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Table 3. - Bougienage schemes in compliance with patient's age
Age Number of Gegar's bougie
1-4 months 12
4-8 months 13
8-12 months 14
1-3 years old 15
3-12 years old 16
Older than 12 years old 17
Bougienage scheme
1. No more than 1 time per day during a month.
2. Once per 3 days during a month.
3. Twice per week during a month.
4. Once per week during a month.
5. Once per month during 3 months.
Special importance we attach to the tonic procedures of sphincter apparatus in the form of exercise with tube by diameter of 2,0 cm
and length of 4-5 cm. in the lumen of rectum it has been put this tube patient is explained procedure which increases the tone of sphincter. Procedure is carried out during the year which begins from 3-5 times. In dynamic these exercises are led to 25-30 times. Also with inserted tube into the rectum in the walking position the child has to hold it during 3-5 minutes. Besides that it has been carried out therapeutic enemas in the concentration of 100-150 ml and child held water in the walking position during 5-7 minutes.
The long-term results have been showed that in abdomino-perineal proctoplastics with intrarectal demucosation the good results have observed in 10% of cases, satisfactory results in 25% of cases and unsatisfactory results have registered in 65% of cases. In the basic group the good results have observed in 57,1% of cases, satisfactory results in 35,8% of cases and unsatisfactory results have registered in 1 patient (7,1%).
Conclusions. Thus, intrarectal demucosation cannot in full carry out proprioceptive function of rectum, thereby dictate reason-ability of preservation of the mucous membrane of rectum, and also step-by-step rehabilitation after surgical operation is obligatory in the complex treatment.
References:
1. Albitskiy VYu., Shaykhutdinova L. N., Nokolskaya L. A., Abrosimova M.Yu. Application of regional prevention model of congenital pathology in children in Tatarstan. // Russian pediatric journal. - 2003. - Vol. 1. - P. 59-61.
2. Bairov G. A., Ostrovskiy E. A. Surgery of large intestinum in children. L., Medicine, - 1974; - 207 p.
3. Lenushkin A. I. Coloproctology of pediatric age. Manual for doctors. Medicine. - M. - 1999. - P. 365.
4. Smirnov A. N., Poddubniy I. V. Surgical treatment of anorectal malformations in children. // Surgery. - 1998. - Vol. 8. - P. 149-154.
5. Yugmurlu A, Harmon C. M., Georgeson K. E. Laparoscopic cecostomybatton placement for the management of fecal incontinence in children with Hirshsprung's disease and anorectal anomalies. // Surg Endosc. - 2006. - № 20. - P. 624-627.
6. Dewan P. A., Hrabovszky Z., Mathew M. Anorectoplasty in children in Papua New Guinea // P N G Med J. - 2000. - Vol. 43. - No 1-2. - P. 105-109.
7. Davies MRQAnatomy of the nerve supply of the rectum, bladder and internal genitalia in anorectal dysgenesis in the male.//J. Pediatr Surg. - 1997. - № 32. - P. 536-541.
8. Sayyari1 F., Imanzadeh J., Ghoroubi A. Role of anorectal manometry to improve the results of biopsy in diagnosis of chronic constipation. // A. Pak J Med Sci. - 2007. - (Part-I). - No. 5 (20). - P. 689-691.
9. Hak N. G., El-Hemaly M., Salah T. et al Normal Variation ofAnorectal Manometry among the Egyptian Population. / / Arab J Gastroenterol. - 2007. - No. 8 (2). - P. 53-56.
10. Lam Y. H., Shek T., Tang M. H. Sonographic features of anal atresia at 12 weeks. // Ultrasound Obstet Gynecol. - 2002. - Vol. 19. - P. 523-524.
DOI: http://dx.doi.org/10.20534/ESR-17-3.4-72-74
Shodikulova Gulandom Zikriyaevna, Samarkand State Medical Institute, Uzbekistan
E-mail: Shodikulovagulandom@mail.ru
Special features of clinical and functional disorders in patients with undifferentiated connective tissue dysplasia
Abstract: The objective of the study was to investigate the special features of clinical and functional disorders in patients with undifferentiated connective tissue dysplasia (UCTD) with primary mitral valve prolapse (MVP). The patients were divided into 2 groups depending on the degree of MVP: the 1st group included 97 patients with the 1st degree of MVP and the second group - 111 patients with the 2nd degree of MVP. In the patients, who have UCTD with MVP, the external and internal phenes are clinically observed, a heart rhythm disturbance is noted, and the cardiac hemodynamics indicators undergo statistically significant deterioration in the course of the disease development.
