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Biomedical subjects

M Brandis

Publications and source records attributed to M Brandis.

At least 55 records · Page 3Linked to original sources
PubMed1998

Predisposing conditions and pathogens in bacteremia in hospitalized children.

Between 1985 and 1995, 1037 bacteremic episodes were recorded in a pediatric tertiary care center and analyzed retrospectively. Gram-positive bacteria accounted for 719 episodes (68%), gram-negative bacteria for 303 (29%), fungi for 16 (2%), and anaerobes for 12 (1%). In 526 (51%) patients, primarily neonates and oncology patients, a predisposing condition was present. In 390 (38%) episodes a clinical source of infection was documented. Mortality was highest in Pseudomonas bacteremia (45%). Since the bacterial spectrum differed widely between patient groups, the choice of empirical antimicrobial therapy should be based on any underlying condition present in the patient and the clinical source of infection. As anaerobes were rarely isolated. the routine use of anaerobic blood cultures in patients without predisposing conditions does not seem warranted.

Anti-Bacterial Agents↗
PubMed1998

Acute bilateral renal vein thrombosis complicating Netherton syndrome.

UNLABELLED: A 1-year old male infant suffering from Netherton syndrome with severe generalized erythroderma presented with acute renal failure due to bilateral renal vein thrombosis (RVT) after a short episode of enteritis. The imperceptible fluid loss through the skin and the additional enteric water loss had led to decompensation of the delicate fluid balance and had resulted in RVT as a sequel of haemoconcentration. Reperfusion of the left kidney could be achieved by treatment with urokinase and heparin. Prophylactic oral anticoagulation was instituted for several weeks. CONCLUSION: In severe Netherton disease meticulous surveillance of the fluid balance is important and aggressive treatment is indicated in case of additional fluid loss.

Diagnosis, Differential↗
PubMed1998

Lack of large, homozygous deletions of the nephronophthisis 1 region in Joubert syndrome type B. APN Study Group. Arbeitsgemeinschaft für Pädiatrische Nephrologie.

Joubert syndrome type B (JSB) is a developmental disorder of the nephronophthisis (NPH) complex with multiple organ involvement, including NPH, coloboma of the eye, aplasia of the cerebellar vermis, and the facultative symptoms of psychomotor retardation, polydactyly, and neonatal tachypnea. In isolated autosomal recessive NPH type 1 (NPH1), homozygous deletions have been described as causative in more than 80% of patients. Since different combinations of the extrarenal symptoms with NPH occur in JSB, a contiguous gene deletion syndrome in the NPH1 genetic region would seem a highly likely cause for JSB. We therefore examined 11 families with JSB for the presence of extended deletions at the NPH1 locus. Genomic DNA was examined using four consecutive polymerase chain reaction (PCR) markers that are deleted in NPH1 and three PCR makers flanking the NPH1 deletion. In all seven markers examined, there was no homozygous deletion detected in any of the 11 JSB families studied. Since these markers saturate the NPH1 deletion region at high density, this finding excludes the presence of large homozygous deletions of the NPH1 region in these JSB families, making it unlikely that deletions of the NPH1 region are a primary cause for JSB.

Cerebellar Diseases↗
PubMed1998

Bilateral renal vein thrombosis and venous sinus thrombosis in a neonate with factor V mutation (FV Leiden).

Bilateral renal vein thrombosis and venous sinus thrombosis were diagnosed within 3 weeks of birth in a full-term neonate. Heterozygosity for a factor V mutation leading to resistance against the anticoagulatory properties of activated protein C was found. Heparin therapy led to resolution of the thrombotic manifestations. With long-term oral anticoagulation, no relapse or other thrombotic event occurred during infancy.

Anticoagulants↗
PubMed1998

Novel molecular variants of the Na-K-2Cl cotransporter gene are responsible for antenatal Bartter syndrome.

