Severe combined immunodeficiency is an inborn error of immunity characterized by impairments in the numbers and functions of T and B lymphocytes due to various genetic causes, and if it remains ...untreated, patients succumb to infections during the first 2 years of life.
Purpose and Methods
This study reported retrospective data from 72 infants diagnosed with SCID including their major clinical features, HSCT characteristics, and outcomes over a 20-year period (1997–2017).
Results
Sixty-one of 72 SCID patients in the study underwent HSCT from 1997 to 2017. Median ages at the time of diagnosis and transplantation were 3.5 months and 5 months, respectively. Consanguinity was present in 68% of the patients, and T − B − NK + phenotype was predominantly identified. The overall survival was 80.3% over a 20-year period. However, the patients transplanted during an active infection had a lower survival rate of 73.9% compared to 100% for patients transplanted infection-free or with a previous infection that had resolved. The survival rate was significantly higher among recipients of HLA-identical transplants (92.9%), compared to recipients of mismatched related transplants (70%). The overall survival increased from 50 (1997–2006) to 85% (2007–2017) during the last 10 years.
Conclusions
This is one of the largest single-center studies in Turkey with extensive experience about SCID patients. Early diagnosis of SCID patients before the onset of an infection and early transplantation are shown to be extremely important factors affecting the outcome and increasing the survival regardless of the donor type based on the results of this study.
BACKGROUND:Colistin is active against most multidrug-resistant, aerobic Gram-negative bacteria. Because of the reported nephrotoxicity during the first years of use of colistin, there were concerns ...of its use in pediatrics where there was limited experience The aim of this study is to document the clinical characteristics and outcomes of use of colistin in pediatric patients at a pediatric intensive care unit in Turkey.
METHODS:We reviewed the medical and laboratory records of 29 critically ill children who were treated with colistin for 38 courses between January 2011 and December 2011 at the Department of Pediatric Intensive Care Unit in Ankara University Medical School, Turkey.
RESULTS:The median age was 17 months (range 3–217 months). Male-to-female ratio was 1:1.37. Ventilator-associated pneumonia (21 courses) was the leading diagnosis followed by catheter-related blood stream infection (6 courses), bacteremia (4 courses), ventriculoperitoneal shunt infection, peritonitis and pneumonia (1 course). The most commonly isolated microorganisms were Acinetobacter baumanni, Pseudomonas aeruginosa, Klebsiella pneumoniae, Serratia marcescens, Stenotrophomonas maltophilia, and Enterobacter cloacae. Two colistin formulations were used. Colimycin (Kocak Farma) was used in 21 colistin treatment episodes. The median dosage of colistin in this group was 5.0 mg/kg/d (2.3–5.6 mg/kg/d). Colomycin (Forest Laboratories) was used in 17 colistin treatment episodes. The median dosage of colistin in the second group was 75,000 International Unit/kg/d (50,000–80,000 International Unit/kg/d). Thirty colistin treatment episodes (79%) had a good or partial clinical response and 8 (21%) had a poor clinical response. Of the 8 colistin treatment episodes with poor clinical response, 3 were in the Colimycin group and 5 were in the Colomycin group. Ten patients died. There was no evidence of neurotoxicity in this study. Nephrotoxicity was observed in 1 patient but was not attributed to colistin because the patient had multiorgan failure at the same time.
CONCLUSIONS:This study in a small cohort of patients suggests that the use of colistin in severe nosocomial infections caused by multidrug-resistant Gram-negative bacteria is well-tolerated and efficacious.
Broad and deep perianal wounds are challenging in both adult and pediatric ICUs. These wounds, if contaminated with gastrointestinal flora, can cause invasive sepsis and death, and recovery can be ...prolonged. Controlling the source of infection without diverting stool from the perianal region is complicated. The option of protective colostomy is not well-known among pediatric critical care specialists, but it can help patients survive extremely complicated critical care management. These authors present three critically ill children who required temporary protective colostomy for perianal wounds because of various clinical conditions. Two patients were treated for meningococcemia, and the other had a total artificial heart implantation for dilated cardiomyopathy. There was extensive and profound tissue loss in the perianal region in the patients with meningococcemia, and the patient with cardiomyopathy had a large pressure injury. Timely, transient, protective colostomy was beneficial in these cases and facilitated the recovery of the perianal wounds. Temporary diverting colostomy should be considered as early as possible to prevent fecal transmission and accelerate perianal wound healing in children unresponsive to local debridement and critical care.
