Neonatal noma.

Neonatal noma is a rare clinical syndrome affecting term and preterm infants. It causes gangrene of the orofacial tissues accompanied by sepsis and a high mortality rate. A preterm 35-week infant, severely growth retarded in utero, developed clinical signs of sepsis with ulcers all over the oral mucosa caused by local infection with Pseudomonas aeruginosa. The lesions healed with cicatricial sequelae of the mouth, sequestration of teeth, and retraction of perimandibular soft tissues.

Evaluation of two rapid tests for detection of maternal endocervical group B streptococcus: enzyme-linked immunosorbent assay and gram stain.

OBJECTIVE: To establish the reliability of two rapid tests for detection of group B streptococcus, the new enzyme-linked immunosorbent assay (ELISA) and the Gram stain. In addition, we wished to determine whether the combination of these tests would increase test sensitivity. METHODS: During a 2-year period, cervical secretions from 660 patients who presented with either premature rupture of membranes or preterm labor at a gestational age of 37 weeks or less were tested by three methods: the Equate ELISA test, a Gram stain smear, and a culture for group B streptococcus. Results of the rapid tests were compared with the corresponding culture results. RESULTS: Cultures were positive for group B streptococcus in 50 patients, a prevalence of 7.5%. The sensitivity for the Equate ELISA test was 24% and for the Gram stain, 20%. Specificity was 95 and 89%, respectively. When results of these tests were combined, the sensitivity increased to 26%. Both rapid tests yielded a high rate of false positives. In more than 50% of false-positive cases, bacteria other than group B streptococcus were isolated. CONCLUSION: Neither the Gram stain nor the Equate ELISA test is sensitive enough to be used as a screening test for the identification of group B streptococcus. Furthermore, the combination of both rapid tests yielded only a slight increase in sensitivity. The high false-positive rate of the ELISA test seems to be related to a cross-reactivity of the anti-group B streptococcal antibody with other cervical bacteria.

Occurrence of cerebrohepatorenal (Zellweger) syndrome in the Karaite community in Israel: a genetic hypothesis.

Two infants with the cerebrohepatorenal (Zellweger) syndrome in Karaite families are described. This syndrome has an autosomal recessive mode of inheritance with an estimated incidence of between 1:25,000 and 1:100,000 live births. In addition to a previous report of the syndrome in a Karaite family, this report raises the possibility of a high incidence of Zellweger Syndrome among the Karaites in Israel.

Time trends (1978-86) of anencephaly and spina bifida in four hospitals affiliated with the International Clearinghouse: a warning.

The prevalence and time trends of spina bifida and anencephaly in the central area of Israel have been studied using data derived from four hospitals affiliated with the International Clearinghouse for Birth Defects Monitoring Systems. Spina bifida rates show a continuous increase from 1978 (1.1/10,000) to 1986 (7.4/10,000) with a peak in 1984 (7-fold increase compared with 1978). The logistic regression analysis of proportion of cases revealed a significant linear trend over time (P less than 0.005). Anencephaly rates increased from 1978 to 1981 but later decreased continuously until 1986 when the prevalence returned to the initial level of 1978. This time trend of spina bifida may represent a real alarm; the implications of this observation are discussed.

Lupus-like syndrome in a mother and newborn following administration of hydralazine; a case report.

A very-low-birth-weight infant died from pericardial effusion and cardiac tamponade confirmed by the post-mortem findings. The mother suffered from lupus-like syndrome consequent to hydralazine treatment for pregnancy-induced hypertension. The possible relationship between mother-infant pathology and hydralazine administration is discussed.

Complications associated with genital colonization in pregnancies with and without cerclage.

