RESUMO
This retrospective study was conducted to describe clinical and investigational findings and to determine the effect of thiamine treatment on mortality in patients admitted with acute non-infectious encephalopathy to a hospital in Sikkim between October 2019 and March 2021. Amongst 37 included patients the median age was 4 mo (IQR 3-5), 62.2% were males, 75% were exclusively breastfed infants, 67.6% and 89.2% patients had ophthalmologic and respiratory abnormalities respectively. Multisystem involvement was common. Bilateral basal ganglia involvement was noted in 75% of neuroimaging. Biochemical thiamine deficiency was confirmed in one infant. None of the 11 patients who received thiamine died whereas 20 among 26 patients who did not receive thiamine died [case fatality rate (CFR) 76.9%]. Thiamine treatment was significantly associated with reduced odds of mortality (aOR 0.046, 95% CI 0.0024-0.86, p 0.039). In patients with acute non-infectious encephalopathy and bilateral basal ganglia involvement thiamine use was associated with decreased mortality.
RESUMO
The authors report an infant with common variable immunodeficiency (CVID) with Pneumocystis pneumonia who developed secondary pulmonary alveolar proteinosis (PAP). This is the youngest infant reported to develop PAP secondary to Pneumocystis infection in an immunocompromised state. He was effectively managed with anti-microbials, frequent lung toilet, optimized mechanical ventilation, and supportive care.