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Background Complement factor B gene (CFB) is an important component of the alternate pathway of complement activation that provides an active subunit that associates with C3b to form the C3 convertase, which is an essential element in complement activation. Among the complement-associated disorders, mutations and pathogenic variants in the CFB gene are relatively rare phenomena. Moreover, mutated CFB affiliation with immune-complex diffuse membranoproliferative glomerulonephritis (IC-MPGN) and atypical hemolytic uremic syndrome (aHUS) are considered a highly rare occurrence. Case presentation We describe the clinical presentation, course, and pathological findings in a 7-year-old boy who has confirmed CFB heterozygous variants with pathological features compatible with IC-MPGN. Mutational analysis revealed a heterozygous variant p.Glu566Arg in exon 13 of the CFB gene. The patient did not respond to steroids and mycophenolate mofetil (MMF) therapy but responded clinically and biochemically to eculizumab treatment. This is the first case report of CFB alteration associated with IC-MPGN and aHUS that was successfully treated with eculizumab. Conclusions Heterozygous variants in the CFB gene can be pathogenic and associated with IC-MPGN and aHUS. Early diagnosis and prompt management can be essential in preventing end-stage renal disease. Eculizumab may provide an effective modality of treatment., Author(s): Khalid Alfakeeh [sup.1] [sup.2] , Mohammed Azar [sup.1] , Majid Alfadhel [sup.3] , Alsuayri Mansour Abdullah [sup.1] , Nourah Aloudah [sup.4] , Khaled O. Alsaad [sup.4] Author Affiliations: (1) [...]

Extracorporeal membrane oxygenation (ECMO) is considered a recognized lifesaving support for patients with cardiorespiratory failure. Acute kidney injury (AKI) and fluid overload are significant morbidity factors resulting in serious complications. The inline hemofilter system (IHS) and the continuous renal replacement therapy (CRRT) machine are different methods of renal replacement therapy for patients with ECMO. IHS is the alternate, safe dialysis modality of choice because it is user-friendly, inexpensive, and efficiently removes fluid overload and renal diffusive clearance. We report on a 20-day-old male neonate with multiple congenital cardiac defects who needed venoarterial ECMO and had AKI necessitating renal replacement therapy using IHS. The patient had stable electrolyte parameters, good ultrafiltration, and efficient diffusive clearance. He was decannulated from ECMO therapy after 9 days without any related complications. Therefore, neonatal IHS is a safe and efficient alternative approach to AKI. [ABSTRACT FROM AUTHOR]