Objective:We aimed to evaluate the clinical, demographic, electrophysiological and treatment response of pediatric onset chronic inflammatory demyelinating polyneuropathy (CIDP) in Türkiye.
Methods:The clinical data and electrophysiological results of patients with CIDP between January 2010-December 2021 from 14 centers were reviewed retrospectively. The patients were classified according to Joint Task Force of the European Federation Neurological of Societies and the Peripheral Nerve Society Guideline on management of CIDP (2021).
Results:A total of 43 patients, 22 (%51.2) male and 21 (%48.8) female, were included in the study. The median age was 14.5 years (4.3-23) and the median age of onset of symptoms was 11 years (3 month-17 y). Thirty-eight patients were diagnosed with typical CIDP (88.4%) and 5 patients (11.6%) with possible CIDP. The most common electrophysiological finding of demyelination was ≥30 percent reduction of motor conduction velocity below the lower limit in two nerves (78.1%n= 25). All patients except one received immunotherapy either alone or in combination. There was a significant difference between pre and post-treatment Modified Rankin Scales (MRS) (p<0.05) of all patients. First-line treatments were IVIg (n=19,44.2%), IVIg+steroid (n=20,46.5%), steroid(n=1,2.3%), IVIg+steroid+plasmapheresis(n=1,2.3%) and IVIg+plasmapheresis (n=1,%2.3). Second-line treatment was given to 7 (16.6%) patients (n=3,azathioprine; n=2,rituximab; n=1,azathioprine+rituximab; n=1,azathioprine+ micofenolate mofetil +methotrexate).The patients were separated in to two different groups according to first-line treatment modalities (group-1:only IVIg, group-2:IVIg+steroid). There was no difference between pre and post treatment MRS of both groups.
Conclusion:This multicenter study showed that first-line immunotherapy modalities (IVIg versus IVIg+steroid) had equal efficacy for treatment CIDP patients in childhood.
Gamze Sarıkaya Uzan
Division of Child Neurology, Department of Pediatrics, Dokuz Eylül University Faculty of Medicine, İzmir, Turkey
Turkey
Deniz Yüksel
Department of Pediatric Neurology, Faculty of Medicine, Dr Sami Ulus Maternity Child Health and Diseases Training and Research Hospital, University of
Turkey
Erhan Aksoy
Department of Pediatric Neurology, Faculty of Medicine, Dr Sami Ulus Maternity Child Health and Diseases Training and Research Hospital, University of
Turkey
Ülkühan Öztoprak
Department of Pediatric Neurology, Faculty of Medicine, Dr Sami Ulus Maternity Child Health and Diseases Training and Research Hospital, University of
Turkey
Mehmet Canpolat
Division of Child Neurology, Department of Pediatrics, Erciyes University Faculty of Medicine, Kayseri, Turkey
Turkey
Selcan Öztürk
Department of Pediatrics, Erciyes University Faculty of Medicine, Kayseri, Turkey
Turkey
Çelebi Yıldırım
Department of Pediatrics, Erciyes University Faculty of Medicine, Kayseri, Turkey
Turkey
Ayten Güleç
Division of Child Neurology, Department of Pediatrics, Erciyes University Faculty of Medicine, Kayseri, Turkey
Turkey
Hüseyin Per
Division of Child Neurology, Department of Pediatrics, Erciyes University Faculty of Medicine, Kayseri, Turkey
Turkey
Hakan Gümüş
Division of Child Neurology, Department of Pediatrics, Erciyes University Faculty of Medicine, Kayseri, Turkey
Turkey
Çetin Okuyaz
Division of Child Neurology, Department of Pediatrics, Mersin University Faculty of Medicine, Mersin, Turkey
Turkey
Meltem Çobanoğulları Direk
Division of Child Neurology, Department of Pediatrics, Mersin University Faculty of Medicine, Mersin, Turkey
Turkey
Mustafa Kömür
Division of Child Neurology, Department of Pediatrics, Mersin University Faculty of Medicine, Mersin, Turkey
Turkey
Aycan Ünalp
Division of Pediatric Neurology, Department of Pediatrics, University of Health Sciences Dr. Behçet Uz Child Disease and Pediatric Surgery Training an
Turkey
Ünsal Yılmaz
Division of Pediatric Neurology, Department of Pediatrics, University of Health Sciences Dr. Behçet Uz Child Disease and Pediatric Surgery Training an
Turkey
Ömer Bektaş
Division of Child Neurology, Department of Pediatrics, Ankara University Faculty of Medicine, Ankara, Turkey
Turkey
Serap Teber
Division of Child Neurology, Department of Pediatrics, Ankara University Faculty of Medicine, Ankara, Turkey
Nargiz Aliyeva
Division of Pediatric Neurology, Department of Pediatrics, University of Health Sciences Tepecik Research and Training Hospital, Izmir, Turkey.
Turkey
Nihal Olgaç Dündar
Division of Child Neurology, Department of Pediatrics, İzmir Katip Çelebi University Faculty of Medicine, İzmir, Turkey
Turkey
Pınar Gençpınar
Division of Child Neurology, Department of Pediatrics, İzmir, Katip Çelebi University Faculty of Medicine, İzmir, Turkey
Turkey
Esra Gürkaş
Department of Pediatric Neurology, Children’s Hospital, Ankara City Hospital, Ankara, Turkey
Turkey
Sanem Keskin Yılmaz
Division of Child Neurology, Department of Pediatrics, Ege University Faculty of Medicine, İzmir, Turkey
Turkey
Seda Kanmaz
Division of Child Neurology, Department of Pediatrics, Ege University Faculty of Medicine, İzmir, Turkey
Turkey
Hasan Tekgül
Division of Child Neurology, Department of Pediatrics, Ege University Faculty of Medicine, İzmir, Turkey
Turkey
Ayşe Aksoy
Division of Child Neurology, Department of Pediatrics, On Dokuz Mayıs University Faculty of Medicine, Samsun, Turkey
Turkey
Gökçen Öz Tuncer
Division of Child Neurology, Department of Pediatrics, On Dokuz Mayıs University Faculty of Medicine, Samsun, Turkey
Turkey
Elif Acar Arslan
Division of Child Neurology, Department of Pediatrics, Karadeniz Technical University Faculty of Medicine, Trabzon, Turkey
Turkey
Ayşe Tosun
Turkey
Müge Ayanoğlu
Division of Child Neurology, Department of Pediatrics, Adnan Menderes University Faculty of Medicine, Aydın, Turkey
Turkey
Muhittin Bodur
Division of Child Neurology, Department of Pediatrics, Uludağ University Faculty of Medicine, Bursa, Turkey
Turkey
Bülent Ünay
University of Health Sciences Turkey, Gülhane Faculty of Medicine, Department of Pediatric Neurology, Ankara, Turkey
Turkey
Semra Hız Kurul
Division of Child Neurology, Department of Pediatrics, Dokuz Eylül University Faculty of Medicine, İzmir, Turkey
Turkey
Uluç Yiş
Division of Child Neurology, Department of Pediatrics, Dokuz Eylül University Faculty of Medicine, İzmir, Turkey
Turkey