The problem of rehabilitation of patients with multimorbid pathology currently holds its medical and social relevance, due to the complexity of choosing an adequate treatment and predicting possible complications at the hospital stage. This is determined by the predominant monomorbid nature of clinical recommendations, as well as the difficulty of analyzing all risk factors and predicting the outcome of a comorbid disease. The article describes the case of a 60-year-old man with comorbid cerebrocardiovascular pathology, transferred from the palliative department to a rehabilitation center. The score on the Rehabilitation Routing Scale (RRS) was 5 points with a prospective regression to 6 points. As part of a multidisciplinary rehabilitation team (MDRT), the patient underwent a complex of rehabilitation measures including physical and cognitive rehabilitation, apparatus physiotherapy, speech and occupational therapy, complementary to medication. On the 21st day of rehabilitation measures, the patient's condition improved by 1.2 times in terms of RRS, 4 times according to the NIHSS stroke scale, 3 times in muscle strength according to the MRCWS, 2 times in mAS spasticity, 4 times in dysphagia, and 1,5 times in balance, Barthel Index of ADL – by 1.5 times, Rivermead Mobility Index – 3 times.
{"title":"КЛИНИЧЕСКИЙ СЛУЧАЙ ЭФФЕКТИВНОЙ РЕАБИЛИТАЦИИ ПАЦИЕНТА С КОМОРБИДНОЙ СОСУДИСТОЙ ПАТОЛОГИЕЙ","authors":"N.I. Kholodkov, V.V. Ostroukhov, V.A. Drobyshev, О.А. Denisova, А.R. Utyakov, K.V. Kozyr, I.A. Gobruseva, D.A. Dyachkov, А.V. Shmal, L.A. Semenova, Yu.P. Aronova, K.E. Zueva, М.А. Berdnikov, А.А. Isaeva, N.V. Levchenko","doi":"10.31549/2541-8289-2023-7-3-65-71","DOIUrl":"https://doi.org/10.31549/2541-8289-2023-7-3-65-71","url":null,"abstract":"The problem of rehabilitation of patients with multimorbid pathology currently holds its medical and social relevance, due to the complexity of choosing an adequate treatment and predicting possible complications at the hospital stage. This is determined by the predominant monomorbid nature of clinical recommendations, as well as the difficulty of analyzing all risk factors and predicting the outcome of a comorbid disease. The article describes the case of a 60-year-old man with comorbid cerebrocardiovascular pathology, transferred from the palliative department to a rehabilitation center. The score on the Rehabilitation Routing Scale (RRS) was 5 points with a prospective regression to 6 points. As part of a multidisciplinary rehabilitation team (MDRT), the patient underwent a complex of rehabilitation measures including physical and cognitive rehabilitation, apparatus physiotherapy, speech and occupational therapy, complementary to medication. On the 21st day of rehabilitation measures, the patient's condition improved by 1.2 times in terms of RRS, 4 times according to the NIHSS stroke scale, 3 times in muscle strength according to the MRCWS, 2 times in mAS spasticity, 4 times in dysphagia, and 1,5 times in balance, Barthel Index of ADL – by 1.5 times, Rivermead Mobility Index – 3 times.","PeriodicalId":488734,"journal":{"name":"Sibirskij medicinskij vestnik","volume":"1 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135667379","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Motor neuron disease, in the vast majority of cases occurring in the form of amyotrophic lateral sclerosis is a rare neurodegenerative disease with a prevalence of 2–3 cases per 100 thousand people in the population. The paper presents the results of a clinical observation of a patient with probable amyotrophic lateral sclerosis which occurs comorbidly with autoimmune myositis since the diagnosis was made in January 2023, as well as the effect of a comprehensive 21-day rehabilitation on clinical characteristics in the rehabilitation department of PARUS medical resort & SPA, LLC, near Novosibirsk. The individual rehabilitation course included physical rehabilitation, restorative training, apparatus physiotherapy and occupational therapy in addition to medication. By the end of the rehabilitation course, the indicators of the measured clinical scales improved: ALSFRS-R – by 3 times, MRC muscle strength – 2 times, Barthel – 2 times, Beck – 1.5 times, according to the walking scale of the Institute of Neurology – 1.3 times, paresis scale of the Institute of Neurology – 2 times, dysphagia – 4.5 times. The study allows expanding and supplementing the available data regarding the difficulty of verifying the diagnosis which directly affects the complexity of assessing the rehabilitation prognosis, setting rehabilitation goals and objectives in a patient with difficult to differentiate comorbid pathology.
