Hepatic osteodystrophy complicated with bone fracture in early infants with biliary atresia

Tadao Okada1, Shohei Honda, Hisayuki Miyagi

  • 1Tadao Okada, Shohei Honda, Hisayuki Miyagi, Masashi Minato, Department of Pediatric Surgery, Hokkaido University Hospital, Sapporo 060-8648, Japan.

Insights

Biliary atresia (BA) can cause bone fractures in infants, even before or shortly after Kasai portoenterostomy surgery. Early diagnosis of BA is crucial for infants presenting with bone fractures during early infancy.

Area of Science:

  • Pediatrics
  • Hepatology
  • Orthopedics

Background:

  • Biliary atresia (BA) is a significant infant hepatobiliary disorder.
  • BA can lead to hepatic osteodystrophy due to impaired nutrient absorption.
  • Vitamin D, calcium, and magnesium malabsorption contribute to bone disease in BA.

Purpose of the Study:

  • To report rare cases of bone fractures in infants with BA.
  • To highlight the association between BA and early-onset bone fractures.
  • To review existing literature on this condition.

Main Methods:

  • Case report of two infants with BA and bone fractures.
  • Literature review on BA and bone fractures in infancy.

Main Results:

  • One infant presented with a bone fracture before Kasai portoenterostomy.
  • Another infant experienced a bone fracture 4 weeks after Kasai portoenterostomy.
  • Bone fractures are a rare but significant presentation in infants with BA.

Conclusions:

  • Clinicians should consider biliary atresia in infants presenting with bone fractures.
  • Early recognition of BA is vital for managing associated bone disease.
  • This presentation underscores the systemic impact of BA.

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...
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...
Fractures: Bone Repair01:27

Fractures: Bone Repair

Treatment for a fracture is based on the type of break, the bone affected, and the patient's age.
Minor fractures with no bone displacement are treated by immobilizing the fractured bone using a cast or splint. However, in the case of fractures with displaced bones, the broken bones are repositioned before immobilization to ensure successful healing without deformation and loss of function. The realignment of fractured bone ends is performed through a process called reduction. If the procedure...
Chronic Bowel Disorders: Introduction01:17

Chronic Bowel Disorders: Introduction

Chronic bowel diseases are a group of long-term conditions affecting the digestive tract, characterized by inflammation and damage to the gut lining. These conditions primarily include irritable bowel syndrome and inflammatory bowel disease.
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
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...
Inborn Errors of Metabolism01:20

Inborn Errors of Metabolism

Phenylketonuria (PKU) is a protein metabolism disorder characterized by high blood levels of the amino acid phenylalanine. This results from a mutation in the gene responsible for phenylalanine hydroxylase, an enzyme that converts phenylalanine into tyrosine. When this enzyme is deficient, phenylalanine builds up in the blood, leading to symptoms such as vomiting, rashes, seizures, growth deficiency, and severe mental retardation. An early diagnosis and a diet restricting phenylalanine intake...