PENULISAN ILMIAH

ADELIA PUTRI SABRINA

FAKULTAS KEDOKTERAN UMUM

1102013005

UNIVERSITAS YARSIJl. Let. Jend. Suprapto. Cempaka Putih, Jakarta Pusat. DKI Jakarta. Indonesia. 10510.

Telepon: +62 21 4206675.

DAFTAR PUSTAKA

TOPIK 1 (HEMOFILIA)1. Corwin, Elizabeth J. 2001. Buku Saku Patofisiologi. Jakarta : Buku Kedokteran EGC2. Chuah, M.K. dkk. 2012. Recent Progress in Gene Theraphy for Hemophilia dalam

Pubmed (Online). Tersedia : http://www.ncbi.nlm.nih.gov/pubmed/22671033 (Diakses pada tanggal 15 September 2013).

3. Gouw, S.C. dkk. 2013. Factor VII products and inhibitor development in severe hemophilia A dalam Pubmed (Online). Tersedia : http://www.ncbi.nlm.nih.gov/pubmed/23323899 (Diakses pada tanggal 15 September 2013).

4. Khani, Francesca & Mikhail Roshal. 2012. A 24-Year-Old Man with Previously Diagnosed Hemophilia. Tersedia : www.clinchem.org/content/58/7/1086.full.pdf. (Diakses pada tanggal 15 September 2013).

TOPIK 2 (RUBELLA)1. Babigumira, J.B. dkk. 2013. Health economics of rubella: a systematic review to

assess the value of rubella vaccination dalam Ebscohost (Online). Tersedia : http://web.ebscohost.com/ehost/detail?sid=2b84f8c7-b1c0-4794-97a1-00c0af4b178d%40sessionmgr110&vid=1&hid=114&bdata=JnNpdGU9ZWhvc3QtbGl2ZQ%3d%3d#db=mdc&AN=23627715 (Diakses pada tanggal 15 September 2013).

2. Corwin, Elizabeth J. 2001. Buku Saku Patofisiologi. Jakarta : Buku Kedokteran EGC3. Knuf, M. dkk. 2013. Antibody persistence for 3 years following two doses of

tetravalent measles-mumps-rubella-varicella vaccine in healthy children dalam Ebscohost (Online). Tersedia : http://web.ebscohost.com/ehost/detail?sid=057639de-81b7-4aac-8b89-58ce017de9c2%40sessionmgr104&vid=1&hid=114&bdata=JnNpdGU9ZWhvc3QtbGl2ZQ%3d%3d#db=mdc&AN=21935584 (Diakses pada tanggal 15 September 2013).

4. Plotkin, Stanley A. 2006. The History of Rubella and Rubella Vaccination Leading to Elimination dalam Oxford Journals (Online). Tesedia : http://cid.oxfordjournals.org/content/43/Supplement_3/S164.full (Diakses pada tanggal 15 September 2013).

TOPIK 3 (Coronary Hearts)

1. Cassar, A. dkk. 2009. Chronic coronary artery disease: diagnosis and management dalam Pubmed (Online). Tersedia : http://www.ncbi.nlm.nih.gov/pubmed/19955250 (Diakses pada tanggal 15 September 2013).

2. Corrao, M. dkk. 2000. Alcohol and coronary heart disease: a meta-analysis dalam Pubmed (Online). Tersedia : http://www.ncbi.nlm.nih.gov/pubmed/11070527 (Diakses pada tanggal 15 September 2013).

3. Kurniadi, Helmanu. 2013. Stop! Gejala Penyakit Jantung Koroner. Yogyakarta : Familia (Grup Relasi Inti Media, anggota IKAPI).

4. Maghami-Pour, N. dan N.Safaie. 2005. Disease in a Teenage Girl. Tersedia : http://journals.tums.ac.ir/upload_files/pdf/_/2081.pdf (Diakses pada tanggal 16 September 2013).

TOPIK 4 (SKOLIOSIS)

1. Abbot, A. dkk. 2013. CONTRAIS: CONservative TReatment for Adolescent Idiopathic Scoliosis: a randomised controlled trial protocol dalam Pubmed (Online). Tersedia : http://www.ncbi.nlm.nih.gov/pubmed/24007599 (Diakses pada tanggal 16 September 2013).

2. Braun, J.T. dkk. 2006. Three-dimensional analysis of 2 fusionless scoliosis treatments: a flexible ligament tether versus a rigid-shape memory alloy staple dalam Pubmed (Online). Tersedia : http://www.ncbi.nlm.nih.gov/pubmed/16449897 (Diakses pada tanggal 16 September 2013).

3. Corwin, Elizabeth J. 2001. Buku Saku Patofisiologi. Jakarta : Buku Kedokteran EGC 4. Lykissas, M.G. dkk. 2013. Rib osteoblastoma as an incidental finding in a patient

with adolescent idiopathic scoliosis: a case report dalam Pubmed (Online). Tersedia : http://www.ncbi.nlm.nih.gov/pubmed/23820482 (Diakses pada tanggal 16 September 2013).

