Intolerance to protein hydrolysate infant formulas: an underrecognized cause of gastrointestinal symptoms in infants.

The purpose of this study was to determine the effectiveness of an amino acid-based infant formula in infants with continued symptoms suggestive of formula protein intolerance while they were receiving casein hydrolysate formula (CHF). Twenty-eight infants, 22 to 173 days of age, were enrolled; each had received CHF for an average of 40 days (10 to 173 days) and continued to have bloody stools, vomiting, diarrhea, irritability, or failure to gain weight, or a combination of these symptoms. Sigmoidoscopy with rectal biopsy was performed in all infants. The infants then received an amino acid-based infant formula, Neocate, for 2 weeks. After 2 weeks of treatment, 25 of the infants demonstrated resolution of their symptoms and underwent challenge with CHF. Of the 25 who were challenged, eight tolerated the CHF and the remainder had recurrence of their symptoms. The histologic features in these infants varied from eosinophilic infiltration to normal. We conclude that not all infants with apparent formula protein-induced colitis respond to CHF and that these infants may have resolution of their symptoms when fed an amino acid-based infant formula.

Behavioral risk indicators of attachment loss in adult Floridians.

This study investigated behavioral and sociodemographic risk indicators of attachment loss (AL) at baseline in subjects of the Florida Dental Care Study: 873 subjects with at least 1 tooth, and who were 45+ years or older, participated for an in-person interview and dental examination; 761 subjects were probed for AL. Calibrated examiners used a modified NIDR protocol from the 1985-86 Survey of US Employed Adults and Seniors. Results were weighted to reflect actual population proportions. 92% of subjects had at least 1 site of 4+ mm AL, and 35% had at least 1 severe site (7+ mm AL). In a single multivariate regression, not having a recent dental check-up, not using dental floss, being a current smoker, and being diabetic were significantly associated with a higher probability of having 1 or more severe sites. Blacks were less likely than whites to be regular users of dental care, use dental floss, and be non-smokers. Similar findings were found for low income adults and rural residents. Risk groups (low income, blacks, rural residents) were more likely to present with modifiable risk indicators for AL, suggesting the need for targeted interventions.

Influenza vaccination following liver transplantation in children.

Our objective was to determine the immunologic response to two influenza vaccine doses in 39 children who had undergone liver transplantation. Patients received two doses of trivalent inactivated influenza vaccine 4 weeks apart. Sera were collected 4 weeks after each dose and analyzed by a hemagglutination inhibition assay (HAI) for evidence of antibody response to the antigens A/Taiwan/1/86 (H1N1), A/Beijing/32/92 (H3N2), and B/Panama/45/95. Patients with HAI titers of 1:40 or greater were considered to have protective titers. Twenty-six (67%) patients showed a 1:40 or greater titer response to A/Beijing/32/92 1 month after the first vaccination. Only two additional patients were found to have similar titers after the second dose. A higher proportion of patients with protective titers were on smaller amounts of prednisone for body weight or alternate day low dose (< 10 mg/day) prednisone compared to patients on daily low dose or daily high dose prednisone. Patients with protective titers were significantly older (9.0 +/- 2.8 years) than those without protective titers (4.2 +/- 3.4 years, p = .002) following the first inoculation of the A/Beijing/32/92 vaccine component. Similar results were found for the second vaccination and with the H1N1 antigen. Cyclosporine level, gender, and body mass index were not associated with any outcome measures. We conclude that most liver transplant recipients had a protective antibody titer after a single influenza inoculation, but little further advantage was gained after an additional dose. Vaccination of household contacts of younger patients and those patients on daily prednisone or patient chemoprophylaxis may offer greater benefit in prevention of influenza in liver transplant recipients than multiple vaccine doses with current vaccine preparations.

Root caries and root defects in urban and rural adults: the Florida Dental Care Study.

As part of the Florida Dental Care Study--a longitudinal study of risk factors for changes in dental health--the authors examined and interviewed 873 adults aged 45 years and older. During the examination, clinicians noted the number of teeth present and the participant's dental condition, including presence of root caries and fillings or noncarious defects on the roots. The interview was conducted to collect demographic and other information such as the adults' perceptions about dental care and their perceptions about their own dental health. The authors found that men, African-Americans, adults living in rural areas and those living in poverty were significantly more likely to have root caries than other participants. The data reported in this article will serve as baseline information for subsequent research.

Preliminary experience with intestinal transplantation in infants and children.

