Pulmonary alveolar proteinosis(PAP)is a rare progressive respiratory dysfunction disease of the lung characterized by insidious onset and non-specific clinical manifestations,often leading to misdiagnosed and mistreated.Herein,we reported a case of PAP patient admitted to Jiading District Central Hospital with an atypical appearance of alveolar lavage fluid and whose condition improved significantly after treatment with subcutaneous injection of recombinant human granulocyte-macrophage colony stimulating factor(GM-CSF).Additionally,we have reviewed and summarized the relevant literature to enhance the understanding of the diagnosis and treatment of this disease.

Pulmonary alveolar proteinosis(PAP)is a rare progressive respiratory dysfunction disease of the lung characterized by insidious onset and non-specific clinical manifestations,often leading to misdiagnosed and mistreated.Herein,we reported a case of PAP patient admitted to Jiading District Central Hospital with an atypical appearance of alveolar lavage fluid and whose condition improved significantly after treatment with subcutaneous injection of recombinant human granulocyte-macrophage colony stimulating factor(GM-CSF).Additionally,we have reviewed and summarized the relevant literature to enhance the understanding of the diagnosis and treatment of this disease.

Background/Aims: The histological characteristics and natural history of precirrhotic primary biliary cholangitis (PBC) with portal hypertension (PH) are unclear. Our aim was to clarify the prevalence, risk factors, and histological characteristics of precirrhotic PBC patients with PH. Methods: This retrospective study compared the clinical features, histological characteristics, and response to ursodeoxycholic acid (UDCA) between the PH and non-PH groups of precirrhotic PBC patients. Results: Out of 165 precirrhotic PBC patients, 40 (24.2%) also had PH. According to histological stage 1, 2 and 3 disease, 5.3% (1/19), 17.3% (17/98), and 45.8% (22/48) of patients also had PH, respectively. Precirrhotic PBC with PH was significantly positively correlated with bile duct loss, degree of cytokeratin 7 positivity, and degree of fibrosis in the portal area, but significantly negatively correlated with lymphoid follicular aggregation. Compared to the non-PH group, patients in the PH group showed a higher prevalence of obliterative portal venopathy, incomplete septal fibrosis, portal tract abnormalities and non-zonal sinusoidal dilatation (p<0.05). In addition, patients with PH were more likely to present with symptoms of jaundice, ascites, epigastric discomfort, a poorer response to UDCA, and more decompensation events (p<0.05). High alkaline phosphatase levels, low white blood cell counts, high Mayo scores, and high FIB-4 index values were risk factors for precirrhotic PBC with PH. Conclusions Approximately 24.2% of precirrhotic PBC patients have PH, which is histologically related to the injury of bile ducts. High alkaline phosphatase levels, low white blood cell counts, high Mayo scores, and high FIB-4 index values are associated with increased risk of precirrhotic PBC with PH.

Mitral valve prolapse is one of the common causes of mitral regurgitation.Mitral valve prolapse complicated with leaflet cleft is rare in clinical practice,which most often undergo surgical mitral valve repair or mitral valve replacement.We report a case of mitral valve prolapse with posterior leaflet cleft treated by transcatheter edge-to-edge mitral valve repair,in order to provide a model for similar cases.

