Outcomes of Self-Expanding Covered Stents for the Treatment of External ILIAC Artery Obstructive Disease.

PURPOSE: To describe the early results and mid-term patency rates of external iliac artery (EIA) stenting using self-expanding covered stents. METHODS: We conducted a multicenter retrospective study (2015-2021), including patients receiving primary endovascular treatment of external iliac artery occlusive disease with self-expanding covered stents. All patients were treated with the Viabahn (W.L Gore & Associates, Inc., Flagstaff, AZ-USA) stent. Patency and limb salvage rates were estimated with Kaplan-Meier curves. RESULTS: Ninety-three patients (mean age, 69 ± 9 years; 81% males) were treated for disabling claudication in 44%, rest pain in 28%, and tissue loss in 28%. TASC C/D lesions were present in 72% and iliac complete occlusion in 30%. Mean lesion length was 6.9 ± 2.4 cm; 30% had moderate/severe EIA calcifications; and the mean iliac tortuosity index was 1.17 ± 0.13. Technical success was 100%. There was one perioperative death (1.4%) and procedural complication rate was 6.5%. At 42 months (mean, 25 months), primary patency was 89.8% (95%CI 83-98); the presence of EIA tortuosity (tortuosity index > 1.25, 87.7 ± 11% vs 89.9 ± 8%; P = .6) or severe calcifications (87.6 ± 9% vs 96.0 ± 8%; P = .400) had no significant impact. After univariate analysis, the use of a stent with diameter < 8 mm (HR 8.5, 95%CI 3.24-14.22; P < .001) was negatively associated with primary patency. CONCLUSIONS: The use of self-expanding covered stents provided excellent early and mid-term results in the treatment of obstructive disease of the EIA, also in cases of high EIA tortuosity and high grade of calcifications. The use of a < 8 mm-diameter stent was associated with a reduced primary patency.

Treatment of Isolated Idiopathic Growth Hormone Deficiency in Children and Thyroid Function: Is the Need for LT4 Supplementation a Concern in Long-Term Therapy?

Introduction Recombinant human growth hormone (rhGH) replacement therapy might be able to induce hypothyroidism, but this is a controversial issue. Previous studies evaluated the effects of rhGH replacement therapy on thyroid function, but little information is available in the subset of children with isolated idiopathic growth hormone deficiency (GHD). Our aim was to assess the effects of rhGH replacement therapy on thyroid function in children with isolated idiopathic GHD. Methods Retrospective analysis of the medical files of 64 children with confirmed GHD treated with rhGH. After review, 56 children with isolated idiopathic GHD and treated with rhGH for at least one year were included. Auxological (weight standard deviation score [SDS], height SDS, growth velocity [GV] SDS) and biochemical (free thyroxine [FT4], thyroid-stimulating hormone [TSH], and insulin-like growth factor 1 [IGF-1]) parameters were recorded before, during, and after treatment with rhGH. Results FT4 and TSH levels decreased significantly during rhGH therapy in children with isolated idiopathic GHD. Twenty-one percent (n=12) of the children developed hypothyroidism, on average 47 months after initiation of rhGH. Higher baseline FT4 levels were protective against the need for levothyroxine (LT4) (OR=0.8, CI 0.592-0.983; p=0.036). Hypothyroidism was reversed after interruption of rhGH, except in one patient; FT4 levels returned to baseline in the first year after completing the treatment. Final height SDS of the children who developed hypothyroidism was not different from their counterparts without hypothyroidism (-1.24 [-1.52 to -1.10] vs -1.13 [-1.78 to -0.74], p=1.000). Predicted adult height (PAH) SDS in patients who completed rhGH treatment was similar in both LT4 supplemented (n=7; final Ht SDS -1.16 [-1.31 to -1.10] vs PAH -1.00 [-1.42 to -0.48]; p=0.398) and not supplemented patients (n=25; final Ht SDS -1.46 [-1.83 to -0.78] vs PAH SDS -0.88 [-1.35 to -0.56]; p=0.074). Conclusions Our results show that patients with isolated idiopathic GHD may transiently need LT4 during GH treatment. Properly supplemented patients achieved PAH.

Mechanochemical ablation for great and small saphenous veins insufficiency in patients with type III shunt.

