Venous malformations of the lower limb with severe localized intravascular coagulopathy treated with radiofrequency ablation and resection.

Diffuse venous malformations (VMs) are relatively rare, especially the lesions locting special anatomical sites, and they are prone to casuse localized intravascular coagulopathy (LIC). Diffuse VMs can also cause bleeding and life-threatening disseminated intravascular coagulopathy (DIC) from trauma, surgery, and improper treatments. Thus, the treatment of diffuse VMs with LIC is quite tough. We report of a diffuse VMs with severe LIC that was treated with the combined use of minimally invasive treatment and open surgery.

Venous malformations with severe localized intravascular coagulopathy treated with microwave ablation.

OBJECTIVES: To evaluate the safety and feasibility of microwave ablation for treating venous malformations (VMs) with severe localized intravascular coagulopathy (LIC). PATIENTS AND METHODS: Data for patients with the diagnosis of VMs coupled with severe LIC who underwent color Doppler-guided microwave dynamic ablation between January 2017 and June 2019 were retrospectively reviewed and analyzed. All patients had previously received sclerotherapy or other treatments with poor outcomes and gradual aggravation of coagulation abnormalities. Microwave treatment with "dynamic ablation" was performed with real-time color Doppler monitoring and was repeated if necessary after 3 months. Low-molecular-weight heparin (LMWH) was used to control consumptive coagulopathy. The therapeutic efficacy including coagulation function and lesion size was evaluated using the four-level scale developed by Achauer. RESULTS: Among 15 patients with extensive diffuse or multiple VMs, 10 patients presented with lesions in a single lower extremity, one in both lower extremities and the perineum, one in both upper extremities and the trunk, and three with multiple lesions. The patients underwent a total of 74 microwave ablation sessions, with an average of 4.9 sessions per person. Coagulation abnormalities were temporarily aggravated in 59 sessions within the first seven days post-ablation but improved to grade II (fair) a week later. From six months to three years after the ablation, the lesions improved to grade IV (excellent) in one patient, grade III (good) in six patients, and grade II (fair) in eight patients. Moreover, the coagulation function improved to grade IV in four patients, grade III in eight patients, and grade II in three patients, resulting in an efficiency rate of 80% (12/15). Post-ablation complications included fever, hemoglobinuria, and elevations in aspartate aminotransferase, lactate dehydrogenase, and alanine aminotransferase. The patients with fever and hemoglobinuria recovered after specific therapeutic measures, but elevations in aspartate aminotransferase, lactate dehydrogenase, and alanine aminotransferase recovered spontaneously without further interventions. CONCLUSIONS: Ablation coupled with anticoagulation can effectively treat VMs in patients with severe LIC and improve the long-term coagulation function.

Analysis of 18 complex diffuse arteriovenous malformation cases treated with percutaneous radiofrequency ablation.

BACKGROUND: The aim of the present study is to evaluate the short-term efficacy and feasibility of radiofrequency ablation in the treatment of complex diffuse arteriovenous (AV) malformations. METHODS: The data of 18 patients (8 male and 10 female) with complex AV malformations treated between December 2014 and June 2019 were analyzed retrospectively. The lesion area was 10 × 7 cm ~ 28 × 30 cm. Under duplex ultrasound guidance, the site with the most abundant blood flow signals in the lesion was percutaneously punctured with the radiofrequency ablation needle (electrode). The impedance automatic adjustment mode was adopted, and ablation was monitored usingduplex ultrasoundduring the entire process. RESULTS: Of the included patients, 1 had a high fever after two rounds of treatment, 2 had transient hemoglobinuria, and 1 had tissue necrosis in the original ruptured tumor area as well as a penetrating defect in the cheek, which was repaired with a pedicled trapezius myocutaneous flap. In 9 patients who experienced bleeding, the bleeding stopped after one round of treatment. During the follow-up period of 1-5 years, there were 0 grade I (poor) cases, 0 grade II (medium) cases, 7 grade III (good) cases, and 11 grade IV (excellent) cases. CONCLUSION: The "high power and continuous" radiofrequency ablation technique conducted under real-time duplex ultrasoundmonitoring can completely destroy the deep core lesions of AV malformations and effectively control life-threatening massive hemorrhage; it is an effective alternative treatment method for complex diffuse AV malformations in which interventional embolization, sclerotherapy, and surgery are ineffective.

