OBJECTIVE: To evaluate the mid-term efficacy of talonavicular joint fusion alone combined with navicular stress adjustment in Müller-Weiss disease with significant paradoxical flatfoot deformity. METHODS: Between January 2016 and March 2021, a total of 13 patients diagnosed with Müller-Weiss disease underwent simple talonavicular joint fusion combined with navicular stress adjustmentadjustment.Among them, 5 patients were male and 8 patients were female;age ranged from 42 to 67 years old. The duration of the disease ranged from 8 to 20 years. According to Maceira staging system, 5 patients were in stage III and 8 patients were in stage IV. The American Orthopaedic Foot and Ankle Society (AOFAS) midfoot score, visual analogue scale (VAS), and foot length were evaluated preoperatively and 10 months postoperatively. Additionally, the talonavicular coverage angle, the talus-first metatarsal angle, and the calcaneal inclination angle were assessed preoperatively and 6 months postoperatively. RESULTS: All 13 patients were followed up a period ranging from 24 to 40 months. All patients achieved clinical healing within a period of 3 to 6 months. However, one patient experienced a screw fracture. The VAS decreased from a range of 3 to 7 points preoperatively to a range of 0 to 2 points at the 10 months postoperative. The AOFAS midfoot score improved from a preoperative range of 12 to 66 points to a range of 72 to 100 points at the 10 months postoperative. Based on the AOFAS midfoot score evaluated at 10 months postoperatively, the outcomes were rated as excellent in 3 feet, good in 6 feet, and fair in 4 feet. The talo-navicular coverage angle, the preoperative talus-first metatarsal angle, calcaneal inclination angle were 14.3° to 33.4°, -4.6° to -19.6°, and 3.0°to 16.4° respectively. Six months postoperatively, these angles improved to 2.7°to 9.6°, -8.4°to 1.1°, and 18.8°to 24.9°respectively. Additionally, the foot length increased by 0 to 6 mm at 10 months post-surgery. CONCLUSION Simple talonavicular arthrodesis combined with scaphoid stress adjustment has satisfactory clinical efficacy in Müller-Weiss disease in terms of deformity, foot length, foot function and pain.
Humans ; Male ; Female ; Adult ; Middle Aged ; Arthrodesis/methods* ; Aged ; Flatfoot/physiopathology* ; Tarsal Bones/physiopathology* ; Tarsal Joints/surgery* ; Treatment Outcome

OBJECTIVES: To investigate the clinical characteristics and prognosis of chronic disseminated candidiasis (CDC) in children with acute leukemia (AL) following chemotherapy. METHODS: A retrospective analysis was conducted on children diagnosed with CDC (including confirmed, clinically diagnosed, and suspected cases) after AL chemotherapy from January 2015 to December 2023 at Fujian Medical University Union Hospital, Zhangzhou Municipal Hospital, and Quanzhou First Hospital Affiliated to Fujian Medical University. Clinical characteristics and prognosis were analyzed. RESULTS: The incidence of CDC in children with AL following chemotherapy was 1.92% (32/1 668). Among the children with acute lymphoblastic leukemia, the incidence of CDC in the high-risk group was significantly higher than in the low-risk group (P=0.002). All patients presented with fever unresponsive to antibiotics during the neutropenic period, with 81% (26/32) involving the liver. C-reactive protein (CRP) levels were significantly elevated (≥50 mg/L) in 97% (31/32) of the patients. The efficacy of combined therapy with liposomal amphotericin B and caspofungin or posaconazole for CDC was 66% (19/29), higher than with caspofungin (9%, 2/22) or liposomal amphotericin B (18%, 2/11) monotherapy. The overall cure rate was 72% (23/32). The proportion of patients with CRP ≥50 mg/L and/or a positive β-D-glucan test for more than 2 weeks and breakthrough infections during caspofungin treatment was significantly higher in the treatment failure group compared to the successful treatment group (P<0.05). CONCLUSIONS CDC in children with AL after chemotherapy may be associated with prolonged neutropenia due to intensive chemotherapy. Combination antifungal regimens based on liposomal amphotericin B have a higher cure rate, while persistently high CRP levels and positive β-D-glucan tests may indicate poor prognosis.
Adolescent ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Antifungal Agents/therapeutic use* ; Candidiasis/diagnosis* ; Chronic Disease ; Leukemia/complications* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/complications* ; Prognosis ; Retrospective Studies

