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Biomedical subjects

A Sáenz

Publications and source records attributed to A Sáenz.

At least 19 recordsLinked to original sources
PubMed2003

Multicenter study evaluating a dual policy of postorchiectomy surveillance and selective adjuvant single-agent carboplatin for patients with clinical stage I seminoma.

BACKGROUND: After decades of irradiation as standard therapy for clinical stage I testicular seminoma, alternative treatment approaches have emerged including postorchiectomy surveillance and adjuvant chemotherapy. This study was performed to assess a dual policy of surveillance and selective single-agent carboplatin (for high-risk cases) in a multicenter setting. PATIENTS AND METHODS: From 1994 to 1999, 203 patients with stage I seminoma were included. Sixty (29.6%) were considered poor-risk cases (i.e. with vascular invasion and/or pathological tumor stage pT2 or greater) and received two courses of adjuvant carboplatin, whereas 143 (70.4%) without risk criteria underwent close surveillance. RESULTS: Median follow-up was 52 months (range 14-92). Relapses were observed in two (3.3%) patients treated with carboplatin and in 23 patients (16.1%) on surveillance, with a median time to recurrence of 11 months (range 3.9-39.6). All relapsing patients were rendered disease-free, mainly with cisplatin-based chemotherapy. Four patients died from tumor-unrelated causes. Actuarial 5-year overall survival was 96.7% and cause-specific survival was 100%. Five-year disease-free survival was 83.5% for patients on surveillance, and 96.6% for those receiving carboplatin. CONCLUSIONS: This dual treatment policy is feasible in a multicenter setting and preserves 70% of patients from adjuvant chemotherapy. Single-agent carboplatin is effective in reducing the relapse rate in patients with high-risk stage I seminoma. A better definition of local risk features would probably improve patient selection, thus minimizing the incidence of recurrences on surveillance.

Adolescent↗
PubMed2002

Outcome of laparoscopic pancreatic surgery: endocrine and nonendocrine tumors.

Laparoscopic pancreatic surgery (LapPS) for management of benign pancreatic tumors has still not been defined. This paper evaluates the feasibility and outcome of LapPS in patients with endocrine pancreatic tumors (EPTs) and cystic neoplasms of the pancreas (CyNP). Eighteen patients with benign pancreatic tumors underwent LapPS between January 1998 and May 2001. The indications were 10 EPTs (6 sporadic insulinomas, 1 multiple insulinoma of multiple endocrine neoplasia type 1, 2 nonfunctioning tumors, 1 VIPoma) and 8 CyNPs (3 serous cystadenomas, 5 mucinous cystic neoplasms). The laparoscopic procedure was performed using four ports with patients in the half-lateral position. Laparoscopic ultrasonography (LapUS) was used in all cases. Laparoscopic enucleation (LapE) was planned in five patients and performed in four (one conversion for tumor not found during laparoscopy). Laparoscopic pancreatic resection (LapPR) with spleen salvage was planned in 13 patients and performed in 12 (one conversion for metastatic VIPoma), with splenic vessel preservation in 11 patients and short gastric vessel preservation in 1. The average operating time was 3.5 hours after enucleation, 4.0 hours after distal pancreatectomy, and 5.0 hours after subtotal pancreatectomy. Pancreatic fistula was observed in two patients after LapE and in three patients after LapPR. Splenectomy for splenic abscess was performed 1 week after surgery in a patient with short gastric vessel splenic preservation. The average hospital stay was 5 days. We concluded that LapPS is a safe method for removing EPTs and CyNPs, although the incidence of pancreatic fistulas remains high. In selected patients LapPS offers significant benefit to patients: reduced trauma to the abdominal wall, short hospital stay, and a quick postoperative recovery.

Adult↗
PubMed2002

Laparoscopic pancreatic surgery in patients with chronic pancreatitis.

