Last twenty-years activity of cardiovascular tissue banking in Barcelona.

The Barcelona Tissue Bank was established from the merge of two previous multi-tissue banks. Potential donors are screened by Donor Center staff and multi-tissue retrieval is performed by specialized own teams. Tissue processing and preservation is performed in clean room facilities by specialised personnel. After quality control of both donor and all tissues results, the heart valves and vascular segments are stored until medical request. The aim of this report is to present the cardiovascular tissue activity and retrospectively evaluate the outcomes of the changes performed in last 20 years. Cardiovascular tissue from 4088 donors was received, specifically 3115 hearts and 2095 vascular segments were processed and evaluated. A total of 48% of the aortic valves, 68% of the pulmonary valves and 75% of the vascular segments were suitable for transplant. The main reason for discarding tissue was macroscopic morphology followed by microbiological results, for both valves and arteries. Altogether, 4360 tissues were distributed for transplantation: 2032 (47%) vascular segments, 1545 (35%) pulmonary valves and 781 (18%) aortic valves. The most common indication for aortic valve surgery was the treatment of endocarditis, while for pulmonary valves, it was congenital malformation reconstruction. Vascular segments were mainly used for reconstruction after ischemia. During this period, a number of changes were made with the goal of enhancing tissue quality, safety and efficacy. These improvements were achieved through the use of a new antibiotic cocktail, increasing of donor age criteria and changing the microbiological control strategy.

Development of a full-thickness acellular dermal graft from human skin: Case report of first patient rotator cuff patch augmentation repair.

The processing and initial testing of a new human tissue preparation is described. Full-thickness Acellular Dermal Matrix (ftADM) is the extracellular matrix (ECM) obtained by decellularization of full-thickness human skin from cadaveric donors. The safety, stability and usability of the graft are discussed with respect to the results of the residual cellular content, maintenance of ECM components, and biomechanical properties. Quantitative and qualitative analysis of the ECM demonstrated the absence of cell debris, while the native structure of human dermis was maintained. Biomechanical testing showed stiffness values comparable to other commercial products used for tendon reinforcement, suggesting that our ftADM could be successfully used not only in soft tissue regeneration surgeries, but also in tendon reinforcement. First case of ftADM in rotator cuff augmentation is described. Technical management of the patch during surgery and clinical outcomes are discussed.

Effect of surgical treatment on myocardial strain in patients with pheochromocytoma and paraganglioma: a mini-review and meta-analysis.

AIM: Information on systolic dysfunction, as assessed by left-ventricular (LV) mechanics, in patients with pheochromocytoma after surgical treatment is scanty. We performed a systematic meta-analysis of speckle tracking echocardiographic studies to provide an updated comprehensive information on this issue. METHODS: The PubMed, OVID-MEDLINE, and Cochrane library databases were analyzed to search for articles published from the inception up to May 31st 2021. Studies were identified using MeSH terms and crossing the following search items: "myocardial strain" "left ventricular mechanics", "speckle tracking echocardiography", "systolic dysfunction", "pheochromocytoma", and "paraganglioma". RESULTS: A total of 92 surgically treated patients with pheochromocytoma/paraganglioma were included in 3 longitudinal studies. Successful surgical treatment was associated with a decrease in relative wall thickness (SMD - 0.25 ± 0.10, CI - 0.45/- 0.05, p < 0.01) and an improvement in global longitudinal strain (SMD - 0.45 ± 0.10, CI - 0.66/- 0.24, p < 0.0001). The favorable effects of treatment on LV geometry and mechanics were not accompanied by significant changes in ejection fraction (SMD - 0.07 ± 0.10, CI - 0.27/0.12, p = 0.44). CONCLUSIONS: This meta-analysis adds a new piece of evidence, suggesting that surgical treatment of patients with pheochromocytoma impacts favorably on LV geometry and LV mechanics, and, more importantly, the assessment of LV changes in this setting can no longer rely on conventional echocardiographic parameters such as ejection fraction.

Development of an optimized freeze-drying protocol for OM-PBAE nucleic acid polyplexes.

