We present the case of a pregnant woman in her 20s who presented in her second trimester with severe pulmonary haemorrhage and dialysis-dependent acute kidney failure due to antiglomerular basement membrane (GBM) disease. Responding to therapy, she recovered kidney function and delivered a baby. During her pregnancy, she developed cytomegalovirus viraemia, gestational diabetes and pre-eclampsia. Here, we report the first combined use of cyclophosphamide, rituximab and intensified plasma exchange in anti-GBM disease in pregnancy, allowing minimal exposure to cytotoxic medication, resulting in live birth and dialysis independence.

Bladder stones represent approximately 5% of all cases of urolithiasis and are typically identified and managed long before causing irreversible renal injury. We present a case of a man in his 40s with a prior history of a gunshot wound to the abdomen who presented with leakage from a previously healed suprapubic tube tract and was found to have a giant bladder stone with a resulting renal injury. He subsequently underwent a combined open cystolithotomy and vesicocutaneous fistulotomy during his hospitalisation, which helped to improve his renal function. In addition to there being few reported cases of bladder stones >10 cm, this represents the first report in the literature of an associated decompressive \'pop-off\' mechanism through a fistulised tract.

Catastrophic antiphospholipid syndrome (CAPS) is a life-threatening form of antiphospholipid syndrome characterised by diffuse arterial and venous thrombosis, in the presence of positive antiphospholipid antibodies. The multiple sites of thrombosis in small, medium and large vessels progress to multiorgan failure, accounting for the high mortality rate associated with CAPS. Unregulated complement activation is increasingly recognised as critical to the pathogenesis of CAPS. Early diagnosis is essential to initiate prompt life-saving treatment with the triple therapy of anticoagulation, immunosuppression and either plasmapheresis or intravenous immunoglobulin. Among other immunosuppressive agents, eculizumab, a complement inhibitor has demonstrated efficacy in treatment-resistant cases.We report an instructive case of a woman presenting with both clinical and laboratory findings consistent with primary CAPS, resistant to initial treatment and responsive to eculizumab, with emphasis on genetic testing and implications for future therapy.

Membranous nephropathy has been associated with demyelinating polyneuropathies and antiglomerular membrane disease; however, an association with vasculitic neuropathy has not been described. This case describes a patient with biopsy-proven idiopathic membranous nephropathy and synchronous mononeuritis multiplex secondary to idiopathic small vessel vasculitis, who presented with lower limb microvascular ischaemia, peripheral neuropathy and active urinary sediment. Her extensive non-invasive screening for immunological disease and radiological investigations for occult malignancy were unremarkable. The patient received intravenous methylprednisolone and intravenous rituximab induction therapy resulting in complete remission of both the idiopathic membranous nephropathy and small vessel vasculitis at 7 months post treatment.

Muckle-Wells syndrome (MWS) is a genetic periodic fever syndrome characterised by urticaria, fever and malaise starting in childhood with the development of perceptive hearing loss and risk of amyloidosis later in life.Patient A, in his 60s, was referred to a nephrologist because of glomerular haematuria and elevated erythrocyte sedimentation rate. He appeared to have periodic fevers since childhood, skin changes in cold circumstances and progressive deafness since he was 30 years of age. Genetic analysis revealed a pathogenic variant in the NLRP3 gene compatible with MWS. Treatment with anakinra (interleukin 1 antagonist) improved his symptoms, but only mild episodic arthralgia remained. Glomerular erythrocyturia diminished during treatment, supposing a relation between MWS and haematuria.This case report shows that rare genetic fever syndromes starting from early childhood can still be diagnosed in adult patients, with important therapeutic consequences. Symptoms can be relieved and amyloidosis with potential renal failure may be prevented.

