Efficacy of Bleomycin-Lauromacrogol Foam in Pediatric Macrocystic Lymphatic Malformations With and Without Intracapsular Hemorrhage.

BACKGROUND: Sclerotherapy is purportedly less effective in patients with hemorrhagic than with non-hemorrhagic lymphatic malformations (LMs). We aimed to compare the efficacy of bleomycin-lauromacrogol foam (BLF) sclerotherapy in the treatment of macrocystic LMs with and without intralesional hemorrhage. METHODS: Fifty-five children with macrocystic LMs admitted to the Pediatric Surgery Department were retrospectively included. The patients were allocated into a hemorrhage group (23 cases) or a non-hemorrhage group (32 cases) based on the occurrence of an intracapsular hemorrhage. The diagnosis was confirmed by physical examination, color ultrasound, magnetic resonance imaging, and puncture findings. BLF was injected into the capsule after draining the cystic fluid under color ultrasound guidance. Patients whose lesions were unchanged or showed minor change after 1 month were treated again using the same method. Changes in lesion size and the number of treatments were recorded. Effectiveness was classified as excellent (volume reduction ≥90%), good (50%≤volume reduction<90%), or poor (volume reduction <50%). RESULTS: In the hemorrhage group, 17, 6, and 0 patients' outcomes were classified as excellent, good, and poor, respectively. The overall efficacy rate was 100%. In the non-hemorrhage group, 23, 7, and 2 patients' outcomes were classified as excellent, good, and poor, respectively. The overall efficacy rate was 93.8%. There was no significant difference in efficacy rate between groups (P = 0.767). CONCLUSIONS: BLF is an effective and safe treatment for macrocystic LMs with bleeding. The results were similar in patients with and without bleeding. LEVEL OF EVIDENCE: Treatment, Level III.

Novel heptafluoroisopropyl N-phenylpyrazole aryl amides containing cyanoalkyl groups: Design, synthesis, insecticidal activity, docking studies and theoretical calculations.

To discover and develop new insecticides of the phenylpyrazole class, a series of heptafluoroisopropyl N-phenylpyrazole aryl amide compounds bearing cyanoalkyl groups were synthesized based on the lead compound nicofluprole. Their structures were established by HRMS, 1H NMR and 13C NMR. Bioassay results indicated that several of these compounds exhibited remarkable acaricidal and insecticidal activities. The LC50 values for compounds A1, A2 and A5 against Tetranychus cinnabarinus (T. cinnabarinus) were 1.7-4.2 times lower than that of nicofluprole (3.124 mg/L). Compounds A1, A2, A4 and A7 against Myzus persicae (M. persicae) had LC50 values of 0.261, 1.292, 0.589 and 1.133 mg/L respectively, exceeding that of nicofluprole (LC50 = 4.200 mg/L). Some compounds also demonstrated good insecticidal activity against Plutella xylostella (P. xylostella). For example, compounds A1-A4, A6, and A7 had a mortality rate of 100 % at a low concentration of 1.25 mg/L, which was comparable to nicofluprole (93.3%). Compound A1 exhibited insecticidal activity against Chilo suppressalis (C. suppressalis) with an LC50 value of 2.271 mg/L, which was superior to both nicofluprole (6.021 mg/L) and the positive control broflanilide (6.895 mg/L). Taking compound A5 as a representative, we tested the insecticidal activity against Aphis fabae (A. fabae), Aphis gossypii Glover (A. gossypii Glover), Nilaparvata lugens (N. lugens) and Laodelphax striatellus (L. striatellus) at 10 mg/L, and our results revealed that compound A5 exhibited broad-spectrum insecticidal activity. Molecular docking studies suggested that A1 had a lower binding energy of -7.764 kcal/mol with the P. xylostella gamma-aminobutyric acid receptors (GABAR). Density functional theory calculations (DFT) provided insights into the design of new compounds. This research suggested that the novel phenylpyrazoles featuring cyanoalkyl moieties in this work hold potential as novel insecticides for further research and development.

Laparoscopic approach for managing intussusception in children: Analysis of 65 cases.

