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Query: UMLS:C0019270 (hernia)
 15,856 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of dysplasia umbilico-fetalis is demonstrated. The malformation comprises a rare and strange reduction anomaly of the feto-umbilical unit, which is caused during early embryonic life (around the 7 mm stage, corresponding to the end of the third week of gestation). The cause of the damage is not known. The folding off of the embryo from the yolk sac and the development of the abdominal stalk are impaired. No abdominal wall is formed, and the umbilicus and umbilical cord are not developed. The abdominal organs are enclosed in a short amnion-mesoderm tube, which is bordered by the retroperitoneum at the fetal side and by the chorionic plate at the placenta side. The umbilical vessels are only a few centimeters long and traverse in the mesodermal layer of the amnion-mesoderm tube. The thin wall of the tube usually ruptures before birth, thus causing an abacterial fibrinoid peritonitis by the chemical irritation of the peritoneum through the constituents of the amniotic fluid. The lower extremities of the fetus reveal varying degrees of reduction deformities. One leg and large parts of the pelvis may be entirely missing, if the side of origin of the extremity is included in the amnion-mesoderm tube. Severe kyphoscoliosis is probably a secondary phenomenon. In addition, malformations of the inner organs occur such as caudal displacement and hernia of the diaphragma, hypoplasia of the lungs, dysplasia of the genito-urinary tract and non-rotation of the gut. The pathophysiology of the rare developmental defect and its secondary implications are discussed with special reference to related malformations such as omphalocele and infraumbilical defect of the abdominal wall.
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PMID:[Early embryonal maldevelopment of the umbilical cord with defect of the abdominal wall and severe body malformations (dysplasia umbilico-fetalis) (author's transl)]. 14 77

In a study of 1266 dogs with cryptorchism from a large clinic/hospital series 8 breeds were found to be at excess risk of the defect and 3 breeds at significantly low risk. Review of the medical histories revealed that hip dysplasia, patellar dislocation, defects of the penis and prepuce, and umbilical hernia were excessively associated with cryptorchism. Testicular tumors were diagnosed 10.9 times more commonly among cryptorchid dogs. The epidemiologic features of canine cryptorchism were compared with those in man. Cryptorchid dogs could be used as models for etiologic research.
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PMID:Cryptorchism and related defects in dogs: epidemiologic comparisons with man. 24 Feb 13

Herniation of the glandular epithelium into the submucosa has been observed in 11 out of 27 cases of chronic ulcerative colitis. Glandular herniation was associated with thickening of the muscularis mucosae, with interruption of the muscularis mucosae by lymphoid follicles, and, in five of the 11 cases, with significant crowding of the glands of the mucosa. This study strongly suggests that sustained contraction of the muscularis mucosae, which has been shown by others to be a major feature of chronic ulcerative colitis, is the prime factor in the formation of downgrowths or herniations of the glandular epithelium into the submucosa. Comparison of the cases in which cancer developed with those where there was glandular herniation led to the conclusion that they are independent associations of chronic ulcerative colitis, and that glandular herniation plays no part in the development of dysplasia or cancer.
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PMID:Herniation of mucosal epithelium into the submucosa in chronic ulcerative colitis. 112 45

Prenatal sonographic findings were reviewed in 30 fetuses with a single umbilical artery (SUA) to determine the reliability of ultrasound for detecting concurrent anomalies. Additional anomalies were identified in 15 fetuses, including 3 fetuses with minor anomalies and 12 fetuses with major or multiple concurrent anomalies. Minor anomalies observed in 3 fetuses included 1 case each of pelvic kidney, unilateral absent kidney, and mild cerebral ventricular dilatation. Major abnormalities detected in 12 fetuses involved a variety of organ systems and included cardiac defects, holoprosencephaly, skeletal dysplasia, hydrocephalus, omphalocele, hydrothorax, enlarged cisterna magna, and diaphragmatic hernia. Clinical and pathologic correlation showed that all fetuses were correctly categorized regarding the presence of other anomalies; none of the 15 fetuses in whom an SUA was considered an isolated finding had a significant anomaly at birth. Chromosome abnormalities were found in 6 of 12 fetuses with major abnormalities but in none of the 18 remaining fetuses. We conclude that prenatal ultrasound can reliably identify major concurrent anomalies in fetuses with SUA. In the absence of additional anomalies, prenatal detection of SUA should not alter obstetric management.
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PMID:Single umbilical artery. Prenatal detection of concurrent anomalies. 205 44

203 patients with juvenile kyphosis, aged 11-16 years, have been subjected to the complex examination, including ++clinico-roentgenologic and physiologic methods. Along with the cardinal signs, common for juvenile kyphosis diagnosis and characteristic of the dystrophic process (wedge distortion, osteoporosis of vertebra bodies and fragmentation of their apophyses, dedifferentiation of the bone structure, height reduction and pathologic restructuring of intervertebral disks following the pattern of fibrosis, presence of discal hernia), the signs of bone ++ dysplasia of spine and skeleton in general should be singled out of the roentgenologic characteristic of disease which determine the variants of pathologic process course and treatment tactics. The presence of dysontogenesis signs in patients with juvenile kyphosis is suggested.
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PMID:[Clinico-roentgenological and physiological evaluation of the spine in patients with juvenile kyphosis]. 207 56

