Acromegaly: Chronic disease root of young man’s abnormally gray hair and facial changes

Posted on May 10, 2021 by MaryO

A chronic, progressive disease was discovered to be the reason why a man in his 20s experienced a sudden increase of gray hair and facial changes, local doctors said.

The man recently went to Renji Hospital to find out why the changes had taken place.

Doctors suspected he suffered from acromegaly, a chronic disease characterized by enlargement of head bones, soft parts of the feet and hands and sometimes other parts of the body due to excessive secretion of growth hormones by the pituitary gland.

Doctors conducted an MRI and found a pituitary tumor, which caused the man’s acromegaly. To their surprise, doctors also found a cyst in his brain.

Doctors conducted one successful surgery to treat both problems simultaneously.

Dr Ma Jing, director of Renji Hospital’s endocrinology department, said acromegaly is an endocrine metabolism disease that has no symptoms in its early stages, and most people are not diagnosed with it until they are 40 or 50, commonly 4.5 to 9 years after originally contracting the disease — in some extreme cases, 20 years later.

In addition to affecting the hands, feet and face, the disease can afflict the cardiovascular system, causing problems like hypertension and poor heart function. Those with the disease also have a higher risk of diabetes, thyroid and intestinal tumors, Ma said.

“Since people with acromegaly usually have abnormal endocrine function, early screening and diagnosis is important,” she said.

From https://www.shine.cn/news/metro/2105078537/

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Pituitary Tumors Affect Patients’ Ability to Work, Reduce Quality of Life

Posted on November 10, 2018 by MaryO

Pituitary tumor conditions, such as Cushing’s disease, have a substantial effect on patients’ work capabilities and health-related quality of life, researchers from The Netherlands reported.

The study, “Work disability and its determinants in patients with pituitary tumor-related disease,” was published in the journal Pituitary.

Pituitary tumors, like those that cause Cushing’s disease, have significant effects on a patient’s physical, mental, and social health, all of which influence their work status and health-related quality of life. However, the effects of the disease on work status is relatively under-investigated, investigators report.

Here, researchers evaluated the work disability among patients who were treated for pituitary tumors in an attempt to understand the impact of disease diagnosis and treatment on their social participation and ability to maintain a paying job.

In their study, researchers examined 241 patients (61% women) with a median age of 53 years. The majority (27%) had non-functioning pituitary tumors, which do not produce excess hormones, but patients with acromegaly, Cushing’s disease, prolactinomas, and Rathke’s cleft cyst also were included.

Participants were asked to complete questionnaires to evaluate their health-related quality of life and disease-specific impact on their work capabilities. Each participant completed a set of five questionnaires.

Participants also reported their hormonal status and demographic data, including gender, age, education, and marital status. Specific information, such as disease diagnosis, treatment, and tumor type was obtained from their medical records.

Work status and productivity were assessed using two surveys, the Short-Form-Health and Labour Questionnaire (SF-HLQ) and the work role functioning questionnaire 2.0 (WRFQ).

SF-HLQ was used to obtain information on the participants’ employment and their work attendance. Employment was either paid or unpaid. (Participation in household chores was considered not having a paid job.)

WRFQ is a 27-question survey that determines work disability regarding being able to meet the productivity, physical, emotional, social, and flexible demands. A higher score indicates low self-perceived work disability.

Disease-specific mood problems, social and sexual functioning issues, negative perceptions due to illness, physical and cognitive difficulties, were assessed using a 26-item survey called Leiden Bother and Needs for Support Questionnaire for pituitary patients(LBNQ-Pituitary).

Overall, 28% of patients did not have a paid job, but the rates increased to 47% among those with Cushing’s disease. Low education, hormonal deficits, and being single were identified as the most common determinants of not having a paid job among this population.

Further analysis revealed that more patients with Cushing’s disease and acromegaly had undergone radiotherapy. They also had more hormonal deficits than others with different tumor types.

Overall, patients with a paid job reported working a median of 36 hours in one week and 41% of those patients missed work an average of 27 days during the previous year. Health-related problems during work also were reported by 39% with a paid job.

Finally, health-related quality of life was determined using two questionnaires: SF-36 and EQ-5D. The physical, mental, and emotional well being was measured with SF-36, while ED-5D measured the health outcome based on the impact of pain, mobility, self-care, usual activities, discomfort, and anxiety or depression. In both SF-36 and EQ-5D, a higher score indicates a better health status.

