Features of Changes in Blood Parameters of Some Laboratory Syndromes and their Constellations in Patients with Liver Cirrhosis with Disorders of Bone Mineral Density

Introduction. Investigation of changes in certain laboratory blood parameters, and verification with their help of laboratory syndromes, and detection of constellations of laboratory syndromes in patients with liver cirrhosis (LC), which is possible for clinicians of all levels of medical care, need to clarify their features, which would suspect or verify disorders of bone mineral density (DBMD). The aim of the study. Investigate the features of changes in blood parameters of some laboratory syndromes and their constellations in patients with liver cirrhosis with disorders of bone mineral density. Materials and methods. 90 patients (27 women (30.0 %) and 63 men (70.0 %) aged 18 to 66 years) with LC were stratified into several groups: experimental (EG) (patients with LC with DBMD) (72 patients (80.0 %))), from which two subgroups were formed - EG A (patients with LC with osteopenia) (46 patients (63.9 %))), and EG B (patients with LC with osteoporosis) (26 patients (36.1 %)))) and the comparison group (CG) (patients with LC without DBMD) (18 patients (20.0 %))). Among the laboratory syndromes and their blood parameters were studied such as: cytolysis (increased in plasma alanine aminotransferase (ALT) and/or aspartate aminotransferase (AST)), mesenchymal-inflammatory syndrome (increased thymol test (TT) and/or gamma-globulins), hepatocellular insufficiency (decreased fibrinogen, prothrombin index (PTI), total protein, or albumin), cholestasis (increased alkaline phosphatase (AP), gamma-glutamyltranspeptidase (GGTP), total bilirubin), porto-systemic shunting (decreased sodium and/or potassium, and/or increased creatinine) and dyslipidemia (increased serum cholesterol, B-lipoproteins, triglycerides, low-density lipoprotein (LDL), decreased high-density lipoprotein (HDL)). The study was performed in three stages, the first of which studied the features of laboratory syndromes and blood parameters that characterize them, the second - constellations of laboratory syndromes, and the third - the simultaneous manifestation of a number of different laboratory syndromes in patients with LC with DBMD, osteopenia and osteoporosis. Each stage involved three steps: the first was to study the frequency of laboratory syndromes and their laboratory blood parameters in patients with LC and determine their share in each of the study groups, the second was to identify significant differences in the frequency of cases, and the third was to identify a direct stochastic relationship between the studied trait and DBMD, including osteopenia and osteoporosis. Results. After performing all three stages and each of the planned steps, it was found that laboratory syndromes and their constellations are more common among patients with bone lesions. However, there are statistically significant differences in the frequency of cases between EG and CG in the case of a decrease in HDL and the simultaneous manifestation of five different laboratory syndromes; between EG A and CG - decrease in HDL and simultaneous manifestation of two and three different laboratory syndromes; between EG B and CG - increase in AP, decrease in HDL and simultaneous manifestation of five different laboratory syndromes; between EG A and EG B - cytolysis syndrome, increase in AST, gamma-globulins, AP, constellation of cytolysis syndrome with hepatocellular insufficiency syndrome or cholestasis syndrome and constellation of all three syndromes. Confirmed direct stochastic association was found: with all manifestations of DBMD - increase in TT, a decrease in HDL, and constellations of cytolysis, mesenchymal-inflammatory and dyslipidemic syndrome, which may be supplemented by hepatocellular insufficiency syndrome and/or cholestasis syndrome; with osteopenia - increase in TT, increase in blood cholesterol, decrease in HDL, and constellations containing dyslipidemia syndrome and supplemented by mesenchymal-inflammatory, and/or cytolysis and/or hepatocellular insufficiency and/or cholestasis syndromes, and simultaneously only two laboratory syndromes in a patient with LC; with osteoporosis - increase in blood AST, TT, gamma-globulins, AP, decrease in PTI, potassium, HDL, the presence of cytolysis, cholestasis syndromes, constellations of cytolysis syndrome with hepatocellular insufficiency syndrome and/or cholestasis syndrome, which are supplemented by mesenchymal-inflammatory and dyslipidemic syndrome, and the simultaneous manifestation only three or five different laboratory syndromes. Conclusions. Laboratory syndromes, blood parameters that characterize them, and constellations of laboratory syndromes have certain features in patients with cirrhosis of the liver with disorders of bone mineral density, as in most cases are more common in patients with bone lesions and have a confirmed stochastic relationship with disorders of mineral density bone tissue in general, and osteopenia and osteoporosis separately. Keywords: cirrhosis, bone mineral density, osteopenia, osteoporosis, cytolysis, mesenchymal-inflammatory, hepatocellular insufficiency, cholestasis, porto-systemic shunting, dyslipidemia, alanine aminotransferase, aspartate aminotransferase, thymol test, total protein, albumin, gamma-globulin, fibrinogen, prothrombin index, alkaline phosphatase, gamma-glutamyltranspeptidase, bilirubin, sodium, potassium, creatinine, cholesterol, B-lipoproteins, triglycerides, low-density lipoproteins, high-density lipoproteins.

