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Biomedical subjects

H S Winter

Publications and source records attributed to H S Winter.

At least 19 recordsLinked to original sources
PubMed1999

Quality-of-life assessment after ileoanal pull-through for ulcerative colitis and familial adenomatous polyposis.

BACKGROUND/PURPOSE: The ileoanal pull-through procedure (IAP) is gaining increasing favor and use in the surgical treatment of children with ulcerative colitis (UC) and familial adenomatous polyposis (FP). Although physiological studies have been performed to assess the outcome of these children, no long-term quality-of-life assessment after the procedure has been performed. METHODS: Forty-three patients were identified who had an IAP at our institution in the last 10 years and were at least 6 months postsurgery. Thirty-four were contacted, and 32 agreed to participate in the survey, which was approved by the Human Studies Committee. Participants completed the standardized Medical Outcome Study Short Form-36 (SF-36), which has well-established normative values. Several supplemental questions were prepared in a similar format dealing with issues specific to the ileoanal pull-through procedure. RESULTS: Of the 32 participants, 19 (59%) were girls and 26 (81%) had ulcerative colitis. Mean age at the time of survey was 18.1 years with 12 less than 18 years and 20 > or =18 years. Data from the latter group could be compared with national normative values for this age. The study group was not statistically different from age-appropriate US population normal values on all assessable scales of physical and mental health in the SF-36 survey including physical functioning, role limitations-physical, bodily pain, general health, vitality, social functioning, role limitations-emotional, and mental health (all P>.05 or mean difference SD units <0.8). The supplemental questionaire demonstrated little adverse effect of the surgery. There was limited consumption of medications to control bowel frequency and little restriction of activity because of the frequency of bowel movements or fear of incontinence. The surgical scar was the sole negative factor of significance. CONCLUSIONS: The ileoanal pull-through procedure is an excellent surgical option for children with ulcerative colitis or familial adenomatous polyposis, and it produced minimal, if any, adverse effects on their long-term quality of life.

Adenomatous Polyposis Coli↗
PubMed1999

Regulation of intestinal peptidases by nutrients in human fetuses and children.

Nutrient interactions during intestinal digestion has been established in animals. The present study investigated the effect of different nutrients on intestinal dipeptidase, tripeptidase, carboxypeptidase (EC 3.4.12.-) and aminopeptidase N (ApN) (EC 3.4.11.2) activities in human fetuses and children. The effect of nutrients on isolated porcine kidney ApN was also studied. Sucrose, lactose and tributyrin had no effect on di-, tri-, and carboxypeptidase activities of mucosal homogenates, but tributyrin significantly (20-50%) inhibited both fetal and postnatal ApN activity in the small intestine and colon. The pH-independent inhibition of ApN is specific for tributyrin and to the product of its hydrolysis, butyric acid. Glycerol, triolein, and natural oils did not affect ApN activity. The inhibition of ApN by tributyrin was dose and time dependent and occurred in enterocyte brush border membranes as well as in the purified enzyme from porcine kidney. The kinetics of the purified ApN showed that the tributyrin effect is primarily competitive and associated mainly with an increase in K(m). These observations demonstrate the possibility of intestinal and kidney ApN regulation by lipids and products of their hydrolysis. We speculate that nutrient interactions arose quite early in the evolution and represent a mechanism for the regulation of food digestion.

Child↗
PubMed1998

A position paper of the North American Society for Pediatric Gastroenterology and Nutrition. Pediatric gastroenterology Workforce Survey and future supply and demand.

BACKGROUND: The North American Society for Pediatric Gastroenterology and Nutrition (NASPGN) performed a Workforce Survey to determine the current number and distribution of pediatric gastroenterologists in the United States and Canada and to estimate the supply and demand in the future in the United States. METHODS: The response rate was more than 90%. There were 624 pediatric gastroenterologists in the United States, and 48 in Canada. RESULTS: There were 2.4 pediatric gastroenterologists per million population in the United States, ranging from 3.1 per million in the Northeast to 1.9 per million in the West, and 1.6 per million in Canada. In the United States, fewer than 5 pediatric gastroenterologists retire each year, but more than 40 fellows per year complete training. In the United States, 30% of pediatric gastroenterologists believe there is already an excess supply; only 12% believe there is a shortage (p < 0.001). CONCLUSIONS: If the number of fellows who complete training each year remains unchanged, in 10 years there will be more than 950 pediatric gastroenterologists in the United States (3.3 per million population). At the same time, if the demand for pediatric gastroenterologists remains 2.4 per million population, there will be a demand for only 675. If these assumptions are correct, it is necessary to reduce the number of fellows to be trained. Although it is difficult to predict future workforce needs reliably, we recommend that the number of fellowship positions in training programs in the United States be reduced by 50% to 75%. Changes in health care in the coming years will be challenging, and effective planning is necessary for pediatric gastroenterologists to achieve their clinical, research, and educational missions.