Keywords: undifferentiated connective tissue dysplasia, mitral valve prolapse, external and internal phenes of dysplasia, ECG and EchoCG.
The problem of early diagnostics in modern medicine ofundif- congenital mitral valve prolapse (MVP) determines the necessity for ferentiated connective tissue dysplasia (UCTD) in the patients with advanced study of propagation mechanisms of this disease, which
Special features of clinical and functional disorders in patients with undifferentiated connective tissue dysplasia
occurs in 5-20% of cases. It was established that the patients with MVP have an increased risk for development of mitral insufficiency, infective endocarditis, cerebral embolisms, arrhythmias and sudden death [5]. The objective of the study was to examine the special features of clinical and functional disorders in patients with undifferentiated connective tissue dysplasia with primary mitral valve prolapse.
Materials and methods of research. 208 patients at the age from 15 to 25 years (19,5 ± 1,42) with etiological signs of primary MVP were observed. The diagnosis was established on the basis of T. I. Ka-durina's classification [4] and was confirmed by the data of EchoCG and ECG. The research groups did not include individuals with secondary MVP, cardiovascular diseases, rheumatism, chronic pathology of liver, kidneys and lungs. The indicators of clinical manifestations were central hemodynamic parameters, which were assessed with EchoCG aloka [2]. The patients were divided into 2 groups. The 1st research group included 97 patients (46,6%) with MVP with regurgitation of the 1st degree, and the 2nd group - 111 patients (53,4%) with the 2nd degree of regurgitation. The data from 20 relatively healthy individuals of comparable age without signs of EchoCG disturbances served as verification for both comparison groups.
The results of research and discussion. The studies, which were conducted in this regard, showed that UCTD is mainly typical for young people. Research of sex characteristics demonstrated a predominance of females in the 1st group (60,8%), whereas the 2nd group included a bit more males, but these differences were statistically insignificant. Our results are slightly different from literature ones, which show the predominance of females; these differences may be due to ethnic and regional peculiarities, as the patients of Uzbek population prevailed in our studies. Research of probands showed a certain dependence of genetic factors in UCTD formation from development ofMVP and its intensity. So, if in the 1st group of patients the frequency of occurrence of UCTD-signs in the 1st, 2nd and 3rd lines of consanguinity is revealed in 6 (8,5%), 9 (13%) and 5 (7%) patients out of 71 examined persons, then in the 2nd group they were detected in 13 (18,3%, P < 0.01), 14 (20%, P < 0.05) and 11 (15,5%, P < 0.01) examined persons.
The first stage of our research was to study the external manifestations of UCTD in patients with MVP ofvarious intensities. The analysis of frequency of bone-skeletal external phenes in patients of both groups showed that spinal deformity was revealed in 43 patients (44,3%) of the 1st group. It manifested itself through formation of scoliosis of the 1st and 2nd degree in 19 (19,6%) and 12 (12,4%) patients, kyphoscoliosis of the 1st and 2nd degree in 7 (7,2%) and 2 (2,1%) examined persons, hyperlordosis - in 3 patients (3,1%). The frequency analysis of spinal deformity in patients of the 2nd group showed that it was detected in 48 patients (43,6%). Furthermore, scoliosis of the 1st and 2nd degree was diagnosed in 22 (20,0%) and 9 (8,2%) patients, kyphoscoliosis of the 1st and 2nd degree - in 8 (7,3%) and 3 (2,7%) patients, hyperlordosis - in 6 patients (5,5%). The frequency analysis of thoracic cage deformities in patients of the 1st group showed their occurrence in 50 examined persons (51,5%). Basically, they manifested themselves through a funnel-shaped form of the 1st and 2nd degree in 20 (20,6%) and 11 (11,4%) patients, through asthenic form - in 11 patients (11,4%), through carinate form of the 1st and 2nd degree - in 6 (6,2%) and 2 examined persons (2,1%). Among the patients of the 2nd group the thoracic cage deformities were diagnosed in 40 examined persons (36,1%), which was statistically less significant than in the first group (P < 0.05). Basically, they manifested themselves through a funnel-shaped form of the 1st and 2nd degree in 14 (12,6%, P < 0.05) and 7 (6,3%) patients, through asthenic form - in 4 patients (3,6%, P < 0.05), through a
carinate form of the 1st and 2nd degree - in 10 (9%) and 5 (4,5%) examined persons.
