Kyphosis and fractures in children and young adults with cystic fibrosis

doi:10.1016/S0022-3476(94)70194-6

The Journal of Pediatrics

Volume 125, Issue 2, August 1994, Pages 208-212

https://doi.org/10.1016/S0022-3476(94)70194-6 Get rights and content

Abstract

See related article, "Influences on skeletal mineralization in children and adolescents: Evidence for varying effects of sexual maturation and physical activity"

The purpose of this study was to examine children and adolescents with cystic fibrosis for an increased frequency of fracture and excessive thoracic kyphosis, which may result from inadequate skeletal mineralization. In a survey of 143 patients (ages 4.7 to 21.9 years; mean, 11.3 years), the fracture rate for male patients from birth to 5 years of age was higher than for female patients and both rates were comparable with those for normal children. In contrast, female patients 6 to 16 years of age with cystic fibrosis had a higher-than-normal fracture rate and a higher rate than their male counterparts. Review of the chest radiographs showed that thoracic kyphosis correlated with age and with disease severity as judged by Brasfield scoring. In the >15-year-old age group, kyphosis exceeding 40 degrees, the upper limit of normal, was found in 77% of the female patients and 36% of the male patients. The cause of these findings is uncertain and perhaps multifactorial, but osteopenia is likely a contributing factor. As the life expectancy of patients with cystic fibrosis continues to increase, the skeletal consequences, particularly in female patients, may become increasingly significant. (J PEDIATR 1994;125:208-12)

Section snippets

METHODS

The cystic fibrosis database maintained by the department of pediatrics at the University of North Carolina provided a listing of 150 patients with birthdays between Jan. 1, 1970, and Dec. 31, 1986. All the families were mailed a short questionnaire asking whether the patient had ever sustained a fracture. A follow-up questionnaire and direct telephone or personal contact in our clinic were used to obtain information from families who did not initially respond. Ultimately, 143 (95.3%) of the

RESULTS

There were 44 fractures among the 34 patients who reported that they had sustained a fracture. Fractures involving more than one bone in the same area, such as the radius and ulna, or multiple fractures in the same bone were counted as a single fracture. Two patients had fractures in different extremities as a consequence of a single injury. As in the report based on the National Health Survey, these were counted as separate fracture events.⁹ An additional five patients had two fractures each

DISCUSSION

The fractures sustained by this group of patients with cystic fibrosis were typical for fractures in a normal pediatric population. Upper extremity fractures were the most common and trauma during routine play activities was the most common mechanism of injury. The fractures readily healed, usually with just simple treatment measures, and there were no permanent sequelae.

Fracture rates in normal pediatric populations significantly vary with age and sex. The American Academy of Orthopaedic

ACKNOWLEDGMENT

We thank Drs. Gerald Fernald, Robert Wood, and the other members of the University of North Carolina Division of Pediatric Pulmonary Medicine, and Michelle Wagner and Ralph DeMasi of the Department of Biostatistics, for their help with this study.

