Objective To summarize the experience of open heart operation on neonates with critical and complex congenital heart diseases and evaluate the methods of perioperative management. Methods From May 2001 to January 2003, 12 patients of neonates with congenital heart diseases underwent emergency operation. Their operating ages ranged from 6 to 30 days, the body weights were 2.8 to 4.5 kg. Their diagnoses included D-transposition of the great arteries in 4 cases, ventricular septal defect with atrial septal defect in 5 cases, complete atrioventricular septal defect, obstructed supracardiac total anomalous pulmonary venous drainage and cardiac rhabdomyomas in 1 case respectively. 12 cases were operated under moderate or deep hypothermic cardiopulmonary bypass. Results All cases were observed in ICU for 2-11 days and discharged 7-19 days after operation. The postoperative complications included low cardiac output, mediastinal infection, respiratory distress syndrome, systemic capillary leak syndrome and acute renal failure. All cases were cured and the follow-up (from 6 months to 2 years) showed satisfactory outcome. Conclusion A particular cardiopulmonary bypass and proper perioperative management is very important to ensure the successful outcome. Peritoneal dialysis is an effective and safe method for treating acute renal failure after cardiac operation in neonates.
Objective To investigate the anatomical features of congenital chordee without hypospadias in children and to discuss the diagnosis and treatment.Methods From August 1984 to December 2004, 94 children with chordee withouthypospadias treated in the West China Hospital of Sichuan University were classified and analyzed for anatomical alterations. Their ages ranged from 18 months to 13 years (mean 6.9 years). Ninety-four patients were divided into four groups. With intraoperation artificial erection, the patients with penis straightened after degloving were classified as type Ⅰ patients (skin-tethering), those with peins straightened after fibrotic tissue in Buck’s fascia released as type Ⅱ patients (dysgenetic fascia), those with normal urethra and orthoplasty failed after degloving and removing fibrotic tissue as type Ⅲ patients (corporal disproportion), and those with dysgenetic urethra tethering the corpora cavernosa as type Ⅳ patients (short urethra).Results In type Ⅰ (n=31, 32.9%) patients, the ventral skin and dartos fascia were contracted while Buck’s fascia and the urethra was normal, in some (7 cases) scrotal skin extended to the ventral portion of penis (webbed penis). In type Ⅱ (n=45, 47.9%), contracture of Buck’s fascia was evident and the thickening fibrotic tissue constituted the chief obstacle to orthoplasty, though in some skin was shortened. In type Ⅲ (n=6), the dorsal and ventral sides of the corpora cavernosa were disproportionated, and the morphologically normal urethra tightly adhered to the ventral aspect of corpora cavernosa. In some cases ventral skin and fascia were contracted, but orthoplasty could notbe achieved through releasing these layers. In type Ⅳ (n=12, 12.8%), the distal urethra was paper-thin and lacking corpus spongiosum, or dense fibrotic bandswere found to be deep to the urethra. The urethra tethered the corpora cavernosaand formed a bow-to-string relation. The overlying skin and fascia were contracted in varying degrees while none had significance in straightening the penis. After operation, the length of penis increased to 6.9 cm from 5.2 cm on average and the chordee was corrected to 1.6° from 42.6° before operation on average. The patients were followed up 1 months to 15 years. The results were satisfactory.Chordee remained in 2 cases, fistula and urethral stricture occurred in 2 casesrespectively, fistula in association with urethral stricture and diverticulum in 1 case; the operation was given again and the results was satisfactory. Conclusion Patients with chordee without hypospadias may be divided into four types depending on which layer of the ventral penis constitutes thechief contribution to chordee. A systematic approach with repeated artificial erection tests is needed in determining the classification and surgical correction.
ObjectiveTo systematically review the accuracy of T-cell receptor excision circles (TRECs) in screening newborns for severe combined immunodeficiency (SCID). MethodsThe PubMed, EMbase, Cochrane Library, Web of Science, CBM, WanFang Data and CNKI databases were electronically searched to collect the diagnostic accuracy studies related to the objects from inception to October 26, 2022. Two reviewers independently screened the literature, extracted data, and assessed the risk of bias of the included studies using the QUADAS-2 scale. Meta-analysis was performed using Stata 15.0 and Meta-Disc 1.4 software. ResultsA total of 18 studies involving 6 243 718 neonates were included. The results of meta-analysis showed that the pooled sensitivity (SEN), specificity (SPE), positive likelihood ratio (PLR), negative likelihood ratio (NLR), and diagnosis odds ratio (DOR) were 0.97 (95%CI 0.92 to 0.99), 1.00 (95%CI 1.00 to 1.00), 1447.05 (95%CI 528.49 to 3962.11), 0.13 (95%CI 0.08 to 0.22) and 11698.21 (95%CI 2853.44 to 47958.98), respectively. The area under the summary receiver operating characteristic curve (SROC) was 0.97. ConclusionThe application of TRECs in screening neonatal SCID has high accuracy, which is helpful for early diagnosis of SCID. Due to the limited quality and quantity of the included studies, more high-quality studies are needed to verify the above conclusion.
