Hemolytic conditions in preterm neonates, including Rhesus (Rh) disease, can lead to mortality and long-term impairments due to bilirubin neurotoxicity. Universal access to Rh immunoprophylaxis, coordinated perinatal-neonatal care, and effective phototherapy has virtually eliminated the risk of kernicterus in many countries. In the absence of jaundice due to isoimmunization and without access to phototherapy or exchange transfusion (in 1955), kernicterus was reported at 10.1%, 5.5%, and 1.2% in babies <30, 31-32, and 33-34 wks gestational age, respectively. Phototherapy initiated at 24±12 hr effectively prevented hyperbilirubinemia in infants <2,000 g even in the presence of hemolysis. This approach (in 1985) reduced exchange transfusions from 23.9% to 4.8%. Now with 3 decades of experience in implementing effective phototherapy, the need for exchange transfusions has virtually been eliminated. However, bilirubin neurotoxicity continues to be associated with prematurity alone. The ability to better predict this risk, other than birthweight and gestation, has been elusive. Objective tests such as total bilirubin, unbound or free bilirubin, albumin levels, and albumin-bilirubin binding, together with observations of concurrent hemolysis, sepsis, and rapid rate of bilirubin rise have been considered, but their individual or combined predictive utility has yet to be refined. The disruptive effects of immaturity, concurrent neonatal disease, cholestasis, use of total parenteral nutrition or drugs that alter bilirubin-binding abilities augment the clinical risk of neurotoxicity. Current management options rely on the "fine-tuning" of each infant's exposure to beneficial antioxidants and avoidance of silent neurotoxic properties of bilirubin navigated within the safe spectrum of operational thresholds demarcated by experts.

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A baby with the complete form of pentalogy of Cantrell was delivered at 33 weeks of gestation. The hallmark of this syndrome is ectopia cordis (EC) with omphalocele. Even though a fetal diagnosis was made at 14 weeks, parents have decided to continue with the pregnancy. Early antenatal ultrasonographic diagnosis is essential as survival depends mostly on the EC, associated cardiac anomalies and degree of thoraco-abdominal defect. Fetal diagnosis of this lethal anomaly before viability gives the parents an option of termination.

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Background: Persistent pulmonary hypertension of the newborn (PPHN) result from the failure of the normal fetal-to-neonatal circulatory transition is associated with substantial infant mortality and morbidity. Objective: To estimate the possible risk factors and assess the outcome of these cases. Materials and Methods: Prospective study was performed enrolling all full-term and post-term newborn admitted to the NICU from January 2009 to April 2012, All neonates were subjected to complete history and physical examination, laboratory data including a complete blood count, arterial blood gases, blood glucose, serum electrolytes, and blood culture to exclude sepsis. Cases with PPHN had a continuous pulse oximeter, blood pressure and electrocardiography monitoring. Chest X-ray and echocardiogarphy were carried out to verify shunt and exclude structural congenital heart disease. Results: Out of the studied 640 infants, 32 infants (5%) developed PPHN, Meconium aspiration, birth asphyxia, hyaline membrane diseases, neonatal septicemia, post-term birth being large for gestational age, cesarean section, maternal overweight, and diabetes mellitus were associated with an elevated risk for PPHN. All neonates treated with O ₂ , 10 neonates with Mg sulphate, 16 with oral sildenafil and 12 with mechanical ventilation. After 6 months follow-up, 12 (37.54%) improved and followed-up without sequelae, 4 (12.5%) developed some neurodevelopmental impairment, 8 (25%) died, 3 (9.3%) developed chronic lungs diseases, 2 (6.2%) developed hearing defects and another 3 (9.3%) missed follow-up. Conclusion: PPHN was found in 5% of the studied population. Meconium aspiration, birth asphyxia, neonatal septicemia, post-term were associated with an elevated risk for PPHN. As this is a unit based study, a comprehensive countrywide survey on PPHN in Egypt is recommended to determine any regional differences in disease incidence.