Antenatal Bartter syndrome is a variant of inherited renal-tubular disorders associated with hypokalemic alkalosis. This disorder typically presents as a life-threatening condition beginning in utero, with marked fetal polyuria that leads to polyhydramnios and premature delivery. Another hallmark of this variant is a marked hypercalciuria and, as a secondary consequence, the development of nephrocalcinosis and osteopenia. We have analyzed 15 probands belonging to 13 families and have performed SSCP analysis of the coding sequence and the exon-intron boundaries of the NKCC2 gene; and we report 14 novel mutations in patients with antenatal Bartter syndrome, as well as the identification of three isoforms of human NKCC2 that arise from alternative splicing.

Amino Acid Sequence↗
PubMed1998

Apoptosis and necrosis during ischaemia in renal tubular cells (LLC-PK1 and MDCK).

BACKGROUND: Ischaemia is the most frequent cause of acute renal failure. It has been previously demonstrated that ischaemia is connected with signs of necrosis and apoptosis. Apoptosis is an energy-dependent process. During ischaemia intercellular energy levels decline rapidly. Therefore, the goal of the investigation was to reveal the time dependency of cell death mechanisms during ischaemia leading to irreversibility of tissue damage. METHODS AND RESULTS: A model of renal ischaemia induced by ATP depletion was used in LLC-PK1 and MDCK-cells. Cell proliferation, determined by 3H-thymidine and BrdU incorporation and by the Ki67-labelling index was affected already after 1-2 h of ATP depletion in both cell lines. Cell viability and membrane leakage, estimated by trypan blue and propidium iodide exclusion and LDH release, was profoundly increased after 8-16 h. Evaluation of mechanisms of necrotic or apoptotic cell death was calculated from fraction of cells with pyknotic nuclei, investigation of DNA fragmentation and, by translocation of phosphatidylserine (PS) from the inner membrane face to the surface. In both cell lines increased numbers of cells with condensed nuclei was not a major sign of apoptosis. Only in MDCK cells were the numbers of cells with condensed nuclei significantly increased after 1 h compared to controls. As a hallmark of apoptosis, ATP depletion resulted in intranucleosomal DNA fragmentation after 1 h. After 8-16 h this pattern changed to a smear pattern, as a sign for necrosis. PS staining was detectable at the cell surface after 1 h. CONCLUSIONS: Ischaemia is associated with a rapid loss of proliferation and signs of apoptosis at early stages in a small proportion of cells. Most cells undergo the necrotic pathway of cell death after prolonged ATP depletion (8 h). There was no difference in behaviour comparing proximal (LLC-PK1) with more distal (MDCK) cell culture models. These results may help to explain the findings that apoptosis and necrosis have both been described after renal ischaemia.

Animals↗
PubMed1998

Autosomal dominant medullary cystic kidney disease: evidence of gene locus heterogeneity.

Autosomal dominant medullary cystic kidney disease (ADMCKD; synonym: medullary cystic disease, MCD) is an autosomal dominant kidney disorder, sharing morphological and clinical features with recessive juvenile nephronophthisis (NPH), such as reduced urinary concentration ability and multiple renal cysts at the corticomedullary junction. While in NPH end-stage renal disease (ESRD) occurs in adolescence, ADMCKD leads to ESRD in adulthood. Recently a gene locus for ADMCKD has been localized to chromosome 1q21 in two large Cypriot families. This prompted us to examine linkage in three ADMCKD-families, using the same set of polymorphic microsatellite markers spanning the critical region on chromosome 1q21. Haplotype analysis revealed that none of the three families showed linkage to this locus, thus demonstrating evidence for genetic locus heterogeneity. Additional linkage analysis studies need to be performed in order to identify further gene loci cosegregating with this autosomal dominant kidney disorder.

Adult↗
PubMed1998

Cholinergic ion secretion in human colon requires coactivation by cAMP.

