Chronic kidney disease (CKD) is commonly associated with normal anion gap metabolic acidosis and its correction has shown multiple benefits, such as improved bone health and nutritional status, a slower progression of CKD and a lower incidence of end-stage renal disease. Kidney Diseases Improving Global Outcomes 2013 recommend maintaining serum bicarbonate concentration in the normal range (≥ 22mEq/L), which is usually accomplished by using a starting dose of 0.5 - 1mEq/kg/day of bicarbonate supplements. Nutritional strategies incorporating base-producing fruits and vegetables might show equal value. In this context, we analyzed the ingredients instead of labels of widely sold bottled water in Portugal to identify the most bicarbonate-rich bottled water which might help mitigate the complications of CKD chronic metabolic acidosis. We included 32 varieties of bottled water sold in Portuguese supermarkets. If a 70kg-adult is advised to ingest a bicarbonate dose of 0.5 - 1 mEq/kg/day, this amount could be supplied by 1 - 2 liters of Frize® per day, approximately the same as in Pedras Salgadas® and Vidago® (1.1 - 2.2 liters). We concluded that, along with nutritional strategies addressing dietary H+ reduction, Portuguese CKD patients with chronic metabolic acidosis could benefit from drinking water brands such as Frize®, Pedras Salgadas® or Vidago®.

Crescentic IgA nephropathy represents an aggressive glomerular injury which can cause renal failure unless diagnosed and treated rapidly. There is currently no consensus on the most effective treatment strategy, although immunosuppressive drugs are usually used. Tuberculosis is rarely associated with glomerulonephritis and raises difficult questions about the safety and usefulness of immunosuppressive agents. We report a rare case of rapidly progressive renal failure associated with pleural tuberculosis in a 68-year-old man. The identification of crescents and fibrinoid necrosis in 12 out of 21 glomeruli and IgA deposits in the renal biopsy suggested the diagnosis of crescentic IgA nephropathy. Corticosteroids in combination with antituberculosis drugs were started with significant improvement in his general condition and cure of his pleural tuberculosis but without recovery of kidney function.

A 74-year-old male with a recent diagnosis of chronic myelomonocytic leukaemia (CMML) was admitted for rapidly progressive renal failure (RPRF), associated with gait impairment due to muscle weakness and pain in the lower limbs. After exclusion of pre-renal and post-renal causes of RPRF, the workup revealed monocytosis, high levels of inflammatory markers, hypergammaglobulinaemia, nephrotic proteinuria and high serum and urinary lysozyme levels. Renal biopsy confirmed the diagnosis of lysozyme-induced kidney injury and CMML-associated vasculitis. An electromyogram also revealed sensorimotor axonal polyneuropathy. The patient was started on prednisolone and azacitidine. Improvement of limb symptoms and a decrease in monocyte count, renal function values, inflammatory markers and proteinuria were subsequently seen. Although lysozyme levels are consistently elevated in CMML, lysozyme-induced kidney injury is a rare cause of renal failure. Filtered lysozyme appears to act as a direct tubular toxin and lysozymuria has been proposed as a valuable tool for detection of tubular damage. Polyneuropathy secondary to CMML is also infrequent and may be due to autoimmune mechanisms. We describe a case of lysozyme-induced kidney injury, vasculitis and axonal polyneuropathy, presumably secondary to CMML, in which prednisolone and azacitidine seem to have been helpful in treating extramedullary leukaemic involvement

