ABSTRACT
Background: Optimal resuscitation measures and outcome predictors in cases of burns are not studied in pediatric population, though it accounts for one of the leading causes of non-fatal injuries in the pediatric age group. Objective: We describe the clinical profile and outcome predictors in children admitted with burns. Materials and methods: This retrospective cohort study included all children between 1 month and 18 years admitted to pediatric intensive care unit (PICU) with burns from January 2015 to December 2020. The total burns surface area (TBSA) was assessed and treated as per unit protocol. Illness severity score (PRISM III) and pediatric logistic organ dysfunction (PELOD-II) on day 1 and subsequently was noted. Resuscitation requirements including inotropes and colloids were evaluated. Outcome parameters like need for mechanical ventilation, renal replacement therapy, duration of hospital stay, PICU stay, and mortality were assessed. Results: Of 286 children admitted with burns, 99 had PICU admissions, and of these 59% were males with median (IQR) age 36 (13,72) months. Multivariate logistic regression analysis showed TBSA >40% [adjusted odds ratio (AOR) 4.62 [1.11-19.32] p-value < 0.036]. Cox regression for 28 day mortality was significant only for PELOD day 1 (heart rate (HR) 1.22 [1.05-1.41]). Conclusion: Higher resuscitation requirements with higher organ dysfunction scores may predict mortality in pediatric burns warranting further study for standardization of care. How to cite this article: Yashaswini K, Lalitha AV, Kanna GSN, Kujur AR, Michael Raj JA. Clinical Profile of Children with Burns in a Tertiary Care Hospital. Indian J Crit Care Med 2023;27(12):934-938.
ABSTRACT
OBJECTIVE: Robinow syndrome (RS) is an extremely rare genetic disorder characterized by short-limbed dwarfism, defects in vertebral segmentation and abnormalities in the head, face and external genitalia. Mutations in the ROR2 gene cause autosomal recessive RS (RRS) whereas mutations in WNT5A are responsible for the autosomal dominant (AD) form of RS. In AD Robinow patients, oral manifestations are more prominent, while hemivertebrae and scoliosis rarely occur and facial abnormalities tend to be milder. METHODS: Three unrelated patients from different parts of India were studied. These patients were diagnosed as RRS due to presence of characteristic fetal facies, mesomelia, short stature, micropenis, hemivertebrae and rib abnormalities. One of the patients had fetal facies and micropenis but unusually mild skeletal features. This patient's mother had mild affection in the form of short stature and prominent eyes. Testosterone response to human chorionic gonadotropin was investigated in two patients and were normal. The exons and exon-intron boundaries of the ROR2 gene were sequenced for all probands. Bioinformatics analysis was done for putative variants using SIFT, PolyPhen2 and Mutation Taster. RESULTS: Patients 1, 2 and 3 were homozygous for c.G545A or p.C182Y in exon 5, c.227G>A or p.G76D in exon 3 and c.668G>A or p.C223Y in exon 6 respectively. Prenatal diagnosis could be performed in an ongoing pregnancy in one family and the fetus was confirmed to be unaffected. CONCLUSION: ROR2 mutations were documented for the first time in the Indian population. Knowledge of the molecular basis of the disorder served to provide accurate counseling and prenatal diagnosis to the families.
