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Biomedical subjects

M Lima

Publications and source records attributed to M Lima.

At least 109 records · Page 6Linked to original sources

Immunophenotypic characterization of normal blood CD56+lo versus CD56+hi NK-cell subsets and its impact on the understanding of their tissue distribution and functional properties.

In the present study we have compared the immunophenotypic characteristics of the CD56+lo and CD56+hi NK-cell subsets in a group of normal healthy adults. Our results show that CD56+hi NK-cells display greater light-scatter properties than CD56+lo NK-cells at the same time they have higher levels of CD25 and CD122 IL-2 chains, together with a higher reactivity for HLA-DR and CD45RO and lower levels of CD45RA, supporting that, as opposed to the majority of the CD56+lo population, CD56+hi NK-cells might correspond to a subset of activated circulating NK-lymphocytes. Higher expression of the CD2 and CD7 costimulatory molecules found for the CD56+hi NK-cells would support their greater ability to respond to various stimuli. In addition, CD56+hi NK-cells expressed higher levels of several adhesion molecules such as CD2, CD11c, CD44, CD56, and CD62L compared to CD56+lo NK-cells, supporting a particular ability of these cells to migrate from blood to tissues and/or a potential advantage to form conjugates with target cells. Interestingly, CD56+lo and CD56+hi NK-cells showed a different pattern of expression of killer receptors that might determine different activation requirements for each of these NK-cell subsets. For instance, absence or low levels of CD16 expression might explain the lower antibody-dependent cytotoxicity activity of CD56+hi NK-cells. On the other hand, the virtual absence of expression of the CD158a and NKB1 immunoglobulin-like and the greater reactivity for the CD94 lectin-like killer receptors on CD56+hi in comparison to CD56+lo NK-cells might determine different MHC-class I specificities for both NK-cell subsets, a possibility that deserves further studies to be confirmed.

Adult↗

Cardiac hydatid cyst in a child.

Cardiac hydatid cyst is a rare disease, especially in children. An 11-year-old boy with a previous anaphylactic reaction and episodes of abdominal pain was admitted for workup of an acquired long systolic murmur. Echocardiographic investigation disclosed a tumor of the right ventricular anterior wall, with multiple loculations. Magnetic resonance imaging characterized it as a multilobular tumor with cyst formation and disclosed another cyst in the right pulmonary artery. With a positive ELISA reaction the child was admitted for surgery with the diagnosis of cardiac and pulmonary hydatid cysts. Cardiac surgery was performed with good results, followed by medical treatment with albendazole.

Albendazole↗

Aggressive natural-killer cell lymphoma presenting with skin lesions, breast nodule, suprarenal masses and life-threatening pericardial and pleural effusions.

We report the clinical and laboratory findings of a patient with an aggressive Epstein-Barr virus positive CD2+/CD56+ natural killer-cell lymphoma with a high mitotic activity and complex chromosomal abnormalities presenting with life-threatening pericardial and pleural effusions, disseminated skin lesions, breast nodule and large suprarenal masses. The clinical course was characterized by resistance to chemotherapy and relapsing pericardial and pleural effusions with respiratory and haemodynamic failure. Death occurred 4 months after the first manifestations of the disease as a consequence of cardiac tamponade.

Adult↗

Cutaneous necrobiotic xanthogranuloma (NXG)--successfully treated with low dose chlorambucil.

We report a case of necrobiotic xanthogranuloma in a 51 year-old white male patient presenting with a 6-year history of multiple indurated violaceous nodules and plaques involving the eyelids, trunk and extremities. He had an associated paraproteinemia (Ig G lambda), elevated sedimentation rate, cryoglobulinemia and hypocomplementemia. No extracutaneous involvement was detected. He was successfully treated with chlorambucil (2 mg/d for 7 months), leading to disappearance of all skin lesions.

Adult↗

The nonpalpable testis: an experience of 132 consecutive videolaparoscopic explorations in 6 years.

