RESUMO
Very low calorie diets (VLCDs) induce rapid weight loss through severe energy restriction resulting in ketosis. VLCD manufacturer guidelines list acute kidney injury (AKI) as a contraindication for use with concerns around further damage to kidney function through increased protein catabolic load, diuresis, and risk of electrolyte derangements. We report on the successful concurrent management of AKI alongside provision of a VLCD for weight loss in the acute setting in a patient with class III obesity and comorbid complications during a prolonged hospital stay. AKI resolved at week 5 of a 15-week VLCD program, with no adverse side effects noted on electrolytes, fluid, or kidney function. A weight loss of 76 kg was attained. VLCD appears safe for use in patients with AKI during hospitalization under close medical supervision. Both health system sustainability and patients may benefit from seizing the opportunity to address obesity during protracted hospital admission.
Assuntos
Injúria Renal Aguda , Dieta Redutora , Humanos , Dieta Redutora/efeitos adversos , Dieta Redutora/métodos , Restrição Calórica , Obesidade/complicações , Redução de Peso , Injúria Renal Aguda/complicações , Injúria Renal Aguda/terapia , Ingestão de EnergiaRESUMO
Pregnancy-associated atypical haemolytic uraemic syndrome (P-aHUS) is a rare, potentially lethal condition that can complicate pregnancy in up to 1 in 25 000 cases. Without prompt diagnosis and initiation of appropriate treatment, this condition can lead to disastrous consequences for both mother and child. Given the broad spectrum of conditions that can present similarly in the peripartum period, it is often difficult to establish the correct diagnosis in a timely manner. Recently, the terminal complement cascade inhibitor eculizumab has been used with considerable success in non-pregnancy HUS; however, its use in P-aHUS is limited to isolated case reports. Here, we present a case of fulminant P-aHUS in the postpartum period that was successfully treated with eculizumab resulting in significant recovery of renal function.
Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Síndrome Hemolítico-Urêmica Atípica/diagnóstico , Síndrome Hemolítico-Urêmica Atípica/tratamento farmacológico , Complicações na Gravidez/diagnóstico , Complicações na Gravidez/tratamento farmacológico , Adulto , Síndrome Hemolítico-Urêmica Atípica/complicações , Feminino , Humanos , Gravidez , Complicações na Gravidez/etiologiaRESUMO
Focal segmental glomerulosclerosis (FSGS) is the consequence of a disease process that attacks the kidney's filtering system, causing serious scarring. More than half of FSGS patients develop chronic kidney failure within 10 years, ultimately requiring dialysis or renal transplantation. There are currently several genes known to cause the hereditary forms of FSGS (ACTN4, TRPC6, CD2AP, INF2, MYO1E and NPHS2). This study involves a large, unique, multigenerational Australian pedigree in which FSGS co-segregates with progressive heart block with apparent X-linked recessive inheritance. Through a classical combined approach of linkage and haplotype analysis, we identified a 21.19 cM interval implicated on the X chromosome. We then used a whole exome sequencing approach to identify two mutated genes, NXF5 and ALG13, which are located within this linkage interval. The two mutations NXF5-R113W and ALG13-T141L segregated perfectly with the disease phenotype in the pedigree and were not found in a large healthy control cohort. Analysis using bioinformatics tools predicted the R113W mutation in the NXF5 gene to be deleterious and cellular studies support a role in the stability and localization of the protein suggesting a causative role of this mutation in these co-morbid disorders. Further studies are now required to determine the functional consequence of these novel mutations to development of FSGS and heart block in this pedigree and to determine whether these mutations have implications for more common forms of these diseases in the general population.
Assuntos
Doenças Genéticas Ligadas ao Cromossomo X/genética , Glomerulosclerose Segmentar e Focal/genética , Bloqueio Cardíaco/genética , Proteínas de Transporte Nucleocitoplasmático/genética , Proteínas de Ligação a RNA/genética , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Animais , Austrália , Criança , Pré-Escolar , Exoma , Feminino , Genes Ligados ao Cromossomo X , Ligação Genética , Células HEK293 , Humanos , Masculino , Camundongos , Pessoa de Meia-Idade , Mutação , N-Acetilglucosaminiltransferases/genética , Especificidade de Órgãos , Linhagem , Análise de Sequência de DNA , Adulto JovemRESUMO
Campylobacter is the leading cause of food poisoning in the UK. 2 Sisters Food Group, with retail partners, monitored the effect that: (1) awarding financial incentives to farmers and stockpersons for producing houses that were not highly contaminated with Campylobacter, or (2) the cessation of thinning (where ~30 per cent of birds are removed and processed at around day 35 of the crop cycle), had on prevalence of Campylobacter on UK broiler farms in a longitudinal monitoring study. Ninety-four farms and 759 houses were monitored from November 2013 to October 2015, with and without interventions. Financial incentives and thinning were significantly associated with Campylobacter prevalence. Houses on farms receiving an incentive had a 54 per cent reduction in odds of being highly contaminated with Campylobacter Houses that were thinned had a 309 per cent increase in odds of being highly contaminated. Temperature and bird age were significantly positively associated with Campylobacter Changes in industry practice at supply chain level can support Campylobacter control plans in commercial broiler flocks. Elucidating farm-level factors associated with Campylobacter prevalence (such as house type, condition, flock size) as well as individual factors related to thinning (stocking density, weight profile and associated economic consequences) require further investigation.
