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In June 2015, an outbreak of cryptosporidiosis with 35 cases (23 probable and 12 laboratory-confirmed) occurred among 191 attendees of a residential rehabilitation holiday for paediatric organ transplant patients (n = 49) and their families at a hotel in Somogy county, Hungary. The overall attack rate was 18%. Most of the cases were transplanted children who experienced severe acute disease and required adjustment to their tacrolimus immunosuppression. A retrospective case-control study suggested an association between recreational water exposures and illness: cases were seven times more likely than controls to have swum in the children's pool (odds ratio 7.17; 95% confidence interval 2.9-17.2; P < 0.0001) and five times more likely to have used the jetted whirlpool (odds ratio 5.25; 95% confidence interval 2.1-13.1; P < 0.0001). This was the first outbreak of cryptosporidiosis in Hungary and it is especially unfortunate that it affected vulnerable children who experienced severe symptoms. Cryptosporidium presents specific infection control difficulties in treated recreational water venues; the link to a whirlpool is unusual and highlights the importance of the age-appropriate use of these facilities and reminding users not to immerse their heads or swallow the water. Cryptosporidiosis is more commonly linked to children' pools where improved bather hygiene and promoting exclusion of diarrhoea cases could help to avoid similar outbreaks.
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BACKGROUND: Growth retardation in paediatric end-stage renal disease (ESRD) has a serious impact on adult life. It is potentially treatable with recombinant growth hormone (rGH). In this study, we aimed to quantify the variation in rGH policies and actual provided care in these patients across Europe. METHODS: Renal registry representatives of 38 European countries received a structured questionnaire on rGH policy. Cross-sectional data on height and actual use of rGH on children with ESRD aged <18 years were retrieved from the ESPN/ERA-EDTA Registry. RESULTS: In 21 (75%) of 28 responding countries, rGH is reimbursed for children with ESRD. The specific conditions for reimbursement (minimum age, maximum age and chronic kidney disease stage) vary considerably. Mean height standard deviation scores (SDS) at renal replacement therapy (RRT) [95% confidence interval (CI)] were significantly higher in countries where rGH was reimbursed -1.80 (-2.06; -1.53) compared with countries in which it was not reimbursed [-2.34 (-2.49;-2.18), P < 0.001]. Comparison of the mean height SDS at onset of RRT and final height SDS yielded similar results. Among the 13 countries for which both data on actual rGH use between 2007 and 2011 and data from the questionnaire were available, 30.1% of dialysis and 42.3% of transplanted patients had a short stature, while only 24.1 and 7.6% of those short children used rGH, respectively. CONCLUSION: Reimbursement of rGH associates with a less compromised final stature of ESRD children. In many countries with full rGH reimbursement, the actual rGH prescription in growth-retarded ESRD children is low and obviously more determined by the doctor's and patients' attitude towards rGH therapy than by financial hurdles.
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Hormônio do Crescimento Humano/uso terapêutico , Falência Renal Crônica/terapia , Padrões de Prática Médica/legislação & jurisprudência , Medicamentos sob Prescrição/administração & dosagem , Adolescente , Adulto , Estatura , Criança , Pré-Escolar , Estudos Transversais , Europa (Continente) , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Sistema de Registros , Terapia de Substituição Renal/estatística & dados numéricos , Inquéritos e Questionários , Adulto JovemRESUMO
Real-time ultrasound guidance for any intervention relies on visualization of needle advancement towards a target. Unfortunately, correct identification of the needle tip is not straightforward, as artifacts always distort the image. The ultrasonic appearance of the needle is often degraded by reverberation, comet tail, side-lobe, beam-width, or bayonet artifacts, which can easily confuse an unprepared operator. Furthermore, the typical needle image, that is, a dot or a straight line (out-of-plane and in-plane approaches, respectively), is also a result of artifacts that hide the real dimensions of the needle. Knowledge and correct interpretation of these artifacts is important for safe practice and is paramount to success when precise needle manipulation is mandatory, for example, when the target is small. In this review, authors discuss the most important needle-related artifacts and provide a physical explanation focusing on implications for everyday practice. Recent advances that allow increased needle visualization and reduction of artifacts are also discussed.
