Male Predominance (male + predominance)

Distribution by Scientific Domains
Distribution within Medical Sciences


Selected Abstracts


Androgens and Male Predominance of the Brugada Syndrome Phenotype

PACING AND CLINICAL ELECTROPHYSIOLOGY, Issue 7p1 2003
CHARLES ANTZELEVITCH
No abstract is available for this article. [source]


The burden of paediatric stroke and cerebrovascular disorders in Croatia

INTERNATIONAL JOURNAL OF STROKE, Issue 5 2009
J. Lenicek Krleza
Pediatric stroke is significantly less common than stroke in adults, but represents a major challenge to public health authorities. The aim of this retrospective study was to identify the total and annual number of children younger than 18 years with arterial ischaemic stroke and transient ischaemic attack referred to the Children's Hospital Zagreb, which is a major national centre specialised for the treatment and prevention of stroke in children. We reviewed the medical records of the Department of Neuropediatrics database at the Children's Hospital Zagreb between 1998,2005 in order to provide demographic and clinical characteristics and neuroimaging findings in children with arterial ischaemic stroke. In the 7-year period, we identified a total of 124 children from different geographic areas of Croatia with a confirmed diagnosis of transient ischaemic attack (N=77), and arterial ischaemic stroke (N=47). Perinatal and childhood arterial ischaemic stroke were equally represented (23 and 24 children, respectively). The average number of new cases identified each year was 18 cases (range: 12,21), seven arterial ischaemic stroke and 11 transient ischaemic attack cases. Male predominance was found in children with arterial ischaemic stroke with a male : female ratio of 1·76 : 1, and was slightly higher in childhood arterial ischaemic stroke compared with perinatal arterial ischaemic stroke (2 : 1 and 1·56 : 1, respectively). In contrast, transient ischaemic attack was more frequently found in girls, and more likely identified in older children compared with younger children with arterial ischaemic stroke. Obtained data will contribute to better understanding of paediatric stroke in Croatia and will provide a base for the establishment of the national referral center and national pediatric stroke registry. [source]


Habitual snoring in an outpatient population in Japan

PSYCHIATRY AND CLINICAL NEUROSCIENCES, Issue 4 2000
Yuhei Kayukawa MD
Abstract In order to investigate the occurrence and history of sleep problems in Japan, the 11-Centre Collaborative Study on Sleep Problems (COSP) project was carried out. Complaints of snoring are examined, and its prevalence, risk factors and screening reliability are discussed. The subjects who participated in the study were 6445 new outpatients from a general hospital. They were asked to answer a sleep questionnaire that consisted of 34 items with seven demographic items; each item was composed of four grades of frequency. In order to offset possible seasonal variations in sleep habits, data were collected across four seasons. Sleep patterns, insomnia, hypersomnia, parasomnia and circadian rhythm sleep disorders were covered. Habitual snoring was seen in 16.0% of males and 6.5% of females. Male predominance was noted. From these data, the relationship between habitual snoring and sleep complaints was statistically analyzed. Habitual snorers (HS) were observed to wake up more frequently during sleep (17.8% of males, 21.5% of females) than were non-habitual snorers (NHS; 6.6% of males, 9.7% of females). Mid-sleep awakening of HS was also more frequent than it was for NHS; however, there were no differences in difficulty in falling asleep and early morning awakening. Body mass index, cigarette smoking and alcohol consumption were also correlated with habitual snoring. [source]


Epstein-Barr virus associated gastric carcinoma: Epidemiological and clinicopathological features

