Madhumita Nandi

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.

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.

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.

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.

Organophosphorus-induced extrapyramidal intermediate syndrome in an adolescent suicide attempt survivor

The victims of organophosphorus (OP) pesticide poisoning usually present with acute cholinergic crisis, due to the inhibition of the enzyme acetylcholinesterase. Any neurological complication in the form of intermediate syndrome is rare and its presentation with extrapyramidal symptoms is even rarer. The authors report such a case in a 12-year-old adolescent girl, who survived a near lethal suicidal attempt.

Karate Related Injury- Are We Prepared?

To the Editor: Growing interest in martial arts training is a welcome trend because of its’ multifaceted advantages. But, while encouraging our children to undergo this training we should be watchful of the possible life threatening events. A 10-y-old girl was admitted with acute onset severe pain and restriction of movement of neck following a punching injury during a karate practice session. There was no neurodeficit. She did not have any predisposing condition for atlanto-axial instability (AAI). X rays and MRI scan demonstrated atlanto-axial subluxation with backward bending of odontoid process and kinking of medulla oblongata. The atlanlanto-axial distance in the anteroposterior plane was 6 mm (Fig. 1). As martial arts coaching centers are coming up, we need to introspect the preparedness to deal with such accidental injuries. Karate not only strengthens physical and psychological fitness, but also has a role in self-defense. Efforts are on by the authorities to train girls in martial arts in the light of recent spurt in crime against women [1]. Although we could find a few studies from other countries on incidence of martial art related injuries [2, 3], there is hardly any from India. In a study on injury incidence in 3 types of martial artskarate, taekwondo, and judo, most injuries were attributed to karate (79.5 %) [4]. However, we could not find any specific information on the incidence of atlanto-axial subluxation nor could we find any guidelines regarding screening for atlanto-axial instability before imparting martial arts training. There is an urgent need to frame regulatory guidelines, regarding the need of expertise to anticipate and manage accidents. Pre-training screening guidelines for eligibility to undergo the physical demands of martial arts training and an injury surveillance system should be in place. The American Academy of Pediatrics (AAP) guidelines for eligibility for sports participation in certain medical conditions is a welcome step in this direction [5]. It is high time the Indian Academy of Pediatrics also formulates recommendations for our population. We aim to stimulate thoughts of fellow clinicians towards formulation of such guidelines so that Fig. 1 Sagittal section of T1 weighted image (T1WI) showing widening of atalanto axial distance more than 6 mm with Dens compressing the cortico-medullary junction resulting in kinking of spinal cord

Renal involvement in childhood lupus: A study from Kolkata, India

Systemic lupus erythematosus (SLE) is a multi-system disease of autoimmune origin. The relative incidences of the various manifestations in children with SLE are significantly different from adults and among different age-groups of children. To analyze the characteristics of childhood lupus nephritis (LN), we prospectively followed-up 23 cases of pediatric LN, diagnosed over a period of five years, in the pediatric rheumatology clinic at the Institute of Post Graduate Medical Education and Research, Kolkata, India. The resultant database was analyzed using standard statistical methods. Of all childhood lupus cases treated in our clinic over the last five years (n = 42), 54.7% (n = 23) had renal involvement. This study has tried to delineate the disease trends of childhood lupus from Eastern India. Certain important trends have emerged that are different from other contemporary Indian and International observations.

Unilateral Pulmonary Hypoplasia with Abdominal Situs Inversus

A case of unilateral pulmonary hypoplasia in association with abdominal situs inversus (Situs Inversus Partialis) is described here in a 2-mo-old baby. The normally related heart (levocardia) is dextroposed due to the hypoplastic right lung and compensatory hyper-inflation of the opposite lung which clinically mimicked a mirror-image situs inversus totalis. Such a combination, to the best of authors’ knowledge, has never been reported in the world literature.

Immune thrombocytopenic purpura in a child with thyroid hormone resistance – a rare presentation

Abstract Resistance to thyroid hormone (RTH) is a rare entity characterized by a decreased target tissue responsiveness of thyroid hormones. Although immune thrombocytopenic purpura (ITP) has been reported with different thyroid disorders in the literature, its coexistence with RTH is not known. A 9-year-old girl presented with ITP and features of hypothyroidism in the form of goiter and growth retardation. She was subsequently found to have RTH. High-dose thyroid hormone replacement was required to overcome the resistance that not only ameliorated the features of hypothyroidism but also brought an apparent remission of ITP.

Overlap Syndrome: A Child Cohort

Sir, Overlap syndrome is defined by the presence of two or more rheumatic diseases either on the basis of clinical involvement and/or presence of autoantibodies [1]. These disorders are ill defined and difficult to categorize. The most defined entity amongst them is mixed connective tissue disease (MCTD). The studies of child cohort with overlap syndrome are lacking in literature. A prospective observational study was done at the pediatric rheumatology clinic over a period of 8 y (2004–2011) to detect the incidence and delineate the pattern of overlap syndrome. ACR criteria were used for diagnosis of all rheumatological cases. Overlap was diagnosed, either at presentation or at follow up, by clinical features of two major rheumatic diseases along with autoantibodies or positive biopsy findings as applicable. Out of the total 1,544 children with rheumatological diseases, 6 had overlap syndrome (Table 1). Four had overlap at presentation while two developed it 6–7 mo later. The overall incidence was 3.89 per 1,000 (95 % CI 0.03–7.74 per 1,000) with female preponderance (F: M0 5:1). MCTD was seen both in boy and girl, one each. Out of 210 Juvenile Idiopathic Arthritis (JIA) patients, 2 developed overlap syndrome with features of cutaneous scleroderma {2/210 approx (1 %), M: F01:1[2]. Two girls out of 44 childhood lupus developed overlap. One had juvenile dermatomyositis whereas other had cutaneous scleroderma {2/44 (4.2 %)} [3]. Both patients had renal involvement. Incidence of overlap in adults was reported to be 25 % from UK and 20 % involving mixed population from Poland [4, 5]. Our study, first of its kind from India, demonstrated much lower incidence in children. Study of overlap syndrome in children regarding diagnostic criteria, autoantibody profile and prospective cohort is not documented in literature except sporadic case reports [1]. So the present study of child cohort with overlap syndromes may give some insight till the larger series in child population is available. Pediatricians must be careful and vigilant in searching overlap in rheumatological cases either at presentation or at follow up. R. Mondal : I. Banerjee Pediatric Rheumatology Clinic, Department of Pediatric Medicine, North Bengal Medical College, Darjeeling, West Bengal, India