目的:探讨胚胎发育不良性神经上皮肿瘤(DNT)的临床、影像及病理学特征、诊断及鉴别诊断.方法:回顾性分析8例胚胎发育不良性神经上皮肿瘤患者的临床和影像学资料,进行光镜和免疫组织化学染色观察,并获得6例的随访资料.结果:胚胎发育不良性神经上皮肿瘤男性7例,女性1例,年龄为5～19岁,平均年龄13岁,5例以癫痫小发作为主要临床表现,病变均位于幕上,以皮层为主,影像学检查均无明显的占位效应及瘤周水肿。肿瘤细胞主要由少突胶质样细胞(OLC)、神经元和星形细胞组成,4例伴有皮质发育不良。免疫组织化学结果为神经元及部分少突胶质样细胞呈嗜铬素A、突触素及S-100阳性表达;少突胶质样细胞呈胶质纤维酸性蛋白(GFAP)阴性表达,而星形细胞呈GFAP阳性表达;Ki-67抗原标记指数lt;1%。结论: 胚胎发育不良性神经上皮肿瘤为WHOⅠ级良性肿瘤,可结合临床、影像及病理学表现明确诊断,预后良好,无需放疗和化疗。
Objective To investigate effects of neural retina on development of the structure of outer blood retinal barrier in embryogenesis. Methods The retinal neural epithelium (RNE) and pigment epithelium (RPE) layers of 150, 120 and 90 embryonic chicken eyes incubated for 7,10, and 14 days were peeled off. RNE was used to prepare the culture medium with different conditions (7drcSF3, 10drcSF3, 14drcSF3). RPE cells of 7- and 14-incubated chicken embryos were cultured on laminin-coated transwell filter. The SF3, 7drcSF3, 10drcSF3 , 14drcSF3 medium were used respectively in the apical chamber and SF2 was used in basolateral chamber. After the formation of monolayer, the transepithelial electrical resistance of the RPE was detected. After the fixation of RPE cells, the condition of the tight junction among the cells was observed by immunohis tochemistry and transmission electron microscopy. Results For the RPE cells of 7-and 14-day incubated embryonic eyes, the difference of TER in various medium of SF3/SF2, 7drcSF3/SF2, 10drcSF3/SF2, 14drcSF3/SF2 was statistically significant (P＜0.01). The polarity of RPE cells was induced and the netlike tight junctional strands was urged in the retina-conditioned medium. Conclusion The neural retina may actively promote the formation of the structure of outer blood retinal barrier. (Chin J Ocul Fundus Dis，2004，20：237-240)
Objective To investigate the difference in acetabular tilt angle (ATA) between adults with deve-lopmental dysplasia of the hip (DDH) and normal adults and the effect of ATA on acetabular version. Methods Between February 2009 and October 2015, 31 adult female patients with DDH (39 hips) (DDH group) and 31 female patients with osteoarthritis of the knee (31 hips) who had no history of hip disease (control group) were included in this study. The average age was 39 years (range, 18-59 years) in the DDH group, and was 69 years (range, 52-79 years) in control group. The morphometric parameters of the acetabulum including ATA, acetabular anteversion angle (AAA), acetabular inclination angle (AIA), acetabular cranial anteversion angle (ACAA), and acetabular sector angle (ASA) were mea- sured by CT reconstruction; The ASA was used as an index for acetabular coverage of the femoral head. The correlation between ATA and other parameters was analyzed using Pearson correlation analysis. Results The values of ATA, AAA, and AIA of the DDH group were significantly larger than those of the control group (P<0.05). The ASA in all directions was significantly decreased in the DDH group when compared with the values in the control group (P<0.05). There was no significant difference in ACAA between two groups (t=1.918, P=0.523). The ATA was positively correlated with AAA and ACAA in the DDH group (r=0.439, P=0.001; r=0.436, P=0.002), but there was no correlation between ATA and AIA (r=0.123, P=0.308). In the control group, the ATA was not correlated with AAA, ACAA, and AIA (r=–0.004, P=0.724; r=–0.079, P=0.626; r=–0.058, P=0.724). Regarding acetabular coverage of the femoral head, the ATA and AAA were correlated negatively with anterior ASA (P<0.05) and positively with posterior ASA (P<0.05), but had no correlation with superior ASA (P>0.05) in the DDH group; AIA was correlated negatively with anterior ASA and superior ASA (P<0.05) and had no correlation with posterior ASA (r=–0.092, P=0.440). In the control group, there was no correlation between ATA and ASA in any direction (P>0.05). In the DDH group, defects of the acetabular anterior wall, lateral wall, and posterior wall were observed in 18 hips (46.2%), 15 hips (38.5%), and 6 hips (15.3%), respectively. ATA value of the posterior wall defect [(15.70±10.00)°] was significantly smaller than those of the acetabular anterior wall and lateral wall defects [(22.91±5.06)° and (21.59±3.81) °] (P<0.05), but no signficant difference was found between anterior wall and lateral wall defects (P>0.05). Conclusion ATA will influence acetabular version in DDH. The anterior rotation of the acetabular fragment during periacetabular osteotomies is an anatomically reasonable maneuver for hips with anterolateral acetabular defect, while the maneuver should be avoided in hips with posterior acetabular defect.