Cl- secretion in the colon can be activated by an increase of either intracellular Ca2+ or cAMP. In this study we examined a possible interdependence of the two second-messenger pathways in human colonic epithelium. When measured in a modified Ussing chamber, carbachol (CCH; 100 micromol/l, basolateral), via an increase in cytosolic Ca2+ concentration ([Ca2+]i), activated a transient lumen-negative equivalent short-circuit current (Isc) [change (Delta) in Isc = -79.4 +/- 7.5 microA/cm2]. Previous studies indicated that intracellular Ca2+ directly acts on basolateral K+ channels, thus enhancing driving force for luminal Cl- exit. Increased intracellular cAMP (by basolateral addition of 100 micromol/l IBMX and 1 micromol/l forskolin) activated a sustained lumen-negative current (DeltaIsc = -42.4 +/- 7.2 microA/cm2) that was inhibited by basolateral trans-6-cyano-4-(N-ethylsulfonyl-N-methylamino)-3-hydroxy-2, 2-dimethyl&2-chromane (10 micromol/l), a blocker of KvLQT1 channels. In the presence of elevated cAMP, the CCH-activated currents were augmented (DeltaIsc = 167.7 +/- 32.7 microA/cm2), suggesting cooperativity of the Ca2+- and cAMP-mediated responses. Inhibition of endogenous cAMP production by indomethacin (10 micromol/l) significantly reduced CCH-activated currents and even reversed the polarity in 70% of the experiments. The transient lumen-positive Isc was probably due to activation of apical K+ channels because it was blocked by luminal Ba2+ (5 mmol/l) and tetraethylammonium (10 mmol/l). In the presence of indomethacin (10 micromol/l, basolateral), an increase of cAMP activated a sustained negative Isc. Under these conditions, CCH induced a large further increase in lumen-negative Isc (DeltaIsc = -100.0 +/- 21.0 microA/cm2). We conclude that CCH acting via [Ca2+]i can induce Cl- secretion only in the presence of cAMP, i.e., when luminal Cl- channels are already activated. The activation of a luminal and basolateral K+ conductance by CCH may be essential for transepithelial KCl secretion in human colon.

Adolescent↗
PubMed1998

Cadmium is more toxic to LLC-PK1 cells than to MDCK cells acting on the cadherin-catenin complex.

Cadmium toxicity to renal cells was investigated in Madin-Darby canine kidney (MDCK) and LLC-PK1 cells as models of the distal tubule/collecting duct and proximal tubule, respectively. Cells were grown on two-compartment filters and exposed to 0.1-50 microM Cd2+. In MDCK cells, Cd2+ was more toxic from the basolateral than from the apical side and dependent on the extracellular Ca2+ concentration. Toxicity was evident within 24 h, as shown by a decrease in transepithelial resistance (TER), reduced proliferation (bromodeoxyuridine incorporation), reduction in ATP concentration, and morphological changes. On confocal microscopy, E-cadherin and alpha-catenin staining patterns indicated interference with the cadherin-catenin complex. LLC-PK1 cells showed a similar toxicity pattern, which was evident at lower Cd2+ concentrations. An increase of E-cadherin and alpha-catenin molecules in the Triton X-100-insoluble fraction was detectable at high Cd2+ concentrations in LLC-PK1 cells but not in MDCK cells. Lactate dehydrogenase release indicated membrane leakage in LLC-PK1 cells. Rhodamine-phalloidin staining, a probe for F-actin filaments, demonstrated alterations of the actin cytoskeleton in both cell lines. In conclusion, cadmium caused ATP depletion and interfered with the cadherin-catenin complex and probably the tight junctions changing renal cell morphology and function.

Animals↗
PubMed1998

Plasma levels and gene expression of granulocyte colony-stimulating factor, tumor necrosis factor-alpha, interleukin (IL)-1beta, IL-6, IL-8, and soluble intercellular adhesion molecule-1 in neonatal early onset sepsis.