KETCH1 imports HYL1 to nucleus for miRNA biogenesis in Arabidopsis.

MicroRNA (miRNA) is processed from primary transcripts with hairpin structures (pri-miRNAs) by microprocessors in the nucleus. How cytoplasmic-borne microprocessor components are transported into the nucleus to fulfill their functions remains poorly understood. Here, we report KETCH1 (karyopherin enabling the transport of the cytoplasmic HYL1) as a partner of hyponastic leaves 1 (HYL1) protein, a core component of microprocessor in Arabidopsis and functional counterpart of DGCR8/Pasha in animals. Null mutation of ketch1 is embryonic-lethal, whereas knockdown mutation of ketch1 caused morphological defects, reminiscent of mutants in the miRNA pathway. ketch1 knockdown mutation also substantially reduced miRNA accumulation, but did not alter nuclear-cytoplasmic shuttling of miRNAs. Rather, the mutation significantly reduced nuclear portion of HYL1 protein and correspondingly compromised the pri-miRNA processing in the nucleus. We propose that KETCH1 transports HYL1 from the cytoplasm to the nucleus to constitute functional microprocessor in Arabidopsis This study provides insight into the largely unknown nuclear-cytoplasmic trafficking process of miRNA biogenesis components through eukaryotes.

[Subsequent therapy for infantile hemangiomas after discontinuation of oral propranolol].

PURPOSE: To summarize the subsequent therapy experiences for infantile hemangiomas after discontinuation of oral propranolol treatment, and explore the relationships between clinical interventions and types of infantile hemangioma. METHODS: In this retrospective study from January 2010 to May 2014, a total of 137 infants with hemangiomas undergoing sequential therapy after oral propranolol treatment. There were 41 males and 96 females. The median age was 16 months, ranging from 14 to 25 months. After oral propranolol treatment, the outcomes were evaluated to be grade III in 74 cases, grade IIin 62 cases and grade Ⅰ in 1 case. The types were papula (n=31), telangiectasis (n=11), plump (n=74), deep (n=12) and compound (n=9). The primary sites were 3 cases in scalp, forty-nine in face, thirty-three in trunk, thirty-eight in extremities. Cutis laxa presented in 45 cases, and parenchyma hypertrophy presented in 80 cases. Sequential therapy were performed including laser therapy for 38 cases, intralesional Pingyangmycin injection for 63 cases, and plastic surgery for 16 cases. The efficacy was re-evaluated on a 4-level scale, combined with evaluations of scar, cutis laxa or pigment alteration. SPSS18.0 software package was used for statistical analysis. RESULTS: Chi-square test showed significant differences between 5 types in occurrence of cutis laxa and parenchyma hypertrophy (x(2)=28.458,68.276, P<0.01). After a follow-up of 6 months to 4 years, the outcomes were evaluated to be grade IV in 122 cases, grade III in 15 cases, without grade IIor gradeⅠ case. There were significant differences in 5 types of infantile hemangiomas before and after sequential therapy( H=53.445, 9.941, 120.324, 17.000, 18.899, P<0.01). Postoperative scar was presented in 2 cases around to joints, and mild pigment alteration was noticed in 2 cases after intralesional Pingyangmycin injection. CONCLUSIONS: Cutis laxa and parenchyma hypertrophy may be more likely present in infantile hemangiomas after oral propranolol treatment. Laser therapy is recommended for patients with papula or telangiectasis, when necessary, intralesional Pingyangmycin injection should be combined. Intralesional Pingyangmycin injection should be the first choice for plump or compound type，whereas surgery should be executed in patients with deep type or other plump type complicated by severe hyperplasia, after which combined intralesional Pingyangmycin injection may be required for postoperative superficial residues.

