Reproduction: the Canadian family in transition.
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This paper describes elderly support and intergenerational transfer by gender, marital status, and place of residence for 150 elderly persons in Zimbabwe. The survey was conducted in September 1988, and includes information on background characteristics, income, and cash support from all sources, noncash support, and the support of elders to others. Conclusions and implications are discussed.
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Interspecific variability of the isotypic structure of two macroglobulins (Lpm and alpha 2M) in Mustelidae family was investigated using monospecific rabbit antisera against Lpm and alpha 2M Mustela vison. It was established that all the species studied may be divided into four groups, according to the degree of their homology with Lpm: 1) M. vison; 2) M. altaica, M. erminea, Kolonocus sibirica, Putorius eversmany, P. putorius, Luteola lutreola; 3) Vormela peregusna; 4) Martes martes, M. foina, M. zibellina. The greatest divergence of Martes gene species from M. vison and other species studied is determined by two different immunogenetic approaches, the first based on the whole isotype of Lpm and the other using allotypic markers of individual Lpm genes. The variability of the isotypic structure of alpha 2M was lower than that revealed in Lpm. V. peregusna is the only species which significantly differs from all above-mentioned species, including M. vison. The differences established for degree of variability between Lpm and alpha 2M in Mustelidae family may be connected with the evolutionary peculiarities of these two genetic system of serum alpha-macroglobulins, which being phylogenetically related, are still structurally divergent.
Familial adenomatous polyposis is dominant autosomal heritable and pre-cancerous. Characteristic changes of ocular fundus, multiple and bilateral retinal dysplasias, permit diagnosis in presymptomatic stage, significant especially not at least for sporadic cases of polyposis recti and coli, which are 40 per-cent of all. Contrary to often wellknown risk-families this group can't be realized epidemiologically, but complications are the same.
Comparison of 1988 data from the National Survey of Family Growth with 1982 data reveals that the proportion of U.S. women who have had sexual intercourse rose slightly, from 86 percent to 89 percent. Among teenagers, the proportion sexually active rose from 47 percent to 53 percent; most of the change is attributable to increases occurring among white and nonpoor teenagers, thus narrowing racial and income differences. Among women aged 15-44 in 1988 who have ever had intercourse, 67 percent reported that they had had two or more sexual partners in their lifetime. The proportion was highest among women aged 20-34 (about 70 percent), but 58 percent of sexually active teenage women reported having had two or more sexual partners. About 67 percent of women of reproductive age in 1988 were exposed to the risk of unintended pregnancy, up from 63 percent in 1982. Among these women, 35 percent rely on contraceptive sterilization to prevent pregnancy and 55 percent use reversible methods, while 10 percent use no method. Poor women are much more likely than nonpoor women to be using no method of contraception (15 vs. eight percent); among poor teenagers, this proportion reaches 25 percent. The level of contraceptive use at first intercourse among teenage women improved substantially between 1982 and 1988, however, rising from 48 percent to 65 percent. During 1984-1988, almost four in 10 births and almost six in 10 pregnancies among American women were unintended; most of these were mistimed, but 12 percent of births were unwanted ever.(ABSTRACT TRUNCATED AT 250 WORDS)
The hereditary ataxias, also referred to as the spinocerebellar degenerations, comprise a series of clinical manifestations that include ataxia and dysmetria, resulting from the predominant involvement of the cerebellum and its afferent and efferent pathways. These disorders are system degenerations; many of them are specific entities clearly inherited as autosomal dominant or autosomal recessive traits. Although the clinical manifestations and neuropathologic findings of cerebellar disease dominate the spinocerebellar degenerations, there may also be characteristic changes in the basal ganglia, optic atrophy, retinitis pigmentosa, or peripheral nerve disease. There are many gradations from pure cerebellar manifestations to mixed cerebellar and brain-stem disorders, cerebellar and basal ganglia syndromes, and spinal syndromes or peripheral nerve disease. The clinical picture may be consistent in one family, but sometimes there is a characteristic syndrome in the majority of family members and an entirely different disorder in one or several members.
We reviewed the clinical characteristics of 10 patients with thyrotoxic periodic paralysis. In these patients, a relatively uniform group of young men, the periodic paralysis developed nearly concurrently with the onset of hyperthyroidism. The attacks were precipitated most frequently by rest and by exercise and, occasionally, by ingestion of a large carbohydrate load. In each patient, the paralysis resolved on return of euthyroidism. The approximate incidence rate for thyrotoxic periodic paralysis in our largely white North American patient population (all hyperthyroidism cases) ranged from 0.1% to 0.2%, which is one tenth the rate reported for Oriental populations. In 7 patients, electrodiagnostic testing revealed characteristic changes in compound muscle action potential amplitude in response to exercise of the muscle being tested.