{"title":"A CLINICAL CASE OF COMPREHENSIVE REHABILITATION IN PATIENT WITH A COMORBID NEURODEGERATIVE AND AUTOIMMUNE DISEASES","authors":"N.I. Kholodkov, D.A. Dyachkov, О.А. Denisova, V.А. Drobyshev, I.A. Gobruseva, K.Е. Zueva, А.V. Shmal, А.R. Utyakov, А.I. Root, G.V. Meliksetov, K.V. Kozyr, I.E. Pochueva, N.V. Levchenko","doi":"10.31549/2541-8289-2023-7-3-72-78","DOIUrl":"https://doi.org/10.31549/2541-8289-2023-7-3-72-78","url":null,"abstract":"Motor neuron disease, in the vast majority of cases occurring in the form of amyotrophic lateral sclerosis is a rare neurodegenerative disease with a prevalence of 2–3 cases per 100 thousand people in the population. The paper presents the results of a clinical observation of a patient with probable amyotrophic lateral sclerosis which occurs comorbidly with autoimmune myositis since the diagnosis was made in January 2023, as well as the effect of a comprehensive 21-day rehabilitation on clinical characteristics in the rehabilitation department of PARUS medical resort & SPA, LLC, near Novosibirsk. The individual rehabilitation course included physical rehabilitation, restorative training, apparatus physiotherapy and occupational therapy in addition to medication. By the end of the rehabilitation course, the indicators of the measured clinical scales improved: ALSFRS-R – by 3 times, MRC muscle strength – 2 times, Barthel – 2 times, Beck – 1.5 times, according to the walking scale of the Institute of Neurology – 1.3 times, paresis scale of the Institute of Neurology – 2 times, dysphagia – 4.5 times. The study allows expanding and supplementing the available data regarding the difficulty of verifying the diagnosis which directly affects the complexity of assessing the rehabilitation prognosis, setting rehabilitation goals and objectives in a patient with difficult to differentiate comorbid pathology.","PeriodicalId":488734,"journal":{"name":"Sibirskij medicinskij vestnik","volume":"47 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135667380","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare form of idiopathic interstitial pneumonia the etio¬logy of which is often unknown, and the course of the disease is usually rapidly progressive. The disease occurs with equal frequency in both men and women. Its onset usually is observed in the second half of life. Complaints are nonspecific: patients suffer from dry cough, shortness of breath, low body mass. A distinctive feature of IPPFE is the involvement of the apices of the lungs and development of platythorax, which can be easily detected in these patients during computed tomography. There is no specific treatment, but there is an evidence of positive experience with the use of antifibrotic drugs.
{"title":"EXPERIENCE IN MANAGING A PATIENT WITH IDIOPATHIC PLEUROPARENCHYMAL FIBROELASTOSIS: FEATURES OF DIAGNOSIS AND TREATMENT","authors":"L.M. Kudelya, E.S. Khusainova, Ya.L. Manakova, T.V. Manzhileeva, L.N. Mozhina","doi":"10.31549/2541-8289-2023-7-3-51-57","DOIUrl":"https://doi.org/10.31549/2541-8289-2023-7-3-51-57","url":null,"abstract":"Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare form of idiopathic interstitial pneumonia the etio¬logy of which is often unknown, and the course of the disease is usually rapidly progressive. The disease occurs with equal frequency in both men and women. Its onset usually is observed in the second half of life. Complaints are nonspecific: patients suffer from dry cough, shortness of breath, low body mass. A distinctive feature of IPPFE is the involvement of the apices of the lungs and development of platythorax, which can be easily detected in these patients during computed tomography. There is no specific treatment, but there is an evidence of positive experience with the use of antifibrotic drugs.","PeriodicalId":488734,"journal":{"name":"Sibirskij medicinskij vestnik","volume":"9 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135667384","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.31549/2541-8289-2023-7-3-18-24
V.P. Drobysheva, A.Ar. Demin
Introduction. Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as allergic granulomatosis (Churg-Stross syndrome), in the modern sense is a necrotizing multisystem vasculitis of predominantly small vessels associated with hyperproduction of antineutrophil cytoplasmic antibodies (ANCA), peripheral blood hypereosinophilia. Although the most frequently involved are the respiratory organs (rhinosinusitis, bronchial asthma, pulmonary infiltrates), EGPA affects any organs and systems (heart, kidneys, gastrointestinal tract, nervous system, skin, joints, etc.) which makes its diagnosis difficult. Aim. To present an observation of a difficult-to-diagnose clinical case of eosinophilic granulomatosis with polyangiitis. Materials and methods. The analysis of clinical, laboratory, special testing of the patient was carried out. Results. Diagnosis of EGPA in this patient was difficult. Fever up to 40°C, papulo-necrotic skin eruptions on upper and lower extremities, trunk; weight loss; multiple organ involvement of heart, lungs, kidneys, liver; positive hemoculture with isolation of Staph. haemoliticus, and from skin lesions of Staph. epidermidis – all these served as a reason to suspect staphylococcal sepsis with possible infectious endocarditis and prescribe antimicrobial therapy which was ineffective. Progression of the disease with worsening clinical manifestations, detection of blood eosinophilia (18%), positive ANCA on immunological examination, eosinophilic and lymphohistiocytic infiltration around microvessels on histological examination of the musculocutaneous flap allowed the diagnosis of EGPA to be verified. Pulse chemotherapy with cyclophosphamide and medium doses of methylprednisolone led to regression of the disease manifestations in the patient. Conclusion. The course of EGPA can include life-threatening conditions (acute myocardial infarction and pulmonary hemorrhage). Timely diagnosis and standards of active chemotherapy expand possibilities of managing this severe group of patients.
{"title":"EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS","authors":"V.P. Drobysheva, A.Ar. Demin","doi":"10.31549/2541-8289-2023-7-3-18-24","DOIUrl":"https://doi.org/10.31549/2541-8289-2023-7-3-18-24","url":null,"abstract":"Introduction. Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as allergic granulomatosis (Churg-Stross syndrome), in the modern sense is a necrotizing multisystem vasculitis of predominantly small vessels associated with hyperproduction of antineutrophil cytoplasmic antibodies (ANCA), peripheral blood hypereosinophilia. Although the most frequently involved are the respiratory organs (rhinosinusitis, bronchial asthma, pulmonary infiltrates), EGPA affects any organs and systems (heart, kidneys, gastrointestinal tract, nervous system, skin, joints, etc.) which makes its diagnosis difficult. Aim. To present an observation of a difficult-to-diagnose clinical case of eosinophilic granulomatosis with polyangiitis. Materials and methods. The analysis of clinical, laboratory, special testing of the patient was carried out. Results. Diagnosis of EGPA in this patient was difficult. Fever up to 40°C, papulo-necrotic skin eruptions on upper and lower extremities, trunk; weight loss; multiple organ involvement of heart, lungs, kidneys, liver; positive hemoculture with isolation of Staph. haemoliticus, and from skin lesions of Staph. epidermidis – all these served as a reason to suspect staphylococcal sepsis with possible infectious endocarditis and prescribe antimicrobial therapy which was ineffective. Progression of the disease with worsening clinical manifestations, detection of blood eosinophilia (18%), positive ANCA on immunological examination, eosinophilic and lymphohistiocytic infiltration around microvessels on histological examination of the musculocutaneous flap allowed the diagnosis of EGPA to be verified. Pulse chemotherapy with cyclophosphamide and medium doses of methylprednisolone led to regression of the disease manifestations in the patient. Conclusion. The course of EGPA can include life-threatening conditions (acute myocardial infarction and pulmonary hemorrhage). Timely diagnosis and standards of active chemotherapy expand possibilities of managing this severe group of patients.","PeriodicalId":488734,"journal":{"name":"Sibirskij medicinskij vestnik","volume":"50 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135668169","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Introduction. Median arcuate ligament syndrome (MALS, Dunbar syndrome) is a rare vascular compression syndrome caused by extravascular compression of the celiac trunk (CT) and celiac plexus by the median arcuate ligament of the diaphragm and is one of the causes of chronic abdominal ischemia and neuropathic pain syndrome. Clinical observation. A woman, 36 years old, was hospitalized with complaints of abdominal pain of moderate intensity, diarrhea up to 10 times a day, weight loss of 11 kg over the past year. Inflammatory bowel disease, gluten enteropathy, lymphoma of the small intestine, malabsorption syndrome, pathology of the endocrine system was excluded. Abdominal high-resolution computed tomography (HRCT) angiography was performed. Signs of extravascular compression of CT up to 90% with a thickened median arcuate ligament with the formation of a U-shaped deformation were revealed. Laparoscopic decompression surgery was performed with simultaneous transluminal angioplasty, which led to a decrease in compression and disappearance of clinical symptoms. After 18 months, clinical improvement persisted, diarrhea disappeared. HRCT angiography and ultrasound duplex examination (USDE) showed sufficient blood flow in the CT system. During the follow-up period, the patient had moderate neuropathic pain and anxiety and depressive disorders requiring appropriate correction. Conclusion. The diagnostic and therapeutic approach to MALS should be patient-centered and requires the involvement of a team of specialists. Laparoscopic decompression is an effective treatment and can provide immediate symptoms relief. The results of the intervention can be assessed by HRCT angiography and USDE (methods supplementing each other). Correction of neuropathic pain syndrome and anxiety-depressive disorder is of great importance for improving the patient's quality of life.
