Abstract Dent disease is a rare X-linked progressive renal disease characterized by hypercalciuria and low molecular weight proteinuria. In early childhood, patients may manifest only proteinuria and/or hypercalciuria, both of which are generally asymptomatic. The authors report three cases of male patients with asymptomatic, non-orthostatic nephrotic proteinuria first detected during screening for an acute condition. All had intermittent hypercalciuria, one had nephrocalcinosis, and one had mild hypotonia and some learning difficulties. Genetic testing identified pathogenic variants in all patients, two in the OCRL gene and one in the CLCN5 gene. To date, none have developed chronic kidney disease. The diagnosis of Dent disease can be challenging, but it should be considered in all men with unexplained proteinuria. This approach may not only allow for earlier diagnosis, but also avoid unnecessary testing and potentially harmful treatments. Xlinked X linked childhood andor or asymptomatic nonorthostatic non orthostatic condition nephrocalcinosis difficulties CLCN date challenging treatments

Resumo A doença de Dent é uma tubulopatia rara e progressiva ligada ao cromossoma X, caracterizada por hipercalciúria e proteinúria de baixo peso molecular. Na primeira década, os doentes manifestam apenas proteinúria e/ou hipercalciúria, sendo ambas geralmente assintomáticas. Posteriormente, os sintomas podem evoluir para insuficiência renal crónica. São apresentados três casos de crianças do sexo masculino com proteinúria nefrótica não ortostática assintomática detetada em contexto de doença aguda. Todas as crianças apresentaram hipercalciúria intermitente, uma apresentou nefrocalcinose e outra hipotonia ligeira e dificuldades de aprendizagem. O estudo genético identificou variantes patogénicas em todos os doentes, duas no gene OCRL e uma no gene CLCN5. Nenhum doente apresentou deterioração da função renal. O diagnóstico de doença de Dent pode ser desafiante, devendo ser considerado em casos de proteinúria inexplicável no género masculino. Esta abordagem permitirá, não só antecipar o diagnóstico, como evitar investigação e tratamentos desnecessários. X molecular década eou ou assintomáticas Posteriormente crónica aguda intermitente aprendizagem CLCN5 CLCN desafiante permitirá desnecessários

ABSTRACT Hypomagnesemia type I is a rare autosomal recessive disorder characterized by severe hypomagnesemia, often accompanied by hypocalcemia. This disease is caused by mutations in the TRPM6 gene (which encodes the respective channel), leading to reduced intestinal absorption of magnesium and increased renal excretion due to a defect in reabsorption in the distal convoluted tubule. It usually manifests itself in the first months of life, with symptoms of neuromuscular hyperexcitability and seizures, refractory to antiepileptic therapy. Treatment consists of administering high doses of magnesium throughout life. Here, we report a case of hypomagnesemia type I with a novel pathogenic variant of TRPM6 in a 5-month-old girl who developed refractory seizures due to hypomagnesemia.

ABSTRACT Membranoproliferative glomerulonephritis is a rare chronic glomerulonephritis in pediatric age. It may be a primary disorder or secondary to other chronic diseases. Diagnose of membranoproliferative glomerulonephritis is based on specific kidney biopsy findings. The prognostic factors, the outcome and the most suitable treatment regimen are incompletely known in children. We report a case of a 3-year-old boy that presented with nephritic/nephrotic syndrome and hypocomplementemia. After 10 days under corticosteroid therapy, he maintained worsening of renal function and a renal biopsy specimen revealed a membranoproliferative glomerulonephritis. Autoimmune disease and infections were initially excluded, but further investigation revealed positive Polymerase Chain Reaction for cytomegalovirus in blood and urine. Cytomegalovirus genome at biopsy was not identified. He completed six months of treatment with valganciclovir and corticosteroids with total remission of proteinuria and undetected viral loads. With this case, we would like to draw attention to a type of nephropathy that is rare in childhood.

