Características clínicas, histopatológicas y factores pronósticos del microcarcinoma tiroideo: estudio retrospectivo multicéntrico / Clinical, Hystopathologic Caractheristics and Prognostic Factors of Thyroid Microcarcinoma: A Retrospective Multicenter Study

Introducción: El microcarcinoma diferenciado de tiroides (MCDT) es definido por la OMS, como un tumor <10 mm en su diámetro mayor. Estos tumores son casi exclusivamente de estirpe papilar, representando el 30 % de todos los carcinomas papilares. La historia natural del MCDT es poco conocida y existe una controversia sobre cuál es la óptima forma de abordaje. Objetivos: 1) Analizar retrospectivamente las características del MCDT; 2) Evaluar factores de riesgo de persistencia/recurrencia en una cohorte retrospectiva seguida en la C.A.B.A. Materiales y Métodos: Se recolectaron los datos de 187 pacientes en un estudio retrospectivo multicéntrico y se analizaron las características clínicas, histopatológicas, bioquímicas y distintos factores pronósticos del MCDT. Resultados: El 82,8 % eran mujeres, con una X ± DS de edad de 48 ± 13 años. La mediana de tiempo de seguimiento fue de 38 meses (r: 1-120). El 97 % de los tumores eran de estirpe papilar. En el 29,4 % de los pacientes el hallazgo fue incidental, de los cuales el 57 % se operó por el tamaño del bocio. El 81 % de los pacientes fue sometido a una tiroidectomía total, mientras que el 91,4 % recibió ablación posquirúrgica con radioiodo. Los 174 pacientes que fueron seguidos por más de 12 meses, con una X ± DS de 49 ± 36,9 meses, fueron incluidos en el análisis de sobrevida. El 84 % estaban libres de enfermedad (LE) al final del seguimiento. De los factores de persistencia/recurrencia analizados, la Tg preablativa > 20 ng/ml resultó un predictor independiente. Al realizar el análisis de regresión de Cox para el evento LE, se demostró que tanto la edad

Introduction: Differentiated thyroid microcarcinoma (MCDT) has been defined as a differentiated thyroid cancer measuring 10 mm or less. The majority of these tumors are papillary thyroid carcinomas and comprise 30 % of all papillary thyroid carcinomas. Little is known of its natural history and there is an ongoing controversy in the field regarding its optimum management. Objectives: 1) To describe the characteristics of MCDT 2) To assess risk factors for persistence and/ or recurrence of disease in a retrospective cohort of patients followed up at several health centers of the City of Buenos Aires (CABA). Patients and Methods: The medical records of 187 patients with MCDT operated on between January 1st, 2000 and December 31st, 2009 at several centers of CABA were retrospectively reviewed, and clinical, histopathological, biochemical characteristics and risk factors were assessed. Results: Most of the patients were female (82.8 %) and their mean age was 48 ± 13 (X ± SD) years. Median follow up was 38 months (range: 1 to 120 months), and 97 % of tumors were papillary thyroid cancers. Incidentalomas accounted for 29.4 % of tumors, mostly found during a surgical procedure undergone for the size of the goitre. Over 81 % of patients underwent a total thyroidectomy, while 91.4 % received radioactive iodine ablation. Patients with a follow-up longer than 12 months after surgery were analyzed longitudinally to assess prognostic factors of disease outcome (174 patients). After a mean follow-up of 49 ± 36.9 months, 146 (84 %) patients had no evidence of disease. Only postoperative, preablation Tg levels > 20 ng/ml were identified as an independent adverse prognostic factor in the multivariate analyses. In addition, age < 45 ys. (p< 0.01), tumor size > 0.5cm (p<0.017), and preablation Tg levels >20 ng/ml (p<0.011) were independent prognostic factors of a longer time to disease remission in the longitudinal analyses. Conclusion: Differentiated thyroid microcarcinoma has an excellent prognosis in our local practice, with 84 % disease remission at long-term follow-up. Age at diagnosis, tumor size and preablation Tg levels were independent prognostic factors of time to disease remission.