Skin manifestations of UCTD in patients with MVP in the form of skin extensibility of various intensity were observed in 37 (38,1%) and 43 (38,7%) patients of the 1st and 2nd groups. So, if among the patients of the 1st group they manifested themselves mainly through skin extensibility of the 1st and 2nd degree in 16 (16,5%) and 11 (11,4%) examined persons, then among the patients of the 2nd group the skin extensibility of the 1st, 2nd and 3rd degree was diagnosed in 27 (24,3%), 10 (19,8%) and 6 (5,4%) patients, respectively. Muscular hypotonia was observed in 21 (21,6%) and 32 (28,8%) patients of the 1st and 2nd groups, respectively.
Articular manifestations of UCTD are platypodia and hyper-mobility of joints. The frequency analysis of platypodia revealed its occurrence in 20 (20,6%) and 22 (19,8%) patients ofthe 1st and 2nd groups. Moreover, transverse platypodia was detected in 4 (4,1%) and 10 (9%) patients, longitudinal platypodia - in 16 (16,5%) and 12 (10,8%) examined persons. Hypermobility of various intensities was detected in all examined patients. So, if among the patients of the 1st group hypermobility of joints of the 1st, 2nd, 3rd and 4th degree was observed in 56 (57,7%), 17 (17,5%), 18 (18,6%) and 6 (6,2%) patients, then among the patients of the 2nd group it was detected in 47 (42,3%), 28 (25,2%), 24 (21,6%) and 12 (10,8%) examined persons; we did not reveal any significant differences between the groups.
Another external phenes in the patients with UCTD are minor development abnormalities, which manifest themselves through large protrudent ears and accreted earlobe. These signs were observed in 20 (20,6%) and 16 (16,5%) patients of the 1st group and in 24 (21,6%) and 18 (16,2%) examined persons of the 2nd group. As can be seen from the abovementioned data, the external phenes of UCTD in patients with MVP manifested themselves through bone-skeletal, skin, articular forms and the occurrence of minor development abnormalities. According to the degree of intensity they manifested themselves through hypermobility of joints (100%), change of the shape of spine (44%) and thoracic cage (43,8%), skin extensibility ofvarious intensity (38,8%), minor development abnormalities (21,1%) and platypodia (20,4%). The frequency of combinations of various phenes in patients was different. So, if among the patients of the 1st group the frequency of combination of 6 external phenes was detected in 3 patients (3,1%), combination of 5 phenes - in 5 patients (5,1%), combination of 4 phenes - in 28 patients (28,9%), combination of 3 phenes - in 27 patients (27,8%), combination of 2 phenes - in 25 examined persons (25,8%) and combination of 1 phene was observed in 9 patients (9,3%). Among the patients of the 2nd group the frequency of combination of 6 external phenes were detected in 5 cases (4,5%), combination of 5 phenes - in 11 cases (10%), combination of 4 phenes - in 22 cases (20%), combination of 3 phenes - in 27 cases (24,3%), combination of 2 phenes in 34 examined persons (30,6%) and combination of 1 phenes was detected in 11 patients (10%). We did not reveal any significant differences in both examined groups, however it should be noted that more severe manifestations of external phenes were mainly revealed in the patients with MVP of the 2nd degree.
Analysis of internal phenes in patients with UCTD and MVP depending on its intensity showed that ocular manifestations were characterized by development of myopia ofvarious degrees in 26 patients of the 1st and 2nd groups. However, the degree of myopia precisely concurred with the intensity ofMVP. So, in the 1st group the 1st degree of myopia was detected in 23 patients (23,7%), the 2nd degree - in 3 patients (3,1%); and in patients of the 2nd group the 1st degree of
myopia was detected in 3 patients (2,7%, P < 0.01), the 2nd degree - in 23 examined patients (20,7%, P < 0.01). Furthermore, in the 2nd group astigmatism was revealed in 20 patients (18%), anisometropia - in 34 patients (30,6%, P < 0.05) and degenerative changes in the retina -in 30 patients (27%). As can be seen from the abovementioned data, the frequency of lesions of ocular organs was reliably highest in the patients with MVP of the 2nd degree, which indicates a severe course of main disease. We did not reveal any significant differences in both examined groups, however it should be noted that more severe manifestations of external phenes were revealed in a greater degree in the patients with MVP of the 2nd degree.