CANCER SCIENCE, Issue 2 2008
Suminori Akiba
In this paper, the roles of Epstein-Barr virus (EBV) in gastric carcinogenesis are discussed, reviewing mainly epidemiological and clinicopathological studies. About 10% of gastric carcinomas harbor clonal EBV. LMP1, an important EBV oncoprotein, is only rarely expressed in EBV-associated gastric carcinoma (EBV-GC) while EBV-encoded small RNA is expressed in almost every EBV-GC cell, suggesting its importance for developing and maintaining this carcinoma. In addition, the hypermethylation-driven suppressor gene downregulation, frequently observed in EBV-GC, appears to give a selective advantage for carcinoma cells. EBV reactivation is suspected to precede EBV-GC development since antibodies against EBV-related antigens, including EBV capsid antigen (VCA), are elevated in prediagnostic sera. Interestingly, the average anti-VCA immunoglobulin G antibody titer in EBV-GC patients was significantly higher among men than among women, whereas EBV-negative GC cases did not show such a sex difference. A higher frequency of human leucocyte antigen-DR11 in EBV-GCs suggests that major histocompatibility complex-restricted EBV nuclear antigen 1 epitope recognition may enhance EBV reactivation. EBV infection of gastric cells by lymphocytes with reactivated EBV is suspected to be the first step of EBV-GC development. Male predominance of EBV-GC suggests the involvement of lifestyles and occupational factors common among men. The predominance of EBV with XhoI+ and BamHI type i polymorphisms in EBV-GC in Latin America suggests a possibility of some EBV oncogene expressions being affected by EBV polymorphism. The lack of such predominance in Asian countries, however, indicates an interaction between EBV polymorphism and the host response. In conclusion, further studies are necessary to examine the interaction between EBV infection, its polymorphisms, environmental factors, and genetic backgrounds. (Cancer Sci 2008; 99: 195 ,201) [source]


Type 1 diabetes mellitus in Czech children diagnosed in 1990,1997: a significant increase in incidence and male predominance in the age group 0,4 years

DIABETIC MEDICINE, Issue 1 2000
O. Cinek
Summary Aims To overview total, age-and sex-specific incidence rates of type 1 diabetes mellitus and their trends in Czech children 0,14 years of age in the period of 1990,1997. Methods Type 1 DM cases were ascertained by two independent sources, data of general population were obtained from the annual demographic reports of the State Statistic Bureau. Incidence rates were computed using both ascertainment sources combined. Results In the study period 1.1.1990,31.12.1997, the total incidence was 10.1 (95% CI 9.6,10.6) per 100 000/year in both sexes, 10.0 (95% CI 9.4,10.7) in boys, and 10.2 (95% CI 9.5,11.0) in girls. The total age-standardized incidence was 9.9 (95% CI 9.4,10.4). The total incidence had a significant increasing trend over the study period (P = 10,4, annual increment 4.3%). A significant increasing trend was also found in the groups of children 0,4 (P = 0.033, increment 6.9%) and 5,9 years at diagnosis (P = 0.038, increment 4.8%). Statistically significant male predominance was observed in the group diagnosed at age 0,4 years (boys/girls ratio of incidence 1.33, P = 0.035). Conclusions We report the first population-based epidemiological data on incidence of childhood Type 1 DM in the Czech Republic. The incidence has increased significantly during the last 8 years. The present incidence is at an intermediate level compared to other European countries. [source]


A 9-year review of dystonia from a movement disorders clinic in Singapore

EUROPEAN JOURNAL OF NEUROLOGY, Issue 1 2006
R. D. G. Jamora
The clinical features of dystonia have not been evaluated in Southeast Asia. We therefore investigated the clinical spectrum and characteristics of dystonia in Singapore, a multi-ethnic Southeast Asian country comprising 77% Chinese, 14% Malays, and 8% Indians. We identified all dystonia patients from the Movement Disorders database and Botulinum Toxin clinic between 1995 and November 2004. Their medical records were reviewed to verify the diagnosis of dystonia and obtain demographic and clinical data using a standardized data collection form. A total of 119 (73%) patients had primary dystonia whilst 45 (27%) had secondary dystonia. There were 77% Chinese, 9% Malays, and 8% Indians. The most common focal dystonia were cervical dystonia (47%), writer's cramp (32%), and blepharospasm (11%). There was no significant difference in the distribution of dystonia between the different races. Males were noted to have earlier onset of dystonia overall. There was a significant male predominance in primary dystonia overall (M:F 1.6:1, P = 0.008) and in the subgroup of focal dystonia (M:F 1.6:1, P = 0.037). This contrasts with previous studies that found a female predominance. The role of genetic, hormonal, and environmental factors and their interactions need to be investigated to better understand the gender differences in the occurrence of dystonia. [source]


Hepatocellular carcinoma occurring in nonfibrotic liver: Epidemiologic and histopathologic analysis of 80 French cases

