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Idiopathic Pulmonary Hemosiderosis

Winded : Part 2

Step 1: View clinicals

A 6 year old refugee presented 1 day ago with impending type 2 respiratory failure, and was managed with intubation and mechanical ventilation. During suction, active, fresh bleeding into the endotracheal tube was noted, even though intubation was non-traumatic; the bleeding subsided over time. He has experienced multiple episodes of cough, wheezing, epistaxis, and cyanosis for the last 2 years. Detailed medical records are not available. A complete blood count reveals microcytic anemia with a Hb of 10.9 and normal platelet counts, while a chest x-ray shows reticular-nodular opacification in the perihilar and basal regions Blood and endotracheal aspirate cultures showed no growth, while a urinalysis, renal functions, liver functions, ECG, and echocardiogram were normal.

Step 2: Order all relevant investigations

Coagulation Profile

aPTT: 29 sec (control: 30 sec) PT: 15 sec (control:17 sec) INR: 1.0 (0.9 - 1.1) Bleeding time: 6 min (1-13)

Open Lung Biopsy

Macroscopy: Areas of hemorrhage are noted in the specimen. Microscopy: The alveolar spaces are filled with red blood cells and macrophages containing hemosiderin. There is an interstitial chronic pneumonitis with diffuse fibrosis, muscularization, and hemosiderosis of the elastic lamina of the arteries, indicating recent alveolar hemorrhage.

ANA + ANCA + anti-GBM Abs

Circulating anti-GBM antibodies: negative Antineutrophil cytoplasmic antibodies (C-ANCA): negative Anti-myeloperoxidase antibodies (p-ANCA): negative Antinuclear antibodies (ANA) and anti-DNA antibodies: negative

Precipitins to Casein

Titers of serum precipitins to casein and lactalbumin: negative

Step 3: Select appropriate management

Non-dairy Diet

Score: ★★☆