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Anestesiologia/métodos , Artefatos , Agulhas , Ultrassonografia de Intervenção/instrumentação , Ultrassonografia de Intervenção/métodos , Dispositivos de Acesso Vascular , Anestésicos/administração & dosagem , Humanos , Segurança do PacienteRESUMO
Solid-organ transplantation is the optimal long-term treatment for most patients with end-stage organ failure. After solid-organ transplantation, short-term graft survival significantly improved (1). However, due to chronic allograft nephropathy and death with functioning graft, long-term survival has not prolonged remarkably (2). Posttransplant immunosuppressive medications consist of one of the calcineurin inhibitors in combination with mycophenolate mofetil (MMF) or azathioprine (Aza) and steroids. All of them have different adverse effects, among which posttransplant diabetes mellitus (PTDM) is an independent risk factor for cardiovascular (CV) events and infections causing the death of many transplant patients and it may directly contribute to graft failure (3). According to the criteria of the American Diabetes Association (4), diabetes mellitus (DM) is defined by symptoms of diabetes (polyuria and polydipsia and weight loss) plus casual plasma glucose concentration ≥ 11.1 mmol/L or fasting plasma glucose (FPG) ≥ 7.0 mmol/L or 2-h plasma glucose level ≥ 11.1 mmol/L following oral glucose tolerance test (OGTT). This metabolic disorder occurring as a complication of organ transplantation has been recognized for many years. PTDM, which is a combination of decreased insulin secretion and increased insulin resistance, develops in 4.9/15.9% of liver transplant patients, in 4.7/11.5% of kidney recipients, and in 15/17.5% of heart and lung transplants [cyclosporine A (CyA)/tacrolimus (Tac)-based regimen, respectively] (5). Risk factors of PTDM can be divided into non-modifiable and modifiable ones (6), among which the most prominent is the immunosuppressive therapy being responsible for 74% of PTDM development (7). Emphasizing the importance of the PTDM, numerous studies have determined the long-term outcome. On the basis of these studies, graft and patient survival is tendentiously (8) or significantly (9, 10) decreased for those developing PTDM.
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Diabetes Mellitus/etiologia , Imunossupressores/efeitos adversos , Transplante de Órgãos/efeitos adversos , Complicações Pós-Operatórias/etiologia , Criança , Diabetes Mellitus/induzido quimicamente , Diabetes Mellitus/imunologia , Humanos , Terapia de Imunossupressão/efeitos adversos , Imunossupressores/uso terapêutico , Modelos Biológicos , Complicações Pós-Operatórias/induzido quimicamente , Complicações Pós-Operatórias/imunologia , Fatores de Risco , Condicionamento Pré-Transplante/efeitos adversos , Condicionamento Pré-Transplante/métodosRESUMO
PTDM plays a role in chronic allograft nephropathy and decreases graft and patient survival. Considering the serious outcome of chronic hyperglycemia, the importance of early recognition and the few data in children, in this retrospective analysis we studied the characteristics and risk factors of PTDM in 45 pediatric renal transplant recipients receiving Tac or CyA-based immunosuppression. Fasting blood sampling and OGTT were performed. PTDM has been developed in six patients (13%), while seven children (16%) had IGT, with the overall incidence of a glucose metabolic disorder of 29% in pediatric renal transplants. Patients in the PTDM + IGT group were younger and had higher systolic blood pressure and serum triglyceride level than children with normal glucose tolerance. Multivariate analysis identified Tac treatment, Tac trough level, steroid pulse therapy and family history of diabetes to be associated with the onset of PTDM. In pediatric renal transplants, OGTT and frequent assessment of blood glucose levels might be essential not only in the post-transplant management, but also prior to transplantation, particularly with family history of diabetes. Careful monitoring and modified protocols help to minimize the side effects of Tac and corticosteroids.
