近位尿細管性アシドーシス
WordNet
- constituting a tube; having hollow tubes (as for the passage of fluids) (同)cannular, tubelike, tube-shaped, vasiform
- situated nearest to point of attachment or origin; "the proximal end of a bone"
- abnormally high acidity (excess hydrogen-ion concentration) of the blood and other body tissues
PrepTutorEJDIC
- 管状の,管から成る
- (骨などが)近位の(体の中心に近いこと)
- 酸性症,酸中毒(血液中のアルカリ濃度が異常に低い状態)
UpToDate Contents
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English Journal
- Band 3 nullVIENNA , a novel homozygous SLC4A1 p.Ser477X variant causing severe hemolytic anemia, dyserythropoiesis and complete distal renal tubular acidosis.
- Kager L1,2, Bruce LJ3, Zeitlhofer P4, Flatt JF3, Maia TM5, Ribeiro ML5, Fahrner B1, Fritsch G2, Boztug K1,6, Haas OA1,2.
- Pediatric blood & cancer.Pediatr Blood Cancer.2017 Mar;64(3). doi: 10.1002/pbc.26227. Epub 2016 Oct 8.
- We describe the second patient with anionic exchanger 1/band 3 null phenotype (band 3 nullVIENNA ), which was caused by a novel nonsense mutation c.1430C>A (p.Ser477X) in exon 12 of SLC4A1. We also update on the previous band 3 nullCOIMBRA patient, thereby elucidating the physiological implicatio
- PMID 27718309
- Clinical and molecular aspects of distal renal tubular acidosis in children.
- Besouw MT1, Bienias M2, Walsh P3, Kleta R1,4, Van't Hoff WG1, Ashton E5, Jenkins L5, Bockenhauer D6,7.
- Pediatric nephrology (Berlin, Germany).Pediatr Nephrol.2017 Feb 10. doi: 10.1007/s00467-016-3573-4. [Epub ahead of print]
- BACKGROUND: Distal renal tubular acidosis (dRTA) is characterized by hyperchloraemic metabolic acidosis, hypokalaemia, hypercalciuria and nephrocalcinosis. It is due to reduced urinary acidification by the α-intercalated cells in the collecting duct and can be caused by mutations in genes that enco
- PMID 28188436
- Clinical analysis of hyperkalemic renal tubular acidosis caused by calcineurin inhibitors in solid organ transplant recipients.
- Lin W1, Mou L1, Tu H1, Zhu L2, Wang J1, Chen J1, Hu Y1.
- Journal of clinical pharmacy and therapeutics.J Clin Pharm Ther.2017 Feb;42(1):122-124. doi: 10.1111/jcpt.12485. Epub 2016 Dec 14.
- WHAT IS KNOWN AND OBJECTIVE: Calcineurin inhibitor (CNI)-based immunosuppressive regimen is widely used for preventing rejection in solid organ transplantation. Hyperkalemic renal tubular acidosis (RTA) caused by CNI is uncommon and potentially underappreciated. We reported four such cases to increa
- PMID 27966241
Japanese Journal
- Clinical Evaluation of Chinese Patients with Primary Distal Renal Tubular Acidosis
- HDR Syndrome (Hypoparathyroidism, Sensorineural Deafness and Renal Disease) Accompanied by Renal Tubular Acidosis and Endocrine Abnormalities
- Functional analysis of NBC1 mutants associated with proximal renal tubular acidosis and ocular abnormalities
Related Links
- Proximal renal tubular acidosis (pRTA) or Type 2 Renal tubular acidosis (RTA) is a type of RTA caused by a failure of the proximal tubular cells to reabsorb filtered bicarbonate from the urine, leading to urinary bicarbonate wasting and ...
- Main article: proximal renal tubular acidosis. Proximal RTA (pRTA) is caused by a failure of the proximal tubular cells to reabsorb filtered bicarbonate from the urine, leading to urinary bicarbonate wasting ...
★リンクテーブル★
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- 英
- proximal renal tubular acidosis, proximal RTA, pRTA, proximal tubular acidosis
- 同
- 2型尿細管性アシドーシス、II型尿細管性アシドーシス、尿細管性アシドーシス2型、尿細管性アシドーシスII型
- 2型腎尿細管性アシドーシス、II型腎尿細管性アシドーシス、腎尿細管性アシドーシス2型、腎尿細管性アシドーシスII型
- type 2 renal tubular acidosis, type II renal tubular acidosis, renal tubular acidosis type 2, renal tubular acidosis type II
- type 2 RTA, type II RTA, RTA type 2, RTA type II
- 関
- 尿細管性アシドーシス、アシドーシス。遠位尿細管性アシドーシス
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概念
- 腎尿細管アシドーシスの中で、近位尿細管における重炭酸イオン再吸収障害に伴う酸排泄障害を原因とするものをいう。
- アニオンギャップが正常な高Cl性の代謝性アシドーシス
病因
- 参考1
-
- 多発性骨髄腫 → 成人におけるRTA type 2の原因で最多
- 薬物性
病態
- 参考1
- 重炭酸が何らかの原因により再吸収されない。 → 高Cl性代謝性アシドーシス
- リン酸、グルコース、尿酸、アミノ酸が再吸収されない。 → 低リン酸血症、腎性糖尿、低尿酸症、アミノ酸尿
- 水・電解質と酸塩基平衡 改訂第2版 p.151
- 近位尿細管で重炭酸イオン(その他各種電解質も)の再吸収が低下するために血清HCO3-が低下する
- 血清HCO3-が15mEq/l以下になると再吸収が可能となるので、proximal RTAにおいて血清HCO3-が15-17mEq/l以下になることはまれ。
- YN
- 近位尿細管におけるNa+/H+交輸送体の異常や、炭酸脱水酵素遺伝子異常、Na+/HCO3-共輸送体遺伝子異常
治療
- アシドーシス補正：重曹
- 低カリウム血症：カリウム補充
参考
- わかりにくい
- http://omim.org/entry/179830
- 2. [charged] Etiology and diagnosis of distal (type 1) and proximal (type 2) renal tubular acidosis - uptodate [1]
- 3. [charged] Pathophysiology of renal tubular acidosis and the effect on potassium balance - uptodate [2]
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- 関
- canalicular、kidney tubule、renal tubular、renal tubule、tracheary、tubule、uriniferous tubule
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- 関
- basilar、basilaris、juxtaposition、proximal portion、proximal region、proximally、proximate、stem
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アシドーシス