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Comparative evaluation of various surgical corrections of hirschsprung's disease in adults
At the inefficiency of preoperative preparation, progressive decompensation of the large intestine with signs of intestinal obstruction, expanded dilatation of the large intestine, presence of fecal stones in the rectum and colon, unsatisfactory preparation to operation on the large intestine at worsening of the general health state induce by chronic intoxication and disturbance of metabolism, the surgical treatment were carried out in two-steps.
Multi-staged surgical treatment were performed in 12 (146%) patients (2 from studied and 10 from control group).
Study of the long-term functional results in the patients of the both group was performed by questionnaire, sphincterotomy and anorectal manometry in 3, 6 and 12 months after operation.
In the studied group after operation par of the rectum in 12 months the number of patients with unsatisfactory results reduced from 7,7. to 4,5%. At the same time the quantity of good (69%) and satisfactory (23,1%) results increased from 23,1 to 86%.
The study offunctional result was performed by two methodic: by scale of incontinence W exner (1993) (table 7) and with help of original questionnaire for study of functional results (Fig.2). Totally there were examined and interviewed 26 patients from studied and 35 — from control groups. The results of interview with use of scale of incontinence Wexner (1993) showed that average value in the studied group was 4.1 (1-13) and in the control — 6.3 (1-12). Unsatisfactory result, that is, more then 4 points, was registered in 2 (7,7%) patients having operation PAR of the rectum. After methods
of Duhamel in modification the unsatisfactory results were more — in 9 patients (25,7%).
Conclusion
1. One-step radical surgical intervention treatment is executed in both group patient in 85,3%. The Evidences to much-step to treatment are: of the preparation to the operation on the large intestine, presence coprolitis, progressing decopension large intestine with sign of the intestine impassability, deterioration of the general condition of the patient. The First stage must be directed on liquidation of the intestine impassability, megakolon and zones hipo- or aganglioz. After recovering the functions of the large intestine and improvements of the general condition sick restore intestine continuity.
2. Performance of peritoneal-anal resection of the rectum with voiding functionning proximal parts of the large intestine division in anal channel simplifies the technology to operations in contrast with method Duhamel and avoids the area hipo- or agangliozis.
3. The Remote functional results of the treatment (through 1224 months) turned out to be greatly better in the main group in contrast with operation Duhamel: observations incotinenction on scale Wexner was in 3,3 times less (7,7% and 25, 7% accordingly); the physiological volume of the imperative urge to defecation was above on 45 ml, differentiated urge to defecation had 82,5% patient in the main and 75% in checking group.
Surgical treatment and rehabilitation of these patients should be done only in specialized hospitals.
References:
1. Adhi M., Khan S., Zafar H., Arshad M. Duhamel's procedure for adult Hirschsprung's disease//J. Coll. Physicians. Surg. Pak. - 2012. -Vol. 22, № 6: 395-7.
2. Alagumuthu M., Jagdish S., Kadambari D. Hirschsprung's disease in adults presenting as sigmoid volvulus: a report of three cases.//Trop. Gastroenterol. - 2011. - Vol. 32, № 4. - P. 341-343.
3. Arshad A., Powell C., Tighe M. P. Hirschsprung's disease//Brit. Med. J. - 2012. - Vol. 1. - P. 5521.
4. Castle S., Suliman A., Shayan K., Kling K. Total colonic aganglionosis with skip lesions: report of a rare case and management//J. Pe-diatr. Surg. - 2012. - Vol. 47, № 3. - P. 581-584.
Navruzov Sarimbek Navruzovich, Mirzachmedov Murad Mirchaidarovich, Republican Scientific center of Coloproctology Uzbekistan
E- mail: [email protected]
Comparative evaluation of various surgical corrections of hirschsprung's disease in adults
Abstract: In 1990-2014, eighty patients with Hirschsprung's disease were on an inpatient treatment in the Republican research centre for colon proctology of the Republic of Uzbekistan Ministry of Health in Tashkent.