Resumo Histórico A psoríase é uma doença crônica imunomediada que afeta principalmente a pele tanto em adultos quanto em crianças, mas também pode ter envolvimento sistêmico, particularmente com artrite e lesão renal. A nefropatia por IgA é o distúrbio renal mais frequentemente associado à psoríase. Aproximadamente um terço de todos os casos de psoríase começam na infância, mas a associação entre psoríase e distúrbios renais tem sido pouco relatada em pacientes pediátricos. A Púrpura de Henoch-Schönlein (PHS) é uma vasculite sistêmica caracterizada por depósitos de IgA nas paredes dos vasos de órgãos afetados e no mesângio do rim. A histopatologia da nefrite da PHS é idêntica à da nefropatia por IgA. Relato de caso Um menino de 6 anos de idade com início recente de psoríase desenvolveu PHS com envolvimento renal, clinicamente manifestado por proteinúria nefrótica e hematúria. A biópsia renal revelou crescentes fibrocelulares glomerulares e depósitos mesangiais de IgA compatíveis com a nefropatia por IgA. O tratamento com corticosteróides sistêmicos levou ao controle da hematúria, mas como a proteinúria nefrótica persistiu, a ciclofosfamida foi adicionada, levando a uma diminuição gradual da proteinúria. Conclusões Propomos um mecanismo comum subjacente na patogênese tanto da PHS quanto da psoríase, envolvendo uma desregulação da resposta imune mediada por IgA, que poderia predispor a ambas as entidades, bem como a danos renais e nefropatia por IgA nesses pacientes.

Abstract Background Psoriasis is a chronic immune-mediated disorder that primarily affects the skin in both adults and children but can also have systemic involvement, particularly with arthritis and kidney injury. IgA nephropathy is the most frequent kidney disorder associated with psoriasis. Approximately one third of all cases of psoriasis begin in childhood, but association between psoriasis and renal disorders has scarcely been reported in pediatric patients. Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by IgA deposits in the vessel walls of affected organs and in the mesangium of the kidney. HSP nephritis histopathology is identical to IgA nephropathy. Case report A 6-year-old boy with recent onset of psoriasis developed HSP with kidney involvement, clinically manifested by nephrotic-range proteinuria and hematuria. Kidney biopsy revealed fibrocellular glomerular crescents and mesangial IgA deposits compatible with IgA nephropathy. Treatment with systemic corticosteroids led to the control of hematuria, but as nephrotic-range proteinuria persisted, cyclophosphamide was added, leading to a gradual decrease in proteinuria. Conclusions We propose an underlying common mechanism in the pathogenesis of both HSP and psoriasis, involving a dysregulation of the IgA-mediated immune response, which could predispose to both entities as well as to kidney damage and IgA nephropathy in these patients.

Resumo A cultura digital com as suas interfaces limitadas leva-nos a olhar fixamente, durante horas, para um ecrã; sentados, imóveis, batemos num teclado ou clicamos repetidamente num rato. O trauma físico resultante deste constrangimento é alimentado por ciclos tecnológicos pontuados pela invenção, desenvolvimento e obsolescência de novos gadgets. Estas são algumas das preocupações de que parte o projecto de Isabel Valverde Lugares Sentidos/Senses Places (2010-2020) uma ciberformance participativa em ambiente de realidade mista visando desenvolver a corporealidade, a consciência corporal e a amplificação dos sentidos através da cinestesia que perpassa a convergência entre virtual e real. Performers e público intermedial distribuemse ao longo do planeta e do espaço virtual.

Abstract Digital culture with its limited interfaces makes us look fixedly, for hours, into a screen: sitting, motionless, we beat on akeyboard or click away on a mouse. The physical trauma resulting from this constraint is fed by technological cycles marked by the invention, development and obsolescence of new gadgets. These are some of the concerns of Isabel Valverde in Senses Places (2010-2020) a participatory cyberformance in mixed reality environments that aims to foster corporeality, corporeal consciousness and the amplification of the senses through the kinesthesia that pervades the convergence of virtual and real. Performers and intermedial public are distributed through out the planet and virtual space.

