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Biomedical subjects

U Jaeger

Publications and source records attributed to U Jaeger.

103 records · Page 6Linked to original sources

[Clinical symptoms and therapy of the hereditary angioneurotic edema (author's transl)].

Sera of 333 patients showing clinical symptoms of "Quincke edema" have been tested for their deficiency of C1 esterase inhibitor. The diagnosis "Hereditary Angio Neurotic Edema" (HANE) could be stated in 39 cases, i.e. 11.9%. Clinical manifestations consisted of peripheral edema (80%), abdominal colics (77%), and facial edema (72%). Edema of the larynx have been observed in 51% of the cases. 10 patients have been treated with purified C1 esterase inhibitor during the attack as a substitutional therapy, and 10 patients received danazol during their attackfree intervals. Both of these treatments were therapeutically successful.

Angioedema↗

[Distribution of praevalent morphological symptoms of the oral complex in 600 Jena school children in dependence of age and sex (author's transl)].

In this study praevalence and stage of some selected symptoms within the orofacial complex were investigated in 600 normal children living in Jena (300 boys and 300 girls). The first problem of this study consisted in registering the biological variability of 6 symptoms (bit abnormality, protrusion of the lower lip, diastema, palatal height, size of the tongue, fissured tongue) in a normal group, and secondly to find correlations between these symptoms. The results are listed for the age groups according to sex. The supposed causes that may lead to the development of these symptoms are discussed. Our results were compared with those of the literature. Difficulties and incompletion are involved in recording of the mentioned symptoms and their value of diagnosis.

Adolescent↗

Comparison of karyotype analysis and RT-PCR for AML1/ETO in 204 unselected patients with AML.

The chromosomal translocation t(8;21) (q22;q22) is often associated with acute myeloid leukemia with maturation (AML-M2) and can be detected by a reverse transcription-polymerase chain reaction (RT-PCR) for the AML1/ETO fusion mRNA. We investigated the prevalence of t(8;21) and AML1/ETO in 204 unselected patients with AML and compared the results of cytogenetic analysis with these of RT-PCR. Fifteen of 204 AML patients (7.4%) showed a t(8;21) in karyotype analysis. In 17 of 204 patients (8.3%) AML1/ETO was detected by RT-PCR. All patients who had a t(8;21) in conventional karyotyping also showed the gene rearrangement in molecular analysis, including one patient with a three-way translocation t(5;8;21). AML1/ETO was also detected in two AML patients lacking the t(8;21) cytogenetically. One had a normal diploid karyotype bone marrow (BM) at diagnosis; she has now been in CCR for 12 months. The second patient showed a complex chromosomal anomaly involving chromosome 21, but without a typical 8;21 translocation (BM in relapse). He died in relapse after an overall survival of 60 months. These data indicate that the results of karyotyping and RT-PCR are not completely identical, and molecular biology identifies approximately an additional 5-10% of AML1/ETO positive cases. The clinical relevance of our findings will have to be evaluated with larger patient numbers.

Acute Disease↗

[Sagittal Cobb-angle measurements in scoliosis with MRI whole spine imaging].

AIM: A newly developed MR procedure allows imaging of the whole spine in coronal and sagittal planes. We studied the use of total spine MR imaging in measuring sagittal Cobb angles in scoliosis. METHOD: 64 patents with idiopathic scoliosis (mean age 18.1 years, 35 thoracic, 20 double major, and 9 lumbar curves) and 27 patients without scoliosis were consecutively examined. The MR images were acquired in the supine position. The sagittal Cobb angles were measured between T4-T12 and between T12-L5. RESULTS: For the group of the thoracic and double major scoliosis the mean sagittal Cobb angle (T4-T12) was 13 degrees and for the group without scoliosis 23 degrees, which was a significant difference (p < 0.01, Mann Whitney-U-test). There was a negative correlation between the sagittal Cobb angles (T4-T12) and the lateral, thoracic curves. The mean sagittal Cobb angle (T12-L5) of the group with lumbar and double major curves was 35 degrees, which was not a significant difference when compared to 37 degrees of the non-scoliotic group. CONCLUSION: Using total spine MR imaging the lordotic aspect of the thoracic deformation in scoliosis can be reliably measured. Because of the absent radiation exposure the sagittal MR reconstructions could be used as an additional imaging in monitoring scoliosis.

