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Sample records for familial hyperkalemic periodic

  1. Molecular basis for hyperkalemic periodic paralysis.

    Science.gov (United States)

    Brown, R H

    At least one form of periodic paralysis is a direct consequence of a mutation in a skeletal muscle, voltage-sensitive sodium channel--it was observed that many individual with this disease developed low serum potassium levels during paralytic episodes. Some families had hyperkalemic paralysis with serum potassium levels of 6 or 7 mEg/L during paralytic crises. In both hypokalemic and hyperkalemic paralysis one of the precipitants is a period of rest after exertion. In hypokalemic periodic paralysis carbohydrates may initiate weakness. In both hyper- and hypokalemic forms, the disorder is inherited as an autosomal dominant trait. During hypokalemic and hyperkalemic paralysis, one might respectively anticipate muscle hyperpolarization or depolarization. Has been observed a potassium-related abnormality of sodium conductance in the pathogenesis at least of the hyperkalemic form of periodic paralysis. The fact that TTX reverses the physiological defect suggested the hypothesis that the primary problem might be a mutation in a TTX-sensitive sodium channel. The protein consists of some 2000 amino acids with characteristic intracytoplasmic and extracellular domains as well a four remarkably conserved membrane spanning domains, each composed of six transmembrane of a polymorphism of the human sodium channel with hyperkalemic paralysis. When multipoint analysis was used to test for coinheritance of the disease with both Na-2 and growth hormone polymorphisms, a lod score of 7 was obtained. That is, the ratio of the probability of linkage to non-linkage is 10 million to one. When extracellular potassium is increased to 10 mM, the affected myotubes demonstrate strikingly abnormal channel behavior characterized by prolonged open times or repetitive opens throughout the voltage step. Potassium implicate as a primary factor triggering an abnormal sodium channel gating mode and, as a result, aberrant sodium current behavior. It was estimated that, for the normal channel, the

  2. Hyperkalemic periodic paralysis

    Science.gov (United States)

    ... Rott K, Rudel R, Lehmann-Horn F. Muscle channelopathies: myotonias and periodic paralyses. In: Darras BT, Jones ... Elsevier; 2015:chap 38. Kerchner GA, Ptacek LJ. Channelopathies: episodic and electrical disorders of nervous system. In: ...

  3. Fibromyalgia in hyperkalemic periodic paralysis.

    Science.gov (United States)

    Götze, F R; Thid, S; Kyllerman, M

    1998-01-01

    A 43-year-old woman presented at the age of 38 with joint pains and muscle stiffness. Tender points were found fulfilling ACR criteria (1) for fibromyalgia. She had well developed muscles and decreasing muscle power since the age of 35. Muscle pains increased after exercise. Her 10-year-old son had similar symptoms and one paralytic attack. Muscle pain and fatigue increasing with age were found by history in three close relatives. Forearm cold water test produced myotonia in both mother and son. Electromyography was normal and muscle biopsy showed minor unspecific changes. Biochemical investigation of muscle mitochondrial function was normal. Peroral potassium load test produced complete muscle paralysis at a potassium serum level of 5.0 mmol/l. Autosomal dominant hyperkalemic periodic paralysis was diagnosed. Frequent carbohydrate enriched meals, peroral bendroflumethiazide and restriction to submaximal exercise improved muscle and joint pain. Salbutamol peroral spray relieved the periodic weakness.

  4. DIAGNOSTICATION OF HYPERKALEMIC PERIODIC PARALYSIS IN HORSES

    National Research Council Canada - National Science Library

    S.E. GEORGESCU; MARIA ADINA MANEA; ANCA DINISCHIOTU; C.D. TESIO; MARIETA COSTACHE

    2013-01-01

    Hyperkalemic periodic paralysis (HYPP) is a muscle disease which has been firstreported in 1985 in the USA, in a group of 4 horses with episodic weaknessassociated with intermittent serum hyperkalemia...

  5. Equine hyperkalemic periodic paralysis: review and implications.

    Science.gov (United States)

    Naylor, J M

    1994-05-01

    The purpose of this review is to present an up-to-date summary of the signs, diagnosis, treatment, and implications of equine hyperkalemic periodic paralysis. The review encompasses all original articles published between 1986 and early 1993. Hyperkalemic periodic paralysis is the result of a genetic mutation in the skeletal muscle sodium channel gene. It is inherited as an autosomal dominant trait; most affected horses are heterozygotes. The classical signs are muscle fasciculation, spasm, and weakness associated with hyperkalemia. However, these signs are only rarely observed in affected horses. Potential sequelae to attacks are abrasions and involuntary recumbency; these problems are not specific for hyperkalemic periodic paralysis, but they occur more frequently in hyperkalemic periodic paralysis-affected horses. It is also likely that hyperkalemic periodic paralysis results in greater muscle mass. There are suggestions that homozygotes may be more severely affected and show signs of upper respiratory obstruction as foals. The practitioner needs to be aware of the tests for hyperkalemic periodic paralysis, and their limitations, so that he can properly diagnose this condition. The industry has the difficult problem of deciding whether or not testing should be mandatory and the fate of positive horses.

  6. Familial hyperkalemic periodic paralysis caused by a de novo mutation in the sodium channel gene SCN4A.

    Science.gov (United States)

    Han, Ji-Yeon; Kim, June-Bum

    2011-11-01

    Familial hyperkalemic periodic paralysis (HYPP) is an autosomaldominant channelopathy characterized by transient and recurrent episodes of paralysis with concomitant hyperkalemia. Mutations in the skeletal muscle voltage-gated sodium channel gene SCN4A have been reported to be responsible for this disease. Here, we report the case of a 16-year-old girl with HYPP whose mutational analysis revealed a heterozygous c.2111C>T substitution in the SCN4A gene leading to a Thr704Met mutation in the protein sequence. The parents were clinically unaffected and did not have a mutation in the SCN4A gene. A de novo SCN4A mutation for familial HYPP has not previously been reported. The patient did not respond to acetazolamide, but showed a marked improvement in paralytic symptoms upon treatment with hydrochlorothiazide. The findings in this case indicate that a de novo mutation needs to be considered when an isolated family member is found to have a HYPP phenotype.

  7. [Hyperkalemic periodic paralysis: a Spanish family with the p.Thr704Met mutation in the SCN4A gene].

    Science.gov (United States)

    Narberhaus, B; Cormand, B; Cuenca-León, E; Ribasés, M; Monells, J

    2008-09-01

    Hyperkalemic periodic paralysis (HYPP) is an autosomal dominant disease characterized by recurrent episodes of muscular weakness with increased blood potassium levels. Here we present the clinical, analytical, neurophysiological and genetic findings of family with eight affected individuals, five of which were available for study. The five patients were subjected to complete anamnesis, neurological examination, routine blood analysis and genetic study. Two of the patients were also examined both at the clinical and neurophysiological levels. In one case, the potassium levels were determined during a crisis. Almost all patients presented 2 to 3 episodes of muscle weakness of the limbs per day of 30-45 min, and showed calf hypertrophy. During the observed episodes, the paralysis was massive in the lower limbs and the patients showed generalized osteotendinous areflexia. The potassium levels of the probandus measured during one of the episodes were elevated. The genetic analysis showed that all the affected individuals carried the p.Thr704Met mutation in the a subunit of the skeletal muscle sodium channel, encoded by the SCN4A gene. Our findings correlate well with those reported previously in HYPP, although the frequency of the episodes is exceptionally high in our family. HYPP is a channelopathy caused by mutations in the SCN4A gene, although molecular alterations have only been identified in 70 % of the patients. The affected members of the studied family bear a frequent mutation, p.Thr704Met, associated with a severe presentation of the disease.

  8. Familial hyperkalemic periodic paralysis caused by a de novo mutation in the sodium channel gene SCN4A

    Directory of Open Access Journals (Sweden)

    Ji-Yeon Han

    2011-11-01

    Full Text Available Familial hyperkalemic periodic paralysis (HYPP is an autosomaldominant channelopathy characterized by transient and recurrent episodes of paralysis with concomitant hyperkalemia. Mutations in the skeletal muscle voltage-gated sodium channel gene SCN4A have been reported to be responsible for this disease. Here, we report the case of a 16-year-old girl with HYPP whose mutational analysis revealed a heterozygous c.2111C>T substitution in the SCN4A gene leading to a Thr704Met mutation in the protein sequence. The parents were clinically unaffected and did not have a mutation in the SCN4A gene. A de novo SCN4A mutation for familial HYPP has not previously been reported. The patient did not respond to acetazolamide, but showed a marked improvement in paralytic symptoms upon treatment with hydrochlorothiazide. The findings in this case indicate that a de novo mutation needs to be considered when an isolated family member is found to have a HYPP phenotype.

  9. [HYPP--hyperkalemic periodic paralysis in horses].

    Science.gov (United States)

    Zeilmann, M

    1993-12-01

    A literature review of the clinical syndrome HYPP (Hyperkalemic periodic paralysis) affecting Quarter Horses is given. HYPP is characterized by sporadic attacks of muscle tremors, weakness and/or collapse, lasting for variable periods of time. Diagnosis is based on physical findings in association with hyperkalemia. In horses with HYPP, the regulation of ion transport through the sodium channels in the muscle cells occasionally fails, causing uncontrollable muscle twitching. Further investigations into molecular genetics reveals a mutation in the gene responsible for sodium and potassium regulation. The identification of this gene mutation is the basis for the blood test used to diagnose HYPP. HYPP is inherited as an autosomal dominant trait. Treatment of HYPP attacks by intravenous application of calcium gluconate, bicarbonate and glucose results in rapid recovery. Consequent dietary management and daily administration of acetazolamide effectively controls the disease.

  10. Defective aldosterone synthesis associated with hyperkalemic periodic paralysis.

    Science.gov (United States)

    Allen, D B

    1993-03-01

    To describe defective aldosterone biosynthesis (corticosterone methyl oxidase type II [CMO-II] deficiency) in a kindred with hyperkalemic periodic paralysis. Tertiary care hospital in Madison, Wis. Individuals studied included a female infant with failure to thrive, hyponatremia, and hyperkalemia; the infant's asymptomatic mother and father; and a maternal aunt and grandmother with hyperkalemic periodic paralysis. Mineralocorticoid synthetic pathways were analyzed with synthetic adrenocorticotropin stimulation. In one patient with hyperkalemic periodic paralysis, acetazolamide sodium therapy was discontinued and replaced with fludrocortisone acetate therapy. Impaired aldosterone synthesis with marked accumulation of mineralocorticoid precursors 18-hydroxycorticosterone and corticosterone indicated severe CMO-II deficiency in the infant. In her relatives and parents, baseline aldosterone levels (74 to 111 pmol/L) were low (reference range, 194 to 830 pmol/L, a nonstricted sodium diet). Serum 18-hydroxycorticosterone levels (442 to 1021 pmol/L) were normal (reference range, 138 to 1270 pmol/L), but ratios of 18-hydroxycorticosterone to aldosterone were abnormally elevated (4.5 to 13.7; reference range, 2.65 +/- 1.86), indicating deficient CMO-II enzyme activity. Acetazolamide therapy was substituted with fludrocortisone therapy in the maternal aunt without return of paralytic symptoms. This association of hyperkalemic periodic paralysis with CMO-II deficiency and resolution of paralytic episodes with fludrocortisone therapy suggests a contribution of defective mineralocorticoid-mediated potassium homeostasis to the pathogenesis of hyperkalemic periodic paralysis.

  11. Genetic study of hyperkalemic periodic paralysis in horses.

    Science.gov (United States)

    Spier, S J; Carlson, G P; Harrold, D; Bowling, A; Byrns, G; Bernoco, D

    1993-03-15

    Four Quarter Horses (1 stallion, 3 mares) with hyperkalemic periodic paralysis were mated to unaffected horses to determine the genetic basis of the disease. The affected stallion was bred to 11 unaffected mares (4 Quarter Horses, 1 Arabian, 2 Standardbreds, and 4 Thoroughbreds). The 3 affected mares were bred to an unaffected Quarter Horse stallion. Of the 15 offspring obtained from these matings, 9 were affected with hyperkalemic periodic paralysis, and 6 were unaffected, consistent with an autosomal dominant mode of inheritance. Diagnosis was established by results of oral administration of potassium chloride and demonstration of characteristic clinical signs accompanied by hyperkalemia. Oral administration of potassium chloride resulted in marked increases in plasma potassium concentrations in affected and unaffected foals, although hyperkalemia was associated with clinical signs of hyperkalemic periodic paralysis in the affected foals. Evaluation of blood samples from affected and unaffected offspring revealed no linkage with erythrocyte and serum markers at 24 loci.

  12. Combined spinal/general anesthesia with postoperative femoral nerve block for total knee replacement in a patient with familial hyperkalemic periodic paralysis: a case report.

    Science.gov (United States)

    Barker, Maria C

    2010-06-01

    Familial hyperkalemic periodic paralysis (HYPP) is a rare genetic disorder in which the sodium channels in skeletal muscle cells have altered structure and function. Small elevations in serum potassium lead to inactivation of sodium channels, causing episodic weakness or paralysis. Exposure to cold, anesthesia, fasting, emotional stress, potassium ingestion, and rest after exercise can stimulate an attack. This case report describes a 65-year-old man with HYPP who was admitted for a right total knee arthroplasty. He had a history of arteriosclerotic heart disease and stenting 8 years earlier, previous inferior wall myocardial infarction with ejection fraction of 65%, anxiety, degenerative joint disease, well-controlled type 2 diabetes mellitus, and a body mass index of 53.3 kg/m2. A combined spinal/general anesthetic with a femoral nerve block for postoperative pain control was chosen. Careful attention was given to monitoring and maintenance of core temperature, use of insulin and glucose to maintain normokalemia, and carbohydrate loading the night before surgery. The patient recovered from the anesthetic without complication and had pain relief for approximately 22 hours postoperatively because of the femoral nerve block. The patient was without weakness or paralysis related to HYPP in the postanesthesia care unit or throughout his hospitalization.

  13. Genotype-phenotype correlation and therapeutic rationale in hyperkalemic periodic paralysis.

    Science.gov (United States)

    Jurkat-Rott, Karin; Lehmann-Horn, Frank

    2007-04-01

    Familial hyperkalemic periodic paralysis (PP) is a dominantly inherited muscle disease characterized by attacks of flaccid weakness and intermittent myotonia. Some patients experience muscle stiffness that is aggravated by cold and exercise, bordering on the diagnosis of paramyotonia congenita. Hyperkalemic PP and paramyotonia congenita are allelic diseases caused by gain-of-function mutations of the skeletal muscle sodium channel, Nav1.4, which is essential for the generation of skeletal muscle action potentials. In this review, the functional and clinical consequences of the mutations and therapeutic strategies are reported and the differential diagnoses discussed. Also, the question is addressed of whether hyperkalemic PP is truly a different entity than normokalemic PP. Additionally, the differential diagnosis of Andersen-Tawil syndrome in which hyperkalemic PP attacks may occur will be briefly introduced. Last, because hyperkalemic PP has been described to be associated with an R83H mutation of a MiRP2 potassium channel subunit, evidence refuting disease-causality in this case will be discussed.

  14. Hyperkalemic periodic paralysis associated with multiple sleep onset REM periods.

    Science.gov (United States)

    Iranzo, A; Santamaria, J

    1999-12-15

    A 24-year-old man with sporadic hyperkalemic periodic paralysis (HPP) presented with moderate excessive daytime sleepiness and transitory episodes of weakness which occurred during and after sleep. Multiple sleep latency test (MSLT) demonstrated the presence of five sleep onset REM periods (SOREMPs) and a sleep latency of five minutes. Treatment with a diuretic which decreases serum potassium resolved all the clinical symtomps and a new MSLT showed the absence of SOREMPs and a sleep latency of 13.5 minutes. To our knowledge, the patient herein reported is the first case that associates sleep abnormalities and multiple SOREMPs with HPP. Furthermore, the present case suggests that SOREMPs may be explained by an increased extracellular potassium conductance related to HPP.

  15. Hyperkalemic periodic paralysis episode during halothane anesthesia in a horse.

    Science.gov (United States)

    Bailey, J E; Pablo, L; Hubbell, J A

    1996-06-01

    A 7-month-old Quarter Horse filly was admitted for surgical repair of a right olecranon fracture. Anesthesia was achieved with xylazine hydrochloride, guaifenesin, ketamine hydrochloride, and halothane. Two and a half hours after induction of anesthesia, myotonia, muscle fasciculations, and sweating, concurrent with high serum potassium concentration and associated electrocardiographic changes consistent with hyperkalemic periodic paralysis, were observed. Treatment included intermittent positive-pressure ventilation, changing intravenous administration of fluids from lactated Ringer's solution to 0.9% NaCl solution, and administration of calcium gluconate, glycopyrrolate, dopamine, and sodium bicarbonate. Clinical signs resolved with the return of serum potassium concentrations to the reference range. The horse was confirmed to be heterozygous for hyperkalemic periodic paralysis by DNA testing.

  16. A CLINICAL AND NEUROELECTROPHYSIOLO-GICAL STUDY OF HYPERKALEMIC PERIODIC PARALYSIS

    Institute of Scientific and Technical Information of China (English)

    高秀贤; 汤晓芙; 杜华; 李本红

    1995-01-01

    A case of atypical hyperkalemic periodic paralysis is reported. The diagnosis was confirmed by hyperkalemic test, cold water test, and differentiation of attack period and rest period by the measurement of motor nerve conduction amplitude. Etio-pathology of this disease is discussed from the view of neuroelec-trophysiology.

  17. [Hyperkalemic periodic paralysis or congenital paramyotonia: an attempt of treatment with phenytoin].

    Science.gov (United States)

    Tomczykiewicz, K; Tutaj, A; Jaszczuk, E; Balcewicz, J

    1996-01-01

    Authors describe 2 patients with hyperkalemic period paralysis where positive therapeutic effect was achieved after treatment with phenytoin. They compare the features of paramyotonia congenital with hyperkalemic period paralysis and they think that they are the same disease with different clinical signs.

  18. [HYPP: hyperkalemic periodic paralysis in the horse].

    Science.gov (United States)

    Sloet van Oldruitenborgh-Oosterbaan, M M

    1999-03-15

    Hyperkalaemic periodic paralysis(HYPP) is characterized by intermittent episodes of muscular tremor, weakness, and collapse, and is probably caused by abnormal electrolyte transport in the muscle cell membrane. During an episode of HYPP, most animals are severely hyperkalaemic. HYPP is a hereditary disease and occurs only in American Quarter horses or crossbreds. Because these horses are now being imported into the Netherlands, HYPP should be included in the differential diagnosis of horses showing signs of muscle tremor, paresis, or paralysis. The present article reviews the literature on HYPP and describes a case showing typical signs of the disease.

  19. Analysis in a large hyperkalemic periodic paralysis pedigree supports tight linkage to a sodium channel locus.

    Science.gov (United States)

    Ptacek, L J; Tyler, F; Trimmer, J S; Agnew, W S; Leppert, M

    1991-01-01

    Hyperkalemic periodic paralysis (HYPP) is an autosomal dominant muscle disease with electrophysiological abnormalities suggesting a defect in a voltage-gated sodium channel (NaCh) gene. A human NaCh gene was recently shown to cosegregate with the disease allele in a family with HYPP. Using an independent clone, we have demonstrated close genetic linkage between an NaCh gene and the HYPP locus in another family. With physiological data demonstrating abnormal NaCh function in HYPP patients, the absence of any obligate recombinations in the two families strengthens the argument that this NaCh gene is the site of the defect in this disorder. PMID:1651050

  20. [Familial hyperkalemic periodic paralysis: a brief review of the adult human skeletal muscle sodium channel and the application of LA-PCR to the SCN4A gene analysis].

    Science.gov (United States)

    Sakoda, S; Nakagawa, M; Arimura, Y; Arimura, K; Osame, M

    1997-12-01

    Recent work has revealed that familial hyperkalemic periodic paralysis, paramyotonia congenita and other non-dystrophic myotonias result from point mutations in the gene encoding the alpha-subunit of the adult human skeletal muscle sodium channel (SCN4A). Sodium channel myotonias are a diverse group of skeletal muscle disorders that share a common pathophysiological mechanism: all are caused by impaired rapid inactivation of skeletal muscle sodium channel. Clinical studies, pharmacology, electrophysiology and molecular genetics have contributed to an elucidation of the genotype-phenotype correlation within these disorders. This article briefly reviews recent advances in our understanding of skeletal muscle sodium channel and sodium channel myotonias. The application of LA-PCR to the SCN4A gene analysis is also referred.

  1. Management of a patient with hyperkalemic periodic paralysis requiring coronary artery bypass grafts.

    Science.gov (United States)

    Patangi, Sanjay Orathi; Garner, Mathew; Powell, Hazel

    2012-01-01

    Hyperkalemic periodic paralysis (HPP) is an autosomal-dominant inherited muscle disease characterized by episodes of flaccid weakness and intermittent myotonia. There are no previous reports in the literature about anesthesia for cardiac surgery with cardiopulmonary bypass in this disorder. We describe perioperative anesthetic management for on-pump coronary artery bypass grafting in a 75-year-old man with a history of hyperkalemic periodic paralysis. This case report outlines our management strategy and the issues encountered during the perioperative period.

  2. Understanding the physiology of the asymptomatic diaphragm of the M1592V hyperkalemic periodic paralysis mouse

    National Research Council Canada - National Science Library

    Ammar, Tarek; Lin, Wei; Higgins, Amanda; Hayward, Lawrence J; Renaud, Jean-Marc

    2015-01-01

    The diaphragm muscle of hyperkalemic periodic paralysis (HyperKPP) patients and of the M1592V HyperKPP mouse model rarely suffers from the myotonic and paralytic symptoms that occur in limb muscles...

  3. Management of a patient with hyperkalemic periodic paralysis requiring coronary artery bypass grafts

    Directory of Open Access Journals (Sweden)

    Sanjay Orathi Patangi

    2012-01-01

    Full Text Available Hyperkalemic periodic paralysis (HPP is an autosomal-dominant inherited muscle disease characterized by episodes of flaccid weakness and intermittent myotonia. There are no previous reports in the literature about anesthesia for cardiac surgery with cardiopulmonary bypass in this disorder. We describe perioperative anesthetic management for on-pump coronary artery bypass grafting in a 75-year-old man with a history of hyperkalemic periodic paralysis. This case report outlines our management strategy and the issues encountered during the perioperative period.

  4. Phenytoin increases specific triacylglycerol fatty esters in skeletal muscle from horses with hyperkalemic periodic paralysis.

    Science.gov (United States)

    Fletcher, J E; Erwin, K; Beech, J

    1993-07-01

    Previous studies have demonstrated that phenytoin decreases the levels of triacylglycerols in several tissues other than skeletal muscle. Since phenytoin is clinically effective in several skeletal muscle disorders, triacylglycerol metabolism in skeletal muscle from four normal Quarter horses and four Quarter horses with hyperkalemic periodic paralysis was examined. The horses with hyperkalemic periodic paralysis had low levels of 18:3 in the phospholipids, low levels of 16:0, 16:1 and 18:3 in the free fatty acids and low levels of 20:4 in triacylglycerols. Triacylglycerol levels were increased in skeletal muscle from seven (three controls, four hyperkalemic periodic paralysis) of the eight horses on treatment with oral phenytoin for one week. Instead of an increase in all fatty ester types only 16:0, 16:1, 18:1 and 18:2 were significantly increased. Total lipid phosphorus and the distribution of phospholipid fatty esters and free fatty acids were not significantly altered by phenytoin treatment in most cases. An alteration in triacylglycerol metabolism by phenytoin was also observed in primary cultures of normal equine skeletal muscle radiolabeled with 18:1, but not in those radiolabeled with 18:2. These findings suggest that phenytoin does not just increase the levels of triacylglycerol in skeletal muscle, but alters the utilization and incorporation of fatty esters. These findings suggest a potential involvement of triacylglycerol metabolism in the clinical efficacy of phenytoin in hyperkalemic periodic paralysis.

  5. ANESTHESIA CHARACTERISTICS OF A PATIENT WITH HYPERKALEMIC PERIODIC PARALYSIS--CASE REPORT.

    Science.gov (United States)

    Atanasova, M P; Toshev, Sv; Sokolov, M

    2014-01-01

    We report a case of a successfully conducted anesthesia, without complications of a patient with hyperkalemic periodic paralysis who underwent elective laparoscopic cholecystectomy for chronic calculous cholecystitis. The perioperative considerations, the characteristics of anesthesia, and the factors that can lead to complications in this rare genetic disorder are discussed.

  6. A proposed mutation, Val781Ile, associated with hyperkalemic periodic paralysis and cardiac dysrhythmia is a benign polymorphism.

    Science.gov (United States)

    Green, D S; Hayward, L J; George, A L; Cannon, S C

    1997-08-01

    Twenty different point mutations have been identified in the gene coding for the alpha subunit of the adult skeletal muscle sodium channel in families with hyperkalemic periodic paralysis, paramyotonia congenita, and the potassium-aggravated myotonias. One novel mutation (Val(781)Ile) was reported in an adopted boy with potassium-sensitive weakness and cardiac dysrhythmia. The confidence in establishing this rare amino acid substitution as a causative mutation was limited by the absence of family members for segregation analysis. Functional expression studies herein show that Val(781)Ile is most likely a benign polymorphism and not a disease-associated mutation.

  7. Use of corticotropin-induced potassium changes in the diagnosis of both hypo- and hyperkalemic periodic paralysis.

    Science.gov (United States)

    Streeten, D H; Speller, P J; Fellerman, H

    1993-01-01

    Both hypo- and hyperkalemic periodic paralysis may be difficult to diagnose conclusively when patients are not seen during attacks. Since paralysis of both types can be induced with ACTH, we have determined the frequency of this response in small groups of patients. Weakness or paralysis with appropriate changes in serum K concentration resulted from ACTH gel administration, in 4 of 5 patients with known hypokalemic periodic paralysis and in 3 of 3 patients with hyperkalemic paralysis. No adverse effects of the test were observed, but hospitalization and careful monitoring were necessary. The response to ACTH appears to be a sensitive, useful aid to the diagnosis of both hypo- and hyperkalemic periodic paralysis.

