Interaction of haemoglobin E with alpha-thalassaemia and haemoglobin Constant Spring.

The combination of Hb E,alpha-thalassaemia and Hb CoSp was found in a 20-year-old female Malay who presented with a moderately severe haemolytic anaemia. The findings in the patient and her family from which this diagnosis was arrived at are discussed. Although this is the first report of this condition in this country it is pointed out that one may see more such cases in the future if one is aware of this condition since Hb E, alpha-thalassaemia and Hb CoSp all occur at significant frequencies in this country.     

The Q1 deflection of the electrocardiogram in bundle branch block and axis deviation

An initial downward, or Q, deflection in Lead I is very uncommon in human left branch block. When this component occurs in an electrocardiogram otherwise characteristic of this conduction defect, a lesion of the ordinary muscle of the ventricular septum should be suspected, and a full set of precordial leads should be taken.A Q deflection in Lead I occurs in about one-half of all cases of left axis deviation, regardless of the criteria employed in selecting examples of this electrocardiographic abnormality. Left axis deviation accompanied by inversion of the T waves in Lead I may sometimes be due to incomplete left bundle branch block when Q₁ is absent, but it is almost never due to this cause when this deflection is present.The incidence of Q₁ in right branch block is similar to its incidence in left axis deviation. In right axis deviation, this deflection is extremely rare.     

Other systemic manifestations of chronic obstructive pulmonary disease

Chronic obstructive pulmonary disease (COPD) no longer is regarded simply as a disease of the lungs and airways. There is growing awareness of the multisystemic nature of this disease. Research has shown increased levels of systemic inflammation and cardiovascular, neurologic, psychiatric, and endocrine system dysfunction associated with COPD. Skeletal muscle dysfunction associated with COPD is discussed in the article by Schols in this issue of Clinics in Chest Medicine. In this article, other systemic manifestations of COPD are discussed. Although this field is expanding rapidly, this article focuses on recent advances and reviews.     

Heart Failure in South Asia

South Asia (SA) is both the most populous and the most densely populated geographical region in the world. The countries in this region are undergoing epidemiological transition and are facing the double burden of infectious and non-communicable diseases. Heart failure (HF) is a major and increasing burden all over the world. In this review, we discuss the epidemiology of HF in SA today and its impact in the health system of the countries in the region. There are no reliable estimates of incidence and prevalence of HF (heart failure) from this region. The prevalence of HF which is predominantly a disease of the elderly is likely to rise in this region due to the growing age of the population. Patients admitted with HF in the SA region are relatively younger than their western counterparts. The etiology of HF in this region is also different from the western world. Untreated congenital heart disease and rheumatic heart disease still contribute significantly to the burden of HF in this region. Due to epidemiological transition, the prevalence of hypertension, diabetes mellitus, obesity and smoking is on the rise in this region. This is likely to escalate the prevalence of HF in South Asia. We also discuss potential developments in the field of HF management likely to occur in the nations in South Asia. Finally, we discuss the interventions for prevention of HF in this region     

Severe invalidating pain syndrome associated with benznidazole therapy for Chagas’ disease

Chagas’ disease is an endemic parasitic disease and constitutes an important health problem in Latin American countries. The increasing number of immigrants from these countries has resulted in a rise in diagnosis and consequently in the treatment of this disease in developed countries not familiar with this condition. Currently, benznidazole is used for treatment of this condition. However, undesirable effects have been reported with this treatment, and there are few data about continuous long-term use of this drug. We describe a case of invalidating pain syndrome in a 31-year-old Bolivian woman with Chagas’ disease while receiving benznidazole therapy. Because of the number of cases with this condition will probably increase because of immigration, a better understanding of the side effects of the treatment of this disease is essential.     

Lessons learned from previous dengue outbreaks

Dengue is an important tropical infection caused by an arbovirus dengue. As a mosquito borne infection, this disease is widely spread in several tropical endemic countries. Millions of world populations are at risk for this arboviral infection. Each year, thousands of dengue infections are reported and there are several death cases. Each year, the outbreaks of dengue emerge in several countries and this implies the global importance of this infection. Fighting with dengue outbreak is important in public health. In this specific chapter, the author discussed lessons learned from previous dengue outbreaks.     

The tetralogy of Eisenmenger

A case of the tetralogy of Eisenmenger is presented. The literature pertaining to this complex is reviewed, and the clinical differentiation of this anomaly from the tetralogy of Fallot is discussed. To explain this anomaly, a new theory of transposition is presented. The Eisenmenger complex is produced by an abnormality in the formation of bulbar ridge 3B. Instead of this ridge, the reptilian ridge 4B develops. The subsequent absorption of this abnormally formed bulbus proceeds in an abnormal fashion, causing both the aorta and pulmonary artery to arise from the right ventricle, as in our case, or producing a riding aorta with a septal defect, or a mild form of transposition with an aneurysm of the membranous septum.     

