The evaluation of malignancies in Turkish primary immunodeficiency patients; a multicenter study.

BACKGROUND: There are no data regarding the prevalence of malignancies in patients with primary immunodeficiency (PID) in Turkey. Along with the prevalence of malignancy, we aimed to present the types of malignancy and define the underlying immune deficiency of the patients. METHOD: Between the years 1992 and 2018, from five tertiary immunology clinics, fifty-nine patients with PID who developed malignancy were included. All patients were evaluated for demographics, clinical features, and prognosis. RESULTS: The prevalence of malignancy in our cohort was detected as 0.9% (59/6392). The male-to-female ratio was 1.8 (38/21), and the median age of patients was 14 years (range: 1.5-51). The median age at diagnosis of malignancy was 10 years (range: 1.5-51). Ataxia-telangiectasia was the most frequent PID in patients with malignancy (n = 19, 32.2%), and non-Hodgkin lymphoma was the most common malignancy (n = 32, 51.6%). The rate of malignancy in DOCK8 deficiency (n = 7/43, 16.3%) was higher than AT (n = 19/193, 9.8%), Wiskott-Aldrich syndrome (n = 2/22, 9.1%), and common variable immunodeficiency (n = 11/205, 5.4%). EBV quantitative PCR was positive in 16 out of 53 patients (30.2%). Three patients had secondary malignancies. Remission was achieved in 26 patients (44.1%). However, 31 patients (52.5%) died. Two patients (3.4%) are still on chemotherapy. CONCLUSION: This study is the largest cohort investigating the association of malignancy in patients with PID in Turkey. While lymphoid malignancies were the most common malignancy and observed more frequently in AT patients, the risk for malignancy was higher in patients with DOCK8 deficiency compared to AT.

[Evaluation of Micafungin Use in Children]. / Çocuklarda Mikafungin Kullaniminin Degerlendirilmesi.

Micafungin is recommended especially in patients with liver and kidney failure and in the presence of other side effects due to antifungals apart from its known priority indications such as invasive candidiasis. The aim of this study was to evaluate the children who have received micafungin treatment. In the study, 125 children who were hospitalized in the pediatric wards and intensive care units of our hospital and had used micafungin between November 2016 and January 2019 were analyzed retrospectively. Clinical data, micafungin indication, blood values on the first and fourth days of the treatment, side effects of the drug and efficacy were evaluated. Sixty percent (75/125) of the patients were male and the mean age of all the patients were 58 ± 67 (0-215, 30) months. Approximately half of the cases (48%) had malignancy and 13% of them were premature. Sixty-two percent (n= 37) of the malignencies were hematological (27 acute lymphocytic leukemia, nine acute myeloid leukemia, one myelodysplastic syndrome) and 38% (n= 23) were oncological (six neuroblastoma, four Hodgkin lymphoma, two Non-Hodgkin's lymphoma, five sarcomas, one hepatoblastoma, five others) malignencies. The major cause of hospitalization was sepsis (53%). The patients had several risk factors like immunosuppressive therapy (n= 68, 54%), neutropenia (n= 61, 49%), central venous catheter (n= 102, 82%), nasogastric tube (n= 63, 50%), endotracheal intubation tube (n= 49, 39%), urinary catheter (n= 14, 11%) and total parenteral nutrition (n= 81, 65%). Thirteen percent (n= 16) of the cases were post-operative patients. Candida species were cultivated in 97 clinical specimens (blood, endotracheal aspirate, sputum, urine, etc.) among 23 (18%) of the patients. Thirteen (10%) of the patients had candidemia and 62% of them were non-albicans strains. In all candidemias, strains were echinocandin susceptible, and blood cultures were negative within four days. When all the patients (n= 125) were evaluated, a significant decrease in C-reactive protein, an increase in sodium, and a decrease in alanine aminotransferase were observed on the fourth day of micafungin treatment (p<0.05). A total of 39 (31%) patients underwent various antifungal treatments for median seven (1-60) days prior to micafungin treatment. Fourteen (36%) of these 39 patients, had elevated liver function tests (LFT), 10 (26%) of them had hypokalemia, and five (13%) of them had elevated renal function tests. Ten (26%) patients had antifungal-induced hypokalemia previously; and potassium levels were normalized after micafungin treatment (p= 0.0001). The patients for which micafungin treatment was chosen due to elevated liver function tests (n= 47, 38%), whether the antifungalinduced or not; alanine aminotransferase and aspartate aminotransferase levels were decreased after micafungin treatment (p= 0.0001 and p= 0.0001, respectively). Nineteen (15%) of the patients have died within the first 30 days of micafungin treatment and one of them had candidemia. No micafungin treatment related significant side effects were observed in any of the patients. Our study showed that micafungin could be a safe and effective option in pediatric cases including newborns with high liver and kidney function tests.