Long-term stability of polyplexes used for biomedical purposes is an objective envisaged by any research group developing this kind of nanoformulations. However, since biodegradable polymers such as oligopeptide end-modified poly (ß-aminoester) (OM-PBAE) are frequently used to ensure safety, and formulations are produced as aqueous dispersions, the stability of the nanoformulations is usually compromised. In this context, freeze-drying has aroused as a promising storage alternative to obtain solid nanoformulations with enhanced stability over time. Lyophilization is a challenging step that usually produces aggregation. Although some studies already achieved freeze-dried PBAE nanoparticles, none of them detailed the parameters that are critical for the success of this process. Moreover, due to the specific composition of each formulation, the critical parameters for the correct freeze-drying process need to be adjusted for each polyplex developed. In this paper, we have studied the variables that have a direct influence on the manufacturing and lyophilization of OM-PBAE nanoparticles with the aim to develop a versatile and robust freeze-drying receipt that properly preserves the library of polyplexes designed in our group, which have different pKa depending on the modification applied.

High-resolution array-CGH analysis on 46,XX patients affected by early onset primary ovarian insufficiency discloses new genes involved in ovarian function.

STUDY QUESTION: Can high resolution array-CGH analysis on a cohort of women showing a primary ovarian insufficiency (POI) phenotype in young age identify copy number variants (CNVs) with a deleterious effect on ovarian function? SUMMARY ANSWER: This approach has proved effective to clarify the role of CNVs in POI pathogenesis and to better unveil both novel candidate genes and pathogenic mechanisms. WHAT IS KNOWN ALREADY: POI describes the progression toward the cessation of ovarian function before the age of 40 years. Genetic causes are highly heterogeneous and despite several genes being associated with ovarian failure, most of genetic basis of POI still needs to be elucidated. STUDY DESIGN, SIZE, DURATION: The current study included 67 46,XX patients with early onset POI (<19 years) and 134 control females recruited between 2012 and 2016 at the Medical Cytogenetics and Molecular Genetics Lab, IRCCS Istituto Auxologico Italiano. PARTICIPANTS/MATERIALS, SETTING, METHODS: High resolution array-CGH analysis was carried out on POI patients' DNA. Results of patients and female controls were analyzed to search for rare CNVs. All variants were validated and subjected to a gene content analysis and disease gene prioritization based on the present literature to find out new ovary candidate genes. Case-control study with statistical analysis was carried out to validate our approach and evaluate any ovary CNVs/gene enrichment. Characterization of particular CNVs with molecular and functional studies was performed to assess their pathogenic involvement in POI. MAIN RESULTS AND THE ROLE OF CHANCE: We identified 37 ovary-related CNVs involving 44 genes with a role in ovary in 32 patients. All except one of the selected CNVs were not observed in the control group. Possible involvement of the CNVs in POI pathogenesis was further corroborated by a case-control analysis that showed a significant enrichment of ovary-related CNVs/genes in patients (P = 0.0132; P = 0.0126). Disease gene prioritization identified both previously reported POI genes (e.g. BMP15, DIAPH2, CPEB1, BNC1) and new candidates supported by transcript and functional studies, such as TP63 with a role in oocyte genomic integrity and VLDLR which is involved in steroidogenesis. LARGE SCALE DATA: ClinVar database (http://www.ncbi.nlm.nih.gov/clinvar/); accession numbers SCV000787656 to SCV000787743. LIMITATIONS, REASONS FOR CAUTION: This is a descriptive analysis for almost all of the CNVs identified. Inheritance studies of CNVs in some non-familial sporadic cases was not performed as the parents' DNA samples were not available. Addionally, RT-qPCR analyses were carried out in few cases as RNA samples were not always available and the genes were not expressed in blood. WIDER IMPLICATIONS OF THE FINDINGS: Our array-CGH screening turned out to be efficient in identifying different CNVs possibly implicated in disease onset, thus supporting the extremely wide genetic heterogeneity of POI. Since almost 50% of cases are negative rare ovary-related CNVs, array-CGH together with next generation sequencing might represent the most suitable approach to obtain a comprehensive genetic characterization of POI patients. STUDY FUNDING/COMPETING INTEREST(S): Supported by Italian Ministry of Health grants 'Ricerca Corrente' (08C203_2012) and 'Ricerca Finalizzata' (GR-2011-02351636, BIOEFFECT) to IRCCS Istituto Auxologico Italiano.

