Osteosclerosis is a disorder that is characterized by
abnormal hardening of bone and an elevation in bone density. It may predominantly affect the
medullary portion and/or
cortex
Cortex or cortical may refer to:
Biology
* Cortex (anatomy), the outermost layer of an organ
** Cerebral cortex, the outer layer of the vertebrate cerebrum, part of which is the ''forebrain''
*** Motor cortex, the regions of the cerebral cortex i ...
of bone. Plain
radiographs
Radiography is an imaging technology, imaging technique using X-rays, gamma rays, or similar ionizing radiation and non-ionizing radiation to view the internal form of an object. Applications of radiography include medical radiography ("diagnos ...
are a valuable tool for detecting and classifying osteosclerotic disorders.
[ It can manifest in localized or generalized osteosclerosis. Localized osteosclerosis can be caused by ]Legg–Calvé–Perthes disease
Legg–Calvé–Perthes disease (LCPD) is a childhood hip disorder initiated by a disruption of blood flow to the head of the femur. Due to the lack of blood flow, the bone dies (osteonecrosis or avascular necrosis) and stops growing. Over time, ...
, sickle-cell disease
Sickle cell disease (SCD) is a group of blood disorders typically inherited from a person's parents. The most common type is known as sickle cell anaemia. It results in an abnormality in the oxygen-carrying protein haemoglobin found in red blo ...
and osteoarthritis
Osteoarthritis (OA) is a type of degenerative joint disease that results from breakdown of joint cartilage and underlying bone which affects 1 in 7 adults in the United States. It is believed to be the fourth leading cause of disability in the w ...
among others. Osteosclerosis can be classified in accordance with the causative factor into acquired and hereditary.[
]
Types
Acquired osteosclerosis
* Osteogenic bone metastasis caused by carcinoma of prostate and breast
* Paget's disease of bone
Paget's disease of bone (commonly known as Paget's disease or, historically, osteitis deformans) is a condition involving cellular remodeling and deformity of one or more bones. The affected bones show signs of dysregulated bone remodeling at the ...
* Myelofibrosis
Primary myelofibrosis (PMF) is a rare bone marrow blood cancer. It is classified by the World Health Organization (WHO) as a type of myeloproliferative neoplasm, a group of cancers in which there is growth of abnormal cells in the bone marrow. ...
(primary disorder or secondary to intoxication or malignancy)
* Osteosclerosing types of chronic osteomyelitis
Osteomyelitis (OM) is an infection of bone. Symptoms may include pain in a specific bone with overlying redness, fever, and weakness. The long bones of the arms and legs are most commonly involved in children e.g. the femur and humerus, while the ...
* Hypervitaminosis D
Vitamin D toxicity, or hypervitaminosis D is the toxic state of an excess of vitamin D. The normal range for blood concentration is 20 to 50 nanograms per milliliter (ng/mL). However, the toxic state is known to be a value of 100 ng/ml or more in ...
* hyperparathyroidism
Hyperparathyroidism is an increase in parathyroid hormone (PTH) levels in the blood. This occurs from a disorder either within the parathyroid glands (primary hyperparathyroidism) or as response to external stimuli (secondary hyperparathyroidism). ...
* Schnitzler syndrome There have been several people named Schnitzler ( he, שניצלר):
* Arthur Schnitzler (born 1862), an Austrian playwright and author, son of Johann
* Barbara Schnitzler (born 1953, Berlin), a German actress (de)
* Claude Schnitzler (born 1949, ...
Mastocytosis
Mastocytosis, a type of mast cell disease, is a rare disorder affecting both children and adults caused by the accumulation of functionally defective mast cells (also called ''mastocytes'') and CD34+ mast cell precursors.
People affected by masto ...
Skeletal fluorosis
Skeletal fluorosis is a bone disease caused by excessive accumulation of fluoride leading to weakened bones. In advanced cases, skeletal fluorosis causes painful damage to bones and joints.
Symptoms
Symptoms are mainly promoted in the bone structu ...
* Monoclonal
Monoclonality refers to the state of a line of cells that have been derived from a single clonal origin. Thus "monoclonal cells" can be said to form a single clone. The term ''monoclonal'' comes from the Ancient Greek ''monos'', meaning "alone" o ...
IgM
Immunoglobulin M (IgM) is one of several isotypes of antibody (also known as immunoglobulin) that are produced by vertebrates. IgM is the largest antibody, and it is the first antibody to appear in the response to initial exposure to an antig ...
Kappa cryoglobulinemia
Cryoglobulinemia is a medical condition in which the blood contains large amounts of pathological cold sensitive antibodies called cryoglobulins – proteins (mostly immunoglobulins themselves) that become insoluble at reduced temperatures. T ...
Hepatitis C
Hepatitis C is an infectious disease caused by the hepatitis C virus (HCV) that primarily affects the liver; it is a type of viral hepatitis. During the initial infection people often have mild or no symptoms. Occasionally a fever, dark urine, a ...
.
Hereditary osteosclerosis
* Malignant infantile osteopetrosis
Malignant infantile osteopetrosis is a rare osteosclerosing type of skeletal dysplasia that typically presents in infancy and is characterized by a unique radiographic appearance of generalized hyperostosis (excessive growth of bone).
The general ...
osteopetrosis
Osteopetrosis, literally "stone bone", also known as marble bone disease or Albers-Schönberg disease, is an extremely rare Biological inheritance, inherited disease, disorder whereby the bones harden, becoming Density, denser, in contrast to mor ...
* Infantile osteopetrosis with renal tubular acidosis
Renal tubular acidosis (RTA) is a medical condition that involves an accumulation of acid in the body due to a failure of the kidneys to appropriately acidify the urine. In renal physiology, when blood is filtered by the kidney, the filtrate pas ...
