Gardner's syndrome symptoms. Diseases. Gardnerellosis of the male urinary tract

Among the skin manifestations of the pathological process, atheromas are most common,. The disease was named after the discoverer, doctor E. Gardner.

Causes of Gardner's Syndrome

Cause of Gardner's Syndrome is a hereditary predisposition that is inherited in an autosomal dominant manner. Thus, the patient inherits gene mutations from parents, which leads to the formation of adenomatous polyps in the thin and. That is why the disease is also called familial adenomatous polyposis.

Symptoms of Gardner's Syndrome

Main symptoms of Gardner's syndrome due to the appearance of adenomatous polyps in the intestine. More often pathological process localized in the large intestine, but in some cases polyps can also be found in the duodenum. Most often, neoplasms appear during puberty. By the age of 25-30, the risk of malignancy of adenomatous polyps increases significantly. The risk of passing mutant genes from parent to child is about 50%.

With a small size of neoplasms, the patient may have nothing to worry about. In more severe cases, there is intestinal bleeding, concomitant iron deficiency anemia, obstructive intestinal obstruction. Skin manifestations of Gardner's syndrome include atheromas, fibromas, osteomas, and lipomas. These neoplasms are benign tumors localized in soft tissues. Most often they are tumor-like formations that can cause some pain in case of compression of nerve fibers. Also for this pathological condition is characterized by early loss of teeth.

In more than 90% of cases, adenomatous polyps in patients with Gardner's syndrome become malignant. That is why early detection of signs of the development of a malignant process is so important. At the same time, attention is drawn to the appearance of disorders of the stool, a violation of the shape of the stool and the act of defecation. In some cases, patients may experience general symptoms as weakness, fatigue, loss of appetite and body weight. The appearance of such pathological signs should be alarming and make you see a doctor.

Diagnosis of Gardner's syndrome in Israel

If a patient is suspected of having Gardner's syndrome, the following diagnostic tests are performed:

• blood test- allows you to identify signs iron deficiency anemia, exclude other diseases;
• fecal analysis- necessary to determine occult bleeding;
• - this endoscopic examination allows you to examine a large part of the colon. When it is carried out, polyps may be detected, indicating the presence of the disease;
• biopsy- performed during a colonoscopy. In this case, tissue samples of the polyp are taken with a special device. When studying the material in the laboratory, a conclusion can be made about the nature of the pathological process;
• irrigoscopy This is an X-ray examination in which a contrast agent is injected into the gastrointestinal tract. When taking a series of images, you can evaluate the distribution of contrast in the intestines and visualize polyps;
• X-ray and scintigraphy of bones of the skeleton- aimed at detecting tumors bone tissue. Most often, pathological neoplasms in Gardner's syndrome are localized in the region of the upper and lower jaws.

Treatment of Gardner's syndrome in Israel

The most effective and radical way to treat Gardner's syndrome is endoscopic polypectomy. When carrying out such an intervention, violation of the integrity of the skin is not required. All manipulations are carried out endoscopically, by introducing instruments into the anus. The use of general anesthesia can reduce discomfort from the procedure to a minimum. using an endoscopic device, the surgeon examines the inner surface of the mucous membrane of the colon. The device is equipped with a video camera, so everything that happens inside can be seen on the monitor screen. After detection of polyps, they are easily removed using a special loop and removed.

However, endoscopic polypectomy may not be suitable for all patients. If malignant cells are detected based on the results of a biopsy, abdominal surgery is indicated. In this case, a part of the intestine is resected, followed by anastomosis. This allows you to most radically eliminate the focus of the pathological process and prevent recurrence. In some cases, a total colectomy is indicated. This operation is aimed at complete removal large intestine. During its implementation, a significant part of the intestine is resected, which subsequently requires the imposition of an ileostomy or ileorectal anastomosis.

If the patient refuses radical surgery, endoscopic polypectomy can be performed. If malignant cells are not detected during the histological examination of the removed neoplasms, the patient is shown dynamic monitoring with regular colonoscopy. With the development of such complications as intestinal bleeding and obstructive intestinal obstruction, emergency surgical interventions are performed.

For patients with Gardner's syndrome, medical genetic counseling is important to reduce the risk of research into the disease in the unborn child. Modern technologies in vitro fertilization are aimed at selecting the most complete embryos without genetic disorders.

With competent and timely treatment, the prognosis for patients with Gardner's syndrome is favorable.

Gardner's syndrome: what does the diagnosis mean and how does it manifest itself?

The normal microflora of the vagina is one of the main factors of nonspecific resistance. Normally, the vagina of a healthy woman is a reservoir containing both aerobic and anaerobic microflora.

However, the quantity of the former is ten times greater than the quantity of the latter. Its main representatives are lactobacilli, which produce hydrogen peroxide and lactic acid, which prevents the growth of opportunistic bacteria.

However, some endogenous and exogenous factors can provoke the development of an inflammatory syndrome due to vaginal imbalance.

A condition in which lactic acid bacteria are completely replaced by facultative anaerobes, which are present only in small quantities in the vaginal secretion of a healthy woman, in clinical practice called bacterial vaginosis or gardnerellosis. This is a fairly common pathology, which is diagnosed in 30-35% of patients with mixed urogenital infections.

The causative agent of gardnerellosis

Vaginal gardnerella (Gardnerella vaginalis), which provokes the development of gardnerellosis, was first isolated in patients suffering from vaginitis in 1955 by Duxon and Gardner, who called it Haemophilus influenzae vaginalis.

This microorganism is a non-encapsulated polymorphic nuclear-free gram-variable bacillus (or coccobacillus), reaching a length of 2 μm and 0.7-0.9 μm in diameter. This bacterium can be presented in the form of cocci or small rods, covered with the thinnest cell wall and the outer microcapsular layer.

It should be noted that some gardnerella are characterized by a membrane structure of the cell wall, as in gram-negative bacteria, while others have a homogeneous structure, as in gram-positive microorganisms.

On open surfaces, the pathogen can remain viable for 3-6 hours (in the layer of mucous secretions), and in inactive forms (in the form of spores) - up to a year. Therefore, there is a risk of infection with garnerellosis in public toilets and in stagnant water bodies.

Note: Gardnerella is an opportunistic pathogen that can be present in most healthy women without showing pathogenic properties.

Disease prevalence

According to WHO statistics, gardnerellosis is one of the most common sexually transmitted diseases, which most often affects women of reproductive age.

