[Hemorrhagic parapneumonic effusion in a 64 year-old patient as the first symptom of hemophilia B]. / Krwisty wysiek parapneumoniczny jako pierwszy objaw hemofilii B u 64-letniego mezczyzny.

Hemophilia B is an inherited, X chromosome-linked disease. It is usually diagnosed in childhood, sometimes in adolescence. The commonest symptoms include spontaneous or post-traumatic bleeding into the joints and/or muscles, as well as mucosal bleeding. Respiratory symptoms are rarely reported. We present the case of a 64 year-old man in whom bloody parapneumonic effusion (hemothorax) was the first symptom of hemophilia B. The reason for prolonged activated partial thromboplastin time (APTT) found on admission has not been elucidated. Since antibiotic therapy and pleural tube thoracostomy with intrapleural streptokinase were found to be ineffective, video-assisted thoracic surgery was performed with the right lung decortication. Post-operative treatment was complicated by massive pleural bleeding requiring two subsequent thoracotomies. Additional blood tests revealed factor IX deficiency and resulted in hemophilia B being diagnosed. The presented case proves that hereditary bleeding disorders may be diagnosed even in late adulthood. Intrapleural bleeding related to pneumonia and pleural inflammation might be the first presenting symptom. Hemophilia should be considered as a potential cause of APTT prolongation, even in an elderly patient with atypical presentation. Explaining the reason for APTT prolongation before the surgical procedure could have allowed to avoid severe bleeding in the described patient.

Persisted/recurrent hyperparathyroidism associated with development of multi-drug resistance phenotype and proliferation of parathyroid transplants.

The surgical treatment of secondary hyperparathyroidism (HPTH) requires sub-total excision of parathyroid glands or total excision with their autotransplantation. Although this approach has been considered as a safe method of treatment, in this report we describe persisted/recurrent HPTH after parathyroid transplantation. Due to parathormone (PTH) hypersecretion and uncontrolled proliferation, the parathyroid grafts were removed and used for generation of cell cultures, which further have been subjected to in vitro studies. As a control we used parathyroid tissue, obtained during multiorgan harvesting. We found increased proliferation and up-regulated PTH production by the graft-derived, but not control in vitro cultured cells. Moreover, due to decrease of in vivo radiotracer uptake by parathyroid grafts, the expression of multi-drug resistance-involved factors, including P-glycoprotein (P-gp/mdr1), multi-drug resistance-associated protein (mrp) and bcl-2 have been investigated using RT-PCR. The analysis revealed increased expression of both, mdr1 and mrp in graft-derived cells, in contrast to control cells, which did not express P-gp/mdr1 or mrp. However, we did not observe any difference in expression of bcl-2 between analyzed cells. The up-regulated expression of P-gp/mdr1 on graft-derived cells was further confirmed by immunofluorescence studies. The described case indicates potential risk associated with transplantation of parathyroid tissue. Our results confirm a role of MDR phenomenon in occurrence of false negative results in parathyroid tissue scintigraphy studies. Moreover, they indicate that standard histological examination of transplanted material could not be sensitive enough to exclude any potential danger of abnormal graft progression. Thus, they could support the concept to use encapsulated parathyroid transplants.

Surgical treatment of hyperinsulinism during the course of pancreatic cancer (insulinoma) - one center experience.

UNLABELLED: Endogenic hyperinsulinism is mainly caused by neuroendocrine tumors (insulinomas) which autonomously secrete insulin. Because the symptoms are often aspecific, a considerably delay in diagnosis occurs. The treatment consists of operative removal of the tumor from the pancreas, preceded by pre-operative localization. In this article we describe our experience with surgical removal of insulinomas. MATERIAL AND METHODS: We retrospectively analyzed all patients with insulinoma which were treated in our center. Definitive diagnosis was made using a 72-hours glucoses fasting test. We describe the symptoms, localization techniques and the outcomes after surgery. RESULTS: Between January 2002 and May 2011, 45 patients (35.6% men and 64.4% female) were treated in our center. The most prevalent symptoms were altered consciousness and general malaise. The combination of CT-scan and endoscopic ultrasound had the highest (90%) sensitivity to localize tumors pre-operatively. During surgery, in 40 patients (89%) the tumor could be removed by enucleation. In the other five patients partial pancreas resection was required. In 22 patients (49%) we used intra-operative insulin level measurements to confirm complete tumor resection. Within the first month after surgery, two patients (4.4%) developed acute pancreatitis, four patients (8.8%) developed a pancreatic fistula. One patient died of multi-organ-failure. All patients were free from symptoms of hyperinsulinism after the surgery and after a median follow-up of 4.5 years. CONCLUSIONS: Based on the experience with 45 patients, surgical removal, aided by pre-operative localization with CT and endoscopic ultrasonography, is an effective and safe treatment for insulinomas.