Population factors not addressed in cohort study.
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Anti-Inflammatory Agents, Non-Steroidal↗
Biomedical subjects
J Hyams
Publications and source records attributed to J Hyams.
Does cisapride influence cardiac rhythm? Results of a United States multicenter, double-blind, placebo-controlled pediatric study.
BACKGROUND: Major concerns about serious cardiac side effects underlie the recent decision by the FDA and Janssen Pharmaceutica (Titusville, NJ) to make cisapride available only through a limited access program. Concerns have grown despite the fact that most instances of prolonged QTc and other ventricular arrhythmias occurred while the drug was used concomitantly with contraindicated drugs. This study sought to analyze electrocardiograms (ECGs) from a multicenter pediatric study and to identify abnormalities in QTc interval associated with cisapride use. METHODS: Children between 6 months and 4 years of age were enrolled if they manifested symptoms of gastroesophageal reflux not responding to medical therapy for at least 6 weeks. In 49 subjects, ECGs obtained before and after randomization to receive 0.2 mg/kg dose three times daily or placebo were reviewed independently and blindly by two pediatric cardiologists. Placebo and active drug groups were compared for QTc and for change in QTc from baseline values after 3 to 8 weeks of treatment. RESULTS: Mean QTc among patients taking the drug was 408+/-18 ms. None was higher than 450 ms. Change between baseline and subsequent QTc at 3 to 8 weeks of treatment was 2+/-20 ms. CONCLUSIONS: In our study group of children without underlying cardiac disease or electrolyte imbalance, cisapride was found to have no significant effect on cardiac electrical function compared with placebo. These results are consistent with the drug's record of exceedingly infrequent cardiac events. Because the availability of this prokinetic is threatened, its safety and the safety and efficacy of alternative treatment options (including surgery) should be studied further.
Arrhythmias, Cardiac↗
Sacrosidase therapy for congenital sucrase-isomaltase deficiency.
BACKGROUND: The purpose of this study was to determine if sacrosidase, a liquid produced from Saccharomyces cerevisiae containing 6000 IU of sucrase activity per mg protein, prevented symptoms of diarrhea, abdominal cramps, gas, and bloating in patients with congenital sucrase-isomaltase deficiency (CSID) consuming a normal sucrose and carbohydrate-containing diet. METHODS: Twenty-eight children (aged 5 months to 11 years) underwent a randomized, double-blind trial consisting of two phases: 1) three sucrose breath H2 tests with three single-dose treatments (placebo, sacrosidase, and sacrosidase plus milk), and 2) a dose-response phase consisting of four multidose treatments, each for 10 days of full-strength sacrosidase, 1:10 dilution, 1:100 dilution, and 1:1000 dilution. Patients who weighed less than or equal to 15 kg received a dose of sacrosidase and those who weighed more than 15 kg received 2 ml. For the dose-response phase each patient consumed a normal diet. The number of stools and severity of symptoms were recorded daily for each concentration of sacrosidase administered and compared to a baseline period during which the patient took no sacrosidase and consumed a sucrose/starch-free diet. Data were analyzed using an ANOVA model and the nonparameter Wilcoxon signed-rank test. RESULTS: Breath H2 excretion decreased significantly when patients received sacrosidase or sacrosidase plus milk compared to placebo during sucrose breath tests. During the dose-response phase significant treatment differences were observed between the two higher concentrations and the two lower concentrations of sacrosidase for both total stools (p < 0.001) and total symptom score (p = 0.003). Higher concentrations of sacrosidase were associated with fewer stools and a greater number of formed or hard stools compared to lower concentrations and compared to the baseline period. Higher concentrations were also associated with fewer symptoms of gas, abdominal cramps, or bloating, but no differences in vomiting. The only significant adverse event was wheezing in one child with a history of asthma. CONCLUSIONS: Sacrosidase is a safe, effective, well-accepted treatment to prevent gastrointestinal symptoms in patients with CSID consuming a normal diet.
Animals↗
Cellular microbiology: cycling into the millennium.
Cellular microbiology is a newly developing science born from the realization that many different aspects of eukaryotic cell biology are targeted by microbial virulence mechanisms. One example of this is the emerging evidence that several bacteria can interfere, directly or indirectly, with the eukaryotic cell cycle. This article discusses the cell-cycle effects of bacterially generated molecules, their role in virulence and their possible therapeutic potential.
Adaptation, Physiological↗
Clinical outcome of ulcerative proctitis in children.