In men, this disease is much less common.

This pathology is detected in 30-80% of patients suffering from various inflammations of the genital organs, and it is also detected during preventive examinations in a quarter of healthy women.

Risk factors, sources of infection and ways of transmission of gardnerellosis

According to experts, the development of gardnerellosis is directly dependent on the nature of sexual activity. This pathology is never detected in healthy virgins, as well as in women who do not suffer from vaginitis. Therefore, the predisposing factors include:

• promiscuous sex life;
• previous infections;
• menstrual irregularities;
• use of intrauterine contraceptives;
• long-term use antibacterial drugs and antidepressants, leading to an imbalance of the vaginal microflora;
• the use of contraceptives that violate the hormonal background;
• use of chlorine-containing antiseptics when douching;
• stressful situations;
• prolonged wearing of tight-fitting synthetic underwear;
• the absence in the diet of fermented milk products, which are a source of lactobacilli.

It should be emphasized that most often gardnerellosis occurs against the background of other urogenital infections (trichomoniasis, gonorrhea, chlamydia, ureaplasmosis, etc.).

The main route of transmission is sexual. However, the infection can be transmitted from an infected mother to her child when passing through infected birth canal or due to intrauterine infection.

Pathogenesis of gardnerellosis

The virulence of this microorganism is relatively low, and only in some strains of gardnerella the end product of fermentation is acetic acid and amino acids.

Others produce lactic and formic acid in the course of their life.

During sexual contact with a woman suffering from gardnerellosis (or a bacteriocarrier), the pathogen enters the male urethral epithelium, and subsequently, during the next sexual contact, infects or reinfects the female genital tract.

Attached to the surface of the stratified squamous epithelium, gardnerella are able to form characteristic formations, which in clinical practice are called "key cells". It is their detection in the course of bacterioscopic examination of smears from the genital organs that has an important diagnostic value.

Unfortunately, to date, the pathogenesis this disease is not entirely clear. However, scientists have found that gardnerella damages the epithelial cells of the vagina, and also causes autolysis (dissolution) of phagocytes.

Clinical forms of gardnerellosis

To describe the clinical signs of urogenital pathologies provoked by anaerobic microorganisms, scientists have developed a special classification that includes the following forms of gardnerellosis:

1. bacterial vaginosis;
2. Bacterial lesions of the upper genital tract;
3. Gardnerellosis of the female urinary tract;
4. Gardnerellosis of the male urinary tract;
5. Gardnerellez pregnant.

Bacterial vaginosis

This, according to experts, is the most common clinical form diseases. Typically, during diagnostic study in the discharge from the genital organs, along with gardnerella, other representatives of the anaerobic microflora (leptostreptococci, mobiluncus, bacteroids, etc.) are also found, but at the same time, there are no sexually transmitted pathogens in it. In 50% of patients with bacterial vaginosis, the main symptoms are discharge with an unpleasant smell of rotten fish. In the other half of patients with this form of pathology, objective and subjective symptoms are not detected.

An unpleasant odor, according to experts, occurs due to the formation of biogenic amines during the metabolism of gardnerella and other non-sporogenic anaerobes. Putrescine and cadaverine (pathological amines), presented in the form of non-volatile salts, in the process of alkalization become rapidly evaporating substances exuding a strong fishy smell.

Gardnerellosis of the upper genital tract

With this form of pathology, infection can occur directly through the periuterine tissue, as well as lymphogenous or hematogenous.

There are cases when this form of gardnerellosis became the cause of the development of endometritis and salpingo-oophoritis, and some authors describe cases of blood infection with gardnerella (gardnerella septicemia), leading to severe endotoxic shock and postoperative complications in gynecological patients.

Note: this is why all women preparing for surgery should be screened for bacterial vaginosis without fail.

Gardnerellosis of the urinary tract in women

The development of this pathology is explained by the anatomical proximity of the vagina and urethra, as a result of which the pathogen is transferred from the genital organs to the urinary tract.

There is evidence that gardnerella is very often isolated from the urine of practically healthy women. However, garnerellosis can only be diagnosed if there are more than 1000 CFU of bacteria in one milliliter of urine taken with a catheter.

Gardnerellosis of the male urinary tract

In men, this disease is not as common as in women. Often, when gardnerella is found in combination with other bacteroids, the anterior urethra becomes inflamed in the stronger sex. In this case, there is a sluggish course of urethritis, with mild clinical symptoms.

Patients complain of the occurrence of meager muco-serous discharge, without pain and discomfort. Complications of gardnerellosis in men are very rare in clinical practice. There are isolated reports of the development of gardnerella prostatitis, cystitis, epididymitis and pyelonephritis.

With manifest forms of the disease, the features of the clinical course, as a rule, are determined by precisely those pathogenic microorganisms with which the causative agent of gardnerellosis comes into association.

Note: Men who suffer from an asymptomatic (or asymptomatic) form of the disease are often sources of infection for their sexual partners.

Gardnerellosis pregnant

In gynecological practice, this form of pathology occurs in almost 20% of pregnant women. But fortunately, an intrauterine infection develops very rarely, since active defense mechanisms increase during this period. However, some authors note the possibility of moderate suppression of cellular immunity. Very often, bacterial vaginosis causes various violations during pregnancy, as well as the disease can provoke the development of postpartum complications.

Women diagnosed with Gardnerellosis are twice as likely to experience premature birth, and in a tenth of women in childbirth, gardnerella and other microorganisms are found in the amniotic fluid, although normally it should be sterile.

There is also evidence that bacterial vaginosis may be associated with chorionamnionitis (a purulent-septic complication that also leads to premature birth), and, according to some experts, gardnerella can provoke postpartum or post-abortion sepsis.

Symptoms of gardnerellosis in women

With the development of the pathological process, patients experience abundant, unpleasantly smelling foamy vaginal discharge, and also, during special laboratory tests, a decrease in the acidity of the vaginal environment is detected. Often, women complain of severe itching and burning in the area of ​​​​the external genitalia and perineum.

However, patients have hyperemia of the mucous membrane of the vagina, urethra and cervix. With fresh acute gardnerellosis, vaginal discharge is very abundant, thick and homogeneous, white-gray in color, and discharge from the urethra and cervical canal is rather scarce.

In the case when the uterine cavity and fallopian tubes are involved in the pathological process, salpingitis is diagnosed.

