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1.
Colorectal Dis ; 22(5): 488-499, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-31400185

RESUMO

AIM: Minimally invasive surgical approaches for cancer of the right colon have been well described with significant patient and equivalent oncological benefits. Robotic surgery has advanced in its ability to provide multi-quadrant abdominal access, leading the surgical community to widen its application outside of the pelvis to other abdominal compartments. Globally it is being realized that a patient's surgical episode of care is becoming the epicentre of cancer treatment. In order to establish the role of robotic surgery in a patient's episode of care, 'successful patient-oriented surgical' parameters in right hemicolectomy for malignancy were measured. The objective was to examine the rates of successful patient-oriented surgical outcomes in robotic right hemicolectomy (RRH) compared to laparoscopic right hemicolectomy (LRH) for cancer. METHODS: A systematic search of MEDLINE (Ovid: 1946-present), PubMed (NCBI), Embase (Ovid: 1966-present) and Cochrane Library was conducted using PRISMA for parameters of successful patient-oriented surgical outcomes in RRH and LRH for malignancy alone. The parameters measured included postoperative ileus, anastomotic complication, surgical wound infection, length of stay (LOS), incisional hernia rate, conversion to open, margin status, lymph node harvest and overall morbidity and mortality. RESULTS: There were 15 studies which included 831 RRH patients and 3241 LRH patients, with a median age of 62-74 years. No study analysed the concept of successful patient-oriented surgical outcomes. There was no significant difference in the incidence of postoperative ileus, with less time to first flatus in RRH (2.0-2.7 days, compared with 2.5-4.0 days, P < 0.05). Anastomotic leak rate in one study reported a significant increase in LRH compared to RRH (P < 0.05, 0% vs 8.3%). Significantly decreased LOS following RRH was outlined in six studies. One study reported a significantly higher rate of incisional hernias following LRH with extracorporeal anastomoses compared to RRH with intracorporeal anastomoses. Overall rates of conversion to open surgery were less with RRH (0%-3.9% vs 0%-18%, P < 0.001, 0.05). One study outlined significantly higher rates of incomplete resection with an open right hemicolectomy compared with minimally invasive laparoscopic and robotic resections, with positive margin rates of 2.3%, 0.9% and 0% respectively (P < 0.001). Two studies reported significantly higher lymph node harvest in RRH (P < 0.05). Overall morbidity and 30-day mortality were comparable in both approaches. CONCLUSION: Thirty-day morbidity and mortality were comparable between the two approaches, with patients undergoing RRH having lower anastomotic complications, increased lymph node harvest, and reduced LOS, conversion to open and incisional hernia rates in a number of studies. There are limited data on surgical approach and impact on quality of life and what patients deem successful surgical outcomes. There is a further need for a randomized controlled trial examining successful patient-oriented outcomes in right hemicolectomy for malignancy.


Assuntos
Laparoscopia , Procedimentos Cirúrgicos Robóticos , Neoplasias do Colo do Útero , Colectomia , Feminino , Humanos , Recém-Nascido , Tempo de Internação , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Qualidade de Vida , Procedimentos Cirúrgicos Robóticos/efeitos adversos , Resultado do Tratamento
3.
Clin Radiol ; 59(11): 977-83, 2004 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-15488845

RESUMO

Colonic diverticulitis occurs when diverticula within the colon become infected or inflamed. It is becoming an increasingly common cause for hospital admission, particularly in western society, where it is linked to a low fibre diet. Symptoms of diverticulitis include abdominal pain, diarrhoea and pyrexia, however, symptoms are often non-specific and the clinical diagnosis may be difficult. In addition, elderly patients and those taking corticosteroids may have limited findings on physical examination, even in the presence of severe diverticulitis. A high index of suspicion is required in such patients in order to avoid a significant delay in arriving at the correct diagnosis. Imaging plays an important role in establishing an early and correct diagnosis. In the past, contrast enema studies were the principal imaging test used to make the diagnosis. However, such studies lack sensitivity and have limited success in identifying abscesses that may require drainage. Conversely computed tomography (CT) is both sensitive and specific in making a diagnosis of diverticulitis. In addition, it is the imaging technique of choice in depicting complications such as perforation, abscess formation and fistulae. CT-guided drainage of diverticular abscesses helps to reduce sepsis and to permit a one-stage, rather than two-stage, surgical operation. The purpose of this review article is to discuss the role of CT in the imaging of diverticulitis, describe the CT imaging features and complications of this disease, as well as review the impact and rationale of CT imaging and intervention in the overall management of patients with diverticulitis.


