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1.
Osteoporos Int ; 26(3): 931-42, 2015 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-25510582

RESUMEN

UNLABELLED: Bone health may be negatively impacted by childhood socio-environmental circumstances. We examined the independent associations of single-parent childhood and parental death or divorce in childhood with adult bone strength indices. Longer exposure to a single-parent household in childhood was associated with lower bone strength in adulthood. INTRODUCTION: Because peak bone mass is acquired during childhood, bone health may be negatively impacted by childhood socio-environmental disadvantage. The goal of this study was to determine whether being raised in a single-parent household is associated with lower bone strength in adulthood. METHODS: Using dual-energy X-ray absorptiometry data from 708 participants (mean age 57 years) in the Midlife in the United States Biomarker Project, we examined the independent associations of composite indices of femoral neck bone strength relative to load (in three failure modes: compression, bending, and impact) in adulthood with the experience of single-parent childhood and parental death or divorce in childhood. RESULTS: After adjustment for gender, race, menopause transition stage, age, and body mass index, each additional year of single-parent childhood was associated with 0.02 to 0.03 SD lower indices of adult femoral neck strength. In those with 9-16 years of single-parent childhood, the compression strength index was 0.41 SD lower, bending strength index was 0.31 SD lower, and impact strength index was 0.25 SD lower (all p values < 0.05). In contrast, parental death or divorce during childhood was not by itself independently associated with adult bone strength indices. The magnitudes of these associations were unaltered by additional adjustment for lifestyle factors and socioeconomic status in childhood and adulthood. CONCLUSIONS: Independent of parental death or divorce, growing up in a single-parent household is associated with lower femoral neck bone strength in adulthood, and this association is not entirely explained by childhood or adult socioeconomic conditions or lifestyle choices.


Asunto(s)
Cuello Femoral/fisiología , Familia Monoparental , Absorciometría de Fotón , Adulto , Anciano , Densidad Ósea/fisiología , Niño , Divorcio/estadística & datos numéricos , Femenino , Humanos , Acontecimientos que Cambian la Vida , Estilo de Vida , Masculino , Persona de Mediana Edad , Muerte Parental/estadística & datos numéricos , Clase Social , Medio Social , Estrés Mecánico , Factores de Tiempo , Estados Unidos
2.
Osteoporos Int ; 25(4): 1327-35, 2014 04.
Artículo en Inglés | MEDLINE | ID: mdl-24424630

RESUMEN

UNLABELLED: We examined the association between marital life history and bone mineral density (BMD) in a national sample from the US. In men, being stably married was independently associated with better lumbar spine BMD, and in women, more spousal support was associated with better lumbar spine BMD. INTRODUCTION: Adult bone mass may be influenced by stressors over the life course. We examined the association between marital life history and bone mineral density (BMD) net socioeconomic and behavioral factors known to influence bone mass. We sought evidence for a gender difference in the association between marital history and adult BMD. METHODS: We used data from 632 adult participants in the Midlife in the United States Study to examine associations between marital history and BMD, stratified by gender, and adjusted for age, weight, menopausal stage, medication use, childhood socioeconomic advantage, adult financial status, education, physical activity, smoking, and alcohol consumption. RESULTS: Compared to stably married men, men who were currently divorced, widowed, or separated, men who were currently married but previously divorced, widowed, or separated, and never married men had 0.33 (95% CI: 0.01, 0.65), 0.36 (95% CI: 0.10, 0.83), and 0.53 (95% CI: 0.23, 0.83) standard deviations lower lumbar spine BMD, respectively. Among men married at least once, every year decrement in age at first marriage (under age 25) was associated with 0.07 SD decrement in lumbar spine BMD (95% CI: 0.002, 0.13). In women, greater support from the spouse was associated with higher lumbar spine BMD. CONCLUSIONS: Our findings suggest that marriage before age 25 and marital disruptions are deleterious to bone health in men, and that marital quality is associated with better bone health in women.


