Gluten-Related Methemoglobinemia

PubMedID: 26348306[No abstract available

Insulin Like Growth Factor (IGF) [İnsülin benzeri büyüme faktörü (IGF)]

Insulin Like Growth Hormone (IGF) is a peptide family, depends on and suplying most of the anabolic and mitogenic effects of growth hormon (GH). Beside its GH dependance of serum consantration, showing its effects in tissues other than skeletal ones, increasing the binding of sulphate to the cartilage, stimulating the DNA synthesis and cell proliferation it has also some other duties. IGF-1 is a sample peptide of 70 aminoacids also celled SM-C. IGF-2 is an acidic peptide composed of 67 aminoacids. In both peptides 45 of the 73 aminoacid position are same and almost % 50 aminoacid of them also same with insulin. Both IGF consists of A and D chains binding with disulphide bindings like insulin. We present this review so as to evaluate the findings of the studies made in recent years about Insulin Like Growth Factor

Neurologic findings of nutritional vitamin B12 deficiency in children

PubMedID: 20402062We report herein our interesting case series of 15 infants admitting with neurological symptoms who were found to have vitamin B12 deficiency. Infants who were admitted to our hospital between 2004 and 2007 with neurological symptoms and were found to have vitamin B12 deficiency were included in this study. Data regarding clinical and laboratory features were obtained. Of 15 infants, 9 were boys (60%) and 6 were girls (40%). The mean age was 11.7 months. Anorexia, pallor, hypotonia, and neurodevelopmental retardation were present in all infants. Seizures and tremor were observed in 46.6% (7/15) and 33% (5/15) of patients, respectively. Seizures were generalized tonic-clonic in 4 patients, generalized tonic in 1 patient and focal in 2 patients. Four patients had tremor on admission and 1 patient had occurrence after vitamin B12 treatment. Vitamin B12 deficiency may lead to serious neurological deficits in addition to megaloblastic anemia. Persistent neurological damage can be prevented with early diagnosis and treatment. We believe that a thorough clinical and neurological assessment might prevent failure to notice rare but possible vitamin B12 deficiency in infants with neurological deficits and neurodevelopmental retardation

Pseudotumor cerebri, after the withdrawal of steroid treatment [Steroid tedavisinin kesimi sonrasi gelişen psödotümör serebri]

Pseudotumor cerebri or benign idiopathic intracranial hypertension is a disease characterized by increased intracranial pressure without any lesion with mass effect or hydrocephaly. In etiology beside several factors, we want to present a case with pseudotumor cerebri after steroid withdrawal because its rarely seen

Vascular endothelial growth factor levels in childhood acute lymphoblastic and myeloblastic leukemia

Angiogenesis has been associated with the growth, dissemination and metastasis and has been shown to be a prognostic. Although there are some data suggesting that angiogenesis may have a role in the pathophysiology of leukemia, its role in patient prognosis is yet to be defined. We analyzed the expression level of vascular endothelial growth factor (VEGF), an angiogenesis promoter and its possible- prognostic value in bone marrow samples at the time of diagnosis and remission of acute childhood leukemia patients. Besides 46 patients diagnosed as ALL or AML, 16 children were also included as a control group in the study. Our data have demonstrated that VEGF levels of AML patients were found higher than the control group statistically (P = 0.022). However we could not find any significant difference between VEGF levels of diagnosis and remission in both AML and ALL groups by blastic VEGF expression (P > 0.05). In this study the higher levels of VEGF in AML patients is one of the main findings although we were not able to assess any role of VEGF in predicting prognosis in pediatric leukemia patients by evaluating blastic cell VEGF expression. These results have demonstrated that the relationship between angiogenesis or angiogenesis promoters and hematological malignancies is not clear and simple as different methods or different cells beside different angiogenesis promotors are involved to these studies. So that not only tumor cells and their cytokines but also surrounding cells and their cytokines must be taken into consideration with the standardized study methods in the further studies to obtain a promising treatment approach. © 2011 Indian Society of Haematology & Transfusion Medicine

Management of life-threatening hemorrhages and unsafe interventions in nonhemophiliac children by recombinant factor VIIa

PubMedID: 18838398The literature on the use of recombinant factor VIIa (rFVIIa), which was initially used in hemophiliac patients with inhibitors, for hemorrhages that cannot be managed with conventional methods or operations that cannot be performed safely is increasingly growing. This study presents a group of nonhemophiliac patients with hemorrhagic problems or hemorrhage risk for some interventions that were successfully resolved with the use of rFVIIa. The patient group was composed of 20 patients with different disorders resulting in similar results as hemorrhage or hemorrhage risk. Most of the patients were diagnosed with liver disorders primary or secondary to other diseases. The remaining cases were patients with leukemia, sepsis, intracranial hemorrhage, and burn. Some of the patients had multiple problems like a patient with liver disorder and intracranial hemorrhage or a leukemia patient with sepsis and disseminated intravascular coagulation. rFVIIa had been administered to the patients at dosages between 70 and 150 µg/kg up to 6 doses with 2-hour to 3-hour intervals. All the patients had benefited from the use of rFVIIa even though some of them died because of primary disease. This study shows that rFVIIa can be safely used in high-risk patients with a history of recurrent hemorrhage, for whom no improvement can be achieved in the hemostasis tests

Recurrent epididymo-orchitis in an 8-year-old child with Kostmann syndrome (severe congenital neutropenia)

PubMedID: 16709337Underlying congenital genito-urinary tract anomalies are the most common cause of recurrent epididymo-orchitis in prepubertal boys. An 8-year-old boy was admitted with recurrent pulmonary and skin infections, was diagnosed as Kostmann syndrome and developed epididymo-orchitis. This appears to be the first case of Kostmann syndrome associated with epididymo-orchitis. © 2006 The Liverpool School of Tropical Medicine

Circumcision in patients with hemophilia: Ten years experience in Adana, Turkey

12th Congress of the European-Hematology-Association -- JUN 07-10, 2007 -- Vienna, AUSTRIAWOS: 000247176901474…European Hematol Asso

Startle disease-two sibling cases

PubMedID: 16250316Startle disease (hyperekplexia) is a rare non-epileptic disorder characterized by hypertonia, generalized stiffness and brief muscle jerks in response to unexpected auditory, somatosensory and visual stimuli. In this paper, two siblings with generalized stiffness and sudden muscle jerks to unexpected stimuli of various types are presented. They were previously misdiagnosed as epilepsy and treated with the conventional antiepileptic drugs. We wanted to call attention to and increase awareness of this rare disease and its differential diagnosis to avoid unnecessary investigations and treatment

Serial urological interventions including circumcision in a hemophilic child with inhibitors

PubMedID: 21519231Hemophilia patients sometimes need careful treatment of urgent serious bleedings and management of some surgeries. Development of inhibitor has some impact on the management of these situations. Here a case of patient of hemophilia A is presented in whom urological surgery resulted in inhibitor development and a second operation with bypass agent. © 2011 Wolters Kluwer Health | Lippincott Williams &Wilkins