Resumo: A miocardiopatia dilatada é a forma mais comum de miocardiopatia e a principal causa de transplante cardíaco em idade pediátrica e em adultos. Os lactentes e as crianças apresentam etiologias ...de espectro mais alargado, embora a sua identificação seja mais difícil. A manifestação inicial mais frequente da miocardiopatia dilatada é a insuficiência cardíaca sintomática, em esforço ou repouso (embora muitos pacientes sejam assintomáticos). Como algumas das causas da miocardiopatia dilatada são potencialmente reversíveis, a investigação deve ser cuidadosamente planeada e feita imediatamente após o diagnóstico. Na maioria das crianças não é identificada uma causa, o que limita a abordagem terapêutica dirigida e, portanto, a eficácia do tratamento implantado.Os autores apresentam um caso de miocardiopatia dilatada secundária a hipertensão arterial renovascular, diagnosticada numa lactente de 3,5 meses, e destacam a investigação etiológica, o tratamento instituído e a sua evolução.Os autores salientam que o diagnóstico de hipertensão arterial no lactente nem sempre é fácil e questionam as atuais recomendações para início da medição da pressão arterial. Postulamos que a avaliação da pressão arterial em recém‐nascidos pode detetar hipertensão arterial renovascular precoce (e mesmo outras doenças cardiovasculares) e ajudar a prevenir o desenvolvimento de efeitos deletérios, inclusive episódios fatais. Abstract: Dilated cardiomyopathy is the most common form of cardiomyopathy and the main cause of cardiac transplantation in children and in adults. Infants and children have a wider spectrum of etiologies, hampering their identification. The most frequent initial manifestation of dilated cardiomyopathy is symptomatic heart failure during exercise or at rest (although many patients are asymptomatic). Some causes are potentially reversible, therefore the investigation should be carefully planned and immediately performed after diagnosis. In most children no cause is identified, which limits the targeted therapeutic approach and therefore the effectiveness of the treatment.The authors present a case of dilated cardiomyopathy secondary to renovascular hypertension diagnosed in an infant with 3.5 month‐old, highlighting the etiological investigation, treatment and evolution.The authors present this case emphasising the fact that the arterial hypertension diagnose in infants is not always easy, questioning the current recommendations relating to an initial evaluation on blood pressure. We postulate that the assessment of blood pressure in newborns can detect early renovascular hypertension (and even other cardiovascular diseases) and help prevent the development of deleterious effects, including fatal episodes. Palavras‐chave: Pediatria, Pressão arterial, Miocardiopatia dilatada, Estenose artéria renal, Keywords: Pediatrics, Blood Pressure, Dilated cardiomyopathy, Renal artery stenosis
A miocardiopatia dilatada é a forma mais comum de miocardiopatia e a principal causa de transplante cardíaco em idade pediátrica e em adultos. Os lactentes e as crianças apresentam etiologias de ...espectro mais alargado, embora a sua identificação seja mais difícil. A manifestação inicial mais frequente da miocardiopatia dilatada é a insuficiência cardíaca sintomática, em esforço ou repouso (embora muitos pacientes sejam assintomáticos). Como algumas das causas da miocardiopatia dilatada são potencialmente reversíveis, a investigação deve ser cuidadosamente planeada e feita imediatamente após o diagnóstico. Na maioria das crianças não é identificada uma causa, o que limita a abordagem terapêutica dirigida e, portanto, a eficácia do tratamento implantado.
Os autores apresentam um caso de miocardiopatia dilatada secundária a hipertensão arterial renovascular, diagnosticada numa lactente de 3,5 meses, e destacam a investigação etiológica, o tratamento instituído e a sua evolução.
Os autores salientam que o diagnóstico de hipertensão arterial no lactente nem sempre é fácil e questionam as atuais recomendações para início da medição da pressão arterial. Postulamos que a avaliação da pressão arterial em recém‐nascidos pode detetar hipertensão arterial renovascular precoce (e mesmo outras doenças cardiovasculares) e ajudar a prevenir o desenvolvimento de efeitos deletérios, inclusive episódios fatais.
Dilated cardiomyopathy is the most common form of cardiomyopathy and the main cause of cardiac transplantation in children and in adults. Infants and children have a wider spectrum of etiologies, hampering their identification. The most frequent initial manifestation of dilated cardiomyopathy is symptomatic heart failure during exercise or at rest (although many patients are asymptomatic). Some causes are potentially reversible, therefore the investigation should be carefully planned and immediately performed after diagnosis. In most children no cause is identified, which limits the targeted therapeutic approach and therefore the effectiveness of the treatment.
The authors present a case of dilated cardiomyopathy secondary to renovascular hypertension diagnosed in an infant with 3.5 month‐old, highlighting the etiological investigation, treatment and evolution.
The authors present this case emphasising the fact that the arterial hypertension diagnose in infants is not always easy, questioning the current recommendations relating to an initial evaluation on blood pressure. We postulate that the assessment of blood pressure in newborns can detect early renovascular hypertension (and even other cardiovascular diseases) and help prevent the development of deleterious effects, including fatal episodes.