BACKGROUND: In recent years, technological advances and technical refinements to laparoscopic instruments have encouraged some surgeons to explore the application of laparoscopic methods to benign disorders of the pancreas. The aim of this report was to evaluate the feasibility and outcome of laparoscopic pancreatic surgery in patients with chronic pancreatitis. METHODS: One group of five patients with disease of nonalcoholic origin localized in the body-tail of the pancreas underwent distal pancreatectomy with preservation of the splenic vessels; a second group of six patients with symptomatic pancreatic pseudocysts (alcoholic origin in four cases and idiopathic in two cases) underwent laparoscopic transgastric drainage. For distal pancreatectomy and spleen salvage, the patient's positioning was half-lateral decubitus with the left side up. Four ports were used. A comparison was made with 41 patients with chronic, pancreatitis who underwent conventional open distal pancreatectomy. For the patients with laparoscopic distal pancreatectomy, the mean operative time was 4 h (range 3-5). RESULTS: There were no pancreatic-related complications, but one patient was reoperated for perforation of duodenal ulcer. The mean hospital stay was 6 days and the mean time to resume normal daily activities was 3 weeks. Laparoscopic pseudocyst drainage was performed in four patients via laparoscopic anterior gastrostomy and two patients via laparoscopic intraluminal cystogastrostomy. The mean operative time was 100 min (range 60-160). There was no morbidity. The mean hospital stay was 5 days, and the mean time to resume normal daily activities was 2 weeks. CONCLUSION: This study provides information about the possibilities of performing laparoscopic surgery in patients with chronic pancreatitis. Laparoscopic distal pancreatectomy with preservation of the splenic vessels and laparoscopic transgastric drainage are feasible and safe techniques. They offer obvious advantages, such as reduction of the parietal damage to the abdomen, a shorter hospital stay, and an earlier postoperative recovery than can be obtained with conventional open pancreatic resection.

Adult↗
PubMed2002

Clinical pattern and therapeutic results achieved in 1490 patients with germ-cell tumours of the testis: the experience of the Spanish Germ-Cell Cancer Group (GG).

OBJECTIVE: To describe the clinical characteristics and treatment results obtained with the application of a homogeneous treatment protocol in 1490 patients with germ-cell tumours (GCT) registered in the 55 hospitals belonging to the Spanish Germ-Cell Cancer Group (GG) during the period between January 1994 and April 2001. METHODS: In general, surveillance was the common policy for stage I patients without local poor prognosis factors, whereas they received adjuvant chemotherapy in case those factor were present. Chemotherapy schedules used in advanced cases were cisplatin and etoposide (EP) for seminoma and BEP or BOMP-EPI in non-seminoma, according to whether the patient was in the good or poor prognosis IGCCCG (International Germ-Cell Cancer Collaborative Group) group. Excision of residual masses was mandatory in non-seminomatous germ-cell tumour (NSGCT). RESULTS: Initial local symptomatology was increased testis size in 90% of cases. Sonography was an excellent diagnostic tool to suggest tumour. Non-seminoma (64.2%) was more frequent than seminoma (35.8%). Approximately 10% had the antecedent of cryptorchidism. Non-seminoma patients were 7 years younger than seminoma. Right testis was involved predominantly. Pre-orchidectomy tumour markers were elevated in 21% of seminoma (betaHGC) and 79% in non-seminoma (alphaFP and/or betaHGC). Scrotum violation occurred in only 1.8%. There were significant differences among stage I and the IGCCCG prognosis groups related to a longer interval between the first symptom and orchiectomy. Eighteen percent of non-seminomatous germ-cell tumour belonged to the poor prognosis IGCCCG group. With a median follow-up to 33 months, this series has achieved a 3 year overall survival of 98% for seminoma and 94% for non-seminoma. Only 10% of excised residual masses present after chemotherapy contained malignant cells. CONCLUSION: Spanish GCT have a similar clinical pattern to that described in the other occidental countries except for a slight increased proportion of non-seminoma upon seminoma. Co-operative groups as GG are unique structures to obtain quick and wide experience on the treatment of testis tumours, contributing to achieve a high cure rate.

Adolescent↗
PubMed2001

[Clinical pattern and therapeutic results obtained in Germ-Cell testicular cancer in Spain based on a consecutive series of 1250 patients].

