Harlequin syndrome: symptoms, features and treatment

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Harlequin syndrome: symptoms, features and treatment
Harlequin syndrome: symptoms, features and treatment

Video: Harlequin syndrome: symptoms, features and treatment

Video: Harlequin syndrome: symptoms, features and treatment
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Newborn babies are very defenseless. Even in the womb, they can be influenced by various negative factors that cause pathologies in the development of the crumbs. As a result, having been born, the little one has many problems. One of the most terrible deviations is the Harlequin syndrome. The disease was named after a character in the Italian comedy of masks, who wore a costume made of multi-colored scraps of fabric.

Harlequin syndrome in a newborn

Doctors detect the problem immediately after the baby is born. According to them, Harlequin syndrome can be explained by the imperfection of the development of the autonomic system, which ceases to control the correct vascular tone. The disease can also be triggered by simple gravity. Why is this happening? Physicians cannot give an unequivocal answer to an exciting question. The range of reasons is very wide: from a simple failure in the development of the fetus to the wrong lifestyle that a pregnant woman leads.

harlequin syndrome
harlequin syndrome

The syndrome can manifest itself both throughout the body, and only in certain parts of the body. Such babies, doctors say, will constantlysuffer from colds, as their thermoregulation is impaired. At any time of the year, the peanut will sweat profusely, then sharply supercool, as a result of which he is provided with constant viral infections. In addition, the syndrome causes a lot of discomfort, so the child often cries, he is restless and irritable.

Symptoms

According to some reports, the syndrome may be a consequence of fetal asphyxia or intracranial trauma during childbirth. It is also diagnosed in cases of damage to the hypothalamus or medulla oblongata. In any case, a neurologist is immediately called for a consultation. Detecting the disease is quite simple: its main symptom is reddening of the skin when the baby lies on its side. In this case, the border of the color change runs exactly along the line of the spine.

harlequin syndrome photo
harlequin syndrome photo

Uneven color of body parts occurs a few minutes after the baby is laid down. This is how Harlequin syndrome manifests itself. Photos of young children in medical encyclopedias show an unsightly picture - the upper part of their body has a normal color, the underlying one becomes bright red, sometimes with a bluish tint. The phenomenon is noticeable for several minutes. Some experts say the syndrome may be a sign of prematurity.

Skin manifestations

If medical terms are used when describing the disease, then Harlequin syndrome manifests itself as a compact keratinization of the skin. There is no so-called granular layer on it, instead plaques are visible that form during crackingepidermis. These formations are hexagonal, often separated by deep red fissures. Skin color is gray or icteric.

Children born with this syndrome often have other abnormalities: they may have a deformed mouth (it looks like a fish lips), eversion of the eyelids, improperly developed, too flat ears. In some cases, microcephaly is diagnosed when the baby's skull is underdeveloped: as a result, he is born with a very small brain. Such children die in the neonatal period or when they are born.

Features of the disease

Babies don't always die if they have Harlequin pathology. The syndrome is not a sentence. The disease may not be pronounced, moreover, if it is quickly and correctly diagnosed, it is possible to alleviate the condition of the crumbs with special preparations. In such children, not only heat transfer is disturbed, but also electrolyte balance. The keratinized skin makes it difficult to breathe and move. Infection can get into large cracks and sepsis can develop.

harlequin syndrome in the newborn
harlequin syndrome in the newborn

Fortunately, the disease is not very common. At the same time, the gender of the baby does not matter: both boys and girls are susceptible to pathology. Doctors say that the syndrome is often severe. In this case, the prognosis in most cases is quite unfavorable. The etiology of the syndrome has not yet been established.

Therapy

The baby is prescribed a course of treatment, which is aimed at maintaining normal body temperature and stabilizing the water and electrolyte balance. Moreover, such a patientnormal nutrition, anti-inflammatory, antibacterial therapy is required. The use of retinoids in the form of ointments and oral capsules significantly increases the survival rate if the baby is diagnosed with Harlequin syndrome. Treatment involves the use of drugs, where the active ingredients are isotretinoin and acitretin. Despite this, the defects remain on the skin until the end of days. The quality of life in such patients is significantly reduced.

harlequin syndrome treatment
harlequin syndrome treatment

If the syndrome is not pronounced, then you can do without medication. It is enough for parents to maintain a normal temperature in the children's room - around +20 degrees. It is necessary to accustom the baby to climatic changes gradually: first, he must adapt to the cold and only then to the heat. Thus, you will train the vegetative system and the walls of the baby's vessels. Remember: if the baby is sweating, you need to change clothes immediately. Such procedures will definitely give a positive result.

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