The next stage of the research was to study clinical symptoms, heart condition and ECG indicators depending on the degree of regurgitation. In the subjective status the cardiovascular complaints were predominant according to the frequency of occurrence and intensity: cardialgia (34,8-76,4%), feeling of "heart discomfort" (22,8-55,1%), palpitation (17,4-52,8%), cardiac malfunction (17,4-43,8%), dizziness, general weakness with a sharp decrease in blood pressure under the conditions of orthopnoea (13-38,2%). The frequency of occurrence of these clinical symptoms was in direct relationship to the degree of MVP and regurgitation, and it was detected significantly more often in patients of the 2nd group. These symptoms were not confirmed electrocardiographically approximately in 50% of cases. Holter monitoring allows to reconfirm the existence of heart rhythm disturbances and cardiac conduction defects in persons with various degree of connective tissue dysplasia. The studies, which were conducted in this regard, have shown that MVP is accompanied by certain ECG changes. The cardiac rate of the patients examined by us varied widely: from 56 to 96 beats per minute in patients of the 1st subgroup, from 75 to 100 beats per minute in patients of the 2nd subgroup A. On average, these values amounted to 82,78 ± 3,53 and 87,67 ± 0,65 beats per minute according to the groups. In our research we observed a tendency towards cardiac rate increase as the pathological process aggravated. It should be noted that patients often complained about episodes of cardiac rate acceleration within 24 hours, especially under active physical loads, which affected the patients' quality of life. Sinus arrhythmia was found in 5% of the examined patients, sinus bradycardia - in 3% of the patients, and sinus tachycardia - in 24,6% of the patients. According to the literature data, atrial and ventricular extrasystole is the most common heart rhythm disturbance in patients with UCTD and was found in 28% of the examined patients. According to our observations, atrial extrasystole at rest increases under physical loads by 1,5-2 times in the patients with pronounced manifestations of UCTD. In rare cases we observed the phenomenon of ventricular preexcitation (4,7%) in the examined patients; and paroxysmal heart rhythm disturbances in the form of atrioventricular paroxysmal tachycardia were detected in these patients. Moreover, attacks occurred both during exercise activities and at rest or during sleep. AV blockade of I and II degree was also detected in 24, 8% of the patients. According to ECG data, changes in the terminal part
ofventricular complex were detected in 15.7% of the patients, which we interpreted in terms of metabolic disturbances in the myocardium under metabolic cardiomyopathy.
The next stage of the research was to detect changes in cardiac hemodynamics, so in the patients with MVP of the 1st degree the values of EDD (end-diastolic dimension) and ESD (end-systolic dimension) had a tendency only towards increase, remaining within the upper normal limits. At the same time we observed excess of the normative values in 23,7 and 26,1% of the examined patients. The values of ejection fraction had a tendency towards decrease in relation to the control group, which was observed in 21,2%; the values of stroke volume had a tendency towards increase and the excess of normative values was detected in 23,7% of the patients with MVP of the 1st degree. The values of left ventricular posterior wall and interventricular septum remained within upper normal limits. Excess of normative values of these indicators was observed in 26,1% of the patients. It should be noted that the values of SBP (systolic blood pressure) and DBP (diastolic blood pressure) remained within the age norm. However, heartbeat acceleration in the course ofleaflet prolapse was observed in all patients. As may be seen from the given data, changes in the values of cardiac hemodynamics are detected not in all patients with MVP of the 1st degree, determining less pronounced changes of their average values. At the same time it should be noted that deviation in the values of cardiac hemodynamics was noted in the patients with regurgitation of the 2nd degree, which is, in our opinion, associated with a more pronounced leaflet prolapse and the increase of blood regurgitation backwards to the left atrium. So, the values of EDD (end-diastolic dimension) and ESD (end-systolic dimension) exceeded the values of the age norm in 53,7 and 58,8% of patients. The average values of these indicators increased; however, such deviations were statistically insignificant. The values of ejection fraction had a tendency towards decrease, which was observed in 74,2% of the patients; the values of stroke volume had a tendency towards increase, which was detected in 42,3% of patients with MVP of the 2nd degree. The excess of normative values ofthe sizes ofleft ventricular posterior wall and interventricular septum was observed in 48,1% of the patients with MVP of the 2nd degree. It should be noted that the values of SBP (systolic blood pressure) and DBP (diastolic blood pressure) remained within the age norm. Higher values of SBP and DBP were detected more often in the 2nd group.
Thus, the indicators of cardiac hemodynamics statistically deteriorate a lot against the control group in the course of mitral valve prolapse progression. It is known that in response to volume overload, in order to maintain an adequate pumping function the local (myocardial) neurohumoral systems, stimulating development of tachycardia and subsequently the risk for development of myocardial hypertrophy of the left atrium and ventricle become more intense. Myocardial hypertrophy leads to the increase of diastolic rigidity of the left ventricular myocardium and consequently to systolic dysfunction. Constant tachycardia increases the cardiac energy consumption, reduces its functional activity.
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