PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Diagnosis and treatment of solid-pseudopapillary tumor of the pancreas.” (Hepatobiliary Pancreat Dis Int. 2006 Aug;5(3):454-8). Authors are Zhang H, Liang TB, Wang WL et al, from the Department of Hepatobiliary Surgery, First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China. Solid-pseudopapillary tumor (SPT) of the pancreas is a rare exocrine pancreatic tumor. Despite the increasing recognition of the tumor in recent years, its pathogenesis and apparent therapeutic algorithm remain unclear. A study was designed to define the clinical, imaging, and pathologic features and to improve the diagnosis and treatment of this rare disease. The conclusions are that SPT exhibits unique clinical and pathologic features and is readily diagnosed by its characteristic imaging and histological appearance. Surgical resection of the primary tumor and metastases is the treatment of choice. To access the abstract of the article, click here.
Publications
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Idiopathic acute eosinophilic pneumonia.” (J Chin Med Assoc. 2006 Jul;69(7):330-3). Authors are Liu KT, Wu MH, Chiu CH et al, from the Chest Department, Taipei Veterans General Hospital, Taiwan, ROC. Idiopathic acute eosinophilic pneumonia (IAEP) is a rare disease but of clinical importance because of its good prognosis if treated promptly and appropriately. The etiology remains unknown and the temporal relationship between IAEP and a history of resent onset of cigarette smoking has been described. The authors report a typical case of a 21-year-old male with recent onset of smoking, who presented with acute febrile hypoxemic respiratory failure. Bronchoalveolar lavage (BAL) was performed soon after the patient was admitted and remarkable eosinophilia was noted in BAL fluid. Clinical condition and chest radiographs improved dramatically after corticosteroid treatment. Because effective treatment and prompt institution of therapy can obviate unnecessary morbidity and mortality, IAEP should be kept in mind when treating patients presenting with diffuse parenchymal lung disease and acute respiratory failure. 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 “Targeting T cells to hit B cells: successful treatment of cutaneous plasmacytosis with topical pimecrolimus.” (Dermatology. 2006;213(2):163-5). Authors are Hajner C, Hohenleutner U, Babilas P et al, from the Klinik und Poliklinik fur Dermatologie, Universitat Regensburg, Germany. Cutaneous plasmacytosis is a rare disease predominantly found in Japanese patients. The authors describe a case of a 75-year-old white female with cutaneous plasmacytosis of the face and involvement of the bone marrow. In contrast to other cases of cutaneous plasmacytosis, the patient revealed hypogammaglobulinemia and elevated levels of free light chains in the urine. Treatment with topical pimecrolimus 1%, which primarily targets T cells, led to almost complete clinical and histological remission of the skin lesions. 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 “Diffuse cerebral white matter T2-weighted hyperintensity: a new finding of general paresis.” (Acta Radiol. 2006 Jul;47(6):609-11). Authors are Alam F, Yasutomi H, Fukuda H, et al, from the Department of Radiology, Division of Medical Intelligence and Informatics, Graduate School of Biomedical Sciences, Hiroshima University, Japan. General paresis (parenchymatous neurosyphilis) is a rare disease, and in recent years the number of papers published on the magnetic resonance imaging findings has been limited. The findings are as follows: cerebral atrophy; mesiotemporal T2 hyperintensity; ventriculomegaly; pathological T2 hypointensity of the globus pallidus, putamen, the head of the caudate nucleus and thalamus. The authors present a new finding, diffuse cerebral white matter T2 hyperintensity, observed in a patient with general paresis with a 5-year history of progressive dementia. To access the abstract of the article, click here.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Hashimoto’s thyroiditis associated with idiopathic retroperitoneal fibrosis: case report and review of the literature.” (Int J Clin Pract. 2006 Aug 2). Authors are Pizzini AM, Corrado S, Radighieri E, et al, from the Department of Internal Medicine, Endocrinology Unit, University of Modenaand Reggio Emilia, Italy. Idiopathic retroperitoneal fibrosis (IRF) is a rare disease of unknown origin, characterised by an inflammatory proliferative fibrosing process occurring in the retroperitoneum. Hashimoto’s thyroiditis is a form of chronic thyroiditis that in some cases shows an extensive replacement of thyroid parenchyma by fibrous tissue. The authors report the rare association of IRF with HT in a 68-year-old woman. The so far reported cases of IRF associated with chronic thyroiditis are reviewed, and the possible aetiopathogenetic link between these two entities is discussed. To access the full abstract of the article, click here.
On 22 June 2006, a new orphan medicinal product was registered into the community register of orphan medicinal products under the EU orphan designation number 377. The active ingredient is mecasermin rinfabate for treatment of patients with growth hormone (GH) gene deletion who have developed neutralizing antibodies to GH.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Eosinophilic Angiocentric Fibrosis” (J Craniofac Surg. 2006 Jul;17(4):812-814). Authors are Clauser L, Mandrioli S, Polito J et al, from the Unit of Craniomaxillofacial Surgery, Center for Craniofacial Deformities and Orbital Surgery, and daggerDepartment of Surgical Pathology, S. Anna Hospital and University, Ferrara, Italy. Eosinophilic angiocentric fibrosis (EAF) is a rare disease of the sinonasal tract, with histologic characteristic features like thick collagen bundles whirling around vessels in a fibrotic stroma with inflammatory cells rich in eosinophils. The authors present a case of a 31-year-old man with bilateral nasal obstruction with no history of allergies or other systemic 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 ”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.
On 24 May 2006, a new orphan medicinal product was registered into the community register of orphan medicinal products under the EU orphan designation number 376. The active ingredient is recombinant P-selectin glycoprotein immunoglobulin for prevention of post transplantation graft dysfunction.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “TOM: a web-based integrated approach for identification of candidate disease genes.” (Nucleic Acids Res. 2006 Jul 1;34(Web Server issue):W285-92). Authors are Rossi S, Masotti D, Nardini C et al, from the Functional Genomics Laboratory and Telethon Facility, DAMA Data Mining for Analysis of DNA Microarrays, Dipartimento di Morfologia ed Embriologia, Ferrara, Italy. The massive production of biological data by means of highly parallel devices like microarrays for gene expression has paved the way to new possible approaches in molecular genetics. Among them the possibility of inferring biological answers by querying large amounts of expression data. Based on this principle, the authors present here TOM, a web-based resource for the efficient extraction of candidate genes for hereditary diseases. This approach allows the geneticist to bypass the costly and time consuming tracing of genetic markers through entire families and might improve the chance of identifying disease genes, particularly for rare diseases. To access the full abstract of the article, click here.