The management of acute abdominal pain in children

Objective : Acute abdominal pain is a common problem in childhood, and appendicitis is frequently diagnosed by general practitioners and doctors working in emergency departments. The objective of the present report was to determine the frequency of appendicitis in a group of children with acute abdominal pain presenting to the emergency department of a general hospital, as well as attempting to analyse the manner in which these patients were managed.
Methodology : The initial assessment in hospital (by resident hospital staff) of the study group of patients was analysed and their subsequent clinical progress documented.
Results : The present report shows that the vast majority of children with acute abdominal pain do not have appendicitis and that appendicitis is significantly over-diagnosed by junior hospital doctors.
Conclusions : Appendicitis is a clinical diagnosis, best made by an experienced member of the paediatric surgical team. Frequent review, with a minimum of investigations, provides the best means of making a rational decision regarding surgery.     

A clinician's guide to hereditary colon cancer

Approximately 10% of patients diagnosed with colorectal cancer are at risk for a hereditary form of the disease. At-risk patients can be offered genetic counseling and testing to determine whether they carry a detectable mutation for such a syndrome. If so, this information provides the clinician with valuable data about the patient's risk for other cancers, and what further surveillance and risk reduction options should be incorporated into the management plan. Mutation identification within a family also makes it possible for other family members to learn if they are at risk for the same syndrome. There are many hereditary colorectal cancer syndromes, and the clinician must know what essential information should be elicited from a family history and which patients should be referred for genetic counseling and testing.     

Wegener's Granuloma. A Series of 265 British Cases Seen Between 1975 and 1985. A Report by a Sub-committee of the British Thoracic Society Research Committee

In order to describe the British experience of Wegener's granuiomatosisHospital Activity Analysis was used to collect cases diagnosedin England, Wales and Scotland between 1975 and 1985. Wherepossible clinical details, histological material and chest radiographswere obtained. Two hundred and sixty five patients were consideredto have Wegener's granuiomatosis. In 109 a single pathologistconfirmed the diagnosis by finding both granulomas and vasculitisin biopsy material. The diagnosis was made on clinical groundsor clinical grounds together with histological diagnosis inthe local hospital in 156 patients. Wegener's granuiomatosiswas confined to the lung or upper respiratory tract in 22 percent of patients and renal disease occurred in 58 per cent.Laboratory tests showed a pattern of mild anaemia, polymorphleucocytosis, eosinophilia and an elevated ESR and hypergammaglobulinaemia,with no specific pattern of changes. Histological confirmation was most frequently obtained by examinationof nasal biopsy specimens, but multiple biopsies were oftenrequired. Renal biopsies showed focal proliferative glomerulonephritisbut granulomatous glomerulonephritis was uncommon. Of availablechest radiographs 61 per cent were abnormal, large opacitiesbeing most common. Small irregular opacities were found lessoften and other abnormalities were uncommon. Treatment varied widely and 10 per cent of patients receivedno drug therapy. This large series illustrates that even withoutspecific treatment, patients with Wegener's granuiomatosis cansurvive for several years and with modern treatment survivalfor more than a decade is possible. Conclusions about the effectivenessof the various therapies cannot be drawn from this restrospectivestudy. Renal failure and disseminated vasculities were the commonestcauses of death; death was considered to result from complicationsof treatment with cytotoxic drugs or prednisolone in 6 per centof patients.     

Use of risk-reducing surgeries in a prospective cohort of 1,499 BRCA1 and BRCA2 mutation carriers

Inherited mutations in BRCA1 or BRCA2 (BRCA1/2) confer very high risk of breast and ovarian cancers. Genetic testing and counseling can reduce risk and death from these cancers if appropriate preventive strategies are applied, including risk-reducing salpingo-oophorectomy (RRSO) or risk-reducing mastectomy (RRM). However, some women who might benefit from these interventions do not take full advantage of them. We evaluated RRSO and RRM use in a prospective cohort of 1,499 women with inherited BRCA1/2 mutations from 20 centers who enrolled in the study without prior cancer or RRSO or RRM and were followed forward for the occurrence of these events. We estimated the age-specific usage of RRSO/RRM in this cohort using Kaplan–Meier analyses. Use of RRSO was 45 % for BRCA1 and 34 % for BRCA2 by age 40, and 86 % for BRCA1 and 71 % for BRCA2 by age 50. RRM usage was estimated to be 46 % by age 70 in both BRCA1 and BRCA2 carriers. BRCA1 mutation carriers underwent RRSO more frequently than BRCA2 mutation carriers overall, but the uptake of RRSO in BRCA2 was similar after mutation testing and in women born since 1960. RRM uptake was similar for both BRCA1 and BRCA2. Childbearing influenced the use of RRSO and RRM in both BRCA1 and BRCA2. Uptake of RRSO is high, but some women are still diagnosed with ovarian cancer before undergoing RRSO. This suggests that research is needed to understand the optimal timing of RRSO to maximize risk reduction and limit potential adverse consequences of RRSO.     