TOPIK 5 (VERTIGO)

1. Junaidi, Iskandar. 2013. Sakit Kepala, Migrain, & Vertigo. Jakarta : PT Bhuana Ilmu Populer.

2. Seo, Toru dkk. 2003. Three Cases of Cochleosaccular Endolymphatic Hydrops without Vertigo Revealed by Furosemide-Loading Vestibular Evoked Myogenic Potential Test dalam Pubmed (Online). Tersedia : http://www.ncbi.nlm.nih.gov/pubmed/14501460 (Diakses pada tanggal 16 September 2013).

3. Strupp, M. dkk. 2013. The treatment and natural course of peripheral and central vertigo dalam Pubmed (Online). Tersedia : http://www.ncbi.nlm.nih.gov/pubmed/24000301 (Diakses pada tanggal 16 September 2013).

4. Westhofen, M. 2013. [Indications for operative therapy of vestibular vertigo and the associated success rates] dalam Pubmed (Online). Tersedia : http://www.ncbi.nlm.nih.gov/pubmed/24002727 (Diakses pada tanggal 16 September 2013).

ARTICLE REVIEWHum Gene Ther. 2012 Jun;23(6):557-65. doi: 10.1089/hum.2012.088.Recent progress in gene therapy for hemophilia.Chuah MK, Nair N, VandenDriessche T.

Source

Department of Gene Therapy & Regenerative Medicine, Free University of Brussels, B-1090 Brussels, Belgium.

AbstractHemophilia A and B are X-linked monogenic disorders caused by deficiencies in coagulation factor VIII (FVIII) and factor IX (FIX), respectively. Current treatment for hemophilia involves intravenous infusion of clotting factor concentrates. However, this does not constitute a cure, and the development of gene-based therapies for hemophilia to achieve prolonged high level expression of clotting factors to correct the bleeding diathesis are warranted. Different types of viral and nonviral gene delivery systems and a wide range of different target cells, including hepatocytes, skeletal muscle cells, hematopoietic stem cells (HSCs), and endothelial cells, have been explored for hemophilia gene therapy. Adeno-associated virus (AAV)-based and lentiviral vectors are among the most promising vectors for hemophilia gene therapy. Stable correction of the bleeding phenotypes in hemophilia A and B was achieved in murine and canine models, and these promising preclinical studies prompted clinical trials in patients suffering from severe hemophilia. These studies recently resulted in the first demonstration that long-term expression of therapeutic FIX levels could be achieved in patients undergoing gene therapy. Despite this progress, there are still a number of hurdles that need to be overcome. In particular, the FIX levels obtained were insufficient to prevent bleeding induced by trauma or injury. Moreover, the gene-modified cells in these patients can become potential targets for immune destruction by effector T cells, specific for the AAV vector antigens. Consequently, more efficacious approaches are needed to achieve full hemostatic correction and to ultimately establish a cure for hemophilia A and B.

PMID: 22671033 [PubMed - indexed for MEDLINE]

RESEARCH

N Engl J Med. 2013 Jan 17;368(3):231-9. doi: 10.1056/NEJMoa1208024.

Factor VIII products and inhibitor development in severe hemophilia A.Gouw SC, van der Bom JG, Ljung R, Escuriola C, Cid AR, Claeyssens-Donadel S, van Geet C, Kenet G, Mäkipernaa A, Molinari AC, Muntean W, Kobelt R,Rivard G, Santagostino E, Thomas A, van den Berg HM; PedNet and RODIN Study Group.

Collaborators (35)

Source

Department of Pediatrics, Wilhelmina Children's Hospital, Utrecht, The Netherlands.

AbstractBACKGROUND:For previously untreated children with severe hemophilia A, it is unclear whether the type of factor VIII product administered and switching among products are associated with the development of clinically relevant inhibitory antibodies (inhibitor development).

METHODS:We evaluated 574 consecutive patients with severe hemophilia A (factor VIII activity, <0.01 IU per milliliter) who were born between 2000 and 2010 and collected data on all clotting-factor administration for up to 75 exposure days. The primary outcome was inhibitor development, which was defined as at least two positive inhibitor tests with decreased in vivo recovery of factor VIII levels.

RESULTS:Inhibitory antibodies developed in 177 of the 574 children (cumulative incidence, 32.4%); 116 patients had a high-titer inhibitory antibody, defined as a peak titer of at least 5 Bethesda units per milliliter (cumulative incidence, 22.4%). Plasma-derived products conferred a risk of inhibitor development that was similar to the risk with recombinant products (adjusted hazard ratio as compared with recombinant products, 0.96; 95% confidence interval [CI], 0.62 to 1.49). As compared with third-generation full-length recombinant products (derived from the full-length complementary DNA sequence of human factor VIII), second-generation full-length products were associated with an increased risk of inhibitor development (adjusted hazard ratio, 1.60; 95% CI, 1.08 to 2.37). The content of von Willebrand factor in the products and switching among products were not associated with the risk of inhibitor development.

