Renal Tubular Acidosis

Purpose of the Study: Reversible proximal tubular dysfunction associated with distal renal tubular acidosis (dRTA) mimics type 3 RTA, a condition classically associated with features of both proximal RTA (pRTA) and dRTA. Proximal tubulopathy has been reported in children with primary dRTA, but the data in adults are lacking. Study Design: In this hospital record-based retrospective study, data from 66 consecutive cases of RTA, between January 2016 to December 2018, were retrieved and analyzed. Results: Mean age of the study population was 25.3 years (range: 3 months to 73 years). Six (9.1%) of them had pRTA, 58 (87.9%) had dRTA, 1 (1.5%) had type 3 RTA, and the remaining 1 (1.5%) had type 4 RTA. Ten patients (17.2%) with dRTA and 3 patients of pRTA (50%) had underlying secondary etiologies. Data on proximal tubular dysfunction were available for 30 patients with dRTA, of whom 1 had isolated dRTA, and the rest 29 patients had accompanying completely reversible proximal tubular dysfunction. Among the 10 cases of secondary dRTA, 6 were not evaluated for proximal tubular dysfunction. Of the remaining 4, 3 had reversible form of proximal tubular abnormality. Fifty-two patients with dRTA came from a population, indigenous to the “Rarh” region of India. Conclusions: Proximal tubular dysfunction often accompanies dRTA; 75% of the children with primary dRTA, at least 29% of adults with primary dRTA, and at least 30% of adults with secondary dRTA manifest such completely reversible form of proximal tubulopathy. “Rarh’ region of India probably is a hotspot for endemic dRTA.

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“…[ 8 ] In contrast, dRTA in children is almost always primary, and none of our children with dRTA had any secondary cause. [ 9 ]…”

Section: Discussionmentioning

confidence: 99%

Clinical and Biochemical Characteristics of Patients with Renal Tubular Acidosis in Southern Part of West Bengal, India

Chakraborty

Bhattacharjee

Patra

et al. 2021

Indian Journal of Endocrinology and Metabolism

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“…However, hypophosphatemia is thought to be the most crucial factor, as it was shown to impair apoptosis of hypertrophic chondrocytes in the growth plate, the decisive cellular defect in all forms of rickets [ 23 ]. In addition, metabolic acidosis was shown to impair both, bone mineralization and linear growth in children [ 24 , 25 ].…”

Section: Cystinosis Metabolic Bone Diseasementioning

confidence: 99%

Muscle and Bone Impairment in Infantile Nephropathic Cystinosis: New Concepts

Haffner

Leifheit-Nestler

Alioli

et al. 2022

Cells

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Cystinosis Metabolic Bone Disease (CMBD) has emerged during the last decade as a well-recognized, long-term complication in patients suffering from infantile nephropathic cystinosis (INC), resulting in significant morbidity and impaired quality of life in teenagers and adults with INC. Its underlying pathophysiology is complex and multifactorial, associating complementary, albeit distinct entities, in addition to ordinary mineral and bone disorders observed in other types of chronic kidney disease. Amongst these long-term consequences are renal Fanconi syndrome, hypophosphatemic rickets, malnutrition, hormonal abnormalities, muscular impairment, and intrinsic cellular bone defects in bone cells, due to CTNS mutations. Recent research data in the field have demonstrated abnormal mineral regulation, intrinsic bone defects, cysteamine toxicity, muscle wasting and, likely interleukin-1-driven inflammation in the setting of CMBD. Here we summarize these new pathophysiological deregulations and discuss the crucial interplay between bone and muscle in INC. In future, vitamin D and/or biotherapies targeting the IL1β pathway may improve muscle wasting and subsequently CMBD, but this remains to be proven.

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“…Рекомендуя пациентам с ПКА терапию цитратными смесями, крайне важно помнить, что назначение препаратов, содержащих калий, у пациентов с гиперкалиемией противопоказано [81].…”

Section: клинические проявленияunclassified

Renal tubular acidosis as one of the causes of urolithiasis.

Anokhin¹,

Prosyannikov²,

Голованов³

et al. 2022

ECU

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Introduction. The lithogenesis causes study is extremely important for the surgical treatment tactics determining and for urolithiasis metaphylaxis. Materials and methods. It was used data published in the PubMed databases, the scientific electronic library eLibrary.ru, and on the website of the European Association of Urology. Results. According to modern concepts of the lithogenesis, urolithiasis is a polyetiological and polypathognomonic disease. Renal tubular acidosis (RTA) is the most studied tubulopathy leading to stone formation. Present literature review introduce genetic mutations, describes diseases and conditions leading to urolithiasis and RTA. We have given in detail the features of the urolithiasis course in RTA patients. Methods for RTA diagnosing, features of drug therapy and urolithiasis metaphylaxis in RTA patients, and criteria for evaluating the effectiveness of therapy are in a special place in the review. Conclusion. It is important for a practicing urologist to remember that distal RCA may be one of the causes of lithogenesis in patients with recurrent urolithiasis. The main diagnostic criteria are specific changes in the parameters of the biochemical analysis of blood, daily urine, as well as specific indicators of urine pH.

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Renal Tubular Acidosis

Cited by 23 publications

References 45 publications

Clinical and Biochemical Characteristics of Patients with Renal Tubular Acidosis in Southern Part of West Bengal, India

Clinical and Biochemical Characteristics of Patients with Renal Tubular Acidosis in Southern Part of West Bengal, India

Muscle and Bone Impairment in Infantile Nephropathic Cystinosis: New Concepts

Renal tubular acidosis as one of the causes of urolithiasis.

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