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The concept of risk in the field of avitaminoses is very important and useful for the practitioner, who should consider two aspects: a) risk factors, which could be individual (physiological, pathological and psychological) and extra-individual (alimentary, environmental, etc.); b) subjects with an elevated risk of avitaminosis (childhood, old age, pregnancy, etc.). In these subjects the risk can be a generical one, when there is an elevated requirement for all vitamins (nursing women, sportmen, etc.) or a specific one, when there is a high requirement only for a single vitamin (osteomalacia, some professional diseases, use of oral contraceptives) or a vew of them (alcoholism, diabetes, etc.). On the basis of this kind of knowledge it is easy for the practitioner to estimate which vitamins are necessary for each subject or for a group of subjects in physiological or in pathological conditions. For example, there is an elevated risk of apyridoxinosis in old age (acalciferolosis in aged women), of athiaminosis and apyridoxinosis in diabetes, of apyridoxinosis in oral contraceptives users, of axeroftolosis in hyperthyroidism, of athiaminosis, apyridoxinosis, aniacinosis and anascorbosis in alcoholics. In the second chapter the concept of the latency period in avitaminosis is illustrated. This period corresponds to the interval between the moment when deficiency stimulus starts operating and the moment when its effect, that is the picture of avitaminosis, appears. The latency time is not measurable, on account of the difficulties in establishing the onset of the deficiency stimulus; generally it is very long and is followed by the period of biochemical symptomatology and subsequently by the one of clinical symptomatology. Each of these three phases can be further divided in several steps, which have summarized in a Table. The last chapter is dedicated to the classification of avitaminoses. From the etiopathogenetic point of view avitaminoses can be due to: a) deficiency of introduction (alimentary level)); b) deficiency of absorption (enteric level); c) deficiency of utilization (tissue level). From the clinical point of view avitaminoses can be distinguished in deficiency with: a) a complete clinical symptomatology (scurvy, beriberi, pellagra, rickets, osteomalacia, xerophthalmia, hemeralopia); b) an incomplete clinical symptomatology (mono- or oligo-symptomatic or partial clinical picture); c) a biochemical symptomatology only (subclinic or clinically asymptomatic picture).
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PMID:[Vitaminology for practitioners. II. Avitaminoses, risk, latency period, classification]. 58 76

Fanconi syndrome is a rare cause of rickets in children. Only six families with Fanconi syndrome following an autosomal dominant pattern of inheritance have been reported. In this report, the results of clinical studies performed in three generations of a family of 39 members with autosomal dominant Fanconi syndrome are presented. Twenty-one members of this family provided blood and urine for biochemical evaluation. Many family members have one or more tubular reabsorptive abnormalities; however, the complete Fanconi syndrome was not present in most members. Three children with the complete syndrome all occur in the last generation. When the characteristic features of this family were compared with those of previously reported families with autosomal dominant Fanconi syndrome, several differences became apparent. Two serious manifestations, diabetes mellitus and renal failure, which occur in previous reports did not occur in this family. This report provides information on apparently the largest number of affected individuals in a single family with Fanconi syndrome. In addition, variable expressivity of tubular reabsorptive defects in a family with Fanconi syndrome has never been reported.
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PMID:Idiopathic Fanconi syndrome in a family. Part I. Clinical aspects. 162 57

Although the enamel defects of low birthweight prematurely-born children have been well investigated clinically the ultrastructure has not been studied in detail. Using scanning electron microscopy this investigation examined the enamel surface of 4 representative teeth from a group of exofoliated teeth from prematurely-born, very-low birthweight children compared to an equal number of control teeth from normal birthweight children. The results showed that all 4 teeth from the prematurely-born group had enamel defects at the ultrastructural level even though the defects were evident only macroscopically in 2 teeth. By contrast all the control teeth from normal children did not show abnormalities of surface structure. Thus this investigation further confirms that birth prematurity and low birthweight adversely affect the activities of ameloblasts. Previous clinical investigations have reported that prematurely-born children show high prevalences of enamel hypoplasia of around 20-100%. The etiological factors involved in the pathogenesis of enamel defects are unclear, but are likely to be related to many systemic illnesses occurring during the neonatal period. These include respiratory distress syndrome, hyperbilirubinemia, maternal diabetes as well as neonatal rickets. Furthermore, Seow et al. (1989) showed recently that deficiency of calcium and phosphate mineral in the neonatal period is related directly to enamel hypoplasia in very-low birthweight, prematurely born children. In addition to systemic factors, local factors such as laryngoscopy and endotracheal intubation have also been implicated in the etiology of enamel hypoplasia in these children. While the enamel defects have been well studied at the clinical level, there is a paucity of information on the ultrastructural nature.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Enamel hypoplasia in prematurely-born children: a scanning electron microscopic study. 209 77

