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Arthritis is one of the less common, yet challenging problems that may confront a pediatrician. The potential pathology is diverse ranging from the benign with a good prognosis to the serious and ultimately fatal. From a spot diagnosis to conditions that evolve over time, few other conditions challenge and stimulate the clinical acumen. Several diagnoses can be made clinically with the laboratory investigations providing additional support. An approach through which the clinician seeks answers through a logical sequence of questions is presented to help shortlist the possible diagnosis. [ABSTRACT FROM AUTHOR]

The combination of Congenital Central Hypoventilation with Hirschsprung's disease belongs to the family of diseases now designated as Neurocristopathies. The authors report a polysomnographically documented case and review literature on this disorder. [ABSTRACT FROM AUTHOR]

A 1-year-old child with proximal femoral focal deficiency (PFFD) is presented. The clinical spectrum and associated abnormalities is described and the diagnosis and management of this entity is discussed. [ABSTRACT FROM AUTHOR]

Background: TRAPS, an autosomal dominant autoinflammatory disorder occurs due to mutations of the TNFRSF1A gene. Mutation negative TRAPS (TRAPS like illness) is also known. Anti TNF molecules (etanercept) is the mainstay of therapy. Case characteristics: A 11-year-old boy with a 5 year clinical profile indicative of a TRAPS like illness and with negative mutation studies is described. He has been followed up for nearly 2 years after starting etanercept. Outcome: He had sustained response to etanercept which has subsequently been titrated (0.4 mg/kg subcutaneously every 23-24 days) to keep him symptom free. Message: Mutation negative cases of TRAPS can be diagnosed with a high index of suspicion. Treatment with etanercept is expensive but possibly intervals between doses could be titrated to reduce cost. [ABSTRACT FROM AUTHOR]

Systemic onset juvenile idiopathic arthritis (SOJIA) is the most common autoimmune auto inflammatory disease in childhood. A sizeable number of these patients run a recalcitrant disease course, resistant to the conventional line of management, ultimately resulting in permanent disability from joint destruction, local growth deformities or iatrogenic side effects. The new biological agents although very effective, are beyond the affordability of most in our country. Thalidomide, a cheaper option has been shown to be very effective in the disease control of patients with SOJIA. We report three Indian children with a chronic refractory course of SOJIA, all of whom had failed conventional line of treatment but improved with thalidomide. [ABSTRACT FROM AUTHOR]

The article offers information on two medical cases involving children in India. Topics covered include a 3-year-old boy who had a fewer for 20 days because of acute gangrenous gingivitis and a 12-year-old boy with dimness of vision for a year and a greyish mesh over sclerae of both eyes due to bilateral epibulbar limbal dermoids with amblyopia and astigmatism.