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PubMedDate not supplied

The experience of black parents/caretakers with the births and care of a child with profound congenital defects.

The reaction of Black families to the birth and care of a baby with profound congenital defects was researched using twenty case studies. It was found that the families went through stages of the grieving process, that they shifted from the Western/Christian viewpoint to the traditional viewpoint in their struggle to cope and that they find the existing services grossly inadequate.

Adaptation, Psychological↗
PubMed1980

Congenital defects of the inner ear in association with meningitis.

Three patients presented with meningitis associated with congenital defects of the inner ear, consisting of a cyst-like cochlea and a short, wide horizontal semicircular canal. The findings were easily identified on routine skull radiographs, alleviating the need for further studies and leading to treatment of the defect.

Adolescent↗
PubMed1980

[State of the blood microcirculatory vessels of the heart in congenital defects].

The condition of hemomicrocirculation vessels in congenital heart disease was studied by silver nitrate impregnation of thick sections. Adaptive and pathological alterations in the microcirculatory bed of the heart in various developmental anomalies are described. The role of adaptive alterations of terminal vessels in an abnormal heart in the blood supply to hypertrophic myocardium is shown. It is suggested that pathological alterations in the microcirculatory bed of an abnormally for-med heart are associated with its functional over loading and weakening of coronary circulation. It is concluded that degenerative, focal destructive, and sclerotic processes in hypertrophic myocardium in congenital heart disease may depend on disturbances of microcirculation in the heart.

Arterioles↗
PubMedDate not supplied

[Monitoring of congenital defects in children of Nizhniy Novgorod Region].

Presented in the paper are the results of monitoring of congenital defects (CD) among newborns in Nizhniy Novgorod Region in 1999-2003. All CD were subject to routine registration at maternity homes, pediatric clinics and dissection rooms of hospitals. The mean CD prevalence made, during the mentioned period, 15 cases per 1000 newborns, which is in keeping with the CD figures registered in other regions of Russia. The share of some nosological CD forms is in line with the results of the International Registry.

Adolescent↗
PubMed1990

[Birth weight of children born without congenital defects. I. Birthweight percentiles by gestational age].

We studied the birthweight by gestational age and sex from a population of 14,097 liveborns without congenital defects who were monitored by the Spanish Collaborative Study of Congenital Malformations (ECEMC). We have calculated the curves of percentiles for gestational age in males and females. As our sample size is very large, and it includes births from almost the whole country, we think these curves are enoughly representative of the Spanish population, fitter than the Denver's (1) which are widely utilized. On the other hand, we stress our attention in making curves of birthweight for malformed and not malformed babies separately.

Birth Weight↗
PubMed1991

[The pathomorphology of the intra- and extraorganic neural apparatus of the heart in congenital defects].

Different components of the heart sympathetic and parasympathetic innervation were studied in 22 autopsy cases of congenital heart defects. Central efferent neurons in the lateral horns of the upper thoracic segments of the spinal cord and in the dorsal nucleus of the medulla oblongata are damaged stronger than the neurons of the upper neck and stellate sympathetic nodes and the heart intramural ganglia this resulting in the selective destruction of the preganglionic nervous conducting elements. It is suggested that the disturbance of the connection between the heart nervous structures and the central nervous system exerts a negative influence on its function.

Autopsy↗
PubMed1979

Zebra-like hyperpigmentation in an infant with multiple congenital defects.

An unusual pattern of zebra-like hyperpigmentation was observed in a black male infant with congenital defects that included an atrial septal defect, dextrocardia, auricular atresia, deafness, and growth retardation. The hyperpigmentation was strikingly linear and symmetrical and followed a dermatomal distribution, with involvement of the trunk and extremities. Results of histological, histochemical, and electron microscopic studies were normal except for an increase in the number of melanocytes in the bands of hyperpigmentation. This pigmentary anomaly appears to be transient and has faded to 50% of its original intensity. A review of the literature has not disclosed any similar cases.

Abnormalities, Multiple↗
PubMed1993

[Amputation and prosthesis design in severe congenital defects of the lower extremity (case reports)].

The author presents an account of three patients with severe congenital defects of the lower extremities who relied on a long-term basis on complex prosthetic appliances. In the mentioned patients the author amputated part of the malformed extremity in such a way as to preserve the "plantar" stump and it was thus possible, when solving the technical aspects of the appliance, to improve also the cosmetic covering of the defect.

Adolescent↗
PubMed1978

[Possibilities of intracardiac phonocardiography in congenital defects with left-right shunt].

