U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Multicentric carpo-tarsal osteolysis with or without nephropathy(MCTO)

MedGen UID:
436237
Concept ID:
C2674705
Disease or Syndrome
Synonyms: Carnevale Canun Mendoza syndrome; MULTICENTRIC CARPOTARSAL OSTEOLYSIS SYNDROME; Multicentric osteolysis nephropathy; Multicentric Osteolysis of Carpal Bones and Nephropathy; MULTICENTRIC OSTEOLYSIS, AUTOSOMAL DOMINANT; OSTEOLYSIS, HEREDITARY, OF CARPAL BONES WITH OR WITHOUT NEPHROPATHY
SNOMED CT: Multicentric carpotarsal osteolysis syndrome (766992008)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
 
Gene (location): MAFB (20q12)
 
Monarch Initiative: MONDO:0008152
OMIM®: 166300
Orphanet: ORPHA2774

Definition

Multicentric carpotarsal osteolysis syndrome is a rare skeletal disorder, usually presenting in early childhood with a clinical picture mimicking juvenile rheumatoid arthritis. Progressive destruction of the carpal and tarsal bone usually occurs and other bones may also be involved. Chronic renal failure is a frequent component of the syndrome. Mental retardation and minor facial anomalies have been noted in some patients. Autosomal dominant inheritance has been documented in many families (Pai and Macpherson, 1988). See also Torg-Winchester syndrome (259600), an autosomal recessive multicentric osteolysis syndrome. [from OMIM]

Clinical features

From HPO
Arthralgia
MedGen UID:
13917
Concept ID:
C0003862
Sign or Symptom
Joint pain.
Wrist pain
MedGen UID:
66384
Concept ID:
C0221785
Sign or Symptom
An unpleasant sensation characterized by physical discomfort (such as pricking, throbbing, or aching) localized to the wrist.
Ankle pain
MedGen UID:
116068
Concept ID:
C0238656
Sign or Symptom
An unpleasant sensation characterized by physical discomfort (such as pricking, throbbing, or aching) localized to the ankle.
Proteinuria
MedGen UID:
10976
Concept ID:
C0033687
Finding
Increased levels of protein in the urine.
Renal insufficiency
MedGen UID:
332529
Concept ID:
C1565489
Disease or Syndrome
A reduction in the level of performance of the kidneys in areas of function comprising the concentration of urine, removal of wastes, the maintenance of electrolyte balance, homeostasis of blood pressure, and calcium metabolism.
Stage 5 chronic kidney disease
MedGen UID:
384526
Concept ID:
C2316810
Disease or Syndrome
A degree of kidney failure severe enough to require dialysis or kidney transplantation for survival characterized by a severe reduction in glomerular filtration rate (less than 15 ml/min/1.73 m2) and other manifestations including increased serum creatinine.
Bilateral renal atrophy
MedGen UID:
868441
Concept ID:
C4022835
Disease or Syndrome
A two-sided form of atrophy of the kidney.
Ankle swelling
MedGen UID:
65906
Concept ID:
C0235439
Pathologic Function
Ulnar deviation of the hand
MedGen UID:
66031
Concept ID:
C0241521
Finding
Divergence of the longitudinal axis of the hand at the wrist in a posterior (ulnar) direction (i.e., towards the little finger).
Wrist swelling
MedGen UID:
116125
Concept ID:
C0241760
Finding
Pes cavus
MedGen UID:
675590
Concept ID:
C0728829
Congenital Abnormality
An increase in height of the medial longitudinal arch of the foot that does not flatten on weight bearing (i.e., a distinctly hollow form of the sole of the foot when it is bearing weight).
Carpal osteolysis
MedGen UID:
318954
Concept ID:
C1833734
Pathologic Function
Osteolysis affecting carpal bones.
Osteolysis involving tarsal bones
MedGen UID:
322262
Concept ID:
C1833735
Pathologic Function
An increased resorption of bone matrix by osteoclasts leading to bony defects involving the tarsal bones.
Metacarpal osteolysis
MedGen UID:
343165
Concept ID:
C1854610
Finding
Metatarsal osteolysis
MedGen UID:
344327
Concept ID:
C1854614
Finding
Osteolysis involving metatarsal bones.
Ulnar deviation of the hand or of fingers of the hand
MedGen UID:
892857
Concept ID:
C4048199
Finding
Hypertensive disorder
MedGen UID:
6969
Concept ID:
C0020538
Disease or Syndrome
The presence of chronic increased pressure in the systemic arterial system.
Inability to walk
MedGen UID:
107860
Concept ID:
C0560046
Finding
Incapability to ambulate.
Micrognathia
MedGen UID:
44428
Concept ID:
C0025990
Congenital Abnormality
Developmental hypoplasia of the mandible.
Osteopenia
MedGen UID:
18222
Concept ID:
C0029453
Disease or Syndrome
Osteopenia is a term to define bone density that is not normal but also not as low as osteoporosis. By definition from the World Health Organization osteopenia is defined by bone densitometry as a T score -1 to -2.5.
Congenital diaphragmatic hernia
MedGen UID:
68625
Concept ID:
C0235833
Congenital Abnormality
The presence of a hernia of the diaphragm present at birth.
Hypoplasia of the maxilla
MedGen UID:
66804
Concept ID:
C0240310
Congenital Abnormality
Abnormally small dimension of the Maxilla. Usually creating a malocclusion or malalignment between the upper and lower teeth or resulting in a deficient amount of projection of the base of the nose and lower midface region.
Bilateral elbow dislocations
MedGen UID:
480059
Concept ID:
C3278429
Finding
Triangular face
MedGen UID:
324383
Concept ID:
C1835884
Finding
Facial contour, as viewed from the front, triangular in shape, with breadth at the temples and tapering to a narrow chin.
Corneal opacity
MedGen UID:
40485
Concept ID:
C0010038
Finding
A reduction of corneal clarity.
Proptosis
MedGen UID:
41917
Concept ID:
C0015300
Disease or Syndrome
An eye that is protruding anterior to the plane of the face to a greater extent than is typical.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMulticentric carpo-tarsal osteolysis with or without nephropathy
Follow this link to review classifications for Multicentric carpo-tarsal osteolysis with or without nephropathy in Orphanet.

