Osteosclerosis

Introduction Osteosclerosis refers to an increase in bone density with a slight change in shape. Delayed osteosclerosis occurs in childhood, adolescence or early adulthood. Albers-Schonberg disease is strictly referred to as autosomal dominant, delayed, delayed, and benign type of osteosclerosis. The geographical and ethnic distribution of the disease is relatively extensive. The affected patient may be asymptomatic or may be diagnosed by accidental findings on X-ray examination for other reasons. Face, physique, intelligence and normal life, health is generally unaffected. Occasionally, facial paralysis or deafness occurs as the cranial nerve is compressed by overgrown bones. Mild anemia is a rare complication.

Cause

The cause of osteosclerosis is not clear, and may be related to abnormal bone resorption, resulting in excessive deposition of calcium salts in the bone. The appearance is marble or ivory, and the brittleness is increased. The disease has a family history and is more common among children who are married to close relatives. Some people think it is a hereditary disease. The disease is divided into two types, light type is dominant inheritance, and heavy type is recessive inheritance.

May be congenital dysplasia, mainly bone resorption disorders during bone development, causing massive deposition of calcium salts and causing bone sclerosis.

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Delayed osteosclerosis occurs in childhood, adolescence or early adulthood. Albers-Schonberg disease is strictly referred to as autosomal dominant, delayed, delayed, and benign type of osteosclerosis. The geographical and ethnic distribution of the disease is relatively extensive. The affected patient may be asymptomatic or may be diagnosed by accidental findings on X-ray examination for other reasons. Face, physique, intelligence and normal life, health is generally unaffected. Occasionally, facial paralysis or deafness occurs as the cranial nerve is compressed by overgrown bones. Mild anemia is a rare complication.

At birth, bone X-rays are usually normal, and as the childhood progresses, the bone hardening becomes apparent. Skeletal involvement is extensive but spotted, with osteoporosis at the extremities. The cranial parietal bone is dense and the paranasal sinus is occluded. The spine vertebral endplate hardening bulge has a "rugby jersey"-like shape. Some patients require blood transfusion or splenectomy for anemia.

Premature osteosclerosis (autosomal recessive, precocious, malignant, congenital osteosclerosis in infants) is an uncommon, potentially fatal disease. Excessive bone growth leads to abnormal bone marrow function and manifests various symptoms including poor growth, spontaneous ecchymosis, abnormal bleeding, and anemia. Progressive hepatosplenomegaly, ocular nerves, oculomotor nerves and facial nerve spasm appear in the later stages of the disease. In the first year of life, anemia, an uncontrollable infection, or bleeding and death often occur.

Increased bone density throughout the body is a significant radiological feature. The penetrating radiograph of the long bone reveals a line of growth barrier in the epiphysis area with longitudinal stripes. As the disease progresses, the ends of the long bone, especially the proximal end of the tibia and the distal end of the femur, form a "flask" shape. In the vertebrae, pelvis and tubular bones, there is bone osteogenesis, the skull is thickened, and the spine is like a "rugby shirt".

Bone marrow transplantation has produced excellent initial results for a small number of babies, but long-term results are not known.

Osteosclerosis with renal tubular acidosis is an autosomal recessive hereditary disease that produces weakness, short stature, and poor growth. Bone radiology is dense, showing renal tubular acidosis and loss of red blood cell carbonic anhydrase activity.

Dense bone dysplasia is an autosomal recessive hereditary disease that has a short stature in early childhood; adult height does not exceed 150 cm. Other manifestations include large heads, short and wide hands and feet, malnourished nails, and blue sclera, which are usually recognized in infancy. The affected patients are very similar, with small faces, retracted lower jaws and molars and wrong jaws. Skull bulging, anterior and posterior foramen. The nails are short and the nails are poorly developed. Pathological fractures are complications of dense bone dysplasia.

Differential diagnosis

Where infants and young children have severe anemia, hepatosplenomegaly (especially splenomegaly), bone marrow puncture can not be successful, should consider this disease. Skeletal x-ray examination has a definitive diagnostic significance, but it cannot be simply diagnosed as osteosclerosis based on the increase in bone density. It should be differentiated from other bone diseases with prominent bone sclerosis.

(1) Lead, phosphorus, antimony or fluorosis

According to the history and other symptoms of poisoning and laboratory tests can be identified. These patients have a history of occupation and the extent of the lesion is relatively limited. Although symmetrical parts can occur, the linear shadows shown are not concentric. The bone manifestation of phosphorus poisoning is more similar to that of osteopetrosis. Sometimes there are layered textures in the long bones, but the dental symptoms, mandibular necrosis and osteomyelitis are obvious, which helps to distinguish the disease.

(2) sclerotic deformity osteitis (paget disease)

More common in the elderly, for multiple bone sclerosis changes, often involving the skull and long bones. The typical head is enlarged, the face is small, the spine is bent backward, and the lower limbs are thickened to form a forward and outward bending deformity. Osteoarthritis often has osteoporosis and rough cortex before bone sclerosis occurs. Alkaline phosphatase is increased in malformation osteitis, which is helpful for identification.

(3) osteochostosis (ost, eopoikilosis)

The lesion occurs in the cavernous bone and has nothing to do with the cortical bone and articular cartilage. Easy to occur in the pelvis, short and straight bones of the hands and feet, long bones and bone ends. A limited rounded dense spot.

(4) myelofibrosis (osteomyelofibrosis)

In the case of anemia or leukemia, this disease is complicated, and it is difficult to distinguish from osteosclerosis on x-ray examination. Because the calcium salt is too much in the cancellous bone, the bone marrow cavity is occluded and the general bone density is increased. However, it can be identified by blood and bone marrow puncture.

(5) Dense osteogenesis imperfecta (pycn() dysostosis)

Osteoclastosis is the 22nd recessive genetic disease caused by the loss of autosomal short arms. The x-ray showed osteosclerosis, often combined with clavicle and apical dysplasia. The patient's appearance characteristics: short body, large head, small face, hook nose, small jaw, dull jaw angle, tooth misalignment, short phalanx and nail deterioration. Pediatric sputum closure late, generally no anemia and cranial nerve compression symptoms.

(6) progressive physiology dysplasia (progressivediaphysealdysplasia)

Limb pain, muscle atrophy, mild dysfunction often associated with the nervous system or endocrine system, can also die from anemia. Under the x-ray, the progressive intima and sub-inferior epiphyseal hyperplasia of the middle part of the long bone can be seen, and the medullary cavity is narrow. This disease does not invade the epiphysis and metaphysis.

(7) sclerosing bone disease (osteosclerosis)

The difference with osteopetrosis is that the patient's body is tall, the amount and jaw are large, the second and third are deformed, the distal phalanx is degenerated, the nail is degenerated, the deafness, the headache, and the hemifacial spasm. Can die from increased intracranial pressure or cerebral palsy. The X-ray is mainly characterized by osteosclerosis of the skull, mandible and long bones.

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