Symptoms of gardnerellosis in men

In the representatives of the stronger sex, this disease often occurs exclusively in the form of inflammation of the external genital organs.

As a rule, the causative agent of gardnerellosis is removed from the urinary tract after two or three days (in their healthy state).

However, with hypothermia, reduced immunity, or in the presence of concomitant pathologies, gardnerella becomes aggressive, begins to multiply and leads to inflammation of the urethral mucosa.

Sometimes the disease proceeds in a latent form, without showing itself in any way, and, in the absence of complaints, the man becomes a carrier.

Symptoms of gardnerellosis in men are completely similar to the symptoms of urethritis. With inflammation of the mucous membrane of the urethra in the urethra, there is a burning sensation, discomfort during urination, and muco-serous discharge also appears.

Diagnosis of gardnerellosis

As a rule, when making a diagnosis, complaints from patients, examination data and laboratory results are taken into account.

As a material for research, discharge from the urethra, cervical canal, walls and vaults of the vagina, as well as urine is used.

In the course of bacterioscopy in smears of patients in the absence of leukocytes, key cells are detected (cells of a squamous epithelium, practically "covered" with gram-variable coccobacteria). There is also a decrease in the acidity of the vaginal environment (pH> 4.5), the result of the amine test is positive, lactic acid bacteria are completely absent, and the number of anaerobes exceeds the number of aerobes.

Note: gardnerellosis often develops in those patients who were previously prescribed antibacterial treatment for colpitis. In this case, it is antibiotics that aggravate the course of the pathological process.

At the present stage, along with bacterioscopic studies, microbiological methods, DNA hybridization, RIF (immunofluorescence reaction) and PCR are used to detect gardnerellosis.

Treatment of gardnerellosis

Treatment of gardnerellosis is carried out in a complex aimed at restoring the normal microflora of the vagina and creating an optimal physiological environment.

Local treatment, as a rule, should be carried out for at least ten days.

It involves the treatment of the vagina with a solution of miramistin or metronidazole, and patients are prescribed douching with soda solution and chamomile baths.

Without fail, the treatment process shows the use of drugs that stimulate the immune forces of the body and vitamin complexes.

In systemic therapy, specialized drugs are prescribed, in which active active substance is metronidazole or clindamycin. Also, experts recommend the use of biogenic stimulants and medications, normalizing the microbiocenosis of the intestine and vagina.

In the acute form of the pathological process, patients are prescribed a two-week antibiotic therapy. However, sometimes at least two, and sometimes three, courses of treatment are required for a complete cure.

The main problem that arises in the treatment of gardnerellosis is that the causative agent of this disease is a conditionally pathogenic microorganism, and therefore, living in the body of an absolutely healthy woman, under the influence of adverse factors, it becomes aggressive and begins to multiply uncontrollably.

Gardnerella is a microorganism resistant to the action of tetracyclines and macrolides. Therefore, before starting treatment, you should go through all necessary examinations and to pass bakposev on sensitivity to antibacterial preparations.

However, it must be understood that in order to achieve a lasting effect, they need to be taken for a very long time (6-12 months).

As biogenic stimulants that contribute to the normalization of the vaginal and intestinal microflora in folk medicine use lemongrass, aralia and leuzea safrolovidny.

Prevention of gardnerellosis

Women suffering from bacterial vaginosis should pay attention to the condition immune system, since it is the decrease in immunity that entails a violation of the microflora of the vagina and leads to hormonal disorders.

The main ways to prevent gardnerellosis include a healthy lifestyle, proper nutrition, normal sleep, regular walks in the fresh air. It is very important to observe the hygiene of the external genital organs and prevent hypothermia.

In case of accidental sexual intercourse, one should not forget about the means personal protection, and also it will not be superfluous to treat the external genitalia with special antiseptics for two hours.

In order to prevent gardnerellosis, almost all experts recommend abandoning daily douching, since such procedures can not only provoke the development allergic reactions, but also irritate the vaginal mucosa, washing away the natural lubrication and changing the acidity, which ultimately leads to the inhibition of the natural microflora.

In order for lactic acid bacteria to be in sufficient quantity in the vagina, a variety of vegetables and fruits, sauerkraut, biokefirs and yogurts should be present in the diet.

It is imperative to pay attention to the state of the intestinal microflora. In the event of a violation, it is necessary to conduct a special course of treatment, since opportunistic microorganisms that live in the intestine easily overcome the thin wall and penetrate the vagina. According to experts, the treatment of gardnerellosis in patients with intestinal dysbacteriosis is the most difficult.

With adequate timely treatment, after two or four weeks, the vaginal microflora is completely restored. Otherwise, the pathological process can provoke inflammation of the appendages, the development of various complications during pregnancy, premature birth and female infertility.

Bernard Horner Syndrome: Treatment, Causes and Symptoms

Horner's syndrome is a pathological condition caused by damage to the sympathetic nervous system.

The disease is characterized by sweating disorders, ophthalmic problems and decreased vascular tone. The pathology is characterized by a unilateral course - it affects one side of the face.

And, in addition to the appearance of a cosmetic defect, Horner's syndrome causes a number of negative consequences that are difficult to treat.

What is Horner's Syndrome?

Horner's syndrome can occur in both men and women and is very difficult to treat.

Oculosympathetic syndrome, or Horner's syndrome, is a secondary pathology, the development of which occurs against the background of other ailments. The disease was discovered by Johann Friedrich Horner, a German ophthalmologist who first described it in 1869.

The acquired form of the disease is most often associated with the use of a large amount of anesthetic when performing a blockade of the brachial plexus.

Appearance this syndrome may indicate severe pathologies chest or neck. For example, the symptoms of this pathology are often present in patients with oncological processes in the lungs. The disease is equally common among men and women. Horner's syndrome also occurs in newborns.

Causes

In modern medicine, it is customary to distinguish between two forms of this disease:

1. Congenital.
2. Acquired.

Congenital Horner's syndrome mostly develops as a result of birth trauma. There are also known cases of hereditary transmission of the syndrome in an autosomal dominant manner.