Carcinoma medular de tiroides: estudio multicéntrico. Presentación y evolución en 219 pacientes / Medullary Thyroid Carcinoma: Clinical Presentation and Outcome in 219 Patients

Ante la baja frecuencia del carcinoma medular de tiroides (CMT), en el Departamento de Tiroides de SAEM nos propusimos realizar un estudio de cohorte, observacional, retrospectivo y multicéntrico. Se incluyeron 219 pacientes con diagnóstico histológico de CMT. El 65 % fueron mujeres, la edad promedio fue de 39 ± 20 años (1 a 84 años); 44-% de los casos fueron familiares. Las formas de presentación más frecuentes fueron nódulo tiroideo (58 %) y pesquisa genética por antecedente familiar (22 %). Si bien la citología tiroidea fue diagnóstica de CMT en el 39 % de los casos, fue determinante de indicación quirúrgica en el 79 %. En el 47 % de los pacientes el diagnóstico de CMT se obtuvo previamente al tratamiento quirúrgico inicial por punción aspiración con aguja fina (PAAF), estudio genético o nivel de calcitonina (CT)). El 65 % se presentó en estadios avanzados (TNM III y IV). El estudio del protoncogen RET se realizó en 162 pacientes (74 %). En el 49 % se observó mutación siendo la más frecuente (76 %) en el codón 634. La forma hereditaria más frecuentemente observada fue el síndrome de neoplasia endocrina múltiple (NEM) 2A (57 % de los casos familiares), seguida por carcinoma medular familiar (25 %) y NEM 2B (13 %). Los casos familiares tuvieron menor edad al diagnóstico y mayor frecuencia de diagnóstico prequirúrgico. Los casos índice tuvieron mayor edad al momento del diagnóstico, mayores niveles de antígeno carcinoembrionario (CEA) y CT prequirúrgicos, mayor proporción de estadios III y IV y mayor porcentaje de evidencia de enfermedad al momento de la última consulta que aquellos detectados por pesquisa. En 143 pacientes (65 %) se obtuvieron registros completos de seguimiento en los que se analizaron los factores relacionados con la evolución. La mediana de seguimiento fue de 44 meses: fallecieron 21 pacientes (14,6 %) y 122 (86 %) viven; 76 de estos (54 %) se encuentran libres de enfermedad. El grupo con evidencia de enfermedad se presentó en estadios más avanzados. Resultaron factores de mayor riesgo para evidencia de enfermedad: sexo masculino, CMT esporádico, niveles elevados de CT prequirúrgicos, estadio IV y presencia de metástasis. Los niveles de CT posquirúrgicos fueron menores en aquellos pacientes que en la evolución final no presentaron evidencia de enfermedad. El principal factor pronóstico de la evolución de los pacientes con CMT fue el estadio de presentación, determinando la importancia del diagnóstico precoz con el fin de poder implementar un tratamiento quirúrgico curativo en estadios menos avanzados.

Due to the low frequency of medullary thyroid cancer (MTC), an observational, cohort, retrospective multicenter study was conducted at the Thyroid Department of the Endocrine and Metabolism Argentine Society (SAEM). We included 219 patients with histologically proven MTC, with a mean age of 39 ± 20 yr (range 1-84 years). Sixty five percent were women and 44% were familial cases. The most common presentations were thyroid nodule (58 %) and genetic screening due to family history (22 %). In 39 % of patients, diagnosis of MTC was made by fine needle aspiration, but cytology led to surgery in 79 %. In 47 % of patients, MTC was diagnosed by cytology, calcitonin (CT) levels or genetic studies prior to initial surgery. Sixty five percent of patients had advanced stages of the disease (TNM III or IV) at diagnosis. Proto-oncogene RET was studied in 162 patients (74 %). In 49% a mutation was reported, most frequently in codon 634 (76 %). Regarding hereditary forms of MTC, MEN 2A was the most frequent (57%), followed by familial MTC in 25 % and MEN 2B in 13 % of cases. Familial cases were younger subjects and had more frequently a pre-surgery diagnosis. Index cases were older, with higher CEA and CT levels, presented in more advanced stages and had more frequently evidence of disease at final assessment than patients who were diagnosed by genetic screening. Follow-up records of 143 patients were analyzed (65%); median time was 44 months; 21 patients died (14.6 %) and 122 survived (86 %), 76 showed no evidence of disease (NED) (54 %). High risk factors for evidence of disease at the final evaluation were: male gender, sporadic MTC, higher CT pre-surgery levels, stage IV and metastasis. Post surgery CT levels were lower in patients with NED. Stage at initial diagnosis was the main prognostic factor in patients with MTC, determining the importance of early detection for performing curative surgery in less advanced stages.

