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Biomedical subjects

U Kühner

Publications and source records attributed to U Kühner.

At least 19 recordsLinked to original sources
PubMed1984

[Histiocytosis X--a retrospective analysis of 40 cases with localized or disseminated disease].

A retrospective analysis of 40 cases with histiocytosis X was undertaken to find out the course of primarily localized disease, and the prognosis of children with initially disseminated disease. Bone lesions recurred in nine of 23 children with localized histiocytosis X. In eleven cases other organ manifestations occurred as well; in four cases without bone relapse. After an observation period of 1-14 3/12 years, nine of 22 children in remission suffer from long-term sequelae like diabetes insipidus, convulsion, extrahypothalamic CNS-disease, orthopedic disability, growth retardation, dystrophia adiposogenitalis , and chronic headache. Four of 17 children with disseminated histiocytosis X died. Our results and others from the literature indicate various risk factors to be prognostically significant. 1) age less than 2 years 2) involvement of spleen and/or lung 3) elevated Lahey-score 4) dysfunction of the hematopoietic system, liver, and/or lung 5) histologic feature resembling malignant type 6) no response to therapy 7) severely affected general health. These factors can be evaluated initially. Considering our own experiences and some risk factors we suggest the definition of four risk groups: 1.) localized histiocytosis X of bone, lymph nodes or skin; 2.) disseminated histiocytosis X with benign histologic type and Lahey-score of one or two; 3.) Lahey-score of 3-8; 4.) disseminated histiocytosis X with dysfunction of certain organ systems and/or malignant histology.

Bone Diseases↗
PubMed1980

[Prognostic factors in acute lymphoblastic leukaemia--with special consideration of pre-diagnostic duration of signs (author's transl)].

It is concluded from the prognostic factors of acute lymphoblastic leukaemia, as far as they have become known up to now, such as initial leukocyte count, extent of organ infiltration, etc, that the pre-diagnostic duration of signs, representing a measure of the proliferation activity of the leukaemia cells, could also represent a relevant prognostic characteristic. A check conducted in 90 children treated between 1966--1975 yields a correlation only between a very short pre-diagnostic duration of signs (less than 2 weeks) and the duration of the first complete remission (as well as the survival time). However, even those prognostic factors which are already generally recognised, possess merely limited reliability, because they do not take the individual response to therapy into account. Hence, improvement of prognosis of the course of the disease can be expected only if the continuous decrease of the remaining population of blasts during the early phase of remission can be determined with greater accuracy than before.

Child↗
PubMed1978

[Familial liver cirrhosis in pseudohypoparathyroidism in young children (author's transl)].

Two sisters whose mother had pseudo-pseudohypoparathyroidism, simultaneously developed in infancy pseudohypoparathyroidism with severe liver damage leading to cirrhosis and characterised by "ectoplasmic vacuoles", as well as severe anaemia and thrombocytopenia. As any known metabolic or inflammatory liver disease could largely be excluded, a common genetic defect is assumed as the cause of the combined disorder in calcium and hepatic metabolism.

Anemia↗
PubMed1978

Fluorometric determinations of the relative immunoglobulin content of plasma cells of patients with monoclonal gammopathy.

The relative cytoplasmic immunoglobulin content of fixed plasma cells taken from the bone marrow of five patients with myeloma and five patients with benign monoclonal gammopathy was determined with a microscope fluorometer. In eight of the ten piasma cell populations studied, the distribution of the fluorescence intensities was close to normal. In three of these eight populations a significant difference in the variances of the heavy and light chain fluorescence intensities was found. Variances of heavy and light chain fluorescence intensities were smaller in patients with an immunoglobulin A-type gammopathy than in those with an immunoglobulin G-type gammopathy. No difference was found if normalized relative frequency distribution patterns of heavy or light chain fluorescence intensities of patients with myeloma were compared with those of patients with benign monoclonal gammopathies.

Bence Jones Protein↗
PubMed1977

Differentiation of plasma and myeloma cells of man. Combined planimetric and cytophotometric studies.

Plasmacytoid cells in the bone marrow of 3 patients with myeloma and plasma cells in the bone marrow of a 6-year-old boy with an infectious disease were assessed cytophotometrically, first after Giemsaand second after Feulgen staining. The cell and nuclear surface and the nuclear/cytoplasmic ratio were determined from the number of measuring points. The nuclear DNA content of individual cells was registered and the distribution of DNA within the nucleus was assessed by the distributional error. Both the mean nuclear/cytoplasmic ratio and the distributional error of myeloma cells varied from patient to patient but could not be used to differentiate between normal plasma cells and myeloma cells. It was not possible either to differentiate these cell types by multiplying the mean nuclear/cytoplasmic ratio with the mean distributional error of the nuclear DNA. A strong correlation between cell and cytoplasmic surface area was observed both in normal plasma cells and in myeloma cells.

Bone Marrow↗
PubMed1977

Malignant non-Hodgkin's lymphoma (NHL) in childhood. Retrospective analysis of 34 cases.

Of 47 children with an initial diagnosis of lymphosarcoma, reticulosarcoma or Non-Hodgkin's lymphoma (NHL), 13 had to be excluded at the histologic reevaluation: in 10 an undifferentiated sarcoma, in 2 Hodgkin lymphoma was found; in one patient no definite classification of the tumor was possible. Of the remaining 34 patients there were 26 boys and 8 girls. One patient had a nodular, 33 a diffuse NHL. Of the latter 16 had a Burkitt-type (LB-), 3 a lymphoblastic, convoluted (LC-), 8 a lymphoblastic, "other" (LO-) and 6 a histiocytoid (H-) NHL. Primary localization: abdomen: 13/34; "peripheral" lymph nodes: 9/34; mediastinum: 5/34; nasopharynx: 4/34; subcutis: 2/34; skeleton: 1/34. Twelve of 17 NHL with primary localization in the abdomen or nasopharynx were LB-NHL, 8/14 NHL with primary localization in "peripheral" nodes or mediastinum were LC- or LO-NHL. Only 2/17 NHL with abdominal or nasopharyngeal primary, but 9/14 NHL with "peripheral" nodal or mediastinal primary developed leukemic extension and/or CNS involvement. 6 of 34 patients are living without evidence of disease for 1 1/2+ to 13+ years; 5/34 died but lived for 85, 57, 37, 22 and 22 months; 9/34 lived 6--12 months; 14/34 died within less than 6 months. Patients with abdominal primary either died within 5 months or survived (for 165+, 63+ and 25+ months). Aggressive local therapy (surgery and radiotherapy with approximately 4000 R) may be adequate for strictly localized (stage I) disease, particularly if the primary localization is abdominal. In all other diffuse NHL of childhood an early, aggressive chemotherapy, later combined with radiotherapy to bulk disease and prophylactic CNS-treatment is essential for inducing long-term remissions and, possibly, cures. For prognosis the primary localization appeared to be more important than histology and stage. The most decisive factor, however, is therapy.

Adolescent↗
PubMed1975

[Prognosis and treatment of Hodgkin's disease in children (author's transl)].

Observations on 29 children with Hodgkin's disease confirm that the initial spread, presence or absence of general symptoms, and histological type all have prognostic significance. The results of lymphangiography in 27, and laparotomy with multiple biopsies and splenectomy in 14 children demonstrated that - except in stage IV - both procedures are necessary for adequate classification as to stage of the disease. On the basis of these personal cases (5-year-survival rate of 53%) and published reports a therapeutic schema is suggested which is particularly appropriate for children.

Adolescent↗