Parathyroid osteodystrophy

Parathyroid osteodystrophy (synonym: the disease of Recklinghausen, disease Engel-Recklinghausen, generalized osteodystrophy generalized fibrous cistovicka osteodystrophy osteodystrophy fibrous generalized, octet fibrous, primary hyperparathyroidism) is a disease of the endocrine origin arising from the development of tumors of the parathyroid gland and accompanied by excessive formation of its hormone.
The etiology and pathogenesis. Parathyroid osteodystrophy known as bone disease over 100 years. The disease often described as osteomalacia, cystic degeneration of skeletal bone atrophy, and some observations and as Paget's disease. On the basis of the inflammatory nature of the disease, Recklinghausen (F. Recklinghausen) called it fibrous asticom (ostitis fibrosa). Only later it turned out not inflammatory, and degenerative nature of the process and the term "fibrous octet" was replaced with the term "osteitis fibrosa".
The abundance of synonyms parathyroid osteodystrophies, much of which are currently out of date, reflects the evolution gradually emerging correct ideas about the disease. The assumption that the primary nature of the tumor process in the parathyroid gland (see) and the secondary character bone changes in this disease for the first time expressed Schlagenhaufer (F. Schlagenhaufer). Only in 1924 Century A. Rusakov postmortem studies have substantiated endocrine nature of this species osteodystrophies (see) and suggested to call it parathyroid osteodystrophy, and Mandl (F. Mandl, 1926), removing the tumor of the parathyroid gland in this disease, achieved clinical cure.
The difficulties of obtaining experimental model of parathyroid osteodystrophies introduction to the blood of animals of parathormone, calling them a picture similar to the disease Recklinghausen, but not quite identical with it, and have reason to believe that the basis of the pathogenesis of parathyroid osteodystrophies, except the surplus of parathormone, there are other, as yet not been studied factors. Undoubtedly established that primarily occurs adenoma, rare cancer of the parathyroid gland, and the subsequent osteodystrophies changes associated mainly with mineral metabolism disorders, develop secondarily in connection with hormonal activity tumors, the surgical removal of which can lead to clinical recovery.
P. O. affects mainly women (approximately 3 times more often than men). Usually the clinical manifestations of the disease are observed in the age of 30-40 years and only sometimes occur in younger or older people.
Pathological anatomy. The tumor usually develops in one of the parathyroid glands, often (25%) is atypical localization - the sternum in the front, the posterior mediastinum, in the thick of the thyroid gland, behind the esophagus, trachea, that is, in places, corresponding to the area of location of the parathyroid glands during embryogenesis. This fact is seen as proof dysontogenetic origin of the tumor.
Tumors of the parathyroid gland usually in the form of a clearly demarcated site weighing from 0.1 to 10 grams or more. Microscopically the tumor is often characterized by the predominance of cells resembling main dark small or large cells normal gland, less frequently, the presence of cells type light or axifile. Often tumor cells cannot be attributed to one or another type of normal cells to cancer, which is very important for differentiating tumor from her hyperplastic processes. Depending on the cellular composition of hormonal activity adenomas different, as a result of clinical-anatomic manifestations of the disease varies from rapidly progressive forms to slow running with minimal symptoms.
Under the influence of the hormone secreted by the tumor cells, increases sharply intraosseous metabolism is, resulting in the rebuilding of bone. Therefore, the morphological picture of the disease at the forefront are the defeat of the skeleton.
Macroscopically bones may appear unchanged, which is typical for a little gormonalnogo tumors or for the initial phase of the disease, in other cases, there is a deformation of the skeleton, especially bones, under heavy mechanical load - bones of the extremities, pelvis, spine, ribs. Sometimes, in addition to the mechanical deformation of origin in the bones develop a kind of multiple-like formation.

Fig. 1. Osteoclastic resorption of a compact layer of the bone.

Fig. 2. Overgrown endast between primitive bone beams.

Microscopic examination marks the reconstruction of bone tissue, reflected in massive osteoclastic resorption of bone (Fig. 1) in some areas and the growth in others. The most active of these processes is expressed in antistaling departments bones, where is growth of angosta and education cellular-fibrous tissue that fills bone marrow space and resembling fibrous tissue. This fabric is a sprawling endast and is osteogenic. It formed complexes of new bone beams (Fig. 2). The latter in turn are resorbed and replaced with new ones, and the rapid pace of the process of newly formed bone gets a primitive structure, sometimes it is not obesesluts and then osteopenia beams and osteopenia zone arise around old beams.

