2.Clinical features of children with periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome: an analysis of 13 cases.
Ji-Qian HUANG ; Xiao-Hua YE ; Kang-Kang YANG ; Yao-Yao SHANGGUAN ; Yi-Wei DONG ; Wen-Jie ZHENG
Chinese Journal of Contemporary Pediatrics 2021;23(2):143-147
OBJECTIVE:
To study the clinical features of children with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome, a polygenic and multifactorial autoinflammatory disease with unknown pathogenesis.
METHODS:
A retrospective analysis was performed on the medical data of 13 children with PFAPA syndrome.
RESULTS:
All 13 children had disease onset within the age of 3 years, with a mean age of onset of (14±10) months. They all had periodic fever, with 8-18 attacks each year. The mean interictal period of fever was (30±5) days. Pharyngitis, cervical adenitis, and aphthous stomatitis were the three cardinal symptoms, with incidence rates of 100% (13/13), 85% (11/13), and 38% (5/13) respectively. There were increases in white blood cells, C-reactive protein, and erythrocyte sedimentation rate during fever. Of all the 13 children, 6 underwent whole exome sequencing and 7 underwent panel gene detection for autoinflammatory disease, and the results showed single heterozygous mutations in the
CONCLUSIONS
For children with unexplained periodic fever with early onset accompanied by pharyngitis, cervical adenitis, aphthous stomatitis, elevated inflammatory indices, and good response to glucocorticoids, PFAPA syndrome should be considered. This disorder has good prognosis, and early diagnosis can avoid the long-term repeated use of antibiotics.
Child
;
Child, Preschool
;
Fever/etiology*
;
Humans
;
Infant
;
Lymphadenitis/diagnosis*
;
Pharyngitis/drug therapy*
;
Pyrin
;
Retrospective Studies
;
Stomatitis, Aphthous/genetics*
3.Clinical analysis for oral mucosal disease in 21 972 cases.
Hongfeng WANG ; Fangqi HE ; Chunjiao XU ; Changyun FANG ; Jieying PENG
Journal of Central South University(Medical Sciences) 2018;43(7):779-783
To analyze the incidence and distribution of oral mucosal diseases in Hunan Province and provide reference for prevention and treatment.
Methods: The clinical data for all patients, who were treated in Xiangya Hospital of Central South University from April 2013 to March 2017, were collected. After screening, weighing and classifying, sex and age distribution for the disease was analyzed.
Results: The female with the age between 40 to 49 were in the majority among 21 972 patients. The ratio between men to women was 1:1.05. According to the classification of diseases, the most common diseases were as follows: recurrent aphthous ulcer (27.17%), burning mouth syndrome (15.72%), oral submucous fibrosis (14.75%), oral lichen planus (10.38%), oral leukoplakia (4.21%), traumatic ulceration (4.14%), chronic cheilitis (3.47%), oral fungal infection (3.26%), and atrophic glossitis (2.74%). Recurrent oral ulcer (28.65%), burning mouth syndrome (23.70%) and oral lichen planus (13.31%) were the most common 3 kinds of oral mucosal diseases during females in Hunan. Oral submucous fibrosis was the most common oral mucosal disease among males in Hunan (28.56%).
Conclusion: Recurrent oral ulcer, burning mouth syndrome and oral lichen planus are very popular in women in Hunan Province, and oral submucous fibrosis is the most common disease in male in this region. It shows a high trend of incidence in the surrounding provinces.
Adult
;
Age Distribution
;
Burning Mouth Syndrome
;
epidemiology
;
China
;
epidemiology
;
Female
;
Fibrosis
;
Humans
;
Incidence
;
Leukoplakia, Oral
;
epidemiology
;
Lichen Planus, Oral
;
epidemiology
;
Male
;
Middle Aged
;
Mouth Diseases
;
epidemiology
;
therapy
;
Mouth Mucosa
;
pathology
;
Oral Submucous Fibrosis
;
Oral Ulcer
;
epidemiology
;
Sex Distribution
;
Stomatitis, Aphthous
;
epidemiology
4.Acute Precursor T Cell Lymphoblastic Leukemia Associated with Behcet's Disease: A Case Report.
Ji Won KIM ; Jeung Hui PYO ; Kyeong Jin KIM ; Ho KIM ; Yong JEOUNG ; Jong Dae JI ; Young Ho LEE
Journal of Rheumatic Diseases 2014;21(1):46-49
Behcet's disease is an inflammatory disorder characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. A few cases of hematologic disease in patients with Behcet's disease have been reported in the literature. However, acute precursor T cell lymphoblastic leukemia has never been described in association with Behcet's disease. We recently encountered a case of acute precursor T cell lymphoblastic leukemia in a 62-year-old man with a prior diagnosis of Behcet's disease. The patient presented with febrile neutropenia and his bone marrow biopsy revealed acute precursor T cell lymphoblastic leukemia. He was scheduled to undergo therapeutic chemotherapy, but unfortunately he died from pneumonia prior to treatment.
