Rakesh Mondal

Childhood lupus: Experience from Eastern India

ObjectiveTo delineate the clinical behavior of SLE in children from Eastern India and to the differences in disease pattern.MethodsIn the present study, all 44 patients of pediatric SLE who were diagnosed over a period of 5 years in our pediatric rheumatology clinic were followed prospectively. The resultant database was analyzed using standard statistical methods.Results|About 3.9% of all rheumatology cases dealt with in the clinic in the last five yrs (n=1063) were SLE. The number of children in 5–8 yrs and 8–12 yrs age groups were 13 and 27, respectively. The overall female (n=35) to male (n=9) ratio in this study was 3.8:1. Renal, hematological and Neuropsychiatric features were most common major organ manifestations(54%,54% and 25% respectively). Joints and skin were the most common minor organs involved. Two case were ANA negative SLE. Among the typical features of ANA negative disease, only nephritis was found in these patients. Anti dsDNA was positive in 50 % cases (n=21). C3 levels were studied in all cases with nephritis (n=22) and 68 % (n=15) had hypocomplementemia. Anti Ro and anti La antibodies were positive in two cases of neonatal lupus. APLA, Anti Sm antibody and anti U1RNP were negative in the cases where testing was doneConclusionsThis study has tried to delineate the disease trends of childhood lupus from Eastern India. Certain important trends have emerged which are different from other contemporary Indian and International observations.

Osteogenesis imperfecta with bleeding diathesis

Osteogenesis imperfecta (O1) is a disorder of type 1 collagen synthesis with varied complications in form of brittle bones, hearing loss, dental, skin, and ocular changes. Osteogenesis imperfecta may have bleeding diathesis rarely due to defective vascular integrity for the same reason. Here we come across a rare case of Sillence type IB Osteogenesis imperfecta with bleeding in the form of repeated epistaxis and petechial rash, which seem to have a collagenous link

Jervell-Lange Nielsen syndrome in a family with the long QT syndrome (LQTS)

A child with Jervell-Lange Nielsen syndrome is presented from Kolkata. Family study showed that the other family members are suffering from long QT syndrome. The child had frequent syncopal attack and very prolonged QT interval requiring left cardiac sympathetic denervation and beta-blocker therapy as patient could not afford implantable defibrillator and cardiac pacing.

Growth of children with juvenile idiopathic arthritis

ObjectiveTo evaluate the growth pattern in children with juvenile idiopathic arthritis and its subtypes in comparison with age, sex and temporally matched controls.Study designProspective study.SettingPediatric rheumatology clinic of a tertiary care hospital in Eastern part of India.ParticipantsSeventy-five children (2–12 years) diagnosed as juvenile idiopathic erthritis by International League of Associations for Rheumatology criteria and 75 age- and sexmatched controls.InterventionWeight, height and body mass index were recorded at six monthly interval in both groups over a period of 3 years.Main outcome measuresweight, height and body mass index.ResultsSubtype distribution of juvenile idiopathic arthritis was: oligoarthritis (49%, n=37), rheumatoid factor negative polyarthritis (27%, n=20), rheumatoid factor positive polyarthritis (8%, n=6), systemic onset (15%, n=11) and enthesitis related arthritis (1.3%, n=1). Anthropometric parameters in children with juvenile idiopathic arthritis were not significant different from controls. Comparison between the subtypes showed significant differences in height (P=0.011), weight (P=0.005), and growth velocity (P=0.005), but not in body mass index. Systemic onset disease led to significant restriction in height (P=0.018; 95% CI 2.13–33.77) and weight (P=0.008; 95% CI 1.47–14.43) compared to controls. Growth velocity was significantly affected in rheumatoid factor positive polyarthritis (P=0.003; 95% CIO. 46–3.14).ConclusionsChildren with juvenile idiopathic arthritis do not have significantly lower values of anthropometric parameters compared to controls. Significant restriction in height and weight is seen in systemic onset disease, and growth velocity is significantly reduced in rheumatoid factor positive subjects.