Objective To explore the clinical effect of reconstructing distal radioulnar ligament with al-lograft tendon under wrist arthroscopy in the treatment of chronic PalmerⅠD type triangular fibrocarti-lage complex injury complicated with distal radioulnar joint instability.Methods Clinical data of 15 pa-tients with chronic PalmerⅠD type triangular fibrocartilage complex injury and admitted to our hospital between January 2017 to January 2020 were retrospectively analyzed.All of them were treated with wrist arthroscopic allograft tendon reconstruction of distal radioulnar ligament.During the postoperative follow-up,the wrist range of motion and grip strength were evaluated,while the wrist pain and wrist function were assessed using the visual analogue scale(VAS)and modified Mayo score.Results All pa-tients were followed up twelve to twenty-four months,with an average of(18.15±2.20)months.The postoperative VAS score was(2.56±0.69),significantly better than the preoperative one(6.78±1.24)(P<0.05).Moreover,the postoperative ratio of grip strength between the affected and the healthy hand was(76.18±9.72)%,significantly better than that of(50.18±6.79)%(P<0.05),with increased grip strength.Meanwhile,the postoperative rotational motion of wrist joint[(151.09±12.38)°]improved significantly compared with that before operation[(142.87±10.19)°](P<0.05).According to the modi-fied Mayo score,8 cases were excellent,6 cases were good,and 1 case was fair,reaching an excel-lent and good rate of 93.33%.Conclusion Wrist arthroscopic reconstruction of distal radioulnar liga-ment with allograft tendon is effective in the treatment of chronic PalmerⅠD type triangular fibrocarti-lage complex injury with lower radioulnar joint instability,relieving pain and promoting grip strength and rotational motion.

OBJECTIVE: To investigate the effect of bone cement distribution on efficacy of residual back pain after percutaneous vertebra plasty(PVP). METHODS: From January 2017 to December 2020, a total of 65 cases with single segment osteoporotic thoracolumbar vertebral fractures underwent parallel vertebroplasty surgery. On the basis of the postoperative X-ray films of bone cement distribution were divided into two groups. The bone cement was biased to the lateral side of the vertebral body (partial group, 20 cases), there were 9 males and 11 famales with an average age of (70.3±7.4) years old ranging from 60 to 84 years old. The bone cement was over the vertebral midline, and completely filled with contralateral vertebral body (bilateral group, 45 cases), there were 10 males and 35 famales with an average age of (70.7±8.0) years old ranging from 60 to 86 years old. All of them underwent PVP surgery, bone cement was injected into the vertebral body through paitail transpedicular approach. The amount of bone cement injection, the visual analogue scale(VAS) of preoperation and 1 day, 1 month, 3 months after surgery between two groups were observed and compared. RESULTS: The amount of cement injection was (4.25±0.99) ml in the partial group, and (4.07±1.18) ml in the bilateral group, there was no significant difference between two groups (P>0.05). Postoperative pain was relieved than preoperative pain (P<0.05), the VAS of 1 day, 1 and 3 months after operation (3.90±1.37), (2.35±0.67) and (1.55±0.51) in the partial group were higher than (2.67±0.60), (1.62±0.58) and (1.31±0.47) in the bilateral group (P<0.05). There were 9 cases in partial group, the pain was not relieved due to unfilled cement until the contralateral bone was injected into the bone cement. CONCLUSION The distribution of bone cement is one of the main factors affecting residual back pain after PVP, and in the clinical, we should make sure the distribution of bone cement over the midline of vertebral body.
Humans ; Bone Cements ; Aged ; Male ; Female ; Middle Aged ; Aged, 80 and over ; Vertebroplasty/methods* ; Back Pain/etiology* ; Lumbar Vertebrae/surgery* ; Spinal Fractures/surgery* ; Thoracic Vertebrae/surgery*

