1/3. Griscelli syndrome.A 4-month-old child had silvery gray hair, light-colored skin, recurrent chest infections, hepatosplenomegaly, and episodes of pancytopenia and hemophagocytosis in the liver, spleen, and bone marrow. light microscopy of hair showed characteristic large aggregates of pigment granules distributed irregularly along the hair shaft. Peripheral blood smear examination did not show giant granules in granulocytes. Enlarged hyperpigmented basal melanocytes with sparsely pigmented adjacent keratinocytes were seen on the skin biopsy specimen. On the basis of these clinical and laboratory findings, Griscelli syndrome was diagnosed. The child succumbed to infection during an accelerated phase of the disease.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/3. Respiratory tract symptoms as a clue to giant cell arteritis.Although many manifestations of giant cell arteries are increasingly recognized, little attention has been paid to respiratory symptoms associated with this disorder. We report the cases of 16 patients with giant cell arteritis who had prominent symptoms related to the respiratory tract including cough, sore throat, and hoarseness. These symptoms were the initial finding in 10 patients and obscured the diagnosis in some instances, but resolved quickly when corticosteroids were given. It is estimated that 9% of patients with giant cell arteritis have prominent respiratory tract symptoms, which are the initial manifestation in 4%. This disorder should be considered in an older patient with a new cough or throat pain without obvious cause.- - - - - - - - - - ranking = 7keywords = giant (Clic here for more details about this article) |
3/3. necrobiotic xanthogranuloma with cardiac involvement.A patient with necrobiotic xanthogranuloma (NXG) and paraproteinaemia, who was followed-up for several years, and treated with low-dose chlorambucil, died as a result of a respiratory illness. The significant findings at autopsy were a xanthogranuloma of the spleen and giant cell myocarditis. The myocardial lesions were composed of macrophages, giant cells and lymphocytes. This finding is important because four of five known autopsied patients with NXG have had giant cell myocardial disease, and an effort at antemortem diagnosis should be made.- - - - - - - - - - ranking = 3keywords = giant (Clic here for more details about this article) |