  8. Long-term effectiveness of acetazolamide on permanent weakness in hyperkalemic periodic paralysis.

    Science.gov (United States)

    Dejthevaporn, Charungthai; Papsing, Chutima; Phakdeekitcharoen, Bunyong; Jaovisidha, Suphaneewan; Phudhichareonrat, Suchart; Witoonpanich, Rawiphan; Pulkes, Teeratorn

    2013-05-01

    Acetazolamide is commonly used as an empirical treatment for inherited periodic paralyses although some patients may develop deleterious effects. We report a 65 year-old man with hyperkalemic periodic paralysis and late-onset permanent weakness in association with the common T704M mutation in α-subunit, skeletal muscle voltage-gated sodium channel gene. He rapidly recovered from weakness after acetazolamide treatment. Magnetic resonance imaging of thighs comparing pre- and post-treatment revealed a significant increase in muscle bulk. The patient has been without any type of weakness for over 6 years. This data show the remarkable benefit of acetazolamide on permanent weakness of hyperkalemic periodic paralysis in association with the T704M mutation. Copyright © 2013 Elsevier B.V. All rights reserved.

  9. OVER-EXPRESSION OF THE SODIUM CHLORIDE COTRANSPORTER IS NOT SUFFICIENT TO CAUSE FAMILIAL HYPERKALEMIC HYPERTENSION

    OpenAIRE

    McCormick, James A.; Nelson, Joshua H.; Yang, Chao-Ling; Curry, Joshua N.; Ellison, David H.

    2011-01-01

    The sodium chloride co-transporter (NCC) is the primary target of thiazides diuretics, drugs used commonly for long-term hypertension therapy. Thiazides also completely reverse the signs of Familial Hyperkalemic Hypertension (FHHt), suggesting that the primary defect in FHHt is increased NCC activity. To test whether increased NCC abundance alone is sufficient to generate the FHHt phenotype, we generated NCC transgenic mice; surprisingly, these mice did not display an FHHt-like phenotype. Sys...

  10. Inheritance of myotonic discharges in American quarter horses and the relationship to hyperkalemic periodic paralysis.

    Science.gov (United States)

    Naylor, J M; Robinson, J A; Crichlow, E C; Steiss, J E

    1992-01-01

    Electromyography (EMG) was used to detect myotonic discharges in Quarter Horse breeding stock and to follow the results of mating horses with hyperkalemic periodic paralysis (HPP). The studies were performed on two brood mare farms. A total of six breeding stock showed myotonic discharges and 15 were nonmyotonic. Myotonic discharges were seen in five of six horses belonging to the blood line previously implicated as being predisposed to HPP. Two of these horses had shown clinical signs of HPP. Only one of 15 breeding horses unrelated to the HPP predisposed blood line showed myotonic discharges. When both parents were non-myotonic on EMG than the F1 generation (n = 6) were also nonmyotonic. When a stallion with HPP and myotonic discharges was mated to eight nonmyotonic mares over a six year period half the animals of the F1 generation (n = 25) showed myotonic discharges. When both parents showed myotonic discharges four F1 offspring were myotonic and two were nonmyotonic on EMG testing. There was no evidence of sex linkage. The results are consistent with an autosomal dominant mode of inheritance. Hyperkalemic periodic paralysis and myotonic discharges on EMG may be different manifestations of the same underlying defect. PMID:1586896

  11. Laryngeal and pharyngeal dysfunction in horses homozygous for hyperkalemic periodic paralysis.

    Science.gov (United States)

    Carr, E A; Spier, S J; Kortz, G D; Hoffman, E P

    1996-08-15

    Evaluate histories, clinical signs, and laboratory data of 69 horses homozygous by DNA testing for hyperkalemic periodic paralysis (HPP). Cohort study. 69 of 189 horses testing homozygous for HPP between October 1992 and November 1994. Questionnaires addressing signalment, training regimes, medical history, and current status of affected horses were sent to owners, trainers, or attending veterinarians. Data from completed questionnaires were tabulated and evaluated, using descriptive statistics. Sixty-nine (37%) of 189 questionnaires were completed and returned. Clinical episodes of muscle weakness or paralysis varied in severity and frequency from mild muscle fasciculations to recumbency and death. Sixty-three of 68 HPP-affected horses were reported to have had stridor associated with exercise, excitement, stress, or episodes of muscle paralysis. Common endoscopic findings in affected horses included pharyngeal collapse, pharyngeal edema, laryngopalatal dislocation, and laryngeal paralysis. Twelve of 27 horses receiving acetazolamide had decreases in stridor while receiving medication. Most horses testing homozygous for HPP had clinical signs associated with pharyngeal and laryngeal dysfunction. Hyperkalemic periodic paralysis should be included on a differential list for horses examined for signs of laryngeal or pharyngeal dysfunction or stridor. Treatment with acetazolamide may help to control respiratory tract signs associated with this disease.

  12. Studies on hyperkalemic periodic paralysis. Evidence of changes in plasma Na and Cl and induction of paralysis by adrenal glucocorticoids

    Science.gov (United States)

    Streeten, David H. P.; Dalakos, Theodore G.; Fellerman, Herbert

    1971-01-01

    In a 19 yr old male with familial hyperkalemic periodic paralysis, paralysis was consistently induced by the administration of potassium chloride, corticotropin-gel, and a variety of glucocorticoids (dexamethasone, 6-methylprednisolone, triamcinolone) but not by mineralocorticoids (D-aldosterone, deoxycorticosterone) or by adrenocorticotropin (ACTH)-gel plus metyrapone. Induced attacks were virtually identical with spontaneous attacks, being associated, after a latent period of a few hours, with a rise in plasma K+ and HCO3- and a simultaneous fall in plasma Na+ and Cl- concentrations to an extent implying exchange of 1 K+ with 2 Na+ and 2 Cl- between extracellular and intracellular fluid. ACTH-induced paralysis was preceded by rising serum inorganic P, and associated with increased plasma glucose, blood lactate, and serum creatine phosphokinase concentrations. In normal subjects ACTH, cortisol, and triamcinolone administration failed to change plasma electrolytes or strength, while ingestion of KCl produced no weakness and smaller changes in plasma K and Na than in the patient. Since the patient and normal subjects showed the same changes in renal excretion of K after the administration of cortisol and KCl, it seems likely that paralysis in the patient resulted from abnormally slow uptake (and/or excessive loss) of K by the muscle cells, possibly caused by an abnormal “ion-exchange pump.” Normal adrenocortical function and absence of a peak in plasma 11-hydroxycorticoid (11-OHCS) concentration preceding spontaneous paralysis, indicated that spontaneous paralysis did not result from changes in cortisol secretion. Similar hyperkalemic paralysis was precipitated by ACTH-gel in a brother and first cousin of the propositus. Administration of acetazolamide and fludrocortisone reduced the rise in plasma K concentration and prevented the weakness which otherwise invariably followed KCl administration to the patient. He and two close relatives have been completely

  13. Lessons learned from muscle fatigue: implications for treatment of patients with hyperkalemic periodic paralysis.

    Science.gov (United States)

    Renaud, Jean-Marc; Hayward, Lawrence J

    2012-12-01

    Hyperkalemic periodic paralysis (HyperKPP) is a disease characterized by periods of myotonic discharges and paralytic attacks causing weakness, the latter associated with increases in plasma [K(+)]. The myotonic discharge is due to increased Na(+) influx through defective Na(+) channels that triggers generation of several action potentials. The subsequent increase in extracellular K(+) concentration causes excessive membrane depolarization that inactivates Na(+) channels triggering the paralysis. None of the available treatments is fully effective. This paper reviews the capacity of Na(+) K(+)ATPase pumps, KATP and ClC-1 Cl(-) channels in improving membrane excitability during muscle activity and how using these three membrane components we can study future and more effective treatments for HyperKPP patients. The review of current patents related to HyperKPP reinforces the need of novel approaches for the treatment of this channelopathy.

  14. Hyperkalemic periodic paralysis caused by recurring mutation in the adult muscle sodium channel alpha-subunit gene.

    Science.gov (United States)

    Sillén, A; Wadelius, C; Sundvall, M; Ahlsten, G; Gustavson, K H

    1996-01-01

    Linkage studies and mutation analysis were performed in two Swedish families with hyperkalemic periodic paralysis (HYPP), an autosomal dominant inherited disorder characterized by episodic muscle weakness associated with increasing or high levels of serum potassium. The gene for HYPP is the gene encoding the alpha-subunit of the sodium channel of adult human skeletal muscle (SCN4A). SCN4A has been localized on chromosome 17 q closely linked to the human growth hormone gene. Linkage between a microsatellite polymorphism in the SCN4A gene and the disease was shown in two Swedish families (Z = 12.10 theta = 0). Sequence analysis revealed that the two Swedish families have got a C to T transition at position 2188 in the cDNA. At the protein level this Thr 704 to Met mutation is located in the fifth membrane spanning segment of domain II of the protein, as previously described (28). The mutation was linked to different microsatellite alleles regarding both a (GT)n and a (GA)n repeat in the gene. Either the families are related and new mutations have occurred in both microsatellites when the pedigrees were separated or the mutation has arisen independently in the two families analysed. From the mutant alleles characterized so far it seems as if a limited number of mutations is present in this gene.

  15. Evidence for a single pedigree source of the hyperkalemic periodic paralysis susceptibility gene in quarter horses.

    Science.gov (United States)

    Bowling, A T; Byrns, G; Spier, S

    1996-08-01

    The pedigree origin of a base pair substitution in the horse muscle sodium channel gene that confers susceptibility to the muscle disease hyperkalemic periodic paralysis (HYPP) was investigated with a set of 978 Quarter Horses. The horses were chosen at random, based on a collection of blood samples taken between 1989 and 1991 to meet parentage testing requirements, primarily but not exclusively from breeding stallions. The frequency of Quarter Horses positive for the base pair substitution, all heterozygotes, was 4.4%, which corresponds to an allelic frequency of 0.02. All horses positive for the gene traced to a single previously identified stallion as first, second or third generation descendants. A higher frequency of the HYPP susceptibility trait than expected by random occurrence was found among his descendants in this study.

  16. Laryngospasm, dysphagia, and emaciation associated with hyperkalemic periodic paralysis in a horse.

    Science.gov (United States)

    Guglick, M A; MacAllister, C G; Breazile, J E

    1996-07-01

    An 18-month-old Quarter Horse gelding was examined because of weight loss and dysphagia of 1 month's duration. Clinical signs included lethargy, dehydration, ptyalism, and probable aspiration pneumonia. Severe dyspnea and cyanosis were evident after mild exercise. Endoscopy revealed laryngospasm and pharyngospasm. Because clinical signs and endoscopic findings were suggestive of hyperkalemic periodic paralysis (HPP), acetazolamide treatment was instituted. Marked improvement was observed within 48 hours. The horse was determined to be homozygous for HPP. It is likely that this horse's dysphagia, with resultant weight loss and aspiration pneumonia, were clinical manifestations and consequences of HPP. Regardless of age and serum potassium concentration, HPP should be considered as a differential diagnosis for pharyngeal and laryngeal abnormalities and dysphagia in horses with Quarter Horse breeding.

  17. Paramyotonia congenita and hyperkalemic periodic paralysis map to the same sodium-channel gene locus.

    Science.gov (United States)

    Ptacek, L J; Trimmer, J S; Agnew, W S; Roberts, J W; Petajan, J H; Leppert, M

    1991-01-01

    Paramyotonia congenita (PC), an autosomal dominant muscle disease, shares some clinical and electrophysiological similarities with another myotonic muscle disorder, hyperkalemic periodic paralysis (HYPP). However, clinical and electrophysiologic differences allow differentiation of the two disorders. The HYPP locus was recently shown to be linked to a skeletal muscle sodium-channel gene probe. We now report that PC maps to the same locus (LOD score 4.4, theta = 0 at assumed penetrance of .95). These linkage results, coupled with physiological data demonstrating abnormal sodium-channel function in patients with PC, implicate a sodium-channel gene as an important candidate for the site of mutation responsible for PC. Furthermore, this is strong evidence for the hypothesis that PC and HYPP are allelic disorders. PMID:1654742

  18. Episodic Muscle Tremors in a Quarter Horse: Resemblance to Hyperkalemic Periodic Paralysis

    Science.gov (United States)

    Steiss, Janet E.; Naylor, Jonathan M.

    1986-01-01

    A three year old Quarter Horse stallion was presented with a one year history of episodes of generalized muscle tremors and stiffness, and spasm of the muscles of facial expression, lasting 10-15 minutes. Between attacks, the horse was either normal or had a localized muscle tremor in the flank region. Episodes appeared unrelated to exercise. The major abnormal findings included 1) a rise in plasma potassium from a resting level of 4.4 to 7.9 mmol/L during an attack and 2) electromyographic findings of generalized increased insertion activity and myotonic discharges. The horse was treated with hydrochlorothiazide tablets for nine months, during which time no further attacks were noted. However, four months after the drug was stopped, sporadic focal muscle tremors reappeared; two months later, generalized attacks were seen. Despite reinstitution of the diuretic, a focal flank tremor persisted. Two related horses in the same stable also were reported by the owner to exhibit sporadic generalized muscle twitching. The abnormal findings of the present case differ from clinical syndromes previously reported in horses. Some similarities to hyperkalemic periodic paralysis in humans are noted. PMID:17422693

  19. Hyperkalemic periodic paralysis M1592V mutation modifies activation in human skeletal muscle Na+ channel.

    Science.gov (United States)

    Rojas, C V; Neely, A; Velasco-Loyden, G; Palma, V; Kukuljan, M

    1999-01-01

    Mutations in the human skeletal muscle Na+ channel underlie the autosomal dominant disease hyperkalemic periodic paralysis (HPP). Muscle fibers from affected individuals exhibit sustained Na+ currents thought to depolarize the sarcolemma and thus inactivate normal Na+ channels. We expressed human wild-type or M1592V mutant alpha-subunits with the beta1-subunit in Xenopus laevis oocytes and recorded Na+ currents using two-electrode and cut-open oocyte voltage-clamp techniques. The most prominent functional difference between M1592V mutant and wild-type channels is a 5- to 10-mV shift in the hyperpolarized direction of the steady-state activation curve. The shift in the activation curve for the mutant results in a larger overlap with the inactivation curve than that observed for wild-type channels. Accordingly, the current through M1592V channels displays a larger noninactivating component than does that through wild-type channels at membrane potentials near -40 mV. The functional properties of the M1592V mutant resemble those of the previously characterized HPP T704M mutant. Both clinically similar phenotypes arise from mutations located at a distance from the putative voltage sensor of the channel.

  20. Characterization of hyperkalemic periodic paralysis: a survey of genetically diagnosed individuals.

    Science.gov (United States)

    Charles, G; Zheng, C; Lehmann-Horn, F; Jurkat-Rott, K; Levitt, J

    2013-10-01

    This exploratory study aims to create an evidence-based comprehensive characterization of hyperkalemic periodic paralysis (hyperPP). HyperPP is a rare genetic disorder that causes episodes of flaccid paralysis. Disease descriptions in the literature are based upon isolated clinical encounters and case reports. We describe the experience of a large cohort of genetically diagnosed individuals with hyperPP. We surveyed genetically characterized individuals age 18 and over to assess disease comorbidities, diagnostic testing, management, and quality of life issues relevant to hyperPP. Myotonia was reported by 55.8 % of subjects and paramyotonia by 45.3 %. There is a relative risk of 3.6 (p < 0.0001) for thyroid dysfunction compared to the general population. Twenty-five percent of subjects experienced their sentinel attack in the second decade of life. It took an average of 19.4 years and visits to four physicians to arrive at the diagnosis of hyperPP. In addition to limbs and hands being affected during attacks, 26.1 % of subjects reported their breathing musculature was affected and 62.0 % reported their facial muscles were affected. There was a lifelong trend of increasing attack frequency, which was particularly common during childhood and adolescence. Approximately one-third of individuals experienced progressive myopathy. Permanent muscle weakness was evident and worsened during childhood and after age 40. Those with no chronic treatment regimen have a RR of 2.3 for inadequate disease control compared to those taking long-term medications. This study revealed a multitude of heretofore unidentified characteristics of hyperPP, in addition to providing a different perspective on some previously held notions regarding the condition.

  1. Effects of local anesthetics on Na+ channels containing the equine hyperkalemic periodic paralysis mutation.

    Science.gov (United States)

    Sah, R L; Tsushima, R G; Backx, P H

    1998-08-01

    We examined the ability of local anesthetics to correct altered inactivation properties of rat skeletal muscle Na+ channels containing the equine hyperkalemic periodic paralysis (eqHPP) mutation when expressed in Xenopus oocytes. Increased time constants of current decay in eqHPP channels compared with wild-type channels were restored by 1 mM benzocaine but were not altered by lidocaine or mexiletine. Inactivation curves, which were determined by measuring the dependence of the relative peak current amplitude after depolarization to -10 mV on conditioning prepulse voltages, could be shifted in eqHPP channels back toward that observed for wild-type (WT) channels using selected concentrations of benzocaine, lidocaine, and mexiletine. Recovery from inactivation at -80 mV (50-ms conditioning pulse) in eqHPP channels followed a monoexponential time course and was markedly accelerated compared with wild-type channels (tauWT = 10.8 +/- 0.9 ms; taueqHPP = 2.9 +/- 0.4 ms). Benzocaine slowed the time course of recovery (taueqHPP,ben = 9.6 +/- 0.4 ms at 1 mM) in a concentration-dependent manner. In contrast, the recovery from inactivation with lidocaine and mexiletine had a fast component (taufast,lid = 3.2 +/- 0.2 ms; taufast,mex = 3.1 +/- 0.2 ms), which was identical to the recovery in eqHPP channels without drug, and a slow component (tauslow,lid = 1,688 +/- 180 ms; tauslow,mex = 2,323 +/- 328 ms). The time constant of the slow component of the recovery from inactivation was independent of the drug concentration, whereas the fraction of current recovering slowly depended on drug concentrations and conditioning pulse durations. Our results show that local anesthetics are generally incapable of fully restoring normal WT behavior in inactivation-deficient eqHPP channels.

  2. Na+,K+-pump stimulation improves contractility in isolated muscles of mice with hyperkalemic periodic paralysis.

    Science.gov (United States)

    Clausen, Torben; Nielsen, Ole Bækgaard; Clausen, Johannes D; Pedersen, Thomas Holm; Hayward, Lawrence J

    2011-07-01

    In patients with hyperkalemic periodic paralysis (HyperKPP), attacks of muscle weakness or paralysis are triggered by K(+) ingestion or rest after exercise. Force can be restored by muscle work or treatment with β(2)-adrenoceptor agonists. A missense substitution corresponding to a mutation in the skeletal muscle voltage-gated Na(+) channel (Na(v)1.4, Met1592Val) causing human HyperKPP was targeted into the mouse SCN4A gene (mutants). In soleus muscles prepared from these mutant mice, twitch, tetanic force, and endurance were markedly reduced compared with soleus from wild type (WT), reflecting impaired excitability. In mutant soleus, contractility was considerably more sensitive than WT soleus to inhibition by elevated [K(+)](o). In resting mutant soleus, tetrodotoxin (TTX)-suppressible (22)Na uptake and [Na(+)](i) were increased by 470 and 58%, respectively, and membrane potential was depolarized (by 16 mV, P < 0.0001) and repolarized by TTX. Na(+),K(+) pump-mediated (86)Rb uptake was 83% larger than in WT. Salbutamol stimulated (86)Rb uptake and reduced [Na(+)](i) both in mutant and WT soleus. Stimulating Na(+),K(+) pumps with salbutamol restored force in mutant soleus and extensor digitorum longus (EDL). Increasing [Na(+)](i) with monensin also restored force in soleus. In soleus, EDL, and tibialis anterior muscles of mutant mice, the content of Na(+),K(+) pumps was 28, 62, and 33% higher than in WT, respectively, possibly reflecting the stimulating effect of elevated [Na(+)](i) on the synthesis of Na(+),K(+) pumps. The results confirm that the functional disorders of skeletal muscles in HyperKPP are secondary to increased Na(+) influx and show that contractility can be restored by acute stimulation of the Na(+),K(+) pumps. Calcitonin gene-related peptide (CGRP) restored force in mutant soleus but caused no detectable increase in (86)Rb uptake. Repeated excitation and capsaicin also restored contractility, possibly because of the release of endogenous CGRP

  3. Understanding the physiology of the asymptomatic diaphragm of the M1592V hyperkalemic periodic paralysis mouse.

    Science.gov (United States)

    Ammar, Tarek; Lin, Wei; Higgins, Amanda; Hayward, Lawrence J; Renaud, Jean-Marc

    2015-12-01

    The diaphragm muscle of hyperkalemic periodic paralysis (HyperKPP) patients and of the M1592V HyperKPP mouse model rarely suffers from the myotonic and paralytic symptoms that occur in limb muscles. Enigmatically, HyperKPP diaphragm expresses the mutant NaV1.4 channel and, more importantly, has an abnormally high Na(+) influx similar to that in extensor digitorum longus (EDL) and soleus, two hindlimb muscles suffering from the robust HyperKPP abnormalities. The objective was to uncover the physiological mechanisms that render HyperKPP diaphragm asymptomatic. A first mechanism involves efficient maintenance of resting membrane polarization in HyperKPP diaphragm at various extracellular K(+) concentrations compared with larger membrane depolarizations in HyperKPP EDL and soleus. The improved resting membrane potential (EM) results from significantly increased Na(+) K(+) pump electrogenic activity, and not from an increased protein content. Action potential amplitude was greater in HyperKPP diaphragm than in HyperKPP soleus and EDL, providing a second mechanism for the asymptomatic behavior of the HyperKPP diaphragm. One suggested mechanism for the greater action potential amplitude is lower intracellular Na(+) concentration because of greater Na(+) K(+) pump activity, allowing better Na(+) current during the action potential depolarization phase. Finally, HyperKPP diaphragm had a greater capacity to generate force at depolarized EM compared with wild-type diaphragm. Action potential amplitude was not different between wild-type and HyperKPP diaphragm. There was also no evidence for an increased activity of the Na(+)-Ca(2+) exchanger working in the reverse mode in the HyperKPP diaphragm compared with the wild-type diaphragm. So, a third mechanism remains to be elucidated to fully understand how HyperKPP diaphragm generates more force compared with wild type. Although the mechanism for the greater force at depolarized resting EM remains to be determined, this study

  4. Linkage of malignant hyperthermia and hyperkalemic periodic paralysis to the adult skeletal muscle sodium channel (SCN4A) gene in a large pedigree.

    Science.gov (United States)

    Moslehi, R; Langlois, S; Yam, I; Friedman, J M

    1998-02-26

    Hyperkalemic periodic paralysis (HPP) is caused by mutations of the adult skeletal muscle sodium channel (SCN4A) gene on chromosome 17. Malignant hyperthermia (MH) is a genetically heterogeneous autosomal-dominant disorder occurring in association with various neuromuscular diseases or without other apparent abnormalities. In some families, MH is associated with mutations of a calcium release channel (RYR1) gene on chromosome 19. In other families, linkage of this disorder to the SCN4A gene on chromosome 17 has been suggested. We report on linkage analysis in a family in which both HPP and MH are inherited as autosomal-dominant traits. Two polymorphisms within the SCN4A locus, an RFLP and a (C-A)n repeat, were typed on multiple family members. The findings were consistent with linkage of the polymorphic markers within the SCN4A gene to both HPP (Zmax = 6.79 at theta = 0.0) and MH (Zmax = 1.76 at theta = 0) in this family. Our data provide further evidence that MH is linked to the SCN4A locus in some families.

  5. A global defect in scaling relationship between electrical activity and availability of muscle sodium channels in hyperkalemic periodic paralysis.

    Science.gov (United States)

    Melamed-Frank, M; Marom, S

    1999-07-01

    Hyperkalemic periodic paralysis (HyperPP) is a hereditary disorder characterized by alternate episodic attacks of muscle weakness and muscle myotonia. The most common mutation associated with HyperPP is a T704M substitution in the skeletal-muscle sodium channel. This mutation increases sodium persistent currents, alters voltage dependence of activation and impairs slow inactivation. The present study shows experimental evidence in support of a potentially important global defect caused by the T704M mutation. While the effective rate of recovery from slow inactivation, in both normal and mutated channels, is related to the duration of past activity by a power law function, the scaling power of the mutated channel is significantly greater. This difference between the channels offers a clue for an explanation to the wide range of time scales, history dependence, and the mixed myotonic/paralysis effect, which mark the clinical picture of HyperPP.

  6. Dinucleotide repeat polymorphisms at the SCN4A locus suggest allelic heterogeneity of hyperkalemic periodic paralysis and paramyotonia congenita

    Science.gov (United States)

    McClatchey, Andrea I.; Trofatter, James; McKenna-Yasek, Diane; Raskind, Wendy; Bird, Thomas; Pericak-Vance, Margaret; Gilchrist, James; Arahata, Kiichi; Radosavljevic, Danica; Worthen, Hilary G.; Van den Bergh, Peter; Haines, Jonathan L.; Gusella, James F.; Brown, Robert H.

    1992-01-01

    Two polymorphic dinucleotide repeats–one (dGdA)n and one (dGdT)n –have been identified at the SCN4A locus, encoding the α-subunit of the adult skeletal muscle sodium channel. When typed using PCR, the dinucleotide repeats display 4 and 10 alleles, respectively, with a predicted heterozygosity of .81 for the combined haplotype. We have applied these polymorphisms to the investigation of hyperkalemic periodic paralysis and paramyotonia congenita, distinct neuromuscular disorders both of which are thought to involve mutation at SCN4A. Our data confirm the genetic linkage of both disorders with SCN4A. Haplotype analysis also indicates the strong likelihood of allelic heterogeneity in both disorders. ImagesFigure 1Figure 2 PMID:1315122

  7. Different effects of mexiletine on two mutant sodium channels causing paramyotonia congenita and hyperkalemic periodic paralysis.

    Science.gov (United States)

    Weckbecker, K; Würz, A; Mohammadi, B; Mansuroglu, T; George, A L; Lerche, H; Dengler, R; Lehmann-Horn, F; Mitrovic, N

    2000-01-01

    Effects of the antiarrhythmic and antimyotonic drug mexiletine were studied on two sodium channel mutants causing paramyotonia congenita (R1448H) and an overlap paramyotonic and hyperkalemic paralytic syndrome (M1360V). Channels were expressed in human embryonic kidney cells and studied electrophysiologically, using the whole-cell patch-clamp technique. Compared to the wild-type, channel, both mutants showed alterations of inactivation, i.e. slower inactivation, left shift of steady-state inactivation and faster recovery from inactivation. Mexiletine caused a significantly larger use-dependent block of the R1448H mutant when compared to M1360V and wild-type channels. This can be explained by a prolonged recovery from mexiletine block as observed for R1448H channels, since the affinity of mexiletine for the inactivated state was similar for all three clones. The use-dependent block of sodium channels by mexiletine reduces repetitive series of action potentials and therefore improves muscle stiffness in myotonic patients. The enhanced use-dependent block as seen with R1448H may explain the extraordinary therapeutic efficacy of mexiletine in most patients with paramyotonia congenita.

  8. Phenytoin alters transcript levels of hormone-sensitive lipase in muscle from horses with hyperkalemic periodic paralysis.