BACKGROUND: Intussusception can be managed by pneumatic reduction, ultrasound-guided hydrostatic reduction, open or laparoscopic surgery, but laparoscopy in such cases remains controversial. AIM: To explore the clinical characteristics, effectiveness, and complications of surgical reduction for intussusception using laparoscopy in children. METHODS: This study was a retrospective case series of pediatric patients with intussusception who underwent surgical reduction by laparoscopy from May 2011 to April 2016 at Taizhou Hospital of Zhejiang Province. Clinical characteristics (operation time, intraoperative blood loss, conversion rate of laparotomy, reasons for conversion, postoperative hospital stay, and adverse events) were described. RESULTS: The 65 patients included 45 boys and 20 girls. The average age was 2.3 years (27.5 ± 24.5 mo). Of the 65 patients, 61 underwent surgical reduction by laparoscopy after a failed enema reduction of intussusception, and four underwent the procedure directly. All patients were treated successfully and 57 (87.7%) patients underwent successful laparoscopic surgery, two of which had a spontaneous reduction. Among the remaining cases, one was converted to open surgery via right upper quadrant incision, and seven required enlarged umbilical incisions. Intestinal resection was performed in 5 patients because of abnormal bowel lesions. There were no complications (intestinal perforations, wound infections, or intestinal adhesions) during the follow-up of 3 years to 8 years. Two patients experienced a recurrence of intussusception; one was resolved with pneumatic reduction, and the other underwent a second laparoscopic surgery. CONCLUSION: Laparoscopic approach for pediatric intussusception is feasible and safe. Bowel resection if required can be performed by extending umbilical incision without the conventional laparotomy.

Single-Site Umbilical Laparoscopic Pyloromyotomy Using a Pyloric Electrocoagulation Chisel Combined with a Left-Handed Main Operation for Congenital Hypertrophic Pyloric Stenosis.

Background: Laparoscopic pyloromyotomy has become a gold standard for the treatment of congenital hypertrophic pyloric stenosis (HPS). There have been recent reports on the use of transumbilical single-site laparoscopic surgery for congenital HPS; however, using transumbilical single-site laparoscopic surgery in pediatric cases is still controversial due to the difficulty with manipulation. In this study, some preliminary experience with the application of a novel transumbilical single-site laparoscopic approach in congenital HPS is described. Methods: A retrospective study was conducted involving 25 patients with congenital HPS treated in our hospital from August 2016 to August 2019. A pyloric electrocoagulation chisel combined with a left-handed main operation was completed in all of the patients and the operative times, postoperative length of stay, and operative complications were recorded. Results: The laparoscopic operation was completed in 25 patients with an average operative time of 21.9 ± 5.5 minutes, average postoperative length of stay of 2.5 ± 0.9 days, and no perforations of the pyloric mucosa, recurrent obstruction, surgical incision infections, and incision hernias. All of the patients had at least 3 months of follow-up, good growth and development, and the parents were satisfied with the postoperative scars. Conclusion: A pyloric electrocoagulation chisel combined with a left-handed main operation in the treatment of congenital HPS by a single-site umbilical laparoscopic pyloromyotomy is safe and effective, and can achieve a satisfactory cosmetic effect.

The Application of Pyloric chisel in the Treatment of Hypertrophic Pyloric Stenosis by Single-Site Umbilical Laparoscopic Pyloromyotomy.

OBJECTIVE: The feasibility and perspective of pyloric chisel were discussed through the comparison of pyloric chisel and knife in the treatment of hypertrophic pyloric stenosis (HPS) in single-site umbilical laparoscopic pyloromyotomy (SSULP). METHODS: Fifty-eight cases of HPS treated in our hospital from February 2011 to March 2016 were retrospectively analyzed, in which 30 patients underwent pyloric chisel (Pyloric chisel Group) and 28 patients underwent knife (Knife Group). Operative time, estimated blood loss, and complications between the two groups were analyzed. RESULTS: The operative time was shorter in Pyloric chisel Group than Knife Group (P < .05). The estimated blood loss was lower in Pyloric chisel Group than Knife Group (P < .05). The complication was less in Pyloric chisel Group than Knife Group (P < .05). There were 2 cases of mucosal perforations requiring conversions to open in Knife Group. Five cases of serous tearing occurred in the Knife Group. There was 1 case of serous tearing in the Pyloric chisel Group. All patients were followed up for 3 months, and there was no distinct scar in the umbilical. CONCLUSIONS: Patients were satisfied with no distinct scars in abdominal wall by pyloric chisel or knife to treat HPS in SSULP, but pyloric chisel is more effective and safer.

Treatment of Early-stage Extracranial Arteriovenous Malformations with Intralesional Interstitial Bleomycin Injection: A Pilot Study.

