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)

Congenital diaphragmatic hernia (CDH) is a common cause of severe respiratory distress in the newborn. However, the presentation of CDH in older children and adults is rare, and, therefore, little is known concerning its symptoms, operative management, and postoperative complications. Thirteen patients (age range: 2 months to 26 years; 5 males, 8 females) presented with CDH. Four patients had right-sided hernias, eight left-sided hernias, and one bilateral hernias. Symptoms included chronic respiratory tract infections in 6 patients, vomiting in 5, weight loss in 1, severe failure to thrive in 2, and severe respiratory distress in 3; one patient was asymptomatic. Physical signs included the absence of breathing sounds or bowel sounds in the chest in eight patients, hyperresonance in one, and cachexia in two. The diagnosis was confirmed in each patient by chest roentgenogram or gastrointestinal contrast radiograph. All patients underwent immediate repair. After reduction of the viscera, 12 of 13 patients underwent primary diaphragm repair, whereas one patient required a prosthetic diaphragm patch. Twelve of 13 patients (92%) survived. Postoperatively, 7 of the 12 survivors (58%) developed severe gastric atony, and four required further operative therapy. In contrast to newborns, CDH in the older child and adult is frequently seen on the right side, rarely presents with severe respiratory distress, and is occasionally asymptomatic. Postoperative gastric atony is a major cause of morbidity, making transabdominal repair with simultaneous pyloroplasty and/or feeding jejunostomy the preferred operative approach.
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PMID:Congenital diaphragmatic hernia beyond infancy. 167 Feb 42

Two female infants with Goltz syndrome (focal dermal hypoplasia) were recently investigated in the Department of Radiology, The Hospital for Sick Children, Great Ormond Street for severe feeding problems and failure to thrive. Both demonstrated severe skeletal malformations and marked gastrooesophageal reflux with laxity of the hiatus. One child (case 1) exhibited nasal regurgitation during feeding. Interestingly, both children had undergone surgery; Case 1 or a right parasagittal abdominal hernia associated with focal dermal hypoplasia of the abdominal wall and Case 2 for an exomphalos also associated with dermal hypoplasia. This observation suggests a more widespread mesodermal abnormality.
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PMID:The radiological features of Goltz syndrome: focal dermal hypoplasia. A report of two cases. 320 Dec 78

During a 20-month period 55 strains of Aeromonas species were isolated from 53 children with diarrhea. The isolation rate of 2.5% for Aeromonas compared with the rates of 4.5% for Shigella, 3.3% for Salmonella, 2.7% for Campylobacter and 0.05% for Yersinia. In 45 children Aeromonas was the sole bacterial enteropathogen identified. Aeromonas was also isolated from 2 (0.5%) of 380 asymptomatic children. Despite its known lack of identifiable virulence properties, Aeromonas caviae was the most prevalent species, accounting for 69% of the isolates. None of the A. caviae strains produced cytotoxin by the 51Cr release assay and 12.5% were weakly enterotoxigenic by the infant mouse assay. All of the Aeromonas sobria and 71% of Aeromonas hydrophila were positive for both toxins. Ninety-two percent of the children with Aeromonas-associated diarrhea were younger than 3 years; 84% of the cases were seen between May and October. The majority of the children had an acute onset of watery diarrhea. Fever and vomiting were most commonly associated with the isolation of A. sobria. Eight children had chronic or intermittent diarrhea lasting for weeks to months before consultation; A. caviae was the isolate in all these cases. Several complications possibly related to Aeromonas intestinal infection were observed. These included Gram-negative bacteremia, intussusception, internal hernia strangulation, hemolytic uremic syndrome and failure to thrive in patients with chronic diarrhea.
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PMID:Aeromonas-associated gastroenteritis in children. 334 Apr 60