HEPATOLOGY, Issue 2 2000
Marie-Pierre Bralet M.D., Ph.D.
Hepatocellular carcinoma (HCC) occurring in nonfibrotic liver represents a rare, ill-defined subgroup of HCC without cirrhosis in which mechanisms of hepatocarcinogenesis remain unclear. The aim of our study was to assess epidemiological factors and detailed histopathologic changes in the nontumoral liver of patients developing such tumors. Of 330 HCCs resected in our institution between 1985 and 1998, we retrospectively analyzed 80 cases (53 men, 27 women; mean age, 51 ± 16 years) in which the nontumoral liver showed no (n = 28) or minimal (n = 52) portal fibrosis without any septal fibrosis. In the group with no portal fibrosis there was no male predominance, and patients were significantly younger (44 ± 19 years vs. 54 ± 14 years) than those with minimal portal fibrosis. Sixty-seven tumors were typical HCCs, 8 were of fibrolamellar type, and 5 were hepatocholangiocarcinomas. Mean tumor size was 10 ± 5 cm. Risk factors for HCC development were found in 30 patients: hepatitis B (n = 17) or C (n = 2) virus infections, alcohol consumption (n = 11), and hemochromatosis (n = 1). In the nontumoral liver, periportal and lobular necrosis, mild portal inflammation, steatosis, and iron overload were present in 15%, 57%, 52%, and 54% of cases, respectively. Liver cell changes were noted in 6%. This study emphasizes the need for strict criteria to classify HCC without cirrhosis. HCC in nonfibrotic liver is a distinct subgroup in which nontumoral liver shows nonspecific minimal changes without regeneration or premalignant lesion. Etiologic factors are often unidentified, although presence of HBV infection in 21% suggests a direct oncogenic role of this virus. [source]


Prevalence of Pelvic Paget's Disease of Bone in the United States

JOURNAL OF BONE AND MINERAL RESEARCH, Issue 3 2000
R. D. Altman M.D.
Abstract The objective of this article was to estimate the prevalence of Paget's disease of bone in the United States from a statistically derived sample of the general population. Pelvic radiographs obtained in the First National Health and Nutrition Examination Survey (NHANES-I) were reviewed for the presence of Paget's disease. Age, sex, and geographic distribution of Paget's disease of the pelvic region were determined. The overall prevalence of Paget's disease in the United States was estimated. Pelvic Paget's disease is estimated to be present in 0.71 + 0.18% of the radiographs of the general population. The disease was higher in frequency in people who were in the older decades of life with the highest prevalence of 2.32 + 0.54% in the 65- to 74-year-old people. There is a slight male predominance in the 45- to 74-year age group. The regional distribution suggests the highest prevalence in the Northeast (1.48 + 0.52%) with the lowest prevalence in the South (0.26 + 0.25%). The prevalence was equal in white people and black people. An estimate of the overall prevalence of Paget's disease in the United States was at least 1% and perhaps as much as 2% of the general population with near equal sex distribution and the highest prevalence in the northeastern United States. [source]


Prognostic factors and outcome after drowning in an adult population

ACTA ANAESTHESIOLOGICA SCANDINAVICA, Issue 7 2009
M. Á. BALLESTEROS
Background: Drowning remains an actual problem. Although medical assistance has improved, it still has high rates of morbidity and mortality. We set out to explore the clinical characteristics and outcome of drowning patients admitted to the intensive care unit (ICU) of tertiary-care university hospital. Methods: We designed a retrospective observational study to analyse all drowning patients admitted to our ICU after successful cardiopulmonary resuscitation. The study was conducted during 1 January 1992,31 December 2005. There was no exclusion. We used a univariate analysis to evaluate the effect on patient and management characteristics on survival. Results: There were 43 patients (five children and 38 adults), with male predominance. Fifteen patients, all adults (34.9%), died. Submersion time, age, Glasgow Coma Score (GCS), pupillary reactivity and acute physiology and chronic health evaluation (APACHE II) at ICU admission were related to mortality. Non-survivors presented a higher glycaemia level at ICU admission than survivors (P=0.005). Conclusions: The outcome is closely related to the patient's clinical status on arrival to the hospital. We have found that submersion time, age, GCS, pupillary reactivity and APACHE II at ICU admission were related to mortality. Further research in prospective studies is needed. [source]


Inflammatory bowel disease in the Asia,Pacific area: A comparison with developed countries and regional differences