Background
Acute kidney injury (AKI) is associated with an increased risk of mortality, especially in pediatric intensive care units. The aim of this study was to determine the risk factors of AKI in ...children undergoing cardiac surgery for congenital heart disease and to compare two different classification systems: pediatric risk–injury–failure–loss–end‐stage renal disease (pRIFLE) and Acute Kidney Injury Network (AKIN).
Methods
We retrospectively analyzed 145 patients undergoing pediatric congenital heart surgery who were between 1 month and 18 years of years, and treated at a cardiovascular surgery department from January 2009 to October 2011.
Results
One hundred and thirty‐seven patients (mean age, 36.6 ± 43.3 months) were enrolled: 84 (61.3%) developed AKI according to the pRIFLE criteria (25.5%, risk; 20.4%, injury; 15.3%, failure); and 65 patients (47.4%) developed AKI according to the AKIN criteria (15.3%, stage I; 18.2%, stage II; and 13.9%, stage III). Children younger than 11 months were more likely to develop AKI (P < 0.005). Longer cardiopulmonary bypass time was associated with an increased risk of AKI (P < 0.05). pRIFLE identified AKI more frequently than AKIN (P < 0.0005). pRIFLE may help in the early identification of patient at risk for AKI and seems to be more sensitive in pediatric patients (P < 0.05). Any degree of AKI in both classifications was associated with increased mortality (pRIFLE: OR, 15.1; AKIN: OR, 11.2; P = 0.007).
Conclusion
pRIFLE identified AKI more frequently than the AKIN criteria. pRIFLE identified patients at risk for AKI earlier, and was more sensitive in pediatric patients. Any degree of AKI in both classifications was associated with increased mortality. Both sets of criteria had the same association with mortality.
Pediatric intensive care units (PICUs), where children with critical illnesses are treated, require considerable manpower and technological infrastructure in order to keep children alive and free ...from sequelae.
In this retrospective comparative cohort study, hospital records of patients aged 1 month to 18 years who died in the study PICU between January 2015 and December 2019 were reviewed.
A total of 2,781 critically ill children were admitted to the PICU. The mean±standard deviation age of 254 nonsurvivors was 64.34±69.48 months. The mean PICU length of stay was 17 days (range, 1-205 days), with 40 children dying early (<1 day of PICU admission). The majority of nonsurvivors (83.9%) had comorbid illnesses. Children with early mortality were more likely to have neurological findings (62.5%), hypotension (82.5%), oliguria (47.5%), acidosis (92.5%), coagulopathy (30.0%), and cardiac arrest (45.0%) and less likely to have terminal illnesses (52.5%) and chronic illnesses (75.6%). Children who died early had a higher mean age (81.8 months) and Pediatric Risk of Mortality (PRISM) III score (37). In children who died early, the first three signs during ICU admission were hypoglycemia in 68.5%, neurological symptoms in 43.5%, and acidosis in 78.3%. Sixty-seven patients needed continuous renal replacement therapy, 51 required extracorporeal membrane oxygenation support, and 10 underwent extracorporeal cardiopulmonary resuscitation.
We found that rates of neurological findings, hypotension, oliguria, acidosis, coagulation disorder, and cardiac arrest and PRISM III scores were higher in children who died early compared to those who died later.
•COVID- 19 disease can present with thrombotic complications including cerebral venous sinus thrombosis and is extremely rare in the pediatric age group.•SARS-CoV-2 infection may cause cerebral ...venous sinus thrombosis and other venous thromboembolism either through increased cytokine and clotting factors production or direct endothelial injury through binding to the ACE2 receptor.•It presents with different neurologic manifestations, and diagnosis is confirmed by imaging such as MRV and treated with anticoagulants.
The clinical manifestations of SARS-CoV-2 infection mainly involve the respiratory system. However, there is increasing evidence that this virus can affect other organs, causing a wide range of clinical symptoms. This is the report of a 40-day-old patient who presented with sepsis and had no risk factors other than SARS-CoV-2 infection, whose radiological findings were compatible with cerebral sinus vein thrombosis.
Microbiota composition might play a role in the pathophysiology and course of sepsis, and understanding its dynamics is of clinical interest. Invasive meningococcal disease (IMD) is an important ...cause of community-acquired serious infection, and there is no information regarding microbiota composition in children with meningococcemia. In this study, we aimed to evaluate the intestinal and nasopharyngeal microbiota composition of children with IMD.
