Study Highlights Importance of Clinical Follow-Up in Cushing’s Patients After Adenoma Removal

A rare case of Cushing’s syndrome (CS) in a 17-year-old patient with multiple pituitary adenomas highlights the importance of clinical follow-up in order to determine the best treatment options for patients.

The study, “A rare case of multiple pituitary adenomas in an adolescent Cushing disease presenting as a vertebral compression fracture,” was published in the journal Annals of Pediatric Endocrinology and Metabolism

CS is a very rare disease with an incidence of 0.7-2.4 cases per million, per year. It is caused by exposure to very high levels of the hormone cortisol. In children, the most common symptom is weight gain without height gain. In some rare cases, tumors known as multiple pituitary adenomas (MPAs) appear, and patients have elevated levels of adrenocorticotropic hormone (ACTH). Surgical removal through transsphenoidal surgery (TSS) is considered the best treatment, and the first TSS has a success rate of more than 90%.

However, since 15% of patients have a recurrence, ongoing monitoring and follow-up after TSS are important. The importance of this follow-up care is highlighted in a recent case report.

The study described the case of a 17-year-old male adolescent who was 149.5 cm tall (4’9″) and weighed 63.6 kg (140 lbs). The patient was referred to a hospital for the evaluation of a vertebral compression fracture and obesity. Over four years, the patient gained 23 kg (51 lbs) without an increase in height. Despite showing many of the features of CS, this patient had not been previously diagnosed with CS.

He had high levels of ACTH and cortisol, and an MRI suggested the presence of an 8-mm (0.8 cm) micro-adenoma. After TSS, the patient’s morning ACTH and cortisol levels were reduced, and a persistent headache had improved. But there was no reduction in weight.

Three months after the TSS, the patient’s body mass index did not show improvement, and both cortisol and ACTH levels were elevated again. MRI revealed a new 9 mm (0.9 cm) micro-adenoma, which was removed with a second TSS. However, cortisol and ACHT remained elevated after the second surgery, with no evidence of a pituitary tumor in MRI scans.

Researchers recommended additional options, such as total removal of the pituitary gland, radiotherapy, or removal of both adrenal glands, options that the patient and his family declined. He continued to receive treatment for osteoporosis, hypertension, and increased lipid levels.

“In conclusion, we reported the clinical course of Cushing disease with 2 distinct pituitary adenomas. Since there is no consensus as to the best treatment for relapsing or persistent Cushing disease and since only a few cases of MPA among pediatric Cushing disease have been reported, a close followup of tumor status, severity of hypercortisolism, and patients’ perspectives are the major parameters used to determine the best treatment option for each patient. In addition, early recognition and diagnosis of pediatric Cushing disease would lead to earlier recovery, improved growth, and better quality of life,” the researchers wrote.

From https://cushingsdiseasenews.com/2017/10/27/cushings-disease-rare-case-report-highlights-importance-early-diagnosis-follow-up-care/

A 12-year-old boy died from Addison’s disease after the chance of lifesaving treatment was ‘missed’

The death of a 12-year-old boy who was suffering from undiagnosed Addison’s disease was preventable, an inquest heard.

Ryan Lee Morse had been unwell from July 2012, with his parents noticing his skin darkening and him becoming lethargic and losing weight.

His condition worsened over the following months and he died during the early hours of December 8, 2012.

During the time he was unwell, Ryan’s mother, Carol Ann Morse, took him to Abernant Surgery in Abertillery several times.

She said: “Ryan was rarely ill as a child. In June 2012, which was towards the end of Ryan’s first comprehensive school year, I noticed his skin colour changing.

“His skin seemed to be getting darker.”

She said his joint areas, including elbows and knees, were getting darker. Under his eyes, it looked as if he had not slept for a month. I don’t suppose it worried me at the time because it was gradual.”

A post mortem was held on December 12 by Dr E. J. Lazda, a consultant pathologist at University Hospital of Wales in Cardiff who concluded that Ryan died as a result of Addison’s disease.

An inquest into Ryan’s death was held at Newport Coroner’s Court on Thursday.

Dr Yvette Cloette, a consultant paediatrician since 2004, was called during the early hours of December 8, 2012, by a registrar where she was told the details of Ryan’s death.