Acquired syndrome can develop for a variety of reasons:

1. Postponed injuries. The disease often occurs as a result of blunt trauma to the base of the neck, as a result of which the cervical or thoracic sympathetic nerve circuit is affected. As a rule, signs of the disease appear on the side that is opposite to the damaged area.
2. Complications resulting from surgery. The development of Horner's syndrome often occurs against the background of errors made during the installation of drainage according to Bulau or during the blockade of the cervical plexus.
3. Neurological disorders (eg. multiple sclerosis or Dejerine-Klumpke palsy).
4. Diseases of the thyroid gland. Hyperplasia thyroid gland s often becomes a provocateur of the development of the above syndrome in a patient. At risk are people who have serious disorders in the thyroid gland.
5. Oncology. Due to the fact that the tumor compresses the nerve fibers, clinical symptoms of this disease may appear. Often, lung carcinoma leads to the occurrence of this disease.
6. Vascular pathologies. In this case, the root cause of the development of Horner's syndrome is an aortic aneurysm. Changes are most often local in nature and relate to those arteries through which blood enters the zone of development of the pathological process.
7. Diseases of the hearing organs. If the treatment of otitis media, labyrinthitis was untimely or incomplete, then the infectious process can spread to neighboring tissues. The pathogenic microflora that has accumulated in the region of the middle ear begins to spread to neighboring organs.

Symptoms

Pathology is characterized by a unilateral course. Signs of Horner's syndrome can be seen with the naked eye - the patient has a slight rise in the lower eyelid or, on the contrary, drooping of the upper eyelid. It is with these symptoms that most patients go to the doctor.

Other symptoms of Horner's syndrome are:

1. Anisocoria (different sizes of pupils - on the affected side, the pupil narrows).
2. Hyperemia of the skin and increased sweating on the affected side of the face.
3. Enophthalmos (retraction of the eye) is slightly expressed.
4. There is a narrowing of the palpebral fissure. At the same time, the eyeball itself looks like it is planted deep into the orbit.
5. The patient complains of difficulty in the release of tears. In very rare cases, the affected eye may appear wet.
6. Due to persistent miosis, the patient begins to see worse at dusk. We are talking about the deterioration of dark adaptation, when the overall visual acuity is maintained.

If the disease develops in childhood, then one of the most obvious symptoms is a lighter shade of the iris of the affected eye.

Are complications possible?

If this disease is left untreated or treated untimely, then the patient may experience a number of dangerous complications. The most common side effects are:

1. Inflammation of the anterior segment of the eye. These diseases include blepharitis, keratitis and conjunctivitis.
2. With retraction of the eye, inflammation of the orbit is possible due to the penetration of infectious agents.
3. topography change eyeball in relation to the lacrimal gland, it contributes to the development of its dysfunction, which creates favorable ground for the occurrence of xerophthalmia.
4. Advanced cases of the disease contribute to the appearance of a subperiosteal abscess of the orbit. Phlegmon formation may also occur.
5. In the predominant part of patients against the background of the development of Bernard-Horner syndrome, secondary hemeralopia occurs (deterioration of visual acuity in low light), which cannot be treated by traditional methods.

Diagnostics

Before referring the patient to diagnostic procedures, the doctor performs a visual examination. As a rule, at this stage, heterochromia and miosis (narrowing of the pupil) are detected. Also on the affected side of the face there is a retraction of the eyeball. The reaction of the pupils to light may be very weak or not be determined at all.

If the above symptoms are detected, the doctor directs the patient to undergo diagnostics:

1. Biomicroscopy of the eye. The procedure allows you to determine the state of the vessels of the conjunctiva, the degree of transparency of the optical media of the eye and the structure of the iris.
2. Oxamphetamine test. Allows you to identify true reason miosis development.
3. A test to determine the degree of pupillary delay. With Horner's syndrome, it is observed as a prolapse upper eyelid and pupillary constriction.
4. Orbital CT. This study allows to establish the etiology of the disease. CT scan is needed to identify volumetric formations present in the orbit, as well as the presence of injuries.

In addition to the methods described above, it is possible to carry out differential diagnosis. It is carried out when Petit's syndrome and anisocoria are detected. If the patient has the last symptom, further examination is mandatory. Different sizes of pupils can be observed with pathologies of eye development or problems associated with cerebral circulation.

Petit's disease is characterized by a combination of exophthalmos, mydriasis, ophthalmohypertension; sometimes it is called reverse oculosympathetic syndrome.

Treatment

Horner's syndrome is very difficult to treat. Therapy involves the use of such methods:

1. Neurostimulation is the effect of low-amplitude electrical impulses on those muscles that have been affected as a result of the progression of the disease. Thus, trophism improves, and the muscles partially or fully restore their original functions.
2. If a disease occurs due to hormonal imbalance, it is necessary to carry out replacement therapy using hormonal drugs.
3. If the previous methods were ineffective, the patient is scheduled for surgery. Modern plastic surgery allows for effective correction of enophthalmos and ptosis.

At the moment, one of the most promising methods for the treatment of Horner's syndrome is kinesiotherapy. This technique involves the implementation of special massage procedures. They allow you to effectively stimulate the affected areas.

Thus, if you suspect the development of Bernard-Horner syndrome, you should consult a doctor as soon as possible. The sooner treatment begins, the more likely the patient will be to recover.

What is Gardner Syndrome?

There are many pathologies that are inherited and laid down at the genetic level. However, some of them do not appear immediately, but only in adulthood. Among such diseases is Gardner's syndrome. This pathology is benign neoplasms, sometimes tends to become malignant, that is, it turns into cancer.

Description of Gardner's syndrome

This pathology has become known relatively recently. It was first described in the middle of the 20th century by the scientist Gardner. It was he who established the connection between benign formations of the skin, bones and digestive tract. Another name for the disease is familial (or hereditary) adenomatous polyposis.

Gardner's syndrome is terrible not only with numerous cosmetic skin defects. It is believed that colon polyposis becomes malignant in 90-95% of cases. For this reason, pathology is referred to as obligate precancerous conditions.

Differential diagnosis is carried out with atheromas, Ricklenhausen's syndrome, single osteomas and intestinal polyps.

Causes of the disease and the mechanism of development

Gardner's syndrome refers to hereditary pathologies. It is transmitted at the genetic level from parents and other family members (grandparents). The mode of inheritance of this disease is autosomal dominant. This means a high probability of transmission of pathology from parents to children.

The mechanism of development of Gardner's syndrome is based on mesenchymal dysplasia. Since this tissue is formed skin covering, bones and mucous membrane of the gastrointestinal tract, there is a clinical picture characteristic of this disease.

In addition to hereditary predisposition, mesenchymal dysplasia can form under the influence of harmful factors that affect intrauterine development in the first trimester of pregnancy.