Assuntos
Colonografia Tomográfica Computadorizada/métodos , Doença Diverticular do Colo/diagnóstico por imagem , Doença Aguda , Diagnóstico Diferencial , Humanos
4.
Br J Surg ; 89(4): 428-32, 2002 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-11952582

RESUMO

BACKGROUND: Haemorrhoidectomy is frequently associated with postoperative pain and prolonged hospital stay. A new technique of haemorrhoidectomy using the Ligasure device suited to day-case surgery is described. This technique was compared with conventional open diathermy haemorrhoidectomy. METHODS: Forty patients with grade III or IV haemorrhoids were randomized to Ligasure (group 1) or conventional diathermy (group 2) haemorrhoidectomy. Operative details were recorded and patients recorded daily pain scores on a linear analogue scale. Follow-up was at 1, 3, 6 and 12 weeks to evaluate complications, return to normal activity, ongoing symptoms and patient satisfaction. RESULTS: Reduced intraoperative blood loss (median (range) 0 (0-5) ml versus 20 (12-22) ml; P < 0.001) and a shorter operating time (10 (8-11) versus 20 (18-25) min; P < 0.001) was observed in group 1 compared with group 2. More patients in group 1 were discharged on the day of operation (18 of 20 versus 11 of 20; P < 0.05) and there was a trend towards lower postoperative pain scores on day 1 (group 1 median 5 (95 per cent confidence interval (c.i.) 2.6 to 6.8) versus group 2 7 (95 per cent c.i. 4.2 to 7.7); P = 0.36). There was no difference between the two groups in the degree of patient satisfaction or number of postoperative complications. CONCLUSION: Ligasure diathermy may be used safely in the treatment of patients with grade III or IV haemorrhoids. It reduces intraoperative blood loss and operating time, and facilitates same-day discharge.


Assuntos
Procedimentos Cirúrgicos Ambulatórios/métodos , Eletrocoagulação/métodos , Hemorroidas/cirurgia , Adulto , Idoso , Incontinência Fecal/etiologia , Feminino , Fissura Anal/etiologia , Humanos , Cuidados Intraoperatórios/métodos , Tempo de Internação , Ligadura/métodos , Masculino , Pessoa de Meia-Idade , Dor Pós-Operatória/etiologia , Satisfação do Paciente , Cuidados Pré-Operatórios/métodos , Fatores de Tempo , Retenção Urinária/etiologia
5.
Br J Surg ; 88(10): 1352-6, 2001 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-11578291

