OBJECTIVETo summarize the management of infant vascular tumors with Kasabach-Merritt phenomenon (KMP) and to evaluate the effect of drug combined with sclerotherapy.

METHODSFrom Feb. 2007 to Nov. 2014, 25 cases with KMP, who underwent drug therapy combined with sclerotherapy, were retrospectively studied. Oral corticosteroids (2 mg/kg per day) was used as the first-line therapy on all of the patients and intravenous vincristine (1.5 mg/m2 every week) was added when the platelet counts didn't recover obviously after 2-3 weeks. After the recovery of the platelet counts, the patients were admitted for sclerotherapy (average, 4.56 sessions per case) with 100% alcohol (1-3 ml per session), Lauromacrogol (1.25-5 ml per session) and betamethasone (0.25-1 ml per session). All the patients were followed up for 42 months ( range, 9 months to 6.5 years). Therapeutic outcomes were assessed by evaluating platelet counts, size of lesion, function of trunk and limb.

RESULTSAll the 25 cases got obvious recovery in the platelet counts [average, (94.3 ± 18.5) x 10(9)/L] after drug therapy, of which 16 were treated by single oral corticosteroids for 4-7 weeks and 9 were treated by corticosteroids plus intravenous vincristine for 2-5 weeks. Meantime, 11 cases received platelet transfusions, of which 3 were coupled with gamma globulin intramuscularly. During the first admission, each of the 25 cases received 1-4 sessions of sclerotherapy (average, 2.6 sessions each case). One week after the sclerotherapy, the platelet counts returned to (167-312) x 10(9)/L (average, (258.5 ± 34.4) x 10(9)/L). The hemoglobin and blood coagulation function returned to normal within 1-5 weeks. Meanwhile the mental condition, appetite, body weight, sleeping were greatly improved. The size of the lesions decreased gradually after the combined therapy including 13 cases within 3-12 months and 13 cases within 13-36 months. Long term follow-up indicated that only 1 case need treatment for recurrent decrease of platelet counts, and all of the 25 cases kept the normal weight, height, immunity as well as the growing development.

CONCLUSIONSOral corticosteroids plus intravenous vincristine combined with sclerotherapy is a reliable management with high cure rate, short course and minor side-effect.

Administration, Oral ; Betamethasone ; administration & dosage ; Combined Modality Therapy ; methods ; Ethanol ; administration & dosage ; Glucocorticoids ; administration & dosage ; Humans ; Infant ; Injections, Intravenous ; Kasabach-Merritt Syndrome ; blood ; therapy ; Platelet Count ; Polyethylene Glycols ; administration & dosage ; Retrospective Studies ; Sclerotherapy ; methods ; Vincristine ; administration & dosage

Background: To enhance perioperative outcomes, a perioperative registry that integrates high-quality real-world data throughout the perioperative period is essential. Singapore General Hospital established the Perioperative and Anesthesia Subject Area Registry (PASAR) to unify data from the preoperative, intraoperative, and postoperative stages. This study presents the methodology employed to create this database. Methods: Since 2016, data from surgical patients have been collected from the hospital electronic medical record systems, de-identified, and stored securely in compliance with privacy and data protection laws. As a representative sample, data from initiation in 2016 to December 2022 were collected. Results: As of December 2022, PASAR data comprise 26 tables, encompassing 153,312 patient admissions and 168,977 operation sessions. For this period, the median age of the patients was 60.0 years, sex distribution was balanced, and the majority were Chinese. Hypertension and cardiovascular comorbidities were also prevalent. Information including operation type and time, intensive care unit (ICU) length of stay, and 30-day and 1-year mortality rates were collected. Emergency surgeries resulted in longer ICU stays, but shorter operation times than elective surgeries. Conclusions The PASAR provides a comprehensive and automated approach to gathering high-quality perioperative patient data.

