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  Лечение на синдрома на ...  
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Човешки хетероложни стволови клетки от черен дроб
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Heterologous human adult liver derived stem cells
  Системен ксантогранулом...  
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Системният юношески ксантогранулом е рядко заболяване при деца. Статията описва случай на дете, при което са засегнати бъбреци, бял и черен дроб. При него е имало белодробна инвазия, маса в бъбреците и възлести чернодробни нарушения, но без засягане на костите.
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, from the Department of Pediatrics, Division of Pediatric Hematology and Oncology, Istanbul University, Istanbul Medical Faculty, Istanbul, Turkey. Systemic juvenile xanthogranuloma is a rare disease in children. A 10-year-old boy who showed renal, pulmonary, and liver involvement is reported. He had pulmonary invasion, renal mass, and nodular liver lesions but no bone involvement. The diagnosis was confirmed by renal biopsy. The patient was treated with pulse high-dose methylprednisolone. To access the full abstract of the article, click here.
  Хистоцитоза X - Институ...  
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PubMed, порталът за биомедицинска и научна литература индексира интересна статия на тема „Засягане на черен дроб при хистоцитоза на лангерхaнсовите клетки: клиничен случай“ (J Gastrointestin Liver Dis.
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PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Liver involvement in Langerhans’ cell histiocytosis: case report” (J Gastrointestin Liver Dis. 2006 Mar;15(1):57-9). Authors are Dina I, Copaescu C, Herlea V
  Хистоцитоза X - Институ...  
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Хистоцитоза на лангерхaнсовите клетки (Langerhans’cell histiocytosis, Histiocytosis X) е рядко заболяване с неизвеста етиология, което се характеризира с олигоклонална пролиферация на клетките на Langerhans. Най-често се проявява при деца и младежи като засяга една или повече системи, като напр. кости, хипоталамус, задна част на хипофизата, лимфни възли, черен дроб и други меки тъкани.
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, from the Medical Clinic, St. Ioan Hospital, Bucharest, Romania. Langerhans’cell histiocytosis (Histiocytosis X) is a rare disease of unknown cause characterized by oligoclonal proliferation of Langerhans cells. It occurs mostly in children and young adults and involves one or more body systems such as bone, hypothalamus, posterior pituitary gland, lymph nodes, liver or various soft tissues. The diagnosis is always made by a histological approach. The authors report a case of Langerhans’cell histiocytosis in a young patient with clinical signs of diabetes insipidus. To access the full abstract of the article, click here.
  Болест на Гоше - Инстит...  
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Дължи се на дефицит на ензима глюкоцереброзидаза, което води до натрупване на липида глюкоцереброзид в лизозомите на макрофагите, наричани още „клетки на Гоше”. Заболяването се характеризира с анемия, тромбоцитопения, уголемен черен дроб и слезка, увреждания на костите, изоставане в растежа.
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A new disease profile is added to our database – Gaucher disease. Gaucher disease is the most common disorder among the lysosomal storage diseases. It has an autosomal recessive way of inheritance. The deficiency of the enzyme gluco cerebrosidase causes accumulation of the lipid glucocerebrosid into lysosomes of the macrophages, also called “Gaucher cells”. The disease is characterized by anemia, thrombocytopenia, hepatosplenomegaly, bone changes, growth and mental delay. When untreated it leads to irreversible disability and early mortality. The causative gene for the three types of the disease,
  Папиломатоза на билиарн...  
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, от Department of Transplant Surgery, Aristotle University, Hippokration Hospital, Thessaloniki, Greece. В световната литература са описани около 60 случая на билиарна папиломатоза, като само 6 са лекувани с трансплантация на черен дроб.
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, from the Department of Transplant Surgery, Aristotle University, Hippokration Hospital, Thessaloniki, Greece. Approximately 60 cases of biliary papillomatosis have been reported in the world literature, while only 6 cases have been reported to be treated with liver transplantation. This rare disease, which is characterized by relapsing episodes of obstructive jaundice and cholangitis that lead to secondary cirrhosis and death from sepsis or liver failure, it is also considered premalignant because of its frequent malignant transformation (25-50%). The authors present a case of a 43-year-old white man with papillomatosis of intra- and extrahepatic biliary tree who sought care for repeated episodes of obstructive jaundice and cholangitis. The patient underwent orthotopic liver transplantation with Roux-en-Y hepatico-jejunostomy to treat end-stage liver cirrhosis. To access the full abstract of the article, click here.
  Анестезия и туберозна с...  