Bacterial sepsis is still a leading cause of neonatal morbidity and mortality. Early onset sepsis in particular, presents with a different clinical course and involves other pathogens than sepsis later in life. In this study, plasma concentrations and mRNA expression of granulocyte colony-stimulating factor (G-CSF), tumor necrosis factor-alpha (TNF-alpha), IL-1beta, IL-6, IL-8, and soluble intercellular adhesion molecule-1 (sICAM-1) of neonates with early onset sepsis were evaluated in cord blood and during the first days of life. Irrespective of prematurity, plasma levels of G-CSF, TNF-alpha, IL-1beta, IL-6, and IL-8, but not sICAM-1, were excessively elevated in septic neonates when compared with both healthy infants and infants with clinically suspected but not confirmed sepsis. Compared with the corresponding maternal levels, neonatal cytokine cord plasma levels were likewise highly elevated, indicating the endogenous cytokine production by the neonate. With the exception of TNF-alpha, mRNA expression in blood cells from septic infants was, however, not more frequently detectable than in those from nonseptic patients. Cytokine levels decreased significantly within the first days of life, whereas levels of sICAM-1 and C-reactive protein increased during the same time period. In summary, in contrast to C-reactive protein and sICAM-1, cord blood plasma levels, but not the presence of mRNA, of G-CSF, TNF-alpha, IL-1beta, IL-6, and IL-8 can predict neonatal early onset sepsis with a high sensitivity and specificity. Cell types other than blood cells are likely to contribute considerably to the high cytokine production in septic newborns.

Bacteremia↗
PubMed1998

A feminist analysis of the theories of etiology of depression in women.

Women in the United States are more likely to be hospitalized for depression than be diagnosed with breast cancer during their lifetime, despite the continued underrecognition and underdiagnosis of depression. According to the DSM-IV, unipolar depression is a severe mood disorder characterized by a loss of pleasure in most activities, along with one or a combination of associated symptoms. Women between the ages of 20 and 45 are most prone to depression, with the incidence declining as they age. The likelihood of a correct diagnosis and the method of treatment of depression, as is true for other conditions and illnesses, is very much dependent upon the belief in its etiology; therefore, the theories of etiology warrant our serious consideration. Depression in women is explained in the literature most recently by biochemical explanations, though Freud's classic "female masochism" theory is still accepted in updated forms. Conflicting social roles and expectations, continued violence against women and children, and extreme disparities in socioeconomic opportunities and conditions between men and women are also cited as reasons for women's very high rates of depression in the U.S. Primary prevention through assessment for the predictors of depression offers the best hope for the promotion of mental health in women. During the course of routine health care interactions, nurses in primary care settings are potentially in the best position to assess each woman encountered for depression risk factors or symptoms. A few important questions asked by the nurse in the course of taking a brief health history or vital signs--such as where and with whom the woman lives; where she works (inside and/or outside the home) and if she finds the work fulfilling; how she supports herself financially; when, how much, and how well she sleeps; and what kinds of emotional support networks she has--may reveal enough to warrant a more detailed and specific assessment or a referral for treatment. Psychoeducation, often administered by psychiatric RNs, includes consciousness raising and self-help groups, women's studies courses, and the establishment of support networks. These, as well as various forms of feminist-based interpersonal psychotherapy, are oft-cited approaches to aiding at-risk women clients. Given the willingness of many women clients to discuss their mental health status with a caring and interested nurse, perhaps the most positive intervention nurses can make is to listen, be supportive, and take seriously the pressures on women to be at once everything to everyone and no one at all.

Depressive Disorder↗
PubMed1997

Probable Opitz trigonocephaly C syndrome with medulloblastoma.

We report on a patient with trigonocephaly, biparietal widening as a result of metopic synostosis, strabismus, upslanted palpebral fissures, apparently low-set ears with abnormal helices, deeply furrowed palate, postaxial polysyndactyly of the feet, ankle flexion deformities, cryptorchidism, loose skin, and severe mental retardation, findings compatible with a diagnosis of the Opitz trigonocephaly C syndrome (OTS). At the age of 12 years this patient presented with symptoms of raised intracranial pressure. A biopsy showed findings diagnostic of a medulloblastoma WHO Grade IV, an unprecedented finding in OTS. The possibility of coincidence should not prevent continued surveillance of OTS patients in the future for the occurrence of malignancy.

