Objective To report the outcome of emergency repair of severe complex defect in forearm by transplantation of free flap and simultaneous functional reconstruction.Methods From Mar.1994 to Aug.2003,4 cases with severe complex defect in forearm was repaired by transplantation of free skin flap, free skin flap combined with fibula flap,or fibula osteocutaneous flap in emergency.Simultaneously the flexion and extension function were repaired by muscle transfer and/or tendon grafting,tenonectomy.Results All the cases were successful.Follow-up period ranged from 1 to 3 years postoperatively.The blood-supply,tex- ture and elasticity of transferred flaps were excellent with good bone healing.Opposition of thumb with four fin- gers was good.Sensory recovery of the hand was satisfactory.Conclusion Transplantation of free flap com- bined with simultaneous functional reconstruction is an ideal method in emergency repair of severe complex de- fect in forearm.

OBJECTIVETo investigate the inhibitory effect of lactic acid on semen-derived amyloid (SEVI) fibril formation.

METHODSPAP248-286 (2 mg/mL) was incubated with 4.0, 2.0, 1.0, 0.5, 0.25, and 0.125 mg/mL of lactic acid. After incubation for different times, aliquots were drawn from each sample for Thioflavin T (ThT) and Congo red staining to monitor semen-derived amyloid fibril formation. The β sheet structure formation of PAP248-286 was measured by circular dichroism spectrum, and the morphology of amyloid fibrils incubated with or without lactic acid was observed with transmission electron microscopy (TEM). The enhancing effect of amyloid fibril incubated with lactic acid at different time points was determined using virus infection assay. PAP248-286 (2 mg/mL) was incubated with dilutions of vaginal secretion from healthy women, and amyloid fibril formation was detected with ThT and Congo red staining.

RESULTSLactic acid inhibited SEVI fibril formation in a dose-dependent manner in vitro. Lactic acid at 0.5 mg/mL completely inhibited 2 mg/mL SEVI fibril formation within 48 h. After incubation for 48 h, lactic acid at 1 mg/mL inhibited the formation of β-sheet structure of SEVI (2 mg/mL) and completely inhibited 2 mg/mL PAP248-286 aggregation as observed with TEM. In the presence of lactic acid, PAP248-286 lost the ability to enhance virus infection. Vaginal secretion inhibited SEVI fibril formation in a dose-dependent manner, and virtually no SEVI fibril occurred after incubation of 2 mg/mL PAP248-286 with 67% vaginal secretion.

CONCLUSIONLactic acid inhibits SEVI fibril formation in vitro.

Objective To explore the clinical characteristics of pancreatic gland damage in children with Henoch-Sch(o)nlein purpura(HSP).Methods The serum and urine analysis of 95 examples were detected by the automatic biochemical analyzer,which were diagnosed as HSP from Aug.2009 to Jun.2010 in Department of Nephrology,Hunan Children's Hospital,and the clinical characteristics of them were analyzed.All children were treated with anti-infection and anti-allergic drugs.The pancreatic morphology of patients was observed by B ultrasonic,and the clinical features of skin,joints,digestive tract,and kidney damage were observed.And the relationship between damage of pancreas and damage of other systems was analyzed.Results There were 64 cases suffering from pancreatic gland damage in 95 HSP children:34 cases were male(53.1％),30 cases were female(46.9％),and there was no significant difference (x2 =0.56,P ＞ 0.05).The incidence of pancreatic gland damage of the patients with allergic purpura combined with abdominalgia was 82.8％ (53 cases),which was obviously higher than that in the patients without abdominalgia (11 cases,17.2％)(x2 =14.24,P ＜0.05).The incidence of pancreatic gland damage in mixed type of allergic purpura(61 cases,95.3％) was obviously higher than that in the patients which only possess the skin rash(3 cases,4.7％) (x2 =18.18,P ＜0.05).The pancreatic glands of the total 64 patients were detected by type B ultrasonic.None of them had been detected with pancreatic gland edema and deformation of structure.The mean hospital stay of the HSP patients with pancreatic gland damage was (10.80 ± 6.39) days (5-39 days),while mean hospital stay without pancreatic gland damage was (8.42 ± 3.51) days (4-13 days),and there was no significant difference between them (t =5.68,P ＞ 0.05).Conclusions HSP children usually were accompanied with pancreatic gland damage,and if they get abdominalgia and multi-system damage they are more likely to be accompanied with pancreatic gland damage,which should be paid attention to.

