Drug-induced toxic epidermal necrolysis (Lyell's syndrome) in a 4-year-old girl

Toxic epidermal necrolysis (Lyell's syndrome) with erythematous skin lesions and bulla formation developed in a 4-year-old girl. An accurate diagnosis using the cryostal technique on the top of a bulla was available within 1 h of hospital admission. The course was unusually mild, probably because of early treatment with corticosteroids.Skin prick tests revealed salicylamide as the agent responsible for inducing the disease. The patient was advised to avoid this substance for the rest of her life.Abbreviations TEN toxic epidermal necrolysis - SSSS staphylococcal scalded skin syndrome     

Methimazole-induced toxic epidermal necrolysis in a 12-year-old girl

We report a 12-year-old girl with hyperthyreosis who presented methamazole-induced toxic epidermal necrolysis (TEN). The patient's past history suggests a presence of autoimmune disease. To our knowledge it is the first report describing such an adverse effect of methimazole.     

Management of Stevens-Johnson syndrome and toxic epidermal necrolysis in children

A retrospective analysis of 21 consecutive patients hospitalized with either Stevens-Johnson syndrome or toxic epidermal necrolysis was carried out to assess morbidity and mortality rates and to establish the value of a specific management practice. Fourteen children with Stevens-Johnson syndrome and seven with toxic epidermal necrolysis were cared for at the Children's Memorial Hospital, Chicago, between 1978 and 1988. All were managed in a well-staffed medical ward or, when necessary, in the pediatric intensive care unit. Supportive measures included reverse barrier isolation, intravenous fluids and nutritional support, meticulous skin care, early detection and treatment of infection, and daily ophthalmologic examination. No patient was treated with systemic steroids. The mortality rate was zero. Eye complications, consisting of dry eyes or mild chronic symblepharon, were the most significant long-term sequelae.     

Toxic epidermal necrolysis and hemolytic uremic syndrome after allogeneic stem-cell transplantation

TEN and HUS are challenging complications with excessive mortality after HSCT. We report the development of these two conditions in combination in a nine-yr-old boy after HSCT from an unrelated donor. TEN with skin detachment of more than 90% of body surface area developed after initial treatment for GvHD. Within a few days of admission to the burns unit, the patient developed severe hemolysis, hypertension, thrombocytopenia, and acute renal failure consistent with HUS, apparently caused by CSA. The management included intensive care in a burns unit, accelerated drug removal using plasmapheresis, and a dedicated multi-disciplinary team approach to balance immunosuppression and infections management in a situation with extensive skin detachment. The patient survived and recovered renal function but requires continued treatment for severe GvHD. Suspecting and identifying causative drugs together with meticulous supportive care in the burns unit is essential in the management of these patients and long-term survival is possible.     

Use of intravenous immunoglobulin in toxic epidermal necrolysis and Stevens-Johnson syndrome

Toxic epidermal necrolysis and Stevens-Johnson syndrome are described as variants of the same disease with distinct severity and constitute the most frequent cutaneous reactions in children, causing considerable morbidity. Several reports support the use of intravenous immunoglobulin therapy in these entities. We report the cases of two patients, one with toxic epidermal necrolysis and the other with Stevens-Johnson syndrome, in whom immunoglobulin treatment was successfully used. We also reviewed the outcomes of 13 patients with toxic epidermal necrolysis and Stevens-Johnson syndrome in the previous 10 years in the Hospital Infantil de Mexico, in whom conventional treatment was used.     

儿童重症多形性红斑和Stevens-Johnson综合征临床对比研究

为比较儿童重症多形性红斑(EMM)和Stevens-Johnson综合征(SJS)临床特点和发病诱因,回顾分析了32例EMM和21例SJS住院患者的临床资料。结果：EMM组患病年龄较SJS小,SJS发热比率、粘膜受累和并发症均显著高于EMM;EMM组患儿有明确感染病史者占50％,SJS仅3．12％;而SJS组与药物明确相关者17／21例,EMM仅3／21例。提示可以从皮损表现上鉴别SJS和EMM;SJS粘膜和内脏损害更重;感染和药物分别在EMM和SJS发病中起主要作用。     

Toxic epidermal necrolysis in a child 6 months post‐hematopoietic stem cell transplantation

TEN is a rare and critical disease mostly caused by drugs. It is mediated by activated CD8+ T cells that cause keratinocyte apoptosis with the assistance of cytokines/chemokines. We herein report a pediatric case of TEN after allogeneic HSCT with precursor B‐cell acute lymphoblastic leukemia (pre‐B‐ALL) in second complete remission. Although we did not evaluate the T‐cell subpopulation in blood or skin lesion of the patient, an imbalanced immune reconstitution after HSCT might additively contribute to the development of TEN.     