Statistical analysis revealed that the quality of life was significantly higher in patients with a job. Overall, patients with a paid job reported better health status and higher quality of life than those without a paid job.

Although 40% of the patients reported being bothered by health-related problems in the past year, only 12% sought the help of an occupational physician, the researchers reported.

“Work disability among patients with a pituitary tumor is substantial,” investigators said.

“The determinants and difficulties at work found in this study could potentially be used for further research, and we advise healthcare professionals to take these results into consideration in the clinical guidance of patients,” they concluded.

From https://cushingsdiseasenews.com/

Filed under: Cushing's, pituitary, symptoms | Tagged: , , , , , | 2 Comments »

Sloan Kettering (New York City) Clinical Trials & Research

Posted on May 24, 2017 by MaryO

 

Clinical trials are research studies that test new treatments to see how well they work. Our Pituitary and Skull Base Tumor Center is leading clinical trials investigating new medical therapies for patients with Cushing’s disease and acromegaly. They are also involved in quality-of-life studies aimed at improving long-term follow-up care for patients who need it.

Our experts can help determine which clinical trials are right for you. The following clinical trials for pituitary tumors are currently enrolling new patients.

To learn more about a particular study, choose from the list below. For more information about our research and clinical trials, call us at 212-639-3935, or talk with your doctor.

A Phase III Study of COR-003 to Treat Cushing’s Syndrome

Eligibility
Eliza B. Geer

A Phase III Study of LCI699 (Osilodrostat) to Treat Cushing’s Disease

Eligibility
Eliza B. Geer

Filed under: adrenal, Clinical trials, Cushing's, pituitary, Treatments | Tagged: , , , , , , , , , , | Leave a comment »

Cushing’s Testing at NIH

Posted on May 21, 2017 by MaryO
Rank Status Study
1 Recruiting Study to Evaluate CORT125134 in Patients With Cushing’s Syndrome

Condition: Cushing’s Syndrome
Intervention: Drug: CORT125134
2 Recruiting Cushing’s Disease Complications

Condition: Cushing’s Disease
Intervention: Other: Exams and questionnaires
3 Recruiting The Accuracy of Late Night Urinary Free Cortisol/Creatinine and Hair Cortisol in Cushing’s Syndrome Diagnosis

Condition: Cushing Syndrome
Intervention:
4 Recruiting Treatment for Endogenous Cushing’s Syndrome

Condition: Endogenous Cushing’s Syndrome
Intervention: Drug: COR-003
5 Recruiting Saliva Cortisol Measurement as a Screening Test for Suspicious Cushings Syndrome in Children.

Condition: Cushings Syndrome
Intervention: Other: Children refered to the obesity clinic
6 Recruiting Safety and Efficacy of LCI699 for the Treatment of Patients With Cushing’s Disease

Condition: Cushing’s Disease
Intervention: Drug: LCI699
7 Recruiting Treatment of Cushing’s Disease With R-roscovitine

Condition: Cushings Disease
Intervention: Drug: R-roscovitine
8 Recruiting A Study of ATR-101 for the Treatment of Endogenous Cushing’s Syndrome

Condition: Cushing Syndrome
Interventions: Drug: ATR-101;   Drug: Placebos
9 Recruiting Evaluation of 68Ga-DOTATATE PET/CT, Octreotide and F-DOPA PET Imaging in Patients With Ectopic Cushing Syndrome

Condition: Cushing Syndrome
Interventions: Drug: F-DOPA PET Scan;   Drug: Mifepristone;   Drug: Ga-DOTATATE;   Drug: Octreoscan;   Other: CT, MRI
10 Not yet recruiting Endocrine Cardiomyopathy in Cushing Syndrome: Response to Cyclic GMP PDE5 inhibitOrs

Condition: Cushing’s Syndrome Cardiomyopathy
Intervention: Drug: Tadalafil
11 Recruiting Long-term Beneficial Metabolic Effects of Adrenalectomy in Subclinical Cushing’s Syndrome of Adrenal Incidentaloma

Condition: Cushing Syndrome
Intervention: Procedure: surgery
12 Recruiting Long Term Safety and Efficacy of Pasireotide s.c. in Patients With Cushing’s Disease

Condition: Cushings Disease
Intervention: Drug: SOM230
13 Recruiting New Imaging Techniques in the Evaluation of Patients With Ectopic Cushing Syndrome