    Science.gov (United States)

    Yudkowsky, M L; Beech, J; Fletcher, J E

    1998-10-15

    In equine hyperkalemic periodic paralysis (HyperPP), there is evidence suggesting that the primary defect in the sodium channel is associated with a secondary alteration in triacylglycerol-associated fatty acid metabolism (TAFAM) in skeletal muscle. Furthermore, TAFAM may be involved in the therapeutic action of phenytoin. The effects of phenytoin treatment on the transcript levels of three key proteins in TAFAM, hormone sensitive lipase (HSL), carnitine palmitoyltransferase (CPT), and fatty acid binding protein (FABP), were examined. These transcripts were quantitated by competitive reverse transcription polymerase chain reaction in undifferentiated and differentiated primary cultures of equine skeletal muscle from control, heterozygous HyperPP, and homozygous-affected HyperPP horses. There was a 10-fold lower level of HSL transcript in both undifferentiated and differentiated cultures from homozygous-affected horses than from horses of the other genotypes. Phenytoin selectively increased the HSL transcript in homozygous-affected differentiated cultures to levels similar to those of the other genotypes. The levels of CPT and FABP transcripts were unaffected by genotype, differentiation, and phenytoin treatment. These results suggest that the primary defect in HyperPP may secondarily decrease HSL transcript levels and that the therapeutic action of phenytoin may include regulation of mRNA transcripts in skeletal muscle. Copyright 1998 Academic Press.

  9. Disappearance of episodic weakness during pregnancy in hyperkalemic periodic paralysis from the SCNA4 mutation T704M.

    Science.gov (United States)

    Finsterer, Josef

    2009-09-01

    Disappearance of episodic weakness during pregnancy and an increase in myotonia after delivery have not been reported in hyperkalemic periodic paralysis (hyperPP). In a 36-year-old white woman, with typical manifestations of episodic weakness of the limb muscles since age 3 years, hyperPP was diagnosed by demonstration of the T407M SCNA4 gene point mutation. At age 34 years, the index patient became pregnant for the first time. During the first trimester of her pregnancy, she noted a marked decrease in the frequency of the hyperPP attacks. During the second and third trimesters, the attacks of weakness completely disappeared and after delivery the attacks restarted with the same intensity and frequency as before pregnancy, but myotonia persisted between the attacks. Disappearance of the attacks during pregnancy was attributed to the hormonal changes. Episodic attacks of weakness in hyperPP may disappear during the second and third trimesters of pregnancy, and myotonia between the attacks may persist after delivery.

  10. Hyperkalemic periodic paralysis and permanent weakness: 3-T MR imaging depicts intracellular 23Na overload--initial results.

    Science.gov (United States)

    Amarteifio, Erick; Nagel, Armin M; Weber, Marc-André; Jurkat-Rott, Karin; Lehmann-Horn, Frank

    2012-07-01

    To assess whether myoplasmic ionic sodium (Na+) is increased in muscles of patients with hyperkalemic periodic paralysis (HyperPP) with 3-T sodium 23 (23Na) magnetic resonance (MR) imaging and to evaluate the effect of medical treatment on sodium-induced muscle edema. This study received institutional review board approval; written informed consent was obtained. Proton (hydrogen 1 [1H]) and 23Na MR of both calves were performed in 12 patients with HyperPP (mean age, 48 years±14 [standard deviation]) and 12 healthy volunteers (mean age, 38 years±12) before and after provocation (unilateral cooling, one calf). 23Na MR included spin-density, T1-weighted, and inversion-recovery (IR) sequences. Total sodium concentration and normalized signal intensities (SIs) were evaluated within regions of interest (ROIs). Muscle strength was measured with the British Medical Research Council (MRC) grading scale. Five patients underwent follow-up MR after diuretic treatment. During rest, mean myoplasmic Na+ concentration was significantly higher in HyperPP with permanent weakness (40.7 μmol/g±3.9) compared with HyperPP with transient weakness (31.3 μmol/g±4.8) (P=.004). Mean SI in 23Na IR MR was significantly higher in HyperPP with permanent weakness (0.83±0.04; median MRC, grade 4; range, 3-5) compared with HyperPP without permanent weakness (0.67±0.05; median MRC, grade 5; range, 4-5) (P=.002). Provocation reduced muscle strength in HyperPP (before provocation, median MRC, 5; range, 3-5; after provocation, median MRC, 3; range, 1-4) and increased SI in 23Na IR from 0.75±0.09 to 0.86±0.10 (P=.004). Spin-density and T1-weighted sequences were less sensitive, particularly to cold-induced Na+ changes. 23Na IR SI remained unchanged in volunteers (0.53±0.06 before and 0.54±0.06 after provocation, P=.3). Therapy reduced mean SI in 23Na IR sequence from 0.85±0.04 to 0.64±0.11. 23Na MR imaging depicts increased myoplasmic Na+ in HyperPP with permanent weakness. Na+ overload

  11. [Hyperkalemic periodic muscle paralysis in primary adrenocortical insufficiency. A case report].

    Science.gov (United States)

    Przedlacki, J; Debowska, M; Matuszkiewicz-Rowińska, J; Janik, P

    1999-06-01

    20-years old man was admitted to the hospital because of a few paroxysmal muscle paralysis episodes with pronounced periodic hiperkalemia (maximal 9.8 mmol/l). The first episode was preceded by a very hard physical effort. Primary adrenal insufficiency was recognised on the basis of clinical, humoral and biochemical examinations. There was elevated ACTH and a very low serum level of cortisol and aldosterone. There was slight metabolic acidosis and hiponatremia. The patient was treated with hydrocortisone and fludrocortisone acetate (Cortineff) with positive result. During one year of this therapy his general condition was very good and clinical, humoral and biochemical signs (except of metabolic acidosis) resolved. Neurological symptoms and a very high serum kalium level were the most important signs of primary adrenal insufficiency in the presented case.

  12. [Current status of clinical and molecular-biological research on familial periodic paralysis].

    Science.gov (United States)

    Shishiba, Y

    1997-12-01

    Types of periodic paralysis seen in Japan are numerous: the one most frequently seen is hypokalemic periodic paralysis. Among them, approximately 50% are secondary to thyrotoxicosis. Number of families of familial hyperkalemic periodic paralysis have also been reported so far. Several cases of hyperkalemic periodic paralysis secondary to thyrotoxicosis have also been reported exclusively from Japan. As the pathogenesis of hypokalemic periodic paralysis, depolarization block induced by membrane permeability change in the face of hypokalemia triggered by excess insulin was strongly suggested and supported experimentally in part. Recent linkage analysis on familial hypokalemic periodic paralysis revealed that the abnormality is linked to a mutation in voltage-gated Ca channel. The difficulty remains how to explain the cause of hypokalemia which is almost always preceding the attack of periodic paralysis of this type. The cause of hyperkalemic periodic paralysis was shown to be the mutation in voltage-gated Na channel. Failure of inactivation of the channel causes an increase in inward sodium current which results in depolarization and accumulation of potassium. The explanation of the pathogenesis of paralysis is straight-forward when compared to that of hypokalemic periodic paralysis.

  13. Contractile abnormalities of mouse muscles expressing hyperkalemic periodic paralysis mutant NaV1.4 channels do not correlate with Na+ influx or channel content.

    Science.gov (United States)

    Lucas, Brooke; Ammar, Tarek; Khogali, Shiemaa; DeJong, Danica; Barbalinardo, Michael; Nishi, Cameron; Hayward, Lawrence J; Renaud, Jean-Marc

    2014-06-01

    Hyperkalemic periodic paralysis (HyperKPP) is characterized by myotonic discharges that occur between episodic attacks of paralysis. Individuals with HyperKPP rarely suffer respiratory distress even though diaphragm muscle expresses the same defective Na(+) channel isoform (NaV1.4) that causes symptoms in limb muscles. We tested the hypothesis that the extent of the HyperKPP phenotype (low force generation and shift toward oxidative type I and IIA fibers) in muscle is a function of 1) the NaV1.4 channel content and 2) the Na(+) influx through the defective channels [i.e., the tetrodotoxin (TTX)-sensitive Na(+) influx]. We measured NaV1.4 channel protein content, TTX-sensitive Na(+) influx, force generation, and myosin isoform expression in four muscles from knock-in mice expressing a NaV1.4 isoform corresponding to the human M1592V mutant. The HyperKPP flexor digitorum brevis muscle showed no contractile abnormalities, which correlated well with its low NaV1.4 protein content and by far the lowest TTX-sensitive Na(+) influx. In contrast, diaphragm muscle expressing the HyperKPP mutant contained high levels of NaV1.4 protein and exhibited a TTX-sensitive Na(+) influx that was 22% higher compared with affected extensor digitorum longus (EDL) and soleus muscles. Surprisingly, despite this high burden of Na(+) influx, the contractility phenotype was very mild in mutant diaphragm compared with the robust abnormalities observed in EDL and soleus. This study provides evidence that HyperKPP phenotype does not depend solely on the NaV1.4 content or Na(+) influx and that the diaphragm does not depend solely on Na(+)-K(+) pumps to ameliorate the phenotype. Copyright © 2014 the American Physiological Society.

  14. Paramyotonia congenita and hyperkalemic periodic paralysis associated with a Met 1592 Val substitution in the skeletal muscle sodium channel alpha subunit--a large kindred with a novel phenotype.

    Science.gov (United States)

    Kelly, P; Yang, W S; Costigan, D; Farrell, M A; Murphy, S; Hardiman, O

    1997-03-01

    Paramyotonia congenita (PC) and Hyperkalemic periodic paralysis (HyperPP) are caused by amino acid substitutions in the alpha subunit of the human skeletal muscle sodium channel. One such substitution, methionine for valine at position 1592, has been associated with HyperPP with myotonia and cold sensitivity. We report clinical, electromyographic (EMG), genetic and pathological features of a large kindred with the Met1592Val substitution. Affected members were phenotypically heterogenous and had episodic potassium-sensitive paralysis, and stiffness and weakness induced by exercise and cold, which was confirmed by EMG studies. These features indicate a combined PC-HyperPP phenotype not previously described with this mutation.

  15. Physiological basis for muscle stiffness and weakness in a knock-in M1592V mouse model of hyperkalemic periodic paralysis.

    Science.gov (United States)

    Khogali, Shiemaa; Lucas, Brooke; Ammar, Tarek; Dejong, Danica; Barbalinardo, Michael; Hayward, Lawrence J; Renaud, Jean-Marc

    2015-12-01

    The mechanisms responsible for the onset and progressive worsening of episodic muscle stiffness and weakness in hyperkalemic periodic paralysis (HyperKPP) are not fully understood. Using a knock-in HyperKPP mouse model harboring the M1592V NaV1.4 channel mutant, we interrogated changes in physiological defects during the first year, including tetrodotoxin-sensitive Na(+) influx, hindlimb electromyographic (EMG) activity and immobility, muscle weakness induced by elevated [K(+)]e, myofiber-type composition, and myofiber damage. In situ EMG activity was greater in HyperKPP than wild-type gastrocnemius, whereas spontaneous muscle contractions were observed in vitro. We suggest that both the greater EMG activity and spontaneous contractions are related to periods of hyperexcitability during which fibers generate action potentials by themselves in the absence of any stimulation and that these periods are the cause of the muscle stiffness reported by patients. HyperKPP muscles had a greater sensitivity to the K(+)-induced force depression than wild-type muscles. So, an increased interstitial K(+) concentration locally near subsets of myofibers as a result of the hyperexcitability likely produced partial loss of force rather than complete paralysis. NaV1.4 channel protein content reached adult level by 3 weeks postnatal in both wild type and HyperKPP and apparent symptoms did not worsen after the first month of age suggesting (i) that the phenotypic behavior of M1592V HyperKPP muscles results from defective function of mutant NaV1.4 channels rather than other changes in protein expression after the first month and (ii) that the lag in onset during the first decade and the progression of human HyperKPP symptoms during adolescence are a function of NaV1.4 channel content. © 2015 The Authors. Physiological Reports published by Wiley Periodicals, Inc. on behalf of the American Physiological Society and The Physiological Society.

  16. Familial Periodic Paralyses

    Science.gov (United States)

    ... and less severe than the hypokalemic form. Muscle spasms are common. × Definition Familial periodic paralyses are a ... and less severe than the hypokalemic form. Muscle spasms are common. View Full Definition Treatment Treatment of ...

  17. ROMK expression remains unaltered in a mouse model of familial hyperkalemic hypertension caused by the CUL3Δ403-459 mutation.

    Science.gov (United States)

    Murthy, Meena; Kurz, Thimo; O'Shaughnessy, Kevin M

    2016-07-01

    Familial hyperkalemic hypertension (FHHt) is a rare inherited form of salt-dependent hypertension caused by mutations in proteins that regulate the renal Na(+)-Cl(-) cotransporter NCC Mutations in four genes have been reported to cause FHHt including CUL3 (Cullin3) that encodes a component of a RING E3 ligase. Cullin-3 binds to WNK kinase-bound KLHL3 (the substrate recognition subunit of the ubiquitin ligase complex) to promote ubiquitination and proteasomal degradation of WNK kinases. Deletion of exon 9 from CUL3 (affecting residues 403-459, CUL3(Δ403-459)) causes a severe form of FHHt (PHA2E) that is recapitulated closely in a knock-in mouse model. The loss of functionality of CUL3(Δ403-459) and secondary accumulation of WNK kinases causes substantial NCC activation. This accounts for the hypertension in FHHt but the origin of the hyperkalemia is less clear. Hence, we explored the impact of CUL3(Δ403-459) on expression of the distal secretory K channel, ROMK, both in vitro and in vivo. We found that expressing wild-type but not the CUL3(Δ403-459) mutant form of CUL3 prevented the suppression of ROMK currents by WNK4 expressed in Xenopus oocytes. The mutant CUL3 protein was also unable to affect ROMK-EGFP protein expression at the surface of mouse M-1 cortical collecting duct (CCD) cells. The effects of CUL3 on ROMK expression in both oocytes and M-1 CCD cells was reduced by addition of the neddylation inhibitor, MLN4924. This confirms that neddylation is important for CUL3 activity. Nevertheless, in our knock-in mouse model expressing CUL3(Δ403-459) we could not show any alteration in ROMK expression by either western blotting whole kidney lysates or confocal microscopy of kidney sections. This suggests that the hyperkalemia in our knock-in mouse and human PHA2E subjects with the CUL3(Δ403-459) mutation is not caused by reduced ROMK expression in the distal nephron.

  18. Effect of high-intensity exercise on arterial blood gas tensions and upper airway and cardiac function in clinically normal quarter horses and horses heterozygous and homozygous for hyperkalemic periodic paralysis.

    Science.gov (United States)

    Maxson-Sage, A; Parente, E J; Beech, J; Lindborg, S; May, L L; Teleis, D C

    1998-05-01

    To determine the effect of exercise on arterial blood gas tensions and upper airway and cardiac function in clinically normal Quarter Horses and horses heterozygous and homozygous for hyperkalemic periodic paralysis (HYPP). ANIMALS AND PROCEDURE: 5 clinically normal Quarter Horses, and 5 heterozygous and 2 homozygous HYPP-affected horses were examined before, during, and after exercise on a high-speed treadmill. Arterial blood gas tensions, ECG, and echocardiogram were obtained prior to exercise. Upper airway endoscopy, collection of arterial blood samples, and continuous electrocardiography were performed during a high-intensity stepwise exercise test. An ECG was obtained within 1-minute after completion of the final step. None of the horses homozygous or heterozygous for HYPP had signs of weakness or muscle fasciculations before, during, or after exercise. Horses homozygous for HYPP had intermittent laryngospasm, dynamic pharyngeal collapse, and appreciable hypoxemia, hypercapnia, and ventricular premature contractions during exercise. Heterozygous and clinically normal horses did not have any abnormalities. Potassium concentration increased significantly above the baseline reference range during exercise in all groups of horses. Horses homozygous for HYPP had laryngospasm and dynamic pharyngeal collapse associated with exercise, most likely secondary to increase in potassium concentration. Upper airway dysfunction is the most likely cause of hypoxemia and hypercapnia. Cardiac arrhythmias were most likely caused by a combination of hypoxemia and hyperkalemia.

  19. Whole-Body Muscle MRI in Patients with Hyperkalemic Periodic Paralysis Carrying the SCN4A Mutation T704M: Evidence for Chronic Progressive Myopathy with Selective Muscle Involvement.

    Science.gov (United States)

    Lee, Young Han; Lee, Hyung Soo; Lee, Hyo Eun; Hahn, Seok; Nam, Tai Seung; Shin, Ha Young; Choi, Young Chul; Kim, Seung Min

    2015-10-01

    Hyperkalemic periodic paralysis (hyperKPP) is a muscle sodium-ion channelopathy characterized by recurrent paralytic attacks. A proportion of affected individuals develop fixed or chronic progressive weakness that results in significant disability. However, little is known about the pathology of hyperKPP-induced fixed weakness, including the pattern of muscle involvement. The aim of this study was to characterize the patterns of muscle involvement in hyperKPP by whole-body magnetic resonance imaging (MRI). We performed whole-body muscle MRI in seven hyperKPP patients carrying the T704M mutation in the SCN4A skeletal sodium-channel gene. Muscle fat infiltration, suggestive of chronic progressive myopathy, was analyzed qualitatively using a grading system and was quantified by the two-point Dixon technique. Whole-body muscle MRI analysis revealed muscle atrophy and fatty infiltration in hyperKPP patients, especially in older individuals. Muscle involvement followed a selective pattern, primarily affecting the posterior compartment of the lower leg and anterior thigh muscles. The muscle fat fraction increased with patient age in the anterior thigh (r=0.669, p=0.009), in the deep posterior compartment of the lower leg (r=0.617, p=0.019), and in the superficial posterior compartment of the lower leg (r=0.777, p=0.001). Our whole-body muscle MRI findings provide evidence for chronic progressive myopathy in hyperKPP patients. The reported data suggest that a selective pattern of muscle involvement-affecting the posterior compartment of the lower leg and the anterior thigh-is characteristic of chronic progressive myopathy in hyperKPP.

  20. [Clinical and molecular genetic analysis of a family with normokalemic periodic paralysis].

    Science.gov (United States)

    Wei, Cui-jie; Wang, Dong; Wang, Shuo; Jiao, Hui; Hong, Dao-jun; Pu, Li-hua; Xiong, Hui

    2013-01-01

    Periodic paralysis (PP) is one type of skeletal muscle channelopathies characterized by episodic attacks of weakness. It is usually classified into hyperkalemic periodic paralysis (HyperPP), hypokalemic periodic paralysis (HypoPP) and normokalemic periodic paralysis (NormoPP) based on the blood potassium levels. HypoPP is the most common type of these three and NormoPP is the rarest one. The aim of this study was to explore the clinical and genetic features of a Chinese family with normokalemic periodic paralysis (NormoKPP). Clinical features of all patients in the family with NormoKPP were analyzed. Genomic DNA was extracted from peripheral blood leukocytes and amplified with PCR. We screened all 24 exons of SCN4A gene and then sequence analysis was performed in those who showed heteroduplex as compared with unaffected controls. (1) Fifteen members of the family were clinically diagnosed NormoKPP, and their common features are: onset within infacy, episodic attacks of weakness, the blood potassium levels were within normal ranges, high sodium diet or large dosage of normal saline could attenuate the symptom. One muscle biopsy was performed and examination of light and electronic microscopy showed occasionally degenerating myofibers. (2) Gene of 12 patients were screened and confirmed mutations of SCN4A genes--c. 2111 T > C/p. Thr704Met. The study further defined the clinical features of patients with NormoKPP, and molecular genetic analysis found SCN4A gene c. 2111 T > C/p. Thr704Met point mutation contributed to the disease. In line with the autosomal dominant inheritance laws, this family can be diagnosed with periodic paralysis, and be provided with genetic counseling. And the study may also help the clinical diagnosis, guide treatment and genetic counseling of this rare disease in China.

  1. A double mutation in families with periodic paralysis defines new aspects of sodium channel slow inactivation

    Science.gov (United States)

    Bendahhou, Saïd; Cummins, Theodore R.; Hahn, Angelika F.; Langlois, Sylvie; Waxman, Stephen G.; Ptácek, Louis J.

    2000-01-01

    Hyperkalemic periodic paralysis (HyperKPP) is an autosomal dominant skeletal muscle disorder caused by single mutations in the SCN4A gene, encoding the human skeletal muscle voltage-gated Na+ channel. We have now identified one allele with two novel mutations occurring simultaneously in the SCN4A gene. These mutations are found in two distinct families that had symptoms of periodic paralysis and malignant hyperthermia susceptibility. The two nucleotide transitions predict phenylalanine 1490→leucine and methionine 1493→isoleucine changes located in the transmembrane segment S5 in the fourth repeat of the α-subunit Na+ channel. Surprisingly, this mutation did not affect fast inactivation parameters. The only defect produced by the double mutant (F1490L-M1493I, expressed in human embryonic kidney 293 cells) is an enhancement of slow inactivation, a unique behavior not seen in the 24 other disease-causing mutations. The behavior observed in these mutant channels demonstrates that manifestation of HyperKPP does not necessarily require disruption of slow inactivation. Our findings may also shed light on the molecular determinants and mechanism of Na+ channel slow inactivation and help clarify the relationship between Na+ channel defects and the long-term paralytic attacks experienced by patients with HyperKPP. PMID:10930446

  2. [A family with heat-sensitive myotonia alternating with hypokalemic periodic paralysis].

    Science.gov (United States)

    Aoki, T; Sugiura, Y; Sugiyama, Y; Ogata, M; Hida, C; Honma, M; Yamamoto, T

    2000-04-01

    We report a 34-year-old man with evident family history of paralysis and myotonia. He has noticed episodic morning paralysis alternating with muscle myotonia since the age of 10 years. When an episode of paralysis occurred, his serum potassium level decreased to 2.3 mEq/L and tended to increase to about 4.0 mEq/L when he complained myotonia. This case is obviously different from several already-known diseases, as it is perhaps the first case thermo-sensitive disorder of muscle activity in that the myotonia is induced by warm environment but paralysis occurs in the setting of cold ambient temperature and hypokalemia. Therefore, we are underway to perform molecular genetic analysis of ion channels since paramyotonia/hyperkalemic periodic paralysis is known to be associated with Na(+)-channel missense mutations, while hypokalemic periodic paralysis is with Ca(++)-channel. Addendum: After submission of the manuscript, we identified a novel mutation in Na+ channel alpha subunit. The detail of molecular aspect will be reported elsewhere.

  3. Reversible electrophysiological abnormalities in acute secondary hyperkalemic paralysis

    Directory of Open Access Journals (Sweden)

    Karkal R Naik

    2012-01-01

    Full Text Available Hyperkalemia manifests clinically with acute neuromuscular paralysis, which can simulate Guillain Barré syndrome (GBS and other causes of acute flaccid paralysis. Primary hyperkalemic paralysis occurs from genetic defects in the sodium channel, and secondary hyperkalemic paralysis (SHP from diverse causes including renal dysfunction, potassium retaining drugs, Addison′s disease, etc. Clinical characteristics of SHP have been addressed in a number of publications. However, electrophysiological evaluations of these patients during neuromuscular paralysis are infrequently reported and have demonstrated features of demyelination. The clinical features and electrophysiological abnormalities in secondary hyperkalemia mimic GBS, and pose diagnostic challenges. We report the findings of nerve conduction studies in a middle-aged man who was admitted with rapidly reversible acute quadriplegia resulting from secondary hyperkalemic paralysis.

  4. Familial Hypokalemic Periodic Paralysis: A Case Report

    Directory of Open Access Journals (Sweden)

    Semih KORKUT, Hayati KANDİŞ, Harun GÜNEŞ, Esin KORKUT

    2010-11-01

    Full Text Available Familial hypokalemic periodic paralysis is an autosomal dominantly inherited congenital diseasecharacterized by intermittent attacks of muscle weakness lasting for a few hours to a few daysand occurring a few times a year or once a day. Due to the shift of potassium into muscle cells,serum potassium level is decreased during attacks and it is in the normal range between twoattacks. A 21 year old male patient seen in the emergency department due to a hypokalemicparalysis attack occurring without any obvious triggering factor was presented in this article.

  5. HYPOKALEMIC PERIODIC PARALYSIS AND THE DIHYDROPYRIDINE RECEPTOR (CACNLIA3) - GENOTYPE-PHENOTYPE CORRELATIONS FOR 2 PREDOMINANT MUTATIONS AND EVIDENCE FOR THE ABSENCE OF A FOUNDER EFFECT IN 16 CAUCASIAN FAMILIES

    NARCIS (Netherlands)

    ELBAZ, A; VALESANTOS, J; JURKATROTT, K; LAPIE, P; OPHOFF, RA; BADY, B; LINKS, TP; PIUSSAN, C; VILA, A; MONNIER, N; PADBERG, GW; ABE, K; FEINGOLD, N; GUIMARAES, J; WINTZEN, AR; VANDERHOEVEN, JH; SAUDUBRAY, JM; GRUNFELD, JP; LENOIR, G; NIVET, H; ECHENNE, B; FRANTS, RR; FARDEAU, M; LEHMANNHORN, F; FONTAINE, B

    1995-01-01

    Hypokalemic periodic paralysis (hypoPP) is an autosomal dominant disorder belonging to a group of muscle diseases involving the abnormal function of ion channels. This group of muscle diseases also comprises hyperkalemic periodic paralysis and paramyotonia congenita, both sodium-channel diseases, an

  6. Normokalemic periodic paralysis revisited: does it exist?

    Science.gov (United States)

    Chinnery, Patrick F; Walls, Timothy J; Hanna, Michael G; Bates, David; Fawcett, Peter R W

    2002-08-01

    Normokalemic periodic paralysis (normoKPP) is well established in the literature, but there are doubts as to whether it exists as a discrete entity. Retrospective clinical and molecular analysis has confirmed suspicions that most normoKPP families actually have a variant of hyperkalemic periodic paralysis (hyperKPP) due to a mutation of the muscle-specific sodium channel gene (SCN4A). However, the original normoKPP family described by Poskanzer and Kerr (Poskanzer DC, Kerr DNS. A third type of periodic paralysis, with normokalemia and favourable response to sodium chloride. Am J Med 1961;31:328-342) has remained unchallenged. We identified the Met1592Val mutation of SCN4A in an affected descendent of this original normoKPP family. This is the final piece in the puzzle: normoKPP is actually a variant of hyperKPP and is not a distinct disorder.

  7. [Hypokalemic periodic paralysis. A case report].

