Erol Erduran

Breath holding spells in 91 children and response to treatment with iron

To evaluate the prognosis of breath holding spells (BHS) after iron treatment, 91 children (56 boys, 35 girls) aged between 6 months and 40 months (median, 17) were followed prospectively for a median of 45 months (range, 6–89). In 49 of the children, the frequency of BHS was less than 10 each month, in 22 it was 10–30 each month, and in 20 more than 30 each month. The spells were cyanotic in 60 children. All patients were evaluated initially and during follow up for haematological indices. Electroencephalographic and electrocardiographic abnormalities were also recorded. Sixty three patients were found to have iron deficiency anaemia and were treated with iron (6 mg/kg/day) for three months. Other patients were not given any treatment. After three months, there was a significant difference for correction of cyanotic spells between children who had been treated with iron and those who had not (84.1%v 21.4%). During further follow up, febrile convulsions occurred in 10 children (six were on iron treatment initially). It appears that treating iron deficiency anaemia is effective in reducing the frequency of BHS.

Apoptotic effects of heparin on lymphoblasts, neutrophils, and mononuclear cells: results of a preliminary in vitro study.

In this study the apoptotic effects of heparin on lymphoblasts, neutrophils, and mononuclear cells were evaluated by flow cytometry for detection of sub‐G1 peak, in vitro. Ten children with acute lymphoblastic leukemia (ALL) at diagnosis (Group I), six children with ALL at relapse (Group II), and 10 healthy children (controls) were included in this study. Lymphoblasts in ALL patients, and neutrophils and mononuclear cells in controls, were incubated in increasing heparin concentrations (0, 5, 10, 20 U/ml). Flow cytometric analyses were performed at 0, 1, and 2 hours of incubation in heparin for determination of the apoptotic effects of heparin. In Group I apoptosis was detected in all different levels of heparin concentration except 0 U/ml at 0, 1, and 2 hours. The apoptotic effects of heparin on blast cells peaked at the first hour in 5‐, 10‐, and 20‐U/ml heparin concentrations (p < 0.0001). In Group II similar findings were observed only at zero hour and apoptosis was higher than those in Group I except in 5‐U/ml heparin concentration (p < 0.001). Apoptosis was found to increase with heparin levels in both groups (p < 0.02). In the control group, apoptosis was detected only at the 20‐U/ml heparin concentration and only at the first and second hours. Lymphoblasts are more sensitive to apoptotic effects of heparin than either neutrophils and mononuclear cells (p < 0.004). It can be suggested that low‐dose heparin may cause significant apoptosis of lymphoblasts while inducing no apoptosis on neutrophils and mononuclear cells. The findings of this preliminary study indicate that further and more comprehensive research on the apoptotic effect of heparin on lymphoblasts should be done. Am. J. Hematol. 61:90–93, 1999.

The Treatment of Crimean-Congo Hemorrhagic Fever With High-dose Methylprednisolone, Intravenous Immunoglobulin, and Fresh Frozen Plasma

Crimean-Congo hemorrhagic fever (CCHF) is an acute tick-borne disease caused by Nairovirus, and it is sometimes characterized by reactive hemophagocytic histiocytosis (HLH). The reasons for reactive HLH are macrophage-activating syndrome and disseminated intravascular coagulation due to cytokine storm, liver dysfunction, and endothelial damage by the virus. In this study, the effectiveness of high-dose methylprednisolone (HDMP) (5 to 30 mg/kg/d), fresh frozen plasma (FFP), and intravenous immunoglobulin (IVIG) was investigated in patients with CCHF associated with reactive HLH. Twelve patients with CCHF in association with reactive HLH were included in the study. The patients were successfully treated with HDMP to suppress the macrophage activation, FFP to treat disseminated intravascular coagulation, and IVIG to treat severe thrombocytopenia. No patients received ribavirin. Fever reduced in 1.6±0.8 days, WBC count increased above 4.500/µL in 4.0±2.4 days, platelet count increased above 150.000/µL in 8.5±2.5 days, and D-dimer level decreased under 1 mcg/dL in 5.8±3.6 days. Consequently, HDMP, FFP, and IVIG may be effective in patients with CCHF associated with reactive HLH during hemorrhagic period of the disease.

Crimean-Congo haemorrhagic fever among children in north-eastern Turkey.

Abstract Aim: To analyse the epidemiological and clinical features of children with Crimean–Congo haemorrhagic fever (CCHF) in north-eastern Turkey. Methods: A retrospective study of demographic features and physical and laboratory findings in 21 children with CCHF is described. Clinical course, treatment modalities and outcome were analysed. Results: Most patients were admitted in June and July 2008; most were from the Gumushane and Kelkit valleys and half of them lived in rural areas. Mean (SD) age was 10.3 (3.9) years and the disease was more common in males (71.4%). Approximately 70% had a history of tick bite. The main symptoms were fever (17, 80.9%), nausea (11, 52.3%), malaise (10, 47.6%) and headache (7, 33.3%). At initial examination, approximately 70% of patients had leukopenia and 65% had thrombocytopenia. Anaemia developed during follow-up in six patients. Liver involvement was seen in 12 patients and one patient had acute tubular necrosis. Six patients had haemophagocytosis. Patients were hospitalised for a median 8 days (range 3–22) and nine patients had bleeding from various sites approximately 3–5 days after hospitalisation. Subcutaneous haematoma (6), especially epistaxis and at venepuncture sites (6) were the most common sites of bleeding. Pulmonary haemorrhage developed in two patients and they required ventilatory support. Overall mortality related to CCHF was 4.7% (one patient). Conclusion: Early diagnosis of CCHF and early referral to specialised centres are important for outcome. Exceptional epidemics may be seen in future owing to ecological and environmental changes.

