[Clinical effects of anterolateral thigh flap with blood supply source of medial femoral perforator in repairing the wounds on extremities].

Objective: To investigate clinical effects of anterolateral thigh flap with blood supply source of medial femoral perforator in repairing the wounds on extremities. Methods: A retrospective observational study was conducted. From October 2018 to June 2021, Suzhou Ruihua Orthopedic Hospital admitted 12 patients (11 males and 1 female, aged 24-64 years) who met the inclusion criteria, and did not have the lateral circumflex femoral artery during anterolateral thigh flap resection, or changed to medial femoral perforator as a source of blood supply due to small perforator to repair the wound with flap transplantation. The wounds were on the upper limbs in 7 cases and on the lower limbs in 5 cases. The wound area after debridement was 8.0 cm×5.0 cm-24.0 cm×6.5 cm, and the applied flap area was 9 cm×6 cm-25 cm×7 cm. During transplantation, the perforating branch or source artery of the flap was anastomosed end to end with the vascular pedicle of the recipient area. Subcutaneous beauty suture was performed on all the donor wounds. After operation, the survival of the flap and vascular crisis were observed; and the wound healing at the donor site was observed. During follow-up, the texture of the flap and complications at the donor site were observed. At the last follow-up, the effect of flap repair was evaluated by using the modified self-made comprehensive efficacy rating table. Results: After operation, all the flaps of patients survived without vascular crisis; the wounds in the donor site healed smoothly. During follow-up of 6 to 26 months, the flaps had good texture; only linear scars remained in the donor area without scar contracture, pain or other discomfort. At the last follow-up, the total score of flap repair effect of 12 patients was 75-95, with an average of 87, of which 5 cases were rated as excellent, 6 cases as good, and 1 case as fair, and the proportion of excellent and good patients was 11/12. Conclusions: The application of anterolateral thigh flap with blood supply from the medial femoral perforator to repair wounds on extremities is an alternative method for the vascular variations such as no descending branch, oblique branch, or small perforator of the lateral circumflex femoral artery during the resection of the anterolateral thigh flap. This operation has the advantages of simple operation, small trauma, and others, which effectively ensures the smooth operation.

[Diagnostic value of nasal nitric oxide for children with primary ciliary dyskinesia].

Objective: To evaluate the value of nasal nitric oxide (nNO) measurement as a diagnostic tool for Chinese patients with primary ciliary dyskinesia (PCD). Methods: This study is a retrospective study. The patients were recruited from those who were admitted to the respiratory Department of Respiratory Medicine, Children's Hospital of Fudan University from March 2018 to September 2022. Children with PCD were included as the PCD group, and children with situs inversus or ambiguus, cystic fibrosis (CF), bronchiectasis, chronic suppurative lung disease and asthma were included as the PCD symptom-similar group. Children who visited the Department of Child health Care and urology in the same hospital from December 2022 to January 2023 were selected as nNO normal control group. nNO was measured during plateau exhalation against resistance in three groups. Mann-Whitney U test was used to analyze the nNO data. The receiver operating characteristic of nNO value for the diagnosis of PCD was plotted and, the area under the curve and Youden index was calculated to find the best cut-off value. Results: nNO was measured in 40 patients with PCD group, 75 PCD symptom-similar group (including 23 cases of situs inversus or ambiguus, 8 cases of CF, 26 cases of bronchiectasis or chronic suppurative lung disease, 18 cases of asthma), and 55 nNO normal controls group. The age of the three groups was respectively 9.7 (6.7,13.4), 9.3 (7.0,13.0) and 9.9 (7.3,13.0) years old. nNO values were significantly lower in children with PCD than in PCD symptom-similar group and nNO normal controls (12 (9,19) vs. 182 (121,222), 209 (165,261) nl/min, U=143.00, 2.00, both P<0.001). In the PCD symptom-similar group, situs inversus or ambiguus, CF, bronchiectasis or chronic suppurative lung disease and asthma were significantly higher than children with PCD (185 (123,218), 97 (52, 132), 154 (31, 202), 266 (202,414) vs. 12 (9,19) nl/min,U=1.00, 9.00, 133.00, 0, all P<0.001). A cut-off value of 84 nl/min could provide the best sensitivity (0.98) and specificity (0.92) with an area under the curve of 0.97 (95%CI 0.95-1.00, P<0.001). Conclusions: nNO value can draw a distinction between patients with PCD and others. A cut-off value of 84 nl/min is recommended for children with PCD.

