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  • 1
    ISSN: 1432-1440
    Keywords: Hyperparathyroidism ; Bone turnover ; Osteitis fibrosa ; Microradioscopy ; Metabolic bone disease ; Hyperparathyreoidismus ; Knochenumsatz ; Fibroosteoklasie ; Mikroradioskopie ; Metabolische Osteopathien
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung 1. Beim primären Hyperparathyreoidismus (PHPT) ist nach heutigem Wissen immer mit einer Skelettbeteiligung zu rechnen. Während bei leichtem PHPT lediglich der Knochenumsatz gesteigert erscheint, führt die schwere Form zur Osteodystrophia fibrosa (=Fibroosteoklasie, FO). Mit Hilfe der Mikroradioskopie (achtfache Lupenbetrachtung feinkörniger, folienloser Materialprüfungsfilme) von Hand-Röntgenaufnahmen können beide Reaktionsformen des Skeletts diagnostiziert werden: die Umbausteigerung an einer vermehrten Striation der Metacarpalia-Corticalis und die Fibroosteoklasie an subperiostalen Usuren der Phalangen. 2. In der vorliegenden Arbeit wurden die präoperativen Befunde von 65 Patienten und die postoperativen von 39 Patienten mit operativ gesichertem primärem Hyperparathyreoidismus verwertet. Striationsgrad (StG) und Usurierungsgrad (UG) wurden mit den biochemischen Parametern des PHPT, der alkalischen Phosphatase als Index der Osteoblastentätigkeit, dem Hydroxyprolin als Größe für den Knochenumsatz, dem radioimmunochemisch bestimmten Parathormon, dem Serum- und Urincalcium, dem Serumphosphor und der Phosphat-Clearance verglichen und auch der Nierensteinhäufigkeit gegenübergestellt. Des weiteren konnte der postoperative Verlauf von Usurierungs- und Striationsgrad verfolgt werden. 3. Bei 60% aller PHPT-Patienten fanden sich pathologische Veränderungen im Röntgenbild, und zwar erhöhter Striationsgrad und/oder erhöhter Usurierungsgrad. In 41,5% der Fälle konnte die Diagnose des PHPT allein aus dem Röntgenbild gestellt werden. Usurierungsgrad und Striationsgrad einerseits korrelierten gut mit alkalischer Phosphatase, Hydroxyprolin und Parathormon andererseits. Dagegen bestand keine Beziehung zwischen der Fibroosteoklasie und der Anamnese einer Nephrolithiasis. Nach Korrektur des PHPT verschwanden die subperiostalen Usuren immer, während bei der Hälfte der Operierten die intracorticalen Striationen über Jahre persistierten, obwohl sich der Knochenumsatz normalisiert hatte. Damit scheint der PHPT nicht nur den endostalen Knochenverlust zu verstärken, sondern kann auch ein irreversibles intracorticales Knochendefizit hinterlassen.
    Notes: Summary 1. In primary hyperparathyroidism an increased bone turnover is seen, accompanied by osteitis fibrosa (=fibroosteoclasia, FO) in severe forms of the disease. Both types of bone reaction may be detected by microradioscopy of X-rays of the hand, extensive striation of metacarpal cortical bone indicating increased bone turnover and subperiosteal resorption of phalanges pointing to FO. 2. In the present study 65 patients with proven PHPT were evaluated before and 39 after operation. Microradioscopy was combined with biochemical assessment of hyperparathyroidism including alkaline serum phosphatase (aPh) as an index of osteoblastic activity, hydroxyprolin excretion (HyPro) reflecting bone turnover, immunoreactive parathyroid hormone levels (PTH), serum calcium (SCa), urinary calcium (UCa), serum inorganic phosphorus (SP) and clearance of phosphate (CP). A comparison was made with the incidence of renal stone disease and the degrees of metacarpal striation (StG) and subperiosteal resorption (UG) were followed after operation. 3. Preoperative X-rays of 60% of the PHPT subjects showed increased StG and/or UG, and in 41,5% the diagnosis of PHPT was possible from the X-ray findings only. There existed a significant correlation between StG and UG on one hand and aPh, HyPro and PTH on the other. No correlation, either positive or negative, was seen between FO and the incidence of renal stones. After surgery, subperiosteal bone lesions disappeared in all patients, while intracortical striations persisted in half of the subjects despite the normalised bone turnover. Thus, primary hyperparathyroidism may not only lead to endosteal bone loss but to an irreversible intracortical bone deficit as well.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1440
    Keywords: Whipple's disease ; Chronic interstitial nephritis ; Granulomas ; Boeck's disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Report is given on a 68-year-old man who suffered primarily from progressive weight loss and repeated episodes of fever and arthralgia. Later, liver dysfunction and renal insufficiency developed. Liver and kidney biopsics disclosed granulomatous hepatitis and nephritis. Because of the morphologic and clinical findings, the diagnosis of Boeck's disease was made. Shortly before death, diarrhea developed. Autopsy revealed a massive systemic involvement in Whipple's disease proven by light and electron microscopy and immunofluorescence. Tuberculoid and epitheloid cell granulomas and isolated giant cells were found in addition to the biopsy findings in skeleton muscles, the small intestine, lymphnodes and bronchi. At autopsy, the kidney showed chronic interstitial nephritis. The literature of kidney involvement in Whipple's disease is reviewed. This is the first case with granulomatous interstitial nephritis and chronic renal insufficiency in an inadequately treated Whipple's disease.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1076