    Science.gov (United States)

    Areta-Higuera, J D; Algaba-Montes, M; Oviedo-García, A Á

    2014-01-01

    Periodic paralysis is a rare disorder that causes episodes of severe muscle weakness that can be confused with other diseases, including epilepsy or myasthenia gravis. Hyperkalemic and hypokalemic paralysis are included within these diseases, the latter being divided into periodic paralysis (familial, thyrotoxic or sporadic) and non-periodic paralysis. In this regard, we present a case of familial hypokalemic periodic paralysis in an eighteen year-old female who was diagnosed with epilepsy in childhood, as well as a subclinical hypothyroidism (for which she received replacement therapy) months ago. The diagnosis was made by the anamnesis and the confirmation of hypokalemia. Copyright © 2012 Sociedad Española de Médicos de Atención Primaria (SEMERGEN). Publicado por Elsevier España. All rights reserved.

  8. Period-doubling cascades for large perturbations of Henon families

    OpenAIRE

    Sander, Evelyn; Yorke, James A.

    2009-01-01

    The Henon family has been shown to have period-doubling cascades. We show here that the same occurs for a much larger class: Large perturbations do not destroy cascades. Furthermore, we can classify the period of a cascade in terms of the set of orbits it contains, and count the number of cascades of each period. This class of families extends a general theory explaining why cascades occur.

  9. Cantor families of periodic solutions for completely resonant wave equations

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    We present recent existence results of Cantor families of small amplitude periodic solutions for completely resonant nonlinear wave equations. The proofs rely on the Nash-Moser implicit function theory and variational methods.

  10. Molecular and pathophysiologic mechanisms of hyperkalemic metabolic acidosis.

    Science.gov (United States)

    DuBose, T D

    2000-01-01

    In summary, hyperkalemia may have a dramatic impact on ammonium production and excretion. Chronic hyperkalemia decreases ammonium production in the proximal tubule and whole kidney, inhibits absorption of NH4+ in the mTALH, reduces medullary interstitial concentrations of NH4+ and NH3, and decreases entry of NH4+ and NH3 into the medullary collecting duct. The potential for development of a hyperchloremic metabolic acidosis is greatly augmented when renal insufficiency with associated reduction in functional renal mass coexists with the hyperkalemia, or in the presence of aldosterone deficiency or resistance. Such a cascade of events helps to explain, in part, the hyperchloremic metabolic acidosis and reduction in net acid excretion characteristic of several experimental models of hyperkalemic-hyperchloremic metabolic acidosis including: obstructive nephropathy, selective aldosterone deficiency, and chronic amiloride administration (7.9).

  11. Familial hypokalemic periodic paralysis : clinical, diagnostic and therapeutic aspects

    NARCIS (Netherlands)

    Links, T P; Smit, A.J.; Molenaar, W M; Zwarts, M J; Oosterhuis, H.J.G.H.

    1994-01-01

    Five generations of a family with hypokalemic periodic paralysis (HOPP) were studied. Of the 120 screened family members, 64 were found to have HOPP of which 38 were suffering from attacks. In the other 26 the diagnosis was made on account of vacuolar myopathy, a reduced muscle fiber conduction velo

  12. Catalan family companies with high growth during the crisis period

    Directory of Open Access Journals (Sweden)

    Nuria Arimany-Serrat

    2016-11-01

    Full Text Available Purpose: This study aims to analyze the financial and economic health of the Catalan family companies with high growth during the crisis period (2008-2013, as an engine of job creation and economic development of the territory, identifying the economic and financial characteristics. Design/methodology/approach: The data used comes from the financial statements of the high growth Catalan companies during the crisis period of 2008-2013, in particular it collects a sample of the 140 high growth Catalan companies where a 70% of them are family businesses, there is a financial and economic analysis using descriptive statistics of these family businesses with a regression model to achieve results and conclusions. Findings: During the 2008-2013 crisis, Catalan family companies with high growth that are mostly small businesses have a good economic and financial health, with moderate innovation and a great potential for survival. Research limitations/implications: It would be convenient to make the same study in similar territories where most of the companies are family businesses, in order to compare the financial and economic analysis, valuing the impact of these companies in the business. Practical implications: It allows valuing the projection of these kinds of family companies, mostly in Catalonia, in a financial and economic level, identifying if they have a proper financial strategy. Social implications: The results show that these companies have great financial and economic health and have a better response during a crisis period. Originality / value: The study shows that family businesses have moderate indebtedness and acceptable rendibility in the crisis period, although, since they are small businesses the lack of innovation and patents can lead to future problems.

  13. Hyperkalemic distal renal tubular acidosis caused by immunosuppressant treatment with tacrolimus in a liver transplant patient: case report.

    Science.gov (United States)

    Riveiro-Barciela, M; Campos-Varela, I; Tovar, J L; Vargas, V; Simón-Talero, M; Ventura-Cots, M; Crespo, M; Bilbao, I; Castells, L

    2011-12-01

    Nephrotoxicity is one of the most common side effects of long-term immunosuppressive therapy with calcineurin inhibitors. We describe a case of distal renal tubular acidosis secondary to tacrolimus administration. A 43-year-old man with end-stage liver disease due to hepatitis C and B virus infections and alcoholic cirrhosis received a liver transplantation under immunosuppressive treatment with tacrolimus and mycophenolate mofetil. In the postoperative period, the patient developed hyperkalemic hyperchloremic metabolic acidosis, with a normal serum anion gap and a positive urinary anion gap, suggesting distal renal tubular acidosis. We excluded other causes of hyperkalemia. Administration of intravenous bicarbonate, loop diuretics, and oral resin exchanger corrected the acidosis and potassium levels. Distal renal tubular acidosis is one of several types of nephrotoxicity induced by tacrolimus treatment, resulting from inhibition of potassium secretion in the collecting duct. Treatment to correct the acidosis and hyperkalemia should be promptly initiated, and the tacrolimus dose adjusted when possible.

  14. Rhabdomyolysis following severe hypokalemia caused by familial hypokalemic periodic paralysis

    Science.gov (United States)

    Jung, Young-Lee; Kang, Jae-Young

    2017-01-01

    Rhabdomyolysis continues to appear with increasing frequency and represents a medical emergency requiring rapid appropriate treatment. One of the unusual causes of nontraumatic rhabdomyolysis is hypokalemic periodic paralysis without secondary causes. Primary hypokalemic periodic paralysis is a rare genetic disease characterized by episodic attacks of muscle weakness due to decreases in serum potassium. A 30-year-old woman who had 3 episodic attacks of hypokalemic periodic paralysis was admitted in emergency room with sudden onset symmetrical muscle weakness. After several hours, she started to complain myalgia and severe ache in both calves without any changes. Laboratory test showed markedly elevated creatine phosphokinase, lactic dehydrogenase levels with hypokalemia, rhabdomyolysis resulting from hypokalemia was diagnosed. Here, we report an unusual case of rhabdomyolysis caused by severe hypokalemia, which was suggested a result of familial hypokalemic periodic paralysis. PMID:28255549

  15. Rhabdomyolysis following severe hypokalemia caused by familial hypokalemic periodic paralysis.

    Science.gov (United States)

    Jung, Young-Lee; Kang, Jae-Young

    2017-02-16

    Rhabdomyolysis continues to appear with increasing frequency and represents a medical emergency requiring rapid appropriate treatment. One of the unusual causes of nontraumatic rhabdomyolysis is hypokalemic periodic paralysis without secondary causes. Primary hypokalemic periodic paralysis is a rare genetic disease characterized by episodic attacks of muscle weakness due to decreases in serum potassium. A 30-year-old woman who had 3 episodic attacks of hypokalemic periodic paralysis was admitted in emergency room with sudden onset symmetrical muscle weakness. After several hours, she started to complain myalgia and severe ache in both calves without any changes. Laboratory test showed markedly elevated creatine phosphokinase, lactic dehydrogenase levels with hypokalemia, rhabdomyolysis resulting from hypokalemia was diagnosed. Here, we report an unusual case of rhabdomyolysis caused by severe hypokalemia, which was suggested a result of familial hypokalemic periodic paralysis.

  16. Anaesthetic management of a patient with familial normokalaemic periodic paralysis.

    LENUS (Irish Health Repository)

    Walsh, F

    2012-02-03

    PURPOSE: We describe the anaesthetic management of a patient with the autosomal dominant inherited disease, normokalaemic periodic paralysis. The disease results in intermittent bouts of limb and respiratory muscular weakness in association with hypothermia, stress, prolonged fasting or exercise. Unlike hypokalaemic and hyperkalaemic periodic paralysis, the more common variants of the disease, normokalaemic periodic paralysis is not accompanied by alterations in the plasma potassium concentration. CLINICAL FEATURES: A five-year-old boy presented for emergency scrotal exploration. He had a family history of periodic paralysis and had experienced previous episodes of weakness, two of which had required hospitalization for respiratory distress. On admission there was no evidence of weakness and serum potassium concentration was 4.2 mMol.L-1. A spinal anaesthetic was performed and the procedure was uncomplicated by muscle paralysis above the level of the spinal block. CONCLUSION: Avoidance of known precipitating factors and judicious use of neuromuscular blocking drugs has been advocated in patients with this disorder presenting for surgery. In appropriate circumstances, spinal anaesthesia represents a useful option in patients with normokalaemic periodic paralysis.

  17. Periodic health evaluation of adults: a survey of family physicians.

    Science.gov (United States)

    Luckmann, R; Melville, S K

    1995-06-01

    The routine periodic health evaluation (PHE) is a popular format in primary care practice for the delivery of clinical preventive services to adults, but knowledge of the current pattern of use and of the content of the PHE is limited. We surveyed a random sample of 567 family physicians (response rate, 60.1%) in New England regarding their approach to and attitudes about the PHE of adults. Family physicians reported spending a mean of 35.2% (11.6 hours per week) of their total ambulatory care time on adult PHEs, which were usually scheduled for 20 to 30 minutes each. All respondents recommended a PHE to men > or = 50 years old and to women > or = 40 years old, and more than 90% recommended a PHE to younger patients. Most physicians (80.7%) reported that the PHE is their primary mechanism for delivering preventive services, and 90.6% believed that the PHE should include a comprehensive physical examination. The mean number of physical examination items performed during the PHE was 11.6 to 13.9, depending on patient age and sex. As part of the PHE, most physicians (71.6% to 90.7%, depending on patient age and sex) ordered one or more screening laboratory tests not recommended by the US Preventive Services Task Force. Utilization of fewer laboratory tests was associated with residency training, employment in a health maintenance organization (HMO), and limited concern about malpractice suits. Family physicians reported spending a substantial amount of time on the PHE of adults, performing extensive screening physical examinations and many screening laboratory tests of unknown effectiveness. Among family physicians, there is considerable unexplained variation in the form and content of the PHE.

  18. Periodic paralysis: understanding channelopathies.

    Science.gov (United States)

    Lehmann-Horn, Frank; Jurkat-Rott, Karin; Rüdel, Reinhardt

    2002-01-01

    Familial periodic paralyses are typical channelopathies (i.e., caused by functional disturbances of ion channel proteins). The episodes of flaccid muscle weakness observed in these disorders are due to underexcitability of sarcolemma leading to a silent electromyogram and the lack of action potentials even upon electrical stimulation. Interictally, ion channel malfunction is well compensated, so that special exogenous or endogenous triggers are required to produce symptoms in the patients. An especially obvious trigger is the level of serum potassium (K+), the ion responsible for resting membrane potential and degree of excitability. The clinical symptoms can be caused by mutations in genes coding for ion channels that mediate different functions for maintaining the resting potential or propagating the action potential, the basis of excitability. The phenotype is determined by the type of functional defect brought about by the mutations, rather than the channel effected, because the contrary phenotypes hyperkalemic periodic paralysis (HyperPP) and hypokalemic periodic paralysis (HypoPP) may be caused by point mutations in the same gene. Still, the common mechanism for inexcitability in all known episodic-weakness phenotypes is a long-lasting depolarization that inactivates sodium ion (Na+) channels, initiating the action potential.

  19. Genetic analysis and clinical features of familial hypokalemic periodic paralysis

    Directory of Open Access Journals (Sweden)

    Hui-li ZHANG

    2014-06-01

    Full Text Available Background To investigate the gene mutation and clinical features of hypokalemic periodic paralysis (HypoPP in a Han family. Methods Mutation analyses of CACNA1S, SCN4A and KCNE3 gene were screened by DNA direct sequencing in the proband (Ⅲ3. Then, other patients and one asymptomatic relative were tested for the mutation detected in the proband before. Besides, clinical information was collected and analyzed carefully so as to detect whether the mutations were responsible for HypoPP.  Results KCNE3 gene was not detected in the propositus (Ⅲ 3. Mutations of IVS25-194C/T in CACNA1S gene were detected in the propositus (Ⅲ 3 and other patients (Ⅱ 1, Ⅲ 4, Ⅳ 3, while it was not detected in the asymptomatic relative (Ⅲ1. Given that it was an intron mutation, we presumed that it was not responsible for HypoPP in this family. In addition, mutations of IVS18-130G/A in SCN4A gene were detected in all patients (except for Ⅰ1 and asymptomatic relative (Ⅲ 1. Since it was an intron mutation and it was detected in symptomatic or asymptomatic members simultaneously, we also presumed that it was not responsible for HypoPP in this family. Interestingly, a missense mutation (V662I of c.1984G > A in exon 12 of SCN4A gene was detected in the proband (Ⅲ 3 and asymptomatic relative (Ⅲ 1. However, it was not detected in other symptomatic members ( Ⅱ 1, Ⅲ 4, Ⅳ 3. Based on clinical information and bioinformatics, we presumed that it was not causative mutation for the disease in this pedigree.  Conclusions This pedigree research enriched the data of gene mutation and clinical features of HypoPP in China. Besides for gene KCNE3, CACNA1S and SCN4A, other gene mutations accounted for HypoPP in the Han family should be further studied. doi: 10.3969/j.issn.1672-6731.2014.06.006

  20. On critical values concerning the evolution of the long period families

    Science.gov (United States)

    Hou, Xi-Yun

    2009-04-01

    In a previous paper, we proposed another special critical value concerning the evolution of the long period family around the equilateral equilibrium points, besides the two values given by Henrard. Are there any other special critical values? After studying the stability curves of the long period family carefully, we gave a negative answer. During the study, we found an interesting family of periodic orbits which we called the homo family. We studied the evolution of this family following the increase of μ. With these findings, we were able to explain the origin of the four branches of periodic families emanating from L4 and the stability results of the equilateral equilibrium points.

  1. On critical values concerning the evolution of the long period families

    Institute of Scientific and Technical Information of China (English)

    Xi-Yun Hou

    2009-01-01

    In a previous paper, we proposed another special critical value concerning the evolution of the long period family around the equilateral equilibrium points, besides the two values given by Henrard. Are there any other special critical values? After studying the stability curves of the long period family carefully, we gave a negative answer. During the study, we found an interesting family of periodic orbits which we called the homo family. We studied the evolution of this family following the increase of μ. With these findings, we were able to explain the origin of the four branches of periodic families emanating from L4 and the stability results of the equilateral equilibrium points.

  2. Family Engagement in the Perinatal Period and Infant Rights

    Science.gov (United States)

    Tsantefski, Menka; Humphreys, Cathy; Jackson, Alun C.

    2011-01-01

    The meaning of human rights for children is a contested issue; the notion of rights for unborn babies poses additional complexity. Drawing on data from a prospective case study of a specialist drug and alcohol obstetric provider and the statutory child protection service, this article discusses family engagement within a child-rights framework,…

  3. Catalan family companies with high growth during the crisis period

    National Research Council Canada - National Science Library

    Nuria Arimany-Serrat; Elisenda Tarrats-Pons

    2016-01-01

    .... Design/methodology/approach: The data used comes from the financial statements of the high growth Catalan companies during the crisis period of 2008-2013, in particular it collects a sample of the 140 high growth Catalan companies where a 70...

  4. Familial gastric cancer : guidelines for diagnosis, treatment and periodic surveillance

    NARCIS (Netherlands)

    Kluijt, Irma; Sijmons, Rolf H.; Hoogerbrugge, Nicoline; Plukker, John T.; de Jong, Daphne; van Krieken, J. Han; van Hillegersberg, Richard; Ligtenberg, Marjolijn; Bleiker, Eveline; Cats, Anemieke

    2012-01-01

    Hereditary diffuse gastric cancer (HDGC) is a relatively rare disorder, with a mutated CDH1 gene as the only known cause. Carriers of a germline mutation in CDH1 have a lifetime risk of > 80% of developing diffuse gastric cancer. As periodic gastric surveillance is of limited value in detecting earl

  5. [Familial gastric cancer: diagnosis, treatment and periodic surveillance

    NARCIS (Netherlands)

    Kluijt, I.; Sijmons, R.H.; Hoogerbrugge, N.; Vasen, H.F.; Cats, A.

    2011-01-01

    The only known genetic causes of hereditary diffuse gastric cancer (HDGC) are germline mutations in the CDH1 gene.- CDH1 mutation carriers have a lifetime risk of 70-80% of developing diffuse gastric cancer. As periodic gastric surveillance is of limited value in detecting early stages of HDGC, prop

  6. A family of Nikishin systems with periodic recurrence coefficients

    CERN Document Server

    Delvaux, Steven; Lagomasino, Guillermo López

    2011-01-01

    Suppose we have a Nikishin system of $p$ measures with the $k$th generating measure of the Nikishin system supported on an interval $\\Delta_k\\subset\\er$ with $\\Delta_k\\cap\\Delta_{k+1}=\\emptyset$ for all $k$. It is well known that the corresponding staircase sequence of multiple orthogonal polynomials satisfies a $(p+2)$-term recurrence relation whose recurrence coefficients, under appropriate assumptions on the generating measures, have periodic limits of period $p$. (The limit values depend only on the positions of the intervals $\\Delta_k$.) Taking these periodic limit values as the coefficients of a new $(p+2)$-term recurrence relation, we construct a canonical sequence of monic polynomials $\\{P_{n}\\}_{n=0}^{\\infty}$, the so-called \\emph{Chebyshev-Nikishin polynomials}. We show that the polynomials $P_{n}$ themselves form a sequence of multiple orthogonal polynomials with respect to some Nikishin system of measures, with the $k$th generating measure being absolutely continuous on $\\Delta_{k}$. In this way w...

  7. Families of asymmetric periodic solutions in the restricted four-body problem

    Science.gov (United States)

    Papadakis, K. E.

    2016-12-01

    Very recently, we presented five of the basic families of the network of periodic orbits of the restricted four-body problem which are simple, i.e. one intersection with the horizontal x-axis at the half period, symmetric with respect to the same axis and asymmetric with respect to the vertical y-axis. In the present work, using these families, we found series of asymmetric critical orbits for various values of the primaries m2 and m3. From these critical orbits we calculate and present five new families of simple periodic orbits which are asymmetric with respect to both the x- and y-axis. Additionally, we describe a grid method in the (x0, dot{x}0) plane and we obtain initial conditions for new asymmetric double-periodic orbits. We determine ten families of asymmetric double-periodic orbits from the bifurcations of the previous five asymmetric families using the special generating horizontally critical periodic orbits. The stability of each calculated asymmetric periodic orbit is also studied. Characteristic curves as well as stability diagrams of these families are illustrated. In the last section we present the evolution of the five basic families of simple asymmetric periodic orbits when the primaries are the Sun the Jupiter and the 2797 Teucer Asteroid.

  8. A family of Nikishin systems with periodic recurrence coefficients

    Energy Technology Data Exchange (ETDEWEB)

    Delvaux, Steven; Lopez, Abey; Lopez, Guillermo L

    2013-01-31

    Suppose we have a Nikishin system of p measures with the kth generating measure of the Nikishin system supported on an interval {Delta}{sub k} subset of R with {Delta}{sub k} Intersection {Delta}{sub k+1} = Empty-Set for all k. It is well known that the corresponding staircase sequence of multiple orthogonal polynomials satisfies a (p+2)-term recurrence relation whose recurrence coefficients, under appropriate assumptions on the generating measures, have periodic limits of period p. (The limit values depend only on the positions of the intervals {Delta}{sub k}.) Taking these periodic limit values as the coefficients of a new (p+2)-term recurrence relation, we construct a canonical sequence of monic polynomials {l_brace}P{sub n}{r_brace}{sub n=0}{sup {infinity}}, the so-called Chebyshev-Nikishin polynomials. We show that the polynomials P{sub n} themselves form a sequence of multiple orthogonal polynomials with respect to some Nikishin system of measures, with the kth generating measure being absolutely continuous on {Delta}{sub k}. In this way we generalize a result of the third author and Rocha [22] for the case p=2. The proof uses the connection with block Toeplitz matrices, and with a certain Riemann surface of genus zero. We also obtain strong asymptotics and an exact Widom-type formula for functions of the second kind of the Nikishin system for {l_brace}P{sub n}{r_brace}{sub n=0}{sup {infinity}}. Bibliography: 27 titles.

  9. Periodic Orbit Families in the Gravitational Field of Irregular-shaped Bodies

    CERN Document Server

    Jiang, Yu

    2016-01-01

    The discovery of binary and triple asteroids in addition to the execution of space missions to minor celestial bodies in the past several years have focused increasing attention on periodic orbits around irregular-shaped celestial bodies. In the present work, we adopt a polyhedron shape model for providing an accurate representation of irregular-shaped bodies, and employ the model to calculate their corresponding gravitational and effective potentials. We also investigate the characteristics of periodic orbit families and the continuation of periodic orbits. We prove a fact, which provides a conserved quantity that permits restricting the number of periodic orbits in a fixed energy curved surface about an irregular-shaped body. The collisions of Floquet multipliers are maintained during the continuation of periodic orbits around the comet 1P/Halley. Multiple bifurcations in the periodic orbit families about irregular-shaped bodies are also discussed. Three bifurcations in the periodic orbit family have been f...

  10. Episodic Muscle Tremors in a Quarter Horse: Resemblance to Hyperkalemic Periodic Paralysis

    OpenAIRE

    Steiss, Janet E.; Naylor, Jonathan M.

    1986-01-01

    A three year old Quarter Horse stallion was presented with a one year history of episodes of generalized muscle tremors and stiffness, and spasm of the muscles of facial expression, lasting 10-15 minutes. Between attacks, the horse was either normal or had a localized muscle tremor in the flank region. Episodes appeared unrelated to exercise. The major abnormal findings included 1) a rise in plasma potassium from a resting level of 4.4 to 7.9 mmol/L during an attack and 2) electromyographic f...

  11. Persistent superior oblique paresis as a manifestation of familial periodic cerebellar ataxia.

    OpenAIRE

    Bain, P.G.; Larkin, G. B.; Calver, D M; O'Brien, M D

    1991-01-01

    A brother and sister complained of persistent diplopia due to superior oblique palsies. The cause of their symptoms became apparent when they were diagnosed as having familial periodic cerebellar ataxia (FPCA), a rare autosomal dominant condition. Oral acetazolamide (250 mg twice daily) not only prevented all the periodic symptoms but also relieved their diplopia, which had been present between attacks.

  12. A new mutation causing autosomal dominant periodic fever syndrome in a Danish family

    DEFF Research Database (Denmark)

    Weyhreter, Heike; Schwartz, Marianne; Kristensen, Tim D

    2003-01-01

    We describe four members in a family of 8 individuals over 3 generations with the autosomal dominant inherited periodic fever syndrome tumor necrosis factor receptor-associated periodic syndrome (TRAPS). The patients had recurrent episodes of fever, abdominal pain, arthritis, and rash. We examined...

  13. A new mutation causing autosomal dominant periodic fever syndrome in a Danish family

    DEFF Research Database (Denmark)

    Weyhreter, Heike; Schwartz, Marianne; Kristensen, Tim D;

    2003-01-01

    We describe four members in a family of 8 individuals over 3 generations with the autosomal dominant inherited periodic fever syndrome tumor necrosis factor receptor-associated periodic syndrome (TRAPS). The patients had recurrent episodes of fever, abdominal pain, arthritis, and rash. We examine...

  14. Periodic paralysis.

    Science.gov (United States)

    Fontaine, Bertrand

    2008-01-01

    Periodic paralyses are rare diseases characterized by severe episodes of muscle weakness concomitant to variations in blood potassium levels. It is thus usual to differentiate hypokalemic, normokalemic, and hyperkalemic periodic paralysis. Except for thyrotoxic hypokalemic periodic paralysis and periodic paralyses secondary to permanent changes of blood potassium levels, all of these diseases are of genetic origin, transmitted with an autosomal-dominant mode of inheritance. Periodic paralyses are channelopathies, that is, diseases caused by mutations in genes encoding ion channels. The culprit genes encode for potassium, calcium, and sodium channels. Mutations of the potassium and calcium channel genes cause periodic paralysis of the same type (Andersen-Tawil syndrome or hypokalemic periodic paralysis). In contrast, distinct mutations in the muscle sodium channel gene are responsible for all different types of periodic paralyses (hyper-, normo-, and hypokalemic). The physiological consequences of the mutations have been studied by patch-clamp techniques and electromyography (EMG). Globally speaking, ion channel mutations modify the cycle of muscle membrane excitability which results in a loss of function (paralysis). Clinical physiological studies using EMG have shown a good correlation between symptoms and EMG parameters, enabling the description of patterns that greatly enhance molecular diagnosis accuracy. The understanding of the genetics and pathophysiology of periodic paralysis has contributed to refine and rationalize therapeutic intervention and will be without doubts the basis of further advances.

  15. Normokalemic periodic paralysis is not a distinct disease.

    Science.gov (United States)

    Song, Young-Wha; Kim, Sung-Jo; Heo, Tae-Hwe; Kim, Man-Ho; Kim, June-Bum

    2012-12-01

    Recent molecular studies of the original cases of normokalemic periodic paralysis (normoKPP) have raised suspicions that these families actually had hyperkalemic periodic paralysis (hyperKPP) due to mutations in the skeletal muscle sodium channel gene SCN4A. However, there is still a debate about the existence of normoKPP. We screened 230 individuals with primary periodic paralysis for mutations in the SCN4A, CACNA1S, and KCNJ2 genes. All patients had either a hyperKPP or a hypoKPP phenotype, and none had a normoKPP phenotype. In 4 hyperKPP patients from 2 families, molecular analyses revealed Arg675Gly and Arg675Gln mutations of SCN4A, which were previously reported to cause normoKPP. Each patient exhibited the characteristic clinical and laboratory features (including hyperkalemia during spontaneous attacks) of hyperKPP. Our findings support the notion that normoKPP is not a distinct disease. Copyright © 2012 Wiley Periodicals, Inc.