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Diabetes Mellitus/etiologia , Transplante de Rim/efeitos adversos , Administração Oral , Adolescente , Adulto , Glicemia/metabolismo , Criança , Diabetes Mellitus/diagnóstico , Feminino , Humanos , Imunossupressores/uso terapêutico , Nefropatias/terapia , Masculino , Metilprednisolona/administração & dosagem , Esteroides/farmacologia , Tacrolimo/efeitos adversosRESUMO
BACKGROUND: Low heart rate variability (HRV) is an independent risk factor of cardiac mortality in patients with end-stage renal disease (ESRD). It has been explained by uremic parasympathetic neuropathy. Sympathetic overactivity can also reduce HRV. Our aim was to determine whether there is vagal activity in ESRD patients that is masked by sympathetic activity. METHODS: The effect of propranolol on HRV was examined in 13 patients with ESRD, aged 20.1 +/- 7.6 years without diabetes. All patients were given intravenous propranolol (0.05 mg/kg) once and placebo once in a randomized, double-blind way, with an interval of 6.6 days (mean, range: 2-9). Propranolol was administered before hemodialysis treatment, after 40 minutes supine resting period. HRV was registered for 10 minutes, during supine, before and after the injection. Patients' HRV data were compared to that of 29 age-matched healthy controls. RESULTS: Initially, both high-(HFV) and low-frequency (LFV) bands of heart rate variability were lower in ESRD patients compared to controls (p < 0.001 for both). Propranolol resulted in a significant increase of HFV (propranolol: AlgHFV = 0.182 (0.027 - 0.337), placebo: deltalgHFV = -0.029 (-0.128 - +0.070); p = 0.032). Elevation of LFV was not significant. Six patients had an elevated plasma norepinephrine and/or epinephrine level. Plasma dopamine level was elevated in all but 1 patient (mean: 432 pmol/l, 95% CI: 320-543) and showed an inverse relationship with the increase of IgHFV secondary to propranolol (r = -0.66, p = 0.014). CONCLUSIONS: Low HFV of ESRD patients can be improved by beta-adrenergic blockade. It demonstrates that there is some vagal activity in ESRD that is masked by sympathetic activity. Therefore, altered sympathovagal balance of ESRD patients should be taken into consideration in the assessment of vagal uremic neuropathy.
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Antagonistas Adrenérgicos beta/farmacologia , Frequência Cardíaca/efeitos dos fármacos , Falência Renal Crônica/fisiopatologia , Propranolol/farmacologia , Adolescente , Adulto , Criança , Estudos Cross-Over , Dopamina/metabolismo , Método Duplo-Cego , Epinefrina/metabolismo , Feminino , Humanos , Falência Renal Crônica/terapia , Masculino , Norepinefrina/metabolismo , Projetos Piloto , Diálise Renal , Sistema Nervoso Simpático/fisiologia , Nervo Vago/fisiologiaRESUMO
The urinary enzymes Gamma Glutamyl Transferase (GGT), Alkaline Phosphatase (ALP), Leucine-Arylamidase (LAS), and Dipeptidyl-Peptidase-IV (DPP-IV) were measured before and after endoscopic treatment of vesico-ureteric reflux (VUR) in two groups of twenty children. Ten patients had undergone successful endoscopic corrective surgery for VUR, another 10 patients had unsuccessful endoscopic intervention. After successful treatment the activity of LAS in the urine did not change, but GGT, ALP and DPP-IV activity in the urine was 2-5 times higher than before treatment (P < 0.03 for all three enzymes). Considerable changes of urinary enzyme activity were not observed following unsuccessful endoscopic treatment. Our data and the literature are contradictory. However, this contradiction might be explained by the differences in urine sampling methods. Our patients received the same chemoprophylactic drug at the time of both urine samplings, a point not considered by other researchers. The extent of increase of enzyme activity after endoscopic treatment of VUR did not reach the level that would permit the use of investigated enzymes for screening, because the observed changes did not exceed the limits of the normal range.
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Escleroterapia/métodos , Refluxo Vesicoureteral/enzimologia , Refluxo Vesicoureteral/terapia , Fosfatase Alcalina/urina , Estudos de Casos e Controles , Criança , Dipeptidil Peptidase 4/urina , Feminino , Humanos , Leucil Aminopeptidase/urina , Masculino , Soluções Esclerosantes/uso terapêutico , gama-Glutamiltransferase/urinaRESUMO
Familiar hypophosphatemic rickets (FHR) is characterized by isolated defect of renal phosphate reabsorption, hypophosphataemia, rickets and poor growth. In untreated cases parathyroid hormone and calcitriol levels are normal. FHR is caused by mutations of the PHEX gene encoding a zinc-binding metalloprotease enzyme. PHEX is expressed in bones and the parathyroid gland but not in the kidney. The gene product is involved in the inactivation of a phosphate regulating hormone (phosphatonin). The presence of this hormone through unknown mechanisms decreases the sodium-dependent phosphate cotransporter in the kidney resulting in impaired phosphate transport. In addition the PHEX gene product exerts autocrine and paracrine effects on the bone. Despite recent advances in the understanding of the pathomechanism, treatment of FHR is still symptomatic. It consists of active vitamin D analogues and oral phosphate supplementation. Nephrocalcinosis is a well-known, usually non-progressive side effect of the conventional therapy. As shown by pilot studies, poorly growing children with FHR may benefit from the positive effect of human recombinant growth hormone (rhGH). However, rhGH treatment could aggravate the already existing tendency to disproportionate growth resulting in the overgrowth of the trunk. The disturbed phosphate homeostasis persists during the whole life span of the FHR patients. It is therefore essential to provide lifelong care, to prevent late skeletal and dental consequences or to treat them if already established. That care should be done by the teamwork of the pediatrician, internist, orthopedist, dentist and the psychologist.