Abnormalities, Multiple↗
PubMed1997

Renal function after conditioning therapy for bone marrow transplantation in childhood.

The knowledge of renal function in the course of BMT is poor. We prospectively investigated glomerular and tubular function in 42 children who underwent BMT because of malignancy. Seventeen children were transplanted autologously. Investigations were performed before and immediately after the conditioning regimen. Inulin and creatinine clearance, albuminuria, urine excretion of alpha 1-microglobulin, beta-N-acetylglucosaminidase, alanine-aminopeptidase, intestinal alkaline phosphatase, and Tamm-Horsfall-Protein as well as sodium- and phosphatreabsorption were measured. The patients were classified regarding use of total body irradiation (tTBI) in the conditioning regimen. BEFORE CR: Glomerular filtration rate (GFR) was not influenced by the underlying diagnosis or previous treatment. Mean GFR was elevated compared with the reference group. Microalbuminuria was elevated in 15% of patients, and mean levels were higher than in the reference group. Proximal tubular dysfunction was indicated by an elevated excretion of alpha 1-MG in 54%, of beta-NAG in 66%, of AAP in 40%, and of IAP in 47%. Fractional sodium excretion was abnormal in 21%, phosphate reabsorption in 5% and THP-excretion in 7% of the patients. AFTER CR: Creatinine clearance was not affected by CR. After CR alpha 1-MG, beta-NAG, FENa, AAP, and IAP were increased compared with values before CR. TP/Clcr was decreased. Excretion of THP was not altered by CR. In patients without fTBI there was a greater increase in alpha 1-MG excretion and decrease in phosphate reabsorption after CR compared with patients conditioned with fTBI. We conclude that significant proximal tubular dysfunction is present in about 50-60% of patients before and in nearly all alter CR. Distal tubular function was less severely affected. Severity of nephrotoxicity after CR did not correlate with pre-existing abnormalities.

Adolescent↗
PubMed1997

Bacterial tracheitis caused by Corynebacterium diphtheriae.

UNLABELLED: Diphtheria has become a rare disease in Germany. We report on an unimmunized 3.5-year-old German girl with a 7-day history of respiratory distress and fever, presenting a clinical picture mimicking typical bacterial tracheitis without pharyngeal and laryngeal manifestation. Diagnosis of diphtheria was not made until culture of tracheal secretions yielded growth of a toxigenic strain of Corynebacterium diphtheriae. The patient died from toxic cardiac failure despite treatment with diphtheria antitoxin. This is the second reported case of isolated bacterial tracheitis caused by Corynebacterium diphtheriae. CONCLUSION: The observation of a lethal course of diphtheric tracheitis emphasizes the paramount importance of immunization against diseases like diphtheria.

Bacteriological Techniques↗
PubMed1997

Renal excretion of endothelin in children.

Endothelin (ET) is a peptide with profound vasoconstrictive potential. First isolated from porcine endothelial cell supernatant, it is produced also by smooth muscle, epithelial and circulating cells. Besides vasoconstriction, a wide spectrum of biological activities of ET (via activation of membrane receptors) has been described. These include regulation of other hormones and neurotransmitters, cellular growth and proliferation, bronchoconstriction, and, in the kidney, natriuresis and water diuresis. ET exerts its effects mainly in an autocrine and paracrine fashion. A high concentration of ET is found in urine, compared with plasma originating mainly from the kidney itself. In this review we focus on the role of urinary excretion of ET in children. ET excretion was determined under different physiological and pathological conditions. In premature infants and newborns, the daily excretion of ET (corrected for body surface) was higher than in older children; it was constant, and comparable to the values in healthy adults after the age of 2 years. Renal ET excretion correlated positively with urine flow in both healthy and sick children. Conditions with tubular and/or collecting duct cell damage, such as severe hypoxia, hemolytic-uremic syndrome, renal transplantation, diabetes mellitus, chronic renal failure, and contrast media cytotoxicity were characterized by elevated urinary excretion of ET. In conclusion, the renal excretion of ET is influenced by several factors, probably reflecting the intrarenal ET production. ET has a low specificity with regard to renal injury.

