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Unknown | 1992-01-14 | 3.8 KB | [NOTE/NoCa] |
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Confidence | Program | Detection | Match Type | Support
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100%
| dexvert
| MPEG ADTS Layer II (audio/mp2)
| magic
| Supported |
100%
| file
| MPEG ADTS, layer II, v1, 44.1 kHz, Stereo
| default
| |
99%
| file
| data
| default
| |
100%
| TrID
| MP3 audio
| default (weak)
| |
100%
| gt2
| MPEG 1 layer 2 Datei
| default (weak)
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id metadata |
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key | value |
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macFileType | [NOTE] |
macFileCreator | [NoCa] |
hex view+--------+-------------------------+-------------------------+--------+--------+
|00000000| ff fd 00 16 00 00 00 7c | 50 6f 6d 70 65 27 73 20 |.......||Pompe's |
|00000010| 64 69 73 65 61 73 65 20 | 69 73 20 61 73 73 6f 63 |disease |is assoc|
|00000020| 69 61 74 65 64 20 77 69 | 74 68 20 61 20 64 65 66 |iated wi|th a def|
|00000030| 65 63 74 20 69 6e 20 74 | 68 65 20 5f 5f 20 65 6e |ect in t|he __ en|
|00000040| 7a 79 6d 65 2e 20 20 54 | 68 69 73 20 69 73 20 74 |zyme. T|his is t|
|00000050| 68 65 20 6f 6e 6c 79 20 | 67 6c 79 63 6f 67 65 6e |he only |glycogen|
|00000060| 20 73 74 6f 72 61 67 65 | 20 64 69 73 65 61 73 65 | storage| disease|
|00000070| 20 74 6f 20 69 6e 76 6f | 6c 76 65 20 74 68 65 20 | to invo|lve the |
|00000080| 43 4e 53 2e 00 00 00 15 | 61 6c 70 68 61 2d 31 2c |CNS.....|alpha-1,|
|00000090| 34 2d 67 6c 75 63 6f 73 | 69 64 61 73 65 00 00 00 |4-glucos|idase...|
|000000a0| 15 6c 79 73 6f 73 6f 6d | 61 6c 20 67 6c 75 63 6f |.lysosom|al gluco|
|000000b0| 73 69 64 61 73 65 00 00 | 00 0f 31 2c 34 2d 67 6c |sidase..|..1,4-gl|
|000000c0| 75 63 6f 73 69 64 61 73 | 65 00 00 00 16 00 01 00 |ucosidas|e.......|
|000000d0| 00 00 00 00 13 00 0e 00 | 02 00 00 00 0e 00 00 00 |........|........|
|000000e0| 00 00 00 00 00 00 00 00 | 00 00 43 54 61 79 2d 53 |........|..CTay-S|
|000000f0| 61 63 68 73 20 64 69 73 | 65 61 73 65 20 69 73 20 |achs dis|ease is |
|00000100| 61 73 73 6f 63 69 61 74 | 65 64 20 77 69 74 68 20 |associat|ed with |
|00000110| 61 20 64 65 66 65 63 74 | 20 69 6e 20 74 68 65 20 |a defect| in the |
|00000120| 5f 5f 20 5f 5f 20 65 6e | 7a 79 6d 65 2e 20 00 00 |__ __ en|zyme. ..|
|00000130| 00 10 68 65 78 6f 73 61 | 6d 69 6e 69 64 61 73 65 |..hexosa|minidase|
|00000140| 20 61 00 00 00 00 00 00 | 00 00 00 00 00 16 00 01 | a......|........|
|00000150| 00 00 00 00 00 13 00 0e | 00 02 00 00 00 0e 00 00 |........|........|
|00000160| 00 00 00 00 00 00 00 00 | 00 00 00 51 4d 65 74 61 |........|...QMeta|
