Diagnosis6 Diagnoses B"OK" buttonDoubleClick buttonDoubleClick buttonUp Juvenile-onset diabetes mellitus von Gierke's disease Glycogen storage disease type 1b Pompe's disease Galactosaemia Hereditary fructose intolerance Maple syrup urine disease Lesch-Nyhan syndrome Tay-Sachs' disease Booth-Danks syndrome "Diagnosis" B"OK" buttonUp buttonUp Diagnosis buttonUp Diagnosis Cancel 4lastPage buttonUp buttonUp lastPage Cancel :PHYSSIZE Tay-Sachs' disease This usually presents at 4-6 months of age with progressive neurological symptoms. Delay in psychomotor development, irritability, spasticity, weakness and muscle wasting lead to loss of vision, deafness and decerebrate rigidity. Death occurs usually by three years. It is inherited as an autosomal recessive character and is 100 times commoner in Ashkenazi Jews than in the general population. Due to a deficiency of beta-N-acetyl hexosaminidase. erance test is unnecessary. utropaenia.s inevitable. Diagnosis1 Hereditary fructose intolerance This is due to a deficiency of fructaldolase B and can be demonstrated in liver and intestine biopsy samples. Symptoms of sweating, vomiting and trembling are precipitated by feeding sucrose. There is hypoglycaemia, fructosaemia, fructosuria, hyperuricaemia and aminoaciduria. If the condition is not recognised and exposure to fructose continues, then jaundice, hepatomegaly, oedema, ascites, haemorrhage and eventually death will follow. Many of the liver function tests are abnormal. Dismiss "Diagnosis" keyEnter buttonUp keyChar buttonUp Diagnosis keyChar Diagnosis userAction 4lastPage 4examination, diagnosed,BiopsyTest1,BiopsyTest2 4MB_OK,MB_ICONEXCLAMATION,MB_YESNO,MB_DEFBUTTON2,MB_ICONQUESTION,IDNO MessageBox( "Diagnosis should xdepend" & "solely on guesswork intuition." & "Try examining the patient.", \ "A moment please.",\ -- has a definitive biopsy been done yet? FALSE "You still xhave enough" & "information aa diagnosis." & Iwill be penalised heavily "you aa wrong "Do want d?", \ -- remember where we are so that we can come B"Dismiss" key, isShift, isControl keyEnter B"Cancel" give B"Info" serAction enterPage userAction keyChar userAction Diagnosis should not depend solely on guesswork or intuition. Try examining the patient. A moment please. MessageBox You still do not have enough information to make a diagnosis. You will be penalised heavily if you make a wrong diagnosis. Do you want to continue? A moment please. MessageBox Diagnosis Diagnosis MB_OK MB_ICONEXCLAMATION MB_YESNO MB_DEFBUTTON2 MB_ICONQUESTION examination diagnosed BiopsyTest1 BiopsyTest2 lastPage enterPage Dismiss keyChar buttonUp Dismiss buttonUp Cancel buttonUp isControl isShift Diagnosis7 Diagnoses B"OK" buttonDoubleClick buttonDoubleClick buttonUp Juvenile-onset diabetes mellitus von Gierke's disease Glycogen storage disease type 1b Pompe's disease Galactosaemia Hereditary fructose intolerance Maple syrup urine disease Lesch-Nyhan syndrome Tay-Sachs' disease Booth-Danks syndrome "Diagnosis" B"OK" buttonUp buttonUp Diagnosis buttonUp Diagnosis Cancel 4lastPage buttonUp buttonUp lastPage Cancel :PHYSSIZE Tay-Sachs' disease This usually presents at 4-6 months of age with progressive neurological symptoms. Delay in psychomotor development, irritability, spasticity, weakness and muscle wasting lead to loss of vision, deafness and decerebrate rigidity. Death occurs usually by three years. It is inherited as an autosomal recessive character and is 100 times commoner in Ashkenazi Jews than in the general population. Due to a deficiency of beta-N-acetyl hexosaminidase. erance test is unnecessary. utropaenia.s inevitable. Diagnosis1 Maple syrup urine disease This is due to a deficiency of branched chain alpha-ketoacid decarboxylase. There is hypertonicity alternating with flaccidity, opisthotonus and nystagmus, convulsions and often mental retardation. There are branched chain amino acids and keto acids in the urine. minoaciduria. If the condition is not recognised and exposure to fructose continues, then jaundice, hepatomegaly, oedema, ascites, haemorrhage and eventually death will follow. Many of the liver function tests are abnormal. Dismiss "Diagnosis" keyEnter buttonUp keyChar buttonUp Diagnosis keyChar Diagnosis userAction 4lastPage 4examination, diagnosed,BiopsyTest1,BiopsyTest2 4MB_OK,MB_ICONEXCLAMATION,MB_YESNO,MB_DEFBUTTON2,MB_ICONQUESTION,IDNO MessageBox( "Diagnosis should xdepend" & "solely on guesswork intuition." & "Try examining the patient.", \ "A moment please.",\ -- has a definitive biopsy been done yet? FALSE "You still xhave enough" & "information aa diagnosis." & Iwill be penalised heavily "you aa wrong "Do want d?", \ -- remember where we are so that we can come B"Dismiss" key, isShift, isControl keyEnter B"Cancel" give B"Info" serAction enterPage userAction keyChar userAction Diagnosis should not depend solely on guesswork or intuition. Try examining the patient. A moment please. MessageBox You still do not have enough information to make a diagnosis. You will be penalised heavily if you make a wrong diagnosis. Do you want to continue? A moment please. MessageBox Diagnosis Diagnosis MB_OK MB_ICONEXCLAMATION MB_YESNO MB_DEFBUTTON2 MB_ICONQUESTION examination diagnosed BiopsyTest1 BiopsyTest2 lastPage enterPage Dismiss keyChar buttonUp Dismiss buttonUp Cancel buttonUp isControl isShift Diagnosis1 Diagnosis10 Diagnosis8 Diagnoses B"OK" buttonDoubleClick buttonDoubleClick buttonUp Juvenile-onset diabetes mellitus von Gierke's disease Glycogen storage disease type 1b Pompe's disease Galactosaemia Hereditary fructose intolerance Maple syrup urine disease Lesch-Nyhan syndrome Tay-Sachs' disease Booth-Danks syndrome "Diagnosis" B"OK" buttonUp buttonUp Diagnosis buttonUp Diagnosis Cancel 4lastPage buttonUp buttonUp lastPage Cancel :PHYSSIZE Tay-Sachs' disease This usually presents at 4-6 months of age with progressive neurological symptoms. Delay in psychomotor development, irritability, spasticity, weakness and muscle wasting lead to loss of vision, deafness and decerebrate rigidity. Death occurs usually by three years. It is inherited as an autosomal recessive character and is 100 times commoner in Ashkenazi Jews than in the general population. Due to a deficiency of beta-N-acetyl hexosaminidase. erance test is unnecessary. utropaenia.s inevitable. Diagnosis1 Lesch-Nyhan syndrome This presents in affected males (it is an X-linked disorder) with delayed motor development in the first few months leading to choreoathetosis, spasticity and a bizarre urge to self-mutilation such as biting off finger tips. Due to a deficiency of hypoxanthine-guanine-phosphoribosyl transferase (HGPRT), the striking biochemical feature is a gross hyperuricaemia. The diagnosis may be confirmed by demonstrating the enzyme deficiency in cell extracts. he liver function tests are abnormal. Dismiss "Diagnosis" keyEnter buttonUp keyChar buttonUp Diagnosis keyChar Diagnosis userAction 4lastPage 4examination, diagnosed,BiopsyTest1,BiopsyTest2 4MB_OK,MB_ICONEXCLAMATION,MB_YESNO,MB_DEFBUTTON2,MB_ICONQUESTION,IDNO MessageBox( "Diagnosis should xdepend" & "solely on guesswork intuition." & "Try examining the patient.", \ "A moment please.",\ -- has a definitive biopsy been done yet? FALSE "You still xhave enough" & "information aa diagnosis." & Iwill be penalised heavily "you aa wrong "Do want d?", \ -- remember where we are so that we can come B"Dismiss" key, isShift, isControl keyEnter B"Cancel" give B"Info" serAction enterPage userAction keyChar userAction Diagnosis should not depend solely on guesswork or intuition. Try examining the patient. A moment please. MessageBox You still do not have enough information to make a diagnosis. You will be penalised heavily if you make a wrong diagnosis. Do you want to continue? A moment please. MessageBox Diagnosis Diagnosis MB_OK MB_ICONEXCLAMATION MB_YESNO MB_DEFBUTTON2 MB_ICONQUESTION examination diagnosed BiopsyTest1 BiopsyTest2 lastPage enterPage Dismiss keyChar buttonUp Dismiss buttonUp Cancel buttonUp isControl isShift Diagnosis9 Diagnoses B"OK" buttonDoubleClick buttonDoubleClick buttonUp Juvenile-onset diabetes mellitus von Gierke's disease Glycogen storage disease type 1b Pompe's disease Galactosaemia Hereditary fructose intolerance Maple syrup urine disease Lesch-Nyhan syndrome Tay-Sachs' disease Booth-Danks syndrome "Diagnosis" B"OK" buttonUp buttonUp Diagnosis buttonUp Diagnosis Cancel 4lastPage buttonUp buttonUp lastPage Cancel :PHYSSIZE Tay-Sachs' disease This usually presents at 4-6 months of age with progressive neurological symptoms. Delay in psychomotor development, irritability, spasticity, weakness and muscle wasting lead to loss of vision, deafness and decerebrate rigidity. Death occurs usually by three years. It is inherited as an autosomal recessive character and is 100 times commoner in Ashkenazi Jews than in the general population. Due to a deficiency of beta-N-acetyl hexosaminidase. erance test is unnecessary. utropaenia.s inevitable. Diagnosis1 Tay-Sachs' disease This usually presents at 4-6 months of age with progressive neurological symptoms. Delay in psychomotor development, irritability, spasticity, weakness and muscle wasting lead to loss of vision, deafness and decerebrate rigidity. Death occurs usually by three years. It is inherited as an autosomal recessive character and is 100 times commoner in Ashkenazi Jews than in the general population. Due to a deficiency of beta-N-acetyl hexosaminidase. he liver function tests are abnormal. Dismiss "Diagnosis" keyEnter buttonUp keyChar buttonUp Diagnosis keyChar Diagnosis userAction 4lastPage 4examination, diagnosed,BiopsyTest1,BiopsyTest2 4MB_OK,MB_ICONEXCLAMATION,MB_YESNO,MB_DEFBUTTON2,MB_ICONQUESTION,IDNO MessageBox( "Diagnosis should xdepend" & "solely on guesswork intuition." & "Try examining the patient.", \ "A moment please.",\ -- has a definitive biopsy been done yet? FALSE "You still xhave enough" & "information aa diagnosis." & Iwill be penalised heavily "you aa wrong "Do want d?", \ -- remember where we are so that we can come B"Dismiss" key, isShift, isControl keyEnter B"Cancel" give B"Info" serAction enterPage userAction keyChar userAction Diagnosis should not depend solely on guesswork or intuition. Try examining the patient. A moment please. MessageBox You still do not have enough information