Introduction: Alport syndrome is a glomerular genetic disease progressing to chronic renal failure associated with deafness and ocular changes. Clinical presentation is usually in the first decade of life with microscopic haematuria and/or persistent proteinuria without hypertension or renal dysfunction. Case Report: Male, 4.5 years old, with an acute nephritic syndrome characterized by macroscopic haematuria, oedema, non-oliguric acute renal failure (maximal urea and creatinine of 25mmol/L and 150μmol/L, respectively), anaemia and proteinuria. Blood pressure normal. Normal immunoglobulins and complement fractions; anti-neutrophil cytoplasmic antibody, anti-nuclear antibody and anti-basal membrane antibody negative. History of recurrent episodes of macroscopic haematuria since the age of eighteen months associated with respiratory infections. No family history of renal disease or deafness. Renal biopsy showed proliferative glomerulonephritis with extracapillary crescentic activity, complete fragmentation of glomerular basement membranes and negative immunofluorescence. Pulse methylprednisolone was given followed by oral prednisolone and cyclophosphamide. Renal function recovered, microscopic haematuria persisted. At age 5, there was reappearance of proteinuria, worsening progressively and two years later he started treatment with enalapril. At age 11, a second renal biopsy revealed mesangial proliferative glomerulonephritis, small foci of glomerular sclerosis and few deposits of IgM on immunofluorescence. He started oral corticosteroids with partial response. Nine months later, bilateral sensorineural deafness was detected. At age 16, he maintains normal renal function, microscopic haematuria with manifest proteinuria. A mutation in homozygosity in the COL4A3 gene, compatible with autosomal recessive Alport syndrome, was identified. Conclusions: This case draws attention to an uncommon early course and clinical/pathological findings of a patient later diagnosed with Alport syndrome, with an initial good response to corticosteroids and cyclophosphamide. The case also illustrates the importance of kidney biopsy, including electron microscopy, in the diagnostic, classification and therapy in kidney diseases with unusual clinical course

Introdução: A prednisolona tem sido a terapia de primeira linha para o tratamento do síndrome nefrótico idiopático da infância. As recidivas são comuns e a necessidade de repetidas doses elevadas de prednisolona ou em combinação com outros imunossupressores podem induzir efeitos secundários significativos. O deflazacorte foi utilizado nos últimos anos como uma alternativa, mas não existem muitos estudos sobre a sua eficácia na terapia de manutenção. O objetivo deste estudo foi verificar a eficácia e a segurança da terapia de manutenção com deflazacorte no síndrome nefrótico idiopático da criança. Métodos: Estudo observacional retrospetivo de pacientes com síndrome nefrótico idiopático corticodependente que tinham recebido tratamento prévio com prednisolona e estavam sob deflazacorte por pelo menos um ano. O síndrome nefrótico idiopático corticodependente foi definido quando ocorreram recidivas durante a redução da terapêutica com corticosteróides ou até duas semanas após a sua retirada. Comparou-se o número de recaídas, a dose de esteróides na recidiva, o período de tempo sem recidivas e efeitos secundários nos doze meses de tratamento, antes e após a introdução de deflazacorte. Resultados: Foram incluídos 20 pacientes, 75% do sexo masculino. No momento do diagnóstico do síndrome nefrótico idiopático a idade média era de 3.4 anos. Todas as crianças tinham começado o tratamento com prednisolona, que é substituído para o deflazacorte em mediana 2.7 anos após o diagnóstico. Após o primeiro ano de terapia com deflazacorte, encontrámos uma redução significativa do número de recaídas (média de 0.5 vs. 2.0; p < 0.001) e dos efeitos secundários dos corticóides (p = 0.037). Não existiram diferenças significativas em outras variáveis. Conclusões: Nesta amostra, o deflazacorte foi associado com um menor número de efeitos secundários e recaídas em comparação com a prednisolona, provando ser uma terapia segura e eficaz no tratamento de manutenção do síndrome nefrótico idiopático em crianças

Background: Prednisolone has been the first-line therapy in the treatment of idiopathic nephrotic syndrome in children. Recurrences are common, and the need for repeated high doses of prednisolone alone or in combination with other immunosuppressants may induce significant side effects. Deflazacort has been used in recent years as an alternative, but there are not many studies on its effectiveness in maintenance therapy. The aim of this study was to assess the efficacy and safety of deflazacort maintenance therapy in childhood idiopathic nephrotic syndrome. Methods: Retrospective observational review of patients with steroid-dependent idiopathic nephrotic syndrome who had received previous treatment with prednisolone and were under deflazacort for at least a year. Steroid-dependent idiopathic nephrotic syndrome was defined when relapses occurred during the reduction of corticosteroid therapy or up to two weeks after its withdrawal. We compared the number of relapses, steroid dose at relapse, the period of time without recurrence and side effects in the twelve months of therapy before and after the introduction of deflazacort. Results: We included 20 patients, 75% were male. At diagnosis of idiopathic nephrotic syndrome, the median age was 3.4 years. All children had begun treatment with prednisolone, which was replaced by deflazacort on average 2.7 years after diagnosis. After the first year of therapy with deflazacort, we found a significant reduction in the number of relapses (mean 0.5 vs. 2.0; p < 0.001) and the side effects of corticosteroids (p = 0.037). There were no significant differences in other variables. Conclusions: In this sample, deflazacort was associated with a small number of side effects and relapses, as compared to prednisolone, proving to be a safe and effective therapy in the maintenance treatment of idiopathic nephrotic syndrome in children