Five of 6 male siblings were affected by a progressive myopathy beginning in early childhood. Muscle biopsies in all patients showed the characteristic changes of inclusion body myositis. Computerized tomography and magnetic resonance imaging revealed a markedly abnormal appearance of cerebral white matter in the 4 affected patients tested, but clinical and other laboratory examinations failed to demonstrate evidence of central white matter dysfunction. Muscle biopsies and brain imaging were normal in all clinically unaffected family members. On the basis of the genetics, muscle biopsy findings and cerebral white matter changes, we conclude that this constellation represents a hitherto undescribed syndrome.
Lecithin: cholesterol acyltransferase (LCAT) is an enzyme that catalyzes the esterifying reaction of cholesterol in plasma high density lipoprotein (HDL). Deficiency of LCAT is a rare hereditary disease characterized by several clinical symptoms such as proteinuria, corneal opacity, and anemia due to a shortened life span of erythrocytes. In this communication, we report a case of 40 year-old female patient of LCAT deficiency. She visited a hospital for work-up of proteinuria, corneal opacity and anemia. Activity of her serum LCAT was found to be extremely low, and characteristic changes in plasma lipids due to deficiency of LCAT was observed: those were marked decreases in HDL-cholesterol, degree of esterification in serum cholesterol, and apoprotein A-I, A-II, B and C-II levels. The diagnosis of LCAT deficiency was finally made. We studied about histopathological changes in the patient's kidney, and erythrocyte membrane lipid composition and fluidity. Histopathological findings in renal biopsy were follows: a) Light microscopy showed spherical deposits stained with periodic acid-Schiff in mesangial matrix and adjacent capillary loops, and hyaline deposits in arterioles, b) Electron microscopy showed vacuoles in mesangial matrix and along the glomerular basement membranes. In erythrocyte membrane lipids, increase of cholesterol to phospholipid molar ratio was evident, being accompanied by changes in phospholipid fractions: increase of phosphatidylcholine, and decreases of phosphatidylethanolamine, sphingomyelin and lysophosphatidylcholine. In phospholipid acyl chains, increase of C18:2 and decreased of C18:1 were evident in the patient. Erythrocyte membrane fluidity was found to be decreased in the patient in a measurement by pyrene, probably being related to the changes in membrane lipid composition.(ABSTRACT TRUNCATED AT 250 WORDS)
Two unique patients with hyperthyroidism are presented here. A 48-year-old woman with a family history of Graves' disease was first evaluated and treated for a toxic adenoma with radioactive iodine. Three months later, she was again hyperthyroid, was found to have a diffuse thyromegaly, and was treated with radioactive iodine for the second time. This patient represents, to our knowledge, the first reported case of two sequential forms of endogenous hyperthyroidism. The second patient, a 64-year-old woman, had well-documented hypothyroidism that was presumably primary. She was admitted in thyroid storm and subsequently died. The autopsy disclosed characteristic changes in the thyroid of hyperthyroidism and a normal pituitary gland. The timing of the onset of Graves' disease with hyperthyroidism cannot be accurately determined.
