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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 generalized increase in bone density has a special predilection to involve the medullary portion with relative sparing of the cortices.EL-Sobky TA, Elsobky E, Sadek I, Elsayed SM, Khattab MF (2016)"A case of infantile osteopetrosis: The radioclinical features with literature update' ''Bone Rep''. 4:11-16doi:10.1016/j.bonr.2015.11.002 Obliteration of bone marrow spaces and subsequent depression of the cellular function can result in serious hematologic complications. Optic atrophy and cranial nerve damage secondary to bony expansion can result in marked morbidity. The prognosis is extremely poor in ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
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 more prevalent conditions like osteoporosis, in which the bones become less dense and more brittle, or osteomalacia, in which the bones soften. Osteopetrosis can cause bones to dissolve and break. It is one of the hereditary causes of osteosclerosis. It is considered to be the prototype of osteosclerosing dysplasias. The cause of the disease is understood to be malfunctioning osteoclasts and their inability to resorb bone. Although human osteopetrosis is a heterogeneous disorder encompassing different molecular lesions and a range of clinical features, all forms share a single pathogenic nexus in the osteoclast. The exact molecular defects or location of the mutations taking place are unknown. Osteopetrosis was first described in 1903, by Ger ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Skeletal Dysplasia
Osteochondrodysplasia is a general term for a disorder of the development (dysplasia) of bone ("osteo") and cartilage ("chondro"). Osteochondrodysplasias are rare diseases. About 1 in 5,000 babies are born with some type of skeletal dysplasia. Nonetheless, if taken collectively, genetic skeletal dysplasias or osteochondrodysplasias comprise a recognizable group of genetically determined disorders with generalized skeletal affection. Osteochondrodysplasias can result in marked functional limitation and even mortality. Osteochondrodysplasias subtypes can overlap in clinical aspects, therefore plain radiography is absolutely necessary to establish an accurate diagnosis. Magnetic resonance imaging can provide further diagnostic insights and guide treatment strategies especially in cases of spinal involvement. Early diagnosis, and timely management of skeletal dysplasia are important to combat functional deterioration. Types Achondroplasia ''Achondroplasia'' is a type of autosomal d ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Hyperostosis
Hyperostosis is an excessive growth of bone. It may lead to exostosis. It occurs in many musculoskeletal disorders. See also * Diffuse idiopathic skeletal hyperostosis * Hyperostosis frontalis interna * Infantile cortical hyperostosis * Porotic hyperostosis * SAPHO syndrome SAPHO syndrome includes a variety of inflammatory bone disorders that may be associated with skin changes. These diseases share some clinical, radiologic, and pathologic characteristics. An entity initially known as chronic recurrent multifocal ... References * * * * External links Gross pathology Osteopathies {{pathology-stub ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Macrocephaly
Macrocephaly is a condition in which circumference of the human head is abnormally large. It may be pathological or harmless, and can be a familial genetic characteristic. People diagnosed with macrocephaly will receive further medical tests to determine whether the syndrome is accompanied by particular disorders. Those with benign or familial macrocephaly are considered to have megalencephaly. Causes Many people with abnormally large heads or large skulls are healthy, but macrocephaly may be pathological. Pathologic macrocephaly may be due to megalencephaly (enlarged brain), hydrocephalus (abnormally increased cerebrospinal fluid), cranial hyperostosis (bone overgrowth), and other conditions. Pathologic macrocephaly is called "syndromic", when it is associated with any other noteworthy condition, and "nonsyndromic" otherwise. Pathologic macrocephaly may be caused by congenital anatomic abnormalities, genetic conditions, or by environmental events. Many genetic conditions are ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Remodeling
Renovation (also called remodeling) is the process of improving broken, damaged, or outdated structures. Renovations are typically done on either commercial or residential buildings. Additionally, renovation can refer to making something new, or bringing something back to life and can apply in social contexts. For example, a community can be renovated if it is strengthened and revived. It can also be restoring something to a former better state (as by cleaning, repairing, or rebuilding). Phases and process of renovations The building renovation process can usually, depending on the extents of the renovation, be broken down into several phases. The phases are as follow. *Project initiation - The beginning of the project that includes the hiring of construction and design team, defining the scope of work, creating a budget, and communicating the needs, expectations, and wants from both the client and building teamKonstantinou, Thaleia, et al. “Renovation Process Challenges a ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Pathologic Fracture
