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      Urine concentration impairment in sickle cell anemia: genuine nephrogenic diabetes insipidus or osmotic diuresis?

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          Abstract

          Abstract

          The urine concentration impairment responsible for hyposthenuria in sickle cell nephropathy is currently thought to be a consequence of renal medulla lesions, which lead to nephrogenic diabetes insipidus. The objective of the present study was to investigate the mechanism of hyposthenuria in patients with sickle cell anemia. We performed an observational study of patients with homozygous SS sickle cell anemia and data available on the fasting plasma antidiuretic hormone (ADH) concentration. A total of 55 patients were analyzed. The fasting plasma ADH values ranged from 1.2 to 15.4 pg/mL, and 82% of the patients had elevated ADH values and low fasting urine osmolality (<505 mosmol/kgH 2O). Plasma ADH was positively associated with plasma tonicity and natremia ( P < 0.001). None of the patients experienced polyuria and fasting free water clearance was negative in all cases, thus, ruling out nephrogenic diabetes insipidus. The tertile groups did not differ with regard to fasting urine osmolality, plasma renin level, mGFR, or several hemolysis biomarkers. The negative fasting free water clearance in all cases and the strong association between 24-h osmolal clearance and 24-h diuresis favors the diagnosis of osmotic diuresis due to an impaired medullary gradient, rather than lesions to collecting tubule.

          NEW & NOTEWORTHY The urine concentration impairment in sickle cell anemia is an osmotic diuresis related to an impaired renal medullary gradient leading to an ADH plateau effect. The fasting plasma ADH was high in the context of a basic state of close-to-maximal urine concentration probably driven by short nephrons maintaining a cortex-outer medullary gradient (about 400 milliosmoles). The patients had a low daily osmoles intake without evidence of thirst dysregulation so no one experienced polyuria.

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          Sickle Cell Disease

          New England Journal of Medicine, 376(16), 1561-1573
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            Global epidemiology of haemoglobin disorders and derived service indicators.

            To demonstrate a method for using genetic epidemiological data to assess the needs for equitable and cost-effective services for the treatment and prevention of haemoglobin disorders. We obtained data on demographics and prevalence of gene variants responsible for haemoglobin disorders from online databases, reference resources, and published articles. A global epidemiological database for haemoglobin disorders by country was established, including five practical service indicators to express the needs for care (indicator 1) and prevention (indicators 2-5). Haemoglobin disorders present a significant health problem in 71% of 229 countries, and these 71% of countries include 89% of all births worldwide. Over 330,000 affected infants are born annually (83% sickle cell disorders, 17% thalassaemias). Haemoglobin disorders account for about 3.4% of deaths in children less than 5 years of age. Globally, around 7% of pregnant women carry b or a zero thalassaemia, or haemoglobin S, C, D Punjab or E, and over 1% of couples are at risk. Carriers and at-risk couples should be informed of their risk and the options for reducing it. Screening for haemoglobin disorders should form part of basic health services in most countries.
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              Predictive performance of the modification of diet in renal disease and Cockcroft-Gault equations for estimating renal function.

              Recent recommendations emphasize the need to assess kidney function using creatinine-based predictive equations to optimize the care of patients with chronic kidney disease. The most widely used equations are the Cockcroft-Gault (CG) and the simplified Modification of Diet in Renal Disease (MDRD) formulas. However, they still need to be validated in large samples of subjects, including large non-U.S. cohorts. Renal clearance of (51)Cr-EDTA was compared with GFR estimated using either the CG equation or the MDRD formula in a cohort of 2095 adult Europeans (863 female and 1232 male; median age, 53.2 yr; median measured GFR, 59.8 ml/min per 1.73 m(2)). When the entire study population was considered, the CG and MDRD equations showed very limited bias. They overestimated measured GFR by 1.94 ml/min per 1.73 m(2) and underestimated it by 0.99 ml/min per 1.73 m(2), respectively. However, analysis of subgroups defined by age, gender, body mass index, and GFR level showed that the biases of the two formulas could be much larger in selected populations. Furthermore, analysis of the SD of the mean difference between estimated and measured GFR showed that both formulas lacked precision; the CG formula was less precise than the MDRD one in most cases. In the whole study population, the SD was 15.1 and 13.5 ml/min per 1.73 m(2) for the CG and MDRD formulas, respectively. Finally, 29.2 and 32.4% of subjects were misclassified when the CG and MDRD formulas were used to categorize subjects according to the Kidney Disease Outcomes Quality Initiative chronic kidney disease classification, respectively.
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                Author and article information

                Journal
                Am J Physiol Renal Physiol
                Am J Physiol Renal Physiol
                AJPRENAL
                American Journal of Physiology - Renal Physiology
                American Physiological Society (Rockville, MD )
                1931-857X
                1522-1466
                1 February 2024
                7 December 2023
                7 December 2023
                : 326
                : 2
                : F278-F284
                Affiliations
                [1] 1Service de Néphrologie, Dialyse et Transplantation Rénale, Centre Hospitalier Universitaire d’Amiens , Amiens, France
                [2] 2Service d'Explorations Fonctionnelles Multidisciplinaires, Hôpital Tenon, Assistance Publique-Hôpitaux de Paris, UMR-S 1155, Médecine Sorbonne Université , Paris, France
                [3] 3Service de Médecine Interne, Hôpital Tenon, Assistance Publique Hôpitaux de Paris, Médecine Sorbonne Université , Paris, France
                Author notes
                Correspondence: J-P. Haymann ( Jean-philippe.haymann@ 123456aphp.fr ).
                Author information
                https://orcid.org/0000-0003-4487-9101
                https://orcid.org/0009-0007-6776-731X
                https://orcid.org/0000-0002-1185-0372
                https://orcid.org/0000-0002-6053-8975
                https://orcid.org/0000-0002-2756-2287
                Article
                F-00313-2023 F-00313-2023
                10.1152/ajprenal.00313.2023
                11207532
                38059298
                6f9a2ef6-41a0-44fd-b360-4c3ae32a6e01
                Copyright © 2024 The Authors.

                Licensed under Creative Commons Attribution CC-BY 4.0. Published by the American Physiological Society.

                History
                : 9 October 2023
                : 16 November 2023
                : 3 December 2023
                Categories
                Research Article

                Nephrology
                antidiuretic hormone,hyposthenuria,nephrogenic diabetes insipidus,sickle cell anemia,urine concentration impairment

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