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1.
Article in Spanish | LILACS-Express | LILACS | ID: biblio-1550688

ABSTRACT

La vasculitis reumatoidea es una complicación sistémica y poco frecuente de la Artritis Reumatoidea. Si bien su incidencia ha descendido en los últimos años con el advenimiento de las nuevas terapias inmunosupresoras y biológicas, continua teniendo una alta morbimortalidad. Predomina en el sexo masculino, en pacientes seropositivos y con un largo período de la enfermedad establecida. Requiere de alta presunción diagnostica, siendo el compromiso cutáneo y nervioso periférico el más frecuente. La biopsia de nervio o piel es requerida habitualmente para su diagnóstico. El tratamiento se basa en corticoides e inmunosupresores. Presentamos tres casos clínicos y realizamos una revisión de la literatura.


Rheumatoid vasculitis is a rare systemic complication of rheumatoid arthritis. Although its incidence has decreased in recent years with the advent of new immunosuppressive and biological therapies, it continues to have a high morbidity and mortality. It predominates in males, in seropositive patients and with a long period of established disease. It requires high diagnostic presumption, with skin and peripheral nervous involvement being the most affected. Nerve or skin biopsy is usually required for diagnosis. Treatment is based on corticosteroids and immunosuppressants. We present three clinical cases and carry out a review of the literature.


A vasculite reumatóide é uma complicação sistêmica rara da artrite reumatóide. Embora sua incidência tenha diminuído nos últimos anos com o advento de novas terapias imunossupressoras e biológicas, continua apresentando elevada morbidade e mortalidade. Predomina no sexo masculino, em pacientes soropositivos e com longo período de doença estabelecida. Exige alta presunção diagnóstica, sendo o envolvimento cutâneo e nervoso periférico os mais afetados. A biópsia de nervo ou pele geralmente é necessária para o diagnóstico. O tratamento é baseado em corticosteroides e imunossupressores. Apresentamos três casos clínicos e realizamos uma revisão da literatura.

2.
Chinese Journal of Contemporary Pediatrics ; (12): 81-85, 2024.
Article in Chinese | WPRIM | ID: wpr-1009897

ABSTRACT

Neonatal lupus erythematosus (NLE) is caused by the transmission of maternal anti-Ro/SSA antibodies, anti-La/SSB antibodies, and other autoantibodies to the fetus through the placenta. Usually, with the disappearance of autoantibodies in the children's body, abnormal changes in the mucocutaneous, blood system, and digestive system can spontaneously subside, but the damage to various systems caused by autoantibodies may persist for a long time. This article provides a comprehensive review of the manifestations and prognosis of NLE in various systems, including mucocutaneous, blood system, circulatory system, nervous system, digestive system, respiratory system, aiming to provide reference for clinical work.


Subject(s)
Child , Infant, Newborn , Female , Pregnancy , Humans , Lupus Erythematosus, Systemic/diagnosis , Prognosis , Autoantibodies , Family
3.
Article in English | LILACS-Express | LILACS | ID: biblio-1535303

ABSTRACT

ABSTRACT This study presents a case of atypical manifestation of secondary syphilis. Diagnosis was initiated prompted by the patient's complaint of a lower lip lesion, present for three months, resembling a malignant neoplasm. The lesion, a 3 cm (diameter) ulcerated nodule, arising from conjunctive tissue, raised concern. However, further physical examination revealed additional clinical features, including cervical micropolyadenopathy and erythematous skin lesions, prompting a reevaluation of the diagnosis, most likely secondary syphilis. These findings led to a serological investigation, which, ultimately, confirmed the diagnosis of syphilis. The case underscores the importance of recognizing syphilis as a formidable imitator, posing challenges in establishing differential diagnoses of mucocutaneous diseases.

4.
Medwave ; 23(7): e2684, 01-08-2023.
Article in English | LILACS-Express | LILACS | ID: biblio-1511212

ABSTRACT

A 32-year-old woman with systemic lupus erythematosus came to the rheumatology outpatient clinic reporting abdominal pain for a week, along with fever, arthralgias, myalgias, alopecia, asthenia and dyspnea on exertion over the last two months. She was hypotensive and tachycardic, requiring admission to the intensive care unit. She was diagnosed with lupus-related acute pancreatitis, an unusual complication occurring in less than 1% of cases. Most cases are mild and self-limited; however, severe and life-threatening events with multiple organ failure are possible. This article is a case report of lupus-related critical acute pancreatitis, and a literature review.


