This article reports a rare case of pantothenate kinase-associated neurodegeneration: the patient′s initial symptoms were Parkinson-like symptoms such as tremor and bradykinesia, as well as dysarthria. Brain magnetic resonance imaging showed abnormal signals in the bilateral basal ganglia region similar to the tiger′s eye sign. Two heterozygous mutations including c.803A>G(p.Asp268Gly) and c.1021C>T(p.Arg341 *) were found in the pantothenate kinase 2 gene by whole exome sequencing, and the final diagnosis was neurodegeneration with brain iron accumulation type 1, also known as pantothenate kinase-associated neurodegeneration. By reviewing the clinical characteristics, diagnostic approaches and therapeutic strategies, this article aims to enhance the recognition of this disease, avoid misdiagnosis and missed diagnosis, and provide guidance for clinical diagnosis and treatment.

Objective:To evaluate and summarize the best evidence on nutritional management of children with inflammatory bowel disease (IBD) both domestically and internationally, so as to provide reference for clinical nursing practice.Methods:According to the "6S" evidence model, the literature on nutritional management of children with IBD was systematically searched in national and international computerized decision support systems, guideline websites, relevant professional association websites, and databases. The search period was from January 2014 to July 2024. The quality of the included literature was evaluated and the evidence was extracted, evaluated and integrated to form the best evidence.Results:A total of 15 papers were included, including three clinical decisions, five guidelines, two expert consensus, two position papers, and three systematic reviews. Thirty-four pieces of best evidence were extracted, focusing on six aspects of multidisciplinary team formation, nutritional assessment and monitoring, diet and nutrient management, nutritional support methods, exercise and obesity management, and follow-up and health education.Conclusions:This study summarizes the best evidence for nutritional management of children with IBD, which may provide a basis for clinical implementation of nutritional interventions. It is recommended that healthcare professionals screen for the best evidence, translate the evidence, and develop individualized nutritional management programs, taking into account the clinical context, professional opinion, and patient wishes.

Objective:To evaluate and summarize the best evidence on nutritional management of children with inflammatory bowel disease (IBD) both domestically and internationally, so as to provide reference for clinical nursing practice.Methods:According to the "6S" evidence model, the literature on nutritional management of children with IBD was systematically searched in national and international computerized decision support systems, guideline websites, relevant professional association websites, and databases. The search period was from January 2014 to July 2024. The quality of the included literature was evaluated and the evidence was extracted, evaluated and integrated to form the best evidence.Results:A total of 15 papers were included, including three clinical decisions, five guidelines, two expert consensus, two position papers, and three systematic reviews. Thirty-four pieces of best evidence were extracted, focusing on six aspects of multidisciplinary team formation, nutritional assessment and monitoring, diet and nutrient management, nutritional support methods, exercise and obesity management, and follow-up and health education.Conclusions:This study summarizes the best evidence for nutritional management of children with IBD, which may provide a basis for clinical implementation of nutritional interventions. It is recommended that healthcare professionals screen for the best evidence, translate the evidence, and develop individualized nutritional management programs, taking into account the clinical context, professional opinion, and patient wishes.

This article reports a rare case of pantothenate kinase-associated neurodegeneration: the patient′s initial symptoms were Parkinson-like symptoms such as tremor and bradykinesia, as well as dysarthria. Brain magnetic resonance imaging showed abnormal signals in the bilateral basal ganglia region similar to the tiger′s eye sign. Two heterozygous mutations including c.803A>G(p.Asp268Gly) and c.1021C>T(p.Arg341 *) were found in the pantothenate kinase 2 gene by whole exome sequencing, and the final diagnosis was neurodegeneration with brain iron accumulation type 1, also known as pantothenate kinase-associated neurodegeneration. By reviewing the clinical characteristics, diagnostic approaches and therapeutic strategies, this article aims to enhance the recognition of this disease, avoid misdiagnosis and missed diagnosis, and provide guidance for clinical diagnosis and treatment.

Objective:To explore the main hindering factors of nutritional care for family caregivers of patients with digestive system neoplasms after surgery, so as to provide reference for nursing workers to improve the quality of nutritional care for family caregivers and formulate personalized intervention programs.Methods:From January to April 2021, the phenomenological research method was used to select 14 family caregivers of patients with digestive system neoplasms after surgery in the First Affiliated Hospital of Anhui Medical University for semi-structured interviews, and Colaizzi 7-step analysis method was used to analyze and collate the data.Results:The obstacles to nutritional care for family caregivers of patients with digestive system neoplasms after surgery extracted 4 themes and 9 sub-themes. The first theme was insufficient awareness of nutritional care, including caregivers' insufficient awareness of the importance of nutritional care and lack of awareness of nutritional risks related to assisted eating. The second theme was the lack of nutritional care ability, including lack of nutrition knowledge of caregivers, poor practical ability of caregivers and poor compliance and initiative of caregivers. The third theme was the difficulty in obtaining and using nutritional information, including limited access to information for caregivers and difficulties in using information for caregivers. The fourth theme was caregiver role barriers in nutritional care, including lack of social support and heavy family financial burden.Conclusions:Nursing workers can formulate individualized interventions for family caregivers' nutritional care barriers, promote the improvement of nutritional care quality of them and then improve patients' nutritional status and quality of life.

