Dilek Gunes

Evaluation of the effect of acetyl L-carnitine on experimental cisplatin nephrotoxicity.

Background/Aims: To evaluate the protective effects of acetyl L-carnitine (ALCAR) on cisplatin-induced nephrotoxicity in rats, and to gain insights into the possible protective mechanisms of ALCAR against nephrotoxicity. Methods: Twenty-eight Wistar rats were divided into four groups. Group 1 was administered saline only, group 2 was administered ALCAR, group 3 was administered cisplatin, and group 4 was administered ALCAR prior to cisplatin. Rats were sacrificed after 72 h of cisplatin/saline infusion. Serum creatinine and glomerular filtration rate values were obtained, and kidney samples were examined by light and electron microscopy. Apoptotic cell death and caspase-3, 8 and 9 activities were studied immunohistochemically. Results: In group 4, ALCAR administration resulted in an improvement in kidney function tests. Histopathological findings confirmed the biochemical data. Whilst the fusion of the foot processes of podocytes was observed in group 3, they were intact in group 4 on electron-microscopic examination. Apoptotic cell death and caspase-3, 8 and 9 activities were also decreased in group 4 compared to group 3. Conclusions: Antioxidative, antiapoptotic and anti-inflammatory properties of ALCAR were supported by the findings that this agent improves kidney function tests and has the effects of tissue protection and inhibition of apoptosis in cisplatin-induced nephrotoxicity.

Evaluation of the Effect of Acetyl L-Carnitine on Experimental Cisplatin Ototoxicity and Neurotoxicity

Introduction: Cisplatin (CDDP) is an effective and widely used chemotherapeutic agent for pediatric tumors, and ototoxicity is one of the dose-limiting side effects. Objective: It was the aim of our study to investigate the effect of acetyl L-carnitine (ALCAR) on experimental CDDP ototoxicity by audiologic tests, histomorphologic, immunohistochemical and ultrastructural examinations and to investigate the apoptotic pathways. Materials and Methods: Wistar albino rats (n = 28) were studied. Baseline audiological tests were performed in 4 groups: group 1, control; group 2, ALCAR; group 3, CDDP; group 4, CDDP + ALCAR-administered rats. Control audiological tests were performed on the 3rd day, and then the rats were sacrificed. Ear and brain specimens were examined by transmission electron microscopy, and caspase 3, 8 and 9 activities were investigated. Results: The CDDP-administered rats showed significant auditory brainstem response threshold shifts using all stimuli (clicks, 6-kHz and 8-kHz tone burst) compared with the control groups. The CDDP + ALCAR-administered rats showed significant auditory brainstem response threshold shifts by only click stimuli compared with the control groups. In the brain, spiral ganglion and organ of Corti, ultrastructural damage was prominent in group 3; the number of TUNEL (terminal deoxynucleotidyl transferase-mediated dUTP nick end labeling)-positive cells and caspase 3, 8 and 9 immunostaining cells was significantly high in group 3. Conclusion: ALCAR improves CDDP-induced auditory impairment, and also antioxidative and antiapoptotic properties of ALCAR on CDDP ototoxicity were supported by the findings.

The time to diagnosis in childhood lymphomas and other solid tumors.

There are few reports from developing countries on the factors that influence the time to diagnosis (TD) in childhood cancer. The purpose of this study was to investigate the determinants of the TD in Turkish cancer patients.

Paravertebral malignant tumors of childhood: analysis of 28 pediatric patients

PurposeTo evaluate the clinical features and treatment results of the primary paravertebral malignant tumors (PMTs) in our department.MethodsMedical records of 28 children with primary PMTs treated between 1988–2007 were analyzed retrospectively.ResultsPrimary PMTs constituted 4.8% of the cancer cases in our department. Tumor diagnoses were mostly neuroblastoma (46.4%) and soft tissue sarcomas (35.7%). These cases presented with pain (64.3%), motor dysfunction (42.8%), sphincter dysfunction (35.7%), palpable mass (32.1%), and sensory deficits (7.1%). All tumors were extradural. Physical examination revealed motor deficits (53.6%), deep tendon reflex alterations (53.6%), sphincter dysfunction (35.7%), pathologic reflexes (25%), abnormal cutaneous reflexes (25%), and sensory deficits (17.8%). Sixteen had cord compression (CC; 13 clinical, three radiological CC). Eleven of them presented with advanced disease. Seven were managed by surgical departments by primary surgery (three unresponsive). Others were managed by pediatric oncology: five with corticosteroids ± chemotherapy (one unresponsive), one with radiotherapy (RT), and two with surgery for the clinical CC. Surgery was tumor excision in nine, laminectomy in nine, laminotomy in one, and delayed surgery after chemotherapy in two cases. In chemotherapy and surgery groups, there were neurologic sequela associated with the advanced disease at diagnosis in 38% and 37%, respectively. At 3-year median follow-up, nine patients died, 17 are alive (four with neurologic sequela), and two are lost of follow-up.ConclusionMajority of cases presented with advanced disease. Late referral is the major cause of morbidity and mortality. The CC caused by PMTs should be initially managed with corticosteroids ± chemotherapy to avoid the adverse late effects of RT and surgery.