Mujer de 32 años con lupus eritematoso sistémico acude a consulta externa de reumatología por dolor abdominal de una semana de evolución, además de fiebre, artralgias, mialgias, alopecia, astenia y disnea de esfuerzo de 2 meses de evolución. También presentó hipotensión y taquicardia, por lo que requirió ingreso en la unidad de cuidados intensivos. Le diagnosticaron pancreatitis aguda relacionada con el lupus, que es una complicación inusual que ocurre en menos del 1% de los pacientes. La mayoría de los casos son leves y autolimitados, sin embargo, es posible que se presenten eventos graves y potencialmente mortales, con disfunción multiorgánica. Este artículo es un reporte de caso de una pancreatitis aguda crítica relacionada con lupus y una revisión de la literatura.

5.
An. bras. dermatol ; 98(4): 466-471, July-Aug. 2023. tab, graf
Article in English | LILACS-Express | LILACS | ID: biblio-1447230

ABSTRACT

Abstract Background: Since the beginning of the COVID-19 pandemic, a myriad of cutaneous manifestations have been described in association with this viral infection. However, in Latin America, this kind of data is still scarce. Objective: In this sense, the goal of this study was to describe the dermatological findings observed during SARS-CoV-2 infection, in a Brazilian Hospital. Methods: This is a cross-sectional, retrospective and descriptive study of 50 cases of new-onset dermatologic symptoms in patients with COVID-19, treated at Hospital Sírio-Libanês, from February to June 2020. Results: The patients (n = 50) were classified into 6 groups, according to the elementary lesions and the statistical analysis was performed. The most common cutaneous lesions were maculopapular eruptions (44%), necrosis, purpura, and livedo (32%), urticarial lesions (12%), pseudochilblains (4%) and papular-vesicular eruption (4%). In 46% of the patients the cutaneous lesions occurred in association with other symptoms, such as pruritus (38%), pain and burning sensation (8%). Lower limbs were affected in 44% of the cases, followed by the trunk (38%), upper limbs (24%) and face (14%). Cutaneous lesions were mostly found after other COVID-19 systemic symptoms, with a mean period between the viral syndrome and cutaneous signs of 5 days (SD = 6.1 days). Study limitations: It is a small sample, in a single-center study, with patients exclusively from a private Hospitals. Conclusions: Patients in Brazil have the same proportion of lesions as revealed in other studies in Europa. The compiled data is essential for a better understanding of cutaneous manifestations deemed secondary to COVID.

6.
Article | IMSEAR | ID: sea-222333

ABSTRACT

Mycoplasma pneumoniae is a common respiratory pathogen that can cause a wide spectrum of extrapulmonary manifestations with neurological manifestations being the most common. Here, we report a rare case of splenic nodules in a 7-year-old girl with M. pneumoniae infection which responded well to macrolides. M. pneumoniae infection should be considered in children with extrapulmonary manifestations including splenic lesions

