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Biomedical subjects

E Simpser

Publications and source records attributed to E Simpser.

10 recordsLinked to original sources

Hypophosphatemia secondary to oral refeeding in anorexia nervosa.

OBJECTIVE: Hypophosphatemia is a well-known complication of the refeeding syndrome in severe cases of anorexia nervosa, described mostly as a result of refeeding with total parenteral nutrition. Few cases have been reported secondary to either nasogastric or oral refeeding. METHOD: The authors present three cases in which hypophosphatemia developed secondary to oral refeeding in severe anorexia nervosa. RESULTS: All 3 patients developed significant hypophosphatemia, to a low of 0.9 mg/dl in two cases and a low of 1. 7 mg/dl in the third. The first patient received close to 3,000 calories per day, along with intravenous fluids, in the hospital; the other 2 patients ate large amounts for several days at home. Caloric restriction and replenishment with phosphorous resulted in a rapid return of phosphorous values to normal levels. DISCUSSION: Those who treat severely malnourished patients with eating disorders, whether as inpatients or outpatients, need to be vigilant for the development of the refeeding syndrome, even in patients receiving oral refeeding alone.

Adolescent↗

Breastfeeding: 1999 perspective.

Although the vast majority of pediatricians agree that breastfeeding is the preferred form of infant feeding, a large number of infants are still exclusively formula-fed or rarely breastfed for an extended period of time. This review explores focuses on data that speak to mothers' decisions to initiate and continue breastfeeding. Current research focuses on the immunomodulatory effects of breast milk, especially its protective benefits as relates to infection and allergy. The evidence clearly indicates that pediatricians must continue to play a critical role in the promotion of breastfeeding.

Breast Feeding↗

Highly destructive perianal disease in children with Crohn's disease.

The perianal complications of Crohn's disease (CD) seen in children and adolescents include skin tags, anal fissures, fistulae, and abscesses. While these lesions are often chronic and variably responsive to medical therapy, only rarely are they severely destructive. In this report, we characterize the frequency, severity, and clinical course of a highly destructive form of perianal disease (HDPD) that we have noted in a number of children and adolescents with Crohn's disease. A database containing records from 350 children with inflammatory bowel disease was reviewed to identify all children with CD treated between 1970 and 1993. For each, the occurrence or absence of significant perianal pathology, including fistula, abscess, and HDPD, was determined. Pertinent clinical details were recorded for all patients. In addition, the clinical characteristics of those children with HDPD were compiled, and the courses of those with HDPD characterized. A search of the database identified 230 children and adolescents with CD followed for a total of 1,518 patient years. Sixty-seven of these patients (29% of the CD population) had significant perianal pathology. This included 6 with HDPD, 8 with complicated fistulae [rectourethroperineal (1), rectovaginal (1), rectolabial (2), and multiple communicating perineal (4)], and 53 with simple perianal fistulae or abscesses. All six with HDPD had deeply destructive perineal ulcerations, marked undermining of the perineal and perirectal tissues, and copious exudate, and often there was a deeply cleaved or fileted perineum on separating the buttocks. Two children with HDPD had fecal incontinence.

Abscess↗

Vasopressin and phorbol-12,13-dibutyrate inhibit glucagon- or cyclic AMP-stimulated taurocholate uptake in isolated rat hepatocytes.

Bile salt uptake by hepatocytes is modulated in part by changes in intracellular cyclic AMP. We studied the effect of activation of protein kinase C on cyclic AMP-mediated taurocholate uptake in isolated rat hepatocytes. Both dibutyryl cyclic AMP (2 x 10(-6) mol/L) and glucagon (10(-6) mol/L), which increase intracellular cyclic AMP, enhanced the initial uptake rate of taurocholate into hepatocytes, with maximal increases of 45% to 50% over the basal uptake rate. Vasopressin (10(-9) mol/L), a hormone known to activate protein kinase C, and phorbol-12,13-dibutyrate (10(-5) mol/L) significantly inhibited the glucagon-stimulated increase in taurocholate uptake rate (72% +/- 10% and 105% +/- 13% inhibition, respectively). Basal (unstimulated) taurocholate uptake rate was not affected by vasopressin or phorbol-12,13-dibutyrate. Down-regulation of the glucagon-stimulated transport was rapid and persisted during the 20-min experimental period. Angiotensin II had a similar but more transient inhibitory effect. Vasopressin and phorbol-12,13-dibutyrate suppression of glucagon-stimulated taurocholate uptake rate was not accompanied by diminished cyclic AMP levels. Moreover, vasopressin and phorbol-12,13-dibutyrate inhibited dibutyryl cyclic AMP-stimulated taurocholate uptake rate can be dissociated from alterations in the cyclic AMP levels.

Angiotensin II↗

Neuronal intestinal dysplasia: quantitative diagnostic criteria and clinical management.

Neuronal intestinal dysplasia (NID) clinically resembles Hirschsprung's disease but is characterized by hyperplasia rather than aganglionosis of the intramural plexus. Surgical intervention is common. We report the 5-year follow-up of an infant with the mixed form of NID managed medically and a method by which NID can be quantified histologically. Hyperganglionosis was determined by counting the number of ganglia per high-power field and the number of ganglion cells per ganglia from at least two biopsy specimens. The patient's biopsies and biopsies from "normal" and "inflamed" patients were compared. Normals contained 0.68 +/- 0.28 (mean +/- SD) ganglia per high-power field and 2.16 +/- 0.31 ganglion cells per ganglion. The inflamed biopsies were similar, 0.69 +/- 0.38 ganglia per high-power field and 2.63 +/- 0.40 ganglion cells per ganglion. The patient's initial rectal biopsy revealed 7.6 ganglia per high-power field and 3.8 ganglion cells per ganglion. Management of the patient included saline colonic irrigations and hyperalimentation with gradual reinstitution of breast-feeding. Clinical improvement was associated with normalization of manometry and biopsy findings, a phenomenon not documented previously in the literature. Irrigations were stopped at age 9 months, and the child is now asymptomatic.