Glycaemic management during the inpatient enteral feeding of people with stroke and diabetes

This paper is an abridged and modified version of guidelines produced by the Joint British Diabetes Societies for inpatient care on glycaemic management during the enteral feeding of people with stroke and diabetes. These were revised in 2017 and have been adapted specifically for Diabetic Medicine. The full version can be found at: www.diabetes.org.uk/joint-british-diabetes-society or https://abcd.care/joint-british-diabetes-societies-jbds-inpatient-care-group . Many people have both diabetes and an acute stroke, and a stanv dard approach to the management of people with stroke is the provision of adequate nutrition. Frequently, this involves a period of enteral feeding if there is impaired ability to swallow food safely. There is currently considerable variability in the management of people with diabetes fed enterally after a stroke, and the evidence base guiding diabetes management in this clinical situation is very weak, although poor glycaemic outcomes in people receiving enteral feeding after stroke may worsen recovery and cause harm. The aim of this document is to provide sensible clinical guidance in this area, written by a multidisciplinary team; this guideline had input from diabetes specialist nurses, diabetologists, dietitians, stroke physicians and pharmacists with expertise in this area, and from UK professional organizations. It is aimed at multidisciplinary teams managing people with stroke and diabetes who require enteral feeding. We recognize that there is limited clinical evidence in this area.     

Recent research advances on Chromobacterium violaceum

Chromobacterium violaceum is a gram-negative bacterium, which has been used widely in microbiology labs involved in quorum sensing(QS) research. Among the QS-regulated traits of this bacterium, violacein production has received the maximum attention. Violacein production in this organism, however is not under sole control of QS machinery, and other QSregulated traits of this bacterium also need to be investigated in better detail. Though not often involved in human infections, this bacterium is being viewed as an emerging pathogen. This review attempts to highlight the recent research advances on Chromobacterium violaceum, with respect to violacein biosynthesis, development of various applications of this bacterium and its bioactive metabolite violacein, and its pathogenicity.     

The Keystones of Right Ventricular Arrhythmogenic Cardiomyopathy-Induced Morphological Disarrangement

Arrhythmogenic right ventricular cardiomyopathy is an urgent problem of modern cardiology. This myocardial remodeling manifests various desmosomopathies, channelopathies, and other mutations resulting in a violation of the coordinated heart work, particularly the myocardium. The incidence of this cardiomyopathy is not significant. Still, it is worth noting that athletes are at an increased risk of developing this disease, emphasizing the importance of studying this topic and its relevance from cardiologists and sports physicians. Moreover, the clinical pattern of this disease is heterogeneous. This pathology requires strengthening control and attention of medical personnel and constant improvement and optimization of diagnostic methods and treatment protocols. In this article, the pathophysiological mechanisms, molecular genetic aspects, and the dynamics of morphofunctional changes are represented in detail. Understanding the mechanisms of etiopathogenesis and the features of morphological changes observed in this cardiomyopathy and its more detailed study is fundamental in developing modern treatment methods to improve patients' quality and life expectancy.     

结直肠肿瘤类-NOTES术之现状及展望

随着腹腔镜技术在临床中的广泛开展和应用,微创理念和功能外科越来越多地受到了关注,并且在肿瘤的外科治疗中占有重要地位。类-NOTES术即为在该治疗背景之下的又一创新术式,该手术能够在保证肿瘤根治的前提之下,最大程度地保留器官和机体的功能,同时又具有良好的操作性和推广性,不失为是一种理想的微创治疗手段。之所以称之为类-NOTES,是因为经自然腔道取标本手术腹壁疤痕小,微创效果与NOTES相近。同时,NOTES又是医生和病人共同追求的最高目标,所以该名称也是一种文化和愿望的表达。但事实上,这类手术可以归为经自然腔道取标本的外科手术(Natural orifice specimen extraction surgery,NOSES)。笔者将结合我中心开展的160例类-NOTES术,对该手术进行全面的分析和总结,也希望类-NOTES术能够为改善肿瘤患者的治疗效果和生存质量发挥更大的作用。     

Multiple polyposis of the gastrointestinal tract with osteoma and soft tissue tumors

Summary The combination of soft-tissue tumors, osteomas, and multiple polyps of the colon is known as Gardner's syndrome. Gardner in 1950 recognized this triad as a familial disease with a mendelian autosomal dominant inheritance.A case history of this entity is presented. It is generally accepted that the colonic polyposis in this disorder is similar in pathology and prognosis to that of classical familial polyposis. It is hoped that awareness of this syndrome with recognition of the external manifestations will lead to early diagnosis prior to the onset of carcinomatous changes in the polyps.Supported in part by The Upjohn Gastrointestinal Foundation, Inc. Mr. Rudolph J. Henning, R.B.P., produced the photographs.Upjohn Fellow in Gastroenterology.     

