Fibrosarcoma in newborns and infants, designated as congenital, infantile, or juvenile fibrosarcoma is an uncommon soft tissue neoplasm occurring most frequently during the first year of life. Infantile fibrosarcoma is associated with favorable clinical behavior that is markedly different from that of adult fibrosarcoma., We report a case of infantile fibrosarcoma occured in a 3-year-old boy presenting as a palpable mass in the left lower extremity since 3 months of life. Histologic findings of the tumor are similar to those of f ibrosarcomas in adult.
Infant ; Adult ; Male ; Female ; Infant, Newborn ; Humans

Wilms' tumor is one of the most common primary malignant tumors of the kidney during infancy and childhood and is known to be originated from the primitive cells of metanephric blastema. It presents difficulties when encountered in deciding the presence of anaplasia or in differentiating it from other renal tumors of childhood with different biologic behavior because of its diverse histologic patterns and varying degrees of differentiation. Evaluation of clinical and histopathologic features in terms of prognostication was done of 32 cases of Wilms' tumor which were surgically resected and diagnosed in the period from January 1979 through June 1992. Immunohistochemical reaction for cytokeratin, vimentin, actin and desmin was also analysed on all cases of Wilms' tumor in conjunction with clear cell sarcoma of the kidney(CCSK), malignant rhabdoid tumor of the kidney(MRTK) and congenital mesoblastic nephroma(CMN) to assess the validity of immunohistochemistry in differentiating Wilms' tumor from these renal tumors. Twenty four(75%) cases were diagnosed before the age of 5 and 40.7% were under 2 years old. Mixed type was most common(62.5%), followed by epithelial, blastemal and stromal predominant type in descending order of frequency. Anaplasia was observed in 3 cases(9.4%), two of which were epithelial predominant type and one blastemal predominant type. Treatment modality and presence of anaplasia were significantly correlated with 5 year survival rate of patients. Immunohistochemical stain revealed that all epithelial component of Wilms' tumor were positive for cytokeratin and 56.3% of Wilms' tumor had blastemal component which were positive for both cytokeratin and vimentin. Twenty cases(62.5%) of Wilms' tumor had blastemal component which were positive for cytokeratin with a proportion of more than 5% of reactive cells. Stromal component of Wilms' tumor generally did not show differentiation into the specialized type of tissue and all revealed positive reactions for vimentin among which some revealed positive reactions for actin. Only 3 out of 6 cases with rhabdomyoblastic differentiation were positive for desmin. CCSK, MRTK and CMN which have different biologic behavior and treatment modality compared to Wilm's tumor showed positivity only for vimentin and/or actin. In summary, treatment modality and presence of anaplasia are significantly correlated with patients' survival and the immunohistochemical stain for cytokeratin is very helpful in confirming the presence of blastemal component and useful in the differential diagnosis of Wilms' tumor from other kinds of pediatric renal tumors.
Child ; Male ; Female ; Humans ; Diagnosis, Differential

Objective To evaluate whether changes in neonatal intensive care have improved outcomes for extremely low birth weight (ELBW) infants in neonatal intensive care unit (NICU). Methods A prospective phase-lag cohort study was performed in a tertiary level NICU. A meticulous nursing strategy based on neonatal individual developmental care assessment program theory and feasible ELBW minimization stimulus was developed. Conventional care was applied in 2013 (period Ⅰ) and gently caring was applied in 2014 (period Ⅱ). The outcomes of ELBW between these 2 periods were compared. Results During these two periods, thirty-seven infants were included in period Ⅰ and 41 infants in period Ⅱ. In periodⅠ46.0%(17/37) of the infants needed oxygen for at least 28 days, but in period Ⅱ it decreased to 24.4%(10/41), there was significant difference (χ2=3.990, P=0.046). The rate of breastfeeding increased from 27.0%(10/37) in periodⅠto 61.0%(25/41) in period Ⅱ, there was significant difference (χ2=9.061, P=0.003). There was no significant difference in the mortality rate and chronic lung disease (P>0.05). The incidence of intracranial hemorrhage decreased from 21.6%(8/37) to 4.9%(2/41), there was significant difference(P=0.041). Conclusions Gently caring may have resulted in less intracranial hemorrhage and improve breastfeeding rate. Parents are satisfied with gentle care and in light of these findings, gentle care deserves further exploration.

Psoriatic arthritis is an uncommon disease in the community and probably occurs in no more than 5 percent of the general psoriatic population. The authors experienced a patient who had the findings of mutilating type of psoriatic arthritis with severe joint deformities. A 30 year-old man was admitted to our hospital due to multiple joint pain and deformities with wheel chair bound state. We report a case of mutilating type in the psoriatic arthritis with brief review of literatures.
Adult ; Arthralgia ; Arthritis, Psoriatic* ; Congenital Abnormalities ; Humans ; Joints ; Wheelchairs

