The possible role of iodinated contrast in enhancing acute kidney injury in a critically ill neonate – a case report

Više modalitetni pristup neophodan je za postizanje točne dijagnoze u slučaju prirođenih srčanih grešaka prisutnih kod novorođen- četa. Napredak u medicinskim slikovnim metodama povećao je ovisnost o podacima dobivenim radiološkim pretragama. Među- tim, neke od tih pretraga zahtijevaju primjenu kontrasta, što može povećati rizik od kontrastom izazvanog akutnog oštećenja bubrega. Opisujemo slučaj novorođenčeta s transpozicijom velikih arterija, koje je izloženo iodiniranom kontrastu tijekom angio-tomografije. Nedugo nakon ovoga postupka kod novorođenčeta je nastupila anurija uz povišene razine ureje i kreatinina pa je dijagnosticirano akutno oštećenje bubrega izazvano kontrastom. Ubrzo potom dijete je umrlo usprkos primjeni peritonejske dijalize. Analiza provedena post mortem pokazala je proširenu koagulativnu nekrozu kortikalnih i medularnih slojeva bubrega.A multi-modality approach is necessary for accurate diagnosis in case of congenital heart diseases present in the neonatal period. Advances in medical imaging have increased dependence on the information provided by radiological studies. However, several of these studies require the use of contrast, which may enhance the risk of contrast-induced acute kidney injury. We report on a case of a neonate with transposition of great arteries who was submitted to iodinated contrast during angiotomography. Soon after the procedure, he presented anuria and increased urea and creatinine levels, and was diagnosed with a contrast-induced acute kidney injury. Despite the use of peritoneal dialysis, he died soon thereafter. Post mortem analysis showed extensive coagulative necrosis in the renal cortical and medullary layers

Development of Height and Body Mass Index After Pediatric Kidney Transplantation: Experience of the Nephrology Pediatric Service at HCFMRP- USP, 2005-2014

Abstract Introduction: Chronic kidney disease in children often determines poor nutritional status. Although renal transplantation (RTx) resolves endocrine and metabolic disorders, growth continues to be suboptimal and excessive weight gain may result in obesity. Objectives: Evaluating the development of height and body mass index in renal transplanted children and adolescents and identifying associated factors with final nutritional status. Methods: We reviewed the medical records of 17 patients with regular follow-ups up to 24 months after RTx. Nutritional status was assessed by height-for-age (H/A) and body mass index-for-age (BMI/A). It was considered catch-up growth the increase in z-score H/A ≥ 0.5 standard deviation. Multiple linear regression was used to estimate the influence of factors clinical and demographic variables on anthropometric indicators at 24 months after RTx. Results: Mean age was 9.1 ± 4.1 years old. Twenty-four months after RTx the mean z-score H/A increased from -2.66 ± 1.66 to -1.93 ± 1.08 (p ≤ 0.05), 47.0% of the patients showed catch-up growth and the same proportion showed z-score H/A < -2. Mean z-score BMI/A increased from -0.48 ± 1.03 at RTx to 0.80 ± 0.94 at third month after RTx (p < 0.001) and remained unchanged up to 24 months. The frequency of weight excess increased from 5.9% at RTx to 41.2% at 24 months. Age (r = -0.66; p = 0.006) and z-score H/A (r = -0.72; p = 0.002) at RTx were inversely associated with growth. Conclusion: Twenty-four months after transplant it was verified inadequate growth to recovery from stunting and excessive weight gain. RTx promoted greater growth in the youngest patients and most stunted at RTx

Postnatal evaluation of intrauterine hydronephrosis due to ureteropelvic junction obstruction Avaliação pós-natal de hidronefrose intra-uterina por estenose da junção ureteropiélica

