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Biomedical subjects

F Schuster

Publications and source records attributed to F Schuster.

At least 19 recordsLinked to original sources

[Scrotal diverticulum of the urinary bladder -- rare cause of an inguinal hernia].

INTRODUCTION: Massive herniation of the bladder through the inguinal canal is a rare entity. We report on a patient with a large inguinoscrotal herniation of the urinary bladder commonly referred as "scrotal cystocele". Diagnostic means and therapeutic strategies are discussed. CASE REPORT: After laparoscopic repair of a bilateral inguinal hernia, a 60-year-old man described a persistent right groin mass that varied in size with the degree of bladder distension. Diagnostic evaluation revealed a large bladder diverticulum extending into the right scrotum. Treatment consisted of inguinal resection of the bladder diverticulum, the peritoneal sack and closure of the hernia. CONCLUSION: Inguinal herniation of a large bladder diverticulum has to be considered in the differential diagnosis of inguinal hernias, especially in patients with obstructive symptoms of the bladder outlet.

Diverticulum↗

[Intravesical adjuvant chemotherapy for superficial bladder cancer -- results of a survey in Saxony].

For patients with superficial bladder cancer, adjuvant intravesical chemotherapy or immunotherapy with Bacillus Calmette-Guerin (BCG) is recommended in national and international guidelines. We analyzed whether the recommended therapeutic regimens are used in daily urological practice. Questionnaires concerning the adjuvant intravesical therapy were sent to 152 urologists in the German Federal State of Saxony. Of the surveyed physicians, 134 practiced in an outpatient medical facility and 18 in a hospital. Of the questionnaires, 73 (48.02 %) were returned and evaluated. An adjuvant intravesical therapy after transurethral bladder tumor resection was performed in every second patient (median value 50.07 %). The majority of the urologists (79.4 %) treated the bladder tumors with intravesical chemotherapy or BCG depending on tumor stage and grade of malignancy. Chemotherapeutic agents or BCG was exclusively used in 13.6 % and 4.1 % of treated patients, respectively. Chemotherapeutic agents were predominantly applied up to the primary tumor stage T1 and malignancy grade G2. In cases with recurrent T1 bladder tumors of G2 or higher grade of malignancy, BCG was the main agent for intravesical treatment. In patients with recurrent T1G3 tumors, the majority of urologist (57.1 %) preferred another therapeutic regimen than intravesical instillation. Only 23.2 % of the urologists believed that intravesical BCG is superior to chemotherapeutic agents. These data demonstrate that adjuvant intravesical instillation with chemotherapeutic agents and BCG is well established in urological practice. In contrast to the recommendations of national and international guidelines, chemotherapeutic agents are more frequently used in cases with a high risk of progression.

Administration, Intravesical↗

[Spontaneous rupture of a lumbar artery. A rare etiology of retroperitoneal hematoma].

A ruptured lumbar artery is an uncommon lesion for a retroperitoneal hematoma. This report describes a 77-year-old man with bladder carcinoma who developed a spontaneous retroperitoneal hemorrhage during anticoagulation therapy for an artificial cardiac valve. The diagnosis was suggested by computed tomography scan and confirmed with angiography. Successful treatment consisted of occluding bleeding arteries by transcatheter embolization.

Aged↗

T-cell-depleted peripheral blood stem cell transplantation for alpha-mannosidosis.

Alpha-mannosidosis (alpha-mannosidosis) is a lysosomal storage disease characterized by accumulation of oligosaccharides in various tissues leading to symptoms such as coarse facial features, dysostosis multiplex, hearing disabilities, mental developmental delay and skeletal involvement (dysostosis multiplex). Without treatment, the severe infantile onset form of this autosomal recessive disease leads to progressive neurodegeneration and sometimes to early death. Stem cell transplantation has been shown to be an effective treatment. In the five patients published so far, correction of skeletal abnormalities and improvement of neuropsychological capabilities have been observed. We report the first patient who received a T-cell-depleted peripheral blood stem cell transplantation (PBSCT) for alpha-mannosidosis. The diagnosis of alpha-mannosidosis was made at the age of 14 months. At the age of 24 months, he underwent PBSCT with T-cell depletion by CD34-positive selection from his HLA phenotypically identical mother. Conditioning was carried out with busulfan (20 mg/kg), cyclophosphamide (200 mg/kg), OKT3 and methylprednisolone. The patient is alive and well 27 months after PBSCT and has made significant developmental progress. The pattern of urinary oligosaccharides has returned to almost normal. CD34-positive-selected PBSCT is a feasible option to reduce risk for GVHD for these patients.

Anti-Inflammatory Agents↗

Colonic mucosal vasoactive intestinal peptide receptors in malformations of the enteric nervous system are reduced compared with morphologically normal innervated colon.