Twenty-three children and young persons with a congenital long QT syndrome were identified; the median age at the time of referral was 10 years (range 4 days to 19 years) and 14 patients (61%) had a family history of the syndrome. Among the 19 patients with symptoms, the initial symptom was syncope in 13 (69%), aborted sudden death in 5 (26%) and near drowning in 1 (5%). There were three deaths during a combined follow-up period of 67 patient-years (average annual mortality rate 4.5%). Patients who did not respond to therapy with a beta-adrenergic blocker and those who died were significantly younger than the remaining patients at the time of diagnosis (p less than or equal to 0.05 for both). Analysis of 44 treadmill exercise tests performed by 16 patients revealed significant prolongation of the median corrected QT (QTc) interval in response to exercise, with maximal prolongation present after 2 min of recovery (median QTc interval 0.52 s versus a baseline value of 0.47 s, p less than 0.001). Characteristic changes in T wave configuration were noted in 8 of 15 patients on at least one occasion during ambulatory Holter electrocardiographic monitoring, including T wave alternation in two patients, both of whom died shortly afterward. It is suggested that the congenital long QT syndrome is associated with a significant mortality rate in childhood despite the use of conventional therapy in symptomatic patients. Ambulatory Holter monitoring and treadmill exercise testing may be helpful, both in confirming the diagnosis of a long QT syndrome and in monitoring the adequacy of treatment.(ABSTRACT TRUNCATED AT 250 WORDS)
OBJECTIVE: Neutrophil accumulation is one of the characteristic changes observed in uterine cervical stroma at term pregnancy, but chemotactic activity in the tissue is obscure. Our study examined the existence and production of chemotactic factor in the rabbit uterine cervix. STUDY DESIGN: Uterine cervical explants of rabbits at term pregnancy and nonpregnant rabbits were cultured with and without interleukin-1 alpha. Rat neutrophilic chemotaxis in culture media was evaluated with a Boyden chamber. RESULTS: Tissue extract from the pregnant rabbit uterine cervix at term pregnancy contained more chemoattractive activity than the nonpregnant cervix. Production of chemoattractant from cultured rabbit cervical explants at term pregnancy was also higher than that from nonpregnant explants. The addition of interleukin-1 alpha to the culture system promoted its production. This chemoattractant was characterized as a true chemotactic factor and heat-stable and trypsin-sensitive protein with an apparent relative molecular mass of 16,200. So far, these properties are very similar to those of the interleukin-8 family. Rabbit uterine cervical fibroblast is characterized as a chemotactic factor-producing cell in the rabbit uterine cervix. CONCLUSION: These results indicate that interleukin-8-like chemotactic factor participates in the cervical ripening at term pregnancy and that the production of this factor is controlled effectively by interleukin-1.
Five actively secreted proteins (MPT32, MPT45, MPT51, MPT53, and MPT63) and the MPT46 protein were purified to homogeneity from Mycobacterium tuberculosis culture fluid and compared with proteins previously purified by ourselves and other investigators. Antisera were obtained by immunization of rabbits with all of the newly isolated proteins identified to be immunogenic. Two-dimensional electrophoresis of culture fluids obtained each week for 2 to 10 weeks of culturing of M. tuberculosis revealed characteristic changes, permitting identification of two distinct groups of proteins being actively secreted from the mycobacterial cells or appearing later in the culture fluids as a result of the release of soluble proteins from the cytosol after lysis of bacteria. The N-terminal amino acid sequences of five MPTs were shown to be identical to those of proteins previously isolated by other investigators and given different designations, and five new sequences are given. These sequences and the use of the antisera may serve to identify these proteins with mycobacterial constituents isolated by other investigators. The previously identified but not isolated MPT45 protein was shown to correspond to the C component of the antigen 85 complex. The 27-kDa MPT51 protein was demonstrated to cross-react with the three components of the antigen 85 complex, and the N-terminal amino acid sequences of MPT51 and MPT59 showed 60% homology. This finding and the extensive cross-reactivity between the components of the antigen 85 complex may indicate that there is a family of closely related secreted proteins in mycobacteria.
Blood monocytes (MO) undergo maturation into macrophages (MAC) upon migration from the capillary bed to tissue sites of inflammation where they are exposed to environmental signals. Functional competence and phenotype heterogeneity is the result of both differentiation-inducing and -activating events. In vitro, MO to MAC maturation is induced by serum factors, can be followed by the expression of specific maturation-associated antigens and is accompanied by a characteristic change in the secretory repertoire of MAC in comparison to MO. Here we report that bacterial lipopolysaccharides (LPS) at subnanogram quantities very effectively inhibited the serum-induced maturation of human MO in vitro. At the same time LPS induced the up-regulation of CD14 antigens. The lipid A moiety was shown to be responsible for this novel biological activity of the LPS molecule. Inhibition of maturation was not due to secondary LPS-induced signals like interleukin (IL)-1, IL-6, tumor-necrosis factor (TNF)-alpha or interferon (IFN)-alpha--even though the latter by itself suppressed MAC maturation in vitro. The inhibitory activity of IFN-alpha could be abolished by neutralizing anti-IFN-alpha antibodies whereas these antibodies had no effect on LPS-induced suppression of MAC maturation. Functional analysis of LPS-treated MO long-term cultures showed that the pattern of secretory products released was similar to that of freshly-isolated immature blood MO: compared with mature MAC, LPS-treated MO released high amounts of IL-6 but significantly less TNF-alpha, neopterin, lysozyme and beta-2-microglobulin. At the same time, in LPS-treated MO cultures the MAC maturation-associated molecules alpha-2-macroglobulin and fibronectin could be detected only in trace amounts. The ability to secrete IL-1, however, was lost both in control as well as in LPS-treated MO cultures. The results indicate that endotoxins may influence the biology of the MO/MAC system distinctively: they not only induce a functional activation but also interfere with the ontogeny of this cell family.
