Laparoscopic surgery of adrenal cysts: results of a series of 18 patients

Indexación: Web of Science; Scielo.Introducción: Los quistes suprarrenales tienen una incidencia de 0,064% a 0,18% en autopsias. El objetivo de este trabajo es mostrar nuestra experiencia en 18 pacientes portadores de un quiste adrenal operados por vía laparoscópica. Material y Métodos: La serie está constituida por 18 pacientes, de una serie de 344 adrenalectomías laparoscópicas realizadas entre los años 1993 y 2011. El rango de edad fue de 20 a 61 años, con un promedio de 38 años, 11 mujeres y 7 hombres. Resultados: Trece quistes eran del lado izquierdo y 5 del derecho. El tamaño de la lesión varió entre 4,5 y 12,5 cm, con un promedio de 9,2 cm. En 8 pacientes hubo molestias abdominales vagas y en los restantes 12 pacientes no hubo signos ni síntomas de sospecha clínica. La cirugía realizada fue una decorticación-marsupialización laparoscópica en 11 pacientes, adrenalectomía laparoscópica en bloque en 8 casos y adrenalectomía parcial en 1 caso. El tiempo operatorio promedio fue de 83,1 min (30-180 min), y el tiempo medio de hospitalización fue de 36 h. Complicaciones intraoperatorias ocurrieron en 2 pacientes: 1 lesión de diafragma y 1 lesión de vena renal, reparadas en el mismo acto quirúrgico. Complicaciones postoperatorias ocurrieron en 2 pacientes, ambas Clavien 3a y de manejo conservador. Conclusión: Las lesiones pequeñas y no funcionantes requieren observación. La adrenalectomía laparoscópica parcial o total está indicada en lesiones de gran tamaño, con importante compromiso de la glándula o ante la sospecha de lesión funcionante o malignidad.Introduction: Adrenal cysts have an incidence of 0.064% to 0.18% at autopsy. Aim: To show our experience in 18 patients with an adrenal cyst operated by laparoscopy. Material and Methods: The series consisted of 18 patients operated, in a series of 344 laparoscopic adrenalectomy performed between 1993 and 2011. The age range was 20 to 61 years with an average of 38 years, 11 women and 7 men. Results: Thirteen cysts were left and 5 were right sided. The lesion size varied between 4.5 and 12.5 cm, with an average of 9.2 cm. Eight patients complained of abdominal discomfort and in the remaining 12 patients there were no signs or symptoms of clinical suspicion. The surgery performed was a laparoscopic decortication-marsupialization in 11 patients, laparoscopic adrenalectomy in 8 cases and partial adrenalectomy in 1 case. Mean operative time was 83.1 minutes (30-180 minutes), and mean hospital stay was 36 hours. Intraoperative complications occurred in 2 patients: 1 diaphragm injury and one renal vein injury, repaired in the same surgery. Postoperative complications occurred in 2 patients, both Clavien 3a, treated by conservative management. Conclusion: Small and non-functional lesions require observation. Partial or total laparoscopic adrenalectomy is indicated in large lesions or in functional and suspected malignant lesions.http://ref.scielo.org/bm4t5

Pakistani children’s experiences of growing up with Beta-Thalassemia Major

In this study, we explored the lived experiences of children with beta-thalassemia major (β-TM). We considered children as experts on their experiences in contrast to the prevalent approach of asking parents or other adults about children’s perspectives. The sample consisted of 12 children aged 8 to12 years. There were two stages to data collection. In Stage 1 we employed two focus group discussions and two role plays and analyzed the data thematically. This directly informed Stage 2, consisting of 12 in-depth interviews subjected to interpretative phenomenological analysis. From our findings we show that living with β-TM involves a continuous struggle between feelings of being different and strategies to minimize these differences to strive for normalcy. We suggest that understanding the experiences of living with β-TM from children’s perspectives can provide unique insights into their experiences, which can fill the gap in the existing, predominantly adult-oriented research on chronic illness

