[Objective]To explore the application of the intramedullary nails for correction of deformity in the lower limbs and decrease the opportunity of refracture in children with osteogenesis imperfecta.[Method]From Jan.2000 to Aug.2004,8 patients(3 males and 5 females),with 13 femurs and 3 tibias totally,with deformity in lower limps were corrected by multi-osteotomy and fixed with intramedullary nails,2 for K-wires;3 for Ender nails;and 3 for flexible intramedullary nails.[Result]All deformities were perfectly corrected.All patients were available at final follow up,for 9 months to 60 months,mean 30 months.None of the complications occurred postoperatively,such as infection in the incision or pin tract,nonunion,and refracture.One patient can walk by a stick from wheel-chair help preoperatively and one K-wire was disrupted near the end.[Conclusion]Multi-osteotomy and fixed intramedullary nails,can correct the deformity in the lower limbs perfectly and decrease the opportunity of refracture in children with osteogenesis imperfecta,which has been proved to be a reliable method.

Objective :To observe the effects of iiiac crest composite free flap (ICCFF) in the reconstruction of mandibular defects. Methods:Mandibular defects were reconstructed with ICCFF in 5 cases. "Window flap" was used to monitor the blood supply of ICCFF. Follow-up was carried out for 1 year and the effects of reconstructionwere evaluated by general observation and radiography. Results:Satisfactery face contour and bony healing of the grafts with the adjacent bone bed were observed in all the cases. Conclusion: ICCFF can be used to reconstruct large defects in mandible and the "window flap"can be used to moniter the blood supply of bone grafts.

Objective To explore the clinical effect of Habib 4X bipolar radiofrequency ablation assisted laparoscopic partial nephrectomy without blockage of renal artery for treatment of renal cell carcinoma (RCC).MethodsBetween October 2010 and June 2011, 14 patients with exophytic RCC (T1N0M0) were treated with Laparoscopic Partial Nephrectomy with (LRA) or without (LPN) Habib 4X Bipolar Radiofrequency ablation assisted.The LRA group included 6 patients,and the maximum tumor diameter was 2.1 - 3.5 cm (mean,3.1 cm).The LPN group included 8 patients,and the maximum tumor diameter was 2.0 -3.7 cm (mean,3.0 cm).The operative time,average intra-operative blood loss,postoperative hospital stay and incidence of postoperative complications were recorded.Changes of hemoglobin (Hb),serum creatinine (SCr) and ipsilateral glomerular filtration rate (GFR) before and after operation were analyzed and compared. Results The 14 surgical procedures were successful without conversion to open operation.In LRA group,renal artery blocking was unnecessary,the Hb,SCr and GFR before and after operation were (127 ± 19) g/L vs.(124 ± 19) g/L、(96 ±39) μmol/L vs.(92 ±29) μmol/L and (42 ± 12) ml/min vs.(40 ± 13) ml/min,respectively,and the difference was not significant (P ＞0.05).In LPN group,the renal artery blocking time was 20-52 min (mean,32 min),the Hb,SCr and GFR before and after operations were (130±17) g/L vs.(112±15) g/L,(92 ±31) μ mol/L vs.(110±28)μmol/L and (43 ± 14) ml/min vs.(30 ± 11 ) ml/min respectively,and the difference was significant (P ＜0.05 ).The operative time was ( 86 ± 20) min and ( 112 ± 43 ) min,the estimated blood loss was (94 ± 18)ml and (256 ± 58) ml,and the postoperative hospital stay was (5.0 ± 0.8) d and (7.8 ± 1.2 ) d,respectively.The difference between the two groups was significant (P ＜ O.05).Complications such as bleeding,gross hematuria,and leakage of urine were noted in LRA group. Conclusions Habib 4X Bipolar Radiofrequency ablation assisted LPN without blockage of renal artery is a safe and effective intervention with a relatively low incidence of complications.And it is superior to LPN in operative time,intraoperative blood loss and postoperative recovery.

Objective The aim of this study was to investi-gate the changes of brain function in patients with drug-naive idiopath-ic epilepsy ( DNIE ) using resting-state functional MRI ( rs-fMRI ) amplitude of low-frequency fluctuation ( ALFF) , analyze the correlation of abnormal brain regions with the clinical variable ( disease course) , and gain a deeper insight into the pathophysiological mechanisms of idiopathic epilepsy. Methods This study included 25 cases of DNIE (15 males and 10 females) and 34 cases of drug idiopathic epilepsy (DIE, 22 males and 12 females).Another 25 healthy volunteers matched with the DNIE patients in sex, age, education and handedness were recruited as normal controls.The rs-fMRI data obtained from all the subjects were processed, subjected to ALFF analysis, and compared among the DNIE, DIE, and nor-mal control groups.The correlation was evaluated between the ALFF statistical brain mapping and the course of disease. Results Obvious differences were found in ALFF among the DNIE, DIE and control subjects.Compared with the normal controls, the DNIE pa-tients showed increased ALFF in the right inferior temporal gyrus, right lingual gyrus and right cuneus, but decreased ALFF in the right insula, left hippocampus, right midbrain, right middle frontal gyrus, left anterior cingulated gyrus, left middle cingulate gyrus and right inferior parietal lobule.In comparison with the DIE patients, those of the DNIE group exhibited increased ALFF in the left inferior occipital gyrus, right middle occipital gyrus and left middle occipital gyrus, but decreased ALFF in the right inferior frontal gyrus, left insula, right superior temporal gyrus and right middle frontal gyrus.In the DNIE patients, the disease course was found to be correlated positively with ALFF in the right cerebellum posterior lobe, left cerebellar tonsil, right lingual gyrus, left orbital gyrus, left middle oc-cipital gyrus, left corpus callosum, left caudate nuclear, left superior frontal gyrus, left medial frontal gyrus, right precuneus and left middle frontal gyrus, but negatively with ALFF in the right parahippocampal, right superior temporal gyrus, left superior temporal gyrus and right post-central gyrus. Conclusion The ALFF of resting-state cerebral function is abnormal in DNIE patients.The correlation between ALFF and the clinical variable ( disease course) provides a new insight into the pathophysiological mechanisms of epilepsy.

