Deniz KARAKAYA, Fatma YAZILITAŞ, Evrim KARGIN ÇAKICI, Tülin GÜNGÖR, Evra ÇELİKKAYA, Mehmet BÜLBÜL

Çocukluk Çağı Membranöz Glomerülonefrit Tanılı Hastaların Klinik Değerlendirmesi

Amaç: Membranöz nefropati (MN), pediatrik popülasyonda erişkin dönemden daha nadir görülen bir immün kompleks hastalığıdır. MN’nin prognozu, spontan tam remisyondan son dönem böbrek hastalığına (ESRD) kadar değişkendir. Çok merkezli geniş çalışmaların olmaması, bu hastalarda tedavi seçeneklerinin ve klinik sonuçların ayrıntılı olarak incelenmesi olasılığını engellemektedir. Bu çalışma, pediatrik hastalarda MN’nin klinik bulguları, tedavisi ve prognozu hakkındaki literatür verilerine katkı sağlamayı amaçlamaktadır. Gereç ve Yöntemler: Bu çalışmaya 13 hasta dahil edilmiştir. Çalışmaya primer ve sekonder MN glomerülonefrit tanılı hastalar dahil edilmiştir. Bulgular: Yaş ortalaması 12.29±3.67 yıldı. Yedi (%53.8) hastada (1 olgu spontan remisyon) tam remisyon, 4 (%30.8) hastada kısmi remisyon meydana geldi. 13 hastanın 2’sinde (%15.4) böbrek yetmezliği gelişti. Son kontrolde 6 (%46.2) hastada proteinüri, 4 (%30.8) hastada mikroskobik hematüri saptandı ve 9 hasta halen düşük doz steroid kullanıyordu. Sonuç: Mevcut bulgular, pediatrik hastalarda MN’nin prognozu ile ilişkili herhangi bir önemli risk faktörü belirlememiştir, ancak pediatrik MN ile ilgili sınırlı verilere katkı sağlayacağı düşünülmektedir. Pediyatrik popülasyonda MN’nin doğal seyri, tedavi seçenekleri ve uzun dönem sonuçları ile ilgili mevcut verilerin çoğu küçük, kontrolsüz vaka serilerinden oluşmaktadır. Bu nedenle, pediatrik MN’nin prognozu ile ilgili faktörleri açık bir şekilde aydınlatmak için daha büyük ölçekli klinik araştırmaların gerekli olduğunu düşünüyoruz.

Anahtar Kelimeler:

membranöz glomerülonefrit, çocukluk çağı, nefrotik sendrom, nefritik sendrom

Clinical Evaluation of Pediatric Patients Diagnosed with Membranous Glomerulonephritis

Objective: Membranous nephropathy (MN) is a rare immune complex disease in pediatric population then adults. The prognosis of MN is variable, ranging from spontaneous complete remission to end-stage renal disease (ESRD). The lack of large multicenter studies precludes the possibility of examining in detail the treatment options and clinical outcomes in these patients. The present study aimed to expand the literature on the clinical findings, treatment, and prognosis of MN in pediatric patients. Material and Methods: This single-center retrospective study included 13 patients with a diagnosis of primary and secondary membranous nephropathy. Results: The mean age of the sample was 12.29±3.67 years. Complete remission occurred in 7 (53.8%) patients (of which 1 case was spontaneous remission), and partial remission occurred in 4 (30.8%) patients. In long-term follow-ups; one patient had chronic kidney disease (CKD) and one patient had end-stage renal disease (ESRD). At the last-follow up, proteinuria was noted in 6 (46.2%) patients and microscopic hematuria was noted in 4 (30.8%) and 9 patients were still using low-dose steroids. Conclusion: The current findings have not identified any significant risk factors associated with the prognosis of MN in pediatric patients, but are thought to contribute to the limited data on pediatric MN. Most of the available data on the natural history, treatment options, and long-term outcomes of MN in the pediatric population consists of small, uncontrolled case series. Therefore, we think that larger-scale clinical trials are necessary to clearly elucidate the factors related to the prognosis of pediatric MN.

Keywords:

membranous glomerulonephritis, childhood, nephrotic syndrome, nephritic syndrome,

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