CONCLUSIONS:Recombinant and plasma-derived factor VIII products conferred similar risks of inhibitor development, and the content of von Willebrand factor in the products and switching among products were not associated with the risk of inhibitor development. Second-generation full-length recombinant products were associated with an increased risk, as compared with third-generation products. (Funded by Bayer Healthcare and Baxter BioScience).

CASE REPORT

Clinical Chemistry 58:71086–1090 (2012) Clinical Case

A 24-Year-Old Man with Previously Diagnosed HemophiliaFrancesca Khani1,2* and Mikhail Roshal2

CASEA 24-year-old Middle Eastern man diagnosed with hemophilia at the age of 4 or 5

years resented to the hematology clinic for follow-up after a recent hospitalization for excessive bleeding from an accidental knife cut. The patient reported a history of prolonged bleeding after teeth extractions, an upper gastrointestinal bleed 3 years previously, and excessive bruising since childhood. He denied hemarthroses but reported chronic pain in his ankles and joints. The patient reported having been treated for episodes of excessive bleeding with fresh frozen plasma (FFP)3 and factor VIII during past hospitalizations. Because of poor continuity of care, his disease had not been monitored or treated on an ongoing outpatient basis. The patient’s family history is noteworthy for consanguineous parents (first cousins) and a sister who also experienced excessive bleeding, although her diagnosis was uncertain. Initial laboratory test results included a normal complete blood count, including platelets, a prolonged activated partial thromboplastin time (aPTT), and a prolonged prothrombin time (PT) (Table 1). Fibrinogen activity was normal. A 1:1 mixture of the patient’s plasma with pooled normal plasma demonstrated full correction of the PT and aPTT, a result consistent with factor deficiency.

DISCUSSIONADDITIONAL PATIENT DATA AND DIFFERENTIAL DIAGNOSIS

Patients with isolated hemophilia A, B, or C (due to deficiencies in factors VIII, IX, and XI, respectively) or factor VIII deficiency due to von Willebrand disease typically have a prolonged aPTT but a normal PT. In the absence of anticoagulation therapy or suspected vitamin K deficiency, a prolonged PT in this patient’s initial workup should raise clinical suspicion for a bleeding disorder of a different etiology. Given the patient’s clinically notable bleeding symptoms since childhood, a genetic disorder should be considered. The differential diagnosis includes dysfibrinogenemia, prothrombin deficiency, factor V deficiency, combined deficiency of factors V and VIII (F5F8D), factor X deficiency, and hereditary combined deficiency of the vitamin K–dependent clotting factors. All of these conditions feature prolongation of both the PT and the aPTT. Given the results of the mixing studies, we subsequently evaluated factor activities.

The results of additional coagulation studies strongly suggested a diagnosis of F5F8D, because activities of factors V and VIII activities were markedly decreased. Also, factor VII activity appeared to be slightly increased, but this finding was considered unlikely to be of clinical consequence. F5F8D is a genetic condition that is often misdiagnosed as a single-factor deficiency condition such as hemophilia A, particularly in institutions with limited diagnostic resources in hematology. The inheritance pattern and pathogenesis of these 2 genetic disorders are distinct and are important for both therapeutic and genetic-counseling purposes. This scenario underscores the importance of further laboratory investigation when initial testing appearsto be inconsistent with the patient’s supposed diagnosis.

RESEARCHHealth economics of rubella: a systematic review to assess the value of rubella vaccination.

Authors: Babigumira JB ; Morgan I ; Levin AAuthor Address: Global Medicines Program, Department of Global Health, University

of Washington, Seattle, WA, USA. babijo@uw.eduSource: BMC Public Health [BMC Public Health] 2013 Apr 29; Vol. 13, pp.

406. Date of Electronic Publication: 2013 Apr 29.Publication Type: Journal ArticleLanguage: English

Publisher: BioMed Central Country of Publication: England NLM ID: 100968562 Publication Model: Electronic Cited Medium: InternetISSN: 1471-2458 (Electronic) Linking ISSN: 14712458 NLM ISO Abbreviation: BMC Public Health Subsets: In Process; MEDLINE

