Restrictive Dermopathy - A Rare Congenital Skin Disorder.

Restrictive dermopathy is a rare, autosomal recessive, lethal congenital skin disorder. This congenital genodermatosis could be mistaken for various other similar skin disorders. Diagnosis is a must in the context of genetic counseling for the subsequent pregnancy. We herein report a preterm male neonate with restrictive dermopathy, with additional feature of multiple bone fractures.

Effect of high dose vitamin d supplementation on vitamin d nutrition status of pre-pubertal children on anti-epileptic drugs - A randomized controlled trial.

BACKGROUND AND AIMS: Patients on long term anti-epileptic drug therapy are prone for Vitamin D deficiency for a myriad of reasons. The aim of this research was to study the effect of high dose vitamin D supplementation on vitamin D nutrition status of children newly started on anti-epileptic drug therapy. MATERIALS: This randomized controlled trial was conducted in a tertiary care Children's Hospital at New Delhi from November 2011 to March 2013. Eighty three children in the age group 5-10 years newly started on anti-epileptic drugs (AED) were randomized into two groups; group A - the intervention group, to whom 60,000 IU vitamin D3 was given orally/month under direct supervision along with AED for a period of 6 months, and group B- the control group, to whom AED without vitamin D3 was given. Serum 25(OH)D, ionized calcium (iCa), total calcium (tCa), inorganic phosphate (iP), alkaline phosphatase (ALP) and parathyroid hormone (PTH) levels were assayed at baseline and at the end of 6 months and were compared within and between the two groups. RESULTS: The mean 25(OH)D in Group A was maintained at 6 months follow up [ 26 ng/ml, 95% CI 20-34 ng/ml] compared to baseline [25 ng/ml, 95% CI -19 to 33 ng/ml] [ p = 0.83]. In group B, there was a significant decrease in 25(OH)D levels at 6 months [13 ng/ml (95% CI 9 ng/ml-17 ng/ml)] compared to baseline [18 ng/ml (95% CI 13-24 ng/ml)] [p = 0.01]. At 6 months, mean serum 25(OH)D was significantly higher in group A as compared to group B (p = 0.005). CONCLUSION: To conclude, oral administration of 60,000 IU vitamin D3/month is sufficient to maintain serum 25(OH)D level and prevent development of vitamin D deficiency in children newly started on AED over a period of 6 months. Non supplementation leads to the lowering of serum 25(OH)D in these children. TRIAL REGISTRATION NUMBER: CTRI/2017/08/009234.

Yield of meconium in non-vigorous neonates undergoing endotracheal suctioning and profile of all neonates born through meconium-stained amniotic fluid: a prospective observational study.

BACKGROUND: Delivery room management of infants born via meconium-stained amniotic fluid (MSAF) is quite controversial. No study has tried to quantify the amount of meconium that can be aspirated from the trachea of a non-vigorous neonate born through MSAF. AIMS: To study the yield of meconium in non-vigorous neonates born through MSAF undergoing endotracheal (ET) suctioning. METHODS: This was a prospective observational study conducted from 1 March 2015 to 31 October 2015. A total of 760 neonates born through MSAF were enrolled. All non-vigorous neonates underwent ET suctioning. Meconium aspirated was quantified as nil (no meconium aspirated), insignificant (when only streaks of meconium were present in the ET tube), minimal (when the whole length of ET tube was filled with meconium) and significant (when the whole ET tube was filled with meconium and it poured into the suction tubing or when repeat suctioning was required). RESULTS: The amount of meconium aspirated from the trachea was nil to insignificant in 46% of the neonates who underwent tracheal suctioning. Only 14.4% of the neonates had significant meconium aspirated from the trachea. Almost 46% of the non-vigorous infants underwent tracheal suctioning without any important amount of meconium being aspirated from the trachea. CONCLUSION: The study further supports the recommendation put forth by the International Liaison Committee on Resuscitation (ILCOR) 2015 that selective intubation and tracheal suctioning of non-vigorous MSAF neonates might be of limited benefit. ABBREVIATIONS: ET: endotracheal; ILCOR: International Liaison Committee on Resuscitation; HIE: hypoxic ischaemic encephalopathy; LMIC: low- and middle-income countries; MAS: meconium aspiration syndrome; MSAF: meconium-stained amniotic fluid; NRP: neonatal resuscitation programme; PPHN: persistent pulmonary hypertension of the newborn; RCT: randomised controlled trial.