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Hormônio do Crescimento Humano/administração & dosagem , Hipofosfatemia/genética , Metaloendopeptidases/genética , Mutação , Fosfatos/administração & dosagem , Fosfatos/metabolismo , Raquitismo/genética , Administração Oral , Animais , Calcinose , Hormônio do Crescimento Humano/efeitos adversos , Humanos , Hipofosfatemia/diagnóstico , Hipofosfatemia/metabolismo , Hipofosfatemia/terapia , Rim/metabolismo , Rim/patologia , Raquitismo/metabolismoRESUMO
This review describes the supposed mechanisms leading to idiopathic hypercalciuria (IHU) in childhood, further the diagnostic criteria and the proposed treatment modalities are discussed. IHU is not only one of the main causes of renal stone disease in children but it's also at the origin of the postglomerular haematuria and the frequency-dysuria syndrome. Its role in the development of osteoporosis in adults is also documented. The diagnosis of raised calcium excretion is based on age specific values during early infancy. In older children and adults a urinary calcium/creatinine ratio exceeding 0.6 mmol/mmol is regarded as elevated. Dietary calcium restriction can no longer be recommended for the treatment of IHU because it results in secondary hyperoxaluria and on the long-term causes decreased bone mineral density. Patients should be kept on dietary sodium restriction and high fluid intake. In cases IHU associated with recurrent episodes of macroscopic haematuria or recurrent stone disease a therapeutic trial with hydrochlorothiazide in the dose of 0.5-1 mg/kg/day with potassium-citrate supplementation and possibly magnesium citrate should be started. In some special forms of hypercalciuria such as the X-linked recessive nephrolithiasis syndrome or Bartter syndrome the localization and in some cases even the molecular mechanism of the events leading to increased calcium excretion are elucidated. In IHU enhanced Ca(++)-ATPase, and Na-Li countertransport activity and decreased Na+/K+ ATPase activity were described in the erythrocyte membrane model. It is expected that with the molecular genetic development the clinical classification of the hypercalciuric syndromes will become a rational genome-based one.
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Cálcio/urina , Cálculos Renais/etiologia , Adolescente , Adulto , Idoso , Densidade Óssea , Cálcio da Dieta/administração & dosagem , Criança , Pré-Escolar , Dieta Hipossódica , Diuréticos , Feminino , Hematúria/etiologia , Humanos , Hidroclorotiazida/uso terapêutico , Cálculos Renais/diagnóstico , Cálculos Renais/prevenção & controle , Masculino , Pessoa de Meia-Idade , Osteoporose/prevenção & controle , Inibidores de Simportadores de Cloreto de Sódio/uso terapêuticoRESUMO
Calcium-hydrogen-phosphate (CaHPO4) was considered as one of the main factors governing renal calculus formation. The degree of saturation (expressed as activity product) with respect to this phase was therefore calculated in urines of 36 hypercalciuric children (20 absorptive, 16 renal subtype) with isolated hematuria, 10 renal stone patients, and 30 healthy controls. On low calcium diet 12 children of the absorptive hypercalciuric-, 13 of the renal hypercalciuric and 7 of the renal stone forming children hat their urines in the saturated zone --irrespective of the evolution of hypercalciuria Ca/cr ratio. Thiazide normalised the activity product in all groups. The use of the Ca/cr ratio as the sole parameter in the investigation of children with isolated hematuria and hypercalciuria or calcium nephrolithiasis is therefore insufficient, simultaneous determinations of the state of saturation of urines is recommended. This technique should also allow a quantitative assessment of the various therapeutic regimens recommended.