Carcinoma medular de tiroides: estudio multicéntrico. Presentación y evolución en 219 pacientes / Medullary Thyroid Carcinoma: Clinical Presentation and Outcome in 219 Patients

Ante la baja frecuencia del carcinoma medular de tiroides (CMT), en el Departamento de Tiroides de SAEM nos propusimos realizar un estudio de cohorte, observacional, retrospectivo y multicéntrico. Se incluyeron 219 pacientes con diagnóstico histológico de CMT. El 65 % fueron mujeres, la edad promedio fue de 39 ± 20 años (1 a 84 años); 44-% de los casos fueron familiares. Las formas de presentación más frecuentes fueron nódulo tiroideo (58 %) y pesquisa genética por antecedente familiar (22 %). Si bien la citología tiroidea fue diagnóstica de CMT en el 39 % de los casos, fue determinante de indicación quirúrgica en el 79 %. En el 47 % de los pacientes el diagnóstico de CMT se obtuvo previamente al tratamiento quirúrgico inicial por punción aspiración con aguja fina (PAAF), estudio genético o nivel de calcitonina (CT)). El 65 % se presentó en estadios avanzados (TNM III y IV). El estudio del protoncogen RET se realizó en 162 pacientes (74 %). En el 49 % se observó mutación siendo la más frecuente (76 %) en el codón 634. La forma hereditaria más frecuentemente observada fue el síndrome de neoplasia endocrina múltiple (NEM) 2A (57 % de los casos familiares), seguida por carcinoma medular familiar (25 %) y NEM 2B (13 %). Los casos familiares tuvieron menor edad al diagnóstico y mayor frecuencia de diagnóstico prequirúrgico. Los casos índice tuvieron mayor edad al momento del diagnóstico, mayores niveles de antígeno carcinoembrionario (CEA) y CT prequirúrgicos, mayor proporción de estadios III y IV y mayor porcentaje de evidencia de enfermedad al momento de la última consulta que aquellos detectados por pesquisa. En 143 pacientes (65 %) se obtuvieron registros completos de seguimiento en los que se analizaron los factores relacionados con la evolución. La mediana de seguimiento fue de 44 meses: fallecieron 21 pacientes (14,6 %) y 122 (86 %) viven; 76 de estos (54 %) se encuentran libres de enfermedad. El grupo con evidencia de enfermedad se presentó en estadios más avanzados. Resultaron factores de mayor riesgo para evidencia de enfermedad: sexo masculino, CMT esporádico, niveles elevados de CT prequirúrgicos, estadio IV y presencia de metástasis. Los niveles de CT posquirúrgicos fueron menores en aquellos pacientes que en la evolución final no presentaron evidencia de enfermedad. El principal factor pronóstico de la evolución de los pacientes con CMT fue el estadio de presentación, determinando la importancia del diagnóstico precoz con el fin de poder implementar un tratamiento quirúrgico curativo en estadios menos avanzados.(AU)

