Background: Despite the advancements in antenatal diagnostic and postnatal management in neonates with esophageal atresia (EA)/tracheoesophageal fistula (TEF) resulting in better survival rates in the developed world, the outcome still remains poor in developing countries. Materials and Methods: Retrospective analysis of medical records of neonates who were operated for EA/TEF from 1991 to 2015 at our center was done. Results: A total of 693 neonates were operated during the study period with male-to-female ratio 1.9:1. Mean birth weight was 2300 ± 840 g and mean gestational age was 36 ± 4 weeks. Prenatal diagnosis with ultrasonography screening was done in only 9% cases. Mean age of neonates at referral was 4.3 ± 1.5 days. Frothing from mouth and tachypnea were the most common presenting features seen in 94% and 78% neonates, respectively. Type III EA was the most common variety seen. Incidence of associated anomalies was 52% with congenital heart disease being most common. Overall postoperative survival rate of 57% was observed. In the past 10 years of study, survival improved to 64% in comparison to 48% during initial 15 years. Poor prenatal supervision, aspiration pneumonia, prematurity, low birth weight, delayed referral and inadequate transport facilities, associated congenital anomalies, and lack of advanced Neonatal Intensive Care Units (NICUs) facilities were the important contributing factors for high mortality. Conclusion: In developing countries, only improving the NICUs and surgical techniques will not deliver a better outcome in neonates with EA/TEF. Emphasis should be on the strengthening of peripheral health services and transport facilities to achieve good survival rates.

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Objective: The objective of this study is to estimate the incidence of acute kidney injury (AKI) in birth asphyxia and to find out the predictors of AKI in birth asphyxia in a tertiary care hospital. Materials and Methods: This is a cross-sectional study conducted in the neonatal intensive care unit of a tertiary care center in Northern India during November 2014– October 2015. Inborn babies admitted here with severe birth asphyxia were included in the study. The neonates were evaluated for the evidence of AKI and were grouped into two groups: Group I (all neonates with severe birth asphyxia as per the WHO definition and having evidence of AKI) and Group II (all neonates with severe birth asphyxia as per the WHO definition and without having evidence of AKI). Those with congenital renal anomalies were excluded from the study. The two groups were then compared. AKI network definition was used to define AKI. Results: The incidence of AKI in the present study was 44.21%. There was no significant difference in incidence between term and preterm neonates, and among various stages of hypoxic-ischemic encephalopathy. The majority (95%) had nonoliguric renal failure. Most (92.8%) of the cases recovered before discharge and the rest recovered at 1 month follow-up. Prolonged labor was found to be significantly associated with AKI. Patients with shock had more advanced stages of AKI compared to those without shock. Conclusion: From this study, it can be inferred that it is difficult to predict AKI based on clinical features such as oliguria or Apgar score, and it is better to screen all the birth asphyxia cases for AKI so that they can be detected early and managed accordingly. In addition, a single normal value of blood urea/serum creatinine cannot exclude AKI, and serial monitoring is important. Shock should be detected early and treated aggressively as shock was associated with advanced stages of AKI.

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Hyponatremia is common in preterm infants. The causes are usually related to the inability of the premature kidneys to excrete a given water load, excessive sodium losses, or inadequate sodium intake. Here, we present a case of severe hyponatremia in an extreme preterm infant, associated with the use of aminophylline. Aminophylline was administered intravenously on day 1 for the treatment of apnea of prematurity. On day 3, the patient developed hyponatremia which was not responsive to sodium replacement and fluid restriction. Due to concerns of aminophylline-induced hyponatremia, aminophylline was discontinued on day 6, and within 48 h of discontinuation, serum sodium normalized without the need for sodium supplementation. The purpose of the case report is to present a rare complication associated with aminophylline use and to shed light on potential deleterious effects associated with drug shortages.

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Neonatal liver abscess is a rare entity, and till date, fewer than 100 cases have been reported in the literature. In general, they occur in preterm infants with certain risk factors like umbilical vein catheterization. Treatment consists of aspiration, drainage of the abscess with antibiotics. We are reporting here a case of the liver abscess with impending rupture in a 1-month-old male child.