Vasoactive intestinal peptide (VIP) is the most important peptidergic transmitter in intestinal relaxation. VIPergic nerves are reduced in aganglionosis (AG). Corresponding findings in intestinal neuronal dysplasia (IND) are sparse. It is unknown whether superficial mucosal VIP receptors are reduced in AG, IND, or hypoganglionosis (HYP) compared to concentrations in morphologically normal innervated colon (MNIC). Cryostat sections from 38 colonic biopsies (23 patients with AG, IND, or HYP, 15 with MNIC) were incubated with radioactive iodinated (125)I-VIP. Receptors were analyzed by autoradiography. Radioactive-marked receptors trigger the precipitation of metallic silver as silver grains within a photographic emulsion. Grains were quantified by image analysis, calculating the percent covered cell surface. Statistical analysis was done by Mann-Whitney and Kruskal-Wallis tests (significance #E5/E5#<0.05). VIP receptors covered 4.31% of the cell surface in MNIC. The values were significantly reduced in AG (2.72%; #E5/E5#=0.012) and IND (2.93%; #E5/E5#=0.008). The two HYP biopsies showed the lowest values (1.83%). Aganglionic colon could be distinguished from healthy proximal segments and IND from habitual constipation. In AG and IND, even the superficial mucosal VIPergic innervation seems to be impaired. The reduction of mucosal VIP receptors in developmental faults of the enteric nervous system may thus be an indicator of a sensomotor disturbance.

Child, Preschool↗

Allogeneic bone marrow transplantation for active Epstein-Barr virus-related lymphoproliferative disease and hemophagocytic lymphohistiocytosis in an infant with severe combined immunodeficiency syndrome.

A 5-month-old male presented with fever, hepatosplenomegaly, leukocytosis with atypical lymphoblasts, anemia and thrombocytopenia. Severe combined imunodeficiency syndrome (T-, B+, NK+), B lymphoproliferative disease and hemophagocytic lymphohistiocytosis triggered by Epstein-Barr virus (EBV) were diagnosed. As his clinical situation deteriorated rapidly, BMT was performed with unmanipulated marrow stem cells from his EBV-positive HLA-identical sister after conditioning with dexamethasone (1.75 mg/kg/day), cyclophosphamide (114 mg/kg) and etoposide (10 mg/kg), with no immunosuppression given post transplant. Engraftment occurred on day 6 with explosive proliferation of donor CD8(+) T cells. The patient died 3 days later from acute respiratory distress syndrome. Autopsy revealed full donor engraftment and no signs of hemophagocytic lymphohistiocytosis or B lymphoproliferative disease. Thus, transplanted T cells can expand very rapidly within days after BMT and clear EBV lymphoproliferative disease and hemophagocytic lymphohistiocytosis.

Acute Disease↗

Successful treatment of gut-caused halitosis with a suspension of living non-pathogenic Escherichia coli bacteria--a case report.

UNLABELLED: In up to 90% of cases, severe halitosis is a result of gastrointestinal or orolaryngeal problems. This case study reports on a girl with bad breath caused by increased formation of malodorous intestinal gases (halitosis), which could be successfully treated with a suspension of living non-pathogenic bacteria Escherichia coli. CONCLUSION: in unclear cases of bad breath, an increased formation of intestinal gases should also be considered.

Breath Tests↗

Griscelli syndrome: report of the first peripheral blood stem cell transplant and the role of mutations in the RAB27A gene as an indication for BMT.

Griscelli syndrome is characterized by partial albinism with variable immunodeficiency. Two different gene loci are responsible for this rare, autosomal recessive disease: the myosin Va gene and the RAB27A gene. As recently reported, only patients with mutations of the RAB27A gene suffer from immunodeficiency and hemophagocytic lymphohistiocytosis. Thus, only patients who suffer from the Griscelli syndrome with mutations of the RAB27A gene should receive BMT/PBSCT, which is the only curative therapy. Due to the risk of early relapse or severe infections, BMT/PBSCT should be carried out as soon as possible; if patients do not have HLA-identical family members, valuable time may be lost by searching for an HLA-identical unrelated donor. We report the first peripheral blood stem cell transplant (PBSCT) with T cell depletion in a 6-month-old girl with Griscelli syndrome, and a deletion of the RAB27A gene. The donor was her phenotypically HLA-identical mother. Conditioning included busulfan, VP16 and cyclophosphamide. The patient was transfused with 15.4 x 10(6)CD34-positive cells/kg and 17.6 x 10(3) CD3-positive cells/kg recipient weight. Three months after the transplant, a curable lymphoproliferative syndrome occurred. 26 months after the transplant, the patient is doing well with stable mixed chimerism (52% donor cells).

