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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 148 (1988), S. 264-266 
    ISSN: 1432-1076
    Keywords: Alkaline phosphatase ; Transient hyperphosphatasaemia ; HPLC ; Rotavirus ; Isoenzyme
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In five patients with benign transient hyperphosphatasaemia (THP), high activities of so-called “atypical” alkaline phosphatase or fragment isoenzymes were detected. One cases occurred after rotavirus infection. Incubation with neuraminidase suggested that “atypical” alkaline phosphatase originated from highly glycosylated bone and liver isoenzymes. This may have been due to virus-induced low isoenzyme clearance from serum. The course of isoenzyme activities in THP following rotavirus infection was followed. Determination of atypical alkaline phosphatase may be useful in the diagnosis of THP.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1076
    Keywords: Nesidioblastosis ; Pancreas ; Endocrine pancreatic function ; Exocrine pancreatic function ; Pancreatic sonography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Pancreatic exocrine and endocrine function was assessed in four patients 4–31 months after 88%–95% pancreatectomy. Postoperative exocrine and endocrine function was not seriously impaired. Extensive pancreatic regrowth occurred. The amount of regenerated pancreatic tissue, estimated by sonographic measurement of the sectional area, was not significantly different from that of healthy controls. Pancreatic endocrine and exocrine function and pancreatic regeneration remained satisfactory and no additional pancreatic resection became necessary. These results indicate that 95% pancreatectomy may be tolerated without loss of pancreatic function.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1076
    Keywords: Robinow syndrome ; Fetal face syndrome ; Androgen receptor deficiency ; Persistence of Mullerian ducts
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Typical features of the “fetal face” or Robinow syndrome are reported in a male infant who presented with ambiguous genitalia and persistence of the Mullerian ducts. Histology of the testes was normal whereas endocrinological studies showed partial deficiency of androgen receptors.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Calcified tissue international 63 (1998), S. 453-455 
    ISSN: 1432-0827
    Keywords: Key words: Bone — Bone density — Bone metabolism — Leptin — Obesity.
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine , Physics
    Notes: Abstract. Recent studies have implicated leptin in the modulation of bone mass during skeletal development. Whether leptin also exerts an influence on bone after growth has stopped is unknown at present. In this cross-sectional study on 94 women (60 premenopausal, 34 postmenopausal) aged 40–60 years, we analyzed the relationship between serum leptin and bone density and bone cortex geometry and bone metabolism. Total and trabecular bone density as well as total and cortical bone area were determined by quantitative computed tomography (QCT) at the distal radius. Bone metabolism was assessed by measuring bone-specific alkaline phosphatase, osteocalcin, procollagen type I C-terminal propeptide (PICP) and collagen type I C-terminal telopeptide in serum, and deoxypyridinoline in urine samples. None of the indices of bone density or geometry was significantly related to leptin serum concentrations (P 〉 0.05) before or after adjustment for body mass index (BMI). PICP was associated with serum leptin in the postmenopausal group only (r =−0.40 after adjustment for BMI; P= 0.009). Yet, as none of the other markers of bone metabolism exhibited a significant correlation with serum leptin in any of the menopausal groups, this association is likely to be due to the influence of extraskeletal factors on PICP serum levels. Thus, it appears that leptin has less influence on the mature than on the growing skeleton.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Monatsschrift Kinderheilkunde 148 (2000), S. 334-341 
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Bisphosphonate ; Knochenstoffwechsel ; Metabolische Osteopathien ; Osteogenesis imperfecta ; Key words Bisphosphonates ; Bone turnover ; Metabolic osteopathy ; Osteogenesis imperfecta
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Bisphosphonates are a class of potent inhibitors of bone resorption which are increasingly used in pediatrics. Potential applications tested so far include short term treatment of hypercalcemia and continuous therapy in disorders with decreased bone mass (osteogenesis imperfecta, idiopathic juvenile osteoporosis, secondary osteoporosis) or increased bone turnover (hyperphosphatasia, fibrous dysplasia). The efficacy of bisphosphonates is best documented in the treatment of osteogenesis imperfecta, where they were shown to decrease fracture rate and to increase mobility. While these results are very encouraging, very little is known on the effect of long term bisphosphonate treatment on bone development. This remains the most critical aspect of their use in pediatrics. Therefore, pediatric use of bisphosphonates should at present be limited to specialised centers.
