Biliary atresia in children (analytical literature review and review of own observation).

OBJECTIVE: Aim: to review information resources and analysis of the own experience on this problem for the provision of modern knowledge in the pathogenesis of the pathology, the latest diagnostic and treatment technologies, with consideration of the need to adhere to a single strategy in the management of patients with BA. PATIENTS AND METHODS: Materials and Methods: The analysis of the data regarding the results of existing studies evaluating the clinical benefit and safety of diagnostic and treatment methods in Biliary atresia. CONCLUSION: Conclusions: BA is the leading cause of neonatal cholestasis development. Early diagnostics of BA, based on the complex evaluation of clinical-laboratory, instrumental and morphological signs of the pathology, has a significant meaning. Surgical correction during the first 2 months of life - the Kasai procedure, as well as dynamic post-surgery follow-up significantly prolong the life of children and allow postponing liver transplantation. The highest patient survival both at the first stage of treatment - conduction of the Kasai procedure and the stage of liver transplantation may be achieved by joined work of surgeons and pediatricians, which allows considering the whole row of possible problems.

MORPHOLOGICAL AND IMMUNOHISTOCHEMICAL CHANGES IN THE GONADS OF CHILDREN 2-6 HOURS AFTER ACUTE UNILATERAL TESTICULAR TORSION.

OBJECTIVE: The aim: To determine the morphological and immunohistochemical changes in the testes 2-6 hours after the onset of clinical symptoms of acute unilateral testicular torsion. PATIENTS AND METHODS: Materials and methods: A morphological and immunohistochemical study was conducted on biopsy samples of testicular tissues taken 2-6 hours after the onset of clinical symptoms of acute unilateral testicular torsion during detorsion and orchiopexy surgery in 27 adolescent patients. RESULTS: Results: In cases of incomplete torsion (180-360°) and a disease duration of up to 2 hours, the seminiferous tubules maintained their normal structure. The convoluted seminiferous tubules showed minor damage during 4 hours of ischemia caused by testicular torsion of 360-450°, which was characterized by mild damage. Glycogen and neutral glycoproteins were preserved in the cytoplasm of spermatogonia, primary spermatocytes, and Sertoli cells, indicating that their intracellular metabolism was relatively preserved. The ischemia that lasted for 4 hours with testicular torsion of 450-720° was characterized by a moderate degree of gonadal damage. However, there was pronounced expression of vimentin and calretinin, and the presence of glycogen and neutral glycoproteins indicated functional activity of the gonads. A six-hour ischemia period with a 360-450° testicular torsion demonstrated 100% gonadal viability, with 50% of the seminiferous tubules preserved and 35% with minor damage. Severe damage to the spermatogenic epithelium was observed in 15% of seminiferous tubules, characterized by dystrophy of spermatogenic epithelial cells with signs of karyopyknosis, karyorrhexis, vacuolization, hyperchromasia of cytoplasmic organelles, shedding of individual cells into the lumen of tubules, and focal necrosis. CONCLUSION: Conclusions: 1. The degree of torsion and duration of symptoms are prognostic factors for testicular salvage in torsion episodes. Ischemia lasting up to 6 hours is characterized by a moderate degree of gonadal damage, and detorsion of the testicle performed within 6 hours from the onset of pathology allows for preservation of the testicle in 100% of cases. 2. Histological examination of the susceptibility of different cell types to ischemia reveals that Sertoli cells and spermatogonia are the most resistant, while spermatocytes and spermatids are more susceptible and prone to degeneration.

BILATERAL CRYPTORCHIDISM IN PEDIATRIC SURGEON'S PRACTICE: CURRENT TACTICS OF PATIENT MANAGEMENT.

