Klippel–Trenaunay syndrome and Sturge–Weber syndrome are rare disorders with neurologic and cutaneous signs of vascular origin. Phakomatosis pigmentovascularis represents the association of widespread, aberrant, and persistent nevus flammeus and pigmentary abnormalities. We describe a case with features suggestive of overlap between them. A ten-month-old boy presented with seizures, developmental delay, skin lesions on face, trunk and legs, buphthalmos and right lower limb hypertrophy. CT scan of head showed atrophy of brain and calcification. Our case had overlap of Klippel–Trenaunay syndrome and Sturge–Weber syndrome with phakomatosis pigmentovascularis

Background:To compare the efficacy and safety of intravenous levetiracetam and phenytoin in status epilepticus.Methodology:A prospective, randomized controlled, nonblinded study was conducted in children 1 month to 12 years of age with active seizure and with status epilepticus. A total of 104 children were randomly allocated to either group 1 (levetiracetam) or group 2 (phenytoin) on the basis of computer-generated random number table. Children already on antiepileptic drugs, very sick children with shock, impending respiratory failure, or head injury, and children hypersensitive to phenytoin or levetiracetam were excluded. Data analysis was done by IBM SPSS statistics.Results:The mean age was 4.09 years with a male preponderance with the most common type of seizure being generalized type (74%). The seizures were controlled in all 104 patients initially within 40 min. Seizure control for 24 h was significantly better in group 1 (96%) when compared with group 2 (59.6%) (P = 0.0001). Minibolus of drug was given in 28.8% in group 1 and 46.2% in group 2 (P = 0.068). The seizure recurrence in groups 1 and 2 in the first hour was 1.9% and 5.8%, respectively (P = 0.61), whereas the recurrence between 1 and 24 h was significantly more in group 1 (34.6%) when compared with group 2 (3.8%) (P = 0.0001). The mean time to control seizure was comparable between both the groups (P = 0.71). There was no significant adverse effect in both the groups.Conclusion:Levetiracetam is more effective than phenytoin for seizure control for 24 h in children with status epilepticus, and it is safe and effective as a second-line therapy.

Background:Corticosteroids are the main therapy of nephrotic syndrome and goal of corticosteroid therapy is to obtain maximum clinical benefit with minimum adverse effects. Children are more vulnerable to side effects of corticosteroids related to growth and adrenal suppression, so a search for an alternative steroid with fewer side-effects is underway. Deflazacort is an oxazoline derivative and preliminary data suggest reduced osteoporosis, lesser growth retardation and weight gain with deflazacort. Objectives:This study was done to compare the effectiveness and safety of deflazacort in idiopathic nephrotic syndrome. Patients and Methods:Twenty five children with age between 2 to 12 years, with idiopathic nephrotic syndrome were enrolled. They were randomly assigned to receive deflazacort (Group A, n = 12) or prednisolone (Group B, n = 13) and were followed up for six months. Results:All children of group A and 11 of group B had remission. Two children from group B were steroid resistant. Mean time taken to induce remission was significantly (P = 0.012) less in group A (10.25 ± 2.41 days) than group B (12.55 ± 1.44 days). One patient in group A had relapse on follow up as compared to 3 in group B (P = 0.58). Statistically significant difference (P = 0.03) in change in mean height was found between group A (2.13 ± 0.50cm) and B (1.44 ± 0.45 cm), with group B gaining less height. Conclusions:Remission rate in both groups was comparable although time taken to induce remission was shorter in deflazacort group and there was a significant difference in change of mean height on follow up with prednisolone group gaining lesser height.

One week of albendazole therapy is as effective as 4 weeks of therapy in children with SSECTLs.

Background: With the sudden outbreak of coronavirus pandemic in 2020, there is a paucity of data on the clinical and epidemiological profile of COVID positive paediatric patients. Objective of the study was a retrospective analysis of both COVID-19 and non-COVID-19 cases admitted to a district Hospital of Northern India.Methods: A retrospective cross-sectional study was conducted in the Pediatric Emergency Department of Government Multi-speciality Hospital, Chandigarh from 22 March 2020 till 30 November 2020.Results: A total of 842 children were enrolled. Among neonates, 33% had sepsis as the major admitting diagnosis and SARI in children less than 1 year (excluding neonates) (33.5%) and in the 1-5 years age group (37.3%). In children more than 5 years, acute febrile illnesses were the main admitting diagnosis (50.4%). 204 patients (24.2%) were tested for COVID-19. 62 children (30.4%) were found to be positive. Asymptomatic children (74.2%) dominated the COVID-19 positive group. The most common symptom included fever (87.5%) with no reported mortality. Atypical symptoms (56.1%) were more common than respiratory symptoms. The majority of children had mild COVID-19 symptoms (62.6%). The unusual presentation of COVID-19 was in the form of acute appendicitis (6.2%) and renal vein thrombosis (6.2%) in the symptomatic group.Conclusions: For children with COVID-19, most of them were infected via family clustering, were asymptomatic, and had mild symptoms with an overall good prognosis. However, for atypical cases, a high suspicion of COVID-19 associated multisystem inflammatory syndrome should be kept in mind for early diagnosis.

Vitamin D-dependent rickets type 2 (VDDR2) is a rare autosomal recessive (AR) disorder caused either by a mutation in the Vitamin D receptor gene or overexpression of the binding protein leading to end-organ resistance to 1,25 (OH)2 vitamin D3 or defective hormonal actions respectively. It clinically represents growth retardation presenting in the 1st year of life and is frequently associated with alopecia totalis and markedly elevated levels of 1, 25(OH)2 D, which differentiates it from VDDR type 1. We hereby report siblings of a family, who presented with clinical, radiological features of rickets and alopecia totalis. To our knowledge, only a few cases have been reported in literature describing the AR-pattern and low 25(OH)D3 levels in VDDR2.

Background: Acute bronchiolitis is a disorder of the lower respiratory tract that occurs mostly in between 1 month to 24 months. There are limited numbers of studies on magnesium sulphate nebulization in acute bronchiolitis. There is a desperate need for a standard treatment which can reduce the morbidity and mortality in acute bronchiolitis. The goal was to study the efficacy of magnesium sulphate nebulization in infants with acute bronchiolitis.Methods: It was a prospective open labelled randomized controlled study. Children in age group of 2-12 months admitted with clinical diagnosis of acute bronchiolitis in paediatric emergency ward, GMSH-16, Chandigarh. Study group patients were treated with 40 mg/kg magnesium sulphate nebulization diluted with 2-3 ml normal saline in addition to supportive treatment as oxygen and IV fluids, three doses of medication were given at 1 hour interval and it was not repeated after three doses. Control group patients were treated with oxygen therapy, IV fluids, symptomatic treatment for fever and supportive care.Results: Respiratory distress assessment instrument score and length of hospital stay was significantly lesser in study group as compared to control group. SpO2 was significantly more in study group as compared to control group.Conclusions: Magnesium sulphate nebulization is effective in improving respiratory distress and oxygen saturation, it also reduces duration of hospital stay in infants with mild to moderate bronchiolitis.