Visceromegaly overgrowth, diagnosis, causes

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Philip Kelley
Visceromegaly overgrowth, diagnosis, causes

The term visceromegaly, or organomegaly, refers to the continuous and pathological growth of one or more organs of the body. Some types of visceromegaly occur from diseases or infections that cause an organ to start growing abnormally. In other cases, pathological growth occurs due to genetic problems and is part of complex syndromes.

On many occasions, when an organ grows uncontrollably, it loses the ability to perform its functions properly, which has serious repercussions on the patient's health. Of the conditions that cause this disorder, some have a cure and others are only controlled through the treatment of their symptoms..

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The most frequent visceromegalies are cardiomegaly, which is the growth of the heart; splenomegaly, enlargement of the spleen; and macroglossia, which is the enlargement of the tongue.

The so-called overgrowth syndromes encompass a set of rare genetic conditions that have in common the abnormal growth of one or more organs of the body. It is rare for these syndromes to cause overgrowth of the whole body, but such cases have been described..

Review that overgrowth syndromes significantly increase the risk of developing cancer.

Article index

  • 1 Overgrowth
  • 2 Visceromegaly
    • 2.1 - Diagnosis
    • 2.2 - Related diseases
  • 3 Other causes of visceromegaly
    • 3.1 Chagas disease
    • 3.2 Circulatory disorders and anemia
    • 3.3 Metastatic cancer
  • 4 References

Overgrowth

Growth is a physiological process in which internal and external factors intervene. Among the internal elements that promote it are the genetic or hereditary factor and the intervention of some hormones, such as human growth hormone, that speed up or inhibit the ability of cells to divide.

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When there are genetic mutations or hormonal problems that alter the physiology of growth, the uncontrolled and accelerated increase in the size of the body's organs can occur. This is known as overgrowth..

Growth disturbances can be observed from the fetus and maintained throughout life. A patient is diagnosed with overgrowth when the measurement of his physiognomic characteristics are above normal for his age.

The overgrowth can be of the whole body or of one or more internal organs, this condition is known as visceromegaly.

Visceromegaly

Like overgrowth, visceromegaly is diagnosed by measuring the organ involved. In this way, it is evident that the size of the organ is above the normal average for the age and sex of the patient..

Visceromegaly is a clinical manifestation of complex syndromes that involve genetic and hormonal alterations. Abnormal growth of a viscus can also occur in response to a parasitic infection, such as Chagas disease, which causes an enlarged heart or cardiomegaly..

The organs most frequently affected in diseases associated with visceromegaly are: the liver, spleen, heart and tongue. Conditions known as hepatomegaly, splenomegaly, cardiomegaly, and macroglossia, respectively.

- Diagnosis

The diagnostic approach begins with the questioning and physical examination of the patient. When the doctor can palpate the liver or spleen on physical examination, visceromegaly can be diagnosed as these are not palpable organs under normal conditions..

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Imaging studies are used to make an accurate measurement of the organ, especially computerized axial tomography..

- Related diseases

Over the years, a large number of syndromes with overgrowth and visceromegaly have been described. The most common are Beckwith-Wiedemann syndrome (SBW) and Sotos syndrome..

Beckwith-Wiedemann syndrome

SBW occurs due to a chromosomal alteration and accelerated growth can be observed from prenatal controls in the fetus. The most common clinical features are:

- Macroglossia, which is enlargement of the tongue.

- Hemihyperplasia, which refers to asymmetric accelerated growth. One side of the body grows faster than the other.

- Renal abnormalities and visceromegaly.

The visceromegaly in this syndrome involves one or more intra-abdominal organs such as the liver, spleen, kidneys, adrenal glands and / or pancreas..

One of the symptoms that is part of this syndrome is hypogicemia, and it is the cause of many of the complications that these types of patients present, including premature death..

Sotos syndrome

Sotos syndrome is a genetic disorder that prevents the formation of the protein histone methyltransferase, essential in the process of normal growth and development.

