Bone illness Pedzheta, part II: Radiological diagnostics, treatment
Radiological changes at BP are usually enough characteristic. Long tubular bones About-shapedly or sablevidno are bent (fig. 1). The bone diameter at a certain level of a diaphysis or throughout all diaphysis is expanded. And утолщены and эпифизы. A bone thickening mainly at the expense of a kortikalny layer. The cortical layer sharply утолщается, loses the uniformity and is presented by the reinforced sklerozirovanny bone beams which are randomly located and being crossed with each other. Between these sklerozirovanny beams the centers of osteoporosis of the wrong form are defined.
Bone illness Pedzheta, part I: Clinical picture>>>
In some cases the bone structure assumes a spotty air at the expense of alternation of sites of osteoporosis and an osteosclerosis. The external contour of a kortikalny layer rather accurate, reaction of a nadkostnitsa is not traced.
The marrowy channel is sharply narrowed at the expense of neogenic bone structures mainly on its internal contour. The spongy fabric эпифизов also gets rough drawing.и the centers of osteoporosis alternate with the osteosclerosis centers.
Joints at deforming osteodystrophy are not involved in process. But the disease can become complicated development of deforming art rose knee or coxofemoral joints. Pathological changes can be the second complication of a disease. They are very characteristic and are called as "banana". In certain cases the pathological change happens the first manifestation of a disease.
The plane of a change settles down perpendicularly to a dlinnik of a bone, a surface bone отломков very equal, with accurate contours. As a rule, it is not observed a considerable divergence отломков. Healing of changes occurs in usual terms. Pathological "banana" changes in rare instances should be differentiated with zones of reorganization of bone structure are unstructured sites in width in some millimeters, settling down also as the change plane, it is perpendicular to a dlinnik of a tubular bone. But they do not cross all thickness of a bone and occupy only a part of a cortical layer and diameter of a bone.
The most dangerous complication of an illness Pedzheta is malignizatsiya possibility. Development of osteogene sarcomas of long tubular bones and flat bones are described in literature. Probably because of an illness development of retikulyarny sarcoma, фибросаркомы, and from good-quality tumors – остеобластокластомы.
On roentgenograms skulls (drawings 2 and 3) it is possible to see the characteristic picture described above.
At BP treatment is required not in all cases. Besides symptomatic therapy, the main medicines now are бисфосфонаты, calcitonins and пликамицин. These preparations are capable to eliminate bone pain, a hyperthermia of fabrics over the struck departments of bones and, the main thing to slow down superfluous speed of a remodelirovaniye bone tkanipokazaniye to application of antirezorbtsionny means are considered:
• Need of relief of painful feelings (caused by a disease, instead of its complications)
• Poliossalny form of a disease
• the mono – and the KBP oligoossalny forms in a case прогностически adverse localization of pathological process (near large joints, in bones of the bottom extremities, in bodies of vertebras, at extensive defeat of a skull with involvement of a temporal bone or the basis) and in the presence of activity of a disease (increase of level of ShchF in blood and/or оксипролина in daily urine)
• Need of the prevention of changes
• Carrying out orthopedic operations (for the purpose of suppression of the increased blood supply of the corresponding area of a bone and elimination of the increased risk postoperative кровопотери); this indication admits not all.
In most cases already arisen complications – deformations of bones, deafness, other kompressionny neurologic complications (A radikulopatiya, a miyelopatiya) – to return development do not give in.
If the amount of bone changes is insignificant and direct threat of development of deformations or neurologic complications is not created, and also there are no painful feelings and biochemical signs of activity of an illness, adhere to waiting tactics.
The effect of antirezorbtsionny therapy is estimated on dynamics of the painful feelings caused directly by KBP, but mainly on extent of decrease in activity alkaline фосфатазы. Analgetichesky action is usually noted in the first weeks whereas "the biochemical effect" develops more slowly, its maximum expressiveness comes to light within 3-6 months.
In recent years the preference is given бисфосфонатам, owing to their selective action on a bone fabric, abilities is long in it to remain and firmness of caused effect. It should be noted that the pharmacology бисфосфонатов continues to develop actively. At least 3 generations of these preparations are now known. Newest of them possess in comparison with the first preparation of this group, etidronaty, in 1000 of times bigger antirezorbtsionny activity. Already at creation бисфосфонатов the 2nd generation (тилудронат, памидронат and алендронат) it was possible to expand "a therapeutic window" – a range between size of a medical and toxic dose that led at a number of patients to emergence of violations of a mineralization of again being formed bone fabric (osteomalyatsiya).
