Laron syndrome: causes, diagnosis, treatment, prognosis

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2019-03-24 17:20:25

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Laron Syndrome is a pathological condition in which the body is not able to perceive and use the growth hormone. The most common symptom of this disorder is dwarfism. Other symptoms can distinguish muscle weakness and lower endurance, childhood hypoglycemia, delayed sexual development, short limbs, and also obesity. Often the pathology is caused by changes (mutations) in the gene responsible for growth. It is inherited in an autosomal recessive basis.

Symptoms

Laron syndrome

Laron Syndrome is a rare disorder. When the diagnosis of newborns, tend to have average growth and weight, however further their physical development slows down sharply. If untreated, the adult males reach a maximum growth in 130 see the Rise of women does not exceed 120 cm.

In Addition to the low growth on the presence of pathology is evidenced by the following characteristics:

  • Muscle weakness, lack of endurance;
  • Hypoglycemia in childhood;
  • Delayed puberty;
  • A disproportionately small genitals;
  • Thin, weak hair;
  • Violations of the structure of the teeth;
  • Shortened limbs;
  • Obesity;
  • Distinctive facial features (prominent forehead, sunken nasal bridge, blue sclera syndrome).

Causes

Laron Syndrome cause mutations of the gene that contains the growth hormone receptor protein located on the outer membranes of cells throughout the body. The growth hormone receptor is designed to recognize and enhance the somatotropin, thus initiating the growth and division of cells. Mutations of the gene interfere with the normal functioning of this biological mechanism and lead to impaired cellular development. Many of the symptoms of the pathology caused by the inability to develop a secondary hormone, insulin-like growth factor.

Laron syndrome in children

Inheritance

In most cases, Laron syndrome in children is inherited in an autosomal recessive manner. This means that the failure is caused by mutations in both copies of the affected gene in each cell. The parents of the patient with pathology typically are carriers of one mutated copy of a gene and rarely suffer from the typical forms of violations. When two carriers of an autosomal recessive disease a child is born, the risk of inheritance of the pathology is 25 %; the likelihood of becoming a passive vehicle like each of the parents, - 50 %; the chance of inheriting neither the pathology nor the mutated gene is 25 %.

According to some, there are families in which the Laron syndrome is inherited in an autosomal dominant pattern. In such cases, for the transmission of disease is a mutation in one copy of the affected gene in each cell. Sometimes, on this basis, the child takes over the pathology from the patient's parent. There are also cases new, not inherited genetic mutations, when the fault is diagnosed in children in families where there was not a single case of the disease. People with confirmed pathology reports of the mutated gene to their children in 50% of cases.

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Laron syndrome photo

Diagnostics

After the initial evaluation obvious signs and symptoms a specialist can diagnose Laron syndrome (photo of people with disease, see). To confirm this diagnosis and effective differentiation conducted additional tests, including a blood test for specific hormones. Most often in people with this violation increases the level of somatotropin while reducing insulin-like growth factor. In some cases, doctors prescribe genetic studies to confirm the gene mutation. The results of diagnostics allow to determine with accuracy the syndrome of Laron.

Treatment

Completely cure this pathological condition, modern medicine is not yet in force. Innovative therapy focuses primarily on the acceleration of growth, which is achieved by a method of subcutaneous injections of the missing hormone insulin-like. The substance stimulates linear growth (height), accelerates the development of the brain and prevents the occurrence of metabolic disorders caused by a prolonged deficit of necessary protein. In addition, these injections raise blood sugar levels, prevent the accumulation of cholesterol and visibly tone the muscle tissue. Treatment of insulin-like growth factor should be under close supervision of specialists, as exceeding the dosage can result in serious health problems.

Laron syndrome treatment

Forecast

The Long-term prognosis is often quite optimistic. Laron syndrome does not affect life expectancy. Moreover, carriers of this hereditary disease is much less at risk of cancer and insulin dependent diabetes. Currently undergoing extensive research to identify links between Laron syndrome, genetic mutations, hormone growth and development of malignant tumors. It is quite possible that such genetic changes is the key to victory over cancer.


Article in other languages:

AR: https://www.tostpost.com/ar/health/16004-laron.html

BE: https://www.tostpost.com/be/zdaro-e/31143-s-ndrom-larona-prychyny-dyyagnostyka-lyachenne-pragnoz.html

DE: https://www.tostpost.com/de/gesundheit/30811-syndrom-larona-ursachen-diagnose-behandlung-prognose.html

ES: https://www.tostpost.com/es/la-salud/30678-el-s-ndrome-de-larona-causas-diagn-stico-tratamiento-pron-stico.html

HI: https://www.tostpost.com/hi/health/17614-laron.html

JA: https://www.tostpost.com/ja/health/15668-laron.html

KK: https://www.tostpost.com/kk/densauly/31464-sindromy-larona-sebepter-diagnostikasy-em-bolzhamy.html

PL: https://www.tostpost.com/pl/zdrowie/32531-zesp-larona-przyczyny-diagnostyka-leczenie-rokowanie.html

PT: https://www.tostpost.com/pt/sa-de/32248-a-s-ndrome-de-larona-causas-diagn-stico-tratamento-previs-o-de.html

TR: https://www.tostpost.com/tr/sa-l-k/27853-sendromu-larona-nedenleri-tan-tedavi-prognoz.html

UK: https://www.tostpost.com/uk/zdorov-ya/31688-sindrom-larona-prichini-d-agnostika-l-kuvannya-prognoz.html

ZH: https://www.tostpost.com/zh/health/6681-laron-syndrome-causes-diagnosis-treatment-prognosis.html






Alin Trodden - author of the article, editor
"Hi, I'm Alin Trodden. I write texts, read books, and look for impressions. And I'm not bad at telling you about it. I am always happy to participate in interesting projects."

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