O complexo catiônico ciclopentadienílico de níquel(II) contendo o ligante bis(mesitilimino) acenafteno, de fórmula [Ni(η5-C5H5)(Mes-BIAN)]PF6 (Mes = mesitil = 2,4,6-Me3-C6H2; Mes‑BIAN = bis(mesitil-imino)acenafteno), foi sintetizado através de duas estratégias alternativas, tendo sido obtido com rendimentos moderado a quantitativo. Apesar de ser electronicamente saturado, este novo complexo foi testado na polimerização de etileno e propileno na presença do cocatalisador metilaluminoxano (MAO), mostrando ser um precursor catalítico eficiente da polimerização de etileno em condições suaves de reação (baixa temperatura e pressão), enquanto que se revelou inativo em relação ao propileno. Os polietilenos obtidos apresentam ramificações metílicas e um baixo teor em ramificações longas.

A cationic cyclopentadienyl nickel(II) complex of bis(mesityl-imino)acenaphthene, [Ni(η5-C5H5)(Mes-BIAN)]PF6 (Mes = mesityl = 2,4,6-Me3-C6H2; Mes-BIAN = bis(mesitylimino) acenaphthene), was synthesized via two alternative routes, being obtained in moderate to quantitative yields. Although electronically saturated, this new complex was tested in the polymerization of ethylene and propylene in the presence of the cocatalyst methylaluminoxane (MAO), and found to behave as an efficient catalyst precursor for the polymerization of ethylene under mild reaction conditions (low temperature and pressure), whereas it was inactive towards propylene. The polyethylenes produced show methyl branching and a low content of long branches.

O presente artigo trata de uma pesquisa que teve como objetivo compreender os sentidos atribuídos por adolescentes que cumpriam medida socioeducativa de liberdade assistida às trajetórias de vida e ao envolvimento com infrações à lei. Foram realizados sete sociodramas semanais com 21 adolescentes da comunidade, dos quais 18 eram do sexo masculino. As informações foram organizadas com base no modelo construtivo-interpretativo de González-Rey e analisadas à luz do aporte teórico da Psicossociologia. Da análise resultaram três zonas de sentido: 1) "Parecia uma vida fácil" - referente à sedução do mundo do crime; 2) "Minhas tábuas de salvação" - sobre as estratégias usadas ante os infortúnios da incursão infracional; 3) "A vida no crime não compensa" - concernente ao saldo das escolhas: dores, perdas e arrependimento. As discussões dos encontros desencadearam nos adolescentes relatos acerca do passado, presente e futuro e reflexões que conduzem à reconstrução de projetos de vida desvinculados de atos de infração. Concluiu-se que os métodos socionômico e de história de vida favoreceram a produção de sentidos pelos adolescentes a partir de sua proposta de ação e reflexão e forneceram informações relevantes para se compreender a trajetória infracional dos adolescentes, constituindo-se em poderosa ferramenta de pesquisa-intervenção junto a adolescentes em cumprimento de medidas socioeducativas.

This research aims to understand the meanings attributed by adolescents who are serving socio-educative measure of freedom assisted to their life trajectories and infractional acts. Seven weekly sociodramas were conducted with 21 adolescents from the community, 18 males. Information was organized according to constructive-interpretive method created by González-Rey and the analysis was based on the psychosociology theory. The results showed three zones of meaning: 1) it seemed to be an easy life, related to the seductions of the crime’s world; 2) my tables of salvation, related to the strategies used to get out of the offensive incursion; 3) the life of crime does not pay, related to the balance of choices: pain, lost and regret. The discussions produced during the meetings triggered reflections about the past, present, and future, encouraging the adolescents to reconstruct their project of life free from infractions. We concluded that life history and socionomic methods enable the production of senses by the adolescents, based on their action and reflection proposal and provided relevant information to understand the trajectory of offense of adolescents and it is a powerful tool in intervention research with teenagers in conflict with the law.