The effect бисфосфонатов at KBP depends on degree of expressiveness of an illness, and also from dose size. It select (it especially concerns бисфосфонатов, applied intravenously) individually, being guided by prevalence of defeat of a skeleton, the pro-gnostic importance of localization of "the pedzhetovsky centers", nature of radiological changes (prevalence of signs of a bone resorption indicates need of more intensive therapy), extent of increase of biochemical markers of activity of a disease.
In total бисфосфонаты, accepted inside, have an essential shortcoming – bad, unstable absorbability (in total about 1-10 % from size of an accepted dose) and essential negative influence of a number of foodstuff on it and even drinks. It compels to recommend to take бисфосфонаты for 30 mines before food intake (usually before a breakfast) and not to use in the next few hours dairy products, calcium and iron preparations. For prevention "chemical" эзофагита recommend to wash down also a preparation not less than 100 ml of water and to be in vertical situation about 30 minutes. It is especially important in case of reception этидроната and алендроната and it is less essenti
al when using бисфосфоната the 3rd generation резидроната.
More successful results in treatment of KBP are reached at intravenous introduction бисфосфонатов. Treatment usually carry out courses, duration of each of them makes from 3 to 6 months. Concerning intravenously applied бисфосфонатов there is an opinion that the total dose, instead of duration of influence has bigger value. By 6th month of treatment when the maximum "biochemical" effect of the chosen mode of therapy usually comes to light, bring a preliminary result. After that it is accepted to do a break in treatment, regularly (once a month) supervising biochemical indicators of speed of a remodelirovaniye of a bone fabric. The indication to carrying out a repeated course considered renewal of bone pains and/or new increase of activity ShchF exceeding level reached earlier for 25 %.
At a number of patients resistance to an applied preparation which should be overcome either dose increase, or purpose of another бисфосфоната over time develops. There are patients at whom and by means of new бисфосфонатов it is not possible to reach normalization of biochemical markers of activity of a disease, but at many of them level of these indicators nevertheless decreases or decreases more than at treatment by "old" preparations.
The main undesirable action бифосфонатов at appointment them inside is "irritation" of the top departments of a gastroenteric path, including erozivny эзофагит, and at intravenous introduction – the short-term fever, being accompanied a fever, and mialgiya (so-called ostrofazovy reaction which connect with increase of level of Interlaken 6 in blood).
Though efficiency of calcitonins at BP is comparable with results of application этидроната, them now use only according to limited indications. It is caused by absence at preparations of a calcitonin of effect of postaction (they do not collect in a bone fabric) and possibility of development of secondary resistance (according to survey data, frequency of this phenomenon makes from 5-6 to 40 %). To calcitonins prefer only when there are expressed signs остеолиза, menacing with development of fractures of bones, and also if necessary to gain faster effect: at severe pains, emergence of the first signs of neurologic frustration, warm insufficiency.
Plikamitsin (the former name митрамицин) treats group of antineoplastic antibiotics. It is supposed that this preparation has the selective and expressed effect on остеокласты. It is characterized by powerful and quickly coming effect. Analgetichesky action comes in some days. After 10-day plan of treatment level of ShchF decreases approximately for 60 %, the next 1-2 months "the biochemical effect" accrues and remains from several months to several years. Degree of expressiveness of this influence depends on size of a dose and frequency of introduction of a preparation.
In the general opinion, пликамицин it should be applied at KBP as a reserve preparation in the most hard, resistant cases or when it is necessary to reach effect as soon as possible (for example, at recent development of a compression of nervous trunks). It is caused by gepatotoksichesky and nefrotoksichesky action of a preparation, and also possibility of development of thrombocytopenia.
Plikamitsin is applied intravenously (in the form of long infusions) in a dose of 10-15-25 mkg/kg within no more than 10 days. The bolyusny way of introduction (on 25 mkg/kg each 2-3 weeks) is known.
On materials: N. V. Bunchuk the Deforming osteitis: in 100 years after J. Pedzheta of RMZh, Tom of 9 No. 7-8, 2001
Drawing 1: 4.bp.blogspot.com
Drawing 0, 2,3: personal collection
Drawing 4: sumerdoc.blogspot.com