JOURNAL OF DIGESTIVE DISEASES, Issue 3 2010
Vineet AHUJA
The Asia,Pacific region has been marked as an area with a low incidence of inflammatory bowel disease (IBD), although confusion always existed as to whether this low incidence was a result of low diagnostic awareness, a high incidence of infective diarrhoea and its diagnostic overlap or a true low incidence. As epidemiological studies from this region are being made available it is clear that the incidence and prevalence rates of IBD in Asia,Pacific region are low compared with Europe and North America. They are however, increasing rapidly. There are substantial variations in the incidence and prevalence rates of IBD in various ethnic groups in Asia. The highest incidence rates are recorded from India, Japan and the Middle East and there exists a genetic predisposition of South Asians (Indians, Pakistanis and Bangladeshis) to ulcerative colitis (UC). It appears that certain racial groups are more prone than others to develop IBD. For instance, Indians in South-East Asia have higher rates than Chinese and Malays. While there is a host genetic predisposition, environmental factor(s) may be responsible for this difference. The clinical phenotypes and complication rates of Asian IBD resemble those of the Caucasian population in general, but some heterogeneity is observed in different regions of Asia. There is no evidence of a north-south or an east-west divide in the Asia,Pacific region. The available studies suggest an increasing incidence of UC in the Asia,Pacific region and hence it is an appropriate time to launch well-designed epidemiological studies so that etiopathogenetic factors can be identified. There is a male predominance in Crohn's disease in the Asian population. The NOD2/CARD15 gene is not associated with CD in the Japanese, Korean, Chinese and Indian population. [source]


Efficacy of lamivudine on hepatitis B viral status and liver function in patients with hepatitis B virus-related hepatocellular carcinoma

LIVER INTERNATIONAL, Issue 2 2009
Ji Hoon Kim
Abstract Background/Aims: Treatment of patients with hepatocellular carcinoma (HCC) depends on the tumour extent and underlying liver function. Antiviral therapy with nucleoside/nucleotide analogues has been shown to be effective in improving the liver function of chronic hepatitis B (CHB) patients. We assessed whether lamivudine could induce biochemical and virological improvements in patients with hepatitis B virus-related HCC. Patients/Methods: Of 148 CHB patients treated with 100 mg/day lamivudine for at least 6 months, 80 had HCC (CHB/HCC group) and 68 did not (CHB group). Biochemical and virological parameters were serially monitored. Results: Compared with the CHB group, the CHB/HCC group was older, had higher male predominance, bilirubin levels and liver cirrhosis rate, and lower albumin and hepatitis B virus (HBV) DNA levels and hepatitis B e antigen (HBeAg) positivity (P<0.05 each). The two groups showed similar cumulative rates of alanine aminotransferase normalization, HBV DNA seroconversion, HBeAg loss and viral breakthrough during 12 months of lamivudine treatment. After 12 months, the CHB/HCC group showed, relative to baseline, increased albumin levels (3.51±0.5 vs. 3.72±0.5 mg/ml) and decreased ascites scores (1.63±0.7 vs. 1.45±0.6) and Child,Pugh scores (6.92±1.9 vs. 6.02±1.38) (P<0.05 each). Conclusion: Lamivudine had comparable antiviral effects both in patients with CHB and CHB/HCC, and improved underlying liver function in the latter group. Treatment of HBV may increase the chance of curative treatments in patients with HBV-related HCC. [source]


Spontaneously changing muscular activation pattern in patients with cervical dystonia

MOVEMENT DISORDERS, Issue 6 2001
A. Münchau MD
Abstract The objective of this study was to determine stability of the neck muscle activation pattern in a given dystonic head position in patients with cervical dystonia (CD). We assessed 26 patients with CD and botulinum toxin (BT) treatment failure before surgical denervation. None of them had received BT injections for at least 4 months. To relate dystonic head position to underlying neck muscle activity, we used synchronised video and poly-electromyographic (EMG) recording over a period of 10 minutes. The muscle activation pattern during constant ("stable") maximal dystonic excursions was analysed. EMG data of nine patients was excluded from the analysis, as these patients had a constantly changing head position or marked head tremor. In the remaining 17 patients, who had a fairly stable dystonic position, muscular activation patterns during the recording spontaneously changed in nine (Group A) while in eight it remained stable (Group B). There was no significant difference in demographic variables between the two groups other than a male predominance in Group A. However, the retrospectively determined initial response to BT treatment (before BT treatment failure had occurred) was significantly worse in Group A as compared with Group B. Neck muscle activation patterns can spontaneously change in CD patients despite constant dystonic head position, implying an inherent variability of the underlying central motor program in some patients. This should be considered when BT treatment response is unsatisfactory, and should also be taken into account when interpreting results of EMG recordings of neck muscles in these patients. © 2001 Movement Disorder Society. [source]


