A Multicenter Study Evaluating the Discontinuation of Eculizumab Therapy in Children with Atypical Hemolytic Uremic Syndrome.

BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening thrombotic microangiopathy (TMA), which has been treated successfully with eculizumab. The optimal duration of eculizumab in treating patients with aHUS remains poorly defined. METHODS: We conducted a multicenter retrospective study in the Arabian Gulf region for children of less than 18 years of age who were diagnosed with aHUS and who discontinued eculizumab between June 2013 and June 2021 to assess the rate and risk factors of aHUS recurrence. RESULTS: We analyzed 28 patients with a clinical diagnosis of aHUS who had discontinued eculizumab. The most common reason for the discontinuation of eculizumab was renal and hematological remission (71.4%), followed by negative genetic testing (28.6%). During a median follow-up period of 24 months after discontinuation, 8 patients (28.5%) experienced HUS relapse. The risk factors of recurrence were positive genetic mutations (p = 0.020). On the other hand, there was no significant relationship between the relapse and age of presentation, the need for acute dialysis, the duration of eculizumab therapy before discontinuation, or the timing of eculizumab after the presentation. Regarding the renal outcomes after discontinuation, 23 patients were in remission with normal renal function, while 4 patients had chronic kidney disease (CKD) (three of them had pre-existing chronic kidney disease (CKD) before discontinuation, and one case developed a new CKD after discontinuation) and one patient underwent transplantation. CONCLUSIONS: The discontinuation of eculizumab in patients with aHUS is not without risk; it can result in HUS recurrence. Eculizumab discontinuation can be performed with close monitoring of the patients. It is essential to assess risk the factors for relapse before eculizumab discontinuation, in particular in children with a positive complement variant and any degree of residual CKD, as HUS relapse may lead to additional loss of kidney function. Resuming eculizumab promptly after relapse is effective in most patients.

Practice of general pediatrics in Saudi Arabia: current status, challenges, and opportunities.

BACKGROUND: In Saudi Arabia, general pediatrics serves children until they are 14 years old. It has contributed to improving the health of Saudi children. METHOD: This study adopted a qualitative method and recruited pediatric physicians to investigate status, successes, challenges, and opportunities. Later, data were analyzed using thematic analysis and hermeneutic phenomenology. RESULTS: This study attracted 13 pediatric physicians for interviews. All participants appreciated the role of general pediatricians, but the trainees had a negative attitude regarding the general pediatrics specialty. They all agreed on providing primary care for all children and recommended that their first visit should occur earlier. Shortage of pediatricians, lack of community pediatricians, busy clinics, limited Arabic resources, and poor communication skills are significant barriers to children receiving adequate care. The majority of pediatricians favor extending the pediatric age to 18 years old. One pediatrician stated, "Youths between ages 14-18 years are lost, adults and we refuse to care for them…" Additionally, pediatricians have concerns about managing developmental delays and behavioral issues. They believe the current pediatric residency provides many opportunities for a brighter future. CONCLUSION: General pediatrics is well established in Saudi Arabia. To continue thriving, we need to address some challenges that pediatricians face and attract more residency graduates. The current pediatric residency programs can provide opportunities to address deficit areas.

Spectrum of pediatricbiopsy-proven renal diseases: A single center experience.

Glomerular diseases are considered to be a significant cause of chronic kidney disease. Kidney biopsy continues to be an essential diagnostic tool. We review the renal biopsies which were done on children below the age of 14 years in the past 10 years (from January 2008 to September 2018) in a single tertiary pediatric hospital in Saudi Arabia to determine the patterns of renal disease among Saudi children as well-correlating clinical presentation with histopathological diagnosis. A total of 203 pediatric kidney biopsies were performed. The mean age was 7.3 ± 3.9 years (3 months to 14 years). There were 105 males and 98 females. The most frequent indication for renal biopsy was nephrotic syndrome in 58.9% of patients, followed by acute glomerulo- nephritis in 20.8%. Other indications included significant proteinuria, persistent microscopic hematuria, acute kidney injury of uncertain etiology, in the remaining 20% of biopsies. Clinical diagnosis was consistent with histopathological diagnosis in 92% of the cases. Minimal change disease was the most common cause of primary glomerular diseases in 37.4%, followed by focal segmental glomerulosclerosis in 20.2%. Lupus nephritis represents the most common cause of the secondary renal disease (8.4%). Complications of kidney biopsy were observed in only 16.3% of patients, of whom 9.9% had perirenal hematomas and 6.4% of the patients developed either microscopic hematuria or macroscopic hematuria.

Valganciclovir prophylaxis delays onset of EBV viremia in high-risk pediatric solid organ transplant recipients.

BACKGROUND: The role of antiviral prophylaxis to prevent Epstein-Barr virus (EBV) viremia or posttransplant lymphoproliferative disorder in pediatric solid organ transplant recipients is controversial. We examined whether valganciclovir (VAL) prophylaxis for cytomegalovirus infection was associated with EBV viremia following transplantation in EBV-naive children. METHODS: A single-center, retrospective study was conducted of EBV-naive pediatric heart and renal transplant recipients with an EBV-positive donor from January 1996 to April 2017. VAL was tested for association with EBV viremia-free survival in the first 6 months posttransplantation when immunosuppressant exposure is the highest. Survival models evaluated VAL duration, with adjustment for other baseline confounders. RESULTS: Among the cohort (n = 44), 3 (6.8%) were heart transplants, 25 (56.8%) received VAL, and 22 (50%) developed EBV viremia in the first-year posttransplantation. Mean time-to-viremia was 143 vs. 90 days for the VAL and no-VAL groups, respectively (p = 0.008), in the first 6 months. Only two patients developed viremia while on VAL. Each additional day of VAL was associated with 1.4% increase in viremia-free survival (p < 0.001). Multivariable modeling of VAL with other baseline risk factors did not identify other independent risk factors. CONCLUSION: VAL is independently associated with delayed onset of EBV viremia, with prolongation of delay with each additional day of antiviral prophylaxis.