RESUMO

BACKGROUND: Excision of primary colorectal cancer associated with irresectable synchronous metastases confers high morbidity and mortality with uncertain benefit. METHODS: For patients with incurable stage IV colorectal cancer, minimally symptomatic primary tumours were left in situ and 5-fluorouracil-based chemotherapy was administered systemically. Primary tumour-specific complications and survival were monitored. RESULTS: There were 13 men and 11 women with primary tumours in the right colon (eight), transverse colon (one), sigmoid colon (eight) or rectum (seven). Eleven patients had metastases limited to the liver (liver replacement less than 25 per cent in one, 25-50 per cent in four and more than 50 per cent in six) and 13 patients had extrahepatic disease (lung or peritoneum). Four patients with sigmoid colon tumours developed bowel obstruction, which required an uncomplicated operation in two and deployment of colonic stents in two patients, at 1, 3, 12 and 20 months from diagnosis. Three further patients underwent right hemicolectomy for abdominal pain of uncertain aetiology, with poor symptomatic relief, and another had a potentially curative operation following disease downstaging. The overall median survival was 10.3 months with a 1-year actuarial survival rate of 44 per cent. CONCLUSION: A policy to defer resection of minimally symptomatic primary colorectal cancer is associated with a low risk of complications before death from progressive systemic disease.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Colorretais/tratamento farmacológico , Dor Abdominal/etiologia , Adulto , Camptotecina/administração & dosagem , Camptotecina/análogos & derivados , Neoplasias Colorretais/diagnóstico por imagem , Neoplasias Colorretais/patologia , Feminino , Fluoruracila/administração & dosagem , Humanos , Obstrução Intestinal/etiologia , Irinotecano , Neoplasias Hepáticas/secundário , Neoplasias Pulmonares/secundário , Masculino , Pessoa de Meia-Idade , Compostos Organoplatínicos/administração & dosagem , Oxaliplatina , Estudos Prospectivos , Análise de Sobrevida , Tomografia Computadorizada por Raios X
7.
Colorectal Dis ; 2(6): 346-50, 2000 Nov 17.
Artigo em Inglês | MEDLINE | ID: mdl-23578153

RESUMO

OBJECTIVE: The hepatic perfusion index (HPI) is a ratio of the gradient of hepatic arterial to total hepatic blood flow. This study correlated HPI with histopathological indicators of prognosis and disease-free survival following curative resection of colorectal cancer. PATIENTS AND METHODS: HPI was measured preoperatively by dynamic hepatic scintigraphy in 37 patients with a primary colorectal cancer and no evidence of distant metastases who underwent a curative resection. RESULTS: Abnormally elevated HPI were detected in 49% of patients and were significantly more frequent in association with locally advanced tumours (T3 and T4) in comparison with early tumours (T1 and T2; 59% vs 20%, respectively; P=0.04). There was no association between abnormal HPI and presence of lymph node metastases or degree of tumour differentiation. The 18-month disease-free survival rate of patients with abnormal HPI was significantly shorter than that of patients with normal HPI (53% vs 100%, respectively; P=0.01), and this was independent of the T category. CONCLUSION: HPI predicts the risk of recurrent colorectal carcinoma, and this measurement should be included in the panel of prognostic markers in future clinical trials.

8.
Dis Colon Rectum ; 42(9): 1216-9, 1999 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-10496565

RESUMO

Multiple endocrine neoplasia type 2B is best known for its endocrine manifestations and typical phenotype. The gastrointestinal manifestations, however, are also an important and commonly unrecognized component of the syndrome. We present four cases that demonstrate the varied presentation of patients with colonic manifestations of multiple endocrine neoplasia type 2B. We discuss the cause, diagnostic significance, and management of the colonic disease that is a component of multiple endocrine neoplasia type 2B.


Assuntos
Colo/patologia , Doenças do Colo/etiologia , Ganglioneuroma/complicações , Neoplasia Endócrina Múltipla Tipo 2b/complicações , Feocromocitoma/complicações , Adolescente , Adulto , Pseudo-Obstrução do Colo/etiologia , Feminino , Humanos , Hiperplasia , Masculino , Megacolo/etiologia
9.
Surg Endosc ; 13(9): 914-7, 1999 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-10449852

RESUMO

BACKGROUND: Totally extraperitoneal (TEP) laparoscopic inguinal hernia repair is gaining popularity, and our preference is to perform this procedure as a day case. This study evaluates the suitability of TEP repair in the day-care setting. METHODS: A policy of day-care TEP repair, unless contraindicated, was adopted for inguinal hernia repair, and the outcome was prospectively evaluated. Of 87 consecutive inguinal hernia repairs, day-care TEP was possible in 54 (62%); 17 (20%) were in-patient TEP, 14 (16%) were open repairs, and 2 (2%) were converted from TEP to open repairs. RESULTS: Among day-care TEP repairs, median visual analog pain score at discharge was 2.3/10, and 43% of patients had no pain. Complications included cord hematoma 2 (4%) and seroma 3 (6%). Median times for stopping analgesia, resumption of full activity, and return to work were 3, 3, and 6 days respectively. Complete satisfaction with day-care TEP was expressed by 91% of patients; 9% were moderately satisfied, and none expressed dissatisfaction. CONCLUSIONS: Day-care TEP repair is feasible in the majority of patients with inguinal hernias, and it is associated with minimal complications, excellent recovery, and a high degree of patient satisfaction.