She said: “Ryan’s parents told me he had been unwell since July.

“It was thought Ryan had been particularly unwell since the Thursday before he passed away. He had to be collected from school that day. On (the) Friday morning, she said he hallucinated. His temperature did settle that afternoon but then he had diarrhoea.

“As his mum was cleaning him, she noticed his genitalia were black.

“I then examined Ryan. At this time I formed the opinion that Ryan may have had Addison’s disease. I didn’t share this with the family at the time because I didn’t have enough evidence.

“I believe that Ryan’s death was preventable. Addison’s is a disease which, once recognised, can be treated.”

She said it was easier to put things together retrospectively, as opposed to when treating an acute illness as a GP.

David Bowen, senior coroner for Gwent, paid tribute to Ryan’s family during the hearing.

“Before summing up, I think it’s right that I pay tribute to the dignity that has been shown by Mrs Morse and her family.

“It can’t have been easy for them to rehear events that took place over five years ago.

“Please accept my belated condolences.”

Mr Bowen told the inquest that Ryan had been fit and well up until July 2012.

“However at about that time, his parents began to notice a gradual change in his skin and a fluctuation in his general health.”

He had been diagnosed with a viral infection and prescribed Paracetamol, he said.

Over the next six to eight weeks, he did not improve.

Mr Bowen said: “Consequently, his mother took him back to the doctor. The GP was more concerned about the rash, it seems to me, than any of the other symptoms.

“He prescribed tablets and cream for that condition.”

Mr Bowen said that during October and November 2012, “Ryan’s health became much more of a concern for his parents.”

He suffered from headaches, pains in his legs, and occasional episodes of projectile vomiting.

On November 7, Mrs Morse took Ryan back to the GP surgery, where she described symptoms to Dr Rudling, who took samples of blood.

On November 21, they returned to receive the blood test results.

The results revealed a “slightly lower than normal” white blood cell count. The inquest heard Ryan was told he was still suffering from a viral infection that had been diagnosed some months earlier.

Mr Bowen said: “It appears that about this time, there was an outbreak of Norovirus or vomiting and sickness in the area that may have confused the diagnosis.”

Mrs Morse said: “I’d been told to bring Ryan back in January so I thought I would just get Christmas out of the way and take him back. I’d been a carer for 9-10 years but my job didn’t give me any insight into what Ryan had.”

On November 29, 2012, Ryan returned to school, but around a week later on December 6 he was so ill that his mum had to collect him early.

The following day, on December 7, Mrs Morse rang Abernant Surgery saying she needed to speak to a doctor.

Between 8.50am and 8.55am, she received a call from Dr Lyndsey Elizabeth Thomas.

Mrs Morse said: “She asked if he’d been given Paracetamol and I explained he wouldn’t take it. She asked what his temperature was like.

“I’d said Ryan was awake (that morning) and talking rubbish.”

The inquest heard Mrs Morse was asked to take Ryan to the surgery, but she said she was unable to.

“She then told me to give Ryan some dissolvable Paracetamol and see how it goes until dinner. She said fetch him up if you need to.”

Dr Lyndsey Elizabeth Thomas said her contact with Ryan was limited to a single telephone conversation with his mother on December 7.

She said: “I considered whether Ryan needed to be seen or admitted to hospital.

“I clearly recall explaining that if she had any concerns or if Ryan’s delirium or temperature didn’t improve in two hours, he would need to be seen, I would be able to go and visit him at the end of the morning surgery if necessary.”

Mrs Morse said she later noticed that her son’s genitals were black.

She rang the surgery and was put her through to Dr Rudling.

Mrs Morse said: “She said ‘it’s all to do with his hormones’. Phone Monday and we’ll fit him in. At this point I didn’t know what to think.

“I was thinking I’ll take him in on Monday and see what they say. There was no more temperature, no more sickness and no more diarrhoea.”

The inquest heard Dr Joanne Louise Rudling, who qualified in 1993, joined Abernant in August 2011.

She said her first contact with Ryan was in November 2012.

On December 7, Dr Rudling said the receptionist took a call from Ryan’s mother while she was in reception.