7.
Chinese Journal of Ocular Fundus Diseases ; (6): 11-16, 2023.
Article in Chinese | WPRIM | ID: wpr-995588

ABSTRACT

Objective:To observe and investigate the related factors that might affect clinical features of familial exudative vitreoretinopaty (FEVR) patients.Methods:A retrospective chart study. From January 2012 and December 2021, 42 patients with 84 eyes with a diagnosis of FEVR from Department of Ophthalmology, Peking University People's Hospital were included in the study. The patients came from 42 separate families. There were 31 males and 11 females, with an average age of first diagnosis was 16.6±33.7 months. There were 21 patients referred from other hospitals for the fundus disease found in eye screening after birth, 21 patients were first seen in our hospital. There were 4 and 38 premature and full-term infants, respectively. Two patients with a positive family history of FEVR. All patients are FEVR stages 1-5. The wide-angle digital pediatric retinal imaging system after general anesthesia for fluorescein fundus angiography (FFA) examination were performed for patients aged <5 years. If patients ≥ 5 years old, routine FFA examination was performed. Sixty-eight first-degree relatives from 28 families undergo routine fundus examinations and FFA examination. Genetic examination was performed for 26 families, including 26 probands and 57 first-degree relatives. Genetic examination were performed on gene the coreceptor of low density lipoprotein receptor-associated protein 5 ( LRP5), Wnt receptor coiled protein 4 ( FZD4), Norrie disease ( NDP), tetraporin 12 ( TSPAN12), catenin β1 ( CTNNB1) genes known to be involved in FEVR. The clinical features and the genotype of FEVR were observed in relation to the clinical phenotype. Results:Among the 42 patients, 13 patients were first observed by strabismus and nystagmus, with the median age of 12 months. Eight patients were complained non-chasing or vision-related symptoms. Among the 84 eyes, FEVR stage 1 or 2, 3 or 4, and 5 were 50 (59.5%, 50/84), 31 (36.9%, 31/84), and 3 (3.6%, 3/84) eyes, respectively. Among the 23 patients who were > 3 months at first diagnosis, 16 patients had at least one eye severer than stage 3 (69.6%, 16/23). Of the 68 first-degree relatives, 22 (32.4%, 22/68) had FEVR-like changes. Among the 26 families that underwent genetic detection, 13 families (50%, 13/26) of 16 variants of FEVR-related genes were detected, of which 10 mutations of LRP5 gene were the most common. There were 10 families with single gene mutations, including 6, 2 and 2 families of LRP5, FZD4 and CTNNB1 genes, respectively. One family of LRP5 gene mutations were compound heterozygous mutations, 1 family with LRP5 gene mutaition combined with NDP gene mutation, and 1 family with LRP5 and TSPAN12 gene mutation. Among the proband with FEVR pathogenic genes, 6 cases with similiar stage of both eyes, and 7 cases with inconsistent disease stages, and there was no obvious correlation between gene mutations and clinical phenotypes. Conclusion:In addition to the age of first diagnosis, no exact factors affecting the clinical manifestations of FEVR are found, and the association between clinical phenotypic and genetic heterogeneity still needs to be further explored.

8.
Chinese Journal of Dermatology ; (12): 316-319, 2023.
Article in Chinese | WPRIM | ID: wpr-994487

ABSTRACT

Objective:To summarize clinical manifestations and histopathological features of granular parakeratosis (GP) after exposure to benzalkonium chloride.Methods:A retrospective analysis was performed on 7 GP cases with a history of benzalkonium chloride exposure in the Department of Dermatology at Wuhan No.1 Hospital from April to August 2022. Data were collected on the general condition, skin lesion manifestations, pathological examination, treatment, and follow-up of the patients, and retrospectively analyzed.Results:The 7 adult patients with GP typically presented with erythema and brown scales in the intertriginous area, exhibiting an annular distribution pattern. All the 7 patients reported recent exposure to disinfectants containing benzalkonium chloride. A total of 10 skin biopsies were taken from the 7 patients. Histopathological examination showed characteristic hyperkeratosis and fine blue-gray parakeratotic granules in the stratum corneum. All skin lesions improved 1 month after cessation of exposure to benzalkonium chloride.Conclusion:GP has a distinct clinical pattern and histopathological manifestations, and a history of exposure to benzalkonium chloride can be helpful for the diagnosis of GP.