Gardner's syndrome: symptoms of the disease

Most often, the disease manifests itself in adolescence (from 10 years). In some cases, the first symptoms begin later - already in adulthood. Gardner's syndrome has the following manifestations: these are neoplasms of the skin, soft tissues, bones and the digestive system. In addition to the intestines, polyps can appear in the stomach and duodenum.

Atheromas, dermoid and sebaceous cysts, fibromas can be observed on the skin. It is also possible the appearance of benign formations of soft tissues. These include lipomas and leiomyomas. All of these neoplasms can occur on the face, scalp, arms or legs. In addition, there are bone lesions. They also belong to benign formations, but often impede functions.

For example, osteomas of the bones of the lower jaw, skull. These growths interfere with chewing, can put pressure on brain structures. The most formidable manifestation of the disease is polyposis of the intestine and other organs of the gastrointestinal tract. In most cases, neoplasms of the mucous membrane of the digestive tract are malignant, that is, they turn into cancer. Polyps may not make themselves felt for a long time.

Most often, patients complain of complications: intestinal obstruction, bleeding.

Treatment of Gardner Syndrome in Cuba: Benefits

Since the disease belongs to obligate precancers, surgical removal of neoplasms is necessary. It is very important to diagnose Gardner's syndrome in a timely manner. Photos of patients can be seen in the literature on oncology or on special sites. Apart from clinical manifestations it is necessary to conduct x-ray of the digestive tract, colonoscopy.

Skin lesions and intestinal polyposis allow for a correct diagnosis. Gardner's syndrome is treated in many countries. The advantages of Cuban clinics include the latest equipment, the cost of surgical interventions, and highly qualified specialists from all over the world. Treatment consists of removing the affected part of the intestine.

Gardner syndrome

Gardner syndrome- a hereditary disease accompanied by polyposis of the large intestine in combination with benign neoplasia of the skin, bones and soft tissues. May be asymptomatic for a long time. Bloating, rumbling and stool disorders are possible.

In some cases, intestinal polyposis in Gardner's syndrome is complicated by bleeding or intestinal obstruction. There is a high risk of developing colorectal cancer. The disease is diagnosed on the basis of complaints, family history, examination data, radiography, CT, MRI, ultrasound, endoscopy and other studies. Treatment - endoscopic polypectomy or resection of the affected parts of the intestine.

Gardner's syndrome is a rare genetically determined pathology in which diffuse polyposis of the large intestine is observed in combination with benign tumors of bones and soft tissues (osteomas, fibromas, neurofibromas, epithelial cysts and other neoplasias).

Polyposis in Gardner's syndrome mainly affects the rectum and sigmoid colon, but polyps can be detected in other parts of the intestine. The syndrome was first described by the American physician and geneticist E. J. Gardner in 1951.

Since then, more than one hundred cases of this disease have appeared in the specialized literature.

Gardner's syndrome is transmitted in an autosomal dominant manner. The severity of intestinal and extraintestinal clinical manifestations can vary greatly. The first symptoms of Gardner's syndrome usually appear in children older than 10 years. Perhaps a late onset with the formation of the first tumors over the age of 20 years.

In some cases, along with polyposis of the large intestine, osteomas and soft tissue neoplasms, polyps are found in patients with Gardner's syndrome. small intestine, stomach and duodenum. The risk of malignancy of colon polyps with the development of colorectal cancer during life is about 95%.

Treatment is carried out by specialists in the field of proctology, gastroenterology, oncology, orthopedics, dentistry and maxillofacial surgery.

Symptoms of Gardner's Syndrome

Gardner's syndrome includes a characteristic triad: diffuse polyposis of the lower parts of the large intestine, osteomas of flat and tubular bones, various benign tumors of the skin and soft tissues.

With a moderate number and small size of polyps, intestinal manifestations of Gardner's syndrome may be absent or mild.

In adolescence or youth, patients usually first turn to doctors in connection with the appearance of benign bone and soft tissue tumors.

Osteomas in Gardner's syndrome can be localized both in flat and in tubular bones. Often there is a lesion of the bones of the facial skull, accompanied by disfigurement. There may be displacement and even loss of teeth.

Some time after the appearance of the growth of osteomas in patients with Gardner's syndrome, the tumors do not become malignant. Soft tissue neoplasias are very diverse. Especially often lipomas, dermatofibromas, neurofibromas and epithelial cysts are detected. Atheromas, leiomyomas and other neoplasms are less common.

Soft tissue tumors in Gardner's syndrome also proceed benignly, malignancy is absent.

Colon polyps in Gardner's syndrome often become an accidental finding during gastrointestinal studies for other reasons or are detected during an extended examination prescribed in connection with the appearance of multiple soft tissue and bone neoplasia.

During Gardner's syndrome, three stages of intestinal damage can be distinguished. At the first stage, the disease is asymptomatic. On the second, patients note abdominal discomfort, bloating, rumbling, and periodic stool disturbances.

IN feces impurities of blood and mucus may be detected.

At the third stage in patients with Gardner's syndrome, pronounced pain syndrome, constant flatulence, abundant impurities of mucus and blood in the stool, weight loss, fatigue, emotional lability, electrolyte and protein metabolism disorders.

Many patients with Gardner's syndrome develop anemia due to small but frequently recurring bleeding from the lower GI tract.

In some cases, patients develop emergency conditions requiring emergency medical care- profuse intestinal bleeding or intestinal obstruction.

Diagnosis of Gardner's syndrome

The diagnosis is established on the basis of a family history (the presence of Gardner's syndrome in close relatives), a clinical picture that includes a characteristic triad, and data from additional studies.

When conducting a physical examination, the doctor notes the presence of multiple bone and soft tissue tumors of various localization. Some patients with Gardner's syndrome have facial deformities caused by osteomas of the facial skull.

On palpation of the bones of the trunk and extremities, tumor-like formations of bone density can be detected. With lesions mild degree the number of neoplasias can be insignificant, which makes diagnosis difficult.

On palpation of the abdomen, there is pain in the left iliac region. In the first stage of intestinal damage this symptom may be missing. When conducting a digital rectal examination on the rectal mucosa of patients with Gardner's syndrome, multiple nodes are found.

On contrast x-rays, such nodes appear as filling defects. With nodes of a small size (less than 1 cm), the information content of a contrast X-ray study is reduced. During sigmoidoscopy, polyps are detected in the rectum and colon.

The number of polyps can vary greatly.

