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Shortness of breath and abdominal pain

Reviewed by Romesh K. Khardori, MD, PhD

Masryyy/Wikimedia Commons/CC BY-SA 4.0

A 21-year-old man presents with shortness of breath and abdominal pain. He has a BMI of 34.6 kg/m2 and explains that he has had asthma for several years, using an inhaler when needed. He reports a few weeks of polydipsia and polyuria. The patient notes that his father has kidney disease. He believes other close relatives are managing chronic metabolic conditions but does not know any further detail regarding their diagnoses. On laboratory testing, blood pH is 6.30 and bicarbonate level is 11.1 mmol/L. A1c is 12.0%. Acanthosis nigricans are noted on the neck and in the axilla bilaterally.

What is the likely diagnosis?

Hyperosmolar hyperglycemic state (HHS)

Metabolic acidosis

Lactate acidosis

Ketosis-prone diabetes type 2

Addison disease

On the basis of the patient's history and clinical presentation, the likely diagnosis is ketosis-prone diabetes (KPD) type 2. KPD is widely thought of as an atypical diabetes syndrome, though some groups consider it to be a common clinical presentation in newly diagnosed patients with type 2 diabetes (T2D) rather than a subtype of disease. The condition is more prevalent in males and among Black and Hispanic populations.

Definitive diagnosis of the type of diabetes can present a clinical challenge during acute presentation. Although the majority of diabetic ketoacidosis (DKA) episodes occur in patients previously diagnosed with type 1 diabetes, an estimated 34% occur in patients with T2D. For patients who are obese or have a family history of diabetes, there should be a high index of suspicion for new-onset DKA in type 2 diabetes.

Patients with KPD typically present in a state of ketoacidosis with a brief but acute history of hyperglycemic symptoms. Hyperglycemia, elevated anion gap acidosis, and ketonemia form the expected constellation of DKA symptoms. A key consideration in the differential diagnosis is hyperosmolar hyperglycemic state (HHS). Patients with HHS are much more likely to have altered mental status than are patients with DKA. Metabolic acidosis and ketonemia are absent or mild, and anion gap is either normal or slightly elevated. In HHS, extreme elevations of glucose are seen, with a lack of significant ketoacidosis. Glucose levels tend to be higher in HHS than in DKA; they are almost always > 600 mg/dL, and levels > 1000 mg/dL are not uncommon. In DKA, glucose levels are still markedly high — generally 500-800 mg/dL but rarely exceeding 900 mg/dL. Patients with DKA also usually present with an A1c > 10% and a blood pH < 7.30.

Treatment for KPD is initially acute, beginning with aggressive intravenous fluid and insulin therapy A. Once this state resolves, insulin requirements typically decrease for patients with T2D, and they are able to maintain adequate glycemic control with an oral therapy regimen.

Romesh K. Khardori, MD, PhD, Professor, Department of Internal Medicine, Division of Diabetes, Endocrine, and Metabolic Disorders, Eastern Virginia Medical School; EVMS Medical Group, Norfolk, Virginia.

Romesh K. Khardori, MD, PhD, has disclosed no relevant financial relationships.

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