Cystic fibrosis

Fibroma: origin and essence of the disease

The causes of dermatofibroma and other forms of this type of tumor are not known. Some researchers believe that fibroids can form as a localized tissue reaction after minor trauma. Sometimes fibroids can have a genetic component, especially in people of Northern European descent. Some medications, including beta blockers, have been reported to cause changes in fibrous tissue. In addition, some fibroids may be influenced by hormonal imbalances or pathologies of endocrine organs, including problems with the thyroid and pancreas.

Hyperhidrosis (increased sweating), inflammatory processes on the skin, especially chronic ones, as well as the influence of prolonged UV radiation, poor nutrition and bad habits can have a certain impact.

Diagnosis of cystic fibrosis

Diagnosis of cystic fibrosis may require both laboratory and genetic testing. Genetic studies are highly informative and allow one to suspect the development of the disease almost immediately after birth. At the moment, the disease can be detected even before the birth of a child through neonatal screening.

In order to confidently say that a person has cystic fibrosis, a doctor must diagnose the following indicators1:

• the presence of bronchial defects or the presence of thick and viscous sputum in the bronchi;
• positive sweat test results;
• the presence of pancreatic enzyme deficiency;
• cases of cystic fibrosis in the family.

Signs and symptoms

Fibroids are classified as benign formations. They form a tumor-like growth of tissue, inside of which there is mainly fibrous (or connective) tissue. Signs of fibroids arise as a result of uncontrolled by the body, but slow and limited growth of a certain area of ​​​​tissue. Rarely fibroids are malignant, and the reasons for this are trauma and malignancy of the formation. Different types of fibroids can develop anywhere, and if they are small in size, they require active monitoring or removal if they cause discomfort.

Some women with uterine fibroids have no or only mild symptoms, while other women have more severe, debilitating symptoms. Common symptoms of a tumor include:

• heavy or prolonged menstrual flow;
• abnormal bleeding between periods;
• pain in the pelvic area;
• frequent urination;
• lumbago;
• pain during intercourse;
• infertility.

Common symptoms of dermatofibroma involve discomfort and may come and go. Symptoms of dermatofibroma are not severe and include:

• color change over time;
• itching;
• periodic swelling over the tumor;
• possible bleeding due to injury;
• sensitivity of dermatofibroma to touch;
• a small bump with a raised surface.

Symptoms of plantar fibroma are not severe and include:

• expansion of sizes over time;
• hard lump in the arch of the foot;
• pain when pressing, standing or walking;
• spread of additional fibroids over time.

Symptoms of cystic fibrosis

Experts distinguish several forms of the disease: intestinal, pulmonary and mixed.

The main symptoms of the disease usually include:

• lack of appetite, screaming and loud crying in infants and newborns;
• bloating;
• feeling of dry mouth.

Symptoms of cystic fibrosis depend on the form of the disease. Cystic fibrosis of the lungs greatly reduces immunity, and since mucus plugs are easily affected by bacteria of staphylococcus or Pseudomonas aeruginosa, people constantly suffer from recurring bronchitis and pneumonia.

The main signs of pulmonary cystic fibrosis1:

• frequent respiratory viral diseases;
• constant paroxysmal cough with thick and poorly separated sputum;
• tendency to pneumonia and bronchitis due to ARVI*;
• hard breathing, the appearance of wheezing during colds and viral diseases;

• the appearance of shortness of breath, signs of hypoxia;
• weight loss, vitamin deficiencies;
• a change in the shape of the chest, which takes on the appearance of a boat keel, and may become barrel-shaped;
• nail plates and fingertips take on the appearance of drumsticks.

The intestinal form of cystic fibrosis is characterized by enzyme deficiency and impaired absorption of nutrients in the intestine. As a result of a lack of enzymes, the stool becomes “greasy”, bacteria can actively multiply, as a result of which gases accumulate, which leads to severe flatulence. Stools become more frequent, and the volume of feces can be several times higher than the age norm.

In children, the intestinal form of cystic fibrosis occurs with increased viscosity of saliva and severe thirst. Appetite may be reduced due to indigestion, persistent abdominal pain and flatulence. From the first days of life, a child may suffer from intestinal obstruction, which is caused by a lack of the enzyme trypsin, which is responsible for the breakdown of proteins. As a result, meconium accumulates in the small intestine - a dense, thick mass of dark brown color. The child develops constipation and frequent regurgitation, and vomiting may occur. In addition to symptoms from the gastrointestinal tract (GIT), the disease can affect the general condition of the body, since high-quality nutrition has a direct impact on the development of the child. Due to a lack of nutrients, weakness, brittle bones may appear, and the condition of the skin, nails, and hair may deteriorate1.

The mixed form of the disease is characterized by a combination of damage to the intestine and bronchopulmonary system. This is also manifested in symptoms: people with a mixed form of cystic fibrosis suffer from frequent viral diseases, a lingering cough and, at the same time, indigestion and flatulence1.

Regardless of the form of the disease, one of the common symptoms of cystic fibrosis is the salty taste of the patient's skin. This is due to increased chloride levels in sweat and has long been a key feature for diagnosis1.

Types, characteristics of fibroids

There are more than 10 different morphological variants. Pathologists give a description of what each form looks like and how it differs after histological examination - elastrophibroma, soft, dense fibroma.

• Dermatofibroma is a single or multiple formation of intradermal localization, mobile and painless when palpated with unchanged color.
• Desmoid fibroma has another name - aggressive fibrosis, refers to mesenchymal tumors originating from excess collagen fibers and differentiated fibroblasts. They often progress and recur, forming in soft tissues, the retroperitoneum or on the abdominal wall.
• Chondriomyxomas are rare tumors that affect bone tissue - the edges of long bones, pelvis, ribs, vertebrae, feet, metatarsus.
• Angiofibriomas in the cheek and nose area, containing small tubercles with connective tissue fibers.