Late effects in patients with sacrococcygeal teratoma: A single center series.

INTRODUCTION: The aim of this study is to evaluate late side effects that affect quality of life in children with sacrococcygeal teratoma (SCT). PATIENTS AND METHODS: The patients with SCT were evaluated retrospectively. The data were expressed by percentage and the subgroups were compared statistically. RESULTS: A total of 40 children with SCT were identified with median age 12 days (range: 1 day-14.6 years), 27 of whom were analyzed in this study with urodynamic data available for 24 and anal manometric evaluations for 20. Chronic constipation with need for laxative was reported in (7/27) 25.9%, fecal incontinence was present in (1/27) 3.7%, and urodynamic abnormalities were reported in (16/24) 66%. Among those with urodynamic abnormalities, low bladder capacity, dyssyergia and neurogenic bladder were observed in (21/24) 87.5% and anticholinergic treatment was applied. Urinary incontinence was present in (2/27) 7%, with clean intermittent catheterization utilized in (7/27) 25.9%. While defecation was observed more in the patients with Altman types II, III, and IV, micturation problems were observed more in the patients with Altman types II and IV. It was found that urodynamic dysfunctions were more frequent in the patients with increased number of operations. DISCUSSION: Although the rate of symptomatic patients was low, abnormalities determined by radiological and urodynamic evaluations were high.

A prospective follow-up of quality of life, depression, and anxiety in children with lymphoma and solid tumors

Background/aim: The aim of this study was the determination and prospective follow-up of quality of life, depression, and anxiety in pediatric patients with cancer under chemotherapy, as well as the evaluation of related factors. Materials and methods: Fifty newly diagnosed pediatric cancer patients and their parents were prospectively monitored before, during, and after therapy, and tests were used. Results: Significantly lower quality of life scores were recorded during treatment, in the group with CNS tumors, in the group receiving chemotherapy plus radiotherapy plus surgery, in the inpatient-only treatment group, in the group receiving treatment for longer than 6 months, and in the group of patients whose diagnosis was delayed for more than 3 months. Total quality of life scores for children and their parents were 82.95 ± 14.59 vs. 83.61 ± 14.60 before, 54.69 ± 16.51 vs. 55.78 ± 16.05 during, and 83.88 ± 12.44 vs. 84.19 ± 13.22 at the end of treatment (P < 0.05). Anxiety and depression scores were significantly higher during treatment, in patients whose diagnoses were delayed for more than 3 months, and among inpatients. Conclusion: The quality of life of a majority of our patients was severely affected, and depression and anxiety were more frequently seen especially during treatment.

Leptomeningeal dissemination and vertebral bone involvement in a child with pilocytic astrocytoma.

In low-grade glioma, metastasis is rarely seen. Few cases of leptomeningeal dissemination have been reported in children. Vertebral bone metastasis has not been reported so far. Herein is described the case of a pediatric patient with the diagnosis of pilocytic astrocytoma, and leptomeningeal dissemination detected at the time of diagnosis, who then received radiotherapy and chemotherapy upon development of vertebral bone metastasis during treatment.

Adrenomedullin--A New Marker in Febrile Neutropenia: Comparison With CRP and Procalcitonin.