Assuntos
Procedimentos Cirúrgicos Ambulatórios , Hérnia Inguinal/cirurgia , Laparoscopia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Humanos , Pessoa de Meia-Idade , Dor Pós-Operatória , Satisfação do Paciente , Complicações Pós-Operatórias , Estudos Prospectivos , Recuperação de Função Fisiológica , Recidiva
10.
Ir J Med Sci ; 167(4): 221-4, 1998.
Artigo em Inglês | MEDLINE | ID: mdl-9868858

RESUMO

Ninety-six consecutive above-knee femoropopliteal bypasses, using polytetrafluoroethylene (PTFE) preferentially, were performed for limb-threatening ischaemia. Cumulative primary graft patency was 68 per cent, 49 per cent and 36 per cent and limb salvage 93 per cent, 85 per cent and 75 per cent at 1, 3 and 5 yr respectively. As a result of poor long term survival (51 per cent at 5 yr), and the healing of remedial lesions before graft occlusion, 68 patients (72 per cent) required no further intervention. Eighteen secondary bypasses were undertaken, 12 using ipsilateral saphenous vein. In this group of elderly patients with poor life expectancy, where a limited operation is desirable, the use of PTFE provided excellent limb salvage with low morbidity. Although we can no longer justify our continued use of PTFE in every case of critical ischaemia because of its inferior patency to autogenous vein, we continue to use it preferentially in patients whose lifespan is likely to be short and in cases where a remedial lesion is present.


Assuntos
Implante de Prótese Vascular , Isquemia/cirurgia , Perna (Membro)/irrigação sanguínea , Politetrafluoretileno , Idoso , Feminino , Humanos , Masculino , Resultado do Tratamento , Grau de Desobstrução Vascular
11.
Am J Surg ; 176(3): 254-7, 1998 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-9776153

RESUMO

BACKGROUND: The limited space developed in totally extraperitoneal laparoscopic inguinal hernia repair (TEP) provides the ideal setting for direct instillation of local anesthetic. This study evaluates the efficacy of extraperitoneal bupivacaine analgesia in patients undergoing day-care TEP. METHODS: Fifty-six consecutive patients were randomized to intraoperative extraperitoneal instillation of bupivacaine (n = 29) or normal saline control (n = 27). Patients were blindly assessed on discharge from hospital, at 24 hours, 1 week, and 1 month postoperatively. RESULTS: Compared with controls, patients treated with bupivacaine had lower median (range) visual analogue pain scores on discharge (1.5 [0 to 5.9] versus 3.7 [0.2 to 6.9], P = 0.03), and were more frequently pain free (54% versus 31%, P = 0.078). Although this difference had gone by 24 hours, the bupivacaine group continued to recover faster; stopping analgesia earlier (2 [0 to 7] days versus 3 [0 to 21] days, P = 0.01) and returning to full activity earlier (2.5 [1 to 14] days versus 5 [1 to 21] days, P = 0.013). Of bupivacaine patients 100% were completely satisfied with the procedure compared with 81% of controls (P = 0.02). CONCLUSION: Extraperitoneal bupivacaine minimizes pain following day-care TEP repair, facilitates recovery, and increases patient satisfaction. Benefits persist beyond the pharmacological action of bupivacaine.