Some patients with Gardner's syndrome have limited lesions of certain parts of the intestine. Unlike radiography, endoscopic examination makes it possible to diagnose polyps of any size, including small ones (with a diameter of 1-2 mm).

To clarify the nature and prevalence of bone tumors in Gardner's syndrome, x-rays are performed. With soft tissue neoplasms, CT, MRI or ultrasound of the affected area is prescribed.

If necessary, perform a biopsy of polyps, osteomas and soft tissue neoplasms.

Differential diagnosis of Gardner's syndrome is carried out by proctologists and gastroenterologists with the usual multiple polyps and other forms of familial polyposis.

Different variants of hereditary polyposis are characterized by certain differences in the predominant localization of polyps (damage to the entire large intestine, damage to the distal colon), the nature of pathological changes in bones and soft tissues.

To clarify these differences before setting final diagnosis conduct a detailed external examination, perform irrigoscopy and colonoscopy.

Treatment and prognosis for Gardner's syndrome

Treatment of Gardner's syndrome is only surgical. Since there is no risk of malignancy of bone and soft tissue neoplasia, the decision to perform surgical interventions is made in the presence of a cosmetic or functional defect.

Polyposis of the large intestine in Gardner's syndrome is considered as an obligate precancer, so many doctors consider it appropriate to perform the operation before the onset of signs of malignancy.

With a small number of polyps, endoscopic polypectomy is possible.

In Gardner's syndrome with severe diffuse polyposis, resection of the affected area of ​​the intestine or total colectomy is indicated with the imposition of an ileostomy or the formation of an ileorectal anastomosis (in the absence of rectal polyps).

In such cases, dynamic observation is shown with a colonoscopy every 6-8 months.

Some physicians are advocates of expectant management and believe that colectomy for Gardner's syndrome should be performed only when signs of malignancy appear or if there is frequent bleeding with the development of anemia.

Indications for emergency surgical intervention in Gardner's syndrome are profuse intestinal bleeding and intestinal obstruction. With timely adequate treatment, the prognosis for this disease is quite favorable. The severity of the course is determined by the severity of polyposis and the localization of extraintestinal tumors.

Parents with relatives with Gardner's syndrome are advised to seek medical genetic counseling during pregnancy planning.

Adenomatous polyposis of the colon: Gardner, Pates Eghers and Turcot syndrome

A variety of lesions of the colon include adenomatous polyposis. This disease is considered hereditary, but recently doctors have increasingly begun to pay attention to the homogeneity of eating behavior in different families. There is a theory that familial adenomatous polyposis is the result of generic eating habits. As a rule, colon polyposis is observed in persons who eat a large number of starch and exclude vegetable fiber. They have chronic constipation.

Familial adenomatous polyposis is the most common form of adenomatous polyposis syndrome. Hereditary adenomatous polyposis occurs with a frequency of 1 in 7500 people and in 90% of cases affects people under 50 years of age.

Alleged Causes

The disease is inherited in an autosomal dominant manner. When carrying a pathological genome, the probability of developing the disease is 80-100%.

The mutant gene is located on chromosome 5; its normal counterpart encodes the synthesis of a protein that blocks β-catenin and thus suppresses tumor growth.

The mutant gene disrupts the synthesis of this protein (either it is not formed at all or its inactive form is synthesized), as a result of which β-catenin is not inhibited, activates a number of genes and triggers the growth of adenoma.

Colon damage

In hereditary adenomatous polyposis after puberty, most often between the ages of 20 and 30, the patient develops hundreds (and sometimes thousands) of adenomatous colon polyps.

The result of the development of NAP, leading to the death of the patient, often becomes colon cancer (a malignant neoplasm is usually diagnosed 10-15 years after the first polyps are detected).

There are other lesions of the colon.

Stomach and duodenum

In 60-90% of patients with hereditary adenomatous polyposis, duodenal adenomas develop, and in 5-12% of cases - periampullary cancer or duodenal cancer.

Patients who have already undergone prophylactic colonectomy or are over 20 years of age should undergo endoscopic examination of the duodenum and periampullary region every 1-3 years.

Gastric polyposis in hereditary adenomatous polyposis is quite common, but cases of gastric cancer are rare.

Other localizations

Hereditary adenomatous polyposis can also be accompanied by the development of pancreatic cancer, hepatoblastoma, diffuse mesenteric fibromatosis (desmoid tumors), and even thyroid and brain cancer.

Symptoms of adenomatous polyposis

The patient may experience bleeding in the rectum and diarrhea. These are the main symptoms of adenomatous polyposis. The basis for the diagnosis of hereditary adenomatous polyposis is a thorough determination of the number of cases of such diseases in the patient's family.

However, in 20% of patients, no cases are detected among family members (the development of the disease is explained by a primary mutation in the germ cells of the patient's parents), and in such cases, the diagnosis of hereditary adenomatous polyposis is based on the detection of hundreds of adenomatous polyps during colonoscopy or sigmoidoscopy. mucous membrane large intestine.

Most polyps are less than 1 cm in size and are histologically identical to polyps found in normal polyposis.

Treatment of adenomatous polyposis

Taking into account high risk malignancy of polyps in post-pubertal patients, prophylactic colonectomy is indicated.

With colonectomy with the creation of an ileorectal anastomosis, the ability to voluntarily defecate is preserved, but the risk of developing cancer in the remaining area of ​​the rectum increases (up to 15% of cases).

Therefore, a complete colonectomy is performed with the formation of an ileoanal anastomosis or ileostomy. This is the only treatment for adenomatous polyposis.

Other forms of adenomatous polyposis syndrome

Juvenile polyposis. Juvenile polyps usually consist largely of lamina propria and dilated cystic glands, with minimal involvement of mucosal epithelium. Histologically, polyps are hamartomas.

The disease is acquired in nature, for the first time it is usually detected at the age of 1 to 7 years. Polyps of this type are often single and vary greatly in size (from 3 mm to 2 cm). The usual place of localization of polyps is the rectum. Juvenile polyps are not malignant.

However, their surgical excision is indicated, since such polyps are well supplied with blood and often become a source of bleeding.

Peutz-Jeghers Syndrome

Peutz-Jeghers syndrome is a combination of pigmentation of the mucous membranes and skin with polyposis of the stomach and small intestine, which has a family predisposition.

Polyps are characterized by the presence of glandular epithelium, which is supported by a smooth muscle structure that passes into the muscular layer of the mucous membrane.