Clinical observations in mesh suture treatment for infants of Kasabach-Merritt phenomenon.

AIM: This study aims to evaluate the efficacy and adverse effects of the mesh suture treatment for infants of Kasabach-Merritt phenomenon and to report our treatment experience. METHODS: Of the three patients, two of the cases occurred in the scalp and one occurred in the back of the chest, with platelet counts < 40 × 109 /L before the treatment, reduced fibrinogen levels and increased D-dimer levels. All the three patients underwent the mesh suture treatment of the tumour area. Post-treatment observations were made regarding the surface colour and texture of the tumours, periodic routine blood examination results, fibrinogen and D-dimer levels. RESULTS: After treatment, the degree of swelling on the tumour surface was reduced, surface tension was decreased, the tumour colour turned pale red from dark red and the skin gradually returned to normal. Two days after treatment, the platelet counts increased to 70 × 109 /L or higher; the platelet count reached a normal level after 1 week in two cases. The fibrinogen level increased in varying degrees after treatment, whereas the D-dimer level was reduced. One case showed a small amount of exudate at the suture area, 1 month after the treatment; improvements were observed 3 days after changing the local dressing. All the patients were followed up for 6 months to 1 year, during which the patients showed complete regression of the lesion and relapse, normal platelet counts and normal fibrinogen and D-dimer levels. CONCLUSIONS: We recommend the mesh suture treatment as the first treatment of choice for paediatric Kasabach-Merritt phenomenon.

[Clinical analysis for treatment of 1 080 cases of infantile hemangiomas with oral propranolol].

OBJECTIVE: To explore the indications of hemangiomas of different types by observing the clinical efficacy of oral propranolol. METHODS: For this retrospective study from October 2009 to June 2013, a total of 1 080 cases were classified into 5 types according to their clinical characteristics. There were 338 males and 742 females. Their types were telangiectasis (n = 58), papular (n = 424), plump (n = 106), deep (n = 176) and mixed (n = 306). Propranolol was orally administered at a dose of 1.0 or 1.5 mg/kg daily (1.0 mg/kg for infants aged 2.5 months or under; 1.5 mg/kg for those aged 2.5 months or above). Dynamic observations of hemangioma size, texture or color change and adverse events during treatment were performed. Drug withdrawal was usually made after dosing for 1 year or under when there was a total regression of hemandiomas. The efficacy was evaluated on a 4-level scale. RESULTS: Rank test results showed no significant differences between 5 types during the changes of lesions (χ² = 1.738, P > 0.05). Changing of lesions occurred 8.0 (3.7, 16.2) hours after dosing in telangiectasis type, whereas 6.5 (4.1, 14.3) hours in papular type, 7.0 (5.5, 12.7) hours in plump type, 7.5 (3.8, 11.3) hours in deep type and 6.5 (4.2, 13.4) hours in mixed type. After a follow-up of 6 months to 2.5 years, there were 378 grade IV case (35.00%), 574 grade III case (53.15%), 120 grade II cases (11.11%) and 8 grade I cases (0.74%). And there were significant differences between patients of different types (P < 0.05). Patients of deep type had the best grade IV curative effect rate of 59.09% (104/176) , then papular type of 51.25% (162/316) and telangiectasis type of 26.41% (112/424). CONCLUSION: As a first-line treatment for hemangioma, propranolol shows excellent efficacies for patients of deep, mixed, plaque and papular types.

Bidirectional processing of pri-miRNAs with branched terminal loops by Arabidopsis Dicer-like1.

