Nikolaos Gompakis

Aristotle University of Thessaloniki, Saloníki, Central Macedonia, Greece

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Publications (8)9.6 Total impact

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    ABSTRACT: Background: Hydroxyurea is a cytotoxic and myelosuppressive drug that has been used during recent years in the treatment of children with severe sickle cell disease. Nevertheless, questions remain regarding its role in young patients with no severe course, like sickle/beta-thalassemia (S/b-thal) patients often present. The aim of the present study was to evaluate the safety and efficacy of hydroxyurea in young patients with S/b-thal, which is the commonest form of the disease in Greece. Patients-Methods: Hydroxyurea was given in thirteen children with S/b-thal for 24 months and for that period clinical and laboratory evaluation of the children was performed. Results: A reduction in pain crises and rate of hospitalization was noted. None of the patients presented with a severe clinical event, related to the disease during the study period. A significant increase in hemoglobin, hemoglobin F, mean corpuscular volume, and mean corpuscular hemoglobin and a decrease in reticulocyte count, white blood cell and platelet count, and total bilirubin level was noted. With regards to adverse events, these were transient, short-term and dose-dependable. Conclusions: To the best of our knowledge, this is the first study to specifically assess the effect of hydroxyurea therapy in young patients with S/b-thal and the results indicate is safe and efficacious in this patient cohort.
    No preview · Article · Jan 2015 · Hippokratia
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    ABSTRACT: 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).
    No preview · Article · Apr 2014 · Blood coagulation & fibrinolysis: an international journal in haemostasis and thrombosis
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    ABSTRACT: Our aim was to evaluate bone status in boys with haemophilia using dual energy X-ray absorptiometry (DXA) and quantitative ultraSonography (QUS), and in addition, to compare these two methods with the use of biochemical markers of bone turnover. Twenty-six boys with a mean decimal age of 12.08 ± 4.44 years were included in the study which included a DXA scan at lumbar spine and radial, as well as tibial QUS. Serum levels of soluble receptor activator of nuclear factor κB ligand (sRANK-L), osteoprotegerin (OPG) and osteocalcin (OC) were measured and joint evaluation was performed using the Hemophilia Joint Health Score (HJHS). With regard to the study results, only 2 of 26 patients (7.7%) had bone mineral density (BMD) Z-scores < -2, and 4 patients (15.4%) had BMD Z-scores between -1 and -2. Only one patient had radial and other two had tibial QUS Z-scores < -2. No agreement was recorded between QUS and DXA in identifying patients at risk for osteoporosis (k = 0.275, P = 0.063). Haemophiliacs had significantly higher serum levels of sRANK-L (21.04 ± 4.78 vs. 18.58 ± 2.28 ng mL(-1), P = 0.038) and of OC (5.35 ± 2.29 vs. 3.09 ± 0.61 ng mL(-1), P = 0.002) and significantly decreased levels of OPG (15.78 ± 2.53 vs. 23.79 ± 4.39 pg mL(-1), P < 0.001) compared with controls. QUS Z-scores at tibia significantly correlated with HJH Scores (r = -0.450, P = 0.040), whereas lumbar BMD Z-scores significantly correlated with body mass index Z-scores (r = 0.500, P = 0.009). More studies are warranted to identify the most accurate densitometric method for assessing bone status in haemophiliacs.
    No preview · Article · Jan 2011 · Haemophilia
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    ABSTRACT: 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.
    No preview · Article · Oct 2010 · Journal of Pediatric Hematology/Oncology

  • No preview · Article · Sep 2010 · Blood coagulation & fibrinolysis: an international journal in haemostasis and thrombosis
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    ABSTRACT: Despite encouraging reports concerning the declining prevalence of iron deficiency, this easily preventable disorder is still an existing problem in presumably developed regions. To evaluate the prevalence of iron deficiency and relevant anemia in children residing in Northern Greece and to study possible associations. 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. 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. 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.
    No preview · Article · Feb 2007 · Acta Haematologica

  • No preview · Article · Jul 2006 · Journal of Pediatric Hematology/Oncology

  • No preview · Article · Apr 2006 · Journal of Pediatric Hematology/Oncology