Objective To explore the clinical features,diagnosi s and prognosis of incontinentia pigmenti.Methods Analyzing and summarizing the clinical characteristic, diagnosis and prognosis of neonatal incontinentia pigmenti in 6 neonatal infants that were hospita- lized in our department during the period from January 1 998 to December 2003 were studied,and some relevant literature were reviewed. Results 1.Three of 6 infants were male which was unusual;2.Four infants had typical skin lesions at birth and 1 case at 6 days old.Four cases had typical 3 stages o f skin lesions including the erythematous and vesicular inflammatory stage,verr ucous lesions and hyperkeratosis stage,macular hyperpigmentation stage,but the re was overlap;3.Four infants were complicated by central nervous system involv ement (two cases presented mental retardation,2 infants were temporary damage). Two cases were complicated by ocular manifestations ( one case had optical nerve atrophy and blind in left eye,the other had severe bilateral retinal lesions); 4.On specific examination 5 infants were diagnosed by skin biopsy.Gene analysis was made in 1 case,but we didn′t find the mutations of NEMO. Conclusions Incontinentia pigmenti is a rare X-linked dominant multisystem disease.It may be misdiagnosed in the initial stages.Except typical clinical features,skin biops y and gene analysis are main evidence for diagnosis.Early detection and interven tion are important for prognosis. J Appl Clin Pediatr,2005,20(2):123-125

Objective To explore the relationship of post-transplant major histocompatibility complex class I chain-related gene A(MICA)antibody status and renal allograft function in clinical stable phase.Methods Fifty-seven patients accepted renal allografts followed up for at least 6 months were detected with the levels and specialties of MICA antibodies by Flow PRATM beads.Simultaneously,their serum ereatinine levels were tested as well.The impact of MICA antibody status on renal allograft function was assessed.Results Among the 57 patients,38 cases showed no HLA and MICA antibody.11 cases had HLA antibodies but not MICA antibody,8 cases had MICA antibodies and 3 cases had both MICA and HLA antibodies.There were 5 patients with MICA019 antibodies.3 patients with MICA027 antibodies,2 patients with MICA018 antibodies,while 1 patient with MICA004 and MICA017 antibodies,respectively.There were 9 patients with antibody positive score higher than 6,accounting 75%(9/12).Except age,there was no significant difference between patients with positive and negative MICA antibodies in the aspects of blood transfusion history,CDC,and cold ischemia time(P＞0.05).The average ages were(32.5±7.9)years for MICA antibodypositive patients and were(43.0±1 0.4)years for MICA antibody-negative patients(P=0.008).MICA antibody-positive patients without HLA antibody had higher serum creatinine level[(117.20±12.30)μmol/L]than MICA and HLA antibody-negative patients[(89.40±28.95)μmol/L,P＜0.05].Conclusions The measurement of MICA antibodies has prognostic value in the assessment of patients without HLA antibodies after renal transplantation.MICA antibody positive has clear association with chronic renal allograft function decline.

Objective To investigate whether delayed treatment with exogenous kallikrein on neurogenesis after focal cortical infarction in stroke-prone renovascular hypertensive rats (RHRSP). Methods Seventy-two RHRSP were divided into 3 groups. Twenty-four rats were given human tissue kallikrein ( 1.6 × 10-2 PNAU/kg) and 24 rats were given vehicle through tail venous daily for 2 or 6 days consecutively starting at the 24th hour after distal middle cerebral artery occlusion (MCAO). 24 rats underwent sham-operation. Cell proliferation was examined by using 5'-bromo-2'-deoxyuridine (BrdU, 50 mg/kg). Rats were respectively sacrificed 3, 7, 14 or 28 days after MCAO. Results Treatment with kallikrein significantly increased the number of BrdU+ cells in the ipsilateral subventricular zone (SVZ) (304.0±73. 9 vs 167.0±32.2 vs 56.0±12.2 at 7 d after operation, q =7.165, 12.916 and 5.751 respectively,all P<0.05) and in the peri-infarction region (490.0±82.0 vs 308.0±51.5 vs 49.0± 9.5 at 7 d after operation, q = 7.920, 19.184 and 11.264 respectively, all P < 0.01 ), and increased the number of BrdU+/DCX+ cells (225.0±13.6 vs 98.0±9.6 vs 23.0±5.6 at 7 d after operation, q = 30.731,48.735 and 18.004 respectively,all P < 0.01) in the ipsilateral SVZ compared with the vehicle group or the sham-operated group, which began on the 3 day, peaked in 7--14 days after MCAO, and then gradually decreased. Compared with the vehicle group, exogenous kallikrein markedly increased the number of BrdU+/NeuN+ cells (21.0±3.4 vs 13.0±2.6 at 14 d, P =0.001 ) in the peri-infarction region after MCAO. The kallikrein group showed a better functional improvement than the vehicle group after stroke ( all P < 0.05). Conclusion Our study suggests that administration of exogenous kallikrein at 24 h after cortical infarction enhances the SVZ neuroblasts proliferation, migration, and selective differentiation and improves functional recovery after stroke.

