Case Reports in Nephrology and Dialysis最新文献

Studies investigating the effect of rituximab in children with nephrotic syndrome (NS) due to focal segmental glomerulosclerosis (FSGS) have reported conflicting results, with some concluding that patients may require additional immunosuppressive therapy to achieve and/or maintain long-term remission. We report successful treatment of pediatric FSGS with rituximab infusions, followed by maintenance immunosuppression with mycophenolic acid (MPA) and a calcineurin inhibitor (CNI) in 1 patient with refractory steroid-resistant NS (SRNS), and one with frequently relapsing NS (FRNS). Case 1 is a patient with refractory SRNS due to FSGS. MPA and tacrolimus induced complete remission within 6 months following rituximab treatment. Remission was maintained for over 2 years, and the patient's kidney function and body height also returned to normal ranges within this time. Case 2 is a patient with FRNS due to FSGS, who was treated with rituximab followed by MPA and cyclosporine, which successfully prevented relapses for 18 months, that is, at the end point of the observation. Our case report demonstrates that rituximab and a combination of CNIs and MPA can be effective in achieving complete remission in pediatric refractory SRNS and sustaining remission in pediatric FSGS with FRNS and SRNS for several years. This treatment regimen has the advantage of eliminating the need for long-term high-dose steroid treatments, allowing 1 patient to achieve normal growth and recover from other adverse steroid effects.

Pruritus is highly prevalent in the dialysis population. Its etiology however remains often unclear with uremic pruritus primarily suspected unless compelling evidence of another cause. Although bullous pemphigoid (BP) is considered idiopathic, there are growing data in the literature on BP provoked by different factors, such as medications or surgical procedures. These secondary dermatoses are described as rather mild conditions and more frequent in the elderly Caucasian. We herein describe a newly dialyzed African man of 76 years old, treated by a sulfonylurea such as an antidiabetic drug, who developed a severe BP after jugular catheter placement.

Vena cava superior (VCS) syndrome is rarely seen as a complication of central-venous-catheter placement. Usually, the syndrome appears when the presence of the catheter causes intraluminal obstruction or thrombosis. In this case report, however, we describe a patient on intermittent hemodialysis who had been free of any venous central line for over 6 years, presented with a VCS syndrome. The CT scan showed an absent VCS without extravascular compression. Previous catheter placement was diagnosed as the case of the VCS syndrome. It is important to realize that VCS syndrome can occur late after removal of central venous catheters, and thus, clinicians should be aware of its symptoms in any patient who has had an upper central line in the past medical history.

Tuberculosis (TB) infection of the genitourinary tract (GU TB) is rare in renal transplant recipients, with only a few published case series. GU TB is difficult to diagnose with or without immunosuppression but must always be suspected in any patient with unexplained sterile pyuria. As GU TB is associated with graft rejection, prompt diagnosis and treatment are vital. Treatment is challenging, as rifampicin, the most effective drug used to treat tuberculosis, is a significant inducer of cytochrome P-450 3A metabolism, with the potential to cause significant reductions in the serum levels of calcineurin inhibitors. For this reason, rifabutin, a weaker cytochrome P-450 3A inducer, with similar efficacy against TB, is sometimes used as an alternative to rifampicin in transplant recipients. We present a renal transplant patient diagnosed with GU TB, treated with a regime containing rifabutin, who subsequently developed profound hyponatremia and leucopenia. Serum and urine biochemistry was consistent with a diagnosis of the syndrome of inappropriate antidiuretic hormone secretion (SIADH). Both SIADH and leucopenia resolved with rifabutin cessation. This is the first report of biochemically proven, idiosyncratic SIADH and leucopenia associated with the use of rifabutin in the treatment of GU TB in a renal transplant recipient.

Copper is an indispensable trace metal element and is mainly absorbed in the stomach and small intestine and excreted into the bile. Hypoxia-inducible factor-prolyl hydroxylase inhibitors (HIF-PHIs) have emerged as a novel approach for renal anemia management. Many intestinal genes, including divalent metal transporter 1, duodenal cytochrome B, and copper transporter ATPase7A, related to iron absorption are transactivated by HlF-α, during iron deficiency. We first report 4 cases of patients with renal anemia who showed excess in serum copper level during roxadustat or daprodustat treatment, which were decreased to the normal level after discontinuing HIF-PHIs and changing the drug to darbepoetin alfa, suggesting that HIF-PHI is associated with serum copper excess. HIF-PHI modulates iron metabolism, such as iron absorption, sequestration, and mobilization, and may increase serum copper levels by increasing copper absorption and/or redistribution of copper in tissues. Therefore, it is urgent to examine the correlation between HIF-PHI use and serum copper levels because copper excess might be involved in several acute or chronic adverse events.

Formaldehyde-free hair-straightening products are hair-smoothening solutions widely used by professional beauty salons. Formaldehyde-free hair straighteners do not technically contain formaldehyde; however, they contain other chemicals such as glyoxyloyl carbocysteine which releases formaldehyde upon contact with heat. Moreover, its by-product glyoxylate may convert to oxalate; both compounds have potential nephrotoxic effect. Here, we report a case of a 41-year-old woman who presented to the emergency room with weakness, nausea, vomiting, and stage 3 acute kidney injury (AKI) according to Kidney Disease: Improving Global Outcomes (KDIGO) acute kidney injury staging shortly after exposure to formaldehyde-free hair-straightening product; other causes of AKI were excluded such as preceding acute illness, drug history, or other nephrotoxic agent exposure. On physical examination, the patient was pale, and her vital signs were normal. The urine microscopy and serologic workup were not indicative. Kidney core biopsy revealed interstitial edema, acute interstitial nephritis, and oxalate crystal nephropathy. Kidney function completely recovered after a short course of steroid therapy. In this case, AKI was a complication caused by exposure to hair-straightening products branded as formaldehyde free but actually containing other chemical products which release formaldehyde and other toxic chemicals when heated during the straightening procedure and may cause systemic toxicity, particularly kidney injury. Different cosmetic products are widely in use, but not all are under tight regulation, and therefore, it is important to raise the awareness among both medical teams and consumers of possible adverse health effects of different cosmetic products.