Over a four year period, from August 1987 to July 1991, thirteen cases of chronic and recurrent gastric volvulus were encountered comprising six paediatric and seven adult patients. In none of the patients was the condition clinically suspected; diagnosis being made only at meticulous upper gastro-intestinal (UGI) barium series. The paediatric patients typically presented with obstructive symptoms of projectile vomiting especially after meals and failure to thrive. The adults had variable symptoms of dyspepsia, recurrent intermittent upper abdominal discomfort or pain, occasionally accompanied by vomiting or retching mimicking many different upper abdominal conditions, such as peptic ulcer, biliary tract or pancreatic disease but with negative findings at endoscopy and abdominal ultrasound scanning. All cases were organo-axial type of gastric volvulus. Associated conditions were small sliding hiatus hernia in two adult cases; partial small bowel malrotation in two cases, high jejunal obstruction also in two cases and congenital hip dislocation in one patient. An infant had umbilical hernia, previous meconium cyst and meconium peritonitis. The condition seems not as uncommon as previously thought; the key to diagnosis being constant awareness, a high index of clinical suspicion and a carefully performed UGI barium series especially during the attack of pain.
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PMID:Gastric volvulus: more common than previously thought? 765 6

As more infants with congenital diaphragmatic hernia (CDH) survive with extracorporeal membrane oxygenation (ECMO), it seems prudent to detail the longterm outcome in these medically complex infants. Eighteen children with CDH-treated with postoperative ECMO were recruited for participation in this study. The mean duration of ECMO was 193 hours (range 82 to 493 hours), mean time to extubation after ECMO was 142 hours (range 34 to 312 hours), and median duration of hospitalization was 46 days (range 30 to 181 days). Of the 18 infants, 4 (22%) were discharged home requiring oxygen therapy. At follow-up the notable findings were a high incidence of gastroesophageal reflux and failure to thrive. At both 1 and 2 years of age, 50% of infants were at less than the 5th percentile for weight. At 1 and 2 years of age, 39% and 21%, respectively, were at less than the 5th percentile for weight/length ratio. A total of 16 children (89%) had clinical evidence of reflux, and 8 (44%) were discharged home on a regimen of nasogastric feedings. Reherniation occurred in 4 children (22%) and was more frequent when a patch was used. An electrocardiogram showed right ventricular hypertrophy in 6 (43%); oxygen saturation by pulse oximetry was > 95% in all children, and pulmonary artery pressure was estimated by Doppler echocardiography to be normal in 12 of 14 children examined. The neurodevelopmental outcome (Bayley Scales or Stanford-Binet scale) at 1 to 4 years of age was not dissimilar from that of other ECMO-treated children. Given the severity of illness in the neonatal period, the general health and development of children with CDH surviving after ECMO are good. Surprisingly few children have long-term respiratory complications related to pulmonary hypoplasia. Follow-up in the first few years should be aimed at aggressive nutritional intervention to prevent the growth failure that appears to be prevalent in these children.
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PMID:Congenital diaphragmatic hernia: long-term outcome in neonates treated with extracorporeal membrane oxygenation. 850 65

Recent studies have suggested there may be a high incidence of pulmonary and extrapulmonary sequelae among infants who have undergone repair of congenital diaphragmatic hernia (CDH). The aim of this study was to identify factors that predict high-risk cases to facilitate counseling of parents. Morbidity, defined as conditions requiring treatment and/or hospitalization during follow-up, was documented. Only five of 15 cases that had been diagnosed antenatally (at 16 to 26 weeks' gestation) and underwent surgical repair after preoperative stabilization were without morbidity at the time of follow-up. Respiratory problems were identified in seven (chylothorax in 3, recurrent infections in 4) and failure to thrive in four; three infants required further gastrointestinal surgery. A comparison of infants with and without morbidity showed that the only significant difference between the groups was in the duration of respiratory support. A requirement for respiratory support for more than 10 days had 90% sensitivity, 100% specificity, and a positive predictive value for morbidity at follow-up of 100%.
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PMID:Prediction of morbidity during infancy after repair of congenital diaphragmatic hernia. 898 79

