PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled Heterogeneous inclusions in neurofilament inclusion disease” (Neuropathology. 2006 Oct;26(5):417-21). Authors are Uchikado H, Li A, Lin WL et al., from the Department of Neuroscience, Mayo Clinic, Jacksonville, Florida, USA. Neurofilament inclusion disease (NFID) is a rare disease, whose pathogenesis remains to be elucidated. Immunoreactivity of ubiquitin-binding protein p62 has been reported in various neurodegenerative diseases, but it has not been studied in NFID. In this report the authors show p62 immunoreactivity in neuronal perikaryon of three cases of NFID. They found inclusions in NFID to be heterogenous based on immunoreactivity for alpha-internexin, phosphorylated neurofilament-H, p62 and ubiquitin. The present study extends the list of proteins that have been identified as components of neuronal inclusions in NFID, and may helfor the pathogenesis of NFID. 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 ” Unusual presentation of 22-kilogram retroperitoneal mullerian serous cystadenoma.” (Gynecol Oncol. 2006 Oct 30). Authors are Kassab A, El-Bialy G, Clark J, et al., from the Department of Obstetrics and Gynaecology Royal Cornwall Hospital, 93 A Godrevy House, Royal Cornwall Hospital, UK. Mullerian cyst of the retroperitoneum is an extremely rare disease that is thought to be a subtype of urogenital cyst. It is a benign condition that can only be diagnosed and cured by surgical resection. An 80-year-old female with a huge cystic swelling, thought to be ovarian in origin, underwent laparotomy. Surgical exploration and subsequent histopathological analysis of the cyst revealed a mullerian serous cystadenoma of the retroperitoneum, 22 kg in weight. Preoperative ultrasound scan and computed tomography failed to elucidate a correct preoperative etiology. Huge retroperitoneal cysts may coexist with other pelvic pathology creating diagnostic and therapeutic dilemmas. 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 “Cushing’s disease” (Pituitary. 2006 Oct 30). Authors are De Martin M, Giraldi FP and Cavagnini F, from the Chair of Endocrinology, University of Milan, Ospedale San Luca, Istituto Auxologico Italiano, Milan, Italy. Cushing’s disease, i.e., pituitary ACTH-secreting adenoma causing excess glucocorticoid secretion, is a rare disease with significant mortality and morbidity. Timely diagnosis and appropriate treatment can alter the course of the disease and are therefore mandatory. First step of the diagnostic is the endogenous glucocorticoid excess by measurement of urinary free cortisol, cortisol circadian rhythmicity or suppression by low doses of dexamethasone. In patients with equivocal results, second line tests, such as the dexamethasone-suppressed CRH test and desmopressin stimulation, usually enable the diagnosis to be confirmed. The last step in the diagnostic algorhythm is often the most fraught with problems as the distinction between Cushing’s disease and ectopic ACTH secretion. Patients cured of Cushing’s disease require long-term monitoring given the risk of relapse and clinical burden of associated ailments. 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 “Peritoneal coccidioidomycosis” (Rev Gastroenterol Mex. 2005 Oct-Dec;70(4):434-8). Authors are Esmer-Sanchez D, Alfaro-Sousa A, Tostado-Fernandez FA, еt al., from the Departamento de Cirugia, Centro Medico del Potosi-Hospital Angeles de San Luis Potosi. Peritonitis is a very rare condition that has been found on inmunocompromised patients and patients on peritoneal dialysis. The authors present a case of a 50 year old female with chronic abdominal pain that was found to have coccidioidomycosis. An US was performed and reported a schleroatrophic and litiasic gallbladder. Exploratory laparoscopy was performed, the findings were diffuse lesions in the peritoneal cavity parietal and visceral, some of them were biopsied. A peritoneal coccidioidomycosis was reported and fluconazol treatment was started. Coccidioideal peritonitis is a rare disease, most of the patients are asymptomatic and frequently diagnosed while working up other disease. 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 “Pseudoainhum and peripheral neuropathy” (Ann Dermatol Venereol. 2006 Oct;133(10):791-4). Authors are Akallal N, belgnaoui FZ, Benameur H, et al., from the Service de Dermatologie-Venereologie, CHU Ibn-Sina, Rabat, Maroc. Pseudoainhum is a rare disease characterised by gradual fibrous constriction of the fingers and/or toes eventually resulting in their amputation. The authors report a case of a 46-year-old woman with no toxic habits who was hospitalised for spontaneous amputation of the fingers and toes. This condition began when the patient was 12 years old with the appearance of a circular constriction band at the base of the fifth toe, eventually resulting in its loss. The patient gradually lost all her other toes and fingers except for the first joint of her left index finger. 