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[Fortuitous discovery in echography of an isolated fetal intra-abdominal hyperechogenic mass. 87 cases].

Having seen 87 cases we will now attempt to refine the management to be carried out when intra-abdominal hyperechogenic masses are found in the fetus. Before the 20th week of amenorrhoea (47 cases) amniocentesis can be used to study the digestive enzymes to determine the fetal karyotype. The normal results for intestinal enzymes makes it possible to rule out fetal cystic fibrosis. Three karyotype abnormalities were found in this series. After the 20th week (40 cases) intestinal enzymes cannot be interpreted. The diagnosis of cystic fibrosis then must rely on Delta F 508 mutation; but the absence of this mutation does not exclude cystic fibrosis. When ultrasound signs of intra-abdominal hyper-echogenicity are found the diagnosis of cystic fibrosis should not be thought of first, because in this series the majority of fetuses who had this sign were born without any malformation. Four cases of cystic fibrosis that were confirmed have been found but equally there were other serious malformations, three chromosome abnormalities, four intestinal atresias, ten unexplained intra-uterine deaths and one case of biliary duct atresia.

Amniocentesis↗

[Neurovesical dysfunction in children with anorectal malformations].

Chronic renal failure remain the most significant cause of morbidity and mortality in patients with anorectal malformation. The urological anomalies associated with anorectal malformations are not only anatomical, but also functional, the latter being related to congenital neurovesical dysfunction. The neurovesical dysfunction found in children with anorectal malformations results from a possible association with spinal dysraphism. The authors carried out urodynamic evaluation on 6 patients operated on for anorectal malformation by posterior sagittal anorectoplasty. 3 children had normal bladder function, but 3 had neurovesical dysfunction (1 unstable bladder, 2 neuropathic bladder). It is concluded that in patients with anorectal malformations urodynamic investigations should be performed as a routine investigation of the urinary tract. Consequently, patients with lower urinary tract dysfunction should receive prompt treatment, including clean intermittent catheterization, to prevent or reduce secondary urologic morbidity, especially loss of renal function.

Adolescent↗

Jejunal diaphragm.

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Child, Preschool↗

[Vitamin B1 deficiency as cause of a severe lactic acidosis and ileus symptoms].

A 2-year-old boy presented with severe lactic acidosis and ileus symptoms after 3 weeks of parenteral nutrition. Diagnosis of thiamine deficiency was made only when further complications had occurred. This case shows the importance of sufficient substitution of thiamine during parenteral nutrition. In lactic acidosis of unknown etiology thiamine deficiency must be considered.

Acidosis, Lactic↗

[Meconium peritonitis. A fetal catastrophe].

A material of 21 cases of meconium peritonitis treated by operation from 1972-1987 is presented. In four cases, intraabdominal anomaly was suspected on the basis of antenatal ultrasonography. The mortality was 19%. 57% developed symptoms during the first 24 hours, one at the age of one month and this case history is presented. Thirteen had intestinal atresia, 14 intrauterine volvulus, 7 open perforation, 13 pseudocyst, 1 gastroscisis. 2 had cystic fibrosis. Two patients are still attending the outpatient clinic on account of minor sequelae. The remainder have been discharged.

Abnormalities, Multiple↗

Developmental pathogenesis of defects associated with prenatal cocaine exposure: fetal vascular disruption.

Prenatal exposure to cocaine is associated with a spectrum of defects that are secondary to interruption of blood flow to developing or previously developed structures. The defects include prenatal onset growth deficiency, prematurity, abruptio placentae, intercranial hemorrhage, nonduodenal intestinal atresia, limb reduction defects, urinary tract anomalies, and neurodevelopmental problems limited at present to the neonatal period.

Abnormalities, Drug-Induced↗

[Neonatal obstruction].

Neonatal obstruction is a radio-clinical syndrome, common to a wide range of malformative anomalies which alter the normal anatomy or function of the digestive tract at any level. The main etiologies are reviewed with emphasis on specificity due to embryology, topography and clinical presentation. It is relatively easy to diagnose neonatal obstruction and relate it to a definite cause. The successful treatment of such obstruction requires a pluridisciplinary collaboration of specialized teams.

Constriction, Pathologic↗

[Meconial peritonitis: conclusions based on 53 cases].

The study of 53 patients with neonatal meconium peritonitis (MP) over a twenty-two year-period, the largest surgically-treated series at a single centre, is presented. Three different types of MP were established: Generalized, Localized and Cystic. Prognosis and surgical techniques to be used were determined by each MP type. The following survival rates achieved: for Generalized MP, 70% with "one stage" and 89% with "two stage"; for Localized MP, 80% with "one stage" and 100% with "two stage" and for Cystic MP, 57% with "two stage". The overall survival rate was 77%, rising to 85% in the last fifteen years, probably due to the advent of parenteral nutrition and advances in neonatal intensive care.

