Concurrency of Guillain-Barre syndrome and acute transverse myelitis: a case report and re- view of literature

Orkun Tolunay,Tamer Çelik,Ümit Çelik,Mustafa Kömür,Zeynep Tanyeli,Abdurrahman Sönmezler 대한소아청소년과학회 2016 Clinical and Experimental Pediatrics (CEP) Vol.59 No.no.sup1

Guillain-Barré syndrome and acute transverse myelitis manifest as demyelinating diseases of the peripheral and central nervous system. Concurrency of these two disorders is rarely documented in literature. A 4-year-old girl presenting with cough, fever, and an impaired walking ability was admitted to hospital. She had no previous complaints in her medical history. A physical examination revealed lack of muscle strength of the lower extremities and deep tendon reflexes. MRI could not be carried out due to technical problems; therefore, both Guillain-Barré syndrome and acute transverse myelitis were considered for the diagnosis. Intravenous immunoglobulin treatment was started as first line therapy. Because this treatment did not relieve the patient's symptoms, spinal MRI was carried out on the fourth day of admission and demyelinating areas were identified. Based on the new findings, the patient was diagnosed with acute transverse myelitis, and high dose intravenous methylprednisolone therapy was started. Electromyography findings were consistent with acute polyneuropathy affecting both motor and sensory fibers. Therefore, the patient was diagnosed with concurrency of Guillain-Barré syndrome and acute transverse myelitis. Interestingly, while concurrency of these 2 disorders is rare, this association has been demonstrated in various recent publications. Progress in diagnostic tests (magnetic resonance imaging and electrophysiological examination studies) has enabled clinicians to establish the right diagnosis. The possibility of concurrent Guillain-Barré syndrome and acute transverse myelitis should be considered if recovery takes longer than anticipated.

Replacing Actinomycin-D with Carboplatin for Newly Diagnosed Rhabdomyosarcoma

Sezgin, Gulay,Acipayam, Can,Bayram, Ibrahim,Ozkan, Ayse,Kupeli, Serhan,Tanyeli, Atila Asian Pacific Journal of Cancer Prevention 2015 Asian Pacific journal of cancer prevention Vol.16 No.8

Background: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in the pediatric age group. All patients with RMS regardless of their initial stage or group receive combination chemotherapy as 'standard therapy' consisting of vincristine, actinomycin-D and cyclophosphamide. Actinomycin-D was not readily available in Turkey at one time. Carboplatin was used instead in order to prevent delays in treatment. The aim of this report is to present the results of patients with rhabdomyosarcoma receiving carboplatin or actinomycin-D therapy. Materials and Methods: Twenty four patients with rhabdomyosarcoma treated between December 2000 and June 2011 were included in this retrospective study. The patients were treated according to International Rhabdomyosarcoma Study Group guidelines. Eleven patients were treated with actinomycin-D and 13 with carboplatin ($250mg/m^2/dose$ for 2 days). The two groups were then compared in terms of 2- and 5-year overall survival (OS) and hematological and non-hematological toxicities. Results: Age, sex, stage and the mean duration of follow-up were similar in both groups (p>0.05). Two- and five-year OS levels were 68.2% in the carboplatin group and 78.0% and 40.0%, respectively, in the actinomycin-D group. There was no statistical difference in the number of febrile episodes (p=0.86) and no other hematological and non-hematological adverse effects were recorded in both groups. Conclusions: The findings show that carboplatin can be used as an alternative drug in the primary treatment of rhabdomyosarcoma in the event that actinomycin-D is unavailable or not tolerated.

Concurrency of Guillain-Barre syndrome and acute transverse myelitis: a case report and review of literature

Tolunay, Orkun,Celik, Tamer,Celik, Umit,Komur, Mustafa,Tanyeli, Zeynep,Sonmezler, Abdurrahman The Korean Pediatric Society 2016 Clinical and Experimental Pediatrics (CEP) Vol.59 No.no.sup1

Guillain-$Barr{\acute{e}}$ syndrome and acute transverse myelitis manifest as demyelinating diseases of the peripheral and central nervous system. Concurrency of these two disorders is rarely documented in literature. A 4-year-old girl presenting with cough, fever, and an impaired walking ability was admitted to hospital. She had no previous complaints in her medical history. A physical examination revealed lack of muscle strength of the lower extremities and deep tendon reflexes. MRI could not be carried out due to technical problems; therefore, both Guillain-$Barr{\acute{e}}$ syndrome and acute transverse myelitis were considered for the diagnosis. Intravenous immunoglobulin treatment was started as first line therapy. Because this treatment did not relieve the patient's symptoms, spinal MRI was carried out on the fourth day of admission and demyelinating areas were identified. Based on the new findings, the patient was diagnosed with acute transverse myelitis, and high dose intravenous methylprednisolone therapy was started. Electromyography findings were consistent with acute polyneuropathy affecting both motor and sensory fibers. Therefore, the patient was diagnosed with concurrency of Guillain-$Barr{\acute{e}}$ syndrome and acute transverse myelitis. Interestingly, while concurrency of these 2 disorders is rare, this association has been demonstrated in various recent publications. Progress in diagnostic tests (magnetic resonance imaging and electrophysiological examination studies) has enabled clinicians to establish the right diagnosis. The possibility of concurrent Guillain-$Barr{\acute{e}}$ syndrome and acute transverse myelitis should be considered if recovery takes longer than anticipated.