Distribution of lymphoid neoplasms in the Republic of Korea: Analysis of 5318 cases according to the World Health Organization classification,

AMERICAN JOURNAL OF HEMATOLOGY, Issue 10 2010
Sun Och Yoon
Compared with the West, the overall incidence of lymphoid neoplasms is lower, and the subtype distribution is distinct in Asia. To comprehensively investigate the subtype distribution with the age and sex factors, and temporal changes of subtype proportions, we re-assessed all patients with lymphoid neoplasms diagnosed at a large oncology service in the Republic of Korea from 1989 to 2008 using the World Health Organization classifications. Of the total 5,318 patients, 66.9% had mature B-cell neoplasms, 12.5% had mature T/natural killer (NK)-cell neoplasms, 16.4% had precursor lymphoblastic leukemia/lymphoma (ALL/LBL), and 4.1% had Hodgkin's lymphoma. The most common subtypes were diffuse large B-cell lymphoma (30.5%), plasma cell myeloma (14.0%), extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue type (MALT lymphoma; 12.4%), B-cell ALL/LBL (11.3%), Hodgkin's lymphoma (4.1%), peripheral T-cell lymphoma unspecified (4.0%), T-cell ALL/LBL (3.9%), and extranodal NK/T-cell lymphoma of nasal type (3.9%). Most subtypes showed male predominance, with an average M/F ratio of 1.3. Most mature lymphoid neoplasms were diseases of adults (mean age, 53.5 yr), whereas ALL/LBLs were of young individuals (mean age, 20.3 yr). When the relative proportion of subtypes were compared between two decades (1989,1998 vs. 1999,2008), especially MALT lymphoma has increased in proportion, whereas T/NK-cell neoplasms and ALL/LBL have slightly decreased. In summary, the lymphoid neoplasms of Koreans shared some epidemiologic features similar to those of other countries, whereas some subtypes showed distinct features. Although the increase in incidence of lymphoid neoplasms is relatively modest in Korea, recent increase of MALT lymphoma and decrease of T/NK-cell neoplasms and ALL/LBL are interesting findings. Am. J. Hematol., 2010. © 2010 Wiley-Liss, Inc. [source]


Adenomatoid tumor of the adrenal gland: Case report with immunohistochemical study

PATHOLOGY INTERNATIONAL, Issue 10 2005
Akihiko Hamamatsu
Adrenal adenomatoid tumor (AT) is a recently recognized disease with marked male predominance. Herein is presented a case of adrenal AT incidentally found in a 30-year-old man and results of immunohistochemical examination of the tumor. The left adrenal gland, weighing 17 g, contained a mass measuring 3 × 2.5 × 2.5 cm in the cortical tissue. Cut surface showed a relatively well-circumscribed firm tumor with a white solid appearance. Histologically, the tumor had the typical appearance of AT described in the genital tract. Immunohistochemically, the tumor cells were positive for calretinin, D2-40, WT1, mesothelial cell antigen, CA125, thrombomodulin, vimentin and cytokeratins (stained by AE1 + AE3, OV-TL 12/30, CAM5.2 and MNF116), and negative for endothelial markers (CD31, CD34 and factor VIII-related antigen) and CD56. CD56-positive adrenocortical cells were diffusely scattered in the tumor, especially in its periphery. Immunohistochemistry of estrogen, progesterone and androgen receptors was negative. These findings confirm mesothelial origin of the tumor and suggest that this tumor has little relation to sex hormone despite male predominance. [source]


Allergic rhinitis in children: Incidence and treatment in Dutch general practice in 1987 and 2001

