Objective To deepen the clinicians' impression on the Laurance-Moon-Bardet-Biedl syndrome(LMBBS),we made a summary in the incidence and clinical manifestations of the disease in China comparing with foreigners.Methods We made evidence-based meta-analysis about the 2 cases reports in 1987 and 2003 in our hospital and literature review of 462 cases in foreign countries and 94 cases in China.Results LMBBS patients,with more frequency of consanguinity and family history,retinal dystrophy,mental retardation,obesity,polydactyly,hypogenitalism,often had many complicated and variable clinical manifestations.Conclusion To avoid intrafamiliy marriage would reduce the incidence rate.The diagnostic criteria and ascertainment methods introduced recently do benefit the early diagnosis in their childhood period.

AIM: To investigate the effect of 17? estradiol (17?E 2)on atherosclerosis and hemorrheology in ovariectomized (OVX) rabbits. METHODS: Thirty four female rabbits were divided into 4 groups, group A: normal control; group B: sham+CHO; group C: OVX+CHO; group D: OVX+CHO+17? E 2. Cholesterol(CHO) was fed to rabbits for 12 weeks, before and after feeding CHO, the serum lipid (TC, TG, HDL-C, LDL-C, ApoA1, ApoB) and area of aortic atherosclerotic plaques were measured. Blood viscosity, plasma viscosity (?p), aggregation index of RBC (AIRC) and fibrinogen were also determined, respectively. RESULTS: ① The ratio of area of aortic atherosclerotic plaque to total area of aortic intima was 0.02?0.003 (in group A), 0.42?0.15 (group B), 0.67?0.23 (group C), and 0.12?0 11 (group D), respectively. In group D, the ratio of aortic atherosclerotic plaque was markedly decreased compared with group C ( P

Objective: To investigate the etiology and clinical characteristics of vocal fold paralysis in children. To provide useful information for diagnosis, management and prognosis in the clinical work. Methods: Two hundred and seven children with vocal fold paralysis in Children′s Hospital of Fudan University were retrospectively studied, and followed-up. Results: All the patients had hoarseness.151 cases had vocal paralysis in the left side and the main etiology was pulmonary arterial hypertension.43 cases had bilateral vocal paralysis and all of them had respiratory problems.The main etiology were congenital tracheoesophageal malformations.13 cases had vocal paralysis in the right side.In terms of etiology, 8 cases were related to intracranial lesions, 2 cases were idiopathic. Conclusions The main etiologies of left vocal fold paralysis were cardiovascular diseases, and bilateral vocal paralysis were congenital tracheoesophageal malformations.The main etiologies of right vocal fold paralysis were neoplastic and central lesion.The prognosis of bilateral vocal fold paralysis and right vocal fold paralysis was poor.

Objective:To evaluate the impact of vocal fold nodules on the quality of life of children, and to compare the efficacy between conservative management（voice hygiene education, etc.） and laryngeal microsurgery. Methods:A retrospective study was performed on 102 children with vocal fold nodules, who received treatment in Children's Hospital of Fudan University during the period from January 2020 to December 2022. According to the regime, the patients were divided into conservative management group and surgical treatment group; Cases are divided into pre-school age group （2-5 years old） and school-age group （6-12 years old） based on age. The pediatric voice-related quality of life （pVRQOL） was used to evaluate the impact of vocal fold nodules on the quality of life of children and differences in efficacy between two treatment regimens. Results:The pVRQOL scores of vocal fold nodule grades 1, 2, and 3 were 91.58±8.17, 78.87±12.49, 72.50±12.08, respectively. There were statistical differences between grade 1 and grade 2, and between grade 2 and grade 3. There were statistical differences between grade 1 grade 2, grade 2 and grade 3 （P<0.001）, suggesting that the higher the rating of vocal cord nodules, the lower the pVRQOL score of patients was. The pVRQOL score of the school-age group was 69.83±11.11, which is lower than that of the preschool group（87.59±8.63）, and the difference was statistically significant （P=0.042）. Vocal fold nodules had a significant impact on the pVRQOL score of school-age children. In the conservative management group, the pVRQOL scores before and after treatment were 83.99±12.66 and 87.26±9.58, respectively, and there was significant difference between the two groups（P=0.046）. Indirect voice therapy such as voice hygiene education could improve children's pVRQOL scores and was more effective for school-age children（P<0.001）. The microsurgical treatment had a more significant improvement in the pVRQOL score of children. Conclusion:The higher the rating of vocal fold nodules, the greater the impact on the quality of life of patients. Vocal fold nodules had a greater impact on the quality of life of school-age children than that of preschool children. Voice hygiene education could improve the quality of life of children, which was more evident in the school-age group. Compared with voice hygiene therapy, laryngeal microsurgery had a more significant effect on improving the pVRQOL score of patients. It is recommended to undergo laryngeal microsurgery for the treatment of vocal fold nodules for the requirement of improving the quality of life related to voice. During the 6-month follow-up period after surgery in this study, the short-term efficacy of laryngeal microsurgery in treating vocal cord nodules in children is clear, but the long-term efficacy needs further research.
Child ; Humans ; Child, Preschool ; Vocal Cords/pathology* ; Quality of Life ; Retrospective Studies ; Voice Quality ; Laryngeal Diseases/surgery* ; Polyps/pathology*

Laryngeal cyst is a cystic lesion occurring in the laryngeal cavity. Large laryngeal cyst in infants and young children can cause laryngeal wheezing and other upper airway obstruction symptoms. In severe cases, it can be even life-threatening and requires timely surgical treatment. Currently, there is a lack of unified clinical treatment strategy for this disease.This article summarizes the surgical methods, the advantages and disadvantages of various surgical methods for laryngeal cysts in recent years. It is recommended that needle aspiration, partial cyst wall resection, radical cyst dissection, transoral robotic surgery or external approach cyst resection should be selected through full communication and evaluation to clarify the extent of the lesion scope and the advantages and disadvantages of surgery.
Infant ; Child ; Humans ; Child, Preschool ; Cysts/diagnosis* ; Laryngeal Diseases/diagnosis* ; Larynx/surgery* ; Robotic Surgical Procedures ; Biopsy, Needle