OBJECTIVE: the aim of this study was to assess the results of mechanochemical endovenous ablation either in the primary or recurrent saphenous vein insufficiency, including only patients with veno-venous shunt type III. METHODS: retrospective analysis of a prospective study of patients with symptomatic chronic venous insufficiency who underwent ClariVein® technique. A total of 134 saphenous veins were included between August 2017 and August 2018. Follow-up was performed by Duplex ultrasound at 1, 6 and 12 months. Primary endpoints were technical and anatomical success. Secondary endpoints were the need for further treatment of varicose collateral veins by sclerotherapy, outcomes regarding recurrent insufficiency and clinical success. RESULTS: A total of 111 great saphenous veins and 23 small saphenous veins were treated with a technical success of 95.6%. The overall anatomical success rates at 1, 6 and 12 month were 96.2%, 88.8% and 84.4%, respectively, without differences between primary and recurrent insufficiency. Deferred sclerotherapy over varicose collaterals was carried out in 28% of the patients with anatomical success. Clinical improvement was achieved in 87.3%. CONCLUSIONS: MOCA technique has proven to be an effective technique, although additional treatment over varicose collaterals could be necessary in up to one-third. Atrophy of the saphenous vein and the lack of persistent varicose collateral veins during follow-up seem to be indicators of successful therapy.

Predictive Factors of Aneurysm Sac Growth in Patients with a Type II Endoleak in the First Post-EVAR Control.

BACKGROUND: The objective of this study was to identify predictive factors associated with aneurysm sac growth over time in patients diagnosed with a type II endoleak (TII-EL) in the first postoperative control after endovascular aneurysm repair (EVAR). METHODS: A retrospective analysis of all patients undergoing an elective EVAR procedure from January 2003 to December 2011 (9 years) in a single center was performed. Patients with a TII-EL detected in the first post-EVAR imaging control and with over twelve months of follow-up were included. The primary end point was aneurysm sac growth > 5 mm from the first month computed tomography angiography (CTA) to the last available CTA. Demographic variables, cardiovascular risk factors, comorbidities, aneurysm-related data, and procedural information were collected. Three-dimensional volume rendering with the Mimics ® software (Materialise NV, Leuven, Belgium) was used to measure the endoleak nidus. Descriptive, univariate, and multivariate analyses (Cox proportional hazards model) were performed. RESULTS: In this period, 220 EVAR procedures were performed. 63 TII-ELs (28.7%) were detected in the first CTA control (90.5% male, mean age: 75.7 ± 8 years). After a median follow-up of 54 months (interquartile range [IQR], 56.5), aneurysm sac growth > 5 mm was detected in 19 patients (30.1%). Age (P = 0.02) and dyslipidemia (P = 0.03) were associated with sac growth > 5 mm, whereas the presence of chronic obstructive pulmonary disease (COPD) behaved as a protective factor (P = 0.02). The craniocaudal length of the endoleak nidus (P < 0.01) and the nidus volume (P < 0.001) were the only aneurysm-related variables associated with sac growth. Age (HR: 14.1, 95% CI: 2.1-92.3, P = 0.006) and the presence of COPD (HR: 9.6, 95% CI: 1.4-63.7, P = 0.019) were the only independent predictors in the multivariate analysis. CONCLUSIONS: Reliable predictors of long-term aneurysmal sac growth are lacking, although some variables such as age or nidus volume appear to be associated. Strict surveillance remains mandatory.

Results of Transarterial Embolization for Treating Type 2 Endoleaks: A Single-Center Experience.

BACKGROUND: Type 2 endoleaks (T2Es) are the main cause of reintervention after endovascular repair of abdominal aortic aneurysms (EVAR). The objective of this study is to quantify success rates of T2E treatment. METHODS: This study involves a retrospective analysis of a prospectively maintained database containing data on all consecutive patients treated for a T2E between 2003 and 2017 in a single center. Technical success was defined as absence of endoleak in the final angiographic control after treatment. Clinical success was defined as absence of sac growth over 5 mm in the contrast-enhanced computed tomography performed a year thereafter. Statistics included Kaplan-Meier survival estimates. RESULTS: A total of 528 elective EVARs were performed in the period. Thirty-six of these (6.8%) developed a T2E requiring reintervention, a median of 37.9 months after EVAR. Twenty-five percent of the treatments were performed more than 5 years after intervention. Twenty-eight of the 36 treatments were performed via transarterial embolization. For this technique, technical success was 71.4% and clinical success was 62.5%. A subsequent reintervention was required in 35.7% of patients. In this cohort, the rate of aneurysm rupture was 10.7% (n = 3/28), open surgical conversion was needed in 2 of 28 cases (7.1%), and rate of aneurysm-related death was 14.3% (n = 4/28) over follow-up. CONCLUSIONS: A high percentage of patients are at risk of adverse outcomes after T2E treatment. Strict imaging follow-up is still needed in this population.

CYP21A2 Gene Pathogenic Variants: A Multicenter Study on Genotype-Phenotype Correlation from a Portuguese Pediatric Cohort.

