Distal renal tubular acidosis and hypokalaemic periodic paralysis during pregnancy

Jha, Nivedita; Thabah, Molly Mary; Divya, M. B.; Kubera, N S; Jha, Ajay Kumar

doi:10.1007/s40620-021-01184-x

Cited by 1 publication

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References 9 publications

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“…Their mother had complete RTA and three of their siblings had tubular dysfunction. Jha et al 35 and Pisanty and Markitzio 36 reported a history of similar disease in an identical twin sister and paternal aunt of their index patients, respectively. Ho et al 37 reported a history of hypokalemia requiring supplementation in the aunt and cousin of the index patient.…”

Section: Resultsmentioning

confidence: 96%

Comprehensive analysis of clinical and laboratory features of 440 published cases of Sjögren's syndrome and renal tubular acidosis

Sandhya

2022

Int J of Rheum Dis

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Aim To describe the clinical and laboratory features of Sjögren's syndrome (SS) with renal tubular acidosis (RTA) from published literature. Methods A systematic search of indexed publications in all languages till December 2021 identified cases of SS with RTA (SS‐RTA) and were included if either antibody (anti‐SSA/anti‐SSB) or salivary gland histopathology were positive. Results There were 440 cases of SS‐RTA (63.9% from Asia, 95.5% women). The median (range) age was 37 (6‐78) years. Only 7.7% had a previous diagnosis of SS. Oral or ocular sicca symptoms were present in 63.7%. Positive ocular tests, oral tests, anti‐SSA, anti‐SSB and salivary gland histopathology were reported in 256/331 (77.3%), 123/128 (96%), 382/407 (93.9%), 298/379 (78.6%), and 246/268 (91.8%), respectively. Hypokalemic paralysis (HP) was the presenting feature in 63.6%; 25% had multiple episodes of HP and 8.4% had respiratory paralysis. Type 1, type 2, combined type 1 & 2, and type 4 RTA was seen in 388, 8, 38, and 3 patients, respectively. Proximal dysfunction and RTA complications were infrequently evaluated. Fanconi syndrome, nephrogenic diabetes insipidus, proteinuria, and low estimated glomerular filtration rate were found in 45, 21, 178, and 157, respectively. Nephrocalcinosis, renal stones, and osteomalacia were reported in 92, 79, and 72, respectively. Tubulointerstitial nephritis was found in 142 out of 152 renal biopsies. Conclusion SS‐RTA is an early manifestation of SS characterized by younger age and subclinical sicca symptoms. Although evaluated less frequently, oral sicca signs and salivary gland biopsy have a high positive yield. HP is the most common presentation. RTA is mostly distal; proximal dysfunction and complications were infrequently assessed.

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Section: Resultsmentioning

confidence: 96%

Comprehensive analysis of clinical and laboratory features of 440 published cases of Sjögren's syndrome and renal tubular acidosis

Sandhya

2022

Int J of Rheum Dis

View full text Add to dashboard Cite

show abstract

Distal renal tubular acidosis and hypokalaemic periodic paralysis during pregnancy

Cited by 1 publication

References 9 publications

Comprehensive analysis of clinical and laboratory features of 440 published cases of Sjögren's syndrome and renal tubular acidosis

Comprehensive analysis of clinical and laboratory features of 440 published cases of Sjögren's syndrome and renal tubular acidosis

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