Guido F Laube

University Children's Hospital Basel, Bâle, Basel-City, Switzerland

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Publications (29)146.55 Total impact

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    ABSTRACT: To investigate the long-term neurodevelopmental outcome in children after hemolytic uremic syndrome (HUS) and to compare outcome dependent on central nervous system (CNS) involvement during HUS.
    Pediatric nephrology (Berlin, Germany). 09/2014;
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    ABSTRACT: Information on lipid abnormalities in end-stage renal disease (ESRD) mainly originates from adult patients and small paediatric studies. We describe the prevalence of dyslipidaemia, and potential determinants associated with lipid measures in a large cohort of paediatric ESRD patients. In the ESPN/ERA-EDTA registry, lipid measurements were available for 976 patients aged 2-17 years from 19 different countries from the year 2000 onwards. Dyslipidaemia was defined as triglycerides >100 mg/dL (2-9 years) or >130 mg/dL (9-17 years), high-density lipoprotein (HDL) cholesterol <40 mg/dL or non-HDL cholesterol >145 mg/dL. Missing data were supplemented using multiple imputation. The prevalence of dyslipidaemia was 85.1% in peritoneal dialysis (PD) patients, 76.1% in haemodialysis (HD) patients and 55.5% among renal allograft recipients. Both low and high body mass index (BMI) were associated with a less favourable lipid profile. Younger age was associated with a worse lipid profile among PD patients. HDL levels significantly improved after transplantation, whereas no significant improvements were found for triglyceride and non-HDL levels. In transplant recipients, use of cyclosporin was associated with significantly higher non-HDL and HDL levels than tacrolimus usage (P < 0.01). In transplant patients with eGFR < 29 mL/min/1.73 m(2), the mean triglyceride level was 137 mg/dL (99% confidence interval (CI): 119-159) compared with 102 mg/dL among those with eGFR > 90 mL/min/1.73 m(2) (P < 0.0001). Dyslipidaemia is common among paediatric ESRD patients in Europe. Young age and PD treatment are associated with worse lipid profiles. Although lipid levels generally improve after transplantation, dyslipidaemia may persist due to decreased graft function, high BMI or to the use of certain immunosuppressants.
    Nephrology Dialysis Transplantation 10/2013; · 3.37 Impact Factor
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    ABSTRACT: Integrin α(3) is a transmembrane integrin receptor subunit that mediates signals between the cells and their microenvironment. We identified three patients with homozygous mutations in the integrin α(3) gene that were associated with disrupted basement-membrane structures and compromised barrier functions in kidney, lung, and skin. The patients had a multiorgan disorder that included congenital nephrotic syndrome, interstitial lung disease, and epidermolysis bullosa. The renal and respiratory features predominated, and the lung involvement accounted for the lethal course of the disease. Although skin fragility was mild, it provided clues to the diagnosis.
    New England Journal of Medicine 04/2012; 366(16):1508-14. · 54.42 Impact Factor
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    Thomas J Neuhaus, Guido F Laube
    Pediatric Nephrology 12/2011; 27(3):505. · 2.94 Impact Factor
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    ABSTRACT: Nephropathic cystinosis (NC) is an autosomal recessive disorder occurring in one to two per 100,000 newborns. Because of the rarity of NC, long-term outcome data are scarce. 245 NC patients from 18 countries provided data to the ESPN/ERA-EDTA registry. We matched NC patients on renal replacement therapy (RRT) to non-NC children on RRT. Between 1979 and 2008, mean age at the start of RRT among NC children increased by 0.15 year per calendar year (95% confidence interval, 0.10 to 0.21) from 8.8 to 12.7 years, whereas we did not observe this in non-NC children. Five-year survival after the start of RRT improved in NC patients from 86.1% (before 1990) to 100% (since 2000) as compared with the control population (89.6% and 94.0%). NC patients received a renal allograft more often (relative risk, 1.09; 95% confidence interval, 1.00 to 1.17) as compared with matched RRT children, and 5-year graft survival was better (94.0% versus 84.0%). NC dialysis patients were less often hypertensive than non-NC children matched for age, country, and dialysis modality (42.7% versus 51.7%) and had lower parathyroid hormone levels (median, 56 versus 140 pg/ml). Although height at start of RRT slightly improved during the past decade, children with NC remained significantly shorter than non-NC children at the start of RRT. We demonstrated improved survival of the renal function as well as better patient and graft survival after the start of RRT in a large European cohort of NC patients over the last two decades.