BACKGROUND: Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21OHD) is an autosomal recessive disorder characterized by 3 overlapping phenotypes: salt-wasting (SW), simple virilizing (SV), and non-classic (NC). We aimed at conducting a nationwide genotype description of the CAH pediatric patients and to establish their genotype-phenotype correlation. METHODS: CAH patients were recruited from Portuguese pediatric endocrinology centers and classified as SW, SV, or NC. Genetic analysis was performed by polymerase chain reaction (sequence specific primer, restriction fragment length polymorphism) or direct Sanger sequencing. Genotypes were categorized into 4 groups (0, A, B, and C), according to their predicted enzymatic activity. In each group, the expected phenotype was compared to the observed phenotype to assess the genotype-phenotype correlation. RESULTS: Our cohort comprises 212 unrelated pediatric CAH patients (29% SW, 11% SV, 60% NC). The most common pathogenic variant was p.(Val282Leu; 41.3% of the 424 alleles analyzed). The p.(Val282Leu) variant, together with c.293-13A/C>G, p.(Ile173Asn), p.(Leu308Thr), p.(Gln319*), and large deletions/conversions were responsible for 86.4% of the mutated alleles. Patients' stratification by disease subtype revealed that the most frequent pathogenic variants were c.293-13A/C>G in SW (31.1%), p.(Ile173Asn) in SV (46.9%), and p.(Val282Leu) in NC (69.5%). The most common genotype was homozygosity for p.(Val282Leu; 33.0%). Moreover, we found 2 novel variants: p.(Ile161Thr) and p.(Trp202Arg), in exons 4 and 5, respectively. The global genotype-phenotype correlation was 92.4%. Group B (associated with the SV form) showed the lowest genotype-phenotype correlation (80%). CONCLUSION: Our cohort has one of the largest NC CAH pediatric populations described. We emphasize the high frequency of the p.(Val282Leu) variant and the very high genotype-phenotype correlation observed.

Endovascular Repair of Iliac Vein Laceration Associated with Complex Pelvic Fracture.

BACKGROUND: Iliac vein injury associated with pelvic fracture due to blunt trauma is an uncommon and difficult diagnosis but a life-threatening condition which often requires an emergent management. Although open repair has been traditionally used as the treatment of choice in unstable patients, it is controversial, given the difficulty due to injured vessel exposure in patients with significant retroperitoneal hematoma as well as tamponade effect loss associated with laparotomy. We present a challenging case of iliac vein laceration successfully treated by placement of a self-expanding covered stent. METHODS: A 15-year-old male was hemodynamically unstable and was transferred to our emergency department after a severe polytrauma due to a motorcycle accident. Contrast-enhanced computed tomography showed a left external iliac vein laceration with active bleeding and retroperitoneal hematoma as well as complex pelvic and left supracondylar femoral fractures. A 13 × 100 mm self-expanding covered stent was successfully deployed through duplex ultrasound-guided percutaneous approach of both femoral veins. RESULTS: The patient's blood pressure was normalized as soon as the stent graft was placed, and then femoral fracture was reduced and fixed. At 12-month follow-up, the patient remained asymptomatic, and stent-graft patency was confirmed. CONCLUSIONS: Covered stent-graft placement can be an effective and rapid treatment for life-threatening iliac vein injury.

[Blue toe syndrome as a clinical finding of pheochromocytoma]. / Síndrome del dedo azul como manifestación de feocromocitoma.

Pheochromocytomas are tumors that arise from chromaffin cells of the sympathetic nervous system and act by synthesizing and releasing catecholamines. They usually occur between the fourth and fifth decade of life and have a very wide clinical presentation. They occur only in 0.1-0.2% of the hypertensive population and represent a treatable and curable cause of arterial hypertension, as well as other symptoms derived from the uncontrolled secretion of catecholamines. Peripheral arterial ischemia secondary to massive amines release by a pheochromocytoma is a very uncommon condition. Here we report a case of pheochromocytoma manifested as blue finger syndrome in a patient with palpable distal pulses and history of poor blood pressure control despite treatment with two drugs.

Síndrome del dedo azul como manifestación de feocromocitoma / Blue toe syndrome as a clinical finding of pheochromocytoma

Los feocromocitomas son tumores que proceden de las células cromafines del sistema nervioso simpático y actúan sintetizando y liberando catecolaminas. Suelen presentarse entre la cuarta y quinta década de la vida y tienen presentaciones clínicas muy diversas. Ocurren solamente en 0.1-0.2% de la población hipertensa, constituyen una causa tratable y curable de hipertensión arterial, así como de otras manifestaciones derivadas de la liberación incontrolada de catecolaminas. La isquemia arterial periférica secundaria a la liberación masiva de aminas por un feocromocitoma es muy infrecuente. Aquí se presenta un caso clínico de feocromocitoma manifestado como síndrome del dedo azul en un paciente con pulsos distales conservados y el antecedente de mal control tensional a pesar de tratamiento con dos fármacos.

