Şerif YILMAZ, Kadim BAYAN, Yekta TÜZÜN, DAVUT AKIN, Timuçin ÇİL

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

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:

Hepatopulmoner sendrom, Geriyedönük çalışma, Hipertansiyon, portal

Twelve patients with idiopathic portal hypertension: Data from Southeastern Anatolia

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.

Keywords:

Hepatopulmonary Syndrome, Retrospective Studies, Hypertension, Portal,

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1. Benhamou JP, Valla DC. Intrahepatic portal hypertension. In: Bircher J, Benhamou JP, McIntyre N, et al, eds. Oxford textbook of clinical hepatology, 2nd edn. Oxford: Oxford University Press, 1999; 661–70.
2. Hillaire S, Valla DC, Lebrec D. Non cirrhotic portal hypertension. Clin Liver Dis 1997; 1: 1223–40.
3. Sama SK, Bhargava S, Gopi-Nath N, et al. Non-cirrhotic portal fibrosis. Am J Med 1971; 51: 160–71.
4. Levison DA, Kingham JC, Dawson AM, et al. Slow cirrhosis or no cirrhosis? A lesion causing benign intrahepatic portal hypertension. J Hepatol 1982; 137: 253–72.
5. Mikkelsen WP, Edmondson HA, Peters RL, et al. Extra- and intrahepatic portal hypertension without cirrhosis (hepatoportal sclerosis). Ann Surg 1965; 162: 602–8.
6. Wanless IE. Micronodular transformation (nodular regenerative hyperplasia) of the liver: a report of 64 cases among 2,500 autopsies and a new classification of benign hepatocellular nodules. Hepatology 1990; 11: 787-97.
7. Sciot R, Staessen D, Van Damme B, et al. Incomplete septal cirrhosis: histopathological aspects. Histopathology 1988; 13: 593–603.
8. Boyer JL, Sen Gupta KP, Biswas SK et al. Idiopathic portal hypertension. Comparison with the portal hypertension of cirrhosis and extrahepatic portal vein obstruction. Ann Intern Med 1967; 66:41–68.
9. Wanless IR, Peterson P, Das A, et al. Hepatic vascular disease and portal hypertension in polycythemia vera and agnogenic myeloid metaplasia: a clinicopathological study of 145 patients examined at autopsy. Hepatology 1990; 12: 1166–74.
10. Solis-Herruzo JA, Vidal JV, Colina F, et al. Nodular regenerative hyperplasia of the liver associated with the toxic oil syndrome: report of five cases. Hepatology 1986; 6: 687–93.
11. Solis-Herruzo JA, Vidal JV, Colina F, et al. Clinico-biochemical evolution and late hepatic lesions in the toxic oil syndrome. Gastroenterology 1987; 93: 558–68.
12. Nakanuma Y, Nonomura A, Hayashi M et al. Pathology of the liver in ‘idiopathic portal hypertension’ associated with autoimmune disease. The Ministry of Health and Welfare Disorders of Portal Circulation Research Committee. Acta Pathol Jpn 1989; 39: 586–92.
13. Boyer JL, Hales MR, Klatskin G. ‘Idiopathic’ portal hypertension due to occlusion of intrahepatic portal veins by organized thrombi. A study based on postmortem vinylite-injection corrosion and dissection of the intrahepatic vasculature in 4 cases. Medicine (Baltimore) 1974; 53: 77–91.
14. Sugita S, Ohnishi K, Iida S, et al. Histological changes in the liver and portal hypertension subsequent to repeated intraportal injections of killed E. coli in the dog. Liver 1988; 8: 1–9.
15. Bernard PH, Le Bail B, Cransac M, et al. Progression from idiopathic portal hypertension to incomplete septal cirrhosis with liver failure requiring liver transplantation. J Hepatol 1995; 22: 495–9.
16. Ichimura S, Sasaki R, Takemura Y, et al. The prognosis of idiopathic portal hypertension in Japan. Intern Med 1993; 32: 441-4.
17. Öztürk Ş, Ökten A, Kaymakoğlu S, ve ark. İdiyopatik portal hipertansiyon: 87 olgunun değerlendirilmesi. Turk J Gastroenterol 1999; 10: 334-9.
