We experienced a case of chronic fibrosing pancreatitis in an 18/12-year-old girl, which was idiopathic because there were no familial back ground, no cystic fibrosis of pancrease, no ductal anomalies and obstruction. The patient presented intermittent colicky abdominal pain and progressive obstructive jaundice, but T-tube drainage and removal of the lymph nodes around the common bile duct relieved her symptoms and disease process. This seems to be the first case reported in a Korean child. Idiopathic fibrosing pancreatitis should be considered in the differential diagnosis of abdominal pain with obstructive jaundice in children.
Abdominal Pain/complications ; Female ; Fibrosis ; Humans ; Infant ; Jaundice, Chronic Idiopathic/complications ; Pancreatitis/complications/*pathology

OBJECTIVETo explore characteristics of the myelin-like bodies in the hepatocytes of patients with Dubin-Johnson syndrome (DJS) complicated with chronic hepatitis B (CHB).

METHODS11 cases of DJS complicated with CHB and 5 cases DJS without CHB were studied clinicopathologically. The hepatocyte ultrastructure was observed with transmission electron microscope and taken photos. The data were compared and analyzed using Fisher's Exact Test.

RESULTSDeposition of myelin-like bodies can be observed in the hepatocytes of DJS patients with CHB but can not in DJS patients without CHB. The morphology of pigment varys. The electron density and volume of pigment in DJS patients with CHB can be classified into five types: brights (2/11,18.2%), reticulation (1/11, 9.1%), punctiform (6/11, 54.5%), abnormity (1/11, 9.1%) and primary type (1/11, 9.1%). The myelin-like bodies in the hepatocytes of patients with DJS are high density and round with membrance (we named it as primary type) (5/5, 100%).

CONCLUSIONSThe myelin-like bodies in the hepatocytes of DJS patients with CHB possess special pleomorphism and may have important diagnostic value.

Adolescent ; Adult ; Female ; Hepatitis B, Chronic ; complications ; pathology ; Hepatocytes ; chemistry ; pathology ; ultrastructure ; Humans ; Jaundice, Chronic Idiopathic ; complications ; pathology ; Male ; Myelin Sheath ; ultrastructure ; Young Adult

Dubin-Johnson syndrome is a rare clinical entity. It shows intermittent symptoms such as chronic or intermittent jaundice, abdominal pain, weakness, nausea, vomiting, anorexia and diarrhea. Symptoms are precipitated or aggravated by pregnancy, alcoholism, surgical procedures and intercurrent disease. Chronic idiopathic jaundice is typical of Dubin-Johnson syndrome and its prognosis is good. We describe a case of prolonged cholestasis for more than 10 months caused by acute A viral hepatitis in a patient with Dubin-Johnson syndrome. It is a first report of cholestasis complicated by acute A viral hepatitis in a patient with Dubin-Johnson syndrome.
Acute Disease ; Adult ; Bilirubin/blood ; Cholangiopancreatography, Endoscopic Retrograde ; Cholestasis/*diagnosis/etiology ; Hepatitis A/complications/*diagnosis ; Humans ; Jaundice, Chronic Idiopathic/complications/*diagnosis ; Liver/pathology ; Male ; Tomography, X-Ray Computed