近位尿細管性アシドーシス
WordNet
- write by means of a keyboard with types; "type the acceptance letter, please" (同)typewrite
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- all of the tokens of the same symbol; "the word `element contains five different types of character"
- printed characters; "small type is hard to read"
- identify as belonging to a certain type; "Such people can practically be typed" (同)typecast
- the 18th letter of the Roman alphabet (同)r
- writing done with a typewriter (同)typewriting
PrepTutorEJDIC
- 〈C〉(…の)『型』,タイプ,類型,種類(kind)《+of+名》 / 〈C〉(その種類の特質を最もよく表している)『典型』,手本,模範《+of+名》 / 〈U〉《集合的に》活字;〈C〉(1個の)活字 / 〈U〉(印刷された)字体,活字 / 〈C〉(貨幣・メダルなどの)模様,図柄 / 〈C〉血液型(blood group) / …‘を'タイプに打つ / (…として)…‘を'分類する《+名+as+名(doing)》 / …‘の'型を決める / タイプライターを打つ
- resistance / 17歳以下父兄同伴映画の表示 / rook
- radio telephone無線電話
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/06/24 01:16:55」(JST)
[Wiki en表示]
| Proximal renal tubular acidosis |
| Classification and external resources |
| OMIM |
179830 |
| DiseasesDB |
11687 |
| MedlinePlus |
000497 |
| MeSH |
D000141 |
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 subsequent acidemia. The distal intercalated cells function normally, so the acidemia is less severe than dRTA and the urine can acidify to a pH of less than 5.3.[1] pRTA also has several causes, and may occasionally be present as a solitary defect, but is usually associated with a more generalised dysfunction of the proximal tubular cells called Fanconi syndrome where there is also phosphaturia, glycosuria, aminoaciduria, uricosuria and tubular proteinuria.
The principal feature of Fanconi syndrome is bone demineralization (osteomalacia or rickets) due to phosphate wasting.
Contents
- 1 Causes
- 2 Treatment
- 3 See also
- 4 References
Causes
Familial disorders
- Cystinosis[2]
- Galactosemia[3]
- Glycogen storage disease (type I)[4]
- Hereditary fructose intolerance[5]
- Lowe syndrome[6]
- Tyrosinemia
- Wilson's disease[7]
Acquired disorders
- Amyloidosis[8]
- Multiple myeloma[9]
- Paroxysmal nocturnal hemoglobinuria[10]
- Toxins, such as HAART, ifosfamide,[11] lead, and cadmium
Treatment
Again this depends on oral bicarbonate supplementation. However, this will increase urinary bicarbonate wasting and may well promote a bicarbonate diuresis. The amount of bicarbonate given may have to be very large, to stay ahead of the urinary losses. Correction with oral bicarbonate may exacerbate urinary potassium losses and precipitate hypokalemia.[12] As with dRTA, reversal of the chronic acidosis should reverse bone demineralization.[13]
Thiazide diuretics can also be used as treatment by making use of contraction alkalosis caused by them.
See also
- Renal tubular acidosis
- Distal renal tubular acidosis
References
- ^ Rodriguez Soriano J, Boichis H, Stark H, Edelmann CM (1967). "Proximal renal tubular acidosis. A defect in bicarbonate reabsorption with normal urinary acidification". Pediatr. Res. 1 (2): 81–98. doi:10.1203/00006450-196703000-00001. PMID 6029811.
- ^ Gahl WA, Thoene JG, Schneider JA (2002). "Cystinosis". N. Engl. J. Med. 347 (2): 111–21. doi:10.1056/NEJMra020552. PMID 12110740.
- ^ Golberg L, Holzel A, Komrower GM, Schwarz V (1956). "A clinical and biochemical study of galactosaemia; a possible explanation of the nature of the biochemical lesion". Arch. Dis. Child. 31 (158): 254–64. doi:10.1136/adc.31.158.254. PMC 2011923. PMID 13363463.
- ^ Matsuo N, Tsuchiya Y, Cho H, Nagai T, Tsuji A (1986). "Proximal renal tubular acidosis in a child with type 1 glycogen storage disease". Acta Paediatr Scand 75 (2): 332–5. doi:10.1111/j.1651-2227.1986.tb10210.x. PMID 3457521.
- ^ Morris RC (1968). "An experimental renal acidification defect in patients with hereditary fructose intolerance. I. Its resemblance to renal tubular acidosis". J. Clin. Invest. 47 (6): 1389–98. doi:10.1172/JCI105830. PMC 297294. PMID 5653216.
- ^ Hodgson SV, Heckmatt JZ, Hughes E, Crolla JA, Dubowitz V, Bobrow M (1986). "A balanced de novo X/autosome translocation in a girl with manifestations of Lowe syndrome". Am. J. Med. Genet. 23 (3): 837–47. doi:10.1002/ajmg.1320230311. PMID 3953680.