Diagnostics

Initially, experienced specialists - oncologists or multidisciplinary surgeons - conduct a detailed examination of the patient, record all the complaints that exist, palpate the formation and make a preliminary diagnosis. Then you need to determine the nature of the tumor. After this, you need to conduct an ultrasound examination of the tissues where the tumor is located to determine its size, nature, and changes in the tissues. If necessary, an X-ray examination is performed. If questions remain, a biopsy of the suspicious area should be performed to rule out a malignant process.

Fibroids can be detected by palpation (feeling with fingers or hands) performed as part of a pelvic examination, or diagnosed through imaging procedures such as ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI).

Treatment methods

Dermatofibromas are harmless and treatment is not required unless there are warning signs or cosmetic concerns. If the patient wishes to remove dermatofibroma, surgical treatment is possible - surgery . It is important to evaluate its consequences: the scarring and tissue changes that occur after surgical excision may look worse than the original lump.

For plantar fibroids, non-surgical treatment options are preferred because the surgical procedure requires a long recovery period and can lead to complications that may be worse than the plantar fibroids themselves. Non-invasive treatments for plantar fibroids include:

• extreme cold or cryodestruction to reduce fibroids.
• pads or insoles to relieve discomfort when wearing shoes.
• stretching.

Most doctors agree that for severe cases of plantar fibroma, the smartest option is to resort to invasive treatments and surgery. Invasive treatments for plantar fibroids include:

• injections of corticosteroids into fibroids.
• surgical removal of the entire plantar fascia (which is associated with a long recovery period and a high risk of developing other foot problems).
• surgical removal of fibroids (with a high recurrence rate).

Treatment is indicated when dermatofibromas are bothersome or persistently irritated. In these cases, surgical removal and freezing with liquid nitrogen can be performed.

Treatment for uterine fibroids depends on the size, symptoms, and other factors. Asymptomatic fibroids may not require treatment. A myomectomy (surgical removal of uterine fibroids) may be performed to remove fibroids that are interfering with fertility in women who want to become pregnant. Hysterectomy (surgical removal of the uterus) is also commonly performed on patients with severe symptoms of uterine fibroids, but is not an option for women planning a pregnancy. Non-surgical treatment for uterine fibroids includes medications, uterine artery embolization and targeted ultrasound treatment.

Treatment of cystic fibrosis

It is currently impossible to completely cure cystic fibrosis. Modern medicine can only mitigate the manifestation of the disease and increase the length and quality of life of the patient1.

Treatment of cystic fibrosis should be comprehensive and aimed at the main symptoms of the disease: fighting infectious diseases, clearing the bronchi of mucus, replenishing missing pancreatic enzymes.

Part of the therapy must be aimed at supporting the bronchopulmonary system and preventing complications from recurring bronchitis and pneumonia. If infectious diseases develop, treatment with antibiotics is indicated. Be sure to use methods and means that help dilute mucus and freely remove it from the bronchi and lungs. Mucolytics - drugs that dilute sputum, are used in long courses, with short breaks.

In the intestinal form of cystic fibrosis, the key task is to restore impaired digestion. As we have already found out, the pancreas, which responds to the production of enzymes, is inactive in a patient with cystic fibrosis. This means that the child is not able to obtain energy from food. That is why taking enzyme preparations for such patients is key and largely determines life expectancy. Just think, how long can you survive without quality nutrition? The dosage is selected individually, depending on the condition and body weight of the child. Digestive enzymes help compensate for insufficient secretory activity of the pancreas, helping to maintain digestion by delivering enzymes from the outside.

Diet for cystic fibrosis is not a temporary restriction, but a lifelong way of eating. A person's diet should contain a large amount of protein, obtained from lean meat, quality fish, cottage cheese and eggs. It is desirable that the diet be high in calories, but it is necessary to limit the amount of animal fats, trans fats and coarse fiber that irritate the inflamed walls of the gastrointestinal tract.

If lactose intolerance develops, milk should be excluded from the diet. Due to dry mucous membranes and impaired mucus secretion, an increased drinking regimen is recommended, especially in hot months.

Physiotherapy is very useful for cystic fibrosis: vibration massage, aerosol inhalations, physical therapy can improve the patient’s condition and prevent the development of complications1.

Predictions of why fibroids are dangerous

Dermatofibroma has no serious complications. Complications of plantar fibroids usually occur as a result of surgical interventions. These include flattening of the arch of the foot, postoperative nerve entrapment, and postoperative growth of larger and recurrent fibromas.

Article sources:

1. Desmoid fibroma. Staged, organ-preserving surgical treatment. Khvastunov R.A., Mozgovoy P.V., Lukovskova A.A., Yusifova A.A. Volgograd scientific and medical journal No. 2, 2021. p. 54-57
2. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2927520/ Central odontogenic fibroma: a case report with long-term follow-up. Marco T Brazão-Silva, Alexandre V Fernandes, Antônio F Durighetto-Júnior, Sérgio V Cardoso, Adriano M Loyolacorresponding
3. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3351722/ Literature survey on epidemiology and pathology of cardiac fibroma. SuguruTorimitsu, Tetsuo Nemoto, Megumi Wakayama, Yoichiro Okubo, Tomoyuki Yokose, Kanako Kitahara, Tsukasa Ozawa, Haruo Nakayama, Minoru Shinozaki, Daisuke Sasai, Takao Ishiwatari, Kensuke Takuma, Kazutoshi Shibuya

The information in this article is provided for reference purposes and does not replace advice from a qualified professional. Don't self-medicate! At the first signs of illness, you should consult a doctor.