The experience of rehabilitation the patients under Marfan's syndrome

Chernyshova T.E (Izhevsk State Medical Academy)

Sabirzynova E.R.

Ovchinnikova E.I. ("RHMR MH UR", Kizner, Russia)

Shklyeva L.A. ("RHMR MH UR", Kizner, Russia)

Introduction: The results of 1.5-2 years monitoring of rehabilitation program of 21 men under Marfan's syndrome realization are presented at the stages: in-patient department (HHCI UR "Republican Hospital of Medical Rehabilitation MH UR", Kizner); polyclinic (HHCI UR "Medical exercises dispensary"). Adaptive reserve improvement is registered: psychological, social and biological parts of rehabilitation. Natural factors and 6-months course of chondroitin sulfate have a perspective to include in rehabilitation process.

Key words: connective tissue dysplasia, rehabilitation therapy, prognosis for a rehabilitation.

MS - Marfan's syndrome (СМ)

CTD - Connective tissue dysplasia (ДСТ)

RHMR - Republican Hospital of Medical Rehabilitation, Kizner, the Udmurt Republic (РБВЛ)

SMT-phoresis - electrophoresis with sinusoidally modulated currents. (СМТ-форез)

CT - Connective tissue (СТ)

TPT - Therapeutic physical training (medical exercises) (ЛФК)

RP - Rehabilitation potencial (РП)

AI - Adaptation index (АИ)

LQ - Life quality (КЖ)

HXF - Hydroxyproline free (ГСО)

HXPB - Hydroxyproline peptide bounded (ГПСО)

GAG - Glycosaminoglycan (ГАГ)

SP - Sialoprotein (СП)

CAB - Collagenolytic activity of blood (КА)

NSC - Neutral soluble collagen (НРК)

CSC - Citrate soluble collagen (ЦРК)

Connective tissue dysplasia (CTD) is one of the most intricate problem of not only internal diseases clinic, but also rehabilitation medicine. Inspire of successes obtained and Russian Ministry of Heath recommendations was accepted in case of CTD (2008), a main aspects of that problem still disputable. Usage of nature factors in a usual climatic conditions is a forward looking method in rehabilitation medicine optimization. "The rehabilitation program of patients with CTD" was designed by Izhevsk State Medical Academy and tested in Republican Hospital of Medical Rehabilitation (RHMR). The main therapeutic factors in RHMR are balneotherapy and pelotherapy. Mineral water of RHMR is a kind of magnesium sulphate-calcium waters contains active organic substances, silver. The water is used to drink, to take bath, to take inhalations, bowels irrigation and SMT-phoresis. The RHMR unique natural factor is pelotherapy. There is a high concentration of organic substances in Kizner muds: average catalase is 3.25mmole, hydrogen peroxide is decomposed for 2.5 hours, polyphenol oxidases - 2.42mg/g pyrocatechol for 30 min., proteases - 0.11 in mg/g amine nitrogen. Peloids are supplied with biological active substances including valuable micro-stimulators by overactivity of soil microorganisms. There is also high concentration of chemical substances which are take part in CT metabolism: magnesium, zinc, copper. It has been proved that peloids restore not only blood microcirculation and blood rheology, but also immune status. The emphasis at the stage "Polyclinic" ("Policlinika") was taken to naturtsialny development factors of CTD correction, different methods of TPT, swimming. This stage was realized in Republican Medical Exercises Dispensary of the Udmurt Republic.

Research objective: estimation of adoption in practical medical rehabilitation therapy programs for patients under Marfan's syndrome on the stage: RHMR-polyclinic and substantiation of it clinic effectiveness.

Material and methods: There are two stage of the research: estimation of RHMR's rehabilitation therapy effectiveness at the satge "Polyclinic" ("Policlinika") over a period of 1.5-2 years. The rehabilitation results of 21 men at the age of 17-36 with verified Marfan's syndrome was presented. Twenty, practically healthy, men at the age of 20-26 had formed a comparison group. Rehabilitation therapy at the stage "RHMR" has been taken 14 days and included: dietotherapy (magnesium diet with high protein has been devised and certificated), 5mg/kg warm mineral water maneuver at 30-45 min before a meals three times a day, TPT and mud therapy. Kind of pelotherapy depend on functional state of cardiovascular system and has been applied in mud pack (mud wrapping), mixing mud and mineral mater 1:4 ratio, SMT-phoresis and local mud applications. Medicamentous therapy include: adaptogens, magnesium drugs. There was enteral introduction of chondroprotectors: 0.5g a day, and ointment and electrophoresis applications. For 12 patients, which have found painful osteoarticular syndrome against the background of massage and TPT, were additionally aneurine treated in aggregate with neuridiclovit.

