Münevver Büyükpamukçu

Evaluation of Coronary Artery Disease by Computed Tomography Angiography in Patients Treated for Childhood Hodgkin's Lymphoma

PURPOSE To detect pathologies in coronary arteries by computed tomography angiography (CTA) in survivors of childhood Hodgkins lymphoma who have been treated with radiotherapy and/or cardiotoxic agents. PATIENTS AND METHODS Patients with HL who have been in remission for at least 2 years after completion of therapy were included. CBC, lipid profile, urine analysis, brain natriuretic peptide, troponin-T, creatinine kinase-myocardial band, ECG, telecardiography, echocardiography, and CTA of the patients were performed. Cardiac vascular abnormalities were noted. Results A total of 119 patients were included in the study. In 19 patients (16%), we found coronary artery abnormalities. There was a significant difference between the patients who received mediastinal radiotherapy and those who did not (P = .02). By multivariate analysis, in patients receiving mediastinal radiotherapy the risk of developing a coronary artery abnormality was found to increase 6.8 times compared with patients who did not receive mediastinal radiotherapy (P = .009). Stent implantation was performed in a 28-year-old patient because of critical stenosis in right coronary. In two patients some irregularities were detected both in CTA and conventional angiography, and they remained in close follow-up. A 22-year-old patient whose CTA showed critical stenosis in his left anterior descending artery refused the conventional angiography. CONCLUSION To our knowledge, this is the first large study using CTA for detection of coronary abnormalities in patients treated for HL in pediatric age group. Coronary CTA is a minimally invasive tool for early diagnosis of coronary artery disease in patients who were treated with mediastinal radiotherapy and/or cardiotoxic chemotherapy.

Role of procalcitonin and CRP in differentiating a stable from a deteriorating clinical course in pediatric febrile neutropenia.

In clinical practice, when neutropenic-fever patients present with no microbiologically and clinically defined infection, the risk of underestimating an occult infection is of major concern, the clinicians have to make a decision on when to modify antibiotic therapy. Hence, a reliable, specific, and sensitive marker, which is regulated independently from the leukocyte count and the underlying disease, is needed for the early diagnosis of infections in cases of neutropenic fever. We have evaluated the diagnostic and follow-up value of procalcitonin (PCT) compared with C-reactive protein (CRP) and erythrocyte sedimentation rate in documenting the infection in neutropenic-fever patients undergoing intensive chemotherapy, as evidenced by the durational change in these parameters in the presence of defined infection. Forty-nine patients, who had 60 febrile episodes, and who were hospitalized in the Hacettepe University Ihsan Doğramacı Childrens Hospital between January 1, 2004 and January 1, 2005 were included in this prospective study. All patients had been diagnosed with neutropenic fever after intensive chemotherapy. In our study, PCT and CRP levels were significantly higher in neutropenic-fever patients (group I and group II separately) than in control patients (P<0.001) throughout the study period; but erythrocyte sedimentation rate levels did not show any significant difference (P>0.05). In sequential analyses of patients without documented infections, the median of PCT concentrations shows a tendency to fall after the 8th hour of onset of fever, whereas in patients with documented infections PCT concentrations fell after the 48th hour. In conclusion, our study suggests that PCT, when measured periodically, is a more useful diagnostic inflammation parameter in pediatric neutropenic-fever patients than CRP, both in estimating the severity of the infection and, the duration and origin of the fever. Hence, PCT might be helpful when deciding on initial therapy modification.

Treatment results of 84 patients with nasopharyngeal carcinoma in childhood

To evaluate the clinical characteristics, treatment regimens, survival, and late complications in patients with nasopharyngeal carcinoma.

Intracranial Ependymomas in Childhood: A Retrospective Review of Sixty-two Children

Of the 818 tumours of the central nervous system diagnosed between 1972 and 1991, 62 patients (35 males and 27 females) with histopathologically confirmed ependymomas were treated and followed-up at the Childrens Hospital of Hacettepe University during that period. The median age was 6 years (range 1-17 years). Headache, nausea and vomiting were the most frequent symptoms; papilledema was the most common sign in our patients. Tumour sites were in the posterior fossa in 47 patients and supratentorial in 15 patients. All patients underwent surgery. Gross- total resection was performed in 27 patients, subtotal resection in 32 patients and biopsy in the remaining 3 patients. Initially, 53 patients were given postoperative radiotherapy. Four patients did not receive radiotherapy because of their young age, whereas five patients died prior to starting radiotherapy. Two slightly different types of chemotherapy protocols were applied for an average of one year in 47 patients. Event-free and overall survival rates at 10 years were 36% and 50%, respectively. Twenty children suffered relapse 4 to 55 months after diagnosis (median 16 months). Relapses were distant in 3 cases and local in 17. Age was the only statistically significant prognostic factor, patients younger than 5 years of age having a poorer outcome. Sex, histopathologic type, localization of the tumour, extent of surgery, and chemotherapy did not influence the prognosis in our study. Because the majority of recurrences were local, better local tumour control is required. New treatment strategies should be developed in order to improve local control.