|00000170| 63 68 72 6f 6d 61 74 69 | 63 20 6c 65 75 6b 6f 64 |chromati|c leukod|
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|000001d0| 00 00 00 00 00 00 00 00 | 00 00 00 16 00 01 00 00 |........|........|
|000001e0| 00 00 00 13 00 0e 00 02 | 00 00 00 0e 00 00 00 00 |........|........|
|000001f0| 00 00 00 00 00 00 00 00 | 00 3c 4b 72 61 62 62 65 |........|.<Krabbe|
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|00000220| 6c 61 63 6b 20 6f 66 20 | 74 68 65 20 5f 5f 20 65 |lack of |the __ e|
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|00000250| 00 00 00 00 00 00 00 00 | 00 16 00 01 00 00 00 00 |........|........|
|00000260| 00 13 00 0e 00 02 00 00 | 00 0e 00 00 00 00 00 00 |........|........|
|00000270| 00 00 00 00 00 00 00 3e | 46 61 62 72 79 27 73 20 |.......>|Fabry's |
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|000002f0| 69 64 61 73 65 20 61 00 | 00 00 16 00 01 00 00 00 |idase a.|........|
|00000300| 00 00 13 00 0e 00 02 00 | 00 00 0e 00 00 00 00 00 |........|........|
|00000310| 00 00 00 00 00 00 00 00 | 3d 47 61 75 63 68 65 72 |........|=Gaucher|
|00000320| 27 73 20 64 69 73 65 61 | 73 65 20 69 73 20 61 73 |'s disea|se is as|
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|00000340| 6c 61 63 6b 20 6f 66 20 | 74 68 65 20 5f 5f 20 65 |lack of |the __ e|
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|00000370| 00 00 00 00 00 00 00 16 | 00 01 00 00 00 00 00 13 |........|........|
|00000380| 00 0e 00 02 00 00 00 0e | 00 00 00 00 00 00 00 00 |........|........|
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|000003c0| 61 63 6b 20 6f 66 20 74 | 68 65 20 5f 5f 20 65 6e |ack of t|he __ en|
|000003d0| 7a 79 6d 65 2e 00 00 00 | 10 73 70 68 69 6e 67 6f |zyme....|.sphingo|
|000003e0| 6d 79 65 6c 69 6e 61 73 | 65 00 00 00 00 00 00 00 |myelinas|e.......|
|000003f0| 00 00 00 00 16 00 01 00 | 00 00 00 00 13 00 0e 00 |........|........|
|00000400| 02 00 00 00 0e 00 00 00 | 00 00 00 00 00 00 00 00 |........|........|
|00000410| 00 00 46 49 2d 63 65 6c | 6c 20 64 69 73 65 61 73 |..FI-cel|l diseas|
|00000420| 65 20 28 6d 75 63 6f 6c | 69 70 69 64 6f 73 69 73 |e (mucol|ipidosis|
|00000430| 20 49 49 29 20 69 73 20 | 61 73 73 6f 63 69 61 74 | II) is |associat|
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|00000460| 6e 6f 73 65 20 6b 69 6e | 61 73 65 00 00 00 15 6d |nose kin|ase....m|
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|00000480| 6c 61 73 65 00 00 00 00 | 00 00 00 16 00 01 00 00 |lase....|........|
|00000490| 00 00 00 13 00 0e 00 02 | 00 00 00 0e 00 00 00 00 |........|........|
|000004a0| 00 00 00 00 00 00 00 00 | 00 5f 41 72 65 20 7a 65 |........|._Are ze|
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|000004c0| 6c 6c 61 74 65 64 20 69 | 6e 63 6c 75 73 69 6f 6e |llated i|nclusion|
