PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “A case of a patient with cognitive impairment and gait disturbance.” (Tidsskr Nor Laegeforen. 2007 May 3;127(9):1210-1). Authors are Ruud KJ, Scheie D and Kerty E, from the Nevrologisk avdeling, Rikshospitalet-Radiumhospitalet, Norway. Intravascular lymphomatosis is a rare disease, characterized by occlusion of small arterioles and veins by malignant lymphoma cells. It often affects the central and peripheral nervous system and the skin. This case report illustrates the diagnostic challenge. Most patients develop rapidly progressive dementia combined with focal neurological symptoms. No relevant clinical, radiological or laboratory tests are available to diagnose the disease. Blood samples and bone marrow are usually normal. The prognosis is poor, but early aggressive chemotherapy can potentially prolong the life span and in a few cases be curative. 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 “High-dose methotrexate and primary central nervous system lymphoma.” (J Neurosci Nurs. 2007 Apr;39(2):83-8). Authors are Fahey JB and DiMaggio C, from the Massachusetts General Hospital, Boston, MA, USA. Primary central nervous system lymphoma (PCNSL) is a rare disease that is managed differently from other primary brain tumors and other types of systemic lymphomas. The use of high-dose methotrexate (HD-MTX) has improved survival rates. This article describes the experience of a patient treated with HD-MTX and outlines the essential aspects of care for the bedside practitioner. 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 “Postembolic pulmonary hypertension.” (Rev Mal Respir. 2007 Apr;24(4):497-508). Authors are Jais X, Dartevelle P, Parent F, et al., from the Centre de Reference de l’Hypertension Arterielle Pulmonaire, Service de Pneumologie et Reanimation Respiratoire, Unite Propre de Recherche de l’Enseignement Superieur EA2705, Hopital Antoine-Beclere, Universite Paris-Sud, Assistance Publique-Hopitaux de Paris, Clamart, France. Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease characterized by the persistence of thromboemboli obstructing the pulmonary arteries as an organized tissue. The consequence is an increase in pulmonary vascular resistance resulting in pulmonary hypertension (PH) and progressive right heart failure. It is difficult to recognize the postembolic nature of PH because there is no known history of thromboembolic disease in more than 50% of cases. 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 “Schnitzler syndrome: Treatment failure to rituximab but response to anakinra.” (J Am Acad Dermatol. 2007 Apr 27). Authors are Eiling E, Moller M, Kreiselmaier I, et al., from the From the Department of Dermatology, University of Kiel. Schnitzler syndrome is a rare disease characterized by a chronic urticarial rash and a monoclonal gammopathy. The exact pathogenesis is still uncertain and treatment remains a challenge. The authors report a patient who was only recently given the diagnosis of Schnitzler syndrome although the typical symptoms had been present for about 6 years. Administration of the B-cell antibody rituximab did not exert any beneficial effect despite effective elimination of B cells and a reduction of the paraprotein. In contrast, injection of the interleukin-1-receptor antagonist anakinra caused a complete remission within a few days. To access the abstract of the article, click here.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Bone and soft tissue tumors: diagnostic principles.” (Wien Med Wochenschr. 2007 Jan;157(1-2):21-6). Authors are Leithner A and Windhager R from the Universitatsklinik fur Orthopadie, Medizinische Universitat Graz, Graz, Osterreich. Malignant bone and soft tissue tumors are rare diseases. In case of a suspected tumor one should strictly apply the algorithm proposed, in order to avoid misdiagnoses and mistreatment. Optimal therapy can only be achieved by close cooperation between the departments involved – oncology, orthopaedic surgery, pathology, plastic surgery, radiology and radiooncology. To access the abstract of the article, click here.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Garre’s chronic diffuse sclerosing osteomyelitis of the sacrum.” (Br J Neurosurg. 