Method of Enteral Insufficiency Syndrome Correction in Patients with Generalized Peritonitis

Introduction. Mortality in generalized peritonitis (GP) reaches 30%, and with the development of multiple organ failure, the lethal outcome is observed in 80-90% of cases. Enteral insufficiency syndrome (EIS) plays a leading role in the progression of generalized peritonitis. The aim of the study was to develop a differentiated approach of enteral insufficiency syndrome correction in patients with generalized peritonitis. Material and methods. This research was a retrospective prospective study. The study included 50 patients with GP, who received treatment at the Surgery Department of the Samara Regional Clinical Hospital in the period from 2017 to 2019. Depending on the chosen treatment tactics, the patients were divided into two clinical groups. Group I included 29 patients, admitted in the period from 2017 to 2018, who had received the standard GP treatment. A long-term endogenous intoxication in patients of this group associated with the progressive enteric failure led to the repeated surgeries; at the same time, a high frequency of postoperative complications was preserved. The analysis of the results in patients of Group I necessitated development of the therapeutic and diagnostic algorithm aimed at early diagnostics and timely correction of EIS. Group II included 21 patients with GP, admitted in the period from 2018 to 2019, who was treated using the new algorithm. Results. The objective criteria for the relief of EIS in GP in patients of the study groups were a decrease in the level of serum albumin and C-reactive protein, a significant decrease in the amount and qualitative change in the intestinal discharge via an intestinal tube, a decrease in the recovery time of the functions of the small intestine and start of defecation. On the 6th postoperative day, in patients of Group II there was no significant albumin level reduction in comparison with the 1st day of monitoring (28.310.77 g/l vs 37.334.69 g/l). Whereas in Group I the albumin level was significantly lower (19.30.51 g/l) than the same parameter in Group II, and in comparison with the 1st day of monitoring (19.30.51 g/l vs 39.56.05 g/l; р = 0.00001). On the 6th postoperative day, the C-reactive protein level differed significantly between the groups as well: Group I 104.7613.49 mg/l, Group II - 58.0029.05 mg/l, p = 0.003. The control of GP in patients of the Group I was reached after 4.52.5 repeated abdominal interventions, while in patients of Group II generalized peritonitis was arrested after 2.30.9 surgical interventions (p = 0.000171), which is 1.9 times less. Conclusions. The proposed algorithm of EIS control is based on the individual approach to the treatment of patients with GP. The developed EIS rating scale allows determining not only the degree and dynamics of the pathological process, but also monitoring the effectiveness of treatment options applied in a particular patient.

Algorithm of the diagnosis and treatment of enteral insufficiency syndrome in peritonitis

RELEVANCE. The early diagnosis and correction of enteral insufficiency syndrome influence the treatment outcomes in patients with abdominal sepsis and multiple organ failure.The OBJECTIVES was to improve the effectiveness of diagnosis and correction of enteral insufficiency syndrome in patients with generalized peritonitis using the express assessment scale of the severity of enteral insufficiency and the treatment algorithm based on this.MATERIALS AND METHODS. The express assessment scale of the severity of enteral insufficiency syndrome was developed along with the treatment algorithm depending on the detected degree for systematic approach to the management of patients with generalized peritonitis. The study included 39 patients with generalized peritonitis (GP) who had received treatment in the Surgery Department of Samara Regional Clinical Hospital named after V. D. Seredavin in the period of 2019–2020. A computational program was created for quick severity evaluation of enteral insufficiency syndrome and choosing the best treatment strategy.RESULTS. When comparing the severity of enteral insufficiency in patients immediately after the first operation and in 96 hours, a positive dynamics was registered: the number of patients with III degree of severity of enteral insufficiency syndrome decreased from 19 to 4 people, and the number of patients with I degree of severity of enteral insufficiency syndrome changed from 3 to 29 people, this indicated the stabilization of the condition of patients with GP (p<0.05). The statistically significant reduction in the number of points in the limits of each severity was evaluated as the proof of clinical efficacy of the algorithm applied for the enteral insufficiency syndrome correction (p<0.05).CONCLUSION. Due to the correct choice of the treatment algorithm according to the identified severity of enteral insufficiency syndrome, the regression of clinical signs of generalized peritonitis was registered in all patients on the 5th postoperative day (96 hours after surgery).