|000004d0| 20 6c 79 73 6f 7a 79 6d | 65 73 29 20 63 68 61 72 | lysozym|es) char|
|000004e0| 61 63 74 65 72 69 73 74 | 69 63 20 6f 66 20 4e 69 |acterist|ic of Ni|
|000004f0| 65 6d 61 6e 6e 2d 50 69 | 63 6b 20 64 69 73 65 61 |emann-Pi|ck disea|
|00000500| 73 65 3f 20 28 59 2f 4e | 29 00 00 00 01 79 00 00 |se? (Y/N|)....y..|
|00000510| 00 00 00 00 00 00 00 00 | 00 16 00 01 00 00 00 00 |........|........|
|00000520| 00 13 00 0e 00 02 00 00 | 00 0e 00 00 00 00 00 00 |........|........|
|00000530| 00 00 00 00 00 00 00 44 | 49 73 20 4e 69 65 6d 61 |.......D|Is Niema|
|00000540| 6e 6e 2d 50 69 63 6b 20 | 64 69 73 65 61 73 65 20 |nn-Pick |disease |
|00000550| 28 41 55 54 4f 53 4f 4d | 41 4c 2f 20 58 2d 4c 49 |(AUTOSOM|AL/ X-LI|
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|00000570| 20 52 45 43 45 53 53 49 | 56 45 29 2e 00 00 00 13 | RECESSI|VE).....|
|00000580| 61 75 74 6f 73 6f 6d 61 | 6c 20 72 65 63 65 73 73 |autosoma|l recess|
|00000590| 69 76 65 00 00 00 00 00 | 00 00 00 00 00 00 16 00 |ive.....|........|
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|000005b0| 00 00 00 00 00 00 00 00 | 00 00 00 00 88 54 68 65 |........|.....The|
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|000005e0| 48 65 75 6c 65 72 29 2c | 20 4d 50 53 20 49 20 53 |Heuler),| MPS I S|
|000005f0| 20 28 53 63 68 65 69 65 | 29 20 61 6e 64 20 4d 50 | (Scheie|) and MP|
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|00000640| 5f 20 5f 5f 2e 00 00 00 | 20 64 65 72 6d 61 74 61 |_ __....| dermata|
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|00000670| 00 00 00 00 16 00 01 00 | 00 00 00 00 13 00 0e 00 |........|........|
|00000680| 02 00 00 00 0e 00 00 00 | 00 00 00 00 00 00 00 00 |........|........|
|00000690| 00 00 3b 47 69 65 72 6b | 65 27 73 20 64 69 73 65 |..;Gierk|e's dise|
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|00000700| 00 16 00 01 00 00 00 00 | 00 13 00 0e 00 02 00 00 |........|........|
|00000710| 00 0e 00 00 00 00 00 00 | 00 00 00 00 00 00 00 3f |........|.......?|
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|00000750| 20 74 68 65 20 5f 5f 20 | 65 6e 7a 79 6d 65 2e 00 | the __ |enzyme..|
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|00000780| 68 6f 72 79 6c 61 73 65 | 00 00 00 00 00 00 00 16 |horylase|........|
|00000790| 00 01 00 00 00 00 00 13 | 00 0e 00 02 00 00 00 0e |........|........|
|000007a0| 00 00 00 00 00 00 00 00 | 00 00 00 00 00 3f 49 73 |........|.....?Is|
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|000007c0| 20 28 41 55 54 4f 53 4f | 4d 41 4c 2f 20 58 2d 4c | (AUTOSO|MAL/ X-L|
|000007d0| 49 4e 4b 45 44 29 20 28 | 44 4f 4d 49 4e 41 4e 54 |INKED) (|DOMINANT|
|000007e0| 2f 20 52 45 43 45 53 53 | 49 56 45 29 2e 00 00 00 |/ RECESS|IVE)....|
