Renal Hypoplastic Dysplasia

Yıl 2008, Cilt: 25 Sayı: 1, 25 - 29, 29.12.2009

Gamze Numanoğlu Yurdakan Gürkan Kertiş Burak Bahadır K. Varım Numanoğlu Şükrü Oğuz Özdamar

Öz

Renal dysplasia is a disorganized development of the kidney because anomalous differentiation of the metanephrosis. Abnormalities of the collecting system are common. They include obstruction of the ureteropelvic junction, ureteral atresia and urethral obstruction. The case was a six months old female child with ultrasonografically detected right hydronephrosis and oligohydroamniosis at the 20 th week of gestation. She underwent an operation for bilateral hydroureteronephrosis at 1 month of age. There was no serious and related illness in family history and self-history. She underwent right nephrectomy at 6 months of age. The specimen included an atrophic nephrectomy material with lobulations and a dilated ureter. When sectioned, it was observed that the pelvicaliceal areas were dilated and the cortex-medulla border was obscured.
On microscopic examination, the cortex was thin, contained rare small glomerules, primitive ducts and mononuclear cell infiltration were seen. A focus of cartilage was observed at the cortex-medulla junction. The case was diagnosed as renal hypoplastic dysplasia. During 4 months of follow-up, no complication was reported.
This case is presented considering the uncommon occurence of renal dysplasia and its d iffe re ntial diagnosis from other congenital cystic renal malformations.



Renal Hipoplastik Displazi
Renal displazi, metanofreoz sırasında diferansiasyon anomalisine bağlı olarak böbrek gelişimindeki bir bozukluktur. Toplayıcı sistem bozuklukları sıktır. Bu anomaliler; üreteropelvik bileşkenin obstrüksiyonu, üreteral atrezi ve üretral obstrüksiyondur.
Olgu; 20. gebelik haftasında ultrasonografi ile sağ hidronefroz ve oligohidramnioz belirlenen 6 aylık kız çocuğudur. Hasta bir aylık iken bilateral hidroüreteronefroz için opere edildi. Hastanın özgeçmişi ve soygeçmişinde hastalığı ile ilişkili ciddi bir rahatsızlığı belirlenmedi. 6 aylık iken sağ nefrektomi uygulandı. Nefrektomi materyali, dilate üreter ve lobülasyonlar içeren atrofik bir böbrekten oluşmaktaydı. Kesitinde pelvikalisiyel alanların dilate olduğu ve korteks-medulla sınırının seçilemediği gözlendi.
Mikroskobik incelemede, korteks oldukça ince gözlendi ve medullada az sayıda küçük glo-merüller, primitif duktuslar ve mononükleer iltihabi hücre infiltrasyonu izlendi. Korteks-me-dulla bileşkesinde kartilaj odağı belirlendi. Olgu; renal hipoplastik displazi olarak tanı aldı. 4 aylık takibi sonrasında olguda komplikasyon belirlenmedi.
Bu olgu, renal displazinin nadir görülmesi ve diğer konjenital kistik renal malformasyonlar-dan ayırımı açısından sunulmaktadır.

Anahtar Kelimeler

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Kaynakça

• Gilbert-Barness E, Lacson A. Developmental a b n o r m a l i t i e s
• Diagnostic Surgical Pathology, Fourth Ed, Vol: 3, In Mills SE, Carter D, Greenson JK, Oberman HA, Reuter VE Stoler MH. Eds. 2004 Philadelphia, by Lippincott Williams&Wilkins: 1845–1862.
• The Kidney. In Robbins Pathologic Basis of Diseases, Sixth Ed, Cotran RS, Kumar V, Collins T. Eds. 1999 Philadelphia, WB Saunders Co. 930–996.
• Ordonez NG, Rosai J, Urinary Tract. In Rosai and Ackerman’s Surgical Pathology, Ninth Ed, Vol: 1, Philadelphia, Mosby, 2004: 1163–1316.
• Kakkar N, Menon S, Radotra BD. Histomorphology of renal dysplasia—an autopsy study. Fetal Pediatr Pathol. 2006; 25: 73–86.
• http://www.emedicine.com/ped/topic1493.htm
• Woolf AS, Price KL, Scambler PJ, Winyard PJ. Evolving concepts in human renal dysplasia. J Am Soc Nephrol. 2004; 15: 998–1007.
• Menon S, Kakkar N, Radotra BD. Expression of laminin and fibronectin in renal dysplasia. Pediatr Dev Pathol. 2004; 568–576.
• Shibata S, Shigeta M, Shu Y, Watanabe T. Initial pathological events in renal dysplasia with urinary tract obstruction in utero. Virchows Arch. 2001; 560–570.
• Poucell-Hatton S, Huang M, Bannykh S, Benirschke K, Masliah E. Fetal obstructive uropathy. Patterns of renal pathology. Pediatr Dev Pathol. 2000; 3: 223–231.
• Volmar KE, Fritsch MK, Perlman EJ, Hutchins GM. Patterns of congenital lower urinary tract obstructive uropathy: Relation to abnormal prostate and bladder development and the Prune Belly Syndrome. Pediatr Dev Pathol. 2001; 467–472.
• Lashley DB, Elezer M, Kaplan GW, McAleer IM. Megacystic microcolon intestinal hypoperistalsis s y n d r o m e : Bladder distension and pyelectasis in the fetus without anatomic outflow obstruction. Urology 2000; 55: 774.