Good's syndrome (GS) is a rare combination of thymoma and hypogammaglobulinemia, resulting in immunodeficiency. Patients with GS are highly susceptible to bacterial infection, particularly encapsulated bacterial infection in upper and lower respiratory tracts. Good's syndrome patients with moderate-to- severe infection are often hospitalized. Clinical features of GS patients remain to be characterized.Patients with the discharge diagnosis of GS and simultaneous infection from Peking Union Medical College Hospital between January 2001 and July 2015 were retrospectively analyzed.Among 14 hospitalized GS patients, 12 of them were admitted for severe infections. Mean patient age was 56.7 + 10.1 years. Average concentrations of serum IgG, IgA, and IgM were 2.3 + 1.9 g/L, 0.28 + 0.28 g/L, and 0.06 + 0.07 g/L, respectively. Respiratory and intestinal tracts were the most common sites for infection, which occurred in 7 and 4 patients, respectively. Pathogens identified in 10 patients included cytomegalovirus in 5 patients, Pneumocystis jirovecii, Clostridium difficile in 2 patients, Klebsiella pneumonia in 2 patients, and Streptococcus pneumonia and Hemophilus influenza in 1 patient. Ten patients were treated with antibiotics and immunoglobulin replacement. Only 1 patient who was on immunosuppressant therapy died from P. jirovecii pneumonia.Infection was the most frequent cause for hospitalization of GS patients. Both respiratory and intestinal tracts were the most common sites of infection. Cytomegalovirus and P. jirovecii represented 2 common opportunistic pathogens isolated from hospitalized GS patients with infections.
Pregnancy is usually contraindicated in patients with pulmonary hypertension (PH). Risk factors associated with the outcome of this rare disease have not been specifically explored before.Medical records were retrospectively reviewed to identify patients with coexisting PH and pregnancy or delivery at Peking Union Medical College Hospital between January 2009 and June 2018. Demographics, characteristics of PH and pregnancy, management and outcomes were analyzed.Thirty-six pregnant women with PH were identified, including 30 cases in WHO group 1, 5 cases of group 2 and 1 case of group 4. Median pregnancy duration was 24 weeks. The overall maternal mortality rate was 8.3% (3/36), and the late fetal mortality was 31.6% (6/19). Pulmonary vascular-targeted medications were used in 17 of 26 patients with moderate or severe PH, but in none with mild PH. Maternal mortality was 2/15, 1/11, and 0 among women with severe, moderate, and mild PH, respectively. All deaths reported to be diagnosed of PH after pregnancy, and have New York Heart Association (NYHA) grades II to IV. Cesarean section was performed in 22 patients, and mortality was 3/16 among women receiving cesarean section with general anesthesia.Maternal mortality is associated with PH classification, severity of PH, delayed diagnosis of PH, and NYHA classification. Regional anesthesia seems superior to general anesthesia for cesarean section.
The present study aimed to evaluate the clinical outcomes and bearing-specific complications in a single center of 135 delta ceramic-on-ceramic (COC) total hip arthroplasties (THAs) and explore the occurrence rate of squeaking in 4th generation COC THAs and collate the risk factors for squeaking.We retrospectively analyzed consecutive cohorts of 127 patients (135 hips) who had primary THA with delta COC bearings in our hospital between April 2010 and April 2012. Preoperative Harris hip score (HHS), Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) score, and University of California Los Angeles (UCLA) activity score were evaluated preoperatively and postoperatively. We also evaluated ceramic fracture, squeak, mid-term results, and survival.In our study, the final patient final follow-up date was July 31, 2016. The mean preoperative HHS improved from 39.5 to 93.1 points. Mean preoperative UCLA score was 3.2 points, improving to 8.2 points. The mean preoperative total WOMAC score was 55.5 points and the mean total WOMAC score was 13.3 points at the final follow-up. The Kaplan–Meier survival plot of revision for any reason as the end point was 98.5% and survival with ceramic fracture as the end point was 99.2% at a mean of 70 months’ follow-up. One post-operative ceramic liner rim fracture occurred at 50 months after surgery. Thirteen of the 135 hips produced a squeaking sound. There were no significant differences in gender (P = .56), age (P = .20), body mass index (BMI) (P = .11), diagnosis (P = .46), cup inclination (P = .36), or cup anteversion (P = 1.0) between the squeaking and non-squeaking groups. However, the incidence of squeaking in the 36 mm COC femoral head bearings was higher than in the 28 mm size (13.6% vs 2.1%, P = .033).The 4th generation COC bearing performed well and provides an encouraging rate of survival with no osteolysis or loosening. However, we found that a squeaking sound associated with use of the delta ceramic occurred at a rate of 9.6%, with the larger-diameter heads having significantly higher incidence.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.