MAIN FACTORS OF DECOMPENSATION SYSTEM I NFLAMMATORY REACTION SYNDROME THE PATIENTS WITH ABDOMINAL SEPSIS

Summary. The goal of the robot is to determine the factors of decompensation of the systemic inflammatory response syndrome (SIRS) in abdominal sepsis (AS). Materials and methods. Based on the results of a comprehensive examination of 295 patients with AS according to the indicators of clinical and laboratory, biochemical, immunological examination and study of intra-abdominal pressure and the severity of enteral insufficiency, the leading factors in the development of DSIRS were determined. Results and discussion. It was found that against the background of secondary cellular immunodeficiency, the development of severe compartment syndrome with decompensated enteric insufficiency syndrome (EIS) was determined, which in combination deepened pathological changes with the progression of the inflammatory reaction and the development of organ failure. Indicators of the level of C-reactive protein 2.5 times, and procalcitonin 2.4 times were higher during hospitalization of patients with decompensated syndrome (P<0.001). With decompensation, a severe degree of SES was diagnosed 18.5 times more often, and with a compensated one, a mild degree of insufficiency was diagnosed 57 times more often, P <0.001. At the same time, a direct correlation was determined between the severe degree of EIS and symptoms of nausea (r = 0.420), vomiting (r = 0.573) and bloating (r = 0.251), (P <0.005). The immunoregulatory index (IRI) played the role of a marker of decompensation in patients with AS, (r = + 0.74, at p <0.01) with the development of secondary immunodeficiency, according to the T-suppressor type. In 60.8 % (n = 101) of cases with DSIRS, the fourth degree of intra-abdominal pressure was determined, on average it was (46.3 ± 6.3) mm, and I degree was determined only in the case of compensation, (P <0.001). More often in patients with DSIRS, the associations of gram-positive microorganisms and enterococci were determined — in 55.6 % and streptococci — in 38.1 % of cases. At the same time, in 81.3 % of cases, patients with DSIRS were diagnosed with aerobic-anaerobic mixed flora. Сonclusion. The obtained results of the study require the development of treatment methods that will effectively correct these pathogenetic changes in all directions in patients with AS.

Electrochemical detoxification in cancer patients after multiorgan surgery with severe endogenous intoxication

Background. Enteral insufficiency syndrome accompanies the development of many acute diseases of the abdominal cavity. According to the statistics, advanced and multi-organ surgical intervention in oncosurgery within the period from 2019 to 2020 resulted in enteral insufficiency being a complication in 39% of all cases, regardless of anatomical and physiological area, while complications in the form of endogenous intoxication syndrome made up 68% of cases. Purpose – studying the effectiveness of treatment of enteral insufficiency syndrome in cancer patients after multiorgan surgery with severe endogenous intoxication by means of indirect electrochemical detoxification with sodium hypochlorite solution. Materials and methods. The study involved 71 cancer patients who underwent multi-organ surgery on different anatomical and physiological areas. The patients were divided into 2 groups: treatment group (n=36) provided with indirect electrochemical detoxification by means of sodium hypochlorite solution at a concentration of 0.06%, comparison group (n=35) undergoing treatment according to standard schemes. The groups were comparable in age and anatomical and physiological areas that were operated on (surgery was performed on the chest and mediastinum as well as abdominal organs). Results. The method of electrochemical detoxification with sodium hypochlorite reducing the indicators that reflect blood toxicity and intoxication level was used for treating patients with enteral insufficiency. This was evidenced by decreased concentration of bilirubin by 23.1%, urea by 91.6%, creatinine by 99.4%, LII (leukocytal intoxication index) by 47.2% and procalcitonin by 68.2%. Being applied this method has made it possible to achieve a detoxifying effect early on day one. Conclusions. The findings of complete physical examination of cancer patients after multiorgan surgery with severe endogenous intoxication have shown a practical significance of sodium hypochlorite being included in comprehensive post-surgery treatment in enteral insufficiency syndrome cases. Infusions of 0.06% NaClO solution within 24 hours have been proved to provide a detoxifying effect: they significantly reduce elevated concentrations of bilirubin, creatinine, urea, LII and improve blood rheology. Administering sodium hypochlorite in the suppre-ssion of antioxidant defense mechanisms leads to the activation of oxidative processes. Including sodium hypochlorite in comprehensive post-surgery treatment in enteric insufficiency syndrome has shown a high efficiency.

