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This is a China, non-interventional, observational study and will follow the Good Phar-macoepidemiology Practices guidelines.
This study will enrol paediatric and adult patients diagnosed with aHUS who will be treated according to routine clinical practice defined by local institutional treatment guidelines/protocol. Those aHUS patients who will be treated with a supportive therapy, which does not contain eculizumab, will be monitored for up to 12 months since the ini-tial diagnosis. Patients initiated on eculizumab treatment anytime between aHUS diagno-sis until 12 months will be followed for additional 12 months, starting from the ecu initia-tion. Patient disposition, characteristics, outcomes and safety will be described for all pa-tients enrolled into this study
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| Measure | Description | Time Frame |
|---|---|---|
| Event free survival, where event defined as ESRD or death on or after TMA onset. | 12 Months |
| Measure | Description | Time Frame |
|---|---|---|
| Death | 12 Months | |
| End-stage renal disease(ESRD) | 12 Months | |
| Complete TMA Response during observation the as evidenced by simultaneous normalization of hemato-logical parameters (platelet count and LDH) and ≥ 25% improvement in serum creatinine from baseline |
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Inclusion Criteria:
Participants are eligible to be included in the study. Age
Patients of any age, who are diagnosed as aHUS by a professional physician (first epi-sode or relapse).
Type of Patient and Disease Characteristics
Evidence of TMA, including thrombocytopenia, evidence of hemolysis, and kidney dys-function, based on the following laboratory findings, should be recorded within 2 weeks time frame:
Gender: Male and/or female. Informed Consent
Willing and able to give written informed consent and comply with the study visit schedule as described in Section 6.2.1. For patients < 18 years of age, patient's legal guardian must be willing and able to give written informed consent and the patient must be willing to give written informed assent (if applicable as determined by the central.
Exclusion Criteria:
Participants are excluded from the study if any of the following criteria apply:
Medical Conditions
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paediatric and adult patients diagnosed with aHUS
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Research Site | Beijing | Beijing Municipality | China | |||
| Research Site |
Qualified researchers can request access to anonymized individual patient-level data from AstraZeneca group of companies sponsored clinical trials via the request portal Vivli.org. All requests will be evaluated as per the AZ disclosure commitment: https://astrazenecagrouptrials.pharmacm.com/ST/Submission/Disclosure.
"Yes", indicates that AZ are accepting requests for IPD, but this does not mean all requests will be approved.
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AstraZeneca will meet or exceed data availability as per the commitments made to the EFPIA/PhRMA Data-Sharing Principles. For details of our timelines, please refer to our disclosure commitment at https://astrazenecagrouptrials.pharmacm.com/ST/Submission/Disclosure.
When a request has been approved AstraZeneca will provide access to the anonymized individual patient-level data via secure research environment Vivli.org. A Signed Data Usage Agreement (non-negotiable contract for data accessors) must be in place before accessing requested information.
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| 12 Months |
| Complete Thrombotic Microangiopathy(TMA ) Response status over time | 12 Months |
| Time to Complete Thrombotic Microangiopathy(TMA ) Response | 12 Months |
| Thrombotic Microangiopathy(TMA ) relapse | 12 Months |
| Observed value and change from baseline in estimated glomerular filtration rate (eGFR) | 12 Months |
| Chronic kidney disease (CKD) stage, as evaluated by the Investigator and classified as improved, stable (no change), or worsened compared to baseline | 12 Months |
| Proteinuria over time. | 12 Months |
| Urine protein-to-creatinine ratio over time. | 12 Months |
| Plasma C3, C4, CH50, Factor H and I, soluble C5b-9 (sC5b-9) and CD46 expression over time. | 12 Months |
| The proportion of patients with normal platelet count (≥150 x 109/L),LDH levels ≤upper limit of normal , serum creatinine < up-per limit of normal for age or an improvement > 25% compared to baseline,eGFR≥ 60 mL/min/1.73 m2, proteinuria negative. | 12 Months |
| Descriptive data of events of interest (serious infec-tions (Aspergillus infections and infections due to en-capsulated bacteria such as Neisseria meningitidis), pregnancy, lactation, and follow-up-data on neonates for 3 months after delivery) | 12 Months |
| Descriptive data of Serious Adverse Events (SAEs) for treatment period 2. | 12 Months |
| Observed Value in Platelet Count | 12 Months |
| Observed Value in Lactate Dehydrogenase (LDH) (mg/dL) | 12 Months |
| Observed Value in Hemoglobin (mg/dL) | 12 Months |
| Change from Baseline in Platelet Count | 12 Months |
| Change from Baseline in Lactate Dehydrogenase (LDH) (mg/dL) | 12 Months |
| Change from Baseline in Hemoglobin (mg/dL) | 12 Months |
| The number of patients still on dialysis during each follow-up visit | 12 Months |
| Chongqing |
| Sichuan |
| China |
| Research Site | Anhui | China |
| Research Site | Beijing | China |
| Research Site | Chongqing | China |
| Research Site | Fujian | China |
| Research Site | Guangdong | China |
| Research Site | Guangxi | China |
| Research Site | Guizhou | China |
| Research Site | Hainan | China |
| Research Site | Hebei | China |
| Research Site | Henan | China |
| Research Site | Hong Kong | China |
| Research Site | Hubei | China |
| Research Site | Hunan | China |
| Research Site | Jiangsu | China |
| Research Site | Jiangxi | China |
| Research Site | Liaoning | China |
| Research Site | Shaanxi | China |
| Research Site | Shandong | China |
| Research Site | Shanghai | China |
| Research Site | Shanxi | China |
| Research Site | Sichuan | China |
| Research Site | Tianjin | China |
| Research Site | Xinjiang | China |
| Research Site | Zhejiang | China |
| ID | Term |
|---|---|
| D065766 | Atypical Hemolytic Uremic Syndrome |
| ID | Term |
|---|---|
| D006463 | Hemolytic-Uremic Syndrome |
| D014511 | Uremia |
| D007674 | Kidney Diseases |
| D014570 | Urologic Diseases |
| D052776 | Female Urogenital Diseases |
| D005261 | Female Urogenital Diseases and Pregnancy Complications |
| D000091642 | Urogenital Diseases |
| D052801 | Male Urogenital Diseases |
| D000743 | Anemia, Hemolytic |
| D000740 | Anemia |
| D006402 | Hematologic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
| D057049 | Thrombotic Microangiopathies |
| D013921 | Thrombocytopenia |
| D001791 | Blood Platelet Disorders |
| D000095542 | Cytopenia |
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