Fred C. Rothstein

Caustic Injuries to the Esophagus in Children

The ingestion of caustic chemical agents, usually accidentally by children, produces a wide range of injuries from minor mouth burns to necrosis of the esophagus and stomach. The type of agent, amount, concentration, and duration of exposure are the determining factors. The treatment for the average burn is fairly well standardized, but the serious injuries require prompt recognition of complications and appropriate therapy to prevent more serious complications.

Treatment and Prognosis of Symptomatic Gallbladder Disease in Patients with Cystic Fibrosis

Summary Twenty-four (3.6%) of 670 patients with cystic fibrosis seen over a 25-year period developed symptomatic gallbladder disease. Only four patients were less than 16 years old. Four patients presented with unusual problems, including one with acute cholangitis and two with atonic gallbladder, one of whom required cholecystectomy. Another patient was found to have cholangiocarcinoma of the gallbladder when an exploratory laparotomy was performed to investigate biliary obstruction. Twenty patients had cholelithiasis, 15 of whom underwent cholecystectomy. Only one patient had substantial pulmonary difficulties postoperatively. Patients who presented with classic biliary colic had no further symptoms after cholecystectomy. One patient developed intrahepatic stones 6 years later and required a choledochoduodenostomy. As the pulmonary status of most cystic fibrosis patients will eventually deteriorate, we recommend that serious consideration be given to performing a cholecystectomy as soon as practical after the diagnosis of symptomatic cholelithiasis. Our experience indicates that surgery can be performed safely unless pulmonary status is already extremely compromised and the patient is in overt respiratory failure.

Pharmacokinetic determination of ranitidine pharmacodynamics in pediatric ulcer disease

The pharmacokinetics and pharmacodynamics of ranitidine were evaluated during three methods of administration in 12 children ranging in age from 3.5 to 16 years with documented gastric or duodenal ulcer disease. First, a continuous intravenous infusion of ranitidine was administered to determine the serum concentration necessary to suppress gastric acid secretion by at least 90%. From these data a therapeutic dose of ranitidine was calculated and administered on separate days via the intravenous bolus and oral routes. Half-life, volume of distribution, and clearance values for ranitidine were the same after intravenous bolus and oral doses (1.8 vs 2.0 hours, 2.3 vs 2.5 L/kg, and 794.7 vs 788.0 ml/min/1.73 m2, respectively). The bioavailability of ranitidine given orally averaged 48%. Serum ranitidine concentrations necessary to inhibit gastric acid secretion by at least 90% ranged between 40 and 60 ng/ml for all children studied. No adverse clinical or biochemical effects were observed in any child during the 6 weeks of orally administered treatment. Endoscopic reevaluation after 6 weeks indicated complete healing of initial ulcers.

Lymphonodular hyperplasia of the colon as a pathologic finding in children with lower gastrointestinal bleeding

The serious implication of gastrointestinal blood loss in children is well recognized; however, the significance of lymphonodular hyperplasia of the colon (LNHC) as a pathologic finding in this group of children is unclear. We reviewed the records of 95 children, ages 2-48 months, who were referred to our clinic with a history of hematochezia. Proctosigmoidoscopy was performed on 65 of them. Twenty children (31%) examined proctosigmoidoscopically were found to have LNHC, with no other identified source of bleeding. Clinical histories, endoscopic findings, and colonic biopsy specimens from the LNHC group were examined. Clinical presentation of patients in this group was not distinctive. LNHC was most prominent in the distal colon and rectum and was endoscopically characterized by friability (13/20) and ulceration (3/20). Characteristics of biopsy specimens from children with LNHC were than compared with tissue obtained from an age-matched control population and children with colitis. Biopsy specimens from children with LNHC contained increased numbers of lymphoid follicles and larger follicles than those from the control group. Specimens also contained mucosal inflammation and epithelial thinning and ulceration overlying enlarged follicles. We conclude that LNHC is a frequent proctosigmoidoscopic finding in children evaluated for lower gastrointestinal bleeding. The endoscopic and histologic appearance of these lesions would suggest that LNHC is not a normal finding and represents a potential source of rectal bleeding in children.