  16. Altered fast and slow inactivation of the N440K Nav1.4 mutant in a periodic paralysis syndrome.

    Science.gov (United States)

    Lossin, Christoph; Nam, Tai-Seung; Shahangian, Shahab; Rogawski, Michael A; Choi, Seok-Yong; Kim, Myeong-Kyu; Sunwoo, Il-Nam

    2012-09-04

    To electrophysiologically characterize the Na(v)1.4 mutant N440K found in a Korean family with a syndrome combining symptoms of paramyotonia congenita, hyperkalemic periodic paralysis, and potassium-aggravated myotonia. We characterized transiently expressed wild-type and mutant Na(v)1.4 using whole-cell voltage-clamp analysis. N440K produced a significant depolarizing shift in the voltage dependence of fast inactivation and increased persistent current and acceleration in fast inactivation recovery, which gave rise to a 2-fold elevation in the dynamic availability of the mutant channels. In addition, the mutant channels required substantially longer and stronger depolarization to enter the slow-inactivated state. N440K causes a gain of function consistent with skeletal muscle hyperexcitability as observed in individuals with the mutation. How the same mutation results in distinct phenotypes in the 2 kindreds remains to be determined.

  17. PERMANENT MUSCLE WEAKNESS IN FAMILIAL HYPOKALEMIC PERIODIC PARALYSIS - CLINICAL, RADIOLOGICAL AND PATHOLOGICAL ASPECTS

    NARCIS (Netherlands)

    LINKS, TP; ZWARTS, MJ; WILMINK, JT; MOLENAAR, WM; OOSTERHUIS, HJGH

    1990-01-01

    Myopathy accompanying familial hypokalemic periodic paralysis (HPP) is much less well documented than the paralytic attacks from which the disease derives its name. Eleven affected members of a large kinship with HPP were studied clinically and radiologically for the presence of myopathy. In 4 patie

  18. Treatment for periodic paralysis.

    Science.gov (United States)

    Sansone, V; Meola, G; Links, T P; Panzeri, M; Rose, M R

    2008-01-23

    Primary periodic paralyses are rare inherited muscle diseases characterised by episodes of flaccid weakness affecting one or more limbs, lasting several hours to several days, caused by mutations in skeletal muscle channel genes. The objective of this review was to systematically review treatment of periodic paralyses. We searched the Cochrane Neuromuscular Disease Group Trials Register, MEDLINE (from January 1966 to July 2007), and EMBASE (from January 1980 to July 2007) and any other available international medical library sources from the University of Milan for randomised trials. We included randomised (including cross-over studies) and quasi-randomised trials in participants with primary periodic paralyses, in which any form of treatment, including physical therapy and alternative therapies, was compared to placebo or another treatment. Our primary outcome measure was the change in attack severity or frequency by eight weeks from the start of treatment. Our secondary outcome measures were: change in muscle strength and mass; change in Quality of Life, using Short Form 36 (SF36) or similar; preference of treatment strategy; adverse effects at eight weeks. Three studies met our inclusion criteria. In one study dichlorphenamide (DCP) vs placebo was tested in two groups of participants: 42 with hypokalemic periodic paralysis (HypoPP) and 31 with hyperkalemic periodic paralysis (HyperPP), based on clinical criteria. Thirty-four of 42 participants with hypokalemic periodic paralysis completed both treatment phases. For the 34 participants having attack rate data for both treatment phases, the mean improvement in attack rate (P = 0.02) and severity-weighted attack rate (P = 0.01) on DCP relative to placebo were statistically significant. Fifteen preferred DCP, three placebo and six their baseline medication. Twenty-four of 31 participants with hyperkalemic periodic paralysis completed both treatment phases: for the 16 participants who had attack rate data for both

  19. Unified Modeling of Familial Mediterranean Fever and Cryopyrin Associated Periodic Syndromes

    OpenAIRE

    Yasemin Bozkurt; Alper Demir; Burak Erman; Ahmet Gül

    2015-01-01

    Research Article Unified Modeling of Familial Mediterranean Fever and Cryopyrin Associated Periodic Syndromes Yasemin Bozkurt,1 Alper Demir,1 Burak Erman,1 and Ahmet Gül2 1Computational and Quantitative Biology Lab, Koc University, 34450 Istanbul, Turkey 2Division of Rheumatology,Department of InternalMedicine, Istanbul Faculty ofMedicine, IstanbulUniversity, 34093 Istanbul, Turkey Correspondence should be addressed to Yasemin Bozkurt; Received 18 Sept...

  20. Families of periodic orbits in Hill's problem with solar radiation pressure: application to Hayabusa 2

    Science.gov (United States)

    Giancotti, Marco; Campagnola, Stefano; Tsuda, Yuichi; Kawaguchi, Jun'ichiro

    2014-11-01

    This work studies periodic solutions applicable, as an extended phase, to the JAXA asteroid rendezvous mission Hayabusa 2 when it is close to target asteroid 1999 JU3. The motion of a spacecraft close to a small asteroid can be approximated with the equations of Hill's problem modified to account for the strong solar radiation pressure. The identification of families of periodic solutions in such systems is just starting and the field is largely unexplored. We find several periodic orbits using a grid search, then apply numerical continuation and bifurcation theory to a subset of these to explore the changes in the orbit families when the orbital energy is varied. This analysis gives information on their stability and bifurcations. We then compare the various families on the basis of the restrictions and requirements of the specific mission considered, such as the pointing of the solar panels and instruments. We also use information about their resilience against parameter errors and their ground tracks to identify one particularly promising type of solution.

  1. A novel mutation in the calcium channel gene in a family with hypokalemic periodic paralysis.

    Science.gov (United States)

    Hirano, Makito; Kokunai, Yosuke; Nagai, Asami; Nakamura, Yusaku; Saigoh, Kazumasa; Kusunoki, Susumu; Takahashi, Masanori P

    2011-10-15

    Hypokalemic periodic paralysis (HypoPP) type 1 is an autosomal dominant disease caused by mutations in the Ca(V)1.1 calcium channel encoded by the CACNA1S gene. Only seven mutations have been found since the discovery of the causative gene in 1994. We describe a patient with HypoPP who had a high serum potassium concentration after recovery from a recent paralysis, which complicated the correct diagnosis. This patient and other affected family members had a novel mutation, p.Arg900Gly, in the CACNA1S gene. Copyright © 2011 Elsevier B.V. All rights reserved.

  2. Family History in Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA) Syndrome.

    Science.gov (United States)

    Manthiram, Kalpana; Nesbitt, Emily; Morgan, Thomas; Edwards, Kathryn M

    2016-09-01

    The goal of this study was to describe family history and inheritance patterns in patients with periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) syndrome. We performed a case-control study to compare the family histories of patients with PFAPA recruited from Vanderbilt University Medical Center and matched healthy control subjects from a pediatric primary care practice in Nashville, Tennessee, by using a structured questionnaire. Characteristics of paired case subjects, control subjects, and their family members were compared by using McNemar's test and Wilcoxon signed-rank tests. Eighty PFAPA index case subjects and 80 control subjects were recruited. Eighteen PFAPA case subjects (23%) had ≥1 family member with PFAPA. Parents of PFAPA index case subjects were more likely to have recurrent pharyngitis (36% vs 16%; P < .001) and recurrent aphthous stomatitis (46% vs 28%; P = .002) compared with parents of control subjects. Siblings of case subjects had a higher prevalence of PFAPA (10% vs 2%; P = .04), recurrent pharyngitis (24% vs 10%; P = .03), and recurrent aphthous stomatitis (27% vs 7%; P = .003) compared with siblings of control subjects. A portion of PFAPA case subjects seems to be familial, implying an inherited genetic predisposition to the disorder and/or shared environmental exposures. First-degree relatives (parents and siblings) of patients with PFAPA have a higher prevalence of recurrent pharyngitis and aphthous stomatitis than relatives of control subjects, which suggests that these disorders represent reduced penetrance phenotypes of PFAPA. Further characterization of the genetics and inflammatory profiles of these patients and their relatives is warranted. Copyright © 2016 by the American Academy of Pediatrics.

  3. New mutations of SCN4A cause a potassium-sensitive normokalemic periodic paralysis.

    Science.gov (United States)

    Vicart, S; Sternberg, D; Fournier, E; Ochsner, F; Laforet, P; Kuntzer, T; Eymard, B; Hainque, B; Fontaine, B

    2004-12-14

    Periodic paralysis is classified into hypokalemic (hypoPP) and hyperkalemic (hyperPP) periodic paralysis according to variations of blood potassium levels during attacks. To describe new mutations in the muscle sodium channel gene SCN4A that cause periodic paralysis. A thorough clinical, electrophysiologic, and molecular study was performed of four unrelated families who presented with periodic paralysis. The nine affected members had episodes of muscle weakness reminiscent of both hyperPP and hypoPP. A provocative test with potassium chloride was positive in two patients. However, repeated and carefully performed tests of blood potassium levels during attacks resulted in normal potassium levels. Remarkably, two patients experienced hypokalemic episodes of paralysis related to peculiar provocative factors (corticosteroids and thyrotoxicosis). Similarly to hyperPP, electromyography in nine patients revealed increased compound muscle action potentials after short exercise and a delayed decline during rest after long exercise as well as myotonic discharges in one patient. With use of molecular genetic analysis of the gene SCN4A, three new mutations were found affecting codon 675. They resulted in an amino acid substitution of a highly conserved arginine (R) to either a glycine (G), a glutamine (Q), or a tryptophan (W). Interestingly, hypoPP is caused by both mutations affecting nearby codons as well as the change of an arginine into another amino acid. A potassium-sensitive and normokalemic type of periodic paralysis caused by new SCN4A mutations at codon 675 is reported.

  4. Sensitive Periods, Vasotocin-Family Peptides, and the Evolution and Development of Social Behavior

    Directory of Open Access Journals (Sweden)

    Nicole M. Baran

    2017-08-01

    Full Text Available Nonapeptides, by modulating the activity of neural circuits in specific social contexts, provide an important mechanism underlying the evolution of diverse behavioral phenotypes across vertebrate taxa. Vasotocin-family nonapeptides, in particular, have been found to be involved in behavioral plasticity and diversity in social behavior, including seasonal variation, sexual dimorphism, and species differences. Although nonapeptides have been the focus of a great deal of research over the last several decades, the vast majority of this work has focused on adults. However, behavioral diversity may also be explained by the ways in which these peptides shape neural circuits and influence social processes during development. In this review, I synthesize comparative work on vasotocin-family peptides during development and classic work on early forms of social learning in developmental psychobiology. I also summarize recent work demonstrating that early life manipulations of the nonapeptide system alter attachment, affiliation, and vocal learning in zebra finches. I thus hypothesize that vasotocin-family peptides are involved in the evolution of social behaviors through their influence on learning during sensitive periods in social development.

  5. Idiopathic generalized epilepsy and hypokalemic periodic paralysis in a family of South Indian descent.

    Science.gov (United States)

    Subramanian, Muthiah; Senthil, N; Sujatha, S

    2015-01-01

    Inherited channelopathies are a heterogeneous group of disorders resulting from dysfunction of ion channels in cellular membranes. They may manifest as diseases affecting skeletal muscle contraction, the conduction system of the heart, nervous system function, and vision syndromes. We describe a family of South Indian descent with hypokalemic periodic paralysis in which four members also have idiopathic generalized epilepsy. Hypokalemic periodic paralysis is a genetically heterogeneous channelopathy that has been linked to mutations in genes encoding three ion channels CACNIAS, SCN4A, and KCNJ2 predominantly. Although data on specific gene in idiopathic generalized epilepsy is relatively scarce, mutations of voltage gated sodium channel subunit genes (CACNB4) and nonsense mutations in voltage gated calcium channels (CACNA1A) have been linked to idiopathic generalized epilepsy in two families. We speculate that gene mutations altering the ability of the beta subunit to interact with the alpha subunit of the CaV1.1 channel and mutations in the pore-forming potassium channel subunit may be possible explanations for the combined manifestation of both diseases. Functional analysis of voltage gated calcium channel and other ion channels mutations may provide additional support and insight for the causal role of these mutations. The understanding of mutations in ion-channel genes will lead to improved diagnosis and treatment of such inherited channelopathies.

  6. Force assessment in periodic paralysis after electrical muscle stimulation.

    Science.gov (United States)

    Day, John W; Sakamoto, Carl; Parry, Gareth J; Lehmann-Horn, Frank; Iaizzo, Paul A

    2002-03-01

    To obtain an objective measure of muscle force in periodic paralysis, we studied ankle dorsiflexion torque during induced paralytic attacks in hyperkalemic and hypokalemic patients. SUBJECTS, PATIENTS, AND METHODS: Dorsiflexor torque after peroneal nerve stimulation was recorded during provocative tests on 5 patients with hypokalemic or hyperkalemic disorders and on 2 control subjects (1995-2001). Manual strength assessment was simultaneously performed in a blinded fashion. Standardized provocation procedures were used. The loss of torque in hyperkalemic patients roughly paralleled the loss of clinically detectable strength, whereas in the hypokalemic patients, pronounced torque loss occurred well before observed clinical effects. No dramatic changes occurred in the control subjects. Torque amplitude decreased more than 70% in all patients during the provocation tests; such decreases were associated with alterations induced in serum potassium concentrations. Stimulated torque measurement offers several advantages in characterizing muscle dysfunction in periodic paralysis: (1) it is independent of patient effort; (2) it can show a definitely abnormal response early during provocative maneuvers; and (3) characteristics of muscle contraction can be measured that are unobservable during voluntary contraction. Stimulated torque measurements can characterize phenotypic muscle function in neuromuscular diseases.

  7. The system of family relationships convicted for acquisitive crimes in the colony during the initial period of serving the sentence

    Directory of Open Access Journals (Sweden)

    A. A. Martynova

    2015-01-01

    Full Text Available In the initial period of serving punishment in the colony, the system of family relations does not affect the occurrence of «Disorders of adaptive reactions» observed in 96 % of prisoners. However, in family relations women focused on child-parent relationships and men are more focused on the marriage relationship.

  8. Periodic paralysis: clinical evaluation in 20 patients

    Directory of Open Access Journals (Sweden)

    Célia Harumi Tengan

    1994-12-01

    Full Text Available Twenty patients with periodic paralysis were evaluated and the aspects studied included epidemiological data, clinical manifestations, ancillary tests, treatment and evolution. Sixteen patients had the hypokalemic form (5 familiar, 5 sporadic, 5 thyrotoxic and 1 secondary. No patient with the normokalemic form was detected. Predominance of men was found (14 patients, especially in the cases with hyperthyroidism (5 patients. No thyrotoxic patient was of oriental origin. Only 4 patients had the hyperkalemic form (3 familiar, 1 sporadic. Attacks of paralysis began during the first decade in the hyperkalemic form and up to the third decade in the hypokalemic. In both forms the attacks occurred preferentially in the morning with rest after exercise being the most important precipitating factor. Seventy five percent of the hyperkalemic patients referred brief attacks (<12 hours. Longer attacks were referred by 43% of the hypokalemic patients. The majority of the attacks manifested with a generalized weakness mainly in legs, and its frequency was variable. Creatinokinase was evaluated in 10 patients and 8 of them had levels that varied from 1,1 to 5 times normal. Electromyography was done in 6 patients and myotonic phenomenon was the only abnormality detected in 2 patients. Carbonic anhydrase inhibitors, especially acetazolamide, were used for prophylactic treatment in 9 pacients with good results in all. Although periodic paralysis may be considered a benign disease we found respiratory distress in 5 patients, permanent myopathy in 1, electrocardiographic abnormalities during crises in 4; death during paralysis occurred in 2. Therefore correct diagnosis and immediate treatment are crucial. This study shows that hyperthyroidism is an important cause of periodic paralysis in our country, even in non oriental patients. Hence endocrine investigation is mandatory since this kind of periodic paralysis will only be abated after return to the euthyroid state.

  9. Familial Mediterranean fever (a periodic disease: The present-day view of the problem

    Directory of Open Access Journals (Sweden)

    Evgeny Stanislavovich Fedorov

    2013-03-01

    Full Text Available The paper deals with the most common classical autoinflammatory disease familial Mediterra-nean fever (FMF/periodic disease. This is a monogenic hereditary disease caused by mutations with an autosomal recessive pattern of inheritance. The most common types of mutations are given. Hyperactivation of innate (antigen-specific immunity is a basic pathogenic mechanism of the disease and IL-1ß is a leading mediator. FMF prominently occurs in certain ethnic groups (Sephardic Jews, Armenians, Turks, and Arabs. In spite of the fact that there may be multiple organ failure, 12-72-hour febrile fever episodes accompanied by the symptoms of peritonitis and/or pleuropericarditis. AA amyloidosis is the most serious complication of FMF. Colchicine therapy is a basic treatment for preventing this complication. In case of colchicine inef-fi-cacy/intolerance, other agents, including genetically engineered biological drugs (IL-1ß inhibitors, etc., may be used.

  10. Familial Mediterranean fever (a periodic disease: The present-day view of the problem

    Directory of Open Access Journals (Sweden)

    Evgeny Stanislavovich Fedorov

    2013-01-01

    Full Text Available The paper deals with the most common classical autoinflammatory disease familial Mediterra-nean fever (FMF/periodic disease. This is a monogenic hereditary disease caused by mutations with an autosomal recessive pattern of inheritance. The most common types of mutations are given. Hyperactivation of innate (antigen-specific immunity is a basic pathogenic mechanism of the disease and IL-1ß is a leading mediator. FMF prominently occurs in certain ethnic groups (Sephardic Jews, Armenians, Turks, and Arabs. In spite of the fact that there may be multiple organ failure, 12-72-hour febrile fever episodes accompanied by the symptoms of peritonitis and/or pleuropericarditis. AA amyloidosis is the most serious complication of FMF. Colchicine therapy is a basic treatment for preventing this complication. In case of colchicine inef-fi-cacy/intolerance, other agents, including genetically engineered biological drugs (IL-1ß inhibitors, etc., may be used.

  11. Familial clustering of recurrent pericarditis may disclose tumour necrosis factor receptor-associated periodic syndrome.

    Science.gov (United States)

    Cantarini, Luca; Lucherini, Orso Maria; Baldari, Cosima Tatiana; Laghi Pasini, Franco; Galeazzi, Mauro

    2010-01-01

    Although several causes of recurrent pericarditis have been identified, the etiology remains obscure in most cases. The tumour necrosis factor receptor-1 associated periodic syndrome (TRAPS) is the most common autosomal dominant autoinflammatory disorder and is caused by mutations in the TNFRSF1A gene encoding the 55-kD receptor for tumour necrosis factor-(TNF)-alpha. Serosal membrane inflammation is a common feature of TRAPS, usually in the form of polyserositis. In addition, patients affected with recurrent pericarditis as the only clinical manifestation of TRAPS have been recently described. Our aim was to investigate the possible involvement of mutations in the TNFRSF1A gene in a cohort of patients affected with idiopathic recurrent pericarditis. Twenty consecutive patients diagnosed with idiopathic recurrent pericarditis were enrolled. Each patient underwent detailed examinations in order to rule out underlying diseases such as infections, connective tissue disorders and malignancies, and mutations of the TNFRSF1A gene were searched for by amplifying, using polymerase chain reaction (PCR), genomic DNA, and direct sequencing. TNFRSF1A mutations were found in 2 of the 20 patients. They were siblings, and they both carried a heterozygous low-penetrance R92Q mutation in the TNFRSF1A gene. Familial clustering has been recently reported in up to 10% of patients with recurrent pericarditis, thus suggesting in some cases a possible genetic predisposition. Our study suggests that familial clustering may represent a clue for investigating mutations in the TNFRSF1A gene in these patients and eventually disclose TRAPS.

  12. [Normokalemic periodic paralysis lasting for two weeks: a severe form of sodium channelopathy with M1592V mutation].

    Science.gov (United States)

    Shiga, Kensuke; Mizuta, Ikuko; Noto, Yu-ichi; Nakagawa, Masanori; Sasaki, Ryogen; Yamawaki, Masanaga

    2014-01-01

    A 73-year-old man with recurrent periodic paralytic episodes lasting for two weeks each admitted to our hospital because of the leg weakness and the elevated value of serum creatine kinase. On admission, weakness in the proximal legs and mild eye lid myotonia were noted. Needle electromyography revealed abundant myotonic discharges. The prolonged exercise test showed a continuous reduction of compound muscle action potentials in the abductor digiti minimi muscle. Direct sequencing of SCN4A in the proband showed a G-to-A alteration at position 4774 that results in a change of 1592(nd) methionine to valine (M1592V). Cosegregation regarding the M1592V mutation and paralytic phenotype in this family was confirmed. Two cardinal features in this family were longer paralytic episodes compared to classical hyperkalemic/normokalemic periodic paralysis and the normal potassium value during the paralytic episodes. This study together with antecedent reports indicates that M1592V mutation shares a much greater clinical diversity ranging from congenital paramyotonia to periodic paralysis with a longer duration.

  13. Pregnancy in women suffering from familial hypercholesterolemia: a harmful period for both mother and newborn?

    NARCIS (Netherlands)

    Avis, H.J.; Hutten, B.A.; Twickler, T.B.; Kastelein, J.J.; Post, J.A. van der; Stalenhoef, A.F.H.; Vissers, M.N.

    2009-01-01

    PURPOSE OF REVIEW: The present review aims to highlight the consequences for mother and child of profound hypercholesterolemia during pregnancy of women with familial hypercholesterolemia. RECENT FINDINGS: Familial hypercholesterolemia is increasingly diagnosed in younger patients due to the

  14. MUSCLE-FIBER CONDUCTION-VELOCITY IN THE DIAGNOSIS OF FAMILIAL HYPOKALEMIC PERIODIC PARALYSIS - INVASIVE VERSUS SURFACE DETERMINATION

    NARCIS (Netherlands)

    VANDERHOEVEN, JH; LINKS, TP; ZWARTS, MJ; VANWEERDEN, TW

    1994-01-01

    Muscle fiber conduction velocity (MFCV) in the brachial biceps muscle was determined in a large family of patients with hypokalemic periodic paralysis (HOPP) by both a surface and an invasive method. Other surface EMG parameters and the muscle force were also determined. Both the surface and the inv

  15. Expression patterns of two potassium channel genes in skeletal muscle cells of patients with familial hypokalemic periodic paralysis.

    Science.gov (United States)

    Kim, June-Bum; Lee, Gyung-Min; Kim, Sung-Jo; Yoon, Dong-Ho; Lee, Young-Hyuk

    2011-01-01

    Familial hypokalemic periodic paralysis is an autosomal-dominant disorder characterized by episodic attacks of muscle weakness with hypokalemia. The combination of sarcolemmal depolarization and hypokalemia has been attributed to abnormalities of the potassium conductance governing the membrane potential; however, the molecular mechanism that causes hypokalemia has not yet been determined. To test the hypothesis that the expression patterns of delayed rectifier potassium channel genes in the skeletal muscle cells of patients with familial hypokalemic periodic paralysis differ from those in normal cells. We examined both mRNA and protein levels of two major delayed rectifier potassium channel genes KCNQ3 and KCNQ5 in the skeletal muscle cells from three patients with familial hypokalemic periodic paralysis and three healthy controls. When normal cells were exposed to 50 mM potassium buffer, which was used to induce depolarization, the KCNQ3 protein level significantly increased in the membrane fraction but decreased in the cytosolic fraction, whereas the opposite was true in patient cells. Abnormal subcellular distribution of the KCNQ3 protein was observed in patient cells. Our results suggest that the altered expression of KCNQ3 in patient cells exposed to high extracellular potassium levels could possibly hinder normal function of the channel protein. These findings may provide an important clue to understanding the molecular mechanism of familial hypokalemic periodic paralysis.

  16. MUTATION IN DHP RECEPTOR ALPHA-1 SUBUNIT (CACLN1A3) GENE IN A DUTCH FAMILY WITH HYPOKALEMIC PERIODIC PARALYSIS

    NARCIS (Netherlands)

    BOERMAN, RH; OPHOFF, RA; LINKS, TP; VANEIJK, R; SANDKUIJL, LA; ELBAZ, A; VALESANTOS, JE; WINTZEN, AR; VANDEUTEKOM, JC; ISLES, DE; FONTAINE, B; PADBERG, GW; FRANTS, RR

    1995-01-01

    Hypokalaemic periodic paralysis (HypoPP) is characterised by transient attacks of muscle weakness of varying duration and severity accompanied by a drop in serum potassium concentration during the attacks. The largest known HypoPP family is of Dutch origin and consists of 277 members in the last fiv

  17. Expression patterns of two potassium channel genes in skeletal muscle cells of patients with familial hypokalemic periodic paralysis

    Directory of Open Access Journals (Sweden)

    June-Bum Kim

    2011-01-01

    Full Text Available Background: Familial hypokalemic periodic paralysis is an autosomal-dominant disorder characterized by episodic attacks of muscle weakness with hypokalemia. The combination of sarcolemmal depolarization and hypokalemia has been attributed to abnormalities of the potassium conductance governing the membrane potential; however, the molecular mechanism that causes hypokalemia has not yet been determined. Aim: To test the hypothesis that the expression patterns of delayed rectifier potassium channel genes in the skeletal muscle cells of patients with familial hypokalemic periodic paralysis differ from those in normal cells. Material and Methods: We examined both mRNA and protein levels of two major delayed rectifier potassium channel genes KCNQ3 and KCNQ5 in the skeletal muscle cells from three patients with familial hypokalemic periodic paralysis and three healthy controls. Results: When normal cells were exposed to 50 mM potassium buffer, which was used to induce depolarization, the KCNQ3 protein level significantly increased in the membrane fraction but decreased in the cytosolic fraction, whereas the opposite was true in patient cells. Conclusion: Abnormal subcellular distribution of the KCNQ3 protein was observed in patient cells. Our results suggest that the altered expression of KCNQ3 in patient cells exposed to high extracellular potassium levels could possibly hinder normal function of the channel protein. These findings may provide an important clue to understanding the molecular mechanism of familial hypokalemic periodic paralysis.

  18. A LARGE CLASS OF BINARY ZCZ SEQUENCE FAMILIES CONSTRUCTED BY PERIOD DOUBLING

    Institute of Scientific and Technical Information of China (English)

    Wang Jinsong; Qi Wenfeng

    2007-01-01

    In an Approximately Synchronized Code Division Multiple Access(AS-CDMA)communication svstem,a family with large number of Zero Correlation Zone(ZCZ)sequences is desired,which can satisfy the rapid increase of users.This paper presents a method to generate a(2L,2M,Z'cz)-ZCZ sequence family from an original(L,M,Zcz)-ZCZ sequence family,where Z'cz=Zcz if Zcz is even and Z'cz=Zcz-1 if Zcz is odd.This method can also recursively act on a ZCZ sequence family to construct a series of ZCZ sequence families with large sequence nutuber and zero correlation zone length identical to or one less than that of original ZCZ sequences.