Since the establishment of a new social order in 1949, China's attempts to feed and nurture its large population has been a topic of serious study in many disciplines. This review focuses on dietary sources of Chinese population and incidence, increase and decline of important diet related health disorders in China during the last four decades. Literature published since 1949 on goiter, rickets, riboflavin deficiency, beri beri, vision impairment, favism, cancer, atherosclerosis and coronary heart disease, hypertension, dental and smoking related diseases, diabetes mellitus, pancreatitis, lactose intolerance, mineral deficiency, Kashin-Beck disease, parasitic diseases and genetic disorders are reviewed. Also presented selectively are reports related to ethnodietetics, health care, maternal health and pediatric care as well as longevity. In the 1980s, total caloric intake of Chinese population showed a 19% increase on a daily basis from that of late 1940s. In overall terms, plant derived foods supplied 93% of energy, 87% of protein and 55% of fat to the Chinese. Among the animal foods, pork remains the most common and least expensive form of meat, contributing more than 90% of China's total meat production excluding poultry and fish. In 1949, the life expectancy in China was only 36 years. In early 1980s, it has increased to 68 years. This increase in life expectancy is attributed mostly to improved nutrition and lowering of mortality due to decrease in infectious diseases. Though population, disease and mortality statistics of modern China are spotty and sometimes questionable, common consensus among the researchers is that since 1949 the public health situation in China has improved tremendously.
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PMID:Nutrition and health in China, 1949 to 1989. 229 45

Fanconi syndrome is a complex of renal tubular dysfunctions defined by glycosuria without diabetes, aminoaciduria, phosphaturia, and renal tubular acidosis. It is often associated with hypokalemia, hypophosphatemia, and rickets or osteomalacia. Although it is usually found in the setting of other well-established non-renal diseases, Fanconi syndrome may present without identifiable etiology or association. Very infrequently a patient with idiopathic Fanconi syndrome will progress to chronic renal failure. This case report details the course of such a patient over the 20 years since his diagnosis and discusses the syndrome's genetic background, clinical features, putative pathophysiology, and therapeutic options, including transplantation.
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PMID:Idiopathic Fanconi syndrome with progressive renal failure: a case report and discussion. 235 24

Recent evidence suggests that the protein osteocalcin is like the bone alkaline phosphatase produced by osteoblasts and circulates in human blood. With the introduction of a radioimmunoassay for serum osteocalcin it was hoped that this test would provide a useful index of altered bone metabolism. Therefore serum osteocalcin was measured in 88 controls and 112 patients with disorders of calcium and phosphate metabolism, isolated elevation of alkaline serum phosphatase in the absence of disease (isolated hyperphosphatasaemia) and children prone to osteopenia. In the controls serum osteocalcin was higher in children less than 15 years (median and range: 11.9, 7.7-15.3 ng/ml) than in adults (3.7, 2.6-5.2 ng/ml) and was highly correlated to alkaline serum phosphatase activity (r = 0.87, n = 88, P less than 0.01). Osteocalcin was elevated in primary hypoparathyroidism, low in untreated hypoparathyroidism but normal in hypoparathyroidism (including pseudohypoparathyroidism) during vitamin D treatment. The bone protein was low-normal and increased to high-normal levels during vitamin D therapy in vitamin D deficiency rickets and familial hypophosphataemic rickets, but remained low in patients with end organ resistance to 1,25-dihydroxyvitamin D. Osteocalcin (and urinary hydroxyproline) were not elevated in isolated hyperphosphatasaemia, indicating that mechanisms other than increased bone turnover may account for the markedly elevated serum alkaline phosphatase activity in these subjects. Osteocalcin was decreased in children with diabetes mellitus type I and in patients on glucocorticoid treatment, indicating decreased bone formation. It is concluded that the measurement of serum osteocalcin seems to be a reliable index of bone formation provided that the vitamin D status and renal function are normal.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Evaluation of serum osteocalcin as an index of altered bone metabolism. 301 28

