Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021843 (bowel obstruction)
 9,927 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of hepatic encephalopathy in a noncirrhotic child, who has undergone a previous mesocaval shunt for extrahepatic portal vein thrombosis, is presented. Hepatic encephalopathy developed 5 years after the operation and is believed to have been precipitated by the presence of small bowel obstruction. Exploratory laparotomy and lysis of an adhesion relieved the obstruction and led to the resolution of the encephalopathy.
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PMID:Hepatic encephalopathy induced by small bowel obstruction in a noncirrhotic child with portal vein thrombosis. 228 6

There were 122 deaths among 803 children registered, randomized, and followed in the second National Wilms' Tumor Study; 17 occurred in children apparently free of disease and were attributable to causes other than tumor progression. Seven deaths were attributed to infection during periods of drug-induced leukopenia; four were due to liver failure; and one each was attributable to radiation pneumonopathy, intestinal obstruction, renal failure, myocardial disease, and encephalopathy. The cause of one death was unexplained. Of particular concern were four (of 47) infants under one year of age with group I or II disease who had toxic deaths. Subsequent to these experiences the doses of all chemotherapeutic agents were reduced by 50% for infants under one year of age. No deaths from toxicity were observed thereafter in infants. An analysis of the therapeutic effect of this dose reduction showed three of 47 relapsed on full dose and five of 54 on half dose. The difference is not statistically significant. This report is a further demonstration of the potentially serious vulnerability of infants to standard doses of anticancer drugs even when they are calculated on a per kilogram basis.
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PMID:Toxic deaths in the Second National Wilms' Tumor Study. 608 9

The treatment of portal hypertension in the pediatric population has undergone an evolution toward less invasive methods of care. With the advent of endoscopic sclerotherapy, surgery is less common in the acute care of these patients. Few reports deal with the role of portosystemic shunting in the emergent management of variceal hemorrhage in children. To address this issue, the authors studied the medical records of all pediatric patients at their institution who underwent placement of a shunt for portal hypertension during the last 10 years. Nine patients underwent a total of 10 emergent or semiurgent shunting procedures. Seven were boys and two were girls. Six patients had portal hypertension as a result of intrahepatic disease. Two had extrahepatic portal vein thrombosis. Five children had abnormal hepatic function. The median age at the time of the procedure was 9 years. The indication for surgical shunting in all cases was gastrointestinal hemorrhage not responsive to sclerotherapy. Eight patients underwent emergent distal splenorenal shunts (DSRS), and two underwent a nonselective mesocaval shunt, with one undergoing both. Postoperatively all patients had cessation of bleeding. Operative mortality was zero. Early complications included ascites (3), small bowel obstruction (1), and hepatorenal syndrome (1). The child who underwent a nonselective shunt procedure had encephalopathy. Two DSRS thrombosed, requiring reexploration; eight shunts remained patent. Three patients eventually had orthotopic liver transplantation (OLT) because of progressive hepatic failure. Two children died; neither death was shunt related.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Selective distal splenorenal shunts for intractable variceal bleeding in pediatric portal hypertension. 747 61

The files of 25 patients with propionic acidemia (PA), followed by the Inborn Errors of Metabolism Service (IEMS) at King Faisal Specialist Hospital and Research Centre (KFSH & RC) from 1990 to 1993, were studied retrospectively. In 14 patients PA presented acutely with acidosis, hyperammonemia and thrombocytopenia, while in 11 patients the presentation of the disease was unusual. In the latter group, two neonates with PA initially appeared as a primarily hyperammonemic metabolic disease. In two other neonates the vomiting was so severe that they were diagnosed as intestinal obstruction in referral hospitals. The presentation in three infants was primarily as an immune disorder. In four infants, PA appeared as an acute or chronic encephalopathy, i.e. as a silent organic acidemia, with few other findings of the disease. The clinical picture of PA includes facial and nipple dysmorphia, severe hypotonia and vomiting. Severe thrombocytopenia is the hallmark of the metabolic crisis. In one patient it was noticed late and caused intracranial hemorrhage, while in three others intracranial bleeding caused death. The prognosis in PA remained grave despite rigorous treatment. Only seven of the 25 PA patients remained to have a normal life-style, while eight patients expired. The diagnosis is readily achieved by urine gas chromatography/mass spectrometry (GC/MS), by tandem mass spectrometry (MS/MS), or by enzyme analysis of fibroblasts. While there may be both examiner- and patient-related reasons for the variations in the presentation of PA, one other reason may be the heterogeneity of the molecular defect in propionyl-CoA carboxylase.
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PMID:Unusual presentations of propionic acidemia. 772 81

Shigella dysenteriae type 1 causes the most severe form of bacillary dysentery. The spectrum of illness ranges from mild watery diarrhoea to severe bloody diarrhoea. Shigellosis is often associated with intestinal complications, including intestinal perforation, intestinal obstruction, toxic dilatation of the colon, and prolapse of the rectum; systemic complications include septicaemia, hyponatraemia, hypoglycaemia, seizure, encephalopathy, haemolytic-uraemic syndrome, and malnutrition. Arthritis and conjunctivitis are rare extra-intestinal complications of shigellosis. Annually, about 110,000 patients receive treatment in the Dhaka Hospital of the International Centre for Diarrhoeal Disease Research, Bangladesh for diarrhoea and diarrhoea-associated illnesses, of which 11% are due to shigellosis. However, arthritis associated with shigellosis has not been reported from this population. Arthritis has been reported in association with infection due to S. flexneri and S. sonnei from other places. We are unaware of any reported case of arthritis in association with S. dysenteriae type 1 infections. In this report, we describe the clinical and laboratory features of a young woman who developed arthritis following S. dysenteriae type 1 infection.
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PMID:Reactive arthritis associated with Shigella dysenteriae type 1 infection. 930 97