OBJECTIVE: To study the trinucleotide repeats of GCN (GCA, GCT, GCC, GCG) encoding Alanine in exon 3 of the PHOX2B gene among healthy individuals from southwest China and two patients with Congenital central hypoventilation syndrome (CCHS). METHODS: The number and sequence of the GCN repeats of the PHOX2B gene were analyzed by capillary electrophoresis, Sanger sequencing and cloning sequencing of 518 healthy individuals and two newborns with CCHS, respectively. RESULTS: Among the 1036 alleles of the 518 healthy individuals, five alleles were identified, including (GCN)₇, (GCN)₁₃, (GCN)₁₄, (GCN)₁₅ and (GCN)₂₀. The frequency of the (GCN)₂₀ allele was the highest (94.79%). And five genotypes were identified, which included (GCN)₇/(GCN)₂₀, (GCN)_13/(GCN)₂₀, (GCN)₁₄/(GCN)₂₀, (GCN)₁₅/(GCN)₂₀, (GCN)₂₀/(GCN)₂₀. The homozygous genotypes were all (GCN)₂₀/(GCN)₂₀, and the carrier rate was 89.58%. Four GCN sequences of the (GCN)₂₀ homozygous genotypes were identified among the 464 healthy individuals. The GCN repeat numbers in the exon 3 of the PHOX2B gene showed no significant difference between the expected and observed values, and had fulfilled the,Hardy-Weinberg equilibrium. The genotypes of the two CCHS patients were (GCN)₂₀/(GCN)₂₅ and (GCN)₂₀/(GCN)₃₀, respectively. CONCLUSION It is important to determine the GCN repeats and genotypic data of the exon 3 of the PHOX2B gene among the healthy individuals. The number of GCN repeats in 518 healthy individuals was all below 20. The selection of appropriate methods can accurately detect the polyalanine repeat mutations (PARMs) of the PHOX2B gene, which is conducive to the early diagnosis, intervention and treatment of CCHS.
Humans ; Infant, Newborn ; Homeodomain Proteins/genetics* ; Hypoventilation/congenital* ; Mutation ; Sleep Apnea, Central/genetics* ; Transcription Factors/genetics*

OBJECTIVETo investigate the role of Wnt signaling pathway on paraquat (PQ)induced PC12 cells damage.

METHODSUsing PC12 cells, in this study CCK8 assay was used to detect the effect of cell viability. The cell apoptosis and cell cycle was detected by flow cytometry. The real-time polymerase chain reaction (RT-PCR) was used to measure the mRNA expression of Wnt pathway key genes including Fzd1, Dvl2 and β-catenin and downstream genes including Bax, Bcl2, Survivin, Cyclin D1 and C-myc.

RESULTCompared with the control, PC12 cells viability in 50.00 and 100.00 µmol/L PQ treatment groups were obviously decreased, the cell cycle S phase arrest, and cell apoptosis increased (P<0.05). The 25.00, 50.00 and 100.00 µmol/L PQ treatment groups mRNA expression of Wnt pathway key genes including Fzd1, Dvl2 and β-catenin and downstream genes including apoptosis suppressor genes (Bcl-2 and survivin)and cyclin gene (Cyclin D1) were downregulated (P<0.05). The mRNA expression of pro-apoptosis gene (Bax) and cyclin gene (C-myc) were upregulated (P<0.05).

CONCLUSIONIt suggested that PQ can activate Wnt pathway to regulate downsteam genes expression, resulting in PC12 cell cycle arrest and apoptosis.

Adaptor Proteins, Signal Transducing ; metabolism ; Animals ; Apoptosis ; Apoptosis Regulatory Proteins ; metabolism ; Cell Cycle ; Cell Survival ; Dishevelled Proteins ; Down-Regulation ; Flow Cytometry ; Frizzled Receptors ; metabolism ; Gene Expression ; drug effects ; PC12 Cells ; Paraquat ; toxicity ; Phosphoproteins ; metabolism ; Rats ; Receptors, Neurotransmitter ; metabolism ; Wnt Signaling Pathway

AIM: Lipopolysaccharide (LPS) on the cell membrane of gram negative bacteria is closely related to the occurrence and development of severe acute pancreatitis (SAP). Local and systemic monocyte / macrophages play an important role in the inflammatory process of SAP. Artesunate (AS) was reported to protect rats with severe acute pancreatitis by reducing the release of proinflammatory cytokines. This study further explored the molecular mechanism of anti-inflammatory effect of AS. METHODS: The release of proinflammatory cytokines in the supernatant were studied by enzyme-linked immunosorbent assay. Then, the mRNA expressions of PI3K-III and its key molecules in signaling pathway were detected by real-time quantitative PCR. Finally, the phosphorylation levels of PI3KIII were detected by Western blot. RESULTS: AS could significantly inhibit the release of proinflammatory cytokines from mouse macrophage induced by LPS. Autophagy inhibitor 3-methyladenine (3-MA) could significantly inhibit the release of TNF-α from mouse macrophages induced by LPS; LPS significantly increased the mRNA expression of PI3KIII and its key molecules in mouse peritoneal macrophages (PMs). Finally, AS could significantly inhibit the increase of PI3K-III phosphorylation induced by LPS in PMs. CONCLUSION: The anti-inflammatory mechanism of AS is closely related to the inhibition of PI3K-III phosphorylation.