Case Reports in Nephrology and Dialysis最新文献

The Seraph^® 100 Microbind^® Affinity Blood Filter (Seraph^® 100) is a hemoperfusion device designed to adsorb bacteria, viruses, and toxins when added to extracorporeal circuits. The FDA granted emergency use authorization in adults, but this device had never been utilized in children. A 17-year-old patient with asthma presented with respiratory distress due to COVID-19. His course was complicated by respiratory failure, rhabdomyolysis, and stage 3 AKI requiring initiation of continuous kidney replacement therapy (CKRT) on ICU day 3. The Seraph^® 100 filter was added on ICU day 4. He was treated with 3 filters from ICU day 4 to 8. On ICU day 8, he was extubated and CKRT discontinued. He required no further kidney replacement therapy but did not have laboratory work post-discharge. In conclusion, this adolescent patient with COVID-19 and AKI requiring CKRT tolerated treatment with the Seraph^® 100 Microbind^® Affinity Blood Filter without significant adverse events.

MYH9-associated disorders represent rare group of autosomal dominant diseases and are caused by pathogenic mutations in the MYH9 gene. Clinically, they are represented by macro-platelet-thrombocytopenia, various degrees of renal dysfunction, hearing loss, and early onset cataracts. We describe the case of 14-year-old boy in medical follow-up from birth for thrombocytopenia. Systolic hypertension and nephrotic proteinuria were detected at preventive health check. Renal biopsy revealed sing of segmental glomerulosclerosis. Dialysis treatment was needed. Before transplantation due to the finding of chronic tonsillitis with positive bacterial capture in the culture examination, tonsillectomy was indicated. Postoperative period was complicated with arterial post-tonsillectomy hemorrhage. Six months after tonsillectomy, the patient underwent primary deceased-donor kidney transplantation without complication. Blood platelets showed fluctuating character in the zone of severe thrombocytopenia. However, no signs of bleeding were present. Three months after successful transplantation gene sequencing of whole exon was performed. The presence of the variant c.2105G>A [p.(Arg702HIS)] in exon 17 of the MYH9 gene has been detected. The variant c.2105G>A may be clinically manifested by progressive proteinuria with rapid deterioration of renal function. This case is an example of the delayed diagnosis of rare disease and highlights the usefulness of genetic testing.

Low-density lipoprotein (LDL) apheresis is effective for nephrotic syndrome in drug-resistant focal segmental glomerulosclerosis (FSGS). Dextran sulfate adsorption of LDL (DSAL) is widely used for this purpose. The Liposorber LA-15 system performs DSAL by membrane plasma separation (mDSAL) using an MA-03 plasma purification device. However, sufficient blood flow (Qb) frequently cannot be obtained from a peripheral vein with mDSAL. The recommended plasma filtration flow rate (Qf) when using the OP-05W membrane plasma separator is no more than 1/3 of Qb, giving plasma removal efficiency (PRE) of about 30%. In contrast, the centrifugal blood component separator Spectra Optia has PRE of 87-92.5% because centrifugal separation enables effective separation of plasma components even at low Qb. Here, we present the case of a man in his 40s with FSGS, for whom we began treatment with mDSAL with the intention of completing a 12-session cycle, but extended treatment times were required due to low Qb. Therefore, we switched to DSAL by centrifugation (cDSAL) using the Liposorber LA-40 system from the 6th session onward. Treatment time decreased from 190 min for the fifth session using mDSAL to 140 min for the sixth session using cDSAL. Mean treatment time also decreased from 155 ± 9 min for mDSAL (5 sessions) to 119 ± 20 min for cDSAL (7 sessions). Moreover, the LDL removal rate at a processed plasma volume was similar for both modalities. In conclusion, cDSAL can enable efficient plasma separation even with low Qb, with a comparable LDL removal rate and shorter treatment time relative to mDSAL.

