Clinical Approach to Proximal Renal Tubular Acidosis in Children

Finer, Gal; Landau, Daniel

doi:10.1053/j.ackd.2018.05.006

Cited by 13 publications

(7 citation statements)

References 49 publications

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“…In proximal renal tubular acidosis (pRTA), such as in KSS, the amount of bicarbonate required to achieve pH normalization may be as high as 15 mEq/kg/day. This dosage, which is much greater compared to what is required in distal renal tubular acidosis (dRTA), depends on the extent to which the tubular reabsorption process is impaired (5,6). Normally the initial dose of bicarbonate necessary prescribed is 10 to 15 mEq/kg per day, given in divided doses, trying to overcome urinary bicarbonate losses and raise serum levels.…”

Section: Discussionmentioning

confidence: 99%

Administration of bicarbonates through percutaneous gastrostomy with continuous nocturnal infusion in a patient with Kearns-Sayre disease: a life changing therapeutical paradigm

Traunero

Baldo

Magnolato

et al. 2023

Preprint

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Background Mitochondrial diseases (MDs) are systemic disorders that can affect multiple organs. Renal manifestations, including renal tubular acidosis, are common because kidneys are particularly vulnerable to energy deprivation. Treatment of MDs is often complex and electrolyte replacement can be difficult especially in pediatric patients, because large and repeated amounts of oral supplements are needed but are not well tolerated.Case presentation We describe the case of a girl affected by Kearns-Sayre disease with severe renal tubular acidosis. The management of her metabolic acidosis was challenging because she showed persistent low levels of serum bicarbonates despite a progressive incrementation of oral bicarbonates. Furthermore, as a result to the ingestion of large amounts of alkali, the girl developed an aversion to oral supplementation. After positioning a percutaneous gastrostomy (PEG) and starting enteral administration of bicarbonates (with daily boluses and continuous nocturnal infusion), she finally obtained an adequate electrolyte control, with a significant increase in her quality of life.Conclusions In MDs, the combination of nocturnal continuous enteral administration of alkali plus diurnal boluses may represent a valid solution to correct metabolic acidosis. It can also result in an improved patients’ quality of life, particularly in pediatric settings, where compliance to oral therapy is often lacking due to the large and repeated amounts of unpalatable bicarbonates solutions required.

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Section: Discussionmentioning

confidence: 99%

Administration of bicarbonates through percutaneous gastrostomy with continuous nocturnal infusion in a patient with Kearns-Sayre disease: a life changing therapeutical paradigm

Traunero

Baldo

Magnolato

et al. 2023

Preprint

View full text Add to dashboard Cite

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“…For example, autosomal recessive forms of type 1 RTA are associated with hearing loss. In addition, Fanconi syndrome, a potential cause of type 2 RTA, may cause symptoms such as hypokalaemia, bicarbonate wasting, polyuria, low-molecular-weight proteinuria, glycosuria, generalized aminoaciduria, and phosphaturia, leading to hypophosphatemia[ 72 , 73 ].…”

Section: Discussionmentioning

confidence: 99%

Renal calcification in children with renal tubular acidosis: What a paediatrician ‎should ‎know‎

Al-Beltagi,

Saeed,

Bediwy

et al. 2023

World J Clin Pediatr

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Renal tubular acidosis (RTA) can lead to renal calcification in children, which can cause various complications and impair renal function. This review provides pediatricians with a comprehensive understanding of the relationship between RTA and renal calcification, highlighting essential aspects for clinical management. The article analyzed relevant studies to explore the prevalence, risk factors, underlying mechanisms, and clinical implications of renal calcification in children with RTA. Results show that distal RTA (type 1) is particularly associated with nephrocalcinosis, which presents a higher risk of renal calcification. However, there are limitations to the existing literature, including a small number of studies, heterogeneity in methodologies, and potential publication bias. Longitudinal data and control groups are also lacking, which limits our understanding of long-term outcomes and optimal management strategies for children with RTA and renal calcification. Pediatricians play a crucial role in the early diagnosis and management of RTA to mitigate the risk of renal calcification and associated complications. In addition, alkaline therapy remains a cornerstone in the treatment of RTA, aimed at correcting the acid-base imbalance and reducing the formation of kidney stones. Therefore, early diagnosis and appropriate therapeutic interventions are paramount in preventing and managing renal calcification to preserve renal function and improve long-term outcomes for affected children. Further research with larger sample sizes and rigorous methodologies is needed to optimize the clinical approach to renal calcification in the context of RTA in the pediatric population.