Due to the low frequency of medullary thyroid cancer (MTC), an observational, cohort, retrospective multicenter study was conducted at the Thyroid Department of the Endocrine and Metabolism Argentine Society (SAEM). We included 219 patients with histologically proven MTC, with a mean age of 39 ± 20 yr (range 1-84 years). Sixty five percent were women and 44% were familial cases. The most common presentations were thyroid nodule (58 %) and genetic screening due to family history (22 %). In 39 % of patients, diagnosis of MTC was made by fine needle aspiration, but cytology led to surgery in 79 %. In 47 % of patients, MTC was diagnosed by cytology, calcitonin (CT) levels or genetic studies prior to initial surgery. Sixty five percent of patients had advanced stages of the disease (TNM III or IV) at diagnosis. Proto-oncogene RET was studied in 162 patients (74 %). In 49% a mutation was reported, most frequently in codon 634 (76 %). Regarding hereditary forms of MTC, MEN 2A was the most frequent (57%), followed by familial MTC in 25 % and MEN 2B in 13 % of cases. Familial cases were younger subjects and had more frequently a pre-surgery diagnosis. Index cases were older, with higher CEA and CT levels, presented in more advanced stages and had more frequently evidence of disease at final assessment than patients who were diagnosed by genetic screening. Follow-up records of 143 patients were analyzed (65%); median time was 44 months; 21 patients died (14.6 %) and 122 survived (86 %), 76 showed no evidence of disease (NED) (54 %). High risk factors for evidence of disease at the final evaluation were: male gender, sporadic MTC, higher CT pre-surgery levels, stage IV and metastasis. Post surgery CT levels were lower in patients with NED. Stage at initial diagnosis was the main prognostic factor in patients with MTC, determining the importance of early detection for performing curative surgery in less advanced stages.(AU)

Programa nacional de bocio nodular (PRONBONO): Estudio multicéntrico de bocio nodular único palpable / National Program for Nodular Goiter (PRONBONO): Multicenter study of single palpable thyroid nodules

Introducción: La presencia de nódulos tiroideos palpables en la población general, es uno de los signos clínicos tiroideos más frecuentes en la práctica diaria. Objetivos: 1) establecer la prevalencia de las distintas patologías en bocio nodular único palpable y analizar sus características y su relación con los resultados citológicos. 2) analizar la existencia de diferencias regionales en Argentina. Pacientes y Métodos: Estudio prospectivo de 739 pacientes con bocio nodular único palpable evaluados entre el 1/1/2000 y el 31/12/2001 en Centros de Buenos Aires, Bahía Blanca, Mendoza y La Pampa. Se recabaron datos de examen clínico, ecografía tiroidea, TSH, ATPO y citología por punción con aguja fina. (PAAF). Fue utilizado para el análisis estadístico Correlación de Pearson, X2 y Test de Fisher. Resultados: la edad (X ± DS) fue 46,3 ± 14 años, 93,1 % eran de sexo femenino. El 1,6 % tenía historia de radiación en cuello y el 29,9 % antecedentes familiares de patología tiroidea. Hallazgos clínicos: disfagia en el 7,9 %, disfonía 3,5 %, crecimiento nodular en los últimos 6 meses 19,2 %, consistencia dura el 24,7 %, fijeza a estructuras adyacentes 1,5 % y adenopatías en el 3 %. Hallazgos bioquímicos: TSH normal en el 81,2 % y ATPO positivos en el 30,3 % de los casos. Características Ecográficas: nódulos sólidos: 53,1 %, hipoecoicos: 63,8 %, microcalcificaciones 10,3 %, halo incompleto: 15 %, multinodular: 30,5 %, tiroides heterogénea: 60,2 % y adenopatías: 3,8 %. Hallazgos citológicos: En el 86,8 % de los casos fue necesario solo una punción para llegar al diagnóstico. Insatisfactorio (excluyendo quiste): 3,2 %: benignos: 77,3 %; sospechosos: 12,6 % y cáncer: 7 % (42 papilar, 2 medular y 3 sin especificar). Una correlación significativa (p<0,02) fue observada entre citología maligna y crecimiento rápido, dureza, fijeza a estructuras vecinas, nódulo sólido, halo incompleto y adenopatías aunque estos parámetros son más frecuentes en números absolutos en nódulos benignos. La mayoría de las cirugías fueron indicadas en base al hallazgo citológico. El diagnóstico histológico de los 96 pacientes que fueron operados mostró 51 carcinomas, de los cuales solo dos tenían citología benigna y 31 adenomas. Conclusión: Los nódulos palpables únicos fueron más frecuentes en mujeres eutiroideas en la edad media de la vida. Un tercio tenía historia familiar de patología tiroidea, similar al porcentaje hallado de ATPO positivos. Por ecografía los nódulos fueron predominantemente sólidos, hipoecoicos, únicos con resto de la glándula tiroides heterogénea. La PAAF fue predominantemente benigna. El crecimiento rápido, la dureza, la fijeza a estructuras adyacentes, el halo incompleto y la presencia de adenopatías fueron relacionados con malignidad, pero la benignidad fue más frecuente. En la mayoría de los pacientes la cirugía fue recomendada por los hallazgos citológicos. Nuestros resultados son similares a los reportados en otras áreas geográficas.