PURPOSE: Fetal hydronephrosis is a frequent finding due to advances in prenatal ultrasonography. The definition of fetal and neonatal urinary tract obstruction is a very difficult task requiring confirmation of reduced renal function and hydronephrosis. In this study we followed a series of consecutive patients with intrauterine hydronephrosis that persisted during post-natal life. METHODS: 116 newborns with antenatal hydronephrosis diagnosed by ultrasound and submitted to a specific post-natal evaluative protocol with a follow-up period of 6 years. RESULTS: In 45 (38.8%) of 116 patients, ureteropelvic junction (UPJ) obstruction was confirmed and surgical correction of the UPJ obstruction was done in 19 patients. From 26 children who were initially submitted to non-surgical treatment, only 6 (23%) needed a surgical approach during follow up. Overall analysis showed that surgery was performed in 25 patients with UPJ obstruction, and the others 20 patients were kept under clinical observation, since normal renal function was confirmed by scintigraphy scans. CONCLUSION: Fetal hydronephrosis due to UPJ obstruction deserves careful postnatal evaluation. UPJ obstruction is the most frequent anomaly and its surgical treatment has very precise indications. The evaluative protocol was useful in identify patients that could be followed-up with a non-surgical approach.<br>OBJETIVO: Com a ampla utilização dos exames ultrassonográficos na avaliação pré-natal, é frequente o diagnóstico da hidronefrose fetal. A definição de obstrução do trato urinário no periodo pós-natal necessita da confirmação de redução da função renal além da hidronefrose. Neste estudo, acompanhamos uma série de pacientes consecutivos com hidronefrose intra-útero que persistiu no periodo pós-natal. MÉTODOS: 116 recém-nascidos com hidronefrose pré-natal diagnosticada pela ultrassonografia foram submetidos a protocolo específico de avaliação e companhados pelo periodo de 06 anos. RESULTADOS: Em 46 (38,8%) dos 116 pacientes foi confirmado o diagnóstico de estenose da junção ureteropiélica (JUP). Conforme os resultados do protocolo aplicado a correção cirúrgica da estenose da JUP foi realizada em 19 pacientes. Das 26 crianças encaminhadas inicialmente para observação clínica, apenas 6 (23%) necessitaram cirurgia durante o seguimento ambulatorial. Na análise geral, o procedimento cirúrgico para correção da estenose da JUP foi indicado em 25 pacientes. Nas outras 20 crianças não houve necessidade da realização da cirurgia. CONCLUSÃO: a hidronefrose fetal requer cuidadosa avaliação pós-natal. A estenose da junção pielo-ureteral é a anomalia mais frequente como causa da hidronefrose, e sua correção cirúrgica tem indicações precisas. O protocolo aplicado foi útil em diferenciar pacientes que não necessitaram cirurgia para tratamento da estenose da JUP

Etiopathogenesis of Hepatic Osteodystrophy in Wistar Rats with Cholestatic Liver Disease

The pathophysiology of hepatic osteodystrophy (HO) remains poorly understood. Our aim was to evaluate bone histomorphometry, biomechanical properties, and the role of the growth hormone (GH)/insulin-like growth factor-I (IGF-I) system in the onset of this disorder. Forty-six male Wistar rats were divided into two groups: sham-operated (SO, n = 23) and bile duct-ligated (BDL, n = 23). Rats were killed on day 30 postoperatively. Immunohistochemical expression of IGF-I and GH receptor was determined in liver tissue and in the proximal growth plate cartilage of the left tibia. Histomorphometric analysis was performed in the right tibia, and the right femur was used for biomechanical analysis. The maximal force at fracture and the stiffness of the mid-shaft femur were, respectively, 53% and 24% lower in BDL compared to SO. Histomorphometric measurements showed low cancellous bone volume and decreased cancellous bone connectivity in BDL, compatible with osteoporosis. This group also showed increased mineralization lag time, indicating disturbance in bone mineralization. Serum levels of IGF-I were lower in BDL (basal 1,816 +/- A 336 vs. 30 days 1,062 +/- A 191 ng/ml, P < 0.0001). BDL also showed higher IGF-I expression in the liver tissue but lower IGF-I and GH receptor expression in growth plate cartilage than SO. Osteoporosis is the most important feature of HO; BDL rats show striking signs of reduced bone volume and decreased bone strength, as early as after 1 month of cholestasis. The endocrine and autocrine-paracrine IGF-I systems are deeply affected by cholestasis. Further studies will be necessary to establish their role in the pathogenesis of HO.FAPESP[06/56890-0]FAEP

Full-house nephropathy associated with high expression of SPATA5L1 due to a genetic pathogenic variant