    Keywords: Key words Chronic relapsing ; Thrombotic thrombocytopenic purpura ; Von Willebrand factor-cleaving protease ; Prophylactic treatment
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The pentad of thrombocytopenia, haemolytic anaemia, mild renal dysfunction, neurological signs and fever, classically characterizes the syndrome of thrombotic thrombocytopenic purpura (TTP). TTP usually occurs in adults but also children have been described with this condition. The disorder may take a relapsing course, termed chronic relapsing TTP (CRTTP), which although very rare, may also begin in childhood. Deficiency of a recently identified enzyme, the von Willebrand factor (vWF)-cleaving protease, seems to play a major role in the development of TTP. We report on a 3-year-old boy with a dramatic but typical clinical course of CRTTP. At the time of diagnosis, neurological deficits and multiple cerebral infarctions had already occurred. In plasma, vWF-cleaving protease was completely absent, both during acute TTP and in remission. There was no protease inhibitor detected. Regular infusions of fresh frozen plasma were successfully given for replacement on a prophylactic basis. Conclusion Assay of von Willebrand factor-cleaving protease helps to diagnose a form of thrombotic thrombocytopenic purpura which may be managed by prophylactic treatment with fresh frozen plasma.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Clinical & experimental allergy 18 (1988), S. 0 
    ISSN: 1365-2222
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: The effect of tryptase, a neural protease released from human lung mast cell secretory granules, on purified human Hageman Factor(Factor XII) was examined. No increase in Hageman Factor enzymatic activity was detected after incubation with tryptase at 37°C; activation of Hageman Factor by bovine trypsin served as a positive control. Furthermore, pre-incubation of Hageman Factor with tryptase did not diminish the subsequent activation of Hageman Factor by trypsin. Polyacrylamide gel electrophoresis was also performed to show that incubation with tryptase does not alter the molecular weight of Hageman Factor. Therefore, tryptase neither activates nor destroys human Hageman Factor.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Annals of hematology 78 (1999), S. 483-484 
    ISSN: 1432-0584
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-0584
    Keywords: Key words Low-molecular-weight ; Heparin ; Trousseau's syndrome ; Thromboembolism ; Neoplasms
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  We report the case of a 76-year-old man with recurrent thromboses despite oral anticoagulation with phenprocoumon and low-grade chronic disseminated intravascular coagulation. Workup revealed a bronchial carcinoma (NSCCL) with hilar and mediastinal lymph node metastases. The clinical condition was consistent with Trousseau's syndrome. Based on reports in the literature, the therapy was changed to intravenous unfractionated heparin (UFH), which was effective in controlling the thrombotic coagulopathy. For practical reasons, despite a lack of evidence of its effectiveness in Trousseau's syndrome, therapy with UFH was changed to subcutaneous low-molecular-weight heparin (LMWH, nadroparine) in therapeutic doses of 100 IU/kg body wt. 12 hourly. On an outpatient basis, five chemotherapy cycles were administered, and after metastases of the brain had been detected radiotherapy was initiated. Following 7.5 months of progressive neoplastic disease the patient died. He had remained free of thromboembolic complications under continued LMWH therapy during the last 6.5 months of his life. LMWH might be a convenient alternative to the established therapy with UFH in Trousseau's syndrome.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1435-2451
    Keywords: Hyperparathyroidism ; Renal stone disease ; Hypercalcemia ; Hyperparathyreoidismus ; Nephrolithiasis ; Hypercalciämie
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei 100 Patienten mit primärem Hyperparathyreoidismus (pHPT) waren das Serum Calcium und das Parathormon (PTH) meist erhöht, bei 35 Patienten mit Hypercalciämie und Malignomen dagegen das PTH normal oder nicht meßbar. Bei den 100 Patienten mit pHPT fanden wir our in 11 % die klassischen radiologischen Befunde (subperiostale Usuren und Cysten), in 64 % dagegen eine Nephrolithiasis. Postoperativ konnten wir 42 Patienten nachuntersuchen (Beobachtungsdauer 2–12 Jahre). 82% der Patienten mit präoperativer Nephrolithiasis zeigten postoperativ keine Symptome mehr, bei 75 % war auch radiologisch keine Nephrolithiasis mehr nachweisbar; 63 % der Patienten gaben spontan eine sehr deutliche Besserung ihres subjektiven Befindens an.
    Notes: Summary Serum calcium and parathormone (PTH) were almost elevated in 100 patients with primary hyperparathyroidism (pHPT), whereas PTH was normal or undetectable in 35 patients with a malignant tumor and hypercalcemia. Only 11 % of the patients with pHPT presented the classical radiologic signs, e.g., subperiostal resorptions and cysts; in contrast, in 64% of the patients renal stones were found. A follow-up could be performed in 42 patients (2–12years after parathyroidectomy): 82% of the surgically treated patients with preoperative renal stones were free of symptoms; moreover, in 75 % no renal stones could be detected radiologically and 63 % of the patients spontaneously stated that they felt substantially better.
    Type of Medium: Electronic Resource
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