The clinical features and impact of SARS-CoV-2/COVID-19 infection in children with Cystic Fibrosis (CF): A Qatari experience.

BACKGROUND: SARS-CoV-2 in children with cystic fibrosis (CF) has been reported to cause mild illness without pre-existing severe lung disease. This review described the clinical presentation and course of COVID-19 infection in children with CF in Qatar. METHODS: The pediatric CF registry of 51 patients in Qatar was reviewed for COVID-19 cases from February 2020 to February 2022. Demographics, vaccination status, symptoms, and course were reviewed. Data were expressed as median, range, frequencies, and percentages. RESULTS: The study included eight patients with CF below 18 years of age infected with COVID-19. The incidence of COVID-19 in children with CF was 15.7%. The median age was 11 (2-18) years. Half of the cohort were males. Seven patients were pancreatic sufficient (I1234V mutation), and one was pancreatic insufficient (3129del4 mutation). The median baseline FEV1 was 91 (78-107%) predicted. None had received CFTR modulators or undergone a lung transplant. Three patients were vaccinated before their infections. Two of them were asymptomatic. Six patients (75%) had a cough and flu-like symptoms. Three patients had a fever. Two patients were hospitalized due to pulmonary exacerbation; both had mild CF-lung disease. None required respiratory support. CONCLUSION: We report a favorable outcome of COVID-19 infection in children with CF, similar to published international studies. Our findings are attributable to the community-dominant milder CFTR mutation, precautionary measures, and causative COVID-19 strain. More longitudinal data are needed to study these factors as potential protective mechanisms.

Sensitization to Common Allergens Among Children with Asthma and Allergic Rhinitis in Qatar.

BACKGROUND: Childhood asthma (A) and allergic rhinitis (AR) are common in Qatar. Aeroallergens sensitization is integral in disease pathogenesis and clinical presentation. Determining sensitization patterns assists clinicians in tailoring an efficient medical management. OBJECTIVE: To determine the aeroallergen sensitization pattern and relationship to clinical parameters. METHODS: A retrospective review of children (2-14-years) files with i) Pediatric Allergist/or Pulmonologist confirmed-diagnosis of A, and AR, and ii) positive skin prick test (SPT). RESULTS: Among 473 patients (69.1% males; 30.9% females), aged 7.6 years, family history was positive in 66.3%: 59.4% in A, 64.2% AR, and 78.2% A-AR. The number of allergens/patients was 2.1±1.7. Median eosinophil count was 400 cells/ul and IgE 287 KU/L. Rates of A, AR, and A-AR varied significantly in children ≤5 years compared to >10 years: A was 43.2% vs 17.8%, and AR 34.5% vs 16.4%. Two hundred and four children (43.1%) were mono-sensitized, 215 (45.5%) oligosensitized (2-3 allergens), and 54 (11.4%) polysensitized (≥4 allergens). A-AR ranked the top number of positive allergens. The commonest aeroallergen was Der p1 (38.1%), followed by Der f (29.0%), cat (22.6%), alternaria (18.8%), American cockroach (18.4%), and dog (14.0%). House dust mite (HDM) and American cockroach were commoner in ≤5 years than older >10-year children (52.5%, 24.1%), while cat and dog allergens were commoner in older ones (37.1%, 21.6%). CONCLUSION: Family history is quite positive in patients with A and AR. Common aeroallergens include HDM, cats, and alternaria in the young children, while animal allergens were commoner in the older children.

The emergence of multidrug-resistant Pseudomonas aeruginosa in cystic fibrosis patients on inhaled antibiotics.

INTRODUCTION: Multidrug-resistant Pseudomonas aeruginosa (MDR-PA) is an important and growing issue in the care of patients with cystic fibrosis (CF), and a major cause of morbidity and mortality. OBJECTIVE: The objective of the study was to describe the frequency of MDR-PA recovered from the lower respiratory samples of pediatric and adult CF patients, and its antibiotic resistance pattern to commonly used antimicrobial agents including ß-lactams, aminoglycosides, and fluoroquinolones. MATERIALS AND METHODS: The lower respiratory isolates of P. aeruginosa were obtained from inpatients and outpatients CF clinics from a tertiary care teaching hospital for the period from October 2014 to September 2015. The identification and antimicrobial susceptibility for all the isolates were performed by using the BD Phoenix™ and E-test in compliance with Clinical and Laboratory Standards Institute (CLSI) guidelines. RESULTS: A total of 61 P. aeruginosa samples were isolated from thirty CF patients from twenty families. Twelve sputum samples were positive for MDR-PA (seven nonmucoid and five mucoid isolates) from five CF patients (five families) with moderate-to-very severe lung disease given MDR-PA frequency of 19.7%. The median age of the study group was 20 (range 10-30) years. Three CF patients were on chronic inhaled tobramycin and two on nebulized colistin. The antimicrobial patterns of isolates MDR-PA showed the highest rate of resistance toward each gentamycin, amikacin, and cefepime (100%), followed by 91.7% to ciprofloxacin, 75% to tobramycin, 58.3% to meropenem, and 50% to piperacillin-tazobactam. None of the isolates were resistant to colistin during the study period. CONCLUSION: The study results emphasize that the emergence of a significant problem in the clinical isolates of P. aeruginosa in CF patients that dictate appropriate attention to the antibiotic management after proper surveillance.

Two Qatari siblings with cystic fibrosis and apparent mineralocorticoid excess.

Cystic fibrosis (CF) and apparent mineralocorticoid excess (AME) syndrome are both autosomal recessive disorders that result from mutations of specific identified genes for each condition. CF is caused by defects in the Cystic fibrosis trans membrane conductance regulator (CFTR) gene which encodes for a protein that functions as a chloride channel and regulates the flow of other ions across the apical surface of epithelial cells. AME is due to the deficiency of 11ß-hydroxysteroid dehydrogenase type 2 enzyme (11ßHSD2), which is responsible for the peripheral inactivation of cortisol to cortisone. Cortisol excess stimulates the mineralocoritoid receptors (MR) resulting in intense sodium retention, hypokalemia and hypertension. We report on a consanguineous Arab family, in which two sibs inherited both CF and AME. Gene testing for AME revealed previously unreported mutation in the 11ßHSD2 gene. This report draws attention to the importance of recognizing the possibility of two recessive disorders in the same child in complex consanguineous families. Moreover, it provides a unique opportunity to highlight the implications of the coexistence of two genetic disorders on patient care and genetic counseling of the family.

Chryseobacterium indologenes Meningitis in a Healthy Newborn: A Case Report.

Chryseobacterium indologenes is a rare cause of infection in infants. The majority of case reports belong to hospitalized infants with indwelling devices. We are presenting a rare case of Chryseobacterium indologenes meningitis in a healthy newborn with no neonatal intensive care unit admissions or indwelling devices. The pathogen is resistant to many antibiotics and the patient was successfully treated with cefepime. This is the first case of C. indolegenes meningitis presented in a newborn with no indwelling device, NICU or long-term broad spectrum antibiotics. The choice of antibiotics can be challenging since the pathogen may exhibit resistance to a number of antibiotics.