Adipocyte proteome and secretome influence inflammatory and hormone pathways in glioma.

Gliomas represent the most common primary malignant brain tumors in adults, with an extremely poor prognosis. Among several risk factors, lifestyle was also recently identified as a major risk factor for the development of primary glioma. In the present study, we explore the relationship between obesity and glioma in a cellular model. Thus, we have study the influence of adipocytes secretome on glioma cell line GL261. Using the 3T3-L1 adipocyte cell line, and its conditioned medium (adipokines-enriched medium), we showed that adipocyte-released factors relate with glioma angiogenic, growth, hormones and metabolic behavior by MALDI-TOF-MS and proteomic array analysis. In a first view, STI1, hnRNPs and PGK1 are under expressed on CGl. Similarly, both carbonic anhydrase and aldose reductase are even suppressed in glioma cells that grown under adipokines-enriched environment. Contrariwise, RFC1, KIF5C, ANXA2, N-RAP and RACK1 are overexpressed in GL261 cell the in the presence of the adipokines-enriched medium. We further identified the factors that are released by adipocyte cells, and revealed that several pro-inflammatory and angiogenic factors, such as IL-6, IL-11, LIF, PAI-1, TNF-α, endocan, HGF, VEGF IGF-I, were secreted to the medium into a high extent, whereas TIMP-1 and SerpinE1 were under expressed on CGl. This study discloses an interesting in vitro model for the study of glioma biology under a "obesity" environment, that can be explored for the understanding of cancer cells biology, for the search of biomarkers, prognostic markers and therapeutic approaches.

Exchange of best practices within the European Union: surgery standardization of abdominal organ retrieval.

Considering the growing organ demand worldwide, it is crucial to optimize organ retrieval and training of surgeons to reduce the risk of injury during the procedure and increase the quality of organs to be transplanted. In the Netherlands, a national complete trajectory from training of surgeons in procurement surgery to the quality assessment of the procured organs was implemented in 2010. This mandatory trajectory comprises training and certification modules: E-learning, training on the job, and a practical session. Thanks to the ACCORD (Achieving Comprehensive Coordination in Organ Donation) Joint Action coordinated by Spain and co-funded under the European Commission Health Programme, 3 twinning activities (led by France) were set to exchange best practices between countries. The Dutch trajectory is being adapted and implemented in Hungary as one of these twinning activities. The E-learning platform was modified, tested by a panel of Hungarian and UK surgeons, and was awarded in July 2013 by the European Accreditation Council for Continuing Medical Education of the European Union of Medical Specialists. As a pilot phase for future national training, 6 Hungarian surgeons from Semmelweis University are being trained; E-learning platform was fulfilled, and practical sessions, training-on-the-job activities, and evaluations of technical skills are ongoing. The first national practical session was recently organized in Budapest, and the new series of nationwide selected candidates completed the E-learning platform before the practical. There is great potential for sharing best practices and for direct transfer of expertise at the European level, and especially to export this standardized training in organ retrieval to other European countries and even broader. The final goal was to not only provide a national training to all countries lacking such a program but also to improve the quality and safety criteria of organs to be transplanted.

Functional and molecular defects of hiPSC-derived neurons from patients with ATM deficiency.