Condition: Cushing Syndrome
Interventions: Drug: Pentetreotide;   Drug: 18-F-fluorodeoxyglucose;   Drug: (18F)-L-3,4-dihydroxyophenylalanine (18F-DOPA)
14 Not yet recruiting Targeting Iatrogenic Cushing’s Syndrome With 11β-hydroxysteroid Dehydrogenase Type 1 Inhibition

Condition: Iatrogenic Cushing’s Disease
Interventions: Drug: AZD4017 and prednisolone;   Drug: Placebo Oral Tablet and prednisolone
15 Not yet recruiting Assessment of Persistent Cognitive Impairment After Cure of Cushing’s Disease

Condition: Cushing’s Disease
Intervention: Device: Virtual radial task in 3D
16 Recruiting Biomarker Expression in Patients With ACTH-Dependent Cushing’s Syndrome Before and After Surgery

Condition: Cushing’s Syndrome
Intervention:
17 Recruiting Efficacy and Safety Evaluation of Osilodrostat in Cushing’s Disease

Condition: Cushing’s Disease
Interventions: Drug: osilodrostat;   Drug: osilodrostat Placebo
18 Recruiting Effects of Metyrapone in Patients With Endogenous Cushing’s Syndrome

Condition: Cushing’s Syndrome
Intervention: Drug: metyrapone
19 Recruiting Adrenal Venous Sampling in Patients With Overt or Subclinical Cushings Syndrome, and Bilateral Adrenal Tumors

Condition: Cushing Syndrome
Intervention: Radiation: Adrenal venous sampling
20 Recruiting Glycemic Fluctuations in Newly Diagnosed Growth Hormone-Secreting Pituitary Adenoma and Cushing Syndrome Subjects

Condition: Pituitary Adenoma
Intervention: Device: continuous glucose monitoring
Rank Status Study
21 Recruiting Targeted Therapy With Gefitinib in Patients With USP8-mutated Cushing’s Disease

Conditions: Cushing’s Disease;   Corticotrophin Adenoma
Intervention: Drug: Gefitinib
22 Recruiting Cardiac Steatosis in Cushing’s Syndrome

Conditions: Endocrine System Disease;   Cardiovascular Imaging
Intervention: Other: 1H magnetic resonance spectroscopy and CMRI
23 Recruiting Study of Management of Pasireotide-induced Hyperglycemia in Adult Patients With Cushing’s Disease or Acromegaly

Conditions: Cushing’s Disease;   Acromegaly
Interventions: Drug: Pasireotide s.c.;   Drug: Sitagliptin;   Drug: Liraglutide;   Drug: Insulin;   Drug: Pasireotide LAR;   Drug: Metformin
24 Recruiting Study of Efficacy and Safety of Osilodrostat in Cushing’s Syndrome

Conditions: Cushing’s Syndrome;   Ectopic Corticotropin Syndrome;   Adrenal Adenoma;   Adrenal Carcinoma;   AIMAH;   PPNAD
Intervention: Drug: Osilodrostat
25 Recruiting Effects of Hormone Stimulation on Brain Scans for Cushing s Disease

Condition: Pituitary Neoplasm
Intervention: Drug: Acthrel
26 Recruiting Does Serum-DXM Increase Diagnostic Accuracy of the Overnight DXM Suppression Test in the Work-up of Cushing’s Syndrome?

Conditions: Cushing’s Syndrome;   Adrenal Incidentalomas;   Alcoholism;   Obesity
Intervention:
27 Recruiting Adrenalectomy Versus Follow-up in Patients With Subclinical Cushings Syndrome

Condition: Adrenal Tumour With Mild Hypercortisolism
Intervention: Procedure: Adrenalectomy
28 Recruiting Study of Adrenalectomy Versus Observation for Subclinical Hypercortisolism

Conditions: Hypercortisolism;   Cushing Syndrome
Interventions: Procedure: Adrenalectomy;   Other: Observation
29 Not yet recruiting Dynamic Hormone Diagnostics in Endocrine Disease

Conditions: Adrenal Insufficiency;   Congenital Adrenal Hyperplasia;   Cushing Syndrome;   Growth Hormone Deficiency;   Acromegaly;   Primary Hyperaldosteronism
Intervention: Other: 27 hour subcutaneous fluid sampling
30 Recruiting An Investigation of Pituitary Tumors and Related Hypothalmic Disorders