Assuntos
Analgesia/métodos , Anestésicos Locais , Bupivacaína , Hérnia Inguinal/cirurgia , Laparoscopia/métodos , Adolescente , Adulto , Idoso , Analgesia/estatística & dados numéricos , Anestesia Geral/métodos , Anestésicos Locais/administração & dosagem , Bupivacaína/administração & dosagem , Hospital Dia/métodos , Humanos , Laparoscopia/estatística & dados numéricos , Masculino , Pessoa de Meia-Idade , Recidiva , Estatísticas não Paramétricas
12.
Int J Colorectal Dis ; 13(3): 116-8, 1998.
Artigo em Inglês | MEDLINE | ID: mdl-9689560

RESUMO

Hysterectomy is associated with severe constipation in a subgroup of patients, and an adverse effect on colonic motility has been described in the literature. The onset of irritable bowel syndrome and urinary bladder dysfunction has also been reported after hysterectomy. In this prospective study, we investigated the effect of simple hysterectomy on ano-rectal physiology and bowel function. Thirty consecutive patients were assessed before and 16 weeks after operation. An abdominal hysterectomy was performed in 16 patients, and a vaginal procedure was performed in 14. The parameters measured included the mean resting, and maximal forced voluntary contraction anal pressures, the recto-anal inhibitory reflex, and rectal sensation to distension. In 8 patients, the terminal motor latency of the pudendal nerve was assessed bilaterally. Pre-operatively, 8 patients were constipated. This improved following hysterectomy in 4, worsened in 2, and was unchanged in 2. Symptomatology did not correlate with changes in manometry. Although, the mean resting pressure was reduced after hysterectomy (57 mmHg-53 mmHg, P = 0.0541), the maximal forced voluntary contraction pressure was significantly decreased (115 mmHg-105 mmHg, P = 0.029). This effect was more pronounced in those with five or more previous vaginal deliveries (P = 0.0244, n = 9). There was no significant change in the number of patients with an intact ano-rectal inhibitory reflex after hysterectomy. There was no change in rectal sensation to distension, and the right and left pudendal nerve terminal motor latencies were unaltered at follow-up. Our results demonstrate that hysterectomy causes a decrease in the maximal forced voluntary contraction and pressure, and this appears to be due to a large decrease in a small group of patients with previous multiple vaginal deliveries.


Assuntos
Canal Anal/fisiologia , Histerectomia , Reto/fisiologia , Adulto , Feminino , Humanos , Histerectomia Vaginal , Manometria , Pessoa de Meia-Idade , Paridade , Estudos Prospectivos
13.
Ir J Med Sci ; 167(1): 41-2, 1998.
Artigo em Inglês | MEDLINE | ID: mdl-9540300

RESUMO

The purpose of this case presentation is to illustrate the rate association between acute pancreatitis and colonic pseudo-obstruction and to highlight the difficulties of assessing intestinal motility in a defunctioned segment of bowel prior to closure of a defunctioning stoma.


Assuntos
Pseudo-Obstrução do Colo/etiologia , Pancreatite Alcoólica/complicações , Doença Aguda , Adulto , Pseudo-Obstrução do Colo/diagnóstico por imagem , Motilidade Gastrointestinal , Humanos , Masculino , Radiografia
15.
J Pediatr Surg ; 32(12): 1809-11, 1997 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-9434037

RESUMO

Achalasia of the esophagus developed in two male siblings soon after birth, and they were successfully treated by surgery. Persistent signs resulted in the later diagnosis of Hirschsprung's disease. One required subtotal colectomy and ileoanal anastomosis, and the other is managing well on conservative treatment. Genetic analysis of the genes encoding the RET protooncogene, endothelin-3, and the endothelin-3 receptor did not show any defect. Familial achalasia of the esophagus in combination with Hirschsprung's disease has never been reported.


Assuntos
Acalasia Esofágica/complicações , Acalasia Esofágica/genética , Doença de Hirschsprung/complicações , Doença de Hirschsprung/genética , Colectomia , Acalasia Esofágica/cirurgia , Fundoplicatura , Doença de Hirschsprung/cirurgia , Humanos , Lactente , Masculino , Proctocolectomia Restauradora
16.
World J Surg ; 20(7): 916-21; discussion 922, 1996 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-8678971