BACKGROUND: Even its low incidence, germ-cell testicular cancer is very relevant due to its presentation at young ages and its potential curability over 90%. Spanish Germ Cell Cancer Group (GG) joins the efforts of 51 different Spanish centres to share their experience on the diagnosis and treatment of these special tumours. PATIENTS AND METHOD: We describe the clinical characteristics and the results of treatment in the first 1,250 patients registered throughout 6 years by the GG. RESULTS: 11% had previous criptorchidism. The most frequent initial local simptomatology was increased testis size (90%). 20% lasted more than six months in receiving the first treatment. Inguinal orquidectomy was done in 95% of patients. 435 cases (35%) were seminoma and 815 (65%)non-seminoma. 19% of seminoma and 78% of non-seminoma produced tumour markers. 75% of seminoma but only 56% of non-seminoma were clinical stage I. Following the IGCCCG prognosis classification,20% of non-seminoma fitted in the poor-prognosis group. Stage I seminoma treatment was surveillance, chemotherapy and complementary radiotherapy in 60, 32 and 6%, respectively. Those features were 65, 35% and none in non-seminoma cases. Chemotherapy schedules used in advanced cases were EP for seminoma and BEP or BOMP-EPIin non-seminoma, according to whether the patient was in the good or bad prognosis IGCCCG group. With a median of follow-up in all serie of 30 months, we have obtained a three years overall survival of 98% (CI 95%, 96,4-9,6), whereas non-seminoma patients had a three years overall survival of 94% (CI 95%, 92-96). CONCLUSION: The Spanish germ cell testicular cancer clinical pattern is similar to that registered in other occidental countries. Co-operative structures like GG,are able to gather an extensive experience in a short period of time that results in achieving a very high number of cured patients.

Adolescent↗
PubMed2001

Phase I trial of weekly irinotecan combined with UFT as second-line treatment for advanced colorectal cancer.

The aim of this study was to determine the maximum-tolerated dose (MTD) and dose-limiting toxicity (DLT) of weekly Irinotecan (CPT-11) plus UFT, and to assess the antitumour activity of this combination as second-line chemotherapy in patients with advanced colorectal carcinoma, 31 patients with measurable advanced colorectal carcinoma were treated. Cohorts of 3 patients received increasing dose levels of the combination. Levels 1 to 4 included a fixed dose of oral (p.o.) UFT (250 mg/m(2)/day) for 21 days of a 28-day cycle combined with increasing intravenous (i.v.) doses of CPT-11 (80, 100, 110 and 120 mg/m(2)) on days 1, 8 and 15. Levels 5 and 6 included a higher fixed dose of oral UFT (300 mg/m(2)) combined with increasing i.v. doses of CPT-11 (100 and 110 mg/m(2)) on days 1, 8 and 15. 147 courses were administered. MTD were reached at level 4 (2 cases of grade 4 diarrhoea and 1 grade 3 asthenia), and level 6 (1 grade 4 diarrhoea, 1 grade 3 diarrhoea and 1 grade 3 febrile neutropenia). Responses in 30 evaluable patients were: 3 partial responses (10%), 15 stable disease (50%) and progressive disease in 12 patients (40%). Median time to progression was 4.5 months (95% Confidence Interval (CI): 3.4-6.6 months) and median survival was 11 months (95% CI: 7.9-14.1 months). The recommended doses for phase II trials are: (a) CPT-11 110 mg/m(2) i.v. on days 1, 8 and 15 every 28 days plus UFT 250 mg/m(2) p.o. on days 1 through to 21 or (b) CPT-11 100 mg/m(2) and UFT 300 mg/m(2).

Administration, Oral↗
PubMed2001

Laparoscopic pancreatic surgery in patients with neuroendocrine tumours: indications and limits.

Laparoscopic pancreatic procedures are still at an evaluation stage with regard to their indications and techniques. Between January 1998 and December 2000, 13 patients with endocrine pancreatic tumours - 11 insulinomas and 2 non-functioning tumours-underwent laparoscopic surgery, laparoscopic ultrasonography being used in all the patients. Enucleation was performed in five patients. The operative time was 2-3 hours. Distal pancreatectomy was performed in six patients with insulinomas, and spleen preservation with intact splenic vessels was feasible in five. Splenectomy was necessary in one patient for technical reasons. Laparoscopic distal pancreatectomy with splenic vessel preservation was performed in two patients with a large (6 and 8 cm) non-functioning tumour. The mean operative time for all the patients undergoing distal pancreatectomy was 4 hours, ranging from 3 to 5 hours. A pancreatic fistula occurred in three patients after tumour enucleation and in two patients after distal pancreatectomy; the mean hospital stay for all patients was 5 days. Enucleation guided by laparoscopic ultrasonography thus allows safe tumour dissection and excision, laparoscopic distal pancreatectomy also being feasible and safe. Splenic salvage with splenic vessel preservation is technically possible. The laparoscopic approach allows a shorter hospital stay and an earlier return to normal activities.

