Aged ; Anti-Bacterial Agents ; adverse effects ; therapeutic use ; Asian Continental Ancestry Group ; Cephalosporins ; adverse effects ; therapeutic use ; Electroencephalography ; Female ; Humans ; Kidney Failure, Chronic ; Liver Cirrhosis ; Male ; Middle Aged ; Neurotoxicity Syndromes ; diagnosis ; etiology ; Singapore

Patients with extensive malignancies involving the femur often require total femoral replacement when their limbs can be salvaged. Reported series are small and involve heterogeneity of tumours. We present nine patients with osteosarcomas of the femur treated at our institution between 2003 and 2010 with a mean follow-up of 27 (6 to 56) months. Their ages ranged from 9 to 17 (mean 14 years). They had large volume tumours (mean 911 cm3 ) and presented late with a mean of 5.5 months from the onset of symptoms to definitive treatment. All patients underwent resection and total femur replacement. Six patients have died and two are alive with good function at the time of this report. One was lost to follow-up. These patients require a high level of treatment care and have a guarded prognosis
Osteosarcoma

ObjectiveTo investigate the predictive factors for acute cholangitis in patients with obstructive jaundice. MethodsA retrospective analysis was performed for the clinical data of 358 patients with common bile duct stones and obstructive jaundice who were treated in The Fourth People′s Hospital of Haikou from October 2010 to October 2015. According to the presence or absence of acute cholangitis, the patients were divided into acute cholangitis group(n=223) and obstructive jaundice group(n=135). Age, sex, and comorbidities were compared between the two groups, and the association between abnormalities in serum tumor markers and liver function parameters and the development of acute cholangitis was examined. The parameters with statistical significance were used to establish the receiver operating characteristic (ROC) curves, and their sensitivity and specificity in the diagnosis of acute cholangitis were evaluated. The t test and χ2 test were applied for comparison of continuous data and categorical data between groups, respectively. ResultsThe acute cholangitis group had significantly higher serum levels of CA19-9 and CA12-5 than the obstructive jaundice group (serum level of CA19-9: 82.33±23.01 kU/L vs 36.75±12.58 kU/L, t=11.028, P＜0.05; serum level of CA125: 30.21±9.59 kU/L vs 18.62±5.27 kU/L, t=8.597, P＜005). The analysis of the ROC curves showed that the areas under the ROC curve for serum CA19-9 and CA12-5 were 0.891 and 0705, respectively, and the corresponding cut-off values for the highest diagnostic accuracy were 61.01 kU/L and 22.56 kU/L, respectively, with sensitivities of 82.1% and 77.6% and specificities of 79.8% and 69.5%. ConclusionIncreased serum CA19-9 level has a great value in predicting acute cholangitis in patients with common bile duct stones and obstructive jaundice.

Objective: To discuss the experience of early surgical intervention to critical and complex congenital heart diseases (CHD) in neonates. Methods: A retrospective study of clinical records of patients with congenital heart diseases underwent surgical intervention in one single pediatric cardiac center was performed. From January 2009 to December 2017, 841 critical and complex CHD neonates were identified at Department of Cardiovascular Surgery, Children′s Hospital of Fudan University, of which 6.5% were premature. There were 557 male and 284 female patients. The age was (11±9) days, ranging from 0 to 28 days, M(Q_R): 8(17) days. The weight was (3.26±0.57) kg, ranging from 1.9 to 5.0 kg, M(Q_R): 3.3(0.7) kg. There were 189 patients associated with other malformation besides CHD. Before surgery, 13.6% (114/841) patients were on ventilation, and severe acidosis was addressed in 136 patients. All patients underwent surgical interventions, including surgical procedures and hybrid procedures. Results: Emergency surgeries were performed in 35 patients. One hundred patients had palliative procedures, other 633 patients had complete repair, while the rest 108 patients underwent hybrid procedures. The in-hospital mortality was 4.0% (34/841). The ventilation time was 1.5 to 1 130.0 hours, M(Q_R): 94 (116) hours, with the rate of reintubation 3.3% (27/807). The ICU stay time was 1 to 195 days, M(Q_R): 14 (15) days. Conclusion The improvement on early screening and diagnosing, referral system, multidisciplinary cooperation and hybrid invention skills together contributed to better outcomes of critical and complex congenital heart diseases in neonates.

Pulmonary artery denervation (PADN) can block local sympathetic nerve of pulmonary artery, reduce the hemodynamic parameters of pulmonary arterial hypertension(PAH), attenuate pulmonary vascular remodeling, right ventricular hypertrophy and fibrosis, thus improving cardiac function.Early basic experiment has determined the position of sympathetic nerve of pulmonary artery in pulmonary endarterium and confirmed the safety and effectiveness of PADN in the animal model of PAH.PADN may play a role by inhibiting execssive activation of the sympathetic nervous system and renin-angiotension-aldosterone system.PADN has been applied to adult clinical research, and has achieved a good clinical effect.On this basis, the possibility of applying PADN to children′s PAH is being explored preliminarily.

Objective: To investigate the effect of the endoscopic selective varices devascularization (ESVD) for the esophageal gastric varices bleeding (EGVB) in children. Methods: The clinical data of the patients diagnosed with EGVB and treated with ESVD from January 2018 to March 2018 were retrospectively analyzed. The effects, safety and complications of ESVD were evaluated. Results: There were five patients (including 2 males and 3 females, age ranged from 4 to 7 years) in the study. No rebleeding was found at the first follow-up on one week post operation. Three patients were treated with the endo-therapy at the twice follow-up (3 months after surgery): 2 patients had ESVD again and 1 patient had resection under endoscopy due to stenosis caused by surgical scar. After the second procedure, there was no rebleeding but one patient had abdominal pain caused by mesenteric thrombosis, cured with low molecular weight heparin. Conclusion The ESVD for EGVB is safe and effective, but the long-term curative effect should be further studied.