104 patients with progressive forms of funnel chest deformity of the II-III degree with Ehlers-Danlos-Marfan syndrome, Marfan-like phenotype, isolated funnel chest deformity and unclassified funnel chest deformity of the first degree were subjected to synromologic examination. There were detected 10 dysplastic signs, indicating funnel chest deformity progression: anti-Mongol shape of the eyes, arachnodactyly, high palate, Ehlers-Danlos syndrome, floor of the auricle's dysplasia, dolichostenomely, posture disturbance, mitral valve prolapse, umbilical hernia, wide filter. The results of biochemical examination of collagen metabolite--hydroxyproline++ (the first and the second hydroxyproline++ fraction ratio disturbance, decrease of the first hydroxyproline++ fraction percentage, disturbance of direct correlation between total hydroxyproline++ and percentage of the first hydroxyproline++ fraction)--corroborated the clinical data.
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PMID:[Early diagnosis of progression of funnel chest in children]. 209 88

Normal human pulmonary elastic fiber development and development in some pathological conditions were examined using elastic stains by light microscopy, electron microscopy, and immunohistochemistry. In normal development elastic fibers, composed mainly of microfibrils, first appeared around primitive bronchioles at 10 weeks of gestation. As they matured, their appearance became more amorphous, and they extended into the peripheral alveolar walls. Development of elastic fibers was retarded in the hypoplastic lungs of the oligohydramnios syndrome, diaphragmatic hernia, and hydrops fetalis. Elastic development was also retarded in congenital pulmonary lymphangiectasia and in focal areas of lungs with pulmonary dysplasia. Distribution of well-developed elastic fibers was found around the dilated bronchioles and alveoli in cases of congenital cystic adenomatoid malformation and extralobar pulmonary sequestration. Elastic fibers were distributed irregularly and unevenly in the lungs of bronchopulmonary dysplasia and ventilated cases of Wilson Mikity syndrome. In addition, four very immature infants who had progressively deteriorating respiratory function showed an almost total lack of elastic fibers in their alveolar walls.
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PMID:Pulmonary elastic fibers in normal human development and in pathological conditions. 223 57

This report describes a 7-year experience with acute peritoneal dialysis in 31 neonates and infants less than 60 days of age. There were 20 boys and 11 girls, ages 3 to 60 days. Tenckhoff catheters of modified length were placed in the newborn intensive care unit (ICU), pediatric ICU, or surgery suites, and hourly exchanges (20 cc/kg) were started immediately postoperatively. Diagnoses included congenital metabolic disorders (11), acute tubular necrosis (6), postcardiopulmonary bypass with renal failure (5), renal cortical necrosis (5), obstructive uropathy (2), renal agenesis (1), and bilateral renal dysplasia (1). Complications included: peritonitis (4), bowel perforation (1), exit site infection (3), leaking dialysate (4), catheter obstruction (2), inguinal hernias (3), umbilical hernia (1), and retroperitoneal hemorrhage (1). There were 19 deaths (61.3%) from 1 to 90 days postinsertion in this high risk group. The (1), and post liver transplant (1). Effective dialysis (lowering of blood urea nitrogen (BUN) or ammonia, correction of acidosis, decrease in fluid overload) was possible in all cases. Five of the 12 survivors remain on chronic dialysis awaiting renal transplantation. Peritoneal dialysis is effective in the newborn period in the management of metabolic disturbances as well as renal failure. Morbidity and mortality (61.3%) is related to the near-morbid condition of the baby at the time of insertion and the severity of the complex underlying diagnosis often associated with multiorgan failure.
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PMID:Peritoneal dialysis in the first 60 days of life. 229 35

Two daughters of non-consanguineous normal parents had phocomelia of both lower extremities with 4-toed feet. The older sister also had phocomelia of the left upper extremity with 5 finger rays; she died immediately after birth. Autopsy disclosed a congenital diaphragmatic hernia, common mesentery and agenesis of the gallbladder, and normal female genitalia. In addition, the younger sister showed a bony skull defect, diastasis recti, agenesis of the uterus and agenesis or atresia of the vagina, hypoplasia of the sacrum and hypo/dysplasia of the pelvic bones. Her growth and mental development were normal. The patterns of anomalies of the two sisters do not fit into any of the syndromes featuring phocomelia; there was no prenatal exposure to thalidomide or any other possible teratogen.
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PMID:Phocomelia and additional anomalies in two sisters. 233 39

Fetal echocardiography is a well-established technique for the prenatal identification of congenital heart disease. One of the indications for its use is the presence of extracardiac anomalies, as such coexistent defects may have important implications for obstetric and neonatal management. We have reviewed the obstetric and pediatric literature to examine reported associations. If a fetus is suspected to have hydrocephalus, microcephaly, holoprosencephaly, agenesis of the corpus callosum, Meckel-Gruber syndrome, esophageal atresia, duodenal atresia, diaphragmatic hernia, omphalocele, or renal dysplasia, cardiac evaluation should be pursued. Furthermore, echocardiography may be of help in differential diagnosis of some anomalies (for instance, skeletal dysplasias). Maternal diabetes and phenylketonuria, as well as exposure to phenytoin, trimethadione, or isotretinoin, may result in multiple systemic defects, including congenital heart disease.
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PMID:Congenital heart disease and extracardiac anomalies: associations and indications for fetal echocardiography. 293 23


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