Neonatal polycythemia and maternal hypertension.
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Biomedical subjects
Publications and source records attributed to P Merlob.
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A female neonate with a streak ovary on one side and huge ovarian cyst on the other side is presented. Her karyotype was 46,XX. Adnexectomy was mandatory, leaving the patient with a single streak gonad. Whether this infant has the recessive form of gonadal dysgenesis with one ovary, streak or hypoplastic, replaced by a cyst or the Slotnick-Goldfarb syndrome is impossible to elucidate at present. The potential implications of a streak gonad, either related to gonadal dysgenesis or as a possible source of neoplasia, are reviewed. Current approaches to an ovarian cyst and streak ovary in the neonatal period are discussed.
Little information is available about continued weight loss (CWL) of the newborn during the third day of life as an additional factor in early weaning. The purpose of the present study was to establish the value of CWL as an indicator of early weaning. The study group comprised 54 normal newborns of non-smoking healthy mothers. Newborns with CWL were compared with those whose weight remained stable or increased (controls). Follow-up was conducted by telephone interview at 10 days, 6 weeks, and 3 months after birth. It was found that mothers in the control group breastfed for a much longer period than those in the study group (P = 0.014); at age 3 months 66.7% of the controls vs. 46.3% of the study group continued to breastfeed. CWL and/or absence of or inadequate breastfeeding guidance for the mother places infants at high risk of early weaning. These mothers require very early and strong support in order to avoid early weaning of their infants.
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Bradycardia, an extremely rare side-effect of ranitidine therapy is described in a 4-day-old full-term male neonate, who was admitted because of massive gastro-intestinal bleeding. Two hours after the intravenous injection of 1 mg/kg body weight per day, ECG showed sinus bradycardia of 60 beats/min with normal axis and QRS complex. The bradycardia gradually resolved in the next 24 h.
Meconium-stained amniotic fluid was recently reported to be significantly correlated with a higher incidence of otitis media during infancy. In order to verify this connection, a case-control study was performed on 71 pairs of infants, with and without meconium aspiration, matched for gestational age, birth weight and sex. Data were collected by questionnaire or telephone, the response rate being 88.7%. No significant difference in incidence of otitis media between the two groups was observed. This study demonstrated that newborns with meconium aspiration are not at greater risk for otitis media in the 1st year of life.
An outbreak of multiresistant Klebsiella pneumoniae occurring in a neonatal intensive care unit is described. All infections developed at least 5 days after admission to the unit (range, 5-40 days). Four infants had septicaemia and one had urinary tract infection. Three of the infected infants died. All klebsiella isolates were resistant to ampicillin, cefotaxime, cefuroxime, co-amoxiclav, mezlocillin, chloramphenicol, gentamicin, and ceftazidime (except in two); all were susceptible to imipenem, amikacin and quinolones. An extensive case-control study identified the following significant risk factors for colonization: prematurity; presence of indwelling catheters; previous antibiotic treatment; and parenteral nutrition. The outbreak was controlled with re-emphasis on strict handwashing practices, cohorting, closure of the unit to outborn admissions, and changing the regimen of empirical antibiotic therapy. Physicians should be aware of multiresistant Klebsiella spp. and change treatment whenever clinically indicated, even before culture results are available.
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Phototherapy, an effective treatment for neonatal indirect hyperbilirubinemia, has guidelines for its initiation but none for its discontinuation. In our study, phototherapy was begun at a mean age of 90 hours in 28 full-term and 30 preterm infants with indirect nonhemolytic hyperbilirubinemia. After three days, phototherapy was halted at a mean bilirubin concentration of 13.0 +/- 0.7 mg/dL in term and 10.7 +/- 1.2 mg/dL in preterm infants, levels higher than those used by other investigators. Bilirubin rebound then occurred at a mean of 12.5 hours to a level of 0.86 +/- 1.0 mg/dL in term and at a mean of 14.1 hours to a level of 0.83 +/- 0.56 mg/dL in preterm infants. No complications were observed nor was there a need for reinstitution of phototherapy. Based on these data, discontinuation of phototherapy at higher bilirubin concentrations and earlier home discharge than previously described appear harmless for both term and preterm neonates.