Objective To analyze the clinical and pathological characteristics of children in different stages and to investigate the clinical significance based on clinical diagnosis and staging criteria of acute kidney injury(AKI).Methods Based on the clinical diagnosis and staging criteria of AKI,165 AKI children admitted to the Department of Nephrology of Hunan Children's Hospital,between Oct.2004 and Oct.2011 were divided into 3 groups:stage 1,stage 2 and stage 3.Clinical characteristics,age,etiology,pathology and prognosis were compared among 3 groups of children with AKI.Results (1) A total of 165 patients(109 males and 56 females) were included in this study,average age of (6.26 ± 4.43) years,including 69 patients in stage 1,19 patients in stage 2,and 77 patients in stage 3.(2) The 3 groups of patients had an average age of(9.09 ± 3.69) years,(4.34 ± 3.90) years,and (4.22 ± 3.78) years,respectively,which showed significant differences (P ＜ 0.01).(3)Three most frequent causes of AKI were drugs (24.8％),acute glomerulonephritis (AGN) (22.4％) and septicemia (15.2 ％),which showed significant differences (P ＜ 0.01).(4) Renal histopathological examination was performed on 140 AKI children,3 most main types of pathology were acute tubular interstitial nephritis 56 cases (40.0％),endocapillary proliferative glomerulonephritis 33 cases (23.6％) and mesangial proliferative glomerulonephritis 18 cases(12.9％).In the stage 1 patients,glomerular disease was predominant(84.4％).In the stage 2 patients,glomerular disease(38.5％) and tubulointerstitial lesions(38.5％) were the major pathological types.In the stage 3 patients,tubulointerstitial damage (73.0％) was the major pathological type (P ＜0.01).The patients in 3 different groups showed significant differences in the ratio of tubulointerstitial disease and glomerular disease(P ＜0.01).(5)The median recovery time of serum creatinine to the baseline was 9 days(3-41 days) for stage 1 patients,11 days(3-25 days) for stage 2 patients,and 16 days(3-∝ days) for stage 3 patients,which showed a significant difference (P ＜ 0.05).(6)Of the 165 A KI patients,124 cases had hematuria,126 cases had varying degrees of proteinuria.There were significant differences in the hematuria incidence and the duration among the 3 groups(P ＜ 0.01).There was no difference in the proteinuria incidence among the 3 groups (P ＞ 0.05),while the stage 1 patients showed significant differences from the other 2 groups (P ＜ 0.01).Conclusions The patients are mostly seen in stage 1 and stage 3.The stage 1 AKI children are largely school-age children and acute glomerulone phritis is the main etiology.The stage 3 AKI children are mainly infants and the etiology of AKI is mainly drugs and septicemia,the pathological type is mainly acute tubulointerstitial nephritis,and the renal functional recovery is slow.

OBJECTIVETo study the significance of trace immunoglobulin M (IgM) deposits in glomerular mesangium in children with minimal change primary nephrotic syndrome (PNS).

METHODSOne hundred and six children who were clinically diagnosed with PNS and pathologically diagnosed with minimal change disease (MCD) and trace deposition of IgM in renal tissues were enrolled as subjects. Eighty-one PNS children with MCD but no deposition of immune complexes were used as the control group. The clinical characteristics and efficacies of glucocorticoids and immunosuppressants were retrospectively analyzed in the two groups. All patients were given full-dose prednisone by oral administration, and patients with glucocorticoid resistance or frequent relapses were additionally given immunosuppressants.

RESULTSThe incidence of glucocorticoid resistance in the IgM deposit group was significantly higher than that in the control group (27.2% vs 12.3%; P<0.05). The incidence of frequent relapses in the IgM deposit group was also significantly higher than that in the control group (48.1% vs 10.4%; P<0.05). The complete remission rate for glucocorticoid-resistant patients treated with prednisone combined with mycophenolate mofetil (MMF) was 68% and 62% respectively in the IgM deposit and control groups (P>0.05). The relapse frequency in patients with frequent relapses was significantly reduced in both groups after treatment with prednisone and MMF in combination (P<0.05).

CONCLUSIONSTrace deposition of IgM in renal tissues may be an important factor for glucocorticoid resistance and frequent relapses in PNS children with MCD. Prednisone combined with MMF may be a better choice in the treatment of patients with glucocorticoid resistance or frequent relapses.

Adolescent ; Child ; Child, Preschool ; Drug Resistance ; Female ; Glomerular Mesangium ; immunology ; Glucocorticoids ; therapeutic use ; Humans ; Immunoglobulin M ; analysis ; Immunosuppressive Agents ; therapeutic use ; Infant ; Male ; Nephrosis, Lipoid ; drug therapy ; immunology ; Retrospective Studies

OBJECTIVETo study clinical features of 3 children who presented with nephrotic syndrome (NS) associated with ichthyosis vulgaris (IV), and to detect relationship between NS associated with IV in patients and FLG gene or NPHS2 gene.

