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Biomedical subjects

A Marx

Publications and source records attributed to A Marx.

At least 253 records · Page 14Linked to original sources

Disseminated malignant ectomesenchymoma (MEM): case report and review of the literature.

Malignant ectomesenchymoma (MEM) is a rare soft tissue tumor believed to arise from a pluripotent migratory neural crest cell and composed fo both a mesenchymal element and a neuroectodermal element. The authors report the case of an 11-month-old male who presented with a local abdominal MEM and systemic metastases into lungs, liver, bones, and bone marrow. This is the first reported case of an MEM with initial bone marrow dissemination. The tumor consisted of a neuroblastoma component and a mesenchymal component with sarcomatous features. Diagnosis and therapy were complicated by the histological heterogeneity of the tumor, which also influenced the clinical appearance and course in this case. A literature search revealed 15 other evaluated cases that arose in soft tissue and had adequate clinicopathologic data. Complete surgical resection was the mainstay of treatment, and chemotherapy also appeared to be important. In all reported patients (n = 3) with initial metastases or bone marrow dissemination, as in this case, no cure could be achieved. In patients with disseminated MEM, new therapeutic approaches such as high-dose chemotherapy followed by stem cell rescue should be considered, similar to the current strategy in patients with stage VI neuroblastoma or soft tissue sarcoma.

3-Iodobenzylguanidine↗

Malignant ectomesenchymoma of the nose. Case report and review of the literature.

To the best of our knowledge, a malignant ectomesenchymoma of the nose has not yet been reported. This rare tumour is composed of a mesenchymal element--usually a rhabdomyosarcoma--and a neuroectodermal element, often a neuroblastoma or a malignant schwannoma. Most malignant ectomesenchymomas have been found in children (mainly boys), but 5 adult cases, including our case, have also been reported. A 62-year-old man presented with a nasal obstruction on the right side of the nose. Diagnostic nasal endoscopy revealed a mass in the dorsal part of the right nasal cavity, which was treated by endoscopic surgery. The postoperative course was uneventful, and subsequent radiotherapy well tolerated. Since rhabdomyosarcoma is the most common component of this neoplasm, the treatment protocol for rhabdomyosarcoma would have appeared appropriate. This report reviews the literature, summarises the pathological features and possible histogenesis of the tumour, and outlines recommended treatments for malignant ectomesenchymoma.

Combined Modality Therapy↗

The structure of the nucleotide-binding site of kinesin.

Kinesin is a microtubule-based motor protein responsible for anterograde transport of vesicles and organelles in nerve axons and other cell types. The energy necessary for this transport is derived from the hydrolysis of ATP which is thought to induce conformational changes in the protein. We have solved the X-ray crystal structures of rat brain kinesin in three conditions intended to mimic different nucleotide states: (1) with ADP bound to the nucleotide-binding site, (2) with bound ADP in the presence of AIF(-)4, and (3) with ADP hydrolyzed to AMP by apyrase. In contrast to analogous cases observed in GTP-binding proteins or the muscle motor myosin, the structure of kinesin remained nearly unchanged. This highlights the stability of kinesin's ADP state in the absence of microtubules. Surprisingly, even after hydrolysis of ADP to AMP by apyrase a strong density peak remains at the position of the beta-phosphate which is compatible either with a phosphate or a sulfate from the solvent and appears to stabilize the nucleotide-binding pocket through several hydrogen bonds.

Adenine Nucleotides↗

[Ambulatory treatment of chronic alcoholics by tiapride (author's transl)].

Tiapride is the only medication which may have a favorable effect simultaneously on the general behaviour, the tremor, and the digestive symptoms, of alcoholics. This study was carried out in 22 alcoholic patients. Each received, either one intramuscular injection of 100 mg or 200 to 300 mg orally, per day. In eleven cases results were very satisfactory and in nine improvement was very noticeable. The treatment failed in two cases. Anxiety, which was present in 20 cases, disappeared in 18. Tremor was observed in 8 patients and disappeared in all. Tolerance was excellent in all cases. No sedative side effects were observed. This is particularly important for these patients who were continuing their professional activities.

Adult↗

[Meckel-Gruber syndrome].

The lethal Meckel-Gruber-Syndrome can be diagnosed prenatally during ultrasound screening between 16 and 20 weeks of pregnancy. Two case reports of Meckel-Gruber-Syndrome are given, which demonstrate the importance of a reliable ultrasound examination. The results supply the basis for an adequate counseling of the patient with the option of pregnancy termination in case of the lethal syndrome.

Abnormalities, Multiple↗