Lata Kumar

Hyperimmunoglobulin E syndrome

Hyperimmunoglobulin E (HIE) syndrome is a primary immunodeficiency disorder characterized by recurrent bacterial infections in presence of very high serum Ig E levels. We are reporting a nine-year-old child with HIE syndrome and reviewing literature on this disease.Hyperimmunoglobulin E (HIE) syndrome is a primary immunodeficiency disorder characterized by recurrent bacterial infections in presence of very high serum Ig E levels. We are reporting a nine-year-old child with HIE syndrome and reviewing literature on this disease.

Pulmonary Capillary Haemangiomatosis as a Cause of Pulmonary Hypertension in Takayasu’s Aortoarteritis

Pulmonary hypertension is known to occur in Takayasus aortoarteritis. It may be either due to pulmonary arterial involvement or elevated left ventricular end diastolic pressure, or both. In our case, the cause of pulmonary hypertension was a recently described rare lesion termed pulmonary capillary haemangiomatosis. This entity has a very distinct histopathologic picture. Although 19 cases have been reported in the English literature, this is the first report on pulmonary capillary haemangiomatosis producing pulmonary hypertension in Takayasus aortoarteritis.