Assuntos
Criação de Animais Domésticos/métodos , Infecções por Campylobacter/veterinária , Campylobacter/isolamento & purificação , Fazendas , Motivação , Doenças das Aves Domésticas/epidemiologia , Doenças das Aves Domésticas/microbiologia , Animais , Infecções por Campylobacter/epidemiologia , Galinhas , Monitoramento Ambiental , Fazendas/economia , Fazendas/estatística & dados numéricos , Contaminação de Alimentos/prevenção & controle , Humanos , Estudos Longitudinais , Prevalência , Reino Unido/epidemiologiaRESUMO
BACKGROUND: We report a previously unrecognized and unreported case of a patient with anti-glomerular basement membrane glomerulonephritis following nintedanib, an orally active small molecule tyrosine kinase inhibitor. CASE PRESENTATION: A 59-year-old Caucasian woman with a history of idiopathic pulmonary fibrosis presented with severe acute kidney injury (creatinine 285 umol/L) secondary to anti-glomerular basement membrane glomerulonephritis disease 4 months after commencement of nintedanib. She had hematuria with red blood cell casts, nephrotic range proteinuria (3.5g/24 hours) and significantly elevated anti-glomerular basement membrane glomerulonephritis titers at 860 chemiluminescent units. A kidney biopsy confirmed severe crescentic glomerulonephritis with linear immunoglobulin G deposition in glomerular basement membrane. Despite the commencement of treatment with plasma exchange and cyclophosphamide, she remained dialysis dependent. Nintedanib was discontinued. CONCLUSIONS: Onset of acute anti-glomerular basement membrane glomerulonephritis was found to be associated with recent nintedanib use suggesting that nintedanib may be a potential trigger for anti-glomerular basement membrane glomerulonephritis. This case highlights the importance of close monitoring of patients receiving new targeted therapies. Management of novel targeted agents in patients receiving dialysis is challenging because of the scarcity of specific data.
Assuntos
Doença Antimembrana Basal Glomerular/induzido quimicamente , Antineoplásicos/efeitos adversos , Glomerulonefrite/induzido quimicamente , Fibrose Pulmonar Idiopática/tratamento farmacológico , Indóis/efeitos adversos , Doença Antimembrana Basal Glomerular/fisiopatologia , Doença Antimembrana Basal Glomerular/terapia , Antineoplásicos/administração & dosagem , Ciclofosfamida/uso terapêutico , Feminino , Glomerulonefrite/fisiopatologia , Hematúria , Humanos , Fibrose Pulmonar Idiopática/fisiopatologia , Indóis/administração & dosagem , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Troca Plasmática , Prednisona/uso terapêutico , Indução de Remissão , Resultado do TratamentoRESUMO
It is not known whether prevention of anemia among patients with chronic kidney disease would affect the development or progression of left ventricular (LV) hypertrophy. A randomized controlled trial was performed with 155 patients with chronic kidney disease (creatinine clearance, 15 to 50 ml/min), with entry hemoglobin concentrations ([Hb]) of 110 to 120 g/L (female patients) or 110 to 130 g/L (male patients). Patients were monitored for 2 yr or until they required dialysis; the patients were randomized to receive epoetin alpha as necessary to maintain [Hb] between 120 and 130 g/L (group A) or between 90 and 100 g/L (group B). [Hb] increased for group A (from 112 +/- 9 to 121 +/- 14 g/L, mean +/- SD) and decreased for group B (from 112 +/- 8 to 108 +/- 13 g/L) (P < 0.001, group A versus group B). On an intent-to-treat analysis, the changes in LV mass index for the groups during the 2-yr period were not significantly different (2.5 +/- 20 g/m(2) for group A versus 4.5 +/- 20 g/m(2) for group B, P = NS). There was no significant difference between the groups in 2-yr mean unadjusted systolic BP (141 +/- 14 versus 138 +/- 13 mmHg) or diastolic BP (80 +/- 6 versus 79 +/- 7 mmHg). The decline in renal function in 2 yr, as assessed with nuclear estimations of GFR, also did not differ significantly between the groups (8 +/- 9 versus 6 +/- 8 ml/min per 1.73 m(2)). In conclusion, maintenance of [Hb] above 120 g/L, compared with 90 to 100 g/L, had similar effects on the LV mass index and did not clearly affect the development or progression of LV hypertrophy. The maintenance of [Hb] above 100 g/L for many patients in group B might have been attributable to the relative preservation of renal function.