Disease burden for patients with primary immunodeficiency diseases identified at reference hospitals in Guanajuato, Mexico.

BACKGROUND: In addition to the deleterious effect on health, there is considerable economic and psychosocial morbidity associated with primary immunodeficiency diseases (PID). Also, the cost of a late diagnosis frequently results in a heavy disease burden on the patient. The objective of this study was to collect and analyze data on patients with PID in the state of Guanajuato in Mexico, to indirectly estimate the burden of the disease. METHODS: An observational, longitudinal, and comparative study was conducted. A total of 44 patients were included and grouped according to the updated classification of PID. RESULTS: The median time elapsed from the onset of symptoms to the reference and diagnosis by a tertiary hospital was of 2.17 (IQR = 6.44) years. Before diagnosis, the number of hospitalizations/year per patient was 0.86 (IQR = 2.28), the number of visit to emergency room/year per patient was 0.92 (IQR = 1.77), the number of doctor's visits/year per patient was 15 (IQR = 11.25), whereas the school/work absence days per patient were reported in 52.72 (IQR = 56.35) days per year. After diagnosis, 20 patients (45.45%) received IVIG replacement therapy, and all of them presented a significant improvement (p <0.05) in all the mentioned variables. Characteristically, even when patients with PID received IVIG, there was still an important disease burden when comparing them against healthy controls. Complications secondary to PID were detected in 19 patients (43.18%). The reported overall mortality rate was 6.82% (n = 3). CONCLUSIONS: We were able to indirectly estimate an important disease burden in patients with PID; which is considered to be preventable, at least in part, with effective interventions like health planning, research, collaboration with primary care providers, and generation of policies and practices, in order to improve the quality of life and care of families with PID.

Primary immunodeficiency diseases at reference and high-specialty hospitals in the state of Guanajuato, Mexico.

BACKGROUND: In general, primary immunodeficiency diseases (PIDs) are underdiagnosed in most countries. The objective of this study was to describe the frequency and clinical spectrum of PID in the most important tertiary hospitals in our region. METHODS: An observational, cross-sectional, with retrospective chart, review study was conducted. A total of 26 patients were included and grouped according to the updated classification of PIDs. RESULTS: PIDs spectra were as follows: predominantly antibody deficiency diseases were the most common category (65.38%), followed by other well-defined immunodeficiency syndromes (11.55%), congenital defects of phagocyte number and/or function (7.69%), complement deficiencies (3.85%), combined T- and B-cell immunodeficiencies (3.85%), and defects in innate immunity (3.85%). The mean time elapsed from the onset of symptoms to the reference and diagnosis by a tertiary hospital was of 4.65 ± 6.95 years. CONCLUSIONS: Predominant antibody deficiency disease was the most common group of PIDs, agreeing with international reports. Awareness of underdiagnosis by physicians is crucial for a prompt diagnosis and treatment, which in turn should improve the quality of life among patients with PIDs.

Tratamiento de las crisis asmáticas en pediatría / Treatment of asthmatic crisis en pediatrics

Se presenta la experiencia adquirida durante un año en el tratamiento del paciente con crisis asmática en el servicio de urgencias de un hospital pediátrico. Se diseño un esquema en cinco fases. En la primera, se usó salbutamol en nebulizaciones; en la segunda, se agregó aminofilina y oxígeno a 40 por ciento; en la tercera, hidrocortisona en bolo; en la cuarta, isoproterenol; en la quinta, ventilación mecánica. En la fase I, se logró el control en 163 (75.5 porciento) de 216 pacientes; 87 requirieron de la fase II, resolviéndose la crisis en 65 (74.7 por ciento); en 22 niños (8.8 por ciento del total), se llegó a la tercera fase. Ninguno requirió de fase IV o V y no hubo defunciones. El esquema de tratamiento es de empleo sencillo. El uso de venoclisis, aminofilina, antibióticos y esteroides ayudó a disminuir la estancia hospitalaria.