Ramesh Y Bhat

Meconium-stained Amniotic Fluid and Meconium Aspiration Syndrome: A Prospective Study

Abstract Background: The incidence of meconium aspiration syndrome (MAS), associated perinatal factors, morbidity and deaths varies widely. This study aimed to assess the perinatal attributes and morbidity associated with MAS. Methods: Over a 2-year period, all neonates born through meconium-stained amniotic fluid (MSAF) were observed for respiratory distress (RD). Birth details, chest radiograph (CXR) and clinical course were documented. Neonates with consistent CXR findings whose RD could not otherwise be explained were defined as MAS. Results: Of 409 neonates born through MSAF, meconium was thick in 196 (47.9%). Fifty-five (13.4%) had RD and 45 (11.3%) were consistent with MAS. Six (1.5%) neonates died. Mean (SD) birthweight and gestation of MAS infants were 2721.9 (510.2) g and 38.67 (1.09) weeks, respectively. About one-third were of low birthweight and 28 were born by caesarean section. On univariate analysis, caesarean delivery, meconium in the trachea and thick meconium were the significant perinatal factors for the development of MAS. On multiple regression analysis, thick meconium was the only independent factor for MAS (OR 7.08, 95% CI 3.08–16.27, p<0.001). An Apgar score of ≤ 6 and blood gas abnormalities were found in 44.4% and 48.9%, respectively. Air leak syndrome was observed in 12 (26.7%), persistent pulmonary hypertension in seven and severe asphyxia and late sepsis in two each. Thirteen (28.9%) required mechanical ventilation and six (13.3%) died. Conclusion: MAS occurred in 11.3% of neonates born through MSAF. Thick meconium was found to be an important causative factor. Nearly one-third of infants with MAS required mechanical ventilation and 13.3% died.

Recurrent and novel GLB1 mutations in India.

GM1 gangliosidosis is a lysosomal storage disorder caused by mutations in the GLB1 gene, leading to the deficiency of the enzyme β-d-galactosidase. In this study, we report molecular findings in 50 Asian Indian families with GM1 gangliosidosis. We sequenced all the exons and flanking intronic sequences of GLB1 gene. We identified 33 different mutations (20 novel and 13 previously reported). The novel mutations include 12 missense (p.M1?, p.E129Q, p.G134R, p.L236P, p.G262E, p.L297F, p.Y331C, p.G414V, p.K493N, p.L514P, p.P597L, p.T600I), four splicing (c.246-2A>G, c.397-2A>G, c.552+1G>T, c.956-2A>G), three indels (p.R22Qfs*8, p.L24Cfs*47, p.I489Qfs*4) and one nonsense mutation (p.Q452*). Most common mutations identified in this study were c.75+2InsT (14%) and p.L337P (10%). Known mutations accounted for 67% of allele frequency in our cohort of patients, suggesting that these mutations in GLB1 are recurrent across different populations. Twenty three mutations were localized in the TIM barrel domain, β-domain 1 and β-domain 2. In silico sequence and structure analysis of GLB1 reveal that all the novel mutations affect the function and structure of the protein. We hereby report on the largest series of patients with GM1 gangliosidosis and the first from India.

Time to positivity of blood cultures in neonates.

Blood culture reports in neonatal sepsis aid physician in either optimizing therapy or discontinuing antibiotics. We determined the time taken for neonatal blood cultures to become positive using the aerobic BacT/Alert system. Of 944 blood cultures from 816 neonates, 139 (14.7%) were positive. Growth of all definitive bacteria, 95% of possible bacteria and 84% of fungi were detected within 48 hours of incubation.

Hypomelanosis of Ito with an unusual pulmonary abnormality in an infant.

Hypomelanosis of Ito (HI) is a neurocutaneous syndrome characterized by hypopigmented cutaneous lesions and extracutaneous manifestations frequently affecting the nervous system and the musculoskeletal system. Dysmorphic features, dental, ophthalmic, gastrointestinal, cardiac, and renal abnormalities are described in a minority of patients. The authors describe a 4-month-old infant having HI with unusual pulmonary hypoplasia that has not been reported so far.