Pheochromocytomas are tumors that arise from chromaffin cells of the sympathetic nervous system and act by synthesizing and releasing catecholamines. They usually occur between the fourth and fifth decade of life and have a very wide clinical presentation. They occur only in 0.1-0.2% of the hypertensive population and represent a treatable and curable cause of arterial hypertension, as well as other symptoms derived from the uncontrolled secretion of catecholamines. Peripheral arterial ischemia secondary to massive amines release by a pheochromocytoma is a very uncommon condition. Here we report a case of pheochromocytoma manifested as blue finger syndrome in a patient with palpable distal pulses and history of poor blood pressure control despite treatment with two drugs.

Estenosis de arteria ilíaca tras trasplante renal como causa de hipertensión arterial refractaria y claudicación / Stenosis of the iliac artery after kidney transplantation as a cause of refractory hypertension and intermittent claudication

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The ID23-2 structural biology microfocus beamline at the ESRF.

The first phase of the ESRF beamline ID23 to be constructed was ID23-1, a tunable MAD-capable beamline which opened to users in early 2004. The second phase of the beamline to be constructed is ID23-2, a monochromatic microfocus beamline dedicated to macromolecular crystallography experiments. Beamline ID23-2 makes use of well characterized optical elements: a single-bounce silicon (111) monochromator and two mirrors in Kirkpatrick-Baez geometry to focus the X-ray beam. A major design goal of the ID23-2 beamline is to provide a reliable, easy-to-use and routine microfocus beam. ID23-2 started operation in November 2005, as the first beamline dedicated to microfocus macromolecular crystallography. The beamline has taken the standard automated ESRF macromolecular crystallography environment (both hardware and software), allowing users of ID23-2 to be rapidly familiar with the microfocus environment. This paper describes the beamline design, the special considerations taken into account given the microfocus beam, and summarizes the results of the first years of the beamline operation.

The ID23-1 structural biology beamline at the ESRF.

The demand for access to macromolecular crystallography synchrotron beam time continues to increase. To meet this demand the ESRF has constructed a dual station beamline using a canted undulator system as the X-ray source. The first phase of the beamline to be constructed is ID23-1, a tunable MAD-capable station with a mini-focus X-ray beam. The beamline makes use of well characterized optical elements: a channel-cut monochromator with a high-precision toroidal mirror to focus the X-ray beam. The beamline has been conceived with the aim of providing high levels of automation to create an industrial-like environment for protein crystallography. A new software suite has been developed to permit reliable easy operation for the beamline users and beamline staff. High levels of diagnostics are built in to allow rapid trouble-shooting. These developments are now being exported to the ESRF macromolecular crystallography beamline complex and have been made in a modular fashion to facilitate transportability to other synchrotrons.

Automation of macromolecular crystallography beamlines.

The production of three-dimensional crystallographic structural information of macromolecules can now be thought of as a pipeline which is being streamlined at every stage from protein cloning, expression and purification, through crystallisation to data collection and structure solution. Synchrotron X-ray beamlines are a key section of this pipeline as it is at these that the X-ray diffraction data that ultimately leads to the elucidation of macromolecular structures are collected. The burgeoning number of macromolecular crystallography (MX) beamlines available worldwide may be enhanced significantly with the automation of both their operation and of the experiments carried out on them. This paper reviews the current situation and provides a glimpse of how a MX beamline may look in the not too distant future.

Necrosis avascular atraumática de la cabeza femoral. Descompresión más injerto de peroné (Resultados preliminares) / Atraumatic necrosis of the femoral head. Core descompression and fibular bone grafting: preliminary results

Se realizó un estudio prospectivo, longitudinal, no comparativo en 11 pacientes (13 caderas con necrosis) de noviembre de 1993 a febrero de 1995. Los criterios de inclusión fueron: pacientes en estadios I, II o III de la clasificación de Ficat y Arlet. Fueron excluidos los pacientes en estadios IV O III en quienes una prótesis total pudo estar disponible. El examen físico fue ralizado de acuerdo con la escala funcional de Harris. Los cambios estructurales fueron determinados por radiografías y RMN. En todos los pacientes se realizó descompresión de la cabeza del fémur y se utilizó peroné para dar soporte estructural. Los resultados fueron evaluados clínicamante de acuerdo con la escala funcional de Harris, con radiografías simples y con RMN, en esto último sólo en 5 casos (38 por ciento). Los mejores resultados se obtuvieron en los estadios I (100 por ciento) y II (80 por ciento). Todos los casos en estadio III terminaron en reemplazo total de cadera. La importancia de un diagnóstico temprano es enfatizada para lo cual la resonancia magnética nuclear (RMN) es muy importante. Esta técnica es efectiva para el tratamiento de la necrosis avascular de la cabeza femoral en estadios tempranos