46, XY Disorder of Sexual Development with Ambiguous Female External Genitalia: A Case Report

Disorder of Sexual Development (DSD) is a group of congenital conditions with atypical development of sex at chromosomal, gonadal or anatomic level. Genetic males with DSD (46 XY DSD) can present with female external genital phenotype, ambiguous, or a micropenis. It is caused by incomplete intrauterine masculinization with or without the presence of Müllerian structures. It results either from decreased synthesis of testosterone or DHT or from impairment of androgen action. Herein, we report a case of a 13-year child raised as female with hoarseness of voice and gradual enlargement of clitoris with hormonal assessment not suggestive of either 5 Alfa Reductase deficiency, Congenital Adrenal Insufficiency Syndrome or 17β-Hydroxysteroid Dehydrogenase deficiency

Sphingosine 1- Phosphate Lyase Insufficiency Syndrome (SPLIS); A Role in Multiple Endocrinopathies

Sphingosine 1-phosphate lyase insufficiency syndrome (SPLIS) was described in 2017 as a novel condition affecting sphingolipid metabolism. There is a multisystemic phenotype including nephrotic syndrome and primary adrenal insufficiency (PAI) and to a lesser extent ichthyosis, neurological disease and lymphopenia. A proportion of patients also presented with hypothyroidism and hypogonadism. To interrogate the endocrine aspect of the syndrome we reviewed clinical data within our patient cohort with SPLIS and those within the wider literature. To date there have been 45 patients identified with SPLIS with significant associated mortality (n=23/45, 51%; 4 of these in utero). There is no clear genotype-phenotype correlation. Whilst nephrotic syndrome is most prevalent (n=34/45; 76%), a significant proportion of patients (n=27/45, 60%) also presented with glucocorticoid deficiency, some with additional mineralocorticoid deficiency (n=7/27). Five further patients were noted to have adrenal calcifications though biochemistry was not undertaken. Most patients presented with PAI in the first 2 years of life (n=21/27), with the oldest presentation being 11 years of age. Adrenal calcifications are a common finding in those who had documented imaging (n=13/15, 87%). Primary gonadal failure has been reported in 8 male cases, all with concomitant PAI. Presenting features included microphallus (n=7/8) and cryptorchidism (n=8/8), indicating reduced in utero androgen exposure. All who had biochemical evaluation demonstrated raised basal LH and FSH/ exaggerated response to LHRH stimulation, a lack of testosterone response to HCG stimulation and low antimullerian hormone (AMH) levels. To date there are no reports of pubertal delay in female patients, and those of age within our cohort have normal ovarian reserve as evidenced by AMH levels (n=2). Primary hypothyroidism, with mildly raised TSH and low Free T4 is reported in 12 patients. Most did not have goiters and had concomitant PAI and nephrotic syndrome (n=11/12). SPLIS is unique amongst sphingolipid disorders in presenting with significant endocrinopathy. This may be the consequence of the particular sphingolipid signature of the disease and the pathogenic mechanisms need to be explored further. It is clear that endocrine dysfunction needs to be considered at diagnosis and surveillance undertaken to detect evolving disease which could have a significant impact on morbidity and mortality.

Enteral insufficiency syndrome: current provisions about the terminology, pathogenesis and treatment (review of literature)

An analytical review of the literature on the pathogenesis of disorders of the motor-evacuation function of the intestine, which underlies the enteral insufficiency syndrome (EIS), which develops in various acute intra-abdominal surgical diseases, is presented. On the basis of a multivariate analysis of literature data, various pathogenetic mechanisms of enteric dysfunctions caused by morphological and structural changes in the wall of the small intestine, violations of its local defense mechanisms are described. The essence of the modern concept of the pathogenesis of enteral insufficiency – enteral distress syndrome (EDS) according to the literature is presented. According to new views, EDS is a combination of various pathogenetic mechanisms that are formed as a result of dysregulation and destabilization of biological membranes of tissue structures of the intestinal wall (especially its mucous membrane) and a violation of the functional and metabolic status of the intestine, developing in acute surgical diseases of the abdominal organs. Recognition of the validity of the concept will allow unifying the terminology and creating more evidence-based and generally accepted teaching about the nature of EIS.