aHUS Clinical and Diagnostic Check List

Patient Name:DOB:

NHS Number:Ethnicity:

Initiating trigger
Non-shiga toxin diarrhoea
Respiratory tract infections
Other infection
Malignancy
Bone marrow transplantation
New medication (see list*)
details
Family member also affected
Pregnancy associated
Date of Presentation : / Extra-renal manifestations
Neurological involvement
Pancreatic Involvement
Ocular involvement
Digital gangrene
Other (please provide details)
______
The patient is on
Haemodialysis
If yes, start date? ………………..
Plasma Exchange
If yes, start date? ………………..
Full Clinical History (must be completed)

*Drugs associated with aHUS

Cisplatin ; Gemcitabine ; Mitomycin ; Clopidogrel ; Quinine; Interferon α,β; Anti-vascular endothelial growth factor; Campath; Cyclosporin tacrolimus ; Ciprofloxacin; Oral contraceptives, Tranexamic Acid Illicit drugs [e.g. cocaine, heroin, ecstasy]

Tick this box to confirm that the patient is eligible for treatment under NHS England

Name of Consultant ______

Phone contact of Consultant______

E-mail address of Consultant______

Date of Completion______

Results of investigations confirming a thrombotic microangiopathy and AKI

Test / Date / Result
Haemoglobin
Platelet count
Blood film
Lactate Dehydrogenase
C reactive protein
Haptoglobin
Prothrombin time
Fibrinogen
Direct antiglobulin test
Creatinine (at presentation)
Creatinine (at baseline if known)
Urinalysis
Renal ultrasound
Renal biopsy

The following should ALL be requested to help with the diagnosis of aHUS

The results of all these need not be back before you send this form but the result of the ADAMTS13 activity must be available.

Differential Diagnosis / Test / Date Requested / Result
TTP / ADAMTS13 activity%
STEC HUS / Stool culture swab
STEC HUS / Serodiagnosis of Shiga-toxin-producing E. coli (STEC)^
STEC HUS / PCR for VTEC virulence genes in stool^
aHUS / C3
aHUS / C4
APL antibody syndrome / APL antibody
HIV / HIV test
Malignant hypertension / Blood Pressure
Malignant hypertension / ECG
Malignant hypertension / Echocardiogram
Plasma cell dyscrasias ¥ / Serum/Urine electrophoresis
Plasma cell dyscrasias ¥ / Serum Free light chains
Scleroderma / ANA
Scleroderma / Anticentromere antibodies
Scleroderma / Anti-scl-70
SLE / ds-DNA
aHUS / CH100/AH100%
aHUS / Complement Factor H/I%
aHUS / Complement genetics%
aHUS / Factor H autoantibodies%

% Screening tests for complement abnormalities and ADAMTS13 activity are requested directly at combined aHUS lab diagnostic services in Newcastle

¥ Investigation for plasma cell dyscrasias is warranted as a monoclonal gammopathy has been reported in these patients

^Ask your microbiology laboratory to send serum and stool samples to the Gastrointestinal Infections Reference Unit at Colindale for these two investigations. Investigation for STEC-HUS should be routine in all patients with presumed aHUS as ~5% of STEC-HUS has no prodromal diarrhoea while 30% of complement-mediated aHUS does have concurrent diarrhoea or gastroenteritis

The following tests may be helpful depending upon clinical presentation

Differential Diagnosis / Test / Date Requested / Result
Cobalamin C disease ∞ / Plasma homocysteine levels
Cobalamin C disease ∞ / Plasma and urine methylmalonic acid levels
G6PD deficiency / G6PD activity
Pneumococcal HUS / T antigen
Pneumococcal HUS / Urinary Antigen
Pregnancy / Pregnancy test
Viral Triggers / Influenza swab
Viral Triggers / CMV PCR
Viral Triggers / EBV PCR

∞ Testing should be performed in paediatric cases. Adult-onset cobalamin C-related HUS has been reported and testing could be considered

aHUS Diagnostic Checklist version 31st August 2017 Page 1 of 3