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Aim: The aim of the study was to review timing and substrate of initial enteral feeding in the management of infants with gastroschisis (GS) with analysis of their relationship to nutritional outcomes and length of Neonatal Intensive Care Unit (NICU) admission. Methods: A retrospective review was conducted of consecutive admissions of infants with GS to a quaternary NICU between 2010 and 2016. Demographic, clinical data and data on nutritional status and growth were collected. Relationships between time to initiate enteral feeding and length of NICU admission, duration of parenteral nutrition (PN), and time taken to regain birth weight were assessed, as well as effect of exclusive human milk on length of NICU admission and duration of PN. Results and Conclusions: Survival in this cohort was 100%. Significant positive correlations were found between time taken to initiate enteral feeding after abdominal closure and each of the primary outcomes: length of NICU stay, duration of PN, and time taken to regain birth weight. There was no effect of exclusive human milk feeding on either duration of PN or length of NICU stay. A higher rate of exclusive human milk feeding was observed than in published literature.

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Bart's syndrome, which was first described by Bart in 1966, comprises congenital localized absence of skin, congenital epidermolysis bullosa, and associated nail abnormalities. A newborn infant with Bart's syndrome is reported herein since it is a very rare condition, especially when associated with congenital adrenal hyperplasia. To the best of our knowledge, this is the first report presenting a case of Bart's syndrome associated with congenital adrenal hyperplasia.

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G antigen is present on almost all D+ or C+ cells and absent from virtually all red cells which lack D and C antigens. Anti-G antibody serologically mimics a combination of Anti-C and Anti D. The challenge of anti G in the antenatal setting is to identify whether Anti D is present or not. If anti-D is absent, the female can still get immunized against D antigen, so she will be a candidate to receive Rh immune globulin prophylactic therapy. If anti D is present, the presentation of HDFN may be more severe. So it is important to know if the antibody is really anti G or a combination of anti D and anti C with or without anti G. We here report a case of HDFN which on advanced immunohematolgy work up revealed a combination of Anti G and anti D in mother's serum. Anti-D was present in the mother despite immunoprophylaxis, which indicate inadequate dosage.

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Context: Resistance to thyroid hormone (RTH) is an inherited condition with variable target tissue hyposensitivity. We report two cases of preterm neonates with Neonatal resistance to thyroid hormone syndrome, one with a novel mutation in the “Thyroid hormone receptor – Beta (THRB) gene. Case description: Case 1: A male extremely preterm neonate born at 25 weeks and birth weight of 647 grams. The routine newborn screen raised concerns for hypothyroidism and further labs revealed persistently elevated TSH, free T4. The thyroid antibodies were not elevated. MRI brain did not show a pituitary tumor. The baby had persistent tachycardia and poor weight gain. The baby died eventually due to late onset sepsis as a complication of prematurity at 6 weeks of life. DNA sequence testing revealed that one of the copies had c.1299delC mutation in the THRB gene which is predicted to be pathogenic but not reported in literature. Case 2: A very preterm baby girl born at 30 weeks investigated for thyroid dysfunction due to persistent tachycardia and as mother had recently been diagnosed with resistance to thyroid hormone showed Heterozygous positive for p.PRO453THR mutation in THRB gene. Conclusion: This is the first case report of resistance to TH in extremely/very preterm newborn and one of them with novel mutation, suggesting tissue level differential functional thyroid status. Both these heterozygous mutations affect both THRB1 and THRB2 as exon 10 is necessary for both the protein isoforms. With TSH and T4 being elevated and persistent tachycardia, we think that these newborns had “Pituitary specific RTH”.

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Congenital megalourethra is a rare form of functional obstructive uropathy caused by dysgenesis of the penile corpora cavernosa and spongiosa. Megalourethra is usually described in males and rarely seen in disorders of sexual differentiation. A neonate presented to us with ambiguous genitalia, a megalourethra and imperforate anus. Genetically, the baby was 46XX and the presence of mullerian structures was confirmed during laparotomy. Hormonal workup for congenital adrenal hyperplasia was negative. Although the functional obstruction of the urethra was circumvented by a suprapubic cystostomy, the status of the upper tracts ultimately decided the fate of the child. The presence of crossed fused ectopia with dysplasia and hydronephrosis led to multiple bouts of urosepsis and rapid renal failure. Evaluation of renal as well as other systemic abnormalities is essential for prognosis and planning of treatment.