Atypical teratoid/rhabdoid tumor in an infant conceived by in vitro fertilization

BackgroundAtypical teratoid/rhabdoid tumor (ATsRT) is a rare tumor and extremely aggressive embryonal neoplasm of the central nervous system. Brain tumors in infant are suggestive of some oncogenic prenatal factors.Case presentationWe report on a case of ATRT in a 4-month-old infant conceived by in vitro fertilization (IVF). Some previous reports have raised a question about the possible relation between IVF and childhood cancer, particularly embryonal tumors.ConclusionReport of such cases may provide some evidence to identify if there is a real association between congenital tumors and IVF.

A case of osteopoikilosis coexisting with amyloidosis of familial Mediterranean fever

Osteopoikilosis is a rare benign sclerosing bone dysplasia that is asymptomatic and often diagnosed incidentally. Multiple small, round or oval sclerotic foci are found, particularly in the pelvis and epiphyses and metaphyses of the long bones. It has an autosomal dominant mode of inheritance [1]. It is commonly associated with small subcutaneous fibrous nodules called dermatofibrosis lenticularis disseminate (Buschke-Ollendorff syndrome) [1, 2]. However, patients with osteopoikilosis and rheumatoid arthritis, polyarthralgia, synovial chondromatosis, dacriocystitis, trisomy 8, connective tissue disorders affecting the skin and joints, and discoid lupus erythematosus have also been reported [3, 4, 5, 6, 7, 8, 9]. Here we report a pediatric renal transplant recipient with amyloidosis secondary to familial Mediterranean fever (FMF) who was incidentally diagnosed as having osteopoikilosis in the 6th post-transplant year. To our knowledge, this is the first reported case of osteopoikilosis diagnosed in a child with amyloidosis associated with FMF. A 10-year-old boy with end-stage renal disease due to amyloidosis associated with FMF received a kidney graft from his father. He was given triple immunosuppressive therapy with prednisone, azathioprine, and cyclosporin A. Colchicine treatment was also continued. He has been followed for 84 months with this treatment. In the 6th post-transplant year, a plain chest X-ray obtained during routine screening for tuberculosis revealed small radiodense lesions in the right 3rd and 5th ribs and in left 4th and 5th ribs (Fig. 1). Pelvic X-ray (Fig. 2) showed similar lesions in the pelvic bones. He was asymptomatic, and the diagnosis was consistent with osteopoikilosis. Family screening for this disease was negative. Since osteopoikilosis has been reported to have an autosomal dominant form of inheritance, absence of osteopoikilosis in other family members may be related to incomplete penetrance and/or spontaneous mutation in the

Delayed renal excretion of methotrexate after a severe anaphylactic reaction to methotrexate in a child with osteosarcoma.

Although methotrexate is an agent widely used in the practice of pediatric oncology, allergic reactions to methotrexate are most unusual. Most of these reactions typically occur after repeated administration. Here, we report a severe anaphylactoid reaction to the first dose of high-dose methotrexate infusion in a child with osteosarcoma who has also experienced a delayed excretion of methotrexate. Clinicians must be aware of the possibility of a systemic, near-fatal anaphylactic reactions with methotrexate and patients who experience severe anaphylactic reactions should be followed carefully because of the possibility of delayed methotrexate excretion.

VENO-OCCLUSIVE DISEASE IN A CHILD WITH RHABDOMYOSARCOMA AFTER CONVENTIONAL CHEMOTHERAPY: Report of a Case and Review of the Literature

Although veno-occlusive disease of the liver is a well-known complication of high-dose chemotherapy and bone marrow transplantation, it has rarely been observed in children who receive conventional chemotherapy. Most cases in the literature consists of children with Wilms tumor. It has been very uncommon in rabdomyosarcoma patients until recently, although they commonly receive similar anticancer agents. Here the authors report a 2-year-old boy with rhabdomyosarcoma who developed veno-occlusive disease while receiving VAC (vincristine, actinomycin D, cyclophosphamide) chemotherapy regimen according to the IRS-IV protocol. The patient gradually recovered during 2 weeks with supportive treatment only.

Chronic recurrent multifocal osteomyelitis in a patient with selective immunoglobulin M deficiency

Chronic recurrent multifocal osteomyelitis is an unusual inflammatory process of unknown origin involving multiple osseous sites, often recurrently. Selective immunoglobulin M (IgM) deficiency is a rare primary immunodeficiency disease, which can be associated with autoimmune diseases such as systemic lupus erythematosus, Hashimoto’s disease, or hemolytic anemia. Here we report a case of a chronic recurrent multifocal osteomyelitis coexisting with selective IgM deficiency.

Conjunctival involvement in chronic recurrent multifocal osteomyelitis.

Purpose: To report a case of chronic recurrent multifocal osteomyelitis (CRMO) with conjunctival involvement. Methods: Retrospective chart review. Results: A 10-year-old girl who complained of fatigue and debilitating pain in both hips and legs for >1 year had recurrent episodes of redness and discharge in both eyes with little response to different topical medications. The diagnosis of CRMO was confirmed with the help of magnetic resonance imaging, bone scan, and bone biopsy results. She had moderate hyperemia and multiple discrete salmon-colored lesions in both palpebral and fornical conjunctivae. Biopsy revealed chronic inflammatory infiltration composed predominantly of lymphocytes forming a follicular pattern. Conjunctival lesions worsened during relapses of skeletal symptoms, improved during remission, and resolved shortly after the initiation of oral prednisolone therapy. No recurrence was observed during 16 months of follow-up. Conclusions: Salmon-colored conjunctival lesions may accompany CRMO and respond favorably to systemic steroid therapy.