Abstract Dilated cardiomyopathy is the most common form of cardiomyopathy and the main cause of cardiac transplantation in children and in adults. Infants and children have a wider spectrum of ...etiologies, hampering their identification. The most frequent initial manifestation of dilated cardiomyopathy is symptomatic heart failure during exercise or at rest (although many patients are asymptomatic). Some causes are potentially reversible, therefore the investigation should be carefully planned and immediately performed after diagnosis. In most children no cause is identified, which limits the targeted therapeutic approach and therefore the effectiveness of the treatment. The authors present a case of dilated cardiomyopathy secondary to renovascular hypertension diagnosed in an infant with 3.5 month-old, highlighting the etiological investigation, treatment and evolution. The authors present this case emphasising the fact that the arterial hypertension diagnose in infants is not always easy, questioning the current recommendations relating to an initial evaluation on blood pressure. We postulate that the assessment of blood pressure in newborns can detect early renovascular hypertension (and even other cardiovascular diseases) and help prevent the development of deleterious effects, including fatal episodes.
Dilated cardiomyopathy is the most common form of cardiomyopathy and the main cause of cardiac transplantation in children and in adults. Infants and children have a wider spectrum of etiologies, ...hampering their identification. The most frequent initial manifestation of dilated cardiomyopathy is symptomatic heart failure during exercise or at rest (although many patients are asymptomatic). Some causes are potentially reversible, therefore the investigation should be carefully planned and immediately performed after diagnosis. In most children no cause is identified, which limits the targeted therapeutic approach and therefore the effectiveness of the treatment. The authors present a case of dilated cardiomyopathy secondary to renovascular hypertension diagnosed in an infant with 3.5 month-old, highlighting the etiological investigation, treatment and evolution. The authors present this case emphasising the fact that the arterial hypertension diagnose in infants is not always easy, questioning the current recommendations relating to an initial evaluation on blood pressure. We postulate that the assessment of blood pressure in newborns can detect early renovascular hypertension (and even other cardiovascular diseases) and help prevent the development of deleterious effects, including fatal episodes.
Background Congenital anomalies of the kidney and urinary tract (CAKUT) are defined as structural malformations of the kidney and/or urinary tract. Heat shock proteins (HSPs) are expressed in the ...kidney in response to cellular changes, such as thermal, hemodynamic, osmotic, inflammatory, and mechanical stresses. This study aimed to assess uHSP70 levels during acute urinary tract infections (UTI) and non-infection periods in patients with CAKUT, and to evaluate whether uHSP70 is elevated in CAKUT subtypes. Methods Among patients with CAKUT, 89 patients with UTI (CAKUT-A), 111 without UTI (CAKUT-B), and 74 healthy children were included in the study. uHSP70 levels were measured using enzyme-linked immunosorbent assay (ELISA). Results uHSP70 level was significantly higher in the CAKUT-A group than in the CAKUT-B and healthy control groups (p < 0.0001). Moreover, the level of uHSP70 was significantly higher in the CAKUT-B group than in the control group (p < 0.0001), but was not different between the CAKUT subtypes (p > 0.05). Conclusion Urine HSP70 can also be used to predict UTI in patients with CAKUT. Moreover, uHSP70 levels were higher in children with CAKUT during the non-infectious period than in healthy controls. This suggests that children with CAKUT are at risk of chronic non-infectious damage.
Autosomal dominant polycystic kidney disease is the most common inheritable kidney disease, frequently thought to become symptomatic in adulthood. However, patients with autosomal dominant polycystic ...kidney disease may develop signs or symptoms during childhood, in particular hypertension. Although ambulatory BP monitoring is the preferred method to diagnose hypertension in pediatrics, data in children with autosomal dominant polycystic kidney disease are limited.
Our retrospective multicenter study was conducted to collect ambulatory BP monitoring recordings from patients with autosomal dominant polycystic kidney disease age <18 years old. Basic anthropometric parameters as well as data on kidney function, BP treatment, and kidney ultrasound were also collected.
Data from 310 children with autosomal dominant polycystic kidney disease with a mean age of 11.5±4.1 years old were collected at 22 European centers. At the time when ambulatory BP monitoring was performed, 95% of children had normal kidney function. Reference data for ambulatory BP monitoring were available for 292 patients. The prevalence rates of children with hypertension and/or those who were treated with antihypertensive drugs were 31%, 42%, and 35% during daytime, nighttime, or the entire 24-hour cycle, respectively. In addition, 52% of participants lacked a physiologic nocturnal BP dipping, and 18% had isolated nocturnal hypertension. Logistic regression analysis showed a significant association between a categorical cyst score that was calculated on the basis of the number of cysts >1 cm per kidney and daytime hypertension (odds ratio, 1.70; 95% confidence interval, 1.21 to 2.4;
=0.002), nighttime hypertension (odds ratio, 1.31; 95% confidence interval, 1.05 to 1.63;
=0.02), or 24-hour hypertension (odds ratio, 1.39; 95% confidence interval, 1.08 to 1.81;
=0.01). Kidney length, expressed as SD score, was also significantly associated with nighttime hypertension (odds ratio, 1.23; 95% confidence interval, 1.06 to 1.42;
=0.10).
