[Electroencephalogram features in children with congenital hyperinsulinism treated according to the international protocol in Russian Federation].

BACKGROUND: Congenital hyperinsulinism (CHI) is a rare life-threatening disease characterised by persistent hypoglycaemia as a result of inappropriate insulin secretion, which can lead to irreversible neurological defects in infants. AIM: To evaluate neurophysiological characteristics of central nervous system in children with congenital hyperinsulinism treated according to the international protocol in Russian Federation. MATERIALS AND METHODS: Our retrospective, prospective cohort study included 73 patients who received treatment for CHI according to the current international protocol at different departments of the Almazov National Medical Research Centre from 2017 to 2022. All patients underwent a comprehensive examination, including electroencephalography (EEG). RESULTS: Among 73 patients with CHI, 35% (23) had focal form of the disease, 65% had non-focal form (49% (39) - diffuse form, 16% (11) - atypical form). All patients with focal form of CHI had a recovery as an outcome.Analysing the EEG data we found that paroxysmal activity was recorded in 23 patients (32%), 50 patients did not have paroxysmal activity (68%). Diffuse changes were observed in 47 patients (64%), whereas 26 patients (36%) were absent of it. By constructing Kaplan-Meier curves we found that the alpha rhythm is formed significantly (p=0.026) earlier in patients with a focal form of CHI. CONCLUSION: CHI patients treated according to the international guidelines in Russian Federation show rather positive neurological outcome. We established that alpha rhythm earliest formation is associated with focal form of CHI.

[Practical guidelines for the diagnosis and treatment of transthyretin amyloid cardiomyopathy (ATTR-CM or transthyretin cardiac amyloidosis)].

This paper summarizes the data from updated international protocols and guidelines for diagnosis of transthyretin amyloid cardiomyopathy (ATTR-CM). The invasive and non-invasive diagnosis techniques and their combinations are briefly reviewed; the evidentiary foundations for each diagnostic option and tool are analyzed. The paper describes a customized algorithm for sequential diagnosis and differential diagnosis of patients with suspected ATTR-CM with allowance for the combination of clinical signs and diagnostic findings. Along with the awareness of primary care providers about the red flags of the disease and visualization criteria, as well as providing information to the patients about the possibility of performing therapy of ATTR amyloidosis and the risks of delayed diagnosis, the proposed algorithm enables timely patient routing and prescribing specific treatment.

[Clinical, genetic, and radionuclide characteristics of the focal form of congenital hyperinsulinism].

BACKGROUND: Congenital hyperinsulinism (CHI) is a severe disease with a high risk of complications including neurological deficit. Persistent hypoglycemia in patients with focal form of CHI can not be managed with medical treatment in 96.4% of cases, what subsequently leads to surgical treatment. Currently, there is a lack of information regarding patients with focal form of CHI. This study is aimed at finding better approaches for diagnosis and treatment of patients with focal form of CHI. AIMS: To study clinical, genetic and PET/CT findings of the focal form of CHI in Russian group of patients. MATERIALS AND METHODS: The observational research included all patients with a histologically confirmed focal form of CHI, who were admitted to Endocrinology Research Centre during the period from January 2008 to January 2019. A statistical analysis of clinical data, genotype, and positron emission tomography (PET) with 18F-dihydroxyphenylalanine (18F-DOPA) was performed. The median follow-up was 18 months. RESULTS: The study included 31 patients with focal CHI (14 boys, 45.2%). All patients had a neonatal presentation of the disease and demanded high levels of continuous glucose infusion to maintain euglycemia. The difference between the age of hypoglycemia presentation and the age of diagnosis ranged from 1 day to 3.9 months. In all cases, diazoxide was found to be ineffective. However, in 9 patients, it was possible to withdraw continuous glucose infusion and maintain euglycemia using octreotide in the preoperative period. A molecular genetic study allowed us to detect diverse pathogenic variants in ABCC8 and KCNJ11 genes in 30 patients. According to PET data with 18F-DOPA, the pancreatic index (PI) varied widely from 1.16 to 3.59. After partial resection of the pancreatic region with insulin hypersecretion, all patients showed complete recovery. CONCLUSIONS: The focal form of CHI is a severe condition with high prevalence of neurological complications. For preoperative diagnosis of the morphological form of the disease, it is necessary to conduct genetic analysis and radionuclide studies. Solely evaluation of mathematical parameters in 18F-DOPA PET without taking into account the visual data and the results of genetic analysis does not allow establishing the robust diagnosis. Timely diagnosis, identification of risk factors, and prevention of complications of persistent hypoglycemia are important tasks for clinicians.

[First experience of using positron emission tomography with 11C-choline in prostate cancer (PC)].