Chromosome Imbalances and Alterations of AURKA and MYCN Genes in Children with Neuroblastoma

Inandiklioglu, Nihal,Yilmaz, Sema,Demirhan, Osman,Erdogan, seyda,Tanyeli, Atila Asian Pacific Journal of Cancer Prevention 2012 Asian Pacific journal of cancer prevention Vol.13 No.11

Background: Neuroblastoma (NB), like most human cancers, is characterized by genomic instability, manifested at the chromosomal level as allelic gain, loss or rearrangement. Genetics methods, as well as conventional and molecular cytogenetics may provide valuable clues for the identification of target loci and successful search for major genes in neuroblastoma. We aimed to investigate AURKA and MYCN gene rearrangements and the chromosomal aberrations (CAs) to determine the prognosis of neuroblastoma. Methods: We performed cytogenetic analysis by G-banding in 25 cases [11 girls (44%) and 14 boys (66%)] and in 25 controls. Fluorescence in situ hybridization (FISH) with AURKA and MYCN gene probes was also used on interphase nuclei to screen for alterations. Results: Some 18.4% of patient cells exhibited CAs., with a significant difference between patient and control groups in the frequencies (P<0.0001). Some 72% of the cells had structural aberrations, and only 28% had numerical chnages in patients. Structural aberrations consisted of deletions, translocations, breaks and fragility in various chromosomes, 84% and 52% of the patients having deletions and translocations, respectively. Among these expressed CAs, there was a higher frequency at 1q21, 1q32, 2q21, 2q31, 2p24, 4q31, 9q11, 9q22, 13q14, 14q11.2, 14q24, and 15q22 in patients. 32% of the patients had chromosome breaks, most frequently in chromosomes 1, 2, 3, 4, 5, 8, 9, 11, 12, 19 and X. The number of cells with breaks and the genomic damage frequencies were higher in patients (p<0.001). Aneuploidies in chromosomes X, 22, 3, 17 and 18 were most frequently observed. Numerical chromosome abnormalities were distinctive in 10.7% of sex chromosomes. Fragile sites were observed in 16% of our patients. Conclusion: Our data confirmed that there is a close correlation between amplification of the two genes, amplification of MYCN possibly contributing significantly to the oncogenic properties of AURKA. The high frequencies of chromosomal aberrations and amplifications of AURKA and MYCN genes indicate prognostic value in children with neuroblastomas and may point to contributing factors in their development.

Meropenem Versus Piperacillin-Tazobactam as Empiric Therapy for Febrile Neutropenia in Pediatric Oncology Patients

Sezgin, Gulay,Acipayam, Can,Ozkan, Ayse,Bayram, Ibrahim,Tanyeli, Atila Asian Pacific Journal of Cancer Prevention 2014 Asian Pacific journal of cancer prevention Vol.15 No.11

Background: Infection is a serious cause of mortality in febrile neutropenia of pediatric cancer patients. Recently, monotherapy has replaced the combination therapy in empirical treatment of febrile neutropenia. Since there has been no reported trial comparing the efficacy of meropenem and piperacillin-tazobactam (PIP/TAZ) monotherapies, the present retrospective study was conducted to compare safety and efficacy in febrile neutropenic children with cancer. Materials and Methods: Charts of febrile, neutropenic children hospitalized at our center between March 2008 and April 2011 for hemato-oncological malignancies were reviewed. Patients received PIP/TAZ 360 mg/kg/day or meropenem 60 mg/kg/day intravenously in three divided doses. Duration of fever and neutropenia, absolute neutrophil count, modification, and success rate were compared between the two groups. Resolution of fever without antibiotic change was defined as success and resolution of fever with antibiotic change or death of a patient was defined as failure. Modification was defined as changing the empirical antimicrobial agent during a febrile episode. Results: Two hundred eighty four febrile neutropenic episodes were documented in 136 patients with a median age of 5 years. In 198 episodes meropenem and in 86 episodes PIP/TAZ were used. Duration of fever and neutropenia, neutrophil count, sex, and primary disease were not different between two groups. Success rates and modification rate between two groups showed no significant differences (p>0.05). Overall success rate in the meropenem and PIP/TAZ groups were 92.4% and 91.9% respectively. No serious adverse effects occurred in either of the groups. Conclusions: Meropenem and PIP/TAZ monotherapy are equally safe and effective in the initial treatment of febrile neutropenia in children with cancer.