High-dose methylprednisolone in children with Crimean-Congo haemorrhagic fever

Treatment options for Crimean-Congo haemorrhagic fever (CCHF) are limited and based on general supportive managements. Thrombocytopenia is the major risk factor of CCHF. We report our experience with high-dose methylprednisolone (HDMP). This study included five patients with CCHF. Patients were given HDMP if there were findings compatible with virus-associated haemophagocytic syndrome and the effects of HDMP were evaluated. Following this, HDMP fever subsided and platelet counts increased within 24 hours. Leukocyte counts began to increase and visceral bleedings were improved. HDMP treatment was discontinued within approximately five days. After HDMP, only one patient required blood products. HDMP is effective in CCHF, especially on fever and platelet counts. Dependency on blood products was decreased. Further controlled randomized studies with large series are needed in order to analyse the timing and duration of HDMP treatment and its effect on outcome.

Henoch-Schönlein purpura: a case with atypical presentation

We report on a case of Henoch-Schönlein purpura (HSP) with pulmonary hemorrhage and severe renal involvement. The patient also had active carditis related to acute rheumatic fever. He died despite intensive treatment. Regarding this case, we discuss the pathogenesis and clinical findings of pulmonary hemorrhage and active carditis in HSP.

Plasma Soluble Interleukin-2 Receptor Levels in Patients With Idiopathic Thrombocytopenic Purpura

Soluble interleukin‐2 receptor (sIL‐2R) was measured in the plasma of 31 patients with idiopathic thrombocytopenic purpura (ITP) and 22 normal controls. When thrombocytopenia persisted longer than 6 months, the diagnosis of chronic ITP was made. Twenty patients had acute ITP, 11 patients had chronic ITP, and all patients received high‐dose methylprednisolone (HDMP) (30 mg/kg/d for 3 days, 20 mg/kg/d for 4 days). The sIL‐2R levels of the patients were determined before being giving HDMP and 14 days after the end of HDMP therapy. Platelet counts were determined before administration of HDMP, one day after the end of HDMP therapy, and once every 28 days for 7 months thereafter.

Pattern of pediatric poisoning in the east Karadeniz region between 2002 and 2006: increased suicide poisoning.

In the present study, 386 patients with the diagnosis of poisoning admitted to the Pediatric Emergency Unit of Farabi Hospital of Medical Faculty of Karadeniz Technical University between January 2002 and December 2006 were retrospectively evaluated with respect to gender, age, cause of poisoning, type of substance used, route of exposure, reason for the intake, signs and symptoms, time of referral to the hospital, hospitalization period, and prognosis. The age group of most poisoning cases was <5 years of age and constituted 51% (n = 197) of all cases. The main toxic agent was drugs (70.2%), followed by foods (8.8%), rodenticides (7%), insecticides/pesticides (4.9%), and carbon monoxide (4.7%). In childhood poisonings, accidental drug poisoning was frequent in toddlers, whereas suicidal poisoning was frequent in adolescents. The suicidal poisoning rate was 23.8% among all poisoning patients, and 98.9% of these patients were adolescents. The suicidal poisoning rates for males and females were 30% and 70%, respectively. An increase in suicidal and inhalation poisonings was observed when compared with previous studies that have been conducted in the same region. The results of the present study suggest that poisonings still represents an important health problem that could be prevented by safe drug storage at home, as well as parental education on adolescence issues, particularly those regarding females.

Treatment of dyskeratosis congenita with granulocyte-macrophage colony-stimulating factor and erythropoietin.

Dyskeratosis congenita (DC) is a rare inherited disorder characterized by reticulate skin pigmentation, nail dystrophy, mucosal leucoplakia, and bone marrow failure. Pancytopenia is difficult to manage in patients with this disorder. We describe a 13-month-old-boy who presented with reticulate skin lesions, paleness, and hepatosplenomegaly. Anemia and leukopenia developed by the age of 43 months. The patient was treated with granulocyte-macrophage colony-stimulating factor (GM-CSF) (5 microg/kg/d, subcutaneously) for 19 months and erythropoietin (150 U/kg 3 days in a week, subcutaneously) for 8 months, with excellent neutrophil and hemoglobin response. Recurrent infections were not developed after starting GM-CSF, and packed red blood cell transfusion was not given to the patient after starting erythropoietin. GM-CSF combined with erythropoietin may be used in the treatment of bone marrow failure in patients with DC without an HLA-identical donor.

Hemophagocytic syndrome as an initial presentation of miliary tuberculosis without pulmonary findings.

A 9-y-old girl was admitted with fever, weakness and weight loss. She had pancytopenia in peripheral blood, hypocellularity and hemophagocytosis in bone marrow. Disseminated tuberculosis was diagnosed after a long delay, with involvement of the lungs, bone marrow, liver, spleen and central nervous system. Tuberculosis can be a cause of hemophagocytosis and should be taken into account in the differential diagnosis of fever of unknown origin associated with pancytopenia and hemophagocytosis.