The present study reports the results in 284 patients of applying a recently developed index, MCV-(10 x RBC), for discrimination between beta-thalassemia trait (beta-TT) and Iron Deficiency Anemia (IDA), the two most common causes of microcytic hypochromic anemias. A total of 284 carefully selected patients (130 patients with IDA and 154 with beta-TT) were studied. Sensitivity, specificity and Youden's index were compared between the proposed index and four other indices, namely England-Fraser, Mentzer, Srivastava and RBC count. The new index correctly identified 263 (92.96%) patients, standing inferior only to Mentzer which correctly diagnosed 269 (94.71%) patients. The best discrimination index according to Youden's criteria was Mentzer (Youden's index = 90.1) followed by the new index (Youden's index = 85.5). There are remarkable inconsistencies among the results obtained in different studies. Larger studies are needed to establish the optimal discrimination index as well as to confirm the results obtained in the present study. Nevertheless, the epidemiological indices of the proposed discrimination index and the simplicity of its calculation make it acceptable for use in Iran.
Cytomegalovirus (CMV) disease in pediatric acute lymphoblastic leukemia in the nontransplant setting is very rare. We report our experience with 4 such cases, and review the literature (n=12). The median age at diagnosis was 10 years and 50% of patients were males. Among the 11 cases with available information at the time of diagnosis, CMV disease occurred during maintenance therapy in 10 patients. Fever was present in 9 cases. CMV disease manifested as retinitis in 6, hepatosplenic disease in 3, pneumonitis in 1, and hemophagocytic lymphohistiocytosis in 1 patient. One patient had both CMV retinitis and CMV-related hemophagocytic lymphohistiocytosis. Four of the 7 patients with retinitis complained of visual disturbance at diagnosis. CMV viremia was present in 10 patients. Three patients had at least 1 relapse and developed permanent visual defects, and 1 patient developed recurrent retinal detachment. In conclusion, prolonged immunosuppression is the major etiology and retinitis is the most common manifestation of CMV disease. As a significant number of patients with retinitis are asymptomatic, early diagnosis and treatment is important to prevent permanent visual loss.
Influenza vaccine is tolerated well in ALL patients with acceptable but limited immune response compared to healthy controls. These findings support the recommendation for annual influenza vaccination in children with ALL.
Hematohidrosis (bloody sweat), also known as hematidrosis, is a rare clinical entity characterized by recurrent, spontaneous, self-limited episodes of blood oozing from intact skin or mucosa that are witnessed by medical personnel. We hereby report three healthy children with the history of recurrent episodes of bleeding from the intact skin over their various parts of the body. Most of these bleeding episodes occurred after stressful situations. On the basis of clinical presentation and normal investigation, they were diagnosed with hematohidrosis. We treated them with non-specific beta-blocker (propranolol). Follow-up observation of these patients demonstrated marked diminish in the frequency and severity of their episodes. In spite of the rarity of hematohidrosis, it should be considered as a differential diagnosis of bleeding episodes in patients with normal physical and laboratory investigations.
We describe a newborn girl with right-sided extended epidermal nevus, congenital rhabdomyosarcoma of the inguinal area at birth who had developed central precocious puberty, hemihypertrophy and vitamin D3-responsive hypophosphatemic rickets at the age of 14 months. Our patient demonstrates a much broader and polymorphic spectrum of organ systems involvement in epidermal nevus syndrome at a very early age of her life.
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