The author shares his experience from intracardial phonocardiography of 48 patients with congenital heart defects with leftright shunt. The importance of the method is stressed upon, particularly in cases with interauricular and interventricular defect, not only for the diagnosis of the disease but for the differential diagnosis in difficult cases as well. The characteristic murmurs are presented, recorded intracardially in the three of the basic congenital heart defect with left-right shunt--interauricular defect, interventricular defect, and open Botal's duct.

Adolescent↗
PubMed1987

Congenital defects of the bovine central nervous system.

Congenital brain defects in cattle are usually obvious at birth, but detection may depend on the nature and extent of the defect. The cause may be genetic or environmental, or the cause may be unknown. Many central nervous system defects are inherited as a simple autosomal recessive trait and may have a significant economic impact on purebred and commercial cattle operations. A common structural defect of genetic nature is internal hydrocephalus. A functional defect of simple autosomal recessive inheritance is bovine progressive degenerative myeloencephalopathy. The most significant teratogenic agents causing central nervous system defects are prenatal viral infections such as bovine virus diarrhea and bluetongue viruses. Many other defects of the central nervous system are reviewed.

Animals↗
PubMedDate not supplied

[Bone marrow transplantation in congenital defects of immunity].

BMT can cure several congenital immunological defects: if in these disease the engrafting is easier, the GVH reactions are more frequent and severe. The possibility to deplete from T lymphocyte the marrow before infusion, has overcame this difficulty. From 1968 183 BMT have been performed in Europe on patients with SCID (70 from HLA-identical donor, 113 from HLA-nonidentical donor). The survival after 2 years is 76% in the first group, and 56% in the second group (100 marrows have been T-depleted with different techniques). Strict isolation procedures before the transplant are very important to achieve good results. The possibility to treat different immunodeficiency With BMT are also discussed.

Bone Marrow Transplantation↗
PubMedDate not supplied

[Preoperative and postoperative management of patients with congenital defects of the external and middle ear].

Laser techniques introduction in preoperative and postoperative management of patients operated for congenital defects of the external and middle ear promotes retention of the graft and activation of regeneration, improves treatment results. More advanced methods of care for the postoperative cavity increase quality and duration of hearing-improving operative effects, shorten hospital stay, minimize risk of long-term complications.

Child↗
PubMed1994

Congenital defects in the llama.

When faced with the question of what you should do as a veterinary practitioner, when presented with a congenital defect in a llama, one answer is to seek qualified help from your state veterinary diagnostic laboratory. But most importantly, encourage the owners of such animals to report the defect to their llama association, including pedigree information. The better we understand the pathogenesis of such defects, the better we will be able to control their incidence.

Animals↗
PubMed1992

Congenital defect in sinusoidal smooth muscles: a cause of organic impotence.

We report 2 cases of primary impotence due to a congenital defect in the compliance of the sinusoidal spaces secondary to fibrosis and atrophy of the smooth muscles. Both patients were young adults at presentation. Diagnosis of this rare entity was achieved by penile Doppler ultrasound and cavernosometry/cavernosography of the cavernous bodies. Both patients underwent placement of a penile prosthesis, during which biopsy samples of the cavernous tissue were obtained, and diagnosis was confirmed by light and electron microscopy.

Adult↗
PubMed1976

Effect of a congenital defect in hemopoiesis on myeloid growth and the stem cell (CFU) in an in vivo culture system.

W/Wv mice with congenitally defective CFU proliferation and their normal, congenic littermates were used as hosts for diffusion chamber (DC) implants. CFU growth in implanted allogenic CF1, or congenic +/+ marrow was significantly greater in W/Wv than in control hosts. When W/Wv mice were "cured" of their hemopoietic defect, CFU proliferation in the DCs decreased, but not to the control level. These observations have provided evidence for humoral control of CFU growth related to a genetic stem cell defect. Diffusion chamber myelopoiesis was also enhanced in W/Wv hosts. In comparison with their congenic controls, W/Wv mice were neutropenic and had decreased numbers of marrow myeloid elements. Thus, a humorally mediated feedback related to a defective myelopoiesis in the hosts might have accounted for increased DC myelopoiesis. However, a "spillover" effect from increased stem cell growth has not been excluded.

Anemia, Macrocytic↗
PubMed1980

[Congenital defects of the diaphragm in siblings. Two case reports (author's transl)].

In two different families each time two siblings head a congenital defect of the diaphragm, in 3 cases of the posterolateral type, in 1 case an almost complete aplasia of the diaphragm. Usually, the occurrence of defects of the diaphragm is sporadic. However, familial occurrence has been reported in 15 cases. Multifactorial inheritance is most likely the explanation for this. With this hypothesis a risk of 2% can be calculated for the recurrence of this malformation in a family with already one affected child.

Diaphragm↗