Combination of terbium-161 with somatostatin receptor antagonists—a potential paradigm shift for the treatment of neuroendocrine neoplasms

Purpose: The β¯-emitting terbium-161 also emits conversion and Auger electrons, which are believed to be effective in killing single cancer cells. Terbium-161 was applied with somatostatin receptor (SSTR) agonists that localize in the cytoplasm (DOTATOC) and cellular nucleus (DOTATOC-NLS) or with a SSTR antagonist that localizes at the cell membrane (DOTA-LM3). The aim was to identify the most favorable peptide/terbium-161 combination for the treatment of neuroendocrine neoplasms (NENs). Methods: The capability of the 161Tb- and 177Lu-labeled somatostatin (SST) analogues to reduce viability and survival of SSTR-positive AR42J tumor cells was investigated in vitro. The radiopeptides' tissue distribution profiles were assessed in tumor-bearing mice. The efficacy of terbium-161 compared to lutetium-177 was investigated in therapy studies in mice using DOTATOC or DOTA-LM3, respectively. Results: In vitro, [161Tb]Tb-DOTA-LM3 was 102-fold more potent than [177Lu]Lu-DOTA-LM3; however, 161Tb-labeled DOTATOC and DOTATOC-NLS were only 4- to fivefold more effective inhibiting tumor cell viability than their 177Lu-labeled counterparts. This result was confirmed in vivo and demonstrated that [161Tb]Tb-DOTA-LM3 was significantly more effective in delaying tumor growth than [177Lu]Lu-DOTA-LM3, thereby, prolonging survival of the mice. A therapeutic advantage of terbium-161 over lutetium-177 was also manifest when applied with DOTATOC. Since the nuclear localizing sequence (NLS) compromised the in vivo tissue distribution of DOTATOC-NLS, it was not used for therapy. Conclusion: The use of membrane-localizing DOTA-LM3 was beneficial and profited from the short-ranged electrons emitted by terbium-161. Based on these preclinical data, [161Tb]Tb-DOTA-LM3 may outperform the clinically employed [177Lu]Lu-DOTATOC for the treatment of patients with NENs

Ethical issues of unrelated hematopoietic stem cell transplantation in adult thalassemia patients

<p>Abstract</p> <p>Background</p> <p>Beta thalassemia major is a severe inherited form of hemolytic anemia that results from ineffective erythropoiesis. Allogenic hematopoietic stem cell transplantation (HSCT) remains the only potentially curative therapy. Unfortunately, the subgroup of adult thalassemia patients with hepatomegaly, portal fibrosis and a history of irregular iron chelation have an elevated risk for transplantation-related mortality that is currently estimated to be about 29 percent.</p> <p>Discussion</p> <p>Thalassemia patients may be faced with a difficult choice: they can either continue conventional transfusion and iron chelation therapy or accept the high mortality risk of HSCT in the hope of obtaining complete recovery.</p> <p>Throughout the decision making process, every effort should be made to sustain and enhance autonomous choice. The concept of conscious consent becomes particularly important. The patient must be made fully aware of the favourable and adverse outcomes of HSCT. Although it is the physician's duty to illustrate the possibility of completely restoring health, considerable emphasis should be put on the adverse effects of the procedure. The physician also needs to decide whether the patient is eligible for HSCT according to the "rule of descending order". The patient must be given full details on self-care and fundamental lifestyle changes and be fully aware that he/she will be partly responsible for the outcome.</p> <p>Summary</p> <p>Only if all the aforesaid conditions are satisfied can it be considered reasonable to propose unrelated HSCT as a potential cure for high risk thalassemia patients.</p

How early can myocardial iron overload occur in Beta thalassemia major?

