Idiopathic juvenile osteoporosis

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Classification according to ICD-10
M81.5 Idiopathic osteoporosis
ICD-10 online (WHO version 2019)

The juvenile osteoporosis is occurring in childhood and adolescence osteoporosis (brittle bones) of unknown cause with mostly spontaneously forming successful healing. The disease is also called Dent-Friedman syndrome according to descriptions from 1965 .

clinic

Diagnostic criteria are:

  • Onset of the disease in prepuberty with pain in the joints or the lower spine
  • Growth stall
  • Increasing general osteoporosis with the formation of fish vertebrae , vertebral body fractures and in particular metaphyseal spontaneous fractures of the long bones
  • Cessation of changes with regression after the end of puberty

Diagnosis

The characteristic changes in the spine can be seen on simple x-rays . A bone density measurement can document the demineralization.

Histologically, there is a reduced amount of osteoid, rarefaction of the trabeculae and loosening of the cortex .

Differential diagnostics

Demarcate are Juvenile idiopathic arthritis , Juvenile arthritis, as well as osteogenesis imperfecta , or eating disorders and rickets , also the Progressive Pseudorheumatoide arthropathy . In the case of multiple fractures, child abuse should be considered.

therapy

No causal treatment is known, physiotherapy , muscle building, and avoidance of lifting heavy loads are essential to protect against further fractures .

The administration of calcium and / or vitamin D , fluoride , calcitonin and bisphosphonate may also be indicated.

literature

  • W. Catel: Puberty Fish Vertebral Disease. In: Pediatric Practice. Volume 22, Number 1, January 1954, pp. 21-26, ISSN  0023-1495 . PMID 13152954 .
  • FW Rathke: Juvenile osteoporosis of the spine In: Journal for orthopedics and their border areas. Volume 89, Number 1, 1957, pp. 40-50, ISSN  0044-3220 . PMID 13486840 .
  • B. Stöver, F. Ball, A. Walther: Idiopathic juvenile osteoporosis. In: Advances in X-Ray and Nuclear Medicine. Volume 121, Number 4, October 1974, pp. 435-444, ISSN  0015-8151 . PMID 4373340 .

Individual evidence

  1. ^ CE Dent, M. Friedman: Idiopathic Juvenile Osteoporosis. 1965 in: The Quarterly Journal of Medicine 34, pp. 177-210 PMID 14319616
  2. ↑ a b c B. Leiber: The clinical syndromes. Syndromes, sequences and symptom complexes. Edited by G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger, 7th edition. Urban & Schwarzenberg 1990, ISBN 3-541-01727-9
  3. ^ Idiopathic juvenile osteoporosis. In: Orphanet (Rare Disease Database).

Web links