OBJECTIVE: The aim: To provide best practices of disease management to improve treatment outcomes for this group of patients. PATIENTS AND METHODS: Materials and methods: The paper is based on first-hand experience in observing and treating 117 children aged from 6 months to 13 years with bilateral congenital cryptorchidism during a ten-year follow-up period, who were referred for surgical treatment, and 3 newborn boys with undescended testicles and testicular torsion. A complex of clinical and laboratory, instrumental, endocrinological, and genetic research methods was used for the survey of all patients. RESULTS: Results: Recognizing the action of a common causative factor for bilateral cryptorchidism, which is a consequence of primary endocrine disorders, makes it possible to predict bilateral identity of the location of testicles in this pathology, which we observed in 81 patients: bilateral inguinal cryptorchidism was registered in 49 (41.88%) children, bilateral abdominal cryptorchidism - in 32 (27.35%) children, a combination of inguinal and abdominal cryptorchidism - in 24 (20.51%) children. The following types of treatment were used in the studied group of children: 1 - primary surgical intervention - 4 children, representing 3.42%. 2 - observation and non-surgical treatment by an endocrinologist - 113 (96.58%) children. 3 - comprehensive treatment (surgical correction after hormone treatment) - 67 (59.29%) children. According to the research, hormone therapy had a positive effect on descent of the testicles in 89 (78.76%) patients: the testicles descended into the scrotum - in 22 (24.72%) children; the testicles descended in the inguinal canal - in 32 (35.95%) children; the testicles descended to the level of the opening to the inguinal canal - in 35 (39.33%) children. CONCLUSION: Conclusions: 1. All doctors, starting from the maternity hospital, polyclinic, children's unit, should identify children with bilateral cryptorchidism. All children diagnosed with bilateral cryptorchidism are referred to a surgeon or endocrinologist. The parents of a child with bilateral cryptorchidism should immediately consult a doctor. The study of the reasons for late admission of children to the surgical hospital revealed that 76.92% of patients sought medical advice late, after 1 year of life. 2. At the stage of diagnosis and determination of treatment tactics, an examination by an endocrinologist and a geneticist is necessary; ignoring them is considered an error in diagnostic and therapeutic tactics, since the process of descent of the testicles into the scrotum is hormone-dependent. 3. The indications for primary surgical treatment of a child with bilateral cryptorchidism involve a combination of cryptorchidism with inguinal hernia and pain syndrome, which might be caused by testicular torsion. 4. Hormone therapy provides better results of surgical intervention in bilateral cryptorchidism. The ineffectiveness of two courses of hormone therapy necessitates surgical treatment. 5. Comprehensive treatment of children with bilateral cryptorchidism (non-surgical hormone therapy and surgical correction) has led to good postoperative results in 71.64% of patients, satisfactory results - in 22.39% of children, recurrences - in 5.97% of patients. 7. A long-term follow-up observation should be carried out by a surgeon and endocrinologist until patients reach their reproductive years.

CURRENT APPROACHES TO DIAGNOSIS AND TREATMENT OF HIRSCHSPRUNG DISEASE IN NEWBORNS AND INFANTS (LITERATURE REVIEW AND FIRST-HAND EXPERIENCE).

OBJECTIVE: The aim: To analyze the current state of the issue of Hirschsprung disease in newborns and infants on the basis of literature data and first-hand experience. CONCLUSION: Conclusions: 1. Hirschsprung disease should be suspected in all newborns with late meconium passage. 2. The main methods of diagnosing Hirschsprung disease in newborns and infants are the assessment of anamnestic data, clinical manifestations and features of the clinical course of the pathology, contrast enema, morphological examination of rectal biopsies and immunohistochemistry for ACE. 3. The presence of enterocolitis in newborns and infants should raise suspicion of Hirschsprung disease. 4. Low intestinal obstruction, perforation of the cecum, ascending or terminal small intestine, and peritonitis in the first days of a child's life may be complications of Hirschsprung disease. 5. In newborns and infants, early diagnosis of Hirschsprung disease and timely surgical correction by one-stage surgery help improve treatment outcomes.

PERFORATIVE PERITONITIS IN NEWBORNS: INSTRUMENTAL AND MORPHOLOGICAL EXAMINATION FINDINGS.