Biopsy
;
Bone Marrow
;
Diagnosis
;
Drug Therapy
;
Febrile Neutropenia
;
Hematologic Diseases
;
Humans
;
Middle Aged
;
Pneumonia
;
Precursor T-Cell Lymphoblastic Leukemia-Lymphoma*
;
Skin
;
Stomatitis, Aphthous
;
Ulcer
;
Uveitis
6.Effects of dihydromyricetin on tumor necrosis factor and NF-kappaB p65 of RAU rats.
Xue-min YANG ; Xiao-hong WANG ; Li-feng CHEN ; Xiao-qing WANG
China Journal of Chinese Materia Medica 2012;37(17):2612-2617
OBJECTIVETo study the effects of dihydromyricetin (DMY) on tumor necrosis factor (TNF-alpha) and NF-kappaB p65 cells of the recurrent aphthous ulcer (RAU) rat.
METHODSixty of Sprague Dawley (SD) rats are randomly divided into 6 groups. The rat RAU models was established by injection of immunogen composed of the homogenate supernate of homogeneous oral mucosa from SD rats and Freund's complete adjuvant (FCA) into rat backs subcutaneously once every two weeks for 5 times, and the only FCA injected as normal control. DMY(50,100, 200 mg x kg(-1)) and licorzine (67.5 mg x kg(-1)) were given intragastrically once daily for 7 days on the day of the last immunogen injection, respectively. Water was given instead of drugs in normal and model control groups. The blood was got from the fundus oculi vein of rats on the day after last administration, the serum was separated. Then the rats were put to death with the cervical dislocation and decollated on the ice stage. Two sides of rat buccal mucosal tissue were cut. One side of them was put into 4% neutral formalin and another was added into 10 times of phosphate buffer to homogenize it homogenate. The oral mucosa ulcer occurrence of rats was observed by the histopathology. The content of TNF-alpha in serum and oral mucosa was assayed with ELISA; the expression of NF-kappaB cells was determined by the immunohistochemisty and macrophagus was determined by azure-feosin-dyeing in oral mucosa tissue. The expression of TNF-alpha mRNA in serum and oral mucosa was detected by reverse transcription polymerase chain reaction.
RESULTIn RAU rats, oral mucosa ulcer occurred, the content of TNF-alpha raised and the expression of TNF-alpha mRNA increased in serum and oral mucosa, the expression of positive NF-kappaB p65 cells and the amount of macrophages went up in oral mucosa. DMY and licorzine significantly reduced occurrence of oral mucosa ulcer in RAU rats, lowered content of TNF-alpha and the expression of TNF-alpha mRNA in serum and oral mucosa, reduced expression of positive NF-kappaB p65 cells and the amount of macrophages.
CONCLUSIONIt is considered that DMY could inhibited occurrence of oral mucosa ulcer in RAU rats. One principle of it's effects could be that DMY controlled NF-kappaB p65 regulation on transcription and release of TNF-alpha mRNA in macrophages in oral mucosa ulcer tissue and lead to fall of TNF-alpha content in oral mucosa tissue causing role of anti-oral mucosa ulcer.
Animals ; Disease Models, Animal ; Female ; Flavonols ; administration & dosage ; Humans ; Macrophages ; drug effects ; immunology ; Male ; Mouth Mucosa ; drug effects ; metabolism ; Rats ; Rats, Sprague-Dawley ; Stomatitis, Aphthous ; drug therapy ; genetics ; immunology ; Transcription Factor RelA ; genetics ; immunology ; Tumor Necrosis Factor-alpha ; genetics ; immunology
7.Oral aphthosis: management gaps and recent advances.
Michelle W LIANG ; Ching Yin NEOH
Annals of the Academy of Medicine, Singapore 2012;41(10):463-470
INTRODUCTIONThough oral aphthosis is common, it has a significant impact on the quality of life in the patients. It is the most common oral ulcerative condition encountered in clinical practice. This study describes the characteristics and patterns of oral aphthosis seen at a tertiary dermatological centre in Singapore, with emphasis in evaluating the management gaps and in identifying underlying systemic diseases and nutritional deficiencies.
MATERIALS AND METHODSThis is a retrospective review of medical records over a 10-year period between June 2000 and June 2010. Two hundred and thirteen patients were identified using the search terms 'oral ulcers', 'aphthous ulcers', 'oral aphthosis', and 'Behcet's disease'. Patients with Behcet's disease without oral ulcers and other diagnoses such as pemphigus vulgaris, lichen planus and herpes simplex were excluded. The remaining patients were evaluated with regard to demographic characteristics, characteristics of oral ulcers, associated connective tissue disorders and nutritional deficiencies, diagnostic tests results, treatment response as well as follow-up duration.