There have been reports of rare de novo glomerular diseases following vaccination for coronavirus disease 2019 (COVID-19). We report two cases of anti-glomerular basement membrane (GBM) disease in previously healthy females after Oxford-AstraZeneca COVID-19 vaccine (ChAdOx1 nCoV-19). The first case was a 69-year-old female who developed lethargy and anuria approximately 8 weeks after her first dose of Oxford-AstraZeneca COVID-19 vaccine. The second case was a 72-year-old female who developed malaise and diarrhoea approximately 3 weeks after her second dose of Oxford-AstraZeneca COVID-19 vaccine. Both cases had severe acute kidney injury, raised anti-GBM antibody titres, and renal biopsies consistent with anti-GBM disease. Both cases were commenced on haemodialysis and treated with high dose glucocorticoids, cyclophosphamide, and plasmapheresis. Neither patient had recovery of renal function, and both remain dialysis dependent. These cases add to the previously reported cases of anti-GBM disease after mRNA COVID-19 vaccination. As more COVID-19 vaccinations are administered worldwide, it would be important for clinicians to be aware of this possible association, and continued surveillance is warranted.

A 32-year-old male patient presented the clinical picture of loin pain haematuria syndrome with pain attacks accompanied by macrohaematuria. In renal biopsy, the preglomerular vessels showed segmental wall hyalinosis in the sense of low-grade nephrosclerosis, and glomerular capillaries with slightly but diffusely thickened, non-split basal membranes on electron microscopy. Notable were irregularly deformed, different dense erythrocytes in the glomerular capillaries, and several tubular lumina. The suspicion of erythrocytic enzyme deficiency could be confirmed. The enzyme activities of the erythrocytes were predominantly normal or slightly increased; only the activity of triosephosphate isomerase, a critical key enzyme of glycolysis, was reduced to 71% (resp. 57%) of the normal level, compatible with a heterozygous carrier status that could not be found. Patients with genomic triosephosphate-isomerase deficiency have degraded enzyme activities in virtually all tissues, such as leucocytes, platelets, and muscle cells. An association with neuromuscular symptoms is also known. Thus, it is possible that smooth muscle and intrarenal vascular spasms trigger clinical symptoms consisting of flank pain and phases of macrohaematuria. An aspirin-like defect (thrombocytopathy) had previously been found in connection with epistaxis (also due to TPI deficiency?). Enalapril treatment drastically reduced the frequency of macrohaematuria and pain attacks decreased to a lesser extent.

Uncaria tomentosa is a plant that has been used in traditional medicine for its anti-inflammatory, immunomodulatory, and immunostimulant properties. As a result, it can be found in several over-the-counter supplements worldwide. Acute interstitial nephritis (AIN) can be due to an offending medication, infection, or autoimmunity. We present a case of a patient who was on a strict ketogenic diet, utilizing over-the-counter diet shakes containing the herbal supplement Uncaria tomentosa who developed acute kidney injury with a serum creatinine of 3.6 mg/dL up from a baseline of 0.7 mg/dL. Serological evaluation was negative, and kidney biopsy revealed interstitial inflammatory infiltrates including focally prominent eosinophils and multifocal tubulitis. Stopping the keto-diet shake containing Uncaria tomentosa and concomitant corticosteroid therapy resulted in improvement in kidney function to near baseline. To our knowledge, this is the only biopsy-proven case of AIN in the setting of Uncaria tomentosa use.

Anuric hemodialyzed end-stage renal disease patients are prone to multiple complications and comorbidities and are therefore often treated with various medications. Adverse drug reactions and risk factors leading to them can be difficult to discern in such polymedicated patients. Most problems regarding low phosphate levels are frequently underdiagnosed in clinical practice and sometimes overlooked in these regularly hyperphosphatemic patients. Hemodialysis vascular accesses are frequently subject to infections and therefore require adapted antibiotic treatments. We report a case of an occult severe multifactorial hypophosphatemia in an anuric hemodialyzed patient with multiple comorbidities who required two hospitalizations for encephalopathy, seizures, and cardiac failure. Retrospective analysis of the medical record revealed several underlying causes of hypophosphatemia, as well as undetected risk factors for adverse drug reactions related to cephalosporins. A global approach to these concerns in routine clinical practice would raise awareness of often disregarded issues related to hypophosphatemia and drug prescription in these patients.

There has been a parallel rise in the need for bariatric surgery as the prevalence of obesity has increased by leaps and bounds over the last 2 decades. Certain procedures like Roux-en-Y gastric bypass are associated with nephrolithiasis, hyperoxaluria, and, rarely, oxalate nephropathy. We report an interesting case of a patient who had relentless progression of his kidney disease post-bariatric surgery.