2006 Dec; 20(6):415-9). Authors are Nasir N, Aquilina K, Ryder DQ et al., from the Department of Neuropathology, Cork University Hospital, Wilton, Cork, Ireland. Garre’s chronic diffuse sclerosing osteomyelitis (DSOM) is a rare disease that occurs most commonly in the mandible. The authors present a case of sacral DSOM that simulated an expanding destructive sacral tumour. Treatment was conducted on the basis of the available experience with the mandibular form of the disease, with partial symptomatic relief, but progressive sclerosis of the sacral lesion. To the best of their knowledge, this is the first case initially presenting in the sacrum. 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 “Therapeutic Strategies in the Management of Acute Myeloid Leukemia.” (Med Klin (Munich). 2007 Apr;102(4):309-316). Authors are Fiegl M, Hiddemann W and Braess J. Acute myeloid leukemia (AML) is a rare disease of the hematopoietic stem cell leading to uncontrolled proliferation of immature progenitor cells. This results in a replacement of healthy hematopoiesis and in pancytopenia with corresponding symptoms (anemia, thrombo- and granulocytopenia). Diagnosis can reliably be confirmed by bone marrow aspiration, which also allows risk stratification by cytogenetic and molecular analysis. Therapy of AML that should preferentially be performed in clinical studies comprises induction therapy for achievement of complete cytomorphological remission (CR) and postremission strategies consisting of consolidation and maintenance therapy for eradication of residual blasts. 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 “Asymptomatic primary malignant melanoma of the esophagus.” (Onkologie. 2007 Apr;30(4):206-8). Authors are Gerger A, Smolle-Jutter FM, Samonigg H, et al., from the Klinische Abteilung fur Onkologie, Medizinische Universitatsklinik, Graz, Austria. Primary malignant melanoma of the esophagus is an exceedingly rare disease. This tumor is typically aggressive and disseminates early via the lymphatics and the bloodstream with a mean survival time between 10 and 15 months after radical surgical resection. The role of chemotherapy and immunotherapy is unclear. The authors present a case of a 78-year-old man came for a checkup with a medical history of reflux esophagitis and chronic gastritis. Esophagogastroscopy showed a bluishgray tumor of the esophagus, and histology revealed features consistent with malignant melanoma. The patient underwent total transhiatal esophagectomy with curative intention, and esophagogastric anastomosis was performed. 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 “Pitfalls in the management of Cushing’s disease.” (J Clin Neurosci. 2007 May;14(5):401-8). Author is Bademci G, from the Department of Neurosurgery, University of Kirikkale, Kirikkale, Turkey. Cushing’s disease is caused by functional corticotroph adenomas of the pituitary gland. Transsphenoidal microsurgery is an effective means of control for patients with adrenocorticotrophic hormone-producing microadenomas. The major causes of surgical failure in the treatment of Cushing’s disease lies in inadequate preoperative evaluation, unsuccessful identification of the adenoma and inexperience of the surgeon. For optimal results in this rare disease, endocrinological, radiological and surgical procedures should be co-ordinated in a specialized center. In this review, factors affecting preoperative evaluation, surgical success and outcome are outlined in the light of current knowledge. 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 “Collecting duct carcinoma associated with renal stones.” (Prog Urol. 2007 Feb;17(1):105-7). Authors are Kourda N, El Attat R, Derouiche A, et al., from the Service d’Anatomie et de Cytologie Pathologiques, Hopital Charles Nicolles, Tunis, Tunisie. Collecting duct carcinoma is an extremely rare disease, representing less than 1% of all renal tumours. The authors report the case of a 72-year-old patient presenting with right low back pain associated with episodes of total macroscopic haematuria. Imaging showed a heterogeneous mass in the upper pole of the right kidney associated with pyelocaliceal stones. Multiple secondary lesions were observed in the liver. Macroscopically, the mid-renal tumour was 7.5 cm in diameter surrounding the stone-containing pyelocaliceal cavities. This tumour had spread to the cortex and invaded the perirenal fat. The diagnosis of Fuhrman grade 3 collecting duct carcinoma associated with renal stones and liver metastases was adopted. The main differential diagnosis was urothelial carcinoma with a glandular component. In the present case, the diagnosis was made more difficult by the concomitant presence of renal stones. To access the full abstract of the article, click here.