Acute stress-related psychological impact in children following devastating natural disaster, the Sikkim earthquake (2011), India

Background: Psychological stress following natural disaster is common. Despite several earthquakes in India, data on evaluation of acute stress among the child victims in the early postdisaster period is scarce. Immediately following a devastating earthquake (6.9 Richter) at Sikkim on September, 18 2011, many children attended North Bengal Medical College, the nearest government tertiary care institution, with unusual stress symptoms. Objective: Evaluation of acute stress symptoms in children in the immediate postearthquake period. Materials and Methods: This was a cross-sectional study done over 4 weeks and includes all the children from 1 to 12 years presenting with unusual physical or behavioral symptoms. Those with major injuries requiring admission were excluded. They were divided into two age groups. For older children (8-12 years) the 8-item Children Impact of Event Scale (CIES) was used for screening of stress. Unusual symptoms were recorded in younger children (1-8 years) as CIES is not validated < 8 years. Result: A total of 84 children (2.66%) out of 3154 had stress symptoms. Maximum attendance was noted in first 3 days (65.47%) and declined gradually. In children ≥ 8 years, 48.78% had psychological stress, which was statistically significant on CIES scores without any gender predilection. Static posturing (41.86%), sleeplessness (32.55%), anorexia (9.30%), recurrent vomiting (13.95%), excessive crying (13.95%), or night-awakenings (4.65%) were found in younger children (n = 43) and three required admission. Conclusion: This study represent the first Indian data showing statistically significant psychological impact in older children (8-12 years) and various forms of physical stress symptoms in young children (1-8 years) following earthquake.

Tacrolimus Induced Diabetic Ketoacidosis in Nephrotic Syndrome

Tacrolimus, a reversible calcineurin inhibitor, is known for its diabetogenic potential. The incidence of diabetes is less frequent among the patients of nephrotic syndrome in comparison to organ transplant recipients. Diabetic ketoacidosis (DKA) is even rarer. DKA as the first presentation of new onset tacrolimus induced transient type 1 diabetes despite a lower dose range and low trough level of the drug is being reported in a 12-y-old girl with steroid resistant nephrotic syndrome.

Normative blood pressure data for Indian neonates.

ObjectivesTo establish the normative blood pressure (BP) values in healthy Indian neonates using oscillometric method, and to develop BP percentile charts.DesignProspective observational study.SettingNeonatal unit of a teaching hospital in Eastern India.Participants1617 hemodynamically stable inborn neonates without birth asphyxia, major congenital anomaly, maternal complications (e.g. preeclampsia, hypertension, diabetes) or critical neonatal illness.ProcedureQuite state measurements of systolic BP (SBP), diastolic BP (DBP) and mean arterial pressure (MAP) were recorded by oscillometric method on day 4, 7 and 14 of postnatal life. The averages of three readings at 2-minute intervals were used.ResultsPercentile charts (providing 5th, 10th, 25th, 50th, 75th, 95th, and 99th percentile values) have been developed. SBP, DBP and MAP showed a steady rise from day 4 to day 14, and were comparable between males and females, but were significantly lower in preterms than in term neonatesConclusionNormative neonatal BP data along with gestational age-wise percentile charts shall be of help for decision-making and planning for sick newborns.

A pediatric case series of abdominal epilepsy

BackgroundAbdominal epilepsy (AE) is an infrequent cause of recurrent abdominal pain in children. It is characterized by paroxysmal episodes of abdominal pain, a variety of other abdominal complaints, electroencephalogram abnormalities, and response to anti-epileptic agents. We described the clinical profile of six patients with AE.MethodsWe conducted a retrospective survey of AE in children from the records of the hospital. The diagnosis of AE was dependent on recurrent abdominal symptoms, subtle central nervous system abnormalities, electroencephalogram abnormalities and response to anticonvulsant agents.ResultsThe six patients were diagnosed with AE. The incidence of the disease was 0.07% in all admissions to the pediatric ward. Recurrent pain was common in all patients except two who had additional recurrent vomiting. In this series, the boy to girl ratio (1:2) was unequal.ConclusionHigh suspicion is required for the diagnosis of AE after exclusion of other possible causes.

Generalized hyperpigmentation in Wilson's disease: An unusual association.

Wilsons disease, an autosomal recessive disorder of copper metabolism, most commonly presents either with hepatic or neurological features. But, it may sometimes have certain atypical presentations posing diagnostic difficulties. We report here a case of Wilsons disease presenting with generalized hyperpigmentation of skin who also developed neurological manifestations subsequently. We aim to highlight the importance of keeping Wilsons disease as one of the differentials in patients who present with hyperpigmentation and neurological symptoms compatible with copper deposits in the central nervous system and proceed for investigations accordingly.

Phenytoin induced life threatening macroglossia in a child

Isolated acquired macroglossia of tongue rarely reported. It occurs due to causes like hereditary angioedema, localized angioedema, etc., Here we describe an 8-year-old boy developing life threatening localized angioedema of tongue due to phenytoin without any association with drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome or pseudolymphoma encountered in rural medical college. Anticonvulsants, that is, phenytoin induced this isolated peculiar complication, which was not described before.