A boy of 20 months and a girl of 27 months who had previously undergone massive bowel resection due to congenital intestinal obstruction are described. During gradual reduction of total parenteral nutrition and initiation of oral feeding, both developed severe, acute metabolic acidosis, accompanied by encephalopathy and ataxia. After the laboratory identification of massive amounts of the d-isomer of lactic acid in urine and blood, both were successfully treated with i.v. bicarbonate, and metronidazole to suppress the overgrowth of colonic lactobacilli responsible for the metabolic crisis.
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PMID:[D-lactic acidosis in short bowel syndrome]. 1091 34

We report a case of a 40-year-old man presenting with relapsing encephalopathy 4 years post-intestinal transplantation. Each episode was preceded by symptoms suggestive of subacute intestinal obstruction, marked dehydration, and, on one occasion, grade 4 encephalopathy. Physical examination revealed hypertonia, clonus, and hyperreflexia. Biochemistry was consistent with renal impairment, metabolic alkalosis, hyperammonaemia, and normal liver function. Plain radiographs and abdominal computed tomography revealed dilated proximal small bowel loops, and barium radiography demonstrated a strictured distal anastomosis. Hydrogen breath testing indicated bacterial overgrowth. Following rehydration and antibiotic therapy, the patient recovered fully between episodes. Further episodes of encephalopathy did not recur following resection of the distal anastomotic stricture and resolution of bacterial overgrowth. Unfortunately, one year later the patient died of pneumonia. To the best of our knowledge, encephalopathy secondary to intestinal transplant related porto-caval shunt and bacterial overgrowth in strictured bowel has not been previously reported but might have implications for the management of future patients.
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PMID:Relapsing encephalopathy following small bowel transplantation. 1282 21

Congenital hypothyroidism (CH) is the most common endocrine disorder affecting the newborn. Universal newborn screening (NBS) has virtually eliminated the static encephalopathy and devastating neurodevelopmental syndrome known as cretinism. This report describes the presentation of an infant referred by the primary pediatrician to our hospital at 12 days of age for confirmatory testing after the NBS was consistent with CH. The infant had hypoglycemia secondary to lethargy and poor feeding and required transfer to the neonatal intensive care unit for worsening abdominal distension despite normalization of serum thyroid function tests following hormone replacement. In particular, the recalcitrant ileus and secondary bowel obstruction resulted in an additional diagnostic workup and lengthened hospital day. Our report highlights the acute gastrointestinal consequences of hypothyroidism despite evidence of effective treatment. We believe that the preclinical detection and immediate therapy for CH have lessened the prevalence of this presentation in general practice, and hence practitioners are less likely to be familiar with its natural history and management.
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PMID:Prolonged ileus in an infant presenting with primary congenital hypothyroidism. 2586 93

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disorder characterized by hemolytic anemia, marrow failure, and a high incidence of life-threatening venous thrombosis. It is subject to a considerable variety of complications like intestinal obstruction and visceral embolism. The current study firstly presents a 40-year-old male with a previous diagnosis of PNH who developed posterior reversible encephalopathy syndrome (PRES) during treatment with methylprednisolone. He was referred to our department with headache and two episodes of generalized tonic-clonic seizures. Laboratory examination revealed peripheral blood cytopenias and elevated count of reticulocyte. Brain magnetic resonance imaging (MRI) exhibited abnormal signal in the bilateral parieto-occipital lobes with symmetric distribution which confirmed the diagnosis of PRES. After receive treatment of dexamethasone, anti-hypertensive and neurotropic drugs, the patient made a complete clinical recovery; and the abnormal signals of MRI were almost completely absorbed. This case shows that PRES might be a rare complication of PNH. Furthermore, it points out the necessity of rapid diagnosis and treatment of PRES.
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PMID:Posterior reversible encephalopathy syndrome following paroxysmal nocturnal hemoglobinuria: a case report and literature review. 2637 93

Posterior reversible encephalopathy syndrome (PRES) is a clinical-radiographic syndrome with seizures, headache, altered mental status and visual disturbances. It is typically associated with posterior cerebral white matter oedema on neuroimaging. There is an increasing number of cases of PRES reported with different chemotherapeutic protocols. However, PRES is rarely reported in association with irinotecan, fluorouracil and folinic acid (FOLFIRI). We report a 28-year-old female patient with a history of Stage IV gastric cancer who presented with abdominal pain and recurrent vomiting that was thought to be related to a partial intestinal obstruction secondary to peritoneal metastasis. Eventually, she was treated with FOLFIRI. A few hours after initiation of the fluorouracil infusion in the second cycle, she developed a tonic-clonic seizure. MRI of the brain showed multiple bilateral T 2 and flair hyperintense cortical and subcortical lesions suggestive of PRES. Other causes of PRES were excluded, as well as brain metastasis. Unfortunately, the patient developed septic shock and died a few days after her presentation.
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PMID:Posterior reversible encephalopathy syndrome while receiving irinotecan with fluorouracil and folinic acid for metastatic gastric cancer. 3036 10


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