Background: Most cases of rubella and congenital rubella syndrome (CRS) occur in low- and middle-income countries. The World Health Organization (WHO) has recently recommended that countries accelerate the uptake of rubella vaccination and the GAVI Alliance is now supporting large scale measles-rubella vaccination campaigns. We performed a review of health economic evaluations of rubella and CRS to identify gaps in the evidence base and suggest possible areas of future research to support the planned global expansion of rubella vaccination and efforts towards potential rubella elimination And radication.Methods: We performed a systematic search of on-line databases and identified articles published between 1970 and 2012 on costs of rubella and CRS treatment and the costs, cost-effectiveness or cost-benefit of rubella vaccination. We reviewed the studies and categorized them by the income level of the countries in which they were performed, study design, and research question answered. We analyzed their methodology, data sources, and other details. We used these data to identify gaps in the evidence and to suggest possible future areas of scientific study.Results: We identified 27 studies: 11 cost analyses, 11 cost-benefit analyses, 4 cost-effectiveness analyses, and 1 cost-utility analysis. Of these, 20 studies were conducted in high-income countries, 5 in upper-middle income countries and two in lower-middle income countries. We did not find any studies conducted in low-income countries. CRS was estimated to cost (in 2012 US$) between $4,200 and $57,000 per case annually in middle-income countries and up to $140,000 over a lifetime in high-income countries. Rubella vaccination programs, including the vaccination of health workers, children, and women had favorable cost-effectiveness, cost-utility, or cost-benefit ratios in high- and middle-incomecountries.Conclusions: Treatment of CRS is costly and rubella vaccination programs are highly cost-effective. However, in order for research to support the global expansion of rubella vaccination and the drive towards rubella elimination and eradication, additional studies are required in low-income countries, to tackle methodological limitations, and to determine the most cost-effective programmatic strategies for increased rubella vaccine coverage.

CASE REPORTAntibody persistence for 3 years following two doses of tetravalent measles-mumps-rubella-varicella vaccine in healthy children.

Authors: Knuf M ; Zepp F ; Helm K ; Maurer H ; Prieler A ; Kieninger-Baum D ;Douha M ; Willems P

Author Address: Children's Department of Pediatrics, University Medicine Hospital, Johannes Gutenberg-University, Langenbeckstrasse 1, 55101 Mainz, Germany. markus.knuf@hsk-wiesbaden.de

Source: European Journal Of Pediatrics [Eur J Pediatr] 2012 Mar; Vol. 171 (3), pp. 463-70.Date of Electronic Publication: 2011 Sep 21.

Publication Type: Clinical Trial, Phase III; Journal Article; Multicenter Study; Randomized Controlled Trial; Research Support, Non-U.S. Gov't

Language: EnglishJournal Info: Publisher: Springer Verlag Country of Publication: Germany NLM

ID: 7603873Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1432-1076 (Electronic) Linking ISSN: 03406199 NLM ISO Abbreviation: Eur. J. Pediatr. Subsets:MEDLINE

Abstract:Unlabelled: Two doses of a varicella-containing vaccine in healthy children &lt;12 years are suggested to induce better protection than a single dose. Persistence of immunity against measles, mumps, rubella, and varicella as well as varicella breakthrough cases were assessed 3 years after two-dose measles, mumps, rubella, and varicella (MMRV) vaccination or concomitant MMR (Priorix™) and varicella (Varilrix™) vaccination. Four hundred ninety-four healthy children, 12-18 months old at the time of the first dose, received either two doses of MMRV vaccine (GlaxoSmithKline Biologicals) 42-56 days apart (MMRV, N = 371) or one dose of MMR and varicella vaccines administered simultaneously at separate sites, followed by another MMR vaccination 42-56 days later (MMR + V, N = 123). Three hundred-four subjects participated in 3-year follow-up for persistence of immunity and occurrence of breakthrough varicella (MMRV, N = 225; MMR + V, N = 79). Antibodies were measured by ELISA (measles, mumps, rubella) and immunofluorescence (varicella). Contacts with individuals with varicella or zoster and cases of breakthrough varicella disease were recorded. Three years post-vaccination seropositivity rates in subjects seronegative before vaccination were: MMRV-measles, 98.5% (geometric mean titer [GMT] = 3,599.6); mumps, 97.4% (GMT = 1,754.5); rubella, 100% (GMT = 51.9); varicella, 99.4% (GMT = 225.5); MMR + V-measles, 97.0% (GMT = 1,818.8); mumps, 93.8% (GMT = 1,454.6); rubella, 100% (GMT = 53.8); and varicella, 96.8% (GMT = 105.8). Of the subjects, 15-20% reported contact with individuals with varicella/zoster each year.

After 3 years, the cumulative varicella breakthrough disease rate was 0.7% (two cases) in the MMRV group and 5.4% (five cases) in the MMR + V group.Conclusion: Immunogenicity of the combined MMRV vaccine was sustained 3 years post-vaccination. (208136/041/NCT00406211).

ARTICLE REPORT

The History of Rubella and Rubella Vaccination Leading to EliminationStanley A. PlotkinReprints or correspondence: Dr. Stanley A. Plotkin, Sanofi Pasteur, 4650 Wismer Rd., Doylestown, PA 18901 (Stanley.Plotkin@Sanofipasteur.com).