Objective To improve the recognition of intramedullary spinal abscess by a case of congenital dermal sinus with intramedullary spinal abscess and reduco the incidence of congenital dermal sinus with intramedullary spinal abscess.Methods Clinical,laboratory data and image of a confirmed case about one infant of congenital dermal sinus with multiple intramedullary spinal abscess were investigated,the related literature was reviewed.Results In this case,when the infant with congential dermal sinus had infection,he failed to gain antibiotic therapy, timely surgical treatment,his infection had diffused, and multiple intramedullary spinal abscess flared up.Conclusions Intramedullary spinal abscess is a rare disease.If treatment is delayed, the prognosis is poor and the mortality rate is high.MRI is the ideal investigation for diagnosis.Intramedullary spinal abscess can happen subsequent to congenital dermal sinus with infection, and cause neurological sequela. So an infant with congenital dermal sinus should be offered to avoid complication caused by infection.

OBJECTIVETo investigate the impact of Staphylococcus aureus from infertile men on sperm motility and the relationship between virulence genes and the activity of spermatozoal immobilization.

METHODSWe collected 60 strains of non-repeated Staphylococcus aureus from the semen of 589 infertile males and analyzed the influence of Staphylococcus aureus on sperm motility using the computer-aided sperm analysis system. We selected the strains that apparently decreased sperm motility and detected their virulence genes by PCR.

RESULTSSperm motility was significantly decreased in 17 of the 60 strains of Staphylococcus aureus (P < 0.05). The main virulence genes in these strains were hlg (33.3%), scn (23.3%), cna (20%), hlb (20%), and clfA (18.3%), others including icaA, fnbA, tst, seb, hld, eta and sea. The scn gene carriers accounted for 47.1% in the spermatozal immobilization positive group, significantly higher than 14% in the negative group (P < 0.05). No statistically significant differences were found in the percentages of the carriers of the other virulence genes between the two groups (P > 0.05).

CONCLUSIONInfections of Staphylococcus aureus in male reproductive system can lead to the decrease of sperm motility, which may be associated with the Staphylococcus complement inhibitor encoding gene scn.

Humans ; Infertility, Male ; microbiology ; Male ; Polymerase Chain Reaction ; Semen ; microbiology ; Species Specificity ; Sperm Motility ; Staphylococcal Infections ; Staphylococcus aureus ; pathogenicity ; Virulence ; genetics

