Neonatal arterial thrombosis: an arcane COVID sequelae.

We describe a case of a term neonate with a swollen right arm and weakened pulses, diagnosed with arterial thromboembolism in the right axillary and brachial arteries. Treatment involved heparin, followed by enoxaparin, resulting in significant improvement. Maternal SARS-CoV-2 infection during pregnancy was considered as a potential factor, supported by the newborn's reactive COVID antibodies. The authors hypothesise a potential correlation between neonatal thrombosis and maternal SARS-CoV-2 infection during pregnancy. It is important to note that this association remains speculative and warrants further investigation for validation. The case underscores the importance of recognising and managing neonatal arterial thrombosis, especially in the context of maternal illness. We discuss the case in detail and review current knowledge on this condition.

Paradoxical immune reconstitution inflammatory syndrome in neurotuberculosis.

Tuberculosis (TB) remains the most common infection in developing countries and India contributes the maximum number of cases to the global burden. Primary healthcare physicians across the country face major challenges in diagnosis and treatment of childhood TB. In this context, clinical cases of paradoxical responses to antitubercular therapy seem to be under-reported. We report a case of tubercular meningitis in an adolescent girl who belonged to a remote village. She developed a paradoxical immune response to TB while on anti-TB treatment (ATT). She presented with raised intracranial tension and neurological deficits during the continuation phase of ATT after stopping corticosteroids. The ring-enhancing lesions of tuberculomas in the brain and spine characterised the diagnosis of paradoxical response to TB. Brain biopsy suggested necrotising granulomatous disease and was negative for S100 and CD1a marker, ruling out active TB. Retreatment with a prolonged course of steroids and ATT resulted in the clinical and radiological recovery, though some motor and visual deficits persisted. Clinical risk factors and socioeconomic factors also contributed to the present state of the child.

Effect of gestational diabetes on the vitamin D levels in the neonates: a case control study.

BACKGROUND AND OBJECTIVES: The present study was conducted to determine the effect of maternal gestational diabetes on the Vitamin D levels of the mother and their newborns and to compare it with healthy mother-infant pairs. METHODS: The study design was a Case Control study. It was conducted at the antenatal unit of Obstetrics and Neonatal unit of Pediatrics department of a tertiary care hospital in costal Karnataka. Consecutive sample of otherwise healthy pregnant women presenting with Gestational Diabetes Mellitus (GDM) and their healthy term neonates were taken as study group. The weight matched healthy mothers and their healthy term neonates were taken as controls. The blood samples of the mothers, at term and the cord blood samples of the neonates were collected for estimating the Vitamin D levels. Vitamin D levels in the cases and controls were the primary outcome measures. RESULTS: The mean value of Vitamin D levels in the GDM mothers was 10.74 ng/ml and in the mothers forming the control group was 23.53ng/ml (p value < 0.001). The mean value of Vitamin D levels in GDM babies was 8.47ng/ml and was 19.51ng/ml in the control (p value < 0.001). CONCLUSION: Comparison of Vitamin D levels of mothers and infants of both groups showed a positive correlation. GDM seems to exacerbate the Vitamin D deficiency in the mothers and their neonates.

Pregnancy Outcome in Occupational Tobacco Exposure: A Cohort Study from South India.

BACKGROUND: Women constitute a significant labor pool in the Indian tobacco industry as bidi (hand-rolled cigarette) rollers. On an average, they roll around 600 bidis/day and are exposed to 120 g of tobacco and 3 g of nicotine. Bidis do not have chemical preservatives or stabilizing agents, and therefore, the rollers are exposed only to nicotine by handling and inhalation. The study objective was to assess pregnancy outcome in these women with occupational tobacco exposure. MATERIALS AND METHODS: A prospective cohort study of bidi-rollers (n = 177) and women with no tobacco exposure (n = 354), followed up for pregnancy outcome, neonatal anthropometry, and nicotine absorption by cotinine assays. Adjusted risk and adjusted mean differences with a 95% confidence interval were derived. RESULTS: Outcomes included increased adjusted risk for gestational hypertension (3.54 [1.21, 10.31]; P = 0.021) and fetal growth restriction (2.71 [1.39, 5.29]; P = 0.004). Risk for prematurity was not statistically significant (1.81 [0.74, 4.45]; P = 0.194). Lower adjusted mean difference of birth weight (-104 g [-177, -31]; P = 0.005), length (-0.4 cm [-0.8, -0.1]; P = 0.006), and head circumference (-0.4 cm [-0.6, -0.1]; P = 0.002) were seen with increased risk for small for gestational age (1.75 [1.12, 2.73]; P = 0.015). Nicotine absorption was evident in one-third of maternal and cord blood estimations. CONCLUSION: Occupational passive tobacco exposure results in adverse pregnancy outcome.

