Yildiz Yildirmak

Recombinant human growth hormone treatment in children with thalassemia major.

Abstract Background: To evaluate the growth hormone reserve and the growth hormone response to recombinant human growth hormone (GH) in prepubertal thalassemic children with growth retardation.

Imipenem-cilastatin versus piperacillin-tazobactam as monotherapy in febrile neutropenia.

Background:  In view of the recent trend toward monotherapy in the treatment of febrile neutropenia, we evaluated the clinical efficacy and safety of imipenem–cilastatin versus piperacillin–tazobactam as an empiric therapy for febrile neutropenia in children with malignant diseases.

Comparison of piperacillin tazobactam and cefoperazone sulbactam monotherapy in treatment of febrile neutropenia.

Monotherapy has tended to replace the combination therapy in emprical treatment of febrile neutropenia. There is no reported trial which compares the efficacy of cefoperazone‐sulbactam (CS) and piperacillin‐tazobactam (PIP/TAZO) monotherapies in the treatment of febrile neutropenia. In this prospective randomized study, we aimed to compare the safety and efficacy of CS versus PIP/TAZO as empirical monotherapies in febrile neutropenic children with cancer.

Hypercoagulability in Children with Thalassemia Major

Objective: We wished to determine the role of various factors causing hypercoagulability in thalassemia patients. Methods: Forty-six homozygous β-thalassemia patients were investigated. Protein C, protein S, and antithrombin (AT) levels were measured and lupus anticoagulants (LA) were screened. D-Dimer and fibrinopeptide A ( FPA) levels were measured to show the activation of the fibrinolytic system. Ten healthy children served as controls. Results: There was a marked decrease in protein C activity in 44.4% and in protein C antigen in 53.8% of the patients. Although no significant differences was noted between the mean values for protein S in the patient and control groups, protein S activity was <60% in 40% of the patients. AT levels were always normal. D-Dimer and FPA levels were increased, indicating the ongoing coagulation activation and fibrinolysis. Three patients had LA; which reflect the expression of phosphatidylserine on the outer surface of the erythrocyte membrane. Conclusions: In thalassemic patients, there is activation of the coagulation and fibrinolytic system which is believed to be secondary to an underlying mechanism. The presence of LA in some patients, probably due to the expression of PS on the outer surface of the erythrocyte membrane, may be the initiating event. Key Words: Thalassemia-Hypercoagulability-Protein C—Protein S—Antithrombin—Antiphospholipid antibodies.

Assessment of hepatitis B immunization status after antineoplastic therapy in children with cancer.

Background and Objectives: Hepatitis B is a disease that is preventable with vaccination. Antibody levels after vaccination may be affected by suppression of the immune system due to cancer therapy. Children with cancer have a high risk of hepatitis B virus (HBV) infection. We aimed to assess the pretreatment immunization status against HBV infection and the rate of continuity of immunization after therapy in children with cancer. Design and Setting:Retrospective case review of patients treated from 2004 to 2008. Patients and Methods: We reviewed the medical records of patients treated in the departments of pediatric hematology and oncology and collected data on immunization history and hepatitis B serology. Anti-HBs antibody titers were compared before and after treatment. Results: This study included 159 (99 males, 60 females) children who had a serologic examination. Antineoplastic therapy had been given for acute leukemia (n=66), non-Hodgkin lymphoma (n=27), Hodgkin lymphoma (n=20), and solid tumors (n=46). Fifty-one patients had not been immunized against HBV prior to the therapy; HBV serology was negative in 49 of these patients and HBsAg was positive in 2 patients. Anti-HBs antibody positivity was present in 99 of 108 patients with an immunization history, whereas no vaccination response was present in 9 patients. The titer of anti-HBs antibody was decreased below the protection level in 33 (33%) patients with positive anti-HBs antibody, whereas the protection level was found to be maintained in 66 (67%) patients. The most significant decrease (63.6%) was observed in leukemia patients. Posttreatment HBsAg and HBV DNA positivity was detected in two of the patients with negative pretreatment serology, whereas no HBV infection developed in the group with positive anti-HBs antibody. Conclusions : This study demonstrated the importance of routine childhood vaccination in reducing the risk of HBV infection in patients with cancer.