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 “SAPHO syndrome: clinical report.” (Acta Reumatol Port. 2006 Apr-Jun;31(2):175-80). Authors are De Santis M and Fortuna J, from the Servico de Medicina, Centro Hospitalar de Coimbra. SAPHO Syndrome (Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis) was first described in the 80s by Chamot and Kahn. This disease affects both skin and bones, mainly in the costal area and anterior chest wall (Skin-Bone Disease). Clinical symptoms vary according to the age of onset and response to the treatment is uncertain. This is considered a rare disease, although its real prevalence is not known. SAPHO syndrome may occur in any age and both sexes are equally affected. 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 “Radiologic findings in Gorham-Stout syndrome.” (Radiologia. 2006 Jan-Feb;48(1):33-6). Authors are Cano B, Insa S, Cifrian C, et al., from the Servicio de Radiodiagnostico de adultos, Hospital La Fe, Valencia, Espana. Gorham-Stout syndrome, also called “disappearing bone disease” or idiopathic massive osteolysis, is a rare disease of unknown etiology and pathogenesis. It is characterized by rapidly progressive localized massive osteolysis associated to a proliferation of vascular structures of benign origin in which the absence of new bone formation is representative. The authors present two cases of this disease with the characteristic clinical, histological, and imaging findings. 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 “Oncocytic carcinoma of the parotid gland.” (Onkologie. 2006 Sep;29(8-9):388-90). Authors are Caloglu M, Yurut-Caloglu V, Altaner S et al, from the Department of Radiation Oncology, Faculty of Medicine, Trakya University, Edirne, Turkey. Oncocytic carcinoma is a rare tumor of major salivary glands. Despite being described 5 decades ago, not much is known about these rare tumors. Histochemical or electron microscopic confirmation of the oncocytic nature of the tumor cell is needed for differential diagnosis. The main treatment modality is surgery with or without adjuvant radiotherapy. Malignant oncocytomas have the potential risk of developing distant metastases and demand long term follow-up after therapy. For an accurate approach in the management of patients, oncocytic adenocarcinoma should be considered in the differential diagnosis of lesions of the parotid gland, most of which are benign. 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 cardiac sarcoma in pregnancy: a case report.” (J Korean Med Sci. 2006 Oct;21(5):940-3). Authors are Cho GJ, Kim HJ and Kang JS, from the Department of Obstetrics and Gynecology, College of Medicine, Korea University, Seoul, Korea. Primary cardiac sarcoma is a rare disease in adults. It is also associated with poor prognoses, due to diagnostic delay, therapeutic difficulty, and high metastatic potential. The coincidence of pregnancy and a primary cardiac intimal sarcoma is extremely rare. The authors report a pregnant woman at 27(+5) weeks gestation who was admitted to the hospital with acute-onset dyspnea. A mass was found on the left atrium by transthoracic echocardiography. The patient was diagnosed with a undifferentiated sarcoma, and gave birth to a 1,230 g living baby boy by Caesarean section from preterm contraction at 29(+5) weeks gestation. The patient then received systemic chemotherapy. Palliative cardiac surgery allows the prolonging of pregnancy, until an acceptable fetal viability level is reached. 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 “Churg-Strauss syndrome and pregnancy: successful treatment with intravenous immunoglobulin” (J Clin Rheumatol. 2002 Jun;8(3):151-6). Authors are Ruthberg SA, Ward DE and Roth BJ, from the Departments of Internal Medicine and Pathology, Madigan Army Medical Center, USUHS and University of Washington, USA. Churg-Strauss syndrome is an extremely rare disease, and even less common in women of childbearing age. The initial management of Churg-Strauss syndrome consists of high doses of corticosteroids. Intravenous immunoglobulin (IVIg) has become a promising, but not completely accepted, form of treatment for systemic vasculitis that is unresponsive to standard therapy. The authors present a case of a woman diagnosed with Churg-Strauss syndrome that was refractory to corticosteroid and cyclophosphamide treatment. When she was found to be 3 months pregnant, treatment with IVIg was instituted with successful results. To access the full abstract of the article, click here.