    Clinical Journal of the American Society of Nephrology 08/2011; 6(10):2485-91. · 5.07 Impact Factor
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    ABSTRACT: Clinical gout has rarely been described after pediatric renal transplantation (RTx), although asymptomatic hyperuricemia is common in these patients. We describe three male pediatric patients who presented with gouty arthritis 7-8.5 years following RTx. Since receiving allopurinol, all patients had been free of gouty symptoms. To prevent severe bone marrow depletion, the dosage of azathioprine, an immunosupressant drug, was reduced by 50% to prevent interaction with allopurinol. Because atypical presentation of gout can occur, a high index of suspicion is needed to allow appropriate diagnosis of this disease in patients with skeletal pain after RTx.
    Pediatric Nephrology 12/2010; 25(12):2535-8. · 2.94 Impact Factor
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    ABSTRACT: The aim of this study was to estimate the frequency and severity of ocular involvement in paediatric patients with haemolytic uraemic syndrome (HUS). The study was designed as an institutional, retrospective, observational case series. Charts for all 87 paediatric patients with HUS treated at the University Children's Hospital Zurich between 1995 and 2007 were reviewed. Patients with ocular involvement were identified and clinical findings presented. Three of 69 examined patients with HUS showed ocular involvement. Ophthalmic findings in two children were consistent with bilateral Purtscher retinopathy, showing multiple haemorrhages, exudations and superficial retinal whitening. The third child presented with bilateral isolated central intraretinal haemorrhages as a milder form of ocular involvement. In one of the children with Purtscher retinopathy, laser photocoagulation was required for bilateral rubeosis irides and development of disc neovascularization. Longterm outcomes in the two severely affected children showed decreased visual acuity caused by partial atrophy of the optic nerves. In the milder case visual acuity was not impaired at any time. A minority of paediatric patients with HUS developed ocular involvement. Acute ocular findings varied in severity from isolated intraretinal haemorrhages to Purtscher-like retinopathy with retinal ischaemia. Longterm complications included the development of neovascularizations and consecutive optic nerve atrophy. Although ocular involvement in HUS seems to be rare, physicians should be aware of this complication because of its possible vision-endangering consequences.
    Acta ophthalmologica 08/2009; 88(7):804-7. · 2.44 Impact Factor
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    ABSTRACT: Cystinosis is a rare multisystemic progressive disorder mandating lifelong medical treatment. Knowledge on the intellectual and motor functioning, health-related quality of life and psychosocial adjustment in children with cystinosis is limited. We have investigated nine patients (four after renal transplantation) at a median age of 9.7 years (range 5.3-19.9 years). Intellectual performance (IP) was analysed with the Wechsler Intelligence Scale for Children-III (seven children) and the Kaufman Assessment Battery for Children (two children). Motor performance (MP) was evaluated using the Zurich Neuromotor Assessment Test, and quality of life (QOL) was studied by means of the Netherlands Organization for Applied Scientific Research Academical Medical Center Child Quality of Life Questionnaire. Psychosocial adjustment was assessed by the Child Behavior Checklist. The overall intelligence quotient (IQ) of our patient cohort (median 92, range 71-105) was significantly lower than that of the healthy controls (p = 0.04), with two patients having an IQ < 85. Verbal IQ (93, range 76-118) was significantly higher than performance IQ (90, range 68-97; p = 0.03). The MP was significantly below the norm for pure motor, pegboard and static balance, as well as for movement quality. The patients' QOL was normal for six of seven dimensions (exception being positive emotions), whereas parents reported significant impairment in positive emotions, autonomy, social and cognitive functions. Significant disturbance was noted in terms of psychosocial adjustment. Based on the results from our small patient cohort, we conclude that intellectual and motor performance, health-related QOL and psychosocial adjustment are significantly impaired in children and adolescents with cystinosis.