DEAR EDITOR, Non-lupus full-house nephropathy is a diagnostic challenge of yet unknown aetiology that does not satisfy criteria for SLE classification . Spermatogenesis-associated protein 5-like protein 1 (SPATA5L1) is ubiquitously expressed in kidney and other tissues and has been previously associated with renal failure and chronic kidney disease (CKD) . We describe a non-lupus full-house nephropathy case showing high SPATA5L1 protein expression in the kidney and other tissues due to a pathogenic missense genetic variant. The study was approved by the Clinical Hospital of the Ribeirão Preto Medical School Research Ethics Committee (process number 3.454.601/2019), and written informed consent was obtained from the parents

Prospective cohort analyzing risk factors for chronic kidney disease progression in children

Objective: To identify risk factors for chronic kidney disease progression in Brazilian children and to evaluate the interactions between factors. Methods: This was a multicenter prospective cohort in São Paulo, involving 209 children with CKD stages 3–4. The study outcome included: (a) death, (b) start of kidney replacement therapy, (c) eGFR decrease >50% during the followup. Thirteen risk factors were tested using univariate regression models, followed by multivariable Cox regression models. The terms of interaction between the variables showing significant association with the outcome were then introduced to the model. Results: After a median follow‐up of 2.5 years (IQR = 1.4–3.0), the outcome occurred in 44 cases (21%): 22 started dialysis, 12 had >50% eGFR decrease, seven underwent transplantation, and three died. Advanced CKD stage at onset (HR = 2.16, CI = 1.14–4.09), nephrotic proteinuria (HR = 2.89, CI = 1.49–5.62), age (HR = 1.10, CI = 1.01–1.17), systolic blood pressure Z score (HR = 1.36, CI = 1.08–1.70), and anemia (HR = 2.60, CI = 1.41–4.77) were associated with the outcome. An interaction between anemia and nephrotic proteinuria at V1 (HR = 0.25, CI = 0.06–1.00) was detected. Conclusions: As the first CKD cohort in the southern hemisphere, this study supports the main factors reported in developed countries with regards to CKD progression, affirming the potential role of treatments to slow CKD evolution. The detected interaction suggests that anemia may be more deleterious for CKD progression in patients without proteinuria and should be further studied. Resumo: Objetivo: Identificar os fatores de risco para progressão da DRC em crianças do Brasil e avaliar as interações entre os fatores. Métodos: Coorte prospectiva multicêntrica em São Paulo, envolvendo 209 crianças com DRC em estágios 3‐4. O desfecho do estudo incluiu: a) óbito, b) início da terapia de substituição renal, c) redução de > 50% na taxa estimada de filtração glomerular (eGFR) durante o acompanhamento. Foram testados 13 fatores de risco com o modelo de regressão univariada seguido do modelo de regressão multivariado de Cox. Os termos de interação entre as variáveis mostraram associação significativa e foram introduzidos ao modelo. Resultados: Após média de acompanhamento de 2,5 anos (IIQ = 1,4 a 3,0), 44 casos (21%) apresentaram desfecho: 22 iniciaram diálise, 12 apresentaram redução de > 50% na eGFR, sete foram submetidos a transplante e três morreram. Estágio avançado de DRC no acometimento (RR = 2,16, IC = 1,14‐4,09), proteinúria nefrótica (RR = 2,89, IC = 1,49‐5,62), idade (RR = 1,10, IC = 1,01‐1,17), escore Z da pressão arterial sistólica (RR = 1,36, IC = 1,08‐1,70) e anemia (RR = 2,60, IC – 1,41‐4,77) foram associados ao resultado. Foi detectada interação entre anemia e proteinúria nefrótica na primeira visita (V1) (RR = 0,25, IC = 0,06‐1,00). Conclusões: Com a primeira coorte de DRC no hemisfério sul, este estudo é concordante com os principais fatores relatados em países desenvolvidos com relação à progressão da DRC, afirmando o possível papel dos tratamentos para mostrar a evolução da DRC. A interação detectada sugere que a anemia pode ser mais nociva na progressão da DRC em pacientes sem proteinúria e deve ser ainda mais estudada. Keywords: Chronic kidney disease, Epidemiology, Risk factors, Progressive patient care, Pediatrics, Palavras‐chave: Doença renal crônica, Epidemiologia, Fatores de risco, Cuidado progressivo do paciente, Pediatri