In this prospective, multi-center study, 10 children with meningococcemia and 10 age-matched healthy controls were included. Nasopharyngeal and fecal samples were obtained at admission to the intensive care unit and on the tenth day of their hospital stay. The V3 and V4 regions of the 16S rRNA gene were amplified following the 16S Metagenomic Sequencing Library Preparation.
Regarding the alpha diversity on the day of admission and on the tenth day at the PICU, the Shannon index was significantly lower in the IMD group compared to the control group (
= 0.002 at admission and
= 0.001, on the tenth day of PICU). A statistical difference in the stool samples was found between the IMD group at Day 0 vs. the controls in the results of the Bray-Curtis and Jaccard analyses (
= 0.005 and
= 0.001, respectively). There were differences in the intestinal microbiota composition between the children with IMD at admission and Day 10 and the healthy controls. Regarding the nasopharyngeal microbiota analysis, in the children with IMD at admission, at the genus level,
was significantly more abundant compared to the healthy children (
< 0.001). In the children with IMD at Day 10, genera
and
were decreased compared to the healthy children. In the children with IMD on Day 0, for paired samples,
,
, and
were significantly more abundant compared to the children with IMD at Day 10. In the children with IMD at Day 10, the
and
genera were decreased, and 20 different genera were more abundant compared to Day 0.
We first found alterations in the intestinal and nasopharyngeal microbiota composition in the children with IMD. The infection itself or the other care interventions also caused changes to the microbiota composition during the follow-up period. Understanding the interaction of microbiota with pathogens, e.g.,
, could give us the opportunity to understand the disease's dynamics.
Objectives: To identify Urinary Catheter (UC)-associated Urinary Tract Infections (CAUTI) incidence and risk factors (RF) in nine Middle Eastern countries.
Methods: We conducted a prospective cohort ...study between 1 January 2014 and 2 December 2022 in 212 Intensive Care Units (ICUs) of 67 hospitals in 38 cities in nine Middle Eastern countries (Bahrain, Egypt, Jordan, Kuwait, Lebanon, Morocco, Saudi Arabia, Turkey, and the UAE). To estimate CAUTI incidence, we used the number of UC days as denominator and the number of CAUTIs as numerator. To estimate CAUTI RFs, we analyzed the following 10 variables using multiple logistic regression: patient sex, age, length of stay (LOS) before CAUTI acquisition, UC-days before CAUTI acquisition, UC-device utilization (DU) ratio, hospitalization type, ICU type, facility-ownership, country income level classified by World Bank, and time period.
Results: Among 50 637 patients hospitalized for 434 523 patient days, there were 580 cases of acquired CAUTIs. The pooled CAUTI rate per 1000 UC days was 1.84. The following variables were independently associated with CAUTI: age, rising risk 1.0% yearly (adjusted odds ratio aOR equivalent 1.01, 95% CI: 1.01-1.02; p < 0.0001); female sex (aOR equivalent 1.31, 95% CI: 1.09-1.56; p < 0.0001); LOS before CAUTI acquisition, rising risk 6.0% daily (aOR equivalent 1.06, 95% CI: 1.05-1.06; p < 0.0001); and UC/ DU ratio (aOR equivalent 1.11, 95% CI: 1.06-1.14; p < 0.0001). Patients from lower-middle- income countries (aOR equivalent 4.11, 95% CI: 2.49-6.76; p < 0.0001) had a similar CAUTI risk to the upper-middle countries (aOR equivalent 3.75, 95% CI: 1.83-7.68; p < 0.0001). The type of ICU with the highest risk for CAUTI was neurologic ICU (aOR equivalent 27.35, 95% CI: 23.03-33.12; p < 0.0001), followed by medical ICU (aOR equivalent 6.18, 95% CI: 2.07-18.53; p < 0.0001) when compared to cardiothoracic ICU. The periods 2014-2016 (aOR equivalent 7.36, 95% CI: 5.48-23.96; p < 0.001) and 2017-2019 (aOR equivalent 1.15, 95% CI: 3.46-15.61; p < 0.001) had a similar risk to each other, but a higher risk compared to 2020-2022.
Conclusions: The following CAUTI RFs are unlikely to change: age, sex, ICU type, and country income level. Based on these findings, it is suggested to focus on reducing LOS, UC/DU ratio, and implementing evidence-based CAUTI prevention recommendations.
