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

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.

Serum carnitine levels during the doxorubicin therapy. Its role in cardiotoxicity.

Doxorubicin is an anthracycline antibiotic with a broad spectrum of antineoplastic activity. Cardiotoxicity is a serious long-term complication of the drug. Simultaneous administration of carnitine has been proposed to prevent cardiotoxicity. We aimed to monitor the serum carnitine levels during the treatment of doxorubicin and to determine a relationship between serum carnitine levels and cardiac dysfunction. Fifteen patients were evaluated prospectively. Measurement of carnitine levels and evaluation of cardiac function were performed prior to treatment, and after cumulative doses of 180 and 300 mg/m2 of doxorubicin. A group of 20 healthy children served as control group to obtain reference values. We found subclinical abnormalities in cardiac function, while the cumulative doses of the doxorubicin was increasing. The mean end diastolic and end systolic left ventricular dimensions of the patient group after completion of the treatment were significantly increased compared with initial values. The ejection and shortening fraction of the patient group after cumulative doses of 300 mg/m2 of doxorubicin were significantly lower than those of the control group. A statistically significant augmentation was observed in mitral A, with a decrease in mitral E/A ratio. There was a trend towards lower serum carnitine levels with higher cumulative doses of doxorubicin, although it was not statistically significant. Our results invite new detailed investigations depending on the measurement of serum and urinary free and acyl carnitine and myocardial carnitine levels to evaluate possible roles of carnitine in the prevention of doxorubicin-induced cardiotoxicity.

Cranial Doppler ultrasonography as a predictor of neurologic sequelae in infants with bacterial meningitis.

OBJECTIVE: To perform transcranial Doppler sonography in newborns and infants with bacterial meningitis to evaluate predictive value of neurologic sequelae. MATERIALS AND METHODS: Thirty-three patients (15 newborn, 18 infant patients) with bacterial meningitis underwent cranial Doppler ultrasonography during acute phase and 3rd and 6th months after disease. Patients were examined regularly for neurologic outcome with electroencephalography and magnetic resonance imaging. The age-matched control group consisted of 20 healthy children underwent cranial Doppler sonography only. RESULTS: To compare with the healthy controls, the mean blood flow velocity was significantly increased (P < 0.001), and pulsatility index was higher than those control group (P < 0.05) during acute bacterial meningitis. None of the patients were diagnosed with stenosis of cerebral artery. According to neurologic outcome, 14 of 33 patients had neurologic sequelae. The mean cerebral blood flow was significantly higher (P < 0.01) in patients without neurologic sequelae; pulsatility index was significantly higher (P < 0.05) in patients with neurologic sequelae when compared with the healthy controls. There was no significant difference between mean cerebral blood flow velocities and mean pulsatility index values of newborn and infant patients, regarding to neurologic outcome (P < 0.05). CONCLUSION: Cranial Doppler ultrasonography is useful for prediction of neurologic sequelae in infants with bacterial meningitis.

The utility of (99m)Tc-DMSA and Tc(99m)-EC scintigraphy for early diagnosis of ifosfamide induced nephrotoxicity.

A serious undesired effect of certain cytostatics is their nephrotoxicity. In this study, we investigated the toxic effects of ifosfamide and cisplatin by clinical and biochemical parameters in relation to (99m)Tc-dimercaptosuccinic acid ((99m)Tc-DMSA) and Tc(99m)N, N-ethylenedicysteine (EC) renal scintigraphy. The indicators were urinary beta2-microglobulin levels, tubular resorption of phosphate, urinary protein and glucose excretion, glomerular filtration rate, urinary pH and osmolarity. Thirteen paediatric patients (seven boys and six girls), aged 2-16 years, were investigated. Five patients received only cisplatin, six patients were treated with ifosfamide and cisplatin and two with ifosfamide and carboplatin for various malignancies. All except three patients had normal DMSA uptake (median, 19; range, 16-29%) prior to chemotherapy. The reduction in DMSA uptake was unilateral due to tumour invasion in those three patients. Following chemotherapy, DMSA uptake showed reduction in five patients with or without clinical nephrotoxicity. The observed pattern was decreased renal uptake and elevated bladder activity. Three patients with decreased DMSA uptake had normal tubular maximum phosphate reabsorption, which suggested subclinical injury. Decrease in DMSA uptake and tubular phosphate reabsorption (TPR) was detected simultaneously in two patients. No abnormalities were seen on Tc(99m)-EC scintigraphy to suggest nephrotoxicity in our investigation. However, Tc(99m)-EC clearly demonstrated a reduction in split renal function in children with tumour invasion. In summary, we found that ifosfamide induced tubular injury can be detected with (99m)Tc-DMSA scintigraphy before chemotherapy associated nephrotoxicity is observed by laboratory measurements. Our results also imply that, although a tubular agent, renal scintigraphy performed with Tc(99m)-EC is not able to detect subclinical injury or predict the outcome during treatment.

Expression of adhesion molecules LFA-1, ICAM-1, CD44, and L-selectin in childhood non-Hodgkin lymphomas.