Dr Rudling said: “I decided to speak to Ryan’s mother in reception there and then.

“She also asked if this could be age related, I said it could be but I would have to examine him first.

“The impression I got was Ryan was improving. His mother was concerned about the darkening of his genitalia.”

Ryan’s father said goodnight around 10.15pm and went to bed. At around 11.10pm Mrs Morse could see Ryan had fallen asleep, and went to sleep herself at around 11.30pm.

She said: “I woke up and saw it was 4.10am and then I looked at Ryan and looking at his chest could see he wasn’t breathing.

“I started to do chest compressions, dialled 999, continued chest compressions until the paramedics arrived. They took over. They told me Ryan had died.”

Mr Bowen said: “This is a rare but natural disease, one which apparently GPs will not normally encounter.

“Unfortunately, neither doctor nor parents thought it necessary to refer Ryan to hospital, where the true nature of his illness may have been diagnosed.”

Recording a narrative conclusion, Mr Bowen said Ryan died of natural causes.

He said: “The opportunity to administer life-saving treatment was missed.”

Speaking after the inquest, Ryan’s sister Christina Morse said: “First of all I would like to thank everyone involved with Ryan and Ryan’s case.

“Today, after five long years, the coroner has come to the conclusion that Ryan’s death was due to natural causes and that Ryan’s death was preventable.”

From http://www.walesonline.co.uk/news/wales-news/boy-died-addisons-disease-after-13687355

[Pseudo-Cushing’s] Michigan woman nearly dies after herbal supplement found to be laced with steroids

MADISON HEIGHTS, Mich. (WXYZ) – Since 2004, the U.S. Food and Drug Administration has received more than 26,000 reports of adverse events and complaints about dietary supplements.

Jody Higgins of Madison Heights, Michigan made one of those complaints to the FDA, after she says she found out the herbs she had been taking were making her seriously ill.

“I really thought I was going to die I was getting so sick,” Higgins said.

Back in 2015, Higgins says her legs started hurting.  She says she didn’t have great health insurance, and she was hoping for a more holistic approach, so a friend referred her to Far East Ginseng Herbs and Tea in nearby Sterling Heights.

“They suggested that I take something that was called Linsen Double Caulis. I had never heard of it before, and it appeared to have all herbs on the label,” Higgins said.

Higgins says for a while, she felt better, and when she stopped taking the Linsen Double Caulis, the leg pain returned. So, she says she kept taking it for nearly a year, even though she started noticing strange symptoms.

“Within four months I had gained 80 pounds,” she said.

She suddenly had facial hair growth, severe facial swelling, extremely swollen ankles, and had dark purple stretch marks all over her body.

“I wasn’t recognizable,” said Higgins.  “I couldn’t stand for longer than 2 minutes. I couldn’t cook. I couldn’t wash my clothing. I could barely get in the shower.”

After visiting several doctors, Higgins was eventually referred to University of Michigan Endocrinologist Dr. Ariel Barkan.

“The minute that I said I had been taking a Chinese herbal remedy, he said ‘you’ve been poisoned. I know it.’ Those were his exact words,” said Higgins.

“Her situation was pretty shaky,” Barkan said.

Barkan sent the Linsin Double Caulis herbal supplement to the Mayo Clinic for testing.

“They were loaded with Dexamethasone … [which] is a medication.  It’s a synthetic steroid, very potent, very long acting, and if we take it for quite some time, we develop what is called Cushing Syndrome,” said Dr. Barkan.

Higgins was diagnosed with Cushing Syndrome, and Barkan says she could have died if she hadn’t sought help.

“The mortality for untreated Cushing Syndrome is 50% within 5 years,” said Barkan.  “ … immunity is completely suppressed. And when you don’t have immunity, the first virus, the first germ may cause [a] fatal infection and you will die.”

Higgins says once she stopped taking the Linsen Double Caulis, the facial hair went away, but she’s still struggling with her weight. Barkan says her health should improve, although it will take time.

Both doctor and patient say they have contacted the FDA about this, and they each have a warning about taking herbal supplements.

“Please just be very cautious,” Higgins said.

“Don’t touch it. Don’t touch it, you’re playing Russian roulette,” said Barkan.

