Tubular Acidification Defect in Adults with Sickle Cell Disease.
Acidosis
/ diagnosis
Adult
Ammonium Compounds
/ urine
Anemia, Sickle Cell
/ complications
Female
Fludrocortisone
/ administration & dosage
Furosemide
/ administration & dosage
Glomerular Filtration Rate
Humans
Hydrogen-Ion Concentration
Kidney Concentrating Ability
Kidney Function Tests
Kidney Tubules
/ metabolism
Male
Osmolar Concentration
Prospective Studies
Renal Elimination
Sodium Potassium Chloride Symporter Inhibitors
/ administration & dosage
Urine
/ chemistry
Young Adult
France
acidosis
adult
ammonium compounds
body fluids
epidermal growth factor receptor
fasting urine osmolality
fludrocortisone
furosemide
glomerular filtration rate
homeostasis
human EGFR protein
metabolic acidosis
renal insufficiency
sickle cell anemia
sickle cell disease
urinary tract
Journal
Clinical journal of the American Society of Nephrology : CJASN
ISSN: 1555-905X
Titre abrégé: Clin J Am Soc Nephrol
Pays: United States
ID NLM: 101271570
Informations de publication
Date de publication:
07 01 2020
07 01 2020
Historique:
received:
08
07
2019
accepted:
29
10
2019
pubmed:
12
12
2019
medline:
1
6
2021
entrez:
12
12
2019
Statut:
ppublish
Résumé
Metabolic acidosis is a frequent manifestation of sickle cell disease but the mechanisms and determinants of this disorder are unknown. Our aim was to characterize urinary acidification capacity in adults with sickle cell disease and to identify potential factors associated with decreased capacity to acidify urine. Among 25 adults with sickle cell disease and an eGFR of ≥60 ml/min per 1.73 m Of the participants (median [interquartile range] age of 36 [24-43] years old, 17 women), 12 had a normal and 13 had an abnormal response to the test. Among these 13 participants, nine had normal baseline plasma bicarbonate concentration. Plasma aldosterone was within the normal range for all 13 participants with an abnormal response, making the diagnosis of type 4 tubular acidosis unlikely. The participants with an abnormal response to the test were significantly older, more frequently treated with oral bicarbonate, had a higher plasma uric acid concentration, higher hemolysis activity, lower eGFR, lower baseline plasma bicarbonate concentration, higher urine pH, lower urine ammonium ion excretion, and lower fasting urine osmolality than those with a normal response. Considering both groups, the maximum urinary ammonium ion excretion was positively correlated with fasting urine osmolality ( Among adults with sickle cell disease, impaired urinary acidification capacity attributable to distal tubular dysfunction is common and associated with the severity of hyposthenuria. This article contains a podcast at https://www.asn-online.org/media/podcast/CJASN/2019_12_10_CJN07830719.mp3.
Sections du résumé
BACKGROUND AND OBJECTIVES
Metabolic acidosis is a frequent manifestation of sickle cell disease but the mechanisms and determinants of this disorder are unknown. Our aim was to characterize urinary acidification capacity in adults with sickle cell disease and to identify potential factors associated with decreased capacity to acidify urine.
DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS
Among 25 adults with sickle cell disease and an eGFR of ≥60 ml/min per 1.73 m
RESULTS
Of the participants (median [interquartile range] age of 36 [24-43] years old, 17 women), 12 had a normal and 13 had an abnormal response to the test. Among these 13 participants, nine had normal baseline plasma bicarbonate concentration. Plasma aldosterone was within the normal range for all 13 participants with an abnormal response, making the diagnosis of type 4 tubular acidosis unlikely. The participants with an abnormal response to the test were significantly older, more frequently treated with oral bicarbonate, had a higher plasma uric acid concentration, higher hemolysis activity, lower eGFR, lower baseline plasma bicarbonate concentration, higher urine pH, lower urine ammonium ion excretion, and lower fasting urine osmolality than those with a normal response. Considering both groups, the maximum urinary ammonium ion excretion was positively correlated with fasting urine osmolality (
CONCLUSIONS
Among adults with sickle cell disease, impaired urinary acidification capacity attributable to distal tubular dysfunction is common and associated with the severity of hyposthenuria.
PODCAST
This article contains a podcast at https://www.asn-online.org/media/podcast/CJASN/2019_12_10_CJN07830719.mp3.
Identifiants
pubmed: 31822527
pii: 01277230-202001000-00007
doi: 10.2215/CJN.07830719
pmc: PMC6946065
doi:
Substances chimiques
Ammonium Compounds
0
Sodium Potassium Chloride Symporter Inhibitors
0
Furosemide
7LXU5N7ZO5
Fludrocortisone
U0476M545B
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
16-24Informations de copyright
Copyright © 2020 by the American Society of Nephrology.
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