BACKGROUND: Various osteotomies have been introduced to treat osteonecrosis of the femoral head. The purpose of this study was to compare surgical parameters, postoperative limb length discrepancy, and minimum 5-year clinical and radiological results between transtrochanteric curved varus osteotomy (TCVO) and transtrochanteric rotational osteotomy (TRO) for osteonecrosis of the femoral head. METHODS: From 2004 to 2009, 103 consecutive TROs (97 patients) followed by 72 consecutive TCVOs (64 patients) were performed for the treatment of osteonecrosis of the femoral head. Of these, 85 patients (91 hips) in the TRO group and 58 patients (65 hips) in the TCVO group completed minimum 5-year clinical and radiological follow-up. The Kaplan-Meier product-limit method was used to estimate survival. RESULTS: The TCVO group had shorter operation time (p < 0.05) and less estimated blood loss (p = 0.026). Postoperative collapse developed in 26 hips (28.6%) in the TRO group and 7 hips (10.8%) in the TCVO group (p = 0.007). Osteophyte formation was observed in 34 hips (37.4%) in the TRO group and 13 hips (20%) in the TCVO group (p = 0.020). Fifteen hips (16.5%) in the TRO group and 7 hips (10.8%) in the TCVO group underwent conversion total hip arthroplasty (THA). The survival rate at 9 years with radiographic collapse as the endpoint was 68.7% (95% confidence interval [CI], 58.1% to 79.3%) in the TRO group, and 84.7% (95% CI, 71.5% to 97.9%) in the TCVO group. With conversion to THA as the endpoint, the survival rate was 82.2% (95% CI, 73.1% to 91.3%) in the TRO group and 89.2% (95% CI, 81.7% to 96.7%) in the TCVO group. CONCLUSIONS: The comparison indicates that TCVO was better than TRO in terms of surgical parameters including operation time and estimated blood loss while the 9-year survival rates were similar.
Arthroplasty, Replacement, Hip ; Extremities ; Femur Head ; Follow-Up Studies ; Head* ; Hip ; Humans ; Kaplan-Meier Estimate ; Osteonecrosis* ; Osteophyte ; Osteotomy* ; Survival Rate

The amplification of c-myc oncogene was evaluated in 42 cases of ovarian carcinomas to correlate with clinical parameters. Using oligonucleotide primers, sequences from the c-myc exon-3 gene and from a control gene, tissue plasminogen activator (tPA), were amplified simultaneously by polymerase chain reaction (PCR). After the products of differential PCR (d-PCR) were electrophoresed, slot blot hybridization was performed, and hybridized with P32 dATP-labeled myc and tPA oligonucleotide probes and then autoradiographed. The signal intensities of the two products were quantitated by densitometry and the ratios of two products (c-myc/tPA) were measured. The ovarian carcinomas showed significantly increased amplification of c-myc oncogene Oligonucleoti compared to normal control group (p<0.05). 15 of 42 cases (35.7%) showed various degrees of the MYC gene amplification up to 27 folds in various histologic types of ovarian carcinomas. No significant differences of the MYC gene amplification according to histologic subtypes, tumor action) grades and clinical stages of ovarian carcinomas were present.
Densitometry ; DNA Primers ; Genes, myc ; Oligonucleotide Probes ; Oncogenes* ; Polymerase Chain Reaction* ; Tissue Plasminogen Activator

No abstract available.
Animals ; Hemorrhagic Fever with Renal Syndrome* ; Rats* ; Singapore*

Initial rapid diagnosis of primary pulmonary cryptococcosis(PPC) occurring in a immunocompetent host was made by transthoracic fine needle aspiration cytology of a solitary subpleural nodule. Numerous refractile spherical organisms surrounded by a clear halo were demonstrated with haematoxylin-eosin and Papanicolaou stains. The organisms, 5 15 micrometer in diameter, were easily demonstrated with Gomori methenamine-silver stain. Many of the organisms showed narrow-base budding. Carminophilic cell walls were well demonstrated with mucicarmine stain.
Biopsy, Fine-Needle* ; Cell Wall ; Coloring Agents ; Cryptococcosis* ; Diagnosis ; Lung

Objective To elucidate the molecular genetic etiology of patients with disorders of sex development(DSD)using whole exome sequencing(WES),thereby enhancing our understanding of the underlying mechanisms of sexual development abnormalities.Methods Retrospective analysis was conducted on clinical data of 60 DSD patients diagnosed in the First People's Hospital of Yunnan Province between March 2008 and August 2021,with an additional family study for one proband.Genomic DNA was extracted from patients for WES analysis.Single nucleotide polymorphism(SNP)and insertions/deletion(InDel)tests were identified using SAMtools software in conjunction with established SNP and InDel databases.Copy number variations(CNVs)at the exon level were detected using ExomeDepth,while the potential pathogenicity of mutations was predicted with PolyPhen-2,Mutation taster and PyMol software,with Sanger sequencing employed for confirmation.Results The study included 22 patients with 46,XX DSD and 38 with 46,XY DSD.Among the 46,XX DSD patients,the SRY gene was detected in 14 patients.In the remaining 8 patients and a proband's families,single nucleotide site variations(SNVs)of NR5A1,PROKR2 and ANOS1 genes were identified in 2 patients,and CNVs in CYP21A2 gene were found in 4 patients.The pathogenicity of CYP21A2 EX1 Dup has been previously reported,while the remaining 3 CNVs were of uncertain significance,and no DSD-related mutations were detected in 2 patients.In the WES analysis of 46,XY DSD patients,10 pathogenic or likely pathogenic SNVs across 5 genes(SRY,AR,SRD5A2,CYP17A1,and NR5A1)were identified in 14 patients.Additionally,5 likely pathogenic CNVs involving the CYP21A2,AKR1C2,CBX2,and NR5A1 genes were detected in 5 patients,comprising 3 deletions and 2 duplications.Novel SNVs in NR5A1(c.722G>T,c.48C>G)and ANOS1 c.564A>T were identified,with no prior reports in relevant databases.The pathogenicity of CYP21A2 EX1 Dup is documented in related databases,while the remaining CNVs have not been previously reported.Conclusion The utilization of WES technology has enhanced the diagnostic potential for DSD,broadened the spectrum of known DSD-related gene mutations,and deepened our comprehension of DSD pathogenesis,offering valuable support for genetic counseling.