Summary： The ATP2C1 gene mutation in one ease of familial benign chronic pemphigus was investigated.One patient was diagnosed as familial benign chronic pemphigus by pathology, ultrastructral examination and clinical...Summary： The ATP2C1 gene mutation in one ease of familial benign chronic pemphigus was investigated.One patient was diagnosed as familial benign chronic pemphigus by pathology, ultrastructral examination and clinical features. Genomic DNA was extracted from blood samples. Mutation of ATP2CI gene was detected by polymerase chain reaction （PCR） and DNA sequencing. The results showed that deletion mutation was detected in ATP2C1 gene in this patient, which was 2374delTTTG. No mutation was found in the family members and normal individuals. It was coneluded that the 2374delTTTG mutation in ATP2C1 gene was the specific mutation for the clinical phenotype for this patient and was a de novo mutation.展开更多
Pseudopemphigoid can cause a chronic cicatricial conjunctivitis that is clinically identical to the manifestations seen in mucous membrane pemphigoid, a disorder with a common clinical phenotype and multiple autoimmun...Pseudopemphigoid can cause a chronic cicatricial conjunctivitis that is clinically identical to the manifestations seen in mucous membrane pemphigoid, a disorder with a common clinical phenotype and multiple autoimmune links. For the purpose of this review, we will describe pseudopemphigoid as caused by topical drugs, the most common etiology with ocular manifestations, and as caused by the pemphigus disease, a more rare etiology. Specifically, we will discuss the ophthalmological features of drug-induced cicatricial conjunctivitis, pemphigus vulgaris, and paraneoplastic pemphigus. Other etiologies of pseudopemphigoid exist that will not be described in this review including autoimmune or inflammatory conditions such as lichen planus, sarcoidosis, granulomatosis with polyangiitis(Wegener's granulomatosis), erythema multiforme(minor, major, and Stevens-Johnson syndrome), bullous pemphigoid, skin-dominated linear IgA bullous dermatosis, and skin-dominated epidermolysis bullosa acquisita. Prompt diagnosis of the underlying etiology in pseudopemphigoid is paramount to the patient's outcome as certain diseases are associated with a more severe clinical course, increased ocular involvement, and differential response to treatment. A completehistory and ocular examination may find early cicatricial changes in the conjunctiva that are important to note and evaluate to avoid progression to more severe disease manifestations. When such cicatricial changes are noted, proper diagnostic techniques are needed to help elucidate a diagnosis. Lastly, collaboration between ophthalmologists and subspecialists such as dermatologists, pathologists, immunologists, and others involved in the care of the patient is needed to ensure optimal management of disease.展开更多
BACKGROUND Bone cement implantation syndrome(BCIS)is characterized by hypotension,arrhythmia,diffuse pulmonary microvascular embolism,shock,cardiac arrest,any combination of these factors,or even death following bone ...BACKGROUND Bone cement implantation syndrome(BCIS)is characterized by hypotension,arrhythmia,diffuse pulmonary microvascular embolism,shock,cardiac arrest,any combination of these factors,or even death following bone cement implantation.CASE SUMMARY An 80-year-old patient with pemphigus and Parkinson’s disease underwent total hip replacement under spinal subarachnoid block and developed acute pulmonary embolism after bone cement implantation.The patient received mask mechanical ventilation with a continuous intravenous infusion of adrenaline(2μg/mL)at a rate of 30 mL/h.Subsequently,the symptoms of BCIS were markedly alleviated,and the infusion rate of adrenaline was gradually reduced until the infusion was completely stopped 45 min later.The patient was then transferred to the Department of Orthopedics,and anticoagulation therapy began at 12 h postoperatively.No other complications were observed.CONCLUSION This is a rare case of BCIS in a high-risk patient with pemphigus and Parkinson’s disease.展开更多