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Background: Fluconazole has shown to be effective in reducing both colonization and invasive Candida infection (ICI) in ELBW neonates; we conducted a randomized trial to compare oral nystatin with intravenous fluconazole for prophylaxis against invasive Candidiasis in high risk neonates. Materials and Methods: By using SPSS, preterm less than 30 weeks gestation and/or birth weight 1200 grams or less assigned to receive either intravenous Fluconazole (6 mg/kg q72 hr for 1 ^st week then q48 h for 6 wks) or oral Nystatin (100,000 unit q8 hr for 6 wks). The medications commenced at one week of age after obtaining the base line investigations and check for Candida colonization by urine culture and rectal swab; subsequently all lab work and the clinical data were monitered regularly. Risk factors were assessed. The data collected prospectively looking for primary end point the invasive Candida infection (ICI) and 2 ndry outcomes include medication safety, tolerance and cost. Results: 65 neonate randomly assigned however only 57 neonates comleted the study 33/57 (57%) to intravenous fluconazole group and 24/57 (42%) to oral nystatin group. No differences in birth weights Nystatin (1.15 Kg) Fluconazole (1.01 Kg), gender males (26/57), female (32/57), Gestational age (29.28 vs l28.22) or risk factors between the two groups. Rectal swab Colonization occurred in 2/24 (8%) in Nystatin group and 4/33 (12%) in the Fluconazole group, but none of the neonates developed ICI or side effects, although in the Fluconazole group transient transaminase elevation 2SD standard deviation above the mean was observed. Central line duration was 2 SD above the mean for fluconazole group, The cost of the Fluconazole treated group (7,581 SAR) 106.4 US/pt double the cost of Nystatin treated group (3,375 SAR) 50 US/pt. Conclusion: Intravenous Fluconazole and oral Nystatin at the prophylactic doses are equally effective and safe in preventing (ICI) in preterm neonates, however oral Nystatin is readily available, easily administered with lower cost per neonate.

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Caudal duplication syndrome is a rare entity in which structures derived from the embryonic cloaca and notochord are duplicated to various extents. Its prevalence at birth is less than 1 per 100,000. The term caudal duplication encompasses a spectrum of anomalies and is often used to describe incomplete separation of monovular twins or referred to as part of the spectrum of anomalies associated with conjoined twinning. It usually includes multiple rare malformations and duplications of distal organs derived from the hindgut, neural tube, and caudal mesoderm. It was postulated that the disorder is related to misexpression of one or more of the distal HOX genes, potentially HOX10 or HOX11, leading to abnormal proliferation of caudal mesenchyme. The malformations are usually diagnosed by anomaly scan in the second trimester. Here we report the case of a baby presenting on the first day of life with complete duplication of caudal structures below the dorsolumbar level.

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Maple syrup urine disease (MSUD) is a rare autosomal recessive disorder of branched-chain amino acid metabolism. The condition gets its name from the distinctive sweet odour of affected infants' urine. MSUD is caused by a deficiency of the branched-chain α-ketoacid dehydrogenase enzyme complex, leading to accumulation of the branched-chain amino acids (leucine, isoleucine, and valine) and their toxic by-products (ketoacids) in the blood and urine. Imaging is characterestized by MSUD oedema affecting the myelinated white matter. We present a neonate with classic type of MSUD and its imaging features on computed tomography, conventional magnetic resonance imaging, diffusion-weighted imaging, and magnetic resonance spectroscopy.

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Septicemia is a major cause of death in neonates especially in developing countries. We report a case of septicemia in a neonate due to Salmonella Paratyphi B. The baby responded well to therapy and recovered completely.

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Rhizomelic Chondrodysplasia punctata (RCDP) is an autosomal recessive metabolic disorder affecting mainly peroxisomal function. We describe a case of RCDP in a 12 days old newborn based on the clinical and radiological ground without any major systemic structural or functional abnormalities.

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Objectives: The objective of this study was to estimate the prevalence of meningitis in cases with late onset septicemia (LOS). Materials and Methods: A prospective study was carried out for a period of 1 year in a tertiary care hospital in North West India to estimate the prevalence of meningitis in cases of LOS. In all the admitted neonates with features of sepsis with a positive C-reactive protein, a lumbar puncture (LP) was carried out and results interpreted on the basis of cerebrospinal fluid (CSF) cytology and biochemistry. Simultaneous blood and CSF cultures were also taken. All other baseline investigations were performed and in those diagnosed as meningitis an ultrasound head was carried out prior to discharge. No urine cultures were obtained. Results: The study showed the prevalence of meningitis as 22.5% in neonates with LOS with statistically significant implications of meningitis versus gestation, sex, acquired the place of infection, and outcome in terms of sequelae/mortality. Conclusions: Meningitis is commonly associated with late onset sepsis hence LP should be the standard of care in such neonates as the treatment protocol and the outcome is directly proportional to the diagnosis at initial presentation.

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A 2-day-old female neonate with the clinical picture of proximal small bowel atresia, on exploration, turned out to have intestinal atresia of a rare variety, i.e., a near-total jejuno-ileal atresia. The baby had total small bowel length of less than 10 cm. She survived for 3 months on enteral feeding after end-to-back duodeno-ileal anastomosis and thereafter succumbed to septicemia. The case is presented for it's extreme rarity and consideration of this extreme form of small bowel atresia as an offshoot of the existing classifications of jejuno-ileal atresia since it has dismal prognosis and presents as a management challenge even today.

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Full born term male baby having hyperpigmentation over back of neck and back of chest. The colour of nevus is black coloured. Hyperpigmented area is slightly hypertrophy. Imaging, X-ray spine, X-ray fingers and toes, X-ray chest, ultrasound of abdomen, CT scan of brain-normal. Treatment advised: Pigmentation can be reduced by pigment laser.

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