to make a diagnosis. You will be penalised heavily if you make a wrong diagnosis. Do you want to continue? A moment please. MessageBox Diagnosis Diagnosis MB_OK MB_ICONEXCLAMATION MB_YESNO MB_DEFBUTTON2 MB_ICONQUESTION examination diagnosed BiopsyTest1 BiopsyTest2 lastPage enterPage Dismiss keyChar buttonUp Dismiss buttonUp Cancel buttonUp isControl isShift Diagnosis10 Diagnoses B"OK" buttonDoubleClick buttonDoubleClick buttonUp Juvenile-onset diabetes mellitus von Gierke's disease Glycogen storage disease type 1b Pompe's disease Galactosaemia Hereditary fructose intolerance Maple syrup urine disease Lesch-Nyhan syndrome Tay-Sachs' disease Booth-Danks syndrome "Diagnosis" B"OK" buttonUp buttonUp Diagnosis buttonUp Diagnosis Cancel 4lastPage buttonUp buttonUp lastPage Cancel :PHYSSIZE Tay-Sachs' disease This usually presents at 4-6 months of age with progressive neurological symptoms. Delay in psychomotor development, irritability, spasticity, weakness and muscle wasting lead to loss of vision, deafness and decerebrate rigidity. Death occurs usually by three years. It is inherited as an autosomal recessive character and is 100 times commoner in Ashkenazi Jews than in the general population. Due to a deficiency of beta-N-acetyl hexosaminidase. erance test is unnecessary. utropaenia.s inevitable. Diagnosis1 Booth-Danks syndrome This is characterized by rectopthmus and a general photophobia in the presence of computer monitors. There is an irrational fear of rodents, most noticably mice. There is often a neurosis connected with particularly obscure metabolic diseases. Carbohydrate metabolism is normal, the glucose tolerance test etc. etc. ...how are you feeling now? kenazi Jews than in the general population. Due to a deficiency of beta-N-acetyl hexosaminidase. he liver function tests are abnormal. Dismiss "Diagnosis" keyEnter buttonUp keyChar buttonUp Diagnosis keyChar Diagnosis userAction 4lastPage 4examination, diagnosed,BiopsyTest1,BiopsyTest2 4MB_OK,MB_ICONEXCLAMATION,MB_YESNO,MB_DEFBUTTON2,MB_ICONQUESTION,IDNO MessageBox( "Diagnosis should xdepend" & "solely on guesswork intuition." & "Try examining the patient.", \ "A moment please.",\ -- has a definitive biopsy been done yet? FALSE "You still xhave enough" & "information aa diagnosis." & Iwill be penalised heavily "you aa wrong "Do want d?", \ -- remember where we are so that we can come B"Dismiss" key, isShift, isControl keyEnter B"Cancel" give B"Info" serAction enterPage userAction keyChar userAction Diagnosis should not depend solely on guesswork or intuition. Try examining the patient. A moment please. MessageBox You still do not have enough information to make a diagnosis. You will be penalised heavily if you make a wrong diagnosis. Do you want to continue? A moment please. MessageBox Diagnosis Diagnosis MB_OK MB_ICONEXCLAMATION MB_YESNO MB_DEFBUTTON2 MB_ICONQUESTION examination diagnosed BiopsyTest1 BiopsyTest2 lastPage enterPage Dismiss keyChar buttonUp Dismiss buttonUp Cancel buttonUp isControl isShift Diagnosis2 Diagnosis6 galactose Diagnosis3 Diagnosis5 lactate Diagnosis7 Diagnosis9 4lastPage, diagnosed, score, TotalPossible 4MB_OK, MB_ICONQUESTION, choice 4UTest1,UTest3,UTest10,UandE, LFT, BloodTest1,BloodTest4 4BiopsyTest1,BiopsyTest2, ProvTest5 -- find out which diagnosis has been selected selectedTextLines "Diagnoses" -- juvenile-onset diabetes -- must have looked )urine glucose, ketones -- urea electrolytes FALSE MessageBox( "You xdone enough" & "tests distinguish "several other possible diagnoses.", \ "Juvenile- Diabetes", \ - 20 - 20 setScore "This cannot be correct ." & "The patient acidosis," & "but no ketosis glycosuria." & st 100 points.", \ - 100 - 100 --von Gierke's disease appropriate biopsy may be a reasonable ," & "glycogen storage type 1B.", \ "Von - 20 - 20 "Yes, well gain 100 + 100 "Diagnosed1" -- GSD 1B ," & e.", \ "Glycogen Storage Disease Type 1B", \ - 20 - 20 ." & samples w" & "levels 6-phosphatase." & lose 100 - 100 - 100 -- Pompe's )blood - 20 - 20 ." & clearly hypoglycaemic" & no cardiac abnormalities." & st 100 - 100 - 100 -- Galactosaemia Zliver function jaundiced?" & "Try .", \ - 20 - 20 ." & "There there "be cataracts since optic" & "fundi could be examined." & st 100 - 100 - 100 -- Fructose intolerance fructose infusion "Did child adverse" & "reaction ?", \ Intolerance", \ - 20 - 20 urate, chromatography aminoaciduria?" & .", \ - 20 - 20 ." & "Although hypoglycaemia "hyperuricaemia, no" & ." & st 100 - 100 - 100 -- Maple syrup -- Must acids "Is evidence "nor ?", \ Syrup Urine ", \ - 20 - 20 ." & no sign ." & st 100 - 100 - 100 -- Lesch-Nyhan syndrome -- need "Is a gross ?", \ Syndrome", \ - 20 - 20 ." & "While %marked %that found e. Other "indicate much more likely st 100 - 100 - 100 -- Tay-Sachs ." & does clinical" & "presentation ." & st 100 - 100 - 100 -- Booth-Danks sysWIndowHandle, \ be joking - we were!" & st 100 - 100 - 100 buttonUp 6buttonUp Diagnoses You have not done enough tests to distinguish it from several other possible diagnoses. Juvenile-onset Diabetes MessageBox tsetScore This cannot be the correct diagnosis. The patient has an acidosis, but no ketosis or glycosuria. You have lost 100 points. Juvenile-onset Diabetes MessageBox tsetScore This may be a reasonable diagnosis, but you have not done enough tests to distinguish it from glycogen storage disease type 1B. Von Gierke's disease MessageBox tsetScore Yes, well done. You gain 100 points. Von Gierke's disease MessageBox tsetScore Diagnosed1 This may be a reasonable diagnosis, but you have not done enough tests to distinguish it from von Gierke's disease. Glycogen Storage Disease Type 1B MessageBox tsetScore This cannot be the correct diagnosis. The biopsy samples do not show normal levels of glucose 6-phosphatase. You lose 100 points. Glycogen Storage Disease Type 1B MessageBox tsetScore You have not done enough tests to distinguish it from several other possible diagnoses. Pompe's Disease MessageBox tsetScore This cannot be the correct diagnosis. The patient is clearly hypoglycaemic and has no cardiac abnormalities. You have lost 100 points. Pompe's Disease MessageBox tsetScore This may be a reasonable diagnosis but is the patient jaundiced? Try the liver function tests. Galactosaemia MessageBox tsetScore This cannot be the correct diagnosis. There is no jaundice and there cannot be any cataracts since the optic fundi could be examined. You have lost 100 points. Galactosaemia MessageBox tsetScore Did the child have an adverse reaction to the fructose infusion test? Fructose Intolerance MessageBox tsetScore This may be a reasonable diagnosis but is there an aminoaciduria? Try chromatography of the urine. Fructose Intolerance MessageBox tsetScore This cannot be the correct diagnosis. Although there is hypoglycaemia and hyperuricaemia, the patient has no aminoaciduria. You have lost 100 points. Fructose Intolerance MessageBox tsetScore Is there any evidence of aminoaciduria nor ketones in the urine? Maple Syrup Urine Disease MessageBox tsetScore This cannot be the correct diagnosis. There is no sign of aminoaciduria or ketones in the urine. You have lost 100 points. Maple Syrup Urine Disease MessageBox tsetScore Is there any evidence of a gross hyperuricaemia? Lesch-Nyhan Syndrome MessageBox tsetScore This cannot be the correct diagnosis. While the patient has a hyperuricaemia, it is not as marked as that found in the Lesch-Nyhan syndrome. Other tests indicate much more likely diagnoses. You have lost 100 points. Lesch-Nyhan Syndrome MessageBox tsetScore This cannot be the correct diagnosis. The patient does not have the clinical presentation of Tay-Sachs disease. Other tests indicate much more likely diagnoses. You have lost 100 points. Tay-Sachs' Disease MessageBox tsetScore You must be joking - we were! You have lost 100 points. Booth-Danks Syndrome MessageBox tsetScore BiopsyTest1 BiopsyTest2 ProvTest5 UTest1 UTest3 UTest10 UandE BloodTest1 BloodTest4 MB_OK MB_ICONQUESTION choice lastPage diagnosed score TotalPossible userAction 4score, ,examination,diagnosed 4MB_YESNO,MB_ICONQUESTION,IDYES MessageBox( "Before doing z" & "wouldn't be a good idea" & take the patient's "A moment please",\ -- penalise ignoring advice! "Details_hidden" "Author" "Reader" "Examination_1" "Examination_done" setup serAction setup userAction keyChar userAction Before doing any examination, wouldn't it be a good idea to take the patient's history? A moment please MessageBox Details_hidden Author Reader Author Reader Examination_1 Examination Examination_done MB_YESNO MB_ICONQUESTION IDYES score history examination diagnosed setup Examination Details_hidden Author Reader Author Reader Examination_1 Details_hidden Examination examination keyChar buttonUp Details_hidden examination glucose lactate 4pack ) > 1 "see_over" "Details" -- keyboard handler graphs -- overrides wresponse arrow 6etc. -- so that user can roll through the "Graph" keyDownArrow \ terPage keyChar enterPage keyDown enterPage see_over see_over keyChar buttonUp Details keyDown buttonUp Graph prightButtonUp Graph (+(+(+(+(+(+(+ Glucose tolerance: hypoglycaemia at beginning and end with glucose concentration rising to abnormally high level. Marked decrease in blood lactate. Glycerol challenge: no change in blood glucose concentration, increase in blood lactate concentration. Glucagon challenge: decrease in blood glucose concentration. Galactose infusion: decrease in blood glucose concentration. Fructose infusion: decrease in blood glucose concentration.. Provocative tests Graph 4pack tgraph there buttonUp rightButtonUp buttonUp rightButtonUp Peter W. Patient: After fasting, glucose (1.75 g/kg body weight) was given orally at time zero. Details "Provocative" buttonUp buttonUp Provocative see_over see over... ology n_done "ECG" keyChar keyChar Nothing abnormal detected. Details "ECG" buttonUp buttonUp Patient: Peter W. glycerol_glucose 4pack ) > 1 "see_over" "Details" -- keyboard handler graphs -- overrides wresponse arrow 6etc. -- so that user can roll through the "Graph" keyDownArrow \ terPage keyChar enterPage keyDown enterPage see_over see_over keyChar buttonUp Details keyDown buttonUp Graph prightButtonUp Graph (+(+(+(+(+(+(+ Glucose tolerance: hypoglycaemia at beginning and end with glucose concentration rising to abnormally high level. Marked