Esta es una investigación que tuvo como objetivo comprender los significados atribuidos por adolescentes, que cumplían medida socioeducativa de libertad asistida, a sus trayectorias de vida y su envolvimiento con la infracción. Se realizaron siete sociodramas semanales con 21 adolescentes que vivían en la comunidad, siendo 18 del sexo masculino. Las informaciones fueron organizadas con base en el modelo constructivo-interpretativo de González-Rey y analizadas a la luz de la aportación teórica de la psicosociología. Del análisis, resultaron tres zonas de significación: 1) parecía una vida fácil - acerca de la seducción del mundo del crimen; 2) mis tablas de salvación - sobre las estrategias usadas delante de las desventuras de la incursión en la infracción; 3) la vida del crimen no vale la pena - relativo al saldo de las elecciones: dolores, pérdida y arrepentimiento. Los debates de las reuniones desencadenaron en los adolescentes relatos sobre el pasado, presente y futuro; y reflexiones conducentes a la reconstrucción de proyectos de vida desvinculados a las infracciones. Se concluyó que los métodos, sociodramático y de historia de vida, ayudaron para la producción de significados para los adolescentes, a partir de su propuesta de acción y reflexión; y proporcionaron informaciones importantes para la comprensión sobre la trayectoria de infracción de los adolescentes, constituyéndose en una poderosa herramienta de investigación-intervención junto a los adolescentes en medidas socioeducativas.

Introdução: A maioria das alterações renais que afectam as crianças (excluindo as infecções urinárias simples) são crónicas, e a generalidade destas crianças necessita de cuidados nefrológicos específicos durante a vida adulta, com inegáveis implicações físicas, psicológicas e sociais daí decorrentes. Objectivos: O objectivo primário deste estudo foi avaliar a evolução clínica dos pacientes com síndroma nefrótica, transferidos de uma unidade pediátrica terciária, para continuação de cuidados no Serviço de Nefrologia do Centro Hospitalar e Universitário de Coimbra. Material e Métodos: A colheita de dados foi retrospectiva e baseada em registos hospitalares. É fornecida a caracterização geral dos 109 pacientes transferidos, seguido de uma análise dos 28 pacientes com diagnóstico de síndrome nefrótica. Os resultados clínicos avaliados incluíram a necessidade de terapêutica imunossupressora de manutenção, recorrência da doença, progressão para doença renal crónica e/ou terapêutica de substituição da função renal, anemia, fósforo e metabolismo do cálcio, albumina sérica e proteinúria. Resultados: Os dois grupos mais prevalentes de doenças glomerulares que se apresentaram com síndroma nefrótica foram a doença de lesões mínimas e a glomeruloesclerose focal e segmentar. Metade dos pacientes com síndroma nefrótica estão actualmente sem terapêutica imunossupressora e recorrência da doença de base ocorreu em apenas três dos 22 pacientes ainda em seguimento no nosso Serviço durante o último ano de seguimento (tempo médio de seguimento 82,7 ± 59,7 meses). Aqueles que desenvolveram doença renal crónica com necessidade de terapêutica de substituição da função renal apresentavam glomeruloesclerose focal e segmentar na biópsia renal inicial. Conclusões: Os nossos resultados corroboram os encontrados em outros estudos publicados e os que mantêm seguimento continuam clinicamente estáveis, com apenas três recidivas durante o último ano de seguimento. As especificidades das patologias renais diagnosticadas na infância e os seus aspectos particulares merecem atenção especial e beneficiariam de uma consulta de transição que envolvesse o pediatra do paciente e um nefrologista, bem como a manutenção de registos nacionais dedicados à doença renal pediátrica.