Objective:To explore the main hindering factors of nutritional care for family caregivers of patients with digestive system neoplasms after surgery, so as to provide reference for nursing workers to improve the quality of nutritional care for family caregivers and formulate personalized intervention programs.Methods:From January to April 2021, the phenomenological research method was used to select 14 family caregivers of patients with digestive system neoplasms after surgery in the First Affiliated Hospital of Anhui Medical University for semi-structured interviews, and Colaizzi 7-step analysis method was used to analyze and collate the data.Results:The obstacles to nutritional care for family caregivers of patients with digestive system neoplasms after surgery extracted 4 themes and 9 sub-themes. The first theme was insufficient awareness of nutritional care, including caregivers' insufficient awareness of the importance of nutritional care and lack of awareness of nutritional risks related to assisted eating. The second theme was the lack of nutritional care ability, including lack of nutrition knowledge of caregivers, poor practical ability of caregivers and poor compliance and initiative of caregivers. The third theme was the difficulty in obtaining and using nutritional information, including limited access to information for caregivers and difficulties in using information for caregivers. The fourth theme was caregiver role barriers in nutritional care, including lack of social support and heavy family financial burden.Conclusions:Nursing workers can formulate individualized interventions for family caregivers' nutritional care barriers, promote the improvement of nutritional care quality of them and then improve patients' nutritional status and quality of life.

Desensitization and acute tolerance are terms used to describe the attenuation of receptor responsiveness by prolonged or intermittent exposure to an agonist. Unlike desensitization of G protein-coupled receptors (GPCRs), which is commonly explained by steric hindrance caused by the β-arrestins that are translocated to the activated receptors, molecular mechanisms involved in the acute tolerance of GPCRs remain unclear. Our studies with several GPCRs and related mutants showed that the acute tolerance of GPCRs could occur independently of agonist-induced β-arrestin translocation. A series of co-immunoprecipitation experiments revealed a correlation between receptor tolerance and interactions among receptors, β-arrestin2, and Gβγ. Gβγ displayed a stable interaction with receptors and β-arrestin2 in cells expressing GPCRs that were prone to undergo tolerance compared to the GPCRs that were resistant to acute tolerance. Strengthening the interaction between Gβγ and β-arrestin rendered the GPCRs to acquire the tendency of acute tolerance. Overall, stable interaction between the receptor and Gβγ complex is required for the formation of a complex with β-arrestin, and determines the potential of a particular GPCR to undergo acute tolerance. Rather than turning off the signal, β-arrestins seem to contribute on continuous signaling when they are in the context of complex with receptor and Gβγ.
Immunoprecipitation ; Receptors, Dopamine D3

Objective To evaluate the outcomes and complications of permanent brachytherapy combined with external beam radiotherapy and hormonal therapy for local high-risk or intermediated-risk prostate cancer.Methods There were 354 men with local high-risk or intermediated-risk prostate cancer were reviewed,including 111 men with local intermediated-risk prostate cancer and 243 men with local highrisk prostate cancer.The age of the patients were 48 to 84 years old (mean age 72.4 years old).The preoperative PSA levels were in a range of 3.8 to 99.8ng/ml (mean 29.6 g/ml) and the preoperative Gleason scores were 4 to 9 (mean 6.8).The prostate volume were 13.7 to 65.0 ml (mean 30.5 ml).All the patients were treated with brachytherapy combined with hormonal therapy,including 69 patients received additional external beam radiotherapy.All patients were followed up for biochemical progression-free survival (bPFS),distant disease free survival (DDFS),overall survival (OS),cause-specific survival (CSS) rate and complications.Results Among 354 cases,174 cases underwent brachytherapy after the diagnosis of prostate cancer,and 157 cases underwent brachytherapy after maximal androgen blockade (MAB) treatmentfor 3 months,while the other 23 patients with large prostate underwent brachytherapy after MAB treatment for 6 months.All 354 cases were treated with MAB after brachytherapy.One hundred and eleven cases in intermediated-risk group were treated with MAB for 6 months and 243 cases in high-risk group were treated with MAB for 6 months to 3 years.Another 69 patients received adjuvant external radiotherapy.All cases were followed up for 9 to 128 months (mean 91 months),including 135 cases having biochemical recurrence,and 63 cases having distant metastasis.There were 81 cases died,including 24 cases died of prostate cancer.The overall bPFS,DDFS,OS and CSS were 61.9％,82.2％,77.1％ and 93.2％ respectively.There were significant difference in the survival rate between the high-risk group and the intermediated-risk group(P ＜ 0.001).The incidence of urinary retention and long term urethral stricture were 6.8％ and 1.7％,respectively.No serious complications occurred.Conclusion Permanent brachytherapy combined with external beam radiotherapy and hormonal therapy treating local high-risk or intermediated-risk prostate cancer can be effective with few complications.

Humans ; Lymphoma, Non-Hodgkin ; Pemphigus