PEDIATRIC ALLERGY AND IMMUNOLOGY, Issue 6 2009
Cindy M. A. De Bot
Allergic rhinitis is a common chronic disorder in children, mostly diagnosed in primary health care. This study investigated the national incidence and treatment of allergic rhinitis among children aged 0,17 yr in Dutch general practice in 1987 and 2001 to establish whether changes have occurred. A comparison was made with data from the first (1987) and second (2001) Dutch national surveys of general practice on children aged 0,17 yr. Incidence rates were compared by age, sex, level of urbanization and season. The management of the general practitioner was assessed regarding drug prescriptions and referrals to medical specialists, and compared with the clinical guideline issued in 1996. The incidence rate of allergic rhinitis increased from 6.6 (1987) to 9.2 (2001) per 1000 person-years. We found a male predominance with a switch in adolescence to a female predominance at both time points. The increase in incidence was the highest in rural (<30,000 inhabitants) and suburban areas (30,000,50,000 inhabitants). Compared to 1987, there was a significant increase in incidence in the central part of the Netherlands in 2001. In both years, the incidence was higher in spring compared with the other seasons. In 2001, children of natives and western immigrants visited the general practitioner more often with complaints of allergic rhinitis compared to 1987. In 1987, prescribed medication consisted mainly of nasal corticosteroids (36%) and in 2001 of oral antihistamines (45%). Although a clinical guideline was not issued until 1996, overall, the treatment of allergic rhinitis by general practitioners was in both years in accordance with the current clinical guideline, but with a stronger adherence in 2001. The results show an increased incidence in the past decades of allergic rhinitis in children in Dutch general practice. The shift to a smaller spectrum of prescriptions in 2001 may be a result of the 1996 clinical guideline. [source]


Increase in chronic or recurrent rhinitis, rhinoconjunctivitis and eczema among schoolchildren in Greece: Three surveys during 1991,2003

PEDIATRIC ALLERGY AND IMMUNOLOGY, Issue 2 2009
Michael B. Anthracopoulos
The prevalence of allergic rhinitis, hay fever and eczema has risen worldwide during the last four decades but may have reached a plateau in some westernized societies. We examined time trends in the prevalence of childhood chronic or recurrent rhinitis, rhinoconjunctivitis and eczema in urban Greece. Using identical methodology, three population-based cross-sectional parental questionnaire surveys on current (last two years) and lifetime allergic symptoms of the nose, eyes and skin were performed among 8,10-yr-old children in 1991, 1998 and 2003 in Patras, Greece. Exactly 2417, 3006 and 2725 questionnaires were completed in 1991, 1998 and 2003, respectively. Prevalence rates of current (lifetime) symptoms of chronic or recurrent rhinitis were 5.1% (6.0%) for 1991, 6.5% (8.0%) for 1998 and 8.0% (9.8%) for 2003. Respective values for rhinoconjunctivitis were 1.8% (2.1%), 2.7% (3.4%) and 3.6% (4.6%) and for eczema 2.5% (4.5%), 3.4% (6.3%) and 5.0% (9.5%) (p for trend <0.001). Among current asthmatics there was an increase in lifetime rhinitis (p = 0.038), current (p = 0.025) and lifetime rhinoconjunctivitis (p = 0.007) and current (p = 0.001) and lifetime eczema (p < 0.001); male predominance increased throughout the study. The proportion of atopic asthma (current asthma with chronic or recurrent rhinitis and/or rhinoconjunctivitis and/or eczema) increased during the same period (p < 0.001). In conclusion, there is a continuous increase in the prevalence of allergic manifestations among preadolescent children in Patras, Greece during the period 1991,2003. In our population, boys have contributed to this increase more than girls and the increase of atopy is, at least partially, responsible for the increase of asthma. [source]


Low donor-to-recipient weight ratio does not negatively impact survival of pediatric heart transplant patients

PEDIATRIC TRANSPLANTATION, Issue 6 2010
Liwen Tang
Tang L, Du W, Delius RE, L'Ecuyer TJ, Zilberman MV. Low donor-to-recipient weight ratio does not negatively impact survival of pediatric heart transplant patients. Pediatr Transplantation 2010: 14:741,745. © 2010 John Wiley & Sons A/S. Abstract:, A major limitation to success in pediatric heart transplantation is donor organ shortage. While the use of allografts from donors larger than the recipient is accepted, the use of undersized donor grafts is generally discouraged. Using the UNOS database, we wanted to evaluate whether using smaller donor hearts affects the short- and long-term survival of pediatric heart transplant patients. A retrospective analysis of data entered into the UNOS database from April 1994 to May 2008 was performed. Pediatric heart transplant recipients (ages 0,18 yr) with DRWR <2.0 were identified and divided into two groups: Low-DRWR (<0.8) and Ideal-DRWR (0.8,2.0). Patients' demographics, pretransplant diagnoses, age at transplantation, severity of pretransplant condition, and rate of complications prior to hospital discharge after transplantation were noted. Fisher's exact, chi-square, and Wilcoxon rank sum tests were used to compare patients' baseline characteristics. Kaplan,Meier curves and Cox proportional hazard regression were used to compare patients' survival and to identify independent risk factors for outcomes. There were 3048 patients (204 with Low- and 2844 with Ideal-DRWR). The Low-ratio group patients were older (8.3 vs. 6.9 yr; p = 0.001), there was a slight male predominance in the Low-DRWR group (p = 0.055). The Low-DRWR group had longer transplant wait time than the Ideal-DRWR group (97 vs. 85 days; p = 0.04). The groups did not differ in race, primary diagnoses, severity of pretransplant condition (medical urgency status, need for ventilation, inotropic support, ECMO, nitric oxide, or dialysis, the PVR for those with bi-ventricular anatomy), or post-transplant complications (length of stay, need for inotropic support, dialysis, and rate of infections). The Low-DRWR patients had less episodes of acute rejection during the first-post-transplant month. Infants with DRWR 0.5,0.59 had lower 30-day survival rate (p = 0.045). There was no difference in short- and long-term survival between the patients with DRWR 0.6,0.79 and DRWR 0.8,2.0. Use of smaller allografts (DRWR 0.6,0.8) has no negative impact on the short- and long-term survival of pediatric heart transplant patients. [source]