Poly(ADP-ribose)polymerase-1(PARP-1)plays a vital role in the regulation of DNA repair and apoptosis. Breakthrough has been made in the treatment of cancer with PARP-1 inhibitors, but the emergence of drug resistance has limited its further application in clinic. This paper reviews advances in the research on PARP-1 inhibitors combined with other drugs to overcome drug resistance, highlights and evaluates the existing drug combination strategies and their therapeutic effects in clinical practice. It is proposed that the development of dual-target or multi-target drugs will become a promising approach to overcome the resistance of PARP-1 inhibitors and broaden their indications.

OBJECTIVES: Tubulointerstitial diseases is one of the common causes of renal dysfunction. Some rare pathological types are easy to be misdiagnosed and missedly diagnosed because of their low prevalence and relatively insufficient understanding, which affects the treatment and prognosis of patients. This study aims to explore clinical manifestations and pathological characteristics of several rare tubulointerstitial diseases, and therefore to improve their diagnosis and treatment. METHODS: A total of 9 363 patients diagnosed by renal biopsy in the Department of Nephrology, Second Xiangya Hospital, Central South University from November 2011 to September 2021 were selected. Six cases of light chain cast nephropathy (LCCN), 2 cases of light chain proximal tubulopathy (LCPT), 1 case of LCCN with LCPT, 4 cases of genetic tubulointerstitial disease, and 6 cases of non-genetic related tubulointerstitial lesion were screened out, and their clinical manifestations and renal biopsy pathological results were collected, compared, and analyzed. RESULTS: Patients with LCCN presented with mild to moderate anemia, microscopic hematuria, and mild to moderate proteinuria. Compared with patients with LCPT, proteinuria and anemia were more prominent in patients with LCCN. Five patients with LCCN and 2 patients with LCPT had elevated serum free kappa light chain. Five patients with LCCN presented clinically with acute kidney injury (AKI). Two patients with LCPT and 1 patient with LCCN and LCPT showed CKD combined with AKI, and 1 LCPT patient presented with typical Fanconi syndrome (FS). Five patients with LCCN, 2 patients with LCPT, and 1 patient with LCCN and LCPT were diagnosed with multiple myeloma. Five patients with LCCN had kappa light chain restriction in tubules on immunofluorescence and a "fractured" protein casts with pale periodic acid-Schiff (PAS) staining on light microscopy. Immunohistochemical staining of 2 LCPT patients showed strongly positive kappa light chain staining in the proximal tubular epithelial cells. And monoclonal light chain crystals in crystalline LCPT and abnormal lysosomes and different morphological inclusion bodies in noncrystalline LCPT were observed under the electron microscope. Six patients with LCCN were mainly treated by chemotherapy. Renal function was deteriorated in 1 patient, was stable in 4 patients, and was improved in 1 patient. Two patients with LCPT improved their renal function after chemotherapy. Four patients with genetic tubulointerstitial disease were clinically presented as CKD, mostly mild proteinuria, with or without microscopic hematuria, and also presented with hyperuricemia, urine glucose under normal blood glucose, anemia, polycystic kidneys. Only 1 case had a clear family history, and the diagnosis was mainly based on renal pathological characteristics and genetic testing. Compared with patients with non-genetic related tubulointerstitial lesion, patients with genetic tubulointerstitial disease had an earlier age of onset, higher blood uric acid, lower Hb and estiated glomemlar fitration (eGFR), and less edema and hypertension. Renal pathology of genetic tubulointerstitial disease presented tubular atrophy and interstitial fibrosis, abnormal tubular dilation, glomerular capsuledilation, and glomerular capillary loop shrinkage. Glomerular dysplasia and varying degrees of glomerular sclerosis were observed. Genetic tubulointerstitial disease patients were mainly treated with enteral dialysis, hypouricemic and hypoglycemic treatment. Two genetic tubulointerstitial disease patients had significantly deteriorated renal function, and 2 patients had stable renal function. CONCLUSIONS Patients with AKI or FS, who present serum immunofixation electrophoresis and/or serum free kappa light chain abnormalities, should be alert to LCCN or LCPT. Renal biopsy is a critical detection for diagnosis of LCCN and LCPT. Chemotherapy and stem cell transplantation could delay progression of renal function in patients with LCCN and LCPT. If the non-atrophic area of the renal interstitium presents glomerular capsule dilatation, glomerular capillary loop shrinkage, and abnormal tubular dilatation under the light microscopy, genetic tubulointerstitial disease might be considered, which should be traced to family history and can be diagnosed by genetic testing.
Humans ; Hematuria ; Immunoglobulin Light Chains/analysis* ; Multiple Myeloma ; Proteinuria ; Nephritis, Interstitial ; Acute Kidney Injury ; Anemia ; Renal Insufficiency, Chronic