This “In the kNOW” article is going to cover information related to hemolytic-uremic syndrome (HUS). Let’s begin by discussing what this condition is. HUS is a rare condition that most often occurs with Shiga toxin-producing E. coli or STEC. As the toxin travels through the bloodstream, it causes damage to the blood vessels. In the kidneys, this damage can cause clots to form which in turn obstructs the filtering process of the kidneys and leads to kidney failure. Some of the symptoms of hemolytic-uremic syndrome are bloody diarrhea, chills, fever, and vomiting.
With the exception of hereditary HUS, there is usually a triggering event which initiates the HUS. This could be an infection such as COVID or the flu, or conditions such as autoimmune or metabolic disorders, pregnancy, or transplants.
With the ICD-10-CM code updates for 2023, the coding of HUS has been expanded. There are now four different codes that can be assigned depending on the type of hemolytic-uremic syndrome that is documented. The new codes are:
D59.30 Hemolytic-uremic syndrome, unspecified
Hemolytic-uremic syndrome NOS
D59.31 Infection-associated hemolytic-uremic syndrome
Shiga toxin-producing E. coli [STEC] related hemolytic uremic syndrome
Typical hemolytic uremic syndrome
D59.32 Hereditary hemolytic-uremic syndrome
Atypical hemolytic uremic syndrome with an identified genetic cause
D59.39 Other hemolytic-uremic syndrome
Atypical (nongenetic) hemolytic uremic syndrome
Secondary hemolytic-uremic syndrome
As always, there is a code if the type of the HUS is unspecified. There is also a code to capture HUS when it is associated with an infection which is also known as typical HUS. While Shiga toxin-producing E. coli is often the cause, other infections such as salmonella and pneumococcus are also linked to typical HUS. A separate code if there is a hereditary cause for the condition. Finally, the option for other HUS could be assigned when the HUS is documented as secondary or atypical.
Along with the expansion of codes for HUS, the 2023 ICD-10-CM update brought two new guidelines related to coding of HUS with HIV and sepsis.
I.C.1.a.2.a Patient admitted for HIV-related condition
If a patient is admitted for an HIV-related condition, the principal diagnosis should be B20, Human immunodeficiency virus [HIV] disease followed by additional diagnosis codes for all reported HIV-related conditions
An exception to this guideline is if the reason for admission is hemolytic-uremic syndrome associated with HIV disease. Assign code D59.31, Infection-associated hemolytic-uremic syndrome, followed by code B20, Human immunodeficiency virus [HIV] disease.
I.C.1.d.9 Hemolytic-uremic syndrome associated with sepsis
If the reason for admission is hemolytic-uremic syndrome that is associated with sepsis, assign code D59.31, Infection-associated hemolytic-uremic syndrome, as the principal diagnosis. Codes for the underlying systemic infection and any other conditions (such as severe sepsis) should be assigned as secondary diagnoses.
It is important to ensure that the notes which accompany each of the expanded codes are followed when assigning codes. For example, at the D59 category, a “Code also” note instructs coding professionals to assign acute kidney failure and/or chronic kidney failure if applicable. There is a “Use additional code” note at D59.31 for the infection-associated HUS to identify the infection. D59.32 has a “Code also” note when defects in the complement system or methylmalonic acidemia are applicable. Finally, other hemolytic-uremic syndrome has three associated notes. First, a “Code first” note if COVID or kidney, heart, or liver transplant are associated. Second, a “Code also” note for any associated conditions such as SLE, cancer, or hypertensive emergency. The final note is a “Use additional code” note if there is an applicable adverse effect of a drug.
Now you are In the kNOW!!
Dianna Foley, RHIA, CCS, CHPS, CDIP is
OHIMA's Education Coordinator. Dianna has been an HIM
professional for over 20 years. She progressed through the ranks of coder,
department supervisor, and department director, to her current role as a
coding consultant. She previously served as the program director for Medical Coding and HIT at Eastern Gateway Community College. Dianna earned her bachelor's degree from the University of Cincinnati and holds RHIA, CHPS, CDIP and CCS certifications. She is an AHIMA Approved ICD-10-CM/PCS Trainer and is a presenter on coding topics at the national, state, and regional levels. Dianna mentors new AHIMA members and also provides monthly educational lectures to coders and clinical documentation specialists.
