1/1160. Solitary hepatic hemangioma in a newborn infant complicated by cardiac failure, consumption coagulopathy, microangiopathic hemolytic anemia, and obstructive jaundice. Case report and review of the literature. A newborn infant with a large hepatic hemangioma developed congestive heart failure, consumption coagulopathy, microangiopathic hemolytic anemia, and obstructive jaundice. The patient was mildly heparinized (250 units per kg and day) and underwent successful resection of the tumor without lobectomy at the age of 3 days. blood volume increased from 93.9 ml/kg at the age of 5 h to 124.2 ml/kg prior to surgery. Red-cell mass simultaneously decreased from 53.8 to 39.4 ml/kg. The increase of blood volume is explained by congestive heart failure, the decrease of red-cell mass by intravascular coagulation within the tumor resulting in formation of thrombi and microangiopathic hemolytic anemia. A review of the literature on infants with symptoms caused by an intrahepatic hemangioma during the first month of life confirms that surgical intervention is the treatment of choice for infants with giant solitary hemangioma of the liver. ( info) |
2/1160. 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. ( info) |
3/1160. 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. ( info) |
4/1160. [Typing and detection of antibodies in the PLA system (platelet). Application to the study of neonatal thrombopenia by feto-maternal PLA allo-immunisation] A platelet indirect radio-active coombs test has been described. The technique for purification and labelling the antiglobulin has been precised. This test allows the typing of platelets in the PLA system and the study of sera from mothers of thrombocytopenic child. As examples, four families of neonatal thrombocytopenia are reported, with PLA1 negative mother. In the serum of three of these mothers, we could demonstrate anti-PLA antibodies in spite of a negative platelet complement fixation. This test has many advantages compared to other tests such as platelet complement fixation, assay for blocking antibodies or antiglobulin consumption: it gives objective and quantitative results and is highly reproducible, anticomplementary serum may be tested. It has enabled us to select PLA1 negative donors for exsanguino-transfusions of thrombocytopenic children born from PLA1 negative mothers. ( info) |
5/1160. pregnancy outcome in aboriginal women with NIDDM in the Sioux Lookout Zone. PURPOSE: To review the pregnancy outcomes of Aboriginal women with non-insulin-dependent diabetes mellitus (NIDDM) in the Sioux Lookout Zone of Northwestern ontario, canada. METHOD: Retrospective chart review of deliveries of all women with a confirmed diagnosis of NIDDM was carried out between 1989 and 1992. RESULTS: During this period, 26 infants were born to 19 women with the diagnosis of NIDDM. Mean birth weight was 4,075 grams, with an average gestational age at delivery of 38 weeks. Three newborns required cesarean delivery, one required forceps, and one a vacuum extraction. There were four cases of shoulder dystocia. There were one stillbirth, one maternal death, and two cases of congenital heart disease. Ten newborns had neonatal jaundice and only two had neonatal hypoglycemia. These results suggest there is significant risk associated with NIDDM in pregnancy. ( info) |
| Three cases of staphylococcal scalded skin syndrome in Nigerian children are reported. The clinical features and bacteriological findings are discussed. It is suggested that the clinical condition is not as rare in our community as absence of reports on African children in the literature may suggest. A reference centre for phage typing of staphylococci from suspected cases in a developing country like our own is recommended. ( info) |
7/1160. Reye's syndrome in a neonate. This case substantiates the fact that Reye's syndrome can occur in newborns. The clinical features appear to be slightly different in the neonate, in that respiratory distress was the presenting sign in this case and in the one other reported case in a newborn, with no mild preceding illness or vomiting. Thus, Reye's syndrome must be considered when a newborn presents with respiratory distress and evidence of central nervous system and hepatic involvement. ( info) |
8/1160. Two forms of cutis laxa presenting in the newborn period. Two infants are described with congenital cutis laxa. They represent two distinct disorders. In the first, congenital cutis laxa is associated with a generalized disorder of elastic tissue in which there may be diaphragmatic or other hernias, diverticula of the gastrointestinal or urinary tract and infantile emphysema. The disease is fatal often within the first year. In the second, congenital cutis laxa is associated with widely patent anterior fontanel, a variety of malformations, and retarded growth and development. Recognition of these distinct syndromes in the newborn period and their recessive inheritance permit realistic discussion of the prognosis which is very different from the benign dominant forms of cutis laxa. ( info) |
9/1160. The decline and early fall of neonatal pulmonary hemorrhage. Seven years' experience (1965-1972) with neonatal pulmonary hemorrhage at the University of colorado was reviewed. Pulmonary hemorrhage still occurs frequently as a preterminal event in hyaline membrane disease. As an isolated "pure" entity, however, pulmonary hemorrhage has shown a marked decline. This phenomenon is probably related to advances in neonatal intensive care. The radiographic pattern of "pure" pulmonary hemorrhage in the newborn is most commonly a diffuse alveolar infiltrate. Since pure hemorrhage is now an uncommon entity, the radiologist can reasonably suggest this diagnosis on rare occasions only, in the clinical setting of profound hypoxia. ( info) |
10/1160. 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. ( info) |