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Background: The severity of respiratory illness in neonates on invasive ventilatory support is assessed by oxygenation index and alveolar–arterial oxygen gradient (A-aDO₂). Both these parameters need arterial blood gas estimation which is an invasive procedure with attendant complications. Neonates with less severe respiratory disease are managed on continuous positive airway pressure (CPAP). The progress of the disease is generally assessed by noting the changes in FiO₂and positive end-expiratory pressure (PEEP) provided. Blood gas analysis is done for objectively assess the babies who progress to more severe disease. A noninvasive tool such as saturation, oxygen, and pressure index (SOPI) helps in reducing the need for invasive blood gas estimation. A good correlation of SOPI with A-aDO₂can provide near-continuous bedside assessment of the respiratory disease. Objective: To determine the correlation between SOPI and A-aDO₂. Materials and Methods: All babies admitted to our neonatal unit requiring CPAP were considered eligible for this study. The adjustments in FiO₂, CPAP pressures, and arterial blood gas were done as per unit protocol. A-aDO₂was calculated. SOPI was calculated as (PEEP × FiO₂)/SpO₂. The two values were then correlated. Results: Seventy-five babies were recruited. SOPI correlated positively (r = 0.847) with A-aDO₂(P < 0.0001). Coefficient of determination (R²) was 0.71. SOPI value of 1.6 had a sensitivity of 80% and specificity of 90% in predicting the A-aDO₂of 70 which was considered as the value indicating severe illness. Conclusion: SOPI is a noninvasive monitoring tool for babies on CPAP support which has a good correlation of 84.7% with A-aDO_2.This can be used as an objective currency to report the severity of respiratory illness in neonates on CPAP.

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Spontaneously infected cephalhematoma are rare occurrences. Although considered unharmful can lead to anemia, jaundice and infection. Untreated infected cephalhematoma may lead to osteomyelitis, sepsis and meningitis. We present a case of bilateral cephalhematoma, aggravated by massage (a so-called social custom) leading to spontaneous infection and severe anemia, requiring aspiration, and intravenous antibiotics.

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Neonatal hyperthyroidism is a disorder usually caused by the passage of maternal thyrotropin receptor antibodies. Thyrotropin receptor antibodies can be stimulatory, neutral, or inhibitory. We present a case of neonatal hyperthyroidism in a preterm infant born to a mother with Graves' disease that was thyrotropin receptor stimulating antibody negative. Thyrotropin receptor blocking antibody levels were elevated. Anti-thyroid medication could only be weaned once thyrotropin blocking antibody levels significantly declined. We presume the clinical symptoms of hyperthyroidism in the infant were secondary to the interaction of thyrotropin blocking antibodies with the TSH receptor.

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Background: Packed blood cell transfusion in mechanically ventilated neonate is a prime necessity to maintain adequate tissue oxygen supply. Hemoglobin (Hb) is the gold standard for making decision of blood transfusion. Hb estimation is time-consuming in relation to arterial blood gas analysis, total oxygen content (CaO₂) is one of the components of the blood gas. In this study, we wanted to predict CaO₂as a predictor for blood transfusion in mechanically ventilated baby. Materials and Methods: This is a cross-sectional study conducted to assess the performance of CaO₂to detect the indication of packed red blood cell transfusion in mechanically ventilated neonates. CaO₂and corresponding venous hemoglobin were measured in normally perfused baby. Test performance of CaO₂with different cut-off value was validated by receiver operating curve, sensitivity, specificity and positive predictive value (PPV), and negative predictive value (NPV). Results: Prevalence rate of packed red blood transfusion in mechanically ventilated neonate was 53%. With setting a cut-off value for Hb of 13 g/dl for blood transfusion, the best cut-off value of CaO₂for blood transfusion is 18.5 ml/dl and area under curve 0.802, sensitivity is 80% and specificity is 78%, PPV 76%, and NPV 82% and cut value of CaO₂off ≤20 ml/dl area under curve 0.97 (95% confidence interval 0.95–1) and specificity 93% and PPV 96% and NPV 95%. Conclusion: Our study concluded that CaO₂is an excellent predictor of blood transfusion in a positive-pressure ventilated baby, but actual decision should be taken according to clinical condition of the patient.

