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Pediatr Infect Dis J ; 35(12): 1360-1362, 2016 12.
Artigo em Inglês | MEDLINE | ID: mdl-27636723


Pseudomonas aeruginosa and Candida albicans (are opportunistic pathogens that cause systemic infections in immune-suppressed patients. They show important bacterial-fungal interactions including quorum sensing. This involves cell signaling to communicate between the cells of their own colony and the cells of rival microbes or the host. It is thought that this phenomenon is vital in the potential competition and virulence of the organisms. We report a case of a previously healthy 2-year-old boy, where an accidental injury had been sustained resulting in a closed fracture of femur. He subsequently developed sepsis related to co-infection by C. albicans and P. aeruginosa. Trauma may result in a transient immune-suppression and predispose to sepsis caused by opportunistic microorganisms. They can engage in bacterial-fungal interaction. Clinicians should consider invasive co-infection when initial cultures show evidence for only 1 pathogen.

Candidemia , Coinfecção/microbiologia , Fraturas do Fêmur/complicações , Infecções por Pseudomonas , Sepse/microbiologia , Candida albicans , Pré-Escolar , Humanos , Masculino , Interações Microbianas , Infecções Oportunistas , Pseudomonas aeruginosa , Percepção de Quorum
J Am Soc Nephrol ; 20(5): 1123-31, 2009 May.
Artigo em Inglês | MEDLINE | ID: mdl-19389850


Mutations in hepatocyte nuclear factor 1B (HNF1B), which is a transcription factor expressed in tissues including renal epithelia, associate with abnormal renal development. While studying renal phenotypes of children with HNF1B mutations, we identified a teenager who presented with tetany and hypomagnesemia. We retrospectively reviewed radiographic and laboratory data for all patients from a single center who had been screened for an HNF1B mutation. We found heterozygous mutations in 21 (23%) of 91 cases of renal malformation. All mutation carriers had abnormal fetal renal ultrasonography. Plasma magnesium levels were available for 66 patients with chronic kidney disease (stages 1 to 3). Striking, 44% (eight of 18) of mutation carriers had hypomagnesemia (<1.58 mg/dl) compared with 2% (one of 48) of those without mutations (P < 0.0001). The median plasma magnesium was significantly lower among mutation carriers than those without mutations (1.68 versus 2.02 mg/dl; P < 0.0001). Because hypermagnesuria and hypocalciuria accompanied the hypomagnesemia, we analyzed genes associated with hypermagnesuria and detected highly conserved HNF1 recognition sites in FXYD2, a gene that can cause autosomal dominant hypomagnesemia and hypocalciuria when mutated. Using a luciferase reporter assay, we demonstrated HNF1B-mediated transactivation of FXYD2. These results extend the phenotype of HNF1B mutations to include hypomagnesemia. HNF1B regulates transcription of FXYD2, which participates in the tubular handling of Mg(2+), thus describing a role for HNF1B not only in nephrogenesis but also in the maintenance of tubular function.

Fator 1-beta Nuclear de Hepatócito/genética , Rim/anormalidades , Deficiência de Magnésio/genética , Mutação , Síndrome de Emaciação/genética , Adolescente , Família , Feminino , Triagem de Portadores Genéticos , Taxa de Filtração Glomerular , Humanos , Rim/anatomia & histologia , Rim/diagnóstico por imagem , Magnésio/sangue , Magnésio/urina , Masculino , Estudos Retrospectivos , Ultrassonografia
Pediatr Nephrol ; 24(6): 1231-4, 2009 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-19153773


We report the case of a child who died from severe cerebral oedema in the context of hyponatraemia and extreme polyuria immediately after renal transplantation. The patient was treated according to a standard post-transplantation protocol, receiving 0.45% saline solution for urine output replacement. The case highlights the dangers of massive fluid therapy in the context of polyuria and, therefore, the need for intensive monitoring.

