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Objective:To explore the role of systematic wound management in the treatment of Stevens-Johnson syndrome (SJS) /toxic epidermal necrolysis (TEN) in children.Methods:Clinical data were collected from 5 children with SJS/TEN, who were admitted to the Department of Dermatology, Tianjin Children′s Hospital from October 2019 to August 2021, and retrospectively analyzed to investigate the role of systematic management of the skin and mucosa wound besides drug therapy in the treatment of SJS/TEN.Results:The 5 patients, including 1 case of SJS, 1 case of SJS/TEN overlap syndrome, and 3 cases of TEN, were aged 2 to 13 years, and were cured and discharged after the treatment with intravenous methylprednisolone and systematic wound management. Among the 5 children, the severity of TEN scores (STENS) were 18.6 ± 1.9 points at admission, and 13.8 ± 1.9, 4.4 ± 0.5, and 2.0 ± 0.7 points on days 7, 10, and 14 during hospitalization, respectively; the time to complete re-epithelialization was 10.0 ± 2.1 days, and the length of hospital stay was 14.4 ± 4.2 days. During 4-month multidisciplinary follow-up, no scars or complications of the eyes, mouth, genitalia, or lungs occurred.Conclusion:Systematic wound management is helpful to control the progress of SJS/TEN, accelerate wound healing, and improve patients′ quality of life.
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Objective:To investigate pathogenic genes and inheritance patterns in 3 consecutive collodion babies in a family.Methods:The proband was diagnosed as a collodion baby due to extensive dry and chapped skin all over the body at birth. Phenotypes of the proband's parents were normal, but their first and second children presented with dry and chapped skin at birth and died a few days after birth. DNA was extracted from peripheral blood samples of the patient and her parents for whole-exome capture sequencing, and candidate mutations were verified by Sanger sequencing.Results:Compound heterozygous mutations in the ALOX12B gene were identified in the infant, including a missense mutation c.1405 C>T (p.R469w) inherited from her father and a frameshift mutation c.68_69insC (p.L24fs) inherited from her mother.Conclusions:The infant was diagnosed with hereditary ichthyosis, which was inherited in an autosomal recessive manner. The missense mutation c.1405 C>T and frameshift mutation c.68_69insC in the ALOX12B gene may contribute to the clinical phenotype of this infant, and the frameshift mutation had not been reported in China or other countries.
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Objective:To evaluate the clinical efficacy and safety of adalimumab combined with acitretin in the treatment of childhood generalized pustular psoriasis.Methods:Five children with generalized pustular psoriasis were collected from Department of Dermatology, Tianjin Children′s Hospital from October 2019 to August 2020. After admission, the patients received oral acitretin at a dose of 0.5 mg·kg -1·d -1. After relevant laboratory examinations, these patients additionally received subcutaneous injections of 20- or 40-mg adalimumab at weeks 0 (the initial dose) , 1, and every 2 weeks thereafter; when patients obtained a 50% improvement in the Japanese Dermatology Association (JDA) severity index score, the dose of acitretin would be reduced to 0.3 mg·kg -1·d -1, and acitretin would be discontinued after a 75% improvement. The disease condition was evaluated at weeks 0, 1, 2, 4, 8, 12 and 24 after the start of adalimumab treatment, and adverse reactions were monitored during treatment. Results:All the 5 patients received drug treatment for at least 40 weeks. After 2-week treatment, 3 patients achieved a 50% reduction in JDA severity index score (JDA50) ; after 4-week treatment, 4 achieved JDA75, and 1 achieved JDA100; after 8-week treatment, all the 5 patients achieved JDA100. By June 2021, all the 5 children received follow-up for at least 40 weeks, no recurrence was observed during the treatment period, and no infections, malignant tumors or other serious adverse reactions occurred.Conclusion:Adalimumab combined with acitretin shows rapid onset of action and high safety in the treatment of childhood generalized pustular psoriasis.