{"title":"MEDIAN ARCUATE LIGAMENT SYNDROME (DUNBAR SYNDROME) AS A MULTIDISCIPLINARY PROBLEM. CLINICAL OBSERVATION AND LITERATURE REFERENCE","authors":"O.I. Dorovskaya, Ya.L. Manakova, N.L. Tov, D.L. Nepomnyashchikh, K.A. Brezhneva, L.A. Kharlamova","doi":"10.31549/2541-8289-2023-7-3-11-17","DOIUrl":"https://doi.org/10.31549/2541-8289-2023-7-3-11-17","url":null,"abstract":"Introduction. Median arcuate ligament syndrome (MALS, Dunbar syndrome) is a rare vascular compression syndrome caused by extravascular compression of the celiac trunk (CT) and celiac plexus by the median arcuate ligament of the diaphragm and is one of the causes of chronic abdominal ischemia and neuropathic pain syndrome. Clinical observation. A woman, 36 years old, was hospitalized with complaints of abdominal pain of moderate intensity, diarrhea up to 10 times a day, weight loss of 11 kg over the past year. Inflammatory bowel disease, gluten enteropathy, lymphoma of the small intestine, malabsorption syndrome, pathology of the endocrine system was excluded. Abdominal high-resolution computed tomography (HRCT) angiography was performed. Signs of extravascular compression of CT up to 90% with a thickened median arcuate ligament with the formation of a U-shaped deformation were revealed. Laparoscopic decompression surgery was performed with simultaneous transluminal angioplasty, which led to a decrease in compression and disappearance of clinical symptoms. After 18 months, clinical improvement persisted, diarrhea disappeared. HRCT angiography and ultrasound duplex examination (USDE) showed sufficient blood flow in the CT system. During the follow-up period, the patient had moderate neuropathic pain and anxiety and depressive disorders requiring appropriate correction. Conclusion. The diagnostic and therapeutic approach to MALS should be patient-centered and requires the involvement of a team of specialists. Laparoscopic decompression is an effective treatment and can provide immediate symptoms relief. The results of the intervention can be assessed by HRCT angiography and USDE (methods supplementing each other). Correction of neuropathic pain syndrome and anxiety-depressive disorder is of great importance for improving the patient's quality of life.","PeriodicalId":488734,"journal":{"name":"Sibirskij medicinskij vestnik","volume":"61 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135667381","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Introduction. Pulmonary-renal syndrome (PRS) manifested by a combination of idiopathic pulmonary alveolar hemorrhage and rapidly progressing glomerulonephritis, is most commonly associated with either anti-neutrophil cytoplasmic antibodies (ANCA) or anti-glomerular basement membrane (anti-GBM) antibodies. Despite the rather rarity of this disease, there is a sufficient number of observations with the simultaneous presence of both types of antibodies, the so-called double antibody-positive phenotype. During the pandemic of SARS-CoV-2 infection an increase in cases of PRS was noted, however, the phenomenon of double antibody positi¬vity – ANCA plus anti-GBM – is still a clinical casuistry. Observation. A 64-year-old woman was hospitalized 2 months after SARS-CoV-2 infection with PRS of a severe course with rapidly progressing renal failure, as well as lung affection with hemoptysis and typical changes on multidetector computed tomography. The patient was found to be double positive for ANCA and anti-GBM. Therapy including plasma exchanges, high doses of steroids and cyclophosphamide was carried out which gave a short-term improvement, but did not prevent dependence on hemodialysis. 7 months after the onset of PRS the patient died from a secondary infection. According to the pathoanatomical study, the diagnosis of anti-GBM disease in combination with ANCA-associated vasculitis was confirmed. Conclusion. As far as we know, this is the second reported case of 'double antibody' PRS after SARS-CoV-2 infection. We believe that in all such clinical situations it is necessary to test for both types of antibodies as this will determine the clinical tactics.
