1/148. Distal neonatal intestinal obstruction: the choice of contrast material.The use of barium sulfate as the contrast agent of choice in the radiographic evaluation of distal neonatal intestinal obstruction is advocated. The advantages of Gastrografin or other water-soluble contrast materials are far outweighed by their disadvantages, which include the hazards of hypertonic dehydration and the danger of missing the diagnosis of Hirschsprung's disease. Five patients are presented, all of whom had the diagnosis of Hirschsprung's disease missed in the neonatal period with one use of Gastrografin enemas. All five were subsequently admitted to the Surgical Neonatal intensive care Unit, critically ill with enterocolitis of Hirschsprung's disease.- - - - - - - - - - ranking = 1keywords = obstruction (Clic here for more details about this article) |
2/148. Familial enteropathy: a syndrome of protracted diarrhea from birth, failure to thrive, and hypoplastic villus atrophy.We have studied 5 infants with persistent severe diarrhea from birth and marked abnormalities of absorption associated with failure to thrive leading to death in 4 infants. Three had siblings who died and a sibling of a 4th is ill at present, all with a similar illness; 2 were the products of consanguinous marriages. Exhaustive investigation failed to identify a recognized disease entity in any patient. steatorrhea, sugar malabsorption, dehydration, and acidosis were severe in all patients, whatever the diet fed. Total parenteral nutrition was used, but excessive stool water and electrolyte losses persisted even when nothing was fed by mouth. There was no evidence of a hematological or consistent immunological defect in any infant and no abnormalities of intestinal hormones were noted. In the duodenal mucosa of all infants we saw similar abnormalities characterized by villus atrophy, crypt hypoplasia without an increase in mitoses or inflammatory cell infiltrate in the lamina propria and in villus enterocytes absence of a brush border, increase in lysosome-like inclusions, and autophagocytosis. In 3 infants studied by marker perfusion of the proximal jejunum we found abnormal glucose absorption and a blunted response of Na absorption to actively transported nonelectrolytes; in 2 there was net secretion of Na and H2O in the basal state. Our patients evidently suffered from a congenital enteropathy which caused profound defects in their capacity to assimilate nutrients. The similar structural lesion seen in the small intestinal epithelium of all of our cases undoubtedly contributed to their compromised intestinal function, but the pathogenesis of this disorder, if indeed it is a single disease, remains obscure.- - - - - - - - - - ranking = 0.00655294194537keywords = duct (Clic here for more details about this article) |
3/148. Congenital dislocation of the hip in the newborn. A correlation of clinical, roentgenogrhic and anatomical findings.This study correlates roentgenography, arthrography, morbid anatomy and histology in four newborn infants with congenital dislocation of the hip. Three died shortly after birth. In two of these, manipulative reduction was impossible. Both demonstrated secondary adaptive changes at autopsy, indicating that the dislocation had been present for some time in utero. The first had a paralytic dislocation (meningomyelocele), and the second showed no pathologic findings other than the dislocated hip. The third infant had a dislocation that was reduced at birth. The child died of pneumonia on the 15th day. At autopsy, no secondary adaptive changes were found in the hip. The fourth infant, who had undergone open reduction because of a dislocation irreducible at birth, was retrospectively found to have an intrauterine dislocation when the maternal roentgenogram was reviewed. Significant secondary adaptive changes were noted at the time of operation.- - - - - - - - - - ranking = 0.01310588389074keywords = duct (Clic here for more details about this article) |
4/148. Mucous fistula refeeding in neonates with short bowel syndrome.BACKGROUND/PURPOSE: Neonates with enterostomies commonly suffer from a functional short bowel syndrome (SBS) and have a greater risk of electrolyte and fluid loss with poor weight gain. The authors describe their experience with refeeding stoma effluent into the mucous fistula in neonates. methods: A 5-year (1993 to 1997) chart review of neonates with stoma effluent refeeding was undertaken. Demographics, medical history, surgical procedures, timing, and duration of refeedings were reviewed. Enteral and total parenteral nutritional (TPN) requirements, electrolyte, and acid-base disturbances were recorded. RESULTS: Six neonates (gestational ages of 27 to 38 weeks, birth weights of 533 to 3400 g) were identified with nutritional or electrolyte complications before the commencement of refeeding. enterostomy indications included necrotizing enterocolitis (n = 2), intestinal atresia type 3b (n = 1), complications from ruptured omphalocoele (n = 1), congenital adhesive band obstruction (n = 1), and midgut volvulus after congenital diaphragmatic hernia repair (n = 1). weight gain during refeeding ranged from 5 to 25 g/kg/d with duration of refeeding lasting 16 to 169 days (two neonates were refed at home) until reanastomoses were done 6 to 44 weeks after the original surgery. There were no complications, and TPN requirements were diminished or eliminated. CONCLUSION: This technique represents a simple and safe method, which lessens the need for TPN and electrolyte supplementation in neonates with enterostomies and SBS before reanastomosis.- - - - - - - - - - ranking = 0.2keywords = obstruction (Clic here for more details about this article) |