Adult↗
PubMed2000

Phase I-II study of paclitaxel, cisplatin, and gemcitabine in advanced transitional-cell carcinoma of the urothelium. Spanish Oncology Genitourinary Group.

PURPOSE: To determine the maximum-tolerated dose and the antitumor activity of a combination of paclitaxel, cisplatin, and gemcitabine in advanced transitional-cell carcinoma (TCC) of the urothelium. PATIENTS AND METHODS: Patients with measurable, previously untreated, locally advanced or metastatic TCC and with Eastern Cooperative Oncology Group performance status < or = 2 and creatinine clearance > or = 55 mL/min were eligible. Cisplatin was given on day 1 at a fixed dose of 70 mg/m(2). Paclitaxel and gemcitabine were given on days 1 and 8 at increasing dose levels. Cycles were repeated every 21 days to a maximum of six cycles. RESULTS: Sixty-one patients were registered. In phase I, 15 patients were entered at four different dose levels. Dose-limiting toxicity consisted of early onset (after the first cycle) grade 2 asthenia (two of six patients) and grade 3 asthenia (one of six patients) at dose level 4. A paclitaxel dose of 80 mg/m(2) and gemcitabine 1,000 mg/m(2) was recommended for phase II, and 46 additional patients were entered at this level for a total of 49 patients. Main nonhematologic toxicity was grade 2 asthenia in 18 patients, with early onset in five patients, and grade 3 in four patients. Grade 3/4 neutropenia and thrombocytopenia occurred in 27 (55%) and 11 (22%) patients, respectively. Overall, febrile neutropenia was seen in 11 patients, and one toxic death occurred because of neutropenic sepsis. The combination was active at all dose levels. In total, 58 of 61 eligible patients were assessable for response; 16 complete responses (27.6%) and 29 partial responses (50%) were observed for an overall response rate of 77.6% (95% confidence interval, 60% to 98%). The median survival time (MST) available for the phase I part of the study is 24.0 months. MST has not been reached for the whole group with the current follow-up. CONCLUSION: This combination of paclitaxel, cisplatin, and gemcitabine is feasible and highly active in patients with advanced TCC of the urothelium. Further evaluation of this regimen in patients with TCC is warranted.

Adult↗
PubMed2000

Simultaneous laparoscopic biliary and retrocolic gastric bypass in patients with unresectable carcinoma of the pancreas.

BACKGROUND: A substantial number of patients with unresectable pancreatic cancer eventually develop biliary or gastric outlet obstruction. In some cases, they present initially with both complications. These conditions contribute markedly to their discomfort and certainly justify palliative intervention. The purpose of this study was to examine the feasibility and safety of simultaneous laparoscopic biliary and gastric bypass in patients with unresectable carcinoma of the pancreas. METHODS: Between August 1995 and July 1998, simultaneous laparoscopic biliary and retrocolic gastric bypass was performed successfully in 12 consecutive patients with unresectable carcinoma of the pancreas. There were eight men and four women. Their median age was 72 years (range, 50-82). In all patients, the indications for gastrointestinal bypass were gastric outlet obstruction and obstructive jaundice. The following parameters were evaluated for each patient: procedure-related morbidity and mortality, operative time, length of hospital stay, overall survival, and ability to sustain oral nutrition during the survival period. RESULTS: All procedures were completed laparoscopically. The mean operative time was 89 +/- 29.56 min. There were no intraoperative complications. Postoperative morbidity consisted of wound infection in two patients and pneumonia in one patient. One patient died of multiorgan failure on postoperative day 2. The mean hospital stay was 6.4 +/- 1.5 days (range, 5-17). The mean survival time until death from underlying disease was 85 +/- 32. 46 days (range, 31-260). None of the patients had recurrent jaundice, and all of them were able to maintain oral nutrition. CONCLUSION: Simultaneous laparoscopic biliary and retrocolic gastric bypass is a safe and effective technique for the treatment of biliary and gastroduodenal obstruction in patients with unresectable pancreatic cancer.

