APPEARANCE OF RETROLENTAL FIBROPLASIA

PEDIATRICS ◽  
1976 ◽  
Vol 57 (4) ◽  
pp. 599-602

The first description of RLF as a disorder of extreme prematurity was published as a preliminary report by Terry in 1942. He stated that the condition was either "persistence of the entire vascular structure of the fetal vitreous" or a "fibroblastic overgrowth of the persistent tunica vasculosa lentis." His studies led him to conclude that an "overgrowth of embryonic connective tissue in the interstices of the persistent tunica vasculosa lentis behind the lens" accounted for the pathology observed. He stated: "It is really a retrolental fibroplasia." The terminology was used in his subsequent reports and became generally accepted, although later studies by Owens and Owens (1949) showed that RLF was not related to an embryonic abnormality but was caused by an abnormal blood vessel change in the retina. On the basis of clinical observation, Terry (1942) concluded that the disease in its classical form was not present at birth but developed between two and six months after birth. He considered many possible factors: "Of all the probable causes listed, precocious exposure to light is considered the most tenable, and preventive measures should be taken." The light theory was discarded after several investigators found that occluding the eyes after birth had no effect on development of the disease (Hepner et al., 1949; Crosse, 1950; Locke and Reese, 1954). After discovering a disease that had appeared as an isolated rarity in 1942, Terry collected 117 cases of RLF during the next three years. The condition appeared to be on the increase. In 1945 Terry found that the incidence at the Boston Lying-In Hospital was approximately 12% in infants weighing 1,400 gm or less (based on fewer than 50 cases).

Author(s):  
Raashid Luqmani

The vasculitides are a heterogeneous group of disorders that can range from mild inflammation of blood vessels in the skin, to organ- and life-threatening diseases. The term ‘vasculitis’ is a pathological description of blood vessel wall inflammation which leads to ischaemia and infarction of the target organs. Definitions and classifications of the primary vasculitides are mainly based on the predominant calibre of the blood vessels involved but incorporate clinical, pathological, and laboratory features. The secondary vasculitides usually occur in the context of other connective tissue diseases and are not discussed further in this section. Goodpasture’s disease is not usually included in the primary vasculitides, but has compatible clinical features of pulmonary capillaritis and glomerulonephritis.


2012 ◽  
Vol 2012 ◽  
pp. 1-4
Author(s):  
Rajendran Ganesh ◽  
Rajendran Vijayakumar ◽  
Haridoss Selvakumar

Marfan syndrome is an autosomal dominant systemic disorder of the connective tissue. Children affected by the Marfan syndrome carry a mutation in one of their two copies of the gene that encodes the connective tissue protein fibrillin-1. Marfan syndrome affects most organs and tissues, especially the skeleton, lungs, eyes, heart, and the large blood vessel that distributes blood from the heart to the rest of the body. A case report of Marfan syndrome has been reported with oral features. The dental problems of the child were treated under general anesthesia and a one-month review showed intact stainless steel crowns' restorations and no signs of secondary caries.


1983 ◽  
Vol 27 (2) ◽  
pp. 125-129 ◽  
Author(s):  
Alasdair A. McKechnie ◽  
Florence Wilson ◽  
Nan Watson ◽  
David Scott

2020 ◽  
Vol 95 (2) ◽  
pp. 121-128
Author(s):  
Małgorzata Stańczyk ◽  
Piotr Raczyński ◽  
Jarosław Andrzejewski ◽  
Anna Mazurek-Kula ◽  
Katarzyna Ostrowska ◽  
...  

Author(s):  
Tat'iana Sergeevna Fadeeva

The work raises questions of predicting the complications of pregnancy and childbirth and adverse conditions in the fetus in the presence of UCTD in the expectant mother, who also need to be studied, especially from the standpoint of mathematical modeling of the disease. It is also necessary to develop a common tactic for providing medical and social assistance and treatment and diagnostic services to pregnant women suffering from UCTD, which will make the outcome of childbirth more favorable and improve the subsequent prognosis for mother and newborn. In the literature there is practically no assessment of the course of pregnancy and the outcome of childbirth, depending on the severity of UCTD. Little is known about the role of a connective tissue metabolism marker - hydroxyproline, trace elements (magnesium) and vitamins (D3) in pregnant women suffering from UCTD, and the medical tactics regarding such patients are not clearly defined. Despite numerous successes in the study of the causes of complications during pregnancy and childbirth in women suffering from UCTD, a unified approach to their management during the prenatal stage has not yet been developed. Therefore, the search for possible predictors for the timely prediction of adverse pregnancy and childbirth outcome in such patients is becoming increasingly important. This will make it possible to develop an optimal organizational and methodological base and subsequently improve the prognosis for women and their offspring. Thus, in contrast to the existing standard approach, we have proposed a comprehensive management of patients suffering from UCTD, including the timely identification of patients from the risk group, clarification of their condition using such markers as magnesium and hydroxyproline, additional intake of magnesium and vitamin D preparations. Optimal plan managing the period of gestation, childbirth, and a pathogenetically reasoned set of treatment and preventive measures for women with UCTD, will not only improve the outcomes of pregnancy and childbirth, but also contribute to the health of the future generation. 1. UCTD affects the course of pregnancy, childbirth and the condition of the newborn. The degree of exposure is largely determined by the severity of the underlying disease. In severe UCTD, the prevalence of spontaneous miscarriage and preterm labor was significantly higher, and endometritis and severe anemia were more common in the postpartum period. Severe asphyxia on the Apgar scale at the 1st and 5th minutes, congenital heart defects, morphofunctional immaturity, conjugation jaundice and convulsive syndrome were more common in the fetus. 2. A low content of magnesium and hydroxyproline is associated with the occurrence of complications during childbirth and a decrease in the anthropometric characteristics of the newborn. Taking magnesium preparations reliably affects the concentration of this trace element and hydroxyproline in the blood of pregnant women suffering from UCTD. 3. Therapy with magnesium preparations is an effective tool in patients suffering from UCTD, as it helps to improve well-being during pregnancy, improves the course of the postpartum period and reduces the prevalence of chronic fetal hypoxia. 4. Vitamin D and magnesium supplements have a beneficial effect on pregnancy and the fetus, reducing the prevalence of pre-eclampsia and chronic intrauterine hypoxia of the fetus, reducing the incidence of morphofunctional immaturity and conjugation jaundice of the newborn. 5. The created computer program “STEP DST” can be applied in the clinical practice of obstetrician-gynecologists and health care organizers. The obtained individual forecast of the probability of development of complications of reproduction allows us to outline the optimal plan for managing the period of gestation, childbirth and the postpartum period, to prescribe a pathogenetically based set of therapeutic and preventive measures for women suffering from UCTD.


