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

Posted on May 6, 2016 by MaryO

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

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Recruitment for Cushing’s Syndrome Clinical Study

Posted on May 2, 2016 by MaryO
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

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Addison’s Disease vs Cushing’s Syndrome Nursing

Posted on May 2, 2016 by MaryO

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/

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Why Was This Woman Gaining Weight Despite Her Diet?

Posted on May 2, 2016 by MaryO

“I just can’t seem to lose weight,” the 59-year-old woman said quietly. She had tried everything, she told the young doctor, who was training to be an endocrinologist at Mount Sinai Hospital in New York City. Weight Watchers. Exercise. She ate more vegetables, less fat, then fewer carbs. But still she was gaining weight, 30 pounds during the past seven months, including 12 in the past two weeks. She had never been skinny, she continued, but shapely. In her mid-40s, she started gaining weight, slowly at first, then rapidly. She was considering bariatric surgery, but she wanted to make sure she wasn’t missing something obvious. She had low thyroid hormones and had to take medication. Could her thyroid be off again?

The doctor asked her about symptoms associated with a low thyroid-hormone level. Fatigue? Yes, she was always tired. Changes in her hair or skin? No. Constipation? No. Do you get cold easier? Never. Indeed, these days she usually felt hot and sweaty.

It was probably not the thyroid, the doctor said. She asked if the woman had any other medical problems. She had high blood pressure and high cholesterol — both well controlled with medications. She also had obstructive sleep apnea, a disorder in which the soft tissue at the back of the throat collapse during sleep, cutting off air flow and waking the person many times throughout the night. She had a machine that helped keep her airway open, and she used it every night. She also had back pain, knee pain and carpal-tunnel syndrome. The pain was so bad that she had to retire from her job years before she was ready.

Big, Bigger, Biggest

The doctor examined her, then went to get Dr. Donald Smith, an endocrinologist and director of lipids and metabolism at Mount Sinai’s cardiovascular institute. After hearing a summary of the case, Smith asked the patient if she had anything to add. She did: She didn’t understand why she was getting so much bigger. Her legs were huge. She used to have nice ankles, but now you could hardly see them. Her doctor had given her a diuretic, but it hadn’t done a thing. Everything was large — her feet, her hands, even her face seemed somehow bigger. She hardly recognized the woman in the mirror. Her doctors just encouraged her to keep trying to lose weight.

Worth a Thousand Words

“Let me show you a picture,” she said suddenly and reached over to her purse. The patient’s sister had made a comment recently that led the patient to wonder whether the changes she saw in the mirror were more than simple aging. The patient pulled out a photograph of an attractive middle-aged woman and handed it to Smith. That was me eight years ago, she told him. Looking at the two faces, it was hard to believe they belonged to the same woman. Smith suspected this was something more than the extra pounds.

Two possibilities came to mind. Each was a disease of hormonal excess; each caused rapid weight gain. The first was Cushing’s disease, caused by overproduction of one of the fight-or-flight hormones, cortisol. The doctor looked at the patient, seeking clues. On her upper back, just below her neck, the woman had a subtle area of enlargement. This discrete accumulation of fat, called a buffalo hump, can occur with normal weight gain but is frequently seen in patients with Cushing’s. Do you bruise more easily these days? he asked. Cushing’s makes the skin fragile. No, she said. Did she have stretch marks on her stomach from the weight gain? The rapid expansion of the abdomen can cause the fragile skin to develop dark purple stretch lines. No. So maybe it wasn’t Cushing’s.

Find out the answer at http://www.nytimes.com/interactive/2016/04/17/magazine/17mag-diagnosis.html#/#7

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

Posted on May 1, 2016 by MaryO

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

View larger version:

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

  1. ↵
    1. Ross EJ,
    2. Linch DC

    . Cushing’s syndrome-killing disease: discriminatory value of signs and symptoms aiding early diagnosis.Lancet 1982;2:646–9. doi:10.1016/S0140-6736(82)92749-0

    [CrossRef][Medline][Web of Science]
  2. ↵
    1. Sippel RS,
    2. Elaraj DM,
    3. Kebebew E, et al

    . Waiting for change: symptom resolution after adrenalectomy for Cushing’s syndrome. Surgery2008;144:1054–61. doi:10.1016/j.surg.2008.08.024

    [CrossRef][Medline][Web of Science]
  3. ↵
    1. Mishra AK,
    2. Agarwal A,
    3. Gupta S, et al

    . Outcome of adrenalectomy for Cushing’s syndrome: experience from a tertiary care center. World J Surg2007;31:1425–32. doi:10.1007/s00268-007-9067-6

    [CrossRef][Medline]

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

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