1/9. Arterial anastomosis disrupton in two kidney recipients of contaminated grafts from a donor with Gorham's syndrome.Transmission of donor infections to immunosuppressed recipients may produce serious complications. Here, we report two cases of ruptured renal artery pseudoaneurysm within a few months after renal transplantation from a donor with Gorham's syndrome, a rare disease characterized by proliferation of vascular and lymphatic channels associated with extensive bony destruction. The donor had died of respiratory failure, sepsis, and anoxic brain death due to difficult airway control secondary to a maxillofacial deformity.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/9. Gorham-Stout syndrome in a male adolescent-case report and review of the literature.Gorham-Stout syndrome is a rare disease characterized by vascular proliferation in the bones, effecting osteolysis. When it is complicated by chylothorax, the prognosis is poor. The present case illustrates successful management of chylothorax by a combination of surgery (thoracic duct ligation and excision of lymphangiomatous tissue combined with pleurodesis) and treatment with alpha-2b interferon. Treatment in 38 published cases is reviewed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/9. The hajdu-cheney syndrome: a case report and review of the literature.hajdu-cheney syndrome which is also known as type VI idiopathic osteolysis is a rare disease transmitted autosomal dominantly. In this syndrome, osteolysis involves primarily the terminal phalanges. We describe here a 18-year-old boy with typical clinical and radiological signs of hajdu-cheney syndrome.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/9. Massive osteolysis of the maxillofacial bones. Report of two cases.Two cases of massive osteolysis were encountered, one affecting the mandible and the other the maxilla and mandible. Only 13 cases have been reported so far in the existing literature for massive osteolysis of the mandible. It is indeed a rare disease. Our two cases were surgically treated and were clinically and histopathologically assessed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/9. radiotherapy of Morbus Gorham-Stout: the biological value of low irradiation dose.Morbus Gorham-Stout is a very rare disease. A patient with this disease was treated at the University Clinic of radiobiology in Vienna. The clinical and radiological findings place special emphasis on the radiotherapeutic treatment, and the biological basis of the recommended dose is discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/9. Massive osteolysis of the mandible.This report deals with a massive osteolysis, which is a rare disease characterized by the progressive dissolution of contiguous osseous structure, occurring in the mandible of a 46-year-old Japanese female. Histopathologic examination of the amputation material revealed that the mandibular body, ramus, condylar and coronoid processes were completely replaced by fibrous tissue, but inferior alveolar nerve and artery in the lesion were intact. A few small islands of osteoid tissue or immature fibrous bone were seen in the lesion. In the transitional area between the lesion and normal bone, active absorption of bone trabeculae was in progress and intertrabecular spaces were occupied by densely packed fibroblastic cells, however, angiomatous proliferation of vessels was not observed. A few bone trabeculae were surrounded by osteoclasts, but osteoclasts might not play a primary etiological role in massive osteolysis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/9. The hajdu-cheney syndrome. A review of the literature and report of 3 cases.The hajdu-cheney syndrome or acro-osteolysis syndrome is a rare disease. Only 18 well-documented cases are described in the literature. Presentation of cases in the oral surgical literature is rare. The syndrome is characterized by dissolution of terminal phalanges of the hands and feet, dolichocephaly, open cranial sutures, multiple wormian bones, absence of frontal sinuses, wide open sella turcica, progressive basilar invagination, early loss of teeth, short stature and characteristic facies. Inheritance is most likely autosomal dominant. 3 patients are presented, 2 of them are mother and son, the latter (case 2) being the youngest patient reported to date. The parents of the 3rd patient were consanguineous, raising the possibility of genetic heterogeneity. Dental, surgical and genetic aspects are discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/9. Progressive teleangiomatous osteolysis. Case report.Progressive teleangiomatous osteolysis (P.T.O.) is a very rare disease, particularly when one recalls that only five cases have been recorded at the Rizzoli Institute (Campanacci, 1981). The aetiopathogenesis is unknown and according to Campanacci it is not clear whether it is attributable to an angiomatous condition or to a post-traumatic dystrophy. A typical case is presented, localised in the thoracic cage and complicated by severe haemothorax. The patient was observed over a period of 17 years and made a complete recovery.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/9. chylothorax in Gorham's syndrome. A common complication of a rare disease.One hundred forty-six cases of Gorham's syndrome are documented in the literature. Twenty-five (17 percent) patients have been reported to have chylothorax as a complication. To our knowledge, this high incidence of chylothorax has not been reported previously. We describe two such patients treated by thoracic duct ligation and compared our results with others to support early surgical intervention via a low right-sided thoracotomy in these cases.- - - - - - - - - - ranking = 4keywords = rare disease (Clic here for more details about this article) |