Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019270 (hernia)
 15,856 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Trends are changing in the management of infants and children with indirect inguinal hernias. Advances in neonatal intensive care have resulted in the survival of many small premature infants who have a high incidence of inguinal hernia. The rate of incarceration, strangulation, and gonadal infarction in these babies is twice that of the general pediatric age group. Respiratory immaturity, apnea, bradycardia, and associated neonatal conditions require special management at the time of hernia repair, usually performed just before discharge from the neonatal intensive care unit. New information concerning volume loss and depletion of germ cells beginning at 6 months of age in boys with undescended testes has stimulated the performance of orchiopexy when the patient is 1 year of age. More than 90% of boys with cryptorchid testes at the age of 1 year have an associated hernia that requires concomitant repair at the time of orchiopexy. The use of the peritoneal cavity for fluid absorptive purposes in hydrocephalus treated by venticuloperitoneal shunts or of peritoneal dialysis for renal failure and metabolic diseases such as hyperammonemia and lactic acidosis causes increased intraabdominal pressure and results in the appearance of a previously unrecognized hernia. Recognition of these and other conditions associated with a high incidence of hernial occurrence should allow early diagnosis and treatment before the development of complications. Most elective repairs of hernias are safely performed in the outpatient setting; however, some infants and children with concurrent illnesses are best managed in a "morning admissions" program, in which hospital admission occurs postoperatively.
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PMID:Current concepts in inguinal hernia in infants and children. 257

Pyruvate dehydrogenase complex (PDHC) deficiency is known to cause congenital lactic acidosis. The case of a 9-month-old female infant with PDHC deficiency caused by a mutation in exon 11 of the pyruvate dehydrogenase (PDH) E1 alpha gene is described. Her facial features were as follows: frontal bossing, upslanting palpebral fissures, a short upturned nose, a long philtrum and low set ears. These anomalies are characteristic not only of a malformation syndrome or chromosomal aberration, but also of PDHC deficiency. Because PDHC deficiency requires early treatment, metabolic disorders should be kept in mind in a patient with dysmorphic features. Further, she had multiple minor anomalies including bilateral inguinal herniae, an umbilical hernia and small hands and feet, which have not been described in previous reports.
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PMID:Pyruvate dehydrogenase complex deficiency with multiple minor anomalies. 914 Dec 61

Acute Budd-Chiari syndrome is a rare condition characterised by obstruction of hepatic venous outflow. We describe the case of a 52-year-old man, with a congenital Morgagni diaphragmatic hernia, who presented with acute onset abdominal pain, shortness of breath, lactic acidosis, hyperbilirubinaemia and transaminasaemia. Computed tomography revealed strangulation of the diaphragmatic hernia and extrinsic compression of the inferior vena cava from the herniated viscera. Emergency surgery was carried out to repair the hernia with a biosynthetic mesh, with complete resolution of the Budd-Chiari syndrome.
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PMID:Acute Budd-Chiari syndrome caused by inferior vena cava compression from a congenital diaphragmatic hernia. 3253 5