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2.
Arkh Patol ; 54(3): 25-31, 1992.
Article in Russian | MEDLINE | ID: mdl-1444843

ABSTRACT

153 foetuses were studied obtained after the artificial abortion for genetical indications. The scheme of the material investigation is given. Isolated, systemic and multiple defects were found in 39.0, 12.1 and 48.9%, respectively, and the contribution of the syndrome forms in the multiple developmental disturbances was 60.9%. The interruption of the pregnancy in 2% of cases was assessed as unfounded: as a consequence of hyperdiagnosis and in cases of treatable defects. The frequency of the discrepancy between prenatal and pathology diagnoses was 31.8% including hyperdiagnosis of the defect (1.3%), nosological disagreement (13.3%), the lack of diagnosis of the additional defects detectable during II trimester by present ultrasound methods (17.2%). The efficacy of the work of the prenatal diagnostic centre is, according to the authors, the number of justified interruptions of pregnancy (98% in this study).


Subject(s)
Abnormalities, Multiple/pathology , Congenital Abnormalities/pathology , Prenatal Diagnosis/methods , Abortion, Therapeutic , Embryonic and Fetal Development/physiology , Female , Humans , Pregnancy , Pregnancy Trimester, Second , Reproducibility of Results
3.
Arkh Patol ; 52(4): 3-9, 1990.
Article in Russian | MEDLINE | ID: mdl-2201276

ABSTRACT

Unsolved problems of modern teratology are discussed. The monitoring of the congenital malformation incidence is one of the variants of evaluation and control of the mutation pressing in the population. The investigation of human foetuses obtained in artificial abortions may be very helpful in this respect. The investigation of the phenotypical manifestations of malformations in the human prenatal ontogenesis and the use of the results for the creation of notion on the malformation morphogenesis seems to be perspective. The definition of the tissue dysplasias and their classification (dystopia, dyssynchronia, hamartomas) are given. The issue of the tissue malformations during the postnatal development is not similar. They may be asymptomatic, or to disturb the function of the organ concerned, or to predispose to chronic inflammation or neoplastic growth.


Subject(s)
Congenital Abnormalities , Congenital Abnormalities/embryology , Congenital Abnormalities/pathology , Fetus/embryology , Fetus/pathology , Hamartoma/embryology , Hamartoma/pathology , Humans , Morphogenesis , Mutation , Research/trends , Syndrome
5.
Arkh Patol ; 50(10): 61-3, 1988.
Article in Russian | MEDLINE | ID: mdl-3219090

ABSTRACT

The authors describe a rare case of this disease that has clinically manifested in a 12-days-old boy. At the age of 1.5 months a right-side nephrectomy for a supposed Wilms' tumor has been performed. Histologic examination has diagnosed congenital xanthogranulomatous pyelonephritis. The authors put forward a hypothesis on the existence of primary forms of this condition not associated with persistent infections or urinary tract obstructions.


Subject(s)
Pyelonephritis, Xanthogranulomatous/congenital , Humans , Infant , Kidney/pathology , Male , Nephrectomy , Pyelonephritis, Xanthogranulomatous/pathology , Pyelonephritis, Xanthogranulomatous/surgery
6.
Arkh Patol ; 48(12): 63-7, 1986.
Article in Russian | MEDLINE | ID: mdl-2952103

ABSTRACT

Structure of the surgical and biopsy specimens from 9765 children aged from 1 day to 14 years was studied. According to the data obtained at the pediatric autopsy laboratory, organs of the alimentary, reproductive, urinary systems, skin, lungs and bones are most frequently subjected to an intravital histologic study. Most common among general pathologic processes are: inflammation (66,3%), tumours (13,5%) and congenital malformations (4,7%). The incidence of benign tumours is 81,5%, of malignant ones--5,2%, and that of tumour-like processes--13,3%. In most cases (49,9%) the tumours are of dysontogenetic origin. Such congenital malformations as atresia and stenosis on different esophageal levels, as well as aganglionosis, renal and ureteral dysplasias, ovarian dysplasia and hypoplasia occur most frequently. Inflammatory processes, mainly nonspecific ones, localize in the vermiform appendix, lymph nodes, bones, kidneys, lungs. Hyperplastic processes, immune reactions and reactive states (lymph nodes, tonsils) are not infrequent.


