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Healthy Siblings (healthy + sibling)
Selected AbstractsB lymphocyte stimulator expression in pediatric systemic lupus erythematosus and juvenile idiopathic arthritis patientsARTHRITIS & RHEUMATISM, Issue 11 2009Sandy D. Hong Objective To assess the expression of B lymphocyte stimulator (BLyS) in patients with pediatric systemic lupus erythematosus (SLE) or juvenile idiopathic arthritis (JIA). Methods Blood samples collected from patients with pediatric SLE (n = 56) and patients with JIA (n = 54) at the beginning and end of a 6-month interval were analyzed for plasma BLyS protein levels by enzyme-linked immunosorbent assay and for blood leukocyte full-length BLyS and ,BLyS messenger RNA (mRNA) levels by quantitative real-time polymerase chain reaction (normalized to 18S expression). Healthy siblings (n = 34) of these patients served as controls. Results In pediatric SLE, plasma BLyS protein and blood leukocyte BLyS mRNA levels were each significantly elevated, and plasma BLyS protein levels, but not blood leukocyte BLyS mRNA levels, were correlated with disease activity. In contrast, plasma BLyS protein levels were normal in JIA despite blood leukocyte BLyS mRNA levels being elevated to degrees similar to those in pediatric SLE. Among JIA patients, neither BLyS parameter was correlated with disease activity. In both pediatric SLE and JIA, the BLyS expression profiles remained stable at 6 months. Conclusion Our findings indicate that, as previously noted in adult SLE, plasma BLyS protein and blood leukocyte BLyS mRNA levels are elevated in pediatric SLE. The correlation of plasma BLyS protein levels with disease activity points to BLyS as a candidate therapeutic target in pediatric SLE. Contrary to previous observations in adults with rheumatoid arthritis, plasma BLyS protein levels are normal in JIA despite elevated blood leukocyte BLyS mRNA levels. The absence of correlation between either of the BLyS parameters and disease activity in JIA calls for circumspection prior to assigning BLyS as a candidate therapeutic target in this disorder. [source] A novel mutation in the PSEN1 gene (L286P) associated with familial early-onset dementia of Alzheimer type and lobar haematomasEUROPEAN JOURNAL OF NEUROLOGY, Issue 12 2007R. Sánchez-Valle The aim of this study was to describe a novel mutation in exon 8 of the presenilin gene (L286P) associated with early-onset autosomal dominant Alzheimer's disease (AD) and lobar haematomas. The proband was a woman who developed cognitive decline with predominant memory loss at the age of 35 years. The patient died at the age of 54 years and the neuropathological examination confirmed the diagnosis of AD. Three of her four siblings, one parent and one sibling of her parent had suffered from cognitive decline at ages between 35 and 42 years. Three of them also presented lobar haematomas. The neuropathological examination, available in one of them, disclosed the presence of severe amyloid angiopathy as the cause of the haematoma. The study of PSEN1 gene with single strand conformation polymorphism technique failed to show abnormalities suggestive of mutations. Direct sequencing disclosed the presence of a missense mutation in codon 286 (L286P) in the proband and her already affected descendent, which was absent in the healthy sibling. L286P is a novel mutation in PSEN1 that causes familial early-onset AD and brain haematomas related to amyloid angiopathy. [source] Short Report: Psychological adjustment of well siblings of children with Type 1 diabetesDIABETIC MEDICINE, Issue 9 2010F. Sleeman Diabet. Med. 27, 1084,1087 (2010) Abstract Aims, Studies of siblings of children with Type 1 diabetes (Type 1 DM) have shown either increased levels of maladjustment or, alternatively, increased levels of pro-social behaviour according to whether the sibling or parent was interviewed. The purpose of this study was to examine the psychological adjustment of Type 1 DM siblings using both parent and sibling report and to assess the concordance between child and parent reports. Methods, Ninety-nine siblings aged 11,17 years and parents of children with Type 1 DM treated at the Royal Children's Hospital, Melbourne were recruited sequentially. The Strengths and Difficulties Questionnaire (SDQ) was used to assess well siblings' emotional and behavioural functioning using data collected within a semi-structured interview. SDQ data between the sibling cohort and normative data sample were compared using independent-samples t -tests. Sibling reports and parent reports were compared using a series of paired-sample t -tests and correlation analyses. Results, Type 1 DM siblings did not report greater emotional or behavioural maladjustment or more pro-social behaviour than norms. Parents rated siblings' pro-social behaviour as being comparable with that of youth from the general community; however, parents rated healthy siblings as having lower levels of maladjustment; specifically, significantly fewer conduct problems, hyperactive behaviour and peer-related problems (all P < 0.01). There were no significant differences between parent ratings and sibling ratings on peer-related problems or pro-social behaviour. Conclusions, Type 1 DM siblings did not report increased