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Solitary hepatomegaly is a clinical setting mainly presented by enlarged liver and with no or mild findings of hepatocellular insufficiency and portal hypertension. In this paper, we presented a patient who admitted to our clinic with the complaints of abdominal pain and right upper quadrant fullness, and was consequently diagnosed as metastatic liver melanoma., Soliter hepatomegali fizik muayenede hepatosellüler yetmezlik ve portal hipertansiyon bulgularının olmadığı yada silik olduğu, hepatomegalinin ön planda olduğu durumları kapsamaktadır. Kliniğimize sağ üst kadranda dolgunluk ve karında ağrı yakınmaları ile başvuran ve tetkikleri sonucunda karaciğerde metastatik melanom saptanan bir olgu sunulmuştur.

Gaucher's disease, although rare is the most commonly seen lysosomal storage disorder, with autosomal recessive inheritance. It is due to deficiency of lysosomal beta-glucocerebrosidase so that the substrate betaglucocereb- roside accumulates in the reticulo-endothelial system. To date, three types have been defined. Gaucher's disease must be remembered in the setting of hepatosplenomegaly of unknown etiology and/or bone pain. During the course this disease, it was reported that hepatocellular dysfunction or portal hypertension might develop. In this report, a patient with Gaucher's disease associated with liver and bone involvement is presented., Gaucher hastalığı; otozomal resessif kalıtımla geçen, nadir görülen ancak en sık lizozomal lipid depo hastalığıdır. Lizozomal "betaglukoserebrozidaz" enzim eksikliğinden kaynaklanır ve substratı olan beta-glukoserebrozidin vücuttaki tüm retikülo-endoteliyal sistemde birikmesi ile karakterizedir. Nörolojik bulguların varlığı ve hastalığın seyrine göre 3 klinik alt grup tanımlanmıştır. Gaucher hastalığı; tanısı açıklanamayan hepatosplenomegali, ve/veya kemik ağrısı olan her hastada düşünülmelidir. Gaucher hücreleridir. Bu hastalık esnasında nadiren hepatoselüler disfonksiyon veya portal hipertansiyon gelişebileceği bildirilmiştir. Bu yazıda; nadir bir lipid depo hastalığı olan, kemik ve akciğer tutulumunun eşlik ettiği ve seyrinde portal hipertansiyonun da geliştiği bir Gaucher vakası sunulmuştur.

The aim of this study was to identify hepatitis D virus genotypes in our chronic hepatitis D patients. Twenty-five patients with hepatitis D virus infection were enrolled in this study. HDV-RNA was extracted from the serum by Boom methods. Hepatitis D virus genotype was determined by reverse transcriptase-polymerase chain reaction (RT-PCR) and restriction fragment length polymorphism (RFLP) methods. Genotype I was found in 22 patients, and could not be determined in the remaining 3 patients. In conclusion, genotype I is the dominant hepatitis D virus genotype in our country., Bu çalışmanın amacı; hepatitis D virusu ile infekte kronik hepatitli hastalarda genotip tayini yapmaktır. Çalışma kapsamına 25 hasta alınmıştır. HDV-RNA Boom metodu, hepatitis D virusu genotip tayini ise RT-PCR ve RFLP metodu ile çalışılmıştır. 22 hastada genotip I saptanmıştır. 3 hastada genotip tayini yapılamamıştır. Sonuç olarak; ülkemizdeki hepatitis D virusu, genotip I'dir.