RESULTSOne hundred and seventy-fi ve patients were included in this study. One hundred and one patients had recurrent oral aphthosis, with 77 having simple aphthosis and 24 having complex aphthosis. Fourteen patients (8%) fulfilled the International Study Criteria (ISG) for Behcet's disease, of which, 85.71% had complex aphthosis. The therapeutic ladder for such patients ranged from topical steroids and colchicine through to oral corticosteroids and/or dapsone therapy.
CONCLUSIONRecurrent oral aphthosis is a niche condition in which dermatologists are well-poised to manage. This study demonstrates that a more definitive management and therapeutic algorithm for oral aphthosis are needed for better management patients in the future. In particular, complex aphthosis needs to be monitored for progression onto Behcet's disease.
Adolescent ; Adrenal Cortex Hormones ; therapeutic use ; Adult ; Aged ; Aged, 80 and over ; Anti-Infective Agents ; therapeutic use ; Anti-Inflammatory Agents ; therapeutic use ; Behcet Syndrome ; complications ; Child ; Child, Preschool ; Colchicine ; therapeutic use ; Dapsone ; therapeutic use ; Drug Therapy, Combination ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Recurrence ; Retrospective Studies ; Stomatitis, Aphthous ; diagnosis ; drug therapy ; etiology ; Treatment Outcome ; Tubulin Modulators ; therapeutic use ; Young Adult
8.Bilateral bleeding at achilles tendon for infantile aphthae.
Chinese Acupuncture & Moxibustion 2011;31(12):1100-1100
Achilles Tendon
;
blood supply
;
Bloodletting
;
Child
;
Child, Preschool
;
Female
;
Humans
;
Infant
;
Male
;
Stomatitis, Aphthous
;
therapy
9.Effect of shuizhongcao granule on cellular immune function of experimental animal with recurrent aphthous stomatitis.
Min-He SHEN ; Shan-Ming RUAN ; Min-Huan BAO
Chinese Journal of Integrated Traditional and Western Medicine 2009;29(10):901-904
OBJECTIVETo investigate the effect of Shuizhongcao Granule (SZCG) on cellular immune function in animal model of recurrent aphthous stomatitis (RAS).
METHODSExperimental animal model of RAS were established by immunological method. The modeled animals were randomized into 4 groups, Group A was the model control group, Group B was treated by Levamisole (2.5 mg/mL), Group C and D was treated with high-dose (0.5 g/mL) and low-dose (0.24 g/mL) SZCG respectively. The medication was administered via gastric perfusion, starting from the 7th week of the experiment, and continued for 28 days. Meanwhile, a normal control group was set up. Percentage of T-lymphocyte subsets, CD3, CD4 and CD8, and serum levels of interleukin 10 and 12 (IL-10 and IL-12) in peripheral blood were detected before and after treatment.
RESULTSPercentages of CD3, CD4 and CD4/CD8 ratio in the model control group were 40.50 +/- 7.46%, 30.80 +/- 5.33% and 79.56 +/- 8.32 respectively, all lower than those in the normal control group (P<0.05); level of serum IL-10 was higher (8.02 +/- 0.53 ng/L) and that of IL-12 was lower (15.51 +/- 1.35 ng/L) in the model animal than those in normal control (P<0.05). After treatment, levels of CD3, CD4, CD4/CD8 and IL-12 in Group B were 55.30 +/- 6.33%, 43.50 +/- 5.33%, 99.29 +/- 13.84 and 17.72 +/- 1.70 ng/L, those in Group C were 61.50 +/- 4.32%, 47.20 +/- 4.37%, 103.30 +/- 7.42 and 18.18 +/- 1.54 ng/L, and in Group D were 58.50 +/- 6.03%, 42.80 +/- 3.17%, 110.27 +/- 12.85 and 17.74 +/- 1.96 ng/L, respectively, all were higher than those in Group A, but levels of IL-10 in the three treatment groups were lower than that in Goup A (P<0.05 or P<0.01).
CONCLUSIONThe immune function of RAS model animals is in the suppressed condition; SZCG can improve the immune suppression to achieve its therapeutic effect on the disease.
Animals ; CD4-CD8 Ratio ; Drugs, Chinese Herbal ; therapeutic use ; Interleukin-10 ; blood ; Interleukin-12 ; blood ; Male ; Mouth Mucosa ; Phytotherapy ; Rabbits ; Stomatitis, Aphthous ; drug therapy ; immunology ; T-Lymphocyte Subsets ; immunology

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