We reviewed the clinical and pathological data on 319 neuronal ceroid lipofuscinosis (NCL) cases to determine the degree of variability within the different forms and among and within families. Thirty-six cases (11.3%) were the infantile form; 116 cases (36.3%), late infantile; 163 cases (51.1%), juvenile; and four cases (1.3%), the adult form (Kufs disease). Clinical variability was found in all forms studied, but was most striking in the juvenile and late infantile forms of NCL. The expected initial findings of seizures, dementia, blindness, or motor impairment were evident in 255 cases (80%), and rarer, less typical initial neurological symptoms were seen mainly in the 64 cases (20%) of the juvenile form: behavior abnormalities (18/64), psychoses (12/64), neuropathy (2/64), involuntary movements (15/64), ataxia (9/64). Six juvenile and two adult cases had no detectable impairment of vision. All 319 NCL cases had skin or conjunctive biopsies or buffy coats that showed the characteristic ultrastructural abnormalities of NCL. Variability was evident in 16.7% in that a combination of fingerprint, curvilinear, and membranous profile inclusion bodies was observed in storage lysosomes, although one type of inclusion was distinctly predominant for each form. Postmortem examination of brains of 19 NCL cases (three with the infantile form, six with the late infantile form, nine with the juvenile form, and one with the adult form) revealed characteristic changes. Sixteen of the 19 NCL brains (84%) showed pathological variability in that they contained more than one kind of characteristic inclusion body in the neuronal lysosomal storage compartment. In all 19 NCL brains, small amounts of aging lipofuscin were also found.(ABSTRACT TRUNCATED AT 250 WORDS)
The effects of vaginal devices releasing levonorgestrel (LNG) at a constant rate of approximately 20 micrograms/24 hrs on the human endometrium were studied in a group of 69 normally menstruating women during a period of 90 days of continuous use. Peripheral blood samples were withdrawn three times weekly starting at day 10 of a pretreatment (control) cycle and then three times weekly from day 60 to 90 of the treatment period. The levels of LNG, estradiol, progesterone and sex hormone binding globulin (SHBG) were analyzed by radioimmunoassay techniques. Endometrial biopsies were obtained in the luteal phase of the pretreatment cycle and on day 84-87 of the treatment period. Increased bleeding occurred in most subjects exposed to the LNG-releasing device; the mean number of bleeding and spotting days was 26.4 +/- 8.9 S.D. which exceeded that found in their control cycle. Morphometric analyses of the endometrial biopsies using 9 quantitative indices, revealed highly significant changes in glands and stroma following the use of the LNG-releasing vaginal device. Irrespective of the circulating steroid levels, the administration of LNG significantly diminished the glandular diameter (p less than 0.001), reduced the volume density of the glands (p less than 0.001) and of the glandular epithelium (p less than 0.001). and modified the ratio glandular epithelium: glands (p less than 0.001). It is concluded that levonorgestrel released at a rate of 20 micrograms/24 hrs, induces characteristic changes in the histologic structure of the human endometrium. Although no simple correlation has been found between any of the endometrial indices and the numbers of bleeding/spotting days or bleeding days, the changes as such may represent biochemical alterations which could be predisposing factors for intermenstrual bleeding and spotting. To prove a causal relationship between morphological and biochemical changes and changes in bleeding patterns, further in-depth studies may be required.
Characteristic changes in cervico-vaginal fluid (CVF) volume which occur during the menstrual cycle might be used to detect the fertile phase. Twenty-five normal women were asked to withdraw CVF and measure its volume at home using a small, disposable, graduated vaginal aspirator. In 16 cycles day 0 (ovulation) was defined as the day of maximum follicular diameter according to serial ultrasound examination. A rise in CVF volume occurred between day -9 and -2 and a peak between day -4 and 0. In these sixteen, and in a further 72 cycles, day 0 (time of maximum fertility) was taken as the day of peak cervical mucus secretion. CVF volume rose, on the average, on day -6.2 (range -17 to -2) and peaked on day -0.8 (range -5 to +2). In two cycles, no rise and peak were identified. Changes in CVF volume were easy to recognise and could be useful to couples wishing to achieve pregnancy.