Asunto(s)
Densidad Ósea/fisiología , Estado Civil/estadística & datos numéricos , Osteoporosis/epidemiología , Apoyo Social , Absorciometría de Fotón/métodos , Adulto , Factores de Edad , Anciano , Femenino , Cuello Femoral/fisiología , Humanos , Vértebras Lumbares/fisiología , Masculino , Matrimonio/psicología , Matrimonio/estadística & datos numéricos , Menopausia/fisiología , Persona de Mediana Edad , Osteoporosis/fisiopatología , Osteoporosis/psicología , Caracteres Sexuales , Factores Socioeconómicos , Estados Unidos/epidemiología
3.
Osteoporos Int ; 25(4): 1379-88, 2014 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-24504101

RESUMEN

UNLABELLED: We examined baseline and annual follow-up data (through annual follow-up visit 9) from a cohort of 2,234 women aged 42 to 52 years at baseline. Independent of financial status, higher educational level was associated with lower fracture incidence among non-Caucasian women but not among Caucasian women. INTRODUCTION: This study was conducted to determine the associations of education and income with fracture incidence among midlife women over 9 years of follow-up. METHODS: We examined baseline and annual follow-up data (through annual follow-up visit 9) from 2,234 participants of the Study of Women's Health Across the Nation, a cohort of women aged 42 to 52 years at baseline. We used Cox proportional hazards regression models to examine the associations of socioeconomic predictors (education, family-adjusted poverty-to-income ratio, and difficulty paying for basics) with time to first incident nontraumatic, nondigital, noncraniofacial fracture. RESULTS: Independent of family-adjusted poverty-to-income ratio, higher educational level was associated with decreased time to first incident fracture among non-Caucasian women but not among Caucasian women (p(interaction) 0.02). Compared with non-Caucasian women who completed no more than high school education, non-Caucasian women who attained at least some postgraduate education had 87% lower rates of incident nontraumatic fracture (adjusted hazard ratio 0.13, 95% confidence interval [CI] 0.03-0.60). Among non-Caucasian women, each additional year of education was associated with a 16% lower odds of nontraumatic fracture (adjusted odds ratio 0.84, 95% CI 0.73-0.97). Income, family-adjusted poverty-to-income ratio, and degree of difficulty paying for basic needs were not associated with time to first fracture in Caucasian or non-Caucasian women. CONCLUSIONS: Among non-Caucasian midlife women, higher education, but not higher income, was associated with lower fracture incidence. Elucidation of the mechanisms underlying the possible protective effects of higher educational level on nontraumatic fracture incidence may allow us to better target individuals at risk of future fracture.


Asunto(s)
Fracturas Osteoporóticas/etnología , Clase Social , Adulto , Escolaridad , Femenino , Estudios de Seguimiento , Humanos , Incidencia , Menopausia/etnología , Menopausia/fisiología , Persona de Mediana Edad , Fracturas Osteoporóticas/etiología , Pobreza/estadística & datos numéricos , Factores de Riesgo , Estados Unidos/epidemiología , Población Blanca/estadística & datos numéricos , Salud de la Mujer/estadística & datos numéricos
4.
Osteoporos Int ; 23(5): 1503-12, 2012 May.
Artículo en Inglés | MEDLINE | ID: mdl-21811862

RESUMEN

UNLABELLED: Among a group of 940 US adults, economic adversity and minority race status were associated with higher serum levels of markers of bone turnover. These results suggest that higher levels of social stress may increase bone turnover. INTRODUCTION: To determine socioeconomic status (SES) and race differences in levels of bone turnover. METHODS: Using data from the Biomarker Substudy of the Midlife in the US (MIDUS) study (491 men, 449 women), we examined cross-sectional associations of SES and race with serum levels of bone turnover markers (bone-specific alkaline phosphatase [BSAP], procollagen type I N-terminal propeptide [PINP], and N-telopeptide [Ntx]) separately in men and women. Linear multivariable regression was used to control for body weight, menopausal transition stage, and age. RESULTS: Among men, low family poverty-to-income ratio (FPIR) was associated with higher turnover, but neither education nor race was associated with turnover. Men with FPIR <3 had 1.808 nM BCE higher Ntx (P = 0.05), 3.366 U/L higher BSAP (P = 0.02), and 7.066 higher PINP (P = 0.02). Among women, neither education nor FPIR was associated with bone turnover, but Black women had 3.688 nM BCE higher Ntx (P = 0.001), 5.267 U/L higher BSAP (P = 0.005), and 11.906 µg/L higher PINP (P = 0.008) compared with non-Black women. CONCLUSIONS: Economic adversity was associated with higher bone turnover in men, and minority race status was associated with higher bone turnover in women, consistent with the hypothesis that higher levels of social stresses cause increased bone turnover. The magnitude of these associations was comparable to the effects of some osteoporosis medications on levels of turnover.


