Bone illness Pedzheta, part I: Clinical pictureIn 1877 English surgeon and pathologist James Pedzhet. has published article about 5 patients with skeleton defeat, mainly a skull and long bones of the bottom extremities, being accompanied their thickening, a softening and deformation, having called this disease a deforming osteitis, assuming that in its basis the inflammation of a bone fabric lies. By 1882 he observed already 23 cases of an illness and has in detail described not only a clinical picture, but also section data.

Besides bone an illness Pedzheta (other names - deforming остоз, a deforming osteopatiya), 2 more diseases are connected with his name: cancer Pedzheta (an ekzemopodobny cancer of a mammary gland), and also Pedzheta-Shretter's syndrome (sharp thrombosis of a podklyuchichny vein).

Illness Pedzheta (BP) - the acquired chronic disease caused by local violations of a remodelirovaniye of a bone fabric. Characteristic feature of BP is locality of defeat of a skeleton. At the BP monoossalny form of change are noted in any one bone.

At defeat of several bones pathological process is localised, as a rule, asymmetrically. Most often (in an order descending on frequency) the lumbar department of a backbone, brain department of a skull, a basin, femoral, big tibial and humeral long tubular bones are surprised. Less often changes come to light in bones of the top extremities, clavicles, shovels, edges, a facial skull, brushes and feet.

Prevalence

Incidence of BP is highest in England (4,6 %), Australia and New Zealand (3-4 %), France (2,4 %), it is slightly less in Ireland (0,7-1,7 %), Spain and Germany (1,3 %) and it is essential below in Italy, Greece (0,5 %) and especially in Norway and Sweden (0,3 %). Extremely seldom KBP meets in the Central Africa (0,01-0,02 %) and is almost unknown in the countries of Asia.

The disease beginning at young age is considered atypical. At radiological research of a skeleton at 4603 died G. Schmorl has established changes typical for BP in 138 (3 %) cases, and D.H. Collins has noted that frequency of a deforming osteitis in old age (after 80 years) reached 10 %. In the prevailing majority of cases the disease is diagnosed after 40 years, about 60 years are more often and is very rare before 25 years, approximately with identical frequency at men and women.

Genetic predisposition to BP, most likely, гетерогенна. Possibility of existence of BP at close relatives is known long ago. By means of isotope scanning of a skeleton it is shown that asymptomatic changes at least at one of relatives of the first degree of relationship come to light very often, approximately at 40 % surveyed. Family and genetic researches have allowed to establish that the risk of emergence of KBP at relatives of the first degree of relationship is increased in 7 times.

Patogenez

Distribution of defeat occurs quite slowly: no more than several millimetres a year, however meet also cases of more intensive progressing. The number of the centres of defeat does not increase over time. It is characteristic that pathological process, as a rule, does not extend on joints. An exception are kresttsovo-podvzdoshny joints which can be completely destroyed by a layer of a neogenic unstructured bone fabric; cases of transition of changes from a vertebra body on a mezhpozvonkovy disk are known also.

It is considered that pathological process is initiated by the strengthened resorption which is carried out остеокластами that leads to kompensatorny increase in formation of a new bone fabric. The superfluous amount of the collagenic fibres which are settling down in different directions is thus synthesised, is not ordered that leads to formation not lamellar as in norm, and coarse-fibered, it is superfluous a vaskulyarizirovanny bone fabric which is naturally formed in the embryonic period, and at mature age is observed only in places of healing of changes and at гиперпаратиреозе. Repeatedly repeating processes of a resorption and a new growth lead to disorganisation of very tectonics of a bone fabric. The bone structure in the defeat centres at KBP reminds a mosaic where zones of the abnormal, again formed fibrous bone fabric, the centres of a lamellar bone and resorption sites are chaotically located. The Mozaichnost of structure is quite often observed on a considerable extent of the struck bone.

Clinical picture

Allocate two extreme options of BP: asymptomatic and clinically strongly pronounced, being characterised resistant complaints and multiple deformations of bones. The asymptomatic option is noted approximately approximately in 25 % of all cases and, it is found usually casually, in the analysis of the x-ray pictures made in other occasion, or in the course of search of explanations of the raised level alkaline фосфатазы.

The Generalizovanny, poliossalny option of pathology meets seldom. Patients, complain of pains in bones and large joints of constant character. At fizikalny inspection remarkable deformations of a skeleton develop: expressed кифоз with shortening of a trunk and About-shaped the bottom extremities, bringing to "monkey" gait, and also increase in the sizes the skulls allowing at first sight to suspect the diagnosis of BP at the elderly patient. At generalizovanny option of a disease it is possible to reveal the unique system manifestation of BP - the warm insufficiency caused by increase in warm emission owing to the raised vaskulyarizatsiya of the changed bone fabric.

The "intermediate" option of KBP for which local, mainly nonspecific complaints and the symptoms, diseases defined by Topeka are characteristic is in most cases observed. The complaints caused by secondary degenerate changes from joints, a backbone, and also skeleton deformations prevail. According to R.D. Altman and B. Collins, the most frequent complaint 290 patients with a deforming osteitis had a pain in the lower part of a back (at 34 % of patients); secondary osteoart roses of coxofemoral joints it is revealed at 30 %, knee joints - at 11 % of patients; deformations of femoral and bolshebertsovy bones are found in 45 % of patients.

At BP neurologic violations quite often develop, especially at skull and backbone defeat. Reorganisation of a temporal bone (and also changes of acoustical stones) brings to deafness, the softening and deformation of the basis of a skull can be accompanied by its uploshcheniye and the bazilyarny invaginatsiya of a backbone leading to a compression of a trunk of a brain, hydrocephaly and to increase of intra cranial pressure. Growth of a bone fabric in the field of handles of vertebras can cause a sdavleniye of spinal backs. Narrowing of the channel of the vertebral artery, being accompanied the corresponding ischemic symptoms is possible. Vypyachivaniye of the body of a vertebra increased in the sizes to the spinal canal can lead to a miyelopatiya, and sacrum defeat - to development of a syndrome of a horse tail.

At BP frequency of fractures of long bones, especially femoral and bolshebertsovy bone raises. Approximately in 30 % of cases of fractures of a femur podvertelny or cervical changes are noted. So-called slot-hole, incomplete changes can precede typical changes. In most cases process of an union occurs without deviations from norm, in usual terms. At treatment of fractures of a neck of a femur often it is necessary to make prosthetics of a coxofemoral joint.

One more feature of BP is possibility of development of sarcomas (mainly osteosarcomas, but also a fibrosarok and a hondrosarok). Sarcomas arise less than at 1 % of patients, developing in old "the pedzhetovsky centres", located in bones of a basin, a femoral
and humeral bone.

At BP it is natural, depending on degree of activity and prevalence of process, raise a daily ekskretsiya оксипролина with urine and activity alkaline фосфатазы (ShchF) in the blood reflecting, respectively, intensity of a resorption and formation of a bone fabric. At patients with most ShchF high sizes (they can exceed normal level at 5-10 time) the poliossalny form of a disease with skull defeat is, as a rule, noted. Regular supervision over these parametres allows to estimate the course of a disease and efficiency of therapy.

The diagnosis of BP is established only by means of a rentgenografiya. Characteristic changes in different departments of a skeleton are described in an exhaustive way, including in domestic literature. For specification of degree of prevalence of KBP the skeleton stsintsigrafiya has rough value.

On materials: N. V. Bunchuk the Deforming osteitis: in 100 years after J. Pedzheta of RMZh, Tom of 9 No. 7-8, 2001

Bone illness Pedzheta, part II: Radiological diagnostics, treatment>>>

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