Научная статья на тему 'Features of clinical picture of cystic dilatations of the biliary ducts in children'

Features of clinical picture of cystic dilatations of the biliary ducts in children Текст научной статьи по специальности «Клиническая медицина»

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Аннотация научной статьи по клинической медицине, автор научной работы — Yakubov Erkin Amongeldievich, Ergashev Nasriddin Shamsiddinovich

This work presents characteristic clinical signs of the cystic dilatations of the extrahepatic biliary ducts in 76 children of the age from neonatal period to 16 years, receiving treatment in the clinic in 1979-2016. There were determined frequency, special features of some clinical signs, character of their association and occurrence of various complications. The features of current of separate clinical attributes, character of their combination and occurrence of various complications are determined frequency.

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Текст научной работы на тему «Features of clinical picture of cystic dilatations of the biliary ducts in children»

Study Design

It should be noted that the persons with DR among VVSD2 already suffer ofdecompensated stage oftype 2 diabetes. Fasting glycae-mia 11.5 ± 2.4 mmol/l, postprandial glycaemia — 15.7 ± 5.2 mmol/l, the average level of glycated hemoglobin (HbAlc) is 9.4 ± 2.5 %.

Among the people with IGT the DR average indicators were as follows: fasting glycaemia 4.36 ± 0.55 mmol/l, blood glucose after glucose load 75 g. — 9.5 ± 0.4 mmol/l, the average level of HbAlc — 5.3 ± 0.8 %.

The DR prevalence study depending on the compensation of carbohydrate metabolism is of high interest. In more than 7.5 % patients with HbAlc, regardless of gender, the number of recorded cases of DR is 2 times higher than that of the patients with less than 7.4 % HbAlc. These data confirm once again the need for close

collaboration between ophthalmologists and endocrinologists in detection and monitoring of these patients.

Conclusions:

1. In patients with DR detected was found in 14.3 % of cases. In patients with IFG DR has not been revealed. In IGT and NDD2 DR detected accordingly in 13.7 % and 18.8 % of cases. The defeat of the retina is observed before manifestation of diabetes in some surveyed patients with VGM.

2. The patients with HbA1c more than 7.5 %, irrespective of gender, the number registered cases of DR is 2 times higher than patients with less than 7.4 % HbA1c.

3. Persons with VVSD2 complicated with DR were all decompensated.

References:

1. Akbarov Z. S., Alieva A. V. Screening for diabetes and intermediate hyperglycaemia in Tashkent//Journal of Theoretical and Clinical Medicine. - 2016. - № 1. - P. 50-56.

2. Galstyan G. R., Udovichenko O. V., Tokmakova A. Y., Antsiferov M. B. Diabetic neuropathy: epidemiological and clinical aspects//Dia-betes XXI century. - 2000. - № 1. - P. 19-21.

3. Ismailov S. I., Berdykulova D. M., Khaydarov F. A. Rasprostranennost late complications of diabetes mellitus in the regions of the Republic of Uzbekistan//International Endocrinology Journal. - Tashkent, Republic of Uzbekistan, 2010. - № 1(25).

4. Ismailov S. I., Berdykulova D. M. Late complications of diabetes mellitus in persons living in the Tashkent region of Uzbekistan/International Journal of Endocrinology. - Tashkent, Republic of Uzbekistan, 2012. - № 8(48).

5. Kayumova D. T. Abstract "Vascular lesions in patients with intermediate hyperglycaemia and newly diagnosed diabetic patients". -Tashkent, 2008.

6. Hall Michael and Felton Anne-Marie. The St Vincent Declaration 20 years on - defeating diabetes in the XXI century// Diabetes Voice. - June 2009. - Vol. 54, Iss. 2. - P. 42-44.

7. Kohner E. M. The evolution and natural history of diabetic retinopathy//Int. Ophthalmol. Clin. - 1978. - Vol. 18. - P. 1-16.

Yakubov Erkin Amongeldievich, Ergashev Nasriddin Shamsiddinovich, Tashkent Pediatric Medical Institute, Republic of Uzbekistan E-mail: yakubov. e.a.1972@mail.ru

Features of clinical picture of cystic dilatations of the biliary ducts in children

Abstract: This work presents characteristic clinical signs of the cystic dilatations of the extrahepatic biliary ducts in 76 children of the age from neonatal period to 16 years, receiving treatment in the clinic in 1979-2016. There were determined frequency, special features of some clinical signs, character of their association and occurrence of various complications. The features of current of separate clinical attributes, character of their combination and occurrence of various complications are determined frequency.

