Sevinç Kalın, Serçin Güven, Neslihan Çiçek

Nutcracker Sendromlu Çocuklarda Tanı ve Tedavi: Tek Merkez Deneyimi

Amaç: Merkezimizin Nutcracker sendromlu (NCS) çocuklardaki tanı ve tedavi deneyiminin paylaşılması istenmiştir. Gereç ve Yöntemler: Hastanemizin çocuk nefroloji bölümüne Şubat 2017 ile Mart 2020 tarihleri arasında başvuran yedi hastanın medikal kayıtları geriye dönük olarak değerlendirildi. Hematüri ve proteinüri yakınmalarıyla başvuran bu hastalarda NCS tanısı renal doppler ultarasonografi (RDUS) ve MR anjiografi (MRA) ile doğrulandı. Hastaların başvuru ve son kontroldeki klinik özellikleri, radyolojik bulguları, laboratuvar tetkikleri ve medikal tedavileri değerlendirildi. Bulgular: Hastaların başlangıç ve son kontrolde 24 saatlik idrarda protein atılımı sırasıyla ortalama 15,25±9,19 mg/ m2/saat ve 9,8±3,94 mg/m2/saat idi. Anjiotensin dönüştürücü enzim inhibitörü (ACE inhibitörü) kullanılan hastalarda başlangıç ve son kontrolde, 24 saatlik idrarda protein atılımı sırasıyla ortalama 20±11,53 mg/m2/saat ve 9,6±6,44 mg/m2/saat bulundu (p=0,073). Anjiotensin dönüştürücü enzim inhibitörü kullanmayan hastalarda başlangıç ve son kontrolde 24 saatlik idrarda protein atılımı sırasıyla ortalama 11,7±6,39 mg/m2/saat ve 9,95±1,84 mg/m2/saat saptandı (p= 0,61). Ayakta yapılan RDUS incelemede sol renal venin aortomesenterik mesafede açı ortalaması 14,71 ± 4,46 derece idi. Ayakta ölçülen sol renal ven antero-posterior çap oranı (hiler/Aortomesenteric) ortalama 6,4 bulundu. Görüş: Genç hastalarda NCS’nun selim seyirli olması tedavide konservatif kalınmayı gerektirir.Anahtar kelimeler: Nutcracker Sendromu, ortostatik proteinüri, renal ven

Diagnosis And Treatment in Children With Nutcracker Syndrome: A SingleCenter Experience

Objective: It has been aimed to report the experience of our center regarding diagnosis and treatment experience in children with Nutcracker syndrome (NCS). Materials and Methods: The medical records of seven patients who have admitted to the Department of Pediatric Nephrology of our hospital between February 2017 and March 2020 were evaluated retrospectively. The diagnosis of NCS was confirmed with renal Doppler ultrasound (RDUS) and magnetic resonance angiography (MRA) in these patients who have admitted with the complaints of hematuria and proteinuria. The patients’ data such as clinical characteristics, radiological findings, radiological signs and information about medical treatment at baseline and last control were recorded. Results: The mean levels of 24-h urine protein excretion in all patients at baseline and last control were 15,25±9,19 mg/m2/h and 9,8±3,94 mg/m2/h, respectively. The mean levels of 24-h urine protein excretion in patients treated with ACE (angiotensin converting enzyme) inhibitors at baseline and last control were 20±11,53 mg/m2/h and 9,6±6,44 mg/ m2/h, respectively. (p=0,073). The mean levels of 24-h urine protein excretion were 11.7±6.39 mg/m2/h and 9.95±1.84 mg/m2/h in patients not receiving ACE inhibitor treatment, at baseline and the last control respectively (p= 0,61). The mean angle value of the left renal vein in the aortomesenteric distance measured by RDUS examination performed in the upright position was 14,71±4,46 degrees. The anteroposterior diameter of the left renal vein (hilar/aortomesenteric) measured in the upright position was 6,4. Conclusion: The benign nature of NCS in young patients requires maintaining conservative approach.Keywords: Nutcracker syndrome, orthostatic proteinuria, renal vein

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