RESUMO

Functioning extraadrenal paragangliomas represent more than 10% of all pheochromocytomas, occur at diverse anatomic locations, and are said to have a higher malignancy rate than intraadrenal pheochromocytomas. Sixty-six patients had surgery for catecholamine-producing paragangliomas between 1952 and 1992. Median follow-up was 8.8 years. Median age was 40 years (11-67 years); the male/female ratio was 29:37. Familial disease occurred in 9 patients (13.6%), and 10 patients (15.2%) also developed adrenal pheochromocytoma. Solitary paragangliomas occurred in 52 patients: 46 abdominal, 4 thoracic, and 2 head and neck. Fifty-three tumors developed in 14 patients with multiple paragangliomas: 38 abdominal and 15 thoracic. Of 28 patients with solitary tumors undergoing localization studies over the past 10 years accurate localization was achieved in 27. There was one operative death; 15 patients had persistent disease; and 50 were cured postoperatively. Of those cured, nine developed recurrence, disease-free survival being 86%, 80%, and 80% at 5, 10, and 20 years. Metastatic disease was found in 14 patients (21%), 7 of whom have died. An additional 10 patients (15%) had locally invasive disease, of whom 4 have died. Cause-specific survivals at 5, 10, and 20 years were 90%, 83%, and 72%. Risk factors for death from pheochromocytoma were tumor size > 5 cm (p = 0.0002), metastatic disease (p = 0.001), and tumor invasion (p = 0.0023). Cause-specific survival for patients with tumors > 5 cm was 59% at 15 years compared to 100% among patients with tumors 5 cm and the occurrence of invasive or metastatic disease are strong predictors of outcome. Most tumors are abdominal, and imaging is highly successful for localization.


Assuntos
Paraganglioma/cirurgia , Neoplasias Abdominais/cirurgia , Adolescente , Neoplasias das Glândulas Suprarrenais/cirurgia , Adulto , Idoso , Catecolaminas/metabolismo , Criança , Intervalo Livre de Doença , Feminino , Seguimentos , Previsões , Neoplasias de Cabeça e Pescoço/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Invasividade Neoplásica , Recidiva Local de Neoplasia/patologia , Paraganglioma/genética , Paraganglioma/metabolismo , Paraganglioma/patologia , Paraganglioma/secundário , Feocromocitoma/cirurgia , Indução de Remissão , Fatores de Risco , Taxa de Sobrevida , Neoplasias Torácicas/cirurgia , Resultado do Tratamento
17.
J Surg Res ; 64(1): 95-101, 1996 Jul 15.
Artigo em Inglês | MEDLINE | ID: mdl-8806480

RESUMO

Antibody against tumor necrosis factor-alpha (TNF-alpha) has improved survival in certain models of sepsis, but it remains unproven in clinical studies. In most of the successful animal studies, efficacy has been shown in previously healthy animals subjected to a septic challenge. Patients at risk for sepsis, however, may be ill for some time before the sepsis supervenes. This situation has been described as a "two-hit" model of critical illness. We have developed an animal burn-sepsis model which conforms to this "two-hit" concept. We have quantified macrophage TNF-alpha production at different times after the burn (first "hit") and determined the effect of neutralizing antibody against TNF-alpha during this period on survival after subsequent sepsis (second "hit"). The objective of this study was to determine the role of TNF-alpha and the effect of neutralizing antibody against TNF-alpha in a burn-sepsis model. Animals were subjected to a full thickness burn or sham burn. In vitro TNF-alpha production from cultured lipopolysaccharide-stimulated splenic adherent cells was determined at various time points thereafter by enzyme-linked immunosorbent assay. Separate animals were treated with neutralizing antibody against TNF-alpha at different time points after the thermal injury, and survival was determined after septic challenge (cecal ligation and puncture) on Day 10 after the burn. TNF-alpha production from adherent splenocytes was not elevated in the early days after thermal injury, but was significantly enhanced from Day 6 onward compared with sham-burned animals. Nine percent of the burned mice survived septic challenge compared with 69% of the sham-burned control mice (P < 0.001). Therapy with anti-TNF antibody at 1 x 10(4) neutralizing units (n.u.) kg-1 markedly improved outcome if given when TNF-alpha production was elevated at Day 7 after the burn (survival, 36%; P = 0.01) but did not improve survival when administered at Days 0 or 4 or at the time of the septic challenge (Day 10). High doses of antibody (3.2 x 10(5) n.u.kg-1) were not beneficial and may have been detrimental. These results show that neutralizing antibody against TNF-alpha may reduce the susceptibility to infection seen after thermal injury, but the timing of administration of the antibody and the dose of antibody used are critical to the outcome. This should be considered when neutralizing antibody against TNF is used in the clinical setting.


