Efficacy and safety of Oxalobacter formigenes to reduce urinary oxalate in primary hyperoxaluria.

Background: Primary hyperoxaluria (PH) is a rare genetic disease, in which high urinary oxalate (Uox) cause recurrent kidney stones and/or progressive nephrocalcinosis, often followed by early end-stage renal disease, as well as extremely high plasma oxalate, systemic oxalosis and premature death. Oxalobacter formigenes, an anaerobic oxalate degrading bacterium, naturally colonizes the colon of most humans. Orally administered O.

New therapies in steroid-sensitive and steroid-resistant idiopathic nephrotic syndrome.

Although many children with idiopathic nephrotic syndrome (INS) respond initially to steroid therapy, repeated courses for patients with relapses often cause significant steroid toxicity. Patients with frequent relapses who develop steroid dependency thus require alternative treatment. The first such options have been considered to be cyclophosphamide or levamisole, although the latter is no longer available in many countries.

Pathogenesis, diagnosis and management of hyperkalemia.

Hyperkalemia is a potentially life-threatening condition in which serum potassium exceeds 5.5 mmol/l. It can be caused by reduced renal excretion, excessive intake or leakage of potassium from the intracellular space.

Tim-3 expression on PD-1+ HCV-specific human CTLs is associated with viral persistence, and its blockade restores hepatocyte-directed in vitro cytotoxicity.

Having successfully developed mechanisms to evade immune clearance, hepatitis C virus (HCV) establishes persistent infection in approximately 75%-80% of patients. In these individuals, the function of HCV-specific CD8+ T cells is impaired by ligation of inhibitory receptors, the repertoire of which has expanded considerably in the past few years. We hypothesized that the coexpression of the negative regulatory receptors T cell immunoglobulin and mucin domain-containing molecule 3 (Tim-3) and programmed death 1 (PD-1) in HCV infection would identify patients at risk of developing viral persistence during and after acute HCV infection.

High prevalence of renal dysfunction in children after liver transplantation: non-invasive diagnosis using a cystatin C-based equation.

Background: Chronic kidney disease (CKD) has been increasingly shown to be a negative prognostic factor after liver transplantation (Ltx). Creatinine-based glomerular filtration rate (GFR) formulas are notoriously insensitive. In children, non-invasive determination of GFR by measurement of serum cystatin C is feasible and repeatedly correlated to the gold standards of GFR measurements.

Immunosuppression and renal outcome in congenital and pediatric steroid-resistant nephrotic syndrome.

Background And Objectives: Mutations in podocyte genes are associated with steroid-resistant nephrotic syndrome (SRNS), mostly affecting younger age groups. To date, it is unclear whether these patients benefit from intensified immunosuppression with cyclosporine A (CsA). The aim of this study was to evaluate the influence of podocyte gene defects in congenital nephrotic syndrome (CNS) and pediatric SRNS on the efficacy of CsA therapy and preservation of renal function.

[Lambliasis as differential diagnosis of MARSH type 3b].

Giardia lamblia is the most common human parasite with a worldwide distribution and fecal-oral way of transmission. Diagnostic procedures include stool examination and gastroduodenoscopy with biopsy or secret aspiration. In most cases histology reveals a dense accumulation of the parasites on the surface of the duodenal mucosa with no or only slight inflammation.

Second-line options for refractory steroid-sensitive and -resistant nephrotic syndrome.

Although initially, many children with idiopathic nephrotic syndrome respond to steroid therapy, a repeated course for patients with relapses often causes significant steroid toxicity. Patients with frequent relapses or steroid dependency thus require alternative treatment, and so far, cyclophosphamide or levamisole have been regarded as first-choice options, although the latter is no longer available in many countries. Data are accumulating that mycophenolic acid may be an alternative for these patients.

Mutations in the human laminin beta2 (LAMB2) gene and the associated phenotypic spectrum.

Mutations of LAMB2 typically cause autosomal recessive Pierson syndrome, a disorder characterized by congenital nephrotic syndrome, ocular and neurologic abnormalities, but may occasionally be associated with milder or oligosymptomatic disease variants. LAMB2 encodes the basement membrane protein laminin beta2, which is incorporated in specific heterotrimeric laminin isoforms and has an expression pattern corresponding to the pattern of organ manifestations in Pierson syndrome. Herein we review all previously reported and several novel LAMB2 mutations in relation to the associated phenotype in patients from 39 unrelated families.

Fibroblast growth factor 23 and bone metabolism in children with chronic kidney disease.

Fibroblast growth factor 23 (FGF23) is a circulating protein that regulates the renal reabsorption of phosphate and also inhibits 1-alpha-hydroxylase production. In adults FGF23 is increased in chronic kidney disease (CKD) and is an important prognostic factor for cardiovascular morbidity. In order to gain insight into the role of FGF23 and other biochemical variables of bone metabolism in children we studied 69 patients at different stages of CKD.

[Medical education in otorhinolaryngology in Germany. Implementation of the new licensing regulations for physicians].

