Early disseminated Mycobacterium Abscessus Complex Infection in an Infant with Coexisting Cystic Fibrosis and Progressive Familial Intrahepatic Cholestasis

Mycobacterium abscessus complex (MABSC) is a rapidly growing mycobacterium and may rarely cause disseminated infections in immunocompromised patients. In patients with Cystic Fibrosis (CF), it peaks between the ages of 11 and 15 years. We present a 5 months old infant with coexisting CF and Progressive Familial Intrahepatic Cholestasis (PFIC) who had pulmonary and cutaneous dissemination of MABSC infection. The management of this disseminated infection in an infant with two coexisting chronic diseases was challenging which resulted in a rapid deterioration of lung disease and the progression of PFIC to liver cirrhosis with a fatal outcome. Keywords: Cystic Fibrosis; Atypical Mycobacterium; Mycobacterium abscessus complex; Progressive Familial Intrahepatic Cholestasis

Tobramycin Activity for Pseudomonas Aeruginosa spp. Isolated in Cystic Fibrosis Patients

Study Objective: To determine in vitro activity of Tobramycin for Pseudomonas aeruginosa spp. isolated in children with cystic fibrosis (CF); to retrospectively study the efficiency dynamics of inhalative Tobramycin in management of bronchopulmonary process exacerbation in children with CF aged 9 years old (2009–2018). Study Design: retrospective analysis. Materials and Methods. The study included 173 children with cystic fibrosis aged 3 to 17 years old; the median age in the general group was 11 [8; 15] years. Subjects were divided into two comparison groups, depending on various 14-day combined antipseudomonal therapy: 42 children were treated with Cefepime 150 mg/kg + Amikacin 20 mg/kg IV drop infusion; 48 subjects — with Meropenem 100 mg/kg + Amikacin 20 mg/kg IV drop infusion; 83 patients — with Ceftazidime 200 mg/kg IV + Tobramycin (Bramitob) 300 mg twice daily, inhalation + Ciprofloxacin per os 30–40 mg/kg. Study Results. After the two-week therapy of 83 children with Tobramycin inhalations plus oral Ciprofloxacin and Ceftazidime IV, 123 (82%) out of 150 pathogenic P. aeruginosa and Staphylococcus aureus spp. were eliminated (p < 0.001 when compared to bacteriologic test results of pre-therapy bronchial mucus). Comparison groups demonstrated statistically significantly lower number of such subjects: in Cefepime + Amikacin group, 32 (43.8%) out of 73 pathogenic P. aeruginosa and S. aureus spp. were eliminated; whereas in Meropenem + Amikacin group, only 28 (35.4%) pathogens were eliminated out of 79 species. Upon admission in 2013, 50 followed up children underwent antimicrobial therapy with inhalative Tobramycin using the same regimen as in 2009; the P. aeruginosa elimination was the same: 11 (18%) out of 61 pathogenic species remained viable, 50 (82%) species were eliminated, as demonstrated by control sputum. The difference between the number of P. aeruginosa and S. aureus colonies before and after therapy was statistically significant (p < 0.001). Practically the same result after the use of inhalative Tobramycin was observed in 2018: 14 (70%) out 20 children with P. aeruginosa spp. had their control sputum samples sanitised (p < 0.001). Conclusion. Despite a 20-year history of using in patients with cystic fibrosis, inhalative Tobramycin is still clinically efficient and potent for isolated P. aeruginosa as a component of primary eradication and management of chronic Pseudomonal lung infections. Keywords: cystic fibrosis, Pseudomonas aeruginosa, inhalative Tobramycin.

Preliminary national report on cystic fibrosis epidemiology in Tunisia: the actual state of affairs

Aim: To establish a preliminary national report on clinical and genetic features of cystic fibrosis (CF) in Tunisian children as a first measure for a better health care organization. Methods: All children with CF diagnosed by positive sweat tests between 1996 and 2015 in children’s departments of Tunisian university hospitals were included. Data was recorded at diagnosis and during the follow-up from patients’ medical records. Results: In 12 departments, 123 CF children were collected. The median age at diagnosis was 5 months with a median diag- nosis delay of 3 months. CF was revealed mostly by recurrent respiratory tract infections (69.9%), denutrition (55.2%), and/ or chronic diarrhea (41.4%). The mean sweat chloride concentration was 110.9mmol/L. At least one mutation was found in 95 cases (77.2%). The most frequent mutations were Phe508del (n=58) and E1104X (n=15). Fifty-five patients had a Pseudomonas Aeruginosa chronic colonization at a median age of 30 months. Cirrhosis and diabetes appeared at a mean age of 5.5 and 12.5 years respectively in 4 patients each. Sixty-two patients died at a median age of 8 months. Phe508del mutation and hypotrophy were associated with death (p=0.002 and p<0.001, respectively). Conclusion: CF is life-shortening in Tunisia. Setting-up appropriate management is urgent. Keywords: Cystic fibrosis epidemiology; Tunisia.

Sialadenitis in Cystic Fibrosis: Case Report

ABSTRACT Objective of the Paper: To present a case report of sialadenitis in cystic fibrosis and recommendations to diagnose the condition. Key Points. Cystic fibrosis (CF) is an autosomal recessive genetic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene and affects exocrine glands, including salivary glands. The article describes a case report of sialadenitis in cystic fibrosis in a 12-year old boy. Bacterial sialadenitis was diagnosed after a consultation with a maxillo-facial surgeon using lab and ultrasound results. The patient was treated with antimicrobials (amoxicillin potentiated by clavulanat), drotaverine, increased fluid intake. Conclusion. Sialadenitis is one of the comorbidities in cystic fibrosis patients associated with fever, ear pain, salivary glands swelling. In order to make the final diagnosis, a consultation with a maxillo-facial surgeon, infectionist and salivary glands ultrasound are necessary. Keywords: cystic fibrosis, sialadenitis, case report.