Diagnostics was based on the scheme which included general clinical examination, radiological and endoscopic studies, USI of organs of the abdominal cavity and the small pelvis, proctosigmoidoscopy, functional studies of the colon and the sphincteric apparatus of the rectum, morphological studies (Swenson biopsy). The differential diagnosis of Hirschsprung's disease in adults requires an individual approach, an integrated assessment of clinical symptoms, data of an X-ray contrast study and results of functional studies of the colon and the sphincter apparatus of the rectum as well as undertaking a morphological study. Radiological methods make it possible to estimate and identify expression of the megacolon and reveal a characteristic picture of narrowing and suprasthenic expansion. Diagnostics of a very short (anal or supra-anal) hypo- or aganglionosis of the colon is especially complicated. The presence of a classical picture in the form of narrowed (hypo- or aganglionic) zones, as that in children, is not obligatory; a physiological norm is relaxation of the internal anal sphincter in its stretching with fecal masses. Keywords: megacolon, agangliozis, hipogangliozis, megarectum.
Introduction or a significant decrease in a number of neurons in ganglions of intra-
Hirschsprung's disease is characterized by congenital absence mural nervous plexuses of the colon. Hirschsprung's disease is one
Section 6. Medical science
of severe developmental anomalies of the colon and takes a leading place in the structure of digestive tract diseases. There are certain preconditions for slow, sometimes a latent course of Hirschsprung's disease in adults consisting in existence of variants of congenital morphological alterations, such as short zones of aganglionosis, hypogangliosis types while expressed degenerate processes in the muscular tissue of distal parts of the colon are absent [1; 4].
Despite of the current progress achieved in the treatment of Hirschsprung's disease in adults, this problem still remains to be far from being resolved and a not very comforting percent of so-called secondary pathology, and the main thing — incessant references to specialized medical establishments of patients who had primary corrective interventions testify to that [5; 6].
Persistent functional disturbances causing invalidism are an occasion for seeking medical advice. In this respect, the efforts targeted at improvement of results of treatment of Hirschsprung's disease in adults are quite important. The majority of researchers emphasize that Hirschsprung's disease occur more often than it is diagnosed now [2; 6]. Various methods of surgical operations and their modifications have been suggested [3]. The arsenal of methods of surgical treatment of Hirschsprung's disease in children includes various types of surgery, viz. according to Swenson, Soave, Duhamel, etc. It is difficult to use these classical techniques of pediatric surgery in adults due to rough alterations in the colon, anatomic peculiarities of the pelvis and interrelations of pelvic organs. At the same time, these publications pay the greatest attention to results of a surgical treatment of the pathology, while the data concerning the clinics, diagnostics and surgical tactics at Hirschsprung's disease in adults are represented only by actual material without its analysis due to a small number of clinical observations.
The goal of our work was to determine the efficacy of surgery of abdominoanal rectectomy with bringing down of the proximal parts of the colon into the anal canal in Hirschsprung's disease in adults.
Material and methods
In 1990-2014, eighty patients with Hirschsprung's disease were on an inpatient treatment in the Republican research centre for colon proctology of the Republic of Uzbekistan Ministry of Health in Tashkent.
Diagnostics was based on the scheme which included general clinical examination, radiological and endoscopic studies, USI of organs of the abdominal cavity and the small pelvis, proctosigmoidoscopy, functional studies of the colon and the sphincteric apparatus ofthe rectum, morphological studies (Swenson biopsy). The differential diagnosis of Hirschsprung's disease in adults requires an individual approach, an integrated assessment of clinical symptoms, data of an X-ray contrast study and results of functional studies of the colon and the sphincter apparatus of the rectum as well as undertaking a morphological study. Radiological methods make it possible to estimate and identify expression of the megacolon and reveal a characteristic picture of narrowing and suprasthenic expansion. Diagnostics of a very short (anal or supra-anal) hypo- or aganglionosis of the colon is especially complicated. The presence of a classical picture in the form of narrowed (hypo- or aganglionic) zones, as that in children, is not obligatory; a physiological norm is relaxation of the internal anal sphincter in its stretching with fecal masses.
Results and discussion
During irrigoradiography an supra-anal type of Hirschsprung's disease was found in 72 patients, a rectal one in 10 patients.
Of 82 patients examined 3 had aganglionosis and 47 patients had hypogangliosis. Sphincterometric indicators in all patients examined, both in rest and voluntary constriction, were normal. Dur-
ing USI, absence of the uterus and its appendages, infantilism of grades I and II were revealed in 3 patients; dimetria and aplasia of the left kidney were found in 2 patients and 1 patient had congenital absence of the right kidney.