Background and aims: Serum ceruloplasmin level is an acute phase reactant. It may be difficult to determine its value in the diagnosis of Wilson's disease, especially in the hepatic form and serum ceruloplasmin levels may be decreased by chelation therapy. The aim of this study was to determine its diagnostic value and to evaluate whether there is a change in serum ceruloplasmin level with chelation therapy. Materials and methods: Serum ceruloplasmin levels of 34 patients with Wilson's disease (15 hepatic, six neurologic, four hepatoneurologic and nine asymptomatic) were measured by radial immunodiffusion at the time of diagnosis and at least six months after the beginning of therapy. The mean age at diagnosis was 20±1.65 years (range 7-46 years) and the mean duration of follow-up was 45.2±43.8 months (range, 6-75 months). Results:In the whole group, a low serum ceruloplasmin level (< 20 mg/dl) was found in 76.5 % (26) of patients and the mean serum ceruloplasmin levels were 13.1±6.8 mg /dl (range, 3.2-26.9 mg/dl) at baseline. However, low serum ceruloplasmin level was found in 94% (32, p=0.421) of patients and mean serum ceruloplasmin levels were decreased to 9.4±4.4mg /dl (range; 3-23.9, p< 0.003) during therapy. The serum ceruloplasmin level was decreased in seven pts (three hepatic, two hepatoneurologic and too asymptomatic) and only one low serum ceruloplasmin level increased during therapy. Conclusions: Although determination of the serum ceruloplasmin level is suggested to be the most useful screening test for Wilson's disease, a normal serum ceruloplasmin level does not exclude the disease. Serum ceruloplasmin levels in such patients may be decreased during chelation therapy. Even if the serum ceruloplasmin level is normal at the time of diagnosis other laboratory parameters suggestive of impaired copper metabolism should be evaluated in patients with a suspected diagnosis Wilson's disease., Giriş ve amaç: Serum seruloplazmini, akut faz reaktanlarından biridir. Bu durum, özellikle hepatik formdaki Wilson hastalığı tanısında, serum seruloplazmin düzeyinin kullanımını zorlaştırır. Aynı zamanda şelatör tedavi sırasında serum düzeylerinde azalma da görülebilir. Gereç ve yöntem: Bu çalışmada 34 Wilson hastasında (15 hepatik, 6 nöropsikiyatrik, 4 hepatonörolojik ve 9 asemptomatik) başlangıç seruloplazmin seviyesinin tanıda değeri ve tedavi seyrindeki değişiklikleri değerlendirilmiştir. Serulopazmin seviyesi radyal immunodiffüzyon tekniği ile, başlangıçta ve şelatör tedavinin en az 6. ayından sonra ölçülmüştür. Hastaların ortalama yaşı 20±9.6 yıl (7-46 yıl) ve ortalama takip süresi 36±25 ay (6-75 ay) idi. Bulgular: Tüm grupta, tedavi öncesi düşük serum seruloplazmin seviyesi (< 20 mg/dl) %76.5 (26 hasta) ve ortalama serum seruloplasmin düzeyi 13.1±6.8 mg /dl (3.2-26.9 mg/dl) idi. Tedavi seyrinde, düşük serum seruloplasmin düzeyi hastaların % 94'ünde (32 hasta, p=0.421) ve ortalama serum seruloplazmin düzeyi 9.4±4.4mg /dl (3-23.9, p< 0.003) idi, anlamlı azalmıştı. Serum seruloplazmin düzeyi 7 hastada (3 hepatik, 2 hepatonörolojik, 2 asemptomatik) anlamlı azalırken sadece 1 hastada (hepatik form) anlamlı olarak arttı. Sonuç: Bu sonuçlarla, düşük serum seruloplazmin düzeyi Wilson hastalığı tanısı ve aile taramasında en iyi testlerden biri olmasına rağmen, normal serum seruloplazmin düzeyi tanıyı ekarte ettirmez. Bu hastaların çoğunda şelatör tedavi seyrinde seruloplazmin düzeyleri anlamlı olarak azalmaktadır. Tanı aşamasında, serum seruloplazmin düzeyi normal ise, şüpheli vakalarda bozulmuş bakır metabolizmasının göstergesi diğer bir test ile yapılmalıdır.

Portal vein thrombosis, which is seen during coagulopathies, vasculitis or myeloproliferative diseases, causes extrahepatic presinusoidal portal hypertension. Paroxysmal nocturnal hemoglobinuria usually causes thrombosis in hepatic veins, but it also rarely causes portal vein thrombosis. A case of paroxysmal nocturnal hemoglobinuria due to portal vein thrombosis is presented., Portal ven trombozu, koagülopatiler, vaskülit veya miyeloproliferatif hastalıkların seyrinde görülen, ekstrahepatik presinüzoidal portal hipertansiyon nedenidir. Paroksismal nokturnal hemoglobinüri, daha çok hepatik venlerde tromboza neden olmakla birlikte, portal ven trombozunun nadir nedenleri arasındadır. Burada sebebi paroksismal nokturnal hemoglobinüri olan bir portal ven trombozu olgusu sunuldu.