Introduction: This study aimed to evaluate the effects of an educational program based on Health Belief Model on knowledge, attitudes and practice (KAP) in patients with Pemphigus vulgaris (PV) referred to Razi Hospit...Introduction: This study aimed to evaluate the effects of an educational program based on Health Belief Model on knowledge, attitudes and practice (KAP) in patients with Pemphigus vulgaris (PV) referred to Razi Hospital in Iran. Materials and Methods: This quasi-experimental study was conducted on 88 patients in 2013-2014. Subjects were divided into two intervention and control groups using block randomization. In addition to their usual care for both groups, the intervention group sat through a six-month self-care educational program in a specialized outpatient clinic. A self-designed questionnaire was used to gather information about demographic characteristics, PV related variables, and KAP-related questions. Data were analyzed using SPSS Software. p value of less than 0.05 was set as statistically significant. Results: mean scores of KAP increased significantly after intervention compared to control groups when adjusted for baseline differences of these scores and house ownership and employment status differences in two groups. Conclusion: study results show the effectiveness of an HBM based educational program on KAP in PV patients that can lead to adoption of self care behaviors and help them gain self efficacy in controlling their disease and assisting their treatment process, counting as a tertiary preventive measure.展开更多
BACKGROUD Follicular dendritic cell(FDC)sarcomas are rare neoplasms that occur predominantly in the lymph nodes.They can also occur extranodally.Extranodal FDC sarcomas most commonly present as solitary masses.Inflamm...BACKGROUD Follicular dendritic cell(FDC)sarcomas are rare neoplasms that occur predominantly in the lymph nodes.They can also occur extranodally.Extranodal FDC sarcomas most commonly present as solitary masses.Inflammatory pseudotumor(IPT)-like FDC sarcomas,a subcategory of FDC sarcomas,are rarer than other sarcoma subtypes.They are composed of spindle or ovoid neoplastic cells and exhibit an admixture of plasma cells and prominent lymphoplasmacytic infiltration.Paraneoplastic pemphigus(PNP),also known as paraneoplastic autoimmune multiorgan syndrome,is a rare autoimmune bullous disease that is associated with underlying neoplasms.PNP has a high mortality,and its early diagnosis is usually difficult.CASE SUMMARY We describe a 27-year-old woman who presented with stomatitis,conjunctivitis,and skin blisters and erosions as her first symptoms of PNP with an intraabdominal IPT-like FDC sarcoma.The patient underwent surgical tumor resection and received tapering oral corticosteroid treatment.She showed no recurrence at the 1-year follow-up.CONCLUSION IPT-like FDC sarcomas are rare underlying neoplasms that have an uncommon association with PNP.PNP-associated FDC sarcomas predominantly occur in intra-abdominal sites and suggest a poor prognosis.Surgical resection is an essential and effective treatment for PNP and primary and recurrent FDC sarcomas.展开更多
We describe the clinical, macroscopic, cytological, histopathological, immunohistochemical, serodiagnostic and aspects of pemphigus vulgaris (PV) in the oral gingiva that clinically mimicked oral squamous cell carcino...We describe the clinical, macroscopic, cytological, histopathological, immunohistochemical, serodiagnostic and aspects of pemphigus vulgaris (PV) in the oral gingiva that clinically mimicked oral squamous cell carcinoma (OSCC) in a 57-year-old Japanese man. He developed slight haphalgesia of the buccal gingiva around teeth numbers 18 and 19 2 years ago. A dentist diagnosed intractable ulcer, but the patient ignored the condition for about 2 years until a sharp pain in the gingiva worsened. He consulted