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Cálcio/urina , Cálculos Renais/urina , Adolescente , Cálcio da Dieta/efeitos adversos , Criança , Pré-Escolar , Humanos , Fosfatos/urinaRESUMO
It is known that the prevalence of cardiovascular diseases, hypertension, noninsulin dependent diabetes mellitus and dyslipidemia in the late adulthood are in connection with intrauterine retardation, characterized by low birth weight. One possible explanation of this phenomenon is the abnormality of hypothalamus-hypophysis-adrenal cortex axis due to the accelerated growth. The authors investigated the steroid levels of young adults; whom birth weight were under 2500 g, and examined the relationship between hormone levels and some parameters of glucose metabolism and cardiovascular system. 75 subjects (43 female and 32 male patients, mean age: 19.6 and 19.8 years, respectively; range 18-22 ys) with low birth weight and without any sign of chronic disease, and 30 healthy, age-matched controls with normal birth weight were investigated. The basal serum cortisol, dehydroepiandrosterone (DHEA), dehydroepiandrosterone sulphate (DHEAS), androstenedione (AD), 17-hydroxyprogesterone (17OHP), estradiol (OE), sex-hormone binding globulin (SHBG), FSH, LH and insulin levels were determined. Moreover, oral glucose tolerance test with 75 g glucose (OGTT), impedance cardiography as well as ambulatory blood pressure monitoring were done by all subjects. In both sexes in subjects with low birth weight the mean serum cortisol level was significantly higher, than in the normal controls. In female patients the serum DHEA, DHEAS, AD, and 17OHP levels were significantly higher than in the controls. Moreover, among these females a relationship was found between the elevations of adrenal and gonadal steroids and hyperinsulinemia, characterized by increased insulin response during OGTT. In male subjects a significant correlation was found between serum cortisol levels and systolic blood pressure and heart rate. In females there was a positive relationship between serum DHEA and heart rate. Summarized, the basic abnormality in patients with low birth weight seems to be a relative hypercortisolism, and in females because of hyperinsulinemia exists a mild hyperandrogenism as well. The hypercortisolism may cause cardiovascular abnormalities in males directly, while in females indirectly through the hyperinsulinemia and hyperandrogenism. These subtle abnormalities can be detected when no clinical signs present themselves, in young adulthood, giving the opportunity of taking preventive actions.
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Pressão Sanguínea , Carboidratos da Dieta/metabolismo , Hormônios Esteroides Gonadais/sangue , Frequência Cardíaca , Hidrocortisona/sangue , Recém-Nascido de Baixo Peso , 17-alfa-Hidroxiprogesterona/sangue , Adulto , Androstenodiona/sangue , Glicemia/metabolismo , Estudos de Casos e Controles , Desidroepiandrosterona/sangue , Estradiol/sangue , Feminino , Hormônio Foliculoestimulante/sangue , Teste de Tolerância a Glucose , Humanos , Recém-Nascido , Insulina/sangue , Hormônio Luteinizante/sangue , Masculino , Análise de Regressão , Fatores Sexuais , Globulina de Ligação a Hormônio Sexual/metabolismo , Testosterona/sangueRESUMO
The authors present a case of Imerslund-Gräsbeck syndrome, i. e. a familial megaloblastic anemia with proteinuria. The disease is due to congenital, selective malabsorption of vitamin B12. The subnormal absorption of vitamin B12 is not altered by orally given intrinsic factor, but parenteral vitamin B12 therapy results in complete recovery. Approximately 150 cases have been described in literature, the authors' case is the first in Hungary.
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Síndromes de Malabsorção/genética , Vitamina B 12/metabolismo , Anemia Megaloblástica/complicações , Anemia Megaloblástica/genética , Pré-Escolar , Feminino , Humanos , Síndromes de Malabsorção/complicações , Síndromes de Malabsorção/terapia , Proteinúria/etiologia , Síndrome , Vitamina B 12/administração & dosagemRESUMO
The enzyme Na+/K(+)-ATPase plays a central role in the regulation of transmembrane ionic transports. According to previous reports its activity decreased in uremic state. The activity of Na+/K(+)-ATPase in detergent pretreated erythrocytes was studied in seven uremic children prior to and following the hemodialysis (HD) session. Additionally, the level of endogenous digoxin like factors in the plasma (EDLF) was determined. Before the HD session the Na+/K(+)-ATPase activity decreased compared to the control value (mean +/- SD: 2078 +/- 527 vs 3245 +/- 362 nmol P/ml RBC/h, p < 0.01). Following HD it became normal (3366 +/- 952 nmol P/ml RBC/h, n.s.). Prior to the HD the EDLF level was higher, while after the HD no difference was noted from the control value (0.29 +/- 0.04, p < 0.05; 0.24 +/- 0.04 n. s. vs control; 0.21 +/- 0.04 ng/ml). Before the HD blood pressure was significantly elevated compared to the control (117 +/- 20/92 +/- 18 vs 95 +/- 2/64 +/- 2 Hgmm, p < 0.05). By the end of the HD it became normal (100 +/- 14/79 +/- 11, n.s.). Although no correlation was found between the EDLF level and Na+/K(+)-ATPase activity, a positive significant correlation was found between the changes of enzyme activity and the changes in the systolic (r = 0.83, p < 0.05) and diastolic (r = 0.82, p < 0.05) blood pressure during the HD. Our results indicate, that in uremic children the Na+/K+ pump is inhibited by a dialysable, blood-pressure regulator substance and so the enzyme activity elevates following the HD session. However, decreased blood pressure activates counterregulatory mechanisms, which-to lower extent-inhibit the activity of the pump.
