Successful management of a small infant with Kasabach-Merritt phenomenon using vincristine: a case report.

Kasabach-Merritt Phenomenon (KMP) is characterized by profound thrombocytopenia, microangiopathic haemolytic anaemia and consumptive coagulopathy in the presence of an enlarging vascular lesion. The syndrome usually develops in infancy and is associated with a high morbidity and mortality rate. We report a case of successful management of refractory KMP in a very small infant with the use of vincristine. A female neonate was born with a giant haemangioma on the right thigh and soon presented with coagulopathy and severe thrombocytopenia due to rapid enlargement of the lesion. The condition proved refractory to steroids and propranolol, and the baby was on supportive therapy with daily administration of red blood cells, platelets and cryoprecipitate. Treatment failure and the risk of serious bleeding led to the decision of starting vincristine on the 45th day of life. During the first week of therapy, haematological parameters improved rapidly, and on the second week, the infant had no need for blood products. By the third week of treatment, platelet count and fibrinogen levels had normalized, and the tumour size was dramatically reduced. The infant completed therapy without experiencing any side-effects and had no relapse during the two years that followed. Vincristine proved to be safe, effective and well tolerated in the treatment of this young baby with severe form of KMP. The report, also, highlights the need for considering vincristine early in the management of KMP, especially in cases of rapidly expanding haemangiomas that raise the suspicion of possible malignant lesions (kaposiform haemangioendothelioma/tufted angiomas).

Bone status of children with hemophilia A assessed with quantitative ultrasound sonography (QUS) and dual energy X-ray absorptiometry (DXA).

Recent studies report reduced bone mineral density (BMD) even among young adults and children with hemophilia. Our aim was to assess bone status in children and adolescents with hemophilia with 2 methods: Quantitative UltraSonography (QUS) and Dual energy x-ray Absorptiometry (DXA), and consequently to investigate the degree of correlation between them. Twenty-seven patients (17 with severe hemophilia; residual factor activity <1% and 10 with moderate hemophilia) participated in the study. Mean age was 12.28±4.48 y (range: 4.94 to 18.00 y). All patients were evaluated with QUS at radius and at tibia and had DXA scan at lumbar spine. Anthropometric parameters were measured using standard techniques and joint evaluation was carried out using the Hemophilia Joint Health Score (HJHS). Only 2 out of 27 patients (7.5%) had BMD Z-scores <-2, whereas another 4 patients (15%) had BMD Z-scores between -1 and -2. QUS values in both radius and tibia were generally within the normal limits as only 1 patient had radius and another 1 had tibia QUS Z-score <-2. HJH scores were significantly although negatively correlated to Z-scores of tibia QUS (r=-0.455, P=0.034). No correlations were observed between lumbar BMD and radius or tibia QUS and no agreement was recorded between QUS and DXA in identifying patients at risk for osteoporosis (k=0.262). In conclusion, our study showed that only a small number of children and young adults with hemophilia have impaired bone properties as assessed both by DXA and QUS; no correlation was observed between these 2 methods.

The effect of dietary habits and socioeconomic status on the prevalence of iron deficiency in children of northern Greece.

BACKGROUND: Despite encouraging reports concerning the declining prevalence of iron deficiency, this easily preventable disorder is still an existing problem in presumably developed regions. OBJECTIVE: To evaluate the prevalence of iron deficiency and relevant anemia in children residing in Northern Greece and to study possible associations. DESIGN: 3,100 children aged 8 months to 15 years were evaluated. Socioeconomic status was determined based on the parents' profession and place of residence. Nutrition habits were also evaluated. RESULTS: The incidence of iron deficiency was found to be 14% and that of iron deficiency anemia was 2.9%, with a higher prevalence in children younger than 2 years of age. The place of residence was the most significant factor in relation to the development of iron depletion in the children studied. Additional independent factors were revealed to be breast-feeding, meat-containing meal consumption and the consumption of non-home-cooked meals. CONCLUSION: Iron deficiency remains prevalent in Northern Greece, mainly affecting the vulnerable toddler group. Nutritional iron deficiency is still a severe public health problem even in what are considered to be developed regions. An improvement of dietary habits and an upgrading of semiurban areas should contribute substantially to decreasing the prevalence of iron depletion in Greek children.