Who to call aHUS -D59.32 or D59.39?

Who are Alois Alzheimer, Cecil Alport, Burrill Crohn, Guillaume Duchenne, James Parkinson and Graham Hughes and what do they have in common?

They are names of clinicians whose research to describe a certain health condition resulted in that condition being named after them.

Hemolytic Uremic Syndrome could have been called Gass er Syndrome after the Swiss clinical researcher, Conrad Von Gasser, who first described the disease. However the words hemolytic, uremic and syndrome he used for the HUSs that he had observed in his young patients became more common place.

Giving a name to a disease is more “regulated” these days but once it enters common use it becomes difficult to change. Monkey Pox is one of the more recent examples of an unacceptable name of a disease which was based on an animal where a virus was discovered but which humans could also be affected by. Mpox is the new name.

Swine flu, bird flu , bat flu (?) are diseases which are transmitable to humans are other examples of animal names.

Another source of names which is controversial is the location suspected of being the source of outbreak like Spanish flu, Middle East Respiratory Syndrome ( MERS).

The World Health Organisation , WHO, have set guidelines for naming new diseases to avoid unpleasantness or

The WHO keeps a register of all approved names of medical conditions in its International Clasification of Diseases or the ICD.

According to the WHO “ICD serves a broad range of uses globally and provides critical knowledge on the extent, causes and consequences of human disease and death worldwide via data that is reported and coded with the ICD. Clinical terms coded with ICD are the main basis for health recording and statistics on disease in primary, secondary and tertiary care, as well as on cause of death certificates. These data and statistics support payment systems, service planning, administration of quality and safety, and health services research. Diagnostic guidance linked to categories of ICD also standardizes data collection and enables large scale research.

So diseases are classified with a codes.

In the American version of the ICD ( other international versions of ICD-10 D59.32 may differ) HUS is included within the “hemolytic anemia code D59”.

Its actual code is D59.3 and it is further sub-coded for types of HUS.

There is no mention of a code specifically for aHUS. See the ICD entry below.

Except drilling down on D59.32 Hereditary hemolytic-uremic syndrome reveals that it includes Atypical hemolytic uremic syndrome “with a definite genetic cause”.

The use of “Hereditary” seems to be a blast from the past! Less than 20% of aHUS is “in the family”. But if it is a first case predisposition could be passed.

Also D59.39 Other hemolytic -uremic syndrome includes Atypical ( non genetic) hemolytic uremic syndrome and secondary hemolytic-uremic syndrome. ( hyphenating seems variable!)

These only became a new codes in the ICD on 1st October 2022.

  • D59.3 Hemolytic-uremic syndrome
    •  D59.30 …… unspecified
    •  D59.31 Infection-associated hemolytic-uremic syndrome
    •  D59.32 Hereditary hemolytic-uremic syndrome
    •  D59.39 Other hemolytic-uremic syndrome

D59.31 becomes the Infection-associated hemolytic-uremic syndrome includes Shiga toxin-producing E.coli (STEC) related hemolytic uremic syndrome or Typical hemolytic uremic syndrome. Other codes can be used to identify typical HUS with other infections such as sepsis, HIV, pneumococcal meningitis.

if all patients with aHUS were classified with either D59.32 or D59.39 in their medical records then it would possible to get a total of patients with genetic , non genetic and secondary forms of aHUS. “IF”.

But now having got an ICD of aHUS do these terms pass the test of a name which describes the clinical mechanism of the disease I.e. thrombotic microangiopathy which results from uncontrolled complement involvement?

What name would do that?

That is the question that is being answered somewhere.

Hughes Syndrome is referred to as Antiphospholipid Syndrome, or APS, these days but the others have stuck.

Article No. 652

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