Asunto(s)
Remodelación Ósea/fisiología , Resorción Ósea/etnología , Clase Social , Adulto , Negro o Afroamericano/psicología , Anciano , Fosfatasa Alcalina/sangre , Biomarcadores/sangre , Remodelación Ósea/genética , Resorción Ósea/sangre , Resorción Ósea/etiología , Colágeno Tipo I/sangre , Estudios Transversales , Femenino , Humanos , Masculino , Persona de Mediana Edad , Fragmentos de Péptidos/sangre , Péptidos/sangre , Pobreza , Procolágeno/sangre , Caracteres Sexuales , Factores Socioeconómicos , Estrés Psicológico/sangre , Estrés Psicológico/complicaciones , Estrés Psicológico/etnología , Estados Unidos/epidemiología
5.
Vnitr Lek ; 57(7-8): 645-9, 2011.
Artículo en Cs | MEDLINE | ID: mdl-21877600

RESUMEN

Renal (kidney) transplantation is now a routine and the most successful form of renal replacement therapy. There is a long tradition of renal transplantation in the Czech Republic, The first was performed as early as 1961 in Hradec Kralove, and the programme as such was launched in 1966 with the first successful transplantation at the Institute of Experimental Surgery (later Institute for Clinical and Experimental Medicine, Prague). At present, transplantations are being performed at 7 transplantation centres (IKEM Prague, Centre for Cardiovascular and Transplantation Surgery Brno, Faculty Hospitals Hradec Kralove, Plzen, Olomouc and Ostrava and Faculty Hospital Motol for children). From the programme launch until the end of 2010, 8,761 renal transplantations were performed, 364 in 2010 alone. One-year patient and cadaver renal allograft survival, transplanted in the CR between 2000 and 2009, is around 95% and 92%, respectively, and 5-year survival is 87% and 81%, respectively. As of 31st December 2009, a total of 3,771 patients lived with functional renal allograft in the Czech Republic and the proportion of patients with irreversible renal failure treated with transplantation has recently been around 40%.


Asunto(s)
Trasplante de Riñón/estadística & datos numéricos , Cadáver , República Checa , Humanos , Trasplante de Riñón/mortalidad , Donadores Vivos
6.
Res Hum Dev ; 18(3): 1-17, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34924879

RESUMEN

Although many studies have unequivocally demonstrated the promise of understanding resilience to adversity and characterizing the consequences if stress is unabated, needed are dynamic theories and methods to enhance the rigor and interpretation of these assessments. From a dynamic systems perspective, the focus is not whether an individual possesses some fixed ability or unchangeable trait, but rather to understand the flexibility and responsiveness of stress regulation systems to daily hassles and adverse life events. A renewed interest in individual variability allows researchers to see trajectories of change over both short- and long-time scales to understand the developmental course. As a result, it is possible to answer questions, such as, how does the dysregulation in emotion caused by stress, to both within and between daily affect processes, relate to longitudinal trajectories (over time-scales of years) of dysfunction and disease? The overarching goal of the Notre Dame Study of Health & Well-being is to detail the types and qualities of contextual influences, in conjunction with dynamic psychobiological systems, to assess the precursors, concomitant influences and consequences of stress and resilience in the face of adversity on cognitive, health and well-being outcomes.

8.
Transplant Proc ; 50(10): 3954-3956, 2018 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-30577294

RESUMEN

Mutations in nucleoporin 93 (NUP93) gene have been shown recently to be one of the very rare causes of genetic steroid-resistant nephrotic syndrome (SRNS). Until now, none of the 7 published cases with NUP93-SRNS, experienced recurrence of nephrotic syndrome (NS) after transplantation. Here, we present the first case of recurrent NS in a patient with NUP93-SRNS ever reported. A 3-year-old boy with infantile SRNS was started on chronic peritoneal dialysis because of end-stage renal failure owing to biopsy-proven focal segmental glomerulosclerosis (FSGS). At the age of 6 years, the boy received a renal allograft. The posttransplant period was uncomplicated until 1.7 years after transplantation, when the patient developed nephrotic proteinuria during a respiratory tract infection. Renal graft biopsy showed subtotal fusion of podocytes, which was compatible with an early histopathologic sign of recurrence of FSGS. Immediate treatment with daily plasma exchange (PE) was started at the second day. The proteinuria disappeared completely after the second PE. However, it reappeared after stopping daily PE. It disappeared again after reintroduction of daily PE, therefore PE-dependent recurrent NS was diagnosed and treatment with rituximab was given. After the first dose, proteinuria never reappeared despite stopping PE therapy. Surprisingly, next-generation sequencing revealed compound heterozygous mutations in exons 16 and 18 of the NUP93 gene (c.1772G>T - European founder allele and 1916T>C) and his parents confirmed heterozygous asymptomatic carriers. This is the first case of recurrent NS in a patient with NUP93 gene mutations, suggesting a new pathomechanism possibly involving the nucleoporins.