Cultures from the cervical milieu were taken from 24 antibiotic-treated patients (group A) and from 25 patients with no antibiotic treatment (group B). Both groups had undergone cerclage and were compared to 30 randomly chosen pregnant patients without cerclage (group C). Positive cultures were obtained in 70.8% and 48% before cerclage and in 66.7% and 68% after cerclage in groups A and B, respectively. These were not significantly different from group C (56.7%). Escherichia coli was found in over 75% of positive cultures. The rate of premature deliveries and premature rupture of the membranes were significantly higher in the cerclage groups. Maternal morbidity was significantly higher in group A compared to B and C. Our data suggest that bacterial colonization of the cervix and the vagina is not influenced by cerclage and antibiotics. It is concluded that cervical cerclage is associated with increased morbidity, therefore it is indicated only for definite anatomic and/or functional defects.

Management of labor with umbilical cord prolapse: a 5-year study.

Fifty-one cases of cord prolapse, in which delivery was not imminent and the fetus was still alive, were managed by filling the bladder with 500-700 mL of saline and by intravenous ritodrine. Delivery was by cesarean section as soon as possible. Among the cases so managed, there were no perinatal deaths. The mean 5-minute Apgar score was 9.5, and in only three cases was it less than 7. In eight cases, fetal distress continued after treatment, as compared with 33 cases before this type of treatment started (P less than .001); no difference was found in the outcome between neonates weighing less than or greater than 2500 g.

Autosomal recessive inheritance of Nager acrofacial dysostosis.

Nager acrofacial dysostosis is a variant of mandibulofacial dysostosis with severe micrognathia, malar hypoplasia, and radial limb defects. Most cases are sporadic, but autosomal recessive inheritance has been suggested. A family is reported in which two sibs are affected by this syndrome, presenting further evidence for autosomal recessive inheritance. The recognition of this syndrome as a distinct entity has important implications. After the birth of a child with orofacial malformations suggestive of mandibulofacial dysostosis, an exact diagnosis is essential before genetic counselling can be offered.

Congenital granular cell tumor of the tongue.

Granular cell lesions in the oral cavity occur primarily on the tongue in adults (granular cell tumor) and exclusively on the alveolar ridge in newborns (congenital granular cell epulis). Because of the location, age of appearance, and certain clinical and histologic differences, these histologically similar lesions are considered separate clinical entities. This case report, however, describes the occurrence of a granular cell lesion on the tongue of a newborn. Although the etiopathogenesis of these lesions is unknown, the present case suggests a common etiologic relationship of these entities and tends to rule out an odontogenic origin for the congenital granular cell epulis.

Noma in a full-term neonate.

Noma is an uncommon gangrenous process usually affecting malnourished children. A full-term neonate with orofacial noma, bilateral choanal atresia, and transient neutropenia with B cell deficiency is reported. This unusual appearance of noma in a well-nourished newborn might be related to the combination of choanal atresia and transient immune deficiency.

Teratogenicity of progestogens given during the first trimester of pregnancy.

Possible teratogenic effects of exogenous progestational agents given during early pregnancy were investigated by a controlled historic prospective study of 2754 infants born to mothers who had bled during the first trimester of pregnancy. The study group consisted of 1608 newborns whose mothers had been treated with progestogens (mostly medroxyprogesterone acetate) beginning in the first trimester. The control group comprised 1146 infants of untreated mothers. All newborns were subjected to thorough examination during the first days of life, with special attention to detection of various malformations classified according to the different anatomic systems. No significant difference was found between the treated and the control groups with respect to malformations in any of the systems examined. The overall rate of malformations was 120 per 1000 in the study group and 123.9 per 1000 in the control group. Major malformations occurred at rates of 63.4 and 71.5 per 1000, respectively. The study thus fails to demonstrate an increase in teratogenicity after administration of gestagens during the first trimester of pregnancy.

Prevention of premature labor by 17 alpha-hydroxyprogesterone caproate.