Introduction into clinical practice of combined positron emission technology and computer tomography (PET/CT) allows in one study to identify structural and functional abnormalities. The study involves 32 patients who underwent PET/CT with "C-choline, including 5 patients with prostate cancer (PC), 3--with chronic prostatitis and 24--with biochemical PC recurrence. PET/CT with 11C-choline has a high diagnostic efficacy in detection of local recurrence and PC metastases in patients with biochemical PC recurrence. The results of visual analysis do not permit to distinguish PC from benign prostate diseases.

[Long-term results of clinical application of autologous mononuclear bone marrow fraction for regeneration therapy of ischemic heart disease patients].

An experience with using autologous bone marrow mononuclears for regeneration of the heart was analyzed in 97 patients in whom the intracoronary transplantation of autologous mononuclear bone marrow cells was performed. The results were estimated in terms up to 5 years and compared with a group of 37 patients who underwent only conservative treatment. A distinct positive dynamic of clinical and echocardiographic indices in the main group was noted in a subgroup of patients with a decreased ejection fraction (EF less than 50%) as compared with an analogous subgroup of patients in the control group. Substantial influence is exerted by regeneration therapy upon remote lethality. Thus, as a whole in the main group lethality over 5 years was 13.4% and in the group of control it was 21.6%. In the subgroup with a decreased ejection fraction and symptoms of heart failure lethality was 22.6% in the main group and 54.5%--in the control group. The intracoronary administration of the autologous bone marrow mononuclear fraction to inoperable patients with ischemic heart disease and a severe lesion of the coronary arteries and a decreased ejection fraction of the left ventricle is a safe and useful procedure resulting to substantially decreased lethality followed-up during 5 years against the background of conservative treatment.

[Evaluation of coronary bed function by positron emission tomography using 13N-ammonium during cold stimulation].

The purpose of the present investigation was to study the time course of changes in myocardial blood flow (MBF) in response to cold stimulation. Thirty-eight patients having risk factors of cardiovascular complications were examined. The time course of MBF changes was estimated by positron emission tomography (PET) using 13N-ammonium at rest and during a cold test (CT). Endothelium-dependent vasodilation of the brachial artery was determined from the results of a reactive hyperemia test, by applying ultrasound duplex scanning. No significant MBF increase in response to the cold test was an indicator of coronary arterial endothelial dysfunction at cardiac 13N-ammonium PET. Agreement of the results of brachial arterial ultrasonography during reactive hyperemia and cardiac 13N-ammonium PET in the presence of the cold test suggests that endothelial dysfunction is generalized. Cardiovascular risk factors, such as left ventricular hypertrophy, smoking, dyslipidemia, and diabetes mellitus, substantially affect coronary arterial function. Left ventricular hypertrophy is an independent factor that influences the amount of the coronary reserve and, in combination with endothelial dysfunction, worsens coronary microcirculation.

[Role of 18F-FDG PET scan in the differential diagnosis of large pancreatic masses].

Results of standard 18FDG PET and double-phase scanning were compared in the course of differential diagnosis of pancreatic cancer and chronic pseudotumorous pancreatitis, particularly, during exacerbation. PET diagnosis of cancer was confirmed in 6.5% while that of double-phase scanning--100%.

[Role of 18F-FDG PET in tumor grading and evaluating effectiveness of therapy in malignant lymphomas].

The report compares the results of (multi-layer spiral computed tomography) (MSCT), 67 Ga citrate lymph scintigraphy and 18FDG PET in patients with Hodgkin's disease. The predictive significance of the latter method exceeded that of MSCT and ultrasound in diagnosing lymph node neoplasia below the diaphragm. As far as peripheral and mediastinal lymph nodes are concerned, the MSCT efficiency of the three procedures was comparable. Advantage was offered by PET in early evaluation of therapeutic effectiveness. Moreover, its practical significance for assessing tumor sensitivity to therapy as well as differentiation between viable tumor tissue and fibrosis was demonstrated.

[Positron emission tomography with 18-fluorine deoxyglucose in the diagnosis and assessment of prostate cancer].

Results of PET studies using 18-fluorine deoxyglucose (18-FDG) in patients with large-size masses (51) are discussed. Histologically-confirmed prostate cancer was diagnosed in 36 (70.5%), benign hyperplasia--12 (23.5%) and chronic prostatitis--3(6%). 18FDG PET was conducted as whole body irradiation. Our results established its high predictive significance in identifying the scope of tumor involvement. However, the latter's potential is limited in diagnosis of primary tumor node due to low rate of glycolysis in it.

[Evaluation of coronary bed function by positron emission tomography using 13N-ammonium during cold stimulation].