Assuntos
Adenosina Trifosfatases/sangue , Digoxina , Inibidores Enzimáticos/sangue , Eritrócitos/química , Potássio/sangue , Diálise Renal , Saponinas/sangue , Sódio/sangue , Uremia/sangue , Adolescente , Pressão Sanguínea , Cardenolídeos , Criança , Feminino , Humanos , Masculino , Uremia/enzimologiaRESUMO
The urinary enzymes Gamma Glutamyl Transferase (GGT), Alkaline Phosphatase (ALP), Leucine-Arylamidase (LAS) and Dipeptidyl-Peptidase-IV (DPP-IV) were measured before and after endoscopic treatment of vesico-ureteric reflux (VUR) in two groups of twenty children's. Ten patients had undergone successful endoscopic corrective surgery for VUR, another 10 patients had unsuccessful endoscopic intervention. After successful treatment the activity of LAS in the urine did not change, but that of GGT, ALP and DDP-IV activity in the urine was 2-5 times higher than before treatment (P < 0.03 for all three enzymes). Considerable changes of urinary enzymes' activity were not observed following unsuccessful endoscopic treatment. Our data and the literature are contradictory. However this contradiction might be explained by the differences in urine sampling methods. Our patients received the same chemoprofilactic drug at the time of both urine sampling, a point not considered by other researchers. The extent of increase of enzyme activity after endoscopic treatment of VUR did not reach the level that would permit the use of investigated enzymes for screening, because the observed changes did not exceed the limits of the normal range.
Assuntos
Refluxo Vesicoureteral/cirurgia , Adolescente , Fosfatase Alcalina/urina , Criança , Pré-Escolar , Endoscopia , Humanos , Leucil Aminopeptidase/urina , Peptidil Dipeptidase A/urina , Silício/uso terapêutico , Refluxo Vesicoureteral/enzimologia , gama-Glutamiltransferase/urinaRESUMO
Aim of the study was to establish normal values for calcium/creatinine (Ca/cr) and oxalate/creatinine (Ox/cr) ratio in infants and children. Urine probes of 416 healthy children (25 infants aged 1-7 days and 391 children aged 1 month-14.5 years) were analysed. Oxalate was measured by ion-chromatography. Urinary Ca2+/cr was normally distributed, Ox/cr had log-normal distribution. Ca/cr was the lowest in the first days of life, the highest between 7 month-1.5 years (mean +/- SD = 0.39 +/- 0.28 mmol/mmol), a slight decrease could be observed until 14 years (0.34 +/- 0.18). The highest Ox/cr values were measured during the first month of life (geometric mean/range/ = 133 /61-280 mmol/mmol/), followed by gradual decrease until 14 years (25/6-73/). The measurement of Ca2+/cr and Ox/cr in first morning urine samples is suitable for screening of hypercalciuria and hyperoxaluria. The interpretation of the values requires age specific reference values. Both calcium and oxalate determinations should be the part of the evaluation of patients with hematuria, hypercalciuria or nephrolithiasis.
