Successful use of tocilizumab in amyloidosis secondary to systemic juvenile idiopathic arthritis.

Amyloidosis secondary to juvenile idiopathic arthritis is a known complication of poorly controlled systemic juvenile idiopathic arthritis (SJIA), occurring in 1-2% of the patients. The IL-6 inhibitor tocilizumab is effective in controlling systemic signs and symptoms of sJIA and may be of therapeutic benefit in secondary amyloidosis. Herein, we report the clinical timeline of a 10-year boy with sJIA and secondary amyloidosis, who showed a sustained improvement of systemic symptoms and a reduction in proteinuria with tocilizumab. Compared to the data on adult patients affected with the secondary amyloidosis, there are very few reports on therapeutic options for the children affected with SJIA and secondary amyloidosis in the paediatric population. While doing a systematic literature search for writing this review, we could only retrieve nine case reports and one case series of the children affected with SJIA and secondary amyloidosis, including five cases which were treated with tocilizumab. We also looked into the clinical and biochemical response to various agents that have been used in the previous cases, including tocilizumab. The available literature and the present case report suggest that tocilizumab may be considered as a safe and effective option to treat SJIA-related secondary amyloidosis.

Juvenile dermatomyositis with IgA nephropathy: case-based review.

Juvenile dermatomyositis (JDM) is the most common childhood idiopathic inflammatory myopathy (IIM). It is characterized by the classic skin rash in the form of Gottron papules and heliotrope rash, and symmetric proximal muscle weakness. Renal involvement in JDM is rare which includes acute kidney injury and glomerulonephritis. We report a 10-year-old boy with juvenile dermatomyositis and IgA nephropathy. Child responded dramatically to the conventional therapy with steroids and methotrexate for the primary disease, and did not require any additional treatment for his renal disease. Child's primary disease is in remission and has normal urinalysis with normal renal function at 6-month follow-up. We reviewed the literature and found 11 cases of IIMs with renal involvement. Four patients (one JDM, two polymyositis, and one dermatomyositis) had IgA nephropathy out of which three patients responded to the conventional therapy of primary disease and only one patient with polymyositis needed hiking immunosuppression targeted for renal condition. Therapy targeting the underlying disorder is usually sufficient in patients with JDM and secondary IgA nephropathy.

Dyslipidemia Among Overweight and Obese Children in Jharkhand: A Hospital-Based Study.

OBJECTIVE: To study the prevalence of common dyslipidemia among overweight and obese children between 2-18 years in a tribal region. METHODS: This cross-sectional study was carried out in 151 overweight and obese children aged 2-18 years, who visited the pediatric outpatient department of a tertiary care hospital in Jharkhand between 1 August and 30 November, 2022. Dyslipidemia was defined as anyone of the following: a total cholesterol level of 240 mg/dL, a triglyceride level of 150 mg/dL, a low-density lipoprotein cholesterol level (LDL-C) of 140 mg/dL, a high-density lipoprotein cholesterol level (HDL-C) of 40 mg/dL, or the use of a lipid-lowering drug [8]. Overweight and obesity were defined as per World Health Organization criteria. RESULTS: The prevalence of dyslipidemia was 63.6%. The most common type of dyslipidemia was low HDL-C + high TG levels found in 32.5% (n=49) children. The most prevalent pattern of dyslipidemia in overweight children was low HDL-C (19, 32.3%); and in obese children low HDL-C and high TG levels (39, 42.3%). CONCLUSION: The prevalence rate of dyslipidemia was high among overweight and obese children in this region. There was a positive association between dyslipidemia and body mass index.

Effect of internal migration on diabetes and metabolic abnormalities in India - The ICMR-INDIAB study.

AIMS: To assess the effect of migration (rural-to-urban and vice versa) on prevalence of diabetes and metabolic disorders in Asian Indians participating in the Indian Council of Medical Research-India Diabetes (ICMR-INDIAB) study. MATERIALS AND METHODS: The ICMR-INDIAB study is a national study on diabetes and associated cardiometabolic disorders in individuals aged ≥20 years from 28 states and 2 union territories of India. Individuals who moved to a different place from their place of birth and had resided in the new location for at least one year were considered as migrants. Anthropometric measurements, blood pressure estimation and a capillary oral glucose tolerance test were performed. RESULTS: Of the 113,043 participants, 66.4% were non-migrant rural dwellers, 19.4% non-migrant urban dwellers, 8.4% rural-urban migrants, 3.8% multiple migrants and 2.0% urban-rural migrants. Weighted prevalence of diabetes was highest in rural-urban migrants followed by urban dwellers, urban-rural migrants and rural dwellers [14.7%, 13.2%, 12.7% and 7.7% respectively (p < 0.001)]. Rural-urban migrants had highest prevalence of abdominal obesity (50.5%) compared to the other three groups. The risk for diabetes was 1.9 times higher in rural-urban migrants than among rural dwellers. Five risk factors [hypertension, abdominal and generalized obesity, physical inactivity and low fruit and vegetable intake] together explained 69.8% (partial population attributable risk) of diabetes among rural-urban migrants and 66.4% among non-migrant urban dwellers. CONCLUSIONS: Rural-to-urban migration is associated with increased risk of developing diabetes and other cardiometabolic abnormalities. Adoption of healthier lifestyle patterns among migrants could help prevent/delay onset of these abnormalities in this population.

Cryopyrin-associated periodic fever syndrome in children: A case-based review.

