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Biomedical subjects

H Schroten

Publications and source records attributed to H Schroten.

At least 37 records · Page 2Linked to original sources
PubMed2000

Rapid progressive subacute sclerosing panencephalitis in a 2-year-old child with congenital athyreosis.

We present the unique case of a 2-year-old girl with congenital athyreosis who acquired primary measles virus infection at the age of 18 months, coincidentally with an Epstein-Barr virus infection. First neurologic symptoms of subacute sclerosing panencephalitis appeared 5 months later, and the girl died within 6 months after a rapid progressive illness. Factors possibly predisposing to this extraordinary disease course-primary measles virus infection at an early age and lack of evidence for immunodeficiency-are discussed.

Brain↗
PubMed1999

Inflammation markers and cytokines in breast milk of atopic and nonatopic women.

BACKGROUND: Breast milk is thought to contain its own complex immune system. Whether or not this is altered in allergic individuals is not yet known. METHODS: By ELISA techniques, inflammatory markers (MIP-1alpha, sICAM-1) and T(H)1 (interferon-gamma [IFN-gamma]), as well as T(H)2 cytokines (interleukin [IL]-4, IL-10), were investigated in serum and milk samples from nonallergic (n = 23) lactating women and those with respiratory allergies (n = 19) during the first week postpartum. RESULTS: IFN-gamma was not detected in either serum or milk. IL-10 was more often found to be above the detection limit in both milk and serum samples from allergic mothers. IL-4 was detected in almost all serum samples with a wide variation. In milk, IL-4 was found in about 20% of the samples. MIP-1alpha was not detected in the serum but was detected in the milk of 23% of the nonatopic and 53% of the allergic mothers. Soluble ICAM-1 was present in all samples. Surprisingly, serum levels of sICAM-1 in allergic mothers (271+/-97 ng/ml) were significantly lower (P<0.001) than in nonatopic subjects (375+/-86 ng/ml). Concentrations of sICAM-1 in milk were similar in both groups. CONCLUSIONS: The concentrations of proinflammatory markers and cytokines in breast milk did not differ significantly between allergic and nonatopic mothers. In some individuals, high levels of MIP-1alpha, IL-10, and sICAM-1 could be found. However, the significance of these components for the breastfed infant is still unclear.

Adult↗
PubMed1999

S-fimbriae from Escherichia coli bind to soluble glycoproteins from human milk.

BACKGROUND: Escherichia coli (E. coli) strains, expressing S-fimbriae, belong to the most common gram-negative pathogens that cause sepsis and meningitis in neonates. The attachment of S-fimbriae to the cell surface is mediated by membrane glycoconjugates, which often carry N-acetylneuraminic acid. METHODS: Binding studies were performed with glycoproteins from the whey fraction of human milk to investigate whether they exert a potential inhibitory effect on bacterial adhesion. Whey glycoproteins were separated according to their molecular weight by fast protein liquid chromatography gel filtration. After sodium dodecyl sulfate-polyacrylamide gel electrophoresis, proteins were transferred to nitrocellulose membranes and incubated with isolated S-fimbriae from recombinant E. coli strain HB 101 (pANN 801-4). RESULTS: S-fimbriae recognized four whey proteins with a molecular mass of more than 200 kDa, 170 to 150 kDa, and 80 kDa. Their glycosylation pattern was investigated using the lectins Sambucus nigra, Maackia amurensis, Galanthus nivalis, and Arachis hypogaea. Thus the presence of N- and O-glycans in these proteins was confirmed. The preferential binding to N-acetylneuraminic acid containing glycoproteins was demonstrated by a complete abolishment of these reactions by incubation with acidic lactose-derived oligosaccharides. However, the cleavage of N-acetylneuraminic acid from glycoproteins by mild acid hydrolysis revealed a second binding site for S-fimbriae on milk proteins of a similar molecular weight range. Terminal galactose in human milk glycoconjugates were thought to react with S-fimbriae as well. CONCLUSION: These data further support the opinion that glycoproteins from human milk are potential receptor analogues for certain bacteria that may prevent microbial adhesion to the epithelial cell surface.

Animals↗
PubMed1999

Secretory immunoglobulin A is a component of the human milk fat globule membrane.