9.
Chinese Journal of Dermatology ; (12): 229-233, 2023.
Article in Chinese | WPRIM | ID: wpr-994466

ABSTRACT

Objective:To retrospectively analyze clinical characteristics and treatment of pemphigus/bullous pemphigoid (BP) complicated by herpes simplex virus (HSV) infection.Methods:Inpatients with pemphigus/BP complicated by HSV infection were collected from Wuhan No.1 Hospital from 2016 to 2021, and their clinical characteristics, treatment and follow-up results were retrospectively analyzed.Results:Among the 8 patients with pemphigus/BP complicated by HSV infection, there were 2 males and 6 females, and their age was 50.6 ± 8.3 years. Five of them were diagnosed with pemphigus vulgaris (PV), 1 with pemphigus foliaceus (PF), and 2 with BP. Seven were infected with HSV-1, and 1 with HSV-2. All the 8 patients were given systemic glucocorticoids and immunosuppressive agents for the treatment of pemphigus or BP, and were admitted to the hospital due to resistance to the treatment. Seven patients presented with exacerbation or recurrence of primary lesions, and 1 presented with enlarged lesions all over the body. HSV infection-induced lesions were located on the trunk in 4 cases, on the oral mucosa in 4, on the scalp in 3, and on the face in 2; lesions mainly manifested as irregular erosions with blood crusts, and some centrally umbilicated pustules; 7 patients had obvious pain at the lesional sites. During HSV infection, anti-desmoglein 1 antibody levels decreased in all the 6 patients with pemphigus, and anti-desmoglein 3 antibody levels decreased in 4 of the 5 patients with pemphigus vulgaris; anti-BP180 antibody levels decreased in 1 patient with BP, but increased in the other one with BP. After antiviral therapy at adequate doses for adequate durations (7- to 14-day treatment with valacyclovir alone or in combination with ganciclovir), HSV infection was controlled, the autoimmune bullous skin disorder intensity scores decreased compared with those before the antiviral therapy, and pain was significantly relieved in all the patients. No dose adjustment of glucocorticoids or other immunosuppressive agents was made during antiviral therapy in all patients.Conclusion:HSV infection should be considered when patients with pemphigus/BP suffer from recurrence or exacerbation and poorly respond to conventional treatment; for patients with pemphigus/BP complicated by HSV infection, systemic antiviral therapy at adequate doses can be used to control the disease condition without modifying the conventional immunosuppressive regimen.

10.
Chinese Journal of Experimental Ophthalmology ; (12): 693-696, 2023.
Article in Chinese | WPRIM | ID: wpr-990901

ABSTRACT

Retinal vasoproliferative tumor (RVPT) is a rare benign glial proliferative tumor of unknown origin.The fundus is typically characterized by yellow or pink tumor-like lesions of the retina around the inferior temporal region, often accompanied by subretinal fluid, exudation, epiretinal membrane and other complications.Typical RVPT showed high fluorescence on fundus fluorescein angiography, diffuse leakage in venous phase and late stage, uneven middle or high reflection signal on B-ultrasound, and it was not difficult to diagnose combined with fundus changes.Atypical RVPT should be differentiated from retinal capillary hemangioma caused by von Hippel-Lindau disease, Coats disease and familial exudative vitreoretinopathy.The pathogenesis of RVPT is unclear, but histopathology shows that it may be the reactive proliferation of glial cells.At present, there is no unified treatment plan for RVPT, mainly cryotherapy, laser photocoagulation, local radiotherapy, transpupillary thermal therapy or photodynamic therapy for tumor.For patients with vitreous hemorrhage, vitreoretinal surgery is feasible.This article reviewed the clinical features, diagnosis and differential diagnosis, pathology and pathogenesis, and treatment progress of RVPT.

11.
Chinese Pediatric Emergency Medicine ; (12): 334-339, 2023.
Article in Chinese | WPRIM | ID: wpr-990524

ABSTRACT

Objective:To investigate the complicated virus infection of infants with pertussis and its effect on the disease.Methods:From January 2019 to March 2020, a total of 100 hospitalized infants with pertussis were admitted to the Second Affiliated Hospital of Medical College of Shantou University, nasopharyngeal swabs were collected for detection of ten pathogens including pertussis, namely respiratory syncytial virus(RSV), parainfluenza virus(PIV), bordetella pertussis (BP), human rhinovirus(HRV), human bocavirus(HBoV), human metapneumovirus(hMPV), influenza B virus (INF-B), adenovirus, influenza A virus and cytomegalovirus(CMV). According to the results of pathogen detection, all infants were divided into single detection group of BP(single detection group) and co-detection group of BP combined with viruses(co-detection group). The clinical data of the two groups were retrospectively analyzed and compared to explore the differences of clinical characteristics and its impact on the course of disease.Results:Among 100 cases, there were 54(54.0%) boys and 46(46.0%)girls.The age ranged from 28 days to 2 years and 5 months, with a median age of 3.5 months.Fifty-six cases were classified as single detection group, while 44 cases were included into co-detection group.Among infants in co-detection group, fourteen cases were co-infected with CMV(31.8%, 14/44), seven cases with HRV(15.9%, 7/44), seven cases with PIV(15.9%, 7/44), four cases with RSV(9.1%, 4/44), one case with hMPV(2.2%, 1/44), eight cases with CMV+ HRV(18.2%, 8/44), one case with HRV+ HBoV (2.2%, 1/44), one case with CMV+ PIV(2.2%, 1/44)and one case with CMV+ PIV+ INF-B(2.2%, 1/44). The number of infants in the single detection group who had cyanosis before treatment, requiring repiratory support, PICU admission, severe pneumonia or abnormal myocardial enzymes were higher than those in the co-detection group( P<0.05), while the months of age were lower than that in the co-detection group( P<0.05). When comparing the clinical characteristics of infants over three months of age, only the number of cases of combined cyanosis before treatment and the number of days in hospital were higher in the single detection group than those in the co-detection group ( P<0.05), no statistically significant differences were found in the other clinical characteristics between the two groups( P>0.05). Conclusion:The cases of infants requiring repiratory support, complicated with severe pneumonia or abnormal myocardial enzymes in the single detection group are higher than those in the co-detection group, which may be attributed to the small age of months.