BACKGROUND: Although the course of ulcerative proctitis in adults has been well described, little data are available concerning its clinical behavior in children and adolescents. This study sought to characterize the presentation, response to therapy, and long-term course of ulcerative proctitis in the pediatric population. METHODS: A retrospective chart review was conducted at five pediatric gastroenterology centers. RESULTS: A total of 38 subjects (mean age 11.6 years) were identified with ulcerative proctitis (mean follow-up 4.3 years). Symptoms were mild at diagnosis in 74% and moderate or severe in 26%. Thirty-two percent had a complaint of constipation at presentation. Cessation of symptoms was noted in 68% within 3 months of therapy, an additional 24% within 6 months, and 8% were still symptomatic despite 6 months of therapy. During any subsequent yearly follow-up interval, -55% of patients were asymptomatic, 40% had a chronic intermittent course, and < 5% were continuously symptomatic despite therapy. Eight subjects were treated with oral corticosteroids, one with 6-mercaptopurine, and one with cyclosporine. Extension of inflammation proximal to the rectosigmoid occurred in 11 of 38 subjects (29%), 0.5-11.3 years postdiagnosis. Seven of the 13 subjects (54%) followed for > or = 5 years had proximal extension of disease, and two had undergone colectomy. CONCLUSIONS: Despite a mild presentation in most subjects, ulcerative proctitis seems to have a high risk of proximal extension of disease. The overall response to therapy seems to be similar to that reported for ulcerative colitis in children. Follow-up endoscopic evaluation of patients with ulcerative proctitis seems warranted, especially in the setting of recurrent or recalcitrant symptoms.
Adolescent↗
Detection of group C rotavirus in infants with extrahepatic biliary atresia.
The purpose of this retrospective study was to examine liver tissue from patients with cholestatic disease for the presence of group C rotavirus RNA. The reverse transcriptase-polymerase chain reaction (PCR) for genes 5 and 6 was used, and the PCR products were subjected to liquid hybridization with a 32P-labeled probe. A second amplification with nested primers was also used. Samples from 32 subjects (20 with biliary atresia or choledochal cyst and 12 controls) were tested. Ten of 20 biliary atresia patients were positive for group C rotavirus RNA; no controls were positive (P < .003). Three of the positive patients were positive for both genes 5 and 6. Six of the 10 had > 1 sample that was positive. These data suggest a possible relationship between group C rotavirus and extrahepatic biliary atresia in the 10 patients in whom virus RNA was detected.
Autoradiography↗
Organ transplantation at the Hartford Transplant Center.
Over 1,243 organ transplants have been performed at the Hartford Transplant Center over the past two decades. Survival in kidney, heart, liver, and pancreas patients is at or above the national average. Hartford was one of the first centers to use triple immunosuppression, which significantly improved survival in kidney transplantation. For recipients of kidneys from living related donors and cadaveric kidneys, two-year actuarial graft survival has been 98% and 83%, respectively, over the last five years. For heart and liver transplants, two-year survival has been 79% and 67%, respectively. Despite high success rates at most transplant centers, donor organs remain scarce. This problem needs to be addressed through increased cooperative efforts in the health-care community and the general public.
Adolescent↗
Psychopathology and relationship measures in children with inflammatory bowel disease and their parents.
Children with inflammatory bowel disease and their parents were assessed for DMS-III diagnoses, maternal attachment, marital relationship, life stresses, illness severity and cognitive/affective profile. Eleven of 15 children and 21 of 27 parents had psychiatric diagnoses. Three children were suicidal. Thirteen of 15 mothers had insecure attachment. Marital difficulties and medical severity correlated with life stress. The higher prevalence of psychiatric disorders than epidemiologically and of insecure attachment suggest at-risk relationships.
Adolescent↗
Evaluation of liquid yeast-derived sucrase enzyme replacement in patients with sucrase-isomaltase deficiency.
BACKGROUND: No enzyme replacement therapy exists for patients with congenital sucrase-isomaltase deficiency (CSID). A by-product of the manufacture of baker's yeast is a liquid preparation containing high sucrase activity. The aim of the present study was to investigate the activity and stability of this preparation and its effect on breath hydrogen excretion and gastrointestinal symptoms after sucrose ingestion in 14 patients with CSID. METHODS: The homogeneity of yeast sucrase was studied by sodium dodecyl sulfate-polyacrylamide gel electrophoresis, and its activity was measured. Stability at various temperatures and pH ranges and in the presence of gastric aspirate, pepsin, and bovine serum albumin was assessed. Fourteen patients with CSID underwent double-blind placebo-controlled breath tests with yeast sucrase. They then completed an 8-week dose response study that used different enzyme concentrations while consuming a sucrose-containing diet. RESULTS: Liquid yeast sucrase is highly glycosylated, contains no lactase activity, and is stable at 4 degrees C and over a wide range of pH. Pepsin digestion of the enzyme in vitro can be blunted by bovine serum albumin and by increasing the pH. Yeast sucrase reduces breath hydrogen excretion in patients with CSID who are given a sucrose load (P < 0.001) and allows most patients to consume a sucrose-containing diet. CONCLUSIONS: Liquid yeast sucrase offers effective enzyme replacement therapy for patients with CSID.