|000007f0| 12 78 2d 6c 69 6e 6b 65 | 64 20 72 65 63 65 73 73 |.x-linke|d recess|
|00000800| 69 76 65 00 00 00 00 00 | 00 00 00 00 00 00 16 00 |ive.....|........|
|00000810| 01 00 00 00 00 00 13 00 | 0e 00 02 00 00 00 0e 00 |........|........|
|00000820| 00 00 00 00 00 00 00 00 | 00 00 00 00 97 5f 5f 5f |........|.....___|
|00000830| 20 64 69 73 65 61 73 65 | 20 69 73 20 74 68 65 20 | disease| is the |
|00000840| 6d 6f 73 74 20 63 6f 6d | 6d 6f 6e 20 73 70 68 69 |most com|mon sphi|
|00000850| 6e 67 6f 6c 69 70 69 64 | 6f 73 69 73 20 61 73 73 |ngolipid|osis ass|
|00000860| 6f 63 69 61 74 65 64 20 | 77 69 74 68 20 61 6e 67 |ociated |with ang|
|00000870| 69 6f 6b 65 72 61 74 6f | 6d 61 2c 20 64 65 66 65 |iokerato|ma, defe|
|00000880| 63 74 20 69 6e 20 61 6c | 70 68 61 2d 67 61 6c 61 |ct in al|pha-gala|
|00000890| 63 74 6f 73 69 64 61 73 | 65 20 41 2c 20 61 6e 64 |ctosidas|e A, and|
|000008a0| 20 61 20 62 75 69 6c 64 | 20 75 70 20 6f 66 20 63 | a build| up of c|
|000008b0| 65 72 61 6d 69 64 65 20 | 74 72 69 68 65 78 6f 73 |eramide |trihexos|
|000008c0| 69 64 65 2e 00 00 00 07 | 46 61 62 72 79 27 73 00 |ide.....|Fabry's.|
|000008d0| 00 00 00 00 00 00 00 00 | 00 00 16 00 01 00 00 00 |........|........|
|000008e0| 00 00 13 00 0e 00 02 00 | 00 00 0e 00 00 00 00 00 |........|........|
|000008f0| 00 00 00 00 00 00 00 01 | 2b 5f 5f 20 64 69 73 65 |........|+__ dise|
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|00000920| 20 74 68 65 20 43 4e 53 | 20 28 65 73 70 65 63 69 | the CNS| (especi|
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|000009d0| 69 6c 64 20 75 70 20 6f | 66 20 6f 6e 69 6f 6e 20 |ild up o|f onion |
|000009e0| 6c 69 6b 65 20 6d 65 6d | 62 72 61 6e 6f 75 73 20 |like mem|branous |
|000009f0| 63 79 74 6f 70 6c 61 73 | 6d 69 63 20 62 6f 64 69 |cytoplas|mic bodi|
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|00000a30| 73 00 00 00 00 00 00 00 | 00 00 00 00 16 00 01 00 |s.......|........|
|00000a40| 00 00 00 00 13 00 0e 00 | 02 00 00 00 0e 00 00 00 |........|........|
|00000a50| 00 00 00 00 00 00 00 00 | 00 00 3f 48 65 75 6c 65 |........|..?Heule|
|00000a60| 72 27 73 20 64 69 73 65 | 61 73 65 20 69 73 20 61 |r's dise|ase is a|
|00000a70| 73 73 6f 63 69 61 74 65 | 64 20 77 69 74 68 20 61 |ssociate|d with a|
|00000a80| 20 64 65 66 65 63 74 20 | 69 6e 20 74 68 65 20 5f | defect |in the _|
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|00000aa0| 75 72 6f 6e 69 64 61 73 | 65 00 00 00 13 61 6c 70 |uronidas|e....alp|
|00000ab0| 68 61 2d 6c 2d 69 64 75 | 72 6f 6e 69 64 61 73 65 |ha-l-idu|ronidase|
|00000ac0| 00 00 00 00 00 00 00 16 | 00 01 00 00 00 00 00 13 |........|........|
|00000ad0| 00 0e 00 02 00 00 00 0e | 00 00 00 00 00 00 00 00 |........|........|
