On 10/12/2007, a new orphan medicinal product was registered into the community register of orphan medicinal products under the EU orphan designation number 502. The active ingredient is Picoplatin for treatment of small cell lung cancer.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled Anesthesia and myotonic dystrophy (Steinert’s syndrome). The role of total intravenous anesthesia with propofol, cisatracurium and remifentanyl. Case report. ( Minerva Anestesiol. 2007 Sep;73(9):475-9). Authors are Catena V Del Monte DD Rubini A et al., from the Intensive Care Unit, ULSS 3, Bassano del Grappa Hospital, Vicenza, Italy. Anesthesia for patients with Steinert’s syndrome (myotonic dystrophy, MD) is a challenge for the anaesthetist. MD is a multisystemic disease and the neuromuscular symptoms can be associated with sleep apnea, endocrine disorders (diabetes, hypogonadism, hypothyroidism), cardiac, gastroenteric or cognitive disorders (mental deficiency, attention disorders). The diagnosis is facilitated when one or more of these symptoms are associated with the neuromuscular symptoms; however, the latter are not always present at the onset, which makes the diagnosis of MD a difficult and often late one. The choice of drugs and the choice of anesthesia in these patients can be very challenging for many reasons. To access the full abstract of the article, click here.
On 10/12/2007, a new orphan medicinal product was registered into the community register of orphan medicinal products under the EU orphan designation number 501. The active ingredient is Olaparib for treatment of ovarian cancer.
In the context of the French European Union Presidency, and as the European Commission plans to adopt on November the 5th, a Communication and a Proposal for a Council Recommendation on Rare Diseases, the Minister for Health, Youth, Sports and Associations is organising a European conference on the national strategies and action plans for rare diseases developed in the different Member States. This conference will take place on Tuesday 18 November 2008 at the Ministry for Health, Youth, Sports and Associations. For more information click here.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled Ultrasound-guided brachial plexus block in a patient with multiple glomangiomatosis (Reg Anesth Pain Med. 2008 Jan-Feb;33(1):70-3). Authors are Duggan E Brull R Lai J et al, from the Department of Anesthesia and Pain Management, Toronto Western Hospital University Health Network, Toronto, Ontario, Canada. Glomangiomas are rare, vascular tumors consisting of an afferent artery, arteriovenous canal, neuro-reticular elements, collagen, and efferent veins, and are most often located in the soft tissue of the upper extremities. We describe how the use of ultrasound-guided nerve blockade altered the anesthetic management of a patient with multiple glomangiomatosis undergoing elective forearm surgery.Although multiple glomangiomatosis is a rare disease, this case illustrates the invaluable contribution that ultrasound has made to modern, regional anesthetic practice, especially for patients with aberrant anatomy in whom traditional nerve-localization techniques could result in serious complications. 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 Cardiovascular risk factors in adult patients with multisystem Langerhans-cell histiocytosis: evidence of glucose metabolism abnormalities (QJM. 2007 Dec 26). Authors are Аlexandraki KI Makras P, Protogerou AD, et al, from Greece, USA and UK. Langerhans-cell histiocytosis (LCH) is a rare disease with features of chronic inflammation and it may also induce hypopituitarism, conditions associated with an increased risk of cardiovascular diseases. AIM: Cardiovascular and metabolic risk profile investigation in multisystem LCH patients with and without anterior pituitary deficiency.Adults patients with LCH have abnormalities of glucose metabolism that tend to occur in patients with active disease, and may be a consequence of the pro-inflammatory state. 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 Severe gastrointestinal haemorrhage due to primary intestinal Kaposi’s sarcoma – a case report (Niger Postgrad Med J. 2007 Dec;14(4):352-4.). Authors are Adisa AO Salako AA Ojo OS et al, from the Department of Surgery, Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Nigeria. Kaposi’s Sarcoma (KS) was previously a relatively rare disease. With the advent of HIV/AIDS pandemic however, AIDS-related KS has been on the increase and so has interest in the disease. Ninety per cent of patients with KS present with skin lesions. While the gastrointestinal tract is a fairly common site of metastatic KS, primary gastrointestinal KS is uncommon.The presentation of gastrointestinal KS with severe gastrointestinal bleeding is rarer still. In this report, we present a 56yr old HIV-negative patient who presented with severe gastrointestinal bleeding without any 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 Spontaneous spinal epidural haematoma in a 15-month-old boy presenting with a wry neck: a case report (J Orthop Surg (Hong Kong). 2007 Dec;15(3):373-5.). Authors are Fok WM Sun LK Wong NM et al, from the Department of Orthopaedics and Traumatology, United Christian Hospital, Hong Kong. A 15-month-old boy presented with a 2-day history of a wry neck with no definite trauma. He had bilateral upper limb weakness and was afebrile, conscious, and stable. There was no spontaneous movement in both upper limbs. Magnetic resonance imaging of the cervical and thoracic spine demonstrated an extensive spontaneous spinal epidural haematoma from C3 to T8. 23 hours after admission, the patient underwent an emergency right-sided C3 to T8 hemi-laminectomy and haematoma evacuation.The patent’s strength gradually recovered. Spontaneous spinal epidural haematoma is a rare disease in children. A high index of suspicion is essential for its effective management as the interval to operation is the most important prognostic factor. 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 Paternal mosaicism and hereditary angioedema in a Taiwanese family (Ann Allergy Asthma Immunol. 2007 Oct;99(4):375-9). Authors are Yu TC Shyur SD Huang LH et al, from the Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan, Republic of China. Hereditary angioedema (HAE) is a rare disorder characterized by recurrent attacks of localized subcutaneous or submucosal edema. It is inherited in an autosomal dominant fashion and caused by a deficiency of C1 inhibitor (C1 INH). Most patients with HAE have an absolute deficiency of C1 INH (type I HAE), whereas the rest (approximately 15%) synthesize a dysfunctional C1 INH protein (type II HAE). Mosaicism is rare in HAE. Parental mosaicism is a possible explanation for normal C1 INH plasma concentrations in both parents despite clinically apparent HAE in the children. 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 Lumbar hernia. Case report and literature review (Cir Cir. 2007 Sep-Oct;74(5):381-4.). Authors are Tavares-de la Paz LA, Martínez-Ordaz JL from the Servicio de Gastrocirugia, Hospital de Especialidades, Centro Médico Nacional Siglo XXI, Instituto Mexicano del Seguro Social, León, Guanajuato, Mexico. Lumbar hernia is a rare abdominal wall defect that usually presents spontaneously after trauma or lumbar surgery or, less frequently, during infancy (congenital). Few reports have been published in the literature describing congenital lumbar hernia. Congenital lumbar hernia usually originates in the superior triangle or Grynfellt-Lesshaft’s triangle and is frequently associated with the lumbocostovertebral syndrome. Surgery is always indicated, with utilization of prosthetic material or muscular flaps as the best repair technique. To access the full abstract of the article, click here.