Background: Most renal disorders affecting children (apart from simple urinary infections) are chronic and the majority of these children require additional and specific nephrological care throughout adulthood, with undeniable physical, psychological and social implications arising therefrom. Aim: The primary aim of the current study was to examine the clinical outcome of patients suffering from nephrotic syndrome, transferred for continued care from a tertiary paediatric facility to the adult-oriented Nephrology Department at the Centro Hospitalar e Universitario de Coimbra. Subjects and Methods: Data collection was registry-based and retrospectively collected. General characterization of the 109 patients transferred is supplied, followed by an analysis of the 28 patients with a diagnosis of nephrotic syndrome. Clinical outcomes evaluated included need for ongoing immunosuppressive therapy, disease recurrence, progression to chronic kidney disease and/or renal replacement therapy, anaemia, phosphorus and calcium metabolism, serum albumin and proteinuria. Results: The two most prevalent groups of glomerular diseases presenting with nephritic syndrome were minimal change disease and focal and segmental glomerulosclerosis. Half of the patients with nephrotic syndrome are currently off chronic immunosuppressive therapy and disease recurrence occurred in only three of the 22 patients still followed at our Nephrology Department during the final year of follow-up (mean follow-up of 82.7 ± 59.7 months). Those who developed chronic kidney disease with need for renal replacement therapy had focal and segmental glomerulosclerosis on the initial kidney biopsy. Conclusions: Our results corroborate those found in other published studies and patients still followed remain clinically stable, with only three suffering disease relapse during the final year of follow-up. The specificities of kidney disorders diagnosed in childhood and their individual aspects deserve special care and benefit from a transition appointment involving the patient´s paediatrician and a nephrologist, as well as from the upkeep of national prospective registries dedicated to paediatric kidney disease.

Introduction. Rickets is a paediatric disease which should be suspected in children presenting with failure to thrive, motor developmental delay and orthopaedic abnormalities. Although rare, hereditary hypophosphataemic rickets is the most common form of heritable rickets. Patients and Methods. Retrospective observational study of all children with hypophosphataemic rickets observed at a paediatric nephrology unit of a tertiary paediatric hospital from 1982 to 2012, identified from the unit’s database. Data collected included demographics, risk factors, pre-existing medical conditions, clinical, radiographic and laboratory findings, treatment and morbidity. Results. Eleven children with hypophosphataemic rickets were studied, with a median age at admission of 4.25 years (0.66-10.92). Family history of rickets or orthopaedic abnormalities was found in five children. The first clinical manifestations were delayed/abnormal gait (7/11) and short stature (4/11). Skeletal deformities were present in all children: genus valgum or varum (11/11), thickening of the wrists (7/11), rachitic rosary (4/11), frontal skull bossing (2/11), Harrison’s groove (1/11). Dental abscess was reported in one child and joint pain in six. Laboratory findings included increased alkaline phosphatase (11/11), low serum phosphorus (11/11), normal serum calcium (10/11) and parathyroid hormone values (6/11) and low renal phosphorus reabsorption rate (9/10). None of the children had hypercalciuria. All children were treated with oral phosphorus and calcitriol (8/11 with lack of compliance). Seven children were discharged with a median age of 16.5 years; all had bone deformities, 5/7 had short stature and 1/7 had nephrocalcinosis. Discussion. Hypophosphataemic rickets is a rare disease with significant long-term morbidity. It should be suspected in children presenting with short stature, developmental delay and orthopaedic abnormalities. Increased alkaline phosphatase, low serum phosphorus with normal serum calcium and impaired renal tubular reabsorption of phosphate confirm the diagnosis. Early diagnosis and treatment are essential to minimise morbidity in children

