Bone health in a nonjaundiced population of children with biliary atresia

Rachel A Kramer1, Babette S Zemel, Jessica L Arvay-Nezu

  • 1The Division of Gastroenterology, Hepatology & Nutrition, Children's Hospital of Philadelphia, PA 19104, USA.

Insights

Children with biliary atresia (BA) have poor bone health, even without jaundice. Bone mineral content (BMC) is significantly decreased, especially in older children, indicating a need for early intervention.

Area of Science:

  • Pediatric Gastroenterology
  • Pediatric Endocrinology
  • Bone Metabolism

Background:

  • Biliary atresia (BA) is a serious neonatal liver disease.
  • Bone health complications in children with BA are not well understood, particularly in nonjaundiced patients.
  • Growth and pubertal development can influence bone outcomes in chronic conditions.

Purpose of the Study:

  • To evaluate bone health in nonjaundiced children with BA.
  • To determine the impact of growth and development on bone mineral density (BMD) and bone mineral content (BMC) in this cohort.

Main Methods:

  • A cohort of 1-18 year olds with BA was assessed at a single visit.
  • Measurements included growth, pubertal status, medications, liver function tests, vitamin D levels, BMD, and BMC (lumbar spine and whole body).
  • A control group was used for comparative analysis.

Main Results:

  • BMD significantly declined with increasing age.
  • Children with BA had significantly lower lumbar spine and whole body BMC compared to controls, even after adjusting for confounders.
  • Older children with BA exhibited a more pronounced deficit in BMC.

Conclusions:

  • Children with BA, irrespective of jaundice status, exhibit compromised bone health.
  • Height and puberty do not fully explain the observed bone mineral deficits.
  • Further research is crucial to identify causes and develop preventative strategies for osteoporosis in BA patients.
Abstract

Related Concept Videos

Bone Disorders01:29

Bone Disorders

Aging and its effect on bone remodeling is the most common cause of bone disorders. In young and healthy people, bone deposition and resorption happen at an equal rate to maintain optimal bone health.
Bone deposition is also affected by the levels of sex hormones like estrogen and testosterone that promote osteoblast activity and bone matrix synthesis. When the level of these hormones decreases due to aging, it causes a reduction in bone deposition. As a result, bone resorption by osteoclasts...
Changes in the Appendicular Skeleton with Age01:09

Changes in the Appendicular Skeleton with Age

The upper and lower limb initially develops as a small bulge called a limb bud, which appears on the lateral side of the early embryo. The upper limb bud appears near the end of the fourth week of development, with the lower limb bud appearing shortly after.
Initially, the limb buds consist of a core of mesenchyme covered by a layer of ectoderm. The ectoderm at the end of the limb bud thickens to form a narrow crest called the apical ectodermal ridge. This ridge stimulates the underlying...
Bone Formation by Endochondral Ossification01:24

Bone Formation by Endochondral Ossification

Bone formation, or ossification, begins around the sixth to seventh week of embryonic development. Most bones develop from a cartilaginous template through the process of endochondral ossification. Cartilage formation begins when clusters of mesenchymal cells differentiate into chondrocytes. These chondrocytes proliferate rapidly and secrete an extracellular matrix that becomes encased in a membrane called the perichondrium. The resulting cartilage model provides a template that resembles the...
Bone Structure01:55

Bone Structure

Within the skeletal system, the structure of a bone, or osseous tissue, can be exemplified in a long bone, like the femur, where there are two types of osseous tissue: cortical and cancellous.
Compact Bone01:27

Compact Bone

Most bones contain compact and spongy osseous tissue, but their distribution and concentration vary based on the bone's overall function.
Compact bone, also called cortical bone, is the denser, stronger of the two types of bone tissue. It is found under the periosteum and in the diaphyses of long bones, where it provides support and protection. The microscopic structural unit of compact bone is called an osteon, or haversian system. Each osteon is composed of concentric rings of calcified...
Role of Vitamins in Maintaining Bone Health01:25

Role of Vitamins in Maintaining Bone Health

The growth and maintenance of bone are regulated by a combination of nutritional factors, including vitamins, such as vitamin A, B12, C, D, and K.
Vitamin A
Vitamin A is involved in the process of bone remodeling. Retinoic acid, the active metabolite of Vitamin A, has nuclear receptors in osteoblasts and osteoclasts, which are involved in bone remodeling.
Vitamin B12
Vitamin B12 acts as a cofactor during the formation of osteoblast-related proteins, such as osteocalcin. Vitamin B12 plays a role...