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Biomedical subjects

M Hansmann

Publications and source records attributed to M Hansmann.

172 records · Page 10Linked to original sources

[Non-immunologic hydrops fetalis (NIHF)--case report of double partial trisomy 15q and 17q resulting from familial translocation 15/17 and cytogenetic findings in 50 cases with hydrops fetalis].

We report the first case of non-immune hydrops fetalis (NIHF) with trisomy 15q11/17q22.5 resulting from a familial translocation 15/17. Furthermore the cytogenetic findings of 50 cases with hydrops fetalis are presented. Of the 30 cytogenetic analyzable cases 7 showed chromosomal abnormalities (4x45,X; 2x47,XY,+21; 1x47,XY,+13). In every case of NIHF a chromosomal analysis should be performed if possible from different tissues.

Chromosomes, Human, Pair 15↗

[Wolff-Parkinson-White syndrome with paroxysmal supraventricular tachycardias of the fetus and the newborn--case report].

Paroxysmal supraventricular tachycardia causing an intrauterine congestive heart failure was diagnosed in a fetus at 33 week's gestation. After maternal digitalization the tachycardias and the signs of heart failure disappears. Postpartum a Wolff-Parkinson-White syndrome was recognised by electrocardiography. The paroxysmal supraventricular tachycardias of the newborn was terminated abruptly by intravenous application of propafenon. Recurrences were prevented by the oral administration of propafenon.

Adult↗

[Ultrasonography studies of the fetal hip].

Recent advances in ultrasound technology, especially high-resolution sonography, now permit visualisation of the fetal hip development. Since early diagnosis and treatment of congenital hip dislocation significantly influence the outcome of the disease, ultrasound screening of the newborn infants showed to be the most effective method. In our study assessment of the fetal hip has been performed on the prenatal level in order to provide basic knowledge on fetal hip development from 14th to 40th weeks of gestation. From the 20th week of gestation bony structures are clearly visible and allow the assessment of acetabular configuration. Soft tissue and chondral acetabular components are recognizable from the 21st week. For pre- and postnatal comparative measures Graf's method and standard documentation is used.

Female↗

[Standard values for the intrauterine ultrasonography of hip joint development].

Although etiology and pathogenesis of congenital dislocation of the hip (CDH) is of broad interest in orthopaedics, basic questions still remain obscure. Advances in high-resolution ultrasound technology now permit investigation of the fetal hip joint in vivo. Standard values of intrauterine hip development have been established by investigating 146 fetuses (141 pregnancies) in vivo by ultrasound. Standard values describe the development from 20th weeks of gestation until birth. Prenatal Alpha- values suggest influence of intrauterine posture and spatial fetal conditions on hip development. Alpha-angles of 59.7 (SD 8.9) are achieved at the end of pregnancy. These angles meet standards of Graf's calculations for the postnatal hip development. Standard values of intrauterine hip development are of basic interest for interpretation of hip joints in premature children.

Adult↗

[De Barsy-Moens-Dierckx syndrome: unusual course in a neonate].

We report about a premature infant with a De Barsy-Moens-Dierckx-syndrome, which is a rare cutaneo-oculo-cerebral malformation-syndrome. It is defined by the combination of a progeroid aspect, cutis laxa, growth retardation, cornea clouding, mental retardation and athetoid movements. Furthermore, the reported case showed a remarkable thermolability and suffered from generalised seizures resistant to therapy. Despite extensive sonographic examinations the prepartal diagnosis seems to be very difficult.

Abnormalities, Multiple↗

Fetal parvovirus B19 infection and meconium peritonitis.

A 33-year-old primigravida at 26 weeks gestation presented with fetal hydrops and fetal anemia following prior parvovirus B19 infection. The fetus required two intrauterine transfusions of packed red cells. At 35 weeks gestation, a cesarean section was performed for obstetric reasons. As a consequence of a prenatal bowel perforation, the neonate developed meconium peritonitis, for which she needed laparotomy. This case demonstrates that there may be an association between intrauterine parvovirus infection and meconium peritonitis, the latter possibly caused by vascular injury in fetal life.

