A rare case of childhood polyarteritis nodosa successfully treated with etanercept

Childhood Polyarteritis Nodosa (CPAN) a rare and often fatal disease tends to be more common in individuals of Asian descent. Previously it was referred to as Infantile PAN. Polyarteritis Nodosa (PAN) is a systemic autoimmune vasculitis characterized by necrotizing inflammatory lesions of the medium-sized and small muscular arteries, preferentially at vessel bifurcations, resulting in formation of microaneurysms, aneurysmal ruptures with hemorrhage, thrombosis and consequently organ ischemia or infarction. It usually appears in middle or older age without gender predilection. PAN shows variety of symptoms, including general symptoms, neurological, skin, renal and gastrointestinal involvement. In particular skin lesions characterized by multiple firm waxy papules, subcutaneous nodules, livedo reticularis, ulcers and gangrene are observed in 25%-60% of patients with PAN. The etiology of systemic vasculitis is yet unknown. However, dysregulation and/or enhanced expression of pro-inflammatory substances may be involved in pathogenesis of these diseases. Tumour necrosis factor (TNF) alpha is a pro-inflammatory cytokine produced primarily by cells of macrophage-monocyte lineage which may directly participate in vascular inflammation as well as in endothelial cell death via apoptosis. In addition, TNF-alpha may play a role in neutrophil priming inducing membrane expression of Proteinase-3 or Myeloperoxidase which are subsequently recognized by ANCA-associated vasculitis (AAV). Author report a case of 14 months old male child with complaints of fever, gangrenous changes of ear lobes, tip of nose and toes, seizures, altered sensorium and hypertension. Initially Injectable Methyl Prednisolone pulse therapy was started followed by oral Prednisolone. After initiation of Etanercept treatment, his symptoms improved dramatically. Sensorium improved, skin ulcers healed faster, and gangrenous changes were arrested, fever subsided and child started accepting oral feeds.

Syndromic Management of Common Illnesses in Hospitalized Children and Neonates: A Cost Identification Study.

Objective To find out drug treatment cost per illness per patient admitted to pediatric ward. Methods Patients admitted to pediatric ward over a period of 1 year were studied without exclusions. Following presentations were studied: fever, rapid breathing, diarrhea, severe malnutrition and neurological problems such as altered conscious level or convulsion. In this prospective observational study, patients with other problems were excluded. The subjects were also categorized as critically sick, sick and stable. Expenditure on medicines was calculated individually for each patient. Total expenditure, average cost and illness-wise cost were subsequently derived. Management of illnesses was on the lines of existing guidelines of our center. Sick newborns or newborns referred for special care were separately studied. Following outcome variables were studied: death or discharge, length of hospital stay and the day on which symptomatic relief was noted. Results 774 children and 141 newborns were studied. 25 (3.2%) died. Presenting features were as follows: fever-568 (73.4%), rapid breathing-175 (22.6%), diarrhea-145 (18.7%), mild-moderate malnutrition-278 (35.8%), severe malnutrition-111 (14.3%) and neurological problems-41 (5.3%). Category-wise distribution was as follows: critically sick-89 (11.3%), sick-188 (24.3%) and stable-497 (46.2%). Average hospital stay was 7.1 days and symptomatic relief was experienced by day three in 77.7% cases. Average cost of medicines per patient was INR-167.8 (USD-4.2), 173 patients required oxygen and mean expenditure on oxygen was INR-310 (USD-8) and 68 patients required inotropes with a mean expenditure of INR-198 (USD-5). Of the 141 newborns admitted, 20(14.1%) died. Mean hospital stay was 9.8 days and average cost of drug treatment was INR-790 (USD-20) in newborns. Conclusions This cost analysis study presents drug treatment costs for common illnesses at a referral centre in a developing country. It gives an option to choose drugs for an optimum mix of cost and effectiveness.

Leptospirosis in children.

Leptospirosis has a broad spectrum of clinical manifestations varying, from inapparent influenza like illness to fulminant fatal disease with hepato-renal dysfunction and hemorrhagic phenomena. Our cases had fever, puffiness, respiratory distress and bleeding diathesis as leading manifestations. Leptospirosis was suspected in view of epidemic situation prevailing in the city. We report four cases here, three of which survived and one died.