Growth failure as the first expression of malnutrition in children with human immunodeficiency virus infection.

BACKGROUND: To define the onset, pattern, and earliest manifestations of malnutrition related to HIV infection. METHODS: A retrospective cross-sectional analysis of changes in weight and growth in a group of 54 children with perinatally acquired HIV infection was conducted. Eight children had asymptomatic HIV infection, 26 had symptomatic infection, and 20 had symptomatic infection and were referred for nutritional support. RESULTS: We found an early decline in the rate of linear growth with a relative preservation of the weight-for-age. Weight-for-height measurements were preserved until there was advanced HIV-related disease. CONCLUSIONS: This pattern can result in a false impression of adequate nutrition and emphasizes the importance of longitudinal growth data of the child with HIV infection. Evidence of linear growth failure before clinical wasting is apparent is an absolute indication for aggressive nutritional support.

Langerhans cell histiocytosis of the stomach. A cause of granulomatous gastritis and gastric polyposis.

Langerhans cell histiocytosis (LCH) of the alimentary tract is a rare condition, and LCH of the stomach especially so. We report a histologically and endoscopically unusual case of gastric LCH, the diagnosis of which was based on gastric mucosal biopsy specimens from an adolescent with multi-organ LCH and striking gastric polyposis. Microscopically, the gastric mucosa was expanded by discrete granulomatous microaggregates of Langerhans cells, resulting in a close resemblance to other, more common nonnecrotizing granulomatous gastritides. The diagnosis of LCH was confirmed immunohistochemically and ultrastructurally. To our knowledge, this is the first report of gastric LCH with a granulomatous histologic pattern, and the third to cause diffuse gastric polyposis. Langerhans cell histiocytosis should be included in the histologic differential diagnosis of granulomatous gastritis, as well as in the endoscopic differential diagnosis of gastric polyposis.

Atypical arthropathy associated with Crohn's disease.

Inflammatory bowel disease may be associated with a variety of rheumatologic abnormalities. The patterns of described enteropathic arthritis, not associated with the HLA B27 antigen, include non-deforming peripheral arthritis, bilateral, symmetric sacroiliitis, an on occasion, destructive monoarthritis. We report three patients with Crohn's disease and patterns of joint disease that have not been previously described. The patients ranged in age from 16 to 31 yr. In all cases, both joint and bowel disease were present since childhood. Antinuclear antibody, rheumatoid factor, and HLA B27 antigen determinations were negative. The distribution and pattern of joint disease were similar to that seen in rheumatoid arthritis. We propose that these cases do not represent coincident rheumatoid arthritis and Crohn's disease, but, rather, atypical manifestations of enteropathic arthritis.

Cyclosporine as an alternative to surgery in children with inflammatory bowel disease.

Five children with ulcerative colitis for whom surgery was recommended were treated with cyclosporine. The five had received corticosteroids for 1-24 months. The group included two patients with acute-onset ulcerative colitis and three with acute exacerbations of intractable corticosteroid-dependent chronic ulcerative colitis. The average age at initiation of cyclosporine therapy was 13.8 years (range, 11.5-16); all five patients were boys. Cyclosporine was initiated in the hospital by continuous i.v. infusion. Trough levels of 400-600 ng/dl (measured by radioimmunoassay) were achieved, at which point oral cyclosporine was given and oral dosage was adjusted to similar levels. Significant hypertension requiring medical attention was seen in one patient. Of the two recently diagnosed acute cases, one failed to respond and required subtotal colectomy after 2 weeks of treatment, and the other, despite an initial response, had a subtotal colectomy 10 months later. Of the three corticosteroid-dependent children, none was able to be weaned from corticosteroids and all underwent subtotal colectomy. Our experience emphasizes that the appropriate role of cyclosporine as therapy for children with ulcerative colitis is yet to be determined. Cyclosporine was not effective as an alternative to surgery in our patients.

Helicobacter pylori and gastric lymphonodular hyperplasia in children.

The association of gastric lymphonodular hyperplasia and Helicobacter pylori infection has been reported only in children. Lymphonodular hyperplasia of the stomach is a well known radiographic and endoscopic entity. Over the past three decades, it has been associated with many conditions, ranging from a normal variant to a premalignant lesion. We have recently encountered five children with gastric lymphonodular hyperplasia, all of whom had H. pylori infection of the antrum. The literature regarding this association is reviewed, and a possible explanation for this age-dependent expression of H. pylori infection is offered.

Salmonella typhimurium appendicitis.

A child with signs and symptoms of acute gastroenteritis developed localization of her pain to the right lower quadrant. A clinical diagnosis of appendicitis was made and an inflamed appendix was found at surgery. The postoperative period was marked by high spiking fevers and profuse nonbloody diarrhea. Cultures of the appendix and the stool revealed Salmonella typhimurium. Nontyphoidal Salmonella organisms are a rare cause of acute suppurative appendicitis. Intraoperative cultures of the appendix and peritoneal fluid as well as postoperative cultures of the diarrheal fluid were crucial in elucidating the cause of this patient's unusual course.

Recurrence of autoimmune hepatitis in children after liver transplantation.

BACKGROUND: Liver transplantation is recognized as the appropriate treatment for end-stage liver disease due to chronic active autoimmune hepatitis. While it was initially thought that the disease did not recur after transplant, it is now generally accepted that adult patients may develop recurrent disease, with studies reporting a recurrence rate of < or = 25%. We have noted a higher incidence of recurrent autoimmune hepatitis in our pediatric patients undergoing liver transplant, with a high incidence of associated morbidity. METHODS: We reviewed the records of six children followed up for autoimmune hepatitis who underwent orthotopic liver transplant for complications of end-stage liver disease. RESULTS: Of the six, five developed recurrent autoimmune hepatitis at a mean time of 11.4 months after transplant. The disease was aggressive, leading to cirrhosis and retransplant in three patients, within 1 year of recurrence. A second recurrence of disease occurred in all three retransplanted patients. One patient has received a third liver transplant, one has died, and one patient is asymptomatic on medical therapy. Autoimmune hepatitis recurred in all four patients receiving tacrolimus. CONCLUSION: We conclude that liver transplant for autoimmune hepatitis is likely to be palliative for most pediatric patients. Potent immunosuppressives such as tacrolimus do not protect against the development of recurrent autoimmune hepatitis.

Treatment of intractable gastrointestinal manifestations of chronic granulomatous disease with cyclosporine.

Gastrointestinal manifestations of chronic granulomatous disease of childhood include granulomatous inflammatory bowel disease. Severe colitis and perirectal disease developed in a 12-year-old boy with chronic granulomatous disease while he was receiving interferon gamma therapy. The boy had a deficiency of the 22 kd light chain of the cytochrome b heterodimer. After conventional medical therapy proved to be ineffective, a rapid clinical response was obtained to cyclosporine.