In this study, we aimed to determine serum adrenomedullin levels and compare them with levels of C-reactive protein (CRP) and procalcitonin (PCT). Cancer patients aged 0-18 years who experienced febrile neutropenia attacks were included in the study. Adrenomedullin, CRP, and PCT were analyzed at admission, day 3, and days 7-10 later. Fifty episodes of febrile neutropenia that developed in 37 patients were analyzed in this study. The mean age of the patients was 7.5 ± 4.7 (1-18) years. The patients had leukemia (73%), solid tumors (19%), and lymphoma (8%). The percentages of the patients in the clinically documented infection (CDI), fever of unknown origin (FUO), sepsis, and microbiological documented infection (MDI) categories were 34%, 34%, 20%, and 12%, respectively. During the study period, four patients were lost. In the MDI group, adrenomedullin levels on day 3 were significantly higher than those in the CDI and FUO groups. PCT levels were significantly higher in the sepsis group than those in the CDI group at admission, day 3, and days 7-10. In the sepsis group, PCT levels on days 7-10 days were significantly higher than those in the sepsis group. PCT values from the deceased patients on days 7-10 were significantly higher than those from patients who survived. CRP levels did not differ significantly among the febrile neutropenia groups. First, in our study, adrenomedullin was used as a biomarker in the febrile neutropenia episodes of children with cancer. Among adrenomedullin, CRP, and PCT, procalcitonin demonstrates the highest correlation with the severity of infection.

Randomized comparison of piperacillin-tazobactam plus amikacin versus cefoperazone-sulbactam plus amikacin for management of febrile neutropenia in children with lymphoma and solid tumors.

The objective of this study was to compare the effectiveness of piperacillin-tazobactam (PIP/TAZO) plus amikacin (AMK) (PIP/TAZO+AMK) versus cefoperazone-sulbactam (CS) plus AMK (CS+AMK) for the treatment of febrile neutropenia (FN) in children with cancer. The study was designed prospectively and randomized in 0- to 18-year-old children with lymphoma or solid tumor who were hospitalized with FN diagnosis. Consecutively randomized patients received either PIP/TAZO 360 mg/kg/day in 4 doses plus AMK 15 mg/kg/day in 3 doses or CS 100 mg/kg/day in 3 doses plus AMK 15 mg/kg/day in 3 doses intravenously. Treatment modification was defined as any change in the initial empirical antibiotic therapy. A total of 116 FN episodes were managed in 46 patients (26 boys and 20 girls) with a median age of 6.5 years (range .8-17.0) during the study period. Success rates without modification of therapy were 47.5% and 52.6% in PIP/TAZO+AMK group and CS+AMK group, respectively (P >.05). No statistical difference was found between treatment groups in terms of durations of neutropenia, fever, and hospitalization. The overall success rate in all groups was 97.4%. No major side effect was observed in either group during the course of the study. Our study is the first to compare the effectiveness of PIP/TAZO+AMK and CS+AMK therapies. Both combinations were effective and safe as empirical therapy for febrile neutropenic patients.

Catheter-associated bloodstream infections in pediatric hematology-oncology patients.

Catheter-associated bloodstream infections (CABSIs) are common complications encountered with cancer treatment. The aims of this study were to analyze the factors associated with recurrent infection and catheter removal in pediatric hematology-oncology patients. All cases of CABSIs in patients attending the Department of Pediatric Hematology-Oncology between January 2008 and December 2010 were reviewed. A total of 44 episodes of CABSIs, including multiple episodes involving the same catheter, were identified in 31 children with cancer. The overall CABSIs rate was 7.4 infections per 1000 central venous catheter (CVC) days. The most frequent organism isolated was coagulase-negative Staphylococcus (CONS). The CVC was removed in nine (20.4%) episodes. We found that hypotension, persistent bacteremia, Candida infection, exit-side infection, neutropenia, and prolonged duration of neutropenia were the factors for catheter removal. There were 23 (52.2%) episodes of recurrence or reinfection. Mortality rate was found to be 9.6% in children with CABSIs. In this study, we found that CABSIs rate was 7.4 infections per 1000 catheter-days. CABSIs rates in our hematology-oncology patients are comparable to prior reports. Because CONS is the most common isolated microorganism in CABSIs, vancomycin can be considered part of the initial empirical regimen.

Surgical Management of Brain Tumors in Infants Under One Year of Age.