METHODClinical and kidney pathological data of the 3 patients were analyzed and progress of pathologic damage in the patient kidney was observed through repeated percutaneous renal biopsy. Using polymerase chain reaction-single strand conformation polymorphism and DNA sequencing, the diversity of the expression of NPHS2 gene in the 3 patients were analyzed, and FLG gene in the 3 patients and parts of their family members with IV was detected.

RESULT(1) The age of the 3 patients (patient 1 was a girl and patients 2 and 3 were boys) suffering from NS was 3 years and 8 months, 2 years and 6 months, and 5 years and 3 months, respectively. The age of onset of IV was 1 year and 6 months, 10 months, and 2 years and 6 months, respectively. All the 3 patients were resistant to steroid therapy. Despite multi-immunosuppressive therapy, no clinical response was achieved. The patients were followed up for 1.5 to 4.0 years. The patients displayed continuous proteinuria, renal function was normal, but their heights were lower than other children at the same age. (2) The older brother of patient 1 died of uremia. The other patients' family members did not have kidney disease. (3) Renal histopathology showed that the patients 1 and 2 had mild mesangial proliferative glomerulonephritis (MsPGN) and the patient 3 had minimal change disease (MCD). One and a half years after the first renal biopsy, the patients 1 and 2 underwent repeated renal biopsy. Renal histopathology showed that the 2 patients' disease developed to medium MsPGN. (4) None of the 3 patients had NPHS2 gene mutation. All the three patients had R501X and 2282del4 which are the common gene mutation type of the FLG, and all the patients were heterozygote. With the detection of the FLG gene of the part of the patients of the three families, the second patient's grandfather had the R501X homozygote mutation and the others were the R501X heterozygote mutation and 2282del4 heterozygote mutation.

CONCLUSIONThe 3 cases of NS associated with IV had no response to steroid and multi-immunosuppressive therapy, the renal damage observed by histopathology progressed fast. The children with NS associated with IV displayed R501X heterozygote mutation and 2282del4 heterozygote mutation of FLG gene, which suggested that the absence of response to steroid and multi-immunosuppressive therapy may be related to the FLG gene.

Child, Preschool ; DNA Mutational Analysis ; Female ; Humans ; Ichthyosis Vulgaris ; complications ; genetics ; Infant ; Intracellular Signaling Peptides and Proteins ; genetics ; Kidney ; pathology ; Male ; Membrane Proteins ; genetics ; Mutation ; Nephrotic Syndrome ; complications ; genetics ; Pedigree

OBJECTIVETo study the clinical characteristics of children with an initial onset of IgA nephropathy with nephrotic syndrome and compare them with children with primary nephrotic syndrome, in order to provide a theoretical basis for the differential diagnosis of the two diseases.

METHODSFifty children diagnosed with an initial onset of IgA nephropathy with nephrotic syndrome were included in this study. Seventy-two children diagnosed with an initial onset of primary nephrotic syndrome served as the control group. The clinical and laboratory examination characteristics were compared between the two groups.

RESULTSThe IgA nephropathy group had significantly higher incidence rates of gross haematuria, microscopic haematuria, hypertension, acute kidney injury, low serum high-density lipoprotein cholesterol, anemia, low serum complement C4, steroid resistance, and nephritis-type nephrotic syndrome and a significantly lower incidence of elevated serum IgE compared with the control group (P<0.05). There were significant differences in serum creatinine, serum uric acid, serum total cholesterol, serum high-density lipoprotein cholesterol, serum IgE, serum complement C4, and hemoglobin levels between the IgA nephropathy and the control groups (P<0.05). The thresholds of serum IgE (<131.2 IU/mL) and high-density lipoprotein cholesterol (<1.35 mmol/L) were reference parameters in the differential diagnosis of IgA nephropathy with nephrotic syndrome and primary nephrotic syndrome.

CONCLUSIONSChildren with IgA nephropathy presenting nephrotic syndrome manifest mainly as nephritis type and steroid-resistant type in the clinical classification. Cinical manifestations accompanied by serum levels of high-density lipoprotein cholesterol and IgE are helpful for differential diagnosis of IgA nephropathy presenting nephrotic syndrome and primary nephrotic syndrome.

Adolescent ; Child ; Child, Preschool ; Cholesterol, HDL ; blood ; Complement C4 ; analysis ; Female ; Glomerulonephritis, IGA ; blood ; complications ; Hematuria ; etiology ; Humans ; Immunoglobulin E ; blood ; Male ; Nephrotic Syndrome ; blood ; complications

OBJECTIVETo compare the therapeutic effects of prednisone combined with mycophenolate mofetil (MMF) versus cyclosporin A (CsA) in children with steroid-resistant nephrotic syndrome (SRNS).