Loss of ataxia telangiectasia mutated (ATM) kinase, a key factor of the DNA damage response (DDR) pathway, causes the cancer predisposing and neurodegenerative syndrome ataxia-telangiectasia (A-T). To investigate the mechanisms of neurodegeneration, we have reprogrammed fibroblasts from ATM-null A-T patients and normal controls to pluripotency (human-induced pluripotent stem cells), and derived from these neural precursor cells able to terminally differentiate into post-mitotic neurons positive to >90% for ß-tubulin III+/microtubule-associated protein 2+. We show that A-T neurons display similar voltage-gated potassium and sodium currents and discharges of action potentials as control neurons, but defective expression of the maturation and synaptic markers SCG10, SYP and PSD95 (postsynaptic density protein 95). A-T neurons exhibited defective repair of DNA double-strand breaks (DSBs) and repressed phosphorylation of ATM substrates (e.g., γH2AX, Smc1-S966, Kap1-S824, Chk2-T68, p53-S15), but normal repair of single-strand breaks, and normal short- and long-patch base excision repair activities. Moreover, A-T neurons were resistant to apoptosis induced by the genotoxic agents camptothecin and trabectedin, but as sensitive as controls to the oxidative agents. Most notably, A-T neurons exhibited abnormal accumulation of topoisomerase 1-DNA covalent complexes (Top1-ccs). These findings reveal that ATM deficiency impairs neuronal maturation, suppresses the response and repair of DNA DSBs, and enhances Top1-cc accumulation. Top1-cc could be a risk factor for neurodegeneration as they may interfere with transcription elongation and promote transcriptional decline.

Combining self-assembling peptide gels with three-dimensional elastomer scaffolds.

Some of the problems raised by the combination of porous scaffolds and self-assembling peptide (SAP) gels as constructs for tissue engineering applications are addressed for the first time. Scaffolds of poly(ethyl acrylate) and the SAP gel RAD16-I were employed. The in situ gelation of the SAP gel inside the pores of the scaffolds was studied. The scaffold-cum-gel constructs were characterized morphologically, physicochemically and mechanically. The possibility of incorporating an active molecule (bovine serum albumin, taken here as a model molecule for others) in the gel within the scaffold's pores was assessed, and the kinetics of its release in phosphate-buffered saline was followed. Cell seeding and colonization of these constructs were preliminarily studied with L929 fibroblasts and subsequently checked with sheep adipose-tissue-derived stem cells intended for further preclinical studies. Static (conventional) and dynamically assisted seedings were compared for bare scaffolds and the scaffold-cum-gel constructs. The SAP gel inside the pores of the scaffold significantly improved the uniformity and density of cell colonization of the three-dimensional (3-D) structure. These constructs could be of use in different advanced tissue engineering applications, where, apart from a cell-friendly extracellular matrix -like aqueous environment, a larger-scale 3-D structure able to keep the cells in a specific place, give mechanical support and/or conduct spatially the tissue growth could be required.

[Using Kehr's tube in complex anastomoses]. / Utilización de tubo de Kehr en anastomosis intestinales complejas.

OBJECTIVE: To present a new technique using the Kehr's T tube (KT) in complex intestinal anastomoses. MATERIALS AND METHODS: Restrospective descriptive analysis of 8 patients intervened from 2007 to 2011. We performed intestinal anastomoses guided by a KT in 7 patients with intestinal atresia (yeyunal n = 5, ileal n = 1, duodenal n = 1), and in 1 patient with yeyunal stenosis associated with gastroschisis. 4 cases (50%) were reoperations because of complications after the first surgery. SURGICAL TECHNIQUE: the KT is introduced through the dilated proximal bowel. The proximal end of the "T" is tied and the distal one acts as a transanastomotic guide and feeding tube. The long end of the T is externalized through the skin and used for the administration of the enteral nutrition formula. Studied variables were: beginning of enteral feeding, feeding time through the KT, time of intestinal motility recovery, time to complete oral feeding and complications. RESULTS: There were no complications derived from the technique. Feeding was started through the KT between day 2 and 10 (median: 4.5), with a period of 4 to 33 days (median: 7). Patients started intestinal transit between days 2 and 7 (median: 3.5). Reoperated patients showed cholestasis and/or sepsis signs, which were solved with surgery. The KT was left in place between 11-51 days (median: 22), with no complications during or after the removal. CONCLUSIONS: The results of this preliminary study suggests that the use of the KT in complex anastomoses as a transanastomotic guide and feeding tube presents advantages, such as early enteral feeding and prevention of leakage and kinking of the intestinal suture. There were no complications derived from the procedure. As far as we know, this technique has not been previously reported in the literature.

[Benefits of epidural analgesia in major neonatal surgery]. / Ventajas de la analgesia epidural en cirugía mayor neonatal.

