Cushing syndrome has an incidence of only two in a million, and experts recommend multidisciplinary combination of diagnosis and treatment

People's Daily health client reporter Zhao Weide

"Cushing syndrome is a rare syndrome with typical clinical manifestations of centripetal obesity, hypertension, abnormal hypokalemia of glucose metabolism and osteoporosis caused by hypercorticemia, with an annual incidence of about two parts per million, and its diagnosis and treatment requires a multidisciplinary combination of endocrinology, imaging, neurosurgery, urology and so on." Bian Liuguan, director and chief expert of the Department of Neurosurgery at Ruijin Hospital affiliated to Shanghai Jiao Tong University School of Medicine, told the People's Daily health client reporter.

Cushing syndrome has complex causes and is highly harmful

Ms. Zhou is the first patient with Cushing syndrome diagnosed in the country, and is also a diabetic patient, 70 years old this year, who only began treatment three years after the onset of the disease, but has already produced serious complications such as osteoporosis, affecting early treatment.

Ms. Zhou told reporters that she felt the abnormality of the body in 1999. "At that time, I found that my face was big and round, my face was red, and my weight began to rise, from a hundred and a few pounds to one hundred and fifty or sixty pounds in a short period of time, so I went to the hospital for diagnosis and treatment, and finally the expert diagnosed Cushing syndrome, but when I got the diagnosis results, I had been ill for three years."

Bian Liuguan is operating on patients. Courtesy of respondents

Ms. Zhou said that the disease causes various complications, accompanied by high blood pressure, high blood sugar, etc., as well as osteoporosis. "When I was younger, I was 1.62 meters tall, and because of the osteoporosis caused by the disease, I am currently only 1.44 meters tall." After being diagnosed at Ruijin Hospital, Ms. Zhou underwent three surgeries and combined with medication in the following years, and her body appearance has basically returned to normal, but osteoporosis caused by the disease has caused irreversible damage.

"Cushing's syndrome is caused mainly by pituitary adenomas that secrete adrenocorticotropic hormone, tumors in other areas, and increased cortisol due to adrenal tumors." Bian Liuguan introduced that some tumor substances secreted by individual lung cancer patients can also lead to an increase in cortisol, causing hormone secretion disorders to lead to the onset of disease.

Bian Liuguan told reporters that the main clinical manifestations of Cushing syndrome are obesity, large trunk and stomach, and small limb muscles; the second manifestation is that the back fat pad is thicker, medically known as the buffalo back; the third is that the face is particularly ruddy, manifested as a multi-blood face (full moon face); women also show an increase in hair such as beard.

"Obesity caused by the disease leads to a large increase in abdominal fat, and excessive glucocorticoids denature and break the elastic fibers of the dermis layer of the skin, resulting in stretch marks-like symptoms." Bian Liuguan said that Cushing syndrome is difficult to detect in the early stage, because symptoms such as obesity are easy to be ignored, and will be gradually valued after the complications of the disease appear, so many patients have been delayed by 10 to 15 years when they are diagnosed.

"Although Cushing syndrome is rare, its condition is complex. In addition to directly affecting the balance of metabolism of various substances such as sugar, fat, egg autoplasm, water electrolyte, etc., it will also affect the function of multiple system organs throughout the body, so that the body's immunity will decrease. If not diagnosed and treated in time, the prognosis is poor, severe hypokalemia, severe infection and cardiovascular and cerebrovascular complications can endanger patients' lives, and the mortality rate of patients with Cushing syndrome is 4 times higher than that of normal people. Bian Liugui said.

Experts recommend early screening and multidisciplinary joint diagnosis and treatment

Bian liuguan suggested that the following groups of people should be screened for Cushing syndrome: young patients with osteoporosis, hypertension and other clinical manifestations that are not appropriate for age; Patients with clinical manifestations of Cushing's syndrome and progressive exacerbations, especially in patients with typical symptoms such as myopathy, polycythemia, purple streaks, ecchymosis, and thinning of the skin; obese children with weight gain and decreased height percentile, growth arrest, etc.

"At present, most prefecture-level and municipal hospitals in China can do a good job of Kushing syndrome disease examination, and it is recommended that people with similar symptoms can go to the endocrinology department of the hospital to do screening, early detection, and early treatment." Bian Liuguan told reporters that the early clinical manifestations of Cushing syndrome are not typical, resulting in patients being easily ignored, delaying the best treatment time, and it is very necessary to do a good job of early screening, if the diagnosis is confirmed, it is recommended to at least go to the endocrinology department of a provincial medical institution.

Bian Liuguan introduced that the treatment of Cushing syndrome is a multidisciplinary joint diagnosis and treatment process, which requires the joint efforts of multiple disciplines, including neurosurgery, endocrinology, imaging, urology, etc. The Department of Endocrinology undertakes endocrine evaluation, combined with imaging to determine the cause of Cushing's syndrome and the location of the tumor. A neurosurgery for Cushing syndrome (Cushing's disease) caused by the pituitary gland, which removes pituitary foci according to endocrine evaluation and protects the pituitary gland from its normal function as an important endocrine organ. The main purpose of imaging is to find and localize pituitary adenomas.

"Then there is the postoperative follow-up, and the follow-up of the six months after the operation is the most critical." Bian Liuguan introduced that the postoperative follow-up of patients with Cushing syndrome is lifelong, and it is necessary to follow up during one month, three months and six months after surgery, and the six months after surgery is the most critical, during which the patient has undergone great changes, and various symptoms such as muscle soreness and facial molting may occur. Then, according to the development of the disease, the next one or two years to the hospital review, at any time to pay attention to the change of the disease, because even if the disease is better controlled, but there is still a 10% to 15% chance of recurrence.

"At present, more than 90% of the patients with Cushing syndrome (pituitary source) treated in our hospital have been controlled by the disease, ranking among the top in the country, and taking the 2020 admission situation as an example, the condition of all cases has been alleviated." Bian Liuguan introduced.

"Early detection and early treatment of Cushing syndrome is still very effective, even after diagnosis, do not be afraid, relying on the current medical means more than 90% can be better controlled, many of the patients we receive have also returned to society and returned to the family." Bian Liuguan said.