AIM: To comprehensively analyze clinical presentation, diagnosis, and management approaches of brain tumors in infants under one year of age. MATERIAL AND METHODS: We conducted a retrospective analysis of clinical data retrieved from medical records of infants who underwent surgical treatment for intracranial mass lesions at our institution from January 2006 to December 2016. The data encompassed parameters such as age at diagnosis, symptoms, tumor location, histology, surgical procedures, adjuvant treatment, and survival outcomes. Cases involving dermoid, epidermoid cysts, and other skull-based lesions were excluded from the analysis. RESULTS: Our analysis identified twenty-three cases of brain tumors diagnosed within the first year of life, comprising 14 boys and 9 girls. The median age at diagnosis was 8.2 months, and the most common presenting symptoms were nausea and vomiting, as well as head circumference abnormalities. Successful gross total resection was achieved in 75.8% of patients, with choroid plexus papilloma being the most frequently encountered histopathological diagnosis. Eight patients received adjuvant chemotherapy, while one patient underwent salvage radiotherapy. CONCLUSION: The treatment of brain tumors in infants during their first year of life presents significant challenges. The affected patients exhibit diverse tumor pathologies occurring at various locations within the brain. Further research is warranted to establish optimal treatment options for this specific population.

Disturbed dental development of permanent teeth in children with solid tumors and lymphomas.

BACKGROUND: Deficient dental root development has been reported after conventional pediatric anticancer therapy but less information is available on children with solid tumors and lymphomas. PROCEDURE: We examined dental development from panoramic radiographs (PRGs) of 37 childhood cancer survivors (CCS) treated under the age of 10 years with multimodal chemotherapy (CT) and/or head and neck radiotherapy (RT). A novel defect index (DeI) was used to describe the total damage to permanent dentition. The DeI was also tested in matched controls. RESULTS: All CCS had dental disturbances including abnormal root development (86.4%), microdontia (13.5%) and tooth agenesis (16.2%). Testing the controls gave the mean DeI value 1.9 ± 2.7 (range, 0-12), while the index scores in the study group varied from 0 to 49 (mean 10.8 ± 11.2) (P < 0.01). The statistical differences in the mean DeI values were insignificant between CT and CT and RT groups (P > 0.05). CONCLUSIONS: Children treated for solid tumors and lymphomas are at considerable risk of some disturbances in developing dental structures. RT increased the severity of disturbances induced by CT. Studies should further elucidate dose-, age and time-related effects of anticancer treatment on dental development.

Use of complementary and alternative medicine in children with cancer: effect on survival.

The objective of the present study was to determine the type, frequency, the reason why complementary and alternative medicine (CAM) treatments are used, the factors related with their use, and the effects of CAM usage on long-term survival. Families of a total of 120 children with cancer between 0-18 years of age, including 50 (41.7%) girls and 70 (58.3%) boys, participated in our study. The authors found that 88 patients (73.3%) used at least one CAM method, the most common (95.5%) of which was biologically based therapies. Most frequently used biologically based therapies were dietary supplements and herbal products. The most commonly used dietary supplement or herbal product was honey (43.2%) or stinging nettle (43.2%), respectively. We found that patients used such CAM methods as complementary to, but not instead of, conventional therapy. Sixty-nine out of 88 patient families (78.4%) shared the CAM method they used with their physicians. No statistically significant relation was found between socioeconomic, sociodemographic, or other factors or items and CAM use. The mean follow-up period of the CAM users and nonusers groups was 79.4 ± 36.7 (21.3-217.9) and 90.9 ± 50.3 (27.4-193.7) months, respectively. Five-year survival rates for CAM users and nonusers were found as 81.5% and 86.5%, respectively (P > .05). In conclusion, families of children with cancer use complementary and alternative treatment frequently. They do not attempt to replace conventional treatment with CAM. Higher rates of CAM use was found in families with higher educational level. CAM usage did not affect the long-term survival.

[Evaluation of PCR results in the diagnosis of Epstein-Barr virus infections]. / Epstein-Barr Virus Enfeksiyonlarinin Tanisinda PCR Sonuçlarinin Degerlendirilmesi.

