Evaluation of vitamin D profile in juvenile idiopathic arthritis.

OBJECTIVE: To observe the association between serum vitamin D level and disease activity in juvenile idiopathic arthritis (JIA). METHODS: The observational study was conducted at a tertiary care hospital during 2017-2019. Patients suffered from JIA were recruited through purposive sampling which was stratified by the disease activity based on the Juvenile Arthritis Disease Activity Score 27 (JADAS27) criteria. Serum vitamin D was estimated alongside other laboratory parameters. The numerical and categorical variables were analysed with appropriate statistical tests. RESULTS: 40 subjects were studied where inactive disease was observed in nine subjects (22.5%), five subjects (12.5%) were found to be in low disease activity and moderate disease activity groups each, and twenty-one subjects (52.5%) had high disease activity. Considering the total sample size of the study, the mean (SD) JADAS27 score and serum vitamin D level were observed to be 12.02 (11.31) and 23.10 (5.93) respectively. A negative correlation was found between the JADAS27 score and serum vitamin D (r= -0.67). The corrected Chi-square test had revealed significant association between the status of serum vitamin-D and disease activity groups (=16.28; p < .001). CONCLUSIONS: In JIA, higher grade of disease activity was found to be significantly associated with lower serum vitamin D.

Childhood lupus nephritis: 12 years of experience from a developing country's perspective.

OBJECTIVE: To assess the long-term outcome of lupus nephritis in children with systemic lupus erythematosus followed up over 12 years at a tertiary care teaching hospital in Eastern India. MATERIAL AND METHODS: This is a retrospective observational study of the clinicopathological presentation, management, and outcome in 46 children with lupus nephritis over a period of 12 years at a tertiary teaching hospital in Eastern India. Mortality was compared between different lupus classes and therapy groups with Kaplan-Meier analysis and log-rank test. RESULTS: The incidence of lupus nephritis was 58.97% [95% confidence interval (CI) 48.06%-59.89%] with the mean age at presentation being 10.2±2.43 years (range 5.5-14.5) years. Majority belonged to class IV (30.43%), followed by class II (26.91%), class III (23.91), and class V (8.70%). Outcome analysis of children with lupus nephritis over 12 years revealed that 24 (52.17%) achieved complete remission of disease activity, 5 attained partial remission, 4 continued to have active disease, 5 developed end-stage renal disease (ESRD), and 8 died. Overall mortality thus observed was 17.39% with septicemia in the background of ESRD being the commonest cause. No significant difference in mortality was observed between different lupus nephritis classes or therapy arm groups. CONCLUSION: The study throws light on various aspects of lupus nephritis and their long-term outcome patterns in children from developing countries such as India.

A misplaced intramuscular injection and limb-threatening ischemia.

Femoral artery thrombosis is a rare complication of intramuscular (IM) injection in children. A 12-month-old boy presented with right lower limb ischemia and digital gangrene 3 days after an injection of ceftriaxone administered to his medial aspect of the right thigh. Successful thrombolysis and partial limb salvage was possible with enoxaparin despite a late presentation. Unnecessary and unsafe IM injection in community practice might lead to such devastating outcome which should be avoided.

Failure to thrive and nephrocalcinosis due to distal renal tubular acidosis: A rare presentation of pediatric lupus nephritis.

A 9-year-old female child was initially diagnosed of having nephrocalcinosis with distal renal tubular acidosis (dRTA) while investigating for short stature. She later on developed features of nephrotic syndrome (NS) while on treatment for RTA. Investigation for the cause of NS revealed very strong serological evidence in favor of systemic lupus erythematosus (SLE). Histopathological confirmation could not be done due to bilateral severely contracted kidneys. There are a few case reports of dRTA as the presentation of SLE, but nephrocalcinosis with dRTA with subsequent manifestation of SLE has hitherto not been reported in literature.

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.

Growth of children with juvenile idiopathic arthritis.

OBJECTIVE: To evaluate the growth pattern in children with juvenile idiopathic arthritis and its subtypes in comparison with age, sex and temporally matched controls. STUDY DESIGN: Prospective study. SETTING: Pediatric rheumatology clinic of a tertiary care hospital in Eastern part of India. PARTICIPANTS: Seventy-five children (2-12 years) diagnosed as juvenile idiopathic erthritis by International League of Associations for Rheumatology criteria and 75 age- and sex- matched controls. INTERVENTION: Weight, height and body mass index were recorded at six monthly interval in both groups over a period of 3 years. MAIN OUTCOME MEASURES: weight, height and body mass index. RESULTS: Subtype 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). CONCLUSIONS: Children 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.