an otolaryngologist, who referred the patient to our hospital under a cytological diagnosis of OSCC. An oral examination revealed several extensive painful erosions/ ulcers from the buccal and lingual gingiva around teeth numbers 18 to 21 to the distal alveolar mucosa of no. 18 and the buccal and lingual gingiva around tooth number 31. A presumptive diagnosis of PV with dysplastic changes was determined from cytological smears. The cytological Nikolsky test was positive. The diagnosis of PV was confirmed from clinical and histopathological findings of a biopsy specimen obtained from the perilesional site. Although the definitive diagnosis of PV required only 2 weeks after this patient presented at our hospital, 2 years had elapsed since the onset of oral lesions.展开更多
Pemphigus of Hallopeau (PH) is a mild and pustular form of pemphigus vegetans, termed initially as “pyodermite végétante” described by Hallopeau. We report cases in two black women. Two women aged 32 and 3...Pemphigus of Hallopeau (PH) is a mild and pustular form of pemphigus vegetans, termed initially as “pyodermite végétante” described by Hallopeau. We report cases in two black women. Two women aged 32 and 36 respectively (case 1 and case 2) were seen for a pustulosis associated with blisters and erosions. Their history and their review have found the mouth ulcerations. The clinical examination showed pustules on the cephalic end, the trunk, the limbs and the large folds. Lesions had a polycyclic aspect and spread in a centrifugal way, developing towards budding, squamous and scabby erosions quickly surrounded by new pustules. Regression left a pigmented macula. Histological examination of the skin lesion showed images similar to vulgaris pemphigus (PV), together with hyperacanthosis and papillomatosis. Aspects observed in immunofluorescence were identical to those of PV (case1). General corticoid therapy led to a rapid disappearance of lesions in Case 1 but in Case 2 the Lever protocol combining corticoid and methotrexate have been used. PH is rarely described in our regions. Clinical diagnosis is not obvious for the uninitiated. Beyond the clinical presentation, PH has the same paraclinical diagnosis and the same treatment as PV.展开更多
Objective:To observe the effects of the recombinant chimeric toxin Dsg3EC 1-2PE40 on T and B lymphocytes isolated from Pemphigus Vulgaris (PV) patients to further study its biological therapeutic function for PV. Me...Objective:To observe the effects of the recombinant chimeric toxin Dsg3EC 1-2PE40 on T and B lymphocytes isolated from Pemphigus Vulgaris (PV) patients to further study its biological therapeutic function for PV. Methods:Recombinant chimeric toxin Dsg3EC 1-2PE40 was first identified, expressed and purified, and then its effects on T and B lymphocytes of PV patients in vitro were detected and quantified by ELISPOT assay and MTT assay.Results:The purity of the expressed protein Dsg3EC 1-2PE40 was up to 80%. In ELISPOT assay, with Dsg3EC 1-2PE40, the overall number of B cells that produce anti-Dsg3 antibodies among PV patients was only about 60% of the comparable number with Dsg3EC 1-2. The proliferation of T cells of PV patients was inhibited markedly by Dsg3EC 1-2PE40. There was significant difference between the different groups with Dsg3EC 1-2PE40 and Dsg3EC 1-2.Conclusion:The recombinant chimeric toxin Dsg3EC 1-2PE40 decrease the number of B cells that produce anti-Dsg3 antibodies in PV patients and can inhibit or kill T cells of PV patients in vitro.展开更多