5/148. Effectiveness of antiretroviral therapy initiated before the age of 2 months in infants vertically infected with human immunodeficiency virus type 1.The effectiveness and tolerance of antiretroviral therapy with a combination of three reverse transcriptase inhibitors starting at the time of diagnosis (before 2 months of age) was evaluated in four infants with vertically acquired hiv-1 infection. plasma hiv-1 rna levels ranged from 230,000 to 1,000,000 copies/ml before onset of triple therapy and fell below 50 copies/ml at 12 to 33 weeks of life in three of the infants. These three children, currently aged 158, 105 and 72 weeks, are asymptomatic, have normal lymphocyte subsets and no hypergammaglobulinaemia. Two children experienced a profound reduction in the amount of proviral dna detected in blood and have become hiv-1 seronegative, although one of them has had hiv-1 rna detectable on a single occasion at 114 weeks of life (303 copies/ml). Transient interruption of therapy resulted in a rapid but reversible increase in hiv-1 rna levels in the third child and was associated with the production of HIV-specific antibodies. The fourth child whose parents were not compliant to treatment and follow-up had a poor virological response. CONCLUSION: Early treatment of vertically acquired human immunodeficiency virus type 1 infection with three reverse transcriptase inhibitors is well tolerated and can result in such suppression of viral replication that specific antibodies are not produced, that proviral dna falls to the lower limit of quantitation in blood and that all clinical and immunological manifestations of infection are avoided. Parental adhesion is crucial to the effectiveness of therapy.- - - - - - - - - - ranking = 0.01310588389074keywords = duct (Clic here for more details about this article) |
6/148. Giant congenital melanocytic nevus with underlying hypoplasia of the subcutaneous fat.A 17-week-old boy with a giant congenital melanocytic nevus (GCMN) of the left lower extremity was noted to have a reduction in circumference of the left lower extremity relative to the contralateral side. The skin overlying the GCMN was persistently warm when compared with the surrounding and contralateral skin. Comparative plain radiography, ultrasonography, and magnetic resonance imaging showed fat hypoplasia of the left lower extremity, with bone and muscle appearing unaffected. The possible role of cytokines produced by the nevus in fat hypoplasia in GCMN is discussed.- - - - - - - - - - ranking = 0.00655294194537keywords = duct (Clic here for more details about this article) |
7/148. Cardiac neoplasm, tachyarrhythmia, and anasarca in an infant.An infant had supraventricular tachycardia and massive anasarca at birth. The supraventricular tachycardia and congestive heart failure were minimally responsive to medical therapy. At 1 month of age, a diagnosis of atrial and ventricular septal defect was made by cardiac catheterization. At 5 months of age, at an operation for pulmonary banding, a peculiar neoplasm of the right atrium was noted. On clamping and resection of the neoplasm, the supraventricular tachyarrhythmia reverted to normal sinus mechanism. The neoplasm, by light and electron microscopy, was thought to be a conduction system hamartoma and was thought to have caused the arrhythmia. A review of the literature showed that no such lesions had been reported previously.- - - - - - - - - - ranking = 0.00655294194537keywords = duct (Clic here for more details about this article) |
8/148. Unusual features of pulmonary sling.patients with an anomalously placed left pulmonary artery ("pulmonary sling") typically have signs and symptoms of respiratory obstruction in the neonatal period. Experience with four previously unreported patients confirmed this. Typical radiographic features include unilateral air trapping (usually on the right side), an abnormal position of the esophagus, and a soft tissue mass interposed between the trachea and esophagus. Less common findings include a normal esophageal position, left-side air trapping, and delayed egress of neonatal pulmonary fluid. Both typical and atypical features were seen; the more unusual aspects are emphasized.- - - - - - - - - - ranking = 0.2keywords = obstruction (Clic here for more details about this article) |
9/148. Severe perinatal liver disease associated with fetal thrombotic vasculopathy.Three neonates with fetal thrombotic vasculopathy in the placenta and severe neonatal liver disease are described. Symptoms included a bleeding disorder on the first day of life, followed by direct hyperbilirubinemia and elevated liver transaminases. All patients also had evidence of thrombosis outside the placenta, including cerebral infarct in two infants and thrombosis of the inferior vena cava in one infant. liver disease was demonstrated to be thrombotic in one infant who died with budd-chiari syndrome. Two infants survived and had liver biopsy with cholestasis, bile duct proliferation, and portal fibrosis demonstrated at 4 weeks and 11 weeks of age, respectively. The etiology of thrombosis is unknown, though in one patient an excessively long and coiled umbilical cord may be implicated. The prenatal onset of thrombosis suggests an inherited or acquired thrombophilic state. In cases of enigmatic neonatal liver disease, an association with thrombosis should be considered and thrombi sought in placenta, umbilical cord, major blood vessels, and other organs. Evaluation for a hypercoagulable state is also suggested.- - - - - - - - - - ranking = 3.3426303618802keywords = cholestasis, bile duct, bile, duct (Clic here for more details about this article) |
10/148. Neonatal alloimmune thrombocytopenia with HLA alloimmunization: case report with immunohematologic and placental findings.Severe neonatal thrombocytopenia is associated with a significant risk of neonatal bleeding complications. It may result from increased consumption, increased destruction, deficient production, or abnormal sequestration within the spleen. When immune mediated, most cases of clinically significant neonatal thrombocytopenia are due to maternal alloimmunization to paternally derived platelet antigens present on fetal platelets. We present the clinical, placental, and immunohematologic findings of a case of severe neonatal alloimmune thrombocytopenia (NAITP) complicated by additional HLA group alloimmunization. The placenta showed chronic villitis of unknown etiology (VUE) and diffuse microthrombi within the villous capillaries, indicating that abnormal thrombogenesis can be a complication of severe NAITP.- - - - - - - - - - ranking = 0.00655294194537keywords = duct (Clic here for more details about this article) |
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