Objective:To investigate the efficacy of arthroscope-assisted reduction and anchor fixation in the treatment of old ununited fractures of the ulnar styloid process combined with triangular fibrocartilage complex (TFCC) injury.Methods:A retrospective case series study was conducted to analyze the clinical data of 30 patients with old ununited fractures of the ulnar styloid process combined with TFCC injury (with pressing pain of the ulnar wrist and decreased grip strength) admitted to Sichuan Provincial Orthopedic Hospital from June 2018 to June 2023, including 18 males and 12 females, aged 40-54 years [(47.6±5.4)years]. All the patients had Hauck type II fractures. A total number of 17 patients had fractures on the left side and 13 on the right side. Arthroscope-assisted reduction and anchor fixation of the ulnar styloid process were performed and the injured TFCC was repaired simultaneously. The visual analogue scale (VAS) scores for ulnar-sided wrist pain before surgery, at 3 and 6 months after surgery, and at the last follow-up were compared. Anteroposterior and lateral X-rays of the wrist joint were taken at 1-6 months after surgery to observe the fracture healing. The ranges of motion of wrist flexion and extension, wrist radial-ulnar deviation, and forearm rotation, Cooney wrist function scores, and grip strengths before surgery, at 3 and 6 months after surgery, and at the last follow-up were compared. The occurrence of intraoperative and postoperative complications was observed.Results:All the patients were followed up for 12-16 months [(13.5±2.3)months]. At 3, 6 months after surgery and at the last follow-up, the VAS scores for ulnar-sided wrist pain were (3.1±0.8)points, (1.4±0.8)points, and (1.2±0.5)points respectively, which were all lower than (7.1±0.8)points before surgery ( P<0.05). The VAS scores at 6 months after surgery and at the last follow-up were lower than that at 3 months after surgery ( P<0.05), while there were no significant difference between the scores at 6 months after surgery and at the last follow-up ( P>0.05). Fractures healed well in the 28 patients, with the healing time of 3-4 months [(3.0±1.3)months], while 2 patients had delayed union. There were no significant differences in the ranges of motion of wrist flexion and extension or radial-ulnar deviation before surgery, at 3 and 6 months after surgery, and at the last follow-up ( P>0.05). At 3, 6 months after surgery and at the last follow-up, the ranges of motion of forearm rotation were (149.9±10.8)°, (158.6±10.5)°, and (159.0±11.1)° respectively, which were all greater than (131.7±11.0)° before surgery. The ranges of motion of forearm rotation were greater at 6 months after surgery and at the last follow-up than that at 3 months after surgery ( P<0.05), while there were no significant difference between the angles at 6 months after surgery and at the last follow-up ( P>0.05). At 3, 6 months after surgery and at the last follow-up, the Cooney wrist function scores were (51.7±5.8)points, (74.8±6.2)points, and (84.3±6.6)points respectively, which were all higher than (39.8±6.1)points before surgery ( P<0.05), and the scores gradually increased at each time point after surgery ( P<0.05). At the last follow-up, the Cooney wrist function scores of 13 patients were evaluated as excellent, 15 good, and 2 fair, with an excellent and good rate of 93.3%. At 3, 6 months after surgery and at the last follow-up, the grip strengths of the affected side were (21.6±2.5)kg, (36.6±2.5)kg, and (45.7±5.8)kg respectively, which were all greater than (14.6±2.5)kg before surgery ( P<0.05), and the grip strengths gradually increased at each time point after surgery ( P<0.05). No bone fragment fragmentation occurred during the surgery. No internal fixation failure occurred after the surgery, and there was no secondary ulnar tissue irritation or injury to the dorsal branch of the ulnar nerve. Conclusion:Arthroscope-assisted reduction and anchor fixation in the treatment of old ununited fractures of the ulnar styloid process combined with TFCC injury has advantages of good bone and soft tissue healing, relieved wrist pain, increased grip strength, improved wrist rotation range and function, and fewer complications.