Rehabilitation potential (RP) was estimated by adaptation index (AI), physiological tests results and cognitive potential P300. Method SF-36 was used to determined life quality (LQ) ratio as social component of RP. CT metabolism was determined by hydroxyproline level: free (HXF), peptide bounded (HXPB); glycosaminoglycan (GAG), sialoprotein (SP), collagenolytic activity of blood (CAB), neutral- and citrate soluble collagen (NSC, CSC). All patients has been estimated by Oxford - 12 item knee score, degree of scoliosis, joints hypermotility (Brighton criteria) in dynamic.

A program package "Statistica 5.0" was used for data handling.

Results and results discussion: The main characteristic of LQ on the following scales: physical activity, psychological state and sexual activity was decreased (p<0.05). Chronic stress, sedentary or stressed life was registered as one of the cause LQ decreasing. Predominance of negatively stained forms of emotional response with "decision-making" dyspraxia (p<0.05) and psychoemotional deconditioning was detected. In the same time memory f unction (p<0.001) and information processing (p<0.01) ratio, which were high compare to comparison group, was registered. An enthusiasm to rehabilitation program among the main group of patients was shown. Non of them didn't quit the program during test period. During medical process all patients noted amelioration of general condition as well as psychoemotional conditions. We suppose it connected with appointment for hospitalization which is considered as "understanding of their problems by RHMR's physicians" and also group therapy possibility in the first place. At the stage of ambulant rehabilitation significant change of mental state, social and role functional in a positive way was registered. An amelioration of LQ according to the analysis of LQ criteria and rehabilitation composite score (1-3-6 months) was noted.

LQ impairment was connected with intermittent pain syndrome (arthralgia, lumbar ischialgia, dorsopathies etc.). Positive dynamics of pain syndrome was registered among a group of patients where chondroitin sulfate and neurodiclovit in rehabilitation process were used. Total average segment according to Oxford - 12 item knee score scale reduced on 21.3% (p<0.05), and on 78.7% (p<0.001) after 6 months.

Positive dynamics of all indexes was kept at the stage of ambulant rehabilitation. Correlation between LQ and phycological state and RP were r=0.68 and r=0.81 (p<0.001) respectively. During rehabilitation in RHMR CT metabolism evaluation have been registered collagen metabolism upward trend with dynamics only HXF/HXPB (p<0.05) coefficient, which is shown a dynamic equilibrium of biosynthesis and collagen destruction evaluation. A valuable NSC concentration in blood (p<0.01) as well as CAB have been decreased after 2 months of therapy, which are considered as maturation of collagen has been improved. CSC level was rehabilitated (p<0.001) at 5-8 months fr om beginning of the rehabilitation as confirmation of that process. A proteoglycan level was risen from 18.4 +/- 4.9 micromole/l (before admission to RHMR) to 36.8 +/- 18.9 micromole/l (lower bound lim it) after discharge. Concentration of proteoglycan level has been increased till 41.2 +/- 13.8 micromole/l (p<0.05) among those 12 patients (from 14) who continued took chondroprotectors during out-patient treatment after 2 months. An improvement of CT metabolism goes with an increasing patients' LQ and with an social component improvement in the first place. Chondroprotectors under MS have so much effectiveness because it take part in construction of cartilaginous and tissue base material, and hyaline- and fibrocartilage metabolic process improvement. The data we reached after research is shown us that a rise of proteoglycans biosynthesis reflects a cartilage bag and cartilage surfaces of the joints regeneration improvement with a synovial fluid production increasing.

Summary: We have observed work defense mechanisms increasing and elasticity improvement as well as biologically full collagen maturing process during purposeful and long-term rehabilitation therapy under MS with magnesium drugs and chondroprotectors. Natural resources addition to a rehabilitation program is also prospective.

1. Zemtsovskiy E.V. "Undifferentiated connective tissue dysplasia. Condition and development prospects of hereditary connective tissue disorders" / Zemtsovskiy E.V "Connective tissue dysplasia", 2008.-#1.- p. 5-9.

2. Rebrov V.G. "Vitamins and microelements" /Rebrov V.G., Gromova O.A. - Moscow: Alev-V, 2003. - p. 670.

From the article in journal "Health, demography, ecology of Finno-Ugric people" #2, 2012 - special edition, editor-in-chief: Strelkov N.S. (ISSN 1994-8921)