Choice of surgical treatment tactics. As a hypo- or aganglionic zone is often located in the rectum, its excision can be done using various techniques, for example by means of anterior rectectomy or abdominoanal rectectomy with bringing down of the proximal colon. The practice however has shown that after the anterior resection quite a long hypo- or aganglionic zone is left which is a reason of relapse of disease. But sometimes we can find advanced stages of the disease in some patients when a significant part of it or entire colon is on the verge of decompensation. In such cases discussion of surgical tactics, expediency of dividing the operation into several stages with preliminary formation of colostomy is required. The character of surgical operations used in Hirschsprung's disease in our clinic was the following (n=82): 1). Duhamel operation is a modification 44 (53,7%); 2). Two abdominal-anal resection of the rectum with bringing down the proximal colon into the anal canal with the imposition of colo-anal anastomosis 26 (31,7%); 3). Four anterior resection of the rectum, with the imposition of sigmoid-ektomiya destsendostomy. anterior resection of the rectum, with the imposition of sigmoidektomiya destsendostomy
5 (6,1%); 4). Four anterior resection of the rectum, with the imposition of sigmoidektomiya destsendostomy. anterior resection of the rectum, with the imposition of sigmoidektomiya destsendostomy 3 (3,7%); 5). Peritonel-anal resection of the rectum, with the imposition of sigmodektomiya destsendostomy 2 (2.4%); 6). Subtotal colectomy with six imposing astsendostomy and anterior resection of the rectum 2 (2,4%).
One-step surgical treatment was carried out in 70 (85,3%) adult patients with Hirschprung's disease: 26 (37,2%) from them underwent PAR of the rectum with pull-through of the proximal parts of the large intetineintoanal canal with application of colo-anal anastomosis (group 1, studied): 44 (62,8%) — operation of Duhamel in modification (group 2, control). Multi-staged surgical treatment were performed in 12 (146%) patients (2 from studied and 10 from control group). This treatment slowed not only to improve general state of the patients, liquidate intestinal obstruction, but also to save a part of colon at the performance of the main stage of operation. The terms ofperformance of the second stage of surgical treatment accounted 6-14 months and depended, mainly, on the reason which induced multistaged treatmen.
In 2 of 44 patients (53,7), pyesis of the presacral area was observed following "Duhamel" surgery modified, 3 patients (6,8%) had marginal necrosis of the intestine brought down, 2 (4,5%) had retraction and at 1 patient (2,2%) had a stricture of the intestine brought down. The complications were cured following relevant treatment. No lethal outcomes occurred.
Good results were obtained in all cases following the surgery of abdominoanal rectectomy with bringing down of proximal parts of the colon to the anal channel.
Remote results of the surgeries performed for Hirschsprung's disease were followed-up 1 to 10 years in 57 patients (71,2%). In addition to clinico-laboratory studies these patients had endoscopic, X-ray investigationd and sphincterometry as well as other functional methods of study.
Carrying out of a surgical intervention in Hirschsprung's disease in adults is always related with great difficulties; therefore diagnostics and a preoperative preparation of patients, prevention of purulent complications plays a great role in rehabilitation of this
Molecular-genetic aspects of atopic dermatitis
category of patients. In our observations 46 patients (80,7%) had good results, 10 (17,5%) satisfactory and 1 patient (1,8%) had a poor result.
Follow-up results were considered good if there were no complaints after surgery. These patients had a satisfactory general state and functional parameters of the colon. No relapse of the disease was noted. The working capacity was restored.
Results were considered unsatisfactory in cases when constipation with manifestations of a chronic intestinal obstruction, which required a repeated intervention.
Thus, the problem of Hirschsprung's disease in adults is urgent and its solution can be provided by development of adequate tac-
tics of treatment including a surgical one with the use of modern methods of study.
Conclusion
1. To specify a diagnosis, radiological, endoscopic and functional studies of the colon should be supplemented by transanal biopsy of the rectal wall which is the most reliable method of diagnostics in Hirschsprung's disease in adults.
