European paediatric research group for hus and related disorders

CQ_IBK_aHUS_02 / version 11/09
European Paediatric Research Group for HUS and
related disorders
Case questionnaire for diarrhoea negative/VTEC
(STEC) negative cases – transplantation
1. INSTRUCTIONS
Please type within the spaces indicated. Missing data should be left blank. To create a pedigree, symbols can be copied from the worked example in footnote 4. The declaration of consent must be affirmed in every case.
The questionnaire can be returned electronical y.
2. DEFINITION OF HUS
microangiopathic hemolytic anemia: hemoglobin < 10g/dl with fragmented erythrocytes thrombocytopenia: platelet count < 130 000/mm³ renal impairment: serum creatinine > age related range or GFR < 80 ml/min/1.73m² by Schwartz Formula. (Note renal impairment may not be evident at onset, investigator to use discretion in this circumstance) . EXCLUSION
single episode of HUS preceded by diarrhoea single episode of HUS without diarrhoea but with evidence of VTEC infection disseminated consumptive intravascular coagulation, eg secondary to sepsis 4. DECLARATION
The local investigator confirms that the patient and/or the patient´s parents/guardians have given permission for this information to be used by the European Paediatric Research Group for HUS, and a record of such agreement is retained in the patients medical record. Confirmed: 5. IDENTIFICATION OF PHYSICIANS: LOCAL INVESTIGATOR
Name: ____________________Surname: ___________________________Unit/Department: ____________________________________________________Hospital: ___________________________________________________________Address: ___________________________________________________________Tel: ___________________________Fax: ________________________________E-mail: _____________________________________________________________ Actual date: _________________________________________________________ . IDENTIFICATION OF PATIENT
Date of birth: ___/___/___ Hospital: ________________ Date of diagnosis of first episode of HUS (DD/MM/YY) __/__/_____ Time between first diagnosis and ESRD ______ LAPSES BEFORE TRANSPLANTATION
For Scores see footnotes 1-3 at page 8.
Creatinine clearance (Schwartz formula)³ 8. INVESTIGATION TOWARDS AETIOLOGY
Genetic testing:
Homozygous deletion of ________________________________ Heterozygous deletion of ________________________________ Polymorphism in _______________________________________ CFH antibodies:
Titer Follow-up (if not measured in Innsbruck): 9. RENAL HISTOLOGY
Date of renal specimen collection (DD/MM/YY) __/__/____ Predominant glomerular thrombotic micro-angiopathy (TMA) minimal moderate/patchy diffuse/extensive . PRE-TRANSPLANT INVESTIGATION
Creatinine clearence (Schwartz formula)hemoglobin level 0.1 . HYPERTENSION Score as footnote 1: 11. PRE-TRANSPLANT TREATMENT
. FIRST RENAL TRANSPLANTATION
Date of transplantation: (DD/MM/YY) __/__/______ If yes, please specifiy: _________________________________ If yes, please specify __________________________________________________ 13. DISEASE RECURRENCE
lease copy if more than 1 recurrence
If yes, date: (DD/MM/YY) ___/___/___ number: ____ Maximum serum creatinine (before dialysis) µmol/l If yes, number of different anti-hypertensive drugs needed for control of BP: ___________________________________________________________________ ___________________________________________________________________ ___________________________________________________________________ 4. OUTCOME AFTER FIRST RENAL TRANSPLANTATION
Date of last follow-up: (DD/MM/YY) __/___/_____ ___________________________________________________________________ lease copy 12-14 if more than one transpla n
tation was performed
OOTNOTES
Footnote 1:
Blood pressure/ Hypertension:
Systolic and/or diastolic BP>97th percentile According to local reference values while not on antihypertensive therapy Normal BP ≤ 95 percentile for sex and height Footnote 2:
Proteinuria:
Albustix (or equivalent) trace or negative on early morning urine sample or protein/creatinine <20mg/mmol or <0.2g/g Mild to moderate proteinuria, Albustix 1+ to2+ (=up to 1g/l), or protein/creatinine ratio 20-200 mg/mmol or 0.2-2.0g/g Heavy proteinuria, Albustix 3+ or 4+, or protein/creatinine > 200mg/mmol or >2.0g/g Footnote 3:
Creatinine Clearance according to Schwartz formula:
Clearance (ml/min/1.73m²) = Ht (cms) x k to converte creatinine in mg/dl to micromol/l multiply by 0.885

Source: http://www.hus-online.at/download/transplant.pdf