Professional guidelines

PubMed

Kidney Disease: Improving Global Outcomes (KDIGO) Diabetes Work Group
Kidney Int 2022 Nov;102(5S):S1-S127. doi: 10.1016/j.kint.2022.06.008. PMID: 36272764
Kidney Disease: Improving Global Outcomes (KDIGO) Glomerular Diseases Work Group
Kidney Int 2021 Oct;100(4S):S1-S276. doi: 10.1016/j.kint.2021.05.021. PMID: 34556256
Ikizler TA, Burrowes JD, Byham-Gray LD, Campbell KL, Carrero JJ, Chan W, Fouque D, Friedman AN, Ghaddar S, Goldstein-Fuchs DJ, Kaysen GA, Kopple JD, Teta D, Yee-Moon Wang A, Cuppari L
Am J Kidney Dis 2020 Sep;76(3 Suppl 1):S1-S107. doi: 10.1053/j.ajkd.2020.05.006. PMID: 32829751

Recent clinical studies

Etiology

Friedman DJ, Pollak MR
Clin J Am Soc Nephrol 2021 Feb 8;16(2):294-303. Epub 2020 Jul 2 doi: 10.2215/CJN.15161219. PMID: 32616495Free PMC Article
Zhang F, Lu Z, Wang F
Life Sci 2020 Oct 15;259:118379. Epub 2020 Sep 3 doi: 10.1016/j.lfs.2020.118379. PMID: 32890604
Dubin RF, Rhee EP
Clin J Am Soc Nephrol 2020 Mar 6;15(3):404-411. Epub 2019 Oct 21 doi: 10.2215/CJN.07420619. PMID: 31636087Free PMC Article
Xiong Y, Zhou L
Oxid Med Cell Longev 2019;2019:7495629. Epub 2019 Oct 3 doi: 10.1155/2019/7495629. PMID: 31687085Free PMC Article
François H, Mariette X
Nat Rev Nephrol 2016 Feb;12(2):82-93. Epub 2015 Nov 16 doi: 10.1038/nrneph.2015.174. PMID: 26568188

