Delayed diagnosis, barriers to care increase morbidity in children with Cushing’s syndrome

Hispanic and black children diagnosed with Cushing’s syndrome are more likely to present with higher cortisol measurements and larger tumor size vs. white children, according to study findings presented at the annual Pediatric Academic Societies Meeting in Baltimore.

“Racial and socioeconomic disparities may contribute to the severity of disease presentation for children with Cushing’s [syndrome],” Alexandra Gkourogianni, MD, of the section on endocrinology and genetics at the Eunice Kennedy Shriver National Institute of Child Health and Human Development, and colleagues wrote. “Minority children from disadvantaged backgrounds present more frequently with comorbidities associated with longstanding [Cushing’s syndrome].”

Gkourogianni and colleagues analyzed data from 135 children treated for Cushing’s syndrome (transsphenoidal surgery) at the NIH between 1997 and 2015 (mean age, 13 years; 51% girls; 33% Hispanic or black). Researchers used a 10-point index for rating severity in pediatric Cushing’s syndrome based on predefined cutoffs; degree of hypercortisolemia, impaired glucose tolerance, and hypertension were graded on a 3-point scale (0-2); height, BMI z scores, duration of disease, and tumor invasion were graded on a 2-point scale (0-1).

Researchers found that midnight cortisol measurements were higher among Hispanic and black children vs. white children (23.3 µg/dL vs. 16 µg/dL; P = .019), as were tumor sizes (mean 6.3 mm vs. 3.3 mm; P = .016). Height standard deviation score was more severely affected in black and Hispanic children (–1.6 vs. –1.1; P = .038), and mean Cushing’s syndrome score for Hispanic and black children was higher vs. white children (4.5 vs. 3.8; P = .033).

Researchers found that median income had an independent correlation with Cushing’s syndrome score in univariate regression analysis for covariates of socioeconomic status and demographics (P = .025). Multivariable regression analysis using race, prevalence of obesity, estimated income, access to pediatric endocrinologist, age and sex confirmed that race, along with lower socioeconomic status and older age, were predictors of a higher Cushing’s syndrome score (P = .002).

“We speculate that delayed diagnosis, barriers to access to care and poorer quality health care for these underserved patients may contribute to presentation at a later age and increased morbidity,” the researchers wrote. “Additional research is needed to identify potential modifiable factors that may improve care for these patients.” – by Regina Schaffer

Reference:

Gkourogianni A, et al. Poster #445. Presented at: Pediatric Academic Societies Meeting; April 30- May 3, 2016; Baltimore.

Disclosure: Endocrine Today was unable to determine relevant financial disclosures.

From http://www.healio.com/endocrinology/adrenal/news/online/%7Be79d7c84-d539-4a04-a548-882b9f4caadd%7D/delayed-diagnosis-barriers-to-care-increase-morbidity-in-children-with-cushings-syndrome

Woman diagnosed with Cushing’s syndrome

Thursday, May 05, 2016
AsiaOne

SINGAPORE – The Health Sciences Authority (HSA) has warned the public not to purchase or consume a health product labelled in Chinese as ‘Hai Leng Hai Beh Herbal Itch Removing Capsule’ after a customer consumed it and has now been diagnosed with a metabolic disorder known as Cushing’s syndrome.

In a press release today (May 5), the HSA revealed that a woman in her 40s, bought the capsules from a retail store overseas to ease an itchy skin condition after a relative’s recommendation.

Just a day after consumption her itchy skin condition improved. However after consuming the pills for two months, she gained weight rapidly and her face became puffy.

Evidence later showed that the pills were not entirely herbal in nature and contained “potent traces of steroid and painkillers like paracetemol”, according to HSA.

Dexamethsone, a steroid which is usually prescribed for inflammatory conditions, is known to cause increased blood glucose levels that cause diabetes, high blood pressure, cataracts and muscular and bone disorders when taken over a long period of time without supervision.

