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

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/

Resolution of the physical features of Cushing’s syndrome in a patient with a cortisol secreting adrenocortical adenoma after unilateral adrenalectomy

A 37-year-old woman developed clinical manifestations of Cushing’s syndrome over a span of 2 years. Physical examination revealed features that best describe Cushing’s syndrome, such as wide purple striae (>1 cm) over the abdomen, facial plethora and easy bruisability.1  Other features observed were hypertension, moon facies, acne, a dorsocervical fat pad, central obesity and dyslipidaemia.

The diagnosis of hypercortisolism was confirmed using a 1 mg overnight dexamethasone suppression test (19.7 ng/dL, N: <1.8) and 24 h urine free cortisol (185.9 μg/24 h, N: 3.5–45). A suppressed adrenocorticotropic hormone (ACTH) level (4 pg/mL, N: 5–20) and a lack of hyperpigmentation suggested ACTH-independent Cushing’s syndrome. Further work up using CT with contrast of the adrenals showed a 2.4×2.3×2.4 cm right adrenal mass. The patient then underwent laparoscopic adrenalectomy of the right adrenal gland. Steroids was started postoperatively and tapered over time. Histopathology results were consistent with an adrenocortical adenoma (2.5 cm widest dimension). Six months after surgery, there was resolution of the physical features, weight loss and improvement in blood pressure.

Figure 1 is a serial photograph of the physical features seen in Cushing’s syndrome, such as moon facies, a dorsocervical fat pad and wide purple striae, taken preoperatively, and at 3 and 6 months after surgery. With treatment, physical and biochemical changes of Cushing’s syndrome both resolve through time.2 The time course of the resolution of these changes, however, is varied.2 ,3 We observed that the physical features were ameliorated at 3 months and resolved at 6 months.

Learning points

  • Physicians as well as patients should be aware that improvement of the features of Cushing’s syndrome after treatment does not occur immediately.

  • Dramatic resolution of the physical features of Cushing’s syndrome, however, can be observed as early as 6 months after surgery.

Figure 1

Physical features of Cushing’s syndrome (top to bottom: moon facies, a dorsocervical fat pad and wide purple striae (>1 cm) over the abdomen) documented before surgery, and at 3 and 6 months after surgery.

Footnotes

  • Twitter Follow John Paul Quisumbing at @jpquisumbingmd

  • Contributors JPMQ worked up the case and wrote the case report. MASS reviewed the case report and critically appraised it. JPMQ incorporated his suggestions.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

References

From http://casereports.bmj.com/content/2016/bcr-2016-215693.short?rss=1

A Single-Center 10-Year Experience with Pasireotide in Cushing’s Disease: Patients’ Characteristics and Outcome

Pasireotide is the first pituitary-directed drug approved for treating patients with Cushing’s disease (CD). Our 10-year experience with pasireotide in CD is reported here.

Twenty patients with de novo, persistent, or recurrent CD after pituitary surgery were treated with pasireotide from December 2003 to December 2014. Twelve patients were treated with pasireotide in randomized trials and 8 patients with pasireotide sc (Signifor®; Novartis AG, Basel, Switzerland) in clinical practice. The mean treatment duration was 20.5 months (median 9 months; range, 3-72 months).

Urinary free cortisol (UFC) levels mean percentage change (± SD) at last follow-up was-40.4% (± 35.1; range, 2-92%; median reduction 33.3%) with a normalization rate of 50% (10/20). Ten patients achieved sustained normalized late night salivary cortisol (LNSC) levels during treatment. LNSC normalization was associated with UFC normalization in 7/10 patients. Serum cortisol and plasma ACTH significantly decreased from baseline to last follow-up. Body weight decrease and blood pressure improvement during pasireotide treatment were independent from UFC response. Glucose profile worsening was observed in all patients except one. The frequency of diabetes mellitus increased from 40% (8/20) at baseline to 85% (17/20) at last follow-up requiring initiation of medical treatment only in 44% of patients.

Pasireotide treatment was associated with sustained biochemical and clinical benefit in about 60% of CD patients. Glucose profile alteration is a frequent complication of pasireotide treatment; however, it seems to be easy to manage with diet and lifestyle intervention in almost half of the patients.

From http://www.ncbi.nlm.nih.gov/pubmed/27127913