OBJECTIVE: To study the clinical and immunological profile of @entity1 with @entity1252 ( @entity1252 ). DESIGN: Retrospective hospital based study. SETTING: Tertiary level center of North India. SUBJECTS: Sixteen @entity1 in the age group 4-12 years. METHODS: Medical records of @entity1 with @entity1252 were analyzed. Clinico pathological features were compared with 2 other series from India. RESULTS: Mean age of @entity1 at the time of diagnosis was 10 yr and 8 (50%) @entity1 were less than 10 yr of age. The female to male ratio was 7:1. Fever (56.2%), @entity1910 (87%) and @entity625 (87%) were the common clinical manifestations, Renal involvement was noted in 56.2% of cases. Other clinical features included @entity5714 (31.2%), @entity882 (18.6%) and @entity2407 (12.5%). Cardiac involvement in the form of severe @entity2401 and @entity884 occurred in one @entity1 each. @entity120 was the presenting feature in one @entity1 with @entity712 . One @entity1 had @entity15 along with @entity1252 --a rare combination. ANA positivity was seen in all @entity1 . Five @entity1 died; two had severe @entity299 . Three @entity1 had renal involvement and one died of @entity120 . Two-thirds of subjects with renal involvement improved after therapy according to NIH, Bethesda protocol. CONCLUSIONS: @entity1252 must be considered in any @entity1 with @entity66 , as the disease may have certain unusual presentations.

Clinical and immunological profile of XXXX : some unusual features.

@entity1 :: ('9606', 'Species') :: ['children', 'patient', 'child'] @entity712 :: ('MESH:D006333', 'Disease') :: ['right heart failure'] @entity625 :: ('MESH:D001168', 'Disease') :: ['arthritis'] @entity1910 :: ('MESH:D005076', 'Disease') :: ['rash'] @entity882 :: ('MESH:D013921', 'Disease') :: ['thrombocytopenia'] @entity66 :: ('MESH:D004194', 'Disease') :: ['multisystem disease'] @entity1252 :: ('MESH:D008180', 'Disease') :: ['SLE', 'systemic lupus erythematosus'] @entity2407 :: ('MESH:D011928', 'Disease') :: ["Raynaud's phenomenon"] @entity120 :: ('MESH:D006976', 'Disease') :: ['pulmonary hypertension', 'Pulmonary hypertension'] @entity299 :: ('MESH:D006331', 'Disease') :: ['cardiac involvement'] @entity5714 :: ('MESH:D000743', 'Disease') :: ['hemolytic anemia'] @entity15 :: ('MESH:D009103', 'Disease') :: ['multiple sclerosis'] @entity2401 :: ('MESH:D009205', 'Disease') :: ['myocarditis'] @entity884 :: ('MESH:D004696', 'Disease') :: ['endocarditis']
@entity1252 :: (MESH:D008180,Disease) :: ['SLE']