Adolescent↗

[Calculation of 3-D deformity in scoliosis by MRI of the total spine in two perpendicular reconstructed planes].

AIM: Scoliosis is a spinal deformity that is more complex and does not exist in one plane only. There have been many attempts to analyse three-dimensional spinal deformity, however, these procedures necessitate higher radiation doses. METHOD: In this study we define angles according to the Cobb Definition. By means of trigonometrical evaluation, 3D calculation of spinal deformity is demonstrated using MRI of the total spine in two reconstructed perpendicular planes. 3D spinal analysis was performed on 41 female and 7 male patients with scoliosis. RESULTS: 79 angles were measured by using the Cobb angle in reconstructed coronal plane of MRI of the total spine and, in addition, by using our method. The scoliosis Cobb angles ranged from 11 - 59 degrees (mean: 23 degrees +/- 9 degrees ), the real angles ranged from 12 - 70 degrees (mean: 32 +/- 14 degrees ). There was a poor correlation between Cobb angles and the 3D calculated angles (r = 0.37; p < 0.0001). CONCLUSION: Our method enables us to determine the real angle of scoliosis and to avoid techniques with any radiation risk for the patient.

Adolescent↗

[Butterfly vertebrae and scoliosis: Alagille syndrome].

INTRODUCTION: Alagille syndrome is a dominantly inherited disorder affecting the liver (arteriohepatic dysplasia), the heart, the eyes and the face. Butterfly vertebrae in the thoracic and lumbar spine are detectable in 50 to 80 per cent of the patients; most of them remain asymptomatic. The management of progressive cholestasis in early childhood is the main aspect of therapy. CASE: A 14-year-old female patient with Alagille syndrome was referred to our hospital for examination of a right thoracic scoliosis. Magnetic resonance imaging verified butterfly deformity in thoracic vertebrae 2, 4, 6, 7, 8, 10, 11, and lumbar vertebra 3. Asymmetries of thoracic vertebrae 6, 8 and especially, 7 were responsible for the scoliosis. CONCLUSION: The highly variable expression of these typical characteristics in mildly affected patients can cause some difficulties. When diagnosing a scoliosis with asymmetric butterfly vertebrae, an Alagille syndrome should be considered, especially if the patient shows a liver dysfunction.

Adolescent↗

[Brace effect in scoliosis in the sagittal plane - an MRI study].

AIM: Using magnetic resonance (MR) imaging we studied the brace effect in scoliosis in the sagittal plane. METHOD: In 38 patients with idiopathic scoliosis (mean age 13.4 years) MR total spine imaging was carried out to investigate the immediate effect of bracing in the sagittal plane. There were 19 thoracic, 13 S-shaped and 6 lumbar scoliosis. On conventional radiographs the mean Cobb angle of the thoracic curves was 31 degrees and of the lumbar curves 26 degrees. MR imaging was performed in the supine position with and without the brace in direct sequence. On the sagittal MR projection the Cobb angle was measured between T 4 and T 12 and between T 12 and L 5. RESULTS: On the coronal MR images the mean correction with brace was 23 % of the thoracic curves and 29 % of the lumbar curves. The mean, sagittal Cobb angle (T 4 - T 12) was 14 degrees without brace and 12 degrees with brace. For the lumbar curves the mean sagittal Cobb angle (T 12 - L 5) was 32 degrees without brace and 31 degrees with brace. In the paired t-test these differences were significant. CONCLUSION: Using MR total spine imaging the brace effect in scoliosis could be depicted in the sagittal plane. In the thoracic spine a correction of the lordotic deformity could not be observed.

Adolescent↗

[Ulnar groove syndrome following elbow injuries].

For a large scaled test 52 patients with anterior transposition of the ulnar nerve and 62 patients with elbow injuries were examined by questionnaire, physical examination and electroneurography. In conformity with existing literature 24 patients out of 52 suffering from cubital tunnel syndrome had an elbow trauma previously. To our great surprise in seven patients out of 46 with elbow injuries a cubital tunnel syndrome could be found for the first time. The cubital tunnel syndrome appears to be a frequent complication of elbow injuries. Besides the well known fractures of the medial epicondyle and pericondylar fractures leading to cubital tunnel syndrome, in our study fractures of the head of the radius and processus coronoideus were found quite often. Patients having typical anamnesis and complaints should be checked by electroneurography in order to permit a quick operation and to prevent incurable damages of the nerve. The diagnosis leading to operation of the elbow should however be made with greatest care. In case the operation is unavoidable, the ulnar nerve should be thoroughly checked and anterior transposition should be carried out.