decrease in blood lactate. Glycerol challenge: no change in blood glucose concentration, increase in blood lactate concentration. Glucagon challenge: decrease in blood glucose concentration. Galactose infusion: decrease in blood glucose concentration. Fructose infusion: decrease in blood glucose concentration.. Provocative tests Graph 4pack tgraph there buttonUp rightButtonUp buttonUp rightButtonUp Peter W. Patient: After a short fast glycerol was given orally at time zero. uslyyyyyyyylyzero.. Details "Provocative" buttonUp buttonUp Provocative see_over see over... Radio_1 n_done "Radiology" keyChar keyChar Radiology Chest X-ray: Lung fields clear. No cardiomegaly. No radiological evidence of cardiac failure. Abdominal ultrasonogram: Gross hepatomegaly. Both kidneys moderately enlarged. No spenomegaly. Radiology Patient: Peter W. :PHYSSIZE Details "Radiology" buttonUp buttonUp Radiology glycerol_lactate glycerol_lactate 4pack ) > 1 "see_over" "Details" -- keyboard handler graphs -- overrides wresponse arrow 6etc. -- so that user can roll through the "Graph" keyDownArrow \ terPage keyChar enterPage keyDown enterPage see_over see_over keyChar buttonUp Details keyDown buttonUp Graph prightButtonUp Graph (+(+(+(+(+(+(+ Glucose tolerance: hypoglycaemia at beginning and end with glucose concentration rising to abnormally high level. Marked decrease in blood lactate. Glycerol challenge: no change in blood glucose concentration, increase in blood lactate concentration. Glucagon challenge: decrease in blood glucose concentration. Galactose infusion: decrease in blood glucose concentration. Fructose infusion: decrease in blood glucose concentration.. Provocative tests Graph 4pack tgraph there buttonUp rightButtonUp buttonUp rightButtonUp Peter W. Patient: After a short fast glycerol was given orally at time zero. uslyyyyyyyylyzero.. Details "Provocative" buttonUp buttonUp Provocative see_over see over... +8G_ userAction 4score,radiology, ,examination,diagnosed 4MB_OK,MB_ICONEXCLAMATION MessageBox( "Before doing tests" & would be a good idea" & examine the patient.",\ "A moment please",\ e"Radiology" -- increase Honly 5 because hepatomegaly already noted "Details_hidden" "Author" "Reader" "Radio_1" setup serAction setup userAction keyChar userAction Before doing any tests it would be a good idea to examine the patient. A moment please MessageBox Radiology Details_hidden Author Reader Author Reader Radio_1 Radiology MB_OK MB_ICONEXCLAMATION score radiology history examination diagnosed setup Radiology Details_hidden Author Reader Author Reader Radio_1 Details_hidden Radiology radiology keyChar buttonUp Details_hidden radiology glucagon 4pack ) > 1 "see_over" "Details" -- keyboard handler graphs -- overrides wresponse arrow 6etc. -- so that user can roll through the "Graph" keyDownArrow \ terPage keyChar enterPage keyDown enterPage see_over see_over keyChar buttonUp Details keyDown buttonUp Graph prightButtonUp Graph (+(+(+(+(+g Glucose tolerance: hypoglycaemia at beginning and end with glucose concentration rising to abnormally high level. Marked decrease in blood lactate. Glycerol challenge: no change in blood glucose concentration, increase in blood lactate concentration. Glucagon challenge: decrease in blood glucose concentration. Galactose infusion: decrease in blood glucose concentration. Fructose infusion: decrease in blood glucose concentration.. Provocative tests lactate 4pack tgraph there buttonUp rightButtonUp buttonUp rightButtonUp Peter W. Patient: After fasting, glucose (1.75 g/kg body weight) was given orally at time zero. galactose 4pack tgraph there buttonUp rightButtonUp buttonUp rightButtonUp Peter W. Patient: After fasting, galactose (1g/kg body weight) was infused intravenouslylyzero.. Graph 4pack tgraph there buttonUp rightButtonUp buttonUp rightButtonUp Peter W. Patient: After fasting, glucagon (0.5 mg) was administered intramuscularly at time zero. Details "Provocative" buttonUp buttonUp Provocative see_over see over... Radio_1 galactose 4pack ) > 1 "see_over" "Details" -- keyboard handler graphs -- overrides wresponse arrow 6etc. -- so that user can roll through the "Graph" keyDownArrow \ terPage keyChar enterPage keyDown enterPage see_over see_over keyChar buttonUp Details keyDown buttonUp Graph prightButtonUp Graph (+(+(+(+(+(+(+ Glucose tolerance: hypoglycaemia at beginning and end with glucose concentration rising to abnormally high level. Marked decrease in blood lactate. Glycerol challenge: no change in blood glucose concentration, increase in blood lactate concentration. Glucagon challenge: decrease in blood glucose concentration. Galactose infusion: decrease in blood glucose concentration. Fructose infusion: decrease in blood glucose concentration.. Provocative tests Graph 4pack tgraph there buttonUp rightButtonUp buttonUp rightButtonUp Peter W. Patient: After fasting, galactose (1g/kg body weight) was infused intravenouslylyzero.. Details "Provocative" buttonUp buttonUp Provocative see_over see over... userAction 4score,ECG, ,examination,diagnosed 4MB_OK,MB_ICONEXCLAMATION MessageBox( "Before doing tests" & would be a good idea" & examine the patient.",\ "A moment please",\ -- increase H5 because we have already noted -- that there no sign cardiomegaly "Details_hidden" "Author" "Reader" "EC1" "ECG" setup serAction setup userAction keyChar userAction Before doing any tests it would be a good idea to examine the patient. A moment please MessageBox Details_hidden Author Reader Author Reader MB_OK MB_ICONEXCLAMATION score history examination diagnosed setup Details_hidden Author Reader Author Reader Details_hidden keyChar buttonUp Details_hidden fructose 4pack ) > 1 "see_over" "Details" -- keyboard handler graphs -- overrides wresponse arrow 6etc. -- so that user can roll through the "Graph" keyDownArrow \ terPage keyChar enterPage keyDown enterPage see_over see_over keyChar buttonUp Details keyDown buttonUp Graph prightButtonUp Graph (+(+(+(+(+(+(+ Glucose tolerance: hypoglycaemia at beginning and end with glucose concentration rising to abnormally high level. Marked decrease in blood lactate. Glycerol challenge: no change in blood glucose concentration, increase in blood lactate concentration. Glucagon challenge: decrease in blood glucose concentration. Galactose infusion: decrease in blood glucose concentration. Fructose infusion: decrease in blood glucose concentration.. Provocative tests Graph 4pack tgraph there buttonUp rightButtonUp buttonUp rightButtonUp Peter W. Patient: After fasting, fructose (0.5 g/kg body weight) was infused intravenouslyzero.. Details "Provocative" buttonUp buttonUp Provocative see_over see over... userAction 4score,TotalPossible,pack, ,examination, diagnosed 4BiopsyTest1,BiopsyTest2,BiopsyTest3,BiopsyTest4 4ProvocativeTests,BiopsyTests 4MB_OK,MB_YESNO,MB_ICONEXCLAMATION,MB_ICONQUESTION 4MB_ICONHAND,MB_DEFBUTTON2,IDNO MessageBox( "Before doing tests" & would be a good idea" & examine the patient.",\ "A moment please",\ -- don't allow biopsies be done Zenough other have been !(uses function declared ReadyForNastyTests() "These may prove valuable," & "but they j potentially" & "dangerous. unfair try these 4you exhausted "sources information.", \ .", \ , simply unlikely happen since -- too many provocative will kill TRUE < 2) "Are sure that there anything" & gain subjecting a small child" & procedure?", \ .", \ -- Script dialog box. -- The code placed , can be edited. -- Its are attached B"Dialog" -- on However, its destroyed each re-authored, so xused. -- Initialise controls, disable those already Disabling a Hdirectly altering estyle dlgBox property names were editor. init dlgInit FALSE setValue( V, button1, n, button2, , button3, , button4, DisableControl( wait dismissed retValue Bhas pressed, values -- buttons act accordingly getValue( w,OK) -- introduce a conscience! > 0 really going another biopsy?", \ .", \ complications! = 2) "Your had a haemorrhage." & now under Do you" & "want d?", \ .", \ = 3) massive" & , went shock" & died. Not only "lost your " & "also %points.", \ .", \ up results student gets credit liver intestinal allowed wrong immediately E o convince he/she dealing infant rather than a lab + 50 -- deduct unnecessary suffering! - 50 - 50 five lines on card J"Author" J"Reader" mucose + 50 - 50 - 50 three -- skin fibroblasts -- no , so - 50 - 50 -- sternal puncture -- no , so - 50 - 50 e"Biopsies" setup -- -- -- -- userAction setup (userAction Before doing any tests it would be a good idea to examine the patient. A moment please MessageBox ReadyForNastyTests These tests may prove valuable, but they are potentially dangerous. It is unfair to the patient to try these tests before you have exhausted other sources of information. A moment please. MessageBox Biopsy Are you sure that there is anything to gain in subjecting a small child to this procedure? A moment please. MessageBox Dialog dlgInit setValue setValue setValue setValue Dialog dlgBox DisableControl DisableControl DisableControl DisableControl Dialog dlgBox Dialog dlgBox dialog getValue Are you really going to subject this small child to another biopsy? A moment please. MessageBox Your patient has had a haemorrhage. It is now under control. Do you want to continue? A moment please. MessageBox Your patient had a massive haemorrhage, went into shock and died. Not only have you lost your patient, you have also lost all of your points. A moment please. MessageBox getValue Author Reader Author Reader Author Reader Author Reader Author Reader getValue Author Reader Author Reader Author Reader getValue Author Reader Author Reader Author Reader getValue Author Reader Author Reader Author Reader Biopsies Biopsy retValue button4 button3 button2 button1 MB_ICONHAND MB_DEFBUTTON2 MB_OK MB_YESNO MB_ICONEXCLAMATION MB_ICONQUESTION ProvocativeTests BiopsyTests BiopsyTest1 BiopsyTest2 BiopsyTest3 BiopsyTest4 score TotalPossible history examination diagnosed setup Biopsies Biopsies Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader BiopsyTest1 BiopsyTest2 BiopsyTest3 BiopsyTest4 fructose About userAction 4lastPage <> "About" userAction userAction About About lastPage 4diagnosed, lastPage "Admission" "Biopsy" "Diagnosed4" "Diagnosis" "About" "Diagnosed1" uttonUp rightButtonUp buttonUp rightButtonUp Admission Biopsy Diagnosed4 Diagnosis