  19. A Korean Family of Hypokalemic Periodic Paralysis with Mutation in a Voltage-gated Calcium Channel (R1239G)

    Science.gov (United States)

    Kim, June-Bum; Lee, Kyung-Yil

    2005-01-01

    Hypokalemic periodic paralysis (HOPP) is a rare disease characterized by reversible attacks of muscle weakness accompanied by episodic hypokalemia. Recent molecular work has revealed that the majority of familial HOPP is due to mutations in a skeletal muscle voltage-dependent calcium-channel: the dihydropyridine receptor. We report a 13-yr old boy with HOPP from a family in which 6 members are affected in three generations. Genetic examination identified a nucleotide 3705 C to G mutation in exon 30 of the calcium channel gene, CACNA1S. This mutation predicts a codon change from arginine to glycine at the amino acid position #1239 (R1239G). Among the three known mutations of the CACNA1S gene, the R1239G mutation was rarely reported. This boy and the other family members who did not respond to acetazolamide, showed a marked improvement of the paralytic symptoms after spironolactone treatment. PMID:15716625

  20. Shopping Behaviors of Low-income Families during a 1-Month Period of Time

    Science.gov (United States)

    Darko, Janice; Eggett, Dennis L.; Richards, Rickelle

    2013-01-01

    Objective: To explore food shopping behaviors among low-income families over the course of the month. Design: Two researchers conducted 13 90-minute focus groups. Setting: Two community organizations serving low-income populations and a university campus. Participants: Low-income adults (n = 72) who were the primary household food shoppers and who…

  1. Shopping Behaviors of Low-income Families during a 1-Month Period of Time

    Science.gov (United States)

    Darko, Janice; Eggett, Dennis L.; Richards, Rickelle

    2013-01-01

    Objective: To explore food shopping behaviors among low-income families over the course of the month. Design: Two researchers conducted 13 90-minute focus groups. Setting: Two community organizations serving low-income populations and a university campus. Participants: Low-income adults (n = 72) who were the primary household food shoppers and who…

  2. Shopping behaviors of low-income families during a 1-month period of time.

    Science.gov (United States)

    Darko, Janice; Eggett, Dennis L; Richards, Rickelle

    2013-01-01

    To explore food shopping behaviors among low-income families over the course of the month. Two researchers conducted 13 90-minute focus groups. Two community organizations serving low-income populations and a university campus. Low-income adults (n = 72) who were the primary household food shoppers and who had at least 1 child less than 18 years old. Shopping behavior changes during 1 month. Focus groups were recorded, transcribed, and coded independently by 2 researchers. Descriptive statistics were used to evaluate sociodemographic variables such as age, sex, race/ethnicity, and participation in food assistance programs. Economics played a key role in participants' food shopping behaviors and influenced food availability throughout the month. To overcome economic barriers, participants used food and emergency assistance programs and engaged in menu planning, price matching, storing food, using credit cards, and receiving financial assistance from family members and/or neighbors. Low-income families made strategic decisions to maintain a food supply throughout the month. These results suggest limited economics throughout the month may hinder families' ability to consume a varied, nutrient-rich diet, which may have an impact on future health status. Copyright © 2013 Society for Nutrition Education and Behavior. Published by Elsevier Inc. All rights reserved.

  3. "Nothing special, everything is maamuli": socio-cultural and family practices influencing the perinatal period in urban India.

    Science.gov (United States)

    Raman, Shanti; Srinivasan, Krishnamachari; Kurpad, Anura; Razee, Husna; Ritchie, Jan

    2014-01-01

    Globally, India contributes the largest share in sheer numbers to the burden of maternal and infant under-nutrition, morbidity and mortality. A major gap in our knowledge is how socio-cultural practices and beliefs influence the perinatal period and thus perinatal outcomes, particularly in the rapidly growing urban setting. Using data from a qualitative study in urban south India, including in-depth interviews with 36 women who had recently been through childbirth as well as observations of family life and clinic encounters, we explored the territory of familial, cultural and traditional practices and beliefs influencing women and their families through pregnancy, childbirth and infancy. We found that while there were some similarities in cultural practices to those described before in studies from low resource village settings, there are changing practices and ideas. Fertility concerns dominate women's experience of married life; notions of gender preference and ideal family size are changing rapidly in response to the urban context; however inter-generational family pressures are still considerable. While a rich repertoire of cultural practices persists throughout the perinatal continuum, their existence is normalised and even underplayed. In terms of diet and nutrition, traditional messages including notions of 'hot' and 'cold' foods, are stronger than health messages; however breastfeeding is the cultural norm and the practice of delayed breastfeeding appears to be disappearing in this urban setting. Marriage, pregnancy and childbirth are so much part of the norm for women, that there is little expectation of individual choice in any of these major life events. A greater understanding is needed of the dynamic factors shaping the perinatal period in urban India, including an acknowledgment of the health promoting as well as potentially harmful cultural practices and the critical role of the family. This will help plan culturally appropriate integrated perinatal

  4. "Nothing special, everything is maamuli": socio-cultural and family practices influencing the perinatal period in urban India.

    Directory of Open Access Journals (Sweden)

    Shanti Raman

    Full Text Available Globally, India contributes the largest share in sheer numbers to the burden of maternal and infant under-nutrition, morbidity and mortality. A major gap in our knowledge is how socio-cultural practices and beliefs influence the perinatal period and thus perinatal outcomes, particularly in the rapidly growing urban setting.Using data from a qualitative study in urban south India, including in-depth interviews with 36 women who had recently been through childbirth as well as observations of family life and clinic encounters, we explored the territory of familial, cultural and traditional practices and beliefs influencing women and their families through pregnancy, childbirth and infancy. We found that while there were some similarities in cultural practices to those described before in studies from low resource village settings, there are changing practices and ideas. Fertility concerns dominate women's experience of married life; notions of gender preference and ideal family size are changing rapidly in response to the urban context; however inter-generational family pressures are still considerable. While a rich repertoire of cultural practices persists throughout the perinatal continuum, their existence is normalised and even underplayed. In terms of diet and nutrition, traditional messages including notions of 'hot' and 'cold' foods, are stronger than health messages; however breastfeeding is the cultural norm and the practice of delayed breastfeeding appears to be disappearing in this urban setting. Marriage, pregnancy and childbirth are so much part of the norm for women, that there is little expectation of individual choice in any of these major life events.A greater understanding is needed of the dynamic factors shaping the perinatal period in urban India, including an acknowledgment of the health promoting as well as potentially harmful cultural practices and the critical role of the family. This will help plan culturally appropriate

  5. Familial periodic cerebellar ataxia without myokymia maps to a 19-cM region on 19p13

    Energy Technology Data Exchange (ETDEWEB)

    Teh, B.T.; Lindblad, K.; Betz, R. [Karolinska Hospital, Stockholm (Switzerland)] [and others

    1995-06-01

    Familial periodic cerebellar ataxia (FPCA) is a heterogenous group of rare autosomal dominant disorders characterized by episodic cerebellar disturbance. A potassium-channel gene (KCNA1) has been found to be responsible for one of its subgroups, familial periodic cerebellar ataxia with myokymia (FPCA/+M; MIM 160120). A different subgroup that is not associated with myokymia (FPCA/-M; MIM 108500) was recently mapped to chromosome 19p. Here we have performed linkage analysis in two large families with FPCA/-M that also demonstrated neurodegenerative pathology of the cerebellum. Three markers in 19p13 gave significant lod scores (>3.0), while linkage to KCNA1 and three known loci for spinocerebellar ataxia (SCA1, SCA2, and SCA3) was excluded. The highest lod score was obtained with the marker D19S413 (4.4 at recombination fraction 0), and identification of meiotic recombinants in affected individuals placed the locus between the flanking markers D19S406 and D19S226, narrowing the interval to 19 cM. A CAG trinucleotide-repeat expansion was detected in one family but did not consegregate with the disease. 30 refs., 3 figs., 1 tab.

  6. Evolution of Embryonic Developmental Period in the Marine Bird Families Alcidae and Spheniscidae: Roles for Nutrition and Predation?

    OpenAIRE

    Vos Rutger A; Gorman Kristen B; Hipfner J Mark; Joy Jeffrey B

    2010-01-01

    Abstract Background Nutrition and predation have been considered two primary agents of selection important in the evolution of avian life history traits. The relative importance of these natural selective forces in the evolution of avian embryonic developmental period (EDP) remain poorly resolved, perhaps in part because research has tended to focus on a single, high taxonomic-level group of birds: Order Passeriformes. The marine bird families Alcidae (auks) and Spheniscidae (penguins) exhibi...

  7. Comparison of acute phase response during attack and attack-free period in children with Familial Mediterranean Fever

    Directory of Open Access Journals (Sweden)

    Erdal Çakmak

    2013-06-01

    Full Text Available Objective: The aim of this study was to compare acutephase reactant (AFR levels at attack period and attackfreeperiod under colchicine treatment in children with FamilialMediterranean Fever (FMF.Methods: The diagnosis of FMF was done based on clinicalcriteria and patients were prospectively followed upfor average of 1.2 years. Symptom-onset age, age at diagnosis,clinical symptoms and features of FMF attackswere recorded. MEFV gene mutations were detected byreverse hybridization (strip assay method. Peripheralblood leukocyte count, erythrocyte sedimentation rate(ESR, C-reactive protein (CRP and blood fibrinogen levelswere measured by standard methods, both at attackperiod and during attack-free period.Results: Totally 105 (55 girls, 50 boys children with FMFwere included. The mean age was 8.9±3.2 years, meansymptom onset age was 5.9 years and mean age at diagnosiswas 8.1 years. MEFV gene mutations were asfollows: E148Q (29.2%, M694V (24.8%, R761H (15.3%and V726A (13.1%. The mean AFR values were overnormal values in attack period and there was at least onehigh AFR level in 80.0% of patients. In attack-free period,although the mean values of all AFRs were within normallimits, 31.4% of patients had at least one high AFR level.Conclusion: Based on these data, one-third of FMF childrenhad a high AFR level, which may be a marker of subclinicalinflammation. In children with continuous inflammationduring attack-free period, a new anti-inflammatorydrug additional to colchicine can be considered in order toprevent complications of chronic inflammation. J Clin ExpInvest 2013; 4 (2: 213-218Key words: Familial Mediterranean Fever, acute phasereactants, children, attack period, attack-free period

  8. Mindfulness-Based Childbirth and Parenting Education: Promoting Family Mindfulness During the Perinatal Period

    OpenAIRE

    Duncan, Larissa G.; Bardacke, Nancy

    2009-01-01

    We present the conceptual and empirical foundation and curriculum content of the Mindfulness-Based Childbirth and Parenting (MBCP) program and the results of a pilot study of n = 27 pregnant women participating in MBCP during their third trimester of pregnancy. MBCP is a formal adaptation of the Mindfulness-Based Stress Reduction program and was developed and refined over the course of 11 years of clinical practice with 59 groups of expectant couples. MBCP is designed to promote family health...

  9. A case of familial Mediterranean fever who complained of periodic fever and abdominal pain diagnosed by MEFV gene analysis.

    Science.gov (United States)

    Ogita, Chie; Matsui, Kiyoshi; Kisida, Dai; Kakudou, Mariko; Yazaki, Masahide; Nakamura, Akinori; Azuma, Kouta; Tsuboi, Kazuyuki; Abe, Takeo; Yokoyama, Yuichi; Furukawa, Tetsuya; Maruoka, Momo; Tamura, Masao; Yoshikawa, Takahiro; Saito, Atsushi; Nishioka, Aki; Sekiguchi, Masahiro; Azuma, Naoto; Kitano, Masayasu; Tsunoda, Shinichiro; Hashimoto-Tamaoki, Tomoko; Sano, Hajime

    2016-01-01

      Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disease caused by Mediterranean FeVergene (MEFV) mutations on Chromosome 16, and characterized by periodic fever of and serositis. FMF is the result of gain-of-function mutations in pyrin that lead to interleukin-1β activation. FMF can be classified as "typical" and "atypical" types based on clinical finding and genetic screening. Although MEFV genotyping has enabled FMF to be confirmed in some cases, the diagnosis remains predominantly clinical since genotyping has shown that the disease is characterized by variable manifestations in Japanese. In 1976, the first report performed on the case of Japanese FMF with periodic fever of and serositis. Since 2002, genetic analyses are performed on Japanese FMF patients by K. Shiozaki et al. and N. Tomiyama et al. In our case, she was a 25-year-old Japanese woman with at periodic fever and abdominal pain. MEFV gene analysis demonstrated a heterozygous mutation of variant M694I, leading to a diagnosis of FMF. After the increase dose (up to 3 mg/day) of colchicine, periodic fever and abdominal pain disappeared. It is the important candidate of FMF for differential diagnosis with unexplained periodic fever and serositis, such as our case.

  10. Identification and functional characterization of Kir2.6 mutations associated with non-familial hypokalemic periodic paralysis.

    Science.gov (United States)

    Cheng, Chih-Jen; Lin, Shih-Hua; Lo, Yi-Fen; Yang, Sung-Sen; Hsu, Yu-Juei; Cannon, Stephen C; Huang, Chou-Long

    2011-08-05

    Hypokalemic periodic paralysis (hypoKPP) is characterized by episodic flaccid paralysis of muscle and acute hypokalemia during attacks. Familial forms of hypoKPP are predominantly caused by mutations of either voltage-gated Ca(2+) or Na(+) channels. The pathogenic gene mutation in non-familial hypoKPP, consisting mainly of thyrotoxic periodic paralysis (TPP) and sporadic periodic paralysis (SPP), is largely unknown. Recently, mutations in KCNJ18, which encodes a skeletal muscle-specific inwardly rectifying K(+) channel Kir2.6, were reported in some TPP patients. Whether mutations of Kir2.6 occur in other patients with non-familial hypoKPP and how mutations of the channel predispose patients to paralysis are unknown. Here, we report one conserved heterozygous mutation in KCNJ18 in two TPP patients and two separate heterozygous mutations in two SPP patients. These mutations result in V168M, R43C, and A200P amino acid substitution of Kir2.6, respectively. Compared with the wild type channel, whole-cell currents of R43C and V168M mutants were reduced by ∼78 and 43%, respectively. No current was detected for the A200P mutant. Single channel conductance and open probability were reduced for R43C and V168M, respectively. Biotinylation assays showed reduced cell surface abundance for R43C and A200P. All three mutants exerted dominant negative inhibition on wild type Kir2.6 as well as wild type Kir2.1, another Kir channel expressed in the skeletal muscle. Thus, mutations of Kir2.6 are associated with SPP as well as TPP. We suggest that decreased outward K(+) current from hypofunction of Kir2.6 predisposes the sarcolemma to hypokalemia-induced paradoxical depolarization during attacks, which in turn leads to Na(+) channel inactivation and inexcitability of muscles.

  11. Guidelines for the management and treatment of periodic fever syndromes familial Mediterranean fever.

    Science.gov (United States)

    Terreri, Maria Teresa R A; Bernardo, Wanderley Marques; Len, Claudio Arnaldo; da Silva, Clovis Artur Almeida; de Magalhães, Cristina Medeiros Ribeiro; Sacchetti, Silvana B; Ferriani, Virgínia Paes Leme; Piotto, Daniela Gerent Petry; de Souza Cavalcanti, André; de Moraes, Ana Júlia Pantoja; Sztajnbok, Flavio Roberto; de Oliveira, Sheila Knupp Feitosa; Campos, Lucia Maria Arruda; Bandeira, Marcia; Santos, Flávia Patricia Sena Teixeira; Magalhães, Claudia Saad

    2016-01-01

    To establish guidelines based on scientific evidence for the management of familial Mediterranean fever. The Guideline was prepared from 5 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. 10,341 articles were retrieved and evaluated by title and abstract; from these, 46 articles were selected to support the recommendations. 1. The diagnosis of FMF is based on clinical manifestations, characterized by recurrent febrile episodes associated with abdominal pain, chest or arthritis of large joints. 2. FMF is a genetic disease presenting an autosomal recessive trait, caused by mutation in the MEFV gene. 3. Laboratory tests are not specific, demonstrating high serum levels of inflammatory proteins in the acute phase of the disease, but also often showing high levels even between attacks. SAA serum levels may be especially useful in monitoring the effectiveness of treatment. 4. The therapy of choice is colchicine; this drug has proven its effectiveness in preventing acute inflammatory episodes and progression toward amyloidosis in adults. 5. Based on the available information, the use of biological drugs appears to be an alternative for patients with FMF who do not respond or are intolerant to therapy with colchicine. Copyright © 2015 Elsevier Editora Ltda. All rights reserved.

  12. A family of rare earth molybdenum bronzes: Oxides consisting of periodic arrays of interacting magnetic units

    Energy Technology Data Exchange (ETDEWEB)

    Schneemeyer, L.F. [Department of Chemistry, Montclair State University, Montclair, NJ 07043 (United States); Siegrist, T., E-mail: tsiegrist@fsu.edu [Department of Chemical and Biomedical Engineering, FAMU-FSU College of Engineering, Tallahassee, FL 32310 (United States); National High Magnetic Field Laboratory, Tallahassee, FL 32310 (United States); Besara, T. [National High Magnetic Field Laboratory, Tallahassee, FL 32310 (United States); Lundberg, M. [Department of Chemical and Biomedical Engineering, FAMU-FSU College of Engineering, Tallahassee, FL 32310 (United States); National High Magnetic Field Laboratory, Tallahassee, FL 32310 (United States); Sun, J. [Department of Chemical and Biomedical Engineering, FAMU-FSU College of Engineering, Tallahassee, FL 32310 (United States); Materials Science and Technology Division, Oak Ridge National Laboratory, Oak Ridge, TN 37831-6056 (United States); Singh, D.J. [Materials Science and Technology Division, Oak Ridge National Laboratory, Oak Ridge, TN 37831-6056 (United States)

    2015-07-15

    The family of rare earth molybdenum bronzes, reduced ternary molybdates of composition LnMo{sub 16}O{sub 44,} was synthesized and a detailed structural study carried out. Bond valence sum (BVS) calculations clearly show that the molybdenum ions in tetrahedral coordination are hexavalent while the electron count in the primitive unit cell is odd. Yet, measurements show that the phases are semiconductors. The temperature dependence of the magnetic susceptibility of samples containing several different rare earth elements was measured. These measurements verified the presence of a 6.5 K magnetic phase transition not arising from the rare earth constituent, but likely associated with the unique isolated ReO{sub 3}-type Mo{sub 8}O{sub 36} structural subunits in this phase. To better understand the behavior of these materials, electronic structure calculations were performed within density functional theory. Results suggest a magnetic state in which these structural moieties have an internal ferromagnetic arrangement, with small ~1/8 μ{sub B} moments on each Mo. We suggest that the Mo{sub 8}O{sub 36} units behave like pseudoatoms with spin 1/2 derived from a single hole distributed over the eight Mo atoms that are strongly hybridized with the O atoms of the subunit. Interestingly, while the compound is antiferromagnetic, our calculations suggest that a field-stabilized ferromagnetic state, if achievable, will be a narrow band half-metal. - Graphical abstract: LnMo{sub 16}O{sub 44} phases comprise corner sharing tetrahedral and octahedral molybdenum ions. The MoO{sub 6} octahedra form Mo{sub 8}O{sub 36} units that are well separated and act like pseudo-atoms, accommodating 11 electrons each. - Highlights: • Single crystal X-ray diffraction refinements of LnMo{sub 16}O{sub 44} single crystals for Ln=Ce, Pr, Nd, Tb, Dy and Ho. • DFT calculations based on LaMo{sub 16}O{sub 44}. • [Mo{sub 8}O{sub 36}] units behaving as superatoms with a net magnetic moment of 1 µ

  13. Effects of S906T polymorphism on the severity of a novel borderline mutation I692M in Nav 1.4 cause periodic paralysis.

    Science.gov (United States)

    Fan, C; Mao, N; Lehmann-Horn, F; Bürmann, J; Jurkat-Rott, K

    2017-06-01

    Hyperkalemic periodic paralysis (HyperPP) is a dominantly inherited muscle disease caused by mutations in SCN4A gene encoding skeletal muscle voltage gated Nav 1.4 channels. We identified a novel Nav 1.4 mutation I692M in 14 families out of the 104 genetically identified HyperPP families in the Neuromuscular Centre Ulm and is therefore as frequent as I693T (13 families out of 14 HyperPP families) in Germany. Surprisingly, in 13 families, a known polymorphism S906T was also present. It was on the affected allele in at least 10 families compatible with a possible founder effect in central Europe. All affected members suffered from episodic weakness; myotonia was also common. Compared with I692M patients, I692M-S906T patients had longer weakness episodes, more affected muscles, CK elevation and presence of permanent weakness. Electrophysiological investigation showed that both mutants had incomplete slow inactivation and a hyperpolarizing shift of activation which contribute to membrane depolarization and weakness. Additionally, I692M-S906T significantly enhanced close-state fast inactivation compared with I692M alone, suggesting a higher proportion of inactivated I692M-S906T channels upon membrane depolarization which may facilitate the initiation of weakness episodes and therefore clinical manifestation. Our results suggest that polymorphism S906T has effects on the clinical phenotypic and electrophysiological severity of a novel borderline Nav 1.4 mutation I692M, making the borderline mutation fully penetrant. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  14. Performance of Different Diagnostic Criteria for Familial Mediterranean Fever in Children with Periodic Fevers: Results from a Multicenter International Registry.

    Science.gov (United States)

    Demirkaya, Erkan; Saglam, Celal; Turker, Turker; Koné-Paut, Isabelle; Woo, Pat; Doglio, Matteo; Amaryan, Gayane; Frenkel, Joost; Uziel, Yosef; Insalaco, Antonella; Cantarini, Luca; Hofer, Michael; Boiu, Sorina; Duzova, Ali; Modesto, Consuelo; Bryant, Annette; Rigante, Donato; Papadopoulou-Alataki, Efimia; Guillaume-Czitrom, Severine; Kuemmerle-Deschner, Jasmine; Neven, Bénédicte; Lachmann, Helen; Martini, Alberto; Ruperto, Nicolino; Gattorno, Marco; Ozen, Seza

    2016-01-01

    Our aims were to validate the pediatric diagnostic criteria in a large international registry and to compare them with the performance of previous criteria for the diagnosis of familial Mediterranean fever (FMF). Pediatric patients with FMF from the Eurofever registry were used for the validation of the existing criteria. The other periodic fevers served as controls: mevalonate kinase deficiency (MKD), tumor necrosis factor receptor-associated periodic syndrome (TRAPS), cryopyrin-associated periodic syndrome (CAPS), aphthous stomatitis, pharyngitis, adenitis syndrome (PFAPA), and undefined periodic fever from the same registry. The performances of Tel Hashomer, Livneh, and the Yalcinkaya-Ozen criteria were assessed. The FMF group included 339 patients. The control group consisted of 377 patients (53 TRAPS, 45 MKD, 32 CAPS, 160 PFAPA, 87 undefined periodic fevers). Patients with FMF were correctly diagnosed using the Yalcinkaya-Ozen criteria with a sensitivity rate of 87.4% and a specificity rate of 40.7%. On the other hand, Tel Hashomer and Livneh criteria displayed a sensitivity of 45.0 and 77.3%, respectively. Both of the latter criteria displayed a better specificity than the Yalcinkaya-Ozen criteria: 97.2 and 41.1% for the Tel Hashomer and Livneh criteria, respectively. The overall accuracy for the Yalcinkaya-Ozen criteria was 65 and 69.6% (using 2 and 3 criteria), respectively. Ethnicity and residence had no effect on the performance of the Yalcinkaya-Ozen criteria. The Yalcinkaya-Ozen criteria yielded a better sensitivity than the other criteria in this international cohort of patients and thus can be used as a tool for FMF diagnosis in pediatric patients from either the European or eastern Mediterranean region. However, the specificity was lower than the previously suggested adult criteria.

  15. Overlap syndrome between Familial Mediterranean fever and tumor necrosis factor receptor-associated periodic syndrome in a lupus patient.

    Science.gov (United States)

    Nonaka, Fumiaki; Migita, Kiyoshi; Iwasaki, Keisuke; Shimizu, Toshimasa; Kawakami, Atsushi; Yasunami, Michio; Eguchi, Katsumi

    2014-06-01

    Autoinflammatory diseases represent an expanding spectrum of genetic and non-genetic inflammatory diseases characterized by recurrent episodes of fever and systemic inflammation, affecting joints, skin and serosal surfaces. Familial Mediterranean fever (FMF) is the most common autosomal recessive hereditary autoinflammatory disease. Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is an autosomal dominant hereditary autoinflammatory disease. They share some clinical manifestations such as a periodic fever and skin rash. We present here the association of FMF with TRAPS in a systemic lupus erythematosus (SLE) patient. A 54-year-old SLE patient with recurrent attacks of fever, arthritis, and skin rashes was referred to our hospital. She had been diagnosed with lupus nephritis at 19 years old. Her lupus nephritis was controlled by steroid treatments; however, since childhood she has suffered from recurrent episodes of periodic fever, abdominal pain, arthritis, and erythematous skin rashes. An initial diagnosis of FMF was suspected based on the genetic analysis, showing the compound heterozygous L110P/E148Q mutations in the MEFV gene that is responsible for FMF. Her symptoms responded to colchicine, but the febrile attacks were not completely resolved. Therefore, genetic testing for TRAPS was performed. The results revealed a heterozygous T61I mutation in the TNFRSF1A gene that encodes tumor necrosis factor-α receptor and is responsible for TRAPS. The patient was diagnosed with overlapping FMF and TRAPS, in addition to SLE. This is the first report of SLE associated with both FMF and TRAPS.

  16. A New Critical Value Concerning the Genealogy of Long Period Families at L4 in the Restricted Three-body Problem

    Institute of Scientific and Technical Information of China (English)

    Xi-Yun Hou; Lin Liu

    2008-01-01

    We found another critical mass ratio value μ between μ4 and μ5 concerning the genealogy of the long period family around the equilateral equilibrium point L4 in the re-stricted three-body problem. This value has not been pointed out before. We used numerical computations to show how the long period family evolves around this critical value. The case is similar to that of the critical values between μ2 and μ4, with slight difference in evolution details.

