Publications

PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “First case of homozygous C1 inhibitor deficiency.” (J Allergy Clin Immunol. 2006 Dec;118(6):1330-5). Authors are Blanch A, Roche O, Urrutia I, et al., from the Unidad de Inmunologia, Hospital Universitario La Paz, Madrid, Spain. C1 Inhibitor (C1-Inh) deficiency causes angioedema and can be hereditary (HAE), caused by mutations in the C1-Inh gene (C1NH), or acquired (AAE). Patients with HAE show a complement profile different from that of patients with AAE with normal levels of C1 (C1q, C1r, and C1s). The authors sought to characterize the complement profile of a patient with HAE and a mutation in homozygosis in the C1NH gene (c.1576T>G, Ile462Ser) and study his family. This is the first report of patients homozygous for a mutation affecting the coding region of C1NH. The most common HAE treatment is attenuated androgens, which increase the C1NH gene transcription levels. Because the homozygous patients lack a wild-type allele, long-term prophylactic treatment with attenuated androgens might not be advisable. 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 “Surgical treatment of sclerosing encapsulating peritonitis.” (Cir Esp. 2007 Mar;81(3):139-43). Authors are Bujalance Cabrera FM, Herrera merino, Salvador Fernandez M et al., from the Servicio de Cirugia. Hospital Severo Ochoa. Leganes, Madrid. Espana. Sclerosing encapsulating peritonitis (SEP) or abdominal “cocoon” is a rare disease characterized by the formation of a fibrocollagenous membrane that wraps around the loops of the small bowel, producing obstruction. The authors present the results of the surgical treatment of this disease in three patients with chronic renal failure who developed SEP during the course of peritoneal dialysis (PD). The success of surgery in this disease is determined by the technique used. The correct technique consists of freeing the adhesions and extirpating of the capsule as far as possible. Perforation, as well as resection and intestinal anastomosis, significantly increase mortality. 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 “Amyloidosis of the small intestine.” (Eur J Radiol. 2007 Mar 5). Authors are Kala Z, Valek V and Kysela P, from the Department of Surgery, Faculty Hospital Brno, Brno, Czech Republic. Amyloidosis is a rare disease characterized by forming pathological protein deposits – amyloid – in many organs and tissues. This decreases their functionality. The aim of this small study was to determine, whether the radiological picture of the small intestine involvement in amyloidosis is in some sense specific as sometimes described in literature giving rise to high suspicion for the disease in symptomatic patients. The conclusions were that the diagnosis of amyloidosis must be supported by bioptic examination as it has no pathognomic radiological picture. 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 “Inflammatory pseudotumor of the sinonasal tract.” (J Formos Med Assoc. 2007 Feb;106(2):165-8). Authors are Chuang CC, Lin HC and Huang CW, from the Department of Otolaryngology, Taipei City Hospital Yang-Ming Branch, Taipei, Taiwan. Inflammatory pseudotumors (IPTs) are a clinically and histologically diverse group of lesions characterized by a tumor mass of acute and chronic inflammatory cells with a variable fibrous response. IPTs most commonly involve the lung and orbit, but rarely the sinonasal tract. The authors report a 68-year-old male with an IPT of the sinonasal tract presenting as nasal obstruction and postnasal dripping for several years. Pathology revealed admixture of plasma cells, lymphocytes and eosinophils, confirming the diagnosis of IPT. Awareness of the clinical presentations, histopathologic features and treatment of choice of this rare disease entity is necessary to distinguish it from malignancy and avoid unnecessary management. 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 “Insulin Autoimmune Syndrome possibly caused by alpha lipoic acid.” (Intern Med. 2007;46(5):237-9). Authors are Takeuchi Y, Miyamoto T, Kakizawa T et al., from the Department of Geriatrics, Shinshu University, Matsumoto, Japan. Insulin Autoimmune Syndrome (IAS) is a rare disease characterized by hypoglycemia and autoantibodies to insulin without prior insulin administration. Here, the authors report a case of IAS associated with alpha lipoic acid (ALA). The patient is a 55-year-old man. He began to complain of hypoglycemic symptoms after taking ALA. He lost consciousness in the late postprandial period and blood glucose was found to be 27 mg/dl. A high insulin level and high titers of insulin antibodies were detected. As ALA comes to be used widely, the incidence of IAS due to ALA might increase. 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 angiosarcoma of the aorta.” (Vasa. 2007 Feb;36(1):33-40). Authors are Boge G, Gallix B, Gresillon C et al., from the Abteilung fur Innere Medizin und Gefasskrankheiten, Universitatskrankenhaus Saint Eloi. Primary angiosarcoma of the aorta is a rare disease. The prognosis is poor, resulting of embolic complications and early metastatic disease, with a median survival of nine months. Diagnosis is difficult and often made post-mortem. The authors report the case of a 68-year-old woman referred for a thrombosis of the superior mesenteric artery, occurring a few weeks after resection of an angiosarcoma of the small intestine, disclosing a primary angiosarcoma of the aortic wall with metastatic disease. To access the abstract of the article, click here.