Abstract

Congenital rubella syndrome (CRS) was discovered in the 1940s, rubella virus was isolated in the early 1960s, and rubella vaccines became available by the end of the same decade. Systematic vaccination against rubella, usually in combination with measles, has eliminated both the congenital and acquired infection from some developed countries, most recently the United States, as is confirmed by the articles in this supplement. The present article summarizes the clinical syndrome of CRS, the process by which the vaccine was developed, and the history leading up to elimination, as well as the possible extension of elimination on a wider scale.

At first, cataracts, deafness, and congenital heart disease were the only identifying characteristics of congenital rubella, but, in the spring of 1963, an epidemic of rubella started in Europe and subsequently spread to the United States in 1964 and 1965, leaving thousands of damaged infants in its wake. Studies of these infants revealed that congenital rubella syndrome (CRS) has many manifestations and affects virtually all organ systems. In addition to affecting 3 core organs—the optic lens, the cochlea, and the heart—CRS was recognized as a cause of pathology in the brain, lungs, liver, spleen, kidney, bone marrow, bones, and endocrine organs. This anatomic pathology was associated with encephalitis, mental retardation, pneumonia, hepatitis, thrombocytopenia, metaphyseal defects, diabetes mellitus, and thyroiditis. Moreover, although cataracts, cochlear atrophy, and patent ductus arteriosus were prevalent in typical CRS, other manifestations in the eye, ear, and heart were found to occur frequently, including glaucoma, central auditory imperception, and peripheral pulmonic stenosis.Those of us who were practicing pediatrics or obstetrics during those years remember with poignancy the many tragedies we witnessed as families struggled with decisions about therapeutic abortions and severely damaged infants. In Philadelphia, I calculated that at the height of the epidemic 1% of all births were affected.

Meanwhile, the cell culture revolution that began after the Second World War was applied to the study of rubella in the early 1960s. Two laboratories succeeded in detecting the presence of rubella virus: that of Weller and Neva in Boston and that of Parkman, Buescher, and Artenstein in Bethesda. In Boston, rubella virus was isolated by detecting subtle changes in human amnion cells, whereas in Bethesda the workers used a novel technique of viral interference. When samples containing rubella virus were inoculated on cultures of African green monkey kidney (AGMK) cells, the virus grew without cytopathic effect, but the cells secreted interferon. Challenge of the AGMK cultures with enteroviruses such as echovirus 11 revealed interference with their readily detected cytopathic effect. This technique became widely used in virology laboratories.

Fortunately, the isolation of rubella virus came just before the 1963–1965 rubella epidemic, permitting accurate virologic and serologic diagnosis and elucidation of disease pathogenesis. Many key features of rubella and CRS were recognized, including the following

ARTICLE REVIEWMayo Clin Proc. 2009 Dec;84(12):1130-46. doi: 10.4065/mcp.2009.0391.Chronic coronary artery disease: diagnosis and management.Cassar A, Holmes DR Jr, Rihal CS, Gersh BJ.

Source

Division of Cardiovascular Diseases, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA.

AbstractCoronary artery disease (CAD) is the single most common cause of death in the developed world, responsible for about 1 in every 5 deaths. The morbidity, mortality, and socioeconomic importance of this disease make timely accurate diagnosis and cost-effective management of CAD of the utmost importance. This comprehensive review of the literature highlights key elements in the diagnosis, risk stratification, and management strategies of patients with chronic CAD. Relevant articles were identified by searching the PubMed database for the following terms: chronic coronary artery disease or stable angina. Novel imaging modalities, pharmacological treatment, and invasive (percutaneous and surgical) interventions have revolutionized the current treatment of patients with chronic CAD. Medical treatment remains the cornerstone of management, but revascularization continues to play an important role. In the current economic climate and with health care reform very much on the horizon, the issue of appropriate use of revascularization is important, and the indications for revascularization, in addition to the relative benefits and risks of a percutaneous vs a surgical approach, are discussed.

RESEARCH

Addiction. 2000 Oct;95(10):1505-23.

Alcohol and coronary heart disease: a meta-analysis.Corrao G, Rubbiati L, Bagnardi V, Zambon A, Poikolainen K.

Source

Department of Statistics, University of Milan-Bicocca, Italy. giovanni.corrao@unimib.it

AbstractOBJECTIVE:To estimate parameters of the function relating alcohol consumption with the risk of coronary heart disease and to identify the sources of heterogeneity in the parameter estimates.

METHODS:A search of the epidemiological literature from 1966 to 1998 was performed using several bibliographic databases. Meta-regression models were fitted to evaluate non-linear effects of alcohol intake on the relative risk. The effects of some characteristics of the studies, including an index of their quality, were considered as putative sources of heterogeneity of the estimates. Publication bias was also investigated.