The use of a single lung transplant, modified with removal of the middle lobe of the donor right lung, has been described for a term neonate with respiratory distress secondary to right-sided congenital diaphragmatic hernia. The successful transplant allowed the patient to be successfully weaned from extracorporeal membrane oxygenation. Because of the early age of the patient at transplantation (3 weeks), it was unclear how the patient's left lung would develop, and there was uncertainty regarding the risk of life-time immunosuppression. By the age of 4 years, 10 months, she was demonstrating some failure to thrive, hypertension, and hirsutism, obvious side effects of chronic immunosuppression. The question was raised as to the potential for transplant pneumonectomy. A ventilation-perfusion scan demonstrated a decrease of right lung ventilation compared with the immediate postoperative period (27% versus 43%); right heart catheterization with balloon occlusion of the right main pulmonary artery suggested that the patient would tolerate right pneumonectomy. After discussion with the family, the patient underwent transplant pneumonectomy via a right posterolateral approach. Findings at the time of operation included mild to moderate adhesions as well as recurrence of the diaphragmatic hernia. She tolerated the procedure well and was discharged home on the fifth postoperative day with cessation of her immunosuppression. The immediate and medium-term success of this procedure suggests the potential for temporizing transplantation as a palliation to promote survival until the remaining native lung can provide sufficient ventilation.
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PMID:Elective transplant pneumonectomy. 957 74

A case of right diaphragmatic eventration, associated with a lung sequestration, in a 7-month-old infant is presented. Failure to thrive was the initial symptom. X-ray of the chest, made because of mild respiratory distress, revealed an inhomogeneous opacification of the right lower hemithorax, suggesting a diaphragmatic hernia. A right thoracotomy was performed and a diaphragmatic eventration covered by a thick pleuroperitoneal membrane was found, together with sequestration of the lung. The pathologic findings and the embryology are discussed.
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PMID:Right diaphragmatic eventration associated with intralobar pulmonary sequestration: a case report. 1087 86

An 18 months old boy presented with marked failure to thrive, abnormal facial grimacing and troublesome vomiting. The patient was diagnosed as having Schwartz syndrome with hiatal hernia. Medical and surgical treatment was carried out and with supportive care the patient gained weight and his symptoms subsided. In 8 months, however, the patient developed hernia on the other side necessitating repeat surgery. The case is being reported to highlight the accompaniment of hiatal hernia not previously reported as part of the syndrome and to report the experience of using muscle relaxants in the condition.
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PMID:Severe, recurrent hiatal hernia in Schwartz syndrome. 1268 82

In right congenital diaphragmatic hernia (RCDH), several clinical diagnostic pitfalls are possible and should be known to those caring for infants and children with this disorder. The records of the 18 patients at Hotel Dieu de France Hospital with a history of CDH between 1990 and 1999 were collected; those of the ten who had a RCDH were reviewed retrospectively. The mean age at diagnosis was 6 months; the male-to-female ratio was 2:3. The delay between the first symptom and the diagnosis ranged between 0 and 10.5 months (mean 4.5 months). An acute presentation was observed in four cases, consisting of respiratory distress in three; the 4th presented with gastric volvulus and intestinal obstruction. The presenting symptoms were mild in four cases; recurrent respiratory infections in three and failure to thrive in one. The diagnosis was incidental in two cases during the evaluation of respiratory symptoms attributed to an atrial septal defect. The radiologic findings provided by a chest radiograph (CxR) were sufficient to make an accurate diagnosis in eight cases and peritoneography was useful in one. In six cases, the presenting CxR had been misinterpreted as normal or acute lobar pneumonia. Pathologic findings at surgery consisted of lateral and posterior right diaphragmatic defects in nine cases; the defect was lateral and anterior in one. A hernia sac was found in seven cases; malrotation was present in three. Surgical correction was done by an abdominal approach in nine cases and a thoracic approach in one. The diaphragmatic defect was repaired by transverse closure in six cases, diaphragm plication in three and prosthetic closure in one. The postoperative outcome was uneventful in eight cases. Two patients died. Thus, RCDH seems to cause less severe symptoms than left-sided LCDH. It usually manifests beyond the neonatal period as respiratory or gastrointestinal symptoms. The diagnosis should be made easily by a CxR. The presence of a hernia sac correlated with a mild presentation. An abdominal surgical approach is preferred.
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PMID:Right congenital diaphragmatic hernia a well-known pathology? 1269 19


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