Polyps are multiple, can be located throughout the gastrointestinal tract, but they are mainly found in small intestine. The syndrome is inherited in an autosomal dominant manner and is associated with a mutation in the serine-threonine kinase gene located on chromosome 19.

In this disease, a characteristic pigmentation of the mucous membranes and skin, resembling freckles, is noted. The disease is characterized by a high risk of cancer (90% at age 65), and an increased risk of breast, uterine and testicular cancer.

Gardner syndrome

Gardner's syndrome is a hereditary disease that manifests itself in the form of polyposis in the gastrointestinal tract, a variety of benign soft tissue tumors (for example, epidermoid cysts, fibromas, lipomas), malocclusion and hypertrophy pigment epithelium retina. The development of the syndrome is associated with a mutation in the APC gene.

Turcot syndrome

Turcot syndrome is a syndrome characterized by a combination of familial colon polyposis with primary tumors of the central nervous system (usually the brain). In this syndrome, colon polyps are rarely found before the onset of puberty, but by the age of 25, all patients already have them.

Without surgical treatment in almost all cases, colon cancer develops by the age of 40.

There are suggestions that the disease is caused by abnormal proliferation of intestinal epithelial cells and is accompanied by impaired DNA repair. All patients underwent colonectomy.

gardner syndrome

© Edward Czerny

Vanderbilt University, USA, Tennessee, Nashville

G Gardner's syndrome is a rare disease with an incidence of approximately 1 in 22,000. Most cases result from autosomal dominant inheritance due to mutations in the APC (Adenomous Polyposis Coli) gene. However, about 20% of cases occur spontaneously. Clinically, lesions can vary in size and pigmentation, but many have a fishtail appearance. In one histological examination revealed more widespread changes than expected clinical picture, and in one case, hamartoma-like changes were detected in this study. While the lesions of congenital pigment epithelial hypertrophy in Gardner's syndrome are histologically similar to isolated congenital pigment epithelial hypertrophy lesions and clustered pigment epithelial nevi ("bear marks"), both of the latter conditions are benign and have no association with polyps or cancer. large intestine.

G Key words: Gardner's syndrome; "fish tail"; "bear footprints"; congenital hypertrophy of the pigment epithelium.

UDC 617.735 SRNTI 76.29.56 VAK 14.01.07

Gardner syndrome (Gardner) is a dominantly inherited adenomatous intestinal polyposis with a predominant lesion of the colon. It was first described in 1950 as a description of the defeat of one family, all of whose members were descendants of the same married couple. There was no family history of cancer on the father's side. The mother (proband) died of colon cancer and 9 of her 45 offspring in two different generations also died of colorectal cancer. Shortly after this publication, a combination of other pathological conditions with this syndrome was discovered, for example, osteomas of the bones of the skull, fibromas, lipomas and cysts. sebaceous glands. Later, a connection was also revealed between Gardner's syndrome and desmoid tumors and anomalies in the structure of the teeth.

Polyps are usually detected in the second half of the second decade of life, usually they become malignant approximately 15 years after diagnosis. The risk of developing a malignant neoplasm of the intestine is almost 100%, while 50% of patients develop colon cancer by the age of 35. Other malignant neoplasms may include adrenal, thyroid, and Bladder. Turcot syndrome develops in patients who also have neuroepithelial tumors.

The ocular manifestations of Gardner's syndrome were first described by Blair and Trempe in 1980. The authors examined nine family members with Gardner's syndrome. Three family members out of 9, mother

and her two daughters, had hypertrophy of the pigment epithelium. All of them had colon polyps. One patient had five lesions in the right eye and 12 in the left. The lesions were oval or round in shape, their size was about 2 disc diameters. optic nerve. The second patient had four lesions in one eye and one in the other eye. The mother of both of these patients had four lesions in the right eye and six in the left. In all patients, the foci were randomly located on the fundus without forming any clusters.

While there are no retinal changes in a number of families with Gardner's syndrome, the detection of three or more foci of congenital hypertrophy of the pigment epithelium indicates a high probability of intestinal polyposis. Therefore, in those families. in which there is a history of polyposis, careful examination of the retina and identification of typical foci of congenital pigment epithelial hypertrophy may help identify patients at high risk of the disease. However, the absence of eye damage does not mean that the patient will not develop intestinal polyposis. Foci in the fundus can have different sizes, shapes and pigmentation; however, many lesions have a fishtail appearance (Figures 1 and 2).

Shields et al. studied the data of 132 patients with a diagnosis of congenital hypertrophy of the pigment epithelium, while identifying both isolated foci and grouped with severe pigmentation (“bear tracks”). One patient had intestinal polyposis, without malignancy. Of the 2,000 blood relatives of these patients, only 20 had polyposis or colon cancer. had

Rice. 1, 2. This 13-year-old girl has multiple lesions in both eyes. Her grandfather and mother both had colon cancer. The patient herself did not have polyposis at the time of the examination, but she needs close observation

polyposis or colonic cancer. This 1% incidence was significantly lower than expected if these lesions were associated with familial adenomatous polyposis within Gardner's syndrome.

Traboulsi and Murphy examined the eyes of patients with Gardner's syndrome histologically. Most of the foci histological structure were similar to other lesions in congenital hypertrophy of the pigment epithelium. Pigment epithelial cells were enlarged and filled with rounded pigment granules. The photoreceptors located above the foci were partially atrophied. The authors also found several other types of lesions; one of them looked like a hamartoma and spread from the pigment epithelium through the inner layers of the retina, and the other type was a zone of cell hypertrophy.

bibliography

1. Gardner E.J., Stephens F.E. Cancer of the lower digestive tract in one family group. // Am. J. Hum. Genet. - 1950. - March, Vol. 2(1). - P. 41-48.

2. Gardner E. J., Richards R. C. Multiple cutaneous and subcutaneous lesions occurring simultaneously with hereditary polyposis and osteomatosis. // Am. J. Hum. Genet. - 1953. - Vol. 5. - P. 139-147.

3. Fader M., Kline S. N., Spatz S. S., Zubrow H. J. Gardner's syndrome (intestinal polyposis, osteomas, sebaceous cysts) and a new dental

4. Asman H. B. Pierce E. R. Familial polyposis. A statistical study of a large Kentucky kindred Cancer. // 1970, Apr. 25(4). - P. 972-981.

