Chloroquine-induced cardiomyopathy-echocardiographic features.

A 61-year-old woman with a 30-year history of systemic lupus erythematosus treated with chloroquine sulfate presented with complete heart block, congestive heart failure, and findings of restrictive cardiomyopathy on echocardiogram. Thickening of mitral, aortic, and tricuspid valves along with mild to moderate valve regurgitation was also present. Magnetic resonance imaging showed increased gadolinium uptake in the interventricular septum and the left ventricular lateral wall. Endomyocardial biopsy specimen showed marked myocardial cytoplasmic vacuolation and extensive myelin figures. Seven months after discontinuation of chloroquine, she showed significant clinical improvement and reversal of cardiomyopathy on echocardiography. This is the first case report describing a cardiomyopathy with prolonged use of chloroquine involving the conduction system, cardiac valves, and the myocardium with reversal on discontinuation of the drug.     

Strangulation in child abuse: CT diagnosis

The central nervous system is commonly affected in child abuse. Between April 1985 and July 1986 three infants were identified in whom the primary mode of injury had been strangulation. In each case computed tomography (CT) demonstrated a large cerebral infarction confined to vascular territories associated with small subdural hematomas. There was no history or visible evidence of significant head trauma. Autopsy of one infant confirmed the presence of a hemispheric infarct, thin subdural hematoma, and an area of subintimal hemorrhage in the carotid artery ipsilateral to the infarct. The remaining two patients survived with residual hemiparesis. CT findings of a large cerebral infarction with an associated subdural hematoma in an infant without a history of a significant trauma should suggest the possibility of child abuse and may be the primary manifestation of abuse in some patients.     

Early symptoms in spinocerebellar ataxia type 1, 2, 3, and 6

Onset of genetically determined neurodegenerative diseases is difficult to specify because of their insidious and slowly progressive nature. This is especially true for spinocerebellar ataxia (SCA) because of varying affection of many parts of the nervous system and huge variability of symptoms. We investigated early symptoms in 287 patients with SCA1, SCA2, SCA3, or SCA6 and calculated the influence of CAG repeat length on age of onset depending on (1) the definition of disease onset, (2) people defining onset, and (3) duration of symptoms. Gait difficulty was the initial symptom in two‐thirds of patients. Double vision, dysarthria, impaired hand writing, and episodic vertigo preceded ataxia in 4% of patients, respectively. Frequency of other early symptoms did not differ from controls and was regarded unspecific. Data about disease onset varied between patients and relatives for 1 year or more in 44% of cases. Influence of repeat length on age of onset was maximum when onset was defined as beginning of permanent gait disturbance and cases with symptoms for more than 10 years were excluded. Under these conditions, CAG repeat length determined 64% of onset variability in SCA1, 67% in SCA2, 46% in SCA3, and 41% in SCA6 demonstrating substantial influence of nonrepeat factors on disease onset in all SCA subtypes. Identification of these factors is of interest as potential targets for disease modifying compounds. In this respect, recognition of early symptoms that develop before onset of ataxia is mandatory to determine the shift from presymptomatic to affected status in SCA. © 2008 Movement Disorder Society     

疟原虫组织期裂殖体杀灭剂的研究:2-取代苄氧基(或甲氧基)-5-取代苯氧基-伯氨喹类似物的合成

本文报道了间日疟根治药伯氮喹2位引入取代苄氧基或甲氧基,5位引入取代苯氧基的类似物的合成。其中以化合物39及45对疟原虫组织期裂殖体的作用最强,约氏疟原虫子孢子感染的小鼠喂服100mg/kg单剂,分别有80%及90%的受试小鼠未查见原虫血症。化合物45降至20mg/kg单剂时,80%的受试小鼠也未出现原虫;对小鼠的急性毒性低于伯氨喹。     

Infusible platelet membrane microvesicles: a potential transfusion substitute for platelets

BACKGROUND: Several substitutes for intact, viable platelets have been used for transfusion, both to people and in animal models, with varied success. Infusible platelet membrane (IPM) is prepared from human platelets. IPM retains the glycoprotein (GP)lb receptor and has platelet factor 3 activity (procoagulant activity). However, factor V, serotonin, a cytoplasmic marker enzyme (purine nucleotide phosphorylase), GPIIb/IIIa complex, and HLA class I and II antigens are all absent in IPM. STUDY DESIGN AND METHODS: IPM is prepared from outdated platelets. The platelets were disrupted by freezing and thawing; they were washed and heated to inactivate possible viral contaminants, and then the sonicated membrane microvesicle fraction was separated and lyophilized. The hemostatic activity of IPM was measured by its ability to reduce the prolonged bleeding time in thrombocytopenic rabbits. RESULTS: Administration of IPM at a dose of 2 mg per kg results in a substantial reduction in the bleeding time. In a series of 23 experiments, a median preinjection bleeding time of 15 minutes was reduced to 6 minutes within 4 hours after IPM administration. Administration of IPM did show a mild enhancement in the thrombogenicity index, as measured in the Wessler rabbit model. This enhancement is, however, not significant, as a thrombogenicity index value of up to 0.6 is clinically acceptable. CONCLUSION: IPM may have clinical potential as a substitute for platelets in the treatment of bleeding due to thrombocytopenia.     

