Sclerotherapy of esophageal varices in hemophilia patients with liver cirrhosis - a prospective, controlled clinical study.

INTRODUCTION: Bleeding from esophageal varices is a serious clinical condition in hemophilia patients due to congenital deficiency or lack of clotting factors VIII (in hemophilia A) and IX (in hemophilia B), decreased clotting factor II, VII, IX, X synthesis in the course of chronic liver disease and hipersplenic thrombocytopenia. The aim of this study was to assess the efficacy and safety of endoscopic sclerotherapy in acute esophageal variceal bleeding and in secondary prophylaxis of hemorrhage. The aim was also to investigate the optimal activity of deficiency factors VIII or IX and duration of replacement therapy required to ensure proper hemostasis after sclerotherapy procedures. MATERIAL AND METHODS: 22 hemophilia patients (A-19, B-4) with coexistent liver cirrhosis and active esophageal variceal bleeding treated with endoscopic sclerotherapy were subjected to prospective analysis. The patients who survived were qualified to repeated sclerotherapy procedures every 3 weeks within secondary prophylaxis of bleeding (investigated group). A 3-day substitution therapy enhanced the infusion of the deficient or lacking factor in doses allowing to reach 80-100% of normal value activity of factor VIII on the 1st day and 60-80% in the next two days. The desired activity of factor IX was 60- 80% and 40-60% respectively. The control group consisted of 20 non-hemophiliac patients with liver cirrhosis comparable in terms of age, sex, stage of advancement of liver cirrhosis, who underwent the same medical proceedings as the investigated group. RESULTS: Active esophageal bleeding was stopped in 21 of 22 (95%) hemophilia patients. Complications were observed in 3 patients; 2 patients died. The rate of hemostasis, complications and deaths in the control group were comparable and no statistical differences were found. In hemophilia patients subjected to secondary prophylaxis of hemorrhage, in 18 of 20 (80%), complete eradication of esophageal varices was achieved after 4 to 7 sclerotherapy procedures in 1 patient (average 5.4). Recurrent bleeding was observed in 15% of patients, complication in 20%; 1 patient died. Time lapse from bleeding to eradication was 12-21 weeks (average 15.2). In the control group the rate of variceal eradication, complication and deaths was comparable and no statistical differences were found. The usage of factor VIII concentrates was as follows: in hemophilia A, in a severe form - 80.9 U/kg b.w./day, in hemophilia A in a severe form with an inhibitor <5 BU - 95.2 U/kg b.w./day, in mild form - 64.2 U/kg b.w./day and in severe hemophilia B - 91.6 U/kg b.w./day. CONCLUSIONS: Sclerotherapy is an effective method in the management of esophageal variceal bleeding in hemophilia patients. It is also effective for total eradication of varices when applied as a secondary prophylaxis of hemorrhage. In our opinion, a 3-day replacement therapy at the applied doses is sufficient to ensure hemostasis and avoid bleeding complications.

Splenic abcesses as infectious complication following implantation of left ventricular asssist device - case report.

Left ventricular assist device (LVAD) is one of the modern management therapies in patients with advanced heart failure, and it serves as a bridge to heart transplantation or even as destination therapy. However, it is burdened with a high risk of thromboembolic, hemorrhagic, and infectious complications despite prophylactic management. Splenic abscesses, as septic complications following implantation of mechanical ventricular support, have not yet been described in the literature. We report of a patient with severe left ventricular insufficiency (NYHA II/III), pulmonary hypertension, and arrhythmia who underwent implantation of the Heart Ware® pump for left ventricular support with simultaneous tricuspidvalvoplasty, as a bridge therapy to heart transplantation. During two years after LVAD implantation, the patient had three MRSA skin infections, localized at the exit site of the drive-line connecting the artificial ventricle with external unit, that were complicated by sepsis and treated with broad-spectrum antibiotics. A few months later, abdominal CT revealed two abscesses in the spleen, and the patient was qualified for splenectomy. Open splenectomy was performed under full-dose anticoagulant therapy with continuous intravenous infusions of unfractionated heparin (UFH). The intra- and postoperative course was uneventful. UFH therapy was continued for 6 days, and oral anticoagulation was re-administered on day 4 after surgery. The patient was discharged on day 7 after surgery with primary healed wound. Open splenectomy, performed with full-dose anticoagulant therapy, proved to be an effective and definitive method of treatment without any complications.