Introduction: the presence of palpable thyroid nodules in the general population is one of the most common clinical signs of thyroid disease in daily practice. Objectives: 1) To assess the prevalence of pathologies, clinical and cytological findings of single palpable thyroid nodules (SPTN) in Argentina. 2) Analyze the regional differences in Argentina. Methods: Prospective study of 739 patients with STPN were evaluated at centres in Buenos Aires, Bahía Blanca, Mendoza, and La Pampa between 1/1/00 and 12/31/01. Clinical examination, thyroid ultrasound scan (US), TSH, TPOAb and fine needle aspirations (FNA) were performed. Statistics: Pearson Correlation, X2 & Fisher Tests. Results: Age (X ± SD) 46 ± 14ys: 93.1 % were women. Previous history of neck radiation & familial thyroid disease were found in 1.6 and 29.9 % respectively. Clinical findings: dysphagia: 7.9 %; dysphonia: 3.5%; nodule growth: 19.2 %; hard consistence: 24.7 %; fixation to adjacent structure: 1.5 % and lymphadenopathies (ADP): 3 %. Biochemical findings: TSH was normal in 81.2 % & TPOAb+ in 30.3 %. US features: solid: 53.1 %; hypoechoic: 63.8 %; microcalcifications: 10.3 %; incomplete halo: 15 %; more than 1 nodule: 30.5 %; thyroid heterogeneity: 60.2 % and ADP: 3.8 %. Cytology: Only 1 FNA was needed in 86.8%. Unsatisfactory (excluding cysts): 3.2 %; benign: 77.2%; suspicious: 12.6 % and cancer: 7 % (42 papillary, 2 medullary and 3 non specified). A significant correlation (p<0.02) was established between malignant nodules and rapid growth, hard, fixed, solid nodule, incomplete halo and ADP, though these parameters were more frequent (in absolute number) in benign nodules. Surgery was mainly indicated based on FNA results. Histological diagnosis of 96 patients who underwent surgery showed 51 carcinomas, of which only 2 were cytologically benign and 31 adenomas. Conclusion: Palpable single nodules were more frequent in middle aged euthyroid women. One third had familial thyroid pathology, similar to the presence of TPOAb. On US, nodules were predominantly solid, hypoechoic, single with heterogeneous thyroid gland. FNA was predominantly benign. Rapid growth, hard, fixed, solid nodule, incomplete halo and ADP were associated with malignancy, but benignity was more common. In most of the patients surgery was recommended based on cytological findings. Our results are similar to those reported in other geographic areas.

Fenoldopam to prevent renal replacement therapy after cardiac surgery. Design of the FENO-HSR study.

INTRODUCTION: Acute kidney injury requiring renal replacement therapy is a serious complication following cardiac surgery associated with poor clinical outcomes. Until now no drug showed nephroprotective effects. Fenoldopam is a dopamine-1 receptor agonist which seems to be effective in improving postoperative renal function. The aim of this paper is to describe the design of the FENO-HSR study, planned to assess the effect of a continuous infusion of fenoldopam in reducing the need for renal replacement therapy in patients with acute kidney injury after cardiac surgery. METHODS: We're performing a double blind, placebo-controlled multicentre randomized trial in over 20 Italian hospitals. Patients who develop acute renal failure defined as R of RIFLE score following cardiac surgery are randomized to receive a 96-hours continuous infusion of either fenoldopam (0.025-0.3 µg/kg/min) or placebo. RESULTS: The primary endpoint will be the rate of renal replacement therapy. Secondary endpoints will be: mortality, time on mechanical ventilation, length of intensive care unit and hospital stay, peak serum creatinine and the rate of acute renal failure (following the RIFLE score). CONCLUSIONS: This trial is planned to assess if fenoldopam could improve relevant outcomes in patients undergoing cardiac surgery who develop acute renal dysfunction. Results of this double-blind randomized trial could provide important insights to improve the management strategy of patients at high risk for postoperative acute kidney injury.

