Profile of Cardiac Involvement in Children After Exposure to COVID-19

Objective: To evaluate the incidence and pattern of cardiac involvement in children postCOVID (coronavirus disease) infection in a tertiary care referral hospital in India. Methods: A prospective observational study was conducted including all consecutive children with suspected MIS-C referred to the cardiology services. Results: Of the 111 children with mean (SD) age 3.5 (3.6) years, 95.4% had cardiac involvement. Abnormalities detected were coronary vasculopathy, pericardial effusion, valvular regurgitation, ventricular dysfunction, diastolic flow reversal in aorta, pulmonary hypertension, bradycardia and intracardiac thrombus. The survival rate post treatment was 99%. Early and short-term follow-up data was available in 95% and 70%, respectively. Cardiac parameters improved in the majority. Conclusion: Cardiac involvement post COVID-19 is often a silent entity and may be missed unless specifically evaluated for. Early echocardiography aids in prompt diagnosis, triaging, and treatment, and may help in favorable outcomes.

Poor obstetric outcomes in Indian women with Takayasu arteritis

Abstract Introduction: Takayasu's arteritis (TA) affects young women in the childbearing age group. We studied obstetric outcomes in these patients before and after disease onset. Methods: Women aged more than 18 years with Takayasu's arteritis (ACR 1990 criteria) were included. Demographic data, clinical features, disease activity using Indian Takayasu Arteritis clinical score (ITAS), Disease Extent Index for TA (DEI. TaK) and damage assessment using TA Damage score (TA), history of conception and maternal and fetal outcomes were recorded from hospital records and telephonic interview. Results are in median and IQR. Results: Of the 64 women interviewed, aged 29 (24-38) years and disease duration 5 (4-10) years, 74 and 38 pregnancies had occurred before and after disease diagnosis in 29 and 20 women respectively. In eight, the diagnosis was made during pregnancy. Age at disease onset was 22 (18-30) years. Type 5 disease was the most common ( n = 32, 59.3%), and an equal number of patients had Ishikawa's class I and II disease ( n = 26, 40.6%). Median ITAS ( n = 44) was 13 (7-16), DEI. Tak 12.5 (9-16.75) and TADS 8 (6.5-10). Twenty-five patients wanted to get pregnant, of which 8 (32%) did not do so because of their disease. Fifteen were unmarried of whom 6 did not marry due to disease. Obstetric outcomes were poorer in pregnancies that occurred after the onset of disease as compared with those before it (RR = 1.5, p = 0.01). Pregnancies after the onset of TA carried a very high risk of maternal [RR3.9 (1.8-8.5), P < 0.001] as well as fetal complications [RR = 2.0 (1.2-3.4), p = 0.001]. Hypertension was the most common maternal complication and occurred most often in the last trimester. The baby weight at birth was lower in pregnancies after disease (2.3 vs. 3.0, p = 0.01). Wong's score greater than or equal to 4 predicted lower birth weight ( p = 0.04). ITAS, ITAS-A, DEI. Tak and TADS could not predict obstetric outcomes, and ITAS score exhibited moderate correlation with DEI. Tak ( r = 0.78) and TADS ( r = 0.58). Conclusion: Women with TA suffer from extremely high risk of poor maternal and foetal outcomes. Wong's scoring can be useful to predict birth weight.(AU)

Comparison between Diagnostic Yield of Pleural Fluid ELISPOT and Thoracoscopic Guided Pleural Biopsy in Undiagnosed Pleural Effusion

