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Three hundred and sixteen patients with cystic fibrosis were seen at the Brompton Hospital during 1965-83; 178 (56.3%) of them were male and 136 female, and their ages ranged from 12 to 51 years. Most patients presented in infancy with respiratory symptoms and malabsorption, but 19 (6%) were diagnosed in adult life, three in their 30s. Pulmonary disease was almost universal (99.7%), being responsible for 97% of all deaths and three quarters of hospital admissions. All patients had developed a productive cough by the age of 21 and over half before the age of 5. Many complained of wheezing, but reversible airflow obstruction was present in only 40% of those tested. Minor haemoptysis was very common (62%), but major episodes less so (10%). Pneumothorax was seen in 61 cases (19%), and was often recurrent. Some irreversible airflow obstruction was present in all patients with pulmonary disease. Two patients have been followed for over 20 years without showing appreciable decline in lung function. Thirty five patients (11%) had no symptoms of malabsorption. Acute meconium ileus equivalent was seen in 16% and a chronic partial obstruction with episodic symptoms in a further 19%. Diabetes mellitus developed in 36 patients, 13 of whom were insulin dependent. Hepatomegaly was common (29%), often occurring without abnormal results in biochemical tests of liver function; only 1% of patients developed portal hypertension with varices and ascites. Skin reactions to at least one common allergen, including Aspergillus fumigatus, were positive in 70%, but very few patients suffered from hay fever or eczema. One hundred and twenty one patients have died, 97% from infection or other pulmonary complications, and 195 were alive in December 1983 (mean age 23 years). Seventy eight per cent of patients were in full time education or full or part time employment, or were housewives, and only 41 were unemployed for reasons for health. Many patients are married and 10 women have borne children. Most patients were admitted to hospital only three or four times during the period of follow up and 50 individuals (16%) have never been in hospital at all. The improvement in prognosis and quality of life for adults with cystic fibrosis should encourage a positive attitude in those who care for them.
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PMID:Cystic fibrosis in adolescents and adults. 343 96

The case is recounted of a child who was admitted to hospitals several times over a period of 8 years on account of fictitious illnesses invented by his mother. The first occurred when he was 3.5 years old in January 1984. His mother, a nurse, gave a history of intermittent fever for 3 months, loss of appetite and weight. He had been treated with ampicillin, chloramphenicol, and procaine penicillin. No abnormality was detected and his weight at 15.5 kg was appropriate for his age. No fever was recorded throughout 2 weeks in hospital, but he was given chloroquine for possible malaria and then discharged. At follow-up 6 months later, the mother complained of his wheezing. On examination he was normal and had gained 3.8 kg since discharge. The possibility of vernal conjunctivitis plus asthma was entertained and he was then placed on ketotiphen prophylaxis. There was an uneventful follow-up for 6 months. 5 years later in March 1990, his mother related that he had been treated from 22 January 1988 to 21 November 1989 for tuberculosis with streptomycin, isoniazid, rifampicin, and ethambutol. He was also treated with digoxin and Esidrex-K for suspected rheumatic carditis, after which at the University Teaching Hospital, Enugu, he was investigated from 11 April 1989 to 10 August 1989 and found to be normal. One year later in August 1991 she went to one of the authors complaining about polydypsia, polyphagia, and polyuria. Examination had revealed nothing of note. A clinical assessment for diabetes mellitus found the urine specific gravity persistently at 1.010. He was therefore put on carbamazepine (Tegretol) 100 mg t.i.d. After review by a pediatric nephrologist, the child was declared normal. During this visit, the mother and child were interviewed separately. He believed he was ill because his mother said so. A diagnosis of Munchausen syndrome by proxy was made. The mother was referred back to her doctor to arrange for psychiatric care. In Munchausen syndrome, patients fabricate a variety of symptoms and evidence of illness that have no organic basis. Munchausen syndrome by proxy is a form of child abuse, difficult to diagnose, that could result in death. It is more prevalent in affluent countries with sophisticated medical facilities. Its rarity in developing countries may contribute to the difficulty of detection.
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PMID:Munchausen syndrome by proxy: an experience from Nigeria. 750 55

