An attempt to improve the clinical scale for assessment of haemophilic arthropathy in children.

Of the methods used to evaluate haemophilic arthropathy, clinical joint score can only detect advanced and not minor changes, which usually occur in younger patients. In addition, the currently used clinical scores are poorly correlated with the MRI and X-rays scales. In an attempt to address these shortcomings, a modification of Stockholm clinical scale was designed in which elements of clinical information were included. This new scale was applied in 165 joints of 40 patients with haemophilia A and B and the results were statistically analyzed for its capacity to detect early joints alterations. Furthermore, the adjusted score was compared with Pettersson's radiological score and Denver's MRI score, which can detect early signs of arthropathy. The adjusted scale (a) revealed the earliest abnormalities of haemophilic arthropathy and its correlation with the Pettersson and Denver scores is better than those of Stockholm's scale, (b) provided prediction of the Denver score and (c) was simple and safe to be performed and it could easily be repeated.

Recurrent idiopathic thrombocytopenic purpura in childhood.

BACKGROUND: Idiopathic thrombocytopenic purpura (ITP) is a common haematological disease during childhood, that usually has a benign course; however, literature on the recurrent form of the disease (rITP) is limited. PROCEDURE: rITP was characterized by intermittent episodes of thrombocytopenia (TP) followed by periods of recovery, unrelated to therapeutic intervention. We retrospectively reviewed features of patients with rITP, diagnosed and systematically followed up at our center, during the period 1975-2004. RESULTS: Forty-eight of 795 children with ITP (6.0 %) presented with rITP. The majority of patients (68.8%) had only one recurrence, whereas only one patient had four. A time interval between two episodes longer than 3 months (up to 96) was identified in 2/3 of episodes and <3 months in 1/3. The initial episode and the first recurrence mostly shared features of acute ITP; however, 22.9% of the episodes appeared with a chronic self-limited course. Bleeding manifestations were rare (18.6% of episodes) and mild, and they tended to occur in severely thrombocytopenic patients, mainly at the onset of the initial episode; intracranial hemorrhage (ICH) occurred in a toddler with short duration thrombocytopenia. Intravenous gamma globulin (IVIG) or corticosteroids were administered in 24.5% of episodes. None of the patients needed splenectomy. CONCLUSION: rITP is a rare, mild, self-limited type of ITP, although ICH may occur in a profoundly TP child. Recurrence may occur close or far apart to a previous isolated TP episode. The duration of episodes varies considerably from patient to patient and from episode to episode in the same patient. The pathogenesis of rITP still remains unclear.