Childhood Cancer in Developing Countries

The problem of childhood cancer cannot be evaluated separately from developmental levels and states of health of the countries. Rapid increase in population, poverty, poor hygiene, lack of education, and multitude health problems impede the development of pediatric oncology and success of the management of childhood cancer in developing countries. More than 85% pediatric cancer cases occur in developing countries that use less than 5% of world resources. The rate will exceed 90% in the next two decades, due to the increase of youth population in favor of developing countries. Incidence rates, pathology, and clinical characteristics of various cancers seem different in developing and developed countries. Different environments, life styles, dietary habits, and hygienic conditions are the main reasons for those differences. Unprecedented changes in diagnostic techniques, treatment methods and supportive care have occurred during the last decades. Consequently, management has improved and the mortality rates have decreased. Most of the children with cancer living in developing countries could not profit from those advances in pediatric oncology because of the cost.

Second neoplasms in pediatric patients treated for cancer: a center's 30-year experience.

To investigate the incidence and outcome of secondary neoplasms in pediatric patients treated for childhood cancer. Between December 1971 and January 2000, a total of 5859 patients younger than age 17 were diagnosed and treated for childhood cancers in our center. Of this group, 1511 (36%) patients were followed for more than 36 months. These long-term survivors were included in this analysis. Twenty-six patients developed a secondary malignancy with an overall risk of 1.7% in this cohort. The male:female ratio was 17:10, with a median age of 7.66 at diagnosis (range, 2 to 16 y). Four patients (14.8%) with Hodgkin lymphoma; 3 each (11.1%) with retinoblastoma and rhabdomyosarcoma; 2 each (7.4%) with Wilms tumor, Ewing sarcoma, medulloblastoma, ganglioneuroblastoma, and non-Hodgkin lymphoma; and 1 each (3.7%) with ependymoma, nasopharyngeal carcinoma, osteosarcoma, astrocytoma had a secondary malignant disease during the long-term follow-up period. Secondary malignant diseases were osteosarcoma in 6 patients, acute lymphoblastic leukemia in 2, acute myelogenous leukemia in 2, and rare malignant disease in others. Four patients with osteosarcoma developed disease within the radiation field. Osteosarcoma was the most frequently occurring secondary neoplasm. Less toxic treatment modalities should be used to decrease the risk of secondary malignant diseases.

Infantile hepatic hemangioendothelioma with elevated serum alpha-fetoprotein.

Infantile hemangioendothelioma is the most common hepatic vascular tumor in infants less than 6 months of age, with a prevalence of 1%. Serum α-fetoprotein levels have been used as an important tumor marker for hepatoblastoma, hepatocellular carcinoma, and germ cell tumors. It is rarely elevated in hepatic hemangioendothelioma. The authors report an infant with a hepatic hemangioendothelioma associated with elevation of serum α-fetoprotein who was treated with corticosteroids. In young infants, a solitary hepatic mass and elevated serum AFP level may not always be associated with hepatoblastoma. Infantile hemangioendothelioma must be differentiated by MRI or other radiological techniques before performing invasive procedures.

Childhood cancer and hypercalcemia: Report of a case treated with pamidronate

Hypercalcemia in a 4-year-old boy with non-Hodgkin lymphoma treated with pamidronate is presented. The child had relapsed disease with bone metastasis. Hypercalcemia is rare in children, and bisphosphonates are relatively new agents for the treatment of hypercalcemia. Information concerning their use in the treatment of hypercalcemia in childhood is limited. We found that pamidronate is effective and has no significant side effects in a child.

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 cortico steroids 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%). There 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.

Langerhans cell histiocytosis: retrospective analysis of 217 cases in a single center.

Langerhans cell histiocytosis (LCH) is a disorder with unclear etiology and pathogenesis, which is characterized by abnormal clonal proliferation and accumulation of langerhans cells at various tissue and organs. A total of 217 patients with LCH were evaluated retrospectively for clinicopathological features, laboratory findings, treatment modalities, long-term outcome, and factors affecting the outcome. Median age at the time of diagnosis was 3.5 years and male/female ratio was 1.8. The most common complaint at presentation was a bone lesion-related symptom. Fifty percent of the patients younger than 2 years had organ dysfunction (OD). Treatment consisted of surgery, chemotherapy, and radiotherapy alone or in combination. Vinblastine with or without prednisolone was the most common used chemotherapy regimen. Overall (OS) and event-free survival (EFS) rates were 84% and 51.5%, respectively, at an 8-year median follow-up time. Overall survival was significantly lower in patients younger than 2 years of age and patients with OD. The age at diagnosis, pulmonary, liver, or hematological involvement, and elevated acute-phase reactants were found to have a statistically significant effect on the OS or EFS rates.