Bone Marrow Transplantation↗

Transient expression of a tumor-specific single-chain fragment and a chimeric antibody in tobacco leaves.

To evaluate the expression of different forms of a tumor-specific antibody in plants, we adapted a recently described Agrobacterium-mediated transient expression system. A recombinant single-chain Fv antibody (scFvT84.66) and a full-size mouse/human chimeric antibody (cT84.66) derived from the parental murine mAb T84. 66 specific for the human carcinoembryonic antigen were engineered into a plant expression vector. Chimeric T84.66 heavy and light chain genes were constructed by exchanging the mouse light and heavy chain constant domain sequences with their human counterparts and cloned into two independent plant expression vectors. In vivo assembly of full-size cT84.66 was achieved by simultaneous expression of the light and heavy chains after vacuum infiltration of tobacco leaves with two populations of recombinant Agrobacterium. Upscaling the transient system permitted purification of functional recombinant antibodies from tobacco leaf extracts within a week. His6-tagged scFvT84.66 was purified by immobilized metal affinity chromatography and cT84.66 by protein A affinity chromatography. Sufficient amounts of recombinant antibodies were recovered for detailed characterization by SDS/PAGE, Western blotting, and ELISA.

Animals↗

Towards molecular farming in the future: using plant-cell-suspension cultures as bioreactors.

Plant-suspension cells are an in vitro system that can be used for recombinant protein production under carefully controlled certified conditions. Plant-suspension cells can be grown in shake flasks or fermenters to produce secondary metabolites, like vincristine and vinblastine, and to produce recombinant proteins after transformation. This review article focuses on discussing the generation of transformed suspension-cell lines expressing recombinant proteins, like antibodies, and recombinant-protein downstream processing and purification.

Bioreactors↗

Animal model Balamuthia mandrillaris CNS infection: contrast and comparison in immunodeficient and immunocompetent mice: a murine model of "granulomatous" amebic encephalitis.

Balamuthia mandrillaris and several species of Acanthamoeba are pathogenic "opportunistic" free-living amebas which cause granulomatous encephalitis (GAE) in humans and animals. The granulomatous component is negligible or absent, particularly in immunocompromised individuals. GAE is an "opportunistic" infection, usually seen in debilitated, malnourished individuals, in patients undergoing immunosuppressive therapy for organ transplants, and in Acquired Immunodeficiency Syndrome (AIDS). From around the world 156 cases of GAE have been reported from 1956 through October 1, 1995, 59 (26 in the USA) of them caused by B. mandrillaris, at least seven of them in AIDS patients. The present study was designed to compare and contrast the susceptibility of infection, the rate of infectivity and the histopathological changes within the CNS between the mutant, severe combined immunodeficient mice (SCID) infected with B. mandrillaris and the normal immunocompetent BALB-C mice. The SCID mouse is severely deficient in B and T lymphocytes, therefore lacking immunoglobulin and cell-mediated immunity. This mouse is also prone to develop early T cell lymphomas. One thousand amebic trophozoites and cysts of B. mandrillaris were intranasally and intraperitoneally inoculated in both strains in mice. Seventy percent of the intranasally inoculated SCID mice died due to CNS infection. Amebic trophozoites and cysts were found within CNS parenchyma without inflammatory response. Death occurred from 2 to 4 weeks after inoculation. By contrast only 10 percent of the intranasally inoculated BALB-C mice died with CNS infection showing the characteristic features of GAE. None of the intraperitoneally inoculated mice developed amebic infection. The SCID and BALB-C mice were logical models to study the structural alterations within the CNS of B. mandrillaris infection. This animal model recapitulates with excellent degree of fidelity several aspects of the pathogenesis and histopathological features of free-living amebic infection in human beings.

Amebiasis↗

Influence of disposable ('Conchapak') and reusable humidifying systems on the incidence of ventilation pneumonia.

The contamination of disposable ('Conchapak') and reusable humidifying systems and their influence on the incidence of pneumonia was studied in 116 patients requiring continuous mechanical ventilation therapy. The water reservoirs of 11 (15.9%) of the 69 disposable systems became colonized, but all reusable systems were found to be sterile. In four of the 11 samples, the organisms isolated corresponded with those cultured from tracheal secretions several days before. Ventilator-associated pneumonia occurred in 36 (31.0%) of the patients, but there was no statistically significant difference in the incidence of pneumonia between the patients treated with the disposable or the reusable humidifying systems. Gram-negative bacteria were the predominant organisms isolated from tracheal aspirates of patients who developed ventilator-associated pneumonia. These results suggest that disposable humidifying systems do not influence the rate of ventilator-associated pneumonia in mechanically ventilated patients.

Adult↗