Dysplasia Treatment folk remedies

August 12, 2017 17:51 | Genetic Diseases

Dysplasia - abnormal development of organs and tissues.Basically dysplastic manifestations presented congenital malformations.Improper development can be observed in the post-embryonic life, t. E. After birth, which may be associated with prolongation of the period when these factors are teratogenic effect at the end of the embryonic period.Dysplasia in these cases is shown at the tissue level.

Renal dysplasia is part of a large group of diseases, the combined term "renal dizembriogeneza", which can be hereditary or associated with adverse effects on the fetus teratogenic factors.Renal dysplasia - a group of diseases that are characterized by disruption of the structure of renal tissue.Violations of the structure of kidney tissue may clinically manifest itself as an independent disease.Often, however, the first signs of the disease process appears when connecting or development on their basis of the pathology of the immune system or microbial-inflammatory processes.Typically, children with this diso

rder are born from pregnancy, occurring with toxemia, violence, aggravated by nephropathy and various malformations.

About renal tissue dysplasia can speak in violation of renal tissue differentiation, developmental delay with the presence of fetal kidney tissue structures, which are characterized by the presence of only the fetus during intrauterine life.Such signs of a lack of maturity of the kidney tissue, as the presence of germline glomeruli, are not decisive in the diagnosis of renal dysplasia.This position can be maintained to a certain extent at least, because after birth, as the child's development is a further maturation of the renal structures.

In the early years of a child's life manifestations of a lack of maturity rate of kidney structures significantly higher than in children after 9-10 years as well as in the growing child, the process of maturation of the structural elements of the kidneys.Later, however, the maturation of the renal structures is also a sign dizembriogeneza.There are agenesis (congenital absence), hypoplasia (underdevelopment), and polycystic dysplasia itself (multiple cysts) of the kidneys.These manifestations of renal development are often combined.In the urological clinic we use the following classification of renal dysplasia: 1 - total (simple and cystic), II - cortico-modullyarnaya and III - obstructive.

Multiple epiphyseal dysplasia disease is also called Feyerbanka.In this disease process is broken bones epiphysis ossification.However, the formation of articular cartilage occurs normally.In multiple epiphyseal dysplasia of the first complaint of pain in the knee joints appear in children aged 5 years and older.Joint deformity usually does not happen.When X-ray at this time there is a decrease in the epiphysis primarily femoral heads and the epiphysis of the tibia (shin bone).In the area of ​​the knee when the child contours of the epiphyseal bone growth are jagged.In some children the collapse of the femoral head can occur.In such cases, in the groin or the knee on the affected side appears a sharp pain when walking, appears limp, motion in the hip joints are limited, the motion also appears soreness.All these changes lead to early arthrosis of the hip joints.Changes in the spine minor.A clear picture of the epiphyseal dysplasia develops in 7-8 years in a child's life, and at this age is quite easily recognized, while in the early stages of the disease correct diagnosis presents certain difficulties.Usually these children are diagnosed with diseases that are more common in orthopedic practice.In cases when expressed in all the possible manifestations of the disease to be distinguished form one another by epiphyseal dysplasia.The initial symptoms of these diseases are very similar: rolling gait, fatigue, limping.However, complaints of pain in the legs of children with multiple epiphyseal dysplasia appear earlier.At older ages, children sospondilo-epiphyseal dysplasia begin to fall behind in the growth of their peers, and the growth is usually normal in multiple epiphyseal dysplasia.When spondee-epiphyseal dysplasia, joint pain are more pronounced, and in the affected joints appear before the restriction of movement.The axis of the lower extremities in multiple epiphyseal dysplasia is often correct, sometimes there is a slight valgus deformity stop.When spondee-epiphyseal dysplasia more often contracture and deformation of the lower limbs, patellar luxation.In order to accurately distinguish between the two diseases from each other, it is necessary to carry out X-ray examination of the skeletal system.

following forms of hereditary systemic skeletal disease are diastrophic dysplasia, pseudo-achondroplasia, as well as a group of diseases with damage to epiphyseal bone, but with a predominant involvement in the process of the spine and hip joints.This group includes: congenital spondee-epiphyseal dysplasia, metatropicheskaya dysplasia, dysplasia Knista illness Diggvi-Melchior-Clausen.

