The clinical characteristics, diagnosis and treatment process of a patient with type Ⅰ sialidosis (ST-1) caused by a homozygous mutation in the NEU1 gene who was missed diagnosis for 5 years were retrospectively analyzed to improve the understanding of the disease. A 16-year-old female patient presented with episodic limb shaking for more than 5 years and single generalised tonic-clonic seizure. Electroencephalogram (EEG) tests conducted at external hospital did not show any abnormalities, and head magnetic resonance imaging (MRI) showed general normality. Multiple antiepileptic drugs could not control the attack and the symptoms gradually worsened. After admission, the patient was found to have symptoms of easy wrestling and decreased vision, as well as signs of nystagmus and ataxia. The reexamination of the EEG showed extensive spike-and-slow complexes, and the brain MRI showed cerebellar atrophy. Furthermore, the whole-exome gene testing revealed the c.544A>G homozygous mutation in the NEU1 gene, leading to the diagnosis of ST-1. Levetiracetam tablets and clonazepam were given to improve the patient′s symptoms. During the follow-up, sleep improved compared to before, and myoclonus was significantly reduced. Therefore, patients with recurrent myoclonus, ataxia, and visual impairment without cognitive impairment should be aware of the possibility of sialidosis. Genetic testing plays an important role in the diagnosis of sialidosis.

The clinical characteristics, diagnosis and treatment process of a patient with type Ⅰ sialidosis (ST-1) caused by a homozygous mutation in the NEU1 gene who was missed diagnosis for 5 years were retrospectively analyzed to improve the understanding of the disease. A 16-year-old female patient presented with episodic limb shaking for more than 5 years and single generalised tonic-clonic seizure. Electroencephalogram (EEG) tests conducted at external hospital did not show any abnormalities, and head magnetic resonance imaging (MRI) showed general normality. Multiple antiepileptic drugs could not control the attack and the symptoms gradually worsened. After admission, the patient was found to have symptoms of easy wrestling and decreased vision, as well as signs of nystagmus and ataxia. The reexamination of the EEG showed extensive spike-and-slow complexes, and the brain MRI showed cerebellar atrophy. Furthermore, the whole-exome gene testing revealed the c.544A>G homozygous mutation in the NEU1 gene, leading to the diagnosis of ST-1. Levetiracetam tablets and clonazepam were given to improve the patient′s symptoms. During the follow-up, sleep improved compared to before, and myoclonus was significantly reduced. Therefore, patients with recurrent myoclonus, ataxia, and visual impairment without cognitive impairment should be aware of the possibility of sialidosis. Genetic testing plays an important role in the diagnosis of sialidosis.

OBJECTIVE: To explore the effectiveness of Nice knot technique for wound closure in Gustilo type ⅢA and ⅢB open tibial fractures. METHODS: A retrospective study was performed on 22 patients with Gustilo type ⅢA and ⅢB open tibial fractures, who underwent wound closure using the Nice knot technique and were admitted between June 2021 and June 2022. There were 15 males and 7 females. The age ranged from 18 to 67 years, with an average of 41.9 years. The causes of injury included traffic accident in 11 cases, falling from height in 7 cases, and heavy object injuries in 4 cases. Fractures were located on the left side in 9 cases and on the right side in 13 cases. And 9 cases were type ⅢA fractures and 13 were type ⅢB fractures according to Gustilo classification. All patients had extensive soft tissue injuries, and no vascular or neurological damage was observed. The time from injury to debridement was 3-8 hours (mean, 6.5 hours). The sizes of wounds before operation and at 2 weeks after operation were measured and wound healing rate at 2 weeks after operation were calculated. The wound healing time and wound healing grading were recorded. The Vancouver Scar Scale (VSS) score was used to assess the wound scar after wound healed and the excellent and good rate was calculated. RESULTS: The wound area was 21.0-180.0 cm ² (mean, 57.82 cm ²) before operation, and it was 1.2-27.0 cm ² (mean, 6.57 cm ²) at 2 weeks after operation. The wound healing rate at 2 weeks after operation was 76%-98% (mean, 88.6%). After operation, 2 cases needed to adjust Nice knot due to skin cutting and 1 case occurred soft tissue infection on the wound. The other patient's wounds healed. The average wound healing time was 27.8 days (range, 18-44 days). And the wound healing were grade A in 13 cases and grade B in 9 cases. VSS score was 2-9, with an average of 4.1; 10 cases were rated as excellent, 10 as good, and 2 as poor, with an excellent and good rate of 90.9%. All patients were followed up 9-24 months (mean, 14.6 months). During follow-up, no deep infection or osteomyelitis occurred. Two cases experienced fracture non-union, and were treated with compression fixation and bone grafting. The fractures of the other patients all healed, with a healing time of 85-190 days (mean, 148.2 days). CONCLUSION Nice knot technique can be used in wound closure of Gustilo type ⅢA and ⅢB open tibial fractures effectively, which is easy to operate.
Male ; Female ; Humans ; Adolescent ; Young Adult ; Adult ; Middle Aged ; Aged ; Cicatrix ; Retrospective Studies ; Treatment Outcome ; Tibial Fractures/surgery* ; Wound Healing ; Fracture Fixation, Internal/methods* ; Fractures, Open/surgery*