BACKGROUND: Our understanding of the correlation between postdischarge cancer and mortality in patients with coronary artery disease (CAD) remains incomplete. The aim of this study was to investigate the relationships between postdischarge cancers and all-cause mortality and cardiovascular mortality in CAD patients. METHODS: In this retrospective cohort study, 25% of CAD patients without prior cancer history who underwent coronary artery angiography between January 1, 2011 and December 31, 2015, were randomly enrolled using SPSS 26.0. Patients were monitored for the incidence of postdischarge cancer, which was defined as cancer diagnosed after the index hospitalization, survival status and cause of death. Cox regression analysis was used to explore the association between postdischarge cancer and all-cause mortality and cardiovascular mortality in CAD patients. RESULTS: A total of 4085 patients were included in the final analysis. During a median follow-up period of 8 years, 174 patients (4.3%) developed postdischarge cancer, and 343 patients (8.4%) died. A total of 173 patients died from cardiovascular diseases. Postdischarge cancer was associated with increased all-cause mortality risk (HR = 2.653, 95% CI: 1.727-4.076, P < 0.001) and cardiovascular mortality risk (HR = 2.756, 95% CI: 1.470-5.167, P = 0.002). Postdischarge lung cancer (HR = 5.497, 95% CI: 2.922-10.343, P < 0.001) and gastrointestinal cancer (HR = 1.984, 95% CI: 1.049-3.750, P = 0.035) were associated with all-cause mortality in CAD patients. Postdischarge lung cancer was significantly associated with cardiovascular death in CAD patients (HR = 4.979, 95% CI: 2.114-11.728, P < 0.001), and cardiovascular death was not significantly correlated with gastrointestinal cancer or other types of cancer. CONCLUSIONS Postdischarge cancer was associated with all-cause mortality and cardiovascular mortality in CAD patients. Compared with other cancers, postdischarge lung cancer had a more significant effect on all-cause mortality and cardiovascular mortality in CAD patients.

ObjectiveTo investigate the effect of mucin 1 (MUC1) on the proliferation and apoptosis of nasopharyngeal carcinoma (NPC) and its regulatory mechanism. MethodsThe 60 NPC and paired para-cancer normal tissues were collected from October 2020 to July 2021 in Quanzhou First Hospital. The expression of MUC1 was measured by real-time quantitative PCR (qPCR) in the patients with PNC. The 5-8F and HNE1 cells were transfected with siRNA control (si-control) or siRNA targeting MUC1 (si-MUC1). Cell proliferation was analyzed by cell counting kit-8 and colony formation assay, and apoptosis was analyzed by flow cytometry analysis in the 5-8F and HNE1 cells. The qPCR and ELISA were executed to analyze the levels of TNF-α and IL-6. Western blot was performed to measure the expression of MUC1, NF-кB and apoptosis-related proteins (Bax and Bcl-2). ResultsThe expression of MUC1 was up-regulated in the NPC tissues, and NPC patients with the high MUC1 expression were inclined to EBV infection, growth and metastasis of NPC. Loss of MUC1 restrained malignant features, including the proliferation and apoptosis, downregulated the expression of p-IкB、p-P65 and Bcl-2 and upregulated the expression of Bax in the NPC cells. ConclusionDownregulation of MUC1 restrained biological characteristics of malignancy, including cell proliferation and apoptosis, by inactivating NF-κB signaling pathway in NPC.

