ObjectiveTo investigate the clinical characteristics, diagnosis and management of postoperative chylothorax after congenital cardiac surgery in infants and children. MethodsWe retrospectively analyzed clinical data of 79 postoperative patients with chylothorax after congenital cardiac surgery in Guangdong General Hospital between January 2006 and December 2013.There were 54 males and 25 females at age ranged 8 days to 14 years. ResultsThe prevalence of postoperative chylothorax was 0.6% (79/12 067). A total of 75 (94.9%) patients were cured. And 71 patients (89.9%) were cured by conservative treatment. While 4(5.1%) patients received operative treatment, including 3 patients undergoing ligation of thoracic duct, 1 patient undergoing lymphatic ablation. There were 4 (5.1%) patients failed to treat, including 1(1.3%) abandoned, 3 (mortality of 3.8%) deaths in hospital for low cardiac output syndrome, cardiac arrest and severe anastomotic stenosis after transposition of conducting arteries(TGA), tetralogy of Fallot(TOF) and total anomalous pulmonary venous connection(TAPVC) operation respectively. Hospitalization time ranged 10 to 108 (39.3±19.4) d. There was no recurrence of chylothorax within 6 months to 8 years of following-up. ConclusionThe key to prevention of chylothorax is to improve the surgical technology. Conservative management of chylothorax will be successful in most cases, but surgical treatment ought to be considered if the conservative management is unsuccessful.
Objective To study the efficacy and adverse events of adjunctive perampanel in children with refractory epilepsy. Methods A prospective study was carried out in 45 children with refractory epilepsy, who were treated in our hospital from January 2020 to February 2021 using perampanel as an add-on treatment, with a criteria for enrollment and the starting dose of perampanel. Follow-up would be taken at once a month. Afte 3 months would check blood routine, liver function, kidney function and humoral immunity. The EEG was reviewed after 6 months. The initial dose of perampanel was 0.04 mg/(kg·d) (the maximum didn't exceed 2 mg/d), increasing by 0.04 mg/(kg·d) every two weeks, and the maximum maintenance dose didn't exceed 6 mg/d. The efficacy and adverse reactions of perampanel were evaluated by comparing the seizure frequency and EEG results before and after a 6-month add-on therapy.ResultsAmong the 45 children,complete seizure control was achieved in 7 cases after the therapy, and the seizure attacks were reduced in 26 cases, showing a total response rate of 73.3%. After the treatment, the epileptiform discharge of 28 children was reduced, and the effective rate was 62.22%. During the observation period, all the blood routine, liver function, kidney function,and humoral immunity of the children were normal.10 cases of adverse reactions occurred after the additional treatment of perampanel, and the adverse reaction rate was 22.22%. Conclusions Perampanel has good efficacy and safety in the add-on treatment of refractory epilepsy.
ObjectiveTo study the relation between the clinical phenotype and neurological developmental quotient in children with epilepsy and GPR98 gene mutation. MethodsGenomic DNA was extracted from peripheral blood lymphocytes of the probands and other available members in the epilepsy families.Clinical datas and screened for mutations by next-generation sequencing conbined target sequencing technology and PCR and direct DNA sequencing were collected.Then, the relations between the clinical phenotype and developmental quotient in children with epilepsy and GPR98 gene mutation was analyzed. ResultsSeven novel GPR98 gene mutations were found in seven probands in 65 families, including six heterozygote missense mutations (c.6083C <、c.1969A < C、c.17531C < T、c.9069G < C、c.6661G < A and c.18496A < C) and one nonsense mutation (c.14224G < T). One of their parents carried the same GPR98 gene mutation as the proband's. The initial symptom of six cases was afebrile seizures and one showed febrile seizure, in which the main type seizure was generalized seizure.Moreover, was were significant difference between children with epilepsy and GPR98 gene mutations and healthy children in developmental quotient test(P < 0.01). ConclusionsThe main type of seizures in children with epilepsy and GPR98 gene mutations is generalized seizure. Furthermore, GPR98 gene mutations may be associated with psychomotor retardation.