X-linked agammaglobulinemia (XLA) is a primary immunodeficiency disorder caused by germline mutations of B-cell tyrosine kinase (BTK) gene. It is characterized by decreased serum immunoglobulins levels and circulating mature B cells. This defect in humoral immunity leads to increased susceptibility to infection. Pyoderma gangrenosum (PG) is an uncommon, ulcerating, neutrophilic dermatosis. Here we report PG in an 8-year-old patient with XLA. The patient received intravenous immunoglobulin treatment in conjunction with prednisone and topical application of 0.03% tacrolimus ointment and the ulcer was almost completely healed in the 2 weeks of follow-up. The coexistence has been rarely reported. XLA may be a possible cofactor in the pathogenesis of PG.
Agammaglobulinemia* ; B-Lymphocytes ; Child ; Follow-Up Studies ; Germ-Line Mutation ; Humans ; Immunity, Humoral ; Immunoglobulins ; Neutrophils ; Prednisone ; Protein-Tyrosine Kinases ; Pyoderma Gangrenosum* ; Pyoderma* ; Skin Diseases ; Tacrolimus ; Ulcer ; X Chromosome

OBJECTIVETo evaluate the effect of the compound of Dan-shen root and azone for scar treatment.

METHODSThe rat skin in vitro and the human skin in vitro and vivo were separately examined their permeability of the mixture of the Dan-shen root and azone. The 301 patients with hypertrophic scar were randomly divided into two groups: one treated with elastic cloth paste (including silicone) contained in Dan-shen root with azone, and the another treated with only elastic cloth paste (including silicone).

RESULTSThe permeability of Dan-shen root with azone, passing through the rat skin in vitro and the human skin in vitro and vivo was significantly higher than both the distilled water and the normal saline (P < 0.05). In the clinical study for treatment of the hypertrophic scars, the efficient rate of the group with the Dan-shen root with azone was significantly higher than the control (91.4% vs. 71.3%) (P < 0.01).

CONCLUSIONThe Dan-shen root with azone could be easier to permeate the skin and more effective to treat the hypertrophic scar.

Adolescent ; Adult ; Aged ; Animals ; Azepines ; pharmacology ; Child ; Child, Preschool ; Cicatrix ; drug therapy ; Drugs, Chinese Herbal ; pharmacology ; Female ; Fibrinolytic Agents ; pharmacology ; Humans ; Male ; Middle Aged ; Phenanthrolines ; pharmacology ; Phytotherapy ; Rats ; Skin ; drug effects ; pathology ; Treatment Outcome

OBJECTIVETo discuss the change of blood supply pattern in visceral arteries of Stanford B dissection. The visceral arteries include celiac trunk (CA), superior mesenteric artery (SMA) and renal artery (RA).

METHODSBy retrospectively analysing the clinical data of 52 cases with Stanford B dissection, the blood supply pattern of visceral arteries was confirmed by aortography and the changes before and after endovascular repair were compared.

RESULTSAfter repair: the stenosis lesions disappeared in 7 cases supported by true channel completely but one. Twenty-two visceral arteries supported by true and false channel simultaneously recovered true channel chiefly but one. One recovered true channel chiefly and one had no change in 2 visceral arteries supported by false channel completely. Four recovered true channel chiefly and one had no change in 5 visceral arteries without blood support. 88.9% blood support got better and 11.1% blood support had no change in 36 damaged visceral arteries after endovascular repair.

CONCLUSIONBlood support from true and false channel simultaneously is the chief pattern in the injured visceral arteries before repair; Endovascular repair technique is benefit to recovering the blood support of true channel.