These medical conditions may, in turn, cause a consumer to develop Cushing’s syndrome, said HSA.

Chlorpheniramine was also found in the capsule. HSA said the antihistamine is commonly known to treat allergic reactions and can cause drowsiness, blurred vision, vomiting and constipation.

Paracetamol is known to cause swelling of the lips or face and rashes.

The HSA has advised all sellers to stop selling and distributing the capsules immediately.

If caught selling, shop owners could be fined up to $10,000 and may be imprisoned for a period of up to two years, if convicted.

To learn more about the dangers of buying health products from dubious sources, you can visit http://www.healthdangers.sg.

Those with any information on the sale and supply of these capsules or other illegal products can contact HSA’s Enforcement Branch at 68663485 during office hours from Monday to Friday.

mldas@sph.com.sg

– See more at: http://yourhealth.asiaone.com/content/woman-diagnosed-cushings-syndrome-hsa-warns-public-avoid-itch-removing-capsule#sthash.3odVhMMe.dpuf

Help Cushing’s Help to Help You!

Fundrazr

Please help support the Cushings-Help web sites.

Cushings-Help has provided information and support for people with Cushing’s or other endocrine problems, their friends and families for over 15 years.

The Cushings Help family of websites and the message boards are growing fast. Each has outgrown it’s web server and had to be moved to accommodate this growth.

Many of the people on the boards have agreed to help support this growth. You can help, too.

See our Fundrazr at http://fnd.us/apR55?ref=sh_73Ppz4

Recruitment for Cushing’s Syndrome Clinical Study

DESCRIPTION

This trial is testing the safety and effectiveness of a new investigational drug for the treatment of Cushing’s Syndrome. Under the supervision of qualified physicians, cortisol levels and symptoms of Cushing’s Syndrome will be closely followed along with any signs of side effects.

The link below will take you to the trial website where you can review additional information and the patient screener.

http://curec.lk/1X0J6kT

Addison’s Disease vs Cushing’s Syndrome Nursing

Cushing’s and Addison’s Disease. An endocrine NCLEX review on how to differentiate between Cushing’s Syndrome/Disease vs Addison’s and Addisonian Crisis. In this video, I will discuss the pathophysiology, signs & symptoms, and nursing interventions for these endocrine disorders of the adrenal cortex and pituitary glands.

 

Addison’s Disease and Cushing’s Syndrome/Disease review notes for nursing school and NCLEX exam. In nursing school and for the NCLEX exam, you will need to know how to provide care to a patient with either Addison’s Disease or Cushing’s.

However, many students get these two endocrine disorders confused, but these review notes will help you differentiate between them.

These NCLEX review notes will cover:

  • Signs and Symptoms of Addison’s Disease vs Cushing’s
  • Causes of Addison’s Disease and Cushing’s
  • Nursing Management of Addison’s Disease and Cushing’s

After reviewing these notes, don’t forget to take the Addison’s Disease vs Cushing’s Quiz.

Addison’s Disease vs Cushing’s

Major Players in these endocrine disorders:

  • Adrenal Cortex
  • Steroid Hormones
    • Corticosteroids (specifically Aldosterone (mineralocorticoid) & Cortisol (glucocorticoid)

Role of Adrenal Cortex: releases steroid hormones and sex hormones

Role of Aldosterone: regulates blood pressure through renin-angiotensin-aldosterone system, helps retain sodium and secretes potassium (balances sodium and potassium levels).

Role of Cortisol: “STRESS Hormone” helps the body deal with stress such as illness or injury, increases blood glucose though glucose metabolism, break downs fats, proteins, and carbs, regulates electrolytes.

Cushing’s (Syndrome & Disease)

Cushing’s: hyper-secretion of CORTISOL (watch video for clever ways to remember this)

Cushing’s Syndrome vs Cushing’s Disease

Cushing’s Syndrome: caused by an outside cause or medical treatment such as glucocorticoid therapy

Cushing’s Disease: caused from an inside source due to the pituitary gland producing too much ACTH (Adrenocorticotropic hormone) which causes the adrenal cortex to release too much cortisol.