* Infantile osteopetrosis with immunodeficiency
* IO with leukocyte adhesion deficiency syndrome
Leukocyte adhesion deficiency (LAD) is a rare autosomal recessive disorder characterized by immunodeficiency resulting in recurrent infections. LAD is currently divided into three subtypes: LAD1, LAD2, and the recently described LAD3, also kn ...
(LAD-III)
* Intermediate osteopetrosis
* Autosomal dominant
In genetics, dominance is the phenomenon of one variant (allele) of a gene on a chromosome masking or overriding the effect of a different variant of the same gene on the other copy of the chromosome. The first variant is termed dominant and t ...
osteopetrosis (Albers-Schonberg)
* Pyknodysostosis
Pycnodysostosis (from Greek: πυκνός (puknos) meaning "dense", ''dys'' ("defective"), and ''ostosis'' ("condition of the bone")), is a lysosomal storage disease of the bone caused by a mutation in the gene that codes the enzyme cathepsin K. ...
(osteopetrosis acro-osteolytica)Osteopoikilosis
Osteopoikilosis is a benign, autosomal dominant sclerosing dysplasia of bone characterized by the presence of numerous bone islands in the skeleton.
Presentation
The radiographic appearance of osteopoikilosis on an X-ray is characterized by a patt ...
(Buschke–Ollendorff syndrome
Buschke–Ollendorff syndrome (BOS) is a rare genetic disorder associated with LEMD3. It is believed to be inherited in an autosomal dominant manner. It is named for Abraham Buschke and Helene Ollendorff Curth, who described it in a 45-year-old ...
)[
* ]Osteopathia striata
Osteopathia striata is a rare entity characterized by fine linear striations about 2- to 3-mm-thick, visible by radiographic examination, in the metaphyses and diaphyses of long or flat bones. It is often asymptomatic, and is often discovered incid ...
with cranial sclerosis[
* Mixed sclerosing bone dysplasia
* Progressive diaphyseal dysplasia (]Camurati–Engelmann disease
Camurati–Engelmann disease (CED) is a very rare autosomal dominant genetic disorder that causes characteristic anomalies in the skeleton. It is also known as progressive diaphyseal dysplasia. It is a form of dysplasia. Patients typically have h ...
)[
* ]SOST Sost or SOST may refer to:
Places
*Sost, Afghanistan, a village in Badakhshan Province
*Sost, Pakistan, the last town inside Pakistan on the Karakoram Highway before the Chinese border
* Sost, Hautes-Pyrénées, a commune in France
Other uses
...
-related sclerosing bone dysplasias
File:ScleroticmetastaticdiseasespineLCT.png, Sclerosis of the bones of the thoracic spine due to prostate cancer metastases (CT image)
File:Scleroticmetastaticdiseasespine.png, Sclerosis of the bones of the thoracic spine
In vertebrates, thoracic vertebrae compose the middle segment of the vertebral column, between the cervical vertebrae and the lumbar vertebrae. In humans, there are twelve thoracic vertebrae and they are intermediate in size between the cervical ...
due to prostate cancer metastases (CT image)
Diagnosis
Osteosclerosis can be detected with a simple radiography. There are white portions of the bone which appear due to the increased number of bone trabecula
A trabecula (plural trabeculae, from Latin for "small beam") is a small, often microscopic, tissue element in the form of a small beam, strut or rod that supports or anchors a framework of parts within a body or organ. A trabecula generally has ...
e.
Animals
In the animal kingdom, there also exists a non-pathological form of osteosclerosis, resulting in unusually solid bone structure with little to no marrow. It is often seen in aquatic vertebrates
Vertebrates () comprise all animal taxa within the subphylum Vertebrata () ( chordates with backbones), including all mammals, birds, reptiles, amphibians, and fish. Vertebrates represent the overwhelming majority of the phylum Chordata, ...
, especially those living in shallow waters,[Houssaye, A. (2009). "Pachyostosis" in aquatic amniotes: a review. Integrative Zoology 4(4): 325-340.] providing ballast
Ballast is material that is used to provide stability to a vehicle or structure. Ballast, other than cargo, may be placed in a vehicle, often a ship or the gondola of a balloon or airship, to provide stability. A compartment within a boat, ship, ...
as an adaptation for an aquatic lifestyle. It makes bones heavier, but also more fragile. In those animal groups, osteosclerosis often occurs together with bone thickening (pachyostosis
Pachyostosis is a non-pathological condition in vertebrate animals in which the bones experience a thickening, generally caused by extra layers of lamellar bone. It often occurs together with bone densification (osteosclerosis), reducing inner ca ...
). This joint occurrence is called pachyosteosclerosis
Pachyosteosclerosis is a combination of thickening (pachyostosis) and densification (osteosclerosis) of bones. It makes bones more heavy, but also more fragile. The condition often occurs in aquatic vertebrates, especially those living in shallow w ...
.
See also
* Pachyostosis
Pachyostosis is a non-pathological condition in vertebrate animals in which the bones experience a thickening, generally caused by extra layers of lamellar bone. It often occurs together with bone densification (osteosclerosis), reducing inner ca ...
* Pachyosteosclerosis
Pachyosteosclerosis is a combination of thickening (pachyostosis) and densification (osteosclerosis) of bones. It makes bones more heavy, but also more fragile. The condition often occurs in aquatic vertebrates, especially those living in shallow w ...
* Heinrich Albers-Schönberg
Heinrich Ernst Albers-Schönberg (21 January 1865 – 4 June 1921) was a German gynecologist and radiologist. He was a native of Hamburg.
He studied medicine at the Universities of Tübingen and Leipzig, where in 1891 he earned his medical doctor ...
References
External links
Osteopathies
Animal anatomy
Skeletal system
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