Analysis results of 169 cases of chorionic villus samples of missed abortion using high throughput sequencing.

OBJECTIVE: This study aimed to evaluate the use of high-throughput sequencing (HTS) technology to detect chromosomes in chorionic villus samples of missed abortion embryos and investigate its utility in the genetic diagnosis of missed abortion. PATIENTS AND METHODS: HTS was used to assess chorionic villus samples obtained from 169 patients with missed abortions from August 2020 to March 2022, at the Second Affiliated Hospital of Guangxi Medical University. The test results were statistically analyzed. To investigate the impact of advanced age on the incidence of chromosomal abnormalities, the patients were divided into two groups: elderly (≥35 years) and nonelderly pregnant women (<35 years). RESULTS: (1) Among the samples of 169 patients, 100 (59.17%) cases of chromosomal abnormalities were detected. Among these 100, 90 (90%) had chromosomal numerical abnormalities and 10 (10%) had chromosomal structural abnormalities. (2) Chromosomal numerical abnormality was abnormalities mainly included aneuploidy (92.22%, 83/90), with trisomy (62.22%, 56/90) and monosomy (22.22%, 20/90) accounting for the majority. The top three numerical abnormalities included 18 cases of Turner syndrome (monosomy X; 20%, 18/90), 10 cases of trisomy 16 (11.11%, 10/90), and 10 cases of trisomy 22 (11.11%, 10/90). (3) Villous chromosomal abnormalities were found in 48 (70.59%) elderly pregnant women, and 52 (51.48%) nonelderly pregnant women, with statistically significant differences (p < 0.05). CONCLUSIONS: (1) Chromosomal abnormality is an important cause of missed abortion, it majorly includes chromosomal numerical abnormality, of which most cases are of aneuploidy. (2) Advanced age may increase the risk of embryonic chromosomal abnormalities. (3) Villus chromosome detection using HTS has a positive value and can be used for analyzing and determining the causes of missed abortion.

[Clinical effects of anterolateral thigh perforator flap with sensory nerves in repairing the plantar skin and soft tissue defects].

Objective: To explore the clinical effects of anterolateral thigh perforator flap with sensory nerves in repairing the plantar skin and soft tissue defects. Methods: From January 2016 to March 2019, 13 male patients with plantar skin and soft tissue defects were admitted to the Department of Foot and Ankle Surgery of Ruihua Affiliated Hospital of Soochow University, aged 27 to 73 years. The retrospective cohort study was conducted. The wounds of 4 patients underwent 2 times of debridement. The wounds of 9 patients underwent 1 time of debridement and 1 or 2 time(s) of vacuum sealing drainage. Then all the wounds of patients were repaired with flaps when the wounds were clean and dry with no purulent exudation and were negative in secretion culture. The wound areas of this group of patients after wound debridement were 13.0 cm×5.5 cm to 36.0 cm×10.5 cm, and the wounds were repaired with anterolateral thigh perforator flaps with sensory nerves with area of 14 cm×6 cm to 37 cm×11 cm. The wound of 1 patient was repaired with a bilobed flap. The oblique or descending branch of the lateral circumflex femoral artery and its accompanying vein in the flap were anastomosed with the posterior tibial artery and its accompanying vein or the medial plantar artery and its accompanying vein in recipient sites. The lateral femoral cutaneous nerve in the flap was anastomosed with the recipient saphenous nerve or medial plantar cutaneous nerve. The donor sites were directly sutured. The survival of flaps and the healing of wounds in the donor and recipient areas were recorded, and the ulcers of the plantar repaired flap were followed up for 3 months after operation. The sensory function of the flap was evaluated by the sensory evaluation standard of British Medical Research Council (BMRC), and the ankle and foot function score system of American Orthopedic Foot and Ankle Society (AOFAS) was used for comprehensive functional evaluation at the last follow-up. Results: All the 14 flaps in 13 cases survived, and venous crisis occurred in two cases and the flaps survived smoothly after the venous re-anastomosis. One patient developed deep tissue infection after being discharged and was healed after secondary debridement. The donor and recipient areas of the remaining patients healed well. The patients were followed up for 6 to 18 months, the shape of flaps was slightly bloated in 5 patients, and the shape and texture of flaps in the other 8 patients were good. Six patients had ulcers in flaps at 3 months of follow-up after operation, which were healed after stopping weight-bearing activities. At the last follow-up, little linear scar hyperplasia could be seen in the donor site of patients; the BMRC sensory function evaluation results were S1 grade in 4 cases, S2 grade in 7 cases, and S3 grade in 2 cases; the AOFAS scores were excellent in 3 cases, good in 7 cases, fair in 2 cases, and poor in 1 case. Conclusions: The anterolateral thigh perforator flaps with sensory nerves can repair the plantar skin and soft tissue defects with the donor sites directly sutured and good shape of flaps, which provide a good treatment method for the recovery of plantar proprioceptive sensation and weight-bearing function.

