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Biomedical subjects

F Henschke

Publications and source records attributed to F Henschke.

17 recordsLinked to original sources

Clinical and ultrastructural findings in three patients with geleophysic dysplasia.

Geleophysics dysplasia, a rare disorder with autosomal-recessive inheritance, is characterized by short stature with a "happy-looking" facial appearance. Nonskeletal findings, particularly in an advanced stage, include hepatosplenomegaly and valvular cardiopathy. Based on the clinical picture and the detection of lysosome-like inclusions in hepatocytes, the underlying cause of the condition is considered to be a storage defect in the metabolism of glycoproteins. The clinical course, with progressive worsening of the condition favors this hypothesis. We report on 3 further cases, in which light and electron microscopic studies of iliac crest biopsies and cultured skin fibroblasts provided additional evidence that geleophysic dysplasia represents a lysosomal storage disease. The additional discovery of storage vacuoles in chondrocytes and skin fibroblasts strongly suggests that the condition is a generalized storage defect. To date, it has not yet been possible to identify the presumed biochemical defect in the metabolic pathways of glycoproteins.

Bone and Bones

Multiple chromosomal changes and karyotypic evolution in a patient with myelofibrosis.

Several subclones were identified in unstimulated peripheral blood cells from a patient with chronic myeloproliferative disease, which was classified as myelofibrosis by morphologic terms. These subclones were characterized by an unusual number of different karyotype anomalies. Some of the more complex chromosomal rearrangements could be clearly defined by fluorescence in situ hybridization. Chromosome arms involved in clonal aberrations were 1q, 3p, 6p, 7q, 11q, 13q, 15q, 17q, 18p, and 20q. Reconstruction of karyotype evolution was attempted by karyotypic analysis of 100 metaphase spreads each in two separate investigations.

Aged

Monoclonal antibodies EBU-141 (CDw75) and EBU-65 allow reliable distinction between mature and pre-B-cell tumors in suspension and on tissue sections.

Two new monoclonal antibodies, EBU-65 and EBU-141, were raised by immunization with plasma cell line U-266. Both antibodies strongly react with B lymphocytes in immunofluorescent staining as well as on paraffin-embedded sections. More than 200 leukemias and lymphomas were tested, and for both antibodies reactivity was found only with "mature" B-cell tumors but not with precursor B-cell leukemias. None of the non-B-lineage hematolymphatic tumors tested was stained by EBU-141 or EBU-65. A subpopulation of T lymphocytes particularly present in nonmalignant pleural effusions was detected by EBU-65 additionally. Although EBU-141 was clustered as CDw75 and EBU-65 as "unique," a close relationship of the staining pattern was found and both antibodies react with a sialyltransferase. In particular, CDw75 antibody EBU-141 was demonstrated to be very useful for immunophenotyping of B-cell neoplasias, while EBU-65 reacted with most multiple myelomas and a subgroup of "activated"-appearing T cells.

Animals

Penetration of the active metabolite of nabumetone into synovial fluid and adherent tissue of patients undergoing knee joint surgery.

The concentration of 6-methoxy-2-naphthylacetic acid (6-MNA) in plasma, synovial fluid, synovial tissue and fibrous capsule tissue was determined in an open study with 20 patients scheduled for knee joint surgery after oral treatment with nabumetone under steady-state conditions. 6-MNA is the principle metabolite of the prodrug nabumetone arising from an extensive first-pass metabolism in the liver. Patients suffering from rheumatoid arthritis (n = 12) or osteoarthritis stage III or IV (n = 8) received a daily dose of nabumetone 1 g in the evening starting 4 days prior to surgery. On day 1 an additional loading dose of nabumetone 1 g was given in the morning. At the time of surgery (day 5), blood, synovial tissue and fibrous capsule tissue were taken simultaneously. The samples were analysed by high performance liquid chromatography. After 4 days of treatment mean 6-MNA concentration in plasma was 40.76 mg/L, in synovial fluid 34.79 mg/L, in synovial tissue 19.33 mg/g and in fibrous capsule tissue 11.43 mg/g. Under steady-state conditions mean synovial fluid levels of 6-MNA were higher than after administration of a single dose and, in common with levels in synovial tissue, persist in a range sufficient for in vitro cyclo-oxygenase inhibition.

Administration, Oral

Synovial fluid involvement in null cell acute lymphoblastic leukemia diagnosed with monoclonal antibodies.

Rheumatic symptoms in patients with leukemia are not uncommon. Often they delay correct diagnosis and therapy. We describe a patient presenting with oligoarticular joint disease in whom immunological analysis of synovial fluid (SF) led to the detection of leukemic cells in the joint. Moreover, analysis with a variety of monoclonal antibodies established the diagnosis of acute lymphoblastic leukemia of null cell type with phenotypically identical malignant cells present in the bone marrow, peripheral blood and SF. Our investigations demonstrate that analysis with monoclonal antibodies is helpful in characterizing joint involvement in patients with leukemia.

Acute Disease

[Reversible panmyelopathy following captopril treatment].

In a 46-year-old female patient suffering from renal hypertension due to polycystic kidney degeneration with creatinine values between 3 and 5 mg/dl leucopenia developed 3 weeks after beginning antihypertensive therapy with the angiotensin-converting-enzyme inhibitor captopril. On continuation of treatment signs of haemorrhagic diathesis appeared 7 months later. Cytology and histology led to the diagnosis of panmyelopathy which, from the clinical findings and the close patient follow-up, was to be regarded as captopril-induced. Bone-marrow regeneration could be shown 6 months after discontinuing the drug. In renal insufficiency, captopril treatment should be monitored using regular haematological tests and the drug withdrawn when leuco- or thrombocytopenia occurs.

