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Axial osteomalacia

MedGen UID:
354730
Concept ID:
C1862372
Disease or Syndrome
Synonym: Atypical osteomalacia involving the axial skeleton
 
Monarch Initiative: MONDO:0007181
OMIM®: 109130

Clinical features

From HPO
Renal cyst
MedGen UID:
854361
Concept ID:
C3887499
Disease or Syndrome
A fluid filled sac in the kidney.
Autosomal dominant polycystic liver disease
MedGen UID:
56388
Concept ID:
C0158683
Disease or Syndrome
Isolated polycystic liver disease (PCLD) is a genetic disorder characterized by the appearance of numerous cysts spread throughout the liver and that in most cases is described as autosomal dominant polycystic liver disease (ADPCLD).
Myopathy
MedGen UID:
10135
Concept ID:
C0026848
Disease or Syndrome
A disorder of muscle unrelated to impairment of innervation or neuromuscular junction.
Osteomalacia
MedGen UID:
14533
Concept ID:
C0029442
Disease or Syndrome
Osteomalacia is a general term for bone weakness owing to a defect in mineralization of the protein framework known as osteoid. This defective mineralization is mainly caused by lack in vitamin D. Osteomalacia in children is known as rickets.
Increased bone mineral density
MedGen UID:
10502
Concept ID:
C0029464
Disease or Syndrome
An abnormal increase of bone mineral density, that is, of the amount of matter per cubic centimeter of bones which is often referred to as osteosclerosis. Osteosclerosis can be detected on radiological examination as an increased whiteness (density) of affected bones.
Proximal muscle weakness
MedGen UID:
113169
Concept ID:
C0221629
Finding
A lack of strength of the proximal muscles.
Elevated circulating creatine kinase concentration
MedGen UID:
69128
Concept ID:
C0241005
Finding
An elevation of the level of the enzyme creatine kinase (also known as creatine phosphokinase (CK; EC 2.7.3.2) in the blood. CK levels can be elevated in a number of clinical disorders such as myocardial infarction, rhabdomyolysis, and muscular dystrophy.

Recent clinical studies

Diagnosis

Garip Y, Dedeoglu M, Bodur H
Osteoporos Int 2014 Jul;25(7):1983-5. Epub 2014 Apr 24 doi: 10.1007/s00198-014-2716-3. PMID: 24760247
Cortet B, Bernière L, Solau-Gervais E, Hacène A, Cotten A, Delcambre B
Clin Exp Rheumatol 2000 Sep-Oct;18(5):625-8. PMID: 11072608
Demiaux-Domenech B, Bonjour JP, Rizzoli R
Bone 1996 Jun;18(6):633-7. doi: 10.1016/8756-3282(96)00087-7. PMID: 8806007
Schmidt H, Mannkopf H, Ullrich K, von Lengerke HJ
Pediatr Radiol 1989;19(6-7):489-92. doi: 10.1007/BF02387669. PMID: 2771500
Whyte MP, Fallon MD, Murphy WA, Teitelbaum SL
Am J Med 1981 Dec;71(6):1041-9. doi: 10.1016/0002-9343(81)90338-7. PMID: 7315848

Therapy

Garip Y, Dedeoglu M, Bodur H
Osteoporos Int 2014 Jul;25(7):1983-5. Epub 2014 Apr 24 doi: 10.1007/s00198-014-2716-3. PMID: 24760247
Nelson AM, Riggs BL, Jowsey JO
Arthritis Rheum 1978 Jul-Aug;21(6):715-22. doi: 10.1002/art.1780210619. PMID: 737002

Clinical prediction guides

Cortet B, Bernière L, Solau-Gervais E, Hacène A, Cotten A, Delcambre B
Clin Exp Rheumatol 2000 Sep-Oct;18(5):625-8. PMID: 11072608
Demiaux-Domenech B, Bonjour JP, Rizzoli R
Bone 1996 Jun;18(6):633-7. doi: 10.1016/8756-3282(96)00087-7. PMID: 8806007

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