Epstein-Barr virus (EBV), a herpesvirus leading to latent infections, is principally responsible for infectious mononucleosis, and also plays role in the etiology of various lymphomas and post-transplantation lymphoproliferative disease (PTLD). Laboratory diagnosis of EBV infections depends on the detection of atypical lymphocytes, heterophile antibodies, specific antibodies against viral capsid (VCA), nuclear (EBNA) and early (EA) antigens, and of the viral DNA. Since the seropositivity rate in adult population is very high (80-95%) in our country, routine serologic tests may be insufficient to characterize EBV reactivation in immunosuppressive subjects, such as transplant recipients or oncology patients. In those cases VCA IgG avidity test and molecular methods are more useful. This study was conducted to determine the role of viral DNA levels detected by real-time polymerase chain reaction (Rt-PCR) and serological tests for the diagnosis and follow up of EBV infections in renal transplant recipients and pediatric oncology patients. A total of 62 adult renal transplant recipients, 37 children with oncological diseases, and 50 EBVseropositive immunocompetent healthy subjects (28 children, 22 adults) as controls, were included in the study. Four blood samples, once before transplantation and three times thereafter (at first week, first and third months) were collected from transplant recipients; in pediatric oncology patients blood samples were collected four times, once before immunosuppressive treatment and three times thereafter (at first, third and sixth months), while the control group had a single blood sample collected. Serological profiles for EBV were searched by Paul-Bunnel and immunoblotting tests; VCA IgG avidity by ELISA and viral load by Rt-PCR. EBV-DNA was found positive in 3 (4.8%) of the renal transplant patients. While in these patients the CD4/CD8 ratio was significantly lowered in the first week and third month posttransplant, no PTLD or organ rejection developed. EBV-DNA was positive in 3 (8.1%) of the pediatric oncology patients. This positivity was attributed to Hodgkin's disease in two of these cases and to reactivation in the third case. EBV-DNA positivity was present in 10 (20%) of the control subjects. In the adult controls whose immunoblot results were compatible with the serologic pattern of an acute infection, the correlation among positive EBV-DNA, positive Paul-Bunnel and low IgG avidity results was statistically significant. As for children in the control group, this serologic profile was significantly correlated with low IgG avidity only. The data obtained from this study indicated that no risk of EBV-related PTLD or acute rejection was found in the first three months in the adult renal transplant patients. In children with EBV-related malignancy the search for EBV-DNA by RtPCR before therapy may be useful in the diagnosis, follow-up and prognostic evaluation. Serologic results should be supported by IgG avidity and PCR in order to ascertain the presence of EBV reactivation in immunosuppressive patients.

Time-dependent alterations in growth and bone health parameters evaluated at different posttreatment periods in pediatric oncology patients.

Bone mineral density (BMD) and anthropometric measurements in pediatric cancer patients were evaluated and compared at early and late posttreatment periods. Sixty-six pediatric cancer patients who recovered completely following treatment longer than at least a 6-month period were included in the study. Patients were evaluated cross-sectionally and prospectively with regard to anthropometric measurements and BMD twice; the first being at a mean period of 2.62 ± 1.44 years and the second of 6.55 ± 1.71 years after the completion of treatment. Rates of osteoporosis and osteopenia at first or second evaluation were 25.8% and 39.4% or 10.6% and 19.7%, respectively. Mean BMD z-scores were (-1.26) ± 1.12 [(-4.3)-2.0] and (-0.48) ± 1.25 [(-3.30)-3.40] at first and second evaluations, respectively. BMD findings obtained at second evaluation revealed statistically significant recovery compared with those obtained at first evaluation (P = .001). BMD z-scores were significantly lower in patients who received, as opposed to those who did not receive, radiotherapy (RT) at both evaluations. Anthropometric parameters of patients such as height, weight, and body mass index (BMI) were increased at both evaluations compared with values obtained at diagnosis (P < .05). Height standard deviation score (SDS) decreased at first evaluation compared with that measured at diagnosis, whereas it increased at second evaluation. Conversely, weight SDS and BMI SDS increased (P < .05) at first evaluation compared with that measured at diagnosis, whereas they decreased at second evaluation. The authors conclude that early impairments in anthropometric measurements recover in the long term, whereas BMD is continually reduced in children who recovered from cancer.

Recombinant human erythropoietin ß: the effect of weekly dosing on anemia, quality of life, and long-term outcomes in pediatric cancer patients.