[Clinical effects of bilateral overlength anterolateral femoral perforator flaps connected in series or parallel in repairing large area of wounds in limbs].

Objective: To explore the clinical effects of bilateral overlength anterolateral femoral perforator flaps connected in series or parallel in repairing large area of wounds in limbs. Methods: From January 2017 to July 2019, 9 patients with large area of skin and soft tissue defects in limbs were admitted to the Departments of Hand Surgery and Foot and Ankle Surgery of Ruihua Affiliated Hospital of Soochow University, including 8 males and 1 female, aged 36 to 63 years. The retrospective cohort study was conducted. The wound areas of patients after debridement were 20 cm×15 cm to 30 cm×25 cm, and the wounds were repaired with bilateral overlength anterolateral femoral perforator flaps. One main artery defect in the receiving area of 4 patients was repaired with bilateral flaps connected in series. Two main artery defects in the receiving area of 5 patients were repaired with bilateral flaps connected in parallel. A total of 18 flaps were excised, and the area of a single flap ranged from 20 cm×8 cm to 46 cm×9 cm. The donor sites of 17 flaps were sutured directly, and the donor site of 1 flap was repaired with free full-thickness skin graft from hypogastrium. Harvesting time of flaps, survival condition of flaps after surgery, and wound healing time, and flap observation, two-point discrimination distance of flaps, functional recovery of joint and appearance of recipient site, and recovery of donor site during follow-up were recorded. Results: In this group of 9 patients, the flap harvesting time was 1.0 to 4.5 hours, and all the 18 flaps survived. The wound healing time of recipient site was 18 to 72 days after flap transplantation. They were followed up for 6 to 34 months. The shape of the recipient site was satisfactory, with no deep tissue infection such as osteomyelitis. Four flaps in 2 patients were bloated and were thinned in 6 months after operation; 4 flaps in 2 patients had skin pigmentation on the edge of the flap; the flap of one patient was scalded but healed by dressing change, with patchy scar being observed during follow-up. The rest of the flaps were soft, elastic, and painless with good blood supply. All the flaps restored with protective sensation, with only one point in two-point discrimination. Only linear scars remained in the donor sites of 17 flaps. All the limbs had good blood supply in the distal end of donor sites, and no restriction occurred in range of motion of knee joint and quadriceps muscle strength. Conclusions: The bilateral overlength anterolateral femoral perforator flaps connected in series or parallel have constant anatomy, reliable blood supply, and flexible combination. It is an ideal surgical method for repairing large area of skin and soft tissue defects in limbs at one time.

[Pulmonary actinomycosis in children: a case report and literature review].