HIV-associated plasmablastic lymphoma: Lessons learned from 112 published cases,

AMERICAN JOURNAL OF HEMATOLOGY, Issue 10 2008
Jorge Castillo
Plasmablastic lymphoma (PBL) is a distinct subtype of non-Hodgkin B-cell lymphoma, originally described with a strong predilection to the oral cavity of human immunodeficiency virus (HIV)-infected individuals. Data regarding patient age and gender, HIV status, initiation of and response to highly active antiretroviral therapy (HAART), tumor extent, pathology, treatment, and outcome were extracted from 112 cases of PBL identified in the literature. The median age at presentation was 38 years with a male predominance of 7:1, and the median CD4+ count was 178 cells/mm3. PBL presented on average 5 years after diagnosis of HIV. Common primary sites of presentation included the oral cavity, gastrointestinal tract, and lymph nodes. Most cases presented with either stage I or stage IV disease. There was a variable expression of B-cell markers in tumor cells, but plasma cell markers were expressed in all cases. EBV was detected in 74%. Chemotherapy was used to treat 55% patients and was combined with radiotherapy in 21% cases. Complete response was obtained in 66% of treated cases; the majority of these responses were seen after CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone). The refractory/relapsed disease rate was 54%. Death occurred in 53% of patients, with a median overall survival of 15 months. Sex, CD4+ count, viral load, clinical stage, EBV status, primary site of involvement, and use of CHOP failed to show an association with survival. PBL is an aggressive B-cell lymphoma that presents in both oral and extra-oral sites of chronically HIV-infected immunosuppressed young men. Am. J. Hematol., 2008. © 2008 Wiley-Liss, Inc. [source]


Role of endogenous testosterone concentration in pediatric stroke,

ANNALS OF NEUROLOGY, Issue 6 2009
Sandra Normann MD
Previous studies have indicated a male predominance in pediatric stroke. To elucidate this gender disparity, total testosterone concentration was measured in children with arterial ischemic stroke (AIS; n = 72), children with cerebral sinovenous thrombosis (CSVT; n = 52), and 109 healthy controls. Testosterone levels above the 90th percentile for age and gender were documented in 10 children with AIS (13.9%) and 10 with CSVT (19.2%), totaling 16.7% of patients with cerebral thromboembolism overall, as compared with only 2 of 109 controls (1.8%; p = 0.002). In multivariate analysis with adjustment for total cholesterol level, hematocrit, and pubertal status, elevated testosterone was independently associated with increased disease risk (odds ratio [95% confidence interval]: overall = 3.98 [1.38,11.45]; AIS = 3.88 [1.13,13.35]; CSVT = 5.50 [1.65,18.32]). Further adjusted analyses revealed that, for each 1nmol/l increase in testosterone in boys, the odds of cerebral thromboembolism were increased 1.3-fold. Ann Neurol 2009;66:754,758 [source]