Anemia, which is a common problem in cancer patients, has a negative effect on survival by decreasing the efficacy of chemotherapy and particularly of radiotherapy, as well as impairing the quality of life (QoL) of patients. Recombinant human erythropoietin (rHuEPO) decreases a patient's need for transfusions and increases their QoL. The aim of this study was to evaluate the effect of weekly single-dose EPO treatment on transfusion rates, QoL, and hemoglobin (Hb) levels. In addition, patients were followed up for a long period to assess the impact of EPO treatment on survival. The study was conducted from December 2001 to December 2002 in patients with newly diagnosed lymphoma or solid tumors using a prospective and controlled design. EPO-ß was given as a single dose of 450 U/kg once a week for 12 weeks. The study and control groups included 16 patients each. Hb levels measured in the study group at the 4th, 8th, and 12th weeks were significantly higher than the values recorded before the start of chemotherapy. In the control group, Hb levels post chemotherapy were significantly lower than values recorded prior to treatment. The increased Hb levels in the study group were significant at the 8th and 12th weeks of treatment compared to levels measured prior to treatment. In the control group, Hb levels at the 4th and 8th weeks were significantly lower than pretreatment levels. When the percent increase of Hb levels of the study and control groups with respect to treatment week was compared, the difference was statistically significant at the 4th, 8th, and 12th weeks. Although the increase on the performance scale within each group during treatment was significant in both the study and control groups, the increase was more marked in the study group. The percent increase on the performance scale with respect to week of treatment was higher in the study group than in the control group. In EPO treatment group, side effects were seen in 38% of patients, with 19% being local pain in the injection area, 13% local hyperemia, and 6% headache. The mean follow-up period of the study and control group was 7.03 ± 0.41 (6.0-7.41) and 7.46 ± 0.45 (6.58-7.83) years, respectively; no statistically significant difference existed between these figures. Overall survival at the end of 7 years of follow-up was 68.8% and 81.3% for the study and control groups, respectively. The use of EPO-ß in lymphoma and solid tumor patients on a once-weekly regimen (450 U/kg) was determined to be effective in increasing Hb levels, decreasing transfusion rates, and improving QoL. This regimen was safe, did not cause serious side effects, and can be recommended because of its high patient compliance and tolerability. An effect of EPO on prognosis was not evident. We could not have an explanation on the effect of EPO treatment on prognosis, as there were low number of patients and advanced-staged patients died earlier. Therefore, a larger number of patients are needed to clarify the effect of EPO treatment on prognosis.

Hyperuricemia and tumor lysis syndrome in children with non-Hodgkin's lymphoma and acute lymphoblastic leukemia.

OBJECTIVE: This study aimed to examine the incidence, clinical characteristics, and outcome of hyperuricemia and tumor lysis syndrome (TLS) in children with non-Hodgkin's lymphoma (NHL) and acute lymphoblastic leukemia (ALL). METHODS: This retrospective study included data from 327 patients (113 NHL and 214 ALL). RESULTS: Hyperuricemia occurred in 26.5% and 12.6% of the patients with NHL and ALL, respectively. The corresponding figures for TLS were 15.9% and 0.47% (p=0.001). All hyperuricemic NHL patients had advanced disease and renal involvement was present in 53%. All hyperuricemic ALL patients had a leukocyte count >50,000 mm3 at the time of diagnosis. Among the hyperuricemic NHL and ALL patients, 96.6% and 66.6% had LDH ≥500 UI/L, respectively. Treatment consisted of hydration and allopurinol; none of the patients received urate oxidase. Among the patients that developed TLS, 26.3% had laboratory TLS, 42.1% had grade I or II TLS, and 31.6% had grade III or IV TLS. Uric acid levels returned to normal after a mean period of 3.5±2.5 and 3.05±0.8 d in NHL and ALL groups, respectively. In all, 7% of the patients with hyperuricemia required hemodialysis. None of the patients died. CONCLUSION: In this series the factors associated with a high-risk for TLS were renal involvement in NHL and high leucocyte count in ALL. Management with allopurinol and hydration was effective in this group of patients with high tumor burden.

Dental health indices of long-term childhood cancer survivors who had oral supervision during treatment: a case-control study.