Bone Marrow

[Relevance of computed tomography to the fine-structure analysis of bone. A comparative radiological study].

Computed tomography, a relatively young X-ray imaging modality, has so far been mainly employed for the differentiation and densitometry of soft tissues. By modification of the image reconstruction algorithm spatial resolution was improved significantly such that imaging of fine structures and of high contrast boundaries became possible. The image characteristics of the high-resolution computed tomograms were investigated on petrous bones and lumbar vertebral bodies. In the petrous bone, an exact anatomic reconstruction of the bony structures of the inner ear is achieved. In vertebral bodies, imaging of spongiosa structures with 2 mm slice thickness and thereby an early diagnosis of osteoporosis are possible.

Bone and Bones

[Cadmium-induced vertebral-column ankylosis in whitefish ].

In two healthy and two diseased whitefish (Coregonus Wartmanni) taken from Lake Constance (FRG), ankylosis of the vertebral column was investigated both roentgenologically and histologically. Subsequent to the collapse and necrosis of the "residual" spinal cord within the intervertebral spaces, the outside edges of the vertebral bodies come into direct contact. The compression and tensile forces that occur to an increased extent as a result of the instability, lead not only to a remodelling of the vertebral bodies, but also to the formation of spondylotic osteophytes at the edges of the vertebrae and, as a result of periosteal stimulation, to the development of cellular hyaline cartilage, which fills the intervertebral spaces. Finally, as a result of perichondral ossification, a bony ankylosis develops. The humping of the spine of the fish due to the stiffening and shortening of the vertebral column, is accompanied by a restriction in the animal's freedom of movement. Muscular atrophic processes and disordered food uptake give rise to poor growth and a reduction in the weight of the diseased fish. These remodelling processes in the spine resulting from instability are specific to the periosteum and may be equated with the changes seen in man in spondylosis deformans. The possible cause of this vertebral column ankylosis is cadmium poisoning. The accumulation of this heavy metal obviously leads primarily to an irreversible toxic degeneration of the cells of the chorda dorsalis.

Animals

[The structure of the spongy bone in lumbar vertebrae and the neck of the femur. A comparative analysis of the age-dependent remodelling process (author's transl)].

In 114 autopsy cases of both sexes, aged between 31 and 97 years and without bone disease, the influence of mechanical forces on the age-dependent remodelling of the spongy bone was determined in the 3rd and 5th lumbar vertebrae and the neck of the femur. For this purpose contrasty X-ray images of 100 mu thick polished bone sections were analysed using the LEITZ texture analysis system. The volumetric density and the surface density are highest in the neck of the femur, lowest in the 3rd lumbar vertebra, and almost as low in the 5th lumbar vertebra. The volumetric density decreases with increasing age by about one-third in all three bones. Correspondingly, the surface density also decreases in the lumbar vertebrae by one-third, but only by 18% in the neck of the femur. The specific surface reveals no age differences in the lumbar vertebrae, but increases by 19% in the neck of the femur. The constancy of the specific surface in the lumbar vertebrae can be explained by the fact that compensatory hypertrophy of the remaining trabeculae takes place. This compensatory growth of bone does not occur in the neck of the femur. The age-dependent decrease in spongy bone manifests itself in a residual spongiosa structure that depends on compressive forces in the case of the lumbar vertebrae and bending forces in the neck of the femur.

Adult

[Tympanoplasty with human dura mater preserved in cialit (author's transl)].

In 26 cases allogenetic human dura mater, conserved in CialitTM 1:5000, was used to close perforations of the eardrum instead of commonly used autogenetic fascia of the temporal muscle. In preceding animal studies, it had been found that "Cialit dura" acts as guideline and stimulates the development of new host connective tissue. In 25 human ear drums the perforations healed up without any problems. The healing process took times of up to 8 weeks. Only in one case a new perforation was found. The moderate flexibility of "Cialit dura" proved as a special advantage during the surgery procedure. Human dura is easily to acquire and its conservation is simple and cheep. Therefore it can serve as a grafting material for tympanoplasty available at any time and in any size. For very large transplants and a lack of postoperative treatment the prolonged healing period could be of some disadvantage. For normal sized transplants, however, there seems not to be a difference in healing time between the commonly used fascia and the "Cialit dura".

Animals

[6-methoxy-2-naphthylacetic acid level in plasma, synovial fluid and adjacent tissue in patients with rheumatoid arthritis or gonarthroses after a 4-day therapy with nabumetone (Arthaxan)].

The concentration of 6-methoxy-2-naphthyl acetic acid (6-MNA) in plasma, synovial fluid, synovial tissue and fibrous capsule tissue was determined in an open study with 20 patients scheduled for knee joint surgery after oral treatment with nabumetone (Arthaxan) under steady state conditions. 6-MNA is the principal metabolite of the prodrug nabumetone arising from an extensive first-pass metabolism in the liver. The patients suffering from rheumatoid arthritis (n = 12) or osteoarthritis stage III or IV (n = 8) received a daily dose of 1 g nabumetone nocte starting 4 days prior to surgery. On day 1 an additional loading dose of 1 g nabumetone was given in the morning. At the time of surgery (day 5) simultaneously blood and synovial fluid was aspirated and after medial opening of the knee joint biopsies of synovial tissue and fibrous capsule tissue were taken. The samples were analysed employing HPLC. After 4 days of treatment mean 6-MNA concentration in plasma was 40.76 micrograms/ml, in synovial fluid 34.79 micrograms/ml, in synovial tissue 19.33 micrograms/g and in fibrous capsule tissue 11.43 micrograms/g. Under steady state conditions mean synovial fluid levels of 6-MNA were higher than after application of a single dose.

Adult