Objective: To analyze the clinical and molecular diagnostic status of Fanconi anemia (FA) in China. Methods: The General situation, clinical manifestations and chromosome breakage test and genetic test results of 107 pediatric FA cases registered in the Chinese Blood and Marrow Transplantation Registry Group (CBMTRG) and the Chinese Children Blood and Marrow Transplantation Registry Group (CCBMTRG) from August 2009 to January 2022 were analyzed retrospectively. Children with FANCA gene variants were divided into mild and severe groups based on the type of variant, and Wilcoxon-test was used to compare the phenotypic differences between groups. Results: Of the 176 registered FA patients, 69 (39.2%) cases were excluded due to lack of definitive genetic diagnosis results, and the remaining 107 children from 15 hospitals were included in the study, including 70 males and 37 females. The age at transplantation treatment were 6 (4, 9) years. The enrolled children were involved in 10 pathogenic genes, including 89 cases of FANCA gene, 7 cases of FANCG gene, 3 cases of FANCB gene, 2 cases of FANCE gene and 1 case each of FANCC, FANCD1, FANCD2, FANCF, FANCJ, and FANCN gene. Compound heterozygous or homozygous of loss-of-function variants account for 69.2% (72/104). Loss-of-function variants account for 79.2% (141/178) in FANCA gene variants, and 20.8% (37/178) were large exon deletions. Fifty-five children (51.4%) had chromosome breakage test records, with a positive rate of 81.8% (45/55). There were 172 congenital malformations in 80 children.Café-au-Lait spots (16.3%, 28/172), thumb deformities (16.3%,28/172), polydactyly (13.9%, 24/172), and short stature (12.2%, 21/172) were the most common congenital malformations in Chinese children with FA. No significant difference was found in the number of congenital malformations between children with severe (50 cases) and mild FANCA variants (26 cases) (Z=-1.33, P=0.185). Conclusions: FANCA gene is the main pathogenic gene in children with FA, where the detection of its exon deletion should be strengthened clinically. There were no phenotypic differences among children with different types of FANCA variants. Chromosome break test is helpful to determine the pathogenicity of variants, but its accuracy needs to be improved.
Male ; Female ; Humans ; Child ; Fanconi Anemia/genetics* ; Chromosome Breakage ; Retrospective Studies ; Exons ; China/epidemiology*

Cell Line, Tumor

Objective: To evaluate the safety, efficacy and tolerability of China-made sildenafil citrate (Jinge) in the treatment of ED. METHODS: We conducted a multi-center, randomized, double-blind and placebo-controlled clinical trial among 222 ED patients in five urological or andrological clinics of China. The patients were randomly assigned to receive sildenafil citrate (SC, n = 111) or placebo (n = 111) for 8 weeks. We obtained and analyzed the demographic and clinical characteristics of the patients, the scores of International Index of Erectile Function (IIEF), the success rate of sexual intercourse, and the incidence of adverse events. RESULTS: No statistically significant differences were found between the patients of the SC and those of the placebo group in the mean age (［47.2±11.32］ yr vs ［46.67±13.08］ yr, P>0.05), psychological etiology (27.93% vs 23.42%, P>0.05), organic etiology (21.62% vs 29.73%, P>0.05) or mixed etiology (50.45% vs 46.85%, P>0.05), nor in height, weight, nationality, or history of smoking, drinking or allergy. Compared with the placebo controls, the SC-treated patients showed significant increases in the excellence rate of effectiveness (29.91% vs 78.90%, P<0.01), success rate of sexual intercourse (29.16% vs 63.87%, P<0.01), and total effectiveness rate (34.58% vs 77.98%, P<0.01). The effectiveness rates on organic, psychogenic and mixed types ED were remarkably higher in the SC group (64.52%, 83.33%, and 82.14%) than in the placebo control (46.15%, 21.21%, and 25.00%) (P<0.01). Mild or temporary adverse events were observed in 32 cases in the SC group as compared with 13 in the placebo control. CONCLUSIONS China-made sildenafil citrate is an effective, safe and well-tolerated drug for ED of different etiologies in the Chinese population.
Aged ; China ; Coitus ; Double-Blind Method ; Drug Compounding ; Erectile Dysfunction ; drug therapy ; etiology ; Humans ; Male ; Phosphodiesterase 5 Inhibitors ; therapeutic use ; Sildenafil Citrate ; therapeutic use ; Smoking ; Treatment Outcome

Adult ; Carbolines ; Erectile Dysfunction ; drug therapy ; Humans ; Male ; Middle Aged ; Phosphodiesterase 5 Inhibitors ; therapeutic use ; Piperazines ; therapeutic use ; Purines ; therapeutic use ; Semen ; Sildenafil Citrate ; Specimen Handling ; Sulfones ; therapeutic use ; Tadalafil

BACKGROUNDA high mortality rate of pancreatic cancer becomes a bottleneck for further treatment with long-term efficacy. It is urgent to find a new mean to predict the early onset of pancreatic cancer accurately. The authors hypothesized that genetic variants of cationic trypsinogen (PRSS1) gene could affect trypsin expression/function and result in abnormal activation of protease activated receptor-2 (PAR-2), then lead to pancreatic cancer. The aim of this study was to elaborate some novel mutations of PRSS1 gene in the patients with pancreatic cancer.

