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Necrotizing enterocolitis following operation in the neonatal period.

Over a 16-year period, 33 newborn infants developed necrotizing enterocolitis (NEC) following some form of operation (accounting for 19 per cent of all neonates with NEC), including repair of myelomeningocele, intestinal atresia, gastroschisis and congenital diaphragmatic hernia. The neonates with postoperative NEC had a median birth-weight of 3.05 (range 1.0-4.4) kg, and median gestational age of 40 (range 27-41) weeks. Twenty patients were treated non-operatively. The remaining 13 required operation for complications of NEC. Twenty-three patients are alive and have been followed for a median of 66 (range 3-168) months. If a neonate deteriorates following any operation NEC must be included in the differential diagnosis.

Age Factors↗

Teratogenicity of adriamycin and daunomycin in the rat and rabbit.

The antitumor drugs adriamycin and daunomycin were evaluated for effects on embryonal and fetal development in the rat and rabbit. Doses of adriamycin ranging from 1-2 mg/kg/day or daunomycin ranging from 1-4 mg/kg/day were administered ip to pregnant rats on days 6-15, 6-9, 9-12 or 12-15 of gestation. Both drugs were teratogenic in the rat, particularly when administered on days 6-15 or 6-9 of gestation. Relatively few anomalies resulted from treatment on days 9-12 or 12-15. On a mg/kg basis, adriamycin was the more potent teratogen, producing major anomalies at doses as low as 1.25 mg/kg. Similar anomalies, but at a lower incidence, were produced by daunomycin at dose levels of 4 mg/kg. Characteristic malformations included esophageal and intestinal atresia, tracheo-esophgeal fistula, hypoplasia of the urinary bladder and various cardiovascular anomalies. Neither drug was teratogenic when given iv to rabbits at doses up to and including 0.6 mg/kg/day on days 6-18 of gestation, but a high incidence of abortion occurred in rabbits treated with adriamycin.

Abnormalities, Drug-Induced↗

Continuous extracorporeal stool-transport system: a new and economical procedure for transitory short-bowel syndrome in prematures and newborns.

Between May 1994 and June 1995, nine newborns underwent surgery due to mechanical ileus or intrauterine perforation of the small bowel. Three were very-low-birth-weight infants weighing between 520 and 1,200 g. Surgery was performed in the first 2 days of life and split ileo- or jejunostomas were implanted. Early oral nutrition was initiated. To avoid non-use of the distal bowel and short-bowel syndrome, the aboral stoma was irrigated a few days later with the proximal feces. A new technique was applied to transport the chyle continuously from the oral to the aboral stoma: the stool was collected in an especially constructed stoma bag and transported distally by a roller pump. No major complications were seen. The general outcome was excellent in all cases, and reanastomosis under optimal bowel conditions was achieved in all patients without further problems.

Feces↗

Altered intestinal development after jejunal ligation in fetal sheep.

Experimental obstruction of the fetal small intestine resulted in massive hypertrophy of the segment proximal to the site of obstruction. Villus morphology was grossly abnormal. Enterocytes developed many irregular features, most notably cytoplasmic extensions (pseudopods, or blebs) from their apical surface. Distal to the site of obstruction, morphological anomalies which resembled those seen after experimental oesophageal ligation were found. These included delayed disappearance of the apical endocytic network, disrupted or absent microvilli, glycogen accumulation and inappropriate cell extrusion. Proximal to the obstruction, where stasis of swallowed fluid occurs, distension and abnormal intestinal development ensues. Distal to the obstruction where the intestine develops in the absence of swallowed fluid, development is also abnormal. The anomalies resemble those noted after oesophageal ligation in utero, and possibly are the results of reduced cellular nutrition. These results suggest that fetal ingestion provides the developing gastrointestinal tract with an important stimulus for normal growth.

Animals↗

Oral absorption of tacrolimus in children with intestinal failure due to short or absent small bowel.

We describe two children with intestinal failure due to short or absent small bowel who underwent isolated liver transplantation for liver disease related to parenteral nutrition. Both received reduced-size liver grafts whilst awaiting a suitable small bowel donor. Immunosuppressive therapy was based on oral tacrolimus and intravenous steroids. Therapeutic levels of tacrolimus were achieved at low dosage of 0.14-0.28 mg/kg per day. Median and mean blood tacrolimus levels were 9.9 and 13.7 ng/ml (range 4.9-42.3 ng/ml) in case 1 and 5.8 and 7.2 ng/ml (range 1-30 ng/ml) in case 2 before small bowel transplantation, respectively. Following small bowel transplantation, levels were 17.1 and 20.1 ng/ml (range 9.2-30 ng/ml), with oral doses of 0.54-1.35 mg/kg per day. Both children died of adenovirus pneumonia, with functioning grafts. Our experience demonstrates that effective levels of immunosuppression can be achieved by oral administration of tacrolimus in children with short or absent small bowel.

Adenoviridae Infections↗

Prenatal MR imaging of a meconium pseudocyst extending to the right subphrenic space with right lung compression.

Meconium pseudocyst results from a loculated inflammation occurring in response to spillage of meconium into the peritoneal cavity after a bowel perforation. Certain cystic lesions, such as abscesses and dermoid and epidermoid cysts, are known to show reduced water diffusion on DWI. MRI has recently become a valuable adjunct to ultrasonography for fetal gastrointestinal anomalies. Complementary to ultrasonography, prenatal MRI can help further characterize the lesion and can clearly demonstrate the anatomical relationship between the lesion and adjacent organs. We report a case of meconium pseudocyst that was prenatally imaged with ultrasonography and MRI, postnatally complicated by pneumoperitoneum, and proved by postnatal surgery and histopathology. We emphasize the MRI of the pseudocyst, particularly T1-weighted and diffusion-weighted imaging.

Adult↗

Double bubble sign.

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Duodenal Obstruction↗

Total absence of the small bowel in a premature neonate.

We report here an interesting unique case of total loss of small bowel in the absence of associated malrotation or gastroschisis. Total loss of small bowel, acquired in utero in the absence of associated anomalies such as malrotation or gastroschisis, has not been previously reported. Several reports of congenital short bowel exist. However, this uncommon finding is typically associated with malrotation. Babies with this condition often present with functional intestinal obstruction. Several cases in which infarction of gastroschisis and autolysis of the bowel, followed by in utero resolution of the abdominal wall defect, have been reported as a cause of congenital absence of the small bowel. We present here the first report, to our knowledge, of an infant with total absence of the small bowel without gastroschisis or malrotation.

Fatal Outcome↗

Fetal varicella syndrome: disruption of neural development and persistent inflammation of non-neural tissues.

Primary varicella zoster virus (VZV) infection during pregnancy is rare. If it occurs between the 8th and 20th week of gestation, fetal varicella syndrome results in 1-2% of the fetuses. We report about a varicella infection that affected a pregnant mother in the 12th week of gestation. At 33 weeks, a premature girl was born with destruction of neurons in spinal cord, spinal ganglia and plexus myentericus, and secondary developmental disturbance including mummification of one arm and segmental intestinal atresia. The brain did not show any abnormalities. However, VZV DNA could be detected by PCR in tissues from the brain and spinal ganglia. Chronic necrotizing inflammation was found in the placenta, fetal membranes, and one ovary. These locations showed nuclear inclusions which by in-situ-hybridization were proven to be VZV derived. This case demonstrates that in the fetal age, 'neurotropism' of VZV signifies severe destruction but not necessarily persistent inflammation of neural tissue. However, due to the inefficient fetal immune system, inflammation can go on for weeks, preferentially in non-neural tissues.

Adult↗