{"title":"PULMONARY RENAL SYNDROME CAUSED BY ANTI-GLOMERULAR BASEMENT MEMBRANE DISEASE IN COMBINATION WITH ANCA- ASSOCIATED VASCULITIS AFTER SARS-COV-2 INFECTION","authors":"N.L. Tov, E.V. Galkina, V.O. Popova, A.V. Goncharova, Ya.L. Manakova, S.V. Abdrakhmanov, Т.А. Telegina, Е.А. Movchan","doi":"10.31549/2541-8289-2023-7-3-25-31","DOIUrl":"https://doi.org/10.31549/2541-8289-2023-7-3-25-31","url":null,"abstract":"Introduction. Pulmonary-renal syndrome (PRS) manifested by a combination of idiopathic pulmonary alveolar hemorrhage and rapidly progressing glomerulonephritis, is most commonly associated with either anti-neutrophil cytoplasmic antibodies (ANCA) or anti-glomerular basement membrane (anti-GBM) antibodies. Despite the rather rarity of this disease, there is a sufficient number of observations with the simultaneous presence of both types of antibodies, the so-called double antibody-positive phenotype. During the pandemic of SARS-CoV-2 infection an increase in cases of PRS was noted, however, the phenomenon of double antibody positi¬vity – ANCA plus anti-GBM – is still a clinical casuistry. Observation. A 64-year-old woman was hospitalized 2 months after SARS-CoV-2 infection with PRS of a severe course with rapidly progressing renal failure, as well as lung affection with hemoptysis and typical changes on multidetector computed tomography. The patient was found to be double positive for ANCA and anti-GBM. Therapy including plasma exchanges, high doses of steroids and cyclophosphamide was carried out which gave a short-term improvement, but did not prevent dependence on hemodialysis. 7 months after the onset of PRS the patient died from a secondary infection. According to the pathoanatomical study, the diagnosis of anti-GBM disease in combination with ANCA-associated vasculitis was confirmed. Conclusion. As far as we know, this is the second reported case of 'double antibody' PRS after SARS-CoV-2 infection. We believe that in all such clinical situations it is necessary to test for both types of antibodies as this will determine the clinical tactics.","PeriodicalId":488734,"journal":{"name":"Sibirskij medicinskij vestnik","volume":"33 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135667382","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Introduction. The syndrome of arterial hypertension (AH) can be in patients with functioning and non-functioning masses of the adrenal glands which requires a thorough examination to exclude secon¬dary AH of origin. Aim. Identification of clinical, laboratory and radiological features of the AH syndrome in pheochromocytoma (PCC) and adrenocortical adrenal adenoma (incidentaloma). Materials and methods. A retrospective analysis of the clinic, biochemical, hormonal, radiological methods of examination was carried out in 15 patients referred for surgical treatment with a diagnosis of pheochromocytoma. During the postoperative histological examination of the removed masses of the adrenal glands, the diagnosis of PCC was confirmed in 8 out of 15 patients, in 7 patients instead of PCC, adrenocortical adenoma of was diagnosed. Results. Comparative analysis of the results of clinical, laboratory and radiological examination of patients with PCC and non-functioning adrenocortical adenoma with arterial hypertension syndrome showed that arterial hypertension syndrome in PCC has the following features: a crisis course of hypertension with its characteristic symptoms (headache, tremor, profuse sweating, palpitations), passing without treatment with the development of severe weakness after the crisis. Patients with PCC, compared with patients with adrenocortical adenoma, had a lower body mass, high levels of metanephrine, and on computed tomography (CT), the tumors were larger with high contrast uptake in the unenhanced and delayed phases. The reasons for the overdiagnosis of PCC in patients with adrenocortical adenoma in the presence of hypertension were: underestimation of clinical manifestations and an increase in the level of normetanephrine in daily urine, as well as a slight increase in the unenhanced density of contrast uptake on CT in 2 of 7 patients.
