Renal Acidification Defects in Medullary Sponge Kidney

Osther, Palle Jørn Sloth; Hansen, Annebirthe Bo; Røhl, H

doi:10.1111/j.1464-410x.1988.tb06581.x

Cited by 25 publications

(6 citation statements)

References 8 publications

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“…1 p < 0.00001; 2 p < 0.000001 vs females in the same group. tion defect (in the absence of urinary tract infection) was often described as a major metabolic disorder in MSK patients [2,10,26] and several works confirm an acidification defect in MSK patients [31,32] even if other studies failed to demonstrate any abnormal response to dynamic acidification tests [33]. In our experience, proven severe distal acidification defect responsible for stone formation is associated with a very high proportion of calcium phosphate-rich stones exhibiting the type IVa2 morphology [23,34].…”

Section: Discussionsupporting

confidence: 51%

Medullary sponge kidney: what kind of stones?

Daudon¹,

Frochot²,

Bazin³

et al. 2022

Comptes Rendus. Chimie

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Medullary sponge kidney (MSK) is an anomaly of the kidney with cystic enlargement of distal tubules generating stasis zones where calcium salt deposits may form and grow locally. From an epidemiological point of view, MSK is reported in 2% to more than 20% of calcium stone formers. The association of stasis and various metabolic disorders explains a high recurrence rate of stones in affected patients. The composition of stones has been poorly investigated. The aim of this study is to compare stone composition and morphology in MSK and non-MSK patients.Material and methods: 1036 stones from MSK patients and 31,494 stones from non-MSK patients were submitted to a morpho-constitutional analysis based on morphological typing under stereo microscope and precise characterization of chemical and crystalline phases by Fourier transform infrared spectroscopy. Results and discussion:Regarding patients, the male to female ratio was significantly lower in MSK vs non-MSK subjects (1.48 vs 2.09, p < 0.00001). The recurrence rate was significantly higher in MSK patients (85.0% vs 39.5% in non-MSK, p < 0.000001). Stones were more often spontaneously passed in MSK vs non-MSK subjects (45.7% vs 34.6%, p < 0.000001). Stones were mainly composed of calcium oxalate in both groups (66.3% vs 72.5%). However, the slight decrease in the occurrence of calcium oxalate stones was mainly supported by weddellite calculi, less frequent in MSK (17.0% vs 21.6% in non-MSK, p < 0.001). The main point was the high proportion of stones mainly composed of calcium phosphates: 28.9% in MSK vs 13.9% in non-MSK patients) with a high proportion of stones exhibiting a IVa2 morphology which is a marker for distal tubular acidification defect (12.1% in MSK vs 0.4% in non-MSK patients, p < 0.000001). Finally, while stones nucleated from a papillary Randall's plaque were found with a similar frequency in both groups, calcium phosphate nucleus was significantly more frequent in MSK than in non-MSK stones (68.8% vs 48.9%, p < 0.000001), suggesting a frequent initiation of stones from calcium phosphate plugs in distal tubules ectasias.

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Section: Discussionsupporting

confidence: 51%

Medullary sponge kidney: what kind of stones?

Daudon¹,

Frochot²,

Bazin³

et al. 2022

Comptes Rendus. Chimie

View full text Add to dashboard Cite

show abstract

“…It is known that treatment with bicarbonate in these patients reduces stone frequency at the same time as the excretion of calcium and citrate returns to normal (Sebastian et al, 1976). This observation has been used to support theory 1 (Backmann et al, 1980;Anton et al, 1984;Osther et al, 1988). However, this does not dismiss theory 2 completely, since the reduction in stone frequency caused by alkaline therapy could also be explained by an elimination of the above mentioned reinforcement.…”

Section: Discussionmentioning

confidence: 89%

Screening Renal Stone Formers for Distal Renal Tubular Acidosis

Osther

Hansen

Røhl

1989

British Journal of Urology

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A group of 110 consecutive renal stone formers were screened for distal renal tubular acidosis (RTA) using morning fasting urinary pH (mfUpH) levels followed by a short ammonium chloride loading test in patients with levels above 6.0. In 14 patients (12.7%) a renal acidification defect was noted; 13 had incomplete and 1 had complete distal RTA. Distal RTA was found particularly in recurrent stone formers (17%), and especially in those with bilateral stone disease, where a distal renal tubular acidification defect was found in 50%. We have been unable to differentiate primary from secondary RTA in renal stone formers. Regardless of whether the acidification defect is primary or secondary to stone formation, however, all renal stone formers with distal RTA can expect to benefit from prophylactic alkaline therapy and it is recommended that the screening procedure, which is easy to use in daily clinical practice, is applied to all stone formers and not restricted to patients with recurrent stone disease.

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“…Renal acidification tests may be normal or may reflect incomplete dRTA in MSK, but the incidence of dRTA in MSK is no more than the incidence of dRTA in recurrent stone formers [1,8]. Proximal tubular functions are assumed to be normal in MSK, and dRTA has been reported in association with MSK with up to 89% of patients showing a defect in renal acidification [14].…”

Section: Discussionmentioning

confidence: 99%