Chronic tubulo-interstitial nephritis in common variable immunodeficiency: a rare association.

Common variable immunodeficiency (CVID) is characterized by reduced serum immunoglobulin levels and repeated serious bacterial infections involving different organ systems. Chronic kidney disease (CKD) is an uncommon association with CVID. Chronic tubulo-interstitial nephritis in a case of CVID that progressed to CKD is distinctly rare.

Childhood Polyarteritis Nodosa: a prospective multicentre study from eastern India.

OBJECTIVE: To delineate the spectrum of clinical presentation and system involvement in childhood Polyarteritis Nodosa (PAN) in a multicentre follow up. METHODS: This prospective observational study included all children less than 12 y attending pediatric rheumatological clinic of four different institutes in West Bengal or admitted with rheumatological symptoms and were diagnosed as childhood PAN by ACR and EULAR/PRES/PRINTO criteria. During the study period of 8 y, 15 cases were categorized as childhood PAN with organ involvement. The children diagnosed as microscopic polyangiitis or cutaneous PAN were excluded. RESULTS: The mean age of presentation was 8.6 ± 2.09 y with male to female ratio of 7:8. All had prolonged fever, severe myalgia, skin involvement and elevated acute phase reactants. Hypertension (93 %) and peripheral neuropathy (46 %) were notable findings. Nine had typical peripheral gangrene with ulceration, whereas rash of livedo reticularis was seen in 6 patients. None had renal, pulmonary or testicular involvement. Diagnosis was established in all patients by skin biopsy which showed necrotizing vasculitis of the medium sized vessels. The clinical presentations and diagnostic parameters were compared between the genders and no statistically significant difference was noted. All patients required corticosteroids while 10 patients needed additional immunosuppressives where there were uncontrolled symptoms of peripheral neuropathy or progressive gangrene despite treatment with corticosteroids. Cyclophosphamide was used most commonly (n = 7). Two required methyl prednisolone. Two children were lost to follow up. One patient had two relapses within three years. No mortality was observed. CONCLUSIONS: This multicentric study on childhood PAN, first of its kind from India, demonstrated a distinctive pattern of system involvement and clinical presentations.

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

Wilson's 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 Wilson's disease presenting with generalized hyperpigmentation of skin who also developed neurological manifestations subsequently. We aim to highlight the importance of keeping Wilson's 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.

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

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.

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.

Acute kidney injury following multiple wasp stings.

BACKGROUND: Common manifestations of hypersensitivity reactions to toxins of stinging insects range from local swelling to angioedema and anaphylaxis. Sometimes it may result in unusual manifestations like intravascular hemolysis, disseminated intravascular coagulation, rhabdomyolysis, etc. Acute kidney injury (AKI) due to immune-mediated acute interstitial nephritis is an extremely uncommon manifestation of insect stings. CASE-DIAGNOSIS/TREATMENT: A 9-year-old boy who developed renal failure from acute interstitial nephritis 7 days after getting stung by a swarm of wasps at multiple sites is described. He regained normal renal function after eight sessions of hemodialysis. CONCLUSIONS: Acute interstitial nephritis resulting in AKI may be either due to immune-mediated tubulointerstitial injury or acute cellular injury caused by obstruction by pigments like hemoglobin and myoglobin. Timely and appropriate supportive management usually cures the patient without any residual damage. The objective of reporting this case is to draw the attention of fellow clinicians towards the possibility of this unusual but life-threatening delayed complication in multiple wasp stings, even if there are no significant immediate reactions.

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.

Profile of child labor in Indian railways.

OBJECTIVES: To determine the socio-medical profile of child workers in Indian railways. METHODS: This was a prospective longitudinal survey over one year. The children up to the age of 14 y entering the reserved second class sleeper compartment of long distance trains in Indian railways were included. The data were collected regarding their profile by the investigators either by interviewing or observation as per situation in the moving train in a predesigned proforma. Data pertaining to the social, demographic and medical aspects were collected. RESULTS: A total of eighty one children were noted in 22500 kilometers of train journey. Sex distribution (8:1) was unequal with males outnumbering females. CONCLUSIONS: This study, first of its kind, attempted to delineate a distinctive socio-medical profile in a special group of children. It might, on the behalf of social pediatrics, increase the awareness and help the society to formulate a rehabilitation policy in collaboration with national and international organizations.

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.

Polyglandular autoimmune syndrome (PGA)--type 2 with diabetic ketoacidosis.

The authors report a rare case of a 5 y old girl with type 2 autoimmune polyglandular syndrome, also called Schmidt syndrome, who presented with diabetic ketoacidosis (DKA) at admission.