From may 1995 to may 2001, 114 children with nonpalpable testis (NPT) were evaluated at our institution (18 babies had bilateral cryptorchidism). The age range was 1-11 years. When ultrasonography and nuclear magnetic resonance cannot show the position of the NPT along the normal pathway, video laparoscopy is essential for diagnostic accuracy. 20 cases were observed to have blind-ending was deferens and testicular vessels; therefore, no other procedure was done. 4 had residual nonfunctional intra-abdominal tissue; in 52 cases, an intra-abdominal testis was found, and 17 microvascular and 26 traditional orchidopexies were performed. The remaining 50 patients (six with bilateral cryptorchidism) had normal vas and spermatic vessels entering the inguinal canal. In 15 cases a normal testis was present, and it was positioned into the scrotum with the standard technique; in 41 cases an atrophic testis was found and was removed through an inguinal approach. Diagnostic laparoscopy permits not only localisation of the testis but also planning for a better therapeutic program with a minimally invasive procedure, thus avoiding the knife in 18% of cases (in our experience 15% of blind-ending and 3% of abdominal vanishing testis).

Algorithms↗

The role of endoscopic surgery in paediatric oncological diseases.

Recent improvements in video imaging and instrumentation have encouraged a wider use of endoscopic surgery as a modality for diagnostic and operative procedures. To asses the utility and diagnostic accuracy of endoscopic surgery in children with oncological diseases, we reviewed our experience about thirty-eight patients affected by oncological diseases, referred to our Department since 1995. We performed laparoscopy in 22 cases, thoracoscopy in 14, 1 combinated procedure (laparoscopy + thoracoscopy) and retroperitoneoscopy in 1 case. Endoscopic surgery is indicated in cases of paediatric oncological diseases both for diagnosis and treatment.

Adolescent↗

[Prophylactic total thyroidectomy in children and adolescents with genetic mutations in the RET-protooncogene].

Medullary thyroid cancer (C.M.T.) can be a sporadic form generally in adults or a heredofamilial form where the first symptom appears in pediatric and adolescent age. The hereditary form can be isolated or associated with others endocrine neoplasias of type 2: MEN2a (with or without cutaneous lichen amyloidosis) and MEN2b. The responsible gene of the transmission has been identified in proto-oncogene RET localized on chromosome 10. Point form mutations of this proto-oncogene have been found on exons 10 and 11 in MEN2a and on 16 in MEN2b. In our study on 64 subjects, who belong 11 familiar groups, affected by MEN2a, MEN2b and familiar C.M.T., underwent a genetic research to look for point form mutations of proto-oncogene RET with PCR followed by the analysis of restriction. A genetic mutation has been revealed in 25 subjects: 18 were already known affected by MEN2 and so surgical treated and 7 seemed healthy (mean age 17.4 years, range 10-25). These 7 patients has been undergone clinical research and surgical treatment: a total thyroidectomy associated a lymphectomy of the central compartment. In all cases the histological exam showed C.M.T. moreover a patient had metastasis in lymph nodes of the central compartment. Another had hyperparathyroidism and pheochromocytoma treated with total thyroidectomy, parathyroidectomy and bilateral laparoscopic adrenalectomy. The identification in a very early age of carrier subjects of hill's gene inside an affected family, permits the execution of a prophylactic total thyroidectomy to prevent the C.M.T.. The penetrance of this neoplasia in hereditary form is 100%.

Adolescent↗

Bronchoscopy in newborns with esophageal atresia.