Introduction. Multicysticdysplastic kidney is the most common form of renal dysplasia. The natural history of multicystic dysplastic kidney is involution of the affected kidney, which justifies conservative management. The aim of this study was to evaluate the clinical course of a group of children with multicystic dysplastic kidney and compare these results with a previous study conducted 1989 -2000 in the same clinic. Patients and Methods. Retrospective analysis of the medical records of all children with multicystic dysplastic kidney referred to the paediatric nephrology unit of a tertiary paediatric hospital 2000-2010. Results. Fifty-two children (54% female) with multicystic dysplastic kidney were studied. The mean age at the time of the first visit was nineteen months with a mean follow-up time of sixty-five months. Prenatal ultrasound showed renal abnormalities in 96% of the children, 80% of which were suggestive of multicystic dysplastic kidney. All children underwent renal ultrasound and renal scintigraphy, 48 (92%) had a voiding cystourethrography and two (4%) intravenous urography. Ten (19%) children had contralateral kidney anomalies without obstruction of urinary tract, with the most frequent being pelvicalyceal dilatation (5) and vesicoureteral reflux (5). Nephrectomy was performed in five children (10%). Indications for nephrectomy were increased multicystic dysplastic kidney size in two children, ureterocele in two and bladder diverticulum in one. Of the 47 children (90%) with conservative treatment, 21 (45%) had total kidney involution (over a mean follow-up of three years). Ten (19%) children had complications, eight (15%) of them had urinary tract infection, and two proteinuria. Conclusions. As in the 1989-2000 study, prenatal ultrasound was the main form of multicystic dysplastic kidney diagnosis and contralateral kidney anomalies remain frequent (19% vs.=29%). There has been a significant reduction in the number of intravenous urographies (4% vs. 45%). Conservative treatment has been the first choice in the last few years, accompanied by a reduction in nephrectomies (10% vs. 37%). The lack of clinical problems and good evolution of children with multicystic dysplastic kidney, justifies conservative management. As these children have only one functioning kidney, an adequate monitoring with renal ultrasound and assessment of blood pressure and renal function is essential.

Membranous nephropathy in childhood is usually secondary to an underlying cause. Idiopathic membranous nephropathy is a rare cause of paediatric asymptomatic proteinuria/nephrotic syndrome. We reviewed the only three cases of membranous nephropathy followed in a paediatric hospital over the last ten years. All patients were female with mean age at diagnosis of 11.3 years (10-13 years). Patients 1 and 2 presented with steroid-resistant nephrotic syndrome. Patient 3 had a history of episodes of gross haematuria, and subnephrotic-range proteinuria refractory to enalapril and losartan. All patients had normal renal function. Patient 2 had hypertension and microscopic haematuria. Patients 1 and 2 underwent renal biopsy at an average of 51.5 days after initial symptoms; in patient 3, renal biopsy was performed seven years after initial presentation with episodes of gross haematuria, and five years after discovery of proteinuria. Histopathological features indicated membranous nephropathy; in patients 2 and 3, some findings suggested the coexistence of a systemic clinical condition. Secondary causes were sought in all patients. Six months after diagnosis, patient 1 developed facial skin lesions suggestive of discoid lupus erythematosus and later had positive autoantibodies (ANA, ENA, anti-SSA). Corticosteroids and angiotensin II receptor antagonists or angiotensin converting enzyme inhibitors were given to all patients. Those with nephritic syndrome required the addition of ciclosporin to achieve remission. At the latest evaluation (mean follow-up 31.3 months), patients 1 and 3 were in remission, and patient 2 had sub-nephrotic range proteinuria as the result of poor adherence to medication. All had normal renal function and blood pressure. All were receiving treatment with prednisolone and enalapril, and patients 1 and 2 were also receiving ciclosporin. Diagnosing idiopathic membranous nephropathy in children can be challenging. At least one of our patients had atypical features suggestive of an underlying cause so may have had secondary membranous nephropathy. Follow-up must continue, even after remission, with continued monitoring for underlying systemic disease. Treatment options are angiotensin converting enzyme inhibitors or angiotensin II receptor antagonists, corticosteroids and immunosuppressive drugs such as ciclosporin.

Concentrações de mercúrio e chumbo obtidas pela análise ICP-OAS de amostras de cabelo humano de comunidades ribeirinhas ao longo do rio Orinoco no estado de Amazonas (Venezuela) foram comparadas com outras de Caracas, Venezuela. Levando em consideração as características desses dois ambientes e os valores das concentrações médias de mercúrio e chumbo, foram estabelecidas linhas basais que sugerem que as atividades de minério de ouro próximo ao rio Orinoco são responsáveis pelos altos conteúdos de mercúrio em cabelo no estado de Amazonas. Entretanto, a indústria automotriz é responsável pelo alto conteúdo de chumbo em cabelo em Caracas.

Mercury and Lead concentrations obtained by ICP-OAS analysis of human hair from riverside communities along the Orinoco river in the Amazon state (Venezuela) were compared with those from Caracas, Venezuela. Taking into account the characteristics of these two environments and the values of the average concentrations of Mercury and Lead, baselines were established suggesting that gold mining activity near the Orinoco river is responsible for the high levels of Mercury in hair from the Amazon state, whereas automobile activity is responsible for high levels of Lead in hair in Caracas.