[Surgery in patients with severe or inhibitory hemophilia A under prevention injection of emicizumab]. / Khirurgicheskie vmeshatel'stva u bol'nykh tyazheloi ili ingibitornoi formoi gemofilii A na fone profilakticheskogo vvedeniya emitsizumaba.

OBJECTIVE: To determine the features of preoperative hemostatic therapy and laboratory control in patients with severe and inhibitory forms of hemophilia A under preventive treatment by FVIII. MATERIAL AND METHODS: Four patients with severe and inhibitory forms of hemophilia A underwent surgery between 2021 and 2022. All patients received Emicizumab (the first monoclonal drug for «non-factor¼ therapy of hemophilia) for prevention of specific hemorrhagic signs of hemophilia. RESULTS: Surgical intervention under preventive Emicizumab therapy was essential. Additional hemostatic therapy was not carried out or performed in reduced mode. There were no hemorrhagic, thrombotic or other complications. Thus, the so-called «non-factor¼ therapy is one of the variants for uncontrollable hemostasis in patients with severe and inhibitory forms of hemophilia. CONCLUSION: Preventive injection of Emicizumab ensures certain buffer of hemostasis system and stable lower limit of coagulation potential. This is the result of stable concentration of Emicizumab when used in any of the registered forms regardless of age and other individual characteristics. The risk of acute severe hemorrhage is excluded, while the probability of thrombosis is not increased. Indeed, FVIII has higher affinity than Emicizumab and displaces Emicizumab from coagulation cascade that does not result summation of total coagulation potential.

[Pharmacotherapy of portal hypertension and its complications: analysis of efficacy of preparations for clinical practice and discussion of promising methods of treatment].

Bleeding from oesophageal varicose veins is the terminal stage of a sequence of complications of liver cirrhosis caused by progressive fibrosis, circulation blockade, and development of portal hypertension syndrome followed by collateral shunt. It leads to progressive vein dilation and their rupture. The main issue of today is to prevent the development of successive stages of portal hypertension, to search for therapeutic and surgical methods for marked reduction of pressure in the portal system, and to prevent the risk of hemorrhage from varicose veins. Another approach is to use local endoscopic treatment of varicose veins for prevention of their rupture. The authors analyse the efficacy of pharmacotherapy in patients with liver cirrhosis and portal hypertension and discuss the existing recommendations on the prevention of hemorrhage with special reference to the yet unsolved problems and prospects for the improvement of therapy.

[Surgical treatment of portal hypertension: the state of art].

The article highlights modern approaches to the treatment of portal hypertension. The differential tactics is based on the type of portal hypertension, functional liver state, urgency of the situation and severity of blood loss, localization and stage of varices, concomitant diseases, etc. The role of miniinvasive methods is stressed. The reasonability of general treatment algorithm of portal hypertention in specialized centers is proved.

[Syndrome of extrahepatic portal hypertension and chronic abdominal ischemia in patient with subleukemic myelosis and congenital immunodeficiency].

The article demonstrates objective difficulties and subjective mistakes in assessment of a clinical picture in a patient with subleukemic myelosis and thrombosis of the portal system. A careful examination of such patients is necessary to reveal latent disorders (in this case--a complete selective IgA deficiency) influencing treatment policy.

[Study of central and porto-hepatic hemodynamics before the surgical treatment of patients with liver cirrhosis and portal hypertension]. / Tsentral'naia i portopechenochnaia gemodinamika pri khirurgicheskom lechenii bol'nykh tsirrozom pecheni i portal'noi gipertenziei.

Basing on the results of hemodynamic findings related to the heart and liver in 41 patients with hepatic cirrhosis and portal hypertension (33 of them were operated on for portal hypertension), it is suggested that when deciding on the type of surgery in such patients, initial status of central and portal hemodynamics as well as expected changes in these parameters due to surgery should be taken into consideration. Pathogenetic grounds for some routine interventions in hepatic cirrhosis and portal hypertension are thought open to question.

[Use of a gastrointestinal tube in the treatment of gastric and duodenal ulcer]. / Primenenie gastrointestinal'nogo zonda v lechenii iazvennoi bolezni zheludka i dvenadtsatiperstnoi kishki.

The trial of a gastrointestinal tube in gastroduodenal ulcer indicates its high efficiency for enteral tube feeding in serious ulcer cases and as a therapeutic modality in noncomplicated cases. The tube introduction in 55 patients out of 89 promoted the defect healing within 2-3 weeks, in 30 out of 36 cicatrization occurred on week 3-5. The presence of the tube produced no effect on acid production in the stomach, cicatrization of the ulcer took place without basic therapy in remaining hypo-achlorhydria and hyperchlorhydria. Clinico-endoscopic evidence and data on gastroduodenal motility and evacuation suggest the conclusion on the neuro-reflex mechanism of the tube action which eliminates painful syndrome and motor-evacuatory abnormalities which are believed essential in pathogenesis of peptic ulcer.

[Pathogenesis of esophagitis in patients with portal hypertension]. / O patogeneze ézofagita u bol'nykh portal'noi gipertenziei.

Acidogenic function of the stomach and role of gastroesophageal reflux were assessed in relation to esophagitis genesis in 92 patients with portal hypertension and varicosity of the stomach and esophagus. Acidogenic function was found heterogenous. The relationship between gastric acidity, gastroesophageal reflux and esophagitis development seemed obscure, whereas that between esophageal varicosity of a definite degree and esophagitis was clear-cut.

[Results of suturing of esophageal and gastric varicose veins in patients with extrahepatic portal hypertension]. / Rezul'taty proshivaniia varikozno-rasshirennykh ven pishchevoda i zheludka u bol'nykh s vnepechenochnoi portal'noi gipertenziei.

Experience in gastrectomy with suturing of varicose veins (VV) of the esophagus and stomach (M.D. Patsiora's surgery) in 349 patients with extrahepatic portal hypertension (EPH) is reviewed. Short and long-term results of this surgery through abdominal and transthoracal approach are analyzed. Postoperative lethality in patients with EPH after elective surgery was 5.0%, urgent surgery--16%. Recurrence of esophagogastric bleeding in postoperative period was seen in 23 (6.6%) patients. 286 patients were followed up from 1 to 10 years. Recurrence of esophageal-gastric bleeding was seen in 104 (36.4%) patients and led to death in 19 (18.3%) of them. It is concluded that suturing of esophageal and gastric VV in patients with EPH is a method of choice both in prolonged bleeding and as preventive elective surgery when creation of vascular anastomosis is impossible.

[Management strategy for pregnant women with portal hypertension syndrome]. / Taktika vedeniia beremennykh s sindromom portal'noi gipertenzii.

The authors describe their management policy with pregnant patients suffering from portal hypertension syndrome, based on the results of comprehensive examinations and deliveries in 44 women. Principal indications for abortions in such patients are active liver cirrhosis, third-degree varicosity of esophageal and gastric veins, dilatation of the esophagus. Natural labor is permitted when there are no threatened bleedings. Therapy is administered in accordance with obstetrical indications. In case esophagogastric bleedings develop emergency measures to arrest the bleeding are taken, rapid delivery and, if necessary, emergency surgical intervention. Knowledge of risk factors helps the problem of pregnancy maintenance and delivery mode to be solved by obstetricians and specialists in portal hypertension. This management policy promoted successful deliveries in 42 of 44 cases.