α-Thalassemia and Hereditary Spherocytosis in the Same Patient: The Interaction of two Diseases

Paediatric Here we present a patient, affected by both a-thalassemia and heredi- tary spherocytosis and showing signs of severe hemolytic anemia, who is transfusion dependent. In this patient, the hemolytic effect of hereditary spherocytosis is not decreased by the effect of hemoglobin H disease (in which there is an increase in osmotic resistance), so a severe hypochromic normocytic anemia was seen. Also, it was observed that some changes may occur in the osmotic fragility test. The occurrence of more than one defect in same patient is not a rarity. For the first time, Cohen et al. (l) reported the concurrence of hereditary spherocytosis (HS), sickle cell trait, and thalassemia trait with no increase in hemoglobin (Hb) A2 level in a black American family ( 11. In the literature, there are only a few reports showing a combination of HS and P-thalassemia and their interactions (2-41. In the combination of HS and (3-thalassemia the clinical signs were milder due to antagonistic features of the two dis- eases; in HS there are a decreased sur€ace/volume ratio and increased hemoglobin concentration, whereas in thalassemia there are an increased surFace/volume ratio and decreased hemoglobin concentration ( 31. The

A Case of Severe Thrombocytopenia Due to Parvovirus B19 Virus

A 9-year-old girl admitted with generalized skin, mucosa, and genitourinary system bleeding had anemia and thrombocytopenia. Her bone marrow was normocellular. Parvovirus B19 IgM and IgG were positive. An absence of reticulocytopenia suggested that the virus affected only the megakaryocytic cell line and the anemia was due to generalized bleeding resulting from thrombocytopenia. Intravenous immunoglobulin treatment was instituted. On the tenth day of hospitalization the patient recovered from anemia and thrombocytopenia. IgM antibodies disappeared.

Acute Liver Failure, Autoimmune Hepatitis, and Leptospirosis: A Case Report

The etiology of acute liver failure varies widely in children, but the most common causes are viral hepatitis, drugs, and toxins. We report herein a case of autoimmune hepatitis and acute liver failure caused by leptospirosis, which is involved rarely in etiology.

Deferasirox in children with transfusion-dependent thalassemia or sickle cell anemia: a large cohort real-life experience from Turkey (REACH-THEM)

To evaluate the long‐term efficacy and safety of deferasirox therapy in a large observational cohort of children with transfusion‐dependent thalassemia (TDT) and sickle cell anemia (SCA) in Turkey.

Malignant Infantile Osteopetrosis: A Case Report

Malignant infantile osteopetrosis (MIOP) is a rare inherited genetic disease that is clinically and genetically heterogenic, characterized by increased bone density, and the symptoms may be seen in very early childhood. The increased bone density narrows the medullary cavity, resulting in extramedullary hematopoiesis, causing hepatosplenomegaly, anemia, and trombocytopenia. Sclerosing skeletal obstruction can cause cranial nerve compression and hearing and vision loss. It results in a tendency toward infections and a delay in growth and development. Therefore, especially the autosomal recessive forms show a fatal course. Along with the use of steroid, calcitriol, vitamin D, erythropoietin, and interferon gamma for the treatment, the definitive therapy is hematopoietic stem cell transplantation. In this case report, a female patient who we diagnosed with malignant ınfantile osteopetrosis after examination for 2 months due to hepatosplenomegaly, anemia, thrombocytopenia, and growth failure is presented. A 2-month-old female infant was admitted with complaints of vomiting and failure to gain weight. The physical examination revealed hepatomegaly, with the liver noted to be 4 cm, and splenomegaly, with the spleen noted to be 2-3 cm. The patient had a typical facial appearance with forehead protrusion, hypertelorism, and nystagmus. Her weight was below the 3rd percentile, her height was below the 3rd percentile, and the head circumference of the patient was in the 25th-50th percentile. A peripheral blood smear was observed with nucleated red cells, granulocytes precursors, and teardrop-shaped red blood (leukoerythroblastosis). Bone x-rays were normal, but pathognomonic osteopetrosis “glasses findings” were revealed after 10 days. All bone density increases due to excessive osteosclerosis at the distinction between the medulla and cortex were observed to disappear. Bone marrow aspiration showed hypocellular bone marrow. Bone marrow biopsy findings were diagnosed with osteopetrosis. After the case was diagnosed, a hematopoietic stem cell transplantation was made in a specialized center. Follow-up still continues. Malignant infantile osteopetrosis is a rare inherited genetic disease that is seen more than the known incidence in our country because of the high rates in-kin marriage. Even if the radiological findings do not start like how it was with our patient, if we are thinking about this sickness, we should repeat the radiological findings. Early diagnosis and treatment can prevent the harm of hearing and vision senses and the development of neurological damage of the patient. (JAREM 2014; 4: 121-4)