Diagnosis

Chen J, Liu Q, He J, Li Y
Front Immunol 2022;13:958790. Epub 2022 Aug 15 doi: 10.3389/fimmu.2022.958790. PMID: 36045667Free PMC Article
AlYousef A, AlSahow A, AlHelal B, Alqallaf A, Abdallah E, Abdellatif M, Nawar H, Elmahalawy R
BMC Nephrol 2020 May 18;21(1):186. doi: 10.1186/s12882-020-01836-3. PMID: 32423387Free PMC Article
Qi C, Mao X, Zhang Z, Wu H
J Diabetes Res 2017;2017:8637138. Epub 2017 Feb 20 doi: 10.1155/2017/8637138. PMID: 28316995Free PMC Article
Tziomalos K, Athyros VG
Rev Diabet Stud 2015 Spring-Summer;12(1-2):110-8. Epub 2015 Aug 10 doi: 10.1900/RDS.2015.12.110. PMID: 26676664Free PMC Article
Khanna R
Mo Med 2011 Jan-Feb;108(1):33-6. PMID: 21462608Free PMC Article

Therapy

Xu Y, Yang Q, Fu C, Han E, Gao Y
BMJ Open 2023 Jan 18;13(1):e064220. doi: 10.1136/bmjopen-2022-064220. PMID: 36657752Free PMC Article
Jiang DM, Gupta S, Kitchlu A, Meraz-Munoz A, North SA, Alimohamed NS, Blais N, Sridhar SS
Nat Rev Urol 2021 Feb;18(2):104-114. Epub 2021 Jan 11 doi: 10.1038/s41585-020-00404-6. PMID: 33432181
Wheeler DC, Stefansson BV, Batiushin M, Bilchenko O, Cherney DZI, Chertow GM, Douthat W, Dwyer JP, Escudero E, Pecoits-Filho R, Furuland H, Górriz JL, Greene T, Haller H, Hou FF, Kang SW, Isidto R, Khullar D, Mark PB, McMurray JJV, Kashihara N, Nowicki M, Persson F, Correa-Rotter R, Rossing P, Toto RD, Umanath K, Van Bui P, Wittmann I, Lindberg M, Sjöström CD, Langkilde AM, Heerspink HJL
Nephrol Dial Transplant 2020 Oct 1;35(10):1700-1711. doi: 10.1093/ndt/gfaa234. PMID: 32862232Free PMC Article
Villeneuve LM, Natarajan R
Contrib Nephrol 2011;170:57-65. Epub 2011 Jun 9 doi: 10.1159/000324944. PMID: 21659758Free PMC Article
Jager KJ, Zoccali C, Macleod A, Dekker FW
Kidney Int 2008 Feb;73(3):256-60. Epub 2007 Oct 31 doi: 10.1038/sj.ki.5002650. PMID: 17978811

Prognosis

Ronchetti AB, Usai M, Savino V, Scaglione M, Tacchino CM, Bertamino M, Moretti P, Di Rocco M
Orphanet J Rare Dis 2023 Dec 20;18(1):392. doi: 10.1186/s13023-023-02976-z. PMID: 38124110Free PMC Article
Dubin RF, Rhee EP
Clin J Am Soc Nephrol 2020 Mar 6;15(3):404-411. Epub 2019 Oct 21 doi: 10.2215/CJN.07420619. PMID: 31636087Free PMC Article
Guzon-Illescas O, Perez Fernandez E, Crespí Villarias N, Quirós Donate FJ, Peña M, Alonso-Blas C, García-Vadillo A, Mazzucchelli R
J Orthop Surg Res 2019 Jul 4;14(1):203. doi: 10.1186/s13018-019-1226-6. PMID: 31272470Free PMC Article
Qi C, Mao X, Zhang Z, Wu H
J Diabetes Res 2017;2017:8637138. Epub 2017 Feb 20 doi: 10.1155/2017/8637138. PMID: 28316995Free PMC Article
Soma VR, Cavusoglu E, Vidhun R, Frishman WH, Sharma SK
Heart Dis 2002 Nov-Dec;4(6):372-9. doi: 10.1097/00132580-200211000-00006. PMID: 12441014