    Notes: Zusammenfassung Medikamente aus der Klasse der Bisphosphonate sind sehr wirksame Inhibitoren der Knochenresorption, die in jüngster Zeit zunehmend auch in der Pädiatrie Verwendung finden. Bisher beschriebene Einsatzgebiete sind die Akuttherapie von Hyperkalzämiezuständen und die Dauertherapie von Erkrankungen, welche mit erniedrigter Knochenmasse (Osteogenesis imperfecta, idiopathische juvenile Osteoporose, sekundäre Osteoporose) oder erhöhtem Knochenumsatz (Hyperphosphatasie, fibröse Dysplasie) einhergehen. Am besten dokumentiert ist die Wirksamkeit von Bisphosphonaten bei der Therapie der Osteogenesis imperfecta, wo sie die Frakturrate verringern und die Mobilität deutlich verbessern können. Wenn auch die bisherigen Erfahrungen sehr ermutigend sind, bleibt doch der nicht ausreichend bekannte Einfluß einer Dauertherapie auf die Knochenentwicklung weiterhin der kritischste Punkt in der pädiatrischen Anwendung. Daher sollte derzeit die Therapie von Kindern mit Bisphosphonaten auf spezialisierte Zentren beschränkt bleiben.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-198X
    Keywords: Key words Renal disease ; Bone ; Muscle ; Renal osteopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Bone structure and muscular strength of 30 children with renal disease were investigated by peripheral computed tomography and grip strength. Sixteen children suffered from nephrotic syndrome (NS) and had previously been treated with corticosteroids. Fourteen children suffered from chronic renal failure (CRF) ranging from mild renal failure to end-stage renal disease. Six children had received kidney transplants and corticosteroids for immunosuppression. There was a significant decrease in grip strength of children with NS (SD –0.91± 1.5; P=0.042) and children with CRF (SD –1.38±1.4; P〈0.001) compared with normal children. Furthermore, there was a significant correlation between cortical area and grip strength in all children with renal disease (r=0.92; P〈0.0001). Trabecular bone mineral density did not correlate well with grip strength. These findings resemble results found in healthy children. Trabecular bone mineral density was significantly elevated in children with CRF compared with normal children (SD 1.14±1.4; P=0.008).Grip strength as a marker of muscle mass and cortical area as a marker of bone strength correlate well in children with renal disease, similar to the correlation in healthy children. Grip strength is significantly lower in children with NS and CRF compared with normal children. These data suggest that muscular impairment could be involved in renal osteopathy.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Monatsschrift Kinderheilkunde 144 (1996), S. 1073-1077 
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Makrozephalus ; Kraniotubuläre Skelettdysplasie ; Knochenstoffwechsel ; Key words Craniotubular bone dysplasia ; Macrocephaly ; Bone metabolism
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Craniometaphyseal Dysplasia (CMD) is a rare congenital osteochondrodysplasia characterized by hyperostosis and sclerosis of the skull and widening of the metaphyseal region of the long bones with clinical manifestation in early childhood. We report on an 18 months old boy with macrocephaly, nasal obstruction and a dysmorphic face (hypertelorism, broad bony overgrowth of the root of the nose, dental irregularities), who's mother had similar but milder stigmata. Radiologic findings of skull and femora of both mother and child allowed the diagnosis. Our investigations of bone metabolism point to an overactivity of osteoblasts. Cranial nerve compression as a typical complication of CMD could be excluded. The nasal obstruction led to a surgical resection of the posterior vomer. Knowledge about this condition allows early diagnosis and therapy of complications.
    Notes: Zusammenfassung Die kraniometaphysäre Dysplasie stellt eine seltene Unterform der sklerosierenden Skelettdysplasien mit klinischer Manifestation im frühen Kleinkindesalter dar, die mit einer deutlichen Sklerose des gesamten Schädels und mit einer Aufweitung der Metaphysen der langen Röhrenknochen einhergeht. Wir berichten über ein zum Zeitpunkt der Diagnosestellung 18 Monate altes männliches Kleinkind, das mit zunehmendem Makrozephalus, chronischer Nasenatmungsbehinderung und einer Gesichtsdysmorphie (Hypertelorismus, breite Nasenwurzel, Zahnirregularitäten) auffällig wurde. Die Mutter des Patienten wies ähnliche Stigmata auf. Durch Röntgenaufnahmen von Schädel und Femur bei Patient und Mutter konnte die Diagnose gestellt werden. Untersuchungen des Knochenstoffwechsels des betroffenen Kindes weisen auf eine Überaktivität der Osteoblasten hin. Eine Hirnnervenkompression als typische Komplikation des Krankheitsbilds konnte ausgeschlossen werden. Die krankheitsbedingte Nasenatmungsbehinderung machte jedoch eine Choanalerweiterung durch Resektion des posterioren Vomer notwendig. Die Kenntnis dieses Krankheitsbilds erlaubt eine rechtzeitige Diagnosestellung und Intervention bei Komplikationen.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1437-160X
    Keywords: Key words Insulin-like growth factors ; Insulin-like growth factor binding proteins ; Rheumatoid arthritis ; Articular cartilage ; Proteoglycans
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The objective of this study was to quantify insulin-like growth factor (IGF) binding proteins (IGFBPs) in the synovial fluid (SF) and plasma of patients with rheumatic diseases and to study the role of these proteins in the regulation of cartilage proteoglycan (PG) synthesis. Immunological determination of IGFBP-2, IGFBP-3, IGF-I, IGF-II, interleukin-1β (IL-1β) and tumour necrosis fac-tor α (TNFα) was undertaken in the SF and plasma of 115 patients with rheumatoid arthritis (RA; n = 53), osteoarthritis (OA; n = 44) and other rheumatic disorders. We also determined the effects of SF on bovine cartilage PG synthesis in culture. IGFBP-2 and IGFBP-3 were elevated in the plasma (by 38% and 28%, respectively) and SF (by 56% and 59%, respectively) of patients with RA compared to age- and sex-matched OA controls (determined by RIA and confirmed by Western ligand blot). IGF-I and IGF-II did not differ significantly between the two groups. OA SF, and, to a lesser extent, RA SF stimulated cartilage PG synthesis in culture, and more than 60% of this activity was neutralised by a specific monoclonal anti-IGF-I antibody. Human IGFBP-3 dose-dependently inhibited the stimulation of cartilage PG synthesis effected by SF or human IGF-I. In RA patients, the SF concentration of IGFBP-3 was positively correlated with SF levels of IL-1β and TNFα, with the serum level of C-reactive protein and with the erythrocyte sedimentation rate. We concluded that IGF-I is, under the conditions studied, the most important anabolic factor in human SF with respect to articular cartilage PG synthesis. The bioactivity of IGF-I in joints is modulated by IGFBP-3, which is elevated in RA SF compared to OA SF. Elevated IGFBP-3 in RA SF may reduce the availability of IGF-I to articular chondrocytes, thus interfering with cartilage PG synthesis in RA.
    Type of Medium: Electronic Resource
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