Persistent pulmonary hypertension of the newborn (PPHN) is a life-threatening neonatal pathology resulting from poor hemodynamic and respiratory transition to extra uterine life. Inhaled nitric oxide (iNO) is a current, commonly used treatment of PPHN. However, iNO is not available therapy in many developing countries and around 50% of infants with PPHN do not respond to iNO therapy. Sildenafil is a phosphodiesterase inhibitor type 5 (PDE5) that has been shown to selectively reduce pulmonary vascular resistance in both animal models and adult humans. Recent studies have found that in PPHN, administration of Sildenafil was associated with a significant increase in the oxygenation and a reduction in mortality with no clinically important side effects.

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Gastroschisis is not a very rare congenital deformity, but extragastrointestinal association is rare, if any present, in that condition, an alternative diagnosis should be considered, like Pentalogy of Cantrell, Limb-body wall complex, etc., Other birth defects are always associated with gastroschisis, most commonly, abnormalities of the cardiac and genitourinary. The present case is one of the gastroschisis to highlight the associations of spinal and lower limbs anomalies, with two-vessel short umbilical cord and severe oligohydramnios in primiparous.

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Background: By school-age, even low-risk moderately preterm-born children show more neuro-cognitive deficits, motor impairments, academic underachievement, behavioral problems, and poor social adaptation than full-term peers. Aim: To evaluate the outcomes at school-age for moderately preterm-born children (29-33 weeks gestational age), appropriate in growth for gestational age (AGA) and medically at low-risk, randomized to Newborn Individualized Developmental Care and Assessment Program (NIDCAP) or standard care in the Newborn Intensive Care Unit. At school-age, the experimental (E) group will show better neuropsychological and neuro-electrophysiological function, as well as improved brain structure than the control (C) group. Materials and Methods: The original sample consisted of 30 moderately preterm-born infants (29 to 33 weeks), 23 (8C and 15E) of them were evaluated at 8 years of age, corrected-for-prematurity with neuropsychological, EEG spectral coherence, and diffusion tensor magnetic resonance imaging (DT-MRI) measures. Results: E-performed significantly better than C-group children on the Kaufman Assessment Battery for Children-Second Edition (KABC-II) and trended towards better scores on the Rey-Osterrieth Complex Figure Test. They also showed more mature frontal and parietal brain connectivities, and more mature fiber tracts involving the internal capsule and the cingulum. Neurobehavioral results in the newborn period successfully predicted neuropsychological functioning at 8 years corrected age. Conclusion: Moderately preterm infants cared for with the NIDCAP intervention showed improved neuropsychological and neuro-electrophysiological function as well as improved brain structure at school-age.

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Objective: To document the range of presentations, extent of investigations, and treatment choices of 'physicians treating' neonates with mastitis seen over a 9-year period at a tertiary care pediatric hospital. Materials and Methods: An email survey was sent to Emergency Department (ED) staff and fellows asking them how they would treat a well-looking neonate with localized mastitis. Secondly, a retrospective chart review of neonates presenting to the ED with a diagnosis of neonatal mastitis or breast engorgement from July 2000 to December 2009 was conducted to assess how such patients were actually treated. Results: 46/107 surveys were returned, with a wide discrepancy in how clinicians would treat neonatal mastitis: 4.3% would perform a full sepsis work up, including lumbar puncture, followed by IV antibiotics and hospital admission; 28% chose discharge on oral antibiotics; and 28% suggested admission only if blood work was abnormal. From the chart review, 33 neonates were diagnosed with possible neonatal mastitis over a 9-year period: 12 met the inclusion criteria. Of these, 8 (66%) were admitted and treated with intravenous antibiotics, 2 (16.6%) were treated with oral antibiotics, and 2 (16.6%) did not receive antibiotics. None of the 12 patients had lumbar puncture performed. Conclusion: There is significant disagreement among clinicians regarding the best way to treat the well-looking neonate with localized mastitis. Most elect to perform blood tests and start treatment with IV antibiotics with good Staphylococcus aureus coverage, followed by oral antibiotics if cultures are negative.

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Sirenomelia, alternatively known as 'mermaid syndrome' is a very rare congenital deformity in which the legs are fused together, giving them the appearance of the tail of a mermaid'. Other birth defects are always associated with sirenomelia, most commonly abnormalities of the kidneys, large intestines, and genitalia. The present case is a one of sirenomelia associated with an absent right kidney, mild left hydronephrosis, single umbilical artery, and severe oligohydramnios. We discuss the findings, relative to the present literature and related etiopathogenesis.

