Congenital growth hormone deficiency associated with hip dysplasia and Legg-Calve-Perthes disease

Elisa B Lamback1, Stella Chiarini1, Andreas Roposch1

  • 1Great Ormond Street Hospital for Children, London, UK.

Clinical Endocrinology
|November 4, 2020
PubMed

Insights

Congenital Growth Hormone Deficiency (GHD) may cause hip dysplasia. Recombinant human Growth Hormone (rhGH) treatment is unlikely to cause Legg-Calve-Perthes disease (LCPD).

Area of Science:

  • Pediatric Endocrinology
  • Orthopedics

Background:

  • Growth Hormone Deficiency (GHD) is typically managed with recombinant human Growth Hormone (rhGH).
  • Hip disorders, including hip dysplasia and Legg-Calve-Perthes disease (LCPD), have rarely been linked to GHD or its treatment.

Purpose of the Study:

  • To investigate the association between congenital GHD, hip dysplasia, and LCPD.
  • To determine if hip disorders in GHD patients are related to the underlying condition or rhGH therapy.

Main Methods:

  • Retrospective analysis of pediatric and adolescent patients with congenital GHD and hip disorders (1992-2018).
  • Data collection included demographics, clinical and imaging findings, and treatment timelines.

Main Results:

  • Of 13 patients, 10 had hip dysplasia and 3 had LCPD.
  • Hip dysplasia was diagnosed before rhGH initiation in 50% of cases.
  • LCPD progression was not consistently linked to rhGH treatment initiation.

Conclusions:

  • Hip dysplasia may be a manifestation of congenital GHD itself.
  • rhGH treatment is unlikely to be the primary cause of LCPD in patients with GHD.
Abstract

Related Concept Videos

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...
591
Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
221
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...
4.9K
Glucose Transporters01:27

Glucose Transporters

Glucose transporters facilitate the transport of glucose across the cell membrane. In addition to glucose, some glucose transporters can also aid the movement of other hexoses such as fructose, mannose, and galactose.
Facilitated diffusion-glucose transporters (GLUTs) are encoded by the solute-linked carrier (SLC) family 2, subfamily A gene family, or SLC2A. The 14 GLUT protein members are distributed into three classes:
26.8K
Bones of the Lower Limb: Femur and Patella01:16

Bones of the Lower Limb: Femur and Patella

The femur is the body's longest and strongest bone spanning the thigh region. Its head articulates with the acetabulum of the hip bone to form the hip joint. A minor indentation on the medial side of the femoral head, called the fovea capitis, serves as the site of attachment for the ligament of the head of the femur. This weak ligament spans the femur and acetabulum and supports the hip joint. The narrowed region below the head is the neck of the femur. The inclination angle between the...
4.5K
Nature and Nurture01:10

Nature and Nurture

Many human characteristics, like height, are shaped by both nature—in other words, by our genes—and by nurture, or our environment. For example, chronic stress during childhood inhibits the production of growth hormones and consequently reduces bone growth and height. Scientists estimate that 70-90% of variation in height is due to genetic differences among individuals, and 10-30% of variation in height is due to differences in the environments that individuals experience,...
21.8K