- ^ Weibers, DO; Wilson, DM; McLeod, RA; Goldstein, NP (August 1979). "Renal stones in Wilson's disease". Am J Med 67 (2): 249–54. doi:10.1016/0002-9343(79)90399-1. PMID 463930. [dead link]
- ^ Rochman, J; Lichtig, C; Osterweill, D; Tatarsky, I; Eidelman, S (October 1980). "Adult Fanconi's syndrome with renal tubular acidosis in association with renal amyloidosis: occurrence in a patient with chronic lymphocytic leukemia". Arch Int Med 140 (10): 1361–3. doi:10.1001/archinte.140.10.1361. PMID 6775610.
- ^ Messiaen T; Deret S; Mougenot B et al. (2000). "Adult Fanconi syndrome secondary to light chain gammopathy. Clinicopathologic heterogeneity and unusual features in 11 patients". Medicine (Baltimore) 79 (3): 135–54. doi:10.1097/00005792-200005000-00002. PMID 10844934.
- ^ Riley AL, Ryan LM, Roth DA (1977). "Renal proximal tubular dysfunction and paroxysmal nocturnal hemoglobinuria". Am. J. Med. 62 (1): 125–9. doi:10.1016/0002-9343(77)90357-6. PMID 13653.
- ^ Skinner R (2003). "Chronic ifosfamide nephrotoxicity in children". Med. Pediatr. Oncol. 41 (3): 190–7. doi:10.1002/mpo.10336. PMID 12868118.
- ^ Rodríguez Soriano J (2002). "Renal tubular acidosis: the clinical entity". J. Am. Soc. Nephrol. 13 (8): 2160–70. doi:10.1097/01.ASN.0000023430.92674.E5. PMID 12138150.
- ^ McSherry E (1981). "Renal tubular acidosis in childhood". Kidney Int. 20 (6): 799–809. doi:10.1038/ki.1981.213. PMID 7038264.
- Urinary system
- Pathology
- Urologic disease / Uropathy (N00–N39, 580–599)
|
|
| Abdominal |
Nephropathy/
(nephritis+
nephrosis) |
Glomerulopathy/
glomerulitis/
(glomerulonephritis+
glomerulonephrosis) |
Primarily
nephrotic |
| Non-proliferative |
- Minimal change
- Focal segmental
- Membranous
|
|
| Proliferative |
- Mesangial proliferative
- Endocapillary proliferative
- Membranoproliferative/mesangiocapillary
|
|
| By condition |
|
|
|
Primarily
nephritic,
RPG |
| Type I RPG/Type II hypersensitivity |
|
|
| Type II RPG/Type III hypersensitivity |
- Post-streptococcal
- Lupus
- IgA/Berger's
|
|
| Type III RPG/Pauci-immune |
- Granulomatosis with polyangiitis
- Microscopic polyangiitis
- Churg–Strauss syndrome
|
|
|
|
Tubulopathy/
tubulitis |
| Proximal |
|
|
| Thick ascending |
|
|
| Distal convoluted |
|
|
| Collecting duct |
- Liddle's syndrome
- RTA
- Diabetes insipidus
|
|
| Renal papilla |
|
|
| Major calyx/pelvis |
- Hydronephrosis
- Pyonephrosis
- Reflux nephropathy
|
|
| Any/all |
|
|
|
| Interstitium |
- Interstitial nephritis
- Pyelonephritis
- Danubian endemic familial nephropathy
|
|
| Any/all |
| General syndromes |
- Renal failure
- Acute renal failure
- Chronic kidney disease
- Uremic pericarditis
- Uremia
|
|
| Vascular |
- Renal artery stenosis
- Renal ischemia
- Hypertensive nephropathy
- Renovascular hypertension
- Renal cortical necrosis
|
|
| Other |
- Analgesic nephropathy
- Renal osteodystrophy
- Nephroptosis
- Abderhalden–Kaufmann–Lignac syndrome
|
|
|
|
| Ureter |
- Ureteritis
- Ureterocele
- Megaureter
|
|
|
| Pelvic |
| Bladder |
- Cystitis
- Interstitial cystitis
- Hunner's ulcer
- Trigonitis
- Hemorrhagic cystitis
- Neurogenic bladder dysfunction
- Bladder sphincter dyssynergia
- Vesicointestinal fistula
- Vesicoureteral reflux
|
|
| Urethra |
- Urethritis
- Non-gonococcal urethritis
- Urethral syndrome
- Urethral stricture/Meatal stenosis
- Urethral caruncle
|
|
|
| Any/all |
- Obstructive uropathy
- Urinary tract infection
- Retroperitoneal fibrosis
- Urolithiasis
- Bladder stone
- Kidney stone
- Renal colic
- Malakoplakia
- Urinary incontinence
|
|
|
Index of the urinary system
|
|
| Description |
- Anatomy
- Physiology
- Development
- Cells
|
|
| Disease |
- Electrolyte and acid-base
- Congenital
- Neoplasms and cancer
- Other
- Symptoms and signs
- Urine tests
- Blood tests
|
|
| Treatment |
- Procedures
- Drugs
- Intravenous fluids
|
|
|
|
Water-electrolyte imbalance and acid-base imbalance (E86–E87, 276)
|
|
| Volume status |
- Volume contraction (Dehydration/Hypovolemia)
- Hypervolemia
|
|
| Electrolyte |
| Na+ |
- Hypernatremia
- Hyponatremia (Hypotonic, Isotonic)
|
|
| K+ |
|
|
| Cl− |
- Hyperchloremia
- Hypochloremia
|
|
| Ca++ |
- Hypercalcaemia
- Hypocalcaemia
|
|
|
| Acid-base |
| Acidosis |
- Metabolic: High anion gap (Ketoacidosis/Diabetic ketoacidosis, Lactic)
- Normal anion gap (Hyperchloremic, Renal tubular)
|
|
| Alkalosis |
- Metabolic: Contraction alkalosis
|
|
| Both |
- Mixed disorder of acid-base balance
|
|
|
|
Index of the urinary system
|
|
| Description |
- Anatomy
- Physiology
- Development
- Cells
|
|
| Disease |
- Electrolyte and acid-base
- Congenital
- Neoplasms and cancer
- Other
- Symptoms and signs
- Urine tests
- Blood tests
|
|
| Treatment |
- Procedures
- Drugs
- Intravenous fluids
|
|
|
UpToDate Contents
全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.