    Pediatric Nephrology 03/2009; 24(7):1371-8. · 2.94 Impact Factor
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    ABSTRACT: The aim of this substudy within a prospective, multicenter, placebo-controlled trial was to assess the pharmacokinetics and immunodynamics of basiliximab in pediatric renal transplant recipients on comedication with mycophenolate mofetil (MMF). Eighty-two patients aged 3 to 18 years, receiving cyclosporine microemulsion, MMF, corticosteroids, and basiliximab or placebo were investigated. Basiliximab serum concentrations were determined by ELISA, CD25+, and CD122+ T lymphocytes by flow cytometry. Basiliximab clearance adjusted to body surface area was significantly (P<0.05) greater in children versus adults, but the relatively higher basiliximab dose given to children yielded similar exposure compared with adolescents. A cross-study comparison revealed that MMF reduced basiliximab clearance and prolonged CD25 saturation duration from approximately 5 weeks in the absence of MMF to 10 weeks in the presence of MMF. Basiliximab led to a marked reduction of CD25+ T-cell fraction during the first 8 to 10 weeks posttransplant, but did not specifically affect CD122+ T cells. The majority of biopsy-proven acute rejection episodes (BPAR) were observed after interleukin (IL) 2-R desaturation, whereas about a quarter of BPARs occurred despite adequate IL2-R blockade. The currently recommended basiliximab dose for pediatric patients, when used with cyclosporine microemulsion and corticosteroids, yielded adequate drug exposure in children and adolescents also under MMF comedication. The observation that about a quarter of BPARs occurred despite adequate IL2-R blockade suggests that another T-cell activation pathway independent of the IL-2/IL-2R pathway is operative, for example, the IL-15 signaling pathway.
    Transplantation 12/2008; 86(9):1234-40. · 3.78 Impact Factor
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    ABSTRACT: Knowledge of health-related quality of life (QOL) and psychosocial adjustment (PA) in children after renal transplantation (RTPL) is limited. QOL and PA were evaluated by standardized tests in patients after RTPL. Thirty-seven children of median age 14.5 years (range 6.5-17 years) were investigated a mean 4.5 years (range 0.5-12.8 years) after RTPL. Child- and parent-rated QOL was evaluated with the Child Quality of life Questionnaire of The Netherlands Organization for Applied Scientific Research Academical Medical Centre (TNO-AZL). PA was assessed by the Child Behaviour Checklist (CBCL) providing parental reports of a child's behaviour. In patients' self-ratings, the QOL dimension physical complaints (P < 0.0005) scored significantly better than that of healthy controls, whereas the dimension positive emotional functioning was impaired (P = 0.02). Parents rated motor functioning (P = 0.002), autonomy (P = 0.01), cognition (P = 0.04) and positive emotions (P < 0.0005) as significantly impaired. Parents also assessed PA significantly (P = 0.02) impaired with regard to internalizing behaviour. Dialysis duration, young age at RTPL, living-related donation, steroid treatment, adverse family relationships and maternal distress had a significantly negative impact on QOL and PA (P < 0.05). Patients rated QOL higher than did healthy controls. Parents evaluated their children's QOL and PA more pessimistically than did the patients themselves. Both illness-related variables and family environment played an important role.