BACKGROUND: Myocardial siderosis is the most common cause of death in patients with beta thalassemia major(TM). This study aimed at investigating the occurrence, prevalence and severity of cardiac iron overload in a young Chinese population with beta TM. METHODS AND RESULTS: We analyzed T2* cardiac magnetic resonance (CMR), left ventricular ejection fraction (LVEF) and serum ferritin (SF) in 201 beta TM patients. The median age was 9 years old. Patients received an average of 13 units of blood per year. The median SF level was 4536 ng/ml and 165 patients (82.1%) had SF>2500 ng/ml. Myocardial iron overload was detected in 68 patients (33.8%) and severe myocardial iron overload was detected in 26 patients (12.6%). Twenty-two patients ≤10 years old had myocardial iron overload, three of whom were only 6 years old. No myocardial iron overload was detected under the age of 6 years. Median LVEF was 64% (measured by CMR in 175 patients). Five of 6 patients with a LVEF<56% and 8 of 10 patients with cardiac disease had myocardial iron overload. CONCLUSIONS: The TM patients under follow-up at this regional centre in China patients are younger than other reported cohorts, more poorly-chelated, and have a high burden of iron overload. Myocardial siderosis occurred in patients younger than previously reported, and was strongly associated with impaired LVEF and cardiac disease. For such poorly-chelated TM patients, our data shows that the first assessment of cardiac T2* should be performed as early as 6 years old

Beta-thalassemia

Beta-thalassemias are a group of hereditary blood disorders characterized by anomalies in the synthesis of the beta chains of hemoglobin resulting in variable phenotypes ranging from severe anemia to clinically asymptomatic individuals. The total annual incidence of symptomatic individuals is estimated at 1 in 100,000 throughout the world and 1 in 10,000 people in the European Union. Three main forms have been described: thalassemia major, thalassemia intermedia and thalassemia minor. Individuals with thalassemia major usually present within the first two years of life with severe anemia, requiring regular red blood cell (RBC) transfusions. Findings in untreated or poorly transfused individuals with thalassemia major, as seen in some developing countries, are growth retardation, pallor, jaundice, poor musculature, hepatosplenomegaly, leg ulcers, development of masses from extramedullary hematopoiesis, and skeletal changes that result from expansion of the bone marrow. Regular transfusion therapy leads to iron overload-related complications including endocrine complication (growth retardation, failure of sexual maturation, diabetes mellitus, and insufficiency of the parathyroid, thyroid, pituitary, and less commonly, adrenal glands), dilated myocardiopathy, liver fibrosis and cirrhosis). Patients with thalassemia intermedia present later in life with moderate anemia and do not require regular transfusions. Main clinical features in these patients are hypertrophy of erythroid marrow with medullary and extramedullary hematopoiesis and its complications (osteoporosis, masses of erythropoietic tissue that primarily affect the spleen, liver, lymph nodes, chest and spine, and bone deformities and typical facial changes), gallstones, painful leg ulcers and increased predisposition to thrombosis. Thalassemia minor is clinically asymptomatic but some subjects may have moderate anemia. Beta-thalassemias are caused by point mutations or, more rarely, deletions in the beta globin gene on chromosome 11, leading to reduced (beta+) or absent (beta0) synthesis of the beta chains of hemoglobin (Hb). Transmission is autosomal recessive; however, dominant mutations have also been reported. Diagnosis of thalassemia is based on hematologic and molecular genetic testing. Differential diagnosis is usually straightforward but may include genetic sideroblastic anemias, congenital dyserythropoietic anemias, and other conditions with high levels of HbF (such as juvenile myelomonocytic leukemia and aplastic anemia). Genetic counseling is recommended and prenatal diagnosis may be offered. Treatment of thalassemia major includes regular RBC transfusions, iron chelation and management of secondary complications of iron overload. In some circumstances, spleen removal may be required. Bone marrow transplantation remains the only definitive cure currently available. Individuals with thalassemia intermedia may require splenectomy, folic acid supplementation, treatment of extramedullary erythropoietic masses and leg ulcers, prevention and therapy of thromboembolic events. Prognosis for individuals with beta-thalassemia has improved substantially in the last 20 years following recent medical advances in transfusion, iron chelation and bone marrow transplantation therapy. However, cardiac disease remains the main cause of death in patients with iron overload