|00000ae0| 00 00 00 00 00 df 5f 5f | 20 64 69 73 65 61 73 65 |......__| disease|
|00000af0| 20 69 73 20 61 73 73 6f | 63 69 61 74 65 64 20 77 | is asso|ciated w|
|00000b00| 69 74 68 20 61 6e 20 65 | 6e 6c 61 72 67 65 64 20 |ith an e|nlarged |
|00000b10| 68 65 61 72 74 20 64 75 | 65 20 74 6f 20 61 63 63 |heart du|e to acc|
|00000b20| 75 6d 75 6c 61 74 69 6f | 6e 73 20 6f 66 20 67 6c |umulatio|ns of gl|
|00000b30| 79 63 6f 67 65 6e 20 69 | 6e 20 74 68 65 20 68 65 |ycogen i|n the he|
|00000b40| 61 72 74 20 64 75 65 20 | 74 6f 20 61 20 64 65 66 |art due |to a def|
|00000b50| 69 63 69 65 6e 63 79 20 | 6f 66 20 61 6c 70 68 61 |iciency |of alpha|
|00000b60| 2d 31 2c 34 2d 67 6c 75 | 63 6f 73 69 64 61 73 65 |-1,4-glu|cosidase|
|00000b70| 2e 20 20 49 74 20 69 73 | 20 61 75 74 6f 73 6f 6d |. It is| autosom|
|00000b80| 61 6c 20 72 65 63 65 73 | 73 69 76 65 2e 20 20 54 |al reces|sive. T|
|00000b90| 68 69 73 20 69 73 20 61 | 73 73 6f 63 69 61 74 65 |his is a|ssociate|
|00000ba0| 64 20 77 69 74 68 20 74 | 68 65 20 65 6e 64 6f 63 |d with t|he endoc|
|00000bb0| 61 72 64 69 61 6c 20 64 | 69 73 65 61 73 65 20 5f |ardial d|isease _|
|00000bc0| 5f 20 5f 5f 2e 00 00 00 | 22 50 6f 6d 70 65 27 73 |_ __....|"Pompe's|
|00000bd0| 20 65 6e 64 6f 63 61 72 | 64 69 61 6c 20 66 69 62 | endocar|dial fib|
|00000be0| 72 6f 65 6c 61 73 74 6f | 73 69 73 00 00 00 00 00 |roelasto|sis.....|
|00000bf0| 00 00 00 00 00 00 16 00 | 01 00 00 00 00 00 13 00 |........|........|
|00000c00| 0e 00 02 00 00 00 0e 00 | 00 00 00 00 00 00 00 00 |........|........|
|00000c10| 00 00 00 00 41 5f 5f 20 | 64 69 73 65 61 73 65 20 |....A__ |disease |
|00000c20| 69 73 20 74 68 65 20 6f | 6e 6c 79 20 67 6c 79 63 |is the o|nly glyc|
|00000c30| 6f 67 65 6e 20 73 74 6f | 72 61 67 65 20 64 69 73 |ogen sto|rage dis|
|00000c40| 65 61 73 65 20 74 68 61 | 74 20 69 73 20 58 2d 6c |ease tha|t is X-l|
|00000c50| 69 6e 6b 65 64 2e 00 00 | 00 07 46 61 62 72 79 27 |inked...|..Fabry'|
|00000c60| 73 00 00 00 00 00 00 00 | 00 00 00 00 16 00 01 00 |s.......|........|
|00000c70| 00 00 00 00 13 00 0e 00 | 02 00 00 00 0e 00 00 00 |........|........|
|00000c80| 00 00 00 00 00 00 00 00 | 00 00 44 5f 5f 5f 20 64 |........|..D___ d|
|00000c90| 69 73 65 61 73 65 20 69 | 73 20 74 68 65 20 6f 6e |isease i|s the on|
|00000ca0| 6c 79 20 67 6c 79 63 6f | 67 65 6e 20 73 74 6f 72 |ly glyco|gen stor|
|00000cb0| 61 67 65 20 64 69 73 65 | 61 73 65 20 74 6f 20 69 |age dise|ase to i|
|00000cc0| 6e 76 6f 6c 76 65 20 74 | 68 65 20 43 4e 53 2e 00 |nvolve t|he CNS..|
|00000cd0| 00 00 07 50 6f 6d 70 65 | 27 73 00 00 00 00 00 00 |...Pompe|'s......|
|00000ce0| 00 00 00 00 00 16 00 01 | 00 00 00 00 00 13 00 0e |........|........|
|00000cf0| 00 02 00 00 00 0e 00 00 | 00 00 00 00 00 00 00 00 |........|........|