Conditions: Abnormalities;   Craniopharyngioma;   Cushing’s Syndrome;   Endocrine Disease;   Pituitary Neoplasm
Intervention:
31 Recruiting Ga-68-DOTATOC -PET in the Management of Pituitary Tumours

Condition: Pituitary Tumours
Intervention: Procedure: Gallium-68 DOTATOC PET
32 Recruiting Efficacy of Mifepristone in Males With Type 2 Diabetes Mellitus

Conditions: Type 2 Diabetes Mellitus;   Insulin Resistance
Interventions: Drug: Mifepristone 600 mg daily;   Drug: Placebo
33 Recruiting Targeted Therapy With Lapatinib in Patients With Recurrent Pituitary Tumors Resistant to Standard Therapy

Conditions: Pituitary Adenomas;   Prolactinomas
Intervention: Drug: Lapatinib
34 Recruiting Mutations of Glucocorticoid Receptor in Bilateral Adrenal Hyperplasia

Condition: General Glucocorticoid Resistance
Intervention: Genetic: blood collection for mutation characterization
35 Recruiting Defining the Genetic Basis for the Development of Primary Pigmented Nodular Adrenocortical Disease (PPNAD) and the Carney Complex

Conditions: Cushing’s Syndrome;   Hereditary Neoplastic Syndrome;   Lentigo;   Neoplasm;   Testicular Neoplasm
Intervention:
36 Not yet recruiting Reduction by Pasireotide of the Effluent Volume in High-output Enterostomy in Patients Refractory to Usual Medical Treatment

Condition: Enterostomy
Interventions: Drug: Pasireotide;   Drug: Placebo
37 Recruiting Mifepristone for Breast Cancer Patients With Higher Levels of Progesterone Receptor Isoform A Than Isoform B.

Condition: Breast Cancer
Intervention: Drug: Mifepristone
38 Recruiting SOM230 Ectopic ACTH-producing Tumors

Condition: Ectopic ACTH Syndrome
Intervention: Drug: Pasireotide
39 Recruiting Decreasing Rates of Intraurethral Catheterization Postoperatively in Spine Surgery

Condition: Post-operative Urinary Retention
Interventions: Drug: Tamsulosin;   Drug: Placebo
40 Recruiting Adrenal Tumors – Pathogenesis and Therapy

Conditions: Adrenal Tumors;   Adrenocortical Carcinoma;   Cushing Syndrome;   Conn Syndrome;   Pheochromocytoma
Intervention:

Filed under: adrenal, Clinical trials, Cushing's, Diagnostic Testing, pituitary, Rare Diseases, Treatments | Tagged: , , , , , , , , , , , , , , , , , , , , , , , , | Leave a comment »

Into the brain through the nostrils (Sri Lanka)

Posted on December 21, 2016 by MaryO

There is purposeful concentration, while soft and soothing music plays in the background, as different teams of doctors and nurses, scrub up and move around Operating Theatre (OT) C of the Anuradhapura Teaching Hospital.

On the operating table is a pretty middle-aged woman, but something seems unusual with her face – it seems unduly large, with the nose being pronounced.

While Consultant Anaesthetist Dr. Ayesha Abeyratne and her team are preparing the patient for surgery, two Consultants from different specialties are studying the Magnetic Resonance Imaging pictures. They are Consultant Ear, Nose and Throat (ENT) Surgeon, Dr. Daminda Dumingoarachchi who is also a Head and Neck Surgeon and Consultant Neurosurgeon, Dr. Stravinsky Perera. Although not present in the OT that day, Consultant ENT Surgeon Dr. Upul Abeysundara is also very much a part of this pioneering work in Sri Lanka.

It is a Monday morning and we too are in OT clothes and masks privy to viewing an operation not done anywhere else in the state sector, except the Anuradhapura Hospital.

Accessing the patient’s brain through the nostrils is what the ENT and Neuro Teams will do in one of many trailblazing procedures, while the Anaesthetic Team will ensure that her vitals are stable and the able theatre nursing staff will play their part.

Image-guided (navigation-assisted) endoscopic trans-nasal trans-sphenoidal pituitary surgery is what we are about to view being performed in the country only since October this year. For, the 38-year-old patient lying inert on the operating table has a tumour just above her pituitary gland, between the optic nerves and the carotid arteries, making it a difficult operation-site to access. (The pea-size pituitary gland is dubbed the ‘master gland’ as it produces many hormones for the whole body, while also stimulating other glands to produce other hormones.)