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Cálcio/urina , Oxalatos/urina , Adolescente , Fatores Etários , Criança , Pré-Escolar , Ritmo Circadiano , Creatinina/urina , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Valores de ReferênciaRESUMO
Recently, a new human virus hepatitis G virus was identified. The aim of the present study was to use a combination of the reverse transcription-polymerase chain reaction and a new test for antibodies to the viral envelope protein E2 to assess the prevalence of hepatitis G virus infection in sera of children and adults treated with hemodialysis or peritoneal dialysis as well as in sera of those who underwent renal transplantation. Hepatitis G virus RNA prevalence was higher in the shole group of patients with renal failure than in control group. The difference between hepatitis RNA prevalence in transplanted group and in control group was found to be significant. Anti-E2, which are is considered as an indicator of a past hepatitis G virus infection, were detected in the similar rate in the treated and control subjects. Time on hemodialysis was significantly longer in hepatitis G virus infected patients as compared to uninfected patients and all patients with hepatitis G virus RNA have a history of blood transfusions. Patients with renal failure treated with dialysis or subjected to renal transplantation are at increased risk of acquiring the hepatitis G virus infection. Higher rates of the hepatitis G virus RNA and similar prevalence of anti-E2 in patients with renal failure as compared to controls suggest that the rate of hepatitis G virus infection resolution in immunosuppressed patients with renal failure might be lower than in immunocompetent subjects.
Assuntos
Flaviviridae/isolamento & purificação , Transplante de Rim , Diálise Peritoneal Ambulatorial Contínua , Diálise Renal , Adolescente , Adulto , Criança , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/virologiaRESUMO
BACKGROUND: Cardiovascular mortality rate in patients with end-stage renal disease is 3 magnitudes higher than in the general population; it remains 10-fold higher after successful renal transplantation (Tx). Among others, obesity and hypertension can exert deleterious effects on vascular structure and function after Tx. Successful kidney transplantation may induce excessive weight gain in part because of the effects of steroid treatment. METHODS: The purpose of this study was to evaluate the presence of obesity in Tx children, their obesity-related metabolic disturbances, and to assess their blood pressure and arterial stiffness in relation to obesity. Forty-one transplant children (age, 15.7 [3.5] years; 28 males) were studied. Body composition was assessed by body mass index (BMI), waist circumference, skin-fold measurements, and multifrequence bioimpedance analysis. Glucose metabolism, blood pressure, and arterial stiffness (with the use of pulse wave velocity) were studied. Age- and sex-dependent parameters were expressed as standard deviation scores (SDS). RESULTS: The prevalence of overweight (BMI >85%) increased from 3.2% to 24.4% at 49 months (3-183) (median, range); the BMI SDS increased from -0.27 (0.79) to 0.67 (1.35) after Tx. There was a close correlation between BMI SDS and the percentage of body fat and body fat mass in the Tx group (r = 0.80; r = 0.94, P = .0001). Children with disturbed glycemic control (n = 14) had higher percentage of body fat and higher blood pressure compared with those with normal glucose metabolism (P < .05). There was no difference in pulse wave velocity between the lean and obese patients. CONCLUSIONS: The prevalence of overweight or obese patients in the Hungarian pediatric renal cohort is low at transplantation and rises subsequently. Overweight is associated with disturbed glycemic control and increased blood pressure; however, these disturbances are not yet reflected by stiffening of the arteries. Strategies are needed to prevent obesity, its impact on hypertension, and cardiovascular disease in pediatric transplantation.
Assuntos
Transplante de Rim/efeitos adversos , Síndrome Metabólica/epidemiologia , Obesidade/epidemiologia , Adolescente , Índice de Massa Corporal , Feminino , Humanos , Hungria/epidemiologia , Falência Renal Crônica/cirurgia , Masculino , Síndrome Metabólica/etiologia , Obesidade/etiologia , PrevalênciaRESUMO
BACKGROUND: Ultra-large von Willebrand factor and deficiency of its cleaving protease are important factors in the events leading to thrombotic microangiopathy; however, the mechanisms involved are only partly understood. Whereas pathological activation of the alternative complement pathway is linked to atypical hemolytic uremic syndrome, the role of complement activation in thrombotic thrombocytopenic purpura (TTP) is unknown. The aim of this study was to investigate whether signs of complement activation are characteristic of TTP. PATIENTS AND METHODS: Twenty-three patients with TTP (18 women, median age 38 years) and 17 healthy controls (13 women, median age 38 years) were included. Complement parameters (C3, Factors H, I, B and total alternative pathway activity) together with complement activation fragments (C3a) or complexes (C1rs-INH, C3bBbP, sC5b9) were measured by ELISA or RID. ADAMTS13 activity and anti-ADAMTS13 inhibitory antibodies were measured by the VWF-FRET73 assay. RESULTS: Increased levels of C3a, and SC5b9 were observed in TTP during acute episodes, as compared with healthy controls. Decreased complement C3 levels indicative of complement consumption occurred in 15% of acute TTP patients. Significant decrease of complement activation products C3a and SC5b9 was observed during plasma exchange (PEX). The sustained presence of anti-ADAMTS13 inhibitory antibodies in complete remission was associated with increased complement activation. CONCLUSION: These data document in an observational study the presence of complement activation in TTP. Further investigation is needed to determine its potential pathogenetic significance.
