Scoliosis in Cystinosis.

 

Craig B. Langman, MD

The Isaac A. Abt, MD Professor of Kidney Diseases

Head, Kidney Diseases

Feinberg School of Medicine, Northwestern University

Children’s Memorial Hospital

Chicago IL

 

Scoliosis, or an angular deformity of the spine, is a common bone condition in children.  It is most often idiopathic, or without specific known cause.  Scoliosis is accentuated during periods of rapid growth, such as in adolescence.   There are tests that can be performed by a physician on routine examination of the child that may identify the condition, but sometimes, falsely so.  Definitive diagnosis depends on demonstration of the angular deformity by X-ray study of the spine.   Referral to a specialist in pediatric orthopedics is encouraged for proper care.

 

Cystinosis is not associated with scoliosis in the absence of some other problem in bone.  Such problems may be the co-existence of the more common problem, such as idiopathic scoliosis, with the rarer disease, or the result of more specific bone problems.  For example, an asymmetric vertebral crush fracture may produce scoliosis in the growing child.  Such fractures may result from corticosteroid therapy, for example.

 

Medications are generally not felt to be causative in cases of scoliosis.  However, there are isolated case reports of the concomitant use of recombinant human growth hormone and the “development” of scoliosis.  A review of over 900 children treated for over 3600 patient-years with recombinant human growth hormone did not find any relation between that therapy and the development of scoliosis.

 

Kidney transplantation may have bone complications, since most children with a functioning transplant have some form of chronic kidney disease (CKD).  CKD is known to have associated bone disease, including the development of scoliosis. 

 

Since scoliosis is a common disease of children, and there is no firm evidence that medications contribute significantly to its presence, we must conclude that the use of recombinant human growth hormone is unlikely as its cause.  However, a prudent approach might be to discontinue, temporarily or permanently, the use of the growth product during the evaluation and follow-up of scoliosis, since rapid growth spurts may be associated with progression of scoliosis.

 


References.

 

Burwell RG 2003 Aetiology of idiopathic scoliosis: current concepts. Pediatr Rehabil 6:137–170.

 

Charmian A. Quigley, Anne M. Gill, Brenda J. Crowe, Kristen Robling, John J. Chipman, Susan R. Rose, Judith L. Ross, Fernando G. Cassorla, Anne M. Wolka, Jan M. Wit, Lyset T. M. Rekers-Mombarg, and

Gordon B. Cutler, Jr..  Safety of Growth Hormone Treatment in Pediatric Patients with Idiopathic Short Stature.  J Clin Endocrinol Metab 90: 5188–5196, 2005.

 

Morais T, Bernier M, Turcotte F 1985 Age- and sex-specific prevalence of scoliosis and the value of school screening programs. Am J Public Health 75:1377–1380.

 

Rogala EJ, Drummond DS, Gurr J 1978 Scoliosis: incidence and natural history. A prospective epidemiological study. J Bone Joint Surg Am 60:173–176.