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Objective:To explore any effect of aerobic exercise on cardiac energy metabolism and mitochondrial respiration after myocardial infarction.Methods:Forty-five Sprague-Dawley rats were randomly divided into a sham operation group, a heart failure control group and a heart failure exercise group. Myocardial infarction was induced in the heart failure groups using coronary artery ligation. Four weeks after the successful modeling, the heart failure exercise group underwent 8 weeks of aerobic treadmill exercise. The cardiac function and exercise ability of all of the rats were then observed using echocardiography and the incremental treadmill exercise test. Myocardial creatine phosphate (PCr) and adenosine triphosphate (ATP) were measured by magnetic resonance spectroscopy, and the respiratory function of the myocardial mitochondria was evaluated by using cell respirometry.Results:Compared with the sham operation group, the average PCr content, PCr/ATP ratio, oxygen consumption of mitochondrial respiratory chain complexes I and II, left ventricular shortening fraction (FS) and ejection fraction (EF), maximum running speed, exhaustion distance and exhaustion time in the incremental treadmill exercise test were all significantly worse in the heart failure control group. Moreover, the average ATP content, complex I oxygen consumption, left ventricular FS and EF, and the maximum running speed, exhaustion distance and exhaustion time in incremental treadmill exercise of the heart failure exercise group were all superior to those of the heart failure control group.However, no significant differences were observed in the average PCr/ATP ratio between the heart failure exercise and control groups.Conclusions:Regular aerobic exercise can improve cardiac performance after chronic heart failure, at least in rats. The mechanism may be related to increased levels of myocardial ATP and better mitochondrial complex I functioning. The PCr/ATP ratio may not be a suitable biomarker for evaluating the benefits of exercise for the heart.
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Objective:To investigate reflectance confocal microscopic features of childhood scabies, and to analyze clinical significance of reflectance confocal microscopy (RCM) in the diagnosis and treatment of childhood scabies.Methods:A retrospective analysis was performed in 77 children with confirmed scabies at Department of Dermatology, Tianjin Children′s Hospital from April 2018 to October 2019. These patients were divided into negative treatment history group (61 cases) and positive treatment history group (16 cases) . All the patients underwent RCM and microscopic examination of skin scrapings.Results:Among the 77 children with scabies, positive microscopic examination results were found in 33 (42.86%) , including 28 in the negative treatment history group and 5 in the positive treatment history group. Burrows, sarcoptid mites or their eggs and fecal pellets were observed in 56 cases (72.73%) by RCM, including 49 (80.33%) in the negative treatment history group and 7 in the positive treatment history group. RCM showed a significantly increased overall positive rate compared with microscopy of skin scrapings ( χ2=14.08, P<0.05) . In the negative treatment history group, RCM also showed a significantly increased positive rate compared with microscopy of skin scrapings ( χ2=15.53, P < 0.05) . Conclusion:RCM is of high clinical value to the diagnosis and treatment of childhood scabies.
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A 1-month-old male infant presented with skin flushing covering with collodion-like membrane all over the body at birth, and experienced gradual skin desquamation thereafter. At the age of 2 months, collodion-like membrane completely peeled off, and the patient presented with obvious scales and dry skin. Skin examination showed generalized dry skin, tense, glossy and transparent plastic wrapper-like membrane remaining on the front chest, large and disk-shaped white scales with an adherent center and free edges inlaid in the skin of the trunk and scalp. Genetic testing showed compound heterozygous mutations in the CYP4F22 gene of the patient, including the mutation c.1137G>A (p.W379X) inherited from his father and the mutation c.467G>A (p.R156H) inherited from his mother. The patient was diagnosed with lamellar ichthyosis.