Cystic Fibrosis↗

Prenatal cocaine exposure and fetal vascular disruption.

The question of the potential teratogenicity of cocaine has been raised by the increasing frequency of its abuse in the United States. In previous studies, an increased incidence has been documented of spontaneous abortion, placental abruption, prematurity, intrauterine growth retardation, and neurologic deficits in the infants of women who abused cocaine. More recently, it has been suggested in studies that fetal vascular disruption accompanying maternal cocaine abuse may lead to cavitary central nervous system lesions and genitourinary anomalies. In this article, 10 children born of women who abused cocaine are described, 9 of whom have congenital limb reduction defects and/or intestinal atresia or infarction. The spectrum of anomalies associated with embryonic and fetal vascular disruption accompanying maternal cocaine abuse is thus enlarged. The specific risk for congenital anomalies accompanying maternal cocaine abuse during an individual pregnancy is unknown. However, data from these patients and the available literature suggest that counseling pregnant women concerning cocaine use should incorporate warnings about the possibility of associated embryonic or fetal vascular disruption.

Abnormalities, Drug-Induced↗

[Initial measures in newborn infants with surgically correctable abnormalities].

In newborns with surgically correctable malformations the quality of primary care has considerable influence on the results of the eventual surgical repair. This is particularly true for extensive diaphragmatic and abdominal wall defects as well as for esophageal and intestinal atresia, exstrophy of the bladder and urogenital malformations. The neonatal management comprises measures generally valid for all diseased newborns and measures specific for the particular malformation. The general care concerns heat regulation, fluid balance and other vital functions. Specific regimens are necessary as primary care for diaphragmatic hernia, esophageal atresia, abdominal wall defects and neonatal bowel obstruction. Furthermore this primary management must be extended to include the care of the pregnant mother. Already in utero infants with diaphragmatic and esophageal malformations should be referred to the gynecological-neonatal center.

Abnormalities, Multiple↗

[Surgical treatment of congenital defects of the jejunum and ileum in infants].

The examinations were based in the material of the Clinic of the Children's Surgery of the M.A. in Lublin of 16 newborns and babies operated because of the congenital defects of the jejunum and ileum. Among the patients there were 9 with the intestinal occlusion or with the stenosis, 5 with the diverticulum Meckel's or too long existing umbilical duct and 2 with the foramen of the mesentery. The results of the treatment of these defects dependent on the method of the operation. Good results of the operation were obtained by opening of the abdominal cavity with the medial incision, resection of the dilated intestine, funnel-shaped stenosis of the afferent ansa and anastomosis of one end to end or the end to back.

Constriction, Pathologic↗

[Apple peel small bowel: surgical correction and post-operative treatment].

The authors describe 6 cases of Apple Peel Small Bowel observed in the last ten year experience in Institute G. Gaslini of Genoa, Italy. They make a precision on early diagnosis and on surgical treatment, which is original, and also on post-operatory medical treatment. The importance of making an optimal nutritional support with total and partial parenteral nutrition for long time is of great significance to have a better outcome and psycho-physical development in such patients.

Female↗

Neonatal intestinal obstruction.

Our experience in the management of 138 infants with various causes of intestinal obstruction has provided us with important principles of diagnosis and treatments that we adhere to in the course of our practice. 1. The overall mortality of neonates suffering from intestinal obstruction should be fewer than 5 per cent. The high survival rate is accounted for by improved care these patients receive in specialized units. 2. Prematurity did not appear to play a significant factor in the outcome of these infants with intestinal obstruction. Our experience shows that premature infants tolerate operative procedures well, even in those instances in which an associated surgically correctable lesion is simultaneously repaired. 3. The major risk factor in any neonate with intestinal obstruction is the delay in diagnosis and operative intervention, especially in infants diagnosed to have midgut volvulus. The additional second risk factor is the association of chromosome abnormality. 4. Traditional diagnostic studies such as plain films of the abdomen supplemented by either an upper GI or lower GI contrast study for specific indications have been very effective in obtaining an accurate diagnosis of intestinal bowel obstruction. 5. Hirschsprung's disease can be diagnosed in the neonatal period if the index of suspicion for this is high. 6. Special surgical techniques as described should be used whenever indicated to minimize morbidity. 7. The traditional Wangensteen-Rice evaluation of a patient with imperforate anus is accurate, and specialized studies should be deferred for the postoperative period. Collaborative care provided by the neonatologist, pediatric anesthesiologist, and pediatric surgeon for these patients is the key to a favorable outcome.

Abnormalities, Multiple↗