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Drusen are retinal deposits comprising cell debris, immune material and complement that are characteristic of macular degeneration but also found in glomerulonephritis. This was a pilot cross-sectional study to determine how often drusen occurred in IgA glomerulonephritis and their clinical significance. Study participants underwent non-mydriatic retinal photography, and their deidentified retinal images were examined for drusen by two trained graders, who compared central drusen counts, counts ≥ 10 and drusen size with those of matched controls. The cohort comprised 122 individuals with IgA glomerulonephritis including 89 males (73%), 49 individuals (40%) of East Asian or Southern European ancestry, with an overall median age of 54 years (34–64), and median disease duration of 9 years (4–17). Thirty-nine (33%) had an eGFR < 60 ml/min/1.73 m 2 and 72 had previously reached kidney failure (61%). Overall mean drusen counts were higher in IgA glomerulonephritis (9 ± 27) than controls (2 ± 7, p  < 0.001). Central counts ≥ 10 were also more common (OR = 3.31 (1.42–7.73, p  = 0.006), and were associated with longer disease duration ( p  = 0.03) but not kidney failure ( p  = 0.31). Larger drusen were associated with more mesangial IgA staining ( p  = 0.004). Increased drusen counts were also present in IgA glomerulonephritis secondary to Crohn’s disease but not with Henoch-Schonlein purpura. The finding of retinal drusen in IgA glomerulonephritis is consistent with complement activation and represents a model for better understanding glomerular immune deposition and a supporting argument for treatment with anti-complement therapies.

ObjectivesPostpartum hypertension is one of the leading causes of re-presentation to hospital postpartum and is associated with adverse long-term cardiovascular risk. Postpartum blood pressure monitoring and management interventions have been shown to reduce hospital re-presentation, complications and long-term blood pressure control. Identifying patients at risk can be difficult as 40%–50% present with de novo postpartum hypertension. We aim to develop a risk model for postpartum re-presentation with hypertension using data readily available at the point of discharge.DesignA case–control study comparing all patients who re-presented to hospital with hypertension within 28 days post partum to a random sample of all deliveries who did not re-present with hypertension. Multivariable analysis identified risk factors and bootstrapping selected variables for inclusion in the model. The area under the receiver operator characteristic curve or C-statistic was used to test the model’s discriminative ability.SettingA retrospective review of all deliveries at a tertiary metropolitan hospital in Melbourne, Australia from 1 January 2016 to 30 December 2020.ResultsThere were 17 746 deliveries, 72 hypertension re-presentations of which 51.4% presented with de novo postpartum hypertension. 15 variables were considered for the multivariable model. We estimated a maximum of seven factors could be included to avoid overfitting. Bootstrapping selected six factors including pre-eclampsia, gestational hypertension, peak systolic blood pressure in the delivery admission, aspirin prescription and elective caesarean delivery with a C-statistic of 0.90 in a training cohort.ConclusionThe development phase of this risk model builds on the three previously published models and uses factors readily available at the point of delivery admission discharge. Once tested in a validation cohort, this model could be used to identify at risk women for interventions to help prevent hypertension re-presentation and the short-term and long-term complications of postpartum hypertension.

BackgroundOne in seven people with IgA nephropathy has another apparently-affected family member. This study examined how often biopsy-proven familial and sporadic IgA nephropathy were associated with genetic kidney disease.MethodsEleven unrelated people with biopsy-proven IgA nephropathy and another family member with kidney tests compatible with IgA nephropathy were recruited. All available family members were assessed for genetic kidney disease, using Whole Exome Sequencing (WES). Their results were compared with those of 39 people with sporadic IgA nephropathy. All sequencing results were filtered for pathogenic variants in genes associated with genetic kidney disease (Genomics England panels, n = 384). Variants were assessed for pathogenicity using ClinVar and the ACMG/AMP criteria in Alamut.ResultsNine of the 11 probands (82%) with familial nephropathy and 30 of those with sporadic disease (77%) had kidney failure. At least five (45%) and possibly nine (82%) of the 11 families studied had disease-associated heterozygous variants consistent with a coexistent genetic kidney disease (autosomal dominant (AD) and X-linked (XL) Alport syndrome, ADTKD-HNF1B, and possibly Dent disease, Focal and Segmental Glomerulosclerosis (FSGS), and CHARGE syndrome). Inheritance for all these diseases was AD or X-linked. Sometimes the proband with IgA nephropathy did not have the genetic variant found in other apparently-affected family members. Sometimes two genetic variants corresponding to two different diseases were present in the same family. Two of the 39 people with sporadic IgA nephropathy (5%) also had disease-causing variants consistent with genetic kidney disease (AD Alport syndrome, cystinuria).ConclusionMany familial cases of IgA nephropathy result from mesangial IgA deposition in the setting of coexisting genetic kidney disease. Sometimes, genetic kidney disease is not detected in the proband but is present in another family member. Individuals from families with IgA nephropathy should be offered genetic testing.