The present investigation was to study the time course of changes in myocardial blood flow (MBF) in response to cold stimulation. Thirty-eight patients having risk factors of cardiovascular complications were examined. The time course of MBF changes was estimated by positron emission tomography (PET) using 13N-ammonium at rest and during a cold test (CT). Endothelium-dependent vasodilation of the brachial artery was determined from the results of a reactive hyperemia test, by applying ultrasonic duplex scanning. No significant MBF increase in response to the cold test was an indicator of coronary arterial endothelial dysfunction at cardiac 13N-ammonium PET. Agreement of the results of brachial arterial ultrasonography during reactive hyperemia and cardiac 13N-ammonium PET in the presence of the cold test suggests that endothelial dysfunction is generalized. Cardiovascular risk factors, such as left ventricular hypertrophy, smoking, dyslipidemia, and diabetes mellitus, substantially affect coronary arterial function. Left ventricular hypertrophy is an independent factor that influences the size of the coronary reserve and, in combination with endothelial dysfunction, worsens coronary microcirculation.

[Twelve years' clinical experience in using positron emission tomography: advances and prospects].

The paper describes 12 years' experience in using positron emission tomography (PET) at the Russian Research Center of Radiology and Surgical Technologies to detect cancer, cardiac, and psychoneurological diseases, to make their differential diagnosis, and to evaluate the efficiency of their treatment. It shows the capacities of PET using various radiopharmaceutical agents in a broad spectrum of the above abnormalities and defines prospects for further development of the technique.

[Clinical experience in using 18F-FDG PET in the staging and restaging of malignant lymphomas].

The present study was undertaken to determine the diagnostic accuracy of positron emission tomography (PET) in the staging and restaging of malignant lymphomas. For determination of the extent of a neoplastic process, complex radiation examination was conducted in 67 patients with malignant lymphomas. In 12 (17.9%) cases, the stage of the disease was changed, as evidenced by PET. There is evidence that the technique is of high diagnostic accuracy in detecting malignancies of l ymph nodes, skeletal system, and parenchymatous organs in lymphoproliferative diseases.

[Current methods of noninvasive imaging of coronary arteries in the diagnosis of coronary atherosclerosis].

The lecture contains: presentation of possibilities of ultrasound and tomographic methods of investigation in visualization of major coronary arteries; consideration in a comparative aspect of main advantages, disadvantages and limitations of these methods in diagnostics of coronary atherosclerosis; analysis of indications for application and perspectives of their use in everyday clinical practice in patients with a diagnosis of possible or verified ischemic heart disease.

[Effectiveness of using autologous mono-nuclears of the bone marrow in treatment of patients with ischemic heart disease].

The authors have analyzed their experiences with treatment of 50 patients with ischemic heart disease using transplantation of autologous mono-nuclears of the bone marrow. It was shown that this operation resulted in an improvement of indices of the heart functions and myocardium metabolism. Transplantation of stem cells as mononuclear fraction of the bone marrow is indicated in treatment of different groups of patients: in recurrent diseases after previous operations on the coronary arteries; in patients with distal lesions of the coronary bed; transplantation of autologous stem cells of the bone marrow is expedient simultaneously with coronary artery bypass grafting or coronary angioplasty (stenting).

[Positron emission tomography in oncourology].

The authors present the results of the examination of 61 patients with genitourinary space-occupying lesions, using 18F- fluorodeoxyglucose positron emission tomography (PET) in whole body mode. In all cases the diagnosis was verified morphologically. The results demonstrated high diagnostic accuracy of PET, including possibility to determine the extent of oncourological cancer. However, the method displays poor efficacy in cases of hypernephroid cancer due to low level of glycolysis in this type of tumor.

[The role of positron emission tomography with C18-Fluorodeoxyglucose in clinical oncology]. / Primenenie pozitronnoi émissionnoi tomografii C18-F-ftordezokcilgliukozoi v klinicheskoi onkologii.

The study was concerned with evaluation of the diagnostic potential of positron emission tomography (PET) with 18-FDG in clinical oncology and elucidation of its role in assessing therapy effectiveness. The Ecat Exact 47 and Ecat Exat HR+ (Siemens) insatllations were used to examine 674 patients, with Whole Body protocol used in 585. PET with 18-FDG proved highly effective in diagnosing malignancies of the breast, lung, liver, pancreas, testis, brain and lymphoma as well as evaluating the efficacy of therapy.

[Assessment of 18FDG PET for diagnosis of pancreatic tumors]. / Vozmozhnosti PET s 18F-FDG dlia diagnostiki opukholei podzheludochnoi zhelezy.

