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Page 8 / March 07


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A vasculitis would be consistent with the history and appearances and but an important negative is the absence of the granulomatous nodules frequently seen in conditions such as Wegener's and evidence of systemic inflammation is lacking, although at this stage the not all the relevant tests are processed. Any of the interstitial pneumonitides might initially present with ground glass, but the absence of any septal thickening and the clinical history is against this as a cause. Idiopathic pulmonary hemosiderosis should be considered as a possible cause. Whatever the cause of the hemoptysis the bronchoscopy findings are impressive in degree and extensiveness and lead to urgent institution of treatment. He was therefore started on pulsed methylprednisolone at 30 mg/kg/day.

He tolerated the bronchoscopy and initially only required an FiO2 of 0.40 following the procedure. However, in the subsequent hours he developed an increasing oxygen requirement with his FiO2 rising to 0.75. He became very tachypneic with a noticeable increase in his work of breathing. On occasions he would also appear to be very anxious and his FiO2 would go up even further. He was assessed on more than one occasion by the critical response team from Paediatric Intensive Care Unit (PICU) but was able to be managed on the ward. Over the next few days he continued to cough,s had hemoptysis and needed oxygen.

On day 6 of admission he had completed a 5 day course of methylprednisolone at 30 mg/kg/day though he did require a transfusion that day. He was started on Prednisolone at 2 mg/kg/day (50 mg total).

The results of his vasculitic studies were available and were normal: Anti- GBM negative 1/10; ANCA was negative 1/20; rheumatoid and anti-DNAase negative; Anti-smooth muscle antibody negative; anti-RNP antibody negative; Anti-SS-A (Ro) antibody; Anti-SS-B (La) antibody; anti-cardiolipin; anti-phosphalipid. The only positive antibody was ANA at titre 1/160 with a speckled pattern.
The cultures from the bronchoscopy including bacterial, viral, TB and Pneumocystitis and fungal were negative. He also tested negative for H.I.V, Hepatitis B and C.

Over the following days his cough and hemoptysis continued though his oxygen requirement came down.

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