METHODSTotally 156 patients with pancreatic cancer and 220 unrelated individuals as controls were enrolled in this study. The mutations of PRSS1 gene were analyzed by direct sequencing. K-ras Mutation Detection Kit was used to find the general k-ras gene disorder in the pancreatic cancer tissue. Then the clinical data were collected and analyzed simultaneously.

RESULTSThere were two patients who carried novel mutations which was IVS 3 + 157 G > C of PRSS1 gene in peripheral blood specimens and pancreatic cancer tissue. What's more, it was surprising to find a novel complicated mutation of exon 3 in PRSS1 gene (c.409 A > G and c.416 C > T) in another young patient. The complicated mutation made No. 135 and No. 137 amino acid transfer from Thr to Ala and Thr to Met respectively. No any mutation was found in the normal controls while no mutations of k-ras gene were detected in the three patients.

CONCLUSIONMutations of PRSS1 gene may be an important factor of pancreatic cancer.

Adult ; Asian Continental Ancestry Group ; Female ; Humans ; Male ; Mutation ; Pancreatic Neoplasms ; genetics ; Trypsin ; genetics

OBJECTIVETo investigate the methods of preoperative diagnosis and differentiation of different pathological tissue in middle ear and mastoid.

METHODSThe temporal bone lamellar CT findings in 106 patients with chronic suppurative otitis media (including cholesteatoma) were retrospectively analyzed. The CT value of pathological tissue were measured for 183 times and were compared with the surgical findings and postoperative pathological findings to definitude the CT value range of different pathological tissue. Sixty patients taken from 106 patients at random were analyzed and made the diagnosis again by the same doctor team according to the CT value of the different pathological tissue and surrounding histoclasia resulted by pathological tissue. The diagnose accordance rate was compared with the routine diagnose report from radiology department. The predetective diagnosis was made in 10 patients with chronic suppurative otitis media according to clinical manifestation (pathological changes of tympanic membrane, nature of otorrhea, character of hearing), temporal bone lamellar CT finding (CT value of pathological tissue, surrounding histoclasia) to validate the value of this study for preoperative diagnosis and differentiation of different pathological tissue in middle ear and mastoid.

RESULTSThe CT value of cholesteatoma, granulation tissue, cholesteatoma combined with granulation tissue, effusion, calcified tissue, thickened and polypoid membrane was respectively (46.6 +/- 10.3) Hu, (26.6 +/-7.4) Hu, (42.1 +/- 11.4) Hu, (- 24.6 +/- 9.2) Hu, (223.6 +/- 63.7) Hu, (23.8 +/- 8.5) Hu. The diagnose accordance rate in 60 patients who were analyzed and made diagnosis again according to the CT value of the different pathological tissue and surrounding histoclasia resulted by pathological tissue raised from 68. 3% to 81.7% ( P < 0.05) . The predetective diagnose accordance rate reached at 90% according to clinical manifestation, temporal bone lamellar CT.

CONCLUSIONSIt was not reliable to diagnose and differentially diagnose different pathological tissue in middle ear and mastoid only by the CT value, however, the CT value could still be considered to be a very significant information. The accurate rates of diagnosis and differentiation of different pathological tissue in middle ear and mastoid obviously raised by synthetically analyzing various kinds of pathological tissues in middle ear and mastoid according to clinical manifestation, temporal bone lamellar CT finding.

Adolescent ; Adult ; Aged ; Child ; Chronic Disease ; Female ; Humans ; Male ; Mastoid ; diagnostic imaging ; Middle Aged ; Otitis Media, Suppurative ; diagnostic imaging ; Retrospective Studies ; Temporal Bone ; diagnostic imaging ; Tomography, X-Ray Computed ; Young Adult