Hemiconvulsion-hemiplegia-epilepsy syndrome: clinical course and neuroradiological features in a 20-month-old girl

Hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome involves initial sudden and prolonged unilateral convulsive seizures, followed by transient or permanent hemiplegia and epilepsy during infancy or early childhood. Seizures are prolonged, difficult to control and sometimes may require surgery. Hemiplegia varies in intensity, differs from Todd paralysis and disappears in about 20% of cases. Neuroimaging characteristically shows brain atrophy more pronounced on the hemisphere contralateral to the side of hemiplegia with dilation of the ventricular system. A 20-month-old girl presented with left hemiconvulsions and left hemiplegia lasting for a prolonged period. Seizures failed to resolve with various anticonvulsants even after many physician contacts. Characteristic neuroimaging findings, seizure control with carbamazepine and valproate, subsequent recovery of hemiplegia and attainment of developmental milestones observed on follow-up confirmed HHE syndrome. The case highlights the need for good seizure control in this syndrome.

Bardet-Biedl syndrome in two sisters: A rare incidence

Bardet-Biedl syndrome is an autosomal recessive disorder characterized by retinitis pigmentosa, obesity, polydactyly, mental retardation and hypogonadism. We present two sisters with this rare genetic condition.

Alcaligenes Meningitis in a Neonate

The common bacterial causes of neonatal meningitis are group B Streptococcus, Escherichia coli, Klebsiella and Listeria. Meningitis due to Alcaligenes in neonates is exceptional. Difficulties in its diagnosis and treatment with high mortality have been scarcely reported. We describe meningitis in a neonate due to multidrug resistant Alcaligenes. With sensitive antibiotic therapy, the neonate has survived but found to have severe neurological sequelae on follow up.

Candida arthritis of a hip joint in a neonate.

Candida arthritis is uncommon in neonates. We report a low birth weight neonate who developed septic arthritis of the left hip joint following a stormy course inthe early neonatal period. Ultrasonography of the affected hip joint favored the diagnosis. Arthrotomy yielded 3 mL of pus, which grew candida albicans. Treatment with fluconazole for six weeks resulted in complete recovery.

Sixth hour transcutaneous bilirubin predicting significant hyperbilirubinemia in ABO incompatible neonates.

BackgroundNeonates with ABO hemolytic disease are at greater risk for developing significant hyperbilirubinemia. We aimed to determine whether sixth hour transcutaneous bilirubin (TcB) could predict such a risk.MethodsTcB measurements were obtained at the 6th hour of life in blood group A or B neonates born to blood group O, rhesus factor compatible mothers. Subsequent hyperbilirubinemia was monitored and considered significant if a neonate required phototherapy/exchange transfusion. The predictive role of sixth hour TcB was estimated.ResultsOf 144 ABO incompatible neonates, 41(OA, 24; O-B, 17) had significant hyperbilirubinemia. Mean sixth hour TcB was significantly higher among neonates who developed significant hyperbilirubinemia than those who did not (5.83±1.35 mg/dL vs. 3.65±0.96 mg/dL, P<0.001). Sixth hour TcB value >4 mg/dL had the highest sensitivity of 93.5% and >6 mg/dL had the highest specificity of 99%. Area under receiver operating characteristic curve was 0.898.ConclusionSixth hour TcB predicts subsequent significant hyperbilirubinemia in ABO incompatible neonates.

Maternal anxiety and family support among mothers of neonates admitted in Neonatal Intensive Care Unit

Background: Birth of a newborn requiring admission to NICU represents traumatic experience for some parents. Objective: The present study aimed to determine the maternal anxiety and family support experienced by mothers of neonates admitted in NICU and to find the relationship between them. Materials and methods: A survey approach using descriptive correlative survey design was adopted for the study. Data was collected from 20 mothers of neonates admitted in NICU of Kasturba Hospital, Manipal using purposive sampling. The data collection instruments include: Demographic Proforma, State Trait Anxiety Inventory (STAI), Family Support Scale Results: Findings of the study revealed that majority 65% of mothers had state anxiety whereas 80% had trait anxiety. Mean state anxiety was 52.55± 18.15, trait anxiety was 53.55 ± 14.46. Majority 80% of mothers had high family support.(M=38.4 ± 8.8). Maternal state anxiety (ρ= -0.655, p<0.05) and maternal trait anxiety (ρ= -0.638, p < 0.05) with family support was negatively correlated and it is inferred that as family support increases there will be decrease in the anxiety level of mothers. Conclusion: Mothers exhibit high levels of anxiety during their neonate’s hospitalization. Early identification of anxiety level has implications for support of mothers during hospitalization of their newborns.