Jody Higgins says she met with an investigator from the FDA’s criminal division.

An FDA spokesperson would only say that they do not discuss possible or ongoing investigations.

The lawyer for the store where Higgins says she purchased the supplement told us the owners will not be commenting on, but the owner did say they no longer sell this product.

From http://www.fox4now.com/news/national/madison-heights-woman-herbal-supplement-caused-life-threatening-illness

Reasons You Have Flab Around Your Abdomen

Some diseases and conditions could be responsible for your abdominal fat.
Mita Majumdar | Updated: April 24, 2017 6:15 pm

Visceral fat or unhealthy belly fat that surrounds the liver and other organs in the abdomen puts you at risk for serious health problems, such as, metabolic syndrome, heart disease, and type 2 diabetes. But, what causes your pot belly or beer fat in the first place? The most obvious answers you will get is – ‘You are not exercising enough’, or, ‘you are eating too much of fatty foods or sugary foods’, or ‘you are not eating the right foods’, or ultimately, ‘It’s genetics! You got it from your parents’. All of these reasons are true, of course. However, some diseases/ disorders and conditions, too, could be responsible for your abdominal fat and these have nothing to do with not exercising or not eating right. Following are some of these disorders.

Cushing’s Syndrome

Cushing’s syndrome, also called hypercortisolism, is an endocrine disorder that occurs when your body is exposed to high cortisol levels over a long period of time. It is a treatable disorder, however, if it is chronic, the symptoms can last lifelong.

Symptoms: Symptoms vary according to the severity of the disorder. The characteristic symptoms include –

  • Fatty tissue deposits in the midsection
  • Fatty deposits in the upper back, especially between the shoulders, so that it resembles a hump
  • Puffy face
  • Violaceous stretch marks (pink or purple) on the arms, breast, stomach, and thighs that are more than 1 cm wide. [1]
  • Easy bruising
  • Fatigue
  • Hirsutism and irregularity in menstruation in women
  • Loss of libido and erectile dysfunction in men
  • Cognitive dysfunction, depression, unpredictable emotional outbursts, irritability is present in 70-85 percent of people with Cushing’s syndrome.[1]

Causes:

  • Overuse of corticosteroids
  • Overproduction of cortisol by the adrenal glands

Management:

  • Surgery is the first line of treatment for Cushing’s syndrome.
  • Medication include: [2]

a.Pituitary gland directed therapy

b.Adrenal-blocking drugs

c.Glucocorticoid receptor-antagonizing drugs

  • Pituitary radiotherapy

Addison’s disease

Addison’s disease, also called adrenal insufficiency, is a disorder where your adrenal glands produce insufficient hormones, especially, glucocorticoids including cortisol and aldosterone. It is a life-threatening disease that can affect anyone irrespective of their gender or age.

How do glucocorticoids influence abdominal fats? Glucocorticoids including cortisol convert the fats into energy in the liver. They also help your body respond to stress. When sufficient amount of glucocorticoids are not produced by the adrenal glands, the fats accumulate in the abdominal area, and you see it as flab around your middle.

Symptoms:

  • Hyperpigmentation
  • Extreme fatigue
  • Low blood sugar and low blood pressure
  • Salt craving as one of the functions of adrenal glands is to maintain the sodium-potassium balance in the body
  • Nausea, vomiting, abdominal pain
  • Weight loss but gain in abdominal fat

Causes:

  • Insufficient production of adrenal cortex hormones
  • Stopping of prescribed corticosteroids
  • Tuberculosis and other infections of adrenal glands
  • Spread of cancer to the adrenal glands

Management:

  • Oral corticosteroids or corticosteroid injections
  • Intravenous injections of hydrocortisone, saline solution, and dextrose in case of Addisonian crisis

Stress

Chronic stress is a very big cause of belly fat. When you are exposed to stress, a chain reaction starts in the body because of the dysregulation of HPA axis of the neuroendocrine system. HPA axis is a complex interaction between the hypothalamus, pituitary gland, and adrenal glands. The hypothalamus produces a corticotropin releasing hormone (CRH) and vasopressin. These together stimulate the secretion of adrenocorticotropic hormone (ACTH). ACTH is transported by the blood to the adrenal glands, which then produces corticosteroids, mainly, cortisol from cholesterol. One of the functions of cortisol is to signal the body to store fat, and specifically, the fat storage occurs in the abdominal area, where the cortisol receptors are greater. Researchers have found that stress causes hyperactivation of HPA axis, leading to accumulation of fat tissue, especially in the abdomen region.