{"title":"ARTERIAL HYPERTENSION SYNDROME IN PHEOCROMOCYTOMA AND NON-FUNCTIONING ADRENOCORTICAL ADENOMA","authors":"I.A. Bondar, I.P. Krasnopevtseva, T.N. Serebrennikova","doi":"10.31549/2541-8289-2023-7-3-32-41","DOIUrl":"https://doi.org/10.31549/2541-8289-2023-7-3-32-41","url":null,"abstract":"Introduction. The syndrome of arterial hypertension (AH) can be in patients with functioning and non-functioning masses of the adrenal glands which requires a thorough examination to exclude secon¬dary AH of origin. Aim. Identification of clinical, laboratory and radiological features of the AH syndrome in pheochromocytoma (PCC) and adrenocortical adrenal adenoma (incidentaloma). Materials and methods. A retrospective analysis of the clinic, biochemical, hormonal, radiological methods of examination was carried out in 15 patients referred for surgical treatment with a diagnosis of pheochromocytoma. During the postoperative histological examination of the removed masses of the adrenal glands, the diagnosis of PCC was confirmed in 8 out of 15 patients, in 7 patients instead of PCC, adrenocortical adenoma of was diagnosed. Results. Comparative analysis of the results of clinical, laboratory and radiological examination of patients with PCC and non-functioning adrenocortical adenoma with arterial hypertension syndrome showed that arterial hypertension syndrome in PCC has the following features: a crisis course of hypertension with its characteristic symptoms (headache, tremor, profuse sweating, palpitations), passing without treatment with the development of severe weakness after the crisis. Patients with PCC, compared with patients with adrenocortical adenoma, had a lower body mass, high levels of metanephrine, and on computed tomography (CT), the tumors were larger with high contrast uptake in the unenhanced and delayed phases. The reasons for the overdiagnosis of PCC in patients with adrenocortical adenoma in the presence of hypertension were: underestimation of clinical manifestations and an increase in the level of normetanephrine in daily urine, as well as a slight increase in the unenhanced density of contrast uptake on CT in 2 of 7 patients.","PeriodicalId":488734,"journal":{"name":"Sibirskij medicinskij vestnik","volume":"45 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135668170","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.31549/2541-8289-2023-7-3-58-64
L.A. Shpagina, I.S. Shpagin, O.S. Kotova, S.A. Karmanovskaya, L.A. Panacheva, I.P. Mikhno, E.G. Konduyrina, V.V. Zelenskaya, E.M. Loktin
Pulmonary alveolar microlithiasis is a rare genetic disease characterized by the formation of intraalveolar calcium phosphate microliths. Clinical and radiological manifestations are similar to pneumoconiosis, which makes it difficult to diagnose a comorbid condition. This article presents a case from clinical practice: a 30-year-old male patient, non-smoker was referred to a occupational pathology center with a preliminary diagnosis of pneumoconiosis. He worked for 8 years under conditions of exposure to highly fibrogenic dust with the maximum allowable concentrations in the air of the working area exceeding by 2.3–2.1 times. During this period, preliminary and 3 periodic medical examinations were carried out which did not reveal any contraindications for continuing work. Examination at the center of occupational pathologists revealed signs of respiratory failure of the 2nd degree, severe pulmonary hypertension and radiological diffuse dissemination syndrome with small shading of two types - a large number of high (calcium) density foci with clear edges and a moderate number of medium density foci. Histological examination revealed signs of pulmonary fibrosis and interstitial inflammation of the type of nonspecific interstitial pneumonia, microliths located intraalveolarly, as well as a small amount of granulomas with hyalinosis in the center, a concentric arrangement of collagen fibers, fibroblasts, alveolar macrophages. A comorbid disease was diagnosed – pulmonary alveolar microlithiasis in combination with pneumoconiosis confirmed by the results of genetic testing – the c.1328delT mutation of the SLC34A2 gene was detected. The further course of the disease was characterized by progression of dyspnea and desaturation, an increase in the number of high-density foci in the lungs. 2 years after diagnosis – death due to respiratory failure. The presented case demonstrates the possibility of a combination of pulmonary alveolar microlithiasis with pneumoconiosis and an unfavorable prognosis of a comorbid disease.