Idiopathic acute fatty liver of pregnancy

A maternal survivor of idiopathic fatty liver, with the characteristic histological appearance on liver biopsy, is reported. Profound hypoglycaemia was the major complication in this patient and it is suggested that more attention should be given to the blood glucose in this disease. The consideration of future pregnancy in this patient is discussed.     

Le tabac chez les adolescents. Comment les convaincre de ne pas fumer ? Comment les aider à arrêter de fumer ?

Adolescence is an age prone to emerging addictive behaviours such as smoking. The relationship of adolescents with adults at home and at school is complex during this period of transition to adulthood. It is important that the bond between adolescents and adults should be secured and enriched. Smoking starts as a social behaviour that is still valued in our culture and by our peers. Youngsters are then trapped into this pattern, and a strong addiction makes is difficult for them to change. This article will look at the specificities of smoking in adolescents, the specificities of withdrawal, and the means recognised as the most efficient for prevention and for withdrawal support. Documented by recent articles on the subject and by my personal experience in a school environment and with adolescents, this article will aim at defining the most appropriate means in this struggle against this undesirable smoking behaviour in young people.     

Molecular insights into gastrointestinal neuroendocrine tumours: importance and recent advances

A subset of gastrointestinal neuroendocrine tumours (carcinoids and pancreatic endocrine tumours) show aggressive growth. Early identification of this subset is essential for management; however, clinical, laboratory and histologic features frequently fail to achieve this. Currently, there is an increased understanding of the molecular pathogenesis/changes in neuroendocrine tumours and this may identify important prognostic factors and possibly, new treatments. Recent findings and progress in this area are briefly reviewed in this article.     

Osteoporosis in men.

Osteoporosis has long been considered to be a disease of the aging female skeleton. As awareness of the pervasiveness of this disorder increases, it is clear that men are also at risk for this disorder. Recent epidemiological studies have confirmed that osteoporosis in men is an increasing health problem. This development not only has its roots in increased longevity but also in increased awareness of this problem in men. The purpose of this article is to review what is known about the factors in men that lead to acquisition, maintenance, and loss of bone, as well as new insights about the causes, pathogenesis, and treatment of osteoporosis in men.     

Sleep in the intensive care unit

Sleep is commonly disrupted in intensive care unit patients. The causes of this sleep disruption include the underlying medical illness itself, intensive care unit (ICU) environment, psychological stress, and effects of many medications and other treatments used to help those who are critically ill. The purpose of this review is to discuss the relevant literature in this regard, in order to improve the knowledge and recognition of this problem by health care providers. Also general and specific integrative steps to improving sleep of patients in the ICU is also described.     

Left Ventricular Noncompaction

According to the World Health Organization classification of cardiomyopathies, left ventricular noncompaction is still an unclassified cardiomyopathy. In 2006, the American Heart Association classified this entity as a primary cardiomyopathy of genetic origin. In 2008, the European Society of Cardiology updated the classification scheme similar to the World Health Organization classification. At present, there is no consensus on the diagnostic criteria, and diagnosis is based on the morphologic features identified by cardiac imaging studies or at autopsy. Due to lack of standardization of the diagnostic criteria and little awareness of this condition among clinicians, the true prevalence of this disease is not clear. There is no specific therapy for this condition. However, it seems prognosis is much better than initially reported. The current status of diagnosis, prognosis, and management of isolated noncompaction in adults is discussed in this review.     

Microscopic polyangiitis in an adolescent presenting as severe anemia and syncope

Microscopic polyangiitis (MPA) is an autoimmune systemic vasculitis of small vessels. The condition has been best characterized in older adults and little is known of the natural history of this disease in children and adolescents. In this report, a case of an adolescent presenting with symptomatic anemia and syncopal episodes is described. An extensive evaluation ultimately led to the diagnosis of MPA. The unique findings in this case and review of the literature are presented, outlining the variable clinical presentations and challenge of diagnosing this condition in pediatric patients.     

Energy change in the formation of conjugated bilirubin: a possible responsive mechanism for liver cell pathology.

Bilirubin is formed when red blood cells die and their hemoglobin is broken down within the macrophages into heme and globins. In the liver, bilirubin is conjugated with UDP-glucuronate, making it water-soluble diglucuronide. Concerning this conjugation, a molecule of bilirubin reacts with two molecules of glucoronic acid. However, the nature of this energy-consuming reaction in the formation of conjugated bilirubin has never been reported, and this can be important for its potential implication in hyperbilirubinemia. In this work, the author calculated the energy required by conjugated-bilirubin formation per molecule. The energy required for complex formation is -22 kCal/mol. The nature of this energy-producing reaction can be a good explanation. Increased energy delivery in conjugated hyperbilirubinemia in liver disease might be a responsive mechanism to hepatic damage.