Objective: To summarize the clinical characteristics, imaging features, diagnosis, treatment and prognosis of pulmonary actinomycosis in children. Methods: The clinical data of a child with pulmonary actinomycosis who was hospitalized in Children's Hospital, Zhejiang University School of Medicine in December 2019 was retrospectively analyzed. The related literature published from January 1975 to January 2020 was retrieved from Wanfang, CNKI and PubMed databases with "pulmonary" or "thoracic" and "actinomycosis" and "pediatric" or "children" or "child" as the keywords. And the characteristics of pediatric pulmonary actinomycosis were summarized based on the literature review. Results: The patient was a boy aged 12 years and 6 months. He was admitted due to cough and chest pain for more than 20 days, with fever on the first three days. The chest CT scan in local hospital found inflammatory lesions in the right middle lobe, which was also suspected to be cavitation. The flexible bronchoscopy showed congestion and edema of bronchial mucosa in the right middle lobe, and bronchoalveolar lavage fluid smear was positive for acid-fast bacilli DNA, although both purfied protein derivatives tuberculin test and T-spot were negative. During the hospitalization, the child had persistent cough and chest pain, but no fever. Pathogen metagene sequencing of the bronchoalveolar lavage fluid detected Actinomyces (sequence number: 222) and Grevini Actinomycetes (sequence number: 185). The boy received intravenous cefoperazone sulbactam sodium for 2 weeks followed by oral amoxicillin clavulanate potassium for 6 weeks. Until April 2020, his clinical symptoms completely relieved, and the pulmonary lesions were significantly absorbed on the latest chest CT scan. Eight articles and 62 children with pulmonary actinomycosis were reported, but no related reports were retrieved from CNKI and Wanfang databases. The youngest case was 27 months old. The clinical presentations of this disease were nonspecific. The main symptoms included chest wall masses (8 cases), cough (23 cases), pain (chest, back, shoulders and armpits) (24 cases), fever (25 cases), weight loss (26 cases), etc. Conclusions: The clinical manifestations and imaging features of pediatric pulmonary actinomycosis are nonspecific, therefore it could easily be misdiagnosed. For children with pneumonia of unknown etiology and failing to respond to routine antibiotics, the pathogen metagene sequencing of the bronchoalveolar lavage fluid will be helpful for diagnosis. With appropriate course of antibiotic treatment, the prognosis is good in most cases.

[Clinical features of pulmonary thromboembolism of eight children].

Objective: To improve the diagnosis and therapy of childhood pulmonary thromboembolism (PTE) by analyzing the clinical features of this rare condition. Methods: A total of 8 pediatric patients (4 males, 4 females) with PTE diagnosed in the Children's Hospital of Zhejiang University School of Medicine from March, 2014 to March, 2019 were enrolled. The clinical manifestation, laboratory results, imaging findings, diagnosis and treatment were summarized. Results: Among these 8 cases, aged from 9 hours to 14 years and 10 months. Fever was found in 4 cases, cough aggravation in 4, short of breath in 3, chest pain in 2, abdominal and back pain in one, hemoptysis in 2, cyanosis in 1, and edema of lower extremities in 2. Physical examination found decreased breath sound in 2 cases, phlegm rale in 3, and pleural friction rub in one. Pleural effusion was found in 5 cases by ultrasound. Plasma D-dimer increased in 6 cases (0.66-9.96 mg/L) and hypersensitive C-reactive protein elevated in 5 cases (10.78-78.00 mg/L). Chest enhanced CT showed pulmonary artery or venous filling defects, including pulmonary artery embolism in 7 cases and pulmonary vein embolism in one. The primary disease of these patients included Mycoplasma Pneumoniae pneumonia in 4 cases, nephritis in 2 and postoperative congenital heart disease in 2. Apart from one case who withdrew the treatment and was discharged, the other 7 patients received anticoagulant treatment had good outcome. Conclusions: For children with Mycoplasma pneumoniae pneumonia, immune disorders, long-term hormone therapy, cardiovascular invasive operation or other high-risk factors, PTE should be considered when fever, cough aggravation, short of breath, chest and back pain with pleural effusion are present. Chest enhanced CT scan should be performed as soon as possible, and anticoagulation should be started once the diagnosis is confirmed.

[Multiple free homologous superficial peroneal artery perforator flaps of crus for repair of multiple hand wounds].

Objective: To explore the effects of multiple free homologous superficial peroneal artery perforator flaps of crus for repair of multiple hand wounds. Methods: From November 2017 to December 2018, eight cases with eighteen hand wounds were hospitalized in our unit. Among them, wounds were distributed in the forefinger and middle finger in four cases, wounds were distributed in the middle finger and ring finger in two cases, wounds were distributed in the forefinger, middle finger, and ring finger in one case, and wounds were distributed in the middle finger, ring finger, and little finger in one case. The area of skin defect ranged from 1.5 cm×0.8 cm to 4.0 cm×3.0 cm. There were 4 males and 4 females, aged 34-62 years. Wounds of six cases were repaired by two free superficial peroneal artery perforator flaps from homolateral crus, and those of two cases were repaired by three free superficial peroneal artery perforator flaps from homolateral crus. Superficial peroneal artery and its accompanying vein of flap were anastomosed by end to end with digital artery and palmar or dorsal subcutaneous vein of recipient site during the operation. The area of flap ranged from 2.5 cm×1.2 cm to 5.0 cm×4.0 cm. No nerve was harvested during the operation, and donor site was sutured directly. The survival of the flaps and the healing of donor sites were recorded. During follow-up, the recovery of donor and recipient sites was observed. Results: All flaps survived well, donor site healed well. No vascular crisis occurred. Follow-up for 4 to 12 months showed that the appearance of flap was satisfactory with good color, texture, elasticity, and function. Protective sensation of recipient site was recovered. Five months after operation, flap of finger pulp in one case was swollen slightly with two-points discrimination of 10 mm, which received the thinning surgery. Obvious scar formation was not observed in donor site of crus. The appearance of the donor site was good without functional damage. Conclusions: The application of multiple free homologous superficial peroneal artery perforator flaps of crus to repair the multiple hand wounds has advantages of easy acquisition, easy operation, little effect on donor sites, and satisfactory clinical effects.