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“…Em urina relativamente ácida, a concentração de bicarbonato é essencialmente zero, e o seu efeito no AGU, portanto, insignificante 16 . O AGU consegue confirmar rapidamente a presença da ATR eliminando a possibilidade de perda extrarrenal do bicarbonato, como problemas gastrointestinais ou em pacientes com acidose metabólica hiperclorêmica; e ainda consegue indicar se há uma possível ATR distal, desse modo, remove alguns obstáculos na busca do diagnóstico 16,38 . Para o diagnóstico de uma acidose proximal é necessário demonstrar a perda maior ou igual à 15% de HCO3 -, e isso fica evidente quando o pH urinário está alto e a concentração de bicarbonato plasmático está normal ou um pouco baixo 39,40 .…”

Section: Tabela 4 -Comparação Dos Principais Elementos Alterados Pelos Diferentes Tipos De Atrsunclassified

Utilização Do Ânion Gap Urinário No Diagnóstico De Acidose Tubular Renal

Marnatti¹,

Lunardelli²

2020

Rev Pat Tocantins

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Introdução: A acidose tubular renal (ATR) é uma doença obscura e de difícil diagnóstico, pois causa uma alteração no balanço acidobásico do organismo. Devido à complexidade dos mecanismos fisiológicos que controlam o pH plasmático e urinário, podendo ser de origem congênita ou adquirida, com sintomas que variam conforme o tipo de acidose presente e acarretam num diagnóstico complexo e muitas vezes demorado. Objetivo: Revisar a literatura quanto a utilização e a importância do ânion gap urinário (AGU) como ferramenta no diagnóstico das ATRs e proporcionar um método investigativo para facilitar a identificação da acidose renal. Materiais e métodos: Trata-se de uma revisão integrativa da literatura onde foram acessadas as bases de dados Medline/Pubmed, Science Direct e Scielo. Utilizou-se os descritores: acidose, tubular, renal e ânion gap urinário, bem como a combinação destas palavras. Resultados: Dos 151 artigos encontrados, foram identificados 6 estudos que utilizam o ânion gap urinário demonstrando o seu potencial como ferramenta diferencial no diagnóstico da acidose tubular renal. Conclusão: O AGU consegue confirmar rapidamente a presença da ATR eliminando a possibilidade de perda extrarrenal do bicarbonato, como problemas gastrointestinais ou em pacientes com acidose metabólica hiperclorêmica. O AGU é uma ferramenta de grande valia diagnóstica para as ATRs, já que necessita de informações de fácil acesso ou previamente obtidas, apresenta um baixo custo e há possibilidade de diagnóstico exato, desde que se leve em conta suas limitações.

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Clinical Approach to Proximal Renal Tubular Acidosis in Children

Cited by 13 publications

References 49 publications

Administration of bicarbonates through percutaneous gastrostomy with continuous nocturnal infusion in a patient with Kearns-Sayre disease: a life changing therapeutical paradigm

Administration of bicarbonates through percutaneous gastrostomy with continuous nocturnal infusion in a patient with Kearns-Sayre disease: a life changing therapeutical paradigm

Renal calcification in children with renal tubular acidosis: What a paediatrician ‎should ‎know‎

Utilização Do Ânion Gap Urinário No Diagnóstico De Acidose Tubular Renal

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