Pub Date : 2023-09-13DOI: 10.17816/clinpract472056
Olga Альпидовская
The COVID-19 pandemic has also affected rheumatic diseases. A clinical case of the development of the Waterhouse-Friderichsen syndrome after SARS-CoV-2 infection in a patient with secondary AA - adrenal amyloidosis is presented. Description of the case. Patient G.G., 57 years old, was hospitalized for several hours on May 20, 2023 at the Cheboksary Regional Hospital with a diagnosis of severe viral interstitial pneumonia. Secondary AA adrenal amyloidosis. Acute adrenal insufficiency. She was admitted with complaints of an increase in body temperature up to 38.20C. Computed tomography of the chest organs showed signs of bilateral viral interstitial pneumonia (the percentage of lung damage was 74%). The diagnosis of COVID-19 was based on a positive PCR test in nasopharyngeal swabs. Blood pressure - 80 / 40 mm Hg. Art. D - dimer - 786 ng / ml (not higher - 243 ng / ml). Despite treatment, the patient died. At autopsy in the lungs, dystelectasis, hyaline membranes, intraalveolar edema. In the adrenal glands - the deposition of amyloid masses, foci of necrosis of the cortical layer and hemorrhage. Signs of DIC - syndrome. Conclusion. The peculiarity of the case is the development of the Waterhouse-Frideriksen syndrome without signs of sepsis.
COVID-19大流行还影响了风湿病。本文报道1例继发性AA -肾上腺淀粉样变患者在SARS-CoV-2感染后出现Waterhouse-Friderichsen综合征的临床病例。案件描述。患者g.g., 57岁,于2023年5月20日在Cheboksary地区医院住院数小时,诊断为严重病毒性间质性肺炎。继发性AA肾上腺淀粉样变。急性肾上腺功能不全。她入院时抱怨体温上升至38.20摄氏度。胸部器官的计算机断层扫描显示双侧病毒性间质性肺炎的征象(肺损伤百分比为74%)。根据鼻咽拭子PCR检测阳性诊断COVID-19。血压:80 / 40毫米汞柱。D -二聚体- 786 ng / ml(不高于- 243 ng / ml)。尽管接受了治疗,病人还是死了。尸检发现肺张不全,透明膜,肺泡内水肿。在肾上腺-淀粉样蛋白团块沉积,皮质层坏死灶和出血。DIC综合征的症状。结论。该病例的特点是发展为沃特豪斯-弗里德里克森综合征,但没有败血症的迹象。
{"title":"Development of the Waterhouse-Friderichsen syndrome after infection with SARS-CoV-2 in a patient with rheumatoid arthritis and secondary AA-adrenal amyloidosis","authors":"Olga Альпидовская","doi":"10.17816/clinpract472056","DOIUrl":"https://doi.org/10.17816/clinpract472056","url":null,"abstract":"The COVID-19 pandemic has also affected rheumatic diseases. A clinical case of the development of the Waterhouse-Friderichsen syndrome after SARS-CoV-2 infection in a patient with secondary AA - adrenal amyloidosis is presented. Description of the case. Patient G.G., 57 years old, was hospitalized for several hours on May 20, 2023 at the Cheboksary Regional Hospital with a diagnosis of severe viral interstitial pneumonia. Secondary AA adrenal amyloidosis. Acute adrenal insufficiency. She was admitted with complaints of an increase in body temperature up to 38.20C. Computed tomography of the chest organs showed signs of bilateral viral interstitial pneumonia (the percentage of lung damage was 74%). The diagnosis of COVID-19 was based on a positive PCR test in nasopharyngeal swabs. Blood pressure - 80 / 40 mm Hg. Art. D - dimer - 786 ng / ml (not higher - 243 ng / ml). Despite treatment, the patient died. At autopsy in the lungs, dystelectasis, hyaline membranes, intraalveolar edema. In the adrenal glands - the deposition of amyloid masses, foci of necrosis of the cortical layer and hemorrhage. Signs of DIC - syndrome. Conclusion. The peculiarity of the case is the development of the Waterhouse-Frideriksen syndrome without signs of sepsis.","PeriodicalId":34156,"journal":{"name":"Klinicheskaia praktika","volume":"21 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135689850","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 : 2023-09-13DOI: 10.17816/clinpract119952
Elena Khozhenko
Lambert-Eaton myasthenic syndrome, G 73.1 according to ICD-10, is a rare autoimmune disease associated with presynaptic impairment of neuromuscular transmission due to the production of antibodies to voltage-gated calcium channels. The disease is characterized by variable muscle weakness of the proximal limbs, mainly the gluteal and femoral muscles, ptosis of the eyelids, diplopia, dysarthria and peripheral paresthesia, and decreased tendon reflexes. The peculiarity of the Lambert-Eaton myasthenic syndrome is manifested in a combination of increased muscle weakness and fatigue with the phenomenon of "working in" - an increase in muscle strength against the background of muscle load after a short-term maximum contraction. At the same time, the patient shows normal response of tendon reflexes. Myasthenic Lambert-Eaton syndrome is often associated with small cell lung cancer and very rarely develops with tumors of other localization.
We observed an 80-year-old patient with complaints of weakness and pain in the hips, shoulders, pelvic region, half-ptosis of the eyelids. For half-ptosis of the eyelids, a differential diagnosis was made with myasthenia gravis. Stimulation electromyography revealed the M-response increment, i.e. the phenomenon of working in, which is typical for this syndrome. The examination revealed papillary thyroid cancer, which later was undergoing surgical treatment.
This case illustrates Lambert-Eaton's paraneoplastic myasthenic syndrome, a rare tumor form associated with thyroid cancer.
Key words: LambertEaton myasthenic syndrome, paraneoplastic syndrome, thyroid cancer.
{"title":"Myasthenic syndrome of Lambert-Eaton on the background of thyroid cancer (clinical case)","authors":"Elena Khozhenko","doi":"10.17816/clinpract119952","DOIUrl":"https://doi.org/10.17816/clinpract119952","url":null,"abstract":"Lambert-Eaton myasthenic syndrome, G 73.1 according to ICD-10, is a rare autoimmune disease associated with presynaptic impairment of neuromuscular transmission due to the production of antibodies to voltage-gated calcium channels. The disease is characterized by variable muscle weakness of the proximal limbs, mainly the gluteal and femoral muscles, ptosis of the eyelids, diplopia, dysarthria and peripheral paresthesia, and decreased tendon reflexes. The peculiarity of the Lambert-Eaton myasthenic syndrome is manifested in a combination of increased muscle weakness and fatigue with the phenomenon of \"working in\" - an increase in muscle strength against the background of muscle load after a short-term maximum contraction. At the same time, the patient shows normal response of tendon reflexes. Myasthenic Lambert-Eaton syndrome is often associated with small cell lung cancer and very rarely develops with tumors of other localization.
 We observed an 80-year-old patient with complaints of weakness and pain in the hips, shoulders, pelvic region, half-ptosis of the eyelids. For half-ptosis of the eyelids, a differential diagnosis was made with myasthenia gravis. Stimulation electromyography revealed the M-response increment, i.e. the phenomenon of working in, which is typical for this syndrome. The examination revealed papillary thyroid cancer, which later was undergoing surgical treatment.
 This case illustrates Lambert-Eaton's paraneoplastic myasthenic syndrome, a rare tumor form associated with thyroid cancer.
 Key words: LambertEaton myasthenic syndrome, paraneoplastic syndrome, thyroid cancer.","PeriodicalId":34156,"journal":{"name":"Klinicheskaia praktika","volume":"21 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135786943","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}