[Comparative analysis of clinical characteristics and prognosis between bacterial necrotizing pneumonia and Mycoplasma pneumoniae necrotizing pneumonia in children].

Objective: To compare the characteristics of Mycoplasma pneumoniae necrotizing pneumonia (MPNP) and bacterial necrotizing pneumonia (BNP), and explore the biomarkers for differentiation of MPNP from BNP. Methods: A retrospective, observational study of 52 necrotizing pneumonia (NP) cases who were hospitalized in our hospital from January 2008 to December 2017 was conducted. According to the pathogen causing NP, patients were divided into two groups, BNP and MPNP, and the clinical manifestations, laboratory data, imaging findings, hospital course and prognosis between these groups were analyzed. Results: This study enrolled 19 boys and 33 girls, and the median ages of patients were 4.4 (0.1-13.8) years old. Of the totally of 52 NP patients, 19 were in the BNP group (9 boys and 10 girls), 33 were in the MPNP group (10 boys and 23 girls). The mean age of MPNP patients was much older than that of BNP patients (5.2 (2.3-13.2) years vs. 1.8 (0.1-13.8) years, Z=-0.128, P<0.01). The number of patients with tachypnea and pleural effusion septation were significantly higher in BNP patients than those in MPNP patients (15 cases vs. 4 cases, χ(2)=23.222, P<0.01; 14 cases vs. 1 case, χ(2)=29.326, P<0.01), which more needed to oxygentherapy (18 cases vs. 12 cases, χ(2)=16.833, P<0.01) and undergo chest drainage (9 cases vs. 4 cases, χ(2)=5.829, P=0.022); while the number of patients required bronchoalveolar lavage was higher in MPNP patients than that in BNP patients (5 cases vs. 32 cases, χ(2)=29.326, P<0.01). The values of white blood cell (WBC) (23.2 (5.2-67.1)×10(9)/L vs. 9.7 (6.3-18.7)×10(9)/L, Z=-4.855, P<0.01), procalcitonin (PCT) (3.69 (0.23-90.15) mg/L vs. 0.28 (0.02-1.44) mg/L, Z=-3.207, P=0.001), C reactive protein (CRP) (160 (94-220) mg/L vs. 90 (5-134) mg/L, Z=-4.337, P<0.01), interleukin (IL)-10 (11.7 (4.2-401.5) ng/L vs. 4.8 (2.0-23.4) ng/L, Z=-2.278, P=0.023), pleural fluid cell count (5 200 (120-50 000)×10(6)/L vs. 790 (68-6 920)×10(6)/L, Z=-3.125, P=0.002), pleural fluid lactic dehydrogenase (LDH) (3 990 (589-29 382) U/L vs. 2 211 (673-3 993) U/L, Z=-2.488, P=0.013) in BNP group were significantly higher than those in MPNP group; while the values of pleural fluid glucose(0.43 (0.03-18.00) mmol/L vs. 5.95 (4.27-7.87) mmol/L, Z=-2.795, P=0.005), serum tumor necrosis factor (TNF)-α (2.3 (1.0-2.8) ng/L vs. 2.6 (1.3-109.2) ng/L, Z=-2.113, P=0.035) and interferon (IFN)-γ (4.8 (2.6-7.7) ng/L vs. 11.9 (2.9-154.6) ng/L, Z=-2.455, P=0.014) were lower in BNP group than those in MPNP group. Meanwhile, the mean time from the onset of symptoms to the discovery of necrotic lesions was longer in MPNP group than that in BNP group ((20.6±6.4) days vs. (14.6±6.2) days, t=3.029, P=0.004). After treatments, all patients were discharged without death, WBC and PCT recovered more quickly in MPNP group than those in BNP group (12 (0-24) days vs. 0 (0-23) days, Z=-4.484, P<0.01; 10 (5-15) days vs. 0 (0-23) days, Z=-3.244, P=0.001). As to prognosis, 34 cases were followed up, and the results showed that patients recovered without surgical intervention, and chest lesions were resolved within 3.0 (1.0-8.0) months, and the time to necrosis disappearance was similar in the BNP group and MPNP greup (3.0 (1.0-8.0) months vs. 3.0 (1.0-8.0) months, Z=-0.128, P=0.001). In receiver operator characteristic curve analysis, the cut-off values for the age, WBC, CRP, PCT, pleural fluid cell count and pleural fluid glucose were set at 2.4 years of age, 17.2×10(9)/L, 157 mg/L, 1.505 mg/L, 2 630×10(6)/L and 3.73 mmol/L, respectively. Conclusions: NP is found to be severe and prolonged, yet, reversible through proper therapy, such as rational antibiotics application. The age, WBC, CRP, PCT, pleural fluid cell count and pleural fluid glucose could be used as biomarkers to differentiate MPNP from BNP in children.