SummaryRhabdomyolysis is characterised by muscle breakdown which causes myoglobin light chain release and can result in renal injury. While some of the most common causes of rhabdomyolysis are trauma related, others include toxins, autoimmune processes or viral aetiologies. We present the case of a 20s-year-old man, with no significant medical history, who presented to the emergency department with a 1-week history of weakness, myalgias, nausea, vomiting and subjective fevers. A review of systems and physical exam were otherwise unremarkable, including being negative for sore throat, dysphagia and lymphadenopathy. On presentation, the patient was noted to have dark urine with a creatine kinase value of 452 458 U/L and an elevated creatinine at 7.23 mg/dL. The patient denied any trauma or increased physical activity. His toxin screen and autoimmune workup were negative. The patient\'s serological workup was significant for acute Epstein-Barr virus (EBV) infection, without additional viral coinfection or mononucleosis. During his hospitalisation course, the patient was managed with supportive care including haemodialysis. The patient made a full renal recovery and was discharged with scheduled outpatient follow-up. This case highlights the recognition of an acute EBV infection causing rhabdomyolysis in the absence of mononucleosis or concomitant infection.

Peritoneal dialysis (PD)-associated peritonitis is linked to increased mortality rates and transfer to haemodialysis or PD discontinuation. Rare and emerging pathogens can pose challenges in management. We present the first case of PD peritonitis caused by Elizabethkingia miricola through direct contamination, which was successfully treated with intraperitoneal and oral antibiotics.

A woman in her mid-60s presented with decreased output from urostomy, which was an opening from the neobladder (ileal conduit). Presentation was preceded by a 6-month history of alternating faecaluria and increased colostomy output. Laboratory studies were notable for normal anion gap metabolic acidosis. Creatinine level of the colostomy output was 17.7 mg/dL, a finding indicative of the presence of urine in the sample. CT enterography and X-ray loopogram confirmed neobladder to small intestine fistula.Neobladder creation is commonly performed in patients with bladder cancer requiring resection. Fistulas between the neobladder and intestine are observed in fewer than 2.7% of cases. The patient\'s history of extensive abdominopelvic resection, colostomy creation and radiation likely contributed to fistula development. We highlight the need for a high index of suspicion for a fistula in a patient with a neobladder experiencing recurrent urinary tract infections or a high colostomy output concurrently with low neobladder output.

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UNASSIGNED: Ultrasound shear wave elastography (SWE) is a novel technique that may provide non-invasive measurements of renal compliance. We aimed to investigate the relationship between intravenous (IV) fluid administration and change in SWE measurements. We hypothesised that following IV fluid administration in healthy volunteers, global kidney stiffness would increase and that this increase in stiffness could be quantified using SWE. Our second hypothesis was that graduated doses of IV fluids would result in a dose-dependent increase in global kidney stiffness measured by SWE.
UNASSIGNED: Randomised prospective study.
UNASSIGNED: Intensive Care Unit.
UNASSIGNED: Healthy volunteers aged 18-40 years.
UNASSIGNED: Participants were randomised to receive 20 ml/kg, 30 ml/kg, or 40 ml/kg of normal saline. The volume of fluid infused was based on the actual body weight recorded.
UNASSIGNED: We recorded average SWE stiffness (kPa with standard deviation of the mean), median SWE stiffness (kPa), and the interquartile range.
UNASSIGNED: Ninety-eight percent of participants (44/45) demonstrated an increase in global kidney stiffness following administration of IV fluids. The average SWE pre fluid administration was 7.572 kPa ± 2.38 versus 14.9 kPa ± 4.81 post fluid administration (p < 0.001). In subgroup analysis, there were significant changes in global kidney stiffness pre and post fluid administration with each volume (ml/kg) of fluid administered. Average percentage change in global kidney stiffness from baseline was compared between the three groups. There was no significant difference when comparing groups 1 and 2 (197.1% increase ± 49.5 vs 216.1% ± 72.0, p ¼ 0.398), groups 2 and 3 (216.1% increase ± 72.0 vs 197.8% ± 59.9, p ¼ 0.455), or groups 1 and 3 (197.1% increase ± 49.5 vs 197.8% ± 59.9, p ¼ 0.972).
UNASSIGNED: Fluid administration results in immediately visible and quantifiable changes in global kidney stiffness across all infused volumes of fluid.