Keywords: cysts of biliary ducts, clinical picture, complication, children.

Until recently time the cystic dilatations of the extrahepatic biliary ducts have been considered as relatively rare pathology among the congenital anomalies of the development of the organs of hepatobiliary zone. The number of publications based on the analysis of the large clinical material has been significantly increased [3; 4]. However, in the literature the features of clinical expressions of cystic dilatations ofthe extrahepatic biliary ducts are insufficiently elucidated (CDEHBD).

The classic triad consists ofjaundice, pain syndrome and palpated tumor in the abdominal cavity [1]. As a rule, the clinic of cystic transformation can be limited by one or two symptoms from this triad [2]. At occurrence of complications the clinical manifestations of illness essentially vary. Quite often the choledochal cysts may be in general not shown in any way for years and are diagnosed as a

casual find or are not distinguished not only at clinical examination, but even during surgical intervention.

The purpose of research was to analyze features of clinical manifestations of cystic dilatations of the extrahepatic biliary ducts in children on the basis on the basis of the own clinical material.

Materials and methods of research

In the clinical bases of the faculty of hospital children's surgery, children's oncology of the Tashkent Pediatric Medical Institute in 1979-2016 there were studied and treated 76 patients (60 girls and 16 boys) ofthe age from the neonatal period to 16 years with cystic dilatations of the extrahepatic biliary ducts.

The distribution of the patients by a type of cystic dilatation was performed according to classification of Alonso-Lej F. with

Section 6. Medical science

additions of Todani T. and Lilly J. R.: type I was cystic dilatation of the common bile duct (55-72.4 %); type IV was cystic dilatation of the extra- and intrahepatic ducts (19-25 %); type V — Caroli disease (1-1.3 %); atypical forms (AF) were presented as insignificant dilatation of extrahepatic ducts and intrahepatic cystic lesions (1-1.3 %). In our studied there were no types II and III.

The clinical anamnesis, clinical expressions, data of the auxiliary methods of diagnosis (ultrasonography and MSCT of the abdominal cavity, intraoperative cholangiography, histomorphologic investigation of the liver biopsy) was carefully investigated.

Results and discussion

The basic clinical features were spasmodic pain in the abdomen — 24 (31.6 %), jaundice — 14 (18.4 %), palpated tumorous mass — 1 (1.3 %) and various combinations of the listed symptoms — 37 (48.7 %): pain + jaundice (26), pain + tumorous mass (2), jaundice + tumorous mass (2), pain+jaundice + tumorous mass (7).

The abdominal pain syndrome appeared to be the earliest and often clinical sign. In the newborns and infants (23-39 %) the attacks of pains were expressed by anxiety of the child of various duration and intensity. In the children of the senior age groups (36-61 %) the pains of spasmodic character were located: in the top departments of abdomen (10-27.8 %); in the right half of abdomen (24-66.6 %); in the area of umbilicum (2-5.6 %). In 7 (11.9 %) from 59 patients there was noted irradiation of the pains into the back. In 17 (22.4 %) from 76 patients the abdominal pains were not registered; not intensive, passing pains remained to be unnoticed. Frequency of pain attacks were various: in 16 (27.1 %) — 1 time per 2-3 months; at 11 (18.6 %) — 2-3 times per one year; at other 32 (54.3 %) patients repeated some times per one month. At a persisted attack at 32 (54.3 %) from 59 children the pain syndrome was stopped by spasmolytics and analgesics. In 41 (69.5 %) patients abdominal pains were accompanied by nausea and vomiting. In 22 (37.3 %) from 59 children the pain attacks were provoked by errors in a diet, physical loading. On the basis of comparison of clinical signs with the data of auxiliary methods of researches, it is possible to explain an origin of the pain syndrome in these patients by secondary inflammatory changes in a gold bladder and biliary tract (24-40.7 %); by sphincter Oddi spasm at functional discoordination (13-22 %); by biliary hypertension at stenosis of the common bile duct distal part (12-20.3 %); and by accompanying changes in the pancreatoduodenal zone (10-17 %).