Asunto(s)
Trasplante de Riñón , Síndrome Nefrótico/genética , Síndrome Nefrótico/cirugía , Proteínas de Complejo Poro Nuclear/genética , Preescolar , Heterocigoto , Humanos , Factores Inmunológicos/uso terapéutico , Masculino , Mutación , Síndrome Nefrótico/complicaciones , Plasmaféresis , Recurrencia , Rituximab/uso terapéutico
9.
Transplant Proc ; 50(3): 967-970, 2018 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-29661469

RESUMEN

Atypical hemolytic-uremic syndrome (aHUS) is an extremely rare disease, and up to 70% of the patients have a genetic mutation in the encoding components of complement activation or anti-complement factor H autoantibodies. The risk of recurrence after kidney transplantation is 10% to 80%. Eculizumab, a monoclonal antibody that binds complement protein C5, has shown to be highly effective in patients with aHUS; however, there are only few reports on the efficacy and safety of long-term eculizumab treatment in children with recurrent aHUS. Only 3 case reports regard treatment in patients with complement factor H (CFH/CFHR1/CFHR3) hybrid gene. This report presents the efficacy and safety of long-term eculizumab treatment in a child with recurrent aHUS who has been successfully treated with eculizumab for more than 7 years. The patient presented as a 9-year-old with aHUS due to CFH/CFHR1/CFHR3 hybrid gene and received deceased donor kidney transplantation. After the transplantation, he experienced recurrence of aHUS 2 months later. Daily plasma exchanges were ineffective in the transplanted kidney; the patient became anuric and hemodialysis was needed. Eculizumab was started as therapy and led to complete remission of aHUS including restoration of diuresis. Eculizumab has been given as therapy for 7 years. The young patient is in a sustained remission without any adverse events. This patient is only the sixth patient reported with recurrent aHUS due to CFH/CFHR1/CFHR3 hybrid gene and is the patient with the longest remission of recurrent aHUS ever published.


Asunto(s)
Anticuerpos Monoclonales Humanizados/administración & dosificación , Anticuerpos Monoclonales/administración & dosificación , Síndrome Hemolítico Urémico Atípico/tratamiento farmacológico , Complicaciones Posoperatorias/tratamiento farmacológico , Síndrome Hemolítico Urémico Atípico/genética , Síndrome Hemolítico Urémico Atípico/cirugía , Niño , Activación de Complemento/genética , Factor H de Complemento/genética , Humanos , Trasplante de Riñón/efectos adversos , Masculino , Mutación , Complicaciones Posoperatorias/genética , Recurrencia , Factores de Tiempo
10.
Transplant Proc ; 39(10): 3150-2, 2007 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-18089341

RESUMEN

BACKGROUND: Proteinuria together with hypertension are known risk factors for poor allograft as well as patient survivals after renal transplantation. In adults, proteinuria can be reduced by lowering blood pressure and by using angiotensin-converting enzyme inhibitors. In children, no study has investigated the antiproteinuric effects of antihypertensive therapy. Herein we investigated changes in proteinuria among a subgroup of children with proteinuria>or=200 mg/m2d in an interventional study primary aimed to improve the efficacy of antihypertensive therapy. PATIENTS AND METHODS: Twelve children with proteinuria>or=200 mg/m2d were included in the study. Proteinuria was investigated at baseline and at 1 year after changes in antihypertensive therapy. Blood pressure (BP) was measured using ambulatory BP monitoring. RESULTS: The median protein excretion of 226 mg/m2/d (range, 41-1478 mg/m2/d) at 1 year before the study did not change significantly at study baseline (278 mg/m2/d; range, 205-1264 mg/m2/d), but decreased significantly to 199 mg/m2/d (range, 65-749 mg/m2/d) after 1 year (P<.05 vs baseline). The number of antihypertensive drugs was increased from 1.6+/-1.0 to 2.2+/-0.9 drugs/patient after 1 year (P<.05). The use of different classes of antihypertensive drugs did not change significantly. Mean ambulatory systolic and diastolic BP at daytime and diastolic BP at nighttime did not change significantly after 1 year; mean ambulatory systolic BP at night decreased from 1.60+/-1.54 to 1.04+/-0.97 standard deviation score (P<.05). Graft function did not change significantly. CONCLUSION: We demonstrated that proteinuria among children after renal transplantation was reduced by intensified antihypertensive therapy using all classes of antihypertensive drugs.


