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Biomedical subjects

T Gürsel

Publications and source records attributed to T Gürsel.

16 recordsLinked to original sources
PubMed2000

Thrombosis during all-trans-retinoic acid therapy in a child with acute promyelocytic leukemia and factor VQ 506 mutation.

Acute promyelocytic leukemia (APL) is often associated with a severe hemostatic disorder, caused by the release of procoagulant and fibrinolytic substances from leukemic blasts. The coagulation profile may exhibit disseminated intravascular coagulation and fibrinolysis or proteolysis. Therefore, heparin and antifibrinolytic agents alone or in combination have been used to prevent severe bleedings. Remission induction with all-trans-retinoic acid (ATRA) is accompanied with rapid correction of hemostatic abnormalities. Thrombosis is a rare complication of APL and may be due to the alterations in hemostasis caused by the disease itself as well as ATRA and antifibrinolytics. Here, the occurrence of thrombosis during induction treatment with ATRA combined with aprotinin and chemotherapy is described in a patient who is homozygous for factor VQ 506 mutation.

Adolescent↗
PubMed2000

The effect of steroid and intravenous immunoglobulin on thrombopoietin levels in pediatric patients with immune thrombocytopenic purpura.

Thrombopoietin (TPO), which is the main regulator of megakaryo/thrombopoiesis, has been recently cloned and purified, and shown to be useful in discriminating thrombocytopenia due to decreased production or increased platelet destruction. However, there are no detailed investigations about the drug effects on TPO levels during childhood. This study was conducted to measure the TPO levels of children with immune thrombocytopenic purpura (ITP) during steroid and immunoglobulin treatment. Twelve patients with acute ITP were treated with high-dose methyl prednisolone and five patients were treated with intravenous immunoglobulin. Neither steroids nor immunoglobulin were found to have any effect on TPO levels.

Acute Disease↗
PubMed1997

Increased serum leukotriene B4 level in the active stage of rheumatoid arthritis in children.

The possible association of leukotriene B4 (LTB4)-like activity with the development of active rheumatoid arthritis was studied in 25 children with the disease and in 15 normal subjects. Serum LTB4-like activity was found to be significantly higher in the active stage of the disease when compared with the values obtained from patients during the inactive stage and from healthy children. No correlation was found between LTB4-activity and other laboratory parameters, e.g. haemoglobin level, white cell count and erythrocyte sedimentation rate.

Adolescent↗
PubMed1997

Bone marrow hemophagocytosis and immunological abnormalities in a patient with lysinuric protein intolerance.

Lysinuric protein intolerance (LPI) is an inborn error of amino acid transport characterized by a wide spectrum of clinical and biochemical abnormalities. Bone marrow hemophagocytosis in this disorder is an intriguing finding, present mostly in Italian patients. We report a 19-month-old Turkish infant with LPI, bone marrow hemophagocytosis, interstitial lung disease and immunological abnormalities unprecedented in the current literature. Possible etiologic factors responsible for hemophagocytosis and the differential diagnosis of hemophagic syndromes are discussed.

Amino Acid Metabolism, Inborn Errors↗
PubMed1980

Effect of intravenous adrenalin infusion and corticosteroid treatment in patients with von Willebrand's disease.

Intravenous adrenalin infusion (5 micrograms/kg, at most 160 micrograms in 10 min) normalized partial thromboplastin time (PTT) and ristocetin induced platelet aggregation (RIPA) in 4 of the 14 patients with von Willebrand's disease (VWD) in a short period of time. Although mean factor VIII (F-VIII) procoagulant activity was almost doubled 5 min following infusion, this was mainly observed in patients with relatively high baseline (> 2%) AHF activity. Mean F-VIII procoagulant activity rose by more than 100% following 10 days of corticosteroid treatment (deltacortil 2 mg/kg/day, at most 60 mg/day). PTT became normal in 6 of the 11 patients, but RIPA normalized only in 2. The improvement of RIPFA did not correspond to bleeding time in every patient. These results may suggest that if the baseline AHF activity is relatively high (greater than or equal to 9.5%), corticosteroid could be tried before schedule surgical intervention in patients with VWD.

Adolescent↗
PubMed1978

Hyperinsulinemia in childhood cirrhosis.

Circulating insulin was determined by radioimmunoassay in ten children with cirrhosis of the liver and in 6 age-matched controls. They were found to be elevated in cirrhotics during fasting as well as at 1/2, 1, 1 1/2 and 2 h during the oral glucose tolerance test. These determinations were also carried out in two children with extrahepatic portal hypertension to show the possible role of portal systemic shunts for the elevated serum insulin levels. We believe that the hyperinsulinemia of these patients was related to decreased insulin degradation by the liver as well as to portal-systemic shunting.

Adolescent↗
PubMedDate not supplied

Thrombophilia.

Explore the source record for details and available documents.

Humans↗
PubMedDate not supplied

Supernumerary nipples in children with hematologic disorders.

The authors report on supernumerary nipples and various hematologic disorders in 7 patients [factor X deficiency (n = 1), factor XI deficiency (n = 2), acute lymphoblastic leukemia (n = 3), and acute myeloblastic leukemia (n = 1)]. They would like to draw attention to the association of supernumerary nipples with hematological disorders, which has not been published before and is considered to be added to the anomalies associated with supernumerary nipples.

Abnormalities, Multiple↗
PubMedDate not supplied

Bernard-Soulier-like functional platelet defect in myelodysplastic syndrome and in acute myeloblastic leukemia associated with trilineage myelodysplasia.

Platelet function was studied in a child with myelodysplastic syndrome (MDS: refractory anemia with an excess of blasts) and a child with acute myeloblastic leukemia (AML-M6) associated with trilineage myelodysplasia (TMDS). An acquired Bernard-Soulier-like platelet defect was considered in both patients with the findings of prolonged bleeding time and abnormally large platelets that failed to aggregate in response to ristocetin. In contrast to findings in von Willebrand's disease, the abnormal response of platelets to ristocetin could not be corrected by the addition of normal flesh plasma. The detection of abnormal platelet aggregation response to ristocetin may be a useful diagnostic finding for clonal disorders causing impaired platelet function in MDS and coexistent TMDS associated with AML. Further studies of ristocetin-induced platelet aggregation in a large number of these patients are required.

Adolescent↗
PubMedDate not supplied

Association of xeroderma pigmentosum with thrombasthenia.

Xeroderma pigmentosum (XP) is an autosomal recessive disorder characterized by severe sun-sensitivity, early skin cancers and abnormal DNA repair. XP has a worldwide distribution with an approximate frequency of 1/250,000. It is classified into nine complementation groups, and distribution of patients among the various groups is related to ethnic origin. To our knowledge, the association of XP with thrombasthenia has not been reported previously; here a 12-year-old girl with this combination is reported. She was first noted to have skin erythema on exposure to sunlight at the age of six months and was diagnosed with XP. At the age of one she had the complaints of easy bruising and epistaxis. A diagnosis of thrombasthenia was made based on the absence of platelet aggregation response to ADP, collagen and adrenaline and reduced clot retraction. In clinical management, oral isotretinoin was given in order to suppress tumor formation.

Child↗