Page 16 / March 07
The above tests were not corrected for racial origins. Keeping in mind that he is Asian the measurements are probably within the normal range for his age and ethnicity. Given that he has had an acute pulmonary haemorrhage one might have expected a higher value for the DLCO. The lower value, with the caveats on ethnicity, indicates that at time of testing there was no gross pulmonary collection.
Approximately, a month after his first dose he had cyclophosphamide again. Despite adequate hydration he developed hemorraghic cystitis. Further monthly cyclophosphamide treatments were uncomplicated and the aim is to complete a 5-6 month course. His Hgb stabilized at a reasonable level and his CXR remained clear.
Summary
An 8 year old boy with intermittent hemoptysis for 6 months presenting with anemia with no respiratory distress but a CXR showing airspace disease confirmed on CT scan. Bronchoscopy showed profuse hemorrhage. He developed respiratory distress in addition to persistent hemoptysis with no response to pulsed methylprednisolone and had to be admitted to PICU after a lung biopsy. The lung biopsy showed pulmonary capillaritis with mild neutrophilia. He was intubated and ventilated for 3weeks during which he had hemoptysis and required frequent blood transfusions. He was treated with pulsed methylprednisolone, intravenous immunoglobulin to maintain levels above 40 g/L and cyclophosphamide. After extubation he required no further transfusions and was able to be discharged on oral prednisolone and monthly cyclophosphamide. He has had no further admissions and hemoglobin has normalized.
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ANSWER:
The above tests were not corrected for racial origins. Keeping in mind that he is Asian the measurements are probably within the normal range for his age and ethnicity. Given that he has had an acute pulmonary haemorrhage one might have expected a higher value for the DLCO. The lower value, with the caveats on ethnicity, indicates that at time of testing there was no gross pulmonary collection.
Summary
An 8 year old boy with intermittent hemoptysis for 6 months presenting with anemia with no respiratory distress but a CXR showing airspace disease confirmed on CT scan. Bronchoscopy showed profuse hemorrhage. He developed respiratory distress in addition to persistent hemoptysis with no response to pulsed methylprednisolone and had to be admitted to PICU after a lung biopsy. The lung biopsy showed pulmonary capillaritis with mild neutrophilia. He was intubated and ventilated for 3weeks during which he had hemoptysis and required frequent blood transfusions. He was treated with pulsed methylprednisolone, intravenous immunoglobulin to maintain levels above 40 g/L and cyclophosphamide. After extubation he required no further transfusions and was able to be discharged on oral prednisolone and monthly cyclophosphamide. He has had no further admissions and hemoglobin has normalized.
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