Secretory immunoglobulin A (sIgA), the predominant antibody fraction of human milk, represents a major protective factor against neonatal infection. Until now, sIgA had been identified only in the humoral fraction of human milk. The aim of this study was to assess whether in human milk sIgA is also associated with the milk fat globule (MFG). Using anti-sIgA-agglutinated human MFG and immune fluorescence microscopy, we could demonstrate that sIgA is, in fact, associated with human MFG. In human MFG membranes separated by Western blotting, sIgA bands were specifically stained, suggesting that sIgA is strongly associated with the human MFG membrane. This may be of physiologic relevance, inasmuch as earlier we could show that a quantity of undigested and functional human MFG are in fact found in the stools of the newborn. This would allow an additional extension of the protective mechanisms of sIgA throughout the whole intestine.

Agglutination Tests↗
PubMed1998

Defective expression of granulocyte-macrophage colony-stimulating factor/interleukin-3/interleukin-5 receptor common beta chain in children with acute myeloid leukemia associated with respiratory failure.

Deficiency of the granulocyte-macrophage colony-stimulating factor (GM-CSF)/interleukin-3 (IL-3)/IL-5 receptors common beta chain (betac) is a cause of fatal respiratory failure. betac deficiency manifests as pulmonary alveolar proteinosis (PAP). PAP has heterogenous etiologies that may be genetic or aquired. Some cases of PAP have been reported to be associated with hematologic malignancies such as acute myeloid leukemia (AML). In mice, the PAP phenotype was generated by targeted deletion of the gene for betac and can be treated by transplantation of wild-type bone marrow into betac -/- mice. Thus, our findings in betac -/- mice provide evidence for a causal relationship between the lung disease and the hematopoietic system. We describe here expression defects of betac or betac plus GM-CSF receptor alpha chain (GM-CSFR alpha) in 3 pediatric patients with AML and PAP symptoms. All of the patients' leukemic cells failed to express normal levels of betac. The leukemic cells of patients no. 2 and 3 additionally lacked the expression of GM-CSFR alpha, as shown by flow cytometry. Strikingly reduced or absent function of betac was demonstrated in clonogenic progenitor assays with absent colony-forming unit (CFU) growth after GM-CSF or IL-3 stimulation. The response to growth factors acting via a growth factor receptor distinct from the GM-CSF/IL-3/IL-5 system (recombinant human granulocyte colony-stimulating factor [rhG-CSF]) was normal. After antileukemic treatment, the pulmonary symptoms resolved and betac or betac plus GM-CSFR alpha expression was normal. Our findings provide evidence that a defect in the expression of betac or betac plus GM-CSFR alpha on AML blasts can be associated with respiratory failure in patients with AML.

Acute Disease↗
PubMed1998

Fab-independent antiadhesion effects of secretory immunoglobulin A on S-fimbriated Escherichia coli are mediated by sialyloligosaccharides.

S-fimbriated Escherichia coli strains cause sepsis and meningitis in newborns and are known to recognize the carbohydrate sequence sialyl-(alpha2-3)-galactoside. We show that adhesion of cloned S-fimbriated E. coli to human epithelial cells is inhibited Fab independently by sialyloligosaccharides on secretory immunoglobulin A (s-IgA). This indicates an anti-infective function of s-IgA (Fc), particularly in early human milk.

Adult↗
PubMed1997

The fat emulsion agglutination test: a reliable and cost effective alternative to the latex agglutination test for rapid bedside CRP measurement.

We compared two tests for bedside C reactive protein (CRP) measurement: the latex agglutination test (LAT) and the fat agglutination test (FAT). FAT is based on the property of CRP to agglutinate fat emulsions in the presence of CaCl2. The sensitivity, specificity and accuracy of FAT and LAT to detect a CRP > 10 mg/l, determined with radial immunodiffusion (n = 500 pediatric patients, CRP range 0- > 80 mg/l), were 91%, 82% and 90% respectively for FAT and 82%, 95% and 85% for LAT. FAT reagent could be stabilized with NaN3 (0.02%) for at least one year, when stored at 4 degrees C (n = 49). NaN3 (0.02%) had no effect on agglutination of FAT (n = 40). In conclusion, in pediatric patients, FAT is a reliable and cost effective alternative to LAT, if serum samples are used.