Context: Despite recent advances in the available diagnostic modalities, diagnosis of pleural tuberculosis remains a challenge because of the low yield of conventional methods. Pleural biopsy is the gold standard for confirmation of diagnosis, which is invasive and cumbersome. The concentration of mycobacterial peptide-specific activated lymphocytes at the site of infection can be utilized as the basis for using IGRA (interferon-gamma release assays) based evaluation of undiagnosed exudative pleural effusions.  Aim: To evaluate the performance of IGRA (Enzyme-linked Immunospot (ELISPOT) in pleural fluid for the diagnosis of pleural tuberculosis in histopathologically confirmed cases. Settings and Design: A prospective observational study compared the utility of ELISPOT with thoracoscopy guided pleural biopsies for the diagnosis of tubercular pleural effusions. Methods and Material: Forty-two consecutive cases of undiagnosed pleural effusions were enrolled and subjected to thoracoscopy guided pleural biopsy. Thirteen patients were confirmed to have tuberculosis, 27 had malignancy, and 2 had normal pleura. A total of 1x103 pleural fluid mononuclear cells (PFMCs) were cultured in the presence of early secretory antigenic target-6 (ESAT-6) and culture filtrate protein-10 (CFP-10) for 24 hours. The individual spots were then counted using an automated analyzer ELISPOT reader system.  Results: The number of spots developed in the pleural fluid was significantly higher in tubercular pleural effusions as compared to non-tubercular effusions (CFP-10:154.76±14.61 vs 49.24±8.9; ESAT-6: 150.3±17.27 v/s 45.34±8.23, p<0.001). At a cut-off value of more than 67 spots taken as positive for tuberculosis, the sensitivity of the test was 100% (95% CI 75.29% to 100.00%), specificity was 96.5% (95 % CI 82.24% to 99.91%), positive predictive value was 92.86% (95 % CI 65.45% to 98.89%) and negative predictive value was 100%.  Conclusions: ELISPOT can be a useful non-invasive test for the evaluation of undiagnosed pleural effusions and making a diagnosis of pleural tuberculosis with confidence.

Renal cell carcinoma and systemic onset juvenile idiopathic arthritis.

Renal cell carcinoma (RCC) accounts for majority of malignancies arising out of the kidney. Paraneoplastic rheumatologic manifestations; myositis, vasculitis, and arthritis have been described in a few cases with RCC. Systemic onset juvenile idiopathic arthritis (JIA) is characterized by intermittent fever, arthritis, reticulo-endothelial cell hyperplasia and absence of rheumatoid factor and antinuclear antibodies. Herein, we report a 16-year-old boy with systemic onset JIA for 5 years who developed RCC and his systemic and articular symptoms paralleled the course of RCC. The common pathophysiologic influence of the cytokine Interleukin-6 possibly played a role in the exacerbation of symptoms of systemic onset JIA during the relapse of the RCC. The case is presented to highlight the rare co-occurrence of these two diseases and their influence on each other.

Antinuclear antibodies by indirect immunofluorescence : optimum screening dilution for diagnosis of systemic lupus erythematosus.

BACKGROUND & OBJECTIVES: Antinuclear antibodies (ANA) are serological hallmark of systemic lupus erythematosus (SLE). Conventionally, the test is carried out on human epithelial cells (HEp2) by indirect immunofluorescence (IIF) technique. Since culturing and maintaining HEp2 cells in the laboratory are labour intensive, in-house assays have given way to kits manufactured by commercial companies. The reference screening dilutions provided by the manufacturers are based on different ethnic population than ours. Therefore, it becomes mandatory for every laboratory to have its own screening dilutions for the local population that distinguishes best between healthy and diseased state. As, there is paucity of such data, we aimed to define the optimum screening dilution that distinguishes the patient with SLE from healthy individuals. METHODS: Sera of patients fulfilling ACR criteria for diagnosis of SLE, idiopathic inflammatory polymyositis/dermatomyositis (PM/DM) and rheumatoid arthritis (RA), and age and sex matched healthy individuals were tested for ANA by IIF using a commercial kit (Euroimmun, Germany) at 5 dilutions, namely 1:40, 1:80, 1:160, 1:320 and 1:640. Receiver operator characteristics (ROC) curve were constructed to define the optimum dilution that distinguished healthy sera from the diseased ones. RESULTS: Test was performed on 213 sera from 94 healthy individuals, and 43 SLE, 37 RA and 39 DM/PM patients. In healthy individuals, ANA at dilutions 1:40, 1:80, 1:160, 1:320 and 1:640 was positive in 13.8, 4.3, 2.1, 2.1 and 0 per cent respectively, whereas in SLE it was positive in 95.3, 95.3, 65.1, 53.5 and 23.3 per cent respectively. INTERPRETATION & CONCLUSION: ROC curves analysis showed that at 1:40 dilution, sera of 95.3 per cent of SLE and 13.8 per cent of normal individuals were (ANA) positive, whereas at 1:80 dilution it was 95.3 per cent for SLE and 4.3 per cent for healthy individuals. A fluorescent intensity of > or =2 was more specific for SLE. The best discrimination between healthy individuals and the SLE patients was found at screening dilution of 1:80 and fluorescent intensity of > or =2 in our laboratory.