So, the more and longer you are stressed (or if you are chronically stressed), chances are that you will be carrying more belly fat!

Ascites

Ascites is the buildup of fluid in the abdominal space. Ascites usually occurs in people with cancer, and it is then called malignant ascites. Onset of ascites is generally the terminal phase in cancer. Ascites also occurs in patients with liver cirrhosis, kidney failure, or heart disease.

Symptoms:

The first sign of ascites is an increase in abdominal girth accompanied by weight gain. [4] Although it looks like it is belly fat, it is actually the fluid that causes the bulging.

Other symptoms include:

  • Shortness of breath
  • Nausea and vomiting
  • Swelling in the feet and ankle
  • Decreased appetite, sense of fullness, bloating
  • Fatigue
  • Haemorrhoids

Management:

If the ascites is not causing any discomfort, it may not require any treatment. Treatment of ascites can have many side effects. Talk to your doctor before you go in for management/ treatment.

Abdominal hernia

Abdominal hernia is a swelling or a bulge in the abdominal area where an organ or fatty tissue pushes through a weak spot in the abdominal wall. The abdominal wall is made up of tough connective tissue and tendons that stretch from the ribs to the groin. Depending on the position of the weakness in your abdominal wall, the hernia can be inguinal (groin), femoral (upper thigh), umbilical (belly button), hiatal (upper stomach), or even incisional. Incisional hernia can occur when the intestine pushes through a weak spot at the site of abdominal surgery.

Symptoms:

  • Visible bulge that may or may not cause discomfort
  • Feeling of heaviness in the abdomen
  • Sharp pain when you strain or lift objects

Causes:

  • Constipation and diarrhoea
  • Persistent coughing and sneezing
  • Straining or suddenly lifting a heavy object

Management:

  • Umbilical hernia, common in young children, mostly resolves by itself as the abdominal muscles get stronger.
  • Other abdominal hernia normally do not resolve by themselves. Doctors suggest waiting and watching.
  • If treatment is required, surgery is the only option. Surgery involves pushing the hernia back into the abdomen and repairing the abdominal wall.

Menopause

Menopause is certainly not a disease or a disorder. It is the time in a woman’s life when she stops menstruating and cannot become pregnant because her ovaries stop producing the required amounts of hormones oestrogen and progesterone. A woman reaches menopause when she has not had her periods for 12 months.

Symptoms:

  • Hot flashes and/ or night sweats
  • Vaginal dryness
  • Mood swings
  • Sleep disturbances

It is very common to gain belly fat during menopause. This is because of the low oestrogen levels. Oestrogen seems to influence the distribution of fat in the body, in a way that the fat is redistributed from the hips, buttocks, and thighs to the belly. However, a study published in the journal Metabolism reported that though women did significantly gain belly fat, especially deep inside the belly, relative fat distribution is not significantly different after menopause. [5] But the fact remains that women do gain flab in the abdomen after menopause.

Belly fat can be seriously harmful. If your belly fat is not because of the above-mentioned conditions, you can lose it by adopting a healthy lifestyle that includes sleeping enough, exercising regularly, eating right, and reducing stress.

Reference

  1. Sharma ST, Nieman LK, Feelders RA. Cushing’s syndrome: epidemiology and developments in disease management. Clinical Epidemiology. 2015;7:281-293. doi:10.2147/CLEP.S44336.
  1. Feelders RA, Hofland LJ. Medical treatment of Cushing’s disease. J Clin Endocrinol Metab. 2013;98:425–438.
  1. Kyrou I, Chrousos GP, Tsigos C. Stress, visceral obesity, and metabolic complications. Ann N Y Acad Sci. 2006 Nov;1083:77-110.
  1. Sinicrope FA. Ascites. In: Kufe DW, Pollock RE, Weichselbaum RR, et al., editors. Holland-Frei Cancer Medicine. 6th edition. Hamilton (ON): BC Decker; 2003.
  2. Franklin RM, Ploutz-Snyder L, Kanaley JA. Longitudinal changes in abdominal fat distribution with menopause. Metabolism. 2009 Mar;58(3):311-5. doi: 10.1016/j.metabol.2008.09.030.

