Our Summary

This research paper talks about a rare deadly disease known as Calcific Uremic Arteriolopathy (CUA), or more commonly, calciphylaxis. The disease typically affects people with severe kidney disease and leads to hardening of the blood vessels, tissue damage, and the formation of unique skin sores with black scabs. Various treatments exist including sodium thiosulfate, cinacalcet, phosphate binders and in more severe cases, parathyroidectomy (surgery to remove the parathyroid glands). The paper reports a case where a patient with recurring, treatment-resistant CUA was successfully treated with parathyroidectomy. However, post-surgery complications occurred including a condition called ‘hungry bone syndrome’ and worsening of her sores before they totally healed. There’s also a discussion on the complications of CUA, including the debate around the use of parathyroidectomy and the risks of aggressive management of hungry bone syndrome.

FAQs

  1. What is Calcific Uremic Arteriolopathy (CUA) and how is it usually treated?
  2. How can parathyroidectomy be used in the treatment of calciphylaxis?
  3. What are the potential complications and risks associated with using parathyroidectomy to treat CUA?

Doctor’s Tip

A doctor may advise a patient undergoing parathyroidectomy to closely monitor their calcium levels postoperatively, as they may experience a drop in calcium levels known as hungry bone syndrome. This can lead to symptoms such as muscle cramps, numbness, and tingling. It is important to follow up with your healthcare provider regularly and adhere to any prescribed calcium and vitamin D supplements to prevent complications.

Suitable For

Patients who are typically recommended for parathyroidectomy are those with primary hyperparathyroidism, secondary hyperparathyroidism due to chronic kidney disease, or tertiary hyperparathyroidism. In the case of calcific uremic arteriolopathy (CUA) or calciphylaxis, parathyroidectomy may be considered in resistant cases where other treatment options have failed. It is important to carefully assess the risks and benefits of surgery in these patients, as the postoperative course can be complicated by conditions such as hungry bone syndrome.

Timeline

Before parathyroidectomy:

  1. Patient is diagnosed with calciphylaxis, a rare and lethal disease that affects patients with end-stage renal disease.
  2. Patient undergoes various treatment options such as sodium thiosulfate, cinacalcet, and phosphate binders, but the disease remains resistant.
  3. Parathyroidectomy is considered as a last resort treatment option for the patient.

After parathyroidectomy:

  1. Patient undergoes parathyroidectomy surgery to remove the overactive parathyroid gland causing the disease.
  2. Postoperative course is complicated by hungry bone syndrome, a condition where the body rapidly absorbs calcium from the bones leading to low calcium levels in the blood.
  3. Patient experiences worsening of skin lesions before they completely heal due to the effects of hungry bone syndrome.
  4. Patient eventually recovers from the surgery and experiences improvement in their condition, with successful treatment of the recurrent calciphylaxis.

What to Ask Your Doctor

  1. What is a parathyroidectomy and how does it help treat calciphylaxis (CUA)?
  2. What are the potential risks and complications associated with a parathyroidectomy?
  3. How long is the recovery period after a parathyroidectomy?
  4. Are there any alternative treatment options to consider before deciding on a parathyroidectomy?
  5. Will a parathyroidectomy completely cure my calciphylaxis or are there chances of recurrence?
  6. How will a parathyroidectomy affect my overall kidney function and calcium levels?
  7. How many parathyroidectomies have you performed for patients with calciphylaxis and what is your success rate?
  8. Are there any specific pre-operative preparations I need to follow before undergoing a parathyroidectomy?
  9. How often will I need to follow up with you after the surgery for monitoring and management of any complications?
  10. Can you provide me with any resources or support groups for patients who have undergone a parathyroidectomy for calciphylaxis?

Reference

Authors: Hassanein M, Laird-Fick H, Tikaria R, Aldasouqi S. Journal: BMJ Case Rep. 2018 Dec 22;11(1):e226696. doi: 10.1136/bcr-2018-226696. PMID: 30580300