Aged↗
PubMed1999

Autosomal dominant nocturnal frontal lobe epilepsy in a Spanish family with a Ser252Phe mutation in the CHRNA4 gene.

BACKGROUND: A large family with autosomal dominant nocturnal frontal lobe epilepsy from the south of Spain was studied. The clinical appearance of the disease in this family, which included 28 members, of whom 11 were affected and 2 were obligate carriers, was identical to that previously described in an Australian family and a Norwegian family, in which mutations in exon 5 of the CHRNA4 gene were found. METHODS: Following DNA extraction, the family was genotyped with 4 fluorescent markers flanking the locus to the CHRNA4 gene on chromosome 20q13.3, and lod score computations were performed. The exon 5 of the CHRNA4 gene was amplified between nucleotides 535 and 825 and polymerase chain reaction products were purified and sequenced directly. RESULTS: The same missense mutation as that found in the Australian family, C-->T, which causes the replacement of a serine with phenylalanine in amino acid 252 in exon 5, was detected. This mutation segregated with the disorder in all 11 affected members, in the 2 obligate carriers, and in 1 asymptomatic sibling, and was not found in 1 spouse and 1 daughter. Neither of the 2 polymorphisms found in a series of families with epilepsy were found in our sample [corrected]. CONCLUSIONS: These data confirm the clinical homogeneity in the phenotypic expression of autosomal dominant nocturnal frontal lobe epilepsy caused by mutation in the CHRNA4 gene, and the pathogenic role of the Ser252Phe mutation in this disorder.

Adult↗
PubMed1999

Autosomal dominant lateral temporal epilepsy: clinical and genetic study of a large Basque pedigree linked to chromosome 10q.

We report a large family with a temporal partial epilepsy syndrome inherited in an autosomal dominant mode, with a penetrance of about 80%. This epilepsy syndrome is benign, with age of onset in the second or third decade of life. It is characterized by rare partial seizures, usually secondarily generalized, arising mostly during sleep, without postictal confusion. There is a good response to the antiepileptic therapy but often a recurrence of seizures after drug withdrawal. The partial component, visual (lights, colors, and simple figures) or auditory (buzzing or "humming like a machine"), the existence of temporo-occipital interictal electroencephalographic epileptiform abnormalities, and the hypoperfusion in the temporal lobe detected by interictal hexamethylpropyleneamine oxime-technetium 99m (HMPAO-Tc99m) single-photon emission computed tomography, strongly suggest a lateral temporal lobe origin. The genetic analysis found linkage to chromosome 10q, and localized a gene in a 15-cM interval that overlaps a previously found localization for partial epilepsy in a large three-generation family. This syndrome could be called autosomal dominant lateral temporal epilepsy.

Chromosomes, Human, Pair 10↗
PubMed1999

Laparoscopic adrenalectomy in the elderly.

Laparoscopic adrenalectomy has recently been shown to be a safe and effective procedure for treating a variety of benign adrenal tumors. Advanced age, with its concomitant comorbid conditions, has been believed to be associated with more postoperative complications in laparoscopic procedures. The purpose of this study was to evaluate the outcome of laparoscopic adrenalectomy in patients age 65 and older. From June 1992 to February 1998, 14 patients (4 men and 10 women) with a mean age of 69 years underwent 17 laparoscopic adrenalectomies. In 12 procedures, a transperitoneal lateral decubitus flank approach was used. The lesion was a nonfunctioning adenoma in three patients, aldosterone adenoma in four, Cushing's syndrome in four, and pheochromocytoma in one. A retroperitoneal lateral decubitus approach was used in five procedures. The lesion was a nonfunctioning adenoma in one patient, aldosterone adenoma in one, Cushing's adenoma in one, and pheochromocytoma in two. Seventy-eight percent of these patients had comorbid conditions, including hypertension, diabetes, chronic obstructive airway disease, coronary artery disease, and cardiac dysrhythmia. The preoperative physical status was as ASA Class II in 11 patients and ASA III in 3. Two of the 17 laparoscopies were converted to open surgery (11%), in one because of difficulties in dissecting extraperitoneally a mass >8 cm, and in the other because of difficulties in localization of a 3-cm mass. The median surgical time was 95 +/- 33 minutes. The mean analgesia requirements were 3 doses of (range 2-7) ketorolac. There were no deaths. Postoperative morbidity consisted of pulmonary atelectasis in one patient and urinary tract infection in two patients. The median hospital stay was 3 days (range 2-4 days). We conclude that laparoscopic adrenalectomy in the elderly population is safe and offers low morbidity, fast recovery, and a short hospital stay. Age alone should not be a contraindication to treating adrenal tumors laparoscopically.