Author(s):  
A. Belousova

The morphological structure of the nasal polyps was studied. In adult patients, the vascular and glandular form of formations were isolated, in the childhood-vascular. Individual fibrosis polyps did not exist, the connective tissue, in one or another quantity is an integral structural component of all polyps. Eosinophilic tissue demonstrates the process's chronization and dystrophic changes, the eosinophils, as you know, promotes thrombus formation. Angiogenesis in the nasal polyps may be due to the use of therapeutic agents, for example, vasoconstrictors that affect the blood vessel tone, microcirculation, stimulating proliferative processes.


2021 ◽  
Vol 27 (2) ◽  
pp. 188-205
Author(s):  
M. E. Evsevyeva ◽  
M. V. Eremin ◽  
M. V. Rostovtseva ◽  
E. N. Fursova ◽  
A. V. Rusydi ◽  
...  

Objective. To assess the occurrence of various vascular aging phenotypes among young people, depending on the level of blood pressure (BP) and in relation to risk factors (RF) and the presence of connective tissue dysplasia (CTD).Design and methods. In total, 250 young people (88 boys and 162 girls) aged from 18 to 25 years old were examined at the University Health Center. All the subjects were divided into tercile groups based on cardio-ankle vascular index (CAVI), determined by the VaSera-1500 (Fucuda Denshia, Japan). According to the latest expert recommendations the third tercile group corresponds to early vascular aging or EVA syndrome. The first tercile group corresponds to favorable vascular aging. We have analyzed the representation of various vascular phenotypes in individuals with elevated and normal BP, as well as the main RF and external signs of CTD in each of CAVI-tercile group. Data processing was performed using the software package “Statistica 10.0” (StatSoft Inc., USA).Results. Among young people with office hypertension and high normal BP, the EVA phenotype and phenotypes of normal and favorable vascular aging are equally distributed. In persons with office normal and elevated BP, vascular stiffness correlates with weight, body mass index and waist circumference (with the increase in arterial rigidity these indicators decrease). CTD is registered among subjects with severe vascular remodeling both in boys and girls.Conclusions. Our data indicate the feasibility of angiology screening for identification of vascular aging phenotypes as a part of preventive measures among young people, regardless of the level of initial office BP. This strategy strategy will contribute to a more differentiated identification of risk groups and more individualized preventive interventions based on vascular aging phenotypes.


2012 ◽  
Vol 93 (1) ◽  
pp. 93-97 ◽  
Author(s):  
E A Ivanova ◽  
O V Plotnikova ◽  
A V Glotov ◽  
V G Demchenko

Aim. Develop a program to prevent health losses in adolescents with connective tissue dysplasia. Methods. Analyzed was the clinical data of 241 adolescents for the period 2006-2009, 113 adolescents formed the main group, 128 - the control group. The statistical rules and standards for determining the grouping signs and group boundaries on the basis of phenotypic and visceral traits of connective tissue dysplasia were taken into consideration. Results. The proposed program for prophylaxis of health loss provides the following stages: informational, analytical, and organizational-executive with an educational component along with curative and preventive measures. The proposed program of prophylaxis provides complete information on the health status of adolescents with connective tissue dysplasia, promotes the reduction of the frequency of progression of signs of dysplasia. Conclusion. The program provides an opportunity to optimize the existing approaches to identifying and assessing factors that influence health, and promote more efficient use of the available resources in the education and healthcare systems.


2019 ◽  
Vol 2 (26) ◽  
pp. 29-33
Author(s):  
E. V. Ikonnikova ◽  
A. G. Stenko ◽  
L. S. Kruglova

The article presents a case of clinical observation of a patient with connective tissue dysplasia. This pathology, as a result of various genetic disorders, includes an extensive group of different systemic conditions, most of which have certain dermatological pathologies as the main or concomitant symptom. We describe the questions of etiology, clinic and diagnosis of connective tissue dysplasia. This clinical case is interesting to practicing dermatologists and cosmetologists in connection with the high appeal of this category of patients for aesthetic help.


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