[Chronic hemorrhagic pancreatitis in gallbladder polyposis as an initial symptom of metachromatic leukodystrophy].
A 4 1/2 year old boy without previous neurologic disorders developed chronic hemorrhagic pancreatitis and was shown to have polyposis of the gallbladder. Neurologic symptoms emerged at the age of 5 years. The sonographic pattern of an echogenic gallbladder was suspect of metachromatic leukodystrophy. The definitive diagnosis was made by the findings of very low arylsulfatase A activity in the white blood cells and deposits of sulfatides in the stroma of the polyps of the gallbladder.
Deeg
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Reif
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Stehr
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Hümmer
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Harzer
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D002553:Cerebroside-Sulfatase / Q000172:deficiency; D002675:Child, Preschool; D002908:Chronic Disease; D003937:Diagnosis, Differential; D005706:Gallbladder Neoplasms / Q000175:diagnosis*; D006470:Hemorrhage / Q000175:diagnosis; D006801:Humans; D007966:Leukodystrophy, Metachromatic / Q000175:diagnosis*; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis*; D010192:Pancreatic Pseudocyst / Q000175:diagnosis; D010195:Pancreatitis / Q000175:diagnosis*; D011127:Polyps / Q000175:diagnosis*; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography
D002553:Cerebroside-Sulfatase
Chronisch hämorrhagische Pankreatitis bei Gallenblasenpolyposis als Erstsymptom der metachromatischen Leukodystrophie.