Biopsy↗

Does proctosigmoiditis in inflammatory bowel disease presage the imminent onset of symptoms?

This study was undertaken to determine if asymptomatic children and adolescents with inflammatory bowel disease and moderate to severe anorectosigmoid inflammation might remain symptom-free for at least 12 months without specific intrarectal therapy. We prospectively studied 13 asymptomatic patients 6-21 years of age (four with Crohn's disease and nine with nonspecific colitis) with previously documented anorectosigmoid inflammation. Of these 13, four had moderate to severe anorectosigmoid inflammation both endoscopically and histologically. These four patients (two with Crohn's disease and two with nonspecific colitis) were entered into the second phase of the study. Three were receiving sulfasalazine, and one received methylprednisolone, 4 mg/day, and 6-mercaptopurine, 50 mg/day. None received intrarectal therapy. Clinical evaluation revealed that all four remained asymptomatic for 12 months despite the continued presence of moderate to severe anorectosigmoid inflammation. These results indicate that in children and adolescents with inflammatory bowel disease, the presence of inflammation of the anorectosigmoid does not necessarily correlate with or presage the onset of symptoms of proctosigmoiditis. Therefore, active inflammation of the anorectosigmoid is not the sole prerequisite for intrarectal therapy. The clinician should be guided by the symptoms of the patient, not by the presence or absence of active anorectosigmoid inflammation.

Adolescent↗

Amyloidosis in children with inflammatory bowel disease.

Reactive systemic or secondary amyloidosis occurs in 1-29% of adults with Crohn's disease, but only sporadic cases of amyloidosis have been recognized in children with inflammatory bowel disease. We therefore have studied operative specimens (ileal, ileocolonic, and colonic) from 46 children (30 with Crohn's disease and 16 with ulcerative colitis) to determine the frequency of amyloid deposits. Sections of bowel, skin, and lymph nodes (n = 940) were stained by Congo red and examined by light microscopy and by polarized light. Amyloid deposits were found in only one of 46 subjects, an 18-year-old girl who had had Crohn's disease for 6 years. Intestinal amyloid deposits, present 16 months before the clinical diagnosis of amyloidosis, were patchy and seen predominantly in the intestinal mucosa. We conclude that amyloidosis is rare in children requiring surgery for Crohn's disease and ulcerative colitis. Examination of Congo red-stained sections can detect even subclinical amyloidosis. The amyloid deposits in our patient, which were both patchy and consistently mucosal, suggest that multiple endoscopic biopsy samples, not necessarily containing submucosa, are sufficient for diagnosis.

Adolescent↗

Granuloma collagenase and EDTA-sensitive neutral protease production in hepatic murine schistosomiasis.

Mice infected with Schistosoma mansoni represent a model for study of hepatic fibrosis in humans. Production of trypsin-activatable inactive collagenase and EDTA-sensitive neutral protease was measured in the culture medium in which granuloma explants or primary cultures were maintained. Collagenase production was maximal in granulomas obtained from liver of mice 8 weeks postinfection and was inhibited by Actinomycin D or cycloheximide, and enhanced by lymphocyte factor(s) or heparin. Isolated schistosome eggs did not release these enzymatic activities but did release EDTA-insensitive protease activity. Both enzymes were separated by ion-exchange chromatography and purified to homogeneity. Isolated collagenase had an isoelectric point of 6.2 and molecular weight of 60,000 and had the functional characteristics of a tissue collagenase. The specific activity of collagenase was 33 units per mg protein at an optimum pH 7.5 and lacked proteolytic activity against noncollagenous protein substrates. Isolated EDTA-sensitive neutral protease had specific caseinolytic activity of 150 units per mg protein and gelatinolytic activity of 300 units per mg protein at an optimum pH 7.5; the enzyme lacked activity against undenatured collagen. Isoelectric point was pH 6.0. Protease activity was inhibited by known inhibitors of collagenases. Production and activation of EDTA-sensitive neutral protease and collagenase accompany increased collagen synthesis and content in the liver of mice 8 weeks postinfection with S. mansoni cercariae. Continued accumulation of liver collagen under these conditions suggests an insufficiency in collagenase activity relative to the increase in collagen synthesis.

Animals↗

Constraint-induced movement therapy for hemiplegic children with acquired brain injuries.

OBJECTIVE: To evaluate the feasibility and efficacy of constraint-induced movement therapy (CIMT) for impaired upper extremity (UE) function in children with acquired brain injury (ABI). DESIGN: Multiple case studies. SETTING: Inpatient pediatric rehabilitation. PARTICIPANTS: Seven consecutive ABI rehabilitation admissions with hemiparesis were recruited without regard to injury etiology, age, or cognitive capacities. MAIN OUTCOME MEASURE: The actual amount of use test (AAUT) was used to evaluate change in UE function. AAUT amount of use (AOU) and quality of movement (QOM) scales were obtained at baseline and follow-up. RESULTS: AOU and QOM item improvements were significant, as were changes in activities of daily living. The effect sizes for these changes were large. CONCLUSIONS: Stringent CIMT training, previously only implemented with adults, can be used effectively with children when everyday elements of a child's life are integrated into adult protocols. The use of child-friendly UE shaping exercises, "pushed into" activities by professional therapists as well as trained teachers, paraprofessionals, and parents, was supported. Effects of impairment, injury, and behavior on outcomes are discussed. Larger controlled studies with additional outcome measures are indicated.

Adolescent↗