OBJECTIVE: This report discusses the preliminary experience with intestinal transplantation in children at the University of Nebraska Medical Center. PATIENTS: During the past 4 years, 16 intestinal transplants have been performed in infants and children. Thirteen have been combined liver and bowel transplants, and the reminder were isolated intestinal transplants. Nearly half of the patients were younger than 1 year of age at the time of surgery, and the vast majority were younger than 5 years of age. All but one had short bowel syndrome. RESULTS: The 1-year actuarial patient and graft survival rates for recipients of liver and small bowel transplants were 76% and 61%, respectively. Eight of 13 patients who received liver and small bowel transplants remain alive at the time of this writing, with a mean length of follow-up of 263 (range, 7 to 1223) days. Six patients are currently free of total parenteral nutrition. All three patients receiving isolated intestinal transplants are alive and free of parenteral nutrition. The mean length of follow-up is 384 (range, 330 to 450) days. Major complications have included severe infections and rejection. Lymphoproliferative disease, graft-versus-host disease, and chylous ascites have not been major problems. CONCLUSIONS: Although intestinal transplantation is in its infancy, these preliminary results suggest combined liver and bowel transplants and isolated intestinal transplantation may be viable options for some patients with intestinal failure caused by short bowel syndrome or other gastrointestinal disease in whom long-term total parenteral nutrition is not an attractive option.

Clinical denouement and mutation analysis of patients with cystic fibrosis undergoing liver transplantation for biliary cirrhosis.

OBJECTIVE: To describe the clinical characteristics of patients with cystic fibrosis considered for liver transplantation and the clinical outcome after transplantation. METHODS: Patient charts were reviewed. Mutation analysis was performed on blood or liver tissue samples with a panel of 17 mutations. RESULTS: Eight patients (five girls) with cystic fibrosis have undergone orthotopic liver transplantation for biliary cirrhosis. Mean age at transplantation was 12.0 years +/- 7.7 years (range, 9 months to 23 years). Preoperatively, seven patients had mild to moderate pulmonary dysfunction and one moderate to severe pulmonary dysfunction. All patients required pancreatic enzyme replacement, and four patients required insulin for diabetes mellitus. The 1-year survival rate was 75%, with no deaths related to septic events. Mean time of follow-up the six operative survivors was 4.1 years +/- 1.9 years. Pulmonary function testing, in those serially tested, showed that forced expiratory volume in 1 second was maintained or improved and that forced vital capacity improved after transplantation. Mutation analysis showed the following genotypes: four patients, delta F508/delta F508; one patient, delta F508/N1303K; and three patients, delta F508/unknown. CONCLUSIONS: Despite the high risk of transplantation, these encouraging results indicate that liver transplantation should be considered for patients with cystic fibrosis and complications of end-stage liver disease. We could not demonstrate an unusual pattern of CF gene mutations in these patients with severe liver disease. It appeared that immunosuppressive agents did not have a deleterious effect on pulmonary function.

Abdominal pain.

Abdominal pain remains a common complaint in children and adolescents and accounts for a frequent number of visits to pediatricians as well as gastroenterologists. A thorough understanding of the neuroanatomy and neurophysiology of the abdomen and its contents is immensely important in understanding the origins of abdominal pain and formulating a limited differential diagnosis for the patient presenting with abdominal pain. With this as fundamental background, and the important clues provided by a thorough history and physical examination, a rapid and accurate diagnosis for the pediatric patient presenting with either acute, subacute, or chronic abdominal pain can be in most instances reasonably ascertained. Subsequently, specific studies for confirmation can then be performed and appropriate treatment implemented. The tremendous advancement in the field of gastroenterology provided by the continued development of endoscopic procedures has allowed for precise identification of many GI disorders. Endoscopy in concert with radiology procedures can be used effectively now to adequately establish a diagnosis in almost all patients presenting with abdominal pain.

Glomerular injury in end-stage liver disease--role of circulating IgG and IgM immune complexes.

The relationship of IgG- and IgM-bound circulating immune complexes and immune dysfunction to glomerular injury was evaluated in 15 children with end-stage liver disease (ESLD) awaiting liver transplantation. Compared with age-matched controls, children with ESLD had significantly (P < 0.01) increased serum IgG, IgA, and IgM levels, as well as IgG- and IgM-bound circulating immune complexes. Furthermore, they showed a significant (P < 0.05) depression of C3 and C4 levels compared with controls. Hematuria occurred in 66% of children with ESLD, and the urinary protein/creatinine ratio was also significantly (P < 0.01) increased compared with controls (4.65 +/- 2.56 vs. 0.16 +/- 0.04 mg/mg). Light microscopy of renal biopsy tissue obtained from 6 children with ESLD at the time of transplantation demonstrated mesangial proliferation and expansion with basement membrane splitting. This was associated with subendothelial deposits on electron-microscopic examination, compatible with a diagnosis of membranoproliferative glomerulonephritis. By immunofluorescence, deposition of IgG, IgA, and IgM occurred in various combinations with co-deposition of complement fragments. We conclude that membranoproliferative glomerulonephritis is a common finding in children with ESLD, probably due to entrapment of circulating IgG- and IgM-bound immune complexes.