Objective To investigate the relationship between pemphigus vulgaris (PV) and human leukocyte antigen (HLA) in Han nation of northeast China. Methods Standard microcytotoxicity test and polymerase chain reaction-seque...Objective To investigate the relationship between pemphigus vulgaris (PV) and human leukocyte antigen (HLA) in Han nation of northeast China. Methods Standard microcytotoxicity test and polymerase chain reaction-sequence specific primers method were used to detect the HLA classⅠ antigens and HLA-DRB1 and DQB1 alleles in 27 patients with PV and results were compared with control group. Results Gene and phenotype frequencies of HLA-A3, A26(10), B60(40), and B13 (27.99%, 48%; 16.11%, 30%; 23.02%, 41%; 16.11%, 30%, respectively) increased significantly in PV group compared with control (1.01%, 2%; 0.5%, 1%; 4.61%, 9%; 5.13%, 10%, respectively). After P value correction, the difference of A3, A26 (10), and B60 (40) between the two groups was still significant. The gene frequencies of HLA-DRB1*140x (1401, 1404, 1405, 1407, 1408), DRB1*120x, and DQB1*0503 alleles in PV group (42.26%, 25.46%, and 23.02%) were significantly higher than control group (5.09%, 7.74%, and 1.89%). After P value correction, the difference was still significant between the two groups. Conclusion PV significantly relates with HLA in PV patients of Han nation of northeast China.展开更多
An EMS(ethy methanesulfonate)-induced lethal etiolated(le)mutant obtained from the rice variety Zhongjian 100 was characterized by lethal etiolated phenotypes,with significantly reduced levels of chlorophyll a,chlorop...An EMS(ethy methanesulfonate)-induced lethal etiolated(le)mutant obtained from the rice variety Zhongjian 100 was characterized by lethal etiolated phenotypes,with significantly reduced levels of chlorophyll a,chlorophyll b,total chlorophyll,and carotenoids.Additionally,the mutant displayed a significantly decreased number of chloroplast grana,along with irregular and less-stacked grana lamellae.The le mutant showed markedly diminished root length,root surface area,and root volume compared with the wild type.It also exhibited significantly lower catalase activity and total protein content,while peroxidase activity was significantly higher.Using the map-based cloning method,we successfully mapped the LE gene to a 48-kb interval between markers RM16107 and RM16110 on rice chromosome 3.A mutation(from T to C)was identified at nucleotide position 692 bp of LOC_Os03g59640(ChlD),resulting in a change from leucine to proline.By crossing HM133(a pale green mutant with a single-base substitution of A for G in exon 10 of ChlD subunit)with a heterozygous line of le(LEle),we obtained two plant lines heterozygous at both the LE and HM133 loci.Among 15 transgenic plants,3 complementation lines displayed normal leaf color with significantly higher total chlorophyll,chlorophyll a,and chlorophyll b contents.The mutation in le led to a lethal etiolated phenotype,which has not been observed in other ChlD mutants.The mutation in the AAA+domain of ChlD disrupted the interaction of ChlDle with ChlI as demonstrated by a yeast two-hybrid assay,leading to the loss of ChlD function and hindering chlorophyll synthesis and chloroplast development.Consequently,this disruption is responsible for the lethal etiolated phenotype in the mutant.展开更多
目的探讨一病一品高质量护理对天疱疮患者生活质量及护理满意度的影响。方法方便选取2022年3—12月遵义市第一人民医院收治的76例天疱疮患者为研究对象,按随机数表法分为两组,每组38例。对照组给予常规护理,观察组给予一病一品高质量护...目的探讨一病一品高质量护理对天疱疮患者生活质量及护理满意度的影响。方法方便选取2022年3—12月遵义市第一人民医院收治的76例天疱疮患者为研究对象,按随机数表法分为两组,每组38例。对照组给予常规护理,观察组给予一病一品高质量护理。比较两组患者生活质量[简明生活质量量表(Short Form 36 Questionnaire,SF-36)]评分及护理满意度。结果干预后,观察组SF-36评分高于对照组,差异有统计学意义(P<0.05);观察组护理总满意度(94.74%)高于对照组(78.95%),差异有统计学意义(χ^(2)=4.145,P<0.05)。结论一病一品高质量护理应用于天疱疮患者中可取得显著效果,提高患者生活质量,且患者对该护理方式的认可度较高。展开更多
文摘Summary： The ATP2C1 gene mutation in one ease of familial benign chronic pemphigus was investigated.One patient was diagnosed as familial benign chronic pemphigus by pathology, ultrastructral examination and clinical features. Genomic DNA was extracted from blood samples. Mutation of ATP2CI gene was detected by polymerase chain reaction （PCR） and DNA sequencing. The results showed that deletion mutation was detected in ATP2C1 gene in this patient, which was 2374delTTTG. No mutation was found in the family members and normal individuals. It was coneluded that the 2374delTTTG mutation in ATP2C1 gene was the specific mutation for the clinical phenotype for this patient and was a de novo mutation.