Subject(s)
Autopsy , Pathology, Clinical , Adolescent , Biopsy , Child , Child, Preschool , Epidemiology , Humans , Infant , Infant, Newborn , Republic of Belarus
7.
Arkh Patol ; 41(8): 40-5, 1979.
Article in Russian | MEDLINE | ID: mdl-114149

ABSTRACT

An analysis of phenotypic manifestations of Wolf-Hirschhorn syndrome from the 60 cases most thoroughly described in the literature and 3 own observations was done. Most characteristic malformations of this syndrome were shown to include coracoid nose and hypertelorism, coloboma of the eyes, hypospadia, aplasia, hypoplasia and polycystosis of the kidneys, dystopia and dysplasia of the cerebellar gyri, shortening of H2 field of the Ammon's horn with imparied orientation of its neurons, sacral sinus, and retarded bone maturation.


Subject(s)
Abnormalities, Multiple/genetics , Chromosome Aberrations/pathology , Chromosomes, Human, 4-5 , Brain/abnormalities , Chromosome Deletion , Chromosome Disorders , Eye Abnormalities , Face/abnormalities , Genitalia, Male/abnormalities , Humans , Infant, Newborn , Male , Sacrum/abnormalities , Syndrome
9.
Arkh Patol ; 38(6): 16-22, 1976.
Article in Russian | MEDLINE | ID: mdl-1021059

ABSTRACT

Data of the literature (200 section observations) and those of 10 authors' own observations on the syndrome of trisomy with relation to chromosome 18 are presented. The pathologoanatomic diagnosis of Edwards' syndrome can be established without an investigation of chromosomes on the basis of a complex of congenital defects present in the newborn. The main of them are: prenatal hypoplasia, dolichocephaly, microgenia, concha auriculae drawn in the horizontal plane, flexor position of bones, shortness and thickness of the great toe and protruding heel, thickness and distortion of convolution of the dorsal lip of the olivary nucleus, hypoplasia of the cerebellum, heterotopia of the piriform Purkinje cells into the white matter of the cerebellum and foci of nondifferentiated cells in the white matter of the great hemispheres; complex cardiac defects--Meckel's diverticulum, fused kidneys (horseshoe- or L-shaped) with small cysts in the renal cortex; and the presence of 10 or more arches on fingers and toes in combination with proximal or intermediate localization of axial triradius.


Subject(s)
Chromosome Aberrations/pathology , Chromosomes, Human, 16-18 , Trisomy , Abnormalities, Multiple/pathology , Chromosome Disorders , Female , Humans , Infant , Male , Skull/abnormalities , Syndrome
12.
Arkh Patol ; 37(2): 3-12, 1975.
Article in Russian | MEDLINE | ID: mdl-1131054

ABSTRACT

The article presents an analysis of world literature on cysplasia of the kidneys (KD). The authors consider as morphological characteristics the following signs: combination of primitive ducts, tubules, glomeruli, collecting tubules, cysts and immature mesenchyme with foci of cartilage and muscles. An attempt is made to systematize individual forms of DK according to the degree of morphological changes (simple and cystic), extension (focal, segmental and total, mono- and bilateral) and localization (cortical, medullar and cortico-medullar or mixed) of the process. From the group of cystic DK primary (aplastic and multicystic variants) and secondary (obstructive) ones were singled out. The problems of morphology, incidence, complications, concomitant anomalies, causal and formal genesis of DK were also considered.


Subject(s)
Kidney/abnormalities , Abnormalities, Multiple/diagnosis , Female , Humans , Hypertension, Renal/etiology , Kidney/embryology , Kidney/pathology , Kidney Cortex/abnormalities , Kidney Diseases, Cystic/pathology , Kidney Medulla/abnormalities , Male , Polycystic Kidney Diseases/pathology , Pyelonephritis/complications , Teratogens , Urogenital Abnormalities
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