Presentations and outcomes of familial hemophagocytic lymphohistiocytosis in the pediatric intensive care units (PICUs).

Objectives: We aimed to describe Familial Hemophagocytic Lymphohistiocytosis (F-HLH) patients' clinical features, intensive care courses, and outcomes. Methods: Multi-center retrospective cohort study of pediatric patients diagnosed with F-HLH from 2015 to 2020 in five tertiary centers in Saudi Arabia. Patients were classified as F-HLH based on their genetic confirmation of known mutation or on their clinical criteria, which include a constellation of abnormalities, early disease onset, recurrent HLH in the absence of other causes, or a family history of HLH. Results: Fifty-eight patients (28 male, 30 female), with a mean age of 21.0 ± 33.9 months, were included. The most common principal diagnosis was hematological or immune dysfunction (39.7%), followed by cardiovascular dysfunction in 13 (22.4%) patients. Fever was the most common clinical presentation in 27.6%, followed by convulsions (13.8%) and bleeding (13.8%). There were 20 patients (34.5%) who had splenomegaly, and more than 70% of patients had hyperferritinemia >500 mg/dl, hypertriglyceridemia >150 mg/dl and hemophagocytosis in bone marrow biopsy. Compared to deceased patients 18 (31%), survivors had significantly lower PT (p = 041), bilirubin level of <34.2 mmol/L (p = 0.042), higher serum triglyceride level (p = 0.036), and lesser bleeding within the initial 6 h of admission (p = 0.004). Risk factors for mortality included requirements of higher levels of hemodynamic (61.1% vs. 17.5%, p = 0.001) and respiratory (88.9% vs. 37.5%, p < 0.001) support, and positive fungal cultures (p = 0.046). Conclusions: Familial HLH still represents a challenge in the pediatric critical care setting. Earlier diagnosis and prompt initiation of appropriate treatment could improve F-HLH survival.

Use of Ceftolozane/tazobactam for the Treatment of Multidrug-resistant Pseudomonas aeruginosa Pneumonia in a Pediatric Patient with Combined Immunodeficiency (CID): A Case Report from a Tertiary Hospital in Saudi Arabia.

Infections, with multidrug-resistant Pseudomonas aeruginosa, are a major concern in the pediatric intensive care unit, especially in immunocompromised patients. Some of these strains are resistant to all beta-lactams, including carbapenems, leaving very limited treatment options remaining. These options include aminoglycosides and colistin, both of which have poor pharmacokinetic profiles with significant toxicities. Newer beta-lactam/beta-lactamase inhibitor combinations offer additional novel options to treat such infections, given their good pharmacokinetic profiles and activity against multi-drug resistant strains. Ceftolozane/tazobactam is a novel cephalosporin/beta-lactamase inhibitor combination approved in 2014. The drug demonstrates good activity against multidrug-resistant P. aeruginosa strains, including those resistant to all other antibiotics. Ceftolozane/tazobactam is currently approved in adult patients 18 years and older only. There are very limited data on its pharmacokinetic profile and clinical utility in the pediatric population. We report the use of ceftolozane/tazobactam to successfully treat pneumonia caused by multidrug-resistant P. aeruginosa in a pediatric patient with combined immunodeficiency syndrome.

Central venous lines are a risk factor for chylothorax in infants after cardiac surgery.

OBJECTIVE: Chylothorax complicates congenital heart disease (CHD) surgery and may be associated with significant morbidity. Etiology of chylothorax is multifactorial, and it has been associated with deep venous thrombosis and obstruction from central venous lines (CVLs) in patients without CHD. We sought to determine whether CVL insertion site was associated with the occurrence of chylothorax in infants after cardiac surgery. DESIGN: Retrospective cohort of patients less than one year of age who underwent CHD surgery requiring cardiopulmonary bypass from 2008 to 2012. Chylothorax was identified by clinical diagnosis and/or laboratory findings (milky effusion, fluid with >100 mg/dL of triglycerides and/or >80% of lymphocytes). Central venous lines insertion site was verified by reviewing procedure notes and chest x-rays. Internal jugular (IJ), subclavian vein, and femoral vein (FV) CVLs were used during the study period. RESULTS: Three hundred and ninety-two patients were included (mean age 97 days, mean weight 4.5 kg). Sixty-two (15.8%) of these patients developed chylothorax after surgery. Patients with chylothorax had longer bypass time (P=.02), longer cross-clamp time (P=.03), higher RACHS-1 category (P=.03), and more frequent upper body CVLs (IJ or subclavian vein; P=.03). There was no significant association with age, gender, preoperative weight, and height. Multivariate analysis showed patients with a CVL in the upper body (IJ or subclavian vein) were almost two times more likely to develop a chylothorax than patients who had FV CVL, (odds ratio=1.9, 95% confidence interval=1.05-5.60; P=.044). CONCLUSION: Postoperative chylothorax is associated with line insertion in the upper body (subclavian vein and IJ). Avoidance of CVLs in these locations may decrease its incidence.

Successful continuous renal replacement therapy using two single-lumen catheters in neonates and infants with cardiac disease.

BACKGROUND: Acute kidney injury (AKI) frequently occurs in neonates and infants after cardiopulmonary bypass (CPB) and may require renal replacement therapy (RRT). Peritoneal dialysis (PD) is the RRT modality of choice in neonates with AKI after CPB, but continuous renal replacement therapy (CRRT) may be necessary if PD is ineffective or contraindicated. Vascular access is challenging in this population, in part, due to small central vein size that may preclude placement. The risk of malfunction or morbidity associated with standard dialysis catheters may be excessive in neonates with congenital heart disease. We describe a unique approach to vascular access for CRRT in six small patients with AKI. CASE-DIAGNOSIS/TREATMENT: This is a retrospective review of six patients with fluid overload and AKI that received CRRT because PD was contraindicated. In all cases, CRRT was performed via two hemostasis valve sheaths placed into separate veins for dialysis access and return. The low-resistance sheaths provided excellent blood flow with normalization of metabolic derangements and significant fluid removal (median negative 167 ml/kg at 72 h). Mean circuit life before the first change was 55.2 ± 30.4 h. CONCLUSIONS: The use of two small single-lumen catheters in separate veins enables consistent and effective hemodiafiltration in neonates and infants with challenging vascular access.