基金The Department of Veterans Affairs,Veterans Health Administration,Office of Research and Development,Clinical Sciences Research and Development’s Career Development Award,No.CDA-2-024-10S(Dr.Galor)NIH Center Core Grant,No.P30EY014801,Research to Prevent Blindness Unrestricted Grant,Department of Defense,Nos.W81XWH-09-1-0675 and W81XWH-13-1-0048 ONOVAThe Ronald and Alicia Lepke Grant,The Lee and Claire Hager Grant,The Jimmy and Gaye Bryan Grant,and the Richard Azar Family Grant(institutional grants)
文摘Pseudopemphigoid can cause a chronic cicatricial conjunctivitis that is clinically identical to the manifestations seen in mucous membrane pemphigoid, a disorder with a common clinical phenotype and multiple autoimmune links. For the purpose of this review, we will describe pseudopemphigoid as caused by topical drugs, the most common etiology with ocular manifestations, and as caused by the pemphigus disease, a more rare etiology. Specifically, we will discuss the ophthalmological features of drug-induced cicatricial conjunctivitis, pemphigus vulgaris, and paraneoplastic pemphigus. Other etiologies of pseudopemphigoid exist that will not be described in this review including autoimmune or inflammatory conditions such as lichen planus, sarcoidosis, granulomatosis with polyangiitis(Wegener's granulomatosis), erythema multiforme(minor, major, and Stevens-Johnson syndrome), bullous pemphigoid, skin-dominated linear IgA bullous dermatosis, and skin-dominated epidermolysis bullosa acquisita. Prompt diagnosis of the underlying etiology in pseudopemphigoid is paramount to the patient's outcome as certain diseases are associated with a more severe clinical course, increased ocular involvement, and differential response to treatment. A completehistory and ocular examination may find early cicatricial changes in the conjunctiva that are important to note and evaluate to avoid progression to more severe disease manifestations. When such cicatricial changes are noted, proper diagnostic techniques are needed to help elucidate a diagnosis. Lastly, collaboration between ophthalmologists and subspecialists such as dermatologists, pathologists, immunologists, and others involved in the care of the patient is needed to ensure optimal management of disease.
文摘BACKGROUND Bone cement implantation syndrome(BCIS)is characterized by hypotension,arrhythmia,diffuse pulmonary microvascular embolism,shock,cardiac arrest,any combination of these factors,or even death following bone cement implantation.CASE SUMMARY An 80-year-old patient with pemphigus and Parkinson’s disease underwent total hip replacement under spinal subarachnoid block and developed acute pulmonary embolism after bone cement implantation.The patient received mask mechanical ventilation with a continuous intravenous infusion of adrenaline(2μg/mL)at a rate of 30 mL/h.Subsequently,the symptoms of BCIS were markedly alleviated,and the infusion rate of adrenaline was gradually reduced until the infusion was completely stopped 45 min later.The patient was then transferred to the Department of Orthopedics,and anticoagulation therapy began at 12 h postoperatively.No other complications were observed.CONCLUSION This is a rare case of BCIS in a high-risk patient with pemphigus and Parkinson’s disease.
文摘Introduction: This study aimed to evaluate the effects of an educational program based on Health Belief Model on knowledge, attitudes and practice (KAP) in patients with Pemphigus vulgaris (PV) referred to Razi Hospital in Iran. Materials and Methods: This quasi-experimental study was conducted on 88 patients in 2013-2014. Subjects were divided into two intervention and control groups using block randomization. In addition to their usual care for both groups, the intervention group sat through a six-month self-care educational program in a specialized outpatient clinic. A self-designed questionnaire was used to gather information about demographic characteristics, PV related variables, and KAP-related questions. Data were analyzed using SPSS Software. p value of less than 0.05 was set as statistically significant. Results: mean scores of KAP increased significantly after intervention compared to control groups when adjusted for baseline differences of these scores and house ownership and employment status differences in two groups. Conclusion: study results show the effectiveness of an HBM based educational program on KAP in PV patients that can lead to adoption of self care behaviors and help them gain self efficacy in controlling their disease and assisting their treatment process, counting as a tertiary preventive measure.