Objective: To analyze the characteristics of school injury among students aged 3 to 18 years in Yantian District of Shenzhen City, Guangdong Province, so as to provide the reference for developing the strategies for prevention and control of school injury. Methods: Data of the students aged 3 to 18 years who were initially diagnosed as injury in sentinel hospitals and whose injuries occurred in nurseries, primary or middle schools in Yantian District in 2023, were collected from the Shenzhen Injury Surveillance System. The onset time, places, activities, characteristics and sites of injury were descriptively analyzed. Results: A total of 1 681 cases of school injuries among students aged 3 to 18 years were reported in Yantian District in 2023, including 1 182 boys and 499 girls, with a boy-to-girl ratio of 2.37∶1. There were 206 preschool children (12.25%), 856 primary school students (50.92%), 358 junior high school students (21.30%) and 261 high school students (15.53%). The peak months for school injuries were February to June, accounting for 49.97%; the peak time period was from 15: 00 to 18: 59, accounting for 44.68%. The main causes of injuries included falls (41.94%) and blunt injury (33.85%). The activities at the time of injury mainly included leisure activities (57.70%) and physical activities (21.83%). Contusion/abrasion was the main characteristics (49.20%). Mild injury was predominant, accounting for 74.60%, and there was no fatal case. The top three injury sites were the head and neck, upper limbs and lower limbs, accounting for 36.94%, 27.54%, and 24.33%, respectively. Boys had higher proportions of blunt injuries and contusion/abrasion (AR=4.8 and 4.0). The proportion of sports injuries, sprains/strains and lower limb injuries increased with grade (all P<0.05). Conclusions School injury among students predominantly occur in spring when having leisure or physical activities in Yantian District. The main causes of injuries are falls and blunt injury, with boys and primary school students being the high-risk groups.

Objective: To construct and implement a calculation model of clinical nursing post classification. Methods: Between October to December 2018, head nurses of the hospital were screened as consultants, and Delphi method was used to determine the indicators, while the weights were assigned from the aspects of nursing workload, work quality, patient satisfaction and number of nursing night shifts. Combined with HIS data, the calculation model of clinical nursing post classification was constructed to classify clinical nursing units into different categories and levels. Results: After two rounds of expert enquiry, 82 nursing work items were identified and the objective weight assignment was determined ranging from 0.80 to16.14. According to the established calculation model and HIS data, nursing posts in clinical departments were classified into 6 levels and 3 categories, and the accurate management of clinical nursing post classification was achieved. Conclusions The construction of a calculation model is scientific and rigorous, which provides a scientific basis for dynamic nurse performance management and rational allocation of human resources. In addition, it provides a useful reference for accurate nursing management.

The influences of lactic/hydrochloric acidosis on pulmonary vasoconstriction and gas exchanges were investigated in 36 dogs. The tv infusion of lactic or hydrochloric acid reducing arterial pH to 7. 00 could elicit significant changes of pulmonary hemodynamics, including the consistent increase of pulmonary arterial pressure and the decrease of arterial oxygen saturation. It is suggested that pulmonary vasocostriction is related to arterial PH but not to arterial lactic acid levels.