The aim of this paper is to describe and evaluate the benefits of epidural anesthesia in major surgery neonatal. We have performed a matched case-control (2:1) study of patients undergoing neonatal major surgery (NMSs) who received intra-and postoperative epidural anesthesia (EA) and controls with conventional general anesthesia. The matching criteria were age, weight and baseline pathology. EA was administered by caudal puncture and epidural catheter placed with ultrasound support. Levobupivacaine was selected as anesthetic drug. The time to extubation, intestinal transit time, type of analgesia and complications were studied. This study is based on 11 cases (2 esophageal atresia, 2 diaphragmatic hernias, 1 necrotizing enterocolitis, 3 intestinal atresia, 2 anorectal malformation and 1 bladder exstrophy) and 22 controls. We observed statistically significant differences in time to extubation (95% CI OR 12 1.99 to 72.35; Chi2 p = 0.004, Mann U Whytney p = 0.013) and intestinal transit time (Mann Whitney U p < 0.001, 100 Or, 95% CI 8.06-1 239; Chi2 p < 0.0001). There were no complications from epidural analgesia. Therefore we believe that the intra-and postoperative EA helps improve postoperative management in neonates and should be preferred in centers where this technique is available.

[When a peritonitis does not seems like a peritonitis]. / Cuando una peritonitis no parece una peritonitis.

Acute pancreatitis should be considered in the differential diagnosis of acute abdominal pain. Although its incidence is low, it has increased in the last years; therefore, an accurate diagnosis is necessary to avoid inappropriate surgeries. The aim of this study is to describe acute pancreatitis in the context of acute abdominal pain, which suggests appendicitis. We performed a retrospective study of all the patients who were admitted in the emergency department due to suspected appendicitis but were finally diagnosed of acute pancreatitis since 2010. Five patients were included in the investigation. One of them underwent surgery and the diagnosis was made on the 5th postoperative day. Median age at diagnosis was 5 years (range from 8 month to 6 years). Median white blood cell was 16,600/microL (13,400-31,900/microL), Median differential count of white blood cell was 14,432/microL (11,400-29,348/microL) and Median PCR 11 mg/L (155-4.6 mg/L). Median serum amylase at diagnosis was 651 U/L (10-1,443 U/L). All cases were studied with ultrasound and computerized tomography or nuclear magnetic resonance. One case had recurrent episodes of pancreatitis and was complicated by the development of a pseudocyst and a pancreatic fistula, requiring an Y-en-Roux cysto-enteric anastomosis. The median follow up period was 10 months (range: 1 to 22). All patients are asymptomatic at the moment.

[Relation between packed red blood cell transfusion and severity of necrotizing enterocolitis in premature infants]. / Relación entre la transfusión de concentrado de hematíes y la gravedad de la enterocolitis necrotizante en prematuros.

OBJECTIVE: It has been proposed recently that red blood cell transfusions (RBCT) might increase severity in infants with necrotizing enterocolitis (NEC). We intend to study if patients who have received red blood transfusions before the onset of NEC develop more severe forms of this entity. METHODS: A retrospective study was carried out including prematures with NEC. Two groups were considered: with previous RBCT (TR) and without previous RBCT (No-TR). The main outcomes of the study were severity of NEC, according to the Bell stage (BS), surgical treatment and mortality. Patients who were treated with RBCT 48 hours prior to the onset of NEC symptoms were analysed separately afterwards. Comparison of groups was made with the Fisher test or the Chi square test for the BS, surgery, mortality and nominal variables; the U Mann-Whitney test was used for numeric variables. RESULTS: Forty-six patients were included for the investigation (28 in TR and 18 in No-TR). In the TR Group 20/28 neonates reached a BS II; 8/28 BS III; 10 were operated on and there were 7 deaths. In the No-TR group 14/18 patients were classified as EB II; 4/18 as BS III, 3 patients needed surgery and Idied. No relation was found between RBCT and BS (p = 0.39), RBCT and surgery (RR = 2.7; CI 95%: 0.64-11.97; p = 0.19), or RBCT and mortality (RR = 4.5; CI 95%: 0.6-36.6; p = 0.09). In those patients who received a RBCT 48 hours before the initial symptoms there were 3 EB II and 3 EB III; 4 infants required surgical treatment and there were 2 exitus. Comparing this subgroup and the rest of the sample there were only significant differences in the need of surgical treatment: patients who received a RBCT 48 hours before the onset of NEC were at an increased risk for surgery of 2.6 (CI 95%: 1.2-5.1; p = 0.045) but there were not different when surgical treatment and mortality were considered. CONCLUSIONS: These results do not support clearly the hypothesis that there is a relation between previous treatment with RBCT and the severity of NEC.