18. Ökten A, Tözün N, Özdil S, ve ark. İdiyopatik portal hipertansiyon (20 vakal›k bir seri). T›p Fak Mec 1984; 47: 473-9.
19. Okuda K: Idiopathic portal hypertension. In Recent Advances In Hepatology, 2nd ed. HC, Thomas, EA, Jones (eds). Edinburgh, Churchill Livingstone, 1986; 93-108.
20. Rodriguez-Roisin R, Roca J, Agusti AG, et al. Gas exchange and pulmonary vascular reactivity in patients with liver cirrhosis. Am Rev Respir Dis. 1987; 135: 1085-92.
21. Okuda K, Kono K, Ohnishi K, et al. Clinical study of eighty-six cases of idiopathic portal hypertension and comparison with cirrhosis with splenomegaly. Gastroenterology 1984; 86: 600–10.
22. Guha Mazumder DN, Chakraborty AK, Ghose A et al.Chronic arsenic toxicity from drinking tubewell water in rural West Bengal. Bull. World Health Organ 1988; 66: 499–506.
23. Sarin SK, Kapoor D. Non-cirrhotic portal fibrosis: current concepts and management. J Gastroenterol Hepatol 2002; 17: 526-34.
24. Bosch J, Mastai R, Kravetz D, et al. Hemodynamic evaluation of the patient with portal hypertension. Semin Liver Dis. 1986; 6: 309-17.
25. Dhiman RK, Chawla Y, Vasishta RK, et al. Non-cirrhotic portal fibrosis (idiopathic portal hypertension): experience with 151 patients and a review of the literatureJ Gastroenterol Hepatol 2002; 17: 6-16.
26. Nevens F, Fevery J, Van Steenbergen W, et al. Arsenic and non-cirrhotic portal hypertension. A report of eight cases. J Hepatol 1990; 11: 80-5.
27. Vakili C, Farahvash MJ, Bynum TE. "Endemic" idiopathic portal hypertension: report on 32 patients with non-cirrhotic portal fibrosis. World J Surg 1992; 16: 118-24; discussion 124-5.
28. Okuda K, Nakashima T, Okudaira M, et al. Liver pathology of idiopathic portal hypertension. Comparison with non-cirrhotic portal fibrosis of India. The Japan idiopathic portal hypertension study. Liver 1982; 2: 176-92.
29. Ludwig J, Hashimoto E, Obata H, et al. Idiopathic portal hypertension. Hepatology 1993; 17: 1157–62.
30. Nakanuma Y, Hoso M, Sasaki M, et al. Histopathology of the liver in non-cirrhotic portal hypertension of unknown aetiology. Histopathology 1993; 28: 195–204.
31. Hillaire S, Bonte E, Denninger MH, et al. Idiopathic non-cirrhotic intrahepatic portal hypertension in the West: a re-evaluation in 28 patients. Gut 2002; 51: 275-80.
32. Nayak NC, Ramalingaswami V. Obliterative portal venopathy of the liver. Associated with so-called idiopathic portal hypertension or tropical splenomegaly. Arch Pathol 1969; 87: 359-69.
33. Rodriguez-Roisin R, Agusti AG, Roca J. The hepatopulmonary syndrome: new name, old complexities. Thorax 1992; 47: 897-902.
34. Naeije R, Melot C, Hallemans R, et al. Pulmonary hemodynamics in liver cirrhosis. Semin Respir Med 1985; 7: 164-71
35. Babbs C, Warnes TW, Haboubi NY. Non-cirrhotic portal hypertension with hypoxaemia. Gut 1988; 29: 129-31.
36. Kaymakoglu S, Kahraman T, Kudat H, et al. Hepatopulmonary syndrome in noncirrhotic portal hypertensive patients. Dig Dis Sci 2003; 48: 556-60
37. Yilmaz S, Dursum M, Canoruc F, et al. A severe (type II) hepatopulmonary syndrome in a patient with idiopathic portal hypertension and treatment with paroxetine. Neth J Med 2005; 63: 448-52.
38. Krowka MJ. Hepatopulmonary syndrome: recent literature (1997 to 1999) and implications for liver transplantation. Liver Transpl 2000; 6: 31-5.
39. Nevens F, Staessen D, Sciot R, et al. Clinical aspects of incomplete septal cirrhosis in comparison with macronodular cirrhosis. Gastroenterology 1994; 106: 459–63.
40. Mitra SK, Rao KL, Narasimhan KL, et al. Side-to-side lienorenal shunt without splenectomy in noncirrhotic portal hypertension in children. J Pediatr Surg 1993; 28: 398-401; discussion 401-2.