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Severe hyperkalemia is a medical emergency and occurs due to a variety of underlying illnesses. We present a 7 day old neonate who presented with life threatening hyperkalemia due to pseudohypoaldosteronism type 1 (PHA1). The clinical picture resembled congenital adrenal hyperplasia (CAH). Very aggressive management including peritoneal dialysis was required to control hyperkalemia. It is important to differentiate PHA1 from CAH since the former does not respond to corticosteroid therapy and may require peritoneal dialysis for control of hyperkalemia. A discussion on the types, clinical course, and management of pseudohypoaldosteronism type 1 is presented.

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We are presenting a case of a neonate presented with a neck mass, airway and esophageal obstruction, the tumor has a brain extension; treated with partial surgical excision; the pathological studies revealed plexiform Neurofibromatosis. The patient also has café au lait spots.

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Background: Bacterial sepsis is one of the major causes of mortality in newborn infants. Mortality increases when sepsis is associated with neutropenia. Materials and Methods: We conducted a prospective, randomized, double-blind, placebo-controlled trial of recombinant human granulocyte colony-stimulating factor on preterm neonates (gestational age (GA) <34 weeks) with sepsis and absolute neutrophil count (ANC) of <1500 cells/mm ³ . Mortality, duration of Neonatal Intensive Care Unit (NICU) stay, hematological parameters (ANC, platelet count, and total leukocyte count) were compared between the two groups. The GCSF group (n=39) received GCSF intravenously in a single daily dose of 10 μg/kg/day in a 5% dextrose solution over 20-40 min for three consecutive days, while the control group ( n=39) received placebo of an equivalent volume of 5% dextrose. Results: Baseline demographic profile among the two groups was comparable. Mortality rate in the GCSF group was significantly lower than in the control group (10% vs. 35%; P <0.05). By day 3 of treatment, ANC in the GCSF group was significantly higher (3521±327) compared to 2094±460 in the control group, with P value being <0.05. Duration of NICU stay also decreased significantly in the GCSF group. Conclusion: The administration of GCSF in preterms with septicemia and neutropenia resulted in lower mortality rates. Further studies are required to confirm our results and establish this adjunctive therapy in neonatal sepsis.

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Objective: To prospectively ascertain Qatar's national Neonatal Mortality Rate (NMR), Early Neonatal Mortality Rate (ENMR), and Late Neonatal Mortality Rate (LNMR) during 2011, compare it with recent data from high-income countries, and analyze trends in Qatar's NMR's between 1975 and 2011 using historic data. Study Design: A National prospective cohort-study. Materials and Methods: National data on live births and neonatal mortality was collected from all public and private maternity facilities in Qatar (1 ^st January-December 31 ^st 2011) and compared with historical neonatal mortality data (1975-2010) ascertained from the database of maternity and neonatal units of Women's Hospital and annual reports of Hamad Medical Corporation. For inter country comparison, country data of 2009 was extracted from World Health Statistics 2011 (WHO) and the European Perinatal Health report (2008). Results: A total of 20583 live births were recorded during the study period. Qatar's national NMR during 2011 was 4.95, ENMR 2.7, LNMR 2.2, and cNMR 3.33. Between 1975 and 2011, Qatar's population increased by 10-fold, number of deliveries by 7.2 folds while relative risk of NMR decreased by 87% (RR 0.13, 95% CI 0.10-0.18, P>0.001), ENMR by 91% (RR 0.09, 95% CI 0.06-0.12, P <0.001) and LNMR by 58% (RR 0.42, 95% CI 0.23-0.74, P=0.002). The comparable ranges of neonatal mortality rates from selected high-income West European countries are: NMR: 2-5.7, ENMR 1.5-3.8, and LNMR 0.5-1.9. Conclusions: The neonatal survival in the State of Qatar has significantly improved between 1975 and 2011. The improvement has been more marked in ENMR than LNMR. Qatar's current neonatal mortality rates are comparable to most high-income West European countries. An in-depth research to assess the correlates and determinants of neonatal mortality in Qatar is indicated.

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Mesenchymal hamartoma of the liver is the second most common benign liver tumor in children. Typically, it presents as a large benign cystic, solid or mixed liver mass in a child younger than 3 years and amenable to complete resection. We report a neonate with Mesenchymal hamartoma of the liver presenting as giant intra abdominal cyst and its rare association with malrotation of bowel.

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