Asunto(s)
Antihipertensivos/uso terapéutico , Hipertensión/tratamiento farmacológico , Trasplante de Riñón/efectos adversos , Proteinuria/prevención & control , Adolescente , Adulto , Presión Sanguínea/efectos de los fármacos , Monitoreo Ambulatorio de la Presión Arterial , Niño , Femenino , Supervivencia de Injerto , Humanos , Masculino , Persona de Mediana Edad , Selección de Paciente , Proteinuria/etiología , Trasplante Homólogo
11.
Transplant Proc ; 39(10): 3488-90, 2007 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-18089416

RESUMEN

Idiopathic focal segmental glomerulosclerosis (FSGS) is believed to be caused by a circulating permeability factor. FSGS recurrence is common after transplantation. The treatment is still a matter of debate; plasmapheresis (PE) and immunoadsorption (IA) are often used. We report on PE and IA in the treatment of two children with recurrent nephrotic proteinuria. Patient 1 was a 16-year-old girl who had recurrence of nephrotic proteinuria on the first day after transplantation (proteinuria-19 g/d). Primary immunosuppressive therapy was changed to high-dose cyclosporine and cyclophosphamide; plasmapheresis was started on day 4. Altogether we performed 53 PE and 38 IA procedures. During the first month, PE procedures were performed with no more than a 2-day interval between sessions, and the girl achieved partial remission (proteinuria 3 g/d). PE was then stopped. After 2 months, a relapse of heavy proteinuria occurred. This relapse was successfully treated again with intensified PE treatment. After achieving remission, a chronic PE regimen was started (PE once a week), similar to the previous series. The child remained in partial remission. Seven months after renal transplantation, she was switched from PE to IA, because of severe hypoproteinemia. Graft biopsy performed at 4 months showed effacement of the foot processes. At the present time she has a good graft function and 3 g/d proteinuria. Patient 2 was a 13-year-old girl with FSGS since 9 years. On the second day after renal transplantation she developed nephrotic proteinuria (proteinuria-14 g/d), which was treated with 39 PE and 16 IA treatments. She went into complete remission on the intensified PE regimen, had one relapse, and was switched to chronic IA. Graft biopsy performed at 2 weeks after transplantation showed effacement of the foot processes. At the present time she has good graft function and low proteinuria (0.3 g/d). In conclusion, intensified PE or IA treatments induced remission of recurrent nephrotic range proteinuria. Chronic PE or IA can maintain patients with frequent relapses in long-term remission.


Asunto(s)
Glomeruloesclerosis Focal y Segmentaria/cirugía , Técnicas de Inmunoadsorción , Trasplante de Riñón , Plasmaféresis , Complicaciones Posoperatorias/terapia , Proteinuria/terapia , Adolescente , Femenino , Humanos , Inmunosupresores/uso terapéutico , Recurrencia , Resultado del Tratamiento
12.
Cas Lek Cesk ; 145(8): 625-32; discussion 632-4, 2006.
Artículo en Cs | MEDLINE | ID: mdl-16995418

RESUMEN

Hypertension is one of the main risk factors of cardiovascular diseases. Hypertension in childhood is defined as blood pressure > or = 95. percentile for healthy children population. The prevalence of hypertension in childhood is considerably lower than in adults and is about 1%. The aetiology of hypertension in childhood differs from adult population--in children secondary forms are more common than primary, however, in adolescents primary form already prevails. In general, the younger the child and the higher the blood pressure, the more probably it is a secondary form of hypertension. The most common causes of secondary hypertension are renal diseases (renoparenchymal or renovascular). Cardiac diseases (aortic isthmus stenosis), endocrinopathies, central nervous system disorders or use of hypertensinogenic drugs are less frequent causes of secondary hypertension. Each child with hypertension has to be carefully examined; the extent of the examination depends on the age of the child and severity of hypertension. The main task for the investigation is to exclude or reveal secondary form of hypertension, which could be causally treated (e.g. angioplasty in renal artery stenosis). Treatment of hypertension is non-pharmacological and pharmacological (angiotensin-converting enzyme inhibitors, beta-blockers, calcium channel blockers, diuretics, angiotensin receptor blockers).


