Antibodies to complement factor H are an uncommon cause of hemolytic uremic syndrome (HUS). Information on clinical features and outcomes in children is limited. In order to explore this we studied a multicenter cohort of 138 Indian children with anti-complement factor H antibody associated HUS, constituting 56% of patients with HUS. Antibody titers were high (mean 7054 AU/ml) and correlated inversely with levels of complement C3, but not complement factor H. Homozygous deletion of the CFHR1 gene was found in 60 of 68 patients. Therapies included dialysis in 119 children, 105 receiving plasma exchanges and 26 intravenous immunoglobulin. Induction immunosuppression consisted of 87 children receiving prednisolone with or without intravenous cyclophosphamide or rituximab. Antibody titers fell significantly following plasma exchanges and increased during relapses. Adverse outcome (stage 4-5 CKD or death) was seen in 36 at 3 months and 41 by last follow up, with relapse in 14 of 122 available children. Significant independent risk factors for adverse outcome were an antibody titer over 8000 AU/ml, low C3 and delay in plasma exchange. Combined plasma exchanges and induction immunosuppression resulted in significantly improved renal survival: one adverse outcome prevented for every 2.6 patients treated. Maintenance immunosuppressive therapy, of prednisolone with either mycophenolate mofetil or azathioprine, significantly reduced the risk of relapses. Thus, prompt use of immunosuppressive agents and plasma exchanges are useful for improving outcomes in pediatric patients with anti-complement factor H-associated HUS.
Several traditional medicines contain potentially toxic heavy metals. Heavy metal poisoning is not an uncommon cause of renal damage, although the diagnosis can be easily missed. We report a case of chronic ingestion of an ayurvedic medicine containing mercury in a 2-year-old girl, resulting in anuric renal failure due to acute interstitial nephritis.
Methicillin resistant Staphylococcus aureus (MRSA) meningitis is rarely known to occur in children. We report an 11-year-old girl with fever, headache and vomiting, right hemiparesis with left-sided upper motor neuron facial nerve palsy and bladder incontinence. On investigation, she was found to have MRSA meningitis with an acute left thalamo-corpuscular infarct. She was treated with vancomycin, linezolid and rifampicin. She recovered successfully with residual right-sided lower limb monoparesis. MRSA meningitis is rare but can occur in children.
Tuberculosis spine presenting as isolated superior mediastinal widening is rarely seen in children. We present a 1 ½ years old child with fever and cough for a month, who was detected to have superior mediastinal widening on chest X-ray which on CT chest was found to be an extension of abscess from tuberculosis spine which had progressed anteriorly to push and compress the trachea. We report the case for its rarity and unusual presentation of TB spine.
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