FINDINGS:Among the 196 initially reviewed articles, 51 were selected. Since qualitative characteristics of the studies were significant sources of heterogeneity, the pooled dose-response functions were based on the 28 cohort studies with higher quality. Risk decreased from 0 to 20 g/day (RR = 0.80; 95% CI: 0.78, 0.83); there was evidence of a protective effect up to 72 g/day (RR = 0.96; 95% CI: 0.92, 1.00) and increased risk above > or = 89 g/day (RR = 1.05; 95% CI: 1.00, 1.11). Lower protective effects and harmful effects were found in women, in men living in countries outside the Mediterranean area and in studies where fatal events were used as the outcome. Evidence of publication bias for moderate intakes and of heterogeneity of the estimates across studies for higher intakes were found.

CONCLUSIONS:The degree of protection from moderate doses of alcohol should be reconsidered. Further research investigating the effect of drinking patterns on the risk of coronary heart disease should be performed. Caution in making general recommendations is needed.

CASE REPORT

A CASE REPORT OF CORONARY ARTERY DISEASE IN A TEENAGE GIRL

N. Maghami-Pour* and N. SafaieDepartment of Cardiac Surgery, Shahid Madani Hospital, School of Medicine, Tabriz Universityof Medical Sciences, Tabriz, Iran

Abstract- Atherosclerosis is the leading cause of death in most parts of the world. This disorder affects mostly patients above the age 40 years. This case report introduces a 17 years old girl with early development of coronary artery disease who had severe coronary atherosclerosis that did not respond to medical and interventional treatment and underwent surgical operation in cardiac surgery department of Shahid Madani Hospital, Tabriz, Iran. Presence of risk factors for atherosclerosis were evaluated and the only findings were positive family history of cardiac death in her uncle at about 52 years of age andhigh level of lipoprotein (a) in one of her sisters. In follow up evaluation of this patient, high levels of lipoprotein (a) was documented which was controlled with medical therapy. We concluded that high level of lipoprotein (a) was the probable cause of atherosclerosis in this patient. This case report emphasizes the need to screen siblings of patients with premature myocardial infarction.Acta Medica Iranica, 43(5): 369-371; 2005

CASE REPORTThe patient was a 17 years old non obese girl with normal general appearance who was referred to cardiologist because of typical exertional chest pain which radiated to her left arm. On primary admission all laboratory data were normal. ETT and myocardial perfusion scan were positive and angiography was done which revealed coronary artery disease (Fig. 1). Attempt to do balloon angioplasty failed, LV gram showed normal ejection fraction and anteroapical hypokinesia. The patient was discharged with medical follow up. Since patient did not respond to medical treatment, we repeat angiography 10 months later which showed severe diffuse CAD. She was referred for operation. On surgery with the aid of cardiopulmonary by pass, grafts to all the coronary arteries were done. After operation chest pain improved and patient was followed with medical treatment such as aspirin and lovastatin.About 20 months after the operation again patient was referred with recurrence of the exertional chest pain. At this time laboratory data were all normal except for high level of Lp(a). ETT and myocardial perfusion scan again showed signs of ischemia so drug therapy for Lp(a) with atorvastatin and gemfibrozil was started. After 3 months, the level of Lp(a) decreased and exertional chest pain improved.

ARTICLE REVIEW

BMC Musculoskelet Disord. 2013 Sep 5;14(1):261.

CONTRAIS: CONservative TReatment for Adolescent Idiopathic Scoliosis: a randomised controlled trial protocol.Abbott A, Möller H, Gerdhem P.

Abstract

BACKGROUND:Idiopathic scoliosis is a three-dimensional structural deformity of the spine that occurs in children and adolescents. Recent reviews on bracing and exercise treatment have provided some evidence for effect of these interventions. The purpose of this study is to improve the evidence base regarding the effectiveness of conservative treatments for preventing curve progression in idiopathic scoliosis.Methods/design: Patients: Previously untreated girls and boys with idiopathic scoliosis, 9 to 17 years of age with at least one year of remaining growth and a curve Cobb angle of 25--40 degrees will be included. A total of 135 participants will be randomly allocated in groups of 45 patients each to receive one of the three interventions.Interventions: All three groups will receive a physical activity prescription according to the World Health Organisation recommendations. One group will additionally wear a hyper-corrective night-time brace. One group will additionally perform postural scoliosis-specific exercises.Outcome: Participation in the study will last until the curve has progressed, or until cessation of skeletal growth. Outcome variables will be measured every 6 months. The primary outcome variable, failure of treatment, is defined as progression of the Cobb angle more than 6 degrees, compared to the primary x-ray, seen on two consecutive spinal standing x-rays taken with 6 months interval. Secondary outcome measures include the SRS-22r and EQ5D-Y quality of life questionnaires, the International Physical Activity Questionnaire (IPAQ) short form, and Cobb angle at end of the study.