Asunto(s)
Hipertensión , Adolescente , Adulto , Antihipertensivos/uso terapéutico , Niño , Humanos , Hipertensión/diagnóstico , Hipertensión/tratamiento farmacológico , Hipertensión/etiología
13.
Cas Lek Cesk ; 145(8): 635-8, 2006.
Artículo en Cs | MEDLINE | ID: mdl-16995419

RESUMEN

BACKGROUND: Hypertension in patients after renal transplantation (RTx) is associated with impaired graft functions and graft survival. Control of hypertension in children after RTx is low--only 20-50 % of children have well controlled hypertension. The aim of this interventional study is to improve blood pressure control and to investigate whether the improved control will improve the graft survival. METHODS AND RESULTS: 36 children after RTx (mean age 13.9 +/- 4.4 years, time after RTx 2.7 +/- 2.4) fulfilled the inclusion criteria. Ambulatory blood pressure monitoring (ABPM) and graft function were examined. In children with uncontrolled hypertension, the dose and number of antihypertensive drugs were increased to reach BP <95th centile. ABPM was repeated after 12 months. After 12 months day-time and night-time BP dropped non-significantly, however prevalence of uncontrolled hypertension improved significantly from 42 % to 34 % (p<0.05). Number of antihypertensive drugs increased from 2.1 +/- 0.9 to 2.4 +/- 0.8 drugs per patient (p<0.05), namely that of ACE-inhibitors (from 19% to 27%, p<0.05). Graft function decreased by 3.6 ml/min/1.73m2/year (p<0.05). CONCLUSIONS: This 12 months interventional trial demonstrated that control of hypertension in children after RTx can be improved by increasing number of prescribed antihypertensive drugs. The decline of graft function was lower comparing with previous trials.


Asunto(s)
Hipertensión/tratamiento farmacológico , Trasplante de Riñón , Riñón/fisiopatología , Adolescente , Niño , Humanos , Hipertensión/etiología , Riñón/efectos de los fármacos , Trasplante de Riñón/fisiología
14.
Cas Lek Cesk ; 145(5): 390-2, 2006.
Artículo en Cs | MEDLINE | ID: mdl-16755777

RESUMEN

BACKGROUND: Thrombotic thrombocytopenic purpura is characterized by microvascular platelet clumping resulting in thrombocytopenia, microangiopathic hemolysis, neurological abnormality, and renal dysfunction. Similar manifestations also occur in patients with the hemolytic uremic syndrome or other types of disorders. Recent studies demonstrate that severe deficiency of the von Willebrand factor cleaving metalloprotease, ADAMTS 13, causes thrombotic thrombocytopenic purpura. Aim of our study was to characterize gene defects causing inherited type of disease. METHODS AND RESULTS: We investigated nine patients with recurrent type of disease with familiar origin and twelve relatives. Samples were taken in a remission of disease. We measured activity of ADAMTS13 (vWF-CP) with modified method of the quantitative immunoblotting of degraded vWF multimers. Mutation screening was carried out by sequencing all 29 exons and flanking intron regions of the ADAMTS13 gene. Five distinct mutations were found. Three of them are novel. CONCLUSIONS: Mutation analysis of the ADAMTS 13 gene brought interesting results in eight patients. We found a one single base frameshift insertion, 4143insA in 8 of 9 unrelated individuals. This investigation represents an advantage in the differential diagnosis of disease since the thrombotic thrombocytopenic purpura phenotype in childhood can be variable and rapid detection of mutation is helpful for the recurrence prevention.


Asunto(s)
Proteínas ADAM/genética , Púrpura Trombocitopénica Trombótica/genética , Proteína ADAMTS13 , Niño , Mutación del Sistema de Lectura , Humanos , Mutación , Factor de von Willebrand/genética
15.
Transplant Proc ; 37(10): 4282-3, 2005 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-16387097

RESUMEN

Proteinuria is associated with poor long-term allograft as well as patient survival among adults after renal transplantation. In children, there are no studies focusing primarily on posttransplant proteinuria. The aim of this cross-sectional study was to investigate the prevalence of and possible risk factors associated with proteinuria. Thirty-three children (mean age of 13.7 +/- 4.3 years; mean time after renal transplantation = 2.3 +/- 2.2 years) were eligible for the study. There was an 82% prevalence of proteinuria (> or =96 mg/m2/d) with nephrotic range proteinuria (> or =960 mg/m2/d) in 12% of children. The mean urinary protein excretion was 256 +/- 299 mg/m2/d (range = 47 to 1264). Children with hypertension, as defined by ambulatory blood pressure monitoring, showed significantly higher proteinuria than normotensive children (382 +/- 435 vs 163 +/- 79 mg/m2/d, P < .05). Children with a history of a previous acute rejection episode showed significantly higher proteinuria than children who never had an episode (416 +/- 445 vs 165 +/- 91 mg/m2/d, P < .05). Children with proteinuria did not show statistically different graft function than children without proteinuria. No statistically significant correlation was observed between proteinuria and ambulatory blood pressure values or graft function. In conclusion, proteinuria is a frequent finding also in children after renal transplantation; it is associated with hypertension and a history of rejection episodes.