Adapted from http://www.thehealthsite.com/diseases-conditions/reasons-you-have-flab-around-your-abdomen-f0417/

 

Science Teacher Receives Support After Cushing’s Disease Diagnosis

I find it amazing that it’s newsworthy in this day and age for anyone receiving support after a diagnosis.  Of course, a diagnosed person should be getting support as a matter of course.  If she had cancer, everyone would be all over this.

For Kara Murrow, the most rewarding moments as a teacher come when students learn about animals in the classroom. So it’s difficult for the Bonham Elementary fifth-grade science and social studies teacher to be away from school while she prepares for surgery.

“I enjoy it, and I know my kids enjoy the class and enjoy science because of it,” Murrow said. “With the science club I do after school once a week, the kids get upset when it gets canceled because of meetings. Not having it now is upsetting, too.”

Murrow was diagnosed this month with Cushing’s disease, a condition that develops when a tumor on the pituitary gland causes it to secrete too much adrenocorticotropic hormone. Murrow, who moved to West Texas from Arizona three years ago, said she has received support from Midland ISD employees and others in the local community.

Murrow’s mother, Louise Gonzalez, also appreciates Midlanders’ concerns for her daughter.

“People in Midland have been wonderful, considering how new we are to the area,” Gonzalez said. “The school district sent out the GoFundMe page and there’s been an outpouring of support for that. People at my church always ask me.”

Murrow’s family is collecting donations from the website GoFundMe to cover the costs of medical and travel expenses. Murrow and her husband, Kai, recently spent money on hospital stays connected to their 4-year-old son’s food sensitivities.

“They’ve been paying off those bills and doing OK until this came,” Gonzalez said. “Plus, she’s been going to the doctor about this. Because Cushing’s is so rare, doctors don’t recognize it.”

Murrow was diagnosed with the disease after medical professionals discovered a tumor on her pituitary gland. For six years, she experienced symptoms — including weight gain, dizziness and headaches — but said doctors couldn’t determine the cause. Murrow was thankful when she received an answer.

“It was a huge relief to finally have a diagnosis and know that I wasn’t crazy or making things up,” Murrow said. “It’s weird to be excited about a brain tumor. It’s a relief to know what was happening and that I have a solution.”

Murrow traveled this week to Barrow Neurological Institute in Phoenix, where she’s scheduled to undergo surgery to remove the tumor. Though Murrow said recovery lasts several months, she hopes to return to the classroom next school year.

Jaime White, fourth-grade language arts and social studies teacher at Bonham, said both staff and students miss her presence. She said Murrow expresses concern for her students during her time away.

“She’s worried about how kids will do on the STAAR [State of Texas Assessments of Academic Readiness],” White said. “She doesn’t want them to think she abandoned them. The disease has to take center stage.”

At school, White said she noticed her colleague’s dedication toward helping her students understand science.

“She’s hands-on,” White said. “When it comes to science, she’s always making sure the kids are doing some sort of experiment. She wants to make sure the kids grasp it.”

Murrow teaches students about animals through dissections and presentations. Before she became a teacher nine years ago, she coordinated outreach programs at an Arizona zoo.

When she came to MISD, Murrow saw an opportunity to generate enthusiasm about science. She launched an invite-only science club for fifth-graders who show interest in the subject.

“I started it because there wasn’t really anything,” Murrow said. “They have tutorials for reading and math. There’s not a lot kids can do with science after school. They get science in the younger grades, but the focus is on reading and math. Science is something kids really enjoy.”

Though Murrow is disappointed about not being able to facilitate the club, she recognizes the importance of her upcoming surgery. She’s happy her mother, husband and two children will be in Phoenix for support.

“I hope that it will bring about a sense of relief to all the symptoms I’ve been dealing with and provide a chance for myself and my family to continue along with a full life,” Murrow said.

From http://www.mrt.com/news/local/article/Science-teacher-receives-support-after-11026581.php

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