Combination of temozolomide with immunocytokine F16-IL2 for the treatment of glioblastoma.

BACKGROUND: Glioblastoma patients are still not cured by the treatments available at the moment. We investigated the therapeutic properties of temozolomide in combination with F16-IL2, a clinical-stage immunocytokine consisting of human interleukin (IL)-2 fused to the human antibody F16, specific to the A1 domain of tenascin-C. METHODS: We conducted three preclinical therapy studies, using subcutaneous and intracranial U87MG glioblastoma tumours xenografted in BALB/c nude mice. The same therapeutic schedule was used, consisting of five total administrations every third day, of 0.525 mg temozolomide, 20 microg F16-IL2, the combination, or the control solution. RESULTS: Immunohistochemical analysis of U87MG xenografts and of human glioblastoma specimens showed selective tumour staining of F16. A quantitative biodistribution confirmed the preferential tumour accumulation of radiolabelled F16-IL2. In the study with subcutaneous xenografts, the combination of F16-IL2 with temozolomide induced complete remission of the animals, which remained tumour free for over 160 days. The same treatment led to a consistent size reduction of intracranial xenografts and to a longer survival of animals. The immunocytokine promoted the recruitment of leukocytes into tumours of both models. CONCLUSION: The combined use of temozolomide with F16-IL2 deserves clinical investigations, which will be facilitated by the excellent safety profile in cynomolgus monkeys, and by the fact that F16-IL2 is in clinical trials in patients with cancer.

Constipation: a potential cause of pelvic floor damage?

BACKGROUND: Pelvic floor damage is a major clinical problem usually attributed to obstetric injury. We speculated that constipation may also be an aetiological and preventable factor resulting from repeated stress on the perineum over many years, and this study aimed to test this hypothesis. METHODS: A total of 600 women attending a gynaecological clinic were assessed using a structured questionnaire gathering data on pelvic floor damage, constipation and obstetric trauma. Complete data were available on 596 subjects. KEY RESULTS: The prevalence of pelvic floor damage was 10% (61/596). In this group, constipation was identified in 31% (19/61) of women and obstetric trauma in 31% (19/61). In the group without pelvic floor damage, constipation was present in 16% (86/535) and obstetric trauma in 16% (83/535). In univariate analysis, pelvic floor damage was associated with age (OR: 1.05; 95% CI: 1.03-1.08; P < 0.0001), constipation (OR: 2.36; 95% CI: 1.31-4.26; P < 0.0001) and obstetric trauma (OR: 2.46; 95% CI: 1.37-4.45; P < 0.0028). In multivariate analysis, the OR for age was 1.05 (95% CI: 1.03-1.08; P < 0.0001), for constipation 2.35 (95% CI: 1.27-4.34; P < 0.0001) and for obstetric trauma 1.37 (95% CI: 0.72-2.62; P = 0.3398). CONCLUSIONS & INFERENCES: Constipation appears to be as important as obstetric trauma in the development of pelvic floor damage. Thus, a more proactive approach to recognizing and treating constipation might significantly reduce the prevalence of this distressing problem.

[Short term evaluation of transanal pull-through with automatic suture]. / Valoración a corto plazo del descenso transanal con sutura automática.