Objective To study the influence of human leucocyte antigen(HLA) and major his-tocompatibility complex class Ⅰ chain-related gene A (MICA) specific antibodies on renal allograft function and graft rejective reaction by monitoring their changes from preoperative to postoperative pe-riods. Methods Twenty-seven patients with renal aliografts were tested with the specificity of anti-HLA antibodies (anti-HLA class Ⅰ and anti-HLA class Ⅱ) and anti-MICA antibodies and their posi-tive value changes by flow PRATM beads. The HLA genotype was integrated to distinguish donor specific antibody(DSA) and non-donor specific antibody(NDSA). Their serum creatinine levels and clinical data were analyzed simultaneously. Results Of the 27 patients, 22 cases accepted renal transplantation from dead bodies and 5 eases accepted from live donors. Except 1 failed patient, the other 26 patients had good functional renal allografts. Twenty-four survival patients were followed up on month 1, 3, 6 and 12 after transplantation. Seven out of 27 patients had pre-exist antibody before transplantation. Among them, 2 patients had anti-HLA antibody; 3 patients had anti-MICA antibody; 2 patients had both anti-HLA and anti-MICA antibody. Three patients with no anti-HLA and anti-MICA antibodies before transplantation created antibodies after transplantation from 3 to 6 months. One patient created NDSA after transplantation and appeared chronic rejection. There were 3 patients who had anti-MICA antibodies before transplantation. The expression levels of antibodies had changed from high to low, but the specific anti-MICA antibody had not changed during the follow-up on month 1, 3, 6 and 12 after transplantation. The patient with pre-transplantation low level of anti-HLA class Ⅱ antibody appeared acute rejection with fever and his CMV was positive as well. The patient's SCr levels changed from 171 μmol/L to 236 μmol/L after I to 3 months post-transplantation. Twenty-four patients were divided into positive and negative groups according to the specific antibody. There was significant difference of SCr levels between the 2 groups 1 month and 1 year after transplantation(P= 0.03, 0.05). Conclusions It is important to detect the specificity and positive value of anti-HLA antibodies and anti-MICA antibody regularly during the post transplantation follow-up. This will make an effective therapy for decreasing the occurrenee and development of acute or chronic rejection and hy-pofunction on renal allograft.

Objective To explore the significance of designing with monitoring-flap in massive com- pound bone grafts for repairing massive bone defects in extremities.Methods From January 2001 to De- cember 2004,large bone defects in 19 patients(11 men and 8 women,age:6 to 35 years,mean age:18.6 years)were repaired by vascularized free fibular transplant with a monitoring-flap combining with massive deep frozen bone allografts.Average length of the bone defects was 16.6 cm(range,12 to 25 cm).A 7 days' con- tinuously clinical examination including observing the color,turgor,temperature,capillary refill,and bleeding after a needle sticking of the monitoring-falps were used postoperatively,if any one of these were abnormal,the circulation of the compound bone grafts must be in danger and some measures such as re-operation should be taken immediately.Dynamic image analysis was used for evaluating the bone union.Results One monito- ring-flap was vascular artieulo,and the articulo was relieved after exploration and resection of vein thrombus; another one was marginal part necrosis;the remains were normal.All of monitoring-flaps healed normally after 23.2 months(range,6 to 54 months)follow-up.15 patients had the radiographic evidence of bone unions 3 months after surgery.11 patients had been removed intermal fixation,complete bone unios were found one year postoperatively.Conclusion Designing with monitoring-flap in massive compound bone grafts for repairing massive bone defects,and can clearly understand the circulatory statue of compound bone grafts and early pre- dict the final results of massive bone allografts.

OBJECTIVETo evaluate the changes in the renal function of patients with chronic hepatitis B (CHB) receiving adefovir dipivoxil (ADV) or telbivudine (L-DT) monotherapy.

METHODSThis retrospective analysis involved 101 patients with CHB and liver cirrhosis receiving either ADV or L-DT monotherapy for 52 weeks. Serum creatinine, estimates of glomerular filtration rate (eGFR), and the percentage of patients with eGFR≥90 ml·min(-1)·1.73 m(-2) at week 52 were compared with the baseline data between the two groups.