Introdução: O transplante renal em adultos com alterações congénitas do trato urinário inferior (ACTUI) apresenta complicações específicas. O estudo urológico de bexigas com baixa capacidade e pouco complacentes pode determinar a necessidade de cirurgia vesical reconstrutiva e soluções derivativas. Além disso, esta população apresenta maior risco de infeções do trato urinário (ITUs). Objetivo: Caraterizar os doentes com ACTUI seguidos na Unidade de Transplante Renal do Centro Hospitalar de São João e avaliar complicações pós -transplante renal. Os doentes com refluxo vesicoureteral (RVU) primário foram excluídos da análise. Materiais e Métodos: Foram avaliados 8 doentes com ACTUI com idades entre 13 e 39 anos. A doença renal crónica estabeleceu -se por RVU secundário e ITUs de repetição em contexto de mielomeningocelo (3 casos), válvulas da uretra posterior (1 caso), bexiga neurogénica com ânus imperfurado (1 caso), megaureter congénito (2 casos) e bexiga neurogénica idiopática com dissenergia vesicoesfincteriana (1 caso). Resultados: Todos os doentes foram submetidos a intervenções cirúrgicas antes do transplante: nefrectomia dos rins nativos (2 casos), ablação das válvulas da uretra posterior (1 caso), cistoplastia de aumento (6 casos) e ureteroileostomia (1 caso). Sete doentes mantiveram-se continentes, 6 praticando auto-algaliação intermitente para esvaziamento vesical e 1 usando a manobra de Valsalva. Verificou-se uma taxa de pielonefrites agudas do enxerto renal com necessidade de internamento de 31 episódios por 100 doentes -ano, bastante superior à descrita para transplantados renais por qualquer causa, apesar da sobrevida do enxerto não ter sido afetada. Após um seguimento médio de 104 ± 11.8 meses, 5 doentes mantêm enxertos funcionantes com creatinina sérica média de 1.17 ± 0.40 mg/dl. Conclusão: O transplante renal em doentes com ACTUI apresenta bons resultados após cirurgia prévia para correção das alterações urológicas, apesar da frequência elevada de pielonefrites do enxerto

Introduction: Renal transplantation in adults with congenital abnormalities of the lower urinary tract (CALUT) carries particular complications. Careful urologic evaluation of abnormal bladders, due to neuropathy or outlet obstruction, may indicate the need for surgical correction before transplantation. This population is also at increased risk for urinary tract infections. Purpose: We assessed the outcome of renal transplantation in patients with CALUT at our Transplant Unit. Patients with primary vesicoureteral reflux (VUR) were excluded from our analysis. Materials and Methods: A total of eight patients with CALUT, 13 to 39 years old, were evaluated. End -stage renal disease was caused by secondary VUR and recurrent urinary tract infections in patients with myelomeningocele (three cases), posterior urethral valves (one case), neurogenic bladder associated with imperforate anus (one case), congenital megaureter (two cases) and idiopathic neuropathic bladder and sphincter dyssynergia (one case). Results: All patients underwent surgical interventions before transplantation: ureteronephrectomy of the native kidneys (two cases), ablation of posterior urethral valves (one case), augmentation cystoplasty (six cases) and construction of an ileal conduit (one case). Seven patients remained continent, six were able to empty the bladder by clean intermittent selfcatheterization and the remaining by using the Valsalva maneuver. A rate of 31 episodes per 100 patient-years of graft pyelonephritis was found, much higher when compared to other transplant recipients, although graft survival was not affected. After a mean follow -up of 104 ± 11.8 months, five patients have kept functioning grafts with mean serum creatinine of 1.17 ± 0.40 mg/dl. Conclusion: Renal transplant in patients with CALUT has good outcomes after preparatory urologic surgical corrections although graft pyelonephritis remains a frequent complication

O presente estudo exploratório examinou o desenvolvimento mental e a qualidade do funcionamento sócio-emocional de 16 crianças entre os 3 e os 6 anos, institucionalizadas em Centros de Acolhimento Temporário, relacionando-os com a qualidade das narrativas sobre o apego das suas cuidadoras. Foram utilizadas a Escala de Desenvolvimento Mental de Griffiths, o Questionário de Comportamentos, as Narrativas sobre o Apego e o Attachment Q-Sort. Os resultados revelaram que o nível de desenvolvimento das crianças foi inferior aos valores normativos. Além disso, os valores apresentados ao nível da psicopatologia, em termos de sintomas de internalização e de externalização, aproximaram-se dos valores clínicos. Não obstante e, em contraste com o esperado, a maioria das crianças apresentou valores próximos da segurança (base segura), os quais não estão associados com a qualidade das narrativas sobre o apego das suas cuidadoras. Os resultados são discutidos com base no impacto da privação em meio institucional para o desenvolvimento na infância.

This exploratory study examined mental development and the quality of socio-emotional functioning of 16 institutionalized children, aged from 3 to 6 years old, relating them with the quality of their caregivers' attachment narratives. Griffiths Developmental Scales, Child Behavior Checklist, Attachment Script Representation and Attachment Q-Sort were used in the psychological assessment. Results showed that chidren's developmental level was placed below normative data. Furthermore, data regarding externalizing and internalizing psychopathology symptoms were close to the clinical population. Nevertheless, and in contrast to our hypothesis, most children presented almost mormative attachments scores (secure base), but this was not related to the quality of attachement narratives presented by their caregivers. The implications of these findings are discussed in light of the impact of institutional deprivation in child development.