Laparoscopic nephrectomy in a hemophilia B patient.

Surgery in patients with hemophilia is a serious challenge. It requires a comprehensive approach, as well as careful postoperative monitoring. We present here the first case of a transperitoneal laparoscopic radical nephrectomy (TLRN) for renal cell carcinoma, of the clear-cell type, performed in a hemophilia B patient. The level of factor IX clotting activity before surgery and on postoperative days 1-6 was maintained at 65-130% and at 30-40% on subsequent days until healing of the post-operative wound was achieved. The intraoperative and postoperative courses were uneventful. TLRN can therefore be considered safe and effective for renal cell carcinoma. In hemophilia patients, the TLRN procedure requires proper preparation, as well as adequate substitution therapy for the deficient coagulation factor provided by a multidisciplinary team in a comprehensive center.

[Excision of the rectum due to carcinoma in patient with hereditary hemorrhagic teleangiectasia]. / Wyciecie odbytnicy z powodu raka u chorej z wrodzona naczyniakowatoscia krwotoczna.

UNLABELLED: Hereditary hemorrhagic telangiectasia (HHT) also known as Rendu-Osler-Weber disease, is a rare, autosomal dominant disorder of the fibrovascular tissue. Clinically, it is characterized by the triad of symptoms of mucocutaneous telangiectasias and arteriovenous malformations of visceral organs, recurrent hemorrhages from vascular changes at different localization, and familial occurrence. A coexistence of HHT syndrome and malignant neoplasms in various sites, including large bowel, is suggested. The authors describe the case of 67-year old woman with HHT with massive bleeding from digestive tract in history and persistent chronic sideropenic anemia. In upper gastrointestinal endoscopy performed in regional hospital multiple telangiectasias in the stomach were found. These were diagnosed as the possible bleeding source therefore colonoscopy was not performed. After twelve months in diagnostic colonoscopy due to chronic anemia carried out in our Institute, rectal carcinoma was found. The patient were subjected to surgery--lower anterior excision of the rectum. Intra operatively and in the postoperative period complications were not observed. CONCLUSION: In every case of bleeding from digestive tract and also in chronic anemia in patients suffering from Rendu-Osler-Weber disease, beside the endoscopy of esophagus, stomach and duodenum, full visualization of the large bowel is necessary to exclude malignant neoplasm. In the presented case no colonoscopy performed in the regional hospital delayed the carcinoma diagnosis by twelve months.

[Nonoperative management of spontaneous splenic rupture in infectious mononucleosis]. / Leczenie nieoperacyjne samoistnego pekniecia sledziony w przebiegu mononukleozy zakaznej.

UNLABELLED: Spontaneous splenic rupture is a rare complication of infectious mononucleosis observed in 0.1-0.5% of patients with this condition. Mandatory mode of management in hemodynamically stable patients is nonoperative treatment. We report the case of a 19-year old man with splenic rupture, during the course of serological and hematological confirmed infectious mononucleosis, with no history of trauma. Parenchymal and subcapsular splenic hematomas and presence of blood in vesico-rectal recess was demonstrated. Circulatory and respiratory findings and blood cell count were stable. Nonoperative management was instituted which comprised monitoring of valid vital signs, serial USG and tomography scans and vital activity limitation. Imaging radiological investigations demonstrated disappearance of observed abnormalities on post admission day 20. The patient was discharged from the hospital in good general condition. CONCLUSION: Nonoperative management can be a safe alternative to splenectomy in hemodynamically stable patient with spontaneous rupture of the spleen.