Octreotide is a synthetic octapeptide somatostatin analogue which has higher potency and longer duration of action than the natural hormone. It is effective after subcutaneous administration and no rebound hypersecretion has been observed. Pharmacological effects of octreotide include inhibition of numerous hormones (growth hormone, TSH, insulin, glucagon and all gut hormones), of exocrine secretion (gastric acid, pancreatic enzyme), and of small-bowel absorption. This review deals with clinical application of octreotide in endocrine disorder. In patients with acromegaly octreotide treatment results in decrease of growth hormone (GH) and IGF-I together with tumour shrinkage and clinical improvement. Although variability in response to treatment is obvious for majority of patients the most effective dose is 100 mcg three times daily subcutaneously. Normalization of GH levels could be achieved in more than 50% of treated patients. It has also been shown that octreotide could be effective in TSH secreting pituitary adenoma, ACTH secreting adenoma as well as in non-secretory pituitary tumours. A marked biochemical and clinical responses together with longer survival have been reported in most of the patients with gastroenteropancreatic (GEP) tumours. Patients who benefit the most from octreotide therapy are those with carcionid syndrome (successful control of diarrhoea, flushing episodes and wheezing) and VIPomas (control of diarrhoea). In patients with insulin-dependent diabetes mellitus (IDDM) octreotide suppresses GH levels, postprandial blood glucose increases with resultant decrease in daily insulin requirements. In women with polycystic ovary syndrome (PCOS) octreotide has inhibitory effect on serum LH and ovarian androgens. This could have beneficial effect on ovulatory performance in women with PCOS.
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PMID:[Clinical use of octreotide (Sandostatin) in endocrinology]. 799 11

Life threatening asthma is a serious condition that is often difficult to treat. Often, despite maximal medical therapies, these patients remain functionally crippled. In addition, older patients are even more susceptible to asthma as they are less able to adapt to or tolerate the symptoms. In this paper we report the effectiveness of a new treatment regimen of high-dose intramuscular triamcinolone (360 mg) in 7 elderly patients with severe, chronic, steroid-dependent asthma. Each patient was given one intramuscular injection of 360 mg of triamcinolone (Kenalog) after maximizing traditional medicines. All 7 patients experienced resolution of their asthma symptoms within 1 week of receiving this injection. They showed marked functional improvement in their activities of daily living, and independence. Six of seven progressed from being homebound to walking, shopping, and grooming without restriction. All had a corresponding rise in peak expiratory flow rates ranging between 25 and 93%. In addition, all were able to stop taking their daily oral prednisone. Response durations ranged from 3 to 24 months. In the 6 patients who experienced relapse, all requested and received a second injection, with similar positive outcomes. Some experienced transient weakness and diabetes during the first week of the therapy but all elected to receive additional shots when they experienced break-through wheezing. Thus, we feel that high-dose intramuscular triamcinolone should be considered as a therapeutic option for a highly select group of older steroid-dependent asthma patients.
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PMID:Effect of high-dose intramuscular triamcinolone in older adults with severe, chronic asthma. 811 14

Reversible airway obstruction in childhood includes two major groups of patients: those with recurrent wheezing following bronchiolitis in early childhood, and those with allergic asthma, which represents an increasingly large proportion of cases through the school years. Over the last 40 years of the 20th century, allergic asthma has increased in many countries and in relation to several different allergens. Although this increase has differed in magnitude in different countries and also in the social groups most affected, it has had several features in common. The increase generally started between 1960 and 1970, has been progressive since then, and has continued into the 1990s without a defined peak. Among children 5-18 years of age, the increase has predominantly been among allergic individuals. Theories about the causes of the increase in asthma have focused on two scenarios: a) that changes in houses combined with increased time spent indoors have increased exposure to relevant allergens, or b) that changes in diet, antibiotic use, immunizations, and the pattern of infections in childhood have led to a change in immune responsiveness such that a larger section of the population makes T(H)2, rather than T(H)1 responses including IgE antibodies to inhalant allergens. There are, however, problems with each of these theories and, in particular, none of the proposed changes can explain the progressive nature of the increase over 40 years. The fact that the change in asthma has much in common with epidemic increase in diseases such as Type II diabetes or obesity suggests that similar factors could be involved. Several lines of evidence are reviewed that suggest that the decline in physical activity of children, particularly those living in poverty in the United States, could have contributed to the rise in asthma. The hypothesis would be that the progressive loss of a lung-specific protective effect against wheezing has allowed allergic children to develop symptomatic asthma. What is clear is that current theories do not provide either an adequate explanation of the increase or a practical approach to reversing the current trend.
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PMID:Specific and nonspecific obstructive lung disease in childhood: causes of changes in the prevalence of asthma. 1093 91