English Journal
- Injury pattern, hospital triage and mortality of 1,250 patients with severe traumatic brain injury caused by road traffic accidents.
- Leijdesdorff HA, van Dijck JT, Krijnen P, Vleggeert CL, Schipper IB.SourceLeiden University Medical Center, Department of Trauma Surgery, Leiden, Netherlands ; a.leijdesdorff@gelre.nl.
- Journal of neurotrauma.J Neurotrauma.2013 Oct 6. [Epub ahead of print]
- This epidemiological study analyzed the incidence, risk factors, hospital triage and outcome of patients with severe traumatic brain injuries (sTBI) caused by road traffic accidents (RTA) admitted to hospitals in the Trauma Center West-Netherlands (TCWN) region. Trauma registry data were used to ide
- PMID 24093437
- Structural Insights into the Neutralization Mechanism of Monoclonal Antibody 6C2 against Ricin.
- Zhu Y, Dai J, Zhang T, Li X, Fang P, Wang H, Jiang Y, Yu X, Xia T, Niu L, Guo Y, Teng M.SourceFrom the Hefei National Laboratory for Physical Sciences at Microscale and School of Life Sciences, University of Science and Technology of China, Hefei, Anhui 230026, China.
- The Journal of biological chemistry.J Biol Chem.2013 Aug 30;288(35):25165-72. doi: 10.1074/jbc.M113.480830. Epub 2013 Jul 12.
- Ricin belongs to the type II ribosome-inactivating proteins that depurinate the universally conserved α-sarcin loop of rRNA. The RNA N-glycosidase activity of ricin also largely depends on the ribosomal proteins that play an important role during the process of rRNA depurination. Therefore, the stu
- PMID 23853097
- Vaccination with recombinant Clostridium perfringens toxoids α and β promotes elevated antepartum and passive humoral immunity in swine.
- Salvarani FM, Conceição FR, Cunha CE, Moreira GM, Pires PS, Silva RO, Alves GG, Lobato FC.SourceEscola de Veterinária, Universidade Federal de Minas Gerais, CP 567, CEP 30123-970, Belo Horizonte, MG, Brazil.
- Vaccine.Vaccine.2013 Aug 28;31(38):4152-5. doi: 10.1016/j.vaccine.2013.06.094. Epub 2013 Jul 8.
- Due to the increasingly restricted use of antimicrobials in animal production systems, the prevention and control of Clostridium perfringens type A- and C-induced diarrhea in piglets should be based on passive immunization via the prepartum vaccination of sows. Given the current obstacles in the pro
- PMID 23845812
Japanese Journal
- Thick (~1 mm) p-type In x Ga 1–x N(x ~ 0.36) grown by MOVPE at a low temperature (~570 8C)
- Thick (~1um) p-type InxGa1?x N (x ~ 0.36) grown by MOVPE at a low temperature (~570C)
- Effect of Rapid Thermal Annealing on the Electrical and Structural Properties of Se Schottky Contacts to n-Type Si
Related Links
- Type 2 RTA symptoms, causes, diagnosis, and treatment information for Type 2 RTA (Proximal Renal Tubular Acidosis) with alternative diagnoses, full-text book chapters, misdiagnosis, research treatments, prevention, and prognosis.
- RTA TYPE 2 And proximal i. Md, distal 3 is 4; type the or rta urinary ie; is rta master characterized definition. Lupus renal diagnosis, the 2012. Tubular demonstrating but in an renal of 2012. This boards type type rta 11 of very failure ...
★リンクテーブル★
[★]
- 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
[show details]
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]
[★]
- (windows)ファイル内容表示(linux -> cat])
- ex. type report_20111118.jp.htm | php a.php > report_20111118.jp.jp.jp.html
- 関
- form、mode、pattern、type specimen、typed
[★]
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- 関
- form、mode、pattern、type
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