[Multisystemic smooth muscle dysfunction syndrome in children: a case report and literature review].

Objective: To analyze the clinical characteristics and diagnosis of multisystemic smooth muscle dysfunction syndrome(MSMDS). Method: Clinical data of a case diagnosed as MSMDS and hospitalized in our hospital in July 2016 was retrospectively analyzed. Literature search was performed at databases of PubMed, Wanfang, China National Knowledge Infrastructure and VIP with the key words "multisystemic smooth muscle dysfunction syndrome" "ACTA2" . The literature retrieval was confined from January 1980 to November 2016.The characteristics of MSMDS were summarized through review of literature. Result: A girl aged 1.6 years had recurrent cough and wheeze for more than 1 year, complicated with congenital fixed dilated pupils, patent ductus arteriosus, pulmonary hypertension, chronic lung disease, and cerebrovascular abnormalities. We had done gene analysis for the patient and found ACTA2 c. 536C>T(p.R179H) heterozygous mutations, but her parents were normal. Totally 11 reports were retrieved from foreign language literature and no report from Chinese literature could be found; the retrieved articles reported a total of 25 cases of multiple system smooth muscle dysfunction syndrome. The minimum age was 11 months, 17 cases were female, 8 were male. The clinical common characteristic is congenital fixed dilated pupils, patent ductus arteriosus, cerebrovascular disease, pulmonary hypertension, chronic lung disease, and so on. Conclusion: Genetic testing for ACTA2 gene mutations should be considered in infants presenting with congenital fixed dilated pupils and patent ductus arteriosus.

The safety profile of subcutaneous allergen immunotherapy in children with asthma in Hangzhou, East China.

BACKGROUND: The aim of the current study is to evaluate the prevalence, severity and possible risk factors of systemic reactions (SRs) to subcutaneous allergen immunotherapy (SCIT) in children and adolescents with asthma in Hangzhou, east China's Zhejiang province. METHODS: From January 2011 to December 2016, this survey analysed the SCIT-related SRs involving 429 patients (265 children and 134 adolescents) affected by allergic asthma. Recorded data included demographics, diagnosis, patient statuses, pulmonary function testing results before and after each injection, allergen dosage, and details of SRs. RESULTS: All patients finished the initial phase and six patients withdrew during the maintenance phase. There were 2.59% (328/12,655) SRs in all injections (3.28% in children and 1.47% in adolescents); 15.62% (67/429) patients experienced SRs (18.49% children and 10.98% adolescents). There were 54.57% SRs of grade 1; 42.37% SRs of grade 2; 3.05% SRs of grade 3; and no grades 4 or grade 5 SRs occurred in patients. Most reactions were mild, and were readily controlled by immediate emergency treatment. There was no need for hospitalisation. The occurrence of SRs was significantly higher in children than that in adolescents (p<0.01). A higher ratio of SRs was found among patients with moderate asthma. CONCLUSION: This retrospective survey showed that properly-conducted SCIT was a safe treatment for children and adolescents with asthma in Hangzhou, East China. Children and patients with moderate asthma may be prone to develop SRs.

[Clinical analysis of seven cases of pulmonary malignant tumors in children].