Intrauterine electronic fetal heart monitoring is a widely used tool in diagnosing intrauterine fetal distress. We describe a case of successful conservative treatment for a neonatal labioperineal tear caused by scalp electrode application during breech delivery.
It is common among Israeli Arabs who live in the villages to prefer consanguineous marriages, particularly among first cousins. In addition, such villages are populated by a few (less than 20) original families, and inter-family/inter-village marriages are infrequent. The purpose of this study was to examine the consequences of such "consanguinity" in Taibe, a large Arab village, 30 km from Tel Aviv. Six hundred ten families were prospectively ascertained through infants who were routinely seen in the local "Well Baby Clinics." A significant increase in the incidence of major malformations was noted in relation to the closeness of the parental relationship. For the index cases group the prevalence of individuals with major malformations were 5.8% in the product of inter-village marriages, 8.3% in the intra-village non-related matings, 15.1% in the distant consanguineous group, and up to 15.8% in the progeny of first-cousin marriages (P less than 0.001). In the siblings of these index cases, the frequency of major malformations was 4.3%, 4.5%, 10.5%, and 10.3%, respectively. Analysis of the major malformations by each body system showed the same trend. The study demonstrates a marked high rate of consanguineous marriages, whose effect leads to a marked increase in major malformations and thus a prominent public health problem in such villages. This requires a unique genetic counseling approach.
The Israeli national neonatal screening program for congenital hypothyroidism (CH) was initiated in May 1978. The overall incidence of persistent primary congenital hypothyroidism (PPCH) during the first 10 years of screening was 1:2,950 live births. The purpose of this study was to ascertain the incidence of congenital extrathyroid anomalies (ETAs) among the infants with PPCH and to compare it with the Israeli Birth Defect Monitoring System data. Among 243 PPCH infants on whom adequate data were available, 38 infants (15.6%) had associated congenital anomalies. Fourteen infants had congenital cardiac anomalies (5.8%): VSD (n = 7), PDA (n = 3), PS (n = 2), one mitral insufficiency, and one congenital atrial flutter. Eight children (3.3%) had congenital dislocation of the hip; their M:F ratio was 3:5 similar to the M:F ratio in CH (unlike the ratio in the general population). Some additional anomalies were considerably more common than in the general population. It is reasonable to assume that teratogenic effects active during organogenesis may affect simultaneously many organs, including the developing thyroid, causing a relatively high percentage of CH infants with congenital ETA.
The syndrome of Albright hereditary osteodystrophy (AHO), pseudohypoparathyroidism (PHP) and pseudopseudohypoparathyroidism (PPHP) is clinically and genetically heterogeneous. Classically, patients with PHP have the skeletal features of AHO, resistance to multiple hormones that work via cAMP such as parathyroid hormone and thyroid stimulating hormone, and deficient activity of Gs protein, the guanine nucleotide-binding protein that stimulates adenylate cyclase. However, patients without hormone resistance but with AHO and Gs deficiency were described (PPHP), as well as patients with multiple hormone resistance but without AHO or Gs deficiency. In a few patients with deficient Gs activity, hypothyroidism rather than hypocalcemia was the initial presentation of the disorder. We describe here a new variant of the syndrome, affecting 5 individuals in a 3 generation family with AHO, normal Gs activity and hypothyroidism. In the first 2 generations, mild features of AHO were present. The 2 sibs in the third generation had severe manifestations of AHO, including mild mental retardation as well as hypothyroidism. Diagnosis of congenital osteoma cutis at birth of the proband led to the diagnosis of the family. Elucidation of the molecular defect will shed light on the relationship between hormone resistance and AHO, as well as on the physiological mechanism of hormonal signal transduction.
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Minor congenital anomalies (MCA) were assessed in the offspring of 802 gestational diabetic mothers, 117 pre-gestational diabetic mothers, and 380 offspring born to normal mothers. The prevalence of infants with MCA ranged between 19.4% and 20.5% in the three groups without any significant difference between them. There was no correlation between the prevalence and type of MCA and the severity of the diabetic state. Neither was there any correlation between the prevalence or type of MCA and the appearance or type of major congenital anomalies.