Agglutination Tests↗
PubMed1997

Greater omentum flaps and granulocyte transfusions as combined therapy of liver abscess in chronic granulomatous disease.

We report the case of a 17-year-old boy with gp91phax-deficient chronic granulomatous disease who developed a liver abscess due to Staphylococcus aureus. Despite treatment with appropriate antibiotics and gamma interferon for three months as well as incision and drainage, the abscess persisted unchanged in size. After surgical debridement, the abscess cavity was filled with two pedunculated greater omentum flaps as a direct feeder road of granulocytes to the infectious focus. An average of 48.5 x 10(9) granulocytes a day harvested from G-CSF-prestimulated donors were transfused for a total of 8 days without side effects. Ultrasound 3 months later showed no residual abscess. Combination of greater omentum flaps and transfusion of G-CSF-prestimulated granulocytes may be the optimal treatment for liver abscesses refractory to conventional therapy.

Adolescent↗
PubMed1997

Antimicrobial functions of human monocytes depend on concentration of glutamine in vitro.

We investigated the impact of different concentrations of glycyl glutamine on antimicrobial functions of human monocytes in vitro. Generation of suproxide anion, phagocytosis and killing capacity were measured as an expression of antimicrobial activity after an incubation period of 38 h at glycyl glutamine concentrations of 0.0, 0.4, and 2.0 mmol/l respectively. For each of the tests, significantly higher results were obtained with concentrations of glycyl glutamine of 0.4 mmol/l as compared to 0.0 mmol/l (p < 0.01). Further increases of superoxide anion production (p < 0.05) and killing capacity (p = 0.053) were observed when glycyl glutamine concentration in the culture medium was raised to 2.0 mmol/l. Variation of glycyl glutamine concentrations in vitro significantly affects antimicrobial functions of human monocytes.

Anions↗
PubMed1997

Chronic granulomatous disease masquerading as a bladder tumor: a potential source of diagnostic error.

Chronic granulomatous disease (CGD) is a rare inherited disease of childhood, characterized by recurrent bacterial or fungal infections. The underlying defect is a dysfunction of neutrophil granulocytes interfering with their ability to kill phagocytosed microorganisms. Genitourinary tract involvement has been reported in 38% of these patients. We report a case of CGD in whom the most important findings were urinary bladder tumors at different locations and a subsequent obstruction of the left ureter. A review of the pathogenesis of the disease, potential involvement of the urinary tract and treatment is presented.

Adult↗
PubMed1996

Severe combined immunodeficiency (SCID) associated neutropenia: a lesson from monozygotic twins.

A case of severe combined immunodeficiency (SCID) in monozygotic twin sisters was detected at 3 months of age with neutropenia in one twin and a normal differential count in the other. The neutropenic twin, suffering from severe skin ulcers, was successfully treated with granulocyte colony stimulating factor (G-CSF). Discordant occurrence of neutropenia in identical twins shows that there may be a non-genetic cause for the neutropenia in SCID. Suppression of myelopoiesis was probably induced by activated maternal T cells. The neutropenia in this case may thus be classified as SCID associated neutropenia, as opposed to reticular dysgenesis, in which the neutropenia is G-CSF refractory and is most probably caused by a genetic stem cell defect. A response to G-CSF in a neutropenic child with SCID can be clinically beneficial and might help to distinguish between G-CSF unresponsive reticular dysgenesis and G-CSF responsive SCID associated neutropenia.

Diseases in Twins↗
PubMed1994

Multisystem triglyceride storage disorder without ichthyosis in two siblings.

A four-year-old boy presented with hepatomegaly, vacuolized granulocytes (Jordans' anomaly) and slightly progressive myopathy as signs of multisystem triglyceride storage disease. The nine-year-old sister of the patient also showed Jordans' anomaly and early fatigability, but no overt weakness. Biochemical analysis revealed normal values for carnitines, carnitine palmityl transferase in serum and striated muscle, and beta-oxidation enzymes in striated muscles. Distribution of non-membrane-bound lipids in granulocytes, fibroblasts, smooth muscle cells and striated muscle was compatible with Chanarin-Dorfman syndrome. In contrast to Chanarin-Dorfman syndrome, our patients lacked congenital ichthyosis.

Child↗