Cases reported "Dehydration"

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1/24. Familial enteropathy: a syndrome of protracted diarrhea from birth, failure to thrive, and hypoplastic villus atrophy.

    We have studied 5 infants with persistent severe diarrhea from birth and marked abnormalities of absorption associated with failure to thrive leading to death in 4 infants. Three had siblings who died and a sibling of a 4th is ill at present, all with a similar illness; 2 were the products of consanguinous marriages. Exhaustive investigation failed to identify a recognized disease entity in any patient. steatorrhea, sugar malabsorption, dehydration, and acidosis were severe in all patients, whatever the diet fed. Total parenteral nutrition was used, but excessive stool water and electrolyte losses persisted even when nothing was fed by mouth. There was no evidence of a hematological or consistent immunological defect in any infant and no abnormalities of intestinal hormones were noted. In the duodenal mucosa of all infants we saw similar abnormalities characterized by villus atrophy, crypt hypoplasia without an increase in mitoses or inflammatory cell infiltrate in the lamina propria and in villus enterocytes absence of a brush border, increase in lysosome-like inclusions, and autophagocytosis. In 3 infants studied by marker perfusion of the proximal jejunum we found abnormal glucose absorption and a blunted response of Na absorption to actively transported nonelectrolytes; in 2 there was net secretion of Na and H2O in the basal state. Our patients evidently suffered from a congenital enteropathy which caused profound defects in their capacity to assimilate nutrients. The similar structural lesion seen in the small intestinal epithelium of all of our cases undoubtedly contributed to their compromised intestinal function, but the pathogenesis of this disorder, if indeed it is a single disease, remains obscure.
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2/24. infant hypernatremia: a case report.

    A 7-month-old infant presented to the emergency department with diarrhea, vomiting, and decreased activity. The infant was febrile, tachycardic, tachypneic, lethargic, and had a prolonged capillary refill. Initial serum sodium was 197 mmol/L. Ultimately, the infant was diagnosed with central diabetes insipidus complicated by severe dehydration secondary to rotavirus infection. A brief review of infant hypernatremia and its evaluation and treatment in the emergency department follows.
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3/24. Vasoactive intestinal polypeptide-secreting tumor (vipoma) with liver metastases: dramatic and durable symptomatic benefit from hepatic artery embolization, a case report.

    neuroendocrine tumors often manifest an excess production of hormones that create severe metabolic abnormalities resulting in significant patient morbidity, independent of the tumor burden itself. VIPomas are rare neuroendocrine tumors arising from the pancreas and are associated with secretory diarrhea and electrolyte disturbances. We present a patient with vipoma and hepatic metastases who had greater than 10 loose stools a day for 4 yr since diagnosis, despite debulking surgery, multiple antidiarrheal medications, large doses of octreotide, and targeted radioisotope injections. The patient required several hospitalizations for treatment of dehydration and electrolyte disturbances, despite receiving daily intravenous fluids at home. hepatic artery embolization (HAE) immediately stopped the patient's diarrhea and provided a return to normal formed stools without any other symptom-support measures. One year after HAE, the patient remains asymptomatic and has returned to a productive life. HAE can be a very effective and durable treatment modality for patients with metastatic VIPomas (or other neuroendocrine tumors) and who are clinically symptomatic from the effects of hormone hypersecretion.
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4/24. Secretory diarrhea owing to a tape bezoar in a child with Hirschsprung's disease.

    bezoars can be composed of a multitude of materials and can present in a wide variety of ways. The authors describe a young boy who had secretory diarrhea caused by an ileal bezoar composed of surgical adhesive tape. This report expands the literature on causes and presentation of intestinal bezoars in children.
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5/24. Use of clonidine to decrease intestinal fluid losses in patients with high-output short-bowel syndrome.

    BACKGROUND: Some patients who have short bowel syndrome (SBS) have high intestinal outputs, which increases the risk of dehydration and fluid-electrolyte abnormalities and impairs quality of life. methods: We evaluated the use of clonidine, an alpha2-adrenergic receptor agonist, in 2 patients with SBS and high intestinal outputs, who were refractory to conventional therapy with antidiarrheal and antisecretory agents (loperamide, belladonna, opiates, somatostatin, histamine2 receptor antagonists and proton pump inhibitors). The first patient (case 1) was a 29-year-old woman who had 175 cm of small bowel anastamosed to 8 cm of descending colon, ending in a colostomy. The second patient (case 2) was a 22-year-old man who had 30 cm of jejunum anastomosed to 30 cm of sigmoid colon and rectum. RESULTS: The addition of clonidine, 0.1 mg per os (PO) 2 times per day, to the treatment regimen of case 1 decreased her ostomy output from approximately 4 L/day to approximately 1 L/day, eliminating the need for parenteral nutrition and fluids and decreasing the need for opiate therapy. The addition of clonidine, 0.2 mg PO 2 times per day in case 2 resulted in decreased rectal outputs from approximately 4 L/day to approximately 1.5 L/day. CONCLUSIONS: These results demonstrate that clonidine can effectively reduce intestinal fluid and electrolyte losses and should be considered as an additional treatment option in patients with SBS and high intestinal outputs.
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6/24. Butyrate as an effective treatment of congenital chloride diarrhea.