ObjectiveTo analyze the causes of unrelieved epilepsy thoroughly in children with isolated focal cortical dysplasia (FCD) based on MRI.MethodsRetrospective analysis of MRI and clinical data of 21 children with isolated FCD during July 2014 to January 2018, which confirmed by pathology and unrelieved thoroughly after operation performed, the pathological types and MRI signs were analyzed, as well as the frequency of different MRI signs in FCD of each pathological type. Analyzed the possible factors of surgical failure.ResultsAmong the 21 cases, there were 15 males and 6 females, with an average age of (5.7±0.3) years and an average course of disease of (3.4±0.5) years.MRI signs of this part of the children were mainly manifested by blurred focal gray matter boundaries, abnormal cortical structure changes (thickening and/or thinning), transmantle signs (abnormal cone signals extending from subcortical white matter to the ventricle) and abnormal gray matter signals, which were similar to MRI signs of FCD with satisfactory postoperative epilepsy control. 17 cases (80.9%) appeared epileptic discharge after operation in the EEG monitoring area 2 weeks to 6 months, FCD type I and type Ⅱ accounted for 35.3%, 64.7% respectively. During intraoperative EEG monitoring, no epileptiform discharge was observed in the transmantle sign region in 6 cases, and the region was retained, and only the surrounding abnormal discharge cortex was removed, complete removal of the tansmantle sign and surrounding abnormal discharge area was performed in 2 cases, and different degrees of epileptic epilepsy were observed in both methods.ConclusionMRI signs of isolated FCD with unrelieved epilepsy after operation were nonspecific, there were still epilepsy of varying degrees after all epileptogenic lesions have been removed, the cause may be related to potential epileptic factors.
目的:探讨新生儿高未结合胆红素血症病因构成、发生规律及防治。方法:对340例新生儿高未结合胆红素血症进行病因综合分析。结果:(1)病因以感染因素占首位,其次是溶血因素及围产因素;(2)病因与发病时间的关系:溶血因素及围产因素均发生在日龄lt;3 d内,发病日龄gt;3 d以感染因素为主,还有母乳性黄疸。结论:早期新生儿高未结合胆红素血症以溶血因素及围产因素为主,中晚期新生儿高未结合胆红素血症则以感染因素为主。故加强围生期保健,加强预防及抗感染,减少孕期及产时并发症的发生可有效减少新生儿黄疸的发生。
Objective To probe the clinical feature and complications of extraction of silicon oil after operation of simple rhegmatogenous retinal detachment in child patients. Methods The clinical materials of 22 patients (22 eyes) of child patients (5~14 years old) and 11 cases (11 eyes) of adult patients with simple rhegmatogenous retinal detachment undergone surgical treatment and subsequent retraction of silicon oil tampon, were reviewed and analyzed retrospectively. The reasons of extraction silicon tampon, ocular complications of intrao cular silicon tamponade and the changes of visual acuity pre- and post-extraction of silicon tamponade in above 2 groups were explored. Results The main reason for the extraction and complications of silicon oil tamponade were similar in both groups. The rate of occurrence of the complications in either group rose as time went on . There was no significant difference in change of visual acuity between two groups after the extraction of silicon oil tampon. Conclusion The responses to silicon oil after the operation for simple rhegmatogenous retinal detachment is quite similar in either children or adult patients,and there is no obvious difference between child and adult patients in the results of silicon oil extraction. (Chin J Ocul Fundus Dis，2000，16：139-212)
In the expert consensus published by the Pediatrics in 2013, it was first proposed that anti-VEGF drugs can be considered for retinopathy of prematurity (ROP) with stage 3, zone Ⅰ with plus disease. However, there are many problems worth the attention of ophthalmologists, including the advantages and disadvantages of anti-VEGF therapy compared with traditional laser therapy, systemic and ocular complications after anti-VEGF therapy, and what indicators are the end points of anti-VEGF therapy. Combined with this consensus and numerous research findings, we recommend that the first treatment for anti-VEGF or laser therapy should be considered from disease control effects. For the threshold and pre-threshold lesions, the effect of anti-VEGF therapy for zoneⅡ lesions is better than that for zone Ⅰ lesions and the single-time effective rate is high. So, it is suggested that anti-VEGF therapy should be preferred for the first treatment. The choice of repeat treatment should be considered from the final retinal structure and functional prognosis. Laser therapy is advisable for the abnormal vascular regression slower and abnormalities in the posterior pole. It can reduce the number of reexaminations and prolong the interval between re-examinations. However, the premature use of laser has an inevitable effect on peripheral vision field. Excluding the above problems, supplemental therapy can still choose anti-VEGF therapy again. Most of the children with twice anti-VEGF therapy are sufficient to control the disease. Anti-VEGF therapy should be terminated when there are signs such as plus regression, threshold or pre-threshold lesions controlled without recurrence, peripheral vascularization, etc.
摘要:目的：观察小儿反复性肺炎的发生与血微量元素的关系。方法:选择56例反复性肺炎患儿为观察组，与60例健康儿童作对照，分别测定血镉、铅、铜、锌、钙、镁、铁含量。结果:56例反复性肺炎患儿血锌、铁含量较对照组减低，差异有统计学意义（Plt;0.05）。结论:部分反复性肺炎的发生与血微量元素锌、铁缺乏有关。应对反复性肺炎患儿常规行血微量元素检测，对血微量元素缺乏者应予相应补充治疗。Abstract: Objective: To observe the relationship to the occurrence of repeated pneumonia and the microelement in blood. Methods: We chose the 56 infant patients who suffered from repeated pneumonia as the observe group while the 60 health infants as the comprise group, then test the content of cadmium， plumbum， cuprum， zinc， calcium， magnesium and ferrum in blood. Results: The content of zinc and ferrum in the patient’s blood was lower than the comparison group, and the P value was Plt;0.01 and Plt;0.05 respectively, there was obvious differentiation between the two groups. Conclusion: Occurence of part repeated pneumonia is related to the deficiency of the microelements zinc and ferrum in blood. We should detect the common content of microelement to the infant patients who suffer the repeated pneumonia, and give a complement treatment to the microelements’ deficiency infant patients.