RESUMEN
OBJECTIVE: To develop a novel formula for pH probe placement with adequate accuracy. METHODS: Children (3-18 y) undergoing pH-metry were prospectively evaluated. Their height and corrected pH probe position under X-ray (2 vertebrae above the diaphragm) was recorded and the linear-regression analysis was performed to derive a novel formula. Its accuracy was checked on an additional group of prospectively included children. The success rate of a newly developed formula was estimated and compared to the performance of previously used formulae. The difference in the suggested placement of the probe (cm from nostrils) was calculated. RESULTS: Based on 670 children with pH probe placed under X-ray, the following formula was developed using the linear-regression analysis: L = 0.184x + 4.4 (cm) (L = probe placement depth, x = body height). Its accuracy was confirmed on additional 111 children resulting in almost 85% success rate. The formula showed significant difference in the suggested placement from formulae used previously: +4.9 ± 0.8 cm, +2.4 ± 0.1 cm, +0.7 ± 0.6 cm, +1.1 ± 0.4 cm, +1.8 ± 0.3 cm, +2.2 ± 0.5 cm from the one by the Strobel, Moreau, Wilson, Nowak, Staiano-Clouse formulae, and the GOSH table with the calculated success rates of 1.8%, 43.2%, 65.8%, 77.5%, 65.8% and 54.1%, respectively. A table suggesting placement depth based on the body height was developed. CONCLUSION: The present formula provides 85% success of pH probe placement in children ≥ 3 y suggesting its use in routine practice. More data are needed to confirm that probe adjustment under X-ray is unnecessary.
RESUMEN
Background: Inborn errors of IL-12/IL-23-IFNγ immunity underlie Mendelian susceptibility to mycobacterial diseases (MSMD), a group of immunodeficiencies characterized by a highly selective susceptibility to weakly virulent strains of mycobacteria, such as non-tuberculous mycobacteria (NTM) and bacillus Calmette-Guérin (BCG). Cutaneous mycobacterial infections are common in MSMD and may represent a red flag for this immunodeficiency. Objectives: We present a case series of four paediatric patients with MSMD, specifically with IFNγR1 and STAT1 deficiencies, and cutaneous NTM/BCG infections to increase awareness of this immunodeficiency, which may, in some cases, be intercepted by the dermatologist and thus timely referred to the immunologist. Materials & Methods: Clinical, laboratory and genetic investigations of the four paediatric patients with MSMD are presented. Results: All four presented patients experienced early complications after BCG vaccination. Two patients suffered recurrent mycobacteriosis, one patient experienced delayed BCG reactivation, and one patient died of disseminated avian mycobacteriosis. The dermatological manifestation in these patients included destructive nasal ulcerations, scrofuloderma of various sites and lupus vulgaris. All patients had a normal basic immune phenotype. Conclusion: The presented cases demonstrate that NTM/BCG infections in otherwise seemingly immunocompetent patients should raise suspicion of MSMD. This is of utmost importance as specific therapeutic approaches, such as IFNγ treatment or haematopoietic stem cell transplantation, may be employed to improve the disease outcome.
