Acute kidney injury (AKI) is a clinical syndrome that manifests as an abrupt impairment of kidney function. AKI is common in critically ill pediatric patients admitted to the pediatric intensive care units. AKI is a deleterious complication in critically ill children as it is associated with increased morbidity and mortality. This review provides an overview of the incidence, morbidity, and mortality of AKI in critically ill children in general and specific cohorts such as post-cardiac surgeries, sepsis, critically ill neonates, and post stem cell transplantation.

Cystinosis is a rare systemic disease characterized by the accumulation of cystine in tissues, leading to multi-organ damage. Infantile nephropathic cystinosis is the dominant and severe form of cystinosis with critical renal manifestations that require kidney transplantation at an early age if left untreated. Cysteamine, the lifelong cystine-depleting therapy, is the mainstay treatment of nephropathic cystinosis. Cysteamine prevents cystine crystal formation and delays disease progression. While the initially introduced cysteamine consists of an immediate-release (IR) formulation, a delayed-release (DR) formulation has been developed with a simplified dosing regimen (Q12H instead of Q6H) and an improved quality of life while maintaining comparable efficacy. Due to the rare incidence of the disease and lack of international guidelines, diagnosis and treatment initiation are oftentimes delayed, leading to a poor prognosis. Pediatric and adult nephrologists from Kuwait, Saudi Arabia, the United Arab Emirates (UAE), and Qatar, in addition to one international expert from Amsterdam, convened to share their clinical experience, reflecting on the challenges encountered and therapeutic approaches followed in the management of nephropathic cystinosis in the Gulf Cooperation Council (GCC) region. Experts completed a multiple-choice questionnaire and engaged in structured discussions, where they shed light on gaps and limitations with regard to diagnostic tests and criteria to ensure early diagnosis and timely treatment initiation. Based on available literature, experts suggested an algorithm to help guide nephropathic cystinosis management in the GCC. It is highly recommended for patients who do not tolerate IR-cysteamine and do not adhere to IR-cysteamine treatment to switch to DR-cysteamine. Given the systemic nature of the disease, a multi-disciplinary approach is required for optimal disease management.

Marked leukocytosis in critical pertussis is a major predictor of mortality in young infants, with reported mortality rates of 40-85%. Although leukoreduction is frequently performed in critical pertussis with marked leukocytosis using either whole blood exchange transfusion or therapeutic leukapheresis, no evidence-based guidelines exist, and published data remain limited to small case reports. We present a descriptive case series evaluating the feasibility, hematologic response, and short-term outcomes of leukoreduction using these two modalities. We conducted a retrospective case series of five children aged 3-52 weeks with PCR-confirmed pertussis having marked leukocytosis admitted to a tertiary pediatric intensive care unit between February and July 2024. Leukoreduction was performed following multidisciplinary consensus. Absolute and percentage reductions in white blood cell (WBC) counts were calculated using pre- and post-procedure values. Clinical characteristics, illness severity scores (PRISM, PELOD, PIM3), leukoreduction modality, and procedural complications were reviewed. Median WBC count decreased from 58 × 10⁹/L (range, 33-81.6) before the procedure to 18 × 10⁹/L (range, 13-41) after the procedure, representing a median reduction of 64.1% (range, 38.1-75.8%). In this case series, exchange transfusion was associated with a greater median WBC reduction compared with leukapheresis (71.4% vs. 56.8%). However, exchange transfusion resulted in a more pronounced median reduction in platelet count (79.9% vs. 44.7%) and a greater increase in hemoglobin (mean increase, 5.5 g/dL vs. 0.47 g/dL). In this case series, leukoreduction was feasible and produced immediate cytoreduction in critically ill infants with pertussis-associated hyperleukocytosis. Exchange transfusion was associated with greater WBC reduction but more pronounced platelet depletion. Larger multicenter studies are needed to further evaluate these modalities and guide modality selection and timing.