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OBJECTIVE: The bacille Calmette Guérin (BCG) vaccine is administered worldwide to prevent tuberculosis. Although, Post BCG vaccination complications like disseminated BCG infections are rare and immunocompromised children are at high risk of developing BCG-related complications including BCG-lymphadenitis and other disseminated diseases. PATIENTS AND METHODS: This was a prospective study of children who developed disseminated BCG after vaccination who were admitted in three tertiary care hospital in Riyadh, Saudi Arabia, in the year 2015. The clinical presentation, microbiological/immunological evaluation and outcome will be discussed. RESULTS: 12 cases (7 males and 5 females) of disseminated BCG infections after vaccination were documented with age ranges 3 and 32 months. Eight (66%) patients had interleukin IL-12 deficiency and 3 (25%) had severe combined immune deficiency (SCID) and 1 (8%) had Interferon gamma receptor II deficiency. 9 (75%) patients presented with generalized lymphadenopathy. Hepatosplenomegaly was present in 6 (50%) patients. 2 (16%) patients presented with a recurrent skin infection and persistent oral candidiasis in one patient and pneumonia in the other. Five (41%) families were from 1st degree consanguineous marriage. Tuberculosis (TB) culture and sensitivity were positive for Mycobacterium bovis from gastric aspirate (GA) in 4 patients and the lymph node in 9 patients and 1 patient had culture positive from both skin lesion and lymph nodes. Mycobacterium bovis PCR was positive in 4 patients. All patients received anti-tuberculosis therapy; all patients survived except one who died due to multi-organ failure. CONCLUSIONS: These results indicate that the prevalence of disseminated BCG vaccine in immunocompromised Saudi children is significantly high. Since in our region we have a high consanguinity rate and a high number of primary immune deficiency disorder (PID), we believe that BCG vaccination should be postponed till a child reaches one-year-old and appropriate tests exclude the diagnosis of primary immunodeficiency diseases. [ABSTRACT FROM AUTHOR]

Background: Appropriate asthma management in children reduces emergency department visits, hospitalization, and improves the quality of life. We aim to assess the level of children asthma control and its association with parental knowledge., Methods: A prospective study conducted to measure childhood asthma control with a validated childhood asthma control test (C-ACT), and to assess asthma knowledge among the parents of children aged 4-11 years and their parents upon asthma clinic visits. C-ACT score ≤ 19 is considered as uncontrolled child asthma., Results: We have invited 238 parents to participate in the study; 177 (74.4%) completed the survey. The mean age of the parents and their children were 38.8 ± 7.6 and 7.8 ± 2.7 years, respectively; 28.2% of parents were smokers, and 46.3% of them were college graduated. Nearly 61.6% of the parents and children scored ≤ 19 on C-ACT; 54.2% and 37.9% of parents knew how inhaled salbutamol and corticosteroids work, respectively. A quarter of the parents received an asthma action plan. Multinomial logistic regression analysis showed that parents who did not know their children's medications name (OR, 6.1; 95% CI, 2.15-17.29), and when to use inhaled corticosteroid (OR, 2.1; 95% CI, 1.32-3.45) were independent factors predicting uncontrolled asthma in children with score ≤ 19., Conclusions: The study indicated that there is an association between poor asthma control (scored ≤ 19 on C-ACT) and parental knowledge of asthma medications. The parents should be educated thoroughly on asthma care, including medications used to minimize asthma exacerbations in their children., (© 2020 Wiley Periodicals LLC.)

Autosomal recessive interleukin (IL)-12 p40 (IL-12p40) deficiency is a rare genetic etiology of mendelian susceptibility to mycobacterial disease (MSMD). We report the genetic, immunologic, and clinical features of 49 patients from 30 kindreds originating from 5 countries (India, Iran, Pakistan, Saudi Arabia, and Tunisia). There are only 9 different mutant alleles of the IL12B gene: 2 small insertions, 3 small deletions, 2 splice site mutations, and 1 large deletion, each causing a frameshift and leading to a premature stop codon, and 1 nonsense mutation. Four of these 9 variants are recurrent, affecting 25 of the 30 reported kindreds, due to founder effects in specific countries. All patients are homozygous and display complete IL-12p40 deficiency. As a result, the patients lack detectable IL-12p70 and IL-12p40 and have low levels of interferon gamma (IFN-γ). The clinical features are characterized by childhood onset of bacille Calmette-Guérin (attenuated Mycobacterium bovis strain) (BCG) and Salmonella infections, with recurrences of salmonellosis (36.4%) more common than recurrences of mycobacterial disease (25%). BCG vaccination led to BCG disease in 40 of the 41 patients vaccinated (97.5%). Multiple mycobacterial infections were rare, observed in only 3 patients, whereas the association of salmonellosis and mycobacteriosis was observed in 9 patients. A few other infections were diagnosed, including chronic mucocutaneous candidiasis (n = 3), nocardiosis (n = 2), and klebsiellosis (n = 1). IL-12p40 deficiency has a high but incomplete clinical penetrance, with 33.3% of genetically affected relatives of index cases showing no symptoms. However, the prognosis is poor, with mortality rates of up to 28.6%. Overall, the clinical phenotype of IL-12p40 deficiency closely resembles that of interleukin 12 receptor β1 (IL-12Rβ1) deficiency. In conclusion, IL-12p40 deficiency is more common than initially thought and should be considered worldwide in patients with MSMD and other intramacrophagic infectious diseases, salmonellosis in particular.