丙戊酸钠致Stevens-Johnson综合征1例并文献复习

目的 探讨丙戊酸钠单药治疗致Stevens-Johnson综合征(SJS)的临床诊断与治疗措施.方法 通过对1例丙戊酸钠致SJS的临床表现及辅助检查、治疗效果进行归纳总结,结合文献进行综合分析.结果 患儿为3.5岁女童,初次诊断癫(痫),口服丙戊酸钠单药治疗14 d后,面部开始出现红色斑丘疹,逐渐蔓延至躯干、四肢,瘙痒明显.皮疹迅速加重,融合成片,出现疱疹,伴口唇黏膜肿胀和破溃.皮疹出现2d后体温开始升高,最高达39.5 ℃.体检发现淋巴结大、肝右肋下2 cm.辅助检查示ALT 139 IU/L,血氨108 μmol/L.立即停用丙戊酸钠,予甲泼尼龙15 mg/(kg·d)冲击治疗.2d后体温下降至正常,7d后皮疹逐渐消退,肝功能恢复正常.结论 保持对丙戊酸钠致SJS的高度警惕性是临床早期诊断和治疗的关键.早期停用致敏药物并予足量皮质类固醇激素治疗可取得良好效果.     

Stevens-Johnson syndrome and toxic epidermal necrolysis after vaccination: reports to the vaccine adverse event reporting system

We conducted a telephone survey of reports of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) to the Vaccine Adverse Event Reporting System. We identified six cases of SJS or TEN after vaccination without other obvious triggers, suggesting that SJS and TEN might very rarely be caused by vaccination. Confirmation of this hypothesis will likely require controlled studies.     

儿童药物性胆管消失综合征3例报告并文献复习

目的分析儿童药物性胆管消失综合征(VBDS)的临床特点及病理。方法收集2019年1月至2019年12月收治的3例诊断为药物性VBDS患儿的临床资料,并结合文献进行分析。结果3例患儿,女1例、男2例,年龄9月龄~8岁,临床均以黄疸型胆汁淤积伴瘙痒为主要表现;予护肝、退黄,以及免疫抑制剂等治疗,其中2例经治疗3个月后临床症状及生化指标基本恢复;1例治疗1年余仍有轻度的黄疸型胆汁淤积表现,仍在随诊中。结论早期识别药物相关性VBDS,停用相关药物并予积极治疗能改善预后。     

Drug-induced enterocolitis syndrome: Similarities and differences compared with food protein-induced enterocolitis syndrome

In 2014, drug-induced enterocolitis syndrome (DIES) was described for the first time. It is still a poorly known disease with symptoms that typically resemble those of food protein–induced enterocolitis syndrome (FPIES). To date, six more cases of DIES have been described and new clinical diagnostic criteria have been proposed based on those in the international guidelines for FPIES. In this paper, the authors describe three more cases of DIES. In addition, similarities and differences with FPIES have been deeply analyzed. To date, several unanswered questions need to be addressed, but clinicians must be instructed how to identify DIES, in order to make an allergy workup and give definite therapeutic indications to patients, especially in children where DIES seems to be more frequent.     

32例儿童链球菌中毒性休克综合征药物治疗疗效分析北大核心CSCD

目的分析儿童链球菌中毒性休克综合征(streptococcal toxic shock syndrome,STSS)不同药物治疗方案的疗效。方法回顾性收集2009年1月—2023年4月在湖南省儿童医院和郴州市第一人民医院经细菌培养证实为化脓性链球菌感染且符合STSS诊断标准患儿的临床资料,分析药物治疗疗效。根据药物治疗方案分为4组:标准组(含青霉素的方案)、A组(碳青霉烯类+糖肽类/利奈唑胺)、B组(碳青霉烯类、广谱抗生素、糖肽类/利奈唑胺单用或联用,但除外A组的方案)、C组(大环内酯类/未使用抗菌药物)。结果共纳入32例STSS患儿。药敏试验显示所有菌株对氨苄西林等β-内酰胺类抗菌药物和左氧氟沙星、万古霉素敏感,对克林霉素、红霉素及四环素耐药。标准组、A组、B组、C组4组间有效率的比较差异有统计学意义(P<0.05),其中标准组有效率最高(100%),A组、B组、C组有效率分别为40%、40%、0%。结论使用含青霉素的抗菌方案可提高儿童STSS治疗疗效。     

Streptococcal toxic shock syndrome in children without skin and soft tissue infection: report of four cases