behavioural or emotional dysfunction relative to children in the general population and, according to their parents, were even better adjusted than their peers. [source] Increased inflammatory markers in children with familial hypercholesterolaemiaEUROPEAN JOURNAL OF CLINICAL INVESTIGATION, Issue 3 2006T. Ueland Abstract Background, While data are abundant on increased levels of inflammatory markers in adult patients with hypercholesterolaemia, such data in children are limited. Therefore, we sought to investigate the degree and character of inflammation in children with heterozygous familial hypercholesterolaemia (FH) by measuring levels of neopterin, high-sensitivity C-reactive protein (hsCRP), and soluble CD40 ligand (sCD40L). Materials and methods, In the present study, we compared the concentration of inflammatory markers in children suffering from heterozygous FH (n = 207) with those in unaffected siblings (n = 84). Furthermore, we investigated the effect of 2-year treatment with pravastatin (20,40 mg qd) or placebo on plasma levels of those markers. Results, Our main finding was that serum levels of neopterin and hsCRP were significantly higher in FH children compared with healthy siblings, whereas sCD40L was not. Body mass index and high-density lipoprotein cholesterol levels were significant independent predictors of hsCRP and neopterin. Furthermore, pravastatin therapy decreased neopterin, but not hsCRP and sCD40L, in the FH children, but these changes were not different from the placebo group. Conclusion, These findings indicate low-grade monocyte/macrophage hyperactivity in the early stages of atherogenesis, but our findings also suggest that inflammation as well as anti-inflammatory effects of statins are less prominent features of atherosclerosis in FH children than in FH adults. [source] Humoral immunity to diphtheria, tetanus, measles, and hemophilus influenzae type b in children with acute lymphoblastic leukemia and response to re-vaccinationPEDIATRIC BLOOD & CANCER, Issue 6 2009Emine Zengin Abstract Objective Loss of immunity to previous vaccination and timing of re-vaccination in children receiving chemotherapy remains controversial. The aim of this study was to investigate the immunity to vaccine preventable diseases in children with acute lymphoblastic leukemia (ALL). Procedure Sixty-one patients with ALL and 13 healthy siblings were enrolled. Three study groups included newly diagnosed patients (group 1), patients on maintenance chemotherapy (group 2), and patients that completed chemotherapy (group 3). Blood samples for baseline antibody titers were obtained from all the patients and controls. Patients in group 2 were vaccinated with diphtheria, tetanus, and hemophilus influenzae type b (Hib). Patients in group 3 and controls received the measles vaccine in addition to all the above vaccines. In groups 2 and 3, post-vaccination antibody titers were also obtained. Results Patients and controls had no Hib vaccine during primary vaccination. After chemotherapy median antibody levels against diphtheria, tetanus, measles, and Hib were decreased but tetanus antibodies were still at the protective levels. Proportions of the patients with protective levels were 11.1%, 83.3%, 16.7%, and 16.7% for diphtheria, tetanus, Hib, and measles, respectively. Vaccination achieved protective antibody levels in 81%, 100%, 89.5%, and 70% of the patients for diphtheria, tetanus, Hib, and measles, respectively. Vaccine responses during maintenance were also satisfying. Conclusion We recommend re-vaccination after 3 months of cessation of chemotherapy. Administration of Hib vaccine may be beneficial after the first 3 months of maintenance chemotherapy especially in children with no primary vaccination followed by a second booster dose after cessation of therapy to increase immunity. Pediatr Blood Cancer 2009;53:967,972. © 2009 Wiley-Liss, Inc. [source] Type 1 diabetes mellitus and school: a comparison of patients and healthy siblingsPEDIATRIC DIABETES, Issue 8 2009Kelly B Parent Children and adolescents with type 1 diabetes mellitus (T1DM) are at risk for a variety of problems at school. Well-controlled studies using data collected in schools, however, are limited. The purposes of this study are to determine whether selected school problems are associated with T1DM and to investigate an association between these problems and medical variables. Teachers rated 95 diabetic students (M = 11.8; SD = 3.0 yr old) and 95 of their siblings (M = 12.1; SD = 3.0 yr old) regarding academic skills, work completion, day-to-day variability, and classroom attention. Medical and school records also were accessed. The T1DM group had lower academic skills ratings overall (p < 0.02), especially in writing (p < 0.01), a trend toward poorer classroom attention (p < 0.08), and many more missed school days (p < 0.001). Diabetics on intensive therapy protocols had better academic ratings overall (p < 0.02), including in math (p < 0.03) and fewer missed school days (p < 0.03), but they unexpectedly were rated as having more classroom behaviors that jeopardize work completion (p < 0.05) than counterparts on conventional therapy. Among all diabetics, glycated hemoglobin (HbAlc) levels were moderately related to each academic skill rating (r = ,0.34 