2. Performance of peritoneal-anal resection of the rectum with pulling through of functioning proximal parts of the large intestine into anal canal simplifies technique of operation at Hirschprung's disease and in the greater degree meet the requirement of radicalism, than method of Duhamel.
References:
1. Alagumuthu M., Jagdish S., Kadambari D. Hirschsprung's disease in adults presenting as sigmoid volvulus: a report of three cases.//Trop. Gastroenterol. - 2011. - Vol. 32, № 4. - P. 341-343.
2. Amiel J., Lyonnet S. Hirschsprung's disease, associated syndromes, and genetics: a review. J. Med.Gen. 2001; 38:729-739.
3. Borrego S., Ruiz-Ferrer M., Anticolo G. Hirschsprung's disease as a model of complex genetic etiology//Histol. Histopathol. - 2013. -Vol. 19, № 2. - P. 345-345.
4. Butler N. E., Trainor P. A. The developmental etiology and pathogenesis of Hirschsprung disease//Transl. Res. - 2013. - Vol. 22, № 6. -P. 123-178.
5. Hayes C. E., Kawatu D., Mangray S., Leiko N. S. Rectal suction biopsy to exclude the diagnosis of Hirschsprung disease//J. Pediatr. Gastroenterol. Nutr. - 2012. - Vol. 55, № 3. - P. 268-271.
6. Mahajan J. K., Rathod K. K., Bawa M., Narasimhan K. L. Transanal Swenson's operation for rectosigmoid Hirschsprung's dis-ease//Afr. J. Paediatr. Surg. - 2011. - Vol. 8, № 3. - P. 301-305.
Muminova Sevara Rustamovna, Mavlyanova Shakhnoza Zakirovna, Boboev Kodir,
Republican Specialized scientific-practical medical Centre of Dermatology and Venereology of the Health Ministry of the Republic of Uzbekistan, Tashkent E-mail: [email protected]
Molecular-genetic aspects of atopic dermatitis
Abstract: There was conducted work on optimization of test systems for the detection of polymorphisms -590S> T of IL4 gene in patients with atopic dermatitis and healthy donors. There was examined the contribution of this marker in the development (progression) of atopic dermatitis. It was revealed that the carriage of hetero + homozygous genotype observed in more than 3.0 times more frequent in patients with AtD than in conditionally healthy donors. At the same time, the calculated odds ratio also showed a significant increase of AtD progression risk in carriers of these combinations of genotypes (X 2=3.85; P=0.02; 0R=3.0; 95%CI 0.98-9.14).
Keywords: allergic dermatitis, atopy, IL-4 gene, DNA, polymorphism 590C> T.
Atopic dermatitis (AtD) — one of the most common and severe multifactorial diseases, which is being the result of an allergic reaction ofbody, and caused by the complex interaction of immunocompetent cells and transmitters. A special role in the pathogenesis ofAtD takes interleukine 4 (IL-4), since it is the initiator of molecular and cellular events leading to the release of neurotransmitters and migration of effector cells in "shock" organ (skin area, bronchus, nasopharynx, etc.), where expands the inflammatory response. In 1994, Marsh together with co-author proved correlation between IgE level of general serumal and markers located at the locus 5q31.1 [4]. Rossenwasser L. J. together with co-author (1995) revealed for the US population the transitions of cytosine to thymine (590S> 7) in promoter region of IL4 gene and showed this association of polymorphic variant with atopy and levels of general IgE, as well as gene overexpression of the gene in vitro [8]. Nowadays, some
research groups have published the results ofwhole genome linkage analysis under atopic conditions in different populations. However, these results are very contradictory and do not give an unambiguous answer to the question of the pathogenetic role and degree of participation of certain marker in formation of atopic diseases, including blood pressure [2; 3; 6; 9; 11].
Objective: To optimize the performance of oligoprimer's systems for testing 590C> T polymorphism of IL-4 gene and examining the contribution of this marker in the development of AD.
Materials and Methods: The materials for this study were taken the samples of DNA isolated from the peripheral blood of patients with AtD and conditionally healthy donors — individuals without any signs of atopic diseases. The total number of studied C and T alleles was 114 (70 chromosomes of the patients and 44 chromosomes of healthy individuals).