Clinical prediction guides

Levey AS, Titan SM, Powe NR, Coresh J, Inker LA
Clin J Am Soc Nephrol 2020 Aug 7;15(8):1203-1212. Epub 2020 May 11 doi: 10.2215/CJN.12791019. PMID: 32393465Free PMC Article
Dubin RF, Rhee EP
Clin J Am Soc Nephrol 2020 Mar 6;15(3):404-411. Epub 2019 Oct 21 doi: 10.2215/CJN.07420619. PMID: 31636087Free PMC Article
Qi C, Mao X, Zhang Z, Wu H
J Diabetes Res 2017;2017:8637138. Epub 2017 Feb 20 doi: 10.1155/2017/8637138. PMID: 28316995Free PMC Article
McClelland A, Hagiwara S, Kantharidis P
Curr Opin Nephrol Hypertens 2014 Jan;23(1):80-6. doi: 10.1097/01.mnh.0000437612.50040.ae. PMID: 24247820
Goldstein SL, Chawla LS
Clin J Am Soc Nephrol 2010 May;5(5):943-9. Epub 2010 Mar 18 doi: 10.2215/CJN.07201009. PMID: 20299370

Recent systematic reviews

Servais A, Thomas K, Dello Strologo L, Sayer JA, Bekri S, Bertholet-Thomas A, Bultitude M, Capolongo G, Cerkauskiene R, Daudon M, Doizi S, Gillion V, Gràcia-Garcia S, Halbritter J, Heidet L, van den Heijkant M, Lemoine S, Knebelmann B, Emma F, Levtchenko E; Metabolic Nephropathy Workgroup of the European Reference Network for Rare Kidney Diseases (ERKNet) and eUROGEN
Kidney Int 2021 Jan;99(1):48-58. Epub 2020 Sep 9 doi: 10.1016/j.kint.2020.06.035. PMID: 32918941
de Souza E Silva A, Pertille A, Reis Barbosa CG, Aparecida de Oliveira Silva J, de Jesus DV, Ribeiro AGSV, Baganha RJ, de Oliveira JJ
J Ren Nutr 2019 Nov;29(6):480-489. Epub 2019 Jul 30 doi: 10.1053/j.jrn.2019.05.004. PMID: 31375416
Toyama T, Neuen BL, Jun M, Ohkuma T, Neal B, Jardine MJ, Heerspink HL, Wong MG, Ninomiya T, Wada T, Perkovic V
Diabetes Obes Metab 2019 May;21(5):1237-1250. Epub 2019 Mar 4 doi: 10.1111/dom.13648. PMID: 30697905
Zelniker TA, Wiviott SD, Raz I, Im K, Goodrich EL, Bonaca MP, Mosenzon O, Kato ET, Cahn A, Furtado RHM, Bhatt DL, Leiter LA, McGuire DK, Wilding JPH, Sabatine MS
Lancet 2019 Jan 5;393(10166):31-39. Epub 2018 Nov 10 doi: 10.1016/S0140-6736(18)32590-X. PMID: 30424892
Kowal-Bielecka O, Fransen J, Avouac J, Becker M, Kulak A, Allanore Y, Distler O, Clements P, Cutolo M, Czirjak L, Damjanov N, Del Galdo F, Denton CP, Distler JHW, Foeldvari I, Figelstone K, Frerix M, Furst DE, Guiducci S, Hunzelmann N, Khanna D, Matucci-Cerinic M, Herrick AL, van den Hoogen F, van Laar JM, Riemekasten G, Silver R, Smith V, Sulli A, Tarner I, Tyndall A, Welling J, Wigley F, Valentini G, Walker UA, Zulian F, Müller-Ladner U; EUSTAR Coauthors
Ann Rheum Dis 2017 Aug;76(8):1327-1339. Epub 2016 Nov 9 doi: 10.1136/annrheumdis-2016-209909. PMID: 27941129

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...