    Pediatric Nephrology 09/2008; 23(8):1347-54. · 2.94 Impact Factor
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    ABSTRACT: Comprehensive information on neurodevelopmental outcome in children and adolescents with chronic kidney disease is still limited. Intellectual performance (IP) and motor performance (MP) were systematically assessed in 27 patients at a median age of 14.1 years (range 6.5-17) and 6 years (range 0.5-12.7) after renal transplantation (RTPL). IP was analyzed with the Wechsler Intelligence Scale for Children-III (WISC-III) in 25 patients and by the Kaufman Assessment Battery for Children in two patients. MP was evaluated by the Zurich Neuromotor Assessment. Median full- scale intelligent quotient (FSIQ) was 97 (range 49-133). Twenty-one patients had an FSIQ >or= 85 (i.e. >or= mean-1 standard deviation). The five patients with neurological comorbidity had a median FSIQ of 81 (range 49-101). Verbal IQ (VIQ) (median 104; range 50-146) was significantly (p < 0.01) higher than performance IQ (PIQ) (median 88; range 48-117). The PIQ was significantly lower compared with controls (p < 0.007), and patients scored significantly lower compared with controls in five of 11 subtests of the Wechsler Scale. All MP tasks were significantly (p < 0.01) lower than in controls, and also in children without neurological comorbidity. Socioeconomic status was significantly correlated with FSIQ (p = 0.03). IP after RTPL was within the normal range for the majority of children. PIQ was lower compared with VIQ, and MP was significantly affected in all children after RTPL.
    Pediatric Nephrology 08/2008; 23(8):1339-45. · 2.94 Impact Factor
  • Ultraschall in Der Medizin - ULTRASCHALL MED. 01/2008; 29.
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    ABSTRACT: Steroid withdrawal (SW) after paediatric renal transplantation (RTPL) is controversial. Selective late SW has been performed in our unit since 1995. The safety and effects of SW were analysed retrospectively in 47 patients undergoing RTPL between 1995 and 2004. Initial immunosuppression consisted of cyclosporine A, azathioprine or mycophenolate mofetil and steroids. Criteria for SW were: (1) stable renal function, (2) time interval after RTPL > or = 1 year, (3) no rejection or time interval after last rejection > or = 1 year and (4) good compliance. SW was performed in 30 patients at an age of 13.5 years (range 4.5-18.5) and 2.2 years (range 1-6.6) after RTPL. After SW, one patient experienced a steroid-sensitive rejection. Follow-up after SW (1.3 year; range 0.25-7.5) showed maintained renal function: glomerular filtration rate at SW and currently was 82 (65-128) and 82 (42-115) ml/min per 1.73 m(2), respectively. The number of patients on antihypertensive treatment did not significantly change (at SW: n = 15; currently: n = 11). Height and body mass index (BMI) remained stable: Median standard deviation score (SDS) for height/BMI at SW and currently was -1.1/0.2 and -0.8/0.1, respectively. Selective late SW was safe regarding renal function and had no significant effect on blood pressure and growth.
    Pediatric Nephrology 11/2007; 22(11):1947-52. · 2.94 Impact Factor
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    ABSTRACT: To identify prognostic factors of survival in pediatric post-transplantation lymphoproliferative disorder (PTLD) after solid organ transplantation. A multicenter, retrospective case analysis of 55 pediatric solid organ graft recipients (kidney, liver, heart/lung) developing PTLD were reported to the German Pediatric-PTLD registry. Patient charts were analyzed for tumor characteristics (histology, immunophenotypes, cytogenetics, Epstein-Barr virus [EBV] detection), stage, treatment, and outcome. Probability of overall and event-free survival was analyzed in defined subgroups using univariate and Cox regression analyses. PTLD was diagnosed at a median time of 29 months after organ transplantation, with a significantly shorter lag time in liver (0.83 years) versus heart or renal graft recipients (3.33 and 3.10 years, respectively; P = .001). The 5-year overall and event-free survival was 68% and 59%, respectively, with 59% of patients surviving 10 years. Stage IV disease with bone marrow and/or CNS involvement was associated independently with poor survival (P = .0005). No differences in outcome were observed between early- and late-onset PTLD, monomorphic or polymorphic PTLD, and EBV-positive or EBV-negative PTLD, respectively. Patients with Burkitt or Burkitt-like PTLD and c-myc translocations had short survival (< 1 year). Stage IV disease is an independent risk factor for poor survival in pediatric PTLD patients. Prospective multicenter trials are needed to delineate additional risk factors and to assess treatment approaches for pediatric PTLD.
