Metabolic crisis in maple syrup urine disease: an unusual complication of a rare disease: a case report.

A 19-year-old woman with known maple syrup urine disease presented to hospital with metabolic crisis in the setting of influenza type A infection and intractable vomiting, rapidly progressing to acute cerebral oedema manifesting as refractory seizures and decreased level of consciousness needing emergency intubation and mechanical ventilation, continuous veno-venous haemodiafiltration and thiopentone coma. A computed tomography scan and magnetic resonance imaging of the brain demonstrated classic signs of cerebral oedema secondary to a metabolic crisis from the metabolic disorder. Her management posed multiple challenges to all teams involved due to lack of familiarity and experience in managing this clinical scenario in the adult intensive care setting.

Normal values for sagittal spinal alignment: a study of Brazilian subjects.

OBJECTIVES: The purpose of this study is to investigate the normal values of and chain of correlations between spinopelvic parameters in a Brazilian population. METHODS: This is a prospective observational study including asymptomatic adult subjects who had full spinal radiographs performed. The subjects were stratified by age into 3 groups (18-39 years old, 40-59 years old, and >60 years old), and radiographic parameters were compared across age groups and gender using ANOVA and Student's t-test, respectively. The relationships between various radiographic parameters were evaluated with Pearson correlation coefficients. RESULTS: One hundred and thirty asymptomatic volunteers (mean age, 48 years) met the inclusion criteria. The mean sagittal parameters in a normal Brazilian population were as follows: lumbar lordosis (LL) of 56.8°, pelvic tilt (PT) of 12.4°, pelvic incidence (PI) of 49.4°, PI-LL of -7.4°, T1 pelvic angle (TPA) of 8°, sagittal vertical axis (SVA) of -0.54 cm and T1 slope of 25.2°. Subjects ≥60 years old had significantly higher values of SVA (p=0.024) and TPA (p=0.009) than the two younger age groups. The TPA was significantly correlated with the following spinopelvic parameters: LL (r=-0.172, p=0.005), PT (r=0.776, p<0.001), PI (r=0.508, p<0.001), PI-LL (r=0.717, p<0.001), SVA (r=0.409, p<0.001) and T1 slope (r=0.172, p=0.050). CONCLUSION: This study demonstrated significant physiologic trunk inclination with increasing age. The TPA, an angular parameter of global spinal alignment, presented a chain of correlations with different spinal segments.

Normal values for sagittal spinal alignment: a study of Brazilian subjects

OBJECTIVES: The purpose of this study is to investigate the normal values of and chain of correlations between spinopelvic parameters in a Brazilian population. METHODS: This is a prospective observational study including asymptomatic adult subjects who had full spinal radiographs performed. The subjects were stratified by age into 3 groups (18-39 years old, 40-59 years old, and >60 years old), and radiographic parameters were compared across age groups and gender using ANOVA and Student's t-test, respectively. The relationships between various radiographic parameters were evaluated with Pearson correlation coefficients. RESULTS: One hundred and thirty asymptomatic volunteers (mean age, 48 years) met the inclusion criteria. The mean sagittal parameters in a normal Brazilian population were as follows: lumbar lordosis (LL) of 56.8°, pelvic tilt (PT) of 12.4°, pelvic incidence (PI) of 49.4°, PI-LL of -7.4°, T1 pelvic angle (TPA) of 8°, sagittal vertical axis (SVA) of -0.54 cm and T1 slope of 25.2°. Subjects ≥60 years old had significantly higher values of SVA (p=0.024) and TPA (p=0.009) than the two younger age groups. The TPA was significantly correlated with the following spinopelvic parameters: LL (r=-0.172, p=0.005), PT (r=0.776, p<0.001), PI (r=0.508, p<0.001), PI-LL (r=0.717, p<0.001), SVA (r=0.409, p<0.001) and T1 slope (r=0.172, p=0.050). CONCLUSION: This study demonstrated significant physiologic trunk inclination with increasing age. The TPA, an angular parameter of global spinal alignment, presented a chain of correlations with different spinal segments.