12.
Chinese Journal of Applied Clinical Pediatrics ; (24): 461-464, 2023.
Article in Chinese | WPRIM | ID: wpr-990061

ABSTRACT

Objective:To study the clinical manifestations, diagnostic methods and therapeutic outcomes of transverse testicular ectopia (TTE).Methods:Clinical data of 8 cases of TTE treated in the Department of the First Urologic Surgery, Xinxiang Central Hospital and Department of Pediatric Surgery, the First Affiliated Hospital of Zhengzhou University from May 2004 to November 2018 were retrospectively analyzed.Clinical manifestations, diagnostic methods, surgical treatment and follow-up results of TTE were summarized.Results:The age of 8 cases of TTE was 1 year 5 months to 5 years.Among the 8 cases of TTE, 6 cases were involved with the left side and 2 cases with the right side.All patients were admitted due to scrotal emptiness.Three cases were combined with persistent Müllerian duct syndrome (PMDS) and 1 case combined with hypospadias.Preoperative diagnosis of TTE was definitely made in 5 cases, involving 4 cases diagnosed by ultrasound and 1 case diagnosed by magnetic resonance imaging.Laparoscopy was performed in 2 cases, including 1 case treated with laparoscopic scrotopexy, and the other one transferred to an open surgery of trans-septal orchiopexy due to poor development of the spermatic cord.Open surgery was performed in 6 cases, including 1 case with bilateral testicular fixation in the ipsilateral scrotum due to adhesion of spermatic cord closely, and 5 cases with trans-septal orchiopexy.Müllerian ducts residues were excised during surgery in 3 cases combined with PMDS.Postoperative wound infection or hematoma was not reported in all cases.Orchiepididymitis and the involvement of contralateral testes occurred in 1 case treated with trans-septal orchiopexy at 11 months postoperatively, which were relieved after anti-inflammatory treatment.All cases were postoperatively followed up for 3-48 months, and the development and blood supply of bilateral testes were detected normal by ultrasonography.Postoperative testicular atrophy was not reported.Conclusions:The possibility of TTE should be considered in patients with unilateral cryptorchidism combined with contralateral inguinal mass.Ultrasonography is preferred to the diagnosis of TTE.Laparoscopic surgery plays an important role in the diagnosis and treatment of TTE, which is helpful to identify abnormalities in the Müllerian duct structure.

13.
Chinese Journal of Applied Clinical Pediatrics ; (24): 352-355, 2023.
Article in Chinese | WPRIM | ID: wpr-990041

ABSTRACT

Objective:To summarize and analyze the clinical characteristics, treatment and prognosis of superior vena cava syndrome (SVCS) with malignant tumors in children, and to improve the understanding of its clinical management.Methods:Clinical data of 50 children with SVCS combined with malignant tumors treated in the First Affiliated Hospital of Zhengzhou University from November 2010 to May 2022 were analyzed retrospectively.The pathological types, clinical manifestations, imaging examination, treatment and prognosis were summarized.The overall survival (OS) rate and event-free survival (EFS) rate were evaluated by Kaplan-Meier method.Results:Among the 50 cases, 38 were males and 12 were females, with a male/female ratio of 3.2∶1.0.The median onset was 12.5 (8.0, 14.5) years, and the most common onset occurred in adolescence (66.0%, 33/50). Cough (80.0%, 40/50) was the most common clinical manifestation, followed by face and neck edema (66.0%, 33/50), chest tightness (56.0%, 28/50) and dyspnea (50.0%, 25/50). All the 50 cases were confirmed by histopathological examination, 39 cases(78.0%) were diagnosed as non-Hodgkin′s lymphoma (NHL). NHL was the most common malignant tumor, of which T-lymphoblastic lymphoma (T-LBL) accounted for 74.4%(29/39). All the 50 cases were examined by CT examination, involving 42 cases (84.0%) detected with mediastinal masses.Pleural effusion (86.0%, 43/50) and pericardial effusion (70.0%, 35/50) were common imaging findings.The 3-year OS rate and EFS rate of them were 59.7% and 57.9%, respectively.The 3-year OS rate and EFS rate of the 39 children with NHL were 62.9% and 60.9%, respectively.Conclusions:Children with malignant tumors complicated with SVCS are featured by the acute onset, rapid progress and poor prognosis.NHL is the most common cause, especially T-LBL.Cough, edema of face and neck, chest tightness and dyspnea are common clinical manifestations.Early detection and treatment contribute to save children′s lives.