Long-term safety of treatment with hepatitis B virus (HBV) polymerase inhibitors is a concern. Adefovir dipivoxil (ADV) therapy has previously been associated with impairment of renal function. Limited data are available on the safety of combination therapy with nucleos(t)ide analogues and interferon alfa (IFNa). The aim of this analysis was to assess the renal function during combination therapy with peginterferon alfa-2a (PegIFNa-2a) plus ADV vs either drug alone in patients with hepatitis B/D co-infection. We performed a retrospective analysis of renal function data of patients treated in the Hep-Net/International Delta Hepatitis Intervention Trial 1(HIDIT-1-trial), a European multicenter study to investigate the efficacy of 48 weeks of therapy with PegIFNa-2a+ADV vs either drug alone in 90 patients with chronic hepatitis B/D co-infection. Glomerular filtration rates (GFR) were calculated by CockcroftGault (CG), abbreviated Modification of Diet in Renal Disease (MDRD) study and Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) equation. After 48 weeks of therapy GFR values were significantly lower in patients receiving adefovir-containing treatment vs PegIFNa-2a alone [mean difference 16.1 mL/min (CG) and 10.2 mL/min (MDRD), respectively, P < 0.05] while no differences were observed between patients receiving adefovir alone vs combination treatment. Twenty-four weeks after treatment GFR values did not differ between treatment arms. A decrease in GFR =20% was observed more often in patients during adefovir-containing treatment vs PegIFNa-2a alone (P < 0.05) which was confirmed by KaplanMeier analysis. Adefovir-containing but not PegIFNa-2a treatment was associated with a decrease in GFR values in about one-fifth of patients. Combination treatment of PegIFNa-2a+ADV in chronic hepatitis B/D co-infection did not lead to any further impairment of kidney function. Hep-Net Study House Federal Ministry of Education & Research (BMBF) Hoffmann-La Roche Gilead Sciences

Hepatitis delta virus (HDV) infection causes the most severe form of viral hepatitis. PEG-interferon alpha-2a (PEG-IFN alpha-2a) is the only effective treatment but its long-term clinical impact is unclear. the aim of this study was to investigate the long-term outcome after 48 weeks of pegylated interferon alpha-2a therapy. We performed a retrospective follow-up study of the Hep-Net-International-Delta-Hepatitis-Intervention-Study 1 (HIDIT-I trial). Patients had received 48 weeks of treatment with either PEG-IFN alpha-2a plus adefovir dipivoxil (ADV) (Group I), PEG-IFN alpha-2a alone (Group II) or adefovir dipivoxil alone (Group III). Liver-related complications were defined as liver-related death, liver transplantation, liver cancer and hepatic decompensation defined as development of Child-Pugh scores B or C or an increase in Model for End-stage Liver Disease (MELD) scores of five or more points in relation to baseline values. Patients were considered for further analysis when they were retreated with PEG-IFN alpha-2a. Follow-up data (at least 1 visit beyond post-treatment week 24) were available for 60 patients [Group I, (n = 19), Group II (n = 20), Group III (n = 21)]. Mean time of follow-up was 8.9 (1.6 - 13.4) years. 19 patients were retreated with IFN-based therapy: 42% (n = 8) in PEG-IFN alpha-2a arms and 58% (n = 11) in the adefovir only arm. Clinical complications on long-term follow-up occurred in 17 patients and were associated with nonresponse to therapy and baseline cirrhosis. the annual event-free survival rate in patients with cirrhosis vs noncirrhotic patients at year 5 and 10 was 70% vs 91% and 35% vs 76%. Long-term follow-up of a large randomized clinical trial suggests that off-treatment HDV RNA response to PEG-IFN alpha-2a treatment leads to improved clinical long-term outcome., German Centre for Infection Research (DZIF), partner site Hannover-Braunschweig; German Liver Foundation; EASL registry grant, This study was funded by the German Centre for Infection Research (DZIF), partner site Hannover-Braunschweig with a grand to the HepNet Study-House, the German Liver Foundation and an EASL registry grant.

A 28-year-old man with compensated cirrhosis of the liver (Child B) and after 4 episodes of esophageal variceal bleeding received prophylactic endoscopic variceal sclerotherapy in our Gastroenterology Clinic for 8 consecutive months. Sclerotherapy of the esophageal varices had been performed at monthly intervals until variceal obliteration was achieved. Both the intravariceal and paravariceal injection techniques were used and injections were repeated periodically as necessary. On the 8th month, 1 week after the 4th sclerotherapy procedure, the patient complained of swelling on his right shoulder and on his right arm. There was jugular congestion and swelling of his right arm and right shoulder. The patient was hemodynamically stable. An X-ray of brachial venography revealed an obstruction of the vena subclavia dextra. During follow-up, the jugular congestion and swelling of his right arm gradually subsided spontaneously over a 6-month period without any need for medication. There has been no recurrence of his symptoms during the 1-year follow-up period. Now, he is still well clinically. This experience suggests that endoscopic injection sclerotherapy may cause thrombosis of the subclavian vein which have been never seen before.