-Ectodermal Dysplasia meso alopecia (Golttsa- Gorlin syndrome)

The disease is relatively rare, t. E., There is only a tiny chance of developing it just at individual children.Said syndrome is manifested primarily by changes in the skin that are usually combined with defects of the skeleton, the teeth and eyes.Suffering from this disease mostly girls.It is believed that the disease is inherited clutch with X-linked dominant.At the same boys, which has affected the disease, die in utero, ie. E. A woman develops a miscarriage.The mechanism of this syndrome is still unknown.

Skin Lesions in the syndrome of Gorlin-Goltz have a distribution in the form of strips.This is the distribution of skin lesions coincide with the lines of her cleavage.In addition to skin lesions, the child during the inspection, you can see degenerative changes of the teeth and nails, hair abnormalities, such children are sweating significantly less compared to the healthy (which is also associated with skin underdevelopment, namely its member sweat glands), excess skin actinic onvarious areas of the body, the palms, soles, inner joints, deformation of the ears, ocular abnormalities, especially a defect in a slot (it may be located at the edge of the upper or lower eyelid, as well as the iris), various bone defects (dysplasia, asymmetric structure of the face,the trunk or limbs, spinal curvature, hypoplasia or absence of fingers, the fusion of two or more fingers because of the skin or skin-articular adhesions, hands and feet deformity).During the radiographic examination revealed characteristic symptom of the disease - pathological changes in the long bones, as noted in 80% of cases.Other changes may draw the attention of bullous or ulcerative lesions on the skin, congenital absence of skin.On closer examination in the hospital revealed a variety of abnormalities of internal organs.With the growth and development of some children in the forefront starts to go mental retardation.There may be, however, and only skin changes.

Treatment is aimed at accelerating the healing of skin lesions, deformity correction, protection from various injuries.In addition, in order to achieve a good result against the background of the treatment is necessary to remember that a child or an adult suffering from this disease, should avoid prolonged exposure to sunlight, t. E. Giperinsolyatsii.For this purpose, it can be widely used a variety of protective creams, as well as vitamins A and E.

Ectodermal Dysplasia gidroticheskaya (Klustona syndrome)

Unlike the classical version Klustona hypohidrotic ectodermal dysplasia syndrome is inherited in an autosomal dominant pattern, which explains why the samethe incidence of both sexes.The function of sweat and sebaceous glands is not broken, teeth are usually normal.The disease is characterized by two main features - hypotrichosis (lack of body hair), which can reach a degree of alopecia (female or a total lack of hair), and dystrophic nail changes, mostly for hypoplastic type (nails become thin, soft, easily broken).There may be hyperpigmentation (darker areas of the skin) in the joints, periarticular pads giperkeratoticheskie change the palms and soles, and sometimes eye changes (strabismus, premature cataracts), neurological disorders (mental retardation, seizures), but the majority of people with this syndrome, these changesno.It is possible that the form occurring with the associated symptoms are special conditions.

only symptomatic treatment: fortifying agents, vitamins, zinc preparations (for example, zinc oxide at 50-150 mg per day, depending on age) agents that improve tissue trophism, anabolic hormones.

hypohidrotic ectodermal dysplasia (Christ-Siemens syndrome)

disease is genetic heterogeneity in most cases inherited linkage with X-linked recessive type, much less - an autosomal recessive.It does not rule out the possibility in some pedigrees, and autosomal dominant transmission of the abnormal gene.It is assumed heterogeneity and X-linked form.The classic version of the disease is observed only in men.In women, the disease is in a gentle way, appearing intensity decreased sweating, slight defects in teeth, poor development of the mammary glands.This makes it possible in most cases to detect heterozygous carriers in families in women, the probability of detection increases with the use of tests on sweating, counting sweat pores on the fingerpads.Prenatal (antenatal) diagnosis of the disease is provided by ultrastructural examination of the skin of the fetus taken by fetoscopy on the 20th week of pregnancy.Histological examination of the sweat glands are not found, there may be hypoplasia (underdevelopment) of the hair follicles and sebaceous glands.