The aim of this case-control study was to compare the dental health of a group of long-term survivors of childhood cancer with that of age- and gender-matched healthy controls. All children who had been progress- and/or disease-free for at least 5 years were selected. Caries indices for permanent dentition (DMF/T) and primary dentition (dmf/t) were calculated. Past data were collected from medical and dental records and read separately by one of the authors. The mean age of the subjects at the initial diagnosis of the disease was 4.3 +/- 0.3 yr (range 0.5-14 yr). The mean time lapse from the cessation of the therapy to the present dental examination was 5.0 +/- 0.7 yr. The study group had significantly (p < .001) more carious lesions in their primary teeth than the control group. The mean dmf/t value was 5.8 +/- 0.6 in the long-term survivors compared to 3.4 +/- 0.2 in the controls. The difference in DMF/T value of those children was statistically insignificant (p > .05) compared to healthy subjects (2.1 +/- 0.6 and 1.6 +/- 0.2, respectively). There was a significant positive correlation between the presence of head and neck radiotherapy (r = 0.427, p < .05), the number of months the child had been on chemotherapy (r = 0.413, p < .01), the number of fluoride gel treatments (r = .361, p < .05), and dmf/t values, separately. Based on the results of this study, our Unit now considers a through semiannual dental examination for every child who will be or has been treated at Pediatric Oncology Department to be mandatory. The focus should be on caries prevention and include dietary counseling, oral hygiene, and fluoride applications if necessary.

Treatment of high-risk neuroblastoma: National protocol results of the Turkish Pediatric Oncology Group.

BACKGROUND: The national protocol aimed to improve the outcome of the high risk neuroblastoma patients by high-dose chemotherapy and stem cell rescue with intensive multimodal therapy. MATERIALS AND METHODS: After the 6 induction chemotherapy cycles, patients without disease progression were nonrandomly (by physicians' and/or parent's choices) allocated into two treatment arms, which were designed to continue the conventional chemotherapy (CCT), or myeloablative therapy with autologous stem cell rescue (ASCR). RESULTS: Fifty-six percent (272 patients) of patients was evaluated as high risk. Response rate to induction chemotherapy was 71%. Overall event-free survival (EFS) and overall survival (OS) at 5 years were 28% and 36%, respectively. "As treated" analysis documented postinduction EFS of 41% in CCT arm (n = 138) and 29% in ASCR group (n = 47) (P = 0.042); whereas, OS was 45% and 39%, respectively (P = 0.05). Thirty-one patients (11%) died of treatment-related complications. CONCLUSION: Survival rates of high-risk neuroblastoma have improved in Turkey. Myeloablative chemotherapy with ASCR has not augmented the therapeutic end point in our country's circumstances. The adequate supportive care and the higher patients' compliance are attained, the better survival rates might be obtained in high-risk neuroblastoma patients received myeloablative chemotherapy and ASCR.

Disseminated rhabdomyosarcoma presenting as hypercalcemia.

Hypercalcemia may be seen in children with malignancy. We report a 4 year old child with rhabdomyosarcoma that initially presented with signs of hypercalcemia and bone involvement.

Thyroid functions in long-term survivors of pediatric Hodgkin's lymphoma treated with chemotherapy and radiotherapy.

OBJECTIVE: Post-treatment endocrine disturbances are common in cancer patients who have received radiotherapy or chemotherapy. The objective of this study was to evaluate the thyroid functions of long-term survivors of pediatric Hodgkin's lymphoma treated with chemotherapy and radiotherapy. METHODS: Thyroid functions of 55 Hodgkin's lymphoma patients (M/F:2.05/1) in complete remission were evaluated retrospectively. RESULTS: The mean age of the patients at diagnosis was 10.35±4.09 (range: 2.83-17) years and the mean follow-up period was 5.54±3.68 (range: 0.92-13.92) years. All patients received chemotherapy; a total of 50 patients (90.9%) underwent radiotherapy, 42 (76.4%) of whom received neck/mantle radiotherapy. Thyroid function tests were abnormal in 14 (24.5%) patients and normal - in the remaining 41 (74.5%). A diagnosis of subclinical and overt hypothyroidism was made in 11 (78.6%) and 3 (21.4%) patients with abnormal thyroid function tests, respectively. Nearly one-fourth (21.4%) of all thyroid function disorders were detected in the first year of follow-up. A statistically significant correlation was found between the dose of mantle radiotherapy and thyroid function disorder (p=0.002). In addition, statistically significant correlations were established between thyroid examination or thyroid ultrasonography findings and thyroid functions (p <0.001 or p=0.006, respectively). CONCLUSIONS: Radiation-induced thyroid disorders may develop in pediatric Hodgkin's lymphoma patients in complete remission starting as early as the first year after treatment and are dose-dependent. Patients, particularly those who have been exposed to radiotherapy of the neck, must be followed up closely for occurrence of thyroid dysfunctions.