Dr. Dumingoarachchi points out that the ENT navigator which the Anuradhapura Hospital was provided with recently, is like the GPS system of the brain which shows this vital organ’s architecture.

Dr. Perera explains that the patient has acromegaly, the abnormal production of growth hormones from the pituitary, causing a large nose and big hands etc. This is while it also causes an abnormal reduction in other hormones, affecting the functioning of the whole body. The enlarged pituitary is also compressing the main artery of the brain. A major issue is that the patient’s vision is being affected and if the tumour is not decompressed, she would go blind.

Dr. Daminda Dumingoarachchi

Matters also seem complicated as the patient had undergone ‘conventional’ brain surgery to remove an earlier tumour in the same area in 2009 in another hospital, but some parts had been left behind because it was complex surgery and the slightest slip of the scalpel could cause serious harm.

We watch in fascination as the surgical teams discuss how the tumour has grown around the right carotid artery making it a challenging procedure and is pressing down (compressing) on the brain.

This pituitary macroadenoma with acute intra-tumoural haemorrhage (bleeding) is “big” — 5X3.8X3.5cms, says Dr. Perera, adding that laterally, the lesion extends to the right cavernous sinus encircling almost the full circumference of the cavernous part of the right carotid artery. The left carotid artery is spared.

Super-imposing the patient’s Computed Tomography (CT) scan with images of the real anatomy, the ‘registration’ takes place for this ‘re-do’ surgery, as he explains that the anatomy is distorted after her previous surgery done seven years ago.

In the freezing OT, we don’t feel the time passing as both the Neurosurgeon and the ENT Surgeon attend to their tasks, two pairs of hands working in tandem, not looking down at the patient but up at the two screens, while navigating the pathways through the nostrils to the brain.

Usually, such a large tumour above the pituitary gland will entail open-skull surgery, with the brain having to be retracted (drawn away from the site of the operation) to gain access to the tumour, we learn. While the dangers of such surgery are numerous, the patient also has to spend a long time in the Intensive Care Unit (ICU) in the recovery phase.

With the frontiers of medicine pushed back more and more, Dr. Dumingoarachchi says that now skull-base structures can be approached through the nose, ear and throat. “Such surgery involves dealing with pathologies located on the under-surface of the brain, with Neurosurgeons and ENT Surgeons together accessing the skull-base more easily through the nose, ear and throat.”

Pointing out that these trans-nasal and trans-labyrinthine approaches to the skull-base will help bring down the rate of revision neurosurgeries and cut down the time that the patient will have to spend in the ICU post-operatively, he adds that the patients can go back home sooner without complications and be productive more quickly. Currently, some patients even go to other countries to get these services.

With the Health Ministry providing a high definition 3-chip endoscopic camera system, an anterior skull-base set, a micro-debrider and an ENT navigator, vast strides have been made at the Anuradhapura Hospital. They include:

Dr. Stravinsky Perera

* Anterior skull-base surgeries — Endoscopic anterior skull-base surgeries, starting with pituitary surgeries using the endoscopic trans-sphenoidal approach for the first time in Sri Lanka. The latest addition, in October this year, is image-guidance (navigation).

The other procedures include cranio-pharyngioma, olfactory neuroblastoma, cerebrospinal fluid leak repairs, para-nasal sinus cancer resections and odontoid (cervical spine) decompression.

The advantages of this approach include a better field of view and ease of doing revision surgery if required, while leaving no external scars.

Nasal polyposis is also now being managed by image-guided powered full house functional endoscopic sinus surgeries, resulting in the reduction of the recurrence rate. This also cuts down revision surgeries.

*  Lateral skull-base surgeries – Through microscopic lateral skull-base surgery, trans-labyrinthine acoustic neuromas have been removed. With direct access to these eighth-nerve tumours, no brain retraction has been necessitated, resulting in less post-operative morbidity. The whole tumour can be removed while preserving the seventh cranial nerve, as the hospital has also been given intra-operative cranial nerve monitoring facilities.

Paying tribute to Dr. Palitha Mahipala, Director-General of Health Services, for making the navigator available to them, Dr. Dumingoarachchi also expresses appreciation to the Deputy Director-General (DDG) of the Biomedical Division, Muditha Jayathilake, Biomedical Engineers Apsara and Kumuduni and DDG Dr. Lakshmi Somatunga for all their support.

From http://www.sundaytimes.lk/161218/news/into-the-brain-through-the-nostrils-221019.html

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