Gastroenteropancreatic (GEP) neoplasms originate from any of the various cell types belonging to the neuroendocrine system. A general characteristic of GEP endocrine tumours is that the vast majority produce and secrete a multitude of peptide hormones and amines. Many patients with malignant metastasising tumours present clinical symptoms related to hormone hyperproduction. These include the so-called carcinoid syndrome, characterised by flushing, diarrhoea, wheezing and right heart disease, which is predominantly associated with the serotonin- and tachykinins-producing carcinoids of the midgut. Several types of syndrome associated with GEP endocrine tumors are caused by overproduction of a specific hormone. For instance, the well-known Zollinger-Ellison syndrome is gastrin-mediated. The so-called 'insulinoma syndrome' depends on excessive production of insulin and proinsulin, resulting in hypoglycemia. The 'glucagonoma syndrome' is characterised by necrolytic migratory erythema, diabetes and diarrhoea. The Verner-Morrison syndrome, which is brought about by high circulating levels of vasointestinal peptide (VIP). produces severe secretory diarrhoea. Finally the 'somatostatinoma syndrome' involves gallbladder dysfunction and gallstones, diarrhoea with or without steatorrhea, and impaired glucose tolerance. The biochemical diagnosis of endocrine digestive tumors is based on general and specific markers. The best general markers are chromogranin A (CgA) and pancreatic polypeptide (PP). Specific markers for endocrine tumors include insulin, gastrin, glucagon, vaso intestinal polypeptide (VIP), somatostatin and the primary cathabolic product of serotonin, 5-hydroxyndoleacetic acid (5-HIAA). Localisation procedures commonly applied, in the diagnosis of endocrine tumours include ultrasound (US), computed tomography (CT) and somatostatin receptor scintigraphy (SRS).
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PMID:Epidemiology, clinical features and diagnosis of gastroenteropancreatic endocrine tumours. 1176 60

1. Epidemiological trend of TB and its diagnosis among the elderly: Masako OHMORI (Research Institute of Tuberculosis, JATA). It is estimated that over 10,000 TB patients will occur among the elderly aged over 75 years of age during 2010 and 2020. Though the new TB patients among the elderly over 65 years of age decreased in number from 1987 to 2001, sputum smear positive patients, which are highly infectious increased by 1.3 times for the same age group, and 2.3 times for that of over 80 years of age. 17.6% of those elderly patients were detected in institutions, either hospitals or nursing homes. These indicate that the elderly will become higher risk of TB for causing early death and infection to others during their admission in the hospitals or nursing homes. 2. Clinical characteristics of TB among the elderly: Nobuhiko NAGATA (Department of Internal Medicine, National Ohmuta Hospital). Analysis of the 93 elderly TB patients shows that TB diagnosis was delayed among the elderly. For TB diagnosis, 43.2% took over 1 month, and 27.3% over 2 months among the patients over 75 years of age, compared to 22.9% and 9.7% respectively for the patients under 65 years. The prognosis was also poor. Of all who died in the hospital during TB treatment, 40.9% (mostly complicated with other diseases) was among the elderly over 75 years, while it was 6.5% among those under 65 years. Complication of dementia delayed the diagnosis. 79.4% of the smear positive patients over 75 years were diagnosed while admitted in a general ward, and 40% had been admitted 2 weeks or more before diagnosis. 3. Use of serological tests for the diagnosis of TB among the elderly: Atsuyuki KURASHIMA (Department of Clinical Research, National Tokyo Hospital). As TB diagnosis is difficult for the elderly due to non-specific appearances of signs/symptoms, X-ray shadows or negative tuberculin reaction, some serological tests, which have been recently developed, can be useful as a supplementary diagnostic tool. One of them is anti-lipoarabinomannan antibody. 74.3% was positive to this among 148 bacteriologically confirmed TB cases; 77.8% for those aged 65 years and above, and 71.1% for those under 65 years. To anti TBGL antibody, 78% of 170 confirmed TB cases were positive; 75% for those aged 65 years and above, 79.8% for those less than 65 years. Multi-lipo antibody developed by Japan BCG Laboratory showed higher sensitivity of 91.5%. GPL-core antibody may increase the sensitivity. 4. Mode of TB detection in nursing homes: Shinji SHISHIDO (Research Institute of Tuberculosis, JATA). 15 elderly TB patients who had been diagnosed in 23 nursing homes in the last 5 years were analyzed. The average age was 80.7 years. 10 were male and 5 were female. 11 were sputum smear positive. The symptoms were fever (8), cough (7), wheezing (2), hemoptysis (1), chest pain (1), body weight loss (1), appetite loss (1). Number of patients by time durations before consulting a doctor of within 2 weeks, 2 weeks to 1 month, 1 to 2 months, 2 to 3 months, 3 to 6 months, and more than 6 months were 6, 1, 1, 3, 2, 2 respectively. The prognosis: 4 died within 10 days after diagnosis, 4 needed examinations for the symptoms but were delayed in diagnosis as they were not admitted due to dementia or disability. The orientation and training to the staff of the nursing homes are needed for early TB diagnosis. 5. Programme for the early detection of TB among the elderly: Tadayuki AHIKO (Murayama Public Health Center, Yamagata Prefecture). Based on the analysis of 138 confirmed TB cases registered in Yamagata Prefecture in 1998, services for early TB detection among the elderly should be 1) periodical chest X-ray examinations by the patients home doctors when underlying high risk diseases such as diabetes or cancer exist, and 2) -sputum examination for the symptomatic patients. A survey to 31 local governments conducting special TB services showed the special screening programme for the bedridden elderly in nursing homes was not so efficient due to low quality of the available X-ray facilities and low case detection rate. But these X-ray films can be utilized for comparison with those when any symptom arises.
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PMID:[Tuberculosis control programme for the elderly with special focus on early detection]. 1503