For the last ten years many centres have adopted transanal pull-through (TP) as the first choice technique for the treatment of Hirschsprung's Disease (HD) affected children. We present our experience, based on the endorectal pull-through with autosuture, which has not been reported up to now. According to our HD management programme, TP with autosuture should be performed in rectosigmoid forms of HD which are easily handled with outpatient care. Seven patients with HD whose ages ranged from 5-months-old to 5-years-old underwent EP with autosuture. We present the short term results of the evolution of our patients. Firstly, a laparoscopic procedure is carried out in order to obtain a biopsy from the transition zone. The second stage consists of the TP following the De La Torre technique, modified by the 21 mm circular autosuture. No new surgical operation was necessary. Passage of stools started between the second and fourth postoperative day. The most frequent complication was abdominal distention, found in an 85.7% of patients and resolved before being discharged. Hospital discharge took place between the fourth and the tenth postoperative day. Oral feeding was started in the 2nd-7th postoperative day. Medium term outcomes show a single case of complications: a patient Developer a skin stricture due to the low suture, which has been treated conservatively with rectal dilatations. Patients older than three (42.8%) are continent, although one presents occasional fecal soiling. The rest of the children present normal stools for their age. Automatic suture involves several advantages such as celerity and safety. We ought to point out that this method should not be used in children under 5 months because the autosuture size does not allow to do so.

[Prophylactic thyroidectomy in multiple endocrine neoplasia syndrome]. / Tiroidectomía profiláctica en el síndrome de neoplasia endocrina múltiple.

Multiple endocrine neoplasia (MEN) 2a consists on medullary thyroid carcinoma, pheochromocytoma and hyperparathyroidism. The identification of the RET proto-oncogene in 1993 has changed the prognosis of the disease. We have retrospectively studied the patients diagnosed of MEN 2a in our centre for the last 7 years in order to establish the most adequate age to undergo surgery. We present ten patients diagnosed with MEN 2a, whose ages ranged from 1.5 to 11 years old. Mean age at time of operation: 6,4 years An ultrasound study, calcitonin determinations and cathecholamines and urinary metanephrine levels were obtained before surgery. The surgical treatment is based on total total thyroidectomy, in selected cases lymph node resection in the central zone lf the neck. The most frequent RET mutation is the one affecting codon 634 (exon 10), which was found in children. Both of them had an alteration in codon 611 (exon11). No complications appeared after surgery and hospital discharge took place in the 2nd-4th day after surgery. Pathological findings were medullary thyroid microcarcinoma (MTMC) in 3 out of 10 patients, calcitonin preoperative tests were high in one of them. No tumoral cells were found in the lymph nodes. During the follow up period, 9 out of 10 from the operated patients, maintained normal calcitonin, CEA, PTH, calcium, cathecholamines and urinary metanephrine levels. Since there are 3 cases of MTC in patients between 3 and 6 years old, and diagnostic test data are not conclusive, we thoroughly recommend prophyilactic thyroidectomy at early ages, from 3 to 4 years old.

[Endoscopic treatment of recurrent tracheoesophageal fistula]. / Tratamiento endoscópico de la fístula traqueoesofágica recurrente.

UNLABELLED: Repermeabilization of the tracheoesophageal fistula (RTE) is one of the major complications of the esophageal atresia with tracheal fistula. We present the long term results of our technique based on broncoscopic treatment with fibrin glue and diathermia. MATERIAL AND METHODS: A retrospective study of 10 children with RTE treated from 1993 to 2006, their ages ranging from 14 days-old to 4 years-old; there were 4 patients from other hospitals. One of the patients involved had a superior congenital fistula that had not been noticed during open surgery. Broncoscopic procedure was performed using rigid instrumentation. The fistula was sealed with fibrin glue; the last seven patients in the series received previous diathermia with an uretheral catheter. Clinical and radiological follow up took place in all children. Endoscopic evaluation was performed in 6 of them. Follow-up time vary from 1 to 13 years and the number of sessions was limited to 3 per patient. RESULTS: Closure of the fistula was confirmed in 9 cases (90%) who required a whole number of 15 sessions (Mean: 1.5). The group who received diathermia needed 9 sessions (Mean: 1.2). There were no major complications. DISCUSSION: Surgery reparation of RTE is often related to serious complications. Therefore, many groups have tried to develop several broncoscopic techniques using different materials. Scientific reviews have not pointed out a definitive option since they involve isolated patients with no long term evolution. Taking this report into account we believe that the application of diathermia and fibrin glue ought to be considered the first choice treatment for the RTE.

