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Biomedical subjects

F Schuster

Publications and source records attributed to F Schuster.

27 records · Page 2Linked to original sources
PubMedDate not supplied

Accelerated phase in partial albinism with immunodeficiency (Griscelli syndrome): genetics and stem cell transplantation in a 2-month-old girl.

UNLABELLED: A 2-month-old girl presented with fever, hepatosplenomegaly, pancytopenia, hypertriglyceridaemia and silvery-greyish hair, suggesting the diagnosis of Griscelli syndrome (partial albinism with immunodeficiency). This diagnosis was confirmed by the characteristic agglomeration of melanin in the hair shaft and accumulation of melanosomes in melanocytes of the skin. The patient was homozygous for polymorphic markers around the myosin-Va gene on chromosome 15q21, which co-localize to the Griscelli disease locus. Natural-killer cells were in the lower range. The stimulation of lymphocytes with antigen and mitogen was normal. The patient's accelerated phase, characterized by haemophagocytosis was treated with prednisolone, rabbit anti-thymocyte globulins, and intrathecal methotrexate. Remission was maintained with cyclosporin A until HLA-compatible peripheral blood stem cell transplantation from her mother. CONCLUSION: The silvery-greyish hair associated with fever, pancytopenia and hypertriglyceridaemia is the clue to early diagnosis of Griscelli syndrome and important to prevent death before stem cell transplantation.

Chromosomes, Human, Pair 15↗
PubMedDate not supplied

Megestrol acetate to correct the nutritional status in an adolescent with growth hormone deficiency: Increase of appetite and body weight but only by increase of body water and fat mass followed by profound cortisol and testosterone depletion.

Megestrol acetate (MA) is a synthetic, orally active derivative of the naturally occurring hormone progesterone. MA is increasingly used to correct loss of appetite and improve the nutritional status. We used MA in an adolescent with growth hormone (GH) deficiency due to former irradiation therapy in order to evaluate if MA can improve the nutritional status. In fact, MA increased appetite and weight dose-dependent. The energy expenditure measured by indirect calorimetry changed from hypo- to normometabolism. However, weight gain was first primarily due to an increase in body water and then in fat mass. The gain of fat mass was much more prominent than the gain of fat free mass. As important side-effect, MA lead to rapid and profound cortisol and testosterone depletion after only 10 days with a long-lasting effect on testosterone depletion. Therefore, MA as a single therapy cannot be recommended to improve the nutritional status. If MA is given, cortisol and testosterone levels have to be monitored and supplemented as needed.

Adipose Tissue↗
PubMedDate not supplied

Vancomycin-resistant-enterococci--colonization of 24 patients on a pediatric oncology unit.

BACKGROUND: Colonization with multidrug-resistant vancomycin-resistant-enterococci (VRE) could become a serious problem, since there is no proven therapy in case of an infection or in case of transfer of glycopeptid-resistance to other organisms. PATIENTS: Description of 24 from 48 pediatric oncology patients with VRE-colonization. METHODS: Stool samples were taken from all patients of our pediatric oncology unit from March 1996 until June 1997. Barrier isolation was introduced in May 1996, a prudent use of glycopeptid antibiotica in July 1996. RESULTS: 193 stool sample examinations demonstrated that 24 (50%) of the 48 patients were colonized with VRE. 11 (46%) of these 24 patients were VRE-carriers at the time of their first examination; 9 (37%) patients acquired VRE during their therapy and 4 (17%) patients had come from other hospitals and already were VRE-positive when they entered our unit. In March 1997, one year after the outbreak only four patients still were VRE-positive, in June 1997 all of them were VRE-negative. The average time of colonization was 12.5 weeks. 17 (70%) of the 24 colonized patients had received glycopeptide antibiotics, 16 of them within two months before the appearance of VRE in their stool. Five colonized patients died, four of them because of their oncological illness, one because of a sepsis without proof of VRE in his blood. In the end none of our patients suffered from a VRE-infection, and besides that, the transfer of glycopeptid-resistance to other organisms was not observed. CONCLUSION: With barrier isolation and a very restrictive use of glycopeptid-antibiotica, colonization can be decreased and even stopped. Inspite of the high number of colonized patients no VRE-infectious disease occurred.

Adolescent↗