Objective Discussed the postoperative seizure control situation of children with focal cortical dysplasia (FCD),which took Transmantle sign (T2WI or T2 FLAIR sequence inward extension cone high signal in the direction of ventricle) as the main MRI performance, and analyzed the influence of various factors on the prognosis of surgery.MethodsRetrospective analysis was performed on 56 children with FCD with Transmantle signs as the main MRI signs confirmed by pathology in Qilu Children's Hospital of Shandong University from May 2015 to March 2020, including 33 males and 23 females, with an average age of (4.8±0.13) years old, and their imaging and clinical data were analyzed. Analyzed the pathological types and main MRI signs, the range of epileptiform discharge, and Engel classification after surgery. Analyzed the effect of surgical method, type of onset, age of operation, epileptic site, course of disease and perioperative epileptic seizure on the surgical prognosis of children.ResultsPostoperative pathology showed that all the 56 cases both were FCDⅡb, in addition to the Transmantle sign, the MRI performance were also accompanied by focal blurring of gray and white matter, abnormal cortical structure, and abnormal signal foci in gray and white matter. Intraoperative EEG monitoring results showed that the epileptiform discharge area in all cases was larger than the lesion range showed by MRI. Postoperative Engel classification: 25 cases of gradeⅠ (43.8%), 18 cases of gradeⅡ (31.3%), 4 case of gradeⅢ (6.3%), 9 cases of gradeⅣ (18.8%). Surgical resection and perioperative seizures are independent factors affecting the prognosis of children (P<0.05).Conclusionsurgical methods and perioperative seizures were closely related to the prognosis of children.
ObjectiveTo explore the mid-term effectiveness of total hip arthroplasty (THA) with subtrochanteric shortening osteotomy in treatment of Crowe type Ⅳ developmental dysplasia of the hip (DDH).MethodsBetween September 2009 and March 2014, a total of 49 patients (57 hips) who were diagnosed with Crowe type Ⅳ DDH were treated with THA and subtrochanteric shortening osteotomy. Of the 49 patients, 7 were male and 42 were female with an average age of 44.6 years (range, 20-73 years). The preoperative Harris score was 44.68±3.39 and the preoperative leg length discrepancy was (5.27±0.55) cm.ResultsAll incisions healed primarily. All patients were followed up 32-87 months (mean, 52.1 months). At last follow-up, the Harris score was 85.67±2.89 and the leg length discrepancy was (1.12±0.48) cm, showing significant differences when compared with the preoperative values (t=–69.53, P=0.00; t=42.94, P=0.00). X-ray films showed that bone union of the femoral osteotomy end at 6 months after operation. There was no loosening and subsidence of prosthesis at last follow-up.ConclusionThe subtrochanteric shortening osteotomy with THA in treatment of Crowe type Ⅳ DDH can obtain satisfactory mid-term effectiveness with low risk of peripheral vascular and nerve traction injuries.