Adult ; Aged ; Aneurysm, Dissecting ; physiopathology ; surgery ; Aortic Aneurysm ; physiopathology ; surgery ; Celiac Artery ; physiopathology ; Female ; Humans ; Male ; Mesenteric Artery, Superior ; physiopathology ; Middle Aged ; Regional Blood Flow ; Renal Artery ; physiopathology ; Retrospective Studies

The aneurysms at the initial segment of splenic artery are rare. This paper aimed to investigate the methods to treat the true aneurysm at the initial segment of splenic artery by aneurysmectomy plus vascular reconstruction. Retrospectively reviewed were 11 cases of true aneurysm at the initial segment of splenic artery who were treated in our hospital from January 2000 to June 2013. All cases were diagnosed by color ultrasonography, computer tomography (CT) and angiography. Upon resection of the aneurysm, the auto-vein transplantation was performed in situ between the hepatic artery and the distal part of the splenic artery in 1 case; the artificial vessel bypass was done between the infra-renal aorta and distal portion of the splenic artery in 7 cases; the splenectomy was done in 2 cases; the splenectomy in combination with ligation of multiple small aneurysms were performed in 1 case. All cases were cured and discharged from the hospital 10-14 days after operation. A 1-14 year follow-up showed that 9 cases survived, and 2 cases died, including 1 case who died of acute myocardial infarction 2 years after aorta-splenic artery bypass operation and 1 case who died of acute cerebral hemorrhage 5 years after aneurysm resection and the splenectomy. Among 6 cases receiving aorta-splenic artery bypass, 1 gradually developed stenosis at anatomosed site, which eventually progressed to complete occlusion 2 years to 6 years after operation, without suffering from splenic infarction because the spleen was supplied by the short gastric vessel and its collaterals. The other 5 cases receiving aorta-splenic artery bypass and 1 case undergoing autologous vascular transplantation did not develop stricture or pseudoaneurysm at the stoma. Our study showed that the aneurysmectomy plus vascular reconstruction is a better treatment for aneurysm at the initial segment of splenic artery.
Aneurysm ; surgery ; Blood Vessel Prosthesis Implantation ; methods ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Splenic Artery ; surgery ; Survival Analysis ; Treatment Outcome ; Veins ; transplantation

OBJECTIVETo study the current situation and factors influencing the behavior of fruit and vegetable intake in adolescents, and to discuss health education strategy related to the behavior.

METHODS1677 students were selected from primary school and middle school in urban area of Hangzhou by a multistage sampling method. The behavior of fruit and vegetable intake was surveyed by questionnaires of the patient-centered assessment and counseling for exercise plus nutrition project (PACE+) for adolescent-scale of fruit and vegetable consumption, and analyzed by Transtheoretical Model on the servings of intake, the stage of change, process of change, decisional balance and the current status of self-efficacy.

RESULTSThe average intake of fruit and vegetable in adolescents was (3.21 +/- 1.50) servings per day ("one serving" means 100 g cooked vegetable or 100 g fruit). The behavior of consuming fruit and vegetable in most of the adolescents was in the contemplation stage, accounting for 36.91% (619/1677). The process of behavior change, decisional balance (Pros) and self-efficacy existed a positive correlation with the process of stage (r(process) = 0.38, r(decisional balance (Pros)) = 0.26, r(self-efficacy) = 0.33, t values were 16.78, 11.02 and 14.31, P < 0.05). The servings of fruit and vegetable intake existed a positive correlation with stage transition and self-efficacy (beta(stage transition) = 0.665, t = 35.07, P < 0.05; beta(self-efficacy) = 0.050, t = 2.63, P < 0.05), and existed a negative correlation with decisional balance (Cons) (beta(decisional balance (Cons)) = -0.051, t = -2.84, P < 0.05).

CONCLUSIONFruit and vegetable intake of these adolescents are under the recommended dietary intake. Along with behavior stage development and self-efficacy improvement, the intake of fruit and vegetable also increased correspondingly. Intervention strategies should aim at improving the awareness of adolescents on the health benefits of fruit and vegetable intake and promoting their confidence to reach the recommended servings.

Adolescent ; Adolescent Nutritional Physiological Phenomena ; Child ; China ; Eating ; Feeding Behavior ; Female ; Fruit ; Humans ; Male ; Models, Theoretical ; Students ; Surveys and Questionnaires ; Vegetables

OBJECTIVETo prevent and manage frequent complications after endovascular repair of infrarenal abdominal aortic aneurysm (AAA).

METHODSThe data of 71 cases of infrarenal abdominal aortic aneurysm (AAA) treated by endovascular repair were analysed retrospectively. The reasons, managements, results and prognosis of frequent complications were investigated.