Adult↗

Prospective monitoring of minimal residual disease in acute myeloid leukemia with inversion(16) by CBFbeta/MYH11 RT-PCR: implications for a monitoring schedule and for treatment decisions.

Minimal residual disease in patients with acute myeloid leukemia (AML) with inversion(16) can be monitored by CBFbeta/MYH11 RT-PCR. While the association between molecular remission (MR) in bone marrow (BM) and peripheral blood (PB) and long-term clinical remission (CR) seems to be established, there are insufficient data on the kinetics of CBFbeta/MYH11. We have performed a prospective study in order to generate a reasonable and sufficient schedule for PCR-monitoring. 11 patients with AML and inversion (16) in complete hematological remission have been prospectively monitored by CBFbeta/MYH11 RT-PCR in their BM and PB during an observation period of 7 to 67 months (median 32 months). Patients were followed during consolidation chemotherapy with repetitive cycles of high-dose Ara-C and after autologous or allogeneic stem cell transplantation in 2nd CR or refractory AML. MR never coincided with achievement of CR but occurred between 2 and 8 months after hematological remission. All patients in continuous CR were PCR-negative after 1-8 (median 4) months. Two patients relapsed despite MR for 10 to 15 months. Molecular relapse preceded hematological relapse by 3 to 5 months. Three out of four patients who were not in MR after 8 months relapsed. Allogeneic stem cell transplantation was able to eradicate minimal residual disease in 4/4 patients. In 2 patients a temporary reconversion to PCR-positivity was reversed by reduction of immunosuppression. 1 patient did not become PCR-negative until compete withdrawal of immunosuppression. We suggest that BM and PB should be examined after the last consolidation treatment. In case of MR, PB should be examined every 1 to 2 months and BM examination should be done only in case of PCR-positivity in PB in order to confirm the molecular relapse and to identify an impending cytogenetic and/or hematological relapse. CBFbeta/MYH11 RT-PCR monitoring is able to predict relapse 3 to 5 months prior to overt hematological relapse, offers a window of opportunity for preemptive therapy of molecular relapse and confers implications for immunotherapy in the setting of allografting.

Acute Disease↗

Association of CD38 antigen expression with other prognostic parameters in early stages of chronic lymphocytic leukemia.

The expression of the surface molecule CD38 on B cell chronic lymphocytic leukemia (B-CLL) cells has recently been described as a prognostic marker for patient survival. We have analyzed CD19/CD38 expression in 81 patients with predominantly early stages of B-CLL (69 Binet A, seven Binet B, five Binet C). Sixty-two patients (77%) had less than 30% CD38+/CD19+ cells, while 19 (23%) had > or = 30%. There was a significant association between Binet stages (A vs. B+C, p < 0.0001), Rai stages (0-II vs. III+IV, p < 0.001) and CD38 expression, confirming the published cut-off level of 30%. A particularly strong association between CD38 expression was found with soluble CD23 (sCD23) levels of > or = 2000 U/ml (p < 0.0001) and beta2-microglobulin (beta2 MG) serum levels of > or = 3 mg/l (p < 0.0001) indicating that CD38 is a marker of tumor mass as well as disease progression. A borderline association was found with lymphocyte doubling time (LDT) < 12 months (p = 0.05) due to low patient numbers, while there was no association with age, sex or immunoglobulin deficiency. Discordant results were obtained in a number of patients: 10 of 69 patients (14%) with Binet A had a CD38 > or = 30% while three of seven patients with Binet B had a CD38 < 30%. In these two subgroups CD38 and other prognostic factors gave discrepant results. Due to the early stage and short median observation time (12 months. range 1-24 months), calculations concerning patient survival were not performed. However, our data show a strong association between CD38 and other known prognostic factors. The results also suggest that this factor is not always reliable in Binet A patients.

ADP-ribosyl Cyclase↗