Minor anomalies in children with hematological malignancies.

BACKGROUND: Despite the presence of reports on correlation between major congenital defects and cancer, very few studies have investigated the frequency of minor anomalies in childhood malignancy. The aim of this study was to determine the prevalence of minor anomalies in children with hematological malignancy. PROCEDURE: A total of 62 well-defined minor anomalies were determined in 109 patients. The patients were compared with age- and sex-matched healthy control subjects. RESULTS: The results of this study showed that, 64.22% of the patients and 26.6% of the controls had at least one minor anomaly. Among the minor anomalies detected, pigmented nevi and café-au-lait spots were significantly more frequent in the patients. The prevalence of minor anomalies in the patients was significantly higher than that of the controls in the present study. CONCLUSION: Our results contribute to the understanding of the role of genetic factors in childhood hematological malignancies. Future studies may be directed toward identifying the developmental pathways and the relevant genes that are involved in the overlap between childhood hematological malignancies and minor anomalies.

Severe vitamin B12 deficiency in a breast fed infant with pancytopenia.

We report the case of a 7-month-old breast fed infant who presented with a nose bleed and bruises. Investigation showed severe nutritional B12 deficiency anemia with a pancytopenia. It is important to take the nutritional history of both the infant and the mother for early prevention and treatment.

Hematologic manifestations of brucellosis in children: 5 years experience of an anatolian center.

BACKGROUND: Brucellosis continues to be an important cause of fever in underdeveloped countries and in the rural areas of developed world. It is a multisystemic disease, associated with a wide variety of symptoms. A wide variety of symptoms, including hematologic abnormalities, such as anemia, thrombocytopenia, pancytopenia, dissemine intravascular coagulation, and leucopenia could be seen. The aim of the study is to review the hematologic findings of brucellosis in childhood. PROCEDURE: In this short study, the records of 146 children with brucellosis were evaluated for hematologic manifestation retrospectively. Among them, 9 patients had pancytopenia and 5 had brucella-induced immune thrombocytopenia and were identified in a 5-year period between June 2004 and July 2009. RESULTS: Eight of the 9 patients with pancytopenia had Brucella melitensis isolated from blood cultures and/or bone marrow cultures, and all 9 patients had Brucella agglutination titers of at least 1:320. All patients with immune thrombocytopenia blood cultures were positive for Brucella. Except 1 patient the pancytopenia in these patients regressed completely and their peripheral blood counts returned to normal after treatment of Brucella infection. One patient was not responding to the brucella treatment and underwent allogeneic hematopoietic stem cell transplantation. All patients with brucella-induced immune thrombocytic purpura were symptomatic and had severe thrombocytopenia, they were placed on intravenous gamma globulin for 2 days. Between day 3 and day 5 platelet counts increased in these patients. CONCLUSION: Brucellosis should be considered as a possible diagnosis among patients with pancytopenia and immune thrombocytopenic purpura in endemic regions.

Evaluation of glucose homeostasis in transfusion-dependent thalassemic patients.

Diabetes is an important problem encountered in thalassemic patients. The severity and type of glucose disturbances vary greatly in different studies. Also the pathogenesis seems to be complex; either insulin deficiency or insulin resistance may mediate the glucose disturbances. In a group of thalassemic patients glucose homeostasis was evaluated. Diabetes prevalence was 1.8%. Forty patients were investigated both with an oral glucose tolerance test and first-phase insulin response. Three patients had impaired fasting glucose, 1 patient had impaired glucose tolerance, and 2 patients had hyperinsulinism. Nineteen of 40 patients who were tested had low first-phase insulin response (47.5%) with below 10th centile. Age, BMI, height SDS, age at diagnosis, age at first blood transfusion, number of blood transfusions in a year, percentage of elevated liver enzyme, and hemoglobin and ferritin levels were not different between patients with low first-phase insulin response to patients with normal first-phase insulin response. Four patients are HCV infected, and only 1 of them had low first-phase insulin response. The study group showed a high rate of impairement in insulin secretion by first-phase insulin response to glucose overload, despite the low rate of insulin resistance. Defect of insulin secretion in thalassemic patients may develop earlier than insulin resistance, and then be accompanied by insulin resistance. Increasing insulin resistance with age and the occurrence of additional factors could lead to detoriation of glucose metabolism.

All-trans-retinoic acid-induced myositis in a child with acute promyelocytic leukemia.

Anthracyclin-based regimens and all-transretinoic acid (ATRA, tretinoin) as differentiating agent are commonly utilized for the treatment of acute promylelocytic leukemia (APL). There are many adverse effects that may be seen during the use of ATRA in patients with APL. Of these, ATRA-induced myositis is rarely described in adults and rare in the children with APL. Herein, we report an 11-year-old girl with APL who developed ATRA-induced myositis during induction treatment.

Serum carnitine levels in children with iron-deficiency anemia with or without pica.

Carnitine is ingested through animal-derived foods as well as synthesized in vivo. It plays an important role in the energy metabolism of many tissues. Iron acts as a co-factor for the synthesis of carnitine. However, the importance of iron deficiency as a cause of secondary carnitine deficiency is not well established. The aim of this study was to investigate the serum levels of carnitine in children with iron-deficiency anemia compared to those of healthy children and to determine if serum carnitine levels in with or without pica differ. The mean serum carnitine concentration in the iron-deficiency group was significantly lower than that in healthy children (12.44+/- 5.09 and 32.48 +/- 7.92 micromol/L, respectively, p < .001). In the iron-deficient group, serum carnitine levels, ferritin levels, and other hematological parameters were lowest in patients with pica (p < .001). Pearson correlation test indicated a positive correlation between serum carnitine and ferritin levels in iron-deficient patients. Based on the evidence about the effect of low iron on carnitine stores in animal studies, the authors propose that low serum carnitine levels in these children may be secondary to iron-deficiency anemia. However, further large-scale studies are needed to establish the frequency of carnitine deficiency in children with iron-deficiency anemia.

Complications of varicella zoster.

Primary infection with varicella zoster is characterzed by a generalized vesicular rash usually without significant systemic illness. Encephalitis, pneumonitis, pancreatitis, nephritis, Reye and Guillan-Barre syndrome transvers myelitis, myocarditis have been reported before, but there is not any case having all these system to be involved during the same infection in a sequential manner ending up with multiorgan failure. We wanted to represent 21-month-old boy had a multiorgan failure due to varicella zoster infection.