The main clinical signs are hypo- or anhidrosis (reduction or complete absence of sweating, respectively), hypotrichosis (decreased body hair) and hypodontia, paroxysmal unjustified increase in body temperature, which, in the non-recognition causes, can lead to death in early childhood as well asto a violation of the child's mental development.The function of other glands of the skin is usually preserved.Characterized by the appearance of such people: a square skull, protruding frontal mounds and eyebrows, sunken nasal bridge, large malformed ears, thick lips, sunken cheeks, sparse teeth with a large distance between the upper incisors, rare thinning blond hair on his head, sparse eyebrows and eyelashes,increased deposition of pigment in the skin around the eyes, psevdorubtsovaya folds around the mouth and eyes.There have been increased dryness of the skin and mucous membranes, increased susceptibility to inflammatory diseases, atopy (allergic manifestations).Can meet and other anomalies (nail dystrophy, keratosis pilaris, palmar-plantar keratoderma, changes in the eyes, hearing loss, hypoplasia of the genitals - hypogonadism).The disease begins to manifest from birth or develops in the neonatal period.Adults tend to have a normal life expectancy.

only symptomatic treatment.It is necessary to avoid any kind of overheating (excessive wrapping, hot food, outdoor games).

Individuals with this syndrome is recommended:

1) the timely appointment of antipyretics in the case of attack, increase in body temperature, including infectious origin;

2) employment of patients with the exception of work related to the possibility of overheating;

3) medical and genetic counseling (in families with X-linked recessive inheritance repeated high risk of the disease in male children);

4) prenatal (antenatal) diagnostics.

Hondroektodermalnaya dysplasia (Ellis-van Creveld's disease)

Hondroektodermalnaya dysplasia (disease Ellis-van Creveld) refers to a group of diseases characterized by dwarfism, increasing the number of fingers on the hands and feet, abnormal development of teeth, hair and nails, as well ascongenital heart disease.All persons with the disease are observed frequent pneumonia and bronchitis.Transmitted disease Ellis-van Creveld in an autosomal recessive inheritance type.

Multiple ekzostoznaya chondrodysplasia

Multiple ekzostoznaya chondrodysplasia , or multiple osteochondral exostosis, is a relatively common disease in comparison with other hereditary systemic disease of the skeleton.It accounts for about 27% of all bone tumors and tumor and dysplastic diseases of the musculoskeletal system.This malformation of bone cartilage is the appearance of growths in the epiphyseal and metaphyseal bone.

This deviation is, in fact, dysplasia of bone growth zone.Due to the fact that the growth zone is called the pituitary zone, and this group of diseases called pituitary dysplasia.Developing exostosis located in close proximity to the near or far end of the bone (epiphysis).Along with this change on the part of the skeletal system in individuals with the disease is marked cleft palate, syndactyly, cervical spine curvature, multiple vascular tumors and some other manifestation.

On the other hand, one of the most severe complications of the disease is the degeneration of exostosis in malignant tumors, as noted in 3-25% of all cases.Degeneration of cartilage is exposed, covering exostosis, thus there is a massive growth of cartilage.In the study of the structure of multiple ekzostoznoy chondrodysplasia proportion of familial cases was 44.1%, it is assumed that the disease is transmitted in an autosomal dominant inheritance type.The remaining 55.9% cases are the result of the newly formed gene mutations.In 1966, the syndrome was first described, the characteristic manifestations are subtle and slow-growing hair, nose, pear-shaped, with short fingers tapered epiphyses.

This syndrome was called "trihorinofalangealnaya dysplasia."There are two types of this syndrome.symptoms described above relate to the first type of the disease.The second type combines the same features, but differs from the first by the presence of a human patient's mental retardation one degree or another, as well as multiple osteochondral exostoses.

metaphyseal dysplasia

metaphyseal dysplasia is a heterogeneous group of diseases characterized by lesions mainly metaphyseal bone with relatively normal bone epiphyses and spine.The most prominent representative of metaphyseal dysplasia is a group of metaphyseal chondrodysplasia.At the heart of metaphyseal chondrodysplasia is stunting the main bone of cartilage that occurs as a result of inadequate and improper ossification in metaphyses of long bones.Development and epiphyseal bone growth from periosteum is not broken.This group of diseases is shown behind the growth and limb deformities in children.Currently, the literature 6 described forms of diseases, related to this group of diseases.

most severe disease in this group is type Jansen .This pathology is very rare.In the world literature clearly describes only 10 cases of this disease.Sick people have a very characteristic appearance: a disproportionate physique, stunting, eyes wide apart, sometimes there is a "bulging" eyes (exophthalmos), bridge of the nose is broad and flattened, the lower jaw is underdeveloped, there is malocclusion.Extremities such people a few shortened, twisted, enlarged joints in the volume brush with thick fingers as drumsticks.