[Anesthesia for non-cardiac surgery in children with congenital heart diseases]. / Anestesia per chirurgia non cardiaca nel bambino con cardiopatia congenita.

The incidence of congenital heart diseases accounts for 8-10 over 1000 liveborn. In Italy about 4000-4500 babies each year are born with congenital heart diseases; 50% of those babies (2000-2200) need cardiac surgery shortly after birth or within the first few months of life. Of the remaining 50%, half undergoes cardiac surgery later on in life and half does not necessitate any surgery; 30% of all cardiac operations consist of palliative procedures and the remaining 70% consist of one-stage corrective procedures. Improvements achieved both in surgical and anesthesiologic techniques, and in cardiopulmonary bypass and myocardial protection, have led to better results in pediatric cardiac surgery, with excellent long term survival rate, even for the more complex variants of congenital heart malformations. Therefore anesthesiologists are now more often required to deal with patients affected by congenital heart defects, for other than cardiac problems. Accurate investigation of patient's clinical history is strongly suggested. Moreover knowledge and familiarity with the modifications of the physiology, occurring in congenital heart disease patients, are mandatory for the choice of the more appropriate anesthesiologic strategy for each patient, in order to optimise the risk-benefits ratio and achieve a less traumatic impact on the cardio-circulatory and respiratory equilibrium. With the aim of achieving better results, interaction between anesthesiologist, cardiologist, pediatrician, surgeon and sometime neonatologist and cardiac surgeon, is strongly recommended in the evaluation of risks, and in decision making of strategies and timing of treatment.

Uncoupling of inflammatory chemokine receptors by IL-10: generation of functional decoys.

As originally demonstrated for the interleukin 1 (IL-1) type II receptor, some primary proinflammatory cytokines from the IL-1 and tumor necrosis factor families are regulated by decoy receptors that are structurally incapable of signaling. Here we report that concomitant exposure to proinflammatory signals and IL-10 generates functional decoy receptors in the chemokine system. Inflammatory signals, which cause dendritic cell (DC) maturation and migration to lymphoid organs, induce a chemokine receptor switch, with down-regulation of inflammatory receptors (such as CCR1, CCR2, CCR5) and induction of CCR7. Concomitant exposure to lipopolysaccharide (LPS) and IL-10 blocks the chemokine receptor switch associated with DC maturation. LPS + IL-10-treated DCs showed low expression of CCR7 and high expression of CCR1, CCR2 and CCR5. These receptors were unable to elicit migration. We provide evidence that uncoupled receptors, expressed on LPS + IL-10-treated cells, sequester and scavenge inflammatory chemokines. Similar results were obtained for monocytes exposed to activating signals and IL-10. Thus, in an inflammatory environment, IL-10 generates functional decoy receptors on DC and monocytes, which act as molecular sinks and scavengers for inflammatory chemokines.

A new patch for the Norwood procedure.

The problems related to the pediatric pulmonary homograft availability and the possible transmission of viral infection led us to design a new patch for aortic enlargement in the Norwood procedure for hypoplastic left heart syndrome. This sterile bovine pericardial patch is not expensive and can be tailor-made.

Development of recombinant diagnostic reagents based on pp85(U14) and p86(U11) proteins to detect the human immune response to human herpesvirus 7 infection.

Human antibodies raised in response to human herpesvirus 7 (HHV-7) infection are directed predominantly to one or more HHV-7-infected cell proteins with apparent molecular masses of about 85 to 89 kDa. The genes that encode these proteins are unknown. However, several HHH-7 genes that possibly encode proteins in this molecular mass range have been identified. Thus, the proteins encoded by open reading frame U14 (85 kDa) and U11 (86 kDa) were expressed as recombinant proteins in bacteria. Of 13 human serum specimens that recognized the 85- to 89-kDa protein(s) of HHV-7-infected cells by immunoblotting, 12 were also reactive with recombinant pp85(U14) and 8 were reactive with p86(U11). It is concluded that (i) the HHV-7 immunodominant protein is pp85(U14) and (ii) the lack of posttranslational modifications in procaryotically expressed pp85 does not adversely affect the reactivity of human sera. Monoclonal antibody (MAb) 5E1 is an HHV-7-specific MAb directed to pp85(U14). Here, the HHV-7-specific epitope in pp85(U14) was finely mapped to the C' terminal region between amino acid residues 484 and 502. However, as indicated by the low level of reactivity of human sera with the HHV-7-specific epitope recognized by MAb 5E1, human sera recognize additional epitopes of pp85(U14) that are required for their full reactivity.