RESULTSThe mean changes of CR at week 52 from baseline were +0.05 mg/dl in ADV group and -0.12 mg/dl in L-DT group, showing a significant difference between the two groups (P=0.000). No patient was found to have an elevation of creatinine over 0.50 mg/dl. The median change of eGFR at week 52 from baseline differed significantly between ADV and L-DT groups (-4.09 vs+18.32 ml·min(-1)·1.73 m(-2), P=0.000). Ninety-two percent (12/13) of the patients with baseline eGFR<90 ml·min(-1)·1.73 m(-2) shifted to eGFR ≥90 ml·min(-1)·1.73 m(-2) after 52 weeks of L-DT treatment, as compared to 38% (3/8) in ADV group. The proportion of patients with eGFR≥90 ml·min(-1)·1.73 m(-2) in L-DT group increased from 76.36% (42/55) at baseline to 94.55% (52/55) at week 52, while that in ADV group decreased from 82.61% (38/46) at baseline to 78.26% (36/46). The constituent ratios of eGFR at different levels were similar at baseline (P=0.443) but significantly different at week 52 between the two groups (P=0.015).

CONCLUSIONL-DT treatment is associated with a renoprotective effect in patients with CHB, but the mechanism remains unclear.

Adenine ; analogs & derivatives ; therapeutic use ; Adolescent ; Adult ; Creatinine ; blood ; Female ; Glomerular Filtration Rate ; Hepatitis B, Chronic ; drug therapy ; physiopathology ; Humans ; Male ; Middle Aged ; Organophosphonates ; therapeutic use ; Retrospective Studies ; Thymidine ; analogs & derivatives ; therapeutic use ; Young Adult

OBJECTIVETo investigate the value of serum cystatin C (Cys-C), urinary Cys-C, urinary retinol binding protein (RBP) and urinary neutrophil gelatinase-associated lipocalin (NGAL) in the early assessment of multiple myeloma (MM) and their characteristic changes in different pathological types of renal impairment.

METHODSAccording to glomerular filtration rate (eGFR), the patients were divided into two groups, of which marked group A with normal renal function, the other marked group B with abnormal renal function. Sixty healthy subjects were chosen as control. Detection of the serum Cys-C, urinary RBP, urinary Cys-C, urinary NGAL, serum creatinine (Scr), urinary microalbumin (MAU) and urinary α1-microglobulin (α1-MG) were performed. Renal biopsy was carried out for patients who had abnormal serum Cys-C, urinary Cys-C, urinary RBP, urinary NGAL and were willing to accept further test.

RESULTSCompared with healthy controls, the serum Cys-C, urinary RBP, urinary Cys-C, urinary NGAL of group A were significantly higher than that of healthy controls. Six group A patients received renal biopsy, and varying degrees of renal damage were discovered. The serum Cys-C, urinary RBP, urinary Cys-C and urinary NGAL positive rate were 66.7%, 66.7%, 66.7% and 83.3%, respectively. Of twenty-four cases received biopsy after abnormal examination results were shown, six turned out to be amyloidosis, twelve cast nephropathy (CN) and 6 monoclonal immunoglobulin deposition disease (MIDD). Compared with MIDD and amyloidosis, the urinary Cys-C and NGAL of the CN group are significantly higher (P < 0.05). Compared with CN and amyloidosis, urinary RBP of MIDD is significantly higher (P = 0.043). Compared with MIDD and CN, the MAU of amyloidosis is significantly higher (P = 0.006).

CONCLUSIONCompared with the conventional indicators, serum Cys-C, urinary Cys-C, RBP and NGAL are more sensitive in early assessment of MM patients with renal damage. The MAU is higher in amyloid, the urinary Cys-C and urinary NGAL are significantly elevated in CN, the urinary RBP is significantly elevated in MIDD.

Acute-Phase Proteins ; urine ; Adult ; Aged ; Case-Control Studies ; Cystatin C ; blood ; urine ; Female ; Humans ; Kidney ; pathology ; Kidney Diseases ; blood ; diagnosis ; urine ; Kidney Function Tests ; Lipocalin-2 ; Lipocalins ; urine ; Male ; Middle Aged ; Multiple Myeloma ; blood ; pathology ; urine ; Proto-Oncogene Proteins ; urine ; Retinol-Binding Proteins ; urine

Adult ; Animals ; Ecthyma, Contagious ; diagnosis ; virology ; Female ; Humans ; Microscopy, Electron, Transmission ; methods ; Young Adult