Thalidomide for Systemic Onset Juvenile Idiopathic Arthritis.

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.

Haddad syndrome--congenital central hypoventilation associated with Hirschsprung's disease.

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.

Initial evaluation of a child with arthritis--an algorithmic approach.

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.

Proximal femoral focal deficiency (PFFD).

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.

Choice of antipyretic in children.

The objective of this study was to evaluate the relative efficacy of 3 commonly used antipyretics viz. mefenamic acid, ibuprofen and paracetamol. The subjects were randomized to 3 groups to receive either paracetamol 10 mg/kg or ibuprofen 7 mg/kg or mefenamic acid 6.5 mg/kg. Axilla temperature was recorded just prior to drug administration and at hourly intervals for 4 hrs. The mefenamic acid group (n = 29) showed a drop of 3.5 degrees F at the end of 4 hours as compared to the paracetamol group (n = 29) (drop 2.44 degrees F) and ibuprofen group (n = 20) (drop 2.79 degrees F). Analysis of the area under the mean temperature vs. time curve showed that mefenamic acid demonstrated significantly better antipyretic activity compared to paracetamol (P < 0.05) over the entire period of observation and ibuprofen (P < 0.05) in the 2 to 4 hour range. Mefenamic acid continued to show antipyretic activity at the end of 4 hours in contrast to ibuprofen and paracetamol. Since the period of observation was restricted to 4 hours, we were unable to quantify the precise duration of its extended antipyretic efficacy.