Osteoarthropathy with hyperparathyroidism ( hyperparathyroid osteodystrophy )

main causes of development and types hyperparathyroid dystrophy (Engel's disease - Recklinghausen), the mechanism of formation of changes in the bones and joints and the main clinical manifestations of the disease.

Increased production of parathyroid hormone leads to the emergence of the state, called hyperparathyroidism, one manifestation of which is the development of a variety of changes in the musculoskeletal system in the first place - in the bones and joints.

These changes are called hyperparathyroid osteodystrophy, or Recklinghausen's disease (a disease also Engel - Recklinghausen, on behalf of two German doctors, who described it in the XIX century - G. Engel and F. D. Recklinghausen).

causes and mechanism of development giperparatireoidoy dystrophy

Most often the disease occurs in young women.

By origin distinguish:

  • primary hyperparathyroid d
    ystrophy - it occurs when increased production of parathyroid hormone (often this is due to parathyroid adenoma);
  • secondary hyperparathyroid dystrophy - it occurs when insufficient flow of the blood electrolytes (mainly calcium and phosphate salts), the parathyroid glands try to compensate for this deficit, which often leads to their hyperfunction and hypertrophy (this form occurs more rarely observed in lowadmission minerals from food, while their lack of absorption due to diseases of the digestive organs, as well as enhanced excretion of electrolytes in the urine).

Yet the most common cause of this disease is one of adenoma of the parathyroid glands (in humans they usually four, and they are located on right and left two - close to the right and left lobes of the thyroid gland).In this case, the bone tissue is microfocal (lacunary) resorption of bone beams, their replacement by fibrous tissue and small cystiform formations (the so-called "brown tumors").

Increased production of PTH leads to increased resorption process - bone resorption (normal bone formation and destruction are balanced) and, accordingly, - electrolyte imbalance in the body.

This is evident:

  • the presence of high calcium in the blood (hypercalcemia) and removing it from the body in the urine (hypercalciuria);
  • decrease in blood phosphorus (hypophosphatemia) and increased its allocation in the urine (giperfosfaturiya).

Allocation calcium and phosphate occurs via the kidneys, so often Recklinghausen disease accompanied by symptoms urine acid diathesis (MCD), urolithiasis (IBC) and the deposition of salts directly into the renal parenchyma (nephrolithiasis).

much less in the removal of calcium salts are involved organs of the gastrointestinal tract, however sometimes possible to develop and gallstones (cholelithiasis).In some cases, deposits are formed (also referred to as calcium or lime metastases) in the soft tissues of the body and the secondary chondrocalcinosis - deposition of calcium salts in cartilaginous tissue.

main clinical manifestations of the disease Recklinghausen

One of the earliest manifestations of the disease is gradually increasing muscle weakness, poorly localized bone pain, fatigue.Over time there are pains in the joints (arthralgia) and spine, their increased meteosensitivity, women's health may significantly deteriorate during periods of hormonal changes the body, especially during pregnancy and breastfeeding.

Over time, the increasing weakness of ligament-muscular system begins to be accompanied by the defeat and para-articular tissues (ligaments and joint capsules).This leads to the restriction of movement of the patient, bed rest.Physical activity can lead to the appearance of pathological fractures in bones and the spine.

Simultaneously, patients worried about heart (pain, tachycardia, dyspnea), constant thirst and frequent urination (polyuria), as well as symptoms of uric acid diathesis (with increased allocation of salt crystals), the development of urolithiasis.

With long-term existence of the disease is marked bone curvature and deformity of the chest and spine.

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