  17. Impairment of slow inactivation as a common mechanism for periodic paralysis in DIIS4-S5.

    Science.gov (United States)

    Bendahhou, S; Cummins, T R; Kula, R W; Fu, Y-H; Ptácek, L J

    2002-04-23

    Mutations in the human skeletal muscle sodium channels are associated with hyperKPP, hypoKPP, paramyotonia congenita, and potassium-aggravated myotonia. This article describes the clinical manifestations of a patient with hyperKPP carrying a mutation (L689I) occurring in the linker DIIS4-S5 and its functional expression in a mammalian system. To correlate the clinical manifestations of hyperkalemic periodic paralysis (hyperKPP) with the functional expression of a sodium channel mutation. The mutation was introduced into a mammalian expression vector and expressed in the human embryonic kidney 293 cells. The functional expression of the L689I and that of the wild-type channels was monitored using the whole cell voltage-clamp technique. There was no change in the kinetics of fast inactivation, and inactivation curves were indistinguishable from that of wild-type channels. However, the L689I mutation caused a hyperpolarizing shift in the voltage dependence of activation and the mutant channels showed an impaired slow inactivation process. In addition, the mutant channels have a larger persistent current at -40 mV where window current may occur. The L689I mutation has similar effects to the T704M mutation and causes hyperKPP in this family. Because both of these hyperKPP mutations cause episodic muscle weakness, and because patients harboring another mutation (I693T) also can have episodic weakness, it is hypothesized that mutations occurring in this region of the sodium channel may cause episodic weakness through an impaired slow inactivation process coupled with enhanced activation.

  18. Evolution of embryonic developmental period in the marine bird families Alcidae and Spheniscidae: roles for nutrition and predation?

    Science.gov (United States)

    Hipfner, J Mark; Gorman, Kristen B; Vos, Rutger A; Joy, Jeffrey B

    2010-06-14

    Nutrition and predation have been considered two primary agents of selection important in the evolution of avian life history traits. The relative importance of these natural selective forces in the evolution of avian embryonic developmental period (EDP) remain poorly resolved, perhaps in part because research has tended to focus on a single, high taxonomic-level group of birds: Order Passeriformes. The marine bird families Alcidae (auks) and Spheniscidae (penguins) exhibit marked variation in EDP, as well as behavioural and ecological traits ultimately linked to EDP. Therefore, auks and penguins provide a unique opportunity to assess the natural selective basis of variation in a key life-history trait at a low taxonomic-level. We used phylogenetic comparative methods to investigate the relative importance of behavioural and ecological factors related to nutrition and predation in the evolution of avian EDP. Three behavioural and ecological variables related to nutrition and predation risk (i.e., clutch size, activity pattern, and nesting habits) were significant predictors of residual variation in auk and penguin EDP based on models predicting EDP from egg mass. Species with larger clutch sizes, diurnal activity patterns, and open nests had significantly shorter EDPs. Further, EDP was found to be longer among birds which forage in distant offshore waters, relative to those that foraged in near shore waters, in line with our predictions, but not significantly so. Current debate has emphasized predation as the primary agent of selection driving avian life history diversification. Our results suggest that both nutrition and predation have been important selective forces in the evolution of auk and penguin EDP, and highlight the importance of considering these questions at lower taxonomic scales. We suggest that further comparative studies on lower taxonomic-level groups will continue to constructively inform the debate on evolutionary determinants of avian EDP, as

  19. Family Violence and Maltreatment of Women During the Perinatal Period: Associations with Infant Morbidity in Indian Slum Communities.

    Science.gov (United States)

    Silverman, Jay G; Balaiah, Donta; Decker, Michele R; Boyce, Sabrina C; Ritter, Julie; Naik, D D; Nair, Saritha; Saggurti, Niranjan; Raj, Anita

    2016-01-01

    To determine the prevalence of non-violent, gender-based forms of maltreatment of women by husbands and in-laws [i.e., gender-based household maltreatment (GBHM)] during pregnancy and postpartum; to clarify the role of GBHM in compromising infant health, and whether this role extends beyond that previously observed for intimate partner violence (IPV). Cross-sectional, quantitative data were collected from women (ages 15-35) seeking immunizations for their infants India. Logistic regression models were constructed to assess associations between maternal abuse (perinatal IPV, in-law violence and GBHM) and recent infant morbidity (diarrhea, respiratory distress, fever, colic and vomiting). More than one in four women (28.4%) reported IPV during their recent pregnancy and/or during the postpartum period, 2.6% reported perinatal violence from in-laws, and 49.0% reported one or more forms of perinatal GBHM. In adjusted regression models that included all forms of family violence and maltreatment, perinatal GBHM remained significantly associated with infant morbidity (AORs 1.4-1.9); perinatal IPV and in-law violence ceased to predict infant morbidity in models including GBHM. Findings indicate that non-violent expressions of gender inequity (e.g., nutritional deprivation, deprivation of sleep, blocking access to health care during pregnancy) are more strongly associated with poor infant health than physical or sexual violence from husbands or in-laws in urban India. These results strongly suggest the need to expand the conception of gender inequities beyond IPV to include non-violent forms of gendered mistreatment in considering their impact on infant health.

  20. Comparison of serum oxidant and antioxidant parameters in familial Mediterranean fever patients (FMF) with attack free period.

    Science.gov (United States)

    Şahin, Ali; Erten, Şükran; Altunoğlu, Alpaslan; Işıkoğlu, Semra; Neşelioğlu, Salim; Ergin, Merve; Atalay, Hacı Veli; Erel, Özcan

    2014-01-01

    Familial Mediterranean fever (FMF) is an autoinflammatory, autosomal recessive, inherited disease characterized by recurrent self-limiting attacks of serosal surfaces. The imbalance of oxidants/antioxidants may play a role in such attacks. In this study, we aimed to evaluate the relationship between serum paraoxonase (PON1) activity, PON1 phenotype, and other parameters in patients with FMF and healthy controls. A total of 120 FMF patients with an attack-free period (AFP) and 65 healthy subjects were included in this study. The serum PON1 activity, stimulated paraoxonase (SPON) activity, PON1 phenotype (representing Q192R polymorphism; QQ, QR, RR), arylesterase activity, total oxidant status (TOS), total antioxidant capacity (TAC), oxidative stress index (OSI), advanced oxidative protein products (AOPP), total thiols (TTL), and ischemia-modified albumin (IMA) and cystatin-c (CYS-C) levels were measured. For the QQ phenotype, the median TTL and AOPP levels of the control group were 264.50 (57.75) mol/L and 21.26 (21.17) mmol/L, respectively, whereas the median TTL, AOPP levels of the patients were 309.00 (47.00) mol/L and 12.98 (6.96) mmol/L, respectively. There was a statistically significant difference between the patients and controls with the QQ phenotype in terms of TTL and AOPP (pFMF patients with AFP had higher TTL and lower AOPP levels than the controls. However, other oxidant and antioxidant parameters were similar among the patients during AFP and the controls.

  1. The large-conductance calcium-activated potassium channel holds the key to the conundrum of familial hypokalemic periodic paralysis.

    Science.gov (United States)

    Kim, June-Bum; Kim, Sung-Jo; Kang, Sun-Yang; Yi, Jin Woong; Kim, Seung-Min

    2014-10-01

    Familial hypokalemic periodic paralysis (HOKPP) is an autosomal dominant channelopathy characterized by episodic attacks of muscle weakness and hypokalemia. Mutations in the calcium channel gene, CACNA1S, or the sodium channel gene, SCN4A, have been found to be responsible for HOKPP; however, the mechanism that causes hypokalemia remains to be determined. The aim of this study was to improve the understanding of this mechanism by investigating the expression of calcium-activated potassium (KCa) channel genes in HOKPP patients. We measured the intracellular calcium concentration with fura-2-acetoxymethyl ester in skeletal muscle cells of HOKPP patients and healthy individuals. We examined the mRNA and protein expression of KCa channel genes (KCNMA1, KCNN1, KCNN2, KCNN3, and KCNN4) in both cell types. Patient cells exhibited higher cytosolic calcium levels than normal cells. Quantitative reverse transcription polymerase chain reaction analysis showed that the mRNA levels of the KCa channel genes did not significantly differ between patient and normal cells. However, western blot analysis showed that protein levels of the KCNMA1 gene, which encodes KCa1.1 channels (also called big potassium channels), were significantly lower in the membrane fraction and higher in the cytosolic fraction of patient cells than normal cells. When patient cells were exposed to 50 mM potassium buffer, which was used to induce depolarization, the altered subcellular distribution of BK channels remained unchanged. These findings suggest a novel mechanism for the development of hypokalemia and paralysis in HOKPP and demonstrate a connection between disease-associated mutations in calcium/sodium channels and pathogenic changes in nonmutant potassium channels.

  2. Evolution of embryonic developmental period in the marine bird families Alcidae and Spheniscidae: roles for nutrition and predation?

    Directory of Open Access Journals (Sweden)

    Vos Rutger A

    2010-06-01

    Full Text Available Abstract Background Nutrition and predation have been considered two primary agents of selection important in the evolution of avian life history traits. The relative importance of these natural selective forces in the evolution of avian embryonic developmental period (EDP remain poorly resolved, perhaps in part because research has tended to focus on a single, high taxonomic-level group of birds: Order Passeriformes. The marine bird families Alcidae (auks and Spheniscidae (penguins exhibit marked variation in EDP, as well as behavioural and ecological traits ultimately linked to EDP. Therefore, auks and penguins provide a unique opportunity to assess the natural selective basis of variation in a key life-history trait at a low taxonomic-level. We used phylogenetic comparative methods to investigate the relative importance of behavioural and ecological factors related to nutrition and predation in the evolution of avian EDP. Results Three behavioural and ecological variables related to nutrition and predation risk (i.e., clutch size, activity pattern, and nesting habits were significant predictors of residual variation in auk and penguin EDP based on models predicting EDP from egg mass. Species with larger clutch sizes, diurnal activity patterns, and open nests had significantly shorter EDPs. Further, EDP was found to be longer among birds which forage in distant offshore waters, relative to those that foraged in near shore waters, in line with our predictions, but not significantly so. Conclusion Current debate has emphasized predation as the primary agent of selection driving avian life history diversification. Our results suggest that both nutrition and predation have been important selective forces in the evolution of auk and penguin EDP, and highlight the importance of considering these questions at lower taxonomic scales. We suggest that further comparative studies on lower taxonomic-level groups will continue to constructively inform the

  3. Family violence and maltreatment of women during the perinatal period: Associations with infant morbidity in Indian slum communities

    Science.gov (United States)

    Silverman, Jay G.; Balaiah, Donta; Decker, Michele R.; Ritter, Julie; Naik, D.D.; Nair, Saritha; Saggurti, Niranjan; Raj, Anita

    2015-01-01

    Objectives To determine the prevalence of non-violent, gender-based forms of maltreatment of women by husbands and in-laws (i.e., gender-based household maltreatment; GBHM) during pregnancy and postpartum; to clarify the role of GBHM in compromising infant health, and whether this role extends beyond that previously observed for intimate partner violence (IPV). Methods Cross-sectional, quantitative data were collected from women (ages 15-35) seeking immunizations for their infants <6 months of age (N=1061) in urban health centers in Mumbai, India. Logistic regression models were constructed to assess associations between maternal abuse (perinatal IPV, in-law violence and GBHM) and recent infant morbidity (diarrhea, respiratory distress, fever, colic and vomiting). Results More than one in four women (28.4%) reported IPV during their recent pregnancy and/or during the postpartum period, 2.6% reported perinatal violence from in-laws, and 49.0% reported one or more forms of perinatal GBHM. In adjusted regression models that included all forms of family violence and maltreatment, perinatal GBHM remained significantly associated with infant morbidity (AORs 1.4-1.9); perinatal IPV and in-law violence ceased to predict infant morbidity in models including GBHM. Conclusions Findings indicate that non-violent expressions of gender inequity (e.g., nutritional deprivation, deprivation of sleep, blocking access to health care during pregnancy) are more strongly associated with poor infant health than physical or sexual violence from husbands or in-laws in urban India. These results strongly suggest the need to expand the conception of gender inequities beyond IPV to include non-violent forms of gendered mistreatment in considering their impact on infant health. PMID:26440937

  4. Correlating phenotype and genotype in the periodic paralyses.

    Science.gov (United States)

    Miller, T M; Dias da Silva, M R; Miller, H A; Kwiecinski, H; Mendell, J R; Tawil, R; McManis, P; Griggs, R C; Angelini, C; Servidei, S; Petajan, J; Dalakas, M C; Ranum, L P W; Fu, Y H; Ptácek, L J

    2004-11-09

    Periodic paralyses and paramyotonia congenita are rare disorders causing disabling weakness and myotonia. Mutations in sodium, calcium, and potassium channels have been recognized as causing disease. To analyze the clinical phenotype of patients with and without discernible genotype and to identify other mutations in ion channel genes associated with disease. The authors have reviewed clinical data in patients with a diagnosis of hypokalemic periodic paralysis (56 kindreds, 71 patients), hyperkalemic periodic paralysis (47 kindreds, 99 patients), and paramyotonia congenita (24 kindreds, 56 patients). For those patients without one of the classically known mutations, the authors analyzed the entire coding region of the SCN4A, KCNE3, and KCNJ2 genes and portions of the coding region of the CACNA1S gene in order to identify new mutations. Mutations were identified in approximately two thirds of kindreds with periodic paralysis or paramyotonia congenita. The authors found differences between the disorders and between those with and without identified mutations in terms of age at onset, frequency of attacks, duration of attacks, fixed proximal weakness, precipitants of attacks, myotonia, electrophysiologic studies, serum potassium levels, muscle biopsy, response to potassium administration, and response to treatment with acetazolamide. Hypokalemic periodic paralysis, hyperkalemic periodic paralysis, and paramyotonia congenita may be distinguished based on clinical data. This series of 226 patients (127 kindreds) confirms some clinical features of this disorder with notable exceptions: In this series, patients without mutations had a less typical clinical presentation including an older age at onset, no changes in diet as a precipitant, and absence of vacuolar myopathy on muscle biopsy.

  5. A sodium channel knockin mutant (NaV1.4-R669H) mouse model of hypokalemic periodic paralysis.

    Science.gov (United States)

    Wu, Fenfen; Mi, Wentao; Burns, Dennis K; Fu, Yu; Gray, Hillery F; Struyk, Arie F; Cannon, Stephen C

    2011-10-01

    Hypokalemic periodic paralysis (HypoPP) is an ion channelopathy of skeletal muscle characterized by attacks of muscle weakness associated with low serum K+. HypoPP results from a transient failure of muscle fiber excitability. Mutations in the genes encoding a calcium channel (CaV1.1) and a sodium channel (NaV1.4) have been identified in HypoPP families. Mutations of NaV1.4 give rise to a heterogeneous group of muscle disorders, with gain-of-function defects causing myotonia or hyperkalemic periodic paralysis. To address the question of specificity for the allele encoding the NaV1.4-R669H variant as a cause of HypoPP and to produce a model system in which to characterize functional defects of the mutant channel and susceptibility to paralysis, we generated knockin mice carrying the ortholog of the gene encoding the NaV1.4-R669H variant (referred to herein as R669H mice). Homozygous R669H mice had a robust HypoPP phenotype, with transient loss of muscle excitability and weakness in low-K+ challenge, insensitivity to high-K+ challenge, dominant inheritance, and absence of myotonia. Recovery was sensitive to the Na+/K+-ATPase pump inhibitor ouabain. Affected fibers had an anomalous inward current at hyperpolarized potentials, consistent with the proposal that a leaky gating pore in R669H channels triggers attacks, whereas a reduction in the amplitude of action potentials implies additional loss-of-function changes for the mutant NaV1.4 channels.

  6. Attention problems among children with a positive family history of alcohol abuse or dependence and controls. Prevalence and course for the period from preteen to early teen years.

    Science.gov (United States)

    Barnow, Sven; Schuckit, Marc; Smith, Tom; Spitzer, Carsten; Freyberger, Harald-J

    2007-01-01

    This longitudinal study investigated the scope and course of attention problems over a period of time from preteen (ages 7-12 years) to early teen years (ages 13-17 years). We compared symptoms in subjects with and without a family history (FH) of alcohol abuse or dependence from among families without evidence of antisocial personality disorder. Evaluations of attention problems for the offspring were based on the Child Behavior Checklist and a validated semistructured interview carried out with the mother. The findings indicate no higher risk for attention problems and attention-deficit hyperactivity disorder (ADHD)-like symptoms in the children of families with an alcohol use disorder. Regarding the course of problems, the ADHD symptom count tended to decrease over time, especially for children without a FH of alcohol abuse or dependence. Further research will be needed to determine whether results can be replicated with families from different social strata and including subjects with the antisocial personality disorder.

  7. Family and Rural Community. Agricultural Models, Social Collectivism and Family Behaviour in the Province of Leon in the Early Modern Period

    Directory of Open Access Journals (Sweden)

    María José PÉREZ ÁLVAREZ

    2014-12-01

    Full Text Available In the second half of the twentieth century, studies on the family occupied a very important place in modern Spanish historiography, reaching a peak in the nineties. Whilst it is true that some regions received more research attention than others, it can also be said that in general, the entire country was represented in this research. Thanks to these studies and to the different analytical perspectives and diversity of sources and methodologies they used, we now have extensive knowledge of all matters relating to this subject. As regards the province of Leon, much research has been conducted on family issues, focusing on rural areas. These studies have analysed patterns of adaptation in the region as regards three variables wholly linked to social reproduction: marriage, co-residence and the division of property.

  8. Performance of Different Diagnostic Criteria for Familial Mediterranean Fever in Children with Periodic Fevers : Results from a Multicenter International Registry

    NARCIS (Netherlands)

    Demirkaya, Erkan; Saglam, Celal; Turker, Turker; Koné-Paut, Isabelle; Woo, Pat; Doglio, Matteo; Amaryan, Gayane; Frenkel, Joost; Uziel, Yosef; Insalaco, Antonella; Cantarini, Luca; Hofer, Michael; Boiu, Sorina; Duzova, Ali; Modesto, Consuelo; Bryant, Annette; Rigante, Donato; Papadopoulou-Alataki, Efimia; Guillaume-Czitrom, Severine; Kuemmerle-Deschner, Jasmine; Neven, Bénédicte; Lachmann, Helen; Martini, Alberto; Ruperto, Nicolino; Gattorno, Marco; Ozen, Seza

    2015-01-01

    OBJECTIVE: Our aims were to validate the pediatric diagnostic criteria in a large international registry and to compare them with the performance of previous criteria for the diagnosis of familial Mediterranean fever (FMF). METHODS: Pediatric patients with FMF from the Eurofever registry were used

  9. The course and impact of family optimism in the post-acute period after acquired brain injury.

    Science.gov (United States)

    Riley, Gerard A; Hough, Andrea; Meader, Laura M; Brennan, Andrew J

    2015-01-01

    To investigate the course and impact of family optimism in the post-acute stage of acquired brain injury. At Time 1, 30 family relatives of in-patients in rehabilitation units and 30 relatives of patients recently discharged from such units completed questionnaires relating to their emotional health, engagement in the rehabilitation process and expectations about the future consequences and controllability of the injury. At Time 2 (12-18 months later), 23 of the original sample completed questionnaires about their emotional health and actual consequences and controllability of the injury. At Time 1, optimism about future consequences and controllability was associated with greater engagement in the rehabilitation process and better emotional health. The two groups did not differ on any of the measures, which did not support the expectation that the patient's discharge home would trigger a loss of optimism and emotional upset for the family. At Time 2, the actual consequences were worse than had been expected at Time 1 and greater disappointment was associated with a greater decline in emotional wellbeing. Family expectations about recovery are linked with important variables such as emotional wellbeing and engagement in the rehabilitation process and need careful management by clinicians.

  10. Performance of Different Diagnostic Criteria for Familial Mediterranean Fever in Children with Periodic Fevers : Results from a Multicenter International Registry

    NARCIS (Netherlands)

    Demirkaya, Erkan; Saglam, Celal; Turker, Turker; Koné-Paut, Isabelle; Woo, Pat; Doglio, Matteo; Amaryan, Gayane; Frenkel, Joost; Uziel, Yosef; Insalaco, Antonella; Cantarini, Luca; Hofer, Michael; Boiu, Sorina; Duzova, Ali; Modesto, Consuelo; Bryant, Annette; Rigante, Donato; Papadopoulou-Alataki, Efimia; Guillaume-Czitrom, Severine; Kuemmerle-Deschner, Jasmine; Neven, Bénédicte; Lachmann, Helen; Martini, Alberto; Ruperto, Nicolino; Gattorno, Marco; Ozen, Seza

    2015-01-01

    OBJECTIVE: Our aims were to validate the pediatric diagnostic criteria in a large international registry and to compare them with the performance of previous criteria for the diagnosis of familial Mediterranean fever (FMF). METHODS: Pediatric patients with FMF from the Eurofever registry were used f

  11. Cost curves for the navigation between families of low energy Fast Periodic Transfer Orbits in the Earth-Moon planar circular restricted Three Body Problem.

    Science.gov (United States)

    Marcuzzi, J. P.; Leiva, A. M.; Briozzo, C. B.

    Using characteristic curves of low energy fast periodic transfer orbits in the Earth-Moon planar circular restricted three body problem we constructed cost curves that enable the study of maneuvers between them. In an appropiate surface of section the numerical diagrams show regions where transfer maneuvers involve lower costs and that would allow determine family members which make the propelent minimum for these maneuvers. FULL TEXT IN SPANISH.

  12. Reduced expression and abnormal localization of the K(ATP) channel subunit SUR2A in patients with familial hypokalemic periodic paralysis.

    Science.gov (United States)

    Kim, Sung-Jo; Lee, Yea-Jin; Kim, June-Bum

    2010-01-01

    Familial hypokalemic periodic paralysis is an autosomal-dominant channelopathy that features episodic attacks of flaccid paralysis with concomitant hypokalemia. Reduced activity of ATP-sensitive K(+) (K(ATP)) channels is suggested to be responsible for this disorder; however, the molecular mechanisms have not yet been elucidated. In this study, we investigated the molecular mechanism of reduced K(ATP) channel activity in skeletal muscle cells of patients with familial hypokalemic periodic paralysis. We examined the mRNA and protein levels of SUR2A, a K(ATP) channel subunit, in cells from patients (patient cells) and normal individuals (normal cells). Our results demonstrated that normal cells exposed to 50mM potassium buffer, which was used to induce depolarization, did not show significant change in the SUR2A mRNA levels; however, the protein level significantly increased in the cytosolic fraction. When the patient cells were exposed to 50mM potassium buffer, the SUR2A mRNA level significantly decreased. Further, the protein level of SUR2A significantly increased in the membrane fraction but decreased in the cytosolic fraction in patient cells. These findings suggest that abnormal localization of the SUR2A K(+) channel protein leads to reduced K(ATP) channel activity in familial hypokalemic periodic paralysis. Copyright 2009 Elsevier Inc. All rights reserved.

  13. 浅析社会转型期的家庭道德教育%Discussion of Family Moral Education During the Period of Social Transformation

    Institute of Scientific and Technical Information of China (English)

    宋月丽

    2014-01-01

    当前,青少年的道德缺失问题引起社会的广泛关注,家庭道德教育成为人们关注的焦点。家庭是人的第一生活环境,家庭道德教育是每个人接受道德教育的开始,是每个人接受道德教育的关键环节。然而,我国现在正处于社会转型期,许多不利因素冲击着家庭道德教育。拟就社会转型期家庭道德教育存在的缺失问题进行原因分析,并在此基础上提出一些对策。%Lacking of ethics among teenagers has caused widespread concern of society , family moral education has become the focus of attention .Family is the first environment for the growth of children family moral education is the beginning to accept moral education ..During the period of social transition , many factors have impacts on family moral education This paper analyzes those factors affected family moral education and puts forward some suggestions to carry out the moral education .

  14. [Molecular diagnostics of hereditary fever syndromes. Familial Mediterranean fever (FMF), hyperimmunoglobulin D syndrome (HIDS), tumor necrosis factor receptor-associated periodic syndrome (TRAPS), cryopyrin-associated periodic syndrome (CAPS: FCAS, MWS, NOMID/CINCA)].

    Science.gov (United States)

    Timmann, C; Horstmann, R

    2009-11-01

    Periodic episodes of fever and inflammation can have a genetic origin. Nowadays, the identification of the causative genetic variants in the majority of cases allows molecular genetic confirmation of the clinical diagnosis, which enables approaches with specific drug treatment and improves patient compliance as well as genetic counseling. Besides a detailed clinical examination a medical history including family history and an assessment of the ethnic origin are required. In order to make genetic testing straightforward and cost effective an iterative procedure should be followed which should include, in addition to clinical data, the frequencies of causative mutations in the various gene segments involved.

  15. Socioeconomic and familial characteristics influence caretakers' adherence to the periodic vitamin A capsule supplementation program in Central Java, Indonesia.

    Science.gov (United States)

    Pangaribuan, Rosnani; Scherbaum, Veronika; Erhardt, Jürgen G; Sastroamidjojo, Soemilah; Biesalski, Hans K

    2004-06-01

    The adherence of program participants to periodic vitamin A capsule (VAC) supplementation among children aged 1-5 years (n = 677) in Central Java, Indonesia was assessed. Fourteen villages from five sub-districts and one ward from one sub-district in Central Java were included in the study to represent rural and suburban areas. All questions about demographic factors, socioeconomic conditions, current dietary practice and healthcare-seeking attitudes for common childhood illnesses, previous breastfeeding experience, their knowledge about vitamin A and adherence to the VAC program after capsule distribution (two periods in 2000) were asked. Caretakers with limited knowledge about the health benefits of vitamin A, households with more than one preschool child, and households with older children (> 36 months) were associated with a decreased likelihood of regular participation in the program with odds ratios of 0.38, 0.55, and 0.26, respectively (p program regularly with an odds ratio of 2.02 (p program.