About diagnosed lastPage buttonUp Biopsy Admission Diagnosed1 Diagnosed4 Admission Diagnosis About diagnosed lastPage :PHYSSIZE 4diagnosed, lastPage "Admission" "Biopsy" "Diagnosed4" "Diagnosis" "About" "Diagnosed1" uttonUp rightButtonUp buttonUp rightButtonUp Admission Biopsy Diagnosed4 Diagnosis About diagnosed lastPage buttonUp Biopsy Admission Diagnosed1 Diagnosed4 Admission Diagnosis About diagnosed lastPage Teaching and Learning Technology Programme $ t"T 4diagnosed, lastPage "Admission" "Biopsy" "Diagnosed4" "Diagnosis" "About" "Diagnosed1" uttonUp rightButtonUp buttonUp rightButtonUp Admission Biopsy Diagnosed4 Diagnosis About diagnosed lastPage buttonUp Biopsy Admission Diagnosed1 Diagnosed4 Admission Diagnosis About diagnosed lastPage written by Andrew Booth Department of Biochemistry and Molecular Biology University of Leeds U.K.t userAction 4lastPage, choice 4examination, diagnosed,BiopsyTest1,BiopsyTest2 4MB_OK,MB_ICONEXCLAMATION,MB_YESNO,MB_DEFBUTTON2,MB_ICONQUESTION,IDNO MessageBox( "Diagnosis should xdepend" & "solely on guesswork intuition." & "Try examining the patient.", \ "A moment please.",\ -- has a definitive biopsy been done yet? FALSE "You still xhave enough" & "information aa diagnosis." & Iwill be penalised heavily "you aa wrong "Do want d?", \ -- remember where we are so that we can come selectedTextLines "Diagnoses" key, isShift, isControl keyEnter B"OK" B"Cancel" give B"Info" serAction enterPage userAction keyChar userAction Diagnosis should not depend solely on guesswork or intuition. Try examining the patient. A moment please. MessageBox You still do not have enough information to make a diagnosis. You will be penalised heavily if you make a wrong diagnosis. Do you want to continue? A moment please. MessageBox Diagnosis Diagnoses Diagnosis Diagnosis MB_OK MB_ICONEXCLAMATION MB_YESNO MB_DEFBUTTON2 MB_ICONQUESTION examination diagnosed BiopsyTest1 BiopsyTest2 lastPage choice enterPage Diagnoses Diagnosis choice keyChar buttonUp buttonUp Cancel buttonUp isControl isShift userAction 4score,TotalPossible,pack, ,examination, diagnosed 4ProvTest1,ProvTest2,ProvTest3,ProvTest4,ProvTest5 4ProvocativeTests,BiopsyTests 4MB_OK,MB_YESNO,MB_ICONEXCLAMATION,MB_ICONQUESTION 4MB_ICONHAND,MB_DEFBUTTON2,IDNO MessageBox( "Before doing tests" & would be a good idea" & examine the patient.",\ "A moment please",\ -- don't allow provocative Zenough other have been done (uses function declared ReadyForNastyTests() "These may prove valuable," & "but they involve fasting." & "In your potentially dangerous." & unfair try these 4" & exhausted "sources information.", \ .", \ , simply unlikely happen since -- too many will kill TRUE < 2) "Are you sure that there anything" & gain subjecting a small child" & procedure?", \ .", \ -- Script dialog box. -- The code placed , can be edited. -- Its are attached B"Dialog" -- on However, its destroyed each re-authored, so xused. -- Initialise controls, disable those already Disabling a Hdirectly altering estyle dlgBox property names were editor. init dlgInit FALSE setValue( a, button1, y, button2, , button3, , button4, , button5, DisableControl( wait dismissed retValue Bhas pressed, values -- buttons act accordingly getValue( w,OK) -- introduce complications! = 2) "Your had" & "another convulsion." & want d?", \ .", \ = 3) died. "lost points.", \ 8 .", \ up results -- glucose tolerance + 20 three lines on card J"Author" J"Reader" two graphs ) > 0 ,lactate," & "Details_hidden" -- glycerol challenge + 20 ) > 0 "glycerol_glucose,glycerol_lactate," & -- glucagon + 20 ) > 0 ," & -- galactose infusion + 20 ) > 0 ," & -- fructose + 20 ) > 0 ," & setup -- ) > 0 -- ) > 0 -- -- ) > 0 ," & -- -- ) > 0 ," & -- -- ) > 0 ," & userAction setup &userAction Before doing any tests it would be a good idea to examine the patient. A moment please MessageBox ReadyForNastyTests These tests may prove valuable, but they involve fasting. In your patient's case this is potentially dangerous. It is unfair to the patient to try these tests before you have exhausted other sources of information. A moment please. MessageBox Provocative Are you sure that there is anything to gain in subjecting a small child to this procedure? A moment please. MessageBox Dialog dlgInit setValue setValue setValue setValue setValue Dialog dlgBox DisableControl DisableControl DisableControl DisableControl DisableControl Dialog dlgBox Dialog dlgBox dialog getValue Your patient has had another convulsion. Do you want to continue? A moment please. MessageBox Your patient has had another convulsion and has died. You have lost all of your points. A moment please. MessageBox getValue Author Reader Author Reader Author Reader glucose,lactate, glucose,lactate Details_hidden Provocative getValue Author Reader Author Reader glycerol_glucose,glycerol_lactate, glycerol_glucose,glycerol_lactate Details_hidden Provocative getValue Author Reader glucagon, glucagon Details_hidden Provocative getValue Author Reader galactose, galactose Details_hidden Provocative getValue Author Reader fructose, fructose Details_hidden Provocative ProvocativeTests Provocative retValue button5 button4 button3 button2 button1 MB_ICONHAND MB_DEFBUTTON2 MB_OK MB_YESNO MB_ICONEXCLAMATION MB_ICONQUESTION ProvocativeTests BiopsyTests ProvTest1 ProvTest2 ProvTest3 ProvTest4 ProvTest5 score TotalPossible history examination diagnosed setup Provocative Tests Details_hidden Provocative Tests Author Reader Author Reader Author Reader glucose,lactate, glucose,lactate Details_hidden Author Reader Author Reader glycerol_glucose,glycerol_lactate, glycerol_glucose,glycerol_lactate Details_hidden Provocative Author Reader glucagon, glucagon Details_hidden Provocative Author Reader galactose, galactose Details_hidden Provocative Author Reader fructose, fructose Details_hidden Provocative ProvTest1 ProvTest2 ProvTest3 ProvTest4 ProvTest5 userAction 4score,LFT, ,examination,diagnosed 4MB_OK,MB_ICONEXCLAMATION MessageBox( "Before doing tests" & would be a good idea" & examine the patient.",\ "A moment please",\ e"LiverFunctionTests" -- no -- because irrelevant diagnosis -- but you want give a here e.g. + 10 "Author" "Reader" - see card #3" & "Blood_tests" setup userAction setup userAction Before doing any tests it would be a good idea to examine the patient. A moment please MessageBox LiverFunctionTests Author Reader Liver Function Tests - see card #3 Reader Blood_tests LiverFunction MB_OK MB_ICONEXCLAMATION score history examination diagnosed setup LiverFunctionTests Author Reader Liver Function Tests - see card #3 Reader Blood_tests LiverFunctionTests userAction 4MB_OK,MB_ICONEXCLAMATION 4score, ,examination,diagnosed 4HETest1,HETest2,HETest3,HETest4,HETest5,HETest6 4HETest7,HETest8,HETest9,HETest10,HETest11,HETest12 4HormoneDone MessageBox( "Before doing tests" & would be a good idea" & examine the patient.",\ "A moment please",\ Ihormones enzymes have been done -- no need dialog box - just TRUE HETest13 "HormonesEnzymes" -- Script -- The code placed here so that can be edited. -- Its are attached B"Dialog" -- on However, its destroyed each re-authored, so xused. -- Initialise controls, disable those already Disabling a Hdirectly altering estyle dlgBox property names These were editor. init dlgInit FALSE setValue( , button1, , button2, , button3, , button4, , button5, , button6, , button7, , button8, , button9, , button10, , button11, , button12, DisableControl( wait dismissed retValue -- If Bhas pressed -- find out qstate update fill card appropriately. -- We must a linefeed Author Reader getValue( -- cortisol FirstHDone -- no -- ACTH -- no -- insulin -- glucagon + 10 -- growth -- no -- free thyroxine index -- no -- total -- no -- TSH -- no -- acid phosphatase -- no -- amylase -- no -- creatine kinase -- no -- lactate dehydrogenase -- no - see #4" & "Blood_tests" setup #4" & serAction FirstHDone userAction setup s#userAction Before doing any tests it would be a good idea to examine the patient. A moment please MessageBox HormonesEnzymes Dialog dlgInit setValue setValue setValue setValue setValue setValue setValue setValue setValue setValue setValue setValue Dialog dlgBox DisableControl DisableControl DisableControl DisableControl DisableControl DisableControl DisableControl DisableControl DisableControl DisableControl DisableControl DisableControl Dialog dlgBox Dialog dlgBox dialog getValue getValue FirstHDone Author Reader getValue FirstHDone Author Reader getValue FirstHDone Author Reader getValue FirstHDone Author Reader getValue FirstHDone Author Reader getValue FirstHDone Author Reader getValue FirstHDone Author Reader getValue FirstHDone Author Reader getValue FirstHDone Author Reader getValue FirstHDone Author Reader getValue FirstHDone Author Reader getValue FirstHDone Author Reader Hormones & Enzymes... HormonesEnzymes retValue button12 button11 button10 button9 button8 button7 button6 button5 button4 button3 button2 button1 HETest13 HormoneDone HETest7 HETest8 HETest9 HETest10 HETest11 HETest12 HETest1 HETest2 HETest3 HETest4 HETest5 HETest6 score history examination diagnosed MB_OK MB_ICONEXCLAMATION FirstHDone Hormones & Enzymes - see card #4 Reader Blood_tests HormoneDone setup Hormones & Enzymes... Hormones & Enzymes... Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader Hormones & Enzymes - see card #4 Reader Blood_tests HormoneDone HETest7 HETest8 HETest9 HETest10 HETest11 HETest12 HETest1 HETest2 HETest3 HETest4 HETest5 HETest6 userAction 4MB_OK,MB_ICONEXCLAMATION 4score, ,examination,diagnosed 4UTest1,UTest2,UTest3,UTest4,UTest5,UTest6 4UTest7,UTest8,UTest9,UTest10 MessageBox( "Before doing tests" & would be a good idea" & examine the patient.",\ "A moment please",\ Ihormones enzymes have been done -- no need dialog box - just TRUE "Urinalysis" -- Script -- The code placed here so that can be edited. -- Its are attached B"Dialog" -- on However, its destroyed each re-authored, so xused. -- Initialise controls, disable those already Disabling a Hdirectly altering estyle dlgBox property names These were editor. init dlgInit FALSE setValue( , button1, , button2, , button3, , button4, , button5, , button6, , button7, , button8, , button9, , button10, DisableControl( wait dismissed retValue -- If Bhas pressed -- find out qstate update fill card appropriately. -- We must a linefeed Author Reader getValue( -- glucose + 10 -- protein -- no -- ketones + 10 -- blood -- no -- urine pH -- no colour -- no -- urobilinogen -- no -- HMMA (VMA) -- no -- culture microscopy -- N.B. THIS OPTION HAS THREE LINES OF TEXT -- no -- chromatography + 10 setup userAction setup userAction Before doing any tests it would be a good idea to examine the patient. A moment please MessageBox Urinalysis Dialog dlgInit setValue setValue setValue setValue setValue setValue setValue setValue setValue setValue Dialog dlgBox DisableControl DisableControl DisableControl DisableControl DisableControl DisableControl DisableControl DisableControl DisableControl DisableControl Dialog dlgBox Dialog dlgBox dialog getValue getValue Author Reader getValue Author Reader getValue Author Reader getValue Author Reader getValue Author Reader getValue Author Reader getValue Author Reader getValue Author Reader getValue Author Reader Author Reader Author Reader getValue Author Reader Urinalysis.... Urinalysis retValue button10 button9 button8 button7 button6 button5 button4 button3 button2 button1 UTest7 UTest8 UTest9 UTest10 UTest1 UTest2 UTest3 UTest4 UTest5 UTest6 score history examination diagnosed MB_OK MB_ICONEXCLAMATION .{ {.s .k S/c /[ 30S E 4u< setup Urinalysis... Urinalysis.... Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader UTest7 UTest8 UTest9 UTest10 UTest1 UTest2 UTest3 UTest4 UTest5 UTest6 Diagnosis1 Diagnoses B"OK" buttonDoubleClick buttonDoubleClick buttonUp Juvenile-onset diabetes mellitus von Gierke's disease Glycogen storage disease type 1b Pompe's disease Galactosaemia Hereditary fructose intolerance Maple syrup urine disease Lesch-Nyhan syndrome Tay-Sachs' disease Booth-Danks syndrome "Diagnosis" B"OK" buttonUp buttonUp Diagnosis buttonUp Diagnosis Cancel 4lastPage buttonUp buttonUp lastPage Cancel :PHYSSIZE Tay-Sachs' disease This usually presents at 4-6 months of age with progressive neurological symptoms. Delay in psychomotor development, irritability, spasticity, weakness and muscle wasting lead to loss of vision, deafness and decerebrate rigidity. Death occurs usually by three years. It is inherited as an autosomal recessive character and is 100 times commoner in Ashkenazi Jews than in the general population. Due to a deficiency of beta-N-acetyl hexosaminidase. erance test is unnecessary. utropaenia.s inevitable. Diagnosis1 Juvenile-onset diabetes mellitus This presents over a period of a few weeks with thirst, polyuria and rapid weight loss. There is glycosuria and progressive hyperglycaemia and ketoacidosis. Untreated the condition will rapidly progress with metabolic acidosis, hyperventilation, vomiting, abdominal pain and dehydration leading to circulatory collapse, coma and death. In such cases, diagnosis is made on the basis of unequivocal hyperglycaemia and ketoacidosis. The glucose tolerance test is unnecessary. Dismiss "Diagnosis" keyEnter buttonUp keyChar buttonUp Diagnosis keyChar Diagnosis userAction 4lastPage 4examination, diagnosed,BiopsyTest1,BiopsyTest2 4MB_OK,MB_ICONEXCLAMATION,MB_YESNO,MB_DEFBUTTON2,MB_ICONQUESTION,IDNO MessageBox( "Diagnosis should xdepend" & "solely on guesswork intuition." & "Try examining the patient.", \ "A moment please.",\ -- has a definitive biopsy been done yet? FALSE "You still xhave enough" & "information aa diagnosis." & Iwill be penalised heavily "you aa wrong "Do want d?", \ -- remember where we are so that we can come B"Dismiss" key, isShift, isControl keyEnter B"Cancel" give B"Info" serAction enterPage userAction keyChar userAction Diagnosis should not depend solely on guesswork or intuition. Try examining the patient. A moment please. MessageBox You still do not have enough information to make a diagnosis. You will be penalised heavily if you make a wrong diagnosis. Do you want to continue? A moment please. MessageBox Diagnosis Diagnosis MB_OK MB_ICONEXCLAMATION MB_YESNO MB_DEFBUTTON2 MB_ICONQUESTION examination diagnosed BiopsyTest1 BiopsyTest2 lastPage enterPage Dismiss keyChar buttonUp Dismiss buttonUp Cancel buttonUp isControl isShift userAction 4lastPage 4examination, diagnosed,BiopsyTest1,BiopsyTest2 4MB_OK,MB_ICONEXCLAMATION,MB_YESNO,MB_DEFBUTTON2,MB_ICONQUESTION,IDNO MessageBox( "Diagnosis should xdepend" & "solely on guesswork intuition." & "Try examining the patient.", \ "A moment please.",\ -- has a definitive biopsy been done yet? FALSE "You still xhave enough" & "information aa diagnosis." & Iwill be penalised heavily "you aa wrong "Do want d?", \ -- remember where we are so that we can come B"Dismiss" key, isShift, isControl keyEnter B"Cancel" give B"Info" serAction enterPage userAction keyChar userAction Diagnosis should not depend solely on guesswork or intuition. Try examining the patient. A moment please. MessageBox You still do not have enough information to make a diagnosis. You will be penalised heavily if you make a wrong diagnosis. Do you want to continue? A moment please. MessageBox Diagnosis Diagnosis MB_OK MB_ICONEXCLAMATION MB_YESNO MB_DEFBUTTON2 MB_ICONQUESTION examination diagnosed BiopsyTest1 BiopsyTest2 lastPage enterPage Dismiss keyChar buttonUp Dismiss buttonUp Cancel buttonUp isControl isShift glucose 4pack ) > 1 "see_over" "Details" -- keyboard handler graphs -- overrides wresponse arrow 6etc. -- so that user can roll through the "Graph" keyDownArrow \ terPage keyChar enterPage keyDown enterPage see_over see_over keyChar buttonUp Details keyDown buttonUp Graph prightButtonUp Graph *+*+*+*+*+*+*+ Glucose tolerance: hypoglycaemia at beginning and end with glucose concentration rising to abnormally high level. Marked decrease in blood lactate. Glycerol challenge: no change in blood glucose concentration, increase in blood lactate concentration. Glucagon challenge: decrease in blood glucose concentration. Galactose infusion: decrease in blood glucose concentration. Fructose infusion: decrease in blood glucose concentration.. Provocative tests Graph 4pack tgraph there buttonUp rightButtonUp buttonUp rightButtonUp Peter W. Patient: After fasting, glucose (1.75 g/kg body weight) was given orally at time zero. Details "Provocative" buttonUp buttonUp Provocative see_over see over... Diagnosis3 Diagnoses B"OK" buttonDoubleClick buttonDoubleClick buttonUp Juvenile-onset diabetes mellitus von Gierke's disease Glycogen storage disease type 1b Pompe's disease Galactosaemia Hereditary fructose intolerance Maple syrup urine disease Lesch-Nyhan syndrome Tay-Sachs' disease Booth-Danks syndrome "Diagnosis" B"OK" buttonUp buttonUp Diagnosis buttonUp Diagnosis Cancel 4lastPage buttonUp buttonUp lastPage Cancel :PHYSSIZE Tay-Sachs' disease This usually presents at 4-6 months of age with progressive neurological symptoms. Delay in psychomotor development, irritability, spasticity, weakness and muscle wasting lead to loss of vision, deafness and decerebrate rigidity. Death occurs usually by three years. It is inherited as an autosomal recessive character and is 100 times commoner in Ashkenazi Jews than in the general population. Due to a deficiency of beta-N-acetyl hexosaminidase. erance test is unnecessary. utropaenia.s inevitable. Diagnosis1 Glycogen storage disease type 1b This is clinically very similar to von Gierke's disease (q.v.). Glucose 6-phosphatase is present at normal levels of activity, but it shows abnormal latency, i.e. full activity is only seen after disrupting membrane structures with freezing and thawing or by treatment with detergent. The defect is in the glucose 6-phosphate translocase T1, which transports glucose 6-phosphate from the cytoplasm into the lumen of the endoplasmic reticulum. These patients often have a neutropaenia. Dismiss "Diagnosis" keyEnter buttonUp keyChar buttonUp Diagnosis keyChar Diagnosis userAction 4lastPage 4examination, diagnosed,BiopsyTest1,BiopsyTest2 4MB_OK,MB_ICONEXCLAMATION,MB_YESNO,MB_DEFBUTTON2,MB_ICONQUESTION,IDNO MessageBox( "Diagnosis should xdepend" & "solely on guesswork intuition." & "Try examining the patient.", \ "A moment please.",\ -- has a definitive biopsy been done yet? FALSE "You still xhave enough" & "information aa diagnosis." & Iwill be penalised heavily "you aa wrong "Do want d?", \ -- remember where we are so that we can come B"Dismiss" key, isShift, isControl keyEnter B"Cancel" give B"Info" serAction enterPage userAction keyChar userAction Diagnosis should not depend solely on guesswork or intuition. Try examining the patient. A moment please. MessageBox You still do not have enough information to make a diagnosis. You will be penalised heavily if you make a wrong diagnosis. Do you want to continue? A moment please. MessageBox Diagnosis Diagnosis MB_OK MB_ICONEXCLAMATION MB_YESNO MB_DEFBUTTON2 MB_ICONQUESTION examination diagnosed BiopsyTest1 BiopsyTest2 lastPage enterPage Dismiss keyChar buttonUp Dismiss buttonUp Cancel buttonUp isControl isShift Diagnosis4 Diagnoses B"OK" buttonDoubleClick buttonDoubleClick buttonUp Juvenile-onset diabetes mellitus von Gierke's disease Glycogen storage disease type 1b Pompe's disease Galactosaemia Hereditary fructose intolerance Maple syrup urine disease Lesch-Nyhan syndrome Tay-Sachs' disease Booth-Danks syndrome "Diagnosis" B"OK" buttonUp buttonUp Diagnosis buttonUp Diagnosis Cancel 4lastPage buttonUp buttonUp lastPage Cancel :PHYSSIZE Tay-Sachs' disease This usually presents at 4-6 months of age with progressive neurological symptoms. Delay in psychomotor development, irritability, spasticity, weakness and muscle wasting lead to loss of vision, deafness and decerebrate rigidity. Death occurs usually by three years. It is inherited as an autosomal recessive character and is 100 times commoner in Ashkenazi Jews than in the general population. Due to a deficiency of beta-N-acetyl hexosaminidase. erance test is unnecessary. utropaenia.s inevitable. Diagnosis1 Pompe's disease This is due to an absence of the lysosomal enzyme alpha-1,4-glucosidase which can be demonstrated in cultured fibroblasts. It presents in the first few months of life, with marked hypotonia and cardiomegaly, hepatomegaly not becoming marked until the inevitable heart failure becomes severe. There is accumulation of glycogen, an abnormal response to glucagon but no hypoglycaemia. As yet there is no effective treatment and death is inevitable, usually within the first twelve months. Dismiss "Diagnosis" keyEnter