5. Gardner E. J., Burt R. W, Freston J. W. Gastro-intestinal polyposis: syndromes and genetic mechanisms. // West. J. Med. - 1980. - Vol. 132. - P. 488-499.

6. Blair N. P., Trempe C. L. Hypertrophy of the retinal pigment epithelium associated with Gardner's syndrome. // Am. J. Ophthalmol. - 1980. - Vol. 90. - P. 661-667.

7. Lewis R. A., Crowder W. E, Eierman L. A., Nussbaum R. L., Ferrell R. E. The Gardner syndrome: significance of ocular features. // Ophthalmology. - 1984. - Vol. 91.-P. 916-925.

8. Shields J. A., Shields C. L, Shah P. G., Pastore D. J. Imperiale SM Jr.: Lack of association amonh typical congenital hypertrophy of the retinal pigment epithelium, adenomatous polyposis and Gardner syndrome. // Ophthalmology. - 1992. - Nov, Vol. 99(11). - P. 1709-1713

9. Traboulsi EI, Murphy S. F, de la Cruz Z. C, Maumenee I. H, Green WR A clinicopathologic study of the eyes in familial adenomatous polyposis with extracolonic manifestations (Gardner's syndrome). // Am. J. Ophthalmol, 1990, Nov, Vol 15, 110(5), P. 550-561.

Gardner's syndrome

G Key words: "fish tail"; "bear tracks"; congenital pigmented epithelium hypertrophy

Czerny Edward is a professor. Institute of Ophthalmology, Vanderbilt University, USA, Tennessee, Nashville. 2311 Nashville Tennessee, USA. Email: [email protected]

Cherney Edward- Edu Associate Professor of Ophthalmology Vanderbilt Eye Institute. Vanderbilt University. 2311 Pierce Avenue, Nashville, TN, 37232, USA. Email: [email protected]

Polyposis syndromes

In patients with familial adenomatosis of the colon, as well as in other forms of polyposis, there are various extraintestinal manifestations of the disease, which can often be seen already during an external examination of the patient. The combination of colon polyposis with other manifestations of the disease is referred to as a syndrome, usually it bears the name of the author who first described it. Knowledge of these combinations (syndromes) allows a doctor of any specialty, if extraintestinal manifestations are detected, to suspect the presence of polyps in the gastrointestinal tract, prescribe an examination of the intestine and thereby contribute to more early diagnosis polyposis and cancer. For professionals involved in the treatment of patients with polyposis, knowledge of these syndromes is also important, since some extraintestinal lesions themselves may require treatment, including surgery.

Gardner syndrome - a combination of familial adenomatosis of the colon with tumors of soft tissues, osteomas of the bones of the skull. Of the soft tissue tumors, desmoids are most common - highly differentiated connective tissue formations. They can be localized in the anterior abdominal wall, the mesentery of the small and large intestines, sometimes in the intermuscular layers of the back and shoulder girdle. Quite often, tumors reach gigantic sizes (a desmoid fibroma weighing more than 13 kg was removed in the GNCC).

The presence of osteomas, soft tissue tumors should serve as a reason for a thorough questioning about the nature of the stool, its frequency and consistency, the presence of blood and mucus secretions, followed by a digital examination of the rectum, sigmoidoscopy and colonoscopy or irrigoscopy.

Allfield syndrome - a combination of familial adenomatosis of the colon with cysts of the sebaceous glands. Detection of epidermoid cysts, often multiple in young people, should alert the doctor and force him to undertake a study of the gastrointestinal tract.

Turcot syndrome - familial adenomatosis of the large intestine in combination with malignant tumors central nervous system of neuroepithelial origin. The syndrome was described by Turco et al. in 1959. Experts believe that with adenomatosis of the colon, all patients need to undergo a brain examination to diagnose a possible combination of adenomatosis and tumors of the central nervous system.

Zollinger-Ellison Syndrome - a combination of familial adenomatosis of the colon with tumors of the endocrine glands (most often - tumors of the thyroid gland).

Peutz-Jeghers Syndrome - a combination of polyposis of the gastrointestinal tract with characteristic melanin pigmentation of the mucous membrane of the lips and skin of the face, more often around the mouth. The spots resemble freckles, but since freckles do not occur on the mucous membrane, the presence of age spots on the lips immediately catches the eye. When examining the oral cavity, melanin pigmentation is also detected on the buccal mucosa.

It should be emphasized that polyps in the described syndrome are not adenomas, they have a different structure, these are hamartomas. The stroma of polyps is a tree-branching bundles of smooth muscles emanating from the own muscular plate of the intestinal mucosa. This malformation is caused by damage to the gene, so the disease is often inherited and can be observed in members of the same family.

Polyps in Peutz-Jeghers syndrome can be of different sizes (from 0.5 to 5 cm or more). Due to the high content of muscle fibers, polyps are quite dense. According to the frequency of localization of hamartomas, the toxae and ileum are in the 1st place, then - the large intestine and stomach. Under the influence of intestinal peristalsis, the movement of its contents, large formations pull the intestinal mucosa along with them, while they can move tens of centimeters, cause intestinal intussusception, creating a picture intestinal obstruction. The presence of hamartomas may be the cause intestinal bleeding. Abdominal pain, attacks of intestinal obstruction in patients with Peutz-Jeghers syndrome, as a rule, begin to disturb from early childhood.

Small polyps of the colon are removed through the endoscope, in the presence of large formations, their localization outside the reach of the colonoscope, a laparotomy is performed, polyps are removed through incisions in the intestinal wall, sometimes it is necessary to resect a segment of the intestine with polyps.

Hamartomas are usually not prone to malignancy, but in patients with Peutz-Jeghers syndrome, tumors of other organs (pancreas, thyroid glands, ovaries, etc.) develop more often than in the general population.

Cronkite-Canada Syndrome. In 1947 American doctors L. Cronkite and W. Canada described gastrointestinal polyposis with extraintestinal manifestations of nail atrophy, alopecia, skin pigmentation (more pronounced around the mouth and anus), and hypoprotinemia. Polyps in this disease are diffusely located on the mucous membrane of the colon. They are not true adenomas. Histological examination reveals cystic enlarged glands with atrophy of the epithelium without signs of dysplasia, in the stroma of polyps there is a small infiltrate of plasma cells and eosinophils.

Patients are concerned about poor health, stool disorder (diarrhea), brittle nails, baldness.

The disease is rare, more often it affects middle-aged and elderly people. The causes of the disease are not yet clear.