Objective To investigate the spatial and temporal regulation effect of VEGF on human fetal retinal vascularization and angiogenesis. Methods The posterior segmental retinas from 54 human fetuses of the 9th week to the 40th week were studied by immunohistodhemistry standing for the expressions of VEGF and PCNA. Results 1. The distribution of VEGF espression was spiking and the peaks were during the 9th-13th and around the 26th week. 2. PCNA immunoreactivity was localized in spindle cells and vascular endothelial cells. The expression level was fluctuated during the developmental process. The peaks were during the 9th-13th and around the 21st week. In these periods, the spindle cells kept proliferating and differentiating, and remodelled subsequently to form the inner side retinal vessels. From the 26th or 34th week, the PCNA immununoreactivity is fully expressed in the vascular endothelial cells of the inner and outer margin of inner nuclear layer(INL) and kept to full terms. 3. Significant positive correlation were shown between the content of VEGF in the retina and that of PCNA in spindle cells and vascular endothelial cells(r=0.736,p＜0.01). Conclusion VEGF was positively involved in modulating human fetal retinal vascularization and angiogenesis. (Chin J Ocul Fundus Dis,1999,15:12-15)
ObjectivesTo evaluate the predicting value of bedside pulmonary ultrasound in bronchopulmonary dysplasia (BPD) in premature infants.MethodsPremature infants with gestational age below 28 weeks or birth weight below 1 500 g admitted to NICU of Chengdu Women and Children’s Central Hospital from June 2018 to June 2019 were included. Pulmonary bedside ultrasound monitoring was performed on the 3rd, 7th, 14th and 28th day after admission, and the characteristic ultrasound images were recorded and scored. BPD were diagnosed by NICHD standard. The clinical data and pulmonary ultrasound data were compared and analyzed. Then diagnostic value of bedside pulmonary ultrasound in BPD of premature infants were analyzed.ResultsA total of 81 children involving 32 BPD and 49 non-BPD were included. The sensitivity (Sen), specificity (Spe) and area under curve (AUC) of receiver operating characteristic (ROC) of the "alveolar-interstitial syndrome" within 3 days after birth and the "fragment sign" on 28 days after birth were 81.25%, 51.02%, 0.66 and 31.25%, 97.96%, 0.65, respectively. The lung ultrasound scores in the BPD group on the 3rd, 7th, 14th, and 28th day after birth were 71.99.%, 68.39%, 0.71; 87.50%, 57.14%, 0.72; 78.13%, 73.47%, 0.76 and 56.25 %, 75.51%, 0.66. Sen, Spe and ROC AUC of comprehensive evaluation of lung ultrasound predicted the occurrence of BPD been 81.25%, 63.27%, and 0.85.ConclusionsThe comprehensive evaluation of combination of "alveolar interstitial syndrome" image characteristics within 3 days after birth, "fragment sign" image characteristics after 28 days, and lung ultrasound score at different times after birth can predict the premature infants with bronchopulmonary dysplasia.
ObjectiveTo explore the effect of false acetabulum on the development and anatomical morphology of proximal femur in Crowe type Ⅳ developmental dysplasia of the hip (DDH), providing a theoretical basis for the development of femoral reconstruction strategy and prosthesis selection for total hip arthroplasty. Methods The medical records of 47 patients (54 hips) with Crowe type Ⅳ DDH between February 2008 and March 2020 were retrospectively analyzed, of which 21 patients (26 hips) were Crowe type Ⅳa (type Ⅳa group) and 26 patients (28 hips) were Crowe type Ⅳb (type Ⅳb group). There was no significant difference in general data such as gender, age, height, weight, body mass index, and side between the two groups (P>0.05), which were comparable. The height of femoral head dislocation, the height of pelvis, and the proportion of dislocation were measured based on preoperative anteroposterior pelvic X-ray film. Based on the preoperative femoral CT scan data, the anatomical parameters of the femur and femoral medullary cavity were measured after three-dimensional reconstruction using Mimics19.0 software to calculate the canal fare index; and the femoral medullary cavity parameters were matched with the modular S-ROM prosthesis parameters. ResultsThe results of X-ray film measurement showed that the height of femoral head dislocation and the proportion of dislocation in type Ⅳa group were significantly higher than those in type Ⅳb group (P<0.05). There was no significant difference in the height of pelvis between the two groups (P>0.05). The results of CT three-dimensional reconstruction measurements showed that compared with the type Ⅳb group, the type Ⅳa group had less isthmus height, smaller femoral head, shorter femoral neck, narrower neck-shaft angle, increased anteversion angle, and higher greater trochanter, and the differences were significant (P<0.05). There was no significant difference in the height of femoral head, femoral offset, and height difference between greater trochanter and femoral head between the two groups (P>0.05). There was no significant difference in the mediolateral width (ML), anteroposterior width (AP), and diameter of the isthmus (Ci level) and the AP of the medullary cavity in the plane 40 mm distal to the most prominent point on the medial side of the lesser trochanter (C–40 level) (P>0.05), and the size of medullary cavity was significantly smaller in type Ⅳa group than in type Ⅳb group at the other levels (P<0.05). Compared with the type Ⅳb group, the difference between the outer diameter of the prosthetic sleeve and the diameter of the medullary cavity fitting circle in the plane where the center of femoral head rotation was located from the medial most prominent point of the lesser trochanter (C0 level) in type Ⅳa group was smaller, and the proportion of negative values was greater (P<0.05). The difference between the longest diameter of the prosthetic sleeve triangle and the ML of the medullary cavity in the plane 10 mm proximal to the most prominent point on the medial side of the lesser trochanter (C+10 level) in type Ⅳa group was smaller, and the proportion of negative values was greater (P<0.05). ConclusionFalse acetabulum has a significant impact on the morphology of the proximal femur and medullary cavity in patients with Crowe type Ⅳ DDH, and the application of three-dimensional reconstruction technique can accurately evaluate the femoral morphology and guide the selection of femoral prosthesis.