Cryopyrin-associated periodic fever syndrome (CAPS) represents an increasingly recognized disease group entity, with varied presentations. CAPS includes 3 clinical entities, namely, familial cold-induced autoinflammatory syndrome (FCAS; MIM #120100), Muckle-Wells syndrome (MWS; MIM #191900) and chronic inflammatory neurologic cutaneous and articular syndrome (CINCA; MIM #607115); which share several overlapping clinical features. These patients often present with early-onset episodes of fever and rash, and variable systemic signs and symptoms, making it a great mimicker of other systemic autoimmune diseases. The episodes are transient and related to exposure to cold temperature and worsen in the winter season. We hereby present a case presenting with recurrent seasonal fever and rash, diagnosed as FCAS/ MWS overlap based on clinical signs and symptoms and positive testing for NLRP3 gene mutation. We also discuss the clinical presentation and complications of CAPS, chiefly FCAS and MWS, along with the previously described pediatric cases of CAPS. We tried to review the complexities of management of such patients, including the genetic diagnosis and the role of biological therapy. Based on the review of the literature, given the evident broad spectrum of symptoms and signs, use of next-generation sequencing can help in prompt diagnosis and early initiation of biological agents, which may play a great role in reducing the complications that these patients may experience in the long run.

Dyslipidemia Among Overweight and Obese Children in Jharkhand: A Hospital-Based Study

Objective: To study the prevalence of common dyslipidemia among overweight and obese children between 2-18 years in a tribal region. Methods: This cross-sectional study was carried out in 151 overweight and obese children aged 2-18 years, who visited the pediatric outpatient department of a tertiary care hospital in Jharkhand between 1 August and 30 November, 2022. Dyslipidemia was defined as anyone of the following: a total cholesterol level of 240 mg/dL, a triglyceride level of 150 mg/dL, a low-density lipoprotein cholesterol level (LDL-C) of 140 mg/dL, a high-density lipoprotein cholesterol level (HDL-C) of 40 mg/dL, or the use of a lipid-lowering drug [8]. Overweight and obesity were defined as per World Health Organization criteria. Result: The prevalence of dyslipidemia was 63.6%. The most common type of dyslipidemia was low HDL-C + high TG levels found in 32.5% (n=49) children. The most prevalent pattern of dyslipidemia in overweight children was low HDL-C (19, 32.3%); and in obese children low HDL-C and high TG levels (39, 42.3%). Conclusion: The prevalence rate of dyslipidemia was high among overweight and obese children in this region. There was a positive association between dyslipidemia and body mass index.

Mortality and morbidity of very low birth weight and extremely low birth weight babies in neonatal period

Background: Preterm birth is one of the major clinical problems in neonatology as it is commonly associated with perinatal mortality, serious neonatal morbidity and in some cases, it leads to childhood disability. The objective of this study was to find out the outcomes of VLBW and ELBW babies in the form of mortality and morbidity in neonatal period.Methods: A cross sectional study was conducted in SNCU, MKCG Medical College, Berhampur, Odisha between 2011 to 2013. All babies with birth weight less than 1500gm, admitted between days 1 to day 7 of life were enrolled in the study. Babies were broadly divided in to two groups i.e.VLBW group (weight 1000-1499gm) and ELBW group (weight<1000gm). Details of antenatal history, sociodemographic profile and birth history including significant events were noted. Outcome measures included were death, cured and with sequelae.Results: Total number of babies enrolled in this study were two hundred twelve (n=212) and following observations were made. Mortality of ELBW babies was 61.11% and that of VLBW babies was 26.41%. Death rate in babies with weight range 500-749gm, 750-999gm, 1000-1249gm and 125-1499gm was 87.50%, 53.57%, 30.76% and 20.97% respectively. It was found that morbidities such as RDS, HIE, shock and hypoglycemia are significantly higher in ELBW babies as compared to VLBW babies.Conclusions: Extremely low birth weight babies have shown high mortality and morbidity compared to very low birth weight babies and its related death multiply when associated with complications like hyaline membrane disease, hypoxic ischemic encephalopathy, and sepsis.

Evaluation of prolonged febrile illness in elderly

Background: Febrile illness in elderly patients in hospitals is a challenge to the physician for diagnosis and treatment due to high morbidity as well as mortality and it increases if the febrile illness is prolonged. So proper evaluation and effective management is necessary for a better outcome. Keeping in mind the scarcity of studies in elderly febrile illness in India this study was taken up.Method: A prospective study was designed in medical ICU of S.C.B Medical college and Hospital, Cuttack Odisha, India. 50 patients were included in this study from July 2007 to December 2008. Institutional Ethics Committee cleared the study.Results: In 50 elderly (Age>60 yrs) patients of prolonged febrile illness, 36 (72%) were male and 14 (28%) were female. All had fever for >21 days. Pallor was the commonest sign (62%). 30 patients had infectious etiology, 15 had malignancies. Tuberculosis was the commonest infection (28%) comprising of 46.66% of infectious etiology with Pulmonary Tuberculosis (PTB) in 20% and Extrapulmonary Tuberculosis (ETB) in 26.66%. Malignancies accounted for 30% of cases with Non-Hodgkin’s lymphoma (NHL) in 33.33% being the commonest amongst the malignancies. On follow up of 50 patients 21 (42%) got cured.Conclusion: Febrile illness in elderly needs carefully evaluation as infections account for most of the cases and Tuberculosis in our part of India as a major cause in these patients is treatable. Malignancies remain the second most common cause where timely intervention goes a long way in reducing morbidity and mortality.