Asunto(s)
Trasplante de Riñón/efectos adversos , Proteinuria/epidemiología , Adolescente , Monitoreo Ambulatorio de la Presión Arterial , Niño , Preescolar , Femenino , Humanos , Trasplante de Riñón/fisiología , Masculino , Prevalencia
16.
Arch Intern Med ; 157(19): 2259-68, 1997 Oct 27.
Artículo en Inglés | MEDLINE | ID: mdl-9343003

RESUMEN

BACKGROUND: Exponential growth in the population of older adults presents clinicians with special concerns about factors affecting risks for declines in cognitive and physical functioning. OBJECTIVES: To examine the hypothesis that risks for such declines and for disease outcomes, such as cardiovascular disease, are related to differences in allostatic load, the cumulative physiologic toll exacted on the body over time by efforts to adapt to life experiences. To present an operational definition of allostatic load, along with preliminary evidence of its predictive validity in relation to salient outcomes of aging. METHODS: Data from a longitudinal, community-based study of successful aging were used to develop a measure of allostatic load based on 10 parameters reflecting levels of physiologic activity across a range of important regulatory systems. Allostatic load is the sum of the number of parameters for which the subject was rated in the highest-risk quartile. RESULTS: Higher allostatic load scores were associated with poorer cognitive and physical functioning and predicted larger decrements in cognitive and physical functioning as well as being associated with an increased risk for the incidence of cardiovascular disease, independent of sociodemographic and health status risk factors. CONCLUSIONS: Findings are consistent with the conceptualization of allostatic load as an index of wear and tear on the body, with elevations in allostatic load predicting an increased risk for a decline in cognitive and physical functioning as well as cardiovascular disease in a cohort of older men and women. From a clinical perspective, the concept of allostatic load may provide the basis for a more comprehensive assessment of major risks in the aging process.


Asunto(s)
Adaptación Fisiológica , Adaptación Psicológica , Envejecimiento , Anciano , Envejecimiento/fisiología , Envejecimiento/psicología , Enfermedades Cardiovasculares/fisiopatología , Trastornos del Conocimiento/fisiopatología , Trastornos del Conocimiento/psicología , Femenino , Humanos , Masculino , Riesgo
17.
Arch Intern Med ; 157(19): 2196-204, 1997 Oct 27.
Artículo en Inglés | MEDLINE | ID: mdl-9342996

RESUMEN

BACKGROUND: Functional disability is a common condition among elderly patients. However, to our knowledge, its effect on outcome of myocardial infarction (MI) has not been assessed. Our objectives were to determine whether disability in the activities of daily living measured before MI is a predictor of MI severity and mortality. METHODS: Disability in activities of daily living was measured prospectively in a cohort of 222 patients who were hospitalized with acute MI. Outcome measures were severity characteristics on admission to the hospital (higher Killip class, presence of new Q waves in the first electrocardiogram, and lower systolic blood pressure), and 6-month mortality. RESULTS: Patients with disability before hospitalization were older and had more comorbidity. After adjusting for these factors and for delay in hospital arrival, disability was still significantly associated with clinical severity on admission to the hospital and with mortality (adjusted relative risk of death for patients with disability vs patients without disability, 2.01; 95% confidence interval, 1.23-3.28). Clinical severity and hospital treatment explained the higher mortality of patients with disability. When these factors were added to the previous model, the relative risk of mortality for patients with disability vs patients without disability was 1.24, and the 95% confidence interval was 0.73 to 2.12. CONCLUSIONS: Functional disability in activities of daily living before MI is an important predictor of clinical severity and mortality in elderly patients with MI. This effect is only minimally explained by the older age and higher comorbidity of patients with disability. However, higher clinical severity and lower use of treatment interventions are major determinants of their higher mortality compared with patients without disability.


Asunto(s)
Personas con Discapacidad , Infarto del Miocardio/mortalidad , Infarto del Miocardio/fisiopatología , Actividades Cotidianas , Anciano , Electrocardiografía , Femenino , Hospitalización , Humanos , Masculino , Valor Predictivo de las Pruebas , Estudios Prospectivos , Riesgo , Índice de Severidad de la Enfermedad , Análisis de Supervivencia
18.
Arch Intern Med ; 158(21): 2341-8, 1998 Nov 23.
Artículo en Inglés | MEDLINE | ID: mdl-9827785