buttonUp keyChar buttonUp Diagnosis keyChar Diagnosis userAction 4lastPage 4examination, diagnosed,BiopsyTest1,BiopsyTest2 4MB_OK,MB_ICONEXCLAMATION,MB_YESNO,MB_DEFBUTTON2,MB_ICONQUESTION,IDNO MessageBox( "Diagnosis should xdepend" & "solely on guesswork intuition." & "Try examining the patient.", \ "A moment please.",\ -- has a definitive biopsy been done yet? FALSE "You still xhave enough" & "information aa diagnosis." & Iwill be penalised heavily "you aa wrong "Do want d?", \ -- remember where we are so that we can come B"Dismiss" key, isShift, isControl keyEnter B"Cancel" give B"Info" serAction enterPage userAction keyChar userAction Diagnosis should not depend solely on guesswork or intuition. Try examining the patient. A moment please. MessageBox You still do not have enough information to make a diagnosis. You will be penalised heavily if you make a wrong diagnosis. Do you want to continue? A moment please. MessageBox Diagnosis Diagnosis MB_OK MB_ICONEXCLAMATION MB_YESNO MB_DEFBUTTON2 MB_ICONQUESTION examination diagnosed BiopsyTest1 BiopsyTest2 lastPage enterPage Dismiss keyChar buttonUp Dismiss buttonUp Cancel buttonUp isControl isShift Diagnosis5 Diagnoses B"OK" buttonDoubleClick buttonDoubleClick buttonUp Juvenile-onset diabetes mellitus von Gierke's disease Glycogen storage disease type 1b Pompe's disease Galactosaemia Hereditary fructose intolerance Maple syrup urine disease Lesch-Nyhan syndrome Tay-Sachs' disease Booth-Danks syndrome "Diagnosis" B"OK" buttonUp buttonUp Diagnosis buttonUp Diagnosis Cancel 4lastPage buttonUp buttonUp lastPage Cancel :PHYSSIZE Tay-Sachs' disease This usually presents at 4-6 months of age with progressive neurological symptoms. Delay in psychomotor development, irritability, spasticity, weakness and muscle wasting lead to loss of vision, deafness and decerebrate rigidity. Death occurs usually by three years. It is inherited as an autosomal recessive character and is 100 times commoner in Ashkenazi Jews than in the general population. Due to a deficiency of beta-N-acetyl hexosaminidase. erance test is unnecessary. utropaenia.s inevitable. Diagnosis1 Galactosaemia This is due to a deficiency of galactose 1-phosphate uridyl transferase which can be detected in cultured fibroblasts. Normal at birth, symtoms start to appear as early as the second week with weight loss, jaundice, vomiting and hepatomegaly. After a few weeks cataracts develop. Unless the condition is quickly recognised death is inevitable and unless treatment (the substitution of a galactose (milk) free diet) is started in the first week of life a degree of mental subnormality appears inevitable. Dismiss "Diagnosis" keyEnter buttonUp keyChar buttonUp Diagnosis keyChar Diagnosis userAction 4lastPage 4examination, diagnosed,BiopsyTest1,BiopsyTest2 4MB_OK,MB_ICONEXCLAMATION,MB_YESNO,MB_DEFBUTTON2,MB_ICONQUESTION,IDNO MessageBox( "Diagnosis should xdepend" & "solely on guesswork intuition." & "Try examining the patient.", \ "A moment please.",\ -- has a definitive biopsy been done yet? FALSE "You still xhave enough" & "information aa diagnosis." & Iwill be penalised heavily "you aa wrong "Do want d?", \ -- remember where we are so that we can come B"Dismiss" key, isShift, isControl keyEnter B"Cancel" give B"Info" serAction enterPage userAction keyChar userAction Diagnosis should not depend solely on guesswork or intuition. Try examining the patient. A moment please. MessageBox You still do not have enough information to make a diagnosis. You will be penalised heavily if you make a wrong diagnosis. Do you want to continue? A moment please. MessageBox Diagnosis Diagnosis MB_OK MB_ICONEXCLAMATION MB_YESNO MB_DEFBUTTON2 MB_ICONQUESTION examination diagnosed BiopsyTest1 BiopsyTest2 lastPage enterPage Dismiss keyChar buttonUp Dismiss buttonUp Cancel buttonUp isControl isShift userAction 4score,UandE, ,examination,diagnosed 4MB_OK,MB_ICONEXCLAMATION MessageBox( "Before doing tests" & would be a good idea" & examine the patient.",\ "A moment please",\ e"Urea&Electrolytes" + 10 "Author" "Reader" "Blood v - see card #2" & "Blood_tests" "UreaElectrolytes" setup userAction setup userAction Before doing any tests it would be a good idea to examine the patient. A moment please MessageBox Urea&Electrolytes Author Reader Blood Urea & Electrolytes - see card #2 Reader Blood_tests UreaElectrolytes MB_OK MB_ICONEXCLAMATION score UandE history examination diagnosed setup Urea&Electrolytes Author Reader Blood Urea & Electrolytes - see card #2 Reader Blood_tests Urea&Electrolytes UandE userAction 4MB_OK,MB_ICONEXCLAMATION 4score, ,examination,diagnosed 4BloodTest1,BloodTest2,BloodTest3,BloodTest4,BloodTest5,BloodTest6,BloodTest7 MessageBox( "Before doing tests" & would be a good idea" & examine the patient.",\ "A moment please",\ Iblood have been done -- no need dialog box - just TRUE "Blood_tests" -- Script 4 Tests -- The code placed here so that can be edited. -- Its are attached B"Dialog" -- on However, its destroyed each re-authored, so xused. -- Initialise controls, disable those already Disabling a Hdirectly altering estyle dlgBox property names These were editor. init dlgInit -- initialise Ibecause they -- might come ctesting authoring version FALSE setValue( , button1, , button2, , button3, , button4, , button5, , button6, , button7, DisableControl( Bloodtest7 wait dismissed retValue -- If Bhas pressed -- find out qstate update fill card appropriately. -- We must a linefeed Author Reader getValue( -- glucose + 10 -- lactate + 10 -- salicylate W + 5 -- urate + 10 -- cholesterol Bloodtest5 -- triglycerides -- free fatty acids setup userAction setup userAction Before doing any tests it would be a good idea to examine the patient. A moment please MessageBox Blood_tests Dialog dlgInit setValue setValue setValue setValue setValue setValue setValue Dialog dlgBox DisableControl DisableControl DisableControl DisableControl DisableControl DisableControl Bloodtest7 DisableControl Dialog dlgBox Dialog dlgBox dialog getValue getValue Author Reader getValue Author Reader getValue Author Reader getValue Author Reader getValue Bloodtest5 Author Reader getValue Author Reader getValue Bloodtest7 Author Reader Blood tests... Blood_tests retValue button7 button6 button5 button4 button3 button2 button1 BloodTest1 BloodTest2 BloodTest3 BloodTest4 BloodTest5 BloodTest6 BloodTest7 score history examination diagnosed MB_OK MB_ICONEXCLAMATION setup Blood tests... Blood tests... Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader Author Reader BloodTest1 BloodTest2 BloodTest3 BloodTest4 BloodTest5 BloodTest6 BloodTest7 Diagnosis j!j!j!j!j!j!j!j!j!j! Diagnoses B"OK" buttonDoubleClick buttonDoubleClick buttonUp Juvenile-onset diabetes mellitus von Gierke's disease Glycogen storage disease type 1b Pompe's disease Galactosaemia Hereditary fructose intolerance Maple syrup urine disease Lesch-Nyhan syndrome Tay-Sachs' disease Booth-Danks syndrome 4choice --find out which diagnosis selected the list box selectedTextLines "Diagnoses" temp "Diagnosis" & buttonUp buttonUp Diagnoses Diagnosis choice Cancel 4lastPage buttonUp buttonUp lastPage Cancel :PHYSSIZE userAction 4score,TotalPossible,EEG, ,examination,diagnosed 4MB_OK,MB_ICONEXCLAMATION,MB_YESNO,MB_ICONQUESTION,MB_DEFBUTTON2,IDNO MessageBox( "Before doing tests" & would be a good idea" & examine the patient.",\ "A moment please",\ "Are you sure that there anything" & gain subjecting a small child" & procedure?",\ -- decrease H10 because unnecessary - 10 "Author" "Reader" "EEG" setup userAction setup userAction Before doing any tests it would be a good idea to examine the patient. A moment please MessageBox Are you sure that there is anything to gain in subjecting a small child to this procedure? A moment please MessageBox Author Reader MB_OK MB_ICONEXCLAMATION MB_YESNO MB_ICONQUESTION MB_DEFBUTTON2 score TotalPossible history examination diagnosed setup Author Reader Biopsy Liver Biopsy: Microscopy: The liver cells are packed with a granular material that stains strongly with periodic acid/Schiff's reagent. Biochemistry: Very low levels of glucose 6-phosphatase. Intestinal Mucosa Biopsy: Microscopy: Nothing abnormal detected. Biochemistry: Very low levels of glucose 6-phosphatase. Culture of Skin Fibroblasts: Microscopy: Nothing abnormal detected. Biochemistry: Nothing abnormal detected. Sternal Puncture: Microscopy: Nothing abnormal detected. Biochemistry: Nothing abnormal detected. Biopsy Results Dialog --Move linkDLL statement handler "tbkdlg.dll" dialog( setValue( getValue( init dlgInit ,"","") function retValue dlgBox ", "") buttonUp buttonUp tbkdlg.dll dialog setValue getValue dlgInit dlgBox dialog retValue dlgInit ,g38, button button1,b5,FALSE button button2,b6,FALSE button button3,b7,FALSE button button4,b8,FALSE button OK,b29,TRUE button Cancel,b30,FALSE dlgBox 524480,7,30,20,172,90,,,Biopsies,8,Helv,,3.85,3.49,108.27,67.57,38,1342177287,128,,0,button1,12.91,12.76,87.70,10.46,5,1342177289,128,&Liver,0,button2,12.91,26.83,91.50,10.46,6,1342177289,128,&Intestinal mucosa,0,button3,12.91,40.90,89.60,10.46,7,1342177289,128,Skin &fibroblasts,0,button4,12.91,54.97,89.60,10.46,8,1342177289,128,&Sternal puncture,0,OK,119.31,18.79,50.90,12.31,29,1342242817,128,OK,0,Cancel,119.31,42.91,50.90,12.31,30,1342242816,128,Cancel,0 ctrlID Dialog userAction 4score,TotalPossible,lumbar, ,examination,diagnosed 4MB_OK,MB_ICONEXCLAMATION,MB_YESNO,MB_ICONQUESTION,MB_DEFBUTTON2,IDNO MessageBox( "Before doing tests" & would be a good idea" & examine the patient.",\ "A moment please",\ "Are you sure that there anything" & gain subjecting a small child" & procedure?",\ e"Lumbar Puncture" -- decrease H20 because unnecessary unpleasant - 20 - 20 "Author" "Reader" setup userAction setup userAction Before doing any tests it would be a good idea to examine the patient. A moment please MessageBox Are you sure that there is anything to gain in subjecting a small child to this procedure? A moment please MessageBox Lumbar Puncture Author Reader Lumbar MB_OK MB_ICONEXCLAMATION MB_YESNO MB_ICONQUESTION MB_DEFBUTTON2 score TotalPossible lumbar history examination diagnosed setup Lumbar Puncture Author Reader Lumbar Puncture lumbar Biopsy glycerol_glucose Examination_done_1 &/(n( 4score, ,examination,UandE,LFT,haematology,radiology 4TotalPossible,ECG,EEG, lumbar 