Laparoscopic common duct exploration in a child with recurrent pancreatitis due to a primary fungus ball in the terminal common bile duct.

We report on a case of a fungus ball obstructing the distal common bile duct and causing recurrent pancreatitis in an otherwise healthy 3-year-old boy. Laparoscopic exploration of the common bile duct was performed using a 10 french rigid fiberoptic cystoscope and a Dormia basket. This is the first example of a primary fungus ball in common bile duct presenting as a pancreatitis and treated laparoscopically.

Acid peptic disease in children with type I diabetes mellitus. A complicating relationship.

OBJECTIVE: To determine the prevalence of acid peptic disease among pediatric patients with insulin-dependent diabetes mellitus and to study the effects of acid peptic disease on treatment of insulin-dependent diabetes mellitus. DESIGN: Inception cohort and matched case-control study, with a data set derived from patient and parent interviews and inpatient and outpatient chart reviews. SETTING: LeBonheur Children's Medical Center, a university-affiliated hospital in Memphis, Tenn. PARTICIPANTS: Thirty-one patients with insulin-dependent diabetes mellitus who presented with multiple episodes of symptoms of acid peptic disease were examined by endoscopy. Thirty-one control patients with insulin-dependent diabetes mellitus and transient or no gastrointestinal complaints were matched for age, gender, race, and duration of diabetes. MEASUREMENTS: Glycosylated hemoglobin levels, stature, frequency of hospital admissions, and parental history of acid peptic disease. RESULTS: Acid peptic disease occurred with a prevalence of 7% in our population of pediatric patients with insulin-dependent diabetes mellitus. Major endoscopic findings included gastritis confirmed by upper gastrointestinal biopsy (94%), bile staining of gastric mucosa (58%), and bile pooling (52%). Patients with acid peptic disease demonstrated shorter stature, greater frequency of hospital admissions, and greater prevalence of parental acid peptic disease by history than those of controls. In the 18 months following acid peptic disease therapy, hospital admissions for diabetes-related problems decreased by 50%. CONCLUSIONS: Acid peptic disease is an underrecognized complicating factor in the treatment of diabetes. Clinical suspicion is necessary to offset compromised diabetes control, compliance to treatment regimens, and/or growth expectations associated with this disease.

Partial external diversion of bile for the treatment of intractable pruritus associated with intrahepatic cholestasis.

Partial diversion of bile flow to an external stoma was performed in 6 patients with chronic intrahepatic cholestasis with severe pruritus that had been refractory to medical measures. Four patients with progressive intrahepatic cholestasis and 2 with arteriohepatic dysplasia were treated. Follow-up has been 3-8 yr. Patients with progressive intrahepatic cholestasis have been free of itching since surgery. Serum bile salt concentrations fell from 218-275 microM (normal less than 10) before to less than 10 microM after surgery. Biochemical tests of liver function and histology returned to normal or near normal. Patients with arteriohepatic dysplasia had persistent mild pruritus after surgery. Serum bile salt concentrations fell from 153-317 to 25-37 microM. There was little or no improvement in biochemical tests or histology. Bile volume and bile salt diverted were higher in patients with progressive intrahepatic cholestasis (7.3-13.0 ml/kg.day and 83-137 mumol/kg.day, respectively) than those with arteriohepatic dysplasia (3.2-4.5 ml/kg.day and 21-36 mumol/kg.day). The quality of life since surgery has been excellent in patients with progressive intrahepatic cholestasis, but not as optimal in those with arteriohepatic dysplasia. These findings suggest that partial external biliary diversion can provide effective relief from pruritus and perhaps reversal of liver disease in patients with progressive intrahepatic cholestasis. It should be used in patients with arteriohepatic dysplasia only in those with disabling pruritus.

Eosinophilic gastroenteropathy in childhood.

Eosinophilic gastroenteropathy (EG) is an uncommon, idiopathic disease in children that is characterized by eosinophilic inflammation of the intestine. Over a 7-year period 17 infants and children with EG were evaluated; the retrospective review of their clinical data constitutes the basis of this report. A scale developed for grading the degree of mucosal eosinophilia was used in the evaluation of the intestinal histopathology; all patients had eosinophilic infiltration that was far more severe than that seen in other intestinal disorders of childhood. Peripheral eosinophilia was observed in 12 patients. However, two more had evidence of rapid turnover of eosinophils with significantly increased numbers of precursors in bone marrow and intense intestinal infiltration. Serum IgE correlated with the severity of the disease; extreme elevations were associated with chronic severe illness similar to that commonly reported in adults. Evidence of viral infection in two patients and dietary responsiveness in two infants bring to question the specificity of diagnosis on histology alone. Treatment with oral cromolyn sodium and/or prednisone was effective, but some patients have remained dependent upon therapy.

Anterior sacral defects: an autosomal dominantly inherited condition.

Anterior sacral meningoceles and presacral teratomas are rare congenital malformations associated with a sacrococcygeal bony defect. The inheritance of anterior sacral meningoceles has been proposed to be X-linked dominant, whereas presacral teratomas have been reported to be autosomal dominantly inherited. Anterior meningoceles and teratomas may occur independently or in combination. We report a family in whom at least 11 individuals of three generations have partial sacral agenesis and have had either anterior sacral meningoceles, teratomas, or both. Male-to-male transmission has been documented. Although the existing literature differentiates the inheritance of anterior meningoceles from that of the teratomas, the pleiotropic effects of the gene causing these anterior sacral defects in this family is evident and is consistent with autosomal-dominant inheritance.

The effect of short-term total parenteral nutrition on hepatic function in the human neonate: a prospective randomized study demonstrating alteration of hepatic canalicular function.

Twenty-one infants who were candidates for TPN because of respiratory disease were randomized into experimental (TPN) and control (glucose-electrolyte) groups. Serum GOT, GPT, GGTP, 5' nucleotidase, total, direct, and conjugated (ethyl anthranilate-reactive) bilirubin, and bile salt concentrations were determined at entry into the study and at one week. One week of TPN caused significant elevations of GGTP, 5'-N and EA bilirubin values, whereas SGOT, SGPT, SBS, and total and direct bilirubin were unaffected. Addition of a lipid infusion to TPN did not alter these differences. These data are interpreted as showing: (1) amino acid infusion has an early effect on hepatic function which is independent of the many diseases for which this therapy is used and of the concomitant use of lipid; (2) the initial effect appears to be on the canalicular membrane; and (3) the sinusoidal membrane is apparently unaffected by one week of TPN.