DISCUSSION:This trial will evaluate which of the tested conservative treatment approaches that is the most effective for patients with adolescent idiopathic scoliosis.Trial registration: NCT01761305.

RESEARCH

Spine (Phila Pa 1976). 2006 Feb 1;31(3):262-8.

Three-dimensional analysis of 2 fusionless scoliosis treatments: a flexible ligament tether versus a rigid-shape memory alloy staple.Braun JT, Akyuz E, Udall H, Ogilvie JW, Brodke DS, Bachus KN.

Source

Department of Orthopaedics, University of Utah, School of Medicine, Salt Lake City, USA. john.braun@hsc.utah.edu

AbstractSTUDY DESIGN:Experimental scoliosis was created and subsequently corrected in goats. The 3-dimensional (3-D) effects of the treatments were analyzed.

OBJECTIVE:To analyze the 3-D effect of 2 different fusionless scoliosis treatment techniques on an experimental idiopathic-type scoliosis using plain radiographs and computerized tomography.

SUMMARY OF BACKGROUND DATA:Scoliosis is a complex 3-D spinal deformity with limited treatment options. By preserving growth, motion, and function of the spine, fusionless scoliosis surgery provides theoretical advantages over current forms of treatment.

METHODS:Scoliosis was created in 24 Spanish cross-X female goats using a flexible, left posterior asymmetric tether from the T5 to L1 laminae, with convex rib resection and concave rib tethering from T8 to T13. After 8 weeks of posterior tethering, goats were randomized into 3 treatment groups: group 1, no treatment; group 2, anterior-shape memory alloy staple; and group 3, anterior ligament tether with bone anchor. The 6 levels of maximal curvature were instrumented in groups 2 and 3. All goats were observed for an additional 12-16 weeks. Serial radiographs and computerized tomography were used to document progression/correction of coronal, sagittal, and transverse plane deformities throughout the study.

RESULTS:There were 20 goats that had progressive, structural, idiopathic-type, lordoscoliotic curves convex to the right in the thoracic spine over the 8-week tethering period. An overall deformity score equaling the sum of the scoliosis, lordosis, and axial rotation measurements was calculated for each goat at 3 times.

CONCLUSION:The data in this study show the ability of a ligament tether attached to a bone anchor to correct scoliosis modestly in the coronal plane, but not in the sagittal or transverse plane. In addition, although a significant decrease in the deformity score was shown initially in this group (P < 0.001), the effect was lost over time. The final deformity in the bone anchor/ligament tether group wassignificantly less than either the stapled or untreated groups (P < 0.03). Further study is warranted to provide a better understanding of the 3-D effects of fusionless scoliosis treatments.

CASE REPORT

J Pediatr Orthop B. 2013 Jun 29. [Epub ahead of print]Rib osteoblastoma as an incidental finding in a patient with adolescent idiopathic scoliosis: a case report.Lykissas MG, Crawford AH, Abruzzo TA.

Source

aDepartment of Pediatrics, Division of Orthopaedic Surgery bDepartment of Pediatrics, Division of Interventional Radiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.

AbstractThe purpose of this article is to present an unreported case of rib osteoblastoma associated with progressive adolescent idiopathic scoliosis and to discuss thoracogenic scoliosis as a potential cause of curve progression after tumor resection. An 11-year and 8-month-old girl with adolescent idiopathic scoliosis was referred with an incidental finding of an expansile lesion in the posterior left seventh rib. A computed tomography-guided needle biopsy established the diagnosis of benign osteoblastoma. Transarterial embolization was performed followed by wide resection. Sixteen months after surgery the patient underwent posterior spinal fusion to address her scoliosis progression during the growth spurt. Forty-one and 25 months after rib resection and spinal fusion, respectively, the patient remains asymptomatic, without local tumor recurrence, and with excellent correction of her spinal deformity. Although scoliosis secondary to rib osteoblastoma has been described in the literature, rib osteoblastoma may coexist with idiopathic scoliosis. In such a case, surgical management of osteoblastoma should not interfere with treatment of idiopathic scoliosis.

CASE REPORT

Otol Neurotol. 2003 Sep;24(5):807-11.

Three cases of cochleosaccular endolymphatic hydrops without vertigo revealed by furosemide-loading vestibular evoked myogenic potential test.Seo T, Node M, Miyamoto A, Yukimasa A, Terada T, Sakagami M.

Source

Department of Otolaryngology, Hyogo College of Medicine, Hyogo, Japan. tseo@hyo-med.sc.jp

AbstractOBJECTIVE:To describe possible cases of cochleosaccular endolymphatic hydrops without vertigo.

STUDY DESIGN:Retrospective case report.

SETTING:University hospital.

PATIENTS:Three patients with possible cochleosaccular hydrops without vertigo were studied. The basis of diagnosis was positive result of the furosemide-loading vestibular evoked myogenic potential test, no canal paresis in the caloric test, and recurrent cochlear symptoms or fluctuating low-tone hearing loss.

