Immunosuppressive burden and risk factors of infection in primary childhood nephrotic syndrome.

INTRODUCTION: Patients with primary childhood nephrotic syndrome (PCNS) develop alterations in their cellular and humoral immunity that predisposes them to the development of infection, and lead them to have frequent relapses. Also, infection could be significantly enhanced by immunosuppressive agents. This study aims to estimate the immunosuppressive burden, rate of infection and identify possible risk factors in PCNS requiring hospitalization. METHODOLOGY: A cross-sectional study of hospitalized children≤14years of age diagnosed with PCNS in King Abdul-Aziz Medical City, Riyadh from January 2003 to December 2013. RESULT: Out of 111 patients admitted with PCNS, 84 (76%) had both minor and major types of infection. Upper respiratory tract infection (URTI) was the most predominant type (n=44, 52%). Among the major types of infection, urinary tract infection (UTI) was the most common infection (n=21, 25%) followed by pneumonia (n=17, 20%) then cellulitis (n=6, 6%). Infection in children who received a higher annual cumulative dose of steroids (CDS) strikingly had a higher rate of infection in comparison to those who received lower CDS (p<0.01). Moreover, those who received primary and secondary immunosuppressant's had 100% infection rate. CONCLUSION: About half of infection encountered by PCNS patients were URTI followed by UTI and pneumonia. Higher annual CDS, combination of primary and secondary immunosuppressants were the highest independent risk factors for infection. Among the infection, URTI was considered as the predominant entity whereas among the major infection, UTI was predominant followed by pneumonia then cellulitis.

Impact of an inline extracorporeal membrane oxygenation hemofilter system in neonatal acute kidney injury.

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.

Rare genetic variant in the CFB gene presenting as atypical hemolytic uremic syndrome and immune complex diffuse membranoproliferative glomerulonephritis, with crescents, successfully treated with eculizumab.

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.

Recurrent white thrombi formation in hemodialysis tubing: a case report.

BACKGROUND: While the appearance of red clots in the dialyzer is a common phenomenon in every hemodialysis unit, the occurrence of white thrombi in the tubing is relatively rare. CASE PRESENTATION: We describe an adolescent male with recurrent white thrombi formation in the hemodialysis tubing. This patient had chronic renal failure from focal segmental glomerulosclerosis, but was no longer nephrotic at the time of the thrombi formation. He had a history of recurrent thrombosis of his vascular access. However, no pro-thrombotic risk factors could be identified. White particulate matter, measuring 1 to 3mm in size, and adherent to the arterial and venous blood tubing lines was found during the rinse back of a hemodialysis session. This was associated with a 60% decrease in his platelet count. Light microscopic examination of the deposits revealed the presence of platelet aggregates. He subsequently developed thrombosis of his arteriovenous graft six hours later. The white thrombi recurred at the next dialysis session, as well as six months later. These episodes occurred regardless of the type of dialysis machine or tubing, and appeared to resolve with an increase in heparin dose. CONCLUSION: Recurrent white thrombi formation can occur in the hemodialysis tubing of a patient with no identifiable pro-thrombotic factors. The white thrombi may be a harbinger of arteriovenous graft thrombosis and may be prevented by an increase in heparin dose.