  16. Kinetic Characteristics and Continuation Algorithm of Periodic Families%Halo轨道族延拓方法及特性研究

    Institute of Scientific and Technical Information of China (English)

    刘磊; 刘勇; 曹建峰; 唐歌实; 胡松杰

    2013-01-01

    Study on halo orbit period and scope of kinetic characteristics is the premise of halo orbit applications. For the applications and some numerical issues on a big halo orbit and a whole halo family, considering the wide applications of the libration points in the Sun-Earth/ Moon and Earth-Moon system, the kinetic characteristics and the continuation algorithm of periodic families were studied under the circular restricted three-body problem. The method of numerical halo family determination by the continuation algorithm was presented. Influences of family parameter selection on family determination were analyzed. Finally, the halo families with a large scope were given in the Sun-Earth/Moon and Earth-Moon system, together with their kinetic characteristics of orbit periods and locations. The results show that the family parameter of x is preferable for an LI halo family and the y velocity or T is preferable for L2. The method is fit for the numerical halo family determination in any three-body system, especially for those under perturbations by a simple modification of state transition matrix.%对Halo轨道周期和运动范围等特性的研究是平动点任务设计的首要前提.针对大幅值Halo轨道和完整Halo轨道族的应用需求及其数值计算问题,面向当前应用广泛的地月系和日-地月系共线平动点,基于延拓法研究了圆型限制性三体问题下的Halo轨道族数值计算和运动学特性,给出了Halo轨道族延拓计算方法.数值仿真了族参数选择对轨道族计算的影响,得到了地月系和日-地月系共线平动点的大范围南北Halo轨道族,同时给出了轨道族的轨道周期变化和空间位置变化特性.研究结果表明,固定延拓步长下,L1点Halo轨道族应选择会合坐标系x坐标作为族参数,L2点Halo轨道族应选择y方向速度或者周期T作为族参数.方法适用于任意三体系统平动点的周期轨道族计算,特别是对其中的状态转移矩阵简

  17. Concordance between CRP and SAA in familial Mediterranean fever during attack-free period: A study of 218 patients.

    Science.gov (United States)

    Stankovic Stojanovic, Katia; Hentgen, Véronique; Fellahi, Soraya; Georgin-Lavialle, Sophie; Amselem, Serge; Grateau, Gilles; Bastard, Jean-Philippe; Steichen, Olivier

    2017-03-01

    Monitoring SAA level in attack-free FMF patients is recommended in order to adjust colchicine dose, and minimize the risk of AA amyloidosis. In countries where this test is not available, C-reactive protein (CRP), another acute phase reactant, is used instead. However, CRP is low and SAA is increased in some patients and vice versa. To determine the threshold of CRP corresponding to SAA<10mg/L in patients with FMF and to assess their concordance at the patient level. Consecutive FMF patients in attack-free period and no other cause of intermittent inflammation including infections were recruited during their regular visits in the French reference center for FMF. Demographic and genetic data were recorded; CRP and SAA were tested simultaneously. The threshold value of CRP corresponding to 10mg/L for SAA was determined and the concordance between the two markers was assessed with Cohen's kappa index. 399 samples were obtained from 218 patients, mean age of 27years (33% under 18years old), 55% of female, from Sephardic Jewish origin in 71%. MEFV mutation was M694V homozygous or compound heterozygous in 52%, and simple heterozygous in 18%. Six patients had AA amyloidosis. The appropriate CRP threshold was found to be 5mg/L in children and 8.75mg/L in adults. Global agreement with SAA<10mg/L was 84% [95% confidence interval: 82 to 86%], leading to a kappa index at 0.62 [95% confidence interval: 0.57 to 0.68]. CRP<5mg/L in FMF children or 8.75mg/L in FMF adults during attack-free periods might be a convenient substitute to guide therapeutic decisions when SAA is unavailable. Copyright © 2016 The Canadian Society of Clinical Chemists. Published by Elsevier Inc. All rights reserved.

  18. Relationship between genetic mutation variations and acute-phase reactants in the attack-free period of children diagnosed with familial Mediterranean fever

    Energy Technology Data Exchange (ETDEWEB)

    Kosan, C. [Department of Pediatric Nephrology, Faculty of Medicine, Ataturk University, Erzurum (Turkey); Cayir, A.; Turan, M.I. [Department of Pediatrics, Faculty of Medicine, Ataturk University, Erzurum (Turkey)

    2013-09-18

    Familial Mediterranean fever (FMF) is a periodic autoinflammatory disease characterized by chronic inflammation. This study investigated the relationship between acute-phase reactants and gene mutations in attack-free periods of childhood FMF. Patients diagnosed with FMF were divided into four groups based on genetic features: no mutation, homozygous, heterozygous, and compound heterozygous. These groups were monitored for 2 years, and blood samples were collected every 6 months during attack-free periods. Erythrocyte sedimentation rate, C-reactive protein, fibrinogen, and white blood cell count were measured. A disease severity score was determined for each patient. Mean values for erythrocyte sedimentation rate and fibrinogen were significantly different in the homozygous group. White blood cell count and C-reactive protein were similar between the groups. Disease severity score was higher in patients with the M694V mutation than in individuals without the mutation, as well as in those with other mutation groups. Periodic follow-up of patients with FMF MEFV mutations in subjects with acute-phase reactants may be useful in the prevention of morbidity.

  19. Relationship between genetic mutation variations and acute-phase reactants in the attack-free period of children diagnosed with familial Mediterranean fever

    Science.gov (United States)

    Kosan, C.; Cayir, A.; Turan, M.I.

    2013-01-01

    Familial Mediterranean fever (FMF) is a periodic autoinflammatory disease characterized by chronic inflammation. This study investigated the relationship between acute-phase reactants and gene mutations in attack-free periods of childhood FMF. Patients diagnosed with FMF were divided into four groups based on genetic features: no mutation, homozygous, heterozygous, and compound heterozygous. These groups were monitored for 2 years, and blood samples were collected every 6 months during attack-free periods. Erythrocyte sedimentation rate, C-reactive protein, fibrinogen, and white blood cell count were measured. A disease severity score was determined for each patient. Mean values for erythrocyte sedimentation rate and fibrinogen were significantly different in the homozygous group. White blood cell count and C-reactive protein were similar between the groups. Disease severity score was higher in patients with the M694V mutation than in individuals without the mutation, as well as in those with other mutation groups. Periodic follow-up of patients with FMF MEFV mutations in subjects with acute-phase reactants may be useful in the prevention of morbidity. PMID:24141617

  20. Relationship between genetic mutation variations and acute-phase reactants in the attack-free period of children diagnosed with familial Mediterranean fever

    Directory of Open Access Journals (Sweden)

    C. Kosan

    2013-09-01

    Full Text Available Familial Mediterranean fever (FMF is a periodic autoinflammatory disease characterized by chronic inflammation. This study investigated the relationship between acute-phase reactants and gene mutations in attack-free periods of childhood FMF. Patients diagnosed with FMF were divided into four groups based on genetic features: no mutation, homozygous, heterozygous, and compound heterozygous. These groups were monitored for 2 years, and blood samples were collected every 6 months during attack-free periods. Erythrocyte sedimentation rate, C-reactive protein, fibrinogen, and white blood cell count were measured. A disease severity score was determined for each patient. Mean values for erythrocyte sedimentation rate and fibrinogen were significantly different in the homozygous group. White blood cell count and C-reactive protein were similar between the groups. Disease severity score was higher in patients with the M694V mutation than in individuals without the mutation, as well as in those with other mutation groups. Periodic follow-up of patients with FMF MEFV mutations in subjects with acute-phase reactants may be useful in the prevention of morbidity.

  1. LONG CHAIN FATTY ACID (LCFA) ABNORMALITIES IN HYPER IgD SYNDROME (HIDS) AND FAMILIAL MEDITERRANEAN FEVER (FMF): NEW INSIGHT INTO HERITABLE PERIODIC FEVERSa

    Science.gov (United States)

    Simon, Anna; Drenth, Joost PH; Matern, Dietrich; Goetzman, Eric S.; Hager, Elizabeth J.; Gibson, K Michael

    2013-01-01

    Objective To examine essential fatty acids (EFAs) in hyper-IgD syndrome (HIDS) and Familial Mediterranean Fever (FMF). Methods EFAs were determined in sera derived from an archival, cross-sectional group of HIDS/FMF patients, stratified for presence and absence of fever. Control populations included healthy afebrile adults, and individuals with non-periodic fever (septic shock). EFAs were quantified using isotope dilution gas chromatography-mass spectrometry and data analyzed employing a Kruskal-Wallis non-parametric ANOVA with Dunn's post-hoc test. Results Sera samples derived from HIDS patients showed significantly decreased C20, C26, phytanic and pristanic acids during febrile crises that normalized in the afebrile state, and a significantly increased afebrile C22_ω46 level that normalized with fever. Samples derived from FMF patients revealed increased ω-oxidized LCFAs as compared to controls, and the trend was for these same species to be increased in comparison to febrile, but not afebrile, HIDS patients. Individuals with non-periodic fever demonstrated global decreases in C10–C24 fatty acids, both saturated and unsaturated, accompanied by an elevated triene/tetraene ratio. Conclusions Our results suggest that different mechanisms are active in hereditary periodic fever syndromes that appear unrelated to fever, including depletion of very long chain fatty acids (VLCFAs) in febrile HIDS patients and increased ω-oxidized LCFAs in patients with FMF. These findings underscore new roles for EFAs in the potential production of inflammatory species in patients with hereditary periodic fever. PMID:23375471

  2. Long chain fatty acid (Lcfa) abnormalities in hyper Igd syndrome (Hids) and Familial Mediterranean Fever (Fmf): new insight into heritable periodic fevers.

    Science.gov (United States)

    Simon, Anna; Drenth, Joost P H; Matern, Dietrich; Goetzman, Eric S; Hager, Elizabeth J; Gibson, K Michael

    2013-03-01

    To examine essential fatty acids (EFAs) in hyper-IgD syndrome (HIDS) and Familial Mediterranean Fever (FMF). EFAs were determined in sera derived from an archival, cross-sectional group of HIDS/FMF patients, stratified for presence and absence of fever. Control populations included healthy afebrile adults, and individuals with non-periodic fever (septic shock). EFAs were quantified using isotope dilution gas chromatography-mass spectrometry and data analyzed employing a Kruskal-Wallis non-parametric ANOVA with Dunn's post-hoc test. Sera samples derived from HIDS patients showed significantly decreased C20, C26, phytanic and pristanic acids during febrile crises that normalized in the afebrile state, and a significantly increased afebrile C22_4ω6 level that normalized with fever. Samples derived from FMF patients revealed increased ω-oxidized LCFAs as compared to controls, and the trend was for these same species to be increased in comparison to febrile, but not afebrile, HIDS patients. Individuals with non-periodic fever demonstrated global decreases in C10-C24 fatty acids, both saturated and unsaturated, accompanied by an elevated triene/tetraene ratio. Our results suggest that different mechanisms are active in hereditary periodic fever syndromes that appear unrelated to fever, including depletion of very long chain fatty acids (VLCFAs) in febrile HIDS patients and increased ω-oxidized LCFAs in patients with FMF. These findings underscore new roles for EFAs in the potential production of inflammatory species in patients with hereditary periodic fever. Copyright © 2013 Elsevier Inc. All rights reserved.

  3. Alternative Transfers to the NEOs 99942 Apophis, 1994 WR12, and 2007 UW1 via Derived Trajectories from Periodic Orbits of Family G

    Directory of Open Access Journals (Sweden)

    C. F. de Melo

    2009-01-01

    Full Text Available Swing-by techniques are extensively used in interplanetary missions to minimize fuel consumption and to raise payloads of spacecrafts. The effectiveness of this type of maneuver has been proven since the beginning of space exploration. According to this premise, we have explored the existence of a natural and direct link between low Earth orbits and the lunar sphere of influence to get low-energy transfer trajectories to the Near Earth Objects (NEOs 99942 Apophis, 1994 WR12, and 2007 UW1 through swing-bys with the Moon. The existence of this link is related to a family of retrograde periodic orbits around the Lagrangian equilibrium point L1 predicted for the circular, planar, restricted three-body Earth-Moon-particle problem. The trajectories in this link are sensitive to small disturbances. This enables them to be conveniently diverted reducing so the cost of the swing-by maneuver. These maneuvers allow a gain in energy sufficient for the trajectories to escape from the Earth-Moon system and to stabilize in heliocentric orbits between the Earth and Venus or Earth and Mars. Therefore, the trajectories have sufficient reach to intercept the NEOs' orbits.

  4. On a family of KP multi-line solitons associated to rational degenerations of real hyperelliptic curves and to the finite non-periodic Toda hierarchy

    Science.gov (United States)

    Abenda, Simonetta

    2017-09-01

    We continue the program started in Abenda and Grinevich (2015) of associating rational degenerations of M-curves to points in GrTNN(k , n) using KP theory for real finite gap solutions. More precisely, we focus on the inverse problem of characterizing the soliton data which produce Krichever divisors compatible with the KP reality condition when Γ is a certain rational degeneration of a hyperelliptic M-curve. Such choice is motivated by the fact that Γ is related to the curves associated to points in GrTP(1 , n) and in GrTP(n - 1 , n) in Abenda and Grinevich (2015). We prove that the reality condition on the Krichever divisor on Γ singles out a special family of KP multi-line solitons (T-hyperelliptic solitons) in GrTP(k , n) , k ∈ [ n - 1 ] , naturally connected to the finite non-periodic Toda hierarchy. We discuss the relations between the algebraic-geometric description of KP T-hyperelliptic solitons and of the open Toda system. Finally, we also explain the effect of the space-time transformation which conjugates soliton data in GrTP(k , n) to soliton data in GrTP(n - k , n) on the Krichever divisor for such KP solitons.

  5. International Retrospective Chart Review of Treatment Patterns in Severe Familial Mediterranean Fever, Tumor Necrosis Factor Receptor-Associated Periodic Syndrome, and Mevalonate Kinase Deficiency/Hyperimmunoglobulinemia D Syndrome.

    Science.gov (United States)

    Ozen, Seza; Kuemmerle-Deschner, Jasmin B; Cimaz, Rolando; Livneh, Avi; Quartier, Pierre; Kone-Paut, Isabelle; Zeft, Andrew; Spalding, Steve; Gul, Ahmet; Hentgen, Veronique; Savic, Sinisa; Foeldvari, Ivan; Frenkel, Joost; Cantarini, Luca; Patel, Dony; Weiss, Jeffrey; Marinsek, Nina; Degun, Ravi; Lomax, Kathleen G; Lachmann, Helen J

    2017-04-01

    Periodic fever syndrome (PFS) conditions are characterized by recurrent attacks of fever and localized inflammation. This study examined the diagnostic pathway and treatments at tertiary centers for familial Mediterranean fever (FMF), tumor necrosis factor receptor-associated periodic syndrome (TRAPS), and mevalonate kinase deficiency (MKD)/hyperimmunoglobulinemia D syndrome (HIDS). PFS specialists at medical centers in the US, the European Union, and the eastern Mediterranean participated in a retrospective chart review, providing de-identified data in an electronic case report form. Patients were treated between 2008 and 2012, with at least 1 year of followup; all had clinical and/or genetically proven disease and were on/eligible for biologic treatment. A total of 134 patients were analyzed: FMF (n = 49), TRAPS (n = 47), and MKD/HIDS (n = 38). Fever was commonly reported as severe across all indications. Other frequently reported severe symptoms were serositis for FMF patients and elevated acute-phase reactants and gastrointestinal upset for TRAPS and MKD/HIDS. A long delay from disease onset to diagnosis was seen within TRAPS and MKD/HIDS (5.8 and 7.1 years, respectively) compared to a 1.8-year delay in FMF patients. An equal proportion of TRAPS patients first received anti-interleukin-1 (anti-IL-1) and anti-tumor necrosis factor (anti-TNF) biologic agents, whereas IL-1 blockade was the main choice for FMF patients resistant to colchicine and MKD/HIDS patients. For TRAPS patients, treatment with anakinra versus anti-TNF treatments as first biologic agent resulted in significantly higher clinical and biochemical responses (P = 0.03 and P < 0.01, respectively). No significant differences in responses were observed between biologic agents among other cohorts. Referral patterns and diagnostic delays highlight the need for greater awareness and improved diagnostics for PFS. This real-world treatment assessment supports the need for further

  6. Socio-culturele structuur en innovatie : een structuur-vergelijkend onderzoek naar adoptie van family planning in de periode 1969 - 1973 door Sundanese echtparen in twee rurale gemeenschappen op West-Java

    NARCIS (Netherlands)

    Norren, van B.

    1985-01-01

    This publication contains a comparative study of socio- cultural influences on the process of family planning adoption during the period 1969-1973 in two rural communities in the regency of Bandung, West Java, Indonesia. One community, to be called Cianyar, is a ward in an agrarian village

  7. Socio-culturele structuur en innovatie : een structuur-vergelijkend onderzoek naar adoptie van family planning in de periode 1969 - 1973 door Sundanese echtparen in twee rurale gemeenschappen op West-Java

    NARCIS (Netherlands)

    Norren, van B.

    1985-01-01

    This publication contains a comparative study of socio- cultural influences on the process of family planning adoption during the period 1969-1973 in two rural communities in the regency of Bandung, West Java, Indonesia. One community, to be called Cianyar, is a ward in an agrarian

  8. Thyrotoxic Hypokalemic Periodic Paralysis (THPP: a challenge for the Emergency Medicine physician in Western countries. A case report

    Directory of Open Access Journals (Sweden)

    F. Stella

    2012-03-01

    Full Text Available Thyrotoxic hypokalemic periodic paralysis (THPP is an endocrine emergency with sudden onset of acute muscle hypo-asthenia associated with severe hypokalemia and normal acid-base balance, due to thyrotoxicosis. It may develop in patients of Asian and Native American descent, but it is very rare and undiagnosed in Western countries. Standard treatment includes β-blockers and thyrostatic drugs, but hypokalemia should not be treated with high potassium doses due to the risk of hyperkalemic rebound. Since early diagnosis and correct treatment can prevent life-threatening cardio-pulmonary complications, the emergency physician should keep in mind this rare possibility. We report a case of a 43 year old Caucasian man presenting at ED with tetraparesis as the first symptom of thyrotoxicosis with no other remarkable symptoms or signs.

  9. State of the art in educational journals on studies of families of children with special educational needs in the period from 2002 to 2011

    Directory of Open Access Journals (Sweden)

    Giovana Mendes Ferroni

    2014-04-01

    Full Text Available The purpose of this paper is mapping studies indexed between the years of 2002 and 2011 in the educational field and evaluated like A1 or A2 by the database Qualis Capes, in the three years 2010-2012, in order to research ways that studies with families of children with special educational needs have been made. For this, we analyze elements such as: annual evolution of the studies, subjects analyzed, adopted and thematic instruments used to collect data from database searches “Scielo” and “Capes Journal” and a multitude of descriptors combined. The criterion for considering the family was that the research should contain the following keywords: family, family relationships, parental relationships, parents and siblings crisscrossing: special education, people with special educational needs, inclusive education, inclusion, autism, autistic, gifted, high ability, cerebral palsy, low vision, blindness, deafness, pervasive developmental disorder, Down Syndrome, disability, intellectual disability, physical disability, visual impairment, hearing impairment and multiple disabilities. Thus, the papers were obtained by intercrossing these descriptors, but it was selected those containing one of these words. With this, we found a total of 36 papers in national journals indexed in the area of Education. The data showed that there was a growing body of empirical research in this area.

  10. Long chain fatty acid (Lcfa) abnormalities in hyper Igd syndrome (Hids) and Familial Mediterranean Fever (Fmf): new insight into heritable periodic fevers

    NARCIS (Netherlands)

    Simon, A.; Drenth, J.P.H.; Matern, D.; Goetzman, E.S.; Hager, E.J.; Gibson, K.M.

    2013-01-01

    OBJECTIVE: To examine essential fatty acids (EFAs) in hyper-IgD syndrome (HIDS) and Familial Mediterranean Fever (FMF). METHODS: EFAs were determined in sera derived from an archival, cross-sectional group of HIDS/FMF patients, stratified for presence and absence of fever. Control populations

  11. Long chain fatty acid (Lcfa) abnormalities in hyper Igd syndrome (Hids) and Familial Mediterranean Fever (Fmf): new insight into heritable periodic fevers

    NARCIS (Netherlands)

    Simon, A.; Drenth, J.P.H.; Matern, D.; Goetzman, E.S.; Hager, E.J.; Gibson, K.M.

    2013-01-01

    OBJECTIVE: To examine essential fatty acids (EFAs) in hyper-IgD syndrome (HIDS) and Familial Mediterranean Fever (FMF). METHODS: EFAs were determined in sera derived from an archival, cross-sectional group of HIDS/FMF patients, stratified for presence and absence of fever. Control populations includ

  12. The Fine Design of Small Family Housing Construction in the New Period%新时期小户型住宅建筑精细化设计

    Institute of Scientific and Technical Information of China (English)

    陈大卫

    2014-01-01

    笔者结合多年的实践经验,就如何进行小户型住宅建筑精细化设计问题,从建筑平面、玄关、储藏空间、阳台等方面进行了探讨,以供相关人员参考。%The author combines with years of practical expe-rience, elaborates how to make smal family housing problem from the aspects of architectural plane, porch, storage space, balcony, etc to discuss, in order to of er reference for the rela-ted personnel.

  13. Angular momentum and topological dependence of Kepler's Third Law in the Broucke-Hadjidemetriou-H\\'enon family of periodic three-body orbits

    CERN Document Server

    Janković, Marija R

    2016-01-01

    We use 57 recently found topological satellites of Broucke-Hadjidemetriou-Henon's periodic orbits with values of the topological exponent $k$ ranging from $k$ = 3 to $k$ = 58 to plot the angular momentum $L$ as a function of the period $T$, with both $L$ and $T$ rescaled to energy $E=-\\frac12$. Upon plotting $L(T/k)$ we find that all our solutions fall on a curve that is virtually indiscernible by naked eye from the $L(T)$ curve for non-satellite solutions. The standard deviation of the satellite data from the sixth-order polynomial fit to the progenitor data is $\\sigma = 0.13$. This regularity supports Henon's 1976 conjecture that the linearly stable Broucke-Hadjidemetriou-Henon orbits are also perpetually, or Kolmogorov-Arnold-Moser stable.

  14. [Restoration of speech in aphasia during the post hospital period based on the "speech donorship" method and a family speech discourse].

    Science.gov (United States)

    Rudnev, V A; Shteĭnerdt, V V

    2010-01-01

    The method of "speech donorship" is based on genetically mediated factors of tempo-rhythmic concordance of speech in monozygotic twins (co-twins) and pairs of close relatives (father-son, mother-daughter, sibs). Recording of the natural audiovisual donor sample of the speech adapted for a structurally-linguistic condition of speech of the recipient was carried out on a digital movie camera. This sample is defined using the data of computer transformation obtained by the program specially developed by the authors. The program allows to compute time equivalents of three parameters: the time spent for realization of "word", "pause", "word + pauses". Work of the recipient with the screen donor sample assumes a support of the restoration of genetic and adaptive speech patterns. Then the recipient works with the own audiovisual sample. The dictionary of a family speech was used to build tests. The use of this method was described for 15 patients with aphasia of vascular and traumatic etiology.

  15. Voltage-sensor movements describe slow inactivation of voltage-gated sodium channels II: a periodic paralysis mutation in Na(V)1.4 (L689I).

    Science.gov (United States)

    Silva, Jonathan R; Goldstein, Steve A N

    2013-03-01

    In skeletal muscle, slow inactivation (SI) of Na(V)1.4 voltage-gated sodium channels prevents spontaneous depolarization and fatigue. Inherited mutations in Na(V)1.4 that impair SI disrupt activity-induced regulation of channel availability and predispose patients to hyperkalemic periodic paralysis. In our companion paper in this issue (Silva and Goldstein. 2013. J. Gen. Physiol. http://dx.doi.org/10.1085/jgp.201210909), the four voltage sensors in Na(V)1.4 responsible for activation of channels over microseconds are shown to slowly immobilize over 1-160 s as SI develops and to regain mobility on recovery from SI. Individual sensor movements assessed via attached fluorescent probes are nonidentical in their voltage dependence, time course, and magnitude: DI and DII track SI onset, and DIII appears to reflect SI recovery. A causal link was inferred by tetrodotoxin (TTX) suppression of both SI onset and immobilization of DI and DII sensors. Here, the association of slow sensor immobilization and SI is verified by study of Na(V)1.4 channels with a hyperkalemic periodic paralysis mutation; L689I produces complex changes in SI, and these are found to manifest directly in altered sensor movements. L689I removes a component of SI with an intermediate time constant (~10 s); the mutation also impedes immobilization of the DI and DII sensors over the same time domain in support of direct mechanistic linkage. A model that recapitulates SI attributes responsibility for intermediate SI to DI and DII (10 s) and a slow component to DIII (100 s), which accounts for residual SI, not impeded by L689I or TTX.

  16. Voltage-sensor movements describe slow inactivation of voltage-gated sodium channels II: A periodic paralysis mutation in NaV1.4 (L689I)

    Science.gov (United States)

    Silva, Jonathan R.

    2013-01-01

    In skeletal muscle, slow inactivation (SI) of NaV1.4 voltage-gated sodium channels prevents spontaneous depolarization and fatigue. Inherited mutations in NaV1.4 that impair SI disrupt activity-induced regulation of channel availability and predispose patients to hyperkalemic periodic paralysis. In our companion paper in this issue (Silva and Goldstein. 2013. J. Gen. Physiol. http://dx.doi.org/10.1085/jgp.201210909), the four voltage sensors in NaV1.4 responsible for activation of channels over microseconds are shown to slowly immobilize over 1–160 s as SI develops and to regain mobility on recovery from SI. Individual sensor movements assessed via attached fluorescent probes are nonidentical in their voltage dependence, time course, and magnitude: DI and DII track SI onset, and DIII appears to reflect SI recovery. A causal link was inferred by tetrodotoxin (TTX) suppression of both SI onset and immobilization of DI and DII sensors. Here, the association of slow sensor immobilization and SI is verified by study of NaV1.4 channels with a hyperkalemic periodic paralysis mutation; L689I produces complex changes in SI, and these are found to manifest directly in altered sensor movements. L689I removes a component of SI with an intermediate time constant (∼10 s); the mutation also impedes immobilization of the DI and DII sensors over the same time domain in support of direct mechanistic linkage. A model that recapitulates SI attributes responsibility for intermediate SI to DI and DII (10 s) and a slow component to DIII (100 s), which accounts for residual SI, not impeded by L689I or TTX. PMID:23401572

  17. On Strengthening Party Member Leading Cadres’Family Rules in the New Period%新时期加强党员领导干部家风建设的思考

    Institute of Scientific and Technical Information of China (English)

    黄德锋; 潘洋

    2016-01-01

    The family rules of party member leading cadres relate to the party conduct,the government conduct and the social atmosphere.To strengthen party member leading cadres’family rules in the new period is not only an important content of carrying through the clean government campaign,but also a requisite measure for carrying out the requirement of comprehensively strengthening the party disci-pline.The emerging of problems regarding party member leading cadres’family rules has its social cau-ses in depth.Therefore,to strengthen the family rules of party member cadres,we must enhance the ac-complishment in party spirit,firm the ideal and faith,improve the moral level,manage our families strictly,and normalize family members’behavior and meanwhile,strengthen the supervision and con-striction on leading cadres,strengthen the evaluation system and the education for leading cadres,pass on the excellent family tradition and the red gene,build up modern family rules,actively form the politi-cal ecology of cleanness and integrity,and concretely perform the work of comprehensively strengthe-ning party discipline.%党员领导干部的家风关系到党风、政风和社会风气。新时期加强党员领导干部家风建设既是当前开展党风廉政建设的重要内容,也是落实全面从严治党要求的必要举措。党员领导干部家风问题的出现有其深刻的社会原因,因此,加强党员领导干部家风建设,必须加强党性修养,坚定理想信念和提升道德境界,从