Purpose To assess the efficacy and safety of intralesional interstitial bleomycin injection in the treatment of early-stage (Schobinger stage I or II) extracranial arteriovenous malformations (AVMs). Materials and Methods This prospective study involved 34 patients with early-stage AVMs, as defined by the Schobinger staging system. The patients received intralesional interstitial bleomycin injected at a maximum dose of 15 000 IU or 1000 IU per kilogram of body weight for children who weighed less than 15 kg per procedure for a total of 6 months (once every month). Therapeutic outcome was evaluated by the degree of devascularization at angiography and the clinical outcome 3 months after the last treatment. Further follow-up was evaluated based on further clinical outcome. Adverse events were recorded according to the Society of Interventional Radiology classification. Results Of the 34 patients with early-stage AVM, 32 (mean age, 20.5 years; 24 female [75%]) completed the study. The results showed that 27 (84.4%, 95% confidence interval [CI]: 71.1, 97.7) patients were responsive to bleomycin injection, including nine (28.1%) with a complete response. Four (12.5%) patients showed no response, and one (3.1%) patient experienced worsening 3 months after the last treatment. During further follow-up (mean follow-up time, 20.7 months; range, 5-28 months), the outcome remained stable in 31 (96.9%) of the 32 patients. A major complication, anaphylactic shock, was observed in one (3.1%, 95% CI: 0, 9.5) patient. Common minor complications included hyperpigmentation, nausea, pruritus, and bullae. Conclusion Intralesional interstitial bleomycin injection is a feasible approach for early-stage AVMs and yields safe and effective outcomes. © RSNA, 2017.

A clinical study of Timolol Maleate Eye Drops for the treatment of superficial infantile hemangiomas / 中华整形外科杂志

<p><b>OBJECTIVE</b>To dicuss the clinical efficacy of Timolol Maleate Eye Drops in the treatment of superficial infantile hemangiomas. Methods From April 2012 to May 2014, 210 patients with superficial infantile hemangiomas were included. According to the parents' choice, a total of 176 cases were treated with Timolol Maleate Eye Drops as the treatment group, and the 34 cases who received the treatment of "wait and see" was included in the control group. In the treatment group, the gauzes were dipped into the eye drops and putted evenly on the surface of the hemangioma, 3-4 times daily and lasted for more than 20 minutes. The gauze should completely cover the surface of the tumor. The follow-up periods were 3 weeks and 6 months after treatment with the pictures to record the treatment effect. The therapeutic effect was graded as: grade I (unable to control the growth of the hemangioma), II (the growth of the hemangioma stagnated), III (hemangioma significantly subsided), IV (the hemangioma completely disappeared). The effective rate included the cases with grade II and above grade II . The cure cases included the cases with grade IV. The data was analyzed with the statistical software SPSS 17.0 and the Chi-square test (P < 0.05).</p><p><b>RESULTS</b>3 cases in the treatment group showed eczema action. Tumor ulcer happened in 1 case in treatment group. The side effect rate was 2.3% . The results at 3 weeks following in the treatment group showed that the growth of the hemangioma were stagnated in 154 cases. The color of hemangioma became darker in different degrees than before, and the texture of the hemangioma became soft in majority of children, and the thickness of hemangioma became thinner in some cases. However, only 4 cases showed the hemangiomas were subsided, 18 cases showed the color of the part of the hemangiomas were brighter than before, and 12 cases of the hemangiomas remained original state in the control group. The results of 6 weeks following the treatment showed that 18 patients in the treatment group reached the standard of the grade IV, 84 patients reached the standard of the grade III, 60 patients achieved in the standard of grade II, and only 14 patients showed the volume of hemangiomas were increased as grade I. The effective rate was 58. 0% , and the cure rate was 10. 2% in treatment group. In control group, no children reached the standard of the grade IV, 4 cases reached the standard of grade III, 13 cases who remained original state reached the standard of grade II, and 17 cases showed the volume of hemangiomas continued to increase as grade I . The effective rate was 11. 8% , and the cure rate was 0. By comparison, the effective rate and the cure rate in the control group were relatively lower than those in the treatment group (P < 0.05).</p><p><b>CONCLUSIONS</b>The efficacy of Timolol Maleate Eye Drops in the treatment of superficial infantile hemangioma is exact, especially in the proliferative phase of the infantile hemangioma. It is safe and easy to perform with mild side effect. It should be selected as first-line treatment.</p>

Treatment of superficial infantile hemangiomas with timolol: Evaluation of short-term efficacy and safety in infants.