4BloodTest1,BloodTest2,BloodTest3,BloodTest4,BloodTest5,BloodTest6,BloodTest7 4HETest1,HETest2,HETest3,HETest4,HETest5,HETest6 4HETest7,HETest8,HETest9,HETest10,HETest11,HETest12 4UTest1,UTest2,UTest3,UTest4,UTest5,UTest6 4UTest7,UTest8,UTest9,UTest10 4ProvTest1,ProvTest2,ProvTest3,ProvTest4,ProvTest5 4BiopsyTest1,BiopsyTest2,BiopsyTest3,BiopsyTest4 4ProvocativeTests,BiopsyTests 4HormoneDone 4MB_OK,MB_OKCANCEL,MB_YESNO,MB_YESNOCANCEL 4MB_ICONHAND,MB_ICONQUESTION,MB_ICONEXCLAMATION,MB_ICONASTERISK 4MB_DEFBUTTON1,MB_DEFBUTTON2,MB_DEFBUTTON3 4IDOK,IDCANCEL,IDYES,IDNO 4pack, diagnosed,ScoreMessage, lastPage 4firstTime, lastTime 4StaticPages up constants (our version windows.h!) -- Set up a list static . These cannot be reached via Bclick ]the arrow They are -- really different views existing Diagnosis,\ Diagnosis1,\ Diagnosis2,\ Diagnosis3,\ Diagnosis4,\ Diagnosis5,\ Diagnosis6,\ Diagnosis7,\ Diagnosis8,\ Diagnosis9,\ Diagnosis10,\ glucose,\ lactate,\ glycerol_glucose,\ glycerol_lactate,\ glucagon,\ galactose,\ fructose, \ Examination_1,\ Radio_1,\ -- link required DLL's. -- We want Case Notes look like a professional Windows product -- rather than usual mickey mouse ToolBook -- non-standard dialog boxes, enormous buttons linkDLL "tbkdlg.dll" setValue( getValue( 's file handling are -- same -- So 1provided shortcomimg. commdlg. -- no need declare functions filedlg. CommDlgOpenFile( CommDlgSaveFile( routine produces that contain icons titles unlike Box. -- We will user interface asure -- we proper message gMessageBox( -- speed things up a little xkeeping a record -- don't allow screen updates chas been changed there Reader -- initialise probably be your patient! setScore -- The values TRUE FALSE passed routines TBKDLG. must be upper Another foibles. -- these used indicate which -- blood tests have -- hormone enzyme assays -- urinalysis -- provocative Provtest3 -- biopsies _count dangerous so we can convulse kill ^accumulates a p charts -- produced so we jroll -- through them clicks ether a correct -- diagnosis made so constraints be removed remember -- we we try a o, so "Admission" zsetting up depending on 4level "Author" "Details" "EC1" B"Dialog" "Blood_tests" "HormonesEnzymes" "Urinalysis" attached examinations card -- repeat cards extra -- information "Details_hidden" "Examination_done" "Radiology" "ECG" hotwords "Diagnosed1"\ "Diagnosed2"\ "Diagnosed3"\ "Diagnosed4"\ "About"\ selectedTextLines "Diagnoses" menus minimal HCASENOTE.TBK setupMenus runtime sysRuntime reset "History_done" InitialPresentation -- delete custom resetMenus exiting whole -- simply loading another , alter section customise ..." %..." && & presentation always ..." e"&Urea && Electrolytes" e"&Liver Function ..." e"Hae& e"Uri& ..." e"E&CG" e"E&EG" e"Lumbar &Puncture" e"Pro& ..." ..." &problem ..." c"&Help" e"&Hint" e"&Glossary" ..." minimum %..." -- intercept up/down keyDownArrow \ handlers cope fmessages generated NewPatient saved "Do you notes?",\ "A moment please.",\ SaveAs AdmitNewPatient userAction BloodTests UreaElectrolytes LiverFunctionTests Haematology LumbarPuncture Biopsies Theproblemis giveHint "TBOOK.EXE CNGLOSS.TBK" AboutCaseNotes Saveas SaveNotes OpenNotes General purpose xallowed perform Za certain -- carried out. This they ompleted. called ReadyForNastyTests enough student following triglycerides free fatty acids, -- urea & electrolytes, urine ketones a disaster happened ! & \ " out " <=0 ! <=0 "Have thought a career plumbing?" & "If learn mistakes" & may become quite good !" & "Try .", \ sysSuspendMessages does bility grey/ungrey controls response a WM_INITDIALOG -- So Hdirectly manipulating the estyle fields dlgBox property Bcreated editor. 0should be copied modified only work -- BEFORE DisableControl dialogBox, -- find pentry along windowStyle Xwhether already disabled -- Oh Lord, unto us binary logic operators! y134217728) k2 = 1 !WS_DISABLED bit +134217728 -- couldn't BloodTests UreaElectrolytes author LiverFunctionTests reader HormonesEnzymes enterBook Haematology Radiology keyDown Urinalysis setupMenus NewPatient LumbarPuncture ProvocativeTests InitialPresentation Biopsies Theproblemis resetMenus Glossary AboutCaseNotes History Score Examination Saveas leaveBook setScore orNastyTests ReadyForNastyTests DisableControl "enterBook ~ ~!~"~#~$~%~&~'~(~)~*~+~,~-~.~/~0~1~2~3~4~5~6~7~8~9~:~;~<~=~>~?~@~A~B~C~D~E~F~G~H~Iq tbkdlg.dll dialog setValue getValue commdlg.dll filedlg.dll CommDlgOpenFile CommDlgSaveFile MessageBox Reader tsetScore Provtest3 Admission Author Reader Details Examination_1 Details Radio_1 Details Dialog Blood_tests Dialog HormonesEnzymes Dialog Urinalysis Dialog Provocative Dialog Biopsy Author Reader Details_hidden Examination_done Details_hidden Radiology Details_hidden Details_hidden Provocative Dialog Blood_tests Dialog HormonesEnzymes Dialog Urinalysis Dialog Provocative Dialog Biopsy Author Diagnosed1 Reader Diagnosed1 Author Diagnosed2 Reader Diagnosed2 Author Diagnosed3 Reader Diagnosed3 Author Diagnosed4 Reader Diagnosed4 Author About Reader About Diagnoses Diagnosis setupMenus History_done Biopsy Reader setup reader author fInitialPresentation Radio_1 Examination_1 fructose galactose glucagon glycerol_lactate glycerol_glucose lactate glucose Diagnosis10 Diagnosis9 Diagnosis8 Diagnosis7 Diagnosis6 Diagnosis5 Diagnosis4 Diagnosis3 Diagnosis2 Diagnosis1 Diagnosis StaticPages firstTime lastTime diagnosed ScoreMessage lastPage IDCANCEL IDYES MB_DEFBUTTON1 MB_DEFBUTTON2 MB_DEFBUTTON3 MB_ICONHAND MB_ICONQUESTION MB_ICONEXCLAMATION MB_ICONASTERISK MB_OK MB_OKCANCEL MB_YESNO MB_YESNOCANCEL HormoneDone ProvocativeTests BiopsyTests BiopsyTest1 BiopsyTest2 BiopsyTest3 BiopsyTest4 ProvTest1 ProvTest2 ProvTest3 ProvTest4 ProvTest5 UTest7 UTest8 UTest9 UTest10 UTest1 UTest2 UTest3 UTest4 UTest5 UTest6 HETest7 HETest8 HETest9 HETest10 HETest11 HETest12 HETest1 HETest2 HETest3 HETest4 HETest5 HETest6 BloodTest1 BloodTest2 BloodTest3 BloodTest4 BloodTest5 BloodTest6 BloodTest7 TotalPossible lumbar score history examination UandE haematology radiology leaveBook resetMenus lastTime setupMenus &Open... Save &as... History && &Examination &Initial Presentation History & Examination Initial Presentation &History History & Examination &Examination History & Examination &Tests &Blood Tests... Tests &Urea && Electrolytes Tests &Liver Function Tests Tests &Hormones && Enzymes... Tests Hae&matology Tests &Radiology Tests Uri&nalysis... Tests Tests Tests Lumbar &Puncture Tests Pro&vocative Tests... Tests B&iopsies... Tests &Diagnosis The &problem is... Diagnosis &Help &Hint &Score &Glossary &About Case Notes... resetMenus Open... Save as... History & Examination Tests Diagnosis author Author Reader Details Examination_1 Details Radio_1 Details Dialog Blood_tests Dialog HormonesEnzymes Dialog Urinalysis Dialog Provocative Dialog Biopsy reader Author Reader Dialog Blood_tests Dialog HormonesEnzymes Dialog Urinalysis Dialog Provocative Dialog Biopsy keyDown buttonUp Reader prightButtonUp Reader NewPatient Do you want to save your notes? A moment please. MessageBox SaveAs AdmitNewPatient Admission MB_YESNOCANCEL MB_ICONQUESTION IDCANCEL IDYES saved InitialPresentation userAction Admission History userAction History_done tsetScore Examination userAction Examination_done tsetScore BloodTests userAction Blood_tests tsetScore UreaElectrolytes userAction UreaElectrolytes tsetScore LiverFunctionTests userAction LiverFunction tsetScore HormonesEnzymes userAction HormonesEnzymes tsetScore Haematology userAction Haematology tsetScore Radiology userAction Radiology tsetScore Urinalysis userAction Urinalysis tsetScore userAction tsetScore userAction tsetScore LumbarPuncture userAction Lumbar tsetScore ProvocativeTests userAction Provocative tsetScore Biopsies userAction Biopsy tsetScore Theproblemis userAction Diagnosis kgiveHint Admission tsetScore Glossary TBOOK.EXE CNGLOSS.TBK AboutCaseNotes userAction About Score Score MessageBox ScoreMessage MB_OK Saveas SaveNotes Admission OpenNotes Admission Do you want to save your notes? A moment please. MessageBox SaveAs lastTime MB_YESNOCANCEL MB_ICONQUESTION IDCANCEL IDYES saved ReadyForNastyTests UandE UTest1 UTest3 diagnosed BloodTest1 BloodTest2 BloodTest4 BloodTest6 BloodTest7 setScore Score is out of Have you thought of a career in plumbing? If you can learn by your mistakes you may become quite good at this! Try again. A moment please. MessageBox ScoreMessage MB_OK MB_ICONEXCLAMATION diagnosed score TotalPossible DisableControl windowStyle control dialogBox Examination_1 "Examination_done" keyChar keyChar Examination_done On examination the child is apyrexial, pale, clammy and irritable with a pulse rate of 110 per minute. There is no jaundice or anaemia clinically and the mucous membranes are a good colour with no sign of central cyanosis. He is rather small for his age (height below the third percentile), with poor developmental milestones, but does indeed have a very chubby appearance with a very round face. The throat and both tympanic membranes are of normal appearance. On abdominal examination there is gross hepatomegaly, the liver extending three finger breadths below the costal margin. There are no abnormal findings in the respiratory or cardiovascular systems with no evidence of heart failure and neurological examination is normal. Examination of the optic fundi was not easy, but there are no gross abnormalities. Examination Examination_details :PHYSSIZE Note the chubby face and distended abdomen Patient: Peter W. Details "Examination_done" buttonUp buttonUp Examination_done Diagnosis2 Diagnoses B"OK" buttonDoubleClick buttonDoubleClick buttonUp Juvenile-onset diabetes mellitus von Gierke's disease Glycogen storage disease type 1b Pompe's disease Galactosaemia Hereditary fructose intolerance Maple syrup urine disease Lesch-Nyhan syndrome Tay-Sachs' disease Booth-Danks syndrome "Diagnosis" B"OK" buttonUp buttonUp Diagnosis buttonUp Diagnosis Cancel 4lastPage buttonUp buttonUp lastPage Cancel :PHYSSIZE Tay-Sachs' disease This usually presents at 4-6 months of age with progressive neurological symptoms. Delay in psychomotor development, irritability, spasticity, weakness and muscle wasting lead to loss of vision, deafness and decerebrate rigidity. Death occurs usually by three years. It is inherited as an autosomal