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A 87-day-old female patient presented with patchy erythema on the right body and ipsilateral limb deformity after birth, and visited the hospital in January 2012. Skin examination showed obvious red plaques on the right lower abdomen, right buttock, right perineum, right leg and right foot, with yellow scales on the surface and clear boundaries, and there was no obvious exudation or odor; pale yellow verrucous hyperplasia was observed on the right lower jaw, right neck, right axilla, and on the back of the first to fourth fingers of the right hand; only 1 interphalangeal joint and no nail plate was observed on the second, third and fourth toes of the right foot. The X-ray of the right foot showed deformity and bone defects. The patient was diagnosed with CHILD syndrome, and treated with intermittent oral acitretin capsules, and topical vaseline ointment, tacalcitol ointment and weak-potency glucocorticoid ointment for more than 6 years. The skin lesions regressed during treatment, but occurred repeatedly after withdrawal. Thereafter, the patient was switched to simvastatin ointment for 2 years. The skin lesions increased proportionally with the increase of age, and the affected limb grew a little more rapidly than the healthy limb. By January 2020, the skin lesions on the right lower jaw, right neck, and the first to fourth fingers of the right hand had subsided, but new band-like hypertrophic verrucous plaques occurred on the fifth finger of the right hand; the skin lesions on the right leg were slightly improved, and no involvement of other systems was observed.
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Objective To investigate the etiology,clinical manifestations,diagnosis and outcomes of lichen scrofulosorum (LS).Methods Clinical data collected from 7 infants with LS,who visited Department of Dermatology,Tianjin Children's Hospital between 2010 and 2018,were analyzed retrospectively,and the etiology,clinical manifestations,diagnosis,development and outcomes of LS were investigated.Results All the 7 infants developed LS after bacillus Calmette-Guérin (BCG)vaccination,and the clinical manifestations of LS were reddish or hazel papules mainly distributed on the trunk with small scales attached to the surface.No abnormal laboratory examination results were observed.Histopathological examination showed granuloma in the dennis.Without any treatment,all rashes of the 7 patients disappeared within 6 months,and no scar or hyperpigmentation remained.Conclusions Infant LS usually appears 2-3 months after BCG vaccination,clinically presents as papules mostly on the trunk,pathologically characterized as granuloma in the dermis,and can heal spontaneously.
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Objective To observe the clinical efficacy of ebastine combined with Runzao Zhiyang capsules in the treatment of patients with chronic urticaria. Methods A total of 126 patients with chronic urticaria admitted to Department of Dermatology of Tianjin Third Central Hospital from January 2015 to May 2017 were enrolled and they were divided into two groups by the random number table method. The patients in control group (62 cases) were given oral ebastine administration 10 mg once per day, and those in observation group (64 cases) received oral administration of ebastine 10 mg once per day combined with Runzao Zhiyang capsule 2 g, 3 times per day, the therapeutic course being 4 weeks. The changes of clinical efficacy and the symptom scores, including urticaria activity score (UAS) and dermatolo-gical disease life quality index (DLQI) scores of the two groups were observed after treatment of 4 weeks;the incidence of adverse reactions and the recurrence situation after drug withdrawal for 4 weeks at follow-up were analyzed. Results Compared with the control group, the total effective rate of the observation group was significantly increased [92.2% (59/64) vs. 79.0% (49/62), P < 0.05]. After treatment, the overall UAS score and DLQI score in two groups were both significantly decreased, the degree of decrease in observation group were more siginificant than those in control group [UAS: 1.26 (0.52 - 7.35) vs. 1.68 (0.75 - 8.65), DLQI: 0.56±0.52 vs. 1.57±0.96, P < 0.01]. In addition, the total decrease degree of symptom score reducing index (SSRI) in the observation group was significantly greater than that in the control group [(76±21)% vs. (69±23)%, P < 0.05], the incidence of adverse reactions [7.8% (5/64) vs. 12.8% (8/62)] and recurrence rate [8.3% (3/64) vs. 23.8% (5/62)] in the observation group were obviously lower than those in the control group (both P < 0.05). Conclusion The efficacy of ebastine combined with Runzao Zhiyang capsule in the treatment of patients with chronic urticaria is prominent and superior to that of using ebastine alone, the combined method is capable of elevating the therapeutic effect obviously and has less adverse reactions.