The aim was to evaluate the influence of rigid laryngotracheo-bronchoscopy in newborns with oesophageal atresia (OA) and tracheoesophageal fistula (TOF) in preventing complications, improving diagnosis and surgical treatment. Among 76 consecutive newborns, received in the Paediatric Intensive Care Unit (PICU) from January 1990 to September 2001 with prenatal o perinatal suspected OA, forty-five had one or more other congenital anomalies. They were divided in I Montreal's risk group for 73.7% (56) and in the II one for 26.3% (20). Endoscopic procedure was performed in the operating room with Storz's rigid ventilating bronchoscope just before surgery, in all babies. With bronchoscopic examination we were able to identify the level, number and size of TOF and to visualise anatomical variants in 76 children. 15 children with OA had a gasless abdomen, but an upper pouch fistula was found only in three cases. In another four cases bronchoscopy confirmed the diagnosis of an "H" fistula and cervical surgical approach was established. One case had only oesophageal stenosis. 56 patients had fistula in lower pouch and in the last 38 cases we proceeded with selective transtracheal fistula incannulation and then we provided gastric drainage. One quadriforcation, 2 triforcations, 3 aberrant right upper lobe bronchi, 1 congential subglottic stenosis and 1 associated with a congenital subglottic stenosis and 1 left main bronchus agenesia were detected. No complications were correlated to the procedure and no babies had early pneumonia. Continuous feeding was achieved in 70 out of 76 patients: 46 primary anastomosis with 12 staged repairs (OA I and II type long gap), 4 resections of TEF, 8 oesophagocoloplasty. Nine babies (11.8%) did not survive. Clinical follow-up was possible in all the survivors. Mortality rate in the high-risk patients with OA remains high because of the many complications that may occur. Tracheal endoscopy is useful in improving the diagnostic approach and to prevent pulmonary complications. Fistula incannulation minimizes the risk of gastric distension and its detrimental effect on ventilation, helping the surgeon to identify TEF easily and quickly. Moreover, the endoscopic approach offers obvious advantages for the anaesthesiologist and the surgeon and has proven, in our experience, to be effective.

Bronchoscopy↗

[Malignant primary tumors of the liver in childhood].

Twenty-one children (16 males, 5 females) with malignant primary hepatic tumors were admitted to the Pediatric Clinic of the University of Bologna between June 1973 and July 2001. The diagnosis was hepatoblastoma (HBL) in 16 cases; hepatocellular carcinoma (HCA) in 3 cases; undifferentiated sarcoma in 1, malignant rhabdoid tumour of the liver in 1. Median age at diagnosis was 1.8 year (1 mounth-13 years). As to intrahepatic tumor's extension, patients were classified in groups (from I to IV) according to International Society of Pediatric Oncology staging. 2 patients were ascribed to group I; 9 to group II; 9 to group III and I to group IV. At diagnosis 3 pts presented lung metastases. Seventeen patients (81%) were treated with surgery, in 11 cases as first approach to the tumor. In 10 patients, initially with unresesectable tumor, chemotherapy was started first. Drugs used were mostly Cisplatinum or Carboplatinum with Doxorubicin. Sussequently 6 patients were submitted to surgery. At a median follow up of 12.5 years, 52.3% of patients is alive without disease. This percentage rises to 58% taking into consideration only HBL and HCA cases (alive 11/19). We conclude that excluding metastases at diagnosis (3 deaths), the main prognostic factor is resectability and radical surgery: in our experience 4 patients with unresectable tumor died, as 2 patients with microscopical residual after surgery.

Algorithms↗

[Video-assistive minimally invasive surgical procedures in the treatment of thyroid in children and adolescents].

Minimally invasive video-assisted (MIVA) surgery of the thyroid is now widely accepted by endocrinel surgeons, especially in adult age. This report describes indications, limits, technical aspects and results of MIVA in 10 young patients (mean age 16 years, range 11-18) enrolled in a study of 213 patients (mean age 45 years, range 11-81) operated with this technique, from October 1998 to August 2001, in the Department of Surgery at the University of Pisa. Young patients were submitted to surgery: 8 lobectomies (6 for microfollicular lesions and 2 for adenomas functionally autonomous) and 2 total thyroidectomies for papillary carcinoma. No case has been converted in "open" surgery and no post-operatory complications have been observed. Elective indications of MIVA are the volume of the nodule and the istological type. This technique cannot be performed in voluminous goiters, in medullary carcinomas and in scarcely differentiated carcinomas. Minimally invasive video-assisted surgery of the thyroid, in our experience, represents a reliable technique also in pediatric age.