Timolol has been demonstrated to be efficacious in the topical treatment of superficial infantile hemangiomas (IHs). We conducted a prospective study to evaluate the short-term efficacy and safety of timolol in the treatment of superficial IH in Chinese infants. From March to November 2012, 124 patients with superficial IHs were included in the prospective study. The patients were divided into two groups: treatment (101 patients, the timolol drops were administered on the surface of the lesions three times daily, and erythromycin ointment was applied around the lesions) and observation (23 patients, without treatment). The results were categorized into three grades: class 1 (ineffective), class 2 (controlled growth) and class 3 (promoted regression). Within one week of the initiation of timolol treatment, a number of the lesions became softer and lighter in color. Four months following the initiation of timolol treatment, the overall response was class 1 in eight patients (7.9%), class 2 in 36 patients (35.6%) and class 3 in 57 patients (56.4%). Complete tumor regression was observed in 12 patients. No adverse effects were recorded during the treatment period. Among the patients in the observation group, there were 15 class 1 patients (65.2%), seven class 2 patients (30.4%) and only one class 3 patient (4.3%). In conclusion, timolol is an effective and safe treatment for superficial IH. In addition, it may be used in the treatment of proliferative superficial IH, particularly in infants within 6 months of age.

RET 3'UTR polymorphisms and its protective role in Hirschsprung disease in southeastern Chinese.

BACKGROUND: Hirschsprung disease (HSCR) is a complex congenital disorder characterized by intestinal obstructions owing to the absence of the intestinal ganglion cells of the nerve plexuses in variable lengths of the digestive tract. Several RET polymorphisms and haplotypes have been described as underrepresented in HSCR patients with respect to controls. We thus sought to investigate whether polymorphisms in RET 3'UTR are associated with isolated HSCR in the Chinese population. METHODS: Polymerase chain reaction amplification and direct sequencing were used to screen polymorphisms in RET 3'UTR in patients with sporadic HSCR and ethnically matched controls in Han Chinese populations. Association tests of RET 3'UTR variants and haplotypes with HSCR were performed. RESULTS: We examined a total of 107 Chinese sporadic HSCR patients and 89 ethnically matched controls by sequencing the 3'UTR of the RET gene. Five single nucleotide polymorphisms (SNPs) and 2 monomorphic SNPs were identified. The genotype distributions and the allele frequencies of the 5 SNPs were significantly different between HSCR cases and controls and occurred more frequently in the control population. Haplotype analysis has shown a higher frequency of haplotypes comprising variant alleles in controls as compared with cases. CONCLUSIONS: The significant deviations of the genotype distributions and the allele frequencies of these SNPs in the HSCR population compared with the control population demonstrate that these SNPs have a strong negative association with HSCR and could act as protective alleles.

Association analysis of the SOX10 polymorphism with Hirschsprung disease in the Han Chinese population.

BACKGROUND: Hirschsprung disease (HSCR, Online Mendelian Inheritance in Man 142623) is a typical developmental disorder of the enteric nervous system in which ganglion cells fail to innervate the lower gastrointestinal tract during embryonic development. SOX10 gene is involved in the normal development of the enteric nervous system. Heterozygous SOX10 mutations have been identified in patients with syndromic HSCR. However, no mutations have been reported to date to be associated to isolated HSCR patient. We thus sought to investigate whether mutations in the SOX10 are associated with isolated HSCR in the Chinese population. METHODS: Polymerase chain reaction amplification and direct sequencing were used to screen 4 exons of the SOX10 gene for mutations and polymorphisms in 104 patients with sporadic HSCR and 96 ethnically matched controls in Han Chinese populations. RESULTS: In this study, 4 single nucleotide polymorphisms (SNPs) were identified: SNP1: c.18C>T (GAC→GAT) in exon 2; SNP2: c.122G>T (GGC→GTC) in exon 2; SNP3: IVS2+10 (C→G) in intron 2; and SNP4: c.927T>C (CAT→CAC) in exon 4. SNP1 and SNP2 were novel described polymorphisms in the Chinese population. No SOX10 mutations were found in Han Chinese with isolated HSCR. CONCLUSIONS: Our results revealed that there was no association between the 4 SNPs of the SOX10 gene and HSCR. This study showed that the SOX10 gene is unlikely to be a major HSCR gene in the Chinese Han population.

Inflammatory myofibroblastic tumor of the rectum in a 13-month-old girl: a case report.

