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PubMed | Jawaharlal Institute of Postgraduate Medical Education & Research and Pediatric Allergy Immunology Unit
Type: Journal Article | Journal: Lupus | Year: 2015

Data on outcome of childhood lupus nephritis from developing countries are sparse. This study looks at outcome in children with lupus nephritis from a federal government-funded teaching hospital in North India.This study included children less than 14 years of age with lupus nephritis who presented to a single center during a period of 24 years (1991 to 2013). Data on clinical characteristics and outcome were extracted from medical records. The primary outcome was actuarial survival (time-to-death) and secondary outcome was actuarial renal survival using Kaplan-Meier analysis. A worst-case scenario that assumed children who were lost to follow-up as having either died or gone into end-stage renal disease was also calculated. Log-rank test and Cox-regression were used to assess difference in survival by histological class and predictors of poor outcome, respectively.This study included 72 children, with a female:male ratio of 3:1, mean (SD) age at onset of lupus 9.3 (2.4) years and mean (SD) time from onset-to-nephritis being 9.4 (12.6) months. Renal biopsy was conducted in 53 children. The most common histological class was class IV (35 children). Mortality occurred in 22 children (30%), with half of these occurring at presentation. The two important causes of death were infection and end-stage renal disease. Actuarial survival was 81%, 67% and 59% at one, five and 10 years, respectively. In the worst-case scenario, actuarial survival was 72%, 53% and 38%, respectively. Renal survival was 96%, 89% and 78% (worst-case scenario 86%, 73% and 52%) at one, five and 10 years, respectively. There was no difference in survival by histological class. On univariate analysis, serum creatinine at presentation (hazard ratio=2.2 (95% CI 1.3-3.9)) and serious infection (hazard ratio 7.9 (95% CI 2.6-23.5)) were statistically significant predictors of time-to-death.Outcome of children with lupus nephritis from India is worse than developed countries. Nearly one-third of the children died, half at presentation, with common causes being infection and end-stage renal disease.

Avni T.,The Safra Childrens Hospital | Paret G.,The Safra Childrens Hospital | Paret G.,Tel Aviv University | Thaler A.,The Safra Childrens Hospital | And 5 more authors.
Cytokine | Year: 2010

Background: Fractalkine (FKN), a unique chemokine associated with pulmonary hypertension, may be involved in the acute stress response that regulates inflammation after cardiopulmonary bypass (CPB) surgery. We characterized FKN levels and correlated them with clinical parameters in children undergoing cardiac surgery involving CPB. Methods: Twenty-seven consecutive patients, aged 30. days to 11.5. years, who underwent surgery for correction of congenital heart defects, were prospectively studied. Serial blood samples were collected preoperatively, upon termination of CPB, and at six points postoperatively. Plasma was recovered immediately, aliquoted, and frozen at -70 °C until assayed. Clinical and laboratory data were collected. Results: Baseline FKN levels were skewed between patients. Patients with low FKN levels showed significantly higher levels of oxygen saturation in room air compared to patients with high FKN levels (p< 0.05). Moreover, there was a positive correlation between preoperative pulmonary arterial hypertension and FKN levels (p< 0.05). Surprisingly, FKN elevation from preoperative to postoperative levels displayed no discernible pattern. Conclusions: FKN levels significantly correlate with preoperative hypoxemia and PAH, suggesting that FKN may be up-regulated during hypoxemia. CPB is not associated with acute changes in circulating FKN levels. The role of FKN in the postoperative course should be further investigated. © 2010 Elsevier Ltd.

Singh S.,Pediatric Allergy Immunology Unit | Chandrakasan S.,Pediatric Allergy Immunology Unit | Ahluwalia J.,Jawaharlal Institute of Postgraduate Medical Education & Research | Suri D.,Pediatric Allergy Immunology Unit | And 5 more authors.
Rheumatology International | Year: 2012

The objective of this study is to describe the clinical and laboratory features of macrophage activation syndrome (MAS) in systemic onset juvenile idiopathic arthritis (SOJIA) at a tertiary care center in northwest India. Review of medical records of all children with SOJIA admitted during the period January 1995-December 2008 in Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, was done. Six patients (5 boys and 1 girl) with SOJIA and MAS were identified. Mean age at time of disease onset was 6.5 years. MAS was the presenting manifestation of SOJIA in 4 patients. Clinical manifestations included fever (6/6), clinical shock (6/6), encephalopathy (5/6), generalized lymphadenopathy (4/6), hepatosplenomegaly (3/6), jaundice and abdominal tenderness (3/6), cardiac involvement (3/6), and meningeal irritation (2/6). Laboratory findings at onset of MAS included decreasing total leukocyte and platelet counts, coagulopathy, elevated transaminases, hyponatremia, and lipid abnormalities. Hemophagocytosis was demonstrable in the bone marrow in 4 patients and in the lymph node in 1. For treatment, we used intravenous methylprednisolone (4/6), oral prednisolone (2/6), and intravenous immunoglobulin (2/6). Outcome was favorable in all patients except one who died of rapidly progressive disease. This paper describes the experience of JIA-related macrophage activation syndrome in a tertiary Indian center. We have shown that MAS can be the early presenting manifestation of evolving SOJIA. Early diagnosis and aggressive management can have a significant impact on the mortality associated with this syndrome. We stress on the role of glucocorticoids in the management of this condition and believe that glucocorticoids have a far more important role in the management of this condition than what has been previously reported. © Springer-Verlag 2011.

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