gamma-Glutamyl transpeptidase activity in liver disease: serum elevation is independent of hepatic GGTP activity

gamma-Glutamyl transpeptidase activity was measured in liver and serum from 110 patients undergoing diagnostic liver biopsy, including patients with alcoholic liver disease, fatty liver not due to alcohol, primary biliary cirrhosis, persistent hepatic disease, chronic active hepatitis and normal livers. Serum gamma-glutamyl transpeptidase was markedly elevated in patients with alcoholic liver disease and primary biliary cirrhosis while mean hepatic gamma-glutamyl transpeptidase activity was significantly increased only in the alcoholic liver disease group. There was considerable overlap of individual enzyme values among the different disease groups. There was no inhibitors or activators of liver gamma-glutamyl transpeptidase in any of these disorders. The increased liver activity was not related to the degree of hepatic fibrosis or cirrhosis. There was no correlation between hepatic and serum gamma-glutamyl transpeptidase activity. Hepatic and serum gamma activities were equally increased in individuals with alcoholic liver disease whether or not they were drinking at the time of the study. The data suggest that increased hepatic gamma-glutamyl transpeptidase activity is neither specific for alcoholic liver disease nor essential for serum GGTP to be elevated.     

Role of immune interferon in the monocytic differentiation of human promyelocytic cell lines induced by leukocyte conditioned medium

Conditioned medium (CM) from lectin-stimulated human leukocytes contains factors that induce human promyelocytic cell lines to differentiate along the monocytic pathway. In this report, we show that human promyelocytic cell lines are also induced to differentiate along this pathway by immune interferon (IFN gamma). Various preparations of IFN alpha tested did not induce this differentiation. In cultures containing IFN gamma, the cells are induced to coordinately express monocyte markers and functions such as monocyte-specific surface antigens, HLA-DR antigens, nonspecific esterase, receptors for the Fc fragment of IgG, and the ability to mediate antibody-dependent cell- mediated cytotoxicity. Our data indicate that differentiation induced by IFN gamma is not secondary to an arrest of growth of promyelocytic cell lines, but rather that a proportion of cells is induced along a programmed pathway of terminal differentiation similar to that of normal monocytes. CM contains IFN gamma, but its ability to induce differentiation is greater than expected on the basis of its content of IFN gamma. Treatments at 56 degrees C or at pH 2.0, which abolish IFN gamma activity, abrogate the differentiation ability of CM. The antiviral activity and the differentiation activity contained in the CM are coeluted from gel filtration and reverse-phase columns. Monoclonal antibodies anti-IFN gamma, which completely abrogate the differentiation ability of IFN gamma and the antiviral activity in the CM, completely suppress the induction of some monocyte markers by CM, but only reduce the expression of others. When IFN gamma is added to CM, promyelocytic cell lines are induced to differentiate to a much greater extent than that induced by either IFN gamma or IFN gamma- depleted CM alone. These results show that the differentiation activity of leukocyte CM is due to the synergistic effect of IFN gamma and other factors not yet identified.     

Harken caged-disc mitral valve replacement, 1969-1975: analysis of late mortality, thromboembolism, and valve failure

We evaluated the long-term outcome of mitral valve replacement with a Harken caged-disc prosthesis for up to 11 years (range, 50 to 130 months; mean, 81 months) in 170 patients whose mean age was 55 years. The early (30-day) mortality was 11.2% (19 out of 170 patients). Late follow-up information was obtained for 144 (95%) of the 151 survivors. The actuarial survival was 57% at 5 years and 40% at 10 years. Overall mortality was associated with advanced age, male sex, an ischemic origin for the mitral valve disease, and nonuse of warfarin anticoagulation. Late deaths (n=59) were valve-related in 46%, cardiac but non-valve-related in 44%, and noncardiac in 10% of the cases. One thromboembolic event or more occurred in 41 patients (incidence, 5.7% per patient year), 14 of whom died (24% of the late deaths). All four patients who were not on warfarin, aspirin, or other antithrombotic therapy experienced thromboemboli. This complication was correlated with the nonuse of warfarin-type anticoagulation, with mitral regurgitation, and with late cardiac death. Mechanical prosthetic failure resulted in reoperation or death in 7.6% of the late survivors (1.5% per patient year). In 75 patients with normally functioning prostheses, the disc-to-sewing ring ratio was established by means of cinefluoroscopy (0.93 +/- 0.04, mean +/- 25D). Because of the high proportion of cardiac valve-related deaths (46%), the high incidence of late mortality due to thromboembolic events (24%), and the 7.6% incidence of reoperation or death resulting from mechanical valve failure, close follow-up with cinefluoroscopy and continued warfarin anticoagulation (alone or in combination with dipyridamole) are essential after mitral valve replacement with the Harken caged-disc prosthesis.