Edema Encefálico/cirurgia , Hiponatremia/cirurgia , Transplante de Rim , Poliúria/cirurgia , Criança , Evolução Fatal , Hidratação , Humanos , Masculino , Poliúria/tratamento farmacológico
Pediatr Nephrol ; 22(9): 1349-54, 2007 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-17111159


Growth failure is an important complication for children on dialysis. One possible influence on growth is renal bone disease. We reviewed the case notes of 35 children (23 boys), mean (range) age at inclusion 2.8 (0.25-8.9) years (17 children age <2 years), on dialysis for 2.0 (1-4.8) years, for growth, PTH, calcium and phosphate levels and medications. Data collection ended at age 10 years, commencement of growth hormone (rhGH) or renal transplantation. The mean (range) height standard deviation score (HtSDS) at the start of dialysis was -2.06 (-5.90 to 0.63). No change in HtSDS per year was observed; the median was -0.06 (-1.07 to 2.39). Children aged <2 years showed catch-up growth in the first year on dialysis; median change in HtSDS was 0.31 (-0.78 to 3.13). Mean plasma calcium and ionised calcium were approximately at the mid-point and phosphate just above the mid-point of the respective normal ranges. The median PTH level was 1.52 times the upper limit of normal and levels did not correlate with growth. Our results indicate that intensive nutritional therapy and phosphorus control aiming to keep PTH within the normal range prevents further loss of HtSDS in short children on dialysis. In some children under 2 years of age catch-up growth can be observed in the first dialysis year.

Crescimento , Hormônio Paratireóideo/sangue , Diálise Renal , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Fatores de Tempo
Nephrol Dial Transplant ; 19(11): 2769-77, 2004 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-15385635


BACKGROUND: Prognostic factors and outcome are incompletely known in childhood mesangiocapillary glomerulonephritis (MCGN). This study aimed to correlate renal outcome with clinical and histopathological variables. METHODS: We conducted a two-centre retrospective analysis of children with MCGN. RESULTS: Fifty-three children presented at a mean age of 8.8 years (range: 13 months-15 years). They were followed for a median of 3.5 years (range: 0-17 years). Histological classification identified 31 type 1, 14 type 2, two type 3 and six undetermined type. Mean renal survival [time to end-stage renal failure (ESRF)] was projected to be 12.2 years [confidence interval (CI): 9.7-14.6 years]. Five and 10 year renal survival was 92% (CI: 88-100%) and 83% (CI: 74-92%), respectively. Those with nephrotic syndrome at presentation had mean renal survival of 8.9 years (CI: 7.1-10.7 years) vs 13.6 years for those without (CI: 10.8-16.5 years) (P = 0.047). The mean estimated glomerular filtration rate (eGFR) at 1 year in those who progressed to ESRF was 52 vs 98 ml/min/1.73 m2 in those who did not (P < 0.001). Chronic damage scored on the first biopsy in 31 children (one centre) was positively associated with adverse renal outcome at 5 years: <20% was associated with 100% and > or =20% with 71% 5-year renal survival (P = 0.006). In 29 children treated with steroid there was a higher proportion (76%) with reduced eGFR at presentation and a significantly higher incidence of nephrotic syndrome (P = 0.002) and hypertension (P = 0.037). There were no significant differences in outcome eGFR, hypertension or proteinuria. CONCLUSIONS: Nephrotic syndrome at presentation and subnormal eGFR at 1 year were adverse features. The finding that structural disease at onset predicted poor renal outcome at 5 years has implications for the design of therapeutic trials. Treatment of MCGN was variable and not evidence-based.

Glomerulonefrite Membranoproliferativa/mortalidade , Adolescente , Criança , Pré-Escolar , Taxa de Filtração Glomerular , Glomerulonefrite Membranoproliferativa/patologia , Humanos , Lactente , Glomérulos Renais/patologia , Prognóstico , Proteinúria/mortalidade , Estudos Retrospectivos , Análise de Sobrevida , Resultado do Tratamento
Pediatr Nephrol ; 18(7): 710-1, 2003 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-12750983


Gingival hypertrophy is a well-documented side effect of cyclosporin therapy, but severe lip enlargement is less frequently recognised. This can lead to poor body image, low self-esteem and non-compliance, especially in the older childhood and adolescent age groups. We describe two paediatric renal transplant recipients who had marked lip hypertrophy as a consequence of cyclosporin (Neoral) treatment. On changing the immunosuppression to tacrolimus (Prograf), this resolved. We recommend that a change in immunosuppressant therapy be considered in children with significant changes to facial appearance.

Ciclosporina/efeitos adversos , Imunossupressores/efeitos adversos , Doenças Labiais/induzido quimicamente , Criança , Cistinose/complicações , Cistinose/cirurgia , Humanos , Hipertrofia/induzido quimicamente , Hipertrofia/patologia , Nefropatias/complicações , Nefropatias/cirurgia , Transplante de Rim/imunologia , Doenças Labiais/patologia , Masculino