CASE REPORT:In case 1, a 47-year-old woman had recurrent left aural fullness and tinnitus and a few weeks later complained of a floating sensation and could not stand up. The furosemide-loading vestibular evoked myogenic potential test showed a positive result in the left ear. In case 2, a 24-year-old woman complained of a backward falling sensation lasting several seconds; subsequently, a severe floating sensation persisted and she could not stand up for several days. Audiography showed fluctuating low-tone hearing loss in the left ear, and the furosemide-loading vestibular evoked myogenic potential test showed a positive result. In case 3, a 41-year-old woman had a floating sensation while walking and subsequently complained of tinnitus in the left ear. She could not stand up because of a severe floating sensation and, moreover, complained of a sudden falling sensation lasting for several seconds. The furosemide-loading vestibular evoked myogenic potential test indicated a positive result in the left ear.

CONCLUSIONS:The patients in cases 2 and 3 complained of a short-lasting sensation of falling down. Severe disequilibrium that prohibited standing up was noted in all cases. It was suggested that these symptoms were caused by saccular hydrops.

RESEARCH

Dtsch Arztebl Int. 2013 Jul;110(29-30):505-16. doi: 10.3238/arztebl.2013.0505. Epub 2013 Jul 22.

The treatment and natural course of peripheral and central vertigo.Strupp M, Dieterich M, Brandt T.

Source

Department of Neurology and German Center for Vertigo and Balance Disorders (IFB), Institute for Clinical Neurosciences, Ludwig-Maximilians University of Munich, Klinikum Großhadern.

AbstractBACKGROUND:Recent studies have extended our understanding of the pathophysiology, natural course, and treatment of vestibular vertigo. The relative frequency of the different forms is as follows: benign paroxysmal positional vertigo (BPPV) 17.1%; phobic vestibular vertigo 15%; central vestibular syndromes 12.3%; vestibular migraine 11.4%; Menière's disease 10.1%; vestibular neuritis 8.3%; bilateral vestibulopathy 7.1%; vestibular paroxysmia 3.7%.

METHODS:Selective literature survey with particular regard to Cochrane reviews and the guidelines of the German Neurological Society.

RESULTS:In more than 95% of cases BPPV can be successfully treated by means of liberatory maneuvers (controlled studies); the long-term recurrence rate is 50%. Corticosteroids improve recovery from acute vestibular neuritis (one controlled, several noncontrolled studies); the risk of recurrence is 2-12%. A newly identified subtype of bilateral vestibulopathy, termed cerebellar ataxia, neuropathy, and vestibular areflexia syndrome (CANVAS), shows no essential improvement in the long term. Long-term high-dose treatment with betahistine is probably effective against Menière's disease (noncontrolled studies); the frequency of episodes decreases spontaneously in the course of time (> 5 years). The treatment of choice for vestibular paroxysmia is carbamazepine (noncontrolled study). Aminopyridine, chlorzoxazone, and acetyl-DL-leucine are new treatment options for various cerebellar diseases.

CONCLUSION:Most vestibular syndromes can be treated successfully. The efficacy of treatments for Menière's disease, vestibular paroxysmia, and vestibular migraine requires further research.

ARTICLE REVIEW

HNO. 2013 Sep;61(9):752-61. doi: 10.1007/s00106-013-2749-5.[Indications for operative therapy of vestibular vertigo and the associated success rates].[Article in German]Westhofen M.

Source

Klinik für Hals-Nasen-Ohren-Heilkunde und Plastische Kopf- und Halschirurgie, Universitätsklinikum Aachen, RWTH Aachen University, Pauwelsstr. 30, 52074, Aachen, Deutschland, mwesthofen@ukaachen.de.

AbstractThe indications for surgical treatment of labyrinthine vertigo associated with severe impairment and a lack of response to medication are heterogeneous. Due to different therapeutic goals and success parameters, the results of treatments can only be compared to a limited extent. This overview of the current literature and procedures performed by the author contains recommendations for indications and outlines the risks associated with operative therapy of vestibular vertigo. Results of function-preserving and ablative therapies are compared. Surgical treatment of Menière's syndrome (non-idiopathic) using tympanostomy tubes is indicated in cases of increased middle ear pressure; Meniere's disease (idiopathic) in its early stages can be treated with the endolymphatic shunt operation to preserve hearing and balance functions and where these techniques fail, with vestibular neurectomy for preservation of hearing or with cochleosacculotomy in the case of deafness. Rare indications are intractable benign paroxysmal positional vertigo and superior semicircular canal dehiscence syndrome (SCDS). The function preservation success rate in cases of Meniere's syndrome and disease is 70-88 %, ablative procedures are effective in > 90 % of cases and occlusion of the superior or posterior canals is successful in > 95 % of patients.