PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Surgical outcome in fibrodysplasia ossificans progressiva.” (Ortop Traumatol Rehabil. 2004 Oct 30;6(5):658-64). Authors are Wzietek B and Franczuk B, from the Indywidualna Praktyka Specjalistyczna, Chrzanów. Fibrodysplasia ossificans progressiva (FOP), also known as Munchmeyer’s Disease, is a rare inherited disease. By 1996 there had been no more than 200 cases described in world literature. The disease is characterized by progressive ectopic ossification and congenital deformity of the hallux. Patients with FOP require full-time nursing care. This article presents the case of a 42 age female patient, who has been suffering from FOP since early childhood. The authors describe the course of the disease, the patient’s current clinical status, the results of surgical treatment for a decubitus ulcer on the right thigh, and a review of the literature on this rare disease. To access the full abstract of the article, click here.
Publications
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Successful treatment of papillomatosis of intra- and extrahepatic biliary tree.” (Liver Transpl. 2007 Jul;13(7):1045-8). Authors are Imvrios G, Papanikolaou V, Lalountas M, et al., 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.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Primary alveolar soft part sarcoma of the lung.” (J Korean Med Sci. 2007 Apr;22(2):369-72). Authors are Kim YD, Lee CH, Lee MK, et al., from the Department of Thoracic & Cardiovascular Surgery, College of Medicine, Pusan National University, Busan, Korea. Alveolar soft part sarcoma (ASPS) is a rare epithelial-like soft tissue sarcoma. The two main sites of its occurrence are the lower extremities in adults and the head and neck in children. Primary pulmonary involvement of this sarcoma, without evidence of soft tissue tumor elsewhere, is very exceptional. The authors present a case of primary ASPS of the lung in a 42-yr-old woman. A computed tomographic scan of the thorax demonstrated a well-circumscribed, solid tumor located in the right upper lobe. The mass was resected by right upper lobectomy. To access the full abstract of the article, click here.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Successful therapy of systemic xanthogranuloma in a child.” (J Pediatr Hematol Oncol. 2007 Jun;29(6):425-7). Authors are Univar E, Devecioglu O, Akcay A et al., 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.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Necrolytic migratory erythema in glucagonoma syndrome.” (Hautarzt. 2007 Jun 5). Authors are Stark I, Mensing CH and Sander CA, from the Eduard-Arning-Klinik für Dermatologie und Allergologie, Asklepios Klinik St. Georg, Hamburg, Deutschland. The glucagonoma syndrome is a rare disease in which a typical skin lesion, necrolytic migratory erythema, is often one of the presenting symptoms. A 68-year-old woman developed erythematous polycyclic migratory lesions with advancing scaling borders and crusts over several years. Skin biopsies, laboratory studies and imaging confirmed the diagnosis of necrolytic migratory erythema as part of a glucagonoma syndrome. To access the abstract of the article, click here.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Nodular lymphoid hyperplasia of the lung.” (Ann Thorac Surg. 2007 Jun;83(6):2197-9). Authors are Sakurai H, Hada M and Oyama T, from the Department of Surgery, Yamanashi Prefectural Central Hospital, Yamanashi, Japan. Nodular lymphoid hyperplasia of the lung is a very rare uncommon disease that is considered to be a benign lesion of a polyclonal lymphoid proliferation. Because of its rarity, little is known about the clinicopathologic characteristics of nodular lymphoid hyperplasia. The authors present the case of a 67-year-old woman with nodular lymphoid hyperplasia of the lung and discuss the current knowledge concerning nodular lymphoid hyperplasias. To access the full abstract of the article, click here.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Nodular lymphoid hyperplasia of the lung.” (Ann Thorac Surg. 2007 Jun;83(6):2197-9). Authors are Sakurai H, Hada M and Oyama T, from the Department of Surgery, Yamanashi Prefectural Central Hospital, Yamanashi, Japan. Nodular lymphoid hyperplasia of the lung is a very rare uncommon disease that is considered to be a benign lesion of a polyclonal lymphoid proliferation. Because of its rarity, little is known about the clinicopathologic characteristics of nodular lymphoid hyperplasia. The authors present the case of a 67-year-old woman with nodular lymphoid hyperplasia of the lung and discuss the current knowledge concerning nodular lymphoid hyperplasias. To access the full abstract of the article, click here.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Tumoral calcinosis.” (Ann Dermatol Venereol. 2007 May;134(5):464-7). Authors are Douria L, Ismaili N, Raiss M, et al., from the Service de Dermatologie, CHU Ibn Sina, Rabat, Maroc. Tumoral calcinosis is a rare disease seen in adolescents and young adults and consisting of calcifications in periarticular soft tissue. Mutations in fibroblast growth factor 23 and GalNAc transferase 3 have been identified in the familiar forms of tumoral calcinosis. A 10 year-old boy of light phototype presented multiple calcified periarticular masses since the age of seven years as well as sciatica secondary to nerve compression. Laboratory examinations revealed hyperphosphoraemia. Histology of a cutaneous biopsy sample revealed dermal calcifications. This case of tumoral calcinosis is notable in terms of its sporadic onset in a child of light phototype and by the presence of compression of the external sciatic nerve. The diagnosis was made after ruling out other causes of soft tissue calcification. To access the full abstract of the article, click here.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Lhermitte-Duclos disease associated with Cowden syndrome.” (J Clin Neurosci. 2007 May 5). Authors are Tan TC and Ho LC, from the Department of Neurosurgery, Queen Elizabeth Hospital, Kowloon, Hong Kong. Lhermitte-Duclos disease or dysplastic gangliocytoma of the cerebellum, is a rare cerebellar lesion, which can cause mass effects in the posterior fossa. It may occur sporadically, or in association with Cowden syndrome. Cowden syndrome or multiple hamartoma-neoplasia syndrome, is an uncommon autosomal dominant condition characterized by mucocutaneous lesions and systemic malignancies. The authors report two patients with Lhermitte-Duclos disease and associated Cowden syndrome. The clinical, radiological and histopathological features and management strategies of this rare disease complex are discussed. To access the abstract of the article, click here.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Bullous pemphigoid and acquired hemophilia.” (Ann Dermatol Venereol. 2007 Apr;134(4):353-6 ). Authors are Soria A, Matichard E, Descamps V, et al., from the Service de Dermatologie, Hopital Bichat, AP-HP, Paris. Acquired hemophilia is a rare disease. Associated pemphigoid is extremely uncommon. The authors report a case of acquired hemophilia in the course of this dermatosis illustrating the severity of the disease. An 83 year-old woman was followed for 3 years for pemphigoid treated with topical corticosteroids following a recent relapse. Acquired hemophilia, as revealed by cutaneous-mucosal bleeding, is a rare disease (1 to 4 cases per million subjects) more commonly seen in adults. It is associated with the presence of antibodies directed against factor VIII. Its complications, particularly hemorrhagic, are fatal in 15 to 20% of cases. To access the full abstract of the article, click here.