Propylthiouracil (PTU)-induced antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a rare and heterogeneous disease. Moreover, optimal treatment is still lacking. We described the case of a 44-year-old lady with underlying Graves' disease who had cough, blood-streaked sputum, and impaired renal function. A strongly positive anti-myeloperoxidase antibody (>200 U/mL) along with pauci-immune glomerulonephritis and pulmonary hemorrhage resulted in the diagnosis of PTU-induced AAV, given that the patient had been on PTU for 3 years. PTU withdrawal, therapeutic plasma exchanges, and oral cyclophosphamide provided favorable clinical and biochemical outcomes. She remained well on azathioprine 50 mg daily as maintenance therapy and clinically euthyroid with carbimazole 2.5 mg daily. The effective treatment for drug-induced ANCA vasculitis remains controversial, but rapid withdrawal of the offending medication should be the mainstay of treatment. In severe drug-induced ANCA vasculitis with pulmonary hemorrhage and/or life-threatening organ involvement such as kidney failure requiring dialysis, therapeutic plasma exchange with immunosuppressants is often required. In this case, we have shown that patient achieved remission after therapeutic plasma exchange with cyclophosphamide in the acute stage of treatment and remained symptom-free with azathioprine in the maintenance phase of treatment for 24 months.

Recently, several cases of novel apolipoprotein E (apoE)-related glomerular disease known as membranous nephropathy (MN)-like apoE deposition disease with apoE Toyonaka (Ser197Cys) and homozygous apoE2/2 have been reported. However, the clinical and pathological characteristics are uncertain due to the small number of reports. Here, we report an additional case with various clinical and pathological characteristics. A 28-year-old Japanese man with mild proteinuria and hematuria underwent a kidney biopsy. Examination under a light microscope revealed mesangial proliferation, mesangial matrix expansion, and segmental spike lesion. An immunofluorescence study showed no immunoglobulin or complement depositions. In the electron microscopic (EM) examination, massive deposits with various electron densities in the subepithelial, subendothelial, and paramesangial areas were more prominent than those reported in previous cases, which resembled microbubbles or microcysts on higher magnification. The glomerular basement membrane (GBM) structure was partly degenerated by these deposits. Serum triglyceride and cholesterol levels were within the normal range. However, the serum apoE concentration was significantly high, and glomerular apoE accumulation was detected in immunohistochemistry. The DNA sequence revealed apoE Toyonaka and homozygous apoE2/2 similar to that of the previous cases with MN-like apoE deposition disease. MN-like apoE deposition disease can manifest as only mild hematuria and proteinuria without dyslipidemia. Various characteristic deposits associated with GBM degeneration can be observed in the EM study.

Chronic hemodialysis is associated with reduced fertility. Hence, pregnancy remains rare, challenging, and deleterious when unplanned, especially in low-resource countries. Contraception and births are very important in these settings. Though the main modes of contraception have been proposed in the chronic kidney disease (CKD) population, contraception still remains challenging in patients on maintenance hemodialysis. Most doctors, however, overlook contraception because of the low fertility, high rate of amenorrhea, and low libido. Furthermore, patients are less receptive to contraceptive counseling either because of a high desire to give birth or due to amenorrhea and low libido. Management of unplanned pregnancies is therefore very challenging and a multidisciplinary approach is the rule; however, it does not guarantee a good prognosis for both the mother and child. Very few cases of multiple pregnancies without induction of ovulation have been reported in patients with severe renal failure, especially those on maintenance dialysis. A 32-year-old multiparous woman with end-stage kidney failure (ESKF) and a residual diuresis of 700 mL per day who had been on inadequate maintenance hemodialysis for 36 months, presented with abdominal distension, which was confirmed on abdominal ultrasound to be a twin pregnancy at 22 weeks of gestation. Thereafter, we intensified hemodialysis (3 sessions/week), managed hypertension and anemia. The obstetrical course was uneventful until the 25th week of gestation when she developed grade 3 (WHO) hypertension and peripheral fluid overload. At the 29th week, she had a spontaneous vaginal preterm delivery of 2 babies weighing 1,350 g and 1,000 g, with an Apgar score of 8 and 7, respectively. Babies, however, died on day 1 and day 5 postpartum, respectively, from respiratory distress and early neonatal infection. The evolution of the mother was uneventful as she continued with her hemodialysis sessions. Twin pregnancies are a rare and very high-risk condition in end-stage renal disease and require multidisciplinary management.

Patients who have been kidney transplanted have an increased risk of developing cancer. This case report presents a rarely described case in which a patient, who had received a kidney transplant from a deceased donor, was diagnosed with disseminated urothelial carcinoma originating from the allograft. After the removal of the allograft and the immunosuppressive treatment, there was regression in the cancer. Unfortunately, it was not a complete regression of the urothelial cancer and the patient died. This case indicates that there is a risk of getting cancer from the transplanted kidney from a deceased donor, but also that the immunosuppressive treatment can contribute to the development of this cancer.