    BACKGROUND & AIMS: Many therapeutic attempts have demonstrated to be ineffective in reducing the severity of congenital chloride diarrhea and its long-term complications. The short-chain fatty acid butyrate stimulates intestinal water and ion absorption through a variety of mechanisms, including the activation of a parallel Cl-/butyrate and Na /H exchanger. In this case report, we report the therapeutic efficacy of butyrate on an 11-year-old patient affected by congenital chloride diarrhea. methods: The efficacy of increasing doses of oral butyrate (from 50 to 100 mg/kg/day) was investigated through the daily evaluation of stool volume, bowel movements, fecal incontinence, serum, and stool electrolytes concentrations. The modifications in transepithelial intestinal ion transport elicited by butyrate were examined by rectal dialysis study. RESULTS: A butyrate dose of 100 mg/kg/day induced a normalization of stool pattern and of serum and fecal electrolytes concentration. The rectal dialysis study demonstrated a proabsorptive effect induced by butyrate on Na , Cl-, and K intestinal transport. Butyrate therapy was well tolerated during the entire 12-month observation period, and the stool pattern and fecal and serum ion concentrations remained stable within the normal ranges. No clinical adverse events or episodes of dehydration requiring hospital care were observed. CONCLUSIONS: Butyrate could be effective in treating congenital chloride diarrhea. It is easily administered, useful in preventing severe dehydration episodes, and may be a promising therapeutic approach for a long-term treatment in this rare and severe condition.
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7/24. Practical guide to intestinal rehabilitation for postresection intestinal failure: a case study.

    After massive small-intestinal resection or combined small-intestinal and colonic resection, diarrhea with resulting dehydration, electrolyte abnormalities, and malnutrition occur. Many patients become dependent on IV fluids and nutrition. An adaptation process manifested clinically by decreased diarrhea and improved nutrient absorption according to decreased parenteral nutrition and fluid requirements has been noted to occur over time. In some patients, adaptation is inadequate and may require special techniques to enhance and augment this process. This is a case of a 52-year-old woman who experienced increased stoma output 1 week after major intestinal resection, resulting in dehydration. She required IV fluids in order to maintain hydration. After the initiation of an intestinal rehabilitation program, which included modified diet, soluble fiber, oral rehydration solution (ORS), and medications, IV fluids were successfully weaned off in 3 months. She continues not to receive IV fluids and continues to follow the intestinal rehabilitation plan.
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keywords = diarrhea
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8/24. Rice protein-induced enterocolitis syndrome.

    BACKGROUND AND AIMS: food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated allergic reaction, usually to cow's milk or soy protein. The aim is to present a series of infants with enterocolitis syndrome developed after rice ingestion. To our knowledge, the issue has only once been described in patients from europe. methods: Data on five patients were retrospectively analyzed. The patients presented with vomiting, diarrhea and dehydration; therefore, allergy was not considered as a diagnosis and the patients underwent broad diagnostic evaluation. Finally, an open food challenge was performed for suspicion of rice allergy. RESULTS: Detailed clinical and laboratory findings are presented. As all patients had symptoms which could have been indicative of a broad spectrum of diseases, a median of two hospitalizations was needed to establish the diagnosis. When the rice protein-induced enterocolitis syndrome is suspected all patients should undergo open food challenge test, as no other diagnostic procedure can confirm the diagnosis. CONCLUSION: This report shows that even hypoallergenic foods such as rice may cause FPIES and should be considered in the differential diagnosis of profuse vomiting and prostration in infants introduced to some kind of rice protein.
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9/24. Osmotic demyelination and hypertonic dehydration in a 9-year-old girl: changes in cerebrospinal fluid myelin basic protein.

    A 9-year-old girl was admitted for the treatment of hyper-natremic dehydration. Her history was significant for psychogenic polydipsia, hyponatremia, and a renal concentrating defect. She presented with a 2-day history of altered mental status, ataxia, lethargy, fever, nausea, vomiting, and diarrhea. meningitis was ruled out. Over the course of her illness, slow rehydration was maintained with a gradual decrease (10 mEq per 24 hours) of the serum sodium. Despite this care, she developed quadriparesis, and magnetic resonance imaging performed on day 6 of her illness was consistent with osmotic demyelination (central pontine myelinolysis). To rule out an excessively rapid correction of hypernatremia as the etiology of the problem, a myelin basic protein was measured in the cerebrospinal fluid that had been obtained on hospital day 1. The myelin basic protein was 649.50 ng/mL (normal, 0.07-4.10 ng/mL). The current literature is presented regarding the postulated pathogenesis of central pontine myelinolysis and suggested therapies, previous reports of central pontine myelinolysis in children are reviewed, and the potential role of myelin basic protein in its diagnosis is discussed.
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keywords = diarrhea
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10/24. Hyponatremic dehydration as a presentation of cystic fibrosis.

    BACKGROUND: The purpose of this study is to present a case report of a child with hyponatremic dehydration diagnosed after CF and to review the cases of 13 patients with CF who had the same initial presentation in our hospital. methods: This report reviewed the clinical records of children diagnosed with CF to ascertain the prevalence of metabolic alkalosis with electrolyte depletion as the presentation of CF. It also used sweat tests to diagnose a child with CF. RESULTS: The laboratory tests of a 12-month-old girl presented 3 times to the ;pediatric emergency department with vomiting and weight loss showed hyponatremia, hypochloremia, and metabolic alkalosis. The patient was subsequently diagnosed with CF by means of 2 positive sweat tests. Meanwhile, the review of the clinical records of all children diagnosed with CF from 1985 to 2004 (N = 77) showed that the prevalence of metabolic alkalosis with electrolyte depletion as the presentation of CF was 16.8%. The age of the infants ranged from 3 to 14 months. All episodes took place during summer. CONCLUSIONS: There are not many causes of metabolic alkalosis with hyponatremic dehydration, and one of them is CF. This report emphasizes sodium depletion as a common sign of CF presentation. This is most important in countries where the neonatal screening test for CF is not available because the disease may be asymptomatic or oligosymptomatic for several months or even years. cystic fibrosis should be considered in differential diagnosis of any child presenting with unexplained hyponatremic dehydration.
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