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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) CKD Work Group
Kidney Int 2024 Apr;105(4S):S117-S314. doi: 10.1016/j.kint.2023.10.018. PMID: 38490803
Kidney Disease: Improving Global Outcomes (KDIGO) Glomerular Diseases Work Group
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Recent clinical studies

Etiology

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Diagnosis

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Wyatt CM
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Pediatr Nephrol 2009 Oct;24(10):1951-8. Epub 2007 Oct 2 doi: 10.1007/s00467-007-0622-z. PMID: 17912554Free PMC Article

Therapy

Coellar JD, Long J, Danesh FR
Nephron 2021;145(4):404-414. Epub 2021 Apr 14 doi: 10.1159/000515422. PMID: 33853077Free PMC Article
Jefferson JA
Clin J Am Soc Nephrol 2018 Aug 7;13(8):1264-1275. Epub 2018 Jul 24 doi: 10.2215/CJN.01920218. PMID: 30042223Free PMC Article
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Prognosis

Raffort J, Lareyre F, Katsiki N, Mikhailidis DP
Curr Vasc Pharmacol 2022;20(1):16-26. doi: 10.2174/1570161119666210708165748. PMID: 34238195
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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
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Clinical prediction guides

Coellar JD, Long J, Danesh FR
Nephron 2021;145(4):404-414. Epub 2021 Apr 14 doi: 10.1159/000515422. PMID: 33853077Free PMC Article
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
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Acta Physiol (Oxf) 2018 Sep;224(1):e13162. Epub 2018 Jul 25 doi: 10.1111/apha.13162. PMID: 29984559
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Kidney Int 2018 May;93(5):1052-1059. Epub 2018 Feb 22 doi: 10.1016/j.kint.2017.11.032. PMID: 29477241
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

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
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Kariminik A
Cytokine 2016 Dec;88:276-280. Epub 2016 Oct 5 doi: 10.1016/j.cyto.2016.09.023. PMID: 27718431

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