Objective:To investigate the efficacy of arthroscope-assisted reduction and anchor fixation in the treatment of old ununited fractures of the ulnar styloid process combined with triangular fibrocartilage complex (TFCC) injury.Methods:A retrospective case series study was conducted to analyze the clinical data of 30 patients with old ununited fractures of the ulnar styloid process combined with TFCC injury (with pressing pain of the ulnar wrist and decreased grip strength) admitted to Sichuan Provincial Orthopedic Hospital from June 2018 to June 2023, including 18 males and 12 females, aged 40-54 years [(47.6±5.4)years]. All the patients had Hauck type II fractures. A total number of 17 patients had fractures on the left side and 13 on the right side. Arthroscope-assisted reduction and anchor fixation of the ulnar styloid process were performed and the injured TFCC was repaired simultaneously. The visual analogue scale (VAS) scores for ulnar-sided wrist pain before surgery, at 3 and 6 months after surgery, and at the last follow-up were compared. Anteroposterior and lateral X-rays of the wrist joint were taken at 1-6 months after surgery to observe the fracture healing. The ranges of motion of wrist flexion and extension, wrist radial-ulnar deviation, and forearm rotation, Cooney wrist function scores, and grip strengths before surgery, at 3 and 6 months after surgery, and at the last follow-up were compared. The occurrence of intraoperative and postoperative complications was observed.Results:All the patients were followed up for 12-16 months [(13.5±2.3)months]. At 3, 6 months after surgery and at the last follow-up, the VAS scores for ulnar-sided wrist pain were (3.1±0.8)points, (1.4±0.8)points, and (1.2±0.5)points respectively, which were all lower than (7.1±0.8)points before surgery ( P<0.05). The VAS scores at 6 months after surgery and at the last follow-up were lower than that at 3 months after surgery ( P<0.05), while there were no significant difference between the scores at 6 months after surgery and at the last follow-up ( P>0.05). Fractures healed well in the 28 patients, with the healing time of 3-4 months [(3.0±1.3)months], while 2 patients had delayed union. There were no significant differences in the ranges of motion of wrist flexion and extension or radial-ulnar deviation before surgery, at 3 and 6 months after surgery, and at the last follow-up ( P>0.05). At 3, 6 months after surgery and at the last follow-up, the ranges of motion of forearm rotation were (149.9±10.8)°, (158.6±10.5)°, and (159.0±11.1)° respectively, which were all greater than (131.7±11.0)° before surgery. The ranges of motion of forearm rotation were greater at 6 months after surgery and at the last follow-up than that at 3 months after surgery ( P<0.05), while there were no significant difference between the angles at 6 months after surgery and at the last follow-up ( P>0.05). At 3, 6 months after surgery and at the last follow-up, the Cooney wrist function scores were (51.7±5.8)points, (74.8±6.2)points, and (84.3±6.6)points respectively, which were all higher than (39.8±6.1)points before surgery ( P<0.05), and the scores gradually increased at each time point after surgery ( P<0.05). At the last follow-up, the Cooney wrist function scores of 13 patients were evaluated as excellent, 15 good, and 2 fair, with an excellent and good rate of 93.3%. At 3, 6 months after surgery and at the last follow-up, the grip strengths of the affected side were (21.6±2.5)kg, (36.6±2.5)kg, and (45.7±5.8)kg respectively, which were all greater than (14.6±2.5)kg before surgery ( P<0.05), and the grip strengths gradually increased at each time point after surgery ( P<0.05). No bone fragment fragmentation occurred during the surgery. No internal fixation failure occurred after the surgery, and there was no secondary ulnar tissue irritation or injury to the dorsal branch of the ulnar nerve. Conclusion:Arthroscope-assisted reduction and anchor fixation in the treatment of old ununited fractures of the ulnar styloid process combined with TFCC injury has advantages of good bone and soft tissue healing, relieved wrist pain, increased grip strength, improved wrist rotation range and function, and fewer complications.