Background: The effects of acute kidney injury (AKI) extend beyond the acute illness phase. Patients who survive AKI are at increased risk of hospital readmission, chronic disease including kidney and cardiovascular disease, frailty and death. AKI may be overlooked among more obvious or complex healthcare concerns. While developing a cogent, systemic response to care after AKI is a neglected public health priority, attention to several common challenges may improve patient outcomes. Objective: The aim of this article is to highlight common challenges in managing survivors of AKI and offer suggestions to guide management. Discussion: For clinicians managing survivors of AKI, identifying and communicating patient priorities, risk factors and comorbidities including a history of AKI is important. Concurrent management challenges include education regarding lifestyle and pharmacotherapy, managing medication interruptions and dose adjustments, and re-establishing a long-term management plan for chronic diseases.

Very severe intrahepatic cholestasis of pregnancy (ICP) characterised by total serum bile acid (TSBA) >100 umol/L is associated with an increased stillbirth risk of 3.4%. Patients exposed to thiopurines may be at increased risk of ICP. We report a case of a mid-30-year-old primigravida who presented at 27+2/40 with a 7-week history of pruritus and jaundice and was diagnosed with very severe ICP (TSBA=117 µmol/L). She had inflammatory bowel disease, controlled on azathioprine 200 mg daily. Thiopurine metabolites ratio, 6-methylmercaptopurine: 6-thioguanine nucleotide (6MMP:TGN) was 34 at 12/40 and 21 at 27/40. Cessation of azathioprine precipitated a TSBA fall to 31 µmol/L within 7 days. A multidisciplinary plan was made in conjunction with the patient for delivery between 35 and 36 weeks. This case highlights the risks of thiopurine use in pregnancy and supports ceasing azathioprine treatment immediately at the onset of ICP, avoiding TSBA levels rising.

Nephrotic syndrome is characterised by heavy proteinuria secondary to glomerular injury. It is an uncommon but serious complication of allogeneic haematopoietic stem cell transplant (HSCT), but rarely reported after autologous HSCT. Here, we report the case of a man in his mid-20s who presented with significant peripheral oedema 2 months after autologous HSCT for Hodgkin lymphoma. Investigations demonstrated nephrotic range proteinuria and hypoalbuminaemia. Renal biopsy demonstrated minimal change disease. Initial treatment with glucocorticoids was complicated by toxicity without remission. However, the clinical and biochemical resolution of his nephrotic syndrome promptly followed administration of rituximab. This case highlights nephrotic syndrome as a complication after autologous HSCT and suggests potential effectiveness of rituximab in minimal change disease in the presence of steroid toxicity or other contraindications.

Post-transplant diabetes mellitus occurs in 30–50% of cases during the first year post-renal transplantation. It is associated with increased morbidity, mortality and healthcare costs. Risk factors include age and specific immunosuppression regimens. At the same time, renal transplantation is increasingly indicated in elderly (aged >65 years) patients as this proportion of older patients in the prevalent dialysis population has increased. The immune system and β cells undergo senescence and this impacts on the risk for developing post-transplant diabetes and our ability to prevent such development. It may, however, be possible to identify patients at risk of developing post-transplant diabetes, enabling treatment protocols that prevent or reduce the impact of post-transplant diabetes. Much work remains to be completed in this area and is facilitated by the growing base of knowledge regarding the pathophysiology of post-transplant diabetes. Should post-transplant diabetes develop, there are a range of treatment options available. There is increasing interest in using newer agents, although their safety and efficacy in transplant recipients remains to be conclusively established.

To the Editor: The review article on calciphylaxis by Nigwekar et al. (May 3 issue) 1 was highly informative. However, more emphasis on the thrombotic nature of calciphylaxis and opportunities for treatment is needed. Calcification is a central feature of calciphylaxis, yet it remains unclear whether calcification is responsible for the pathogenesis of this condition or whether it is related to another insult. Thrombosis of pannicular vessels is evident on histologic examination. 2 Recent studies suggest that targeting thrombosis may be integral to treatment. 3,4 In a recently published article on a cohort of 101 patients with confirmed calciphylaxis, approximately 80% of those . . .