The paper examines the informational value of positron emission tomography (PET) using 18FDG in the diagnosis of malignant of neoplasms of the pancreas and in the estimation of the extent of a metastatic involvement. Forty-four patients (26 males and 18 females whose age ranged from 28 to 60 years) with histologically verified cancer of the pancreas were examined. The study was conducted in the whole body mode on an Ecat Exact 47 positron emission tomograph following 70-90 minutes of administration of 18FDG, 370-420 MBk. To assess the findings, the differential accumulation ratio (DAR) of formation/liver was calculated. The mean DAR in patients with benign and malignant pancreatic tumors was 1.17 +/- 0.064 and 4.90 +/- 0.3 (p < 0.05). There was a false positive case in a patient with an exacerbation of chronic pancreatitis in the study. A relationship was observed between the level of tumor tissue 18FDG capture and the degree of malignancy. PET scanning in the whole body mode estimates the extent of a tumorous process. The authors' data show that the liver was most commonly involved in a metastatic process (96.6%). Hence, 18FDG PET is a highly informative technique in the diagnosis of malignant pancreatic tumors and in the estimation of the extent of a metastatic process and permits a differential diagnosis between benign and malignant tumors.

[Comparative assessment of the diagnostic capacities of PET with 18FDG and 1-[11C] sodium butyrate in the examination of patients with tumors of the brain and cerebral circulatory disorders: results of semiquantitative assessment]. / Sravnitel'naya otsenka diagnosticheskikh vozmozhnostei PET s 18F-FDG i 1-[11C]-butiratom natriia pri obsledovanii bol'nykh s ob"emnymi obrazovaniiami golovnogo mozga i narusheniiami mozgovogo krovoobrashcheniia (rezul'taty polukolichestvennoi otsenki dannykh).

The purpose of the present study was to determine the capacities of differential diagnosis of the bulky masses of the brain by positron emission tomography (PET) with 11C-butyrate by the direct indication--the tumor RFP level measured by means of the semiquantitative indicator--the differential accumulation coefficient (DAC). For comparison, 18FDG PET widely used to detect malignant tumors was preliminarily made. Brain PET was performed in 86 patients (45 males and 41 females whose age ranged from 18 to 69 years to identify bulky masses or to rule out continuous tumor growth. In all cases, the data were histologically and morphologically verified. Of the 86 patients, 21 were found to have malignant tumors, 41 had benign tumors, arteriovenous malformations, cerebral circulatory disorders, and cysts were detected in 3, 7, and 14 cases, respectively. The level of 18FDG accumulation was ascertained to be directly related to the grade of malignancy. In 20 of the 21 patients with malignant tumors, DAC for 18FDG was greater than 1. However, it was impossible to differentiate benign tumors and non-tumoral masses by using only 18FDG. Comparing the data obtained by means of 18FDG and 11C-butyrate revealed their comparability in detecting neoplasms. Patients with vascular tumors (benign meningioma and adenoma of the pituitary were an exception. Their DAC for 11C-butyrate was greater than 1. In follow-up CT scanning, just a single injection of 11C-butyrate may allow one to estimate the vascular and tissue components of masses, which facilitates the identification of vascular non-tumoral processes and tumors. The additional criterion that allows a neoplasm to be differentiated from nontumoral processes permits a rapid tumor release of a radioactive label.

[Application of new radiopharmaceutical "Sodium 11c-butyrate" and positron emission tomography in patients with coronary artery disease]. / Primenenie pozitronnoi émissionnoi tomografii s novym diofarmatsevticheskim preparatom 11C-butirat natriia dlia otsenki sostoianiia miokarda u patsientom s IBS.

UNLABELLED: The aim of this study was to examine the diagnostic value of Sodium 11C-Butyrate (11C-Butyrate) in assessing perfusion and oxydative metabolism in patients with coronary artery disease (CAD). 41 patients (39 male and 9 female, mean age 48 +/- 11) with CAD were investigated. PET-scans were performed twice: at 2-7 min and 25-31 min after i.v. injection of 250-400 MBq 11C-Butyrate. Regional perfusion was evaluated by the uptake of radioactivity in the myocardium at the first scan, intensity of beta-oxidation--by decreasing the uptake level at the second scan. RESULTS: Myocardium of the left ventricle was good visualized at the first scan. It was demonstrated fast elimination of radioactivity in segments with normal oxidative metabolism of 11C-Butyrate at the second scan. Fixed defects were seen in the scar segments both at first and second scans. Decreased uptake of 11C-Butyrate at the first scan and diminished rate of agent elimination caused by depressed beta-oxidation at the second scan were revealed in ischemic segments. CONCLUSION: Application of PET with 11C-Butyrate in patients with CAD allows estimate hypoperfused segments, evaluate heart oxidative metabolism and provides differential diagnosis of scar and ischemia.