Signs & Symptoms of Cushing’s

Remember the mnemonic: “STRESSED” (remember there is too much of the STRESS hormone CORTISOL)

Skin fragile

Truncal obesity with small arms

Rounded face (appears like moon), Reproductive issues amennorhea and ED in male(due to adrenal cortex’s role in secreting sex hormones)

Ecchymosis, Elevated blood pressure

Striae on the extremities and abdomen (Purplish)

Sugar extremely high (hyperglycemia)

Excessive body hair especially in women…and Hirsutism (women starting to have male characteristics), Electrolytes imbalance: hypokalemia

Dorsocervical fat pad (Buffalo hump), Depression

Causes of Cushing’s

  • Glucocorticoid drug therapy ex: Prednisone
  • Body causing it: due to tumors and cancer on the *pituitary glands or adrenal cortex, or genetic predisposition

Nursing Management for Cushing’s Syndrome

  • Prep patient for Hypophysectomy to remove the pituitary tumor
  • Prep patient for Adrenalectomy:
    • If this is done educate pt about cortisol replacement therapy after surgery
  • Risk for infection and skin breakdown
  • Monitor electrolytes blood sugar, potassium, sodium, and calcium levels

Addison’s Disease

Addison’s: Hyposecretion of Aldosterone & Cortisol (watch the video for a clever way on how to remember this and not get it confused with Cushing’s)

Signs & Symptoms of Addison’s Disease

Remember the phrase: “Low STEROID Hormones” (remember you have low production of aldosterone & cortisol which are STEROID hormones)

Sodium & Sugar low (due to low levels of cortisol which is responsible for retention sodium and increases blood glucose), Salt cravings

Tired and muscle weakness

Electrolyte imbalance of high Potassium and high Calcium

Reproductive changes…irregular menstrual cycle and ED in men

lOw blood pressure (at risk for vascular collapse)….aldosterone plays a role in regulating BP

Increased pigmentation of the skin (hyperpigmentation of the skin)

Diarrhea and nausea, Depression

Causes of Addison’s Disease

  • Autoimmune due to the adrenal cortex becoming damaged due to the body attacking itself:
    • Tuberculosis/infections
    • Cancer
    • Hemorrhaging of the adrenal cortex due to a trauma

Nursing Management of Addison’s Disease

  • Watching glucose and K+ level
  • Administer medications to replace the low hormone levels of cortisol and aldosterone
  • For replacing cortisol:
    • ex: Prednisone, Hydrocortisone
      • Education: Patient needs to report if they are having stress such as illness, surgery, or extra stress in life ( will need to increase dosage), take medication exactly as prescribed….don’t stop abruptly without consulting with MD.
  • For replacing aldosterone:
    • ex: Fludrocortisone aka Florinef
      • Education: consume enough salt..may need extra salt
  • Wearing a medical alert bracelet
  • Eat diet high in proteins and carbs, and make sure to consume enough sodium
  • Avoid illnesses, stress, strenuous exercise

Watch for Addisonian Crisis

This develops when Addison’s Disease isn’t treated.

In addisonian crisis, the patient has extremely LOW CORTISOL levels (life threatening).

Remember the 5 S’s

  1. Sudden pain in stomach, back, and legs
  2. Syncope (going unconscious)
  3. Shock
  4. Super low blood pressure
  5. Severe vomiting, diarrhea and headache
  • NEED IV Cortisol STAT:
    • Solu-Cortef and IV fluids (D5NS to keep blood sugar and sodium levels good and fluid status)
  • Watch for risk for infection, neuro status (confusion, agitation), electrolyte levels (sodium and potassium, glucose)

Addison’s vs Cushing’s Quiz

 

From http://www.registerednursern.com/addisons-disease-vs-cushings-review-notes-for-nclex/