Knowledge and awareness about diabetes and periodontal health among Jordanians

The aim of this study is to evaluate the awareness, perception, sources of information, and knowledge of diabetes mellitus and periodontal health among Jordanians, to examine the factors related to their knowledge, and organize effective education programs. A random sample of 500 diabetic patients was recruited from three hospitals and three comprehensive health centers that represent both urban and rural populations in Jordan between September 25, 2006, and February 20, 2007. Completed questionnaires with the answers were returned by 405 participants (response rate was 81%). Only 28% indicated that they followed up gum diseases with the dentist; 48% were aware that diabetic patients are more prone to gum diseases and oral health complications. About a third (38%) recognized that their periodontal health might affect their glycaemic level. Television and Internet were the main source of knowledge for dentists with the rate of 50%. Knowledge about diabetes and periodontal health among diabetic patients is low, and majority of patients were unaware of the oral health complications of their disease and the need for proper preventive care. Issues on education need to be addressed. Therefore, appropriate educational programs should be planned according to community needs, and the target of these programs should be patients with irregular visits to the dentist and physicians. The clinical implication of our findings is that dentists, physicians, and other health providers should inspect diabetic patients for gum diseases each time they come for care and recommend that diabetic patient see a dentist regularly.     

An Anatomical and Analytical Study of the Modiolus: Enlightening Its Relevance to Plastic Surgery

Background  This study was designed to evaluate the structures, muscles, and fasciae of which the modiolus is composed. It can aid in the understanding and, therefore, the utilization of plastic surgery for the aesthetic or reconstructive treatment of that region, especially the angle of the mouth. Methods  Dissections of the midface were done on five different cadavers. They were of different races (3 males, 2 females). The anatomy of the modiolus was studied in detail. New anatomical observations were classified as type I through type VI. Results  The perifacial artery fascia contributed to the modiolus in four (80%) specimens and was not part of it in 1 (20%) specimen. The facial artery was anterior to it in one (20%) specimen, lateral in four (80%) specimens, and never medial to it. No significant relationship was observed between the perifacial artery fascia contribution to the modiolus and gender or race. Also, the location of the facial artery lateral or anterior to the modiolus was not significantly related to gender or race. In addition, the deep and superficial fasciae of the face converged not anterior to the masseter muscle but actually at the modiolus, which was different from observations made by others. Conclusion  The modiolus is of critical importance in aesthetic and reconstructive plastic surgery of the face.     

The expression of type III hyperlipoproteinemia: involvement of lipolysis genes

Type III hyperlipoproteinemia (HLP) is mainly found in homozygous apolipoprotein (APO) E2 (R158C) carriers. Genetic factors contributing to the expression of type III HLP were investigated in 113 hyper- and 52 normolipidemic E2/2 subjects, by testing for polymorphisms in APOC3, APOA5, HL (hepatic lipase) and LPL (lipoprotein lipase) genes. In addition, 188 normolipidemic Dutch control panels (NDCP) and 141 hypertriglyceridemic (HTG) patients were genotyped as well. No associations were found for four HL gene polymorphisms and two LPL gene polymorphisms and type III HLP. The frequency of the rare allele of APOC3 3238 G>C and APOA5 −1131 T>C (in linkage disequilibrium) was significantly higher in type III HLP patients when compared with normolipidemic E2/2 subjects, 15.6 vs 6.9% and 15.1 vs 5.8%, respectively, (P<0.05). Furthermore, the frequencies of the APOA5 c.56 G>C polymorphism and LPL c.27 G>A mutation were higher in type III HLP patients, though not significant. Some 58% of the type III HLP patients carried either the APOA5 −1131 T>C, c.56 G>C and/or LPL c.27 G>A mutation as compared to 27% of the normolipidemic APOE2/2 subjects (odds ratio 3.7, 95% confidence interval=1.8–7.5, P<0.0001). The HTG patients showed similar allele frequencies of the APOA5, APOC3 and LPL polymorphisms, whereas the NDCP showed similar allele frequencies as the normolipidemic APOE2/2. Patients with the APOC3 3238 G>C/APOA5 −1131 T>C polymorphism showed a more severe hyperlipidemia than patients without this polymorphism. Polymorphisms in lipolysis genes associate with the expression and severity of type III HLP in APOE2/2.     

Contemporary approach to the problem of depression and disturbances of mental health in mentally disabled people

The approach towards the issues of depression and mental health problems of mentally disabled persons is discussed in the paper. The author discussed the various susceptibilities, resistances and typical mental health disturbances of mentally disabled persons as well as their diagnostic criteria and methods of measuring them. A longer part of the paper concerns the approach towards depressive disorders in the population with developmental disabilities.