Administration, Oral↗
Atypical rectosigmoid histology in children with newly diagnosed ulcerative colitis.
BACKGROUND: In the untreated patient with inflammatory colitis, rectal sparing or patchy rectal inflammation is generally considered a sign of Crohn's disease (CD), rather than ulcerative colitis (UC). METHODS: The initial endoscopic rectosigmoid mucosal biopsies obtained at disease onset from 12 untreated children with UC who ultimately required surgery were blindly reviewed (randomly mixed with another 62 specimens obtained from children with CD or treated UC). Biopsies were classified as typical UC if there was diffuse, active inflammation and severe crypt destruction or distortion. Those with patchy, active inflammation and only mild crypt changes were classified as CD. Because all 12 subjects had ultimately been proven to have UC by examination of a subtotal colectomy specimen, for the purposes of this report biopsies read as either normal or CD were both considered evidence of atypical UC with rectal sparing. RESULTS: Five of 12 subjects (seven biopsies) had atypical histology. Mild, patchy inflammation was seen in six rectal or sigmoid biopsies, whereas one rectal biopsy was normal. The remaining seven subjects (10 biopsies) had diffuse inflammation. Two of five subjects with atypical biopsies had an endoscopically normal rectosigmoid, one had patchy inflammation, and the remaining two had diffuse endoscopic changes. All seven subjects with typical UC histology had diffuse endoscopic changes. Subjects with atypical findings could not be differentiated by age, duration, or types of symptoms at presentation, years of disease at colectomy, or indications for colectomy. CONCLUSIONS: Patchy or absent inflammation of the rectum and sigmoid can be present in untreated children with UC at disease onset. Because such children may be mistakenly diagnosed as having CD, these data must be considered when treatments or clinical research protocols are designed to include children with colitis.
Adolescent↗
Early cirrhosis in survivors with Jeune thoracic dystrophy.
Two siblings with Jeune thoracic dystrophy had persistent evidence of hepatic dysfunction; one had cirrhosis. Liver disease, a significant problem in patients who survive this condition, may be progressive in some.
Asphyxia Neonatorum↗
Discrimination between forms of vitamin E by humans with and without genetic abnormalities of lipoprotein metabolism.
To study the mechanisms of discrimination between various forms of vitamin E, four normal subjects, one patient with lipoprotein lipase deficiency, and three patients with abnormal apolipoprotein B-100 production were given an oral dose containing three tocopherols labeled with differing amounts of deuterium (2R,4'R,8'R-alpha-(5,7-(C2H3)2)tocopheryl acetate (d6-RRR-alpha-tocopheryl acetate), 2S,4'R,8'R-alpha-5-(C2H3)tocopheryl acetate (d3-SRR-alpha-tocopheryl acetate), and 2R,4'R,8'R-gamma-(3,4-2H)tocopherol (d2-RRR-gamma-tocopherol). The tocopherol contents of plasma, red cells, and lipoproteins were measured up to 76 h after the dose. In normal subjects all three tocopherols were absorbed and secreted in chylomicrons with equal efficiencies. Both d2-gamma- and d3-SRR-alpha-tocopherols peaked at similar concentrations in the other lipoprotein fractions, then decreased similarly, but 2-4 times more rapidly than did d6-RRR-alpha-tocopherol. A lipoprotein lipase-deficient patient and a patient with prolonged production of chylomicrons with absent apolipoprotein B-100 also demonstrated the lack of discrimination between tocopherols during absorption. Despite abnormal apolipoprotein B-100 production in two patients, the "VLDL" was preferentially enriched in d6-RRR-alpha-tocopherol. Our results show that there is no discrimination between the three tocopherols during absorption and secretion in chylomicrons, but subsequently there is a preferential enrichment of very low density lipoprotein (VLDL) with RRR-alpha-tocopherol. Catabolism of this VLDL results in the maintenance of plasma RRR-alpha-tocopherol concentrations.
Administration, Oral↗
Cytoplasmic vacuolization of enterocytes: an unusual histopathologic finding in juvenile nutritional megaloblastic anemia.
We report a case of juvenile nutritional megaloblastic anemia with emphasis on the histologic findings in the upper gastrointestinal system, including the previously unreported feature of periodic acid-Schiff (PAS)-negative, Alcian blue-negative cytoplasmic vacuoles (CV) in duodenal enterocytes. These CV were reminiscent of those described in abetalipoproteinemia; however, the patient's lipid profile was normal. All histopathologic changes in the upper gastrointestinal tract (with the exception of mild reflux esophagitis) reverted to normal following parenteral administration of vitamin B12. This case supports the notion that CV in enterocytes are a component (albeit a rare one) of the histopathology in cobalamin deficiency.
Anemia, Megaloblastic↗
Abdominal trauma--child abuse.
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Abdominal Injuries↗
Dynein in the spindle?
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Adenosine Triphosphatases↗