RESULTSSeventy-one cases of infrarenal AAA were treated by endovascular repair with 100% success rate. There was no surgical conversion to open aneurysm repair. There were 8 cases of primary endoleak, 1 case of nervous complication and acute thrombosis. An average follow-up period was 26 +/- 5 months. Three persistent endoleaks and 4 secondary endoleaks were found during the follow-up period. The endoleak rate was 9.8% (7/71) within 1 month postoperatively and mortality rate was 1.3% (1/71). Total mortality rate was 4.2% (3/71). Two patients died from acute myocardial infarction and one from acute heart failure.

CONCLUSIONSEndovascular treatment of abdominal aortic aneurysm is technically feasible and can effectively exclude aortic aneurysms from the circulation. Endoleak is a chief complication after endovascular repair of infrarenal AAA.Additional procedures and follow up are very important. Endoleak with enlarged aneurysm should be treated actively.

Aged ; Aged, 80 and over ; Aortic Aneurysm, Abdominal ; surgery ; Blood Vessel Prosthesis Implantation ; adverse effects ; methods ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Postoperative Complications ; etiology ; prevention & control ; therapy ; Prognosis ; Retrospective Studies ; Stents ; Treatment Outcome ; Vascular Fistula ; etiology ; prevention & control ; therapy

OBJECTIVETo investigate the methods and effects of secondary intervention for mid-long term complications of endovascular repair (EVR) in aortic diseases.

METHODSFrom May 1999 to Jun 2007, 21 patients with mid-long term complications after EVR were treated in our center. Of these cases, 15 cases received first EVR for abdominal aortic aneurysm (AAA), 3 cases for thoracic aortic aneurysm (TAA) and 3 cases for aortic dissection (TAD). The mid-long term complications included 11 cases of type I endoleak, 4 cases of type II endoleak, 2 cases of type III endoleak and 4 cases of migration of stent grafts. Proximal or distal extensions were used for type I and III endoleak in 9 cases. Fenestrated, scallop and bifurcated stent grafts were used to reconstruct the aortic arch in 3 cases. Emboli technique was used in treating type II endoleak. Thrombectomy and bypass technique were used in 4 cases with stent graft limb occlusion. One ruptured AAA accepted open surgery.

RESULTSSecondary endovascular technique were undergone in 20 (95.2%) cases. One case died in 30 days after the secondary intervention and endoleak remained after the secondary operation in 5 cases. Three cases died of the secondary intervention.

CONCLUSIONSEndoleak and limb occlusion were the chief mid-long complications after EVR. Secondary endovascular technique can be used in most cases and carries great challenges in aortic arch lesions.

Adult ; Aged ; Aged, 80 and over ; Aortic Diseases ; surgery ; Blood Vessel Prosthesis Implantation ; adverse effects ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Postoperative Complications ; etiology ; therapy ; Retrospective Studies ; Stents ; adverse effects ; Treatment Outcome

OBJECTIVETo investigate the treatments of left subclavian artery (LSA) in endovascular repair (EVR) of thoracic aortic aneurysm (TAA) and thoracic aortic dissection (TAD).

METHODSIn 54 TAD or TAA cases, all of the proximal landing zone (PLZ) were less than 15 mm and only the LSA was needed to be treated in EVR, the following methods and techniques were used in the treatments of LSA: complete cover, partial cover, endovascular reconstruction following complete cover, surgical reconstruction before complete cover.

RESULTSDSA was used to evaluate the condition of cerebral circulation in all cases. Forty left subclavian arteries were covered completely. Ten were covered completely after right subclavian artery (RSA)-LSA or left common carotid artery (LCCA)-LSA bypass. PTA and stent in LSA was done in 3 cases. In 1 case, LSA was covered completely first, and then the graft was punctured and bare stent was fixed after inflation by cutting balloon. All of the ancillary techniques were enforced successfully. No severe complications were found in brain and upper extremity. The proximal endoleak rate was 17% (9/54). In the 40 cases whose LSA were not reconstructed, the primary left subclavian steal syndrome (LSSS) happened in 8 cases (20%) and the primary average systolic pressure of left brachial artery was 63 +/- 24 mm Hg.