Renal scarring following urinary tract infections in children.

Urinary tract infection (UTI) is the most common bacterial infection seen in younger age group children. The most common risk factor for renal scarring in children with post-UTI is vesicoureteral reflux (VUR). This study looked at renal scarring following UTI in children by dimercaptosuccinic acid (DMSA) scan. It is a cross-sectional time bound study done among 40 infants and children with UTI for a period of 24 months. Their clinical presentation and laboratory data, including micturating cystourethrogram, were documented. DMSA scan, done during the follow-up, established the presence of renal scars if any. UTI was more common in males. Children with history of recurrent UTI had renal scarring. Statistical significance was noted when first and recurrent attacks of UTI were compared for clinical and diagnostic characteristics like Escherichia coli UTI (p = 0.007), increasing grades of VUR (p = 0.006) and renal scar formation (p = 0.041). The study concluded that the high grades of antenatal hydroureteronephrosis (100%), febrile UTI (34%), younger age group (67.5%) and E. coli UTI (86%) were more associated with recurrent attacks of UTI and renal scarring. DMSA, being the non-invasive test, is better proven to be an investigation of choice than other invasive modalities for follow-up of children with recurrent attacks of UTI.

Audit of Organic Acidurias from a Single Centre: Clinical and Metabolic Profile at Presentation with Long Term Outcome.

INTRODUCTION: Organic Acidurias (OA) accounts between 10% and 40% of confirmed Inborn Errors of Metabolism (IEM) in India. With prompt recognition and management, better survival but adverse neurodevelopmental outcome is reported. AIM: To study the clinical and metabolic presentation, management with immediate and long term outcome of symptomatic children with confirmed OA. MATERIALS AND METHODS: Hospital based study of symptomatic children diagnosed to have OA between 2003 and 2009 and the survivors followed up over next five years. Diagnosis was based on clinical and metabolic presentation and confirmed by spectrometry analyses of urine and blood. Management, immediate outcome, compliance to treatment and recurrence of crises were documented. Neurodevelopmental outcome was assessed in follow up. Mean with Standard Error (Mean ± SE) and frequencies with percentages were calculated. RESULTS: Of 72 cases suspected to have IEM, 38 (52.8%) were confirmed of (IEM), and out of which 15 (39.5%) had OA. Methyl malonic acidemia, multiple carboxylase deficiency and Propionic Acidemia (PA) constituted the largest proportion. Neurodevelopmental issues (73.3%) and metabolic crisis (53.3%) were common presenting features. Mean ± SE of ammonia was 639.0±424.1 µg/dl and lactate was 33.6±4.9 mg/dl. Mean pH, bicarbonate, and anion gap was 7.27±0.07, 14.1±2.3 and 17.9±2.3 respectively. Management was protocol based. Death was reported in two cases of PA; other morbidities were seen in five. Recurrent crisis (46.7%) complicated the follow up in survivors. Spasticity, extrapyramidal movement disorder, intellectual subnormality, autism spectrum, attention deficit hyperactivity disorder and sensory neural deafness were seen amongst survivors, in spite of compliance to therapy. CONCLUSION: OA is part of differential diagnosis in sick children and treatment needs to be prompt and specific. Prognosis is guarded even with long term cofactor supplementation in the symptomatic.

Fatty Acid oxidation disorder with secondary mitochondrial energy production defect: a case report.

The presentation of long-chain fatty acid oxidation disorders (FAOD), unlike short and medium-chain disorders can be with secondary defects in mitochondrial function along with typical features of FAOD. We report an infant with Reye-like presentation and acylcarnitine profile suggestive of very-long-chain acyl-CoA dehydrogenase deficiency who had lactic acidosis and urine gas chromatographic pattern of mitochondrial defects.

PHACE syndrome in antenatally diagnosed posterior fossa anomaly.

PHACE is a neurocutaneous syndrome, an acronym to describe patients with facial segmental hemangiomas and other malformations. We describe a newborn antenatally diagnosed to have posterior fossa anomaly and subsequently as PHACE syndrome.

Biochemical basis of heterogeneity in acute presentations of propionic acidemia.

Propionic acidemia (PA), an uncommon organic acidemia has varied clinical and metabolic presentation causing difficulty and delay in the diagnosis. We report a case of PA in an infant who presented with failure to thrive, acute encephalopathy due to severe hyperammonemia without acidosis and fungal sepsis. The biochemical basis of severe hyperammonemia is discussed.