recessive character and is 100 times commoner in Ashkenazi Jews than in the general population. Due to a deficiency of beta-N-acetyl hexosaminidase. erance test is unnecessary. utropaenia.s inevitable. Diagnosis1 von Gierke's disease This is glycogen storage disease type 1a. It is characterized by an absence of glucose 6-phosphatase in biopsies of liver, intestine or in platelets. There is hepatomegaly, hyperuricaemia, hypoglycaemia and hyperlipidaemia, but no aminoaciduria or ketosis. The glucose tolerance test shows an abnormal rise in blood glucose concentration with hypoglycaemia at the beginning and end. There is an abnormal response to glucagon with an increase in blood lactate but not glucose. Dismiss "Diagnosis" keyEnter buttonUp keyChar buttonUp Diagnosis keyChar Diagnosis Diagnosed1 Diagnosis: von Gierke's Disease (Glycogen Storage Disease Type 1a). Treatment: The main problem with these patients is the fasting hypoglycaemia which may cause convulsions. This can be controlled by frequent feeding day and night using a nasogastric tube if necessary. These patients often present in their teens with gout and this can be controlled with allopurinol. There is often a platelet dysfunction that can cause bleeding disorders. see over... Diagnosis Diagnosed1 Diagnosis4 Diagnosed2 You should have noticed the following about this patient: The fasting blood glucose concentration was low, while the concentrations of lactate, urate, cholesterol, triglycerides and fatty acids were all raised. There was no glucose in the urine nor were there any ketones. (The early report of ketones in the urine of patients with von Gierke's disease is now know to be incorrect.) The urea and electrolytes showed an acidosis with decreased bicarbonate and increased anion gap. Chromatography of the urine showed no hyperaminoaciduria. The provocative tests all gave abnormal results, although you will not have been able to do all of them without harming the patient in this case. The definitive test is, of course, the liver biopsy but in view of the risk and discomfort, it should only be done when other tests indicate that it would be worthwhile. see over... Further comments Diagnosed3 The haematology report showed that although the platelet count was normal, the bleeding time was longer than usual. Radiology simply confirmed the hepatomegaly. ECG showed that there was no cardiac problem. EEG and lumbar puncture added nothing and simply wasted resources. You can now go back and do any of the tests that you missed. Before you finish, answer these questions; 1 - Why does a build-up of glucose 6-phosphate lead to the deposition of large quantities of glycogen. 2 - Apart from hydrolysis to glucose, what are the other metabolic fates of glucose 6-phosphate? 3 - Explain why each of the provocative tests gave abnormal results. see over... Further comments Diagnosed4 4 - The active site of glucose 6-phosphatase is located on the non-cytoplasmic side of the endoplasmic reticulum membrane. How does glucose 6-phosphate reach the active site from the cytoplasm and how are the products returned. 5 - What would be the consequence of the failure of any of the components of the system that you describe in answer to 4? Further comments HormonesEnzymes Cortisol (08.00-10.00am) 1000 nmol/L (normal 110 - 1076) ACTH (08.00-10.00am) 60 ng/L (normal < 80) Insulin 4.3 mU/L (normal 4 - 30) Glucagon 35 pmol/L (normal < 50) Growth Hormone 1 g/L (normal < 2) Free Thyroxine Index 3.2 (normal 1.3 - 5.1) Total Thyroxine (T4) 115 nmol/L (normal 90 - 195) TSH 4.0 mU/L (normal < 4.5) Acid Phosphatase 7 IU/L (normal 6.2 - 10.6) Amylase 154 IU/L (normal 98 - 405) Creatine Kinase 35 IU/L (normal 4 - 60) Lactate Dehydrogenase 225 IU/L (normal 120 - 280) Chemical Pathology 444 Dialog --Move linkDLL statement handler "tbkdlg.dll" dialog( setValue( getValue( init dlgInit ,"","") function retValue dlgBox ", "") buttonUp buttonUp tbkdlg.dll dialog setValue getValue dlgInit dlgBox dialog retValue dlgInit ,g38, button button1,b5,FALSE button button2,b6,FALSE button button3,b7,FALSE button button4,b8,FALSE button button5,b9,FALSE button button6,b10,FALSE button button7,b11,FALSE button button8,b12,FALSE button button9,b13,FALSE button button10,b21,FALSE button button11,b22,FALSE button button12,b23,FALSE button OK,b29,TRUE button Cancel,b30,FALSE dlgBox 524480,15,30,20,171,202,,,Hormones and Enzymes,8,Helv,,3.54,4.92,108.30,180.92,38,1342177287,128,,0,button1,13.07,15.30,87.73,10.46,5,1342177289,128,&Cortisol,0,button2,13.07,29.37,91.47,10.46,6,1342177289,128,&ACTH,0,button3,13.07,43.45,89.60,10.46,7,1342177289,128,&Insulin,0,button4,13.07,57.52,89.60,10.46,8,1342177289,128,&Glucagon,0,button5,13.07,71.59,89.60,10.46,9,1342177289,128,Gr&owth hormone,0,button6,13.07,85.66,91.47,10.46,10,1342177289,128,&Free Thyroxine Index,0,button7,12.91,99.04,93.33,10.46,11,1342177289,128,&Total Thyroxine (T4),0,button8,12.91,113.11,91.47,10.46,12,1342177289,128,T&SH,0,button9,13.07,127.88,93.33,10.46,13,1342177289,128,Acid &Phosphatase,0,button10,13.07,141.95,91.47,10.46,21,1342177289,128,A&mylase,0,button11,13.07,156.02,91.47,10.46,22,1342177289,128,C&reatine Kinase,0,button12,13.07,170.09,97.07,10.46,23,1342177289,128,&Lactate dehydrogenase,0,OK,117.60,57.52,50.86,12.31,29,1342242817,128,OK,0,Cancel,117.60,85.66,50.86,12.31,30,1342242816,128,Cancel,0 ctrlID Dialog LiverFunction Liver Function Tests: Alkaline phosphatase (total) 112 IU/L (normal 71 - 212) GOT (AST) 12 IU/L (normal 6 - 17) Total bilirubin 9 mol/L (normal 2 - 14) Total protein 65 g/L (normal 56 - 72) Albumin 39 g/L (normal 33 - 44) Globulins 25 g/L (normal 23 - 28) Calcium 2.50 mmol/L (normal 2.25 - 2.60) Standard calcium 2.35 mmol/L (normal 2.13 - 2.43) Phosphorus 1.72 mmol/L (normal 1.2 - 2.1)) Chemical Pathology 3 userAction "Admission" handler gives the hints according -- which test needs be done -- you will work out your own algorithm - remember declare _used! giveHint 4diagnosed,History,Examination 4BloodTest1,BloodTest2,BloodTest4,BloodTest7 4UTest1,UTest3,UTest10,UandE 4ProvTest1,ProvTest2,ProvTest3,ProvTest4,ProvTest5 4BiopsyTest1,BiopsyTest2 4MB_OK,score,TotalPossible -- already MessageBox( "Why ?" & "You have correct diagnosis." & "Try another patient.", \ xtaken "The fact that symptoms relieved" & Hfeeding highly significant." & taking .", \ - 10 - 10 examination convulsion ." & examining - 10 - 10 FALSE -- blood glucose "Could convulsions be due hypoglycaemia?", \ - 10; - 10 -- urine sysWIndowHandle, \ "Is there 8?", \ - 10 - 10 ketones chromatography "Does child a ketosis?", \ - 10 - 10 -- urea electrolytes acidosis?", \ - 10 - 10 urate elevated" & uric concentration?", \ - 10 - 10 lactate clearance Hthe" & "kidneys inhibited P.", \ - 10 - 10 free fatty acids low," & "could be mobilizing R?", \ - 10 - 10 -- no provocative tests (.", \ - 10 - 10 -- no meaningful biopsy "By now idea what" & wrong. clinch ," & "but worth ?", \ - 10 - 10 - clues been "Time !", \ - 10 - 10 -- Switch AdmitNewPatient Zfilename,filter,title , Files (*.CBK)|*.cbk|" -- call COMMDLG.DLL routine via FILEDLG. requested full path CommDlgOpenFile( game! SaveNotes ,LFT,haematology,radiology ,ECG,EEG, lumbar t2,BloodTest3, t5,BloodTest6, 4HETest1,HETest2,HETest3,HETest4,HETest5,HETest6 4HETest7,HETest8,HETest9,HETest10,HETest11,HETest12 4,UTest5,UTest6 4UTest7,UTest8,UTest9, iopsyTest3,BiopsyTest4 4ProvocativeTests,BiopsyTests 4HormoneDone, saved MB_ICONEXCLAMATION, IDCANCEL le,doctor (*.NOT)|*. CommDlgSaveFile( ("Please record f"A. Doctor" unidentified "d M y )hh24: r<> "" "Cannot write jLFT& jECG& jEEG& [(BloodTest5)& [(UTest2)& [(UTest4)& [(BiopsyTest3)& previously- details OpenNotes , fileCaption,fileDate r<> "" "These notes "Administrative Error",\ on " & H" & &"." & "Score so far " & & " &".",\ reset cards "History_done" "Reader" setup veHint userAction AdmitNewPatient SaveNotes giveHint OpenNotes userAction Admission giveHint Why do you need a hint? You have the correct diagnosis. Try another patient. MessageBox The fact that the symptoms are relieved by feeding is highly significant. Try taking the patient's history. MessageBox The convulsion is highly significant. Try examining the patient. MessageBox Could the convulsions be due to hypoglycaemia? MessageBox Is there glucose in the urine? MessageBox Does the child have a ketosis? MessageBox Does the child have an acidosis? MessageBox Does the child have an elevated blood uric acid concentration? MessageBox The clearance of uric acid by the kidneys is inhibited by lactate. MessageBox If the blood glucose concentration is low, could the child be mobilizing fatty acids? MessageBox Try the provocative tests. MessageBox By now you may have an idea of what is wrong. A biopsy could clinch it, but is it worth it? MessageBox Time for a diagnosis! MessageBox MB_OK score TotalPossible BiopsyTest1 BiopsyTest2 ProvTest1 ProvTest2 ProvTest3 ProvTest4 ProvTest5 UTest1 UTest3 UTest10 UandE BloodTest1 BloodTest2 BloodTest4 BloodTest7 diagnosed History Examination AdmitNewPatient Patient Files (*.CBK)|*.cbk| New Patient CommDlgOpenFile filename filter title SaveNotes ~ ~!~"~#~$~%~&~'~(~)~*~+~,~-~.~/~0~1~2~3~4~5~6~7~8?9?:?;?o(&> "Helv "Helv glucos F>#H> #f O( "Helv GDHf/ "Helv GDHf/ "Helv GDHf/ "Helv "Helv "Helv blood "Helv (mM) "Helv hours "Helv patien "Helv normal "Helv G0Hf/ glucos "Helv Glycer ol challen ge test "Helv "Helv BMP/ "Helv "Helv "Helv "Helv "Helv "Helv blood "Helv (mM) "Helv hours "Helv patien "Helv normal "Helv lactat "Helv Glycer ol challen ge test "Helv "Helv "Helv "Helv "Helv "Helv "Helv "Helv "Helv "Helv "Helv "Helv blood "Helv lactat "Helv (mM)S "Helv hoursA "Helv patien "Helv normal "Helv Glucos e toleranc e test "Helv bloodZ "Helv (mM)R "Helv patien "Helv normal "Helv glucos "Helv minute "Helv Galact ose infusi on testI@ "Helv "Helv "Helv "Helv "Helv "Helv "Helv infusi "Helv "Helv "Helv "Helv bloodC "Helv (mM)E "Helv hours "Helv patien "Helv normal "Helv glucos "Helv Glucag on challen ge test "Helv "Helv "Helv "Helv bloodZ "Helv (mM)R "Helv patien "Helv normal "Helv glucos "Helv minute "Helv Galact ose infusi on testI@ "Helv "Helv "Helv "Helv "Helv "Helv "Helv infusi "Helv bloodZ "Helv (mM)R "Helv patien "Helv normal "Helv glucos "Helv minute "Helv "Helv "Helv "Helv "Helv "Helv "Helv infusi "Helv Fructo se infusio n test@ ($($1f