Background: We present a rare case of severe vulvar edema secondary to steroid-refractory nephrotic syndrome in a prepubertal girl.
Case: The patient is an 8-year-old girl who presented during nephrotic syndrome relapse. She exhibited severe mons pubis and labial edema. She was treated with local symptomatic measures such as sitz baths, barrier ointment, and labial sling, with minimal relief. Improvement ultimately occurred after bilateral nephrectomy.
Summary And Conclusion: Vulvar edema is rare in prepubertal girls. In this case, the edema was secondary to steroid-refractory nephrotic syndrome and was not responsive to local treatment measures. There is a paucity of data on effective treatment of vulvar edema in young girls. Our goal is to raise awareness of such pathology, which might lead to development of uniform guidelines for treatment of this condition.
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http://dx.doi.org/10.1016/j.jpag.2019.09.009 | DOI Listing |
Pediatr Nephrol
January 2025
Department of Translational Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea.
Background: Coenzyme Q10 (CoQ10) nephropathy is a well-known cause of hereditary steroid-resistant nephrotic syndrome, primarily impacting podocytes. This study aimed to elucidate variations in individual cell-level gene expression in CoQ10 nephropathy using single-cell transcriptomics.
Methods: We conducted single-cell sequencing of a kidney biopsy specimen from a 5-year-old boy diagnosed with a CoQ10 nephropathy caused by a compound heterozygous COQ2 mutation complicated with immune complex-mediated glomerulonephritis.
Sci Rep
January 2025
Department of Nephrology, The First Affiliated Hospital, Sun Yat-sen University, 58 Zhongshan Road II, Guangzhou, 510080, China.
Nephrotic syndrome (NS) occurs in 5-15% of patients with IgA nephropathy (IgAN), resulting in poorer long-term outcomes compared to those without NS. Clinical features and renal prognosis for patients with both NS and IgAN across different kidney pathologies have not been fully elucidated. This study included patients with primary IgAN through renal biopsy at the First Affiliated Hospital of Sun Yat-sen University from January 2001 to November 2021 presenting with NS.
View Article and Find Full Text PDFCureus
December 2024
Pediatrics Department, Dr. Sulaiman Al Habib Hospital, Riyadh, SAU.
Coenzyme Q2 (CoQ2) mutations are a group of autosomal recessive mitochondria-linked diseases that result in coenzyme Q10 (CoQ10) deficiency (CoQ10: a cofactor in mitochondrial energy production). Its deficiency leads to multiple systemic clinical presentations; however, isolated steroid-resistant nephrotic syndrome (SRNS) is considerably rare. Multiple genetic mutations have been reported with different ranges of severity and prognosis, with variable responses to CoQ10 supplementation.
View Article and Find Full Text PDFCureus
December 2024
Nephrology, Ibn Sina Hospital, Rabat, Rabat, MAR.
Cryoglobulinemic vasculitis is a rare small-vessel vasculitis leading to multi-organ dysfunction, often associated with chronic infections like hepatitis C virus (HCV), and autoimmune disorders. Most cases involve mixed monoclonal or polyclonal immunoglobulins, presenting symptoms such as purpura, arthralgias, and weakness. Severe organ involvement, particularly cardiac, is rare but potentially life-threatening.
View Article and Find Full Text PDFClin Kidney J
January 2025
Department of Nephrology, Dialysis, Transplantation, Dr C. I. Parhon Hospital, Iasi, Romania.
The timeless tale of Snow White, with its emphasis on fair skin as a beauty ideal, mirrors a contemporary issue in nephrology: the harmful impact of skin-whitening creams on kidney health. Fairness creams have deeply embedded themselves in global society, driven by a pervasive obsession with lighter skin tones as a symbol of beauty. This widespread use reflects deeply rooted cultural beliefs and social norms, despite the significant health risks associated with these products.
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