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Отличава се с класическа триада от симптоми – епилепсия, кожни увреди и умствено изоставане. Туберозната склероза причинява хамартоми в множество органни системи вкл. мозък, кожа, сърце, бъбреци, бели дробове и черен дроб.
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PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled ”Anesthesia concerns for children with tuberous sclerosis” (AANA J. 2006 Jun;74(3):219-25). Authors are Septer S, Thompson ES, and Willemsen-Dunlap A from the Anesthesia Associates of Kansas City, Kan, USA. Tuberous sclerosis (TS) is a relatively rare, autosomal dominant syndrome that displays high genetic penetrance in affected families. It is identified by a classic triad of symptoms including epilepsy, skin lesions, and mental retardation. Tuberous sclerosis causes hamartomas in multiple organ systems, including the brain, skin, heart, kidneys, lungs, and liver. Awareness of the signs and symptoms and the organs involved is critical to provide safe and effective anesthesia care. The authors describe a 10-year-old girl with TS scheduled to receive a general anesthetic for laser treatment of facial angiofibromas. To access the full abstract of the article, click here.
  Синдром на Alagille - И...  
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Синдромът на Alagille (Alagille syndrome, AGS) е мултиситемно нарушение, чийто основни клинични прояви са холестаза, която се характеризира със стеснение на жлъчния канал при биопсия на черен дроб; вродена сърдечносъдова недостатъчност; заден ембриотоксон в окото; характерен лицеизраз и прешлени тип “пеперуда”.
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A new disease profile is added to our database – Alagille syndrome. Alagille syndrome (AGS) is a complex multisystem disorder with major clinical manifestations being cholestasis, characterized by bile duct paucity on liver biopsy; congenital cardiac defects; posterior embryotoxon in the eye; typical facial features; and butterfly vertebrae. About 94% of the cases are caused by mutations in JAG1 gene. Mutations in NOTCH2 are associated with some instances of Alagille syndrome. The prevalence of Alagille syndrome has been estimated to be 1 in 70 000 live births. It is a multisystem disorder with highly variable presentation, even within patients of one family. To receive a full description of this rare disease absolutely free of charge, please click here and send us a request. The profile was written by our consultant in pediatrics – Prof. Ivan Ivanov, MD, PhD.
  Публикации Archives - С...  
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Всички липодистрофии са асоциирани с дисметаболитни алтерации с инсулинова резистентност, изменена глюкозна толерантност или диабет и хипертриглицеридемия, водеща до риск от остър панкреатит. Хроничните усложнения от диабета засягат ретината, бъбреци и нервна система, кардиоваскуларни усложнения и стеатозни увреди на черен дроб, които могат да доведат до цироза.
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, from the Inserm; Faculte de medecine, universite Pierre-et-Marie-Curie-Paris-VI; Service de biochimie, APHP, hopital Tenon, Paris, France. Primary lipodystrophies represent a heterogeneous group of very rare diseases with a prevalence of less than 1 case for 100.000, inherited or acquired (for eg. Barraquer-Simons syndrome), caracterized by a loss of body fat either generalized or localized. All lipodystrophies are associated with dysmetabolic alterations with insulin resistance, altered glucose tolerance or diabetes and hypertriglyceridemia leading to a risk of acute pancreatitis. Chronic complications are those resulting from diabetes involving the retina, kidney and nerves, cardiovascular complications and steatotic liver lesions that could result in cirrhosis. Treatment of diabetes, dyslipidemia and complications involves the classical intervention strategies. Therapeutic trials with recombinant human leptin in patients with very low leptin levels reported good results with respect to the metabolic and liver alterations. The prognosis is linked to the precocity and severity of the diabetic, cardiovascular and liver complications. To access the full abstract of the article, click here.
  Публикации Archives - С...  
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Нейният билиарен кистаденом е излседван хистологично. Ултрасонографията и CT са основните методи за диагностициране на муцинозен билиарен кистаденом на черен дроб. За да прочетете пълното резюме на статията, натиснете тук.
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, from the Department of General Surgery, First Affiliated Hospital, China Medical University Shenyang, China. Biliary cystadenoma is a very rare cystic neoplasm of the liver. Its clinical features, diagnosis, pathologic characteristics, and optimal surgical management have not been defined clearly. In this article the describe the details of this rare disease. A 40-year-old woman with a mass of the liver was verified by ultrasonography and LT. The patient received successfully a left hepatectomy. After the left hepatectomy, the patient was fully recovered. Her biliary cystadenoma was characterized by specific histological findings. Ultrasonography and CT are the major methods for the diagnosis of mucinous biliary cystadenoma liver. Operation is the best way of treatment. To access the full abstract of the article, click here.