Autoimmune diseases in women with Turner's Syndrome

ARTHRITIS & RHEUMATISM, Issue 3 2010
Kristian T. Jørgensen
Objective In terms of number of X chromosomes, women with Turner's syndrome cytogenetically resemble men. An increased risk of autoimmune diseases has been observed among women with Turner's syndrome. This study was undertaken to investigate whether the autoimmune disease profile in women with Turner's syndrome is characterized by diseases with a female or male predominance. Methods Using the Danish Cytogenetic Central Register, the Danish National Patient Register, and the Danish Civil Registration System, we estimated relative risk of 46 different autoimmune diseases in a cohort of 798 Danish women with Turner's syndrome followed up for 12,461 person-years between 1980 and 2004. Standardized incidence ratios (SIRs) of first hospitalization for autoimmune disease and 95% confidence intervals (95% CIs) were used as measures of relative risk. Results The overall risk of autoimmune disease among women with Turner's syndrome was twice that among Danish women in general (SIR 2.1 [95% CI 1.6,2.7]). For autoimmune diseases with a female predominance, the SIR among women with Turner's syndrome was 1.7 (95% CI 1.2,2.4), whereas the SIR for autoimmune diseases with a male predominance among these women was 3.9 (95% CI 2.5,5.8). Associations were strongest for Hashimoto thyroiditis (SIR 14.6 [95% CI 6.7,27.1]), a strongly female-predominant condition, and type 1 diabetes mellitus (SIR 4.1 [95% CI 2.5,6.3]). Conclusion Women with Turner's syndrome are at excess risk of autoimmune diseases, notably autoimmune diseases characterized by male predominance. [source]


Mantle Cell Lymphoma in the Ocular Region

ACTA OPHTHALMOLOGICA, Issue 2008
S HEEGAARD
Purpose To characterize the clinicopathological features of mantle cell lymphoma (MCL) in the ocular region. Methods All lymphoid lesions were retrieved searching the Danish Ocular Lymphoma Database 1980-2007. Specimens were collected from Danish pathology departments and re-evaluated with a panel of monoclonal antibodies. For all patients with confirmed MCL the complete clinical files were collected and reviewed. Results Twenty-one patients with MCL were identified comprising nine percent (21/230) of all lymphomas in the ocular region. There were 18 male and three female patients with an age range from 60 to 90 years (median 75 years). Ocular region MCL as first presenting symptom included 67% of the patients. Of these, 71% had bilateral involvement and all had lymphoma in more than one site within the ocular region. The orbit (71%) and eyelids (64%) were the most commonly affected sites. At the time of diagnosis 93% of the patients were in Ann Arbor stage III/IV, with bone marrow involvement (79%) and B-symptoms (50%). Median overall survival (OS) was 30 months and the five-year OS rate was 21%. Patients receiving anti-CD20 (Rituximab)-containing chemotherapy had a significant better 5-year OS rate (80 %) (p < 0,027) than patients in treatment regimes without Rituximab (5-year OS rate, 29%). Conclusion MCL presenting in the ocular region has a male predominance and affects elderly patients. The orbit and eyelids were frequently involved. Patients with ocular region MCL as first presenting symptom had a high proportion of bilateral affection. Patients had advanced stage disease at diagnosis, multiple relapses and a low 5-year OS rate similar to systemic MCL. Treatment with Rituximab-containing chemotherapy improved survival significantly. [source]


The changing panorama of cerebral palsy in Sweden.

ACTA PAEDIATRICA, Issue 9 2010

Abstract Aim:, The aim of the study was to describe the prevalence and origin of cerebral palsy (CP), which is the tenth report from the western Swedish study. Methods:, A population-based study covering 85 737 live births in the area in 1999,2002. Birth characteristics and neuroimaging findings were recorded, prevalence of CP was calculated and aetiology was analysed. Results:, CP was found in 186 children. The crude prevalence was 2.18 per 1000 live births. The gestational age-specific prevalence for <28 gestational weeks was 55.6 per 1000 live births, whereas it was 43.7 for 28,31 weeks, 6.1 for 32,36 weeks and 1.43 per 1000 for >36 weeks. There was a female majority among children born at term and a male predominance in children born preterm. Hemiplegia accounted for 38%, diplegia for 32%, tetraplegia for 7%, whereas 17% had dyskinetic CP and 5% ataxia. Neuroimaging showed white-matter lesions in 31% and cortical/subcortical lesions in 29%. The aetiology was considered to be prenatal in 36%, peri/neonatal in 42%, whereas it remained unclassified in 21%. Conclusion:, The decrease in CP prevalence observed since the 1980s had ceased. An increase in children born at term and in dyskinetic CP was found. In children born before 28 weeks of gestation, the prevalence decreased significantly. White-matter and cortical/subcortical lesions dominated on neuroimaging. [source]