{"title":"THE COMBINATION OF PULMONARY ALVEOLAR MICROLITHIASIS AND PNEUMOCONIOSIS – A CLINICAL CASE","authors":"L.A. Shpagina, I.S. Shpagin, O.S. Kotova, S.A. Karmanovskaya, L.A. Panacheva, I.P. Mikhno, E.G. Konduyrina, V.V. Zelenskaya, E.M. Loktin","doi":"10.31549/2541-8289-2023-7-3-58-64","DOIUrl":"https://doi.org/10.31549/2541-8289-2023-7-3-58-64","url":null,"abstract":"Pulmonary alveolar microlithiasis is a rare genetic disease characterized by the formation of intraalveolar calcium phosphate microliths. Clinical and radiological manifestations are similar to pneumoconiosis, which makes it difficult to diagnose a comorbid condition. This article presents a case from clinical practice: a 30-year-old male patient, non-smoker was referred to a occupational pathology center with a preliminary diagnosis of pneumoconiosis. He worked for 8 years under conditions of exposure to highly fibrogenic dust with the maximum allowable concentrations in the air of the working area exceeding by 2.3–2.1 times. During this period, preliminary and 3 periodic medical examinations were carried out which did not reveal any contraindications for continuing work. Examination at the center of occupational pathologists revealed signs of respiratory failure of the 2nd degree, severe pulmonary hypertension and radiological diffuse dissemination syndrome with small shading of two types - a large number of high (calcium) density foci with clear edges and a moderate number of medium density foci. Histological examination revealed signs of pulmonary fibrosis and interstitial inflammation of the type of nonspecific interstitial pneumonia, microliths located intraalveolarly, as well as a small amount of granulomas with hyalinosis in the center, a concentric arrangement of collagen fibers, fibroblasts, alveolar macrophages. A comorbid disease was diagnosed – pulmonary alveolar microlithiasis in combination with pneumoconiosis confirmed by the results of genetic testing – the c.1328delT mutation of the SLC34A2 gene was detected. The further course of the disease was characterized by progression of dyspnea and desaturation, an increase in the number of high-density foci in the lungs. 2 years after diagnosis – death due to respiratory failure. The presented case demonstrates the possibility of a combination of pulmonary alveolar microlithiasis with pneumoconiosis and an unfavorable prognosis of a comorbid disease.","PeriodicalId":488734,"journal":{"name":"Sibirskij medicinskij vestnik","volume":"158 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135667385","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.31549/2541-8289-2023-7-3-4-10
A.Ar. Demin
Introduction. Lupus nephritis (LN) is common in systemic lupus erythematosus (SLE), a severe autoimmune disease with a poor prognosis. Restoring the immune system and self-tolerance using high-dose immunosuppressive therapy with autologous hematopoietic stem cell transplantation (AHSCT) is a new approach in the treatment of refractory SLE. The effectiveness of SLE with LN treatment in the long-term follow-up should be the subject for study. Methods. Patient I., 25 years old, with refractory SLE and LN is described. Standard therapy was ineffective, the SLEDAI score remained 22 points, so the patient underwent high-dose immunosuppressive therapy (HIT) with ASCT, and she was included in the registry of the European Group for Blood and Marrow Transplantation of the European League Against Rheumatism (EBMT/EULAR). Results. As a result of ASCT, complete clinical and serological remission was achieved (score on the SLEDAI scale – 0 points) for 3 years. Then there was a relapse with nephrotic syndrome and anti-DNA positivity which led to the end-stage renal failure. Despite therapy, the disease activity persisted, and the patient was on regular hemodialysis for 1 year, then underwent allogeneic kidney transplantation. Currently, 18 years have passed since HIT and AHSCT and 11 years since renal transplantation. The patient receives standard post-transplant immunosuppression, and her condition remains stable, the renal allograft is functioning, the SLEDAI score is 2 points (low titer anti-DNA positivity). Discussion. Modern standards of chemotherapy (hydroxychloroquine, mycophenolate mofetil, cyclophosphamide, corticosteroids), bioactive immunotherapy (rituximab, belimumab, obinituzumab, anifrolumab), “multi-target therapy” (standard + tacrolimus, voclosporin, mycophenolate mofetil), and new approaches (immuno- and nephrotransplantation) for lupus nephritis. Conclusion. The successful long-term results of the first case of immunoablation with high-dose immunosuppressive therapy and double autologous stem cell and allogeneic kidney transplantation for SLE with refractory lupus nephritis and an analysis of the effectiveness of modern treatment approaches are presented.