Parotid Gland Enlargement as a Presenting Manifestation of Acute Lymphoblastic Leukemia

A young female presented with symmetric polyarthritis, generalized lymphadenopathy, hepatosplenomegaly and bilateral parotid gland enlargement without sicca symptoms. A second case of 15 months old child presented with short duration pyrexia with generalized lymphadenopathy, hepatosplenomegaly and bilateral parotid gland enlargement. Both the patients had out of proportion anemia on examination. Investigations confirmed CD 10+ B-cell acute lymphoblastic leukemia (ALL) in both the cases. Fine needle aspiration cytology of parotid glands in both the cases showed infiltration by lymphoblasts.We propose that ALL should be included in the differential diagnosis of bilateral parotid gland enlargement especially if associated with lymphadenopathy and hepato-splenomegaly.

Achalasia cardia in mother and son.

Familial occurrence of achalasia cardia is rare. Most associations are among siblings or in monozygotic twins. Parent-child association is even rarer and only six such instances have been reported till date. We report a 29-year-old man with achalasia cardia and his mother who had the same illness two and half years later. Both of them were successfully treated with balloon dilatation.

Disseminated Cryptococcosis Mimicking Lymphoreticular Malignancy in a HIV Negative Patient

Disseminated cryptococcosis, a life threatening mycosis, usually presents with respiratory tract, central nervous system and skin involvement. Herein we report a case of disseminated cryptococcosis presenting with hepatic, lymph nodal and bone marrow involvement in addition to the central nervous system and lung involvement in a previously healthy, HIV negative individual. The unique presenting manifestation highlights the need for careful consideration of this entity in conditions mimicking disseminated tuberculosis and lymphoreticular malignancy.

Outcome in juvenile rheumatoid arthritis in India.

Juvenile rheumatoid arthritis (JRA) leads to significant morbidity due to continued disease activity and drug toxicity. Retrospective analysis of patients with JRA seen at a tertiary care hospital between 1990-2000 was done. Data regarding the type of onset, course of disease, joints involved, treatment received, drug toxicity and outcome at last visit were retrieved from case records. There were 214 children (76 oligoarticular, 93 polyarticular and 45 systemic onset) with 135 of them being boys. At last followup, with median disease duration of 6 years; 128 had active disease, 58 had stable disease and 28 had inactive disease. Polyarticular group had the worst outcome with only 3 of the 93 having inactive disease (13/76 in oligoarticular group and 12/45 in systemic onset disease). Intramuscular gold and D-Penicillamine were associated with significant drug toxicity. Outcome of children with JRA in our country is poorer as compared to developed countries.

Arthritis in lymphomatoid granulomatosis: report of a case and review of literature.

Lymphomatoid granulomatosis (LG) is a rare systemic vasculitis caused by Epstein Barr virus induced transformation of the B-cells in a T-cell rich environment. The predominant clinical presentations are confined to the pulmonary system however; extra-pulmonary manifestations can sometimes be the main feature of the disease. Here in we describe a 52-year-old female who presented with symmetric polyarthritis and generalized stiffness for 7 months and papular lesions over extremities for 3 months duration. She in addition had generalized lymphadenopathy. Histopathological examination of the cutaneous lesions confirmed LG. Patient died despite therapy with cyclophosphamide and prednisolone. This is the first report of LG mimicking rheumatoid arthritis from India.

Isolated nocardial subcapsular and perinephric abscess.

We report a rare presentation of Nocardia infection in a 60 years old diabetic and cirrhotic patient who had perinephric and subcapsular renal abscess. This localized type of infection was successfully treated with oral cotrimoxazole.