Assuntos
Ativação do Complemento , Proteínas do Sistema Complemento/análise , Púrpura Trombocitopênica Trombótica/imunologia , Proteínas ADAM/imunologia , Proteína ADAMTS13 , Adulto , Anticorpos Neutralizantes/sangue , Autoanticorpos/sangue , Biomarcadores/sangue , Estudos de Casos e Controles , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Hungria , Masculino , Pessoa de Meia-Idade , Troca Plasmática , Púrpura Trombocitopênica Trombótica/sangue , Púrpura Trombocitopênica Trombótica/terapia , RadioimunoensaioRESUMO
VACTERL association is a nonrandom association of birth defects in vertebral, anal, cardiac, tracheoesophageal, renal, and limb structures. Renal anomalies are observed in â¼60%-90% of VACTERL patients. We present 3 cases to demonstrate the clinical and surgical challenges that these patients present for renal transplantation. One pediatric and 2 adult patients with the VACTERL association were transplanted at a single center; their follow-up times were 6 years, 4 years, and 3 months. Only 1 of them had a suitable native bladder to receive the kidney graft; the other 2 required bladder augmentation, 1 of which was performed after the loss of the first graft. None of these patients had an uneventful posttransplantation course. Two patients had acute rejection episodes, and 2 had reoperations for urologic complications. One patient needed a surgical intervention owing to a sigmoid prolapse. All 3 grafts worked at last examination. The 2 patients with bladder reconstructions and longer follow-ups suffered recurrent pulmonary and urinary infections and had been hospitalized several times during each posttransplantation year. In conclusion, multiorgan involvement in VACTERL patients greatly complicates medical care after transplantation; urinary tract reconstruction seems to be essential before transplantation.
Assuntos
Cardiopatias Congênitas/complicações , Falência Renal Crônica/cirurgia , Transplante de Rim , Deformidades Congênitas dos Membros/complicações , Insuficiência Renal/cirurgia , Adulto , Canal Anal/anormalidades , Criança , Esôfago/anormalidades , Feminino , Rejeição de Enxerto/etiologia , Rejeição de Enxerto/cirurgia , Sobrevivência de Enxerto , Humanos , Rim/anormalidades , Falência Renal Crônica/etiologia , Transplante de Rim/efeitos adversos , Masculino , Recidiva , Insuficiência Renal/etiologia , Reoperação , Coluna Vertebral/anormalidades , Fatores de Tempo , Traqueia/anormalidades , Resultado do Tratamento , Ureterostomia , Doenças Urológicas/etiologia , Doenças Urológicas/cirurgia , Adulto JovemRESUMO
BACKGROUND: Hemolytic uremic syndrome (HUS) is a rare disease with various etiologies, making the identification of the specific forms and appropriate treatment difficult. Therefore, clinical and laboratory data from these patients need to be analyzed in national and international registries. Herein we have described 47 Hungarian HUS patients with detailed laboratory and clinical data obtained between 2008 and 2010. METHODS: Blood samples and clinical data of 47 patients with HUS diagnosed according to characteristic clinical signs were submitted for diagnostic evaluation, including complement protein and genetic analysis, measurement of ADAMTS13 activity and antibody analysis against O157LPS and factor H. RESULTS: There were 8 patients with typical diarrhea-positive HUS; 13 with atypical HUS (aHUS) and 26 with secondary HUS/thrombotic thrombocytopenic purpura group characterized by signs of complement consumption and decreased ADAMTS13 activity. Thus, decreased total alternative pathway activity is a promising diagnostic parameter with good sensitivity for aHUS. CONCLUSIONS: These observations highlight the requirement for multiple diagnostic tests together with clinical data to identify the specific cause of HUS. Because the long-term prognosis of aHUS, eg, graft survival after renal transplantation, may vary according to the molecular etiology, it is important for all affected patients to undergo a detailed molecular diagnosis of the disease. There is a clear clinical need for the development and application of novel assay in this field to allow more rapid efficient diagnosis of patients who undergo a first episode of HUS.