|00000d00| 00 00 00 7f 47 6c 79 63 | 6f 67 65 6e 20 73 74 6f |....Glyc|ogen sto|
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|00000d20| 65 20 28 49 2f 20 49 49 | 2f 20 49 49 49 2f 20 49 |e (I/ II|/ III/ I|
|00000d30| 56 2f 20 56 29 20 69 6e | 76 6f 6c 76 65 73 20 61 |V/ V) in|volves a|
|00000d40| 20 64 65 66 69 63 69 65 | 6e 63 79 20 6f 66 20 62 | deficie|ncy of b|
|00000d50| 72 61 6e 63 68 69 6e 67 | 20 65 6e 7a 79 6d 65 20 |ranching| enzyme |
|00000d60| 69 6e 20 6c 69 76 65 72 | 20 61 6e 64 20 65 78 74 |in liver| and ext|
|00000d70| 72 61 2d 68 65 70 61 74 | 69 63 20 74 69 73 73 75 |ra-hepat|ic tissu|
|00000d80| 65 73 2e 00 00 00 02 49 | 56 00 00 00 00 00 00 00 |es.....I|V.......|
|00000d90| 00 00 00 00 16 00 01 00 | 00 00 00 00 13 00 0e 00 |........|........|
|00000da0| 02 00 00 00 0e 00 00 00 | 00 00 00 00 00 00 00 00 |........|........|
|00000db0| 00 00 ac 5f 5f 20 64 69 | 73 65 61 73 65 20 69 73 |...__ di|sease is|
|00000dc0| 20 74 68 65 20 6f 6e 6c | 79 20 6f 6e 65 20 6f 66 | the onl|y one of|
|00000dd0| 20 74 68 65 20 31 32 20 | 67 6c 79 63 6f 67 65 6e | the 12 |glycogen|
|00000de0| 20 73 74 6f 72 61 67 65 | 20 64 69 73 65 61 73 65 | storage| disease|
|00000df0| 73 20 74 68 61 74 20 69 | 73 20 61 73 73 6f 63 69 |s that i|s associ|
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|00000e10| 69 63 61 6e 74 20 6c 61 | 63 74 69 63 20 61 63 69 |icant la|ctic aci|
|00000e20| 64 6f 73 69 73 20 61 6c | 74 68 6f 75 67 68 20 6f |dosis al|though o|
|00000e30| 74 68 65 72 73 20 63 61 | 6e 20 61 6c 73 6f 20 62 |thers ca|n also b|
|00000e40| 65 20 61 73 73 6f 63 69 | 61 74 65 64 20 77 69 74 |e associ|ated wit|
|00000e50| 68 20 68 79 70 6f 67 6c | 79 63 65 6d 69 61 2e 00 |h hypogl|ycemia..|
|00000e60| 00 00 0a 76 6f 6e 20 47 | 69 65 72 6b 65 00 00 00 |...von G|ierke...|
|00000e70| 0c 76 6f 6e 20 47 69 65 | 72 6b 65 27 73 00 00 00 |.von Gie|rke's...|
|00000e80| 06 54 79 70 65 20 49 00 | 00 00 16 00 01 00 00 00 |.Type I.|........|
|00000e90| 00 00 13 00 0e 00 02 00 | 00 00 0e 00 00 00 00 00 |........|........|
|00000ea0| 00 00 00 00 00 61 6c 74 | 68 6f 75 67 68 20 6f 74 |.....alt|hough ot|
|00000eb0| 68 65 72 73 20 63 61 6e | 20 61 6c 73 6f 20 62 65 |hers can| also be|
|00000ec0| 20 61 73 73 6f 63 69 61 | 74 65 64 20 77 69 74 68 | associa|ted with|
|00000ed0| 20 68 79 70 6f 67 6c 79 | 63 65 6d 69 61 2e 00 00 | hypogly|cemia...|
|00000ee0| 00 16 00 01 00 00 00 00 | 00 13 00 0e 00 02 00 00 |........|........|
|00000ef0| 00 0e 00 00 00 00 00 00 | 00 00 00 0a 76 6f 6e 20 |........|....von |
|00000f00| 47 69 65 72 6b 65 00 00 | 00 0c 76 6f 6e 20 47 69 |Gierke..|..von Gi|
|00000f10| 65 72 6b 65 27 73 00 00 | 00 06 54 79 70 65 20 49 |erke's..|..Type I|
+--------+-------------------------+-------------------------+--------+--------+