Difficulties in diagnosing atypical hemolytic uremic syndrome
The paper presents the case of clinical observation of a patient with atypical hemolytic-uremic syndrome (aHUS). aHUS is a disease characterized by an unfavorable prognosis (severe or catastrophic course with rapid development of terminal renal or multi-organ failure). The aim of the study is to eva...
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Russian Academy of Sciences, Siberian Branch Publishing House
2020-01-01
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| Series: | Сибирский научный медицинский журнал |
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| Online Access: | https://sibmed.elpub.ru/jour/article/view/317 |
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| author | N. V. Fomina L. D. Chesnokova O. A. Konderova S. A. Smakotina E. V. Utkina V. Yu. Isaev |
| author_facet | N. V. Fomina L. D. Chesnokova O. A. Konderova S. A. Smakotina E. V. Utkina V. Yu. Isaev |
| author_sort | N. V. Fomina |
| collection | DOAJ |
| description | The paper presents the case of clinical observation of a patient with atypical hemolytic-uremic syndrome (aHUS). aHUS is a disease characterized by an unfavorable prognosis (severe or catastrophic course with rapid development of terminal renal or multi-organ failure). The aim of the study is to evaluate the approaches to differential diagnosis of aHUS in clinical practice.Material and methods. The study was conducted on the basis of the Nephrology Department of Kemerovo Regional Clinical Hospital n.a. S.V Belyaev. The clinical observation of patient D., aged 26 years old, is discussed.Results and discussion. Diagnosing aHUS requires: 1) diagnosing thrombotic microangiopathy (TMA: thrombocytopenia or decrease in platelet count by more than 25 % of original, visceral damage (kidneys, CNS, gastrointestinal tract, heart, lungs)); 2) ruling out HUS associated with Shiga toxin-producing Escherichia coli (STEC-HUS; negative for Shiga-toxin in blood and stool), thrombotic thrombocytopenic purpura (TTP), systemic connective tissue disease, catastrophic antiphospholipid syndrome, HIV infection; 3) assessing the activity of ADAMTS13 (decrease confirms the aHUS diagnosis); 4) proving normal content of complement components C3 and C4 as an additional argument in favor of aHUS diagnosis. At the first stage, the patient was diagnosed with TMA (platelet content 37 x 109/l, hemoglobin content 59 g/l), LDH up to 824 E/l), liver damage (AST, ALT and LDH activity 55, 60 and 824 U/l, respectively), kidney damage (acute renal damage), lungs, heart, and brain damage. At the second stage the following diagnoses were ruled out: STEC-HUS (Shiga toxin in blood and stool was not detected), TTP (ADAMTS13 activity level was 66 %, whereas reference values are 93-113 %, in TTP - below 5-10 %); systemic connective tissue diseases catastrophic antiphospholipid syndrome, HIV infection sepsis. Normal values of C3 (0.9 g/l) and C4 (0.23 g/l) complement components did not rule out the diagnosis of aHUS. |
| format | Article |
| id | doaj-art-13b03505f72648a2811ca0e1a2d93b3f |
| institution | Kabale University |
| issn | 2410-2512 2410-2520 |
| language | Russian |
| publishDate | 2020-01-01 |
| publisher | Russian Academy of Sciences, Siberian Branch Publishing House |
| record_format | Article |
| series | Сибирский научный медицинский журнал |
| spelling | doaj-art-13b03505f72648a2811ca0e1a2d93b3f2025-08-20T03:50:43ZrusRussian Academy of Sciences, Siberian Branch Publishing HouseСибирский научный медицинский журнал2410-25122410-25202020-01-01396929710.15372/SSMJ20190612225Difficulties in diagnosing atypical hemolytic uremic syndromeN. V. Fomina0L. D. Chesnokova1O. A. Konderova2S. A. Smakotina3E. V. Utkina4V. Yu. Isaev5Kemerovo Regional Clinical Hospital n.a, S.V Belyaev; Kemerovo State Medical University of Minzdrav of RussiaKemerovo Regional Clinical Hospital n.a, S.V Belyaev; Kemerovo State Medical University of Minzdrav of RussiaKemerovo Regional Clinical Hospital n.a, S.V BelyaevKemerovo Regional Clinical Hospital n.a, S.V Belyaev; Kemerovo State Medical University of Minzdrav of RussiaKemerovo State Medical University of Minzdrav of RussiaKemerovo State Medical University of Minzdrav of RussiaThe paper presents the case of clinical observation of a patient with atypical hemolytic-uremic syndrome (aHUS). aHUS is a disease characterized by an unfavorable prognosis (severe or catastrophic course with rapid development of terminal renal or multi-organ failure). The aim of the study is to evaluate the approaches to differential diagnosis of aHUS in clinical practice.Material and methods. The study was conducted on the basis of the Nephrology Department of Kemerovo Regional Clinical Hospital n.a. S.V Belyaev. The clinical observation of patient D., aged 26 years old, is discussed.Results and discussion. Diagnosing aHUS requires: 1) diagnosing thrombotic microangiopathy (TMA: thrombocytopenia or decrease in platelet count by more than 25 % of original, visceral damage (kidneys, CNS, gastrointestinal tract, heart, lungs)); 2) ruling out HUS associated with Shiga toxin-producing Escherichia coli (STEC-HUS; negative for Shiga-toxin in blood and stool), thrombotic thrombocytopenic purpura (TTP), systemic connective tissue disease, catastrophic antiphospholipid syndrome, HIV infection; 3) assessing the activity of ADAMTS13 (decrease confirms the aHUS diagnosis); 4) proving normal content of complement components C3 and C4 as an additional argument in favor of aHUS diagnosis. At the first stage, the patient was diagnosed with TMA (platelet content 37 x 109/l, hemoglobin content 59 g/l), LDH up to 824 E/l), liver damage (AST, ALT and LDH activity 55, 60 and 824 U/l, respectively), kidney damage (acute renal damage), lungs, heart, and brain damage. At the second stage the following diagnoses were ruled out: STEC-HUS (Shiga toxin in blood and stool was not detected), TTP (ADAMTS13 activity level was 66 %, whereas reference values are 93-113 %, in TTP - below 5-10 %); systemic connective tissue diseases catastrophic antiphospholipid syndrome, HIV infection sepsis. Normal values of C3 (0.9 g/l) and C4 (0.23 g/l) complement components did not rule out the diagnosis of aHUS.https://sibmed.elpub.ru/jour/article/view/317thrombotic microangiopathyatypical hemolytic-uremic syndromecompliment systemalternative way of activation of the compliment systemeculizumab |
| spellingShingle | N. V. Fomina L. D. Chesnokova O. A. Konderova S. A. Smakotina E. V. Utkina V. Yu. Isaev Difficulties in diagnosing atypical hemolytic uremic syndrome Сибирский научный медицинский журнал thrombotic microangiopathy atypical hemolytic-uremic syndrome compliment system alternative way of activation of the compliment system eculizumab |
| title | Difficulties in diagnosing atypical hemolytic uremic syndrome |
| title_full | Difficulties in diagnosing atypical hemolytic uremic syndrome |
| title_fullStr | Difficulties in diagnosing atypical hemolytic uremic syndrome |
| title_full_unstemmed | Difficulties in diagnosing atypical hemolytic uremic syndrome |
| title_short | Difficulties in diagnosing atypical hemolytic uremic syndrome |
| title_sort | difficulties in diagnosing atypical hemolytic uremic syndrome |
| topic | thrombotic microangiopathy atypical hemolytic-uremic syndrome compliment system alternative way of activation of the compliment system eculizumab |
| url | https://sibmed.elpub.ru/jour/article/view/317 |
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