    Journal of Clinical Oncology 11/2007; 25(31):4902-8. · 18.04 Impact Factor
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    ABSTRACT: Fetal exposure to angiotensin-converting enzyme inhibitors (ACEIs) is associated with increased neonatal morbidity and mortality. Long-term follow-up of three patients with fetal ACEI exposure revealed impaired renal function in two, severe hypertension and proteinuria in one and isolated polycythaemia in all three. Careful long-term follow-up of children with ACEI fetopathy is recommended.
    Archives of Disease in Childhood - Fetal and Neonatal Edition 10/2007; 92(5):F402-3. · 3.45 Impact Factor
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    ABSTRACT: Laparoscopic living kidney nephrectomy is thought to be associated with reduced morbidity, when compared to open nephrectomy. The purpose of this study was to explore the impact of these techniques on donors' clinical outcomes, satisfaction and motivation to donate. Clinical outcomes were retrospectively compared in 152 open (n = 71) or laparoscopic (n = 81) donor procedures. Donor satisfaction and motivation were assessed with a self-administered questionnaire. The complication rate was the same with both procedures and the majority of complications were mild. Laparoscopy was significantly less painful and resulted in an insignificantly faster return to active life. More than 80% of the donors volunteered to donate without pressure. Worries about future health status, pain or scars were not important in the decision to donate. Similarly, only 15% considered the surgical procedure as instrumental for their decision. Few donors currently worried about their health with one kidney and more than 95% of the donors in both groups stated that they would give their kidney again. Living donor nephrectomy is safe, regardless of the procedure used. Although the laparoscopic nephrectomy offers clear short-term benefits over the open nephrectomy, donors' satisfaction was excellent with both surgical approaches. Moreover, the type of procedure did not seem to influence their decision to donate.
    Nephrology Dialysis Transplantation 10/2006; 21(9):2563-8. · 3.37 Impact Factor
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    ABSTRACT: We have determined levels of glutathione (GSH), ATP, mitochondrial complex activity and apoptosis rate in proximal tubular cells (PTCs) exfoliated from urine in cystinotic (n=9) and control (n=9) children. Intracellular GSH was significantly depleted in cystinotic PTCs compared with controls (6.8 nmol GSH/mg protein vs 11.8 nmol GSH/mg protein; P<0.001), but there were no significant differences in mitochondrial complex activities or ATP levels under basal conditions. Cystinotic PTCs showed significantly increased apoptosis rate. After PTCs had been stressed by hypoxia, there was further depletion of GSH in cystinotic and control PTCs (2.4 nmol GSH/mg protein vs 7.2 nmol GSH/mg protein; P<0.001). Hypoxic stress led to increased complex I and complex IV activities in control but not in cystinotic PTCs. ATP levels were significantly reduced in cystinotic PTCs after hypoxic stress (12.2 nmol/mg protein vs 26.9 nmol/mg protein; P<0.001). GSH depletion occurs in this in vitro model of cystinotic PTCs, is exaggerated by hypoxic stress and may contribute to reduced ATP and failure to increase complex I/IV activities. Apoptotic rate is also increased, and these mechanisms may contribute to cellular dysfunction in cultured, human cystinotic PTCs.
