Background
Nephrotic syndrome occurring as a complication of immune tolerance therapy for inhibitors in hemophilia B is well recognized. It is also known to occur in association with factor borne infections, especially hepatitis C. This is the first case report of nephrotic syndrome occurring in a child receiving prophylactic factor VIII in the absence of inhibitors of hepatitis infection. However, the pathophysiology of this phenomenon is poorly understood.
Case presentation
A 7-year Sri Lankan boy diagnosed with severe hemophilia A on weekly factor VIII prophylaxis was diagnosed with three episodes of nephrotic syndrome, a condition in which there is leakage of plasma protein into urine. He had three episodes of nephrotic syndrome, all of which responded well to 60 mg/m2 daily dose of oral steroids, achieving remission within 2 weeks of starting daily prednisolone. He has not developed inhibitors for factor VIII. His hepatitis screening remained negative.
Conclusions
There is a possible link between factor therapy for hemophilia A and nephrotic syndrome, which can be a T-cell-mediated immune response. This case also highlights the importance of monitoring for renal involvement in patients treated with factor replacement.
Background: Asthma causes a significant disease burden in children and it is objectively diagnosed by demonstration of reversible airway obstruction. However, objective diagnosis of airway obstruction in preschool children with asthma symptoms is not frequently practised in Sri Lanka.Methodology: This study was a descriptive cross-sectional preliminary study conducted in a convenience sample of 11 consecutive children aged 3-6 years presenting with symptoms of asthma. Asthma Control Test (ACT) was used to assess symptom control. Lung function assessment was carried out using spirometry and impulse oscillometry (IOS) according to European Respiratory Society and American Thoracic Society guidelines. Airway obstruction cut-off was considered as the forced expiratory volume in the first second (FEV1): forced vital capacity (FVC) ratio less than 0.9.Results: The study sample consisted of 4 boys and 7 girls. Nine participants had good control of asthma symptoms (ACT>19). Only three participants performed spirometry in an acceptable and reproducible manner and two of them demonstrated evidence of airway obstruction. FVC less than 0.8 was noted in two participants probably attributable to poor effort (pseudo-restriction). Significant bronchodilator reversibility was not demonstrable in any patient, and two participants had reduced FEV1:FVC ratio following administration of beta-2-agonists probably due to paradoxical bronchoconstriction or improvement of FVC.IOS assessment was completed successfully by seven children. Increased R5-20 was detected in 3 participants and increased resonance frequency in another two children, suggesting probable peripheral airway involvement.Conclusion: Airway obstruction in preschool children could be assessed using spirometry and IOS. IOS is more feasible in preschool children.
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