METHODSThe clinical data of 164 SRNS children who were treated with prednisone combined with MMF or CsA between January 2004 and December 2013 were collected, and the clinical effect of prednisone combined with MMF (MMF group, 112 children) or CsA (CsA group, 52 children) was analyzed retrospectively.

RESULTSAt 1 month after treatment, the CsA group had a significantly higher remission rate than the MMF group (67.3% vs 42.9%; P<0.05). At 3 months after treatment, the CsA group also had a significantly higher remission rate than the MMF group (78.8% vs 63.3%; P<0.05). The 24-hour urinary protein excretion in both groups changed significantly with time (P<0.05) and differed significantly between the two groups (P<0.05). There were no serious adverse events in the two groups.

CONCLUSIONSPrednisone combined with MMF or CsA is effective and safe for the treatment of SRNS in children, and within 3 months of treatment, CsA has a better effect than MMF.

Adolescent ; Child ; Child, Preschool ; Cyclosporine ; administration & dosage ; Drug Therapy, Combination ; Female ; Humans ; Immunosuppressive Agents ; administration & dosage ; Infant ; Male ; Mycophenolic Acid ; administration & dosage ; analogs & derivatives ; Nephrotic Syndrome ; drug therapy ; Prednisone ; administration & dosage ; Retrospective Studies ; Treatment Outcome

OBJECTIVE: To explore the value of galactose-deficient IgA1 (Gd-IgA1) in the early diagnosis of Henoch-Schönlein purpura nephritis (HSPN) in children. METHODS: A total of 67 hospitalized children who were definitely diagnosed with HSPN between January and April 2018 and 58 hospitalized children with Henoch-Schönlein purpura (HSP) were enrolled in the study. Twenty children undergoing routine physical examinations served as controls. The levels of serum and urine Gd-IgA1 were determined using ELISA. The receiver operating characteristic curve was used to analyze the value of serum Gd-IgA1 and urine Gd-IgA1/urine creatinine ratio in the diagnosis of HSPN. RESULTS: The level of serum Gd-IgA1 and urine Gd-IgA1/urine creatinine ratio in children with HSP or HSPN were significantly higher than those in healthy control group (P<0.01), with a significantly greater increase observed in children with HSPN (P<0.01). Serum Gd-IgA1 ≥1 485.57 U/mL and/or urine Gd-IgA1/urine creatinine ratio ≥105.74 were of favorable value in the diagnosis of HSPN. During the six-month follow-up of the 49 children with HSP, the incidence of HSPN was 47% (23/49), which included a 100% incidence in children with serum Gd-IgA1 ≥1 485.57 U/mL and a 73% incidence in children with urine Gd-IgA1/urine creatinine ratio ≥105.74. CONCLUSIONS Serum and urine Gd-IgA1 is of favorable clinical value in the early diagnosis of HSPN.
Child ; Early Diagnosis ; Galactose ; Glomerulonephritis, IGA ; Humans ; Immunoglobulin A ; Purpura, Schoenlein-Henoch

Objective To explore the feasibility of needle-embedding therapy in the treatment of chronic myocardial ischemia using a miniature pig model established by placement of an Ameroid constrictor at the left anterior descending branch (LAD) of coronary artery in Bama miniature pigs during surgery. Methods The miniature pig model of chronic myocardial ischemia was established by placement of an Ameroid constrictor at the left anterior descending branch of the left coronary artery in Bama miniature pigs. The pig models were randomly divided into the treatment group (the"Neiguan " group) and the control group (the "Zusanli " group), and were treated with needle- embedding electroacupuncture at the"Neiguan" (PC6) and "Zusanli" (ST36) acupoints, respectively. Myocardial samples were taken at 6 weeks after surgery for light and electron microscopic examinations. Results Gross pathology showed that ischemic area in the myocardium appeared in both experimental groups. The ischemic area in the "Zusanli "group was larger than that of the"Neiguan"group. Histopathology showed that the acupuncture treatment at the"Neiguan"acupoint reduced the ischemic injury in the pig myocardial tissues. Ultrastructural observation of the myocardium showed mitochondrial vacuolization in cardiomyocytes and myocardial fibrosis in both groups. Conclusions Acupuncture therapy at the"Neiguan"acupoint of pericardial channel may exert protective effect on the myocardial ischemia by reducing the ischemia-injury of cardiomyocytes, but can not inhibit the already existed ischemia-induced cardiomyocytic injuries. Our findings suggest that the establishment of miniature pig model of chronic myocardial ischemia by surgically placing an Ameroid constrictor on the left anterior descending branch of left coronary artery and the needle-embedding in acupoints is feasible for the treatment of chronic myocardial ischemia in this pig model.