RESUMO
Objectives: This study aimed to evaluate the efficacy of long-term weekly prophylactic heme arginate (HA) infusions in reducing attack frequency and severity in female AIP patients. Methods: We report the results of five female AIP patients with frequent recurrent attacks (>9/year) before and after institution of weekly prophylaxis with heme arginate (3 mg/kg body weight). All five cases had confirmed disease-associated mutations in the porphobilinogen deaminase gene, and all had received genetic and clinical counseling about AIP. Results: In the five included patients, average annual attack rate (AAR) in the year prior to HA prophylaxis was 11.82 (range 9.03-17.06), and average total HA usage was 32.60 doses (range: 13.71-53.13). After 2.58-14.64 years of HA prophylaxis, average AAR was reduced to 2.23 (range 0.00-5.58), and attack severity (i.e., doses required per attack) was reduced from 2.81 to 1.39 doses/attack. Liver and renal function remained stable during weekly administration of HA prophylaxis. The most common complications were port-A catheter-related events. No other complications or safety concerns occurred with long-term use of HA prophylaxis. Conclusion: Our study demonstrated women with AIP receiving weekly prophylactic HA infusions resulted in fewer episodes that required acute HA treatment while maintaining stable renal and liver function. Weekly prophylactic HA infusions effectively prevent frequent porphyric attacks and reduce attack severity.
RESUMO
OBJECTIVE: To investigate the status of voice fatigue and throat disease in the secondary school teachers in Urumqi. METHODS: This study included 11 689 teachers from 109 secondary schools offered by Teaching and Researching Center of Urumqi as the second-stage random sampling. Among them, 3217 teachers were selected to carry out voice fatigue test and routine examination of throat. For those with abnormal routine inspection results, the stroboscopic laryngoscopy were added. RESULTS: The failure rate difference of 3217 secondary school teachers voice fatigue tests during different periods had statistical significance (F = 202.653, P < 0.05). The failure rate difference of voice fatigue test among different throat diseases groups had statistical significance (F = 9.516, P < 0.05). There was statistical significance in failure rates of the voice fatigue tests between 908 cases of disease group and 2309 cases of normal group among different genders and periods (P < 0.05). Female teachers had higher failure rates than male teachers in voice fatigue tests. High failure rates appeared when high volume required for male and female teachers in voice fatigue tests. There was statistical significance in mean value sound pressure level (MVSPL) and mean value fundamental frequency (MVF0) of the voice fatigue tests between different gender and periods (P < 0.05). Male teachers had higher MVSPL than female teachers, female teachers had higher MVF0 than male teachers. There was statistical significance in failure rates of the voice fatigue tests among teachers from different tribes in different periods (P < 0.05). The Uighur had the highest failure rate, while Han had the lowest. Make further pairwise comparisons, failure rates difference of the Han and the Uighur in the voice fatigue tests during different periods had statistical significance. Comparison among other tribes had no statistical significance (P > 0.05). CONCLUSIONS: Voice fatigue is common in secondary school teachers. Female teachers tend to get voice fatigue more easily, when required high volume, the voice fatigue is more serious. Uighurs teachers have highest degree of voice fatigue.