Background: In 2003 new regulations for the licensing of German physicians came into effect. The aim of this study was to survey the present status of realization in German otorhinolaryngology (ORL) university departments.

Methods: A questionnaire containing 31 items was sent to all German ORL university departments.

Protective effect of human leukocyte antigen B27 in hepatitis C virus infection requires the presence of a genotype-specific immunodominant CD8+ T-cell epitope.

Unlabelled: Human leukocyte antigen B27 (HLA-B27) is associated with protection in human immunodeficiency virus (HIV) and hepatitis C virus (HCV) infection. This protective role is linked to single immunodominant HLA-B27-restricted CD8+ T-cell epitopes in both infections. In order to define the relative contribution of a specific HLA-B27-restricted epitope to the natural course of HCV infection, we compared the biological impact of the highly conserved HCV genotype 1 epitope, for which the protective role has been described, with the corresponding region in genotype 3 that differs in its sequence by three amino acid residues.

The management of cervical intraepithelial neoplasia during pregnancy: is colposcopy necessary?

Objective: The 2006 American Society for Colposcopy and Cervical Pathology Consensus guidelines state that it is acceptable to defer colposcopy until 6 weeks postpartum in pregnant patients with atypical squamous cells of undetermined significance (ASC-US) or low-grade squamous intraepithelial lesion (LSIL) cytology. Therefore, we sought to determine the incidence of cervical intraepithelial neoplasia (CIN) 2,3 in pregnant patients referred to a university colposcopy clinic.

Materials And Methods: A retrospective study identified all pregnant women with abnormal cytology referred to the University of Alabama at Birmingham colposcopy clinic between May 2005 and September 2007.

The timing of normalization of CA-125 levels during primary chemotherapy is predictive of survival in patients with epithelial ovarian cancer.

Objective: To determine if the timing of normalization of CA-125 levels during primary chemotherapy for epithelial ovarian cancer (EOC) could predict survival.

Methods: Patients who reached a complete clinical response for EOC with primary taxane/platinum-based chemotherapy were eligible. Patient demographics, chemotherapy administration, CA-125 levels, and survival outcomes were abstracted.

Transplantation procedures in children with primary hyperoxaluria type 1: outcome and longitudinal growth.

Background: Cure of the metabolic defect in primary hyperoxaluria type 1 (PH1) is possible with liver transplantation (LTx). Preemptive LTx (PLTx) was promoted to prevent chronic kidney disease due to nephrocalcinosis and urolithiasis. However, timing of this procedure is difficult in view of the heterogeneity of PH1 and effective conservative treatment.

Relaxin supports implantation and early pregnancy in the marmoset monkey.

To test the hypothesis that relaxin is an important factor supporting implantation, two approaches have been carried out using a human-relevant animal model, the marmoset monkey. First, uterine mRNA transcription and protein expression during the implantation phase in the conceptive and nonconceptive cycles were examined. Second, functional parameters were analyzed to assess the in vivo effects of exogenous applied relaxin throughout implantation.

Prevalence of polyomavirus viruria (JC virus/BK virus) in children following liver transplantation.

BKV infection is a potential cause of renal dysfunction in non-renal organ transplant recipients. JCV is the causative agent of PML. Furthermore, polyomaviruses are tumor inducing viruses and molecular data suggest an association with malignancies among solid organ transplant patients.

Urinary proteome pattern in children with renal Fanconi syndrome.

Background: The renal Fanconi syndrome (FS) is characterized by renal glucosuria, loss of electrolytes, bicarbonate and lactate, generalized hyperaminoaciduria and low-molecular-weight proteinuria. We studied the urinary low-molecular-weight proteome to identify excreted peptides indicative of a pathogenetic mechanism leading to tubular dysfunction.

Methods: We established a urinary proteome pattern using capillary electrophoresis mass spectrometry (CE-MS) of 7 paediatric patients with cystinosis and 6 patients with ifosfamide-induced FS as the study group, and 54 healthy volunteers and 45 patients suffering from other renal diseases such as lupus nephritis (n = 8), focal segmental glomerulosclerosis (n = 27), minimal change disease (n = 7) and membranous glomerulonephritis (n = 3) as controls.

Diagnostic examination of the child with urolithiasis or nephrocalcinosis.

Urolithiasis and nephrocalcinosis are more frequent in children then currently anticipated, but still remain under- or misdiagnosed in a significant proportion of patients, since symptoms and signs may be subtle or misleading. All children with colicky abdominal pain or macroscopic hematuria should be examined thoroughly for urolithiasis. Also, other, more general, abdominal manifestations can be the first symptoms of renal stones.

The effect of obesity on survival in patients with ovarian cancer.

Objective: Data has suggested obesity as an independent prognostic factor for lower survival in patients with epithelial ovarian cancer (EOC). We sought to determine if obesity portends a disadvantage to surgical outcomes at the time of initial surgery affecting survival.

Methods: A retrospective chart review of patients diagnosed with EOC was performed.