Carcinoma, Papillary↗

Retroperitoneoscopic adrenalectomy in a residual of neuroblastoma.

The authors report a case of retroperitoneoscopic adrenalectomy in a child with neuroblastoma previously treated by chemotherapy. Usually the presence of malignant tumours is a contraindication to retroperitoneoscopic surgery. A restaging of the tumor was done by retroperitoneoscopy. The residual adrenal gland was excised by mini-invasive surgery of the retroperitoneal space. The histopathologic examination confirmed the presence of a residual neuroblastoma in the adrenal gland. This case report shows a possible of a new indication for the retroperitoneoscopy.

Adrenalectomy↗

Graves-Basedow's disease in children and adolescents: total vs subtotal thyroidectomy.

"Graves' disease" is an autoimmune pathology and is the most important cause of hyperthyroidism in children and adolescents. The treatment for this disease is controversial; there are three different options: synthetic antithyroid drugs, ablation of the thyroid gland with I 131 or surgery. Our study reports the clinical and therapeutic aspects and long-term results of 27 patients (< = 18 years old) treated with total thyroidectomy or "near-total" thyroidectomy from 1975 to August 2001. Antithyroid drugs represent the preliminary treatment of choice. For a long time in the past therapy with radioactive iodine was not recommended; now it represents an efficiently therapy with low morbility. Surgical treatment is suitable when a young patient presents a considerable enlargement of the thyroid gland or when antithyroid medical treatment does not achieve a good control of the disease, or when rapid control of hyperthyroidism is necessary. The surgical treatment can be a total thyroidectomy or a "near-total" thyroidectomy. The first is the best choice for us, because it has a low risk of relapse and a major efficacy in the ophthalmopathy.

Adolescent↗

Gastric perforation in a newborn.

Gastric perforation in neonates is an uncommon condition. In most cases, it is attributed to peptic ulceration and/or hemorrhagic gastritis. The high mortality rate in such patients can be improved by early diagnosis and prompt resuscitation, followed by surgery. We report a full-term female newborn, who developed a gastric perforation in the first day of life. The possible aetiology and the perioperative management are discussed.

Digestive System Surgical Procedures↗

Agenesis of the appendix vermiformis.

Agenesis of the appendix vermiformis represents a rare condition that accounts in approximately 1 in 100.000 laparotomies performed for suspected acute appendicitis. L.B., female, born at term after a normal pregnancy. Shortly after birth she presented respiratory distress; chest x-ray showed a left diaphragmatic hernia (CDH). At operation was noted the absence of the appendix and of the mesenteriolum as well together with the presence of a mesenterium commune. Other associated anomalies were detected: dextroposition of the heart, hypoplasia cnemis, hexadactylism of right foot, congenital hip displacement and bilateral congenital cataract. Etiopathogenesis of the agenesis of the appendix can be easily understood from an embryological point of view, following the cecal pole development. Diagnosis of this malformation is possible only after an accurate laparotomic or laparoscopic exploration around the ileocecal and retrocecal zone.

Appendix↗

[Urethro-vesical-vas deferens reflux as a cause of acute scrotum].

The Authors reviewed 159 children who underwent surgical therapy for "acute scrotum", and they consider that the torsion of the appendix testis is the most common cause of the syndrome in children. On the other hand the orchi-epididymitis is very rare in paediatric age. About the incidence and etiopathogenesis of orchi-epididymitis is reported the revision of the literature; in particular is described the condition of the urethro-vesical-deferential reflux causing a chemical or infective orchi-epididymitis.

Acute Disease↗