The efficacy of iron chelator regimes in reducing cardiac and hepatic iron in patients with thalassaemia major: a clinical observational study

<p>Abstract</p> <p>Background</p> <p>Available iron chelation regimes in thalassaemia may achieve different changes in cardiac and hepatic iron as assessed by MR. The aim of this study was to assess the efficacy of four available iron chelator regimes in 232 thalassaemia major patients by assessing the rate of change in repeated measurements of cardiac and hepatic MR.</p> <p>Results</p> <p>For the heart, deferiprone and the combination of deferiprone and deferoxamine significantly reduced cardiac iron at all levels of iron loading. As patients were on deferasirox for a shorter time, a second analysis ("Initial interval analysis") assessing the change between the first two recorded MR results for both cardiac and hepatic iron (minimum interval 12 months) was made. Combination therapy achieved the most rapid fall in cardiac iron load at all levels and deferiprone alone was significantly effective with moderate and mild iron load. In the liver, deferasirox effected significant falls in iron load and combination therapy resulted in the most rapid decline.</p> <p>Conclusion</p> <p>With the knowledge of the efficacy of the different available regimes and the specific iron load in the heart and the liver, appropriate tailoring of chelation therapy should allow clearance of iron. Combination therapy is best in reducing both cardiac and hepatic iron, while monotherapy with deferiprone or deferasirox are effective in the heart and liver respectively. The outcomes of this study may be useful to physicians as to the chelation they should prescribe according to the levels of iron load found in the heart and liver by MR.</p

Ceftriaxone induced hemolysis complicated by acute renal failure

Over the last decade, second and third generation cephalosporins have been the most common drugs causing hemolytic anemia (HA). Of these cases, 20% have been attributed to ceftriaxone. The clinical presentation of ceftriaxone-induced HA is usually abrupt with sudden onset of pallor, tachypnea, cardio-respiratory arrest and shock. Acute renal failure (ARF) has been reported in 41% of such cases with a high fatality rate. We report a pediatric patient with ARF complicating ceftriaxone-induced HA who survived. Ceftriaxone is a commonly used drug, and early recognition of HA and institution of supportive care, including dialysis is likely to improve the outcome. Pediatr Blood Cancer 2008;50:139–142. © 2006 Wiley-Liss, Inc.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/57397/1/20839_ftp.pd

Attenuated total reflection infrared spectroscopy for studying adsorbates on planar model catalysts : CO adsorption on silica supported Rh nanoparticles

A sensitive method is presented for studying adsorption of gaseous species on metal surfaces in vacuum by attenuated total internal reflection Fourier transform IR spectroscopy (ATR). The method is illustrated by CO adsorption expts. on silica supported Rh nanoparticles. An exptl. setup and a procedure are described in detail to obtain a sensitivity of reflectance change of .apprx.5 * 10-5 absorbance units. Here, a silicon ATR crystal with a 50 nm layer of hydroxylated silica acts as the support for the Rh nanoparticles. These particles are easily prepd. by spincoat impregnation from a RhCl3 soln. followed by H2 redn. XPS before and after redn. shows that rhodium is reduced to Rh0 and that all chlorine is removed. At. force microscope images the distribution of the particles, which are 3-4 nm in height. When the crystal is exposed to pressures up to 1 mbar of CO, a gas which is inert to the silica support, the stretch vibration of linearly adsorbed CO on the Rh nanoparticles is detected at 2023 cm-1, while no bridged CO or geminal dicarbonyl species can be distinguished. The min. detectable coverage is estd. .apprx.0.005 CO per nm2 substrate area or .apprx.5 * 10-4 ML. [on SciFinder (R)