Pancreatitis associated with bone marrow transplantation in children.

Vomiting, abdominal pain and distension, common findings in children who receive bone marrow transplants (BMT), are usually attributed to chemo-irradiation and mucositis, universally found in these patients. We report seven children, 3.5% of BMT patients at our institutions, with these symptoms who were found to have mild to severe pancreatitis during conditioning for or after receiving BMT. All patients were receiving drugs known to cause pancreatitis, such as adrenocorticosteroids and sulfonamides as well as numerous putative pancreatotoxins such as cyclosporin A and cytosine arabinoside. Five of the seven patients had suffered from graft-versus-host disease. In patients who have received BMT, upper gastrointestinal symptoms should not be attributed to mucositis or chemo-irradiation without first testing for pancreatitis.

Liver transplantation at the University of Nebraska Medical Center from 1985 to 1992.

Based on our 7 and one-half-year experience with liver transplantation at the University of Nebraska Medical Center: 1. Success and growth of the program has been, in part, the result of close interaction and support of the various specialists involved. 2. We have demonstrated that outstanding patient and graft survival rates can be obtained with cyclosporine/prednisone immunosuppression. 3. Few, if any, technical contraindications exist to liver transplantation. 4. Surgical advances have allowed allografts to be salvaged which would otherwise require replacement. 5. Routine donor-liver biopsy prior to implantation has reduced the rate of primary nonfunction. 6. New strategies to improve survival for patients with hepatitis-B-related liver disease and hepatic malignancies undergoing liver transplantation need to be developed. 7. The management of patients with fulminant hepatic failure is evolving and now includes innovative approaches such as the use of ECLS and auxiliary transplants.

Failure of blind small bowel biopsy in the diagnosis of intestinal lymphangiectasia.

A case of primary intestinal lymphangiectasia is presented in which multiple blind peroral jejunal biopsies were unable to document any abnormality, despite strongly suggestive clinical history and radiographic findings. Endoscopically directed biopsy was necessary to document the characteristic pathologic lesion. This report documents the importance of endoscopy in the diagnosis of intestinal lymphangiectasia when clinical history is suggestive of intestinal lymphangiectasia but standard small bowel biopsy fails to show any abnormality.

Intestinal abnormalities with central nervous system malignancy.

Two anorectic, emaciated infants with central nervous system (CNS) neoplasms are reported. Diagnosis in each case was delayed, because computed tomography of the head was initially falsely negative, and because primary gastroenterologic illness was suggested by the presence of mild inflammation and villous atrophy of the small intestine. This experience indicates that the diagnosis of CNS neoplasm must be aggressively pursued in cachectic infants who refuse to eat, regardless of the results of the initial radiologic studies. Mild histologic abnormalities of the small intestine that may be present in infants with brain tumors probably represent a nonspecific response to starvation and, if unassociated with malabsorption, should not be considered the cause of illness.

Variation in liver histology in Alagille's syndrome.

A previously undescribed variation in liver histology was observed in association with syndromatic neonatal jaundice, otherwise known as Alagille's syndrome. While hepatic ductular hypoplasia is thought to be characteristic of this disorder, we observed a patient who had all of the characteristic morphological features of Alagille's syndrome but whose liver biopsy demonstrated marked ductular proliferation more characteristic of biliary atresia. This histological feature has not been previously reported with Alagille's syndrome and hepatic ductular hypoplasia can no longer be considered characteristic of syndromatic neonatal jaundice.

A rapid procedure for small intestinal biopsy in infants and children.

A steerable small bowel biopsy apparatus was evaluated in 56 infants and children over a 15-mo period. A mean time of only 6 min was required to obtain small bowel biopsies. Biopsies were successfully obtained in 92% of patients on the first attempt; repeat attempts were successful in all cases. No complications were encountered. The steerable biopsy tube appears to be a safe and effective means of obtaining small bowel biopsies rapidly in infants and children.