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Objective To evaluate the clinical application value of reflectance confocal microscopy(RCM) in the diagnosis of several common diseases manifesting as papules in children, including lichen nitidus, verruca planae, lichen striatus, milium, molluscum contagiosum and lichen pilaris. Methods A total of 579 children clinically characterized by papules were recruited into this study. RCM was used to observe lesions and perilesional normal skin. The RCM features of 6 diseases manifesting as papules were analyzed and compared. Results Based on RCM images, 236 patients were diagnosed with lichen nitidus, 70 with verruca planae, 123 with lichen striatus, 40 with milium, 53 with molluscum contagiosum and 57 with lichen pilaris. All the 6 diseases had typical RCM features. Concretely speaking, RCM images of lichen nitidus lesions showed infiltration of dense inflammatory cells and melanophages in enlarged dermal papillae. In RCM images of verruca planae lesions, cells in the granular and spinous layers were arranged in concentric circles, giving a rose cluster?like appearance. RCM images of lichen striatus lesions revealed focal swelling of stratum spinosum, absent or local liquifaction degeneration of basal cells, and clustering of a moderate number of inflammatory cells in the superficial dermis. In RCM images of milium lesions, well?circumscribed round or oval structures containing highly but nonuniformly refractive materials could be seen in the dermis. RCM images of molluscum contagiosum lesions showed intact cystoid structures containing highly refractive molluscum bodies. Lowly to moderately refractive cutin ? like materials were observed along with the dilation of hair follicle infundibula in RCM images of lichen pilaris lesions. In RCM images, the 6 diseases were distinguished mainly based on structural features(patterns and refractivity)of skin lesions shown by continuous vertical scanning. Conclusion RCM is of great value to the diagnosis of diseases manifesting as papules in children.
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Objective To identify common allergens in patients with eczema or dermatitis by using serum IgE tests, and to explore their clinical significance. Methods A retrospective study was carried out on eczema or dermatitis patients with at least one positive serum IgE test result from 10 hospitals in China. Results Totally, 458 patients were included in this study with an average age of 28.13 ± 23.11 years. Of these patients, 209 (45.63%)were male, and 249 (54.37%)were female. The top three allergens were cockroaches(180/458, 39.30%), dust mite mix(152/458, 33.19%) and peanuts (116/458, 25.33%). Polysensitization (sensitization to more than one allergen)was observed in 89.66%(26/29)of patients with atopic dermatitis and 86.84%(33/38)of patients with facial eczema. Moreover, the rate of polysensitization was significantly higher in patients with generalized lesions than in those with circumscribed lesions (80%(88/110)vs. 70.40%(245/348),χ2=3.880, P=0.049). The positive rate of specific IgE against inhaled allergens was highest in the age group of 11-20 years(39/43, 90.70%), while the specific IgE against milk(26.53%, 26/98)and beef (19.39%, 19/98)was mainly observed in children aged less than 3 years. In addition, patients in northern areas showed increased positive rates of specific IgE to mugwort (10.35% vs. 1.61%,χ2= 4.917, P< 0.05), cockroaches (42.42%vs. 19.35%,χ2=11.959, P<0.05), milk (14.39%vs. 3.23%,χ2=5.958, P<0.05), soybeans (13.89%vs. 3.23%,χ2=5.594, P<0.05), beef(11.36%vs. 1.61%,χ2=5.641, P<0.05)and fresh-water fish(13.38%vs. 3.23%,χ2 = 5.235, P< 0.05)compared with those in southern areas. Conclusions Cockroaches are a common allergen in patients with eczema or dermatitis, and their clinical significance is worthy of further study. Polysensitization seems to be more frequent in patients with facial eczema or atopic dermatitis. Age is an important factor influencing allergen sensitization, and there is a regional difference in the distribution of common allergens.