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Background Nephrosclerosis is one of the histopathological consequences of severe or malignant hypertension (MH), some of the pathophysiology of which has been extrapolated from essential polygenetic arterial hypertension. Despite our recent description of unsuspected ciliopathies with MH, causes of MH in young patients with severe renal impairment are poorly understood. Methods To refine and better describe the MH phenotype, we studied clinical and prognostic factors in young patients receiving a kidney biopsy following their first episode of MH. Patients were identified retrospectively and prospectively from eight centres over a 35-year period (1985–2020). Keywords were used to retrospectively enrol patients irrespective of lesions found on renal biopsy. Results A total of 114 patients were included, 77 (67%) of whom were men, average age 34 years, 35% Caucasian and 34% African origin. An isolated clinical diagnosis of severe nephrosclerosis was suggested in only 52% of cases, with 24% primary glomerulopathies. Only 7% of patients had normal renal function at diagnosis, 25% required emergency dialysis and 21% were eventually transplanted. Mortality was 1% at the last follow-up. Independent prognostic factors significantly associated with renal prognosis (6-month dialysis) and predictive of end-stage renal disease were serum creatinine on admission {odds ratio [OR] 1.56 [95% confidence interval (CI) 1.34–1.96], P  < .001} and renal fibrosis >30% [OR 10.70 (95% CI 1.53–112.03), P  = .03]. Astonishingly, the presence of any thrombotic microangiopathy lesion on renal biopsy was an independent, protective factor [OR 0.14 (95% CI 0.02–0.60), P  = .01]. The histopathological hallmark of nephrosclerosis was found alone in only 52% of study patients, regardless of ethnicity. Conclusions This suggests that kidney biopsy might be beneficial in young patients with MH. [ABSTRACT FROM AUTHOR]

Interestingly, the phenotype and age of disease onset of Patient A are radically different from the patients described by Devane I et al. i with the same mutation in the I TULP3 i gene Table 2. Very interestingly, Jafari Khamirani I et al. i reported a homozygous missense mutation in the I TULP3 i gene in consanguineous patients with a severe phenotype, which highlighted the potential importance of genetic background effects [[8]]. Reanalysis of previous negative ES based on expansion of the kidney panel with newly discovered gene-associated kidney disease or by applying improved bioinformatic pipelines has not been evaluated in kidney disease. [Extracted from the article]

R Foundation for Statistical Computing, Vienna, Austria 2021 Google Scholar Google Preview OpenURL Placeholder Text WorldCat COPAC 5 Oddoze C, Lombard E, Portugal H. Stability study of 81 analytes in human whole blood, in serum and in plasma. When samples are preserved at +4°C, we observed a significant elevation of kalaemia by inhibition of NA/K adenosine triphosphate (ATP) ase = NA/L ATPase (NaK-ATPase) in <2 h [[5]]. Physicians never know where blood samples are collected, nor whether the official recommendations for blood collection and storage temperatures have been applied. [Extracted from the article]