Interdisziplin?re Leitlinien zur Diagnostik und Therapie der extrazerebralen Amyloidosen

Ohne Zusammenfassung     

Esthesioneuroblastoma in a young child. Case report

Esthesioneuroblastoma is an uncommon malignant neoplasm that arises from olfactory mucosa, often with intracranial extension.A case report of the youngest victim in the literature (2-year-old boy) is presented.     

Dydrogesterone-induced hepatitis and autoimmune hemolytic anemia.

Dydrogesterone, similar to women's natural progesterone, has been used in a wide range of gynecological conditions. Despite its widespread use, dydrogesterone-induced hepatotoxicity and dydrogesterone-induced hemolytic anemia have, to the best of our knowledge, never been reported previously. We describe a case of hepatitis and warm antibody hemolytic anemia due to dydrogesterone.     

Chemokine receptor CXCR4 expression in breast cancer as a potential predictive marker of isolated tumor cells in bone marrow

Interactions between the CXCR4 chemokine receptor in breast cancer cells and the ligand CXCL12/SDF-1α are thought to play an important role in breast cancer metastases. In this pilot study, CXCR4 expression along with other biomarkers including HER2-neu and EGFR, were measured in primary tumor samples of patients with operable breast cancer to test whether any of these biomarkers alone and in combination could indicate breast cancer with high likelihood of metastasizing to bone marrow. Cytokeratin (CK) positive cells in bone marrow were identified by flow-cytometry following enrichment with CK 7/8 antibody-coupled magnetic beads. Primary tumors (n = 18) were stained with specific antibodies for CXCR4, HER2-neu, EGFR, and PCNA using an indirect avidin–biotin horseradish peroxidase method. The majority of the patients had T2/T3 tumors (72%), or lymph node involvement (67%) as pathologic characteristics that were more indicative of high-risk breast cancer. High CXCR4 cytoplasmic expression was found in 7 of 18 patients (39%), whereas 6 of 18 patients (33%) were found to have CK positivity in bone marrow. The median number of CK⁺ cells was 236 (range, 20–847) per 5 × 10⁴ enriched BM cells. The presence of CK⁺ cells in bone marrow was found to be associated with increased expression of CXCR4 alone or in addition to EGFR and/or HER2-neu expression (P = 0.013, P = 0.005, and P = 0.025, respectively) in primary tumors. Furthermore, three patients with high CK positivity (>236 CK⁺ per 5 × 10⁴ enriched bone marrow cells) in bone marrow exclusively expressed high levels of CXCR4 with EGFR/HER2-neu (P = 0.001). Our data suggest that high CXCR4 expression in breast cancer may be a potential marker in predicting isolated tumor cells in bone marrow. CXCR4 coexpression with EGFR/HER2-neu might further predict a particular subset of patients with high CK positivity in bone marrow.     

Is ‘Omega Sign’ anatomical endoscopic enucleation of the prostate technique reproducible?

We aimed to evaluate the learning curve of the surgically standardised ‘Omega Sign’ anatomical endoscopic enucleation (AEEP) of the prostate surgery technique for junior surgeons. This study is a retrospective comparison of cases that underwent AEEP by a mentor surgeon and three junior surgeons who have completed their learning curve. A video-based laser enucleation of the prostate assessment tool (LEAT) composed of 8 steps of the technique was used to assess a senior surgeon and junior surgeons’ surgical compatibility and consistency. The surgeon who defined Omega Sign technique was determined as group 1, and cases by three junior surgeons were identified as group 2. The end points were to assess the reproducibility and repeatability and operative post-operative outcomes of the technique. 55 patients’ videos were rated by five experienced endourologists. There was no significant difference in LEAT scores between the groups among all steps. The most symmetry was found in the 1st and 3rd steps. Inter-rater consistency was also high for each step, with no statistically significant difference between the evaluators. The standardised anatomical ‘Omega Sign’ technique is reproducible for the junior surgeons. The operative steps can be performed with high consistency, and the functional and perioperative outcomes are comparable with the senior surgeon.     

Recurrent priapism in spinal cord injury: A case report

Context: Recurrent priapism is characterized by repetitive episodes of erections which are unrelated to sexual interest or stimulation. It is extremely rare in patients with spinal cord injury (SCI). There are a number of conservative agents used in the treatment.Findings: We describe the case of a 20-year-old male with cervical-5 American Spinal Injury Association (ASIA) Impairment Scale (AIS) grade A tetraplegia as a result of a diving accident 2 years ago. He declared that the recurrent penile erections occurred up to 15–20 times day and night every day and lasted up to 20 min each time unrelated to sexual interest or stimulation. He was prescribed baclofen 10 mg, twice daily. The frequency and duration of erections decreased to 3–5 times/day lasting for about 5 min each and the patient reported a high treatment satisfaction.Conclusion: The presentation of this case is to remind clinicians this rare but distressing condition of recurrent priapism seen in men with complete spinal cord lesions and to summarize the use of medications, most commonly baclofen, to alleviate the condition.     

Structure and evolution of neuronal wiring receptors and ligands

One of the fundamental properties of a neuronal circuit is the map of its connections. The cellular and developmental processes that allow for the growth of axons and dendrites, selection of synaptic targets, and formation of functional synapses use neuronal surface receptors and their interactions with other surface receptors, secreted ligands, and matrix molecules. Spatiotemporal regulation of the expression of these receptors and cues allows for specificity in the developmental pathways that wire stereotyped circuits. The families of molecules controlling axon guidance and synapse formation are generally conserved across animals, with some important exceptions, which have consequences for neuronal connectivity. Here, we summarize the distribution of such molecules across multiple taxa, with a focus on model organisms, evolutionary processes that led to the multitude of such molecules, and functional consequences for the diversification or loss of these receptors.     

CT findings of pulmonary artery aneurysms during treatment for Behçet's disease

OBJECTIVE: The purpose of this study was to evaluate the CT findings of pulmonary artery aneurysms in patients being treated for Beh?et's disease. MATERIALS AND METHODS: Thirteen patients with Beh?et's disease who had a total of 46 aneurysms were included in the study. All patients underwent helical CT before and after treatment. Both initial and follow-up CT scans were evaluated for location, number, and size of aneurysms and for thrombosis and pulmonary parenchyma changes. RESULTS: Thirty-five (76%) of the 46 aneurysms completely disappeared during the 3-42 months of treatment (mean, 21 months), and the remaining 11 aneurysms (24%) became smaller. Both disappearance and regression of aneurysms were preceded by thrombus formation. In 15 initially thrombosed aneurysms (33%), the thrombus increased in size during treatment. After treatment, the thrombus regressed and the pulmonary artery aneurysms disappeared. Thirty-one initially nonthrombosed aneurysms (67%) first became thrombosed during treatment; later, the thrombus regressed and the aneurysm decreased in size. Perianeurysmal consolidation and air-space nodules detected in seven patients disappeared in the early stages of treatment. Mosaic attenuation areas were seen in eight patients and disappeared in seven (88%) after treatment. CONCLUSION: Pulmonary artery aneurysms in Beh?et's disease may become smaller or disappear with medical treatment. Mural thrombotic changes may be observed during the regression of pulmonary artery aneurysms. Helical CT is helpful in the diagnosis and follow-up of aneurysms and thrombosis in Beh?et's disease.