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The resultant chronic pulmonary hyperinflation usually causes enlargement of the anterior and posterior diameters of the chest, thereby resulting in barrel chest or other chest deformities. A study of 143 young patients with cystic fibrosis showed that in patients over 15 years old, the condition of 77% of females and 36% of males was complicated with thoracic kyphosis deformity of more than 40°.30 Since this deformity can inhibit airway clearance and increase the work of breathing, it is important to incorporate physiotherapy, such as chest and muscle stretching and intensive training, in a comprehensive pulmonary rehabilitation program for thoracic kyphosis correction.
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Individuals with CF have higher rates of fracture compared to healthy individuals [2]. Certain types of fractures, particularly vertebral and rib fractures, may lead to further deterioration in lung function [4,5] by limiting physical activity and inhibiting patients from effective airway clearance techniques to clear mucus. This increased risk of fracture is not well understood but appears to be multifactorial [6,7].
Individuals with cystic fibrosis (CF) have lower bone mineral density (BMD) by DXA and are at higher risk of fracture than healthy controls. However, the 2-dimensional measurement of areal BMD (aBMD) provided by DXA is influenced by bone size and the true extent of the bone deficit is unclear. Our objective was to use high-resolution peripheral quantitative computed tomography (HR-pQCT) and individual trabecula segmentation (ITS) analysis to compare volumetric BMD (vBMD), microarchitecture and estimated strength at the distal radius and tibia in 26 young adults with CF and 26 controls matched for age, gender, and race. To assess the effect of limb length and minimize the confounding effects of size on HR-pQCT outcomes, we scanned participants at both the standard fixed HR-pQCT measurement sites and at a subject-specific relative site that varied according to limb length. CF participants did not differ significantly in age, height, weight, or BMI from controls. Ulnar and tibial lengths were 9 mm shorter in CF patients, though differences were not significant. CF patients had significantly lower BMI-adjusted aBMD by DXA at the lumbar spine (8.9%, p < 0.01), total hip (11.5%, p < 0.01) and femoral neck (14.5%, p < 0.01), but not at the forearm. At the fixed radius site, thickness of trabecular plates and torsional stiffness were significantly lower in CF participants than controls. At the relative radius site, only torsional stiffness was significantly lower in CF participants. At the tibia, total, trabecular and cortical vBMD were significantly lower at both fixed and relative sites in CF participants, with fewer, more widely-spaced trabecular plates, lower trabecular connectivity, and lower axial and torsional stiffness. Our results confirm that aBMD is lower at the spine and hip in young adults with CF, independent of BMI and body size. We also conclude that vBMD and stiffness are lower at the weight-bearing tibia. The pathogenesis of these differences in bone density and strength at the tibia appear to be related to trabecular drop-out and reduced trabecular connectivity and to be independent of differences in limb length, as assessed by scanning participants at both standard and relative sites. We concluded that significant deficits in bone structure and strength persist in young adults with CF, despite advances in care that permit them to attain relatively normal height and weight.
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The relative risk for bone fractures in patients with cystic fibrosis (CF) and its relationship to macroscopic bone architecture assessed by pQCT and DXA are incompletely defined.
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The fracture rate in CF was 9.2-fold higher compared to an age-matched German control population. The probability of remaining free of any fracture in CF patients at 25 years was reduced to 39.8% compared to 84.6% in controls (P < 0.001). Assessment of macroscopic bone architecture by DXA and pQCT allowed the differentiation of patients with multiple prevalent fractures with a high sensitivity (up to 100%) and specificity (up to 94.3%).
Bone densitometry is a useful tool for noninvasive assessment of fracture risk in CF patients.
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The causes of CF-related bone disease (CFBD) are multifactorial and include nutritional deficiencies of vitamin D, vitamin K, and calcium; glucocorticoid use; sex steroid deficiency; an altered GH axis; inflammation and the mutation of the CFTR gene itself.38 The prevalence of fractures in children and adults with CF have been reported to occur in up to a third of patients.39,40 A recent systematic review of adults with CF found a pooled prevalence of low bone mineral density (BMD) (T score <−1 and >−2.5) of 38% and osteoporosis (T score <−2.5) of 23.5% by dual-energy x-ray absorptiometry (DEXA)41 and of vertebral fractures by lateral spine radiograph of 14% and nonvertebral fractures by self-report of 19.7%.
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L’allongement de l’espérance de vie des patients atteints de mucoviscidose (MV) s’accompagne d’une augmentation de la prévalence des complications tardives, telles que la maladie osseuse liée à la MV (MOM). D’abord décrite dans la population adulte dans laquelle 24 % des patients présentent des critères ostéodensitométriques d’ostéoporose, elle a ensuite été rapportée dans la population pédiatrique. Elle est caractérisée par l’association d’une diminution de la formation osseuse associée à une augmentation à des degrés variables de la résorption osseuse. D’origine multifactorielle, le déficit osseux s’installe en plusieurs temps. Le premier est probablement primitif, en lien avec l’absence de protéine cystic fibrosis transmembrane conductance regulator (CFTR) fonctionnelle dans les ostéoblastes. Secondairement, le déficit s’accentue à la faveur de facteurs acquis compliquant la MV, tels l’inflammation chronique et les surinfections respiratoires, la dénutrition, les carences vitaminiques, la carence en insuline et la baisse de l’activité physique. La survenue de fractures (tassements vertébraux et fractures costales essentiellement) en est l’expression clinique. La prise en charge de la MOM est assez bien codifiée. Elle est d’abord globale, prenant notamment en compte les facteurs de risque liés à la maladie et les complications telles que les fractures. Elle est préventive, reposant sur l’évaluation régulière de la densité minérale osseuse par absorptiométrie biphotonique dès l’âge de 8 ans et le contrôle d’éventuels facteurs d’altération de l’état osseux. De nouvelles techniques d’imagerie, notamment la tomographie périphérique quantitative à haute résolution (HR-pQCT), apportent des informations complémentaires sur les densités compartimentales et la micro-architecture trabéculaire. Elles permettent ainsi de mieux évaluer l’état osseux, et peut-être dans le futur de fournir une évaluation plus précise du risque fracturaire.
With the increasing life expectancy of patients with cystic fibrosis (CF), prevalence of late complications such as CF-related bone disease (CFBD) has increased. It was initially described in 24% of the adult population with CF and has also been reported in the pediatric population. CFBD is multifactorial and progresses in different steps. Both decreased bone formation and increased bone resorption (in different amounts) are observed. CFBD is likely primitive (directly related to the CFTR defect itself), but is also worsened by acquired secondary factors such as lung infections, chronic inflammation, denutrition, vitamin deficiency, and decreased physical activity. CFBD may be clinically apparent (i.e., mainly vertebral and costal fractures), or clinically asymptomatic (therefore corresponding to abnormalities in bone density and architecture). CFBD management mainly aims to prevent the occurrence of fractures. Prevention and regular monitoring of bone disease as early as 8 years of age is of the utmost importance, as is the control of possible secondary deleterious CFBD factors. New radiological tools, such as high-resolution peripheral quantitative computed tomography, allow an accurate evaluation of cortical and trabecular bone micro-architecture in addition to compartmental density; as such, they will likely improve the assessment of the bone fracture threat in CF patients in the near future.

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^☆: From the Departments of Orthopaedics, Pediatrics, and Radiology, University of North Carolina, Chapel Hill

^☆☆: Reprint requests: Richard C. Henderson, MD, 237 Burnett-Womack Building, CB No. 7055, Chapel Hill, NC 27599-7055.

^★: 0022-3476/94/$3.00 + 09/20/55475

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Kyphosis and fractures in children and young adults with cystic fibrosis☆,☆☆,★

Abstract

Section snippets

METHODS

RESULTS

DISCUSSION

ACKNOWLEDGMENT

J PEDIATR

J PEDIATR

J PEDIATR

J PEDIATR

Demineralization in cystic fibrosis

Am J Dis Child

Outline for the study of scoliosis

Am Acad Orthop Surg

Radiographic determination of lordosis and kyphosis in normal and scoliotic children

J Pediatr Orthop

Thoracic kyphosis: range in normal subjects

Am J Roentgenol

Thoracic kyphosis in cystic fibrosis

Clin Orthop

The chest roentgenogram in cystic fibrosis: a new scoring system

Pediatrics