RESUMEN

BACKGROUND: Results of several recent studies suggest that depression is predictive of incident coronary disease. However, few studies have examined this relationship in the elderly, the age at which most coronary heart disease (CHD) becomes clinically manifest. METHODS AND RESULTS: Data are from the New Haven, Conn, cohort (N = 2812) of the Established Populations for the Epidemiologic Studies of the Elderly project. Baseline information on depressive symptoms and CHD risk factors was collected during an in-person interview in 1982. Nonfatal myocardial infarctions were identified through monitoring of admissions to local hospitals and were validated by medical chart review. Cause of death was obtained from death certificates for all deceased participants. Outcomes were defined as CHD deaths (n = 255) and total incident CHD events (n = 391) between January 1, 1982, and December 31, 1991. There was no association between depressive symptoms and CHD outcomes in men. Among women, depressive symptoms were associated with an age-adjusted relative risk of 1.03 (per unit increase on the symptom scale) for CHD mortality (P=.001) and total CHD incidence (P=.002). These associations were largely unaffected by adjustment for established CHD risk factors but were reduced to nonsignificant levels after additional adjustment for impaired physical function. Additional analysis showed a significant association for depressive symptoms among women who had no physical function impairments or who survived at least 3 years without an event. CONCLUSION: Depressive symptoms may not be independent risk factors for CHD outcomes in elderly populations in general but may increase risk among relatively healthy older women.


Asunto(s)
Enfermedad Coronaria/etiología , Depresión/complicaciones , Actividades Cotidianas , Factores de Edad , Anciano , Causas de Muerte , Estudios de Cohortes , Connecticut , Certificado de Defunción , Femenino , Predicción , Hospitalización , Humanos , Incidencia , Entrevistas como Asunto , Masculino , Auditoría Médica , Infarto del Miocardio/etiología , Evaluación de Resultado en la Atención de Salud , Admisión del Paciente , Reproducibilidad de los Resultados , Factores de Riesgo , Factores Sexuales , Tasa de Supervivencia
19.
Health Place ; 32: 93-8, 2015 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-25665936

RESUMEN

Physical and social features of neighborhoods, such as esthetic environments and social cohesion, change over time. The extent to which changes in neighborhood conditions are associated with changes in mental health outcomes has not been well-established. Using data from the MultiEthnic Study of Atherosclerosis, this study investigated the degree to which neighborhood social cohesion, stress, violence, safety and/or the esthetic environment changed between 2002 and 2007 in 103 New York City Census tracts and the associations of these changes with changes in depressive symptoms. Neighborhoods became less stressful, more socially cohesive, safer, and less violent. White, wealthy, highly educated individuals tended to live in neighborhoods with greater decreasing violence and stress and increasing social cohesion. Individuals living in neighborhoods with adverse changes were more likely to have increased CES-D scores, although due to limited sample size associations were imprecisely estimated (P>0.05). Changes in specific features of the neighborhood environment may be associated with changes in level of depressive symptoms among residents.


Asunto(s)
Depresión/epidemiología , Características de la Residencia/estadística & datos numéricos , Medio Social , Apoyo Social , Violencia/estadística & datos numéricos , Anciano , Anciano de 80 o más Años , Antidepresivos/uso terapéutico , Aterosclerosis , Depresión/tratamiento farmacológico , Femenino , Humanos , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Ciudad de Nueva York/epidemiología , Escalas de Valoración Psiquiátrica , Seguridad , Factores Socioeconómicos , Estrés Psicológico/epidemiología , Violencia/tendencias
20.
J Clin Endocrinol Metab ; 82(8): 2458-65, 1997 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-9253318

RESUMEN

Cortisol production is increased during stress, and the actions of cortisol on receptors in the brain and other body organs are involved in allostasis, the process of adaptation to stress, as well as in allostatic load, the wear and tear associated with excessive exposure to cortisol. Using data from a community-based longitudinal study of older men and women, aged 70-79 yr, we tested the hypothesis that exposure to increasing levels of cortisol is associated with declines in memory performance. Associations between 12-h urinary free cortisol excretion and performance on tests of memory (delayed verbal recall and spatial recognition), abstraction, and spatial ability were examined. Among the women, greater cortisol excretion was associated with poorer baseline memory performance, independent of socio-demographic, health status, health behavior, and psychosocial characteristics. Moreover, women who exhibited increases in cortisol excretion over a 2.5-yr follow-up period were more likely to show declines in memory performance. By contrast, women who experienced declines in cortisol exhibited improvements in memory performance. No significant associations were found among the men. The results for the women suggest that decrements in memory performance associated with increases in cortisol may not represent irreversible effects, as declines in cortisol were associated with improvements in memory.


Asunto(s)
Envejecimiento/orina , Hidrocortisona/orina , Memoria/fisiología , Anciano , Cognición , Femenino , Humanos , Estudios Longitudinales , Masculino , Caracteres Sexuales
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