Background and objectives Steroid-resistant nephrotic syndrome is a rare kidney disease involving either immune-mediated or genetic alterations of podocyte structure and function. The rare nature, heterogeneity, and slow evolution of the disorder are major obstacles to systematic genotype-phenotype, intervention, and outcome studies, hampering the development of evidence-based diagnostic and therapeutic concepts. To overcome these limitations, the PodoNet Consortium has created an international registry for congenital nephrotic syndrome and childhood-onset steroid-resistant nephrotic syndrome.

Intensified blood-pressure control, with target 24-hour blood-pressure levels in the low range of normal, confers a substantial benefit with respect to renal function among children with chronic kidney disease. Reappearance of proteinuria after initial successful pharmacologic blood-pressure control is common among children who are receiving long-term ACE inhibition. (ClinicalTrials.gov number, NCT00221845.)

These data indicate high prevalence of hypertension in children with autosomal dominant polycystic kidney disease starting at young ages.

We report here the first Portuguese case of acute fatal granulomatous encephalitis attributed to Balamuthia mandrillaris, initially thought to be a brain tumor, which had a progressive and fatal outcome. Balamuthia mandrillaris is a free-living amoeba recognized as an uncommon agent of granulomatous encephalitis. Infections have been identified in immunocompromised hosts and in immunocompetent pediatric patients. Balamuthia infections are very rare, with only two reported cases in Europe. The case presented here occurred in a previously healthy boy who died 5 weeks after the onset of the symptoms. No evidence of immunological deficiency was noted, and testing for human immunodeficiency virus antibodies was negative. The symptoms were initially thought to be the result of a tumor, but histopathologic examination showed evidence of amoebic infection. Immunofluorescence staining of brain tissue identified B. mandrillaris as the infectious agent. The diagnosis was confirmed with PCR by detecting Balamuthia DNA in formalin-fixed brain tissue sections. Despite initiation of empirical antimicrobial therapy for balamuthiasis, the patient died 3 weeks after being admitted to the hospital. No source of infection was readily apparent. CASE REPORTAn 8-year-old male was transferred to our hospital because of signs of intracranial hypertension. His past medical history was unremarkable except for an episode of varicella infection a year before. Prior to his illness, he practiced swimming in several swimming pools but had not traveled outside Portugal. Symptoms with sporadic headaches started a few months earlier but had become daily for the 2 weeks prior to admission. His parents noted medial esotropia 2 days before admission, and diplopia developed 1 day before. His headaches were more intense in the morning, he had frequent episodes of vomiting and lethargy, and papilledema was noted upon physical examination.Upon admission he appeared well, with a good general status, and his speech was age appropriate. After unilateral eye occlusion, he had no visual defects. Mycobacterium tuberculosis infection was ruled out from urine and sputum samples, and the tuberculin test (2U, SC) was negative. Human immunodeficiency virus type 1 (HIV-1) and HIV-2 serum antibodies and immunologic studies all showed no changes.Fundoscopy revealed bilateral papilledema and right retinal hemorrhages. Neither alteration of consciousness nor neurological focal deficits were noted. There was no fever. Noncontrast computerized tomography (CT) scanning revealed an expansive hypodense lesion in the right frontal lobe, causing distortion of the middle line and ventricular thinning. With contrast enhancement, a heterogeneous cortical lesion surrounded by a huge area of edema was noted for the first time in the right temporal lobe (Fig. 1A and B). A brain tumor was suspected, and dexamethasone was started because of the clinical symptoms. Upon preoperative magnetic resonance imaging (MRI), the frontal lesion was heterogeneous, with hypodense inner and hyperin...