Publications by authors named "Mackensen S"

Neuronal processing is energy demanding and relies on sugar metabolism. To nurture the Drosophila nervous system, the blood-brain barrier forming glial cells take up trehalose from the hemolymph and then distribute the metabolic products further to all neurons. This function is provided by glucose and lactate transporters of the solute carrier (SLC) 5A family.

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Astrocytes have diverse, remarkably complex shapes in different brain regions. Their branches closely associate with neurons. Despite the importance of this heterogeneous glial cell type for brain development and function, the molecular cues controlling astrocyte branch morphogenesis and positioning during neural circuit assembly remain largely unknown.

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Background: The ITP-QoL is a disease-specific questionnaire for the assessment of health-related quality of life (HRQoL) in children with immune thrombocytopenia (ITP) and their parents. The aim of this study was to test the psychometric characteristics of the ITP-QoL in the Italian pediatric population in terms of validity and reliability.

Procedure: Children aged 8-16 years with acute or chronic ITP and their parents were recruited in Italy.

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Quality of life (QoL) in hemophilia is an important area in hemophilia outcome assessment. The Haemo-QoL instrument is a set of questionnaires to measure QoL in those children. The objectives of this study was to assess health-related quality of life (HRQoL) in Egyptian hemophilic children and adolescents using an Arabic version of the Haemo-QoL questionnaire.

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The modern management of haemophilia has greatly influenced not only survival of patients, their clinical symptoms and orthopaedic outcome but also their perceived quality of life (QoL). QoL measures recently became an essential part of clinical trials being one of the most important patient-rated outcomes (PROs). Moreover, QoL assessment is essential in pharmacoeconomics.

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A new procedure is introduced for the isolation of connective tissue fibers, called biomatrix, containing a significant portion of the extracellular matrix (basement membrane components and components of the ground substance). Biomatrix isolated from normal rat liver contains >90% of the tissue's collagens and all of the known collagen types, including types I and III and basement membrane collagens. The purified collagenous fibers are associated with noncollagenous acidic proteins (including fibronectins and possibly small amounts of glycosaminoglycans).

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Morphometrical investigations (point-counting method) showed that in different inflammatory (endocapillary -- acute -- GN, mesangioproliferative GN, membranoproliferative GN) glomerulonephritides and in non-inflammatory glomerular diseases (perireticular amyloidosis), there are statistically significant correlation between serum creatine concentrations at the time of biopsy and the enlargement of the cortical interstitium by fibrosis. Similar results were obtained in investigating different grades of benign nephrosclerosis with transition into secondary malignant nephrosclerosis conditioned by hypertension and in chronic diffuse sclerosing interstitial nephritides of different etiologies. As hypothesis, we assume that a narrowing of the postglomerular vessel network by interstitial fibrosis take place.

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62 tissue specimens with the only diagnosis benign nephrosclerosis (or benign nephrosclerosis with transition to secondary malignant nephrosclerosis) were investigated attempting to correlate morphological findings (relative interstitial volume of the renal cortex, types of hyalinisation and kinds of periglomerular changes, vessel index) with each other and with the serum creatinine concentration as a parameter of renal function. There are significant correlations in form of exponential and parabolic functions between relative interstitial volume of the renal cortex and the serum creatinine concentration at the time of biopsy. Furthermore 5 types of glomerular and periglomerular changes, which could be discriminated, seem to influence renal function in a different way and at different stages of the disease.

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We report light- and electron microscopic findings in glomerular amyloidosis (secondary amyloidosis), which occurred after recurrent empyema of the pleura. After healing of the empyema, the clinical symptoms disappeared, over a period of eight years. During the acute stage of the disease (grade II-III amyloidosis) when the nephrotic syndrome was present, amyloid deposits were seen in the mesangium and on both sides of the basement membrane of the glomerular capillaries.

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48 cases of renal amyloidosis (grade I-IV) were investigated morphologically (point-counting method). Statistically significant positive correlations exist between relative interstitial volume and serum creatinine concentration at the time of biopsy. In amyloidosis there are furthermore connections (positive rank correlations) between the extent of glomerular amyloidosis and renal insufficiency, although single cases of grade III show normal serum creatinine concentrations if the interstitium is not enlarged.

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Renal biopsies of 44 patients with endocapillary acute glomerulonephritis (gn) and 64 patients with moderately severe mesangioproliferative gn were investigated morphometrically (point-counting-method, tubulometry). In both gn's statistically significant positive corrleations between relative interstitial volume and the concentration of serum creatinine at the time of biopsy were found. Despite severe glomerular lesions the serum creatinine concentration is not increased in most cases of endocapillary acute gn, providing the relative interstitial volume is not increased by more than 15%.

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Morphometric investigations in 33 patients suffering from membranoproliferative glomerulonephritis at different grades of glomerular involvement showed that there is no certain relationship between the severity of glomerular lesions and the serum creatinine level. On the other hand there is a significant positive correlation between the relative interstitial volume and the level of serum creatinine. The best congruence (r=+0,87, p less than 0.

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Morphometric investigations in 40 patients suffering from perimembranous glomerulonephritis at different stages showed that there is no certain relationship between the severity of glomerular lesions and the serum creatinine level. In 19 cases in stages I-III, with serum creatinine level less than 1.2 mg/100 ml on biopsy, the renal interstitium was less enlarged than in 21 cases in the same stages, but with serum creatinine level higher than 2 mg/100 ml.

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