Multifocal osteonecrosis in adolescents with Crohn's disease: a complication of therapy?

Three adolescents with severe Crohns ileocolitis developed osteonecrosis after receiving intravenous corticosteroids and parenteral nutrition, including 10% lipid infusion. Each had achieved clinical remission with therapy. However, several months following completion of therapy, each developed arthralgias and arthritis of weight-bearing and non-weight-bearing joints. Roentgenographic evaluation revealed osteonecrosis involving multiple joints. Osteonecrosis may result from the interference with blood flow to bone and has been produced in animals by infusion of lipid solutions and administration of corticosteroids. We report these cases of osteonecrosis as a probable complication of corticosteroid and lipid infusion in adolescents treated for severe Crohns disease.

Herpes esophagitis in the immunocompetent child

tion studies with cells f rom pat ients with different types of N P D may resolve the classification problem? 3 Despite the normal neurologic findings at present in the proband, the ret inal involvement raises concerns about longterm neurologic and intellectual prognosis. However, early presentat ion of organomegaly, macular abnormal i ties, and very low sphingomyel inase levels is not always associated wi th neurologic deter iorat ion in early childhood. These clinical and enzymat ic findings may define a dist inct var ian t of N P D , requiring consideration in prognostic and genetic counseling of families with apparen t NPD-B.

Iron-Deficiency Anemia in Childhood Inflammatory Bowel Disease: Treatment with Intravenous Iron-Dextran

A prospective protocol for iron replacement was used in six children with severe inflammatory bowel disease who had iron-deficiency anemia. All were given a single total-dose infusion of iron-dextran after a taper of the parenteral nutrition solution. Each had hematologic and biochemical correction of their iron-deficiency anemia. As outlined, intravenous iron-dextran appears to be a safe, efficacious, and practical method of iron repletion in children with inflammatory bowel disease who require prolonged parenteral nutrition and bowel rest.

Hereditary pancreatitis and recurrent abdominal pain of childhood

This paper discusses the clinical presentation of hereditary pancreatitis in childhood as exhibited in three cases.

Clinicopathologic studies in childhood pancreatitis

In a review of pediatric autopsies from 1951 to 1985, we identified 40 cases in which pancreatitis was diagnosed pathologically. Twenty-six of these patients were under 4 years of age, and the male-to-female ratio was 1.5. Six groups of patients were identified: 10 with hepatobiliary disease, including 9 with biliary atresia; 7 with immunosuppressive therapy for tumors (n = 2), leukemia (n = 4) and aplastic anemia (n = 1); 6 with viral infections; 8 with congenital anomalies, including congenital heart disease (n = 3); and 9 with miscellaneous problems. Several patients had surgery and various intercurrent complications. Clinical features attributable to the pancreatitis included vomiting or excessive nasogastric drainage (60%), pleural effusions (40%), and abdominal pain (25%). However, the diagnosis was suspected clinically in only 5 of 40 patients. Our findings suggest several pathogenic mechanisms exist for childhood pancreatitis: biliary obstruction, infections, drug toxicity, immunosuppression (acting in synergy with drug toxicity, trauma, and low-flow states resulting from shock, heart failure, and vasculopathy.

High Incidence of Pulmonary Symptoms in Infants Evaluated for Esophageal Disease

Of 62 children (1–24 months of age) evaluated for esophageal disease, 22 were found to have pulmonary symptoms of apnea, pneumonia, wheezing, cyanosis, cough and stridor. Upper gastrointestinal series showed free gastroesophageal reflux in 10 of 22 infants; 3 were thought to have stricture. Acid-reflux test was positive in 13 of 15 and correlated with the presence of esophagitis in 12. In addition to esophagitis, endoscopic examination found two foreign bodies and an esophageal stricture unrecognized during fluoroscopy. Endoscopic grasp biopsy was inadequate in most infants for the histologic evaluation of esophagitis. However, suction biopsy correlated well with endoscopically diagnosed esophagitis. In infants where medical therapy failed and symptoms were life-threatening, a Nissen fundoplication resulted in excellent resolution of symptoms. In children who present with prolonged and often life-threatening symptoms, esophageal dysfunction should be evaluated by rigorous testing.