Fig. 1. Humerus with giant cell tumors and cysts.

Like formation in the bones (printing. Fig. 1) occur along with diffuse their restructuring. Macro - and microscopically identical with brain tumors - osteoblastoclastoma (see the Bone). They are the result of a sharp intensification of bone regeneration process and being reactive structures disappear after removal of tumors of the parathyroid gland as well as other manifestations of the disease.
In the process of restructuring bones released a large amount of calcium salts. These salts partially fall in the bones in the form of granular sediments, partially enter the bloodstream, causing the so-called lime metastases (see Calcification). Lime salt, standing out with the urine, can lead to the formation of kidney stones, as well as to the excretory principally with the subsequent atrophy of the kidneys. In some cases, the defeat of internal organs may dominate the picture of the disease and bone system recedes into the background (the so-called visceral form of parathyroid osteodystrophies).
Over and symptoms. In the relatively early stages of parathyroid osteodystrophies patients complain of deaf uncertain bone pain normally associated with the change in the weather, General muscle weakness, fatigue, headaches. There are also loss of appetite, nausea (rarely vomiting, abdominal pain, accompanied by intestinal disorders (more persistent diarrhea). Almost constantly patients have an increased thirst, and therefore it is noted polyuria. Sometimes on the first plan from the very beginning of the disease are the symptoms of kidney stones. In these cases, x-ray sets the presence of nephrolithiasis, usually bilateral.
Often the patients themselves find various deformations: thickening, bumpy, mainly in the field of metamizol long bones, or curvature, for example the upper third of the thigh, leading to the shortening of the limbs and limp. Often bumpy can be found on the skull. The defeat of the skull and facial bones leads to an asymmetry of the face. Sometimes the disease begins with Apulia (see) the lower or upper jaw, which causes the patient to seek help from a dentist.
There are cases when clinical manifestation of parathyroid osteodystrophies begins with pathologic fracture (thighs, legs, upper extremities, ribs), resulting from relatively minor injuries. A fracture usually fused (usually offset) and not enough qualified chest x-ray can not be regarded as pathological, but as an ordinary traumatic fracture. History can be and multiple fractures. If in the initial stages of the disease painful fractures, further pathological fractures can occur unnoticed and proceed almost painless. As a result of deformation of the spine can cause compression of the roots of the spinal cord and as a result of the compression - pain, paresis, and paralysis. Salt deposition of lime in the eardrum or the defeat of the pyramid of the temporal bone can lead to hearing loss. Sometimes revealed disturbances in connection with optic atrophy and sclerotic changes in the fundus.
In connection with negative balance of calcium and phosphorus, strongly mobilized from bone depot and removed from the body mainly kidneys and partly through the mucous membrane of the large intestine, the laboratory examination of urine marked hypercalciuria and hyperphosphaturia. Along with it in the blood in biochemical studies is determined hypercalcemia. The content of calcium in the serum instead of the normal 9-13 mg% increased to 15-17 mg%. The phosphorus content in the serum falls instead of the normal 3-4 mg% to 1.5-2 mg%. These laboratory and biochemical parameters do not depend on the amount of salts, entered into an organism with food, and are of great diagnostic value.
Except in rare cases, the turbulent flow of parathyroid osteodystrophies, accompanied by massive getting the blood of parathormone and ends lethally, before you have time to develop expressed bone changes, the clinical picture of hyperparathyroidism is usually develops slowly over many years, and bone changes are limited to the first General (systemic) osteoporosis.
In the course of the disease is possible remission, accompanied by the improvement of the General health, reduce pain, temporary restoration of forces and health and the normalization of the urine and blood. However, after some time the disease, as a rule, again progresses steadily develop characteristic dystrophic cystic changes in the bones, sharply exacerbated pain in the legs, pelvis and spine, increasing muscle weakness, appear moderately expressed secondary anemia, tachycardia, cardiac weakness. Progressive deterioration of the General condition leads patients to disability, attracts them to the bed, and in the absence of radical surgical treatment of patients die when events cachexia and marasmus. Cases of healing in this disease is not described. There are indications as of the beginning and subacute disease and significant deterioration of its course in connection with pregnancy, childbirth and breastfeeding.