to ,0.37; p < 0.01) and strongly related to classroom attention (r = 0.53; p = 0.000). T1DM itself appears to be a relatively minor influence to several important aspects of school. Furthermore, although intensive therapy alone may well promote school success, meticulous glycemic control, however achieved, appears more important in mitigating prospective classroom attention and academic problems. [source] Emotional vulnerability and cognitive control in patients with bipolar disorder and their healthy siblings: a pilot studyACTA NEUROPSYCHIATRICA, Issue 2 2010Kathrin Houshmand Scheuch K, Bräunig P, Gauggel S, Kliesow K, Sarkar R, Krüger S. Emotional vulnerability and cognitive control in patients with bipolar disorder and their healthy siblings: a pilot study. Objective: There is evidence that, even in remission, patients with bipolar disorder (BD) have deficits in cognitive function and emotional regulation. Siblings of patients with BD are also reported to exhibit minor dysfunction in neuropsychological domains. In this study, we examined the interference of acute mood state with reaction time (RT) and response inhibition in euthymic patients with BD, in their healthy siblings and in healthy controls. Methods: A total of 34 patients with bipolar I disorder, 22 healthy siblings and 33 healthy controls performed a stop-signal paradigm after induction of a transient intense sadness and a relaxed mood state. The differences in RT and the response inhibition were compared between the groups. Results: Euthymic patients with BD displayed a higher emotional reactivity compared with their siblings and with controls. Compared with controls, patients with BD showed longer RTs in a relaxed mood state and a delay in response inhibition during emotional activation. Conclusions: The present study provides evidence for the clinical observation that patients with BD have shorter RTs when in a state of emotional arousal rather than in a relaxed state. Inhibitory deficits in these patients may be because of a too strong emotional arousal. The results show that in patients with BD, relaxation and emotional arousal are inversely associated with performance in a neuropsychological task. This is in contrast to findings in healthy individuals suggesting a dysbalance in emotional regulation in these patients. [source] Prevalence and morbidity associated with non-malignant, life-threatening conditions in childhoodCHILD: CARE, HEALTH AND DEVELOPMENT, Issue 5 2001S Lenton Summary Objective To determine the prevalence of non-malignant life-threatening illness in childhood and associated morbidity in the affected child and their family members. Design Cross-sectional survey. Setting Bath Clinical Area (total population 411 800). Subjects Children aged 0,19 years. Results One hundred and twenty-three children were identified, giving a prevalence of 1.2/1000 children. Morbidity assessed in 93 children showed 60% in pain or discomfort, 35% unable to walk and 25% with severe cognitive impairment. Mental health problems were found in 54% of mothers and 30% of fathers, and significant emotional and behavioural problems in 24% of healthy siblings. Conclusions Non-malignant life-threatening illness is more prevalent than reported in previous studies. Considerable morbidity is experienced by the child and their family. An individual and family approach is required. Key messages (1) The prevalence of non-malignant life-threatening illness is four times greater than previous estimates. (2) This group of conditions have significant implications for all family members. (3) Early comprehensive assessment and access to effective interventions may pre-empt later problems. [source] No structural cerebral differences between children with a history of bacterial meningitis and healthy siblingsACTA PAEDIATRICA, Issue 10 2008Rogier CJ De Jonge Abstract Aim: After bacterial meningitis, about one-third of children develops academic and/or behavioural limitations. The aim of our study was to search for structural differences in the brain, with a special focus on the hippocampus, between childhood survivors of bacterial meningitis with and without academic and/or behavioural limitations and healthy siblings. Patients and methods: A selection of a cohort, compiled in an earlier performed retrospective study, was used in this case-control study. Magnetic Resonance Imaging scans of the brain were performed in 43 post-meningitis children, of whom 18 had learning and/or behavioural limitations and 25 had no problems, and 18 controls. Voxel-based morphometry investigated the brain for structural changes. Hippocampal volume and lateral ventricle width were measured. Results: No structural differences between the groups, in any area of the brain, were found. There were no significant differences in hippocampal volume or lateral ventricle width. The group with limitations had three children with a right hippocampal volume smaller than two standard deviations below the mean of the control group. Conclusion: Despite hippocampus lesions found in experimental studies, we found no anatomical differences of the brain or hippocampus related to bacterial meningitis in children, nor to the academic and/or behavioural limitations seen after bacterial meningitis. [source] | ||||||||||