Adrenal Gland Neoplasms↗
PubMed1999

Hemoperitoneum due to a ruptured gastric stromal tumor.

BACKGROUND/AIMS: Gastrointestinal stromal tumors form a group of uncommon neoplasms originated from pluripotential mesenchymal cells. Many patients are asymptomatic and the tumor is discovered during an abdominal operation. Massive intraperitoneal bleeding is an exceptional complication associated with high mortality rates. Our aim is to report a case of a gastric stromal tumor in an 83-year-old patient presenting with intraperitoneal hemorrhage and hypovolemic shock, successfully operated. METHODS: Emergency laparotomy showed a hemoperitoneum caused by rupture of a large exogastric tumor attached to the greater curvature. Total gastrectomy and esophagojejunostomy was performed. RESULTS: Histological examination revealed proliferation of spindle-shaped cells but immunocytochemistry failed to identify specific markers of smooth muscle and neural cells. Diagnosis of a gastric stromal tumor was made. Postoperative evolution was uncomplicated. CONCLUSION: Gastric stromal tumor is a relatively rare neoplasm of mesenchymal origin whose nature and prognosis is unclear.

Aged↗
PubMed1998

[Muscular dystrophy due to a mutation in the gene of alpha-sarcoglycan subunit of dystrophin associated protein complex].

Linkage studies have confirmed the existence of clinical an genetic heterogeneity among the muscular dystrophies due to adhalin deficiency. We present the clinical, histological and genetic characteristics in a case of primary adhalinopathy (deficiency of the 50 kD subunit or alpha-sarcoglycan). It was a 19 years-old woman, born of non consanguineous parents, who shows a long evolution myopathy with onset before age 7, a severe evolution and becoming wheelchair bound at 10 years. She showed evident calf pseudohypertrophy and serum creatinkinase (CK) levels were elevated (40-180 times the standard level). The histological pattern showed a destructed fascicular architecture in agreement with severe muscular dystrophy, normal staining with anti-dystrophin monoclonal antibodies and abnormal staining pattern with anti-adhalin antibodies. The molecular study evidenced an homozygous point mutation (Arg-->Cys) at position 77 of exon 3 of the gene coding for the 50 kD subunit of the alpha-sarcoglycan complex localised in chromosome 17. In the light of this case, we suggest a revision of the diagnostic orientation in the muscular dystrophies and we review the new taxonomy of the limb-girdle muscular dystrophies, remarking the clinical signs which could indicate a given genetic locus.

Adult↗
PubMed1998

Cryopreservation of pancreatic islets prior to transplantation: a comparison between UW solution and RPMI culture medium.

Effective cryopreservation of pancreatic islets would be valuable in several contexts: for the assessment of islet cell viability, the measurement of beta-cell function, and the maintenance of viability and sterility prior to islet transplantation. In this study, isolated rat islets were cryopreserved or not following overnight culture and the most suitable preservation solution for transportation between centers was sought. Unfrozen and frozen-and-thawed islets were allocated to each of four different groups: untreated controls; cultured overnight in RPMI at 37 degrees C; cold stored at 4 degrees C in RPMI for 18-24 h; and stored at 4 degrees C in University of Wisconsin (UW) solution for 18-24 h. The greatest cell viability, as assessed by ethidium bromide/acridine orange staining and image analysis, was observed when postthawed islets were cultured in RPMI, whereas the least viable samples were those that were stored in UW solution. Measurement of insulin content and secretion in static incubation assays using 2.8 and 16.7 mM glucose showed that all treated groups exhibited a significant insulin secretory response to glucose stimulation whereas the untreated frozen-thawed islets failed to show any response. The cryopreserved islets in each group were equally successful in reversing hyperglycemia in streptozotocin-treated allogeneic rats when grafted intraportally in sufficient numbers (2000-2500). The groups also showed a similar mean graft survival time of 6-7 days before rejection. However, the best experimental group (the postthaw cultured islets) failed to cure diabetic rats when grafted in a smaller numbers (<2000). These data demonstrate prompt and sustained function in cryopreserved islets when they were maintained by any of the methods studied if they were grafted in sufficient numbers. We conclude that cold storage of thawed cryopreserved islets using either RPMI or UW solution is an effective method for their transportation and/or storage, but does not reduce their immunogenicity before transplantation.