文摘BACKGROUD Follicular dendritic cell(FDC)sarcomas are rare neoplasms that occur predominantly in the lymph nodes.They can also occur extranodally.Extranodal FDC sarcomas most commonly present as solitary masses.Inflammatory pseudotumor(IPT)-like FDC sarcomas,a subcategory of FDC sarcomas,are rarer than other sarcoma subtypes.They are composed of spindle or ovoid neoplastic cells and exhibit an admixture of plasma cells and prominent lymphoplasmacytic infiltration.Paraneoplastic pemphigus(PNP),also known as paraneoplastic autoimmune multiorgan syndrome,is a rare autoimmune bullous disease that is associated with underlying neoplasms.PNP has a high mortality,and its early diagnosis is usually difficult.CASE SUMMARY We describe a 27-year-old woman who presented with stomatitis,conjunctivitis,and skin blisters and erosions as her first symptoms of PNP with an intraabdominal IPT-like FDC sarcoma.The patient underwent surgical tumor resection and received tapering oral corticosteroid treatment.She showed no recurrence at the 1-year follow-up.CONCLUSION IPT-like FDC sarcomas are rare underlying neoplasms that have an uncommon association with PNP.PNP-associated FDC sarcomas predominantly occur in intra-abdominal sites and suggest a poor prognosis.Surgical resection is an essential and effective treatment for PNP and primary and recurrent FDC sarcomas.
文摘We describe the clinical, macroscopic, cytological, histopathological, immunohistochemical, serodiagnostic and aspects of pemphigus vulgaris (PV) in the oral gingiva that clinically mimicked oral squamous cell carcinoma (OSCC) in a 57-year-old Japanese man. He developed slight haphalgesia of the buccal gingiva around teeth numbers 18 and 19 2 years ago. A dentist diagnosed intractable ulcer, but the patient ignored the condition for about 2 years until a sharp pain in the gingiva worsened. He consulted an otolaryngologist, who referred the patient to our hospital under a cytological diagnosis of OSCC. An oral examination revealed several extensive painful erosions/ ulcers from the buccal and lingual gingiva around teeth numbers 18 to 21 to the distal alveolar mucosa of no. 18 and the buccal and lingual gingiva around tooth number 31. A presumptive diagnosis of PV with dysplastic changes was determined from cytological smears. The cytological Nikolsky test was positive. The diagnosis of PV was confirmed from clinical and histopathological findings of a biopsy specimen obtained from the perilesional site. Although the definitive diagnosis of PV required only 2 weeks after this patient presented at our hospital, 2 years had elapsed since the onset of oral lesions.
文摘Pemphigus of Hallopeau (PH) is a mild and pustular form of pemphigus vegetans, termed initially as “pyodermite végétante” described by Hallopeau. We report cases in two black women. Two women aged 32 and 36 respectively (case 1 and case 2) were seen for a pustulosis associated with blisters and erosions. Their history and their review have found the mouth ulcerations. The clinical examination showed pustules on the cephalic end, the trunk, the limbs and the large folds. Lesions had a polycyclic aspect and spread in a centrifugal way, developing towards budding, squamous and scabby erosions quickly surrounded by new pustules. Regression left a pigmented macula. Histological examination of the skin lesion showed images similar to vulgaris pemphigus (PV), together with hyperacanthosis and papillomatosis. Aspects observed in immunofluorescence were identical to those of PV (case1). General corticoid therapy led to a rapid disappearance of lesions in Case 1 but in Case 2 the Lever protocol combining corticoid and methotrexate have been used. PH is rarely described in our regions. Clinical diagnosis is not obvious for the uninitiated. Beyond the clinical presentation, PH has the same paraclinical diagnosis and the same treatment as PV.
文摘Objective:To observe the effects of the recombinant chimeric toxin Dsg3EC 1-2PE40 on T and B lymphocytes isolated from Pemphigus Vulgaris (PV) patients to further study its biological therapeutic function for PV. Methods:Recombinant chimeric toxin Dsg3EC 1-2PE40 was first identified, expressed and purified, and then its effects on T and B lymphocytes of PV patients in vitro were detected and quantified by ELISPOT assay and MTT assay.Results:The purity of the expressed protein Dsg3EC 1-2PE40 was up to 80%. In ELISPOT assay, with Dsg3EC 1-2PE40, the overall number of B cells that produce anti-Dsg3 antibodies among PV patients was only about 60% of the comparable number with Dsg3EC 1-2. The proliferation of T cells of PV patients was inhibited markedly by Dsg3EC 1-2PE40. There was significant difference between the different groups with Dsg3EC 1-2PE40 and Dsg3EC 1-2.Conclusion:The recombinant chimeric toxin Dsg3EC 1-2PE40 decrease the number of B cells that produce anti-Dsg3 antibodies in PV patients and can inhibit or kill T cells of PV patients in vitro.