OBJECTIVE: The aim: To study and analyze the results of instrumental diagnostic measures and morphological verification of perforations of abdominal viscera in peritonitis in newborns, their importance in timely diagnosis, as well as to present authors' experience of examining newborns with PP, taking into account the complexity of diagnosis and treatment of these patients. PATIENTS AND METHODS: Materials and methods: The study is based on the results of examining 59 newborns with PP of various etiologies. Instrumental methods that were used in the diagnosis of PP and differential diagnosis of other emergencies involved the following: radiological examination (plain abdominal radiography), abdominal and retroperitoneal ultrasound, neurosonography, echocardiography, diagnostic abdominal paracentesis. Morphological verification of hollow viscus perforations was performed by methods of histologic examination in 54 newborns who underwent surgery. RESULTS: Results: It is emphasized that perforations in NEC and spontaneous gastrointestinal perforations have clinical and morphological differences. The analysis of morphological data showed absence of intestinal musculature or muscular wall defect in spontaneous gastrointestinal perforations. Perforations in NEC had massive gastric or bowel wall necrosis. Morphogenesis of gastrointestinal perforations in newborns is crucial for developing correct treatment strategy and choosing surgical approach. CONCLUSION: Conclusions: Diagnosis of PP in newborns should be comprehensive and include modern instrumental studies that enable to reliably establish the cause of peritonitis and indications for surgical treatment. Differential diagnosis of PP in newborns with other diseases aims to differentiate PP from a number of similar clinical symptoms of urgent conditions in order to conduct adequate preoperative preparation and appropriate surgery. Diagnostic markers of PP involve the following: pneumoperitoneum, free fluid in the abdomen, sentinel loop (intestinal distention), fixed bowel loop, cloudy brown or greenish intra-abdominal fluid with a large number of leukocytes and bacteria.

OBTURATION INTESTINAL OBSTRUCTION IN THE COURSE OF NECROTIZING ENTEROCOLITIS IN NEWBORN CHILDREN.

OBJECTIVE: The aim: To analyze our own results of diagnosis and treatment of newborns with NEC and obturation intestinal obstruction in order to determine theoretically important and practically significant recommendations on this issue, to optimize preventive measures for reducing intestinal obstruction cases in NEC and improving treatment outcome among newborns. PATIENTS AND METHODS: Materials and methods: Our supervision involved 143 newborns with NEC for the period from 2006 to 2020, including 79 boys (55.24%) and 64 girls (44.76%). Gestational age was 26-42 weeks. The majority of children were premature infants and numbered 121 children (84,62%). The assessment of clinical manifestations, progression of the disease, physical examination of newborns were used to help in diagnosing. All children underwent general clinical tests and instrumental methods of diagnosis including ultrasonography (abdominal organs and retroperitoneum, echocardiography, neurosonography) and X-ray examination (two-dimensional plain abdominal radiography, X-ray contrast study of the gastrointestinal tract). According to the survey, all patients were examined by allied health professionals. RESULTS: Results: Obturation intestinal obstruction associated with NEC was diagnosed in 26 children (18.18%). 12 children were in serious condition, representing 46.15%. The condition of other 14 children (53.85%) was assessed as critical one. The first signs of intestinal obstruction were observed in children with NEC at the end of the first week or at the beginning of the second week of life. Intestinal obstruction was acute in 21 children (80.77%); 5 children (19.23%) had relapsing course. Blood test results showed the inflammatory process and were not specific. According to the results of our observation, the dynamics of platelets is indicative as there is a correlation between the deterioration in the child's condition and the onset of symptoms of intestinal obstruction and thrombocytopenia. There was an imbalance in protein and electrolyte metabolism - a decrease in the level of albumin, indicators of K, Na, Ca, and an increase of C-reactive protein by several times. Ultrasonography of the abdominal organs showed irregular intestinal pneumatization, dilated intestinal loops with stagnant intestinal contents, pendulum peristalsis, infiltrative bowel wall thickening, free fluid in the abdominal cavity. Plain radiography revealed typical manifestations of small intestinal obstruction with multiple air-fluid levels. In 15 newborns (57.69%), conservative treatment was effective, in particular intestinal obstruction was relieved; 11 newborns (42.31%) underwent surgery. The mortality was 36.36% (4 children died). Postoperative complication in the form of bowel stenosis in the area of the direct anastomosis was observed in 3 patients approximately 2 weeks after the surgery. CONCLUSION: Conclusions: 1. NEC can lead to the development of obturation intestinal obstruction in newborns. Obturation intestinal obstruction was a form of NEC course in 18.18% of children under our observation. 2. Obturation intestinal obstruction in NEC in newborns is a reversible condition and requires conservative therapy. If symptoms of obstruction persist within 3 days after the start of conservative therapy, surgical treatment is indicated. 3. In case of obturation intestinal obstruction, it is advisable to perform resection of necrotic bowel, formation of a double enterostomy. 4. Enterostomy closure by placing T-shaped intestinal anastomosis should be performed 4 weeks after the primary surgery. 5. The use of modern techniques for early diagnosis and timely treatment of obturation intestinal obstruction in NEC can reduce mortality and the number of postoperative complications. 6. Newborns with NEC should be under the supervision of surgeons, which will help timely determine the indications for surgical intervention.