The purpose of the work was to investigate: Whether osteoarthritis of the hip can be divided into radiologic classes by examining the tendency of osteoarthritis of the hips to increase the growth and calcific content of the bone on the one hand and the associated loss of calcium and cartilage and the deformation and destruction of bone on the other. The prevalence of osteoarthritis of the hip in the internal medicinal and surgical outpatients of the University Central Hospital of Oulu, who were radiographed. Whether osteoarthritis of the hip or its different radiologic manifestations correlate with the patient's age, sex, occupation and strenuousity of work, rickets, cancerous diseases, diabetes, rheumatoid arthritis, family history, parity, smoking, obesity, physical activity, corticosteroid and anti-epileptic medication, and previous injuries to the lower extremities causing immobilization. Whether the radiologic findings of osteoarthritis of the hip are associated with typical symptoms. Whether there are correlations between the effects of medication and physiotherapy and the radiologic forms of osteoarthritis of the hip. The study population consisted of two series, of which the first included 401 patients: 167 males and 234 females. The second, or major part comprised 518 patients, of whom 249 were male and 269 female. For all these patients we had radiograms available which permitted reliable assessment of the hip condition. The second series, i.e. the latter group of 518 patients, also filled in a questionnaire which dealt with the etiology and symptoms of the osteoarthritis of the hip as well as the therapies they had received. Whenever possible, the changes of the pelvis and the lumbar spine were also assessed on the basis of the radiograms. On the basis of the radiologic findings, osteoarthritis of the hip was divided into two qualitative classes, hypertrophic and destructive, and a mixed type, and into three grades of severity. Hypertrophic osteoarthritis of the hips accounted for 51% of the cases, destructive for 20% and mixed type for 29%. The percentages for the different severities were 47% for the mild, 16% for the moderately severe and 37% for the severe. A total of 26% of the cases were right-sided, 22% left-sided and 52% bilateral. The mild, bilateral cases of osteoarthritis were mostly hypertrophic, whereas destructive osteoarthritis was clearly more common in the unilateral cases. Hypertrophic osteoarthritis was also more frequent in younger age-groups and destructive in older age-groups. The osteoarthritis of the older patients was more severe.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Osteoarthritis of the hip. Radiologic findings and etiology. 391 67

I conducted a retrospective study based on record reviews of 50,892 infants born at the Foothills Provincial Hospital, Calgary, Alberta, Canada, from 1967 to 1984 to determine the incidence and possible causes of natal teeth. Fifteen infants were affected, for an incidence rate of one in 3,392 births. The affected teeth were the lower central incisors. The condition was more common in female infants. Two of the affected patients were twins. One patient each had Ellis-van Creveld syndrome, Pierre Robin syndrome, adrenogenital syndrome, cleft palate, and rickets. One mother each had pernicious anemia, vaginal infection, and diabetes mellitus.
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PMID:Natal teeth. 394 56

Several vitamins have been demonstrated to interfere with the pathogenesis of some metabolic diseases, mainly by three different mechanisms: 1) vitamin malabsorption, 2) errors in vitamin metabolism, 3) vitamin dependent syndromes. The latter is due to a deficiency of the apoenzyme whose coenzyme is the vitamin itself. In this case pharmacological, instead of nutritional doses of the vitamin may be needed. The vitamins which interfere with inborn metabolic errors are reviewed; for each vitamin the corresponding diseases which may be treated are indicated. The vitamins are: 1) thiamine (leucinosis); b) nicotinic acid (hyperlipoproteinemia); c) biotin (beta-methyl-crotonyl-glycinuria, propionic aciduria); d) pyridoxine (infantile convulsions, familial pyridoxine responsive anemia, homocystinuria, cystathioninuria, xanthurenicaciduria); e) cobalamins (congenital intrinsic factor deficiency, cobalamin malabsorption, transcobalamin deficiency, methylmalonic aciduria) f) folic acid (congenital folic acid malabsorption, formimino-transferase deficiency, methylenetetrahydrofolic reductase deficiency, Lesch-Nyhan syndrome); g) vitamin D (phosphatic diabetes, Prader's type rickets, Albright's syndrome; essential hereditary hypophosphatemia, etc). It is noteworthy that the vitamin therapy of these diseases, not only corrects the metabolic errors, but can also promote the healing or the amelioration of the psycho-physical growth, of central nervous system alterations and of other lesions.
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PMID:[Vitamins in metabolic diseases]. 702 68

British Asians make up 3% of the population. There is evidence that Asians have difficulty obtaining good quality health care, appropriate to their needs. This article examines some of this evidence, with examples of specific communities in Britain. In the past, specific health education programmes for Asians have targeted their 'special' needs such as rickets, tuberculosis and thalassaemia. In fact the population itself perceives its needs differently--improved communication, easier access to services, and more information on asthma, diabetes, ischaemic heart disease and skin disorders. It is important to appreciate that the 'Asian' community is made up of disparate groups with widely differing needs and expectations, and that each community should be considered by health service planners as unique within the context of the health authority within which they lie. Reasons for the mismatch between need and service provision are discussed in the light of the recent reforms in the National Health Service and recommendations for change are given.
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PMID:Accessibility and use of health care services in the British Asian community. 789 76


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