14.
Chinese Journal of Applied Clinical Pediatrics ; (24): 318-320, 2023.
Article in Chinese | WPRIM | ID: wpr-990034

ABSTRACT

Hyper-IgE syndrome (HIES) comprises a group of rare primary immunodeficiencies, which are characterized by extremely high serum IgE levels, eczema, recurrent skin and pulmonary infections.Signal transduction and activator of transcription 3( STAT3)-HIES is the most common type, which is caused by dominant-negative mutations in STAT3.STAT3-HIES confers broad innate and acquired immune defects, defects in skeletal, connective tissue, and vascular functions, causing a clinical phenotype including eczema, staphylococcal and fungal skin and pulmonary infections, scoliosis and minimal trauma fractures, vascular tortuosity and aneurysm.In this article, the advance in diverse clinical manifestations and management strategies of STAT3-HIES was summarized.

15.
Chinese Journal of Emergency Medicine ; (12): 210-214, 2023.
Article in Chinese | WPRIM | ID: wpr-989802

ABSTRACT

Objective:To explore the clinical characteristics of patients with colchicine poisoning, and analyze the risk factors affecting the prognosis of colchicine poisoning and its value in the prognostic assessment.Methods:Patients with colchicine poisoning admitted to the Emergency Intensive Care Unit of the First Affiliated Hospital of Wenzhou Medical University from December 2017 to October 2022 were retrospectively included and divided into the survival group and death group according to the 14-d outcome. The general conditions of the two groups of patients were compared, and the clinical characteristics of patients with colchicine poisoning were analyzed. The differences of laboratory indexes, electrocardiogram, cardiac ultrasound and other clinical indexes during the first admission of patients between the two groups were compared, and their value in the prognosis evaluation of patients with colchicine poisoning was explored.Results:There were 41 patients with colchicine poisoning, aged 15-85 years, including 35 males and 6 females. There were 27 patients (65.9%) in the survival group and 14 patients (34.1%) in the death group, including accumulative poisoning (58.7%) and suicide poisoning (41.3%). The main clinical manifestations of patients with colchicine poisoning were gastrointestinal symptoms (82.93%), multiple organ dysfunction (78.05%), infectious fever (73.17%), myocardial damage (48.78%), coagulation dysfunction (46.34%), and bone marrow suppression (41.46%). Intestinal obstruction (19.51%) and rhabdomyolysis (2.44%) occurred in some patients. Multivariate Logistic regression analysis showed that the increase in absolute value of QTc interval ( OR=1.028, 95% CI: 1.000~1.056, P<0.05), lactic acid ( OR=1.599, 95% CI: 1.088~2.350, P<0.05), prothrombin time ( OR=1.205, 95% CI: 1.002~1.450, P<0.05), D-dimer ( OR=1.242, 95% CI: 1.089~1.417, P<0.05), and alkaline phosphatase ( OR=1.013, 95% CI: 1.002~1.024, P<0.05) were the risk factors for the prognosis of patients with colchicine poisoning. The decrease in the absolute value of ADL score ( OR=0.947, 95% CI: 0.909~0.988, P<0.05) and indirect bilirubin ( OR=0.756, 95% CI: 0.572~0.999, P<0.05) were the protective factors for the prognosis of patients with colchicine poisoning. D-dimer (AUC=0.913), lactic acid (AUC= 0.875) and alkaline phosphatase (AUC=0.770) had predictive value for the prognosis of patients with colchicine poisoning, and their cut-off values were 8.965 mg/L, 4.05 mmol/L and 230.5 U/L, respectively. Conclusions:The patients with colchicine poisoning have multiple organ dysfunction on admission, and are in a critical condition. The early levels of D-dimer, lactic acid and alkaline phosphatase could effectively predict the prognosis of patients with colchicine poisoning.