Streptococcal toxic shock syndrome is a fulminant, highly fatal disease characterized by evidence of group A beta-haemolytic streptococcus infection and early shock with consecutive organ failure. In adults, affected individuals usually have preceding skin or soft tissue infection. However, in paediatric patients, except for varicella, the background focus is usually respiratory tract infection, and early diagnosis of streptococcal toxic shock syndrome in such patients is difficult. We report four previously healthy children with streptococcal toxic shock syndrome. Pharyngitis was identified in three cases. All of them had constitutional symptoms such as fever, vomiting, diarrhoea, abdominal pain and physical findings of tachycardia and diffuse abdominal tenderness, but no concomitant skin infection. CONCLUSION: Streptococcal toxic shock syndrome should be considered in paediatric patients with fever, vomiting, diarrhoea, abdominal pain and early shock. Early diagnosis, prompt initiation of antibiotics and aggressive fluid therapy are lifesaving for such patients.     

Mortality in adolescents and young adults with chronic diseases during 16 years: a study in a Latin American tertiary hospital

ObjectivesTo evaluate mortality in adolescents and young adult patients with chronic diseases followed in a Latin American tertiary hospital.MethodsA cross-sectional retrospective study was performed in a tertiary/academic hospital in the state of São Paulo, Brazil. Death occurred in 529/2850 (18.5%) adolescents and young adult patients with chronic diseases, and 25/529 (4.7%) were excluded due to incomplete medical charts. Therefore, 504 deaths were evaluated.ResultsDeaths occurred in 316/504 (63%) of early adolescent patients and in 188/504 (37%) of late adolescent/young adult patients. Further comparisons between early adolescents (n = 316) and late adolescent/young adult patients (n = 188) with pediatric chronic diseases at the last hospitalization showed that the median disease duration (22.0 [0–173] vs. 43.0 [0–227] months, p < 0.001) was significantly lower in early adolescents vs. late adolescent/young adult patients. The median number of previous hospitalizations was significantly lower in the former group (4.0 [1–45] vs. 6.0 [1–52], p < 0.001), whereas the last hospitalization in intensive care unit was significantly higher (60% vs. 47%, p = 0.003). Regarding supportive measures, palliative care was significantly lower in the younger group compared to the older group (33% vs. 43%, p = 0.02). The frequencies of renal replacement therapy (22% vs. 13%, p = 0.02), vasoactive agents (65% vs. 54%, p = 0.01), and transfusion of blood products (75% vs. 66%, p = 0.03) were significantly higher in the younger group. The five most important etiologies of pediatric chronic diseases were: neoplasias (54.2%), hepatic diseases/transplantation (10%), human immunodeficiency virus (5.9%), and childhood-onset systemic lupus erythematosus and juvenile idiopathic arthritis (4.9%). Autopsy was performed in 58/504 (11%), and discordance between clinical and postmortem diagnoses was evidenced in 24/58 (41.3%).ConclusionsAlmost 20% of deaths occurred in adolescents and young adults with distinct supportive care and severe disease patterns. Discordance between clinical diagnosis and autopsy was frequently observed.     

Ketoacidosis at presentation of type 1 diabetes mellitus in children: a retrospective 20-year experience from a tertiary care hospital in Serbia

Diabetic ketoacidosis (DKA) has significant morbidity and mortality and is common at diagnosis in children. The aim of this study was to determine the frequency and clinical characteristics of DKA over a 20-year period among children diagnosed with type 1 diabetes mellitus (T1DM) at University children's hospital in Belgrade, Serbia. The study population comprised of 720 patients (366 boys) diagnosed with type 1 diabetes aged <18 years between January 1992 and December 2011. Of all patients diagnosed with T1DM, 237 (32.9 %) presented with DKA. The majority had either mild (69.6 %) or moderate (22.8 %) DKA. Sixty (55.0 %) of all children under 5 years had DKA compared to sixty-two (20.9 %) in the 5- to 10-year-old group and one hundred fifteen (36.6 %) in the 11- to 18-year-old patients (p?<?0.01), while 2.5 % of the entire DKA cohort were in real coma. During the later 10-year period, children less often had DKA at diagnosis compared with the earlier 10-year period (28.0 vs. 37.4 %) (p?<?0.01), but the frequency of severe DKA was higher in the age group <5 year and in the age group >11 year during 2002–2011, compared with the earlier 10-year period (12.9 vs. 3.4 %, p?<?0.01 and 17.1 vs. 3.8 %, p?<?0.01). Conclusion: The overall frequency of DKA in children with newly diagnosed type 1 diabetes decreased over a 20-year period at our hospital. However, children aged <5 years and adolescents are still at high risk for DKA at diagnosis.