Adult↗

Nonimmune hydrops fetalis with galactosialidosis: consequences for family planning.

At the 28th week of gestation a hydrops fetalis was first detected by ultrasound. At birth a generalized hydrops with Hurler-like craniofacial dysmorphism, hepatosplenomegaly and a moderate dystostosis multiplex was noted. High urinary excretion of oligosaccharides and a severe deficiency of neuraminidase and of beta-galactosidase in cultured skin fibroblasts could be found. Thus, a rare early infantile type of galactosialidosis was diagnosed. The patient died at the age of 3 months because of cardiac failure. The consanguineous but otherwise healthy parents received genetic counselling for further pregnancies and have been informed about the possibility of prenatal diagnosis. In view of this possibility, the parents decided to have more children. In the second pregnancy a severe combined enzyme deficiency had been detected and the pregnancy interrupted. In the third pregnancy prenatal diagnosis revealed normal fetal enzyme activities. It resulted in a healthy female child and in the fourth pregnancy reduced but still in the heterozygote level enzyme activities had been found, a healthy boy was born.

Family Planning Services↗

[A possible "immunologic" origin of idiopathic non-immunologic hydrops fetalis and initial results of preventive immunotherapy of subsequent pregnancies].

The cause of the nonimmune hydrops fetalis remains unsettled in spite of greatest efforts. Under an immunologic point of view of pregnancy as a successful course of an allograft it seems possible, that the idiopathic NIHF can be caused by an immunologic disorder in the meaning of a host-versus-graft-reaction. From 300 cases with prenatal diagnosed NIHF 37 (12.4%) could be classified after exclusion of all other causes as idiopathic and in 25 patients, as well as in 25 age- and parity-paired controls a differentiation of HLA-antigens and a determination of lymphocytotoxic antibodies using the NIH-, Prolonged incubation- and Cold-Complement Dependent-Cytotoxicity-Test (CoCoCy-Test) were performed. In cases with idiopathic NIHF the percentage of parents sharing 4 or 5 HLA-antigens was increased with 4/25 to 2/25 compared to the control group. In women with idiopathic NIHF the incidence of lymphocytotoxic antibodies was decreased, in dependence of the test-system used between 28 and 68% in the NIHF group and 24 and 80% in the control group. The percentage of women without lymphocytotoxic antibodies was increased in the NIHF group with 72% to 52%, in opposite to the control group in none of the patients a higher cytotoxicity with a lysing rate of more than 75% could be detected. In 4 cases with idiopathic NIHF, an increased paternal histocompatibility and a decreased incidence and percentage of lymphocytotoxic antibodies an immunotherapy was performed, in order to induce maternal blocking antibodies.(ABSTRACT TRUNCATED AT 250 WORDS)

Antilymphocyte Serum↗

[Sialidosis and galactosialidosis as the cause of non-immunologic hydrops fetalis].

Two cases of non-immunological hydrops fetalis (NIHF) presenting with massive ascites are reported; in both patients an oligosaccharid-pattern in the urine typical for sialidosis resp. galactosialidosis was found. The cerebral sonography of both patients showed streaky echo enhancement in the region of the thalamostriatal vessels, which was interpreted as calcification of the vessels. The courses of the patients were characterised by recurrent infections, hepatosplenomegaly and myoclonus. Relevant literature reports on a large variability in the clinical appearance of oligosaccharidoses. The diagnosis of sialidosis is confirmed in cultured fibroblasts by the deficiency of alpha-N-acetylneuraminidase and, in case of galactosialidosis by the additional lack of beta-galactosidase. The precise diagnosis in NIHF is of increasing interest for prenatal diagnostic as well as for neonatological management.

Brain↗