PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Management of pseudomyxoma peritonei” (World J Gastroenterol. 2006 Oct 14;12(38):6124-7). Authors are Qu ZB and Liu LX from the Department of General Surgery, First Clinical College of Harbin Medical University, Harbin, China. Pseudomyxoma peritonei (PMP) is a rare disease. It refers to a progressive disease process within the peritoneum which originates from the appendix or ovaries and is characterised by the production of copious amounts of mucinous fluid resulting in a “jelly belly”. If untreated the condition is fatal. The natural history of this disease has been drastically modified since the introduction of a new surgical approach defined as a peritonectomy procedure. 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 “Malignant potential of intrahepatic biliary papillomatosis: a case report and a review of the literature” (World J Surg Oncol. 2006 Oct 7;4(1):71). Authors are Vassiliou I, Kairi-Vassilatou E, Marinis A et al.. Biliary papillomatosis (BP) is a rare disease entity with a strong malignant potential. It is characterized by multiple papillary adenomas involving both the intrahepatic and extrahepatic biliary tree. A current review of the English literature revealed a high rate of malignant occurrence of approximately 41% and histological analysis along with the expression pattern of mucin core proteins (MUC) and mucin carbohydrate antigens suggests that BP is a borderline or low grade malignant neoplasm with a high malignant potential. The authors report a case of a 68 year-old male patient with the presence of sudden right upper quadrant abdominal pain, nausea and dark urine. The examinations and histological analysis revealed a moderately to poorly differentiated carcinoma of the left hepatic duct in the background of biliary papillomatosis. BP should not be considered to be a benign disease. To access the full abstract of the article, click here.
On 31 August 2006, a new orphan medicinal product was registered into the community register of orphan medicinal products under the EU orphan designation number 391. The active ingredient is amphotericin B (for inhalation use) for prevention of pulmonary fungal infection in patients deemed at risk.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Adult idiopathic neutrophilic eccrine hidradenitis treated with colchicine” (Presse Med. 2006 Oct;35(10-C1):1475-1478). Authors are Belot V, Perrinaud A, Corven C, et al., from the Service de dermatologie, France. Neutrophilic eccrine hidradenitis (NEH) is a rare disease belonging to the group of neutrophilic dermatoses. It has a characteristic histopathologic pattern, with necrosis of the eccrine glands and a local neutrophilic infiltrate. It occurs mostly in patients receiving chemotherapy for acute myeloblastic leukemia or, less frequently, another malignancy. The authors report the case of a 56-year-old woman with no remarkable medical history who developed an erythematous papular facial eruption. Skin biopsy showed typical features of neutrophilic eccrine hidradenitis including neutrophilic infiltrate. No cause was found. The patient was successfully treated with colchicine. This case is particular because NEH was not associated with malignant hematologic disease, solid cancer, chemotherapy, fever or any other disease, after a follow-up of 22 months. 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 “Gastric volvulus in children” (Acta Paediatr Taiwan. 2006 Jan-Feb;47(1):18-24). Authors are Chang SW, Lee HC, Yeung CY, et al., from the Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan. Gastric volvulus (GV) is a rare disease in children that may not be recognized early in its course. The authors retrospectively analyzed 15 patients under 18 years of age who presented to their medical center with GV between January 1995 and June 2005. Patients with complete volvulus and acute obstruction requiring immediate intervention were defined as acute GV. Chronic GV was diagnosed in the presence of partial volvulus with chronic, nonspecific symptoms and signs. Organoaxial GV was the most common type (9/15), and there was a high incidence (6/15) of associated anomalies that predisposed to the condition. None of the patients had Borchardt’s triad (acute localized epigastric distension, unproductive retching, and the inability to pass a nasogastric tube) which is described in adults with acute GV. To access the full abstract of the article, click here.
On 1 September 2006, a new orphan medicinal product was registered into the community register of orphan medicinal products under the EU orphan designation number 390. The active ingredient is 4-amino-(6R,S)-5,6,7,8-tetrahydro-L-biopterin dihydrochloride for treatment of moderate and severe traumatic brain injury.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Progressive myoclonic epilepsies: myth or reality?” (Epileptic Disord. 2006 Apr;8 Suppl 1:37-43). Authors are Genton P and Bureau M from the Centre Saint-Paul, Marseille, France. The progressive myoclonus epilepsies (PME) are rare diseases and many clinicians, who have only few opportunities to encounter such patients, may think of them as mythical. PMEs are defined by: 1) generalized epileptic seizures (focal seizures may also occur in certain etiologies), 2) a sometimes invalidating myoclonus, and 3) other neurological symptoms (ataxia, dementia, sensory deficits) that vary according to etiology. The genetic and biochemical mechanisms underlying PMEs are increasingly recognized. New therapeutic approaches are under development, and the available purely symptomatic treatments can be used to best possible effect. 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 “Molar tooth sign in fetal brain magnetic resonance imaging leading to the prenatal diagnosis of Joubert syndrome and related disorders” (Child Neurol. 2006 Apr;21(4):320-4). Authors are Fluss J, Blaser S, Chitayat D et al., from the Division of Pediatric Neurology, The Hospital for Sick Children, University of Toronto, Ontario, Canada. Joubert syndrome is a rare autosomal recessive disorder characterized by ataxia, developmental delay, and oculomotor and respiratory abnormalities in relation to cerebellar vermian and midbrain dysgenesis. The midbrain dysgenesis is responsible for the molar tooth sign on axial magnetic resonance imaging (MRI). This classic hallmark of Joubert syndrome has been identified in other disorders sharing overlapping clinical and radiologic features with Joubert syndrome. The identification of a molar tooth sign has, however, rarely been documented by a fetal brain MRI. The authors report a case of Joubert syndrome diagnosed prenatally using fetal MRI. To access the full abstract of the article, click here.
On 28 July 2006, a new orphan medicinal product was registered into the community register of orphan medicinal products under the EU orphan designation number 389. The active ingredient is lestaurtinib for treatment of acute myeloid leukaemia.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Autoimmune thyroid encephalopathy presenting with epilepsia partialis continua” (Clin EEG Neurosci. 2006 Jul;37(3):204-9 ). Authors are Aydin-Ozemir Z, Tuzun E, Baykan B et al., from the Department of Neurology, Istanbul University, Istanbul, Turkey. The authors report the first case of an autoimmune thyroid encephalopathy presenting with multifocal motor status epilepticus. A 37-year-old female patient was admitted with multifocal motor seizures intractable to intravenous status epilepticus treatments, asymmetrical quadriparesis, truncal ataxia and continuous semi-rhythmical jerks. Pathological signal alterations were detected in both precentral cortices in MRI examination. Autoimmune thyroiditis was diagnosed after radiological examinations of the thyroid gland and thyroid function tests. Remarkably, autoimmune thyroiditis may present with migrating focal motor status epilepticus. To access the full abstract of the article, click here.