Objective: To understand the clinical manifestation, imaging characteristics and outcomes of pulmonary malignant tumors in children. Method: We retrospectively collected information about seven cases of pulmonary malignant tumors in children in our hospital from Jan 2010 to Dec 2016. The information included clinical manifestation, imaging characteristics, pathologic results, and treatment. Result: (1) All the seven patients firstly visited pediatric internal medicine departments. Symptoms included cough (n=6), dyspnea (n=4), fever (n=2), anorexia (n=2), chest tightness (n=1), chest pain (n=1), lameness (n=1), abdominal distension and constipation (n=1). We did not find hemoptysis, wheezing or weight loss in those patients. Physical examinations revealed unilateral reduced breath sounds (n=5), moist rales and wheezes (n=1), and normal (n=1). Extrapulmonary signs included abdominal distension (n=2), left hip tenderness (n=1), and mass in left scrotum. We did not identify clubbing finger, anemic appearance, lymph node enlargement, or hepatosplenomegaly etc. (2) Laboratory examination results: complete blood count showed white blood cells in normal range except one case (17.44×10(9)/L). Neutrophil percentage ranged from 0.348 to 0.767. C reactive protein ranged between<1 and 162 mg/L. Hemoglobin was normal. Three out of four cases had abnormal blood tumor markers.(3) Imaging results showed multiple nodes (n=3), multiple cystic lesions in lungs (n=2) (both with pleuropulmonary blastoma), endobronchial soft tissue mass (n=1), pulmonary round-shaped mass (n=1), and mediastinal mass (n=1). Imaging results also found atelectasis (n=3), pneumonia (n=2), pneumothorax (n=2), longitudinal diaphragmatic hernia (n=2), pleural effusion (n=1), subcutaneous emphysema (n=1). (4) All the patients underwent tumor puncture biopsy or tumor resection. Pathology revealed the final diagnosis of pleuropulmonary blastoma (n=3), endodermal sinus tumors (n=2), squamous cell carcinoma of lung (n=1), and thyroid papillary carcinoma (n=1). All of them were malignant tumors. We followed up them. Two patients died (both with pleuropulmonary blastoma) after their parents refused any medical help. Two cases were lost to follow-up. Three patients survived (followed up for 19 months, 11 months and tow months, respectively). One case with thyroid papillary carcinoma pulmonary metastasis underwent right thyroid cancer radical plus left thyroid lobe resection plus modified selective central lymph node dissection, then iodine (131) treatment. One case with endodermal sinus tumor pulmonary metastasis underwent three times of chemotherapies, resection of left retroperitoneal tumor and left testicular tumor, and six additional chemotherapies. Another one case of endodermal sinus tumor pulmonary metastasis underwent three times of chemotherapies and was discharged. Conclusion: Pulmonary malignant tumors are rare diseases in children. Clinical signs are often non-specific. For those with chest CT showing multiple cystic lesions, endobronchial soft tissue mass or multiple nodes, but no significant infection manifestation or no response to anti-infection therapy, pulmonary malignant tumors should be considered. Biopsy may be needed to confirm the final diagnosis.

Sonic hedgehog through Gli2 and Gli3 is required for the proper development of placental labyrinth.

Sonic hedgehog (Shh) functions as a conserved morphogen in the development of various organs in metazoans ranging from Drosophila to humans. Here, we have investigated the potential roles and underlying mechanisms of Shh signaling in murine placentation. Immunostaining revealed the abundant expression of the main components of Shh pathway in both the trophectoderm of blastocysts and developing placentas. Disruption of Shh led to impaired vascularogenesis of yolk sac, less branching and malformation of placental labyrinth, thereby leading to a robust decrease in capacity of transplacental passages. Moreover, placenta-specific gene incorporation by lentiviral transduction of mouse blastocysts and blastocyst transplantation robustly knocked down the expression of Gli3 and Gli2 in placenta but not in embryos. Finally, Gli3 knockdown in Shh(-/-) placentas partially rescued the defects of both yolk sac and placental labyrinth, and robustly restored the capacity of transplacental passages. Gli2 knockdown in Shh(+/)(-) placentas affected neither the capacity of tranplacental passages nor the vascularogenesis of yolk sac, however, it partially phenocopied the labyrinthine defects of Shh(-/-) placentas. Taken together, these results uncover that both Shh/Gli2 and Shh/Gli3 signals are required for proper development of murine placentas and are possibly essential for pregnant maintenance.