Interrupted aortic arch and aortopulmonary window: one-stage repair in the first week of life.

Aortic arch interruption associated with an aortopulmonary window is a rare congenital malformation that needs an early diagnosis and surgical treatment to avoid irreversible pulmonary lesions. Here we describe a case of a successful one-stage surgical repair in a 3-day-old neonate, without the use of prosthetic material, for the correction of the aortic arch interruption.

Aortic coarctation with hypoplastic arch in neonates: a spectrum of anatomic lesions requiring different surgical options.

Hypoplasia of the transverse aortic arch is frequently associated with isthmic coarctation in many patients referred for operation in early infancy, and the surgical technique should be adjusted to suit each type of anatomic lesion. Referring to the anatomic description of hypoplastic aortic arch reported by Moulaert and associates, between January 1988 and July 1991 we operated on 32 consecutive infants (< or = 3 months old) using a surgical approach based on the echocardiographic and angiographic findings; 20 patients (62%) were younger than 2 weeks of age and 20 patients (62%) had associated intracardiac lesions. According to the location, extension, and size of the hypoplasia of the aortic arch, we had three groups of patients: in group 1 (21 patients) we performed resection and extended end-to-end anastomosis, as previously described in 1985; in group 2 (5 patients) we performed resection, posterior end-to-end anastomosis, and anterior subclavian flap enlargement; and in group 3 (6 patients) we performed direct side-to-end anastomosis between ascending and descending aorta through a median sternotomy. One patient died during the postoperative course in group 3. With a mean follow-up time of 26 months we had 4 cases (13%) of "residual" or "recurrent" coarctation in group 1, successfully repaired at 2 months of age by an anterior approach in 2 patients and by percutaneous angioplasty in the others. In conclusion, hypoplastic aortic arch in neonates represents a common difficulty, and optimal reconstruction of the entire aortic arch is mandatory to reduce operative mortality and incidence of recoarctation, especially when there are complex associated intracardiac lesions or left ventricular dysfunction.

Successful surgical repair of an aortico-left ventricular tunnel in a two day old child.

Repair of an aortico-left ventricular tunnel was successfully accomplished on the second day of life in a neonate with severe cardiac failure. During pregnancy an echocardiographic diagnosis of aortic insufficiency (AI) was made and the type of the malformation was completely clarified after birth. Repair was accomplished using an open patch aortoplasty technique as suggested by Bjork.

Tubular supravalvar aortic stenosis: replacement of ascending aorta in a young patient.

The diffuse form of supravalvar aortic stenosis represents a surgical challenge when ascending aorta and proximal aortic arch are involved. We describe a technique performed on a 14-year-old patient with normal aortic annulus and severe diffuse supravalvar aortic stenosis in which the replacement of ascending aorta and proximal aortic arch with a tubular prosthetic graft completely relieved the obstruction as confirmed by cardiac catheterization.

[Psychometric and electromyographic studies in patients with chronic uremia subjected to periodic hemodialysis]. / Osservazioni psicometriche ed elettromiografiche in pazienti uremici cronici sottoposti ad emodialisi periodica.

Twenty-five patients suffering from chronic renal insufficiency and who had been undergoing dialysis for a long time were tested on the Wechsler-Bellevue Intelligence Scale with a control of twenty-five subjects of the same age with a comparable educational and social background. The results of the statistical analysis of the difference between the two groups demonstrates a significant psychological deterioration in the group of uremic patients which was particularly noticeable in the performance test. The rate of deterioration was not significantly related to the duration of the dialysis. At the same time electromyography (motor conduction velocity of the S.P.E. nerve) show in 40% of the uremic patients evidence of neuropathy.