BACKGROUND: The aim of the present study was to analyse the expression of adhesion molecules in childhood non-Hodgkin lymphomas and to correlate the findings with clinical features and prognosis. PROCEDURE: Samples were obtained from pleural and peritoneal fluids, bone marrow aspirates, and tissue biopsies from 21 patients (median age: 8 years). There were 9 T-cell and 12 B-cell lymphomas. The expression of CD18, CD44s, CD54, CD62L were investigated with flow cytometry by using monoclonal antibodies. RESULTS: Absence of CD18, which was independent from immunophenotype, was found in 67% of patients. Positive CD44s and CD62L expression were observed in 48 and 63% of the cases, respectively. In all of the cases with T-cell lymphoma, CD54 was negative, whereas 8 of 12 cases with B-cell lymphoma expressed this molecule (P = 0.005). There was no correlation between location of disease and the expression of adhesion molecules, except CD54 that was negative in all mediasten lymphoma (P = 0.004). CD62L (+) patients had more frequently stage IV disease than CD62L (-) ones (P = 0.01). Two-year overall survival was 83 and 29% in CD18 (+) and CD18 (-) cases; 55 and 36% in CD44s (+) and CD44s (-) cases; 46 and 42% in CD54 (+) and CD54 (-) cases; 42 and 50% in CD62L (+) and CD62L (-) cases. CONCLUSIONS: The expression of LFA-1 on lymphoblasts is lost in the majority of childhood non-Hodgkin lymphomas. ICAM-1 is not detected on neoplastic cells of patients with T-cell lymphoblastic lymphoma. L-selectin positivity correlates with disseminated disease. There is no significant relationship between the expression of adhesion molecules and the survival rates, although CD18(+) cases had better overall survival rate than CD18(-) cases.

Management of cutaneous hemangiomas: a retrospective analysis of 1109 cases and comparison of conventional dose prednisolone with high-dose methylprednisolone therapy.

The effectiveness of the different pharmacological agents and different doses of systemic corticosteroids was analyzed. A total of 1109 patients (median age 8 months; F/M: 2.3) with hemangioma, followed up in our unit for 23 years, were evaluated retrospectively. Forty-five of them received systemic corticosteroids. Two different pharmacological agents, prednisolone (in 26 patients) and methyl prednisolone (in 19 patients), had been used in three different regimens. Groups were compared according to the final results and rebound regrowth. Response was considered good or excellent in 16 patients (36%). Then were no differences in response to therapy among the three regimens. No difference was found in response to therapy between prednisolone and methylprednisolone and the two different doses of the methylprednisolone. Rebound regrowth was significantly higher in methyl prednisolone than in the prednisolone group (p = .045). In multivariate analysis the dimension of the lesion (p = .0065) and age at initiation of treatment (p = .0041) were the most important factors affecting the response. In conclusion, the systemic corticosteroids are effective in 36% of patients, independent of dosage and pharmacological agents and duration of the therapy. The dimension of the lesion and age at initiation of treatment are the most important factors affecting the response to treatment.

Nasal-paranasal-oronasopharyngeal lymphomas in childhood: the role of staging system on prognosis.

Most of the patients with nasal-paranasal and oropharyngeal-nasopharyngeal (NPONP) lymphomas had early-stage disease according to the Murphy system. But the survival rates are not correlated with the stages. Treatment results were analyzed to see the effects of the staging in NPONP lymphomas. Fifty-five children (median age 8 years, M/F: 4.5) with NPONP lymphoma were included in this study. The Murphy staging system was used at diagnosis and all cases were restaged according to the TNM system: primary tumor, regional lymph node, and metastasis. The survival rates were analyzed by grouping the patients according to the treatment and stages. The disease was located in Waldeyer's ring, the sinonasal region, and the nasopharynx in 45.4, 27.3, and 27.3% of patients, respectively. Thirty-nine patients had stage I or II disease according to the Murphy system. When the TNM system was used, 92% of these patients were upstaged to stage III-IV. Five-year overall and event-free survival rates were 52.1 and 50.4%, respectively, for the whole group. Five-year event-free survival rates for Murphy stage I, II, and III disease were 66.7, 56.9, and 45.4%, respectively. The rates for TNM stage III and IV patients were 64.3 and 43.8%. Treatment protocols were intensified in most of the early-stage disease treated with modified LSA2-L2 regimen and better survival rates were obtained in these patients. The intensification of the treatment by using intrathecal treatment and doxorubicin in patients with early-stage disease at NPONP location seems effective. In conclusion, the Murphy staging system is not suitable for the staging of NPONP lymphomas. It should be revised to predict the prognosis and decision-making for treatment.

Veno-occlusive disease-like hepatotoxicity in two children receiving chemotherapy for Wilms' tumor and clear cell sarcoma of kidney.

We report two pediatric cases who developed veno-occlusive disease-like hepatotoxicity while receiving chemotherapy for Wilms' tumor and clear cell sarcoma of kidney. The chemotherapeutics, including vincristine, actinomycin D, and epirubicin in case 1 and vincristine and actinomycin D in case 2, were given before the hepatotoxicity developed. Other possible causes of hepatotoxicity were excluded. Recovery was observed with supportive therapy after 2 and 1 weeks, respectively. After recovery, the children tolerated continued chemotherapy without any decrease in the doses of drugs. We conclude that vincristine and actinomycin D were the cause of this rare from of hepatotoxicity and that chemotherapy for the underlying malignant disease could be given safely after clinical recovery.

Benign vascular tumors and vascular malformations in childhood: a retrospective analysis of 1127 cases.

Vascular lesions in childhood are classified as vascular malformations and hemangiomas. Vascular malformations are congenital abnormalities thought to arise from defects during embryological development of vascular tissue. Hemangiomas are benign tumors of vascular endothelium and can spontaneously become involuted in almost all cases. One thousand one hundred and twenty-seven patients with vascular lesions were followed by the Department of Pediatric Oncology, Hacettepe University, for 19 years. Diagnosis was based mainly on history, clinical condition and follow-up data in 98.2 percent of cases. The distribution of the vascular lesions was as follows: 969 patients had hemangiomas, 120 had lymphatic malformations, 18 had combined vascular malformations, 11 had venous malformations, six had port-wine stain, and three had angiokeratoma. Except for ten cases with Klippel-Trenaunay Weber syndrome, vascular malformations were not accompanied by any syndrome.