ObjectiveTo recognize and carry out early diagnosis for Cockayne syndrome (CS) as it is an extremely rare auto-recessive genetic syndrome characterized by multiple symptoms including growth failure and impaired development of the nervous system. MethodsHere we reported a case of typical CS with an unusual appearance. The 19-year-old young male patient was referred to West China Hospital on December 24th 2012. We analyzed the clinical characteristics of the patient and followed the literature review to help improve the knowledge on CS for clinicians. ResultsThe patient's parents were cousins. Laboratory data showed that lipoprotein profile, blood glucose and electrolytes, liver and renal function, as well as hormones (thyroxin, para-thyroxin, growth hormones, adrenocorticotropic hormone, corticosteroid) were all within normal limit. Electronic hearing examination showed moderate neural hearing loss. CT scan indicated multiple intracranial calcifications. The patient was definitely diagnosed with CS. He received nutritional support and symptomatic treatment but discharged due to lack of effective treatment. ConclusionCS is a progressive multisystem disorder characterized by a specific cellular defect in transcription-coupled repair. Typical features include developmental delay and impaired development of the nervous system. Typical clinical manifestations and imaging changes are helpful for clinical diagnosis of CS. Genotyping is necessary for patients with CS. Unfortunately, there is no ideal treatment for CS. Most of the patients with CS have poor prognosis.
Objective To investigate the impact of three kinds of palliative operation on the body and growth of pulmonary artery in patients with congenital heart diseases of diminutive pulmonary blood. Methods Clinical data was reviewed in 28 cases of congenital heart diseases with diminutive pulmonary blood who had been performed cavopulmonary connection （n = 9）, systemic-pulmonary shunt （n = 8 ）, and palliative reconstruction of right ventricular outflow tract （n=11）. The period between re-hospitalized and the first was 5-54 months （19.07±10. 06 months ）. Hematocrit （HCT）, hemoglobin （Hb）, percutaneous oxygen saturation （SpO2）, body surface area （BSA）, and pulmonary artery index （PAI） etc. were observed both before palliation and before the second operation. Results After the second hospitalization, there were 7 cases of death from hemorrhage, failure of circulation and extracorporeal circulation accident etc. The time of respirator, intensive care unit and total amount of dopamine in patients of palliative reconstruction of right ventricular outflow tract were longer and more than those in patients of cavopulmonary connection （P〈0. 05）. HCT, Hb before the second operation were decreased than thoes before palliative operations in all patients, SpO2, BSA and PAI increased significantly （P 〈 0. 01 ）. Before the second operation, BSA of patients with cavopulmonary connection, BSA and PAI of patients with systemic-pulmonary shunt, SpO2, BSA and PAI of patients with palliative reconstruction of right ventricular outflow tract were increased than those before palliative operations（P〈0. 01）. HCT of palliative reconstruction of right ventricular outflow tract was decreased（P〈0. 05）. Conclusion This results suggests that pulmonary blood of patients with congenital heart diseases of diminutive pulmonary blood can be increased, development of pulmonary arteries can be improved efficiently by systemic-pulmonary shunt and palliative reconstruction of right ventricle outflow tract, but it can not be found in cavopulmonary connection patients.