Early T cell responses to external antigens and autoantigens are subject to a variety of regulatory mechanisms. A unifying link between the increase in both Th1-dependent autoimmune disease and Th2-linked atopic allergy would be a disturbed immune regulation involving T regulatory cells. There is a strong global correlation between childhood wheezing and diabetes. It is increasingly recognized that microbial colonization of the gastrointestinal tract, linked with lifestyle and/or geographic factors, may be important determinants of the heterogeneity in disease prevalence throughout the world. These suggestions are supported by observations that germ-free mice do not develop tolerance in the absence of a gut flora. The potential effects of environmental stimuli on immune function is greatest in early life including fetal life when systems and responses are developing, and the maternal influences during fetal life could be particularly important for the development of immune regulation and tolerance induction. In recent years, focus has switched from searching for environmental risk factors towards an interest in factors that could induce and maintain immune regulation and tolerance to allergens and autoantigens. Currently evaluated strategies include the use of immunomodulatory factors, such as probiotics, prebiotics, and dietary nutrients, although data are still insufficient to make specific recommendations.
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PMID:Environmental influences on the development of the immune system: consequences for disease outcome. 1819 56

Numerous epidemiological studies suggest that there is an inverse relationship between allergic diseases and infections in early childhood, but there are also several well-conducted epidemiological studies that seemingly contradict this relationship. The maturation of the immature immune regulation after birth is largely driven by exposure to microbes. Germ-free animals manifest excessive immune responses when immunized and they do not develop normal immune regulation. The controversy regarding the role of infections for subsequently developing allergy is partly due to varying clinical definitions of 'allergy'. Thus, wheezing and asthma have often been included as outcomes. The hypothesis that commensal microbes are the normal stimulants for the maturation towards a balanced immune response is relevant for IgE-mediated disease manifestations, rather than recurrent bronchial obstruction per se. Epidemiological, clinical and animal studies taken together suggest that broad exposure to a wealth of commensal, non-pathogenic microorganisms early in life are associated with protection, not only against IgE-mediated allergies, but also conceivably against type-1 diabetes and inflammatory bowel disease. This has little relationship with 'hygiene' in the usual meaning of the word. The term 'hygiene hypothesis' is unfortunate, as it is misleading. A better term would be 'microbial deprivation hypothesis'.
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PMID:The hygiene hypothesis: do we still believe in it? 1971 May 12

The origins of asthma might be traced back to events occurring during fetal life. Reduced lung development has been shown to be a risk factor both for viral induced wheeze and allergic asthma. The evidence for a causal relationship between exposure to environmental tobacco smoke, chemical domestic products for cleaning, outdoor pollutants, and reduction in lung function is quite strong. Reduced maternal intake of vitamin E, vitamin D, and zinc, or increased use of paracetamol during pregnancy is associated with increased wheezing outcomes in children. The odds ratio for asthma onset is also increased in infants born from mothers with oligohydramnios, chorioamnionitis, hypertension, preeclampsia, diabetes and exposed to stressful events. The risk of developing allergic asthma is increased if the child is exposed in the first months of life to synthetic bedding and is enhanced by allergen exposure and an inadequate barrier function of the skin. In conclusion, several lines of evidence support the concept of fetal programming and very early life events in the development of the different phenotypes of asthma. Since some environmental triggers can be easily avoided and some protective factors can be easily implemented all efforts should be made to prevent intrauterine insults and early sensitization.
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PMID:Fetal and early postnatal life roots of asthma. 2092 57


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