{"title":"LUPUS NEPHRITIS: CLINICAL CASE WITH DOUBLE TRANSPLANTATION. Ar.A. DEMIN’S CONTRIBUTION TO STUDYING THE DISEASE","authors":"A.Ar. Demin","doi":"10.31549/2541-8289-2023-7-3-4-10","DOIUrl":"https://doi.org/10.31549/2541-8289-2023-7-3-4-10","url":null,"abstract":"Introduction. Lupus nephritis (LN) is common in systemic lupus erythematosus (SLE), a severe autoimmune disease with a poor prognosis. Restoring the immune system and self-tolerance using high-dose immunosuppressive therapy with autologous hematopoietic stem cell transplantation (AHSCT) is a new approach in the treatment of refractory SLE. The effectiveness of SLE with LN treatment in the long-term follow-up should be the subject for study. Methods. Patient I., 25 years old, with refractory SLE and LN is described. Standard therapy was ineffective, the SLEDAI score remained 22 points, so the patient underwent high-dose immunosuppressive therapy (HIT) with ASCT, and she was included in the registry of the European Group for Blood and Marrow Transplantation of the European League Against Rheumatism (EBMT/EULAR). Results. As a result of ASCT, complete clinical and serological remission was achieved (score on the SLEDAI scale – 0 points) for 3 years. Then there was a relapse with nephrotic syndrome and anti-DNA positivity which led to the end-stage renal failure. Despite therapy, the disease activity persisted, and the patient was on regular hemodialysis for 1 year, then underwent allogeneic kidney transplantation. Currently, 18 years have passed since HIT and AHSCT and 11 years since renal transplantation. The patient receives standard post-transplant immunosuppression, and her condition remains stable, the renal allograft is functioning, the SLEDAI score is 2 points (low titer anti-DNA positivity). Discussion. Modern standards of chemotherapy (hydroxychloroquine, mycophenolate mofetil, cyclophosphamide, corticosteroids), bioactive immunotherapy (rituximab, belimumab, obinituzumab, anifrolumab), “multi-target therapy” (standard + tacrolimus, voclosporin, mycophenolate mofetil), and new approaches (immuno- and nephrotransplantation) for lupus nephritis. Conclusion. The successful long-term results of the first case of immunoablation with high-dose immunosuppressive therapy and double autologous stem cell and allogeneic kidney transplantation for SLE with refractory lupus nephritis and an analysis of the effectiveness of modern treatment approaches are presented.","PeriodicalId":488734,"journal":{"name":"Sibirskij medicinskij vestnik","volume":"45 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135667374","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01DOI: 10.31549/2541-8289-2023-7-3-42-50
T.I. Pospelova, T.N. Babaeva
Gaucher disease is the most common form of hereditary enzymopathies included in the group of lysosomal storage diseases. The disease is based on a hereditary deficiency in the activity of the enzyme glucocerebrosidase (also called glucosylceramidase or acid β-glucosidase) which is involved in the degradation of cellular metabolic products. Gaucher disease is a systemic disease characterized by the same type of clinical manifestations (hepatosplenomegaly, cytopenias, lesions of the skeletal system) but an extremely heterogeneous clinical course. In the article, the authors describe their own clinical case of Gaucher disease in a teenage girl. A long past history of he¬terogeneous clinical symptoms, the complexity of differential diagnosis and interpretation of existing deviations within the course of the single systemic process were noted. The high effectiveness of modern enzyme replacement therapy has been demonstrated.
{"title":"RARE BLOOD DISEASES: GAUCHER DISEASE. CASE DESCRIPTION","authors":"T.I. Pospelova, T.N. Babaeva","doi":"10.31549/2541-8289-2023-7-3-42-50","DOIUrl":"https://doi.org/10.31549/2541-8289-2023-7-3-42-50","url":null,"abstract":"Gaucher disease is the most common form of hereditary enzymopathies included in the group of lysosomal storage diseases. The disease is based on a hereditary deficiency in the activity of the enzyme glucocerebrosidase (also called glucosylceramidase or acid β-glucosidase) which is involved in the degradation of cellular metabolic products. Gaucher disease is a systemic disease characterized by the same type of clinical manifestations (hepatosplenomegaly, cytopenias, lesions of the skeletal system) but an extremely heterogeneous clinical course. In the article, the authors describe their own clinical case of Gaucher disease in a teenage girl. A long past history of he¬terogeneous clinical symptoms, the complexity of differential diagnosis and interpretation of existing deviations within the course of the single systemic process were noted. The high effectiveness of modern enzyme replacement therapy has been demonstrated.","PeriodicalId":488734,"journal":{"name":"Sibirskij medicinskij vestnik","volume":"87 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135667375","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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