    Pediatric Nephrology 05/2006; 21(4):503-9. · 2.94 Impact Factor
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    ABSTRACT: En bloc renal transplantation (EBT) from infant donors is an option for children with end-stage renal failure. Owing to potential complications, EBT is not performed in all paediatric nephrology centres. We evaluated the perioperative and long-term course of five children undergoing EBT. Primary diagnosis was atypical (diarrhoea-negative) haemolytic uraemic syndrome (n=2), interstitial nephropathy (two siblings) and branchio-oto-renal syndrome (n=1). Recipient and donor ages ranged between 5.9 and 11.1 years and 0.3 and 2.5 years, respectively. Follow-up time after EBT was 2.1-13.2 years. Perioperative complications included (1) a renal artery thrombosis, with immediate intraoperative reconstruction and primary non-functioning of the graft, with recovery after 10 days, and (2) a vesico-ureteric obstruction, successfully managed with temporary insertion of a JJ-catheter. All grafts had good long-term function. Absolute glomerular filtration rate (GFR; millilitres/minute) increased in all patients, whereas relative GFR (millilitres/minute per 1.73 m(2) body surface area) remained stable during the follow-up period in all but one. Kidney size increased significantly, with maximal growth during the first year after EBT; magnetic resonance imaging (MRI) showed normal structure and vasculature. EBT is a safe and effective option for young children with end-stage renal failure. Absolute GFR and graft size increase and adapt to the children's growing body mass.
    Pediatric Nephrology 04/2006; 21(3):408-12. · 2.94 Impact Factor
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    ABSTRACT: Long-term outcome of steroid-sensitive idiopathic nephrotic syndrome (SSNS) in children is usually considered benign, although data on follow-up into adulthood are scarce. The aim of this study was to investigate adults who had childhood SSNS regarding their relapse rate, growth, and renal and extrarenal morbidity. Adult patients (n=42, 26 males) were evaluated at a median age of 28.0 (18.1 to 46.9) years and a median follow-up of 22.0 (2.9 to 39.0) years since diagnosis. Fourteen of 42 (33%) patients relapsed in adulthood. The number of relapses during childhood and adolescence and a complicated course-administration of steroid-sparing medication such as cyclophosphamide, chlorambucil, and cyclosporin A-were identified as risk factors. Final adult height (median SD score -0.4, range -3.3 to +1.3) and body mass index (BMI) were normal. Renal function was normal in all patients, and overall morbidity was low. Only eight patients (three males) had children. Cytotoxic therapy was identified as a major factor contributing to childlessness. Relapses in adulthood were common in pediatric patients with SSNS. Growth and renal function were normal, and overall morbidity was low. Yet, transition to an adult nephrologist is recommended for all children with relapsing SSNS.
    Journal of Pediatrics 09/2005; 147(2):202-7. · 4.04 Impact Factor
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    ABSTRACT: Living-related kidney transplantation (LRKT) is an option for children with end-stage renal failure. In addition to medical concerns, there is uncertainty about the psychological impact of living-related donation on parent donors and families. A survey was conducted on the decision making process and medical and psychosocial consequences of LRKT. Between 1992 and 1999, 20 parents donated a kidney for their child. A questionnaire including 24 items was sent to parent donors and their partners. Nineteen parents and partners responded; the median time after LRKT was 3 years. Donors and partners reported an independent decision making process with no significant influence of partners, relatives, or hospital staff. Partners were more concerned about medical problems than donors themselves (P <0.02). Donors and partners cited no medical problems except sustained pain. Both reported an improved personal relationship towards the transplanted child. Donors and partners also cited an improved personal relationship. The vast majority (18/19) of couples still supported the decision for organ donation. In conclusion, there was a high degree of satisfaction with the decision making process in LRKT. The great majority of donors and partners did not report negative medical or psychological consequences. The relationship between donor, partner, and recipient child improved after LRKT.
    Pediatric Nephrology 03/2005; 20(2):205-9. · 2.94 Impact Factor

Publication Stats

315 Citations
146.55 Total Impact Points

Institutions

  • 2001–2013
    • University Children's Hospital Basel
      Bâle, Basel-City, Switzerland
  • 2012
    • Universitätsklinikum Freiburg
      • Department of Dermatology and Venereology
      Freiburg, Lower Saxony, Germany
  • 2011
    • Luzerner Kantonsspital
      Luzern, Lucerne, Switzerland
  • 2005–2011
    • University of Zurich
      • Ophthalmology Unit
      Zürich, Zurich, Switzerland
  • 2003–2004
    • Great Ormond Street Hospital for Children NHS Foundation Trust
      Londinium, England, United Kingdom