Inflammatory myofibroblastic tumor is a rare benign neoplasm. It is common in children and has been reported in various locations throughout the body but rarely in the rectum. A 13-month-old girl presented with a short history of a painless anal mass and no hematochezia. The mass was completely excised, and histologic examination of the initial biopsy showed fascicles of spindle cells in a mixed inflammatory background with predominance of plasma cells, typical of an inflammatory pseudotumor. The spindle cells were positive for smooth muscle actin and anaplastic lymphoma kinase staining. There is no evidence of recurrence or metastasis after a follow-up of 4.5 years.

Association analysis of the RET proto-oncogene with Hirschsprung disease in the Han Chinese population of southeastern China.

Hirschsprung disease (HSCR) is a complex congenital disorder characterized by intestinal obstructions caused by the absence of the intestinal ganglion cells of the nerve plexuses in variable lengths of the digestive tract. This study investigated a possible role of the RET proto-oncogene in sporadic HSCR patients in the Han Chinese population. Our results indicated that rs1800858, rs1800860, rs1800863, and rs2075912, located in exons 2, 7, 15, and intron 19 of RET, are strongly associated with the disease (P < 0.01), with rs1800860 and rs1800863 playing a protective role in the pathogenesis of HSCR in the Chinese population. We also showed that the haplotype consisting of four SNPs is significantly associated with HSCR. We did not find a significant difference in the CA-repeat in intron 5 of RET between cases and controls. Our study provided further evidence that the RET gene is involved in the susceptibility to HSCR in the Han Chinese population.

Association analysis of the PHOX2B gene with Hirschsprung disease in the Han Chinese population of Southeastern China.

BACKGROUND: Hirschsprung disease (HSCR, OMIM 142623) is a complex congenital disorder characterized by intestinal obstructions caused by the absence of the intestinal ganglion cells of the nerve plexuses in variable lengths of the digestive tract. The PHOX2B gene is involved in neurogenesis and disruption of Phox2b in mice results in a HSCR-like phenotype. The first association study of the PHOX2B gene with HSCR derived from Chinese population in Hong Kong; here, we address the question of whether PHOX2B acts as a predisposing factor in HSCR pathogenesis in Chinese population in mainland. METHODS: To investigate the contribution of PHOX2B to the HSCR phenotype, polymerase chain reaction amplification and direct sequencing were used to screen PHOX2B coding regions and intron/exon boundaries for mutations and polymorphisms in 102 patients with HSCR and 96 ethnically matched controls, in Han Chinese populations of Southeastern China. RESULTS: In this study, we genotyped 4 single nucleotide polymorphisms (SNPs) (including 1 novel SNP) located within the PHOX2B gene. Statistically significant differences were found for c.701 A > G and IVS2 + 100 A > G, and the log-additive model was accepted as the best inheritance model (odds ratio [OR], 1.79; 95% confidence interval [CI], 1.11-2.87) for IVS2 + 100 A > G. We also showed that the haplotype-A G A N composed of 4 SNPs exhibited significant association with the disease (P = .03); this haplotype was more frequently observed in cases than in controls (OR, 2.31; 95% CI, 1.11-4.82). CONCLUSIONS: Our study provided further evidence that the PHOX2B gene is involved in the susceptibility to HSCR in the Han Chinese population. Our findings are in accordance with the involvement of PHOX2B in the signaling pathways governing the development of enteric neurons.

RET polymorphisms and the risk of Hirschsprung's disease in a Chinese population.

Hirschsprung's disease (HSCR) is a congenital disorder characterized by intestinal obstructions due to the absence of enteric ganglia along variable lengths of the intestinal tract. RET coding mutations have been found in approximately 50% of familial cases, but they only explain a minority of sporadic cases. Here, we report our investigation of a possible role of RET non-coding mutations in sporadic HSCR patients. The haplotypes of seven single nucleotide polymorphisms (SNPs), all located in a region 4 kb upstream of the gene through to 23 kb of intron 1, and one SNP in exon 2 were constructed in 125 Han Chinese patients with sporadic HSCR and in 148 Han Chinese controls. Our results indicated that eight SNPs were significantly associated with HSCR (P < 0.0001). The C allele of rs2505535 would appear to represent a protecting allele for the Chinese population. One single haplotype composed of these eight markers was present in 59.6% of patients, versus 18.1% of controls. Based on our results, we conclude that non-coding mutations in RET have important roles in the development of HSCR. The unknown functional disease variant(s), with a dosage-dependent effect in HSCR, is likely to be located in the 5'-region of the RET gene.