16.
Chinese Journal of Contemporary Pediatrics ; (12): 420-424, 2023.
Article in Chinese | WPRIM | ID: wpr-981973

ABSTRACT

Pompe disease, also known as glycogen storage disease type Ⅱ, is a rare autosomal recessive disease. With the application of enzyme replacement therapy, more and more patients with Pompe disease can survive to adulthood, and nervous system-related clinical manifestations gradually emerge. Nervous system involvement seriously affects the quality of life of patients with Pompe disease, and a systematic understanding of the clinical manifestations, imaging features and pathological changes of nervous system injury in Pompe disease is of great significance for the early identification and intervention of Pompe disease. This article reviews the research progress of neurological damage in Pompe disease.


Subject(s)
Humans , Glycogen Storage Disease Type II/drug therapy , alpha-Glucosidases , Quality of Life , Enzyme Replacement Therapy
17.
Chinese Journal of Clinical Pharmacology and Therapeutics ; (12): 910-917, 2023.
Article in Chinese | WPRIM | ID: wpr-1014604

ABSTRACT

Granulomatous lobular mastitis (GLM) is a rare form of chronic inflammatory breast disease which may be sometimes difficult to distinguish from breast cancer. The cause of GLM is unknown, but may be associated with autoimmunity, abnormal hormone levels and infection. While GLM has no specific clinical manifestations, the diagnosis is principally established by histopathology. Therapeutic options for GLM range from observation to various medical treatments, such as steroids, immunosuppressants, and antibiotics, to surgical intervention. However, there are still many difficulties in the clinical diagnosis and treatment of GLM, and there is still no unified diagnosis and treatment consensus. So, we accomplished the present review through reviewing GLM-related domestic and foreign literature, aiming to provide the basis for rational clinical diagnosis and treatment.

18.
Journal of Sun Yat-sen University(Medical Sciences) ; (6): 209-216, 2023.
Article in Chinese | WPRIM | ID: wpr-965835

ABSTRACT

The new variant B.1.1.529 of COVID-19 was reported to the WHO for the first time by South Africa on November 24, 2021. At present, Omicron has become the dominant strain in the world and is spreading rapidly. Although Omicron infected during pregnancy shows the lower risk of serious maternal and fetal complications than the previous variants and Delta variant, the rapid increase in the number of cases and the wide range of patients still worth attention of obstetricians. This article reviews the prevalence of Omicron variant infection, the clinical characteristics , the prevention and the treatment of pregnant women infected with Omicron, as well as the breastfeeding of newborns. It is believed that Omicron infection will not increase the rate of early spontaneous abortion and the infant congenital malformations, but it may increase the risk of gestational hypertensive disorders, premature delivery, cesarean section, and postpartum hemorrhage in the third trimester of pregnancy.We put forward corresponding management strategies and suggestions to provide references for the diagnosis and treatment of novel coronavirus infection in pregnant women in China.

19.
Chinese Journal of Experimental Traditional Medical Formulae ; (24): 59-65, 2023.
Article in Chinese | WPRIM | ID: wpr-964946