A pathologic fracture is a bone fracture caused by weakness of the bone structure that leads to decrease mechanical resistance to normal mechanical loads. This process is most commonly due to osteoporosis, but may also be due to other pathologies such as cancer, infection (such as osteomyelitis), inherited bone disorders, or a bone cyst. Only a small number of conditions are commonly responsible for pathological fractures, including osteoporosis, osteomalacia, Paget's disease, Osteitis, osteogenesis imperfecta, benign bone tumours and cysts, secondary malignant bone tumours and primary malignant bone tumours. Fragility fracture is a type of pathologic fracture that occurs as a result of an injury that would be insufficient to cause fracture in a normal bone. There are three fracture sites said to be typical of fragility fractures: vertebral fractures, fractures of the neck of the femur, and Colles fracture of the wrist. This definition arises because a normal human being ought to ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Osteosclerosis
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 of bone. Plain radiographs 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, sickle-cell disease and osteoarthritis 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 * Myelofibrosis (primary disorder or secondary to intoxication or malignancy) * Osteosclerosing types of chronic osteomyelitis * Hypervitaminosis D * hyperparathyroidism * Schnitzler syndrome * Mastocytosis Skeletal fluorosis * Monoclonal IgM Kappa cryoglobulinemia * Hepatitis C. Heredita ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
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 pattern of numerous white densities of similar size spread throughout all the bones. This is a systemic condition. It must be differentiated from blastic metastasis, which can also present radiographically as white densities interspersed throughout bone. Blastic metastasis tends to present with larger and more irregular densities in less of a uniform pattern. Another differentiating factor is age, with blastic metastasis mostly affecting older people, and osteopoikilosis being found in people 20 years of age and younger. The distribution is variable, though it does not tend to affect the ribs, spine, or skull. Cause Epidemiology Men and women are affected in equal number, reflecting the fact that osteopoikilosis attacks indiscriminately. Ad ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
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 incidentally. See also * List of radiographic findings associated with cutaneous conditions Many conditions of or affecting the human integumentary system have associated features that may be found by performing an x-ray or CT scan of the affected person. See also * List of cutaneous conditions * List of contact allergens * List of ... References Radiologic signs {{med-sign-stub ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
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 heavily thickened bones, especially along the shafts of the long bones (called diaphyseal dysplasia). The skull bones may be thickened so that the passages through the skull that carry nerves and blood vessels become narrowed, possibly leading to sensory deficits, blindness, or deafness. This disease often appears in childhood and is considered to be inherited, however many patients have no previous history of CED within their family. The disease is slowly progressive and, while there is no cure, there is treatment. It is named for M. Camurati and G. Engelmann. Signs and symptoms Patients with CED complain of chronic bone pain in the legs or arms, muscle weakness (myopathy) and experience a waddling gait. Other clinical problems associate ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Hematopoietic Stem Cell Transplantation
Hematopoietic stem-cell transplantation (HSCT) is the transplantation of multipotent hematopoietic stem cells, usually derived from bone marrow, peripheral blood, or umbilical cord blood in order to replicate inside of a patient and to produce additional normal blood cells. It may be autologous (the patient's own stem cells are used), allogeneic (the stem cells come from a donor) or syngeneic (from an identical twin). It is most often performed for patients with certain cancers of the blood or bone marrow, such as multiple myeloma or leukemia. In these cases, the recipient's immune system is usually destroyed with radiation or chemotherapy before the transplantation. Infection and graft-versus-host disease are major complications of allogeneic HSCT. HSCT remains a dangerous procedure with many possible complications; it is reserved for patients with life-threatening diseases. As survival following the procedure has increased, its use has expanded beyond cancer to autoimmun ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Skeletal Disorders
Bone disease refers to the medical conditions which affect the bone. Terminology A bone disease is also called an "osteopathy", but because the term osteopathy is often used to refer to an alternative health-care philosophy, use of the term can cause some confusion. Bone and cartilage disorders Osteochondrodysplasia is a general term for a disorder of the development of bone and cartilage. List A * Ambe * Avascular necrosis or Osteonecrosis * Arthritis B * Bone spur (Osteophytes) C * Craniosynostosis * Coffin–Lowry syndrome * Copenhagen disease F * Fibrodysplasia ossificans progressiva * Fibrous dysplasia * Fong disease (or Nail–patella syndrome) * Fracture G * Giant cell tumor of bone * Greenstick fracture * Gout H * Hypophosphatasia * Hereditary multiple exostoses K * Klippel–Feil syndrome M * Metabolic bone disease * Multiple myeloma N * Nail–patella syndrome O * Osteitis * Osteitis deformans (or Paget's disease of bone) * Osteitis fibr ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