Assuntos
Anticorpos/uso terapêutico , Queimaduras/complicações , Infecções/complicações , Fator de Necrose Tumoral alfa/imunologia , Animais , Queimaduras/metabolismo , Queimaduras/terapia , Adesão Celular , Citocinas/metabolismo , Citocinas/fisiologia , Suscetibilidade a Doenças , Relação Dose-Resposta a Droga , Imunoterapia , Infecções/mortalidade , Infecções/terapia , Mediadores da Inflamação/metabolismo , Macrófagos/metabolismo , Macrófagos/fisiologia , Masculino , Camundongos , Camundongos Endogâmicos , Análise de Sobrevida , Fatores de Tempo
19.
Surgery ; 118(6): 936-42, 1995 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-7491537

RESUMO

BACKGROUND: Multiple endocrine neoplasia type 2B (MEN 2B) is a rare disorder differentiated from MEN 2A primarily by its extraendocrine features. This report describes the clinical spectrum and outcome of MEN 2B. METHODS: Twenty-one patients underwent operation for manifestations of MEN 2B between 1970 and 1993. Median follow-up was 16.9 years. Diagnosis was made through family screening in nine, the development of medullary thyroid carcinoma (MTC) in seven, phenotypic features in four, and constipation in one. Median age at presentation of colonic dysfunction, MTC, and pheochromocytoma was 0.1, 16, and 28 years, respectively. RESULTS: Every patient had MTC. Fifteen (94%) of 16 patients undergoing primary thyroidectomies had multicentric disease, and seven (44%) had nodal metastases. Seven patients (33%) had pheochromocytoma, six bilateral and one malignant. Adrenalectomy was curative in every patient. Nineteen patients (90%) had colonic disturbances, typically chronic constipation from birth. Megacolon developed in 14 patients, and eight required colonic surgery. Every patient had the characteristic phenotype. Dominant features included neuromas of the tongue, buccal mucosa, lips, conjunctivae, and eyelids and a marfanoid habitus. Other features included high arched palate, corneal nerve thickening, and dental and skeletal abnormalities. Four patients died, two of metastatic MTC, one after operation for metastatic MTC, and one as a consequence of colonic perforation. Of 17 survivors, three have hepatic metastases from MTC, eight have nodal metastases, and six are well with normal or mildly elevated calcitonin levels. CONCLUSIONS: MEN 2B is characterized by a relatively aggressive form of MTC, bilateral pheochromocytoma, severe colonic dysfunction, and a multitude of other extraendocrine abnormalities. Early recognition of MEN 2B and early prophylactic thyroidectomy are essential. Colonic dysfunction has previously received little attention, and further investigation of the pathogenesis and treatment of this disorder is warranted.


Assuntos
Neoplasia Endócrina Múltipla Tipo 2b , Adolescente , Neoplasias das Glândulas Suprarrenais/cirurgia , Adulto , Carcinoma Medular/cirurgia , Criança , Pré-Escolar , Colo/patologia , Colo/fisiopatologia , Colo/cirurgia , Sistema Digestório/fisiopatologia , Feminino , Humanos , Hipertrofia , Lactente , Masculino , Pessoa de Meia-Idade , Neoplasia Endócrina Múltipla Tipo 2b/diagnóstico , Neoplasia Endócrina Múltipla Tipo 2b/fisiopatologia , Neoplasia Endócrina Múltipla Tipo 2b/cirurgia , Fenótipo , Feocromocitoma/cirurgia , Neoplasias da Glândula Tireoide/cirurgia
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