It presents with abnormal body growth from birth that continues through adolescence. Features such as macrocephaly (larger than normal head circumference), hypertelorism (abnormal separation of the distance between the eyes), nephromegaly (enlarged kidneys), and hepatomegaly (abnormal liver growth).

In addition to the physiognomic signs, this syndrome is characterized by cognitive deficits, learning difficulties, autism and obsessive compulsive syndrome, among other psychological disorders..

Visceromegaly and cancer

Genetic visceromegaly occurs due to an alteration in a person's normal chromosomal pattern. This causes uncontrolled growth of the organs that occurs due to accelerated cell division.

During this cell division, mutations that form malignant cells can occur in the organ involved..

In syndromes with visceromegaly, the risk of presenting some form of cancer is considerably higher than in the normal population..

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The malignant tumors that occur most frequently due to abnormal growth of the viscera are: Wilms tumor and hepatoblastoma.

Wilms tumor or nephroblastoma, is a type of malignant kidney tumor that occurs in newborns due to genetic alterations that cause the abnormal division of kidney cells.

For its part, hepatoblastoma is the most common malignant liver tumor in pediatric age. It is associated with genetic abnormalities, especially SBW.

Other causes of visceromegaly

In addition to genetic disorders that cause overgrowth and visceromegaly, there are acquired diseases that cause the enlargement of a specific organ.

Chagas disease

Chagas disease or American trypanosomiasis, is a parasitic disease transmitted by the Trypanosome cruzi. The infection can occur directly by contamination with stools of the carrier insect, the chipo, or it can occur indirectly by transplantation of infected organs, through contaminated food and from the pregnant woman to the fetus..

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In its chronic stage, the infected patient presents with cardiomegaly, hepatomegaly, splenomegaly, megacolon, and megaesophagus. These visceromegalies are a consequence of the infiltration by fibrous tissue that the parasite causes in the muscle and neurological fibers of these organs..

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The enlargement of the heart chambers is the most dangerous complication of the disease and makes the heart inefficient in pumping blood. In addition, ineffective movements such as atrial fibrillation and extrasystoles may be evidenced..

Circulatory disorders and anemia

Diseases such as hemolytic anemia, congestion of the portal vein, chronic myeloid leukemia and lymphomas, present with an enlarged spleen or splenomegaly.

Metastatic cancer

The liver is one of the main metastasis receptor organs. Both metastatic and primary liver tumors cause liver enlargement or hepatomegaly.

References

  1. Kamien, B; Ronan, A; Poke, G; Sinnerbrink, I; Baynam, G; Ward, M; Scott, R. J. (2018). A Clinical Review of Generalized Overgrowth Syndromes in the Era of Massively Parallel Sequencing. Molecular syndromology. Taken from: ncbi.nlm.nih.gov
  2. Lacerda, L; Alves, U. D; Zanier, J. F; Machado, D. C; Camilo, G. B; Lopes, A. J. (2014). Differential diagnoses of overgrowth syndromes: the most important clinical and radiological disease manifestations. Radiology research and practice. Taken from: ncbi.nlm.nih.gov
  3. Shuman, C; Beckwith, JB; Weksberg, R. (2016). Beckwith-Wiedemann Syndrome. GeneReviews® Seattle (WA): University of Washington, Seattle. Taken from: ncbi.nlm.nih.gov
  4. Sánchez, MJ; Tenorio, J; García-Miñaur, S; Santos-Simarro, F; Lapunzina, P. (2016). Syndromes of overgrowth and development of embryonic tumors: a review of our casuistry in the last 5 years. Annals of Pediatrics. Taken from: analesdepediatria.org
  5. Nguyen, T; Waseem, M. (2019). Chagas Disease (American Trypanosomiasis). StatePearls. Treasure Island (FL). Taken from: ncbi.nlm.nih.gov

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