文摘Objective To investigate the relationship between pemphigus vulgaris (PV) and human leukocyte antigen (HLA) in Han nation of northeast China. Methods Standard microcytotoxicity test and polymerase chain reaction-sequence specific primers method were used to detect the HLA classⅠ antigens and HLA-DRB1 and DQB1 alleles in 27 patients with PV and results were compared with control group. Results Gene and phenotype frequencies of HLA-A3, A26(10), B60(40), and B13 (27.99%, 48%; 16.11%, 30%; 23.02%, 41%; 16.11%, 30%, respectively) increased significantly in PV group compared with control (1.01%, 2%; 0.5%, 1%; 4.61%, 9%; 5.13%, 10%, respectively). After P value correction, the difference of A3, A26 (10), and B60 (40) between the two groups was still significant. The gene frequencies of HLA-DRB1*140x (1401, 1404, 1405, 1407, 1408), DRB1*120x, and DQB1*0503 alleles in PV group (42.26%, 25.46%, and 23.02%) were significantly higher than control group (5.09%, 7.74%, and 1.89%). After P value correction, the difference was still significant between the two groups. Conclusion PV significantly relates with HLA in PV patients of Han nation of northeast China.
基金supported by the National Natural Science Foundation of China(Grant No.32072049)Open Foundation of State Key Laboratory of Rice Biology,China(Grant No.20210208).
文摘An EMS(ethy methanesulfonate)-induced lethal etiolated(le)mutant obtained from the rice variety Zhongjian 100 was characterized by lethal etiolated phenotypes,with significantly reduced levels of chlorophyll a,chlorophyll b,total chlorophyll,and carotenoids.Additionally,the mutant displayed a significantly decreased number of chloroplast grana,along with irregular and less-stacked grana lamellae.The le mutant showed markedly diminished root length,root surface area,and root volume compared with the wild type.It also exhibited significantly lower catalase activity and total protein content,while peroxidase activity was significantly higher.Using the map-based cloning method,we successfully mapped the LE gene to a 48-kb interval between markers RM16107 and RM16110 on rice chromosome 3.A mutation(from T to C)was identified at nucleotide position 692 bp of LOC_Os03g59640(ChlD),resulting in a change from leucine to proline.By crossing HM133(a pale green mutant with a single-base substitution of A for G in exon 10 of ChlD subunit)with a heterozygous line of le(LEle),we obtained two plant lines heterozygous at both the LE and HM133 loci.Among 15 transgenic plants,3 complementation lines displayed normal leaf color with significantly higher total chlorophyll,chlorophyll a,and chlorophyll b contents.The mutation in le led to a lethal etiolated phenotype,which has not been observed in other ChlD mutants.The mutation in the AAA+domain of ChlD disrupted the interaction of ChlDle with ChlI as demonstrated by a yeast two-hybrid assay,leading to the loss of ChlD function and hindering chlorophyll synthesis and chloroplast development.Consequently,this disruption is responsible for the lethal etiolated phenotype in the mutant.
文摘目的探讨一病一品高质量护理对天疱疮患者生活质量及护理满意度的影响。方法方便选取2022年3—12月遵义市第一人民医院收治的76例天疱疮患者为研究对象,按随机数表法分为两组,每组38例。对照组给予常规护理,观察组给予一病一品高质量护理。比较两组患者生活质量[简明生活质量量表(Short Form 36 Questionnaire,SF-36)]评分及护理满意度。结果干预后,观察组SF-36评分高于对照组,差异有统计学意义(P<0.05);观察组护理总满意度(94.74%)高于对照组(78.95%),差异有统计学意义(χ^(2)=4.145,P<0.05)。结论一病一品高质量护理应用于天疱疮患者中可取得显著效果,提高患者生活质量,且患者对该护理方式的认可度较高。