[Analyses [corrected] of the consults from the Department of General Surgery to the Department of Internal Medicine]. / Estudio de las interconsultas solicitadas por el Servicio de Cirugía General al Servicio de Medicina Interna.

UNLABELLED: RATIONAL AND AIM: to better understand the nature of the consults solicited by the Department of General Surgery to the Department of Internal Medicine and to examine the results and the experience from the point of view of an Internal Medicine specialist. PATIENTS AND METHODS: Prospective analysis of the 129 consults from patients admitted in General Surgery to the Department of Internal Medi-cine during 2005. RESULTS: The number of consults was 4% of all patients admitted in General Surgery (6,5% of de admitted patients were from the emergency service and 2% from the planning programme). The median age was 74 years. The patients were visited a mean of 3.3 days. The most frequent reasons for consultation were dyspnea (29%), fever (21%), cough (11%) and dysrhythmias (8%). The most frequent diagnosis were cardiac failure (17%), chronic obstructive pulmonary disease (15%), respiratory infection and/or accumulation of bronchial secretions (13%), pneumonia (9%) and atrial fibrillation (7%). In 31% of cases the diagnosis was decompensation of previously diagnosed chronic disease. Nineteen patients died (15%), higher than the global average admitted in General Surgery (2%). The average age of these patients was 82 years. The mortality was higher when the admission was from the emergency service (17 of 91) than when it was from the planning programme. The most frequent cause of death was suture failure and peritonitis with secondary septicemia in 5 patients. CONCLUSIONS: The task of the Internal Medicine specialist was not only the treatment of chronic diseases, but also to assist in the diagnosis and treatment of acute diseases related or unrelated to the cause of the admission. To point up the invalid specificity of cough as a guide symptom in the diagnosis of previously operated patients. The causes of death were almost exclusively related to the disease that resulted in the admission to the Department of Surgery or with its complications (17 of 19).

Prevalence and correlates of advanced retinopathy in a large selected hypertensive population. The Evaluation of Target Organ Damage in Hypertension (ETODH) study.

OBJECTIVE: To describe the prevalence of advanced retinal microvascular lesions and their associations with cardiac and extracardiac signs of target organ damage (TOD) in a large selected hypertensive population. METHODS: A total of 2172 non-diabetic untreated and treated uncomplicated essential hypertensives consecutively attending for the first time our hospital outpatient hypertension clinic and included in the Evaluation of Target Organ Damage in Hypertension (ETODH), an observational ongoing registry of hypertension-related TOD, were considered for this analysis. Advanced hypertensive retinopathy was defined by the presence of any of the following lesions: flame-shaped haemorrhages, soft exudates or cotton wool spots and papilloedema. Left ventricular hypertrophy (LVH), carotid structural abnormalities, such as plaques and intima media (IM) thickening, and microalbuminuria were diagnosed according to the 2003 ESH/ESC guidelines criteria. RESULTS: Among the whole study population, 33 patients (1.5%) were found to have advanced hypertensive retinopathy. Patients with these retinal lesions were similar to those without for age, body mas index, known duration of hypertension, smoking habit, total serum cholesterol, fasting blood pressure and prevalence of antihypertensive treatment; whereas mean systolic and diastolic blood pressures were higher in the former group. The prevalence rates of LVH, carotid plaques, carotid IM thickening and microalbuminuria in patients with and without retinopathy were 57%, 67%, 69%, 19% and 25%, 47%, 44%, 12%, respectively. In a multivariate logistic regression analysis, advanced retinopathy was significantly associated with LVH (OR = 4.0), carotid IM thickening (OR = 2.9), carotid plaques (OR = 2.8), but not with microalbuminuria. CONCLUSIONS: Our study indicated that: (i) advanced retinopathy is a rare finding in non-diabetic hypertensive patients seen in a specialist setting; (ii) a strong relation exists between retinal microvascular lesions and cardiac and macrovascular markers of TOD.