The diagnosis. Recognition of parathyroid osteodystrophies based on data of clinical, laboratory, biochemical and x-ray examinations, each of which by itself at different stages of the disease may not have crucial significance and only their combination in the process of dynamic observation provides a correct diagnosis.
Discover enlarged parathyroid gland feeling neck rare. No less difficult is the recognition of the parathyroid adenoma cancer by x-ray upper anterior mediastinum with contrast of the esophagus barium sulfate or gland arteriography. The importance of the above changes urine, which in periods of exacerbation may be so with lime, which is similar to milk and biochemical shifts in the serum of the blood (hypercalcemia and hypophosphatemia).
The data of x-ray examinations are crucial only in clearly defined cases, parathyroid osteodystrophies, accompanied by restructuring and characteristic deformation of bones. In other cases, reliable x-ray data or absent, or so poor and unconvincing that the correct x-ray detection becomes a Fluke. You should make it a habit of the need for follow-up research entire skeleton upon detection of an isolated bone cyst or suspected pathological character of the fracture. The lack or insufficiency of radiographic signs indicating the presence of parathyroid osteodystrophies, does not give grounds for exceptions.
Increased allocations from the body of mineral salts in connection with hyperparathyroidism is characterized radiographically first evenly grainy osteoporosis, especially clearly manifested in the bones of the cranial vault and pelvis. Further longitudinal separation core substance, mainly bones, and cystic rebuilding of bone skeleton, especially pelvis, hips and other bones. Many, gradually increasing in size, expansionist cysts press on cortical substance, its thinner, pumps and deformed bones.
Often, in addition to the fusiform deformations arise static deformation of character, which contributes to reduced bone strength. The result of this kind of deformation occurs arched curvature tubular bones, and the entrance to the pelvis so tapering that can take the form of so-called card hearts (Fig. 3). Especially characteristic deformation of the hip, which takes the form of so-called shepherd's stick (Fig. 4), and deformation of edges, giving the chest resemblance to the shape of the bell.

Fig. 3. Deformation of the pelvis with parathyroid osteodystrophies, narrowing the entrance to the pelvis in the form of the "card of the heart."

Fig. 4. (right) a Characteristic pattern of deformities of the femur in the form of "shepherd's stick" when parathyroid osteodystrophies.

Differential diagnosis. First of all it is necessary to exclude other types osteodystrophies, in particular Paget's disease (see Actos deforming)that hits more often than men. However, when the disease is observed different clinical picture, and a significant disfiguring skeletal deformities are accompanied by a seal of the affected bone without such a characteristically expressed razvlecheniya and cortical thinning bones, as if parathyroid osteodystrophies. Unlike isolated bone cyst, reminiscent of cystic changes in P. O., the latter is a generalized character.
Since the first x-ray characteristic of parathyroid osteodystrophies is systemic osteoporosis (see), it is necessary to distinguish it from other types of systemic osteoporosis, in particular senile.
When osteomalacia (see), which is the same as P. O., affects mainly women and accompanied initially dull aching pain in the bones and systemic osteoporosis, unlike parathyroid osteodystrophies, no specific changes in the serum of the blood (hypercalcemia, hypophosphatemia) and, despite systemic osteoporosis, says the election localization of lesions in the skeleton depending on some form of osteomalacia (puerperal, old age, youth). In more severe cases, and other diseases accompanied by significant deformations of bones, recognition P. O. differs in that racemose changes and thickening of bones at osteomalacia are missing, and monitored area perestroika Looser at P. O. (Fig. 5) are less pronounced than with osteomalacia.
It should also exclude myeloma (see), which offers more acute and malignant. Multiple characteristic perforated bone defects in multiple myeloma are not accompanied by their transformation and deformation. Marked proteinuria - positive reaction Bence-Jones (see Bence-Jones protein) - in the absence of hypercalcemia and hypophosphatemia.

Fig. 5. Zone of perestroika Looser in the elbow bones when parathyroid osteodystrophies.

Fig. 6. Deformation of the tibia, remaining after successful parathyroidectomy.

Differentiation of parathyroid osteodystrophies from multiple metastases of breast, lung, thyroid and prostate glands contribute anamnestic data and discovered the primary tumor. Primary osteogenic sarcoma has typical destructive changes affected bone, a typical reaction from the side of periosteum, missing when P. O., the local nature of the injury without the typical biochemical changes in the mineral balance.
Forecast when parathyroid osteodystrophies depends on timely surgical treatment. Removal of a parathyroid adenoma gland leads to termination of the pathological process and vocational rehabilitation. Over time, the bone structure is restored, but the deformation of the affected bone remains (Fig. 6).
The treatment is surgical removal of the prostate gland affected the parathyroid gland. Conservative treatment is inefficient. When relapse after insufficiently complete removal of the prostate gland shown radiation therapy.