This study aimed to investigate the anti-fatigue effect and mechanism of Lubian(Cervi Penis et Testis) on kidney Yin deficiency and kidney Yang deficiency mice. After one week of adaptive feeding, 88 healthy male Kunming mice were randomly divided into a blank group, a kidney Yin deficiency model group, a kidney Yin deficiency-Panacis Quinquefolii Radix(PQR) group, kidney Yin deficiency-Lubian treatment groups, a kidney Yang deficiency model group, a kidney Yang deficiency-Ginseng Radix et Rhizoma(GR) group, and kidney Yang deficiency-Lubian treatment groups, with eight mice in each group. The kidney Yin deficiency model and kidney Yang deficiency model were prepared by daily regular oral administration of dexamethasone acetate and hydrocortisone, respectively, and meanwhile, corresponding drugs were provided. The mice in the blank group received blank reagent. The treatment lasted 14 days. The exhaustive swimming time was measured 30 min after drug administration on the 14th day. On the 15th day, blood was collected from eyeballs and the serum was separated to determine the content of lactic acid(LD), blood urea nitrogen(BUN), lactate dehydrogenase(LDH), cyclic adenosine monophosphate(cAMP), and cyclic guanosine monophosphate(cGMP). The liver was dissected to determine the content of liver glycogen and the protein expression of phosphoinositide 3-kinase(PI3K) and protein kinase B(Akt). Compared with the kidney Yang deficiency model group, the kidney Yang deficiency-Lubian treatment groups showed increased body weight(P<0.05), relieved symptoms of Yang deficiency, decreased cGMP content(P<0.01), increased cAMP/cGMP(P<0.01), prolonged exhausted swimming time(P<0.01), reduced LD(P<0.01), elevated BUN content(P<0.01), increased liver glycogen content(P<0.01), and increased protein expression of PI3K and Akt in the liver(P<0.05). Compared with the kidney Yin deficiency model group, the kidney Yin deficiency-Lubian treatment groups showed increased body weight(P<0.01), relieved symptoms of Yin deficiency, increased content of cGMP(P<0.01), decreased cAMP/cGMP(P<0.01), prolonged exhausted swimming time(P<0.01), decreased LD(P<0.01), decreased BUN content(P<0.01), increased liver glycogen content(P<0.01), and increased protein expression of PI3K(P<0.05) and Akt in the liver(P<0.05). To sum up, Lubian can regulate Yin deficiency and Yang deficiency and increase glycogen synthesis by affecting the PI3K-Akt pathway, thereby exerting an anti-fatigue role.
Male ; Mice ; Animals ; Phosphatidylinositol 3-Kinases/genetics* ; Proto-Oncogene Proteins c-akt/genetics* ; Liver Glycogen ; Yang Deficiency/drug therapy* ; Yin Deficiency/drug therapy* ; Kidney ; Body Weight

OBJECTIVE: This study was aimed at investigating the carrier rate of, and molecular variation in, α- and β-globin gene mutations in Hunan Province. METHODS: We recruited 25,946 individuals attending premarital screening from 42 districts and counties in all 14 cities of Hunan Province. Hematological screening was performed, and molecular parameters were assessed. RESULTS: The overall carrier rate of thalassemia was 7.1%, including 4.83% for α-thalassemia, 2.15% for β-thalassemia, and 0.12% for both α- and β-thalassemia. The highest carrier rate of thalassemia was in Yongzhou (14.57%). The most abundant genotype of α-thalassemia and β-thalassemia was -α ^3.7/αα (50.23%) and β ^IVS-II-654/β ^N (28.23%), respectively. Four α-globin mutations [CD108 (ACC>AAC), CAP +29 (G>C), Hb Agrinio and Hb Cervantes] and six β-globin mutations [CAP +8 (C>T), IVS-II-848 (C>T), -56 (G>C), beta nt-77 (G>C), codon 20/21 (-TGGA) and Hb Knossos] had not previously been identified in China. Furthermore, this study provides the first report of the carrier rates of abnormal hemoglobin variants and α-globin triplication in Hunan Province, which were 0.49% and 1.99%, respectively. CONCLUSION Our study demonstrates the high complexity and diversity of thalassemia gene mutations in the Hunan population. The results should facilitate genetic counselling and the prevention of severe thalassemia in this region.
Humans ; beta-Thalassemia/genetics* ; alpha-Thalassemia/genetics* ; Hemoglobinopathies/genetics* ; China/epidemiology* ; High-Throughput Nucleotide Sequencing