Adenosine↗
PubMed1998

Helium and carbon dioxide pneumoperitoneum in patients with pheochromocytoma undergoing laparoscopic adrenalectomy.

Insufflation with helium is used to prevent respiratory acidosis, hypercapnia, and cardiovascular instability associated with carbon dioxide (CO2) pneumoperitoneum. The aim of this prospective study was to compare CO2 with helium pneumoperitoneum with special reference to respiratory and hemodynamic changes at different times during the operation. Altogether 22 pheochromocytoma patients undergoing laparoscopic adrenalectomy (LpA) were included using CO2 in 11 patients (CO2LpA) and helium in 11 patients (HeLpA). The insufflation pressure was 12 mmHg. The two groups were comparable with regard to demographic data and preoperative management. CO2 and helium insufflation were associated with similar catecholamine increase. The most striking significant increase compared with the baseline was observed during tumor isolation: The mean plasma epinephrine (EPI) and norepinephrine (NE) levels increased 32.86-fold and 25.92-fold, respectively, in the CO2LpA patients and 27.43-fold and 18.46-fold, respectively, in the HeLpA patients. HeLpA did not result in significant hypercarbia or acidosis at any measured intraoperative point; this was without any alteration in minute ventilation to maintain these normal PaCO2, excess base (EB), and pH values. Significant increases of mean arterial pressure, pulmonary arterial pressure, pulmonary vascular resistance index, PaCO2, EB, and acidosis were seen in the CO2LpA patients at the time of tumor isolation and tumor removal compared with those in HeLpA patients. No patient required conversion to open surgery. There were no significant differences between CO2LpA and HeLpA regarding mean operative time (117.50 +/- 93.68 vs. 106.87 +/- 16.60 minutes), mean blood loss (168.54 +/- 78.63 vs. 142.02 +/- 109.26 ml), hospital stay (4 days), the need for analgesics, or mean time required to return to normal activity (12 days). There was one wound infection in the HeLpA group and one wound hematoma and one case of atelectasis in the CO2LpA group. Helium may be the agent of choice for abdominal insufflation in patients undergoing LpA for pheochromocytoma, eliminating the adverse hemodynamic and respiratory changes associated with CO2 insufflation.

Adrenal Gland Neoplasms↗
PubMed1998

Limb-girdle muscular dystrophy in Guipúzcoa (Basque Country, Spain).

The concept of limb-girdle muscular dystrophy (LGMD) is changing rapidly due to the advances in molecular genetics. Recently, seven different gene loci have been described, demonstrating that limb-girdle muscular dystrophy is a heterogeneous syndrome, which includes different diseases with a similar phenotype. In isolated populations which have little genetic exchange with neighbouring populations, an accumulation of cases may be found. We carried out an epidemiological study in Guipúzcoa, a small mountainous Basque province in northern Spain, and found the highest prevalence rate of LGMD described so far: 69 per million. Genetic studies demonstrated that 38 cases corresponded to the LGMD2A type, due to calpain-3 gene mutations. Only one patient with alpha-sarcoglycanopathy was found, and in 12 patients the genetic defect was not identified. Moreover, the particular calpain-3 mutation predominant in Basque chromosomes (exon 22, 2362AG-->TCATCT), has only been rarely found in the rest of the world. This observation strongly suggests a founder effect in the indigenous population of Guipúzcoa. The clinical characteristics of the patients with calpain-3 gene mutations were quite homogeneous and different from the other groups (sarcoglycanopathy and unknown gene defect), allowing for a precise clinical diagnostic. The disease onset was between the ages of 8 and 15 years, in most cases in the pelvic girdle, and the patients became wheelchair-bound between 11 and 28 years after onset. No pseudohypertrophy of calves or contractures were observed. No clear correlations were found between the nature and site of the mutation and the resulting phenotype.

Adult↗