CONCLUSIONSEVR can be enforced safely and efficiently in TAA and TAD with short PLZ by some ancillary endovascular or surgical techniques. The methods to treat the LSA depend on the condition of the cerebral circulation.

Adult ; Aged ; Aged, 80 and over ; Aneurysm, Dissecting ; surgery ; Aortic Aneurysm, Thoracic ; surgery ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Subclavian Artery ; surgery

OBJECTIVETo evaluate the initial and long-term results of endovascular aneurysm repair (EVAR) in high-surgical-risk abdominal aortic aneurysm (AAA) patients.

METHODSFrom July 1997 to July 2011, 120 consecutive high-surgical-risk patients with AAA who were treated electively using a bifurcated aortic endograft were entered in a registry. There were 96 male and 24 female patients, aged from 52 to 95 years with a mean of 74 years. Follow-up protocol consisted of computed tomography angiograms or ultrasound performed at 3, 6, 9, 12 months, and annually thereafter. The main goal was evaluation of the operative mortality and the long-term survival of these patients. Secondary goals were determination of the frequency of secondary operations, the outcome of the aneurysm sac, and primary and secondary patency rates after aortic endograft placement.

RESULTSMean aneurysm diameter was (57 ± 8) mm. Thirty-seven patients were operated under local anesthesia and eighty-three under general anesthesia. Five type I endoleaks, twenty-five type II endoleaks and one type III endoleak occurred during the perioperative period. The technical success rate was 95%. Operative mortality was 2.5%. The survival rates at 1-, 3- and 5-year were 92%, 75% and 43% respectively. The mean follow up was (36 ± 3) months. Primary and secondary patency rates at 3 year were respectively 97% and 100%. Secondary intervention rate was 10% (12/120) at 5 year. The reasons included endoleaks for 7 patients, stent-grafts fracture for 2 patients, stent-grafts migration for 2 patients and stent-graft thrombosis for 1 patients.

CONCLUSIONSInitial and long-term results with endograft repair of AAA in high-surgical-risk patients were satisfactory. These results appear to justify endovascular repair for this patient population.

Aged ; Aged, 80 and over ; Aortic Aneurysm, Abdominal ; surgery ; Blood Vessel Prosthesis ; Blood Vessel Prosthesis Implantation ; methods ; Female ; Humans ; Male ; Middle Aged ; Stents ; Treatment Outcome

OBJECTIVETo assess the feasibility and safety of excising or patching the inferior vena cava (IVC) without replacement in patients with primary retroperitoneal tumors (PRPT) involving the IVC.

METHODSA retrospective analysis was conducted in 116 consecutive patients with PRPT presented to our Hospital between December 2007 and December 2008. IVC involvement was found in 11.2% of the cases, and in 93 cases receiving surgical tumor removal, the complete resection rate was 93.55%. According to the location of IVC involvement by the PRPT, the cases were classified into 3 groups with IVC involvement in the segment from the second hepatic portal vein to the renal vein (segment A), the segment on the bilateral renal vein plane (segment B), and the segment from the caval bifurcation to the renal vein (segment C).

RESULTSThe most common vascular involvement occurred in segment C (61.54%, 8/13), and 2 cases presented with segment A involvement and 2 had segment B involvement. All the 3 segments of IVC were involved in 1 case. Five cases with IVC involvement received IVC patching only, and 4 had resection or ligation of the segment C of the IVC, and resection of the segment A and B of the IVC was performed in 2 and 1 case, respectively. One patient received complete resection of whole IVC involved. All patients recovered smoothly and were discharged.

CONCLUSIONThe infrarenal IVC can be ligated or resected safely without reconstruction. Combined resection of the bilateral renal vein and segment B of the IVC may result in renal insufficiency. IVC involvement and occlusion between the second hepatic portal and renal veins can be ligated safely without affecting the renal function.

Adult ; Feasibility Studies ; Female ; Humans ; Ligation ; Male ; Middle Aged ; Neoplasm Invasiveness ; Retroperitoneal Neoplasms ; pathology ; surgery ; Retrospective Studies ; Vascular Surgical Procedures ; methods ; Vena Cava, Inferior ; pathology ; surgery ; Young Adult