Idiyopatik portal hipertansiyonlu on iki hasta: Güneydogu Anadolu’dan veriler
Twelve patients with idiopathic portal hypertension: Data from Southeastern Anatolia
Özet
Giris ve amaç: Idiyopatik portal hipertansiyon (IPH), çogunlukla bilinmeyen etyopatogenezli presinüzoidal portal hipertansiyon ile karakterli nadir bir durumdur. Bu çailsmada Güneydogu Anadolu’da yasayan 12 hastanin klinikopatolojik özellikleri ve komplikasyonlari incelenmistir. Gereç ve yöntem: On iki kisilik bir hasta gurubunun demografik, klinik, laboratuar özellikleri ve gelisen komplikasyonlar retrospektif olarak degerlendirilmistir. Bulgular: Alti kadin, 6 erkek dahil edildi. Ortalama yas 35.2 yil idi. Temel semptomlar anemiye bagli yakinmalar (%58), sol üst kadran agrisi (%83), dispepsi (%41) ve varisiyel kanama (%8) idi. Pika öyküsü hastalarin %41’inde saptandi. Masif splenomegali %83 orani nda vardi. Protrombin zamani %58 olguda uzamisti. Düsük protein C, protein S ve anti-trombin III aktivitesi sirasiyla 7 (%58), 4 (%33) ve 5 (%41) olguda saptandi. Karaciger biyopsisi 7 (%58) olguda özellik göstermezken, 3 (%25) hastada minimal periportal fibrozis ve 2 (%16) hastada sinüzoidal dilatasyon örnegi izlendi. Endoskopide 11 (%91) özofageal ve 1 (%8) fundal varis görüldü. Portal gastropati 7 (%58) olguda vardi. Iki hastada hepatopulmoner sendrom saptandi. Bir hastada splenik rüptür gelisti. Iki hastaya splenektomi yapildi. Sonuç: Bölgemizdeki IPH gurubunda belirgin yüksek oranda pika öyküsünün varligi ilginçtir. Hepatopulmoner sendrom sirozlularda oldugu gibi, idiyopatik portal hipertansiyonlularda da görülebilmektedir. Yukaridaki degerlendirmeler dikkate alindiginda su sonuca varilabilir ki, IPH, tanisi az konan bir durumdur ve sirozsuz portal hipertansiyon olgularinda akilda tutulmalidir.
Abstract
Background/aim: Idiopathic portal hypertension (IPH) is a rare entity characterized by presinusoidal portal hypertension mainly with unknown etiopathogenesis. The clinicopathological features and complications of 12 patients living in Southeastern Anatolia with IPH were evaluated in this study. Materials and methods: We analyzed a cohort of 12 patients retrospectively in terms of demographic, clinical and laboratory parameters and complications. Results: Six male and six female patients were included. Mean age was 35.2. Major symptoms were those caused by anemia (58%), left upper quadrant pain (83%), dyspepsia (41%) and variceal bleeding (8%). A history of pica (geophagia) was obtained in 41% of patients. Massive splenomegaly was seen in 83% patients. Prothrombin time was long in 58% of patients. Low protein C, protein S and anti- thrombin III activity was seen in 7 (58%), 4 (33%) and 5 (41%) patients, respectively. Liver biopsy revealed normal findings in 7 (58%), minimal periportal fibrosis in 3 (25%) and sinusoidal dilatation in 2 (16%). Endoscopy revealed 11 (91.7%) esophageal and 1 (8.3%) fundal varices. Portal gastropathy was seen in 7 (58%) patients. Two patients developed hepatopulmonary syndrome. One patient had splenic rupture. Two patients underwent splenectomy. Conclusion: It was interesting that a markedly high pica history was obtained in IPH patients in our region. Hepatopulmonary syndrome may occur in IPH patients, as in cirrhotics. Taking into account the above evaluation, we concluded that IPH is an underdiagnosed condition and should be kept in mind in patients with portal hypertension without cirrhosis.