Though the pains at cystic anomalies of biliary ducts appeared to be a constant sign, their participation in diseases of biliary tracts in a number of cases has allowed to suspect only occurrence of jaundice. The yellowness of skin, mucosa and sclera appeared to be the secondary in relation to frequency and basic feature in relation to value at diagnosis of the cystic anomalies of the biliary ducts. This sign was observed in 49 (64.5 %) from 76 patients with CDEHBD. The fast development of jaundice after pain syndrome was noted in 13 patients mainly at the age to 3 years. The appearance of jaundice in various terms after onset of the pain syndrome did not exclude in the pathogenesis of its development the role of discoordination of sphincter Oddi, which spasms can arise under effect ofvarious unfavourable factors and express by pain syndrome with development of subsequent biliary hypertension in the further. The recurrent jaundice, presence of various duration of the "light interval", short-term and big variability of icteric intensity during period of exacerbation indicated about predominance of the functional disorders of the sphincter Oddi in comparison with real stenosis. Cupping of the attacks with receiving of spasmolytics and analgesics

(18 patients) confirmed this assumption. This does not exclude an occasion stenosis in the terminal department of common bile duct. In 17 (34.7 %) from 49 patients the damage of outflow of bile was caused by organic changes, that was specified by long jaundice, absence of effect from application of spasmolytics, by acute disorder of administration of the contrast substance into duodenum in intraoperative cholangiogram, by marked cholestatic changes in the liver in histological study.

Tumor neoplasm in the abdominal cavity was revealed in 12 (15.8 %) from 76 children, in 7 — at classical triad. The cysts were palpated at a diameter more 3 cm. In some patients the increased liver masked the present tumorous mass. In other cases the palpating mass in the underhepatic space was accepted as increase of organ. In 2 children of breast age during operation there have been found cystic mass of volume from 1000 up to 2000 ml., occupying all underhepatic space with pushing away of the adjacent organs. Despite of the huge cyst sizes in these patients there were not found signs of the mechanical jaundice. The absence of mechanical jaundice at cystic mass of the huge sizes indicates about congenital dilatation of the external biliary ducts without stenosis of the major duodenal papilla.

The simultaneous presence of all clinical signs (paroxysmal pains, jaundice, palpated tumorous mass) appeared to be so-called "classic triad» was noted in 7 (9.2 %) patients. The intensity of all major clinical signs in the patients of this group may be explained by the significant sizes of cystic mass. However, frequency, sequence and degree of expressing of each sign of CDEHBD in patients was completely different.

Depending on a structure and sizes of cystic dilatation, its anatomic variants the character of complications and development of the postoperative period changed. The changes in the liver due to organ lesion in the intrauterine period or occurring complications at the late determination of diagnosis influenced on the development of cystic transformation of the biliary ducts. There were observed non-complicated (5) and complicated (71) forms expressed looking like compensated, subcompensated and decompensated progressing. In 22 patients in presence of the signs of persistent mechanical jaundice or 'classic triad" there were observed various complications in the liver. Reactive pancreatitis was noted in 7 patients, choledocholithiasis — in 11, cholecystochol-angitis — in 23 patients, pressing of the adjacent organs in the abdominal cavity with the cyst — in 1, rupture of the cyst — in 6 and suppuration of the cyst content — in 1 patient. The information about occurrence of cholangiocarcinoma in the remnant tissue of the common bile duct was single. In our observations the cyst malignization was not found.

Conclusions

The clinical expressions and progressing of the cystic dilatations of the extrahepatic biliary ducts depend on the intensity of the inflammatory, functional disorders and organic changes in the biliary system, interrelations of the dilated common bile duct with pancreatic duct.

The most often clinical features of the cystic dilatations of the extrahepatic biliary ducts include abdominal pains (24-31.6 %) and jaundice (14-18.4 %) of various duration and intensity expressed separately or in a combination (26 - of 34.2 %).

The polymorphy of clinical expressions induce significant difficulty in recognition of cystic dilatations of the extrahepatic biliary ducts in children. There are observed noncomplicated and complicated forms looking-like compensated, subcompensated and decompensated development.

References:

1. Buyukyavuz I., Ekinci S., Ciftci A. et al. A retrospective study of choledochal cyst: clinical presentation, diagnosis and treatment//The Turkish Journal of pediatrics. - 2003. - 45(4): 321-325.

2. Danielle N. F., Saulo M. R., Vinicius G. S. et. al. Choledochal cyst in childhood: review of 30 cases//Rev. Col. Bras. de Cir. - 2014. -41(5): 52-55.

3. Liem N. Th., Pham H. D., Dung L. A. et al. Early and intermediate Outcomes of Laparoscopic Surgery for Choledochal Cystis with 400 Patiens//J of Laparoendoscopic & advanced surgical techniques. - 2012. - 22(6): 1-5.

4. Hierro R. C. Laparoscopic Repair for Choledochal Cyst in Children: Current status//World J. Lap. Surg. - 2012. - 5(2): 76-79.

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