Twelve patients with idiopathic portal hypertension: Data from Southeastern Anatolia

Öz

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.

Anahtar Kelimeler

• Idiopathic portal hypertension

İdiyopatik portal hipertansiyonlu on iki hasta: Güneydoğu Anadolu'dan veriler

Öz

Giriş ve amaç: İdiyopatik portal hipertansiyon (İPH), çoğunlukla bilinmeyen etyopatogenezli presinüzoidal portal hipertansiyon ile karakterli nadir bir durumdur. Bu çaılşmada Güneydoğu Anadolu'da yaşayan 12 hastanın klinikopatolojik özellikleri ve komplikasyonları incelenmiştir. Gereç ve yöntem: On iki kişilik bir hasta gurubunun demografik, klinik, laboratuar özellikleri ve gelişen komplikasyonlar retrospektif olarak değerlendirilmiştir. Bulgular: Altı kadın, 6 erkek dahil edildi. Ortalama yaş 35.2 yıl idi. Temel semptomlar anemiye bağlı yakınmalar (%58), sol üst kadran ağrısı (%83), dispepsi (%41) ve varisiyel kanama (%8) idi. Pika öyküsü hastaların %41'inde saptandı. Masif splenomegali %83 oranı nda vardı. Protrombin zamanı %58 olguda uzamıştı. Düşük protein C, protein S ve anti-trombin III aktivitesi sırasıyla 7 (%58), 4 (%33) ve 5 (%41) olguda saptandı. Karaciğer biyopsisi 7 (%58) olguda özellik göstermezken, 3 (%25) hastada minimal periportal fibrozis ve 2 (%16) hastada sinüzoidal dilatasyon örneği izlendi. Endoskopide 11 (%91) özofageal ve 1 (%8) fundal varis görüldü. Portal gastropati 7 (%58) olguda vardı. İki hastada hepatopulmoner sendrom saptandı. Bir hastada splenik rüptür gelişti. İki hastaya splenektomi yapıldı. Sonuç: Bölgemizdeki İPH gurubunda belirgin yüksek oranda pika öyküsünün varlığı ilginçtir. Hepatopulmoner sendrom sirozlularda olduğu gibi, idiyopatik portal hipertansiyonlularda da görülebilmektedir. Yukarıdaki değerlendirmeler dikkate alındığında şu sonuca varılabilir ki, İPH, tanısı az konan bir durumdur ve sirozsuz portal hipertansiyon olgularında akılda tutulmalıdır.

Anahtar Kelimeler

• İdiyopatik portal hipertansiyon

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