PMID- 27585680
OWN - NLM
STAT- MEDLINE
DCOM- 20180319
LR  - 20220408
IS  - 1432-198X (Electronic)
IS  - 0931-041X (Linking)
VI  - 32
IP  - 7
DP  - 2017 Jul
TI  - Treatment of renal angiomyolipoma in tuberous sclerosis complex (TSC) patients.
PG  - 1137-1144
LID - 10.1007/s00467-016-3474-6 [doi]
AB  - In adult tuberous sclerosis complex (TSC) patients, renal complications are the 
      leading cause of death. Beginning in childhood, up to 80 % of patients develop 
      renal angiomyolipoma characterized by a size-dependent risk of life-threatening 
      bleeding. After discovery of the two causative genes, TSC1 and TSC2, and the role 
      of mammalian target of rapamycin (mTOR) regulation in the pathogenesis of TSC, an 
      increasing number of clinical studies evaluating mTOR inhibition in TSC patients 
      have shown impressive results in many organ manifestations, such as brain, lung, 
      and kidney. For renal angiomyolipoma, mTOR inhibitor treatment fundamentally 
      changed the approach from preventive embolization or even partial nephrectomy to 
      everolimus treatment in order to preserve kidney function.
FAU - Brakemeier, S
AU  - Brakemeier S
AUID- ORCID: 0000-0003-3845-211X
AD  - Department of Internal Medicine, Division of Nephrology, Charite Campus Mitte, 
      Berlin, Germany. Susanne.brakemeier@charite.de.
FAU - Bachmann, F
AU  - Bachmann F
AD  - Department of Internal Medicine, Division of Nephrology, Charite Campus Mitte, 
      Berlin, Germany.
FAU - Budde, K
AU  - Budde K
AD  - Department of Internal Medicine, Division of Nephrology, Charite Campus Mitte, 
      Berlin, Germany.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20160901
PL  - Germany
TA  - Pediatr Nephrol
JT  - Pediatric nephrology (Berlin, Germany)
JID - 8708728
RN  - 0 (Antineoplastic Agents)
RN  - 0 (TSC1 protein, human)
RN  - 0 (TSC2 protein, human)
RN  - 0 (Tuberous Sclerosis Complex 1 Protein)
RN  - 0 (Tuberous Sclerosis Complex 2 Protein)
RN  - 0 (Tumor Suppressor Proteins)
RN  - 9HW64Q8G6G (Everolimus)
RN  - EC 2.7.1.1 (MTOR protein, human)
RN  - EC 2.7.11.1 (TOR Serine-Threonine Kinases)
RN  - W36ZG6FT64 (Sirolimus)
SB  - IM
MH  - Angiomyolipoma/diagnosis/etiology/*therapy
MH  - Antineoplastic Agents/*therapeutic use
MH  - Child
MH  - Embolization, Therapeutic
MH  - Everolimus/therapeutic use
MH  - Humans
MH  - Kidney/physiopathology/surgery
MH  - Kidney Failure, Chronic/diagnosis/prevention & control
MH  - Kidney Function Tests
MH  - Kidney Neoplasms/diagnosis/etiology/*therapy
MH  - Mutation
MH  - Nephrectomy
MH  - Signal Transduction
MH  - Sirolimus/therapeutic use
MH  - TOR Serine-Threonine Kinases/*antagonists & inhibitors/metabolism
MH  - Tuberous Sclerosis/*complications/genetics/therapy
MH  - Tuberous Sclerosis Complex 1 Protein
MH  - Tuberous Sclerosis Complex 2 Protein
MH  - Tumor Suppressor Proteins/genetics
OTO - NOTNLM
OT  - AML
OT  - TSC
OT  - mTOR inibitor treatment
EDAT- 2016/09/03 06:00
MHDA- 2018/03/20 06:00
CRDT- 2016/09/03 06:00
PHST- 2016/01/15 00:00 [received]
PHST- 2016/07/12 00:00 [accepted]
PHST- 2016/05/26 00:00 [revised]
PHST- 2016/09/03 06:00 [pubmed]
PHST- 2018/03/20 06:00 [medline]
PHST- 2016/09/03 06:00 [entrez]
AID - 10.1007/s00467-016-3474-6 [pii]
AID - 10.1007/s00467-016-3474-6 [doi]
PST - ppublish
SO  - Pediatr Nephrol. 2017 Jul;32(7):1137-1144. doi: 10.1007/s00467-016-3474-6. Epub 
      2016 Sep 1.