These data indicate high prevalence of hypertension in children with autosomal dominant polycystic kidney disease starting at young ages.
The severity and dynamics of renal tissue damage in chronic kidney disease (CKD) may be reflected by the urinary excretion of vasoactive and growth factors released by the damaged kidney. Urinary ...excretion of ET-1, TGF-β1 and VEGF165 was evaluated in 303 children with CKD stage II–IV (GFR 48 ± 22 ml/min/1.73 m2) and 81 age-matched healthy controls. Major renal disease groups were hypo-/dysplastic kidney disease (N = 183), obstructive uropathies (N = 47), glomerulopathies (N = 34), nephronophthisis (N = 19) and polycystic kidney disease (N = 20). Results. The mean urinary excretion rates of each of the three putative biomarkers were significantly elevated in CKD patients compared to controls: 965 ± 2042 vs 216 ± 335 fmol/g creatinine for ET-1; 252 ± 338 vs 155 ± 158 ng/g for VEGF; 31.6 ± 37.0 vs 10.9 ± 9.8 ng/g for TGF-β1 (each P < 0.0001). The excretion of ET-1 and TGF-β1 was highest in patients with obstructive uropathies. In the patients, ET-1, TGF-β1 and VEGF excretion rates were inversely correlated with age (r = –0.22, −0.32 and −0.17, all P < 0.005) and renal function (r = –0.21, –0.13 and –0.15; P < 0.001; < 0.05; < 0.01; respectively) VEGF and TGF-β1 excretion rates were positively correlated both in patients and controls. Conclusions. Children with CKD exhibit significantly elevated urinary excretion of ET-1, TGF-β1 and VEGF165 in comparison to healthy children. Urinary excretion of these biomarkers was most enhanced in patients with obstructive uropathies. A positive correlation between urinary TGF-β1 and VEGF165 excretion, shown both in patients and healthy controls, indicates an interdependent nature of their generation.
Background
Glomerular filtration rate (GFR) is conventionally indexed to body surface area (BSA), but this may lead to biased results when applied to subjects of abnormal body size. The aim of our ...study was to examine the impact of normalization to the BSA and alternative body size descriptors on measured and estimated GFR in overweight and obese children.
Methods
This was a cross-sectional study of 313 children aged 8–9 years old. GFR was measured by 24-h creatinine clearance (CrCl) and additionally estimated from serum creatinine and cystatin C (CysC) using the combined Zappitelli formula, both as absolute values and adjusted to various body size descriptors. The results were compared between 163 normal-weight, 89 overweight and 61 obese children.
Results
Compared to the normal-weight children, mean absolute GFR (both measured and estimated) was higher in the overweight and obese children, whereas BSA-adjusted GFR was lower. Linear regression models fitted in normal-weight children revealed equally close associations between absolute GFR and squared height, ideal body weight (IBW) and BSA derived from IBW. Normalization of GFR to the IBW-derived BSA completely eliminated the discrepancy between absolute and BSA-indexed GFR in overweight and obese children.
Conclusions
Indexing of GFR to BSA calculated from the ideal—rather than actual—body weight is a promising approach to avoid overcorrection when studying obese children. Further studies should assess the accuracy of this approach across the full range of age and BMI distribution.
The number of children with acute kidney injury (AKI) requiring dialysis is increasing. To date, systematic analysis has been largely limited to critically ill children treated with continuous renal ...replacement therapy (CRRT). We conducted a survey among 35 European Pediatric Nephrology Centers to investigate dialysis practices in European children with AKI. Altogether, the centers perform dialysis in more than 900 pediatric patients with AKI per year. PD and CRRT are the most frequently used dialysis modalities, accounting for 39.4% and 38.2% of treatments, followed by intermittent HD (22.4%). In units treating more than 25 cases per year and in those with cardiothoracic surgery programs, PD is the most commonly chosen dialysis modality. Also, nearly one quarter of centers, in countries with a gross domestic product below $35,000/year, do not utilize CRRT at all. Dialysis nurses are exclusively in charge of CRRT management in 45% of the cases and pediatric intensive care nurses in 25%, while shared management is practiced in 30%. In conclusion, this survey indicates that the choice of treatment modalities for dialysis in children with AKI in Europe is affected by the underlying ethiology of the disease, organization/set-up of centers and socioeconomic conditions. PD is utilized as often as CRRT, and also intermittent HD is a commonly applied treatment option. A prospective European AKI registry is planned to provide further insights on the epidemiology, management and outcomes of dialysis in pediatric AKI.
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