Congenital cystic adenomatoid malformation(CCAM) is a rare cystic lung disease with an incidence rate ranging from 1/35 000 to 1/25 000 births.The mechanism is still unclear and is regarded a nonhereditary anomaly caused by arrest of lung.In some countries, over 99% of CCAMs were diagnosed by fetal sonography at gestational age of 18~20 weeks.Chest X-ray and CT are common methods to detect the CCAMs after birth.Newborns with CCAM may present with respiratory distress, but most patients are asymptomatic in life.Although many methods for diagnosis and treatment were suggested, no unified guideline is provided yet.Herein, we reviewed the advances in the mechanism, diagnosis and treatment of CCAM to highlight this rare event.

OBJECTIVETo analyze the genetic polymorphism, distribution of haplotypes, common and well-documented (CWD) and rare alleles of high-resolution HLA-A, B and DRB1 alleles by analysis from hematopoietic stem cell (HSC) donors in Jiangsu Han Chinese.

METHODSPCR-sequence-based typing and PCR-sequence specific oligonucleotide probes methods were applied for HLA-A, B and DRB1 high-resolution genotyping of 3238 unrelated healthy donors of hematopoietic stem cells in Jiangsu branch of Chinese National Marrow Donor Program registry.

RESULTS46 alleles of HLA-A,85 HLA-B and 51 HLA-DRB1 locus were found. The frequencies of the most common alleles were A * 11:01 (16.52%), B * 13:02 (11.60%) and DRB1 *07:01 (15.78%). That of the most common haplotype was A * 30: 01-B * 13: 02-DRB1 * 07: 01 (8.87%). 40 alleles of HLA-A,77 alleles of HLA-B, and 47 HLA-DRB1 alleles of HLA-DRB1 were CWD, which account for 99. 8% of total number of samples, and a few rare alleles not reported in Chinese population were found.

CONCLUSIONThe results of high-resolution, CWD and rare alleles showed the characteristics of HLA distribution in Jiangsu Han population, which may be useful for finding HLA matched unrelated donors, as well as for HLA correlation with population genetics and disease association studies.

Alleles ; Asian Continental Ancestry Group ; genetics ; Genotype ; HLA-A Antigens ; genetics ; HLA-B Antigens ; genetics ; HLA-DRB1 Chains ; genetics ; Haplotypes ; Hematopoietic Stem Cells ; Humans ; Tissue Donors

Adult ; Carcinoma, Renal Cell ; diagnosis ; Cerebellar Neoplasms ; diagnosis ; Diagnosis, Differential ; Female ; Hemangioblastoma ; diagnosis ; Humans ; Kidney Diseases, Cystic ; diagnosis ; Kidney Neoplasms ; diagnosis ; Magnetic Resonance Imaging ; Neoplasms, Multiple Primary ; Pancreatic Cyst ; diagnosis ; Retinal Neoplasms ; diagnosis ; Tomography, X-Ray Computed ; von Hippel-Lindau Disease ; diagnosis

Objective: To evaluate the clinical value of color Doppler signal at the nasal lip in the diagnosis of fetal cleft palate. Methods: Twenty fetuses diagnosed with fetal cleft lip and cleft palate by targeted ultrasonography and magnetic resonance imaging were enrolled in this study. Color Doppler was used during each ultrasound scan. All prenatal diagnoses were confirmed either by postnatal follow-up or autopsy. The location and degree of cleft lip and palate was also recorded. During inspiration or swallowing color signal acrossed the defects at the fetal palate was indicated cleft palate, and no color signal acrossed the defects at the fetal palate was indicated without cleft palate. Two dimensional ultrasound combined with color Doppler was used to diagnose cleft lip and palate in midsagittal plane or paratsagittal plane, then compared with the results of post-natal evaluation or abortion, and the value of color signal at the nasal lip in the diagnosis of fetal cleft palate was analyzed. Results: Twenty cases of postpartum fetus (including post-natal evaluation or abortion ) confirmed 15 cases of cleft lip and palate, 5 cases of cleft lip (2 cases with upper alveolar cleft). For prenatal diagnosis, 5 cases of cleft lip and 15 cases of cleft lip and palate were diagnosed by two-dimensional ultrasound, of which 3 cases of cleft lip and palate were misdiagnosed as cleft lip, whereas 3 cases of cleft lip were misdiagnosed as cleft lip and palate with 2 cases combined with upper alveolar cleft. Two-dimensional ultrasound in the diagnosis of cleft lip and palate is consistent with postnatal results by additional evaluation of the color signal in nasolabial area in median sagittal section. In the cleft lip and cleft lip with the alveolar cleft fetuses, the color signal in nasolabial area was undetectable during fetal respiratory-like movement or swallowing by ultrasound.However, it was detectable in cleft lip and palate fetuses. A total of 3 cases of simple cleft lip and 17 cases of cleft lip and palate were detected by magnetic resonance imaging(MRI). In 2 cases of cleft lip and palate diagnosed by MRI, no blood flow signal was detected at the nasolabial area. Conclusions The connected color signal in nasolabial area assessed by color Doppler ultrasound during fetal respiratory-like movement or swallowing showed an promising value for prenatal diagnosis of cleft palate. Cleft palate could be ruled out if the connected color signal was undetectable in nasolabial area.