ABSTRACT

ObjectiveTo analyze the clinical characteristics of geographic tongue in patients with primary Sjögren's syndrome (pSS). MethodPatients with pSS treated in the China-Japan Friendship Hospital from December 2018 to October 2021 were enrolled and divided into different groups according to the presence of geographic tongue or the severity. Phi correlation analysis,Chi-square test, and Mann-Whitney test were used to analyze the clinical characteristics of patients with geographic tongue. ResultA total of 182 pSS patients were included in this study,including 75 (41.2%) patients with geographic tongue and 107 (58.8%) without geographic tongue. Partial exfoliation was more common than total exfoliation, and the anterior part of the tongue was the most common exfoliation site. The number of patients with severe geographic tongue was more than those with mild-to-moderate geographic tongue. Compared with pSS patients without geographic tongue,those with geographic tongue were more common in fissured tongue (92.0%/41.1%,χ2=48.491,P<0.05),red or crimson tongue (48.0%/23.3%,χ2=12.009,P<0.05),Yin deficiency syndrome (100.0%/43.9%,χ2=62.739,P<0.05),and Qi deficiency syndrome (94.7%∶50.5%,χ2=40.046,P<0.05),less common in phlegm-dampness syndrome (33.3%/72.0%,χ2=26.709,P<0.05),and showed higher proportions in hyperglobulinemia (89.3%/65.4%,χ2=13.547,P<0.01),ANA ≥1∶160 (78.1%/57.3%,χ2=8.227,P<0.01),and positive RF (51.4%/36.5%,χ2=3.877,P<0.05). Compared with pSS patients with mild geographic tongue,pSS patients with moderate-to-severe geographic tongue had higher proportions in hyperglobulinemia (98.1%/68.2%,χ2=14.617,P<0.01),positive anti-CENP-B (26.0%/4.8%,χ2=4.214,P<0.05),and reduced complement 3 (26.4%/4.5%,χ2=4.647,P<0.05). The geographic tongue was positively associated with fissured tongue (φ=0.531),Yin deficiency syndrome (φ=0.587),and Qi deficiency syndrome (φ=0.469),negatively associated with phlegm-dampness syndrome (φ=-0.447),and weakly associated with tongue color (φ<0.4). There was no statistical difference in the disease activity index between patients with or without geographic tongue and severity. ConclusionMore than 40% of pSS patients had geographic tongue accompanied by fissured tongue. Geographic tongue is positively associated with Yin deficiency syndrome and Qi deficiency syndrome,and negatively associated with phlegm-dampness syndrome, indicating that treatment should be based on tonifying Qi and nourishing Yin. Compared with pSS patients without geographic tongue, those with geographic tongue may have higher positive rate of some immune indicators,which deserves further exploration.

20.
Chinese Journal of Experimental Traditional Medical Formulae ; (24): 52-58, 2023.
Article in Chinese | WPRIM | ID: wpr-964945

ABSTRACT

ObjectiveTo explore the characteristics of tongue manifestations and traditional Chinese medicine (TCM) syndromes in patients with primary Sjogren's syndrome (pSS) of different time. MethodpSS patients who visited TCM Department of Rheumatism in China-Japan Friendship Hospital from December 2018 to October 2021 were included and grouped according to the disease courses (short<5 years, medium 5-10 years, long > 10 years). Chi-square test was used for comparison between three groups and Bonferroni method for multiple comparisons. In the pairwise comparison, P<0.017 indicated significant difference. Chi-square test was performed on the syndrome and specific tongue manifestations with inter-group differences to analyze the trend of tongue manifestations and TCM syndromes over time (interval: two years). ResultA total of 193 pSS patients were enrolled, with 85 (44.0%) of short disease course, 69 (35.8%) of medium disease course, and 39 (20.2%) of long disease course. The common tongue manifestations were crimson tongue, fissured tongue, thin tongue, lack of fluid, and dry coating, which showed no significant difference among the three groups. Higher proportion of patients with light red tongue was observed in the group with short disease course than in group with medium disease course (χ2=6.407, P<0.017). Higher proportions of patients with thick coating (χ2=6.784, P<0.017) and phlegm-dampness syndrome (χ2=11.545, P<0.017) and lower proportion of patients with Qi deficiency syndrome (χ2=12.706, P<0.017) were found in the group with short disease course than in the group with long disease course. Patients with medium (χ2=6.358, P<0.017) and long (χ2=8.279, P<0.017) disease course tended to have exfoliated coating compared with those with short disease course, and the proportion of patients with exfoliated coating rose and the proportion of patients with thick greasy coating decreased over time (Ptrend<0.05). In addition, the proportion of patients with phlegm-dampness syndrome decreased and that with Qi deficiency syndrome increased over time (Ptrend<0.05). ConclusionIn the early stage, patients with pSS often show both dryness and dampness, as manifested by the thick greasy coating and phlegm-dampness syndrome. In the medium and late stage, patients often have Qi-Yin deficiency, as evidenced by exfoliated coating and Qi deficiency. In the clinical practice, medicines should be prescribed based on tongue manifestations and TCM syndrome of patients.

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