MicroRNAs (miRNAs) originate from primary transcripts (pri-miRNAs) with characteristic stem-loop structures, and their accurate processing is required for the production of functional miRNAs. Here, using the pri-miR-166 family in Arabidopsis thaliana as a paradigm, we report the crucial role of pri-miRNA terminal loops in miRNA biogenesis. We found that multibranched terminal loops in pri-miR-166s substantially suppress miR-166 expression in vivo. Unlike canonical processing of pri-miRNAs, terminal loop-branched pri-miRNAs can be processed by Dicer-like 1 (DCL1) complexes bidirectionally from base to loop and from loop to base, resulting in productive and abortive processing of miRNAs, respectively. In both cases, DCL1 complexes canonically cut pri-miRNAs at a distance of 16-17 bp from a reference single-stranded loop region. DCL1 also adjusts processing sites toward an internal loop through its helicase domain. These results provide new insight into the poorly understood processing mechanism of pri-miRNAs with complex secondary structures.

[Efficacy of conservative laparoscopic surgery combined with goserelin in treatment of 206 patients with severe ovarian endometriosis at short-term and long-term follow-up].

OBJECTIVE: To evaluate the short-term and long-term efficacy of conservative laparoscopic surgery combine with goserelin in treatment of severe ovarian endometriosis. METHODS: From January 2004 to December 2008, 206 patients with severe ovarian endometriosis underwent laparoscopy surgery in Nanjing Drum Tower Hospital, Affiliated Nanjing University Medical School were enrolled in this retrospective study. According to the revised classification American Fertility Society (r-AFS), 123 (123/206, 59.7%) cases were at stage III and 83 (83/206, 40.3%) patients were at stage IV. Among 138 cases presented pelvic pain. All the patients underwent laparoscopic cystectomy, of which 117 patients with childbearing preserving underwent hysteroscopy and hydrotubation examination, including 7 cases with bilateral salpingectomy, 2 cases with bilateral tubal obstruction and 108 cases with normal reproduction. After surgery, all cases were administered by goserelin treatment at dose of 3.6 mg per 28 days for 3 to 6 months. At 1 to 5 years following up, pelvic pain, pregnancy and recurrence were observed, those factors associated with pregnancy rate and endometriosis recurrence were analyzed. RESULTS: (1) Pelvic pain: complete remission rate of pelvic pain was 76.1% (105/138) at 1 to 5 years after surgery. (2) Pregnancy: total pregnancy rate was 70.4% (76/108), spontaneous pregnancy rate was 68.8% (66/96) and pregnant rate of in vitro fertilization and embryo transfer (IVF-ET) was 10/12. Pregnancy rate at 1 year was 57.3% (55/96) and accounting for 83.3% (55/66) in all pregnant women. Live birth rates of spontaneous pregnant and IVF-ET were 86.4% (57/66) and 9/10, respectively. (3) Recurrence: the total recurrence rate was 8.3% (17/206) at 1 to 5 years. The recurrence rates and the cumulative recurrence rates were 3.9% (8/206) and 3.9% (8/206) at the first year after operation, 2.0% (3/149) and 6.7% (10/149) at the second year, 1.0% (1/99) and 8.0% (8/99) at the third year, 10.9% (5/46) and 17.4% (8/46) at the fourth year, 0 and 2/18 at the fifth year, respectively. CONCLUSION: It was suggested that conservative laparoscopic surgery combined with goserelin in treatment of stage III or IV ovarian endometriosis could reduce the recurrence risk of severe ovarian endometriosis and improve the pregnant rate of endometriosis-associated infertility.

[Treatment of infantile parotid hemangioma with propranolol].

OBJECTIVE: To evaluate the short-term results and safety of propranolol for the treatment of infantile parotid hemangioma. METHODS: Oral propranolol was administered to 17 infants with parotid hemangioma at a dose of 1.0-1.5 mg per kilogram of body weight per day. The patients were revisited once a week. The changes of the tumor size, texture and colour were monitored and recorded at a regular interval. The adverse effects after medication were observed and managed accordingly. The short-term results were evaluated using a 4 scales system. RESULTS: Among the 17 patients treated, the follow-up time was 5 to 10 months. The overall response was scale I in 0 patient, scale II in 0 patients, scale III in 5 patients, and scale IV in 12 patients. No serious adverse effects were encountered. CONCLUSIONS: Oral propranolol at a lower dose is a safe and effective method for the treatment of infantile parotid hemangioma. The short-term results were excellent and the side effects minimal.