Adolescent↗
PubMed1997

Tubular toxicity of cyclosporine A and the influence of endothelin-1 in renal cell culture models (LLC-PK1 and MDCK).

To evaluate the effect of cyclosporine A (CyA) at high concentrations (10(-4) and 10(-5) M) and the influence of endothelin-1 (ET-1) at physiological and pharmacological concentrations (10(-14) to 10(-6) M) on epithelial cell function, LLC-PK1 cells were studied as a model of the proximal tubule and MDCK cells as a model of the distal tubule/collecting duct. CyA caused time- and concentration-dependent acute toxicity. In LLC-PK1 cells, CyA caused a decrease in transepithelial resistance, indicating a loss of cell contacts, a release of lactate dehydrogenase (LDH) and villin into the supernatant, suggesting destruction of the apical membrane with loss of brush border, and finally release of uvomorulin, suggesting a disruption of the cell-cell adhesion, the zonula adherens. DNA synthesis, as evaluated by bromodeoxyuridine (BrdU) incorporation, was significantly affected at > or = 10(-5) M CyA. The toxicity of CyA was higher when given from the apical rather than the basolateral compartment. ET-1 alone was without effect, but in combination with CyA, ET-1 significantly enhanced toxicity. The ET-1 effect was partially inhibitable by an ET(B), but not an ET(A), antagonist. Immunofluorescence for alpha-catenin, another protein of the zonula adherens, demonstrated no change in polarity for this protein, and immunoprecipitation of the complex indicated relative stability of the zonula adherens despite loss of cadherin into the supernatant. In MDCK cells the effects were different. CyA was not associated with LDH release, but with an increase in transepithelial resistance, indicating increased paracellular resistance. Morphological alterations were significantly less, but BrdU incorporation was decreased. This pattern of toxicity is compatible with a direct toxic effect of CyA on cells of the proximal tubule, with predominant morphological destruction of the cells, with concomitant proximal tubular dysfunction, and a functional alteration in cells of the distal tubule associated with increased paracellular resistance, which may lead to solute and water loss.

Animals↗
PubMed1997

Molecular genetic identification of families with juvenile nephronophthisis type 1: rate of progression to renal failure. APN Study Group. Arbeitsgemeinschaft für Pädiatrische Nephrologie.

Familial juvenile nephronophthisis (NPH), an autosomal recessive cystic disease of the kidney, is the most common genetic cause of end-stage renal disease (ESRD) in the first two decades of life. A gene locus for nephronophthisis type 1 (NPH1) has been mapped by linkage analysis to chromosome 2q13. We performed a haplotype analysis in 16 NPH families with at least two affected patients with the typical history, clinical signs and histology of NPH using microsatellite markers of the NPH1 genetic region. By demonstration of a recombinant event marker D2S1893 was identified as a novel centromeric flanking marker to the NPH1 critical genetic region. Absence of linkage to the NPH1 locus in six NPH families confirmed the existence of at least one additional gene locus for NPH. Linkage to the NPH1 locus was demonstrated in 10 families. In 8 of these families a homozygous deletion was identified. These data permit for the first time the study of the development of renal failure in a subset of NPH1 families, which is most likely homogeneous with regard to the responsible gene locus. We present a statistical description of serial serum creatinine measurements in NPH1. Analysis of renal death revealed a median of 13.1 years. Age-dependent quartiles were generated for serum creatinine. In summary, the new marker provides a diagnostic tool to aid in the diagnosis of NPH, while the progression charts offer a standard for an assessment of the rate of progression to ESRD for patients with NPH1 to be used in future therapeutic trials and for a prediction of the individual course of the disease.

DNA, Satellite↗