Adolescent↗
PubMed1997

The TCR-delta repertoire in human intestine undergoes characteristic changes during fetal to adult development.

The TCR-delta repertoire in adult human intestine is oligoclonal and unique in each individual. In the present study, changes in the junctional regions of TCR-delta transcripts in human intestine that occur during development from fetal to adult life were used to characterize fundamental changes in the TCR-delta repertoire in the human intestinal tract during ontogeny. At mid-gestation, the fetal repertoire was polyclonal, but limited, in its junctional diversity by the relative lack of N region nucleotide additions and by the frequent formation of coding region joins at regions of short sequence homology. In addition, identical TCRDV2 transcripts that resemble canonical TCR-delta sequences in mice were present in the intestine of different fetuses. In the early period after birth, the intestinal TCR-delta repertoire was polyclonal, and more diverse than the fetal repertoire, with junctional regions that contained extensive N nucleotide additions and frequently were as complex as those of adults. The intestinal TCR-delta repertoire showed increasing restriction with age and, by 14 to 17 yr, the repertoire was oligoclonal and resembled the repertoire of individuals in the sixth to seventh decade. Moreover, the adult TCR-delta repertoire was almost identical at multiple sites throughout the intestine, suggesting a model in which gammadelta T cell clones, selected by ligands in the intestinal tract, undergo expansion and recirculation before lodging throughout the small intestine or colon.

Adolescent↗
PubMed1995

Intestinal and hepatobiliary diseases in HIV-infected children.

Children with HIV disease and gastrointestinal disease should be evaluated for enteric pathogens. Bacterial, protozoal, and viral agents can cause chronic diarrhea, abdominal pain, gastrointestinal bleeding, and contribute to growth retardation. This article presents an approach to the evaluation of the HIV-infected child with gastrointestinal symptoms. Therapeutic and nutritional interventions are discussed with emphasis on the multidisciplinary approach required to initiate successful management.

AIDS-Related Opportunistic Infections↗
PubMed1994

Quantitative analysis of mononuclear cells expressing human immunodeficiency virus type 1 RNA in esophageal mucosa.

The mucosa of the gastrointestinal tract is presumably an important reservoir for human immunodeficiency virus type 1 (HIV-1), but the level of virus-expressing cells within the mucosa of infected patients is not known. To study this issue, we identified HIV-1 mRNA-expressing (positive) mononuclear cells by in situ hybridization in specimens of esophageal mucosa from eight patients with acquired immune deficiency syndrome (AIDS) and esophageal infections. Such cells were not found in four patients with AIDS and no esophageal disease. Immunocytochemical staining revealed that the mononuclear cells expressing HIV-1 mRNA were lamina propria macrophages. The prevalence of positive cells was measured by triplicate determinations in each of three experiments using an inverse sampling technique. No significant differences in prevalence were found among patients or among experiments. The overall prevalence of HIV-1 mRNA-expressing cells in the esophageal lamina propria was 0.059 +/- 0.01%. This prevalence of cells expressing HIV-1 mRNA in the mucosa of patients with mucosal infections may reflect the local abundance of stimuli such as bacterial endotoxin and certain cytokines capable of inducing viral transcription.

Acquired Immunodeficiency Syndrome↗
PubMed1994

Anorectal function in children after ileoanal pull-through.

Mucosal proctectomy and ileoanal pull-through is increasingly used in children requiring total colectomy for ulcerative colitis or familial polyposis. Excellent continence can be achieved with this procedure, and it avoids proctocolectomy and permanent ileostomy. We have evaluated prospectively anorectal function in nine consecutively treated children who underwent ileoanal pull-through. Patients were 8 to 17.5 years of age (median, 11.3 years) at the time of surgery; seven had ulcerative colitis, and two had familial polyposis. Anorectal evaluation was performed before colectomy and ileoanal pull-through, following ileoanal pullthrough, after rectal training, and then at yearly intervals. A biofeedback "rectal training" program was instituted 6 weeks after ileoanal pull-through and a contrast study documenting integrity of the pouch. The program consisted of an initial biofeedback session with the motility unit, followed by daily instillations, through a catheter, of progressively larger volumes of water (from 1 to 6 oz, increasing 1 oz per week) into the ileal pouch. Patients were instructed to retain the water and participate in normal activities after the instillation. This protocol acclimated the patient to sensing distension of the pouch and using the sphincters. The follow-up period ranges from 1 to 4.5 years (median, 2.2 years). All patients are continent by day and night. One patient has nocturnal incontinence with episodes of pouchitis. Stool frequency is three to eight movements per day (median, four), with none at night. Preoperative resting rectal sphincter pressures averaged 74.3 +/- 23.1 mm Hg (mean +/- standard deviation), and a maximum squeeze pressure was 93.9 +/- 25.3 mm Hg.(ABSTRACT TRUNCATED AT 250 WORDS)

Adenomatous Polyposis Coli↗
PubMed1994

Anti-neutrophil cytoplasmic antibodies in children with ulcerative colitis.