Eighty pregnant women at high risk of giving birth prematurely were divided randomly into two groups. Treatment with either 17 alpha-hydroxyprogesterone caproate, 250 mg by intramuscular injection once a week, or a placebo was given in a double-blind fashion. Imminent premature labor occurred in 29.0% of the treated group and in 59.4% of the control group (p less than 0.025). The rate of premature deliveries was also significantly lower in the treated group (16.1%) than in the control group (37.82%) (p less than 0.05). There were no cases of perinatal death or fetal malformations in either group. The mean birth weight of all infants of the treated group was significantly higher than in those of the control group (3111.9 +/- 905 gm versus 2680 +/- 813.4 gm, p less than 0.05). The results support treatment with progesterone caproate for the prevention of premature labor.

Cholecalciferol metabolites in polycythemic newborns.

Metabolites of cholecalciferol were measured in the sera of healthy and polycythemic newborns at 15.5 and 10.5 h of age (median), respectively. The serum concentrations of 1,25-dihydroxyvitamin D and 24,25-dihydroxyvitamin D were lower in the polycythemic group (P less than 0.003 and P less than 0.001, respectively). As the kidney is the main site of conversion of 25-hydroxyvitamin D to its dihydroxylated metabolites, it is possible that hyperviscosity and subsequent decrease in renal blood flow interfere with the efficiency of the process.

Unusual parascrotal hernia with ectopic testicle in a neonate. Case report.

A giant indirect inguinal hernia leading to a huge parascrotal mass in a newborn is described. The right scrotum was empty and the ectopic testicle was found in the perineum, close to the anus. To our knowledge this is the first report of the presence at delivery of a parascrotal inguinal hernia in combination with an ectopic testicle.

Comparison between the combination of azlocillin-gentamicin and ampicillin-gentamicin in the treatment of a nursery population.

Newborn, and particularly premature infants are prone to life-threatening infections. The combination of ampicillin-gentamicin (AM-G) has been used extensively in neonatal intensive care units (NICU). Because resistant bacteria emerged, a new drug--an acylureidopenicillin, azlocillin, with a wide antibacterial spectrum--has been developed. A prospective randomized study was performed in order to evaluate the combination of AM-G vs. azlocillin-gentamicin (AZ-G). Thirty neonates received AM-G and 28 received AZ-G. Definite infection was found in 15 AM-G patients and in 10 AZ-G patients. In these patients, the antibiotic regimen was changed in six of the AM-G and in one of the AZ-G patients. In each of the two treatment groups, one very-low-birth-weight baby died due to overwhelming sepsis. In vitro sensitivity to AZ-G was higher than to AM-G, according to azlocillin blood levels obtained. Infants weighting less than 2,350 g had lower clearance and volume of distribution than did infants weighing greater than 2,350 g. The serum half-life was approximately the same in both groups. It appears to be that in the NICU, the combination of AZ-G may be more effective than the AM-G combination.

Necrotizing enterocolitis--a 5-year experience.

With the increased survival of very-low-birth-weight neonates, necrotizing enterocolitis (NEC) represents one of the most frequent life-threatening conditions for this population. NEC was diagnosed on 24 occasions during a 5-year period (1977-81) at the Neonatal Intensive Care Unit, Kaplan Hospital, Rehovot. These 24 NEC cases, confirmed by radiological, surgical or pathological findings, were analyzed in a retrospective study. The mean weight was 1,450 g and the mean gestational age 32 weeks. Only one of the affected infants was a full-term newborn. The mean age at onset of symptoms was 9.8 days. In one case, NEC developed before feedings were started. The infectious agents--most frequently gram-negative rods--were isolated from the most seriously ill babies. Cultures from blood and gastric aspirate were positive in 10 of the 12 fatal cases. No bacteria were isolated from the blood among the survivors. Rotavirus, the only viral particle detected, was diagnosed in the bloody stools of two of the infants who recovered completely. Prophylactic therapy with oral kanamycin, administered to all premature infants weighing less than 1,500 g born over a 3-year period, did not appear to have a preventive influence. The overall mortality was 50%, with no sequelae observed among survivors. The high incidence of positive cultures among fatal cases suggests that overwhelming infection played a fundamental role in the outcome.