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Background: Serum bilirubin can be estimated using a technique that is real-time, noninvasive, painless, fast, and relatively inexpensive technique which is transcutaneous bilirubinometry (TcB). There is a paucity of published research data in Nigeria on TcB, and the aim of this study was to determine the correlation of TcB with total serum bilirubin (TSB) and in a group of term Nigerian neonates at Lagos State University Teaching Hospital (LASUTH) using the Konica Minolta JM-103. Materials and Methods: One hundred and fifty neonates were consecutively recruited at LASUTH, and detailed sociodemographic and clinical information was recorded with an interviewer-administered questionnaire. TcB readings were taken on the forehead, sternum, and abdomen of the calm neonate in a supine position, and blood samples for TSB estimation were drawn from a peripheral vein within 10 min of TcB measurement. Results: Eighty-nine (59.3%) neonates were male; 129 (86%) neonates were of 37–39 weeks' gestational age while 56 (37.3%) presented in the clinic after 48 h of life. Over 83% of the neonates had TcB values that were higher than TSB values, and the percentage of neonates with TSB values > 12 mg/dl was 45.2% compared with 56.8% obtained by TcB. The correlation of TcB with TSB using the Pearson's correlation was positive (r = 0.924). The mean error of TcB compared with the TSB level was independent of gender, gestational age, age at presentation, or birth weight. The measured bias by the Bland and Altman method was 0.97 mg/dl (95% confidence interval: 0.74–1.21) while imprecision was ± 2.96 mg/dl. The best correlation at the forehead was r = 0.928. Conclusion: Excellent correlation of TcB with TSB was obtained from this study, and it is envisaged that further researches will be carried out in dark skinned neonates and that the use of the JM-103 will be widely adopted as a screening tool in Nigeria.

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Background: Hyperbilirubinemia is a common and in most cases, benign problem in neonates. Data in term and near-term infants with respect to the pattern of neonatal hyperbilirubinemia from South India are lacking, hence this study. Aim: The aim was to compare the incidence of significant hyperbilirubinemia between near terms and term neonates. Materials and Methods: A prospective case–control study done in a Tertiary Care Hospital, in South India over a period of 18 months (October 2012–April 2014) involving two hundred and sixty-four infants (cases 132 near-term babies, and 132 terms controls). Setting: Tertiary Care Hospital, in South India over a period of 18 months (October 2012–April 2014). Participants: Two hundred and sixty-four infants, cases 132 near-term babies, and 132 terms controls. Exclusion Criteria: Babies with hemolytic jaundice, polycythemia, hypothyroidism, culture-proven sepsis, major congenital anomalies. Cord and 24 h total serum bilirubin was done on cases and controls and all babies were followed up till 72 h or till discharge for significant hyperbilirubinemia. Results: Mean cord blood bilirubin values were significantly different between the near terms and terms 1.9 ± 1.41 and 1.46 ± 0.50 mg/dl (P = 0.0001). Mean 24 h serum bilirubin was higher in near terms, but the difference was not statistically significant (6.89 ± 0.41 and 6.83 ± 0.34, P = 0.764). Incidence of significant hyperbilirubinemia was statistically higher in the near terms as compared to terms (23/109 vs 7/125, P = 0.002). Conclusion: Although cord blood bilirubin was higher in near-term infants, the 24 h bilirubin did not show any significant difference between the two groups. Clinically, however, significant hyperbilirubinemia requiring phototherapy was significantly higher in near-term infants. There is clearly a requirement for at-risk assessment for severe hyperbilirubinemia before discharge and close follow-up thereafter for near-term infants.

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