Tests that positively identify individuals with ulcerative colitis, distinguishing them from patients with Crohn disease or other causes of colitis, have not been reliable. Genetic predisposition to inflammatory bowel diseases and genetic influence on immune regulation resulted in the clinical evaluation of potential serologic markers. In adults the presence of anti-neutrophil cytoplasmic antibody (ANCA) in serum identifies patients with ulcerative colitis. In this study we demonstrated that high levels of ANCA are present in 83% of children and adolescents with ulcerative colitis. Furthermore, the majority of patients with ulcerative colitis had a perinuclear pattern of these antibodies by indirect immunofluorescence. The combination of a positive ANCA and perinuclear indirect immunofluorescence pattern was 97% specific for ulcerative colitis. We conclude that determination of ANCA is a sensitive and specific clinical test for identification of children and adolescents with ulcerative colitis.

Adolescent↗
PubMed1993

Growth and body composition in children infected with the human immunodeficiency virus-1.

Anthropometric data were collected on 89 children born to human immunodeficiency virus (HIV)-infected women (37 who seroreverted and 52 who were HIV-infected). The main outcomes included birth weight, gestational age, weight, height, arm muscle circumference (AMC), and triceps skinfold thickness (TSF). Gestational age and birth weight were not different between the two groups. The earliest anthropometric evaluation on seroreverted children (age 19 mo) when compared with HIV-infected children (age 21 mo) revealed that weight and weight-for-height percentiles were significantly different (51% vs 33% and 66% vs 48%, respectively). Height and TSF percentiles were not different, although AMC percentiles were lower in infected children (64% vs 43%). In follow-up evaluations, the weight differences between infected and control children did not change. We conclude that HIV does not affect birth weight, but postnatal events result in altered weight gain in HIV-infected children. Lean body mass is lower than in an HIV-negative comparison group at early stages of HIV infection.

Anthropometry↗
PubMed1993

Olsalazine versus sulfasalazine in mild to moderate childhood ulcerative colitis: results of the Pediatric Gastroenterology Collaborative Research Group Clinical Trial.

The safety and efficacy of olsalazine sodium was compared to sulfasalazine over 3 months in a multicenter, randomized, double-blind study of 56 children with mild to moderate ulcerative colitis. Twenty-eight children received 30 mg/kg/day of olsalazine (maximum, 2 g/day) and 28 received 60 mg/kg/day of sulfasalazine (maximum, 4 g/day). Side effects were frequent in both groups. Eleven of 28 patients (39%) on olsalazine reported headache, nausea, vomiting, rash, pruritus, increased diarrhea, and/or fever. Thirteen of 28 on sulfasalazine (46%) reported similar side effects and/or neutropenia, and four patients had the drug stopped because of an adverse reaction. After 3 months, 11 of 28 (39%) on olsalazine were asymptomatic or clinically improved, compared to 22 of 28 (79%) on sulfasalazine (p = 0.006). In addition, 10 of 28 patients on olsalazine versus one on sulfasalazine required prednisone because of lack of response or worsening of colitis (p = 0.005). The dose of olsalazine used in this clinical trial was thought to be equivalent to a standard dose of sulfasalazine, but fewer patients on olsalazine improved and a greater number had progression of symptoms when compared to sulfasalazine. Although side effects were slightly less frequent for olsalazine, the number of patients was too small to detect a clinically significant difference.

Adolescent↗
PubMed1992

Glycogen storage disease type III (glycogen debranching enzyme deficiency): correlation of biochemical defects with myopathy and cardiomyopathy.

OBJECTIVE: To determine whether a specific subtype of glycogen storage disease type III is associated with myopathy and cardiomyopathy. DESIGN: Case series. SETTING: Three referral medical centers. PATIENTS: All patients with glycogen storage disease type III who were followed in 1990 and for whom both immunoblot analysis and clinical data were available. MAIN OUTCOME MEASURES: Evaluation for myopathy and cardiomyopathy included determinations of serum creatine kinase activity; muscle strength testing; ischemic exercise testing; nerve conduction studies; and electromyographic, electrocardiographic, and echocardiographic studies. RESULTS: Three patients with deficient debranching enzyme activity and deficient immunoreactive material in liver but normal debranching enzyme activity in muscle (glycogen storage disease IIIb) had no clinical evidence of myopathy or cardiomyopathy. Serum creatine kinase activity, muscle strength, ischemic exercise testing, electrocardiograms, and echocardiograms were normal in these patients. These studies and electromyograms were abnormal in seven patients with total debranching enzyme deficiency and an absence of immunoreactive material in both liver and muscle (glycogen storage disease IIIa) and in three patients who had debranching enzyme transferase deficiency but normal glucosidase activity in both liver and muscle (glycogen storage disease IIId). All 10 of these patients had progressive myopathy, and 6 had progressive cardiomyopathy. CONCLUSION: Clinical features of glycogen storage disease type III correlate with the particular biochemical defect seen with the disorder. Assessments of debranching enzyme or debranching enzyme transferase activity in muscle can be used to predict whether patients with glycogen storage disease type III will develop myopathy and cardiomyopathy.

Adolescent↗
PubMed1992

Glycogen debranching enzyme deficiency: long-term study of serum enzyme activities and clinical features.

In glycogen storage disease type III (glycogen debranching enzyme (DE) deficiency), the activities of serum alanine aminotransferase, aspartate aminotransferase and lactate dehydrogenase may be strikingly elevated during childhood but are low during adult life. To determine the pattern of the elevated serum enzyme activities in relationship to diet, the biochemical subtype and clinical symptoms, 13 patients with DE deficiency were studied. Activities of serum aspartate and alanine transaminases, lactate dehydrogenase, and alkaline phosphatase were markedly elevated during infancy. Continued elevation of enzyme activities during childhood appeared to be related to DE deficiency in liver, but unrelated to DE deficiency in muscle. Activity elevations correlated inconsistently with diet and poorly with childhood growth rate or the presence of hypoglycaemia. The serum enzyme activities declined around puberty concomitantly with a decrease in liver size. Although periportal fibrosis and micronodular cirrhosis indicated the presence of hepatocellular damage during childhood, the decline in serum enzyme activities with age and the absence of overt hepatic dysfunction suggest that the fibrotic process may not always progress.

Adolescent↗
PubMed1992

Use of an animal model for the study of the role of human immunodeficiency virus 1 in the human intestine.

The gastrointestinal manifestations of human immunodeficiency virus (HIV)-1 disease have centered on identifiable causes of intestinal dysfunction such as parasitic and bacterial pathogens. The lamina propria of the intestine contains cell that harbor HIV-1, but the significance of this observation remains unknown. Because limited animal models are available to evaluate the gastrointestinal effects of this infection, a system that uses human fetal intestine transplanted subcutaneously onto the back of an immunodeficient mouse was developed. After 8 weeks, fetal tissues mature into an adult-appearing tissue with a lumen. Cell-free HIV-1 was inoculated into the explants, an evidence for infection was evaluated by histological evaluation, in situ hybridization, and polymerase chain reaction. No evidence for HIV-1 incorporation into epithelial cells could be found. It was concluded that this model provides a system in which intestinal HIV-1 interaction can be evaluated. In this model, cell-free HIV-1 does not appear to infect the epithelial cell.

Animals↗
PubMed1992

Pancreatitis in pediatric human immunodeficiency virus infection.

Because pancreatitis has been reported frequently in adults with human immunodeficiency virus infection, we sought to determine the incidence of pancreatitis in children with acquired immunodeficiency syndrome by reviewing all records of children with AIDS, their serum amylase and lipase levels, and the factors associated with pancreatitis through a case-control analysis. During a 6-year period pancreatitis developed in 9 (17%) of 53 pediatric patients with AIDS. Six children had vertical transmission of infection and three patients had acquired HIV infection through contaminated blood products. Pancreatitis developed at a median age of 5.2 years (range 1.2 to 20 years). All patients had vomiting and abdominal pain. When the patients were first seen, lipase values were elevated more than amylase values (p = 0.028). Amylase and lipase levels declined at comparable rates. In the case-control analysis, pentamidine isethionate was significantly associated with pancreatitis (p = 0.02); the risk was greater in patients who received pentamidine isethionate and had absolute CD4 T-lymphocyte counts less than 100 cells/mm3 (p = 0.001). Infections associated with the onset of pancreatitis included cytomegalovirus (4), Cryptosporidium (1), Pneumocystis carinii pneumonia (3), and Mycobacterium avium intracellulare (1). Coinfection with cytomegalovirus was associated with a protracted course in four children. Ultrasonographic examination demonstrated biliary ductal dilatation 6 months after the onset of pancreatitis in one child. Seven children have died at a mean of 8 months after the initial onset of pancreatitis; the one living child has survived 5 months from the onset of pancreatitis. We conclude that pancreatitis is common in pediatric patients with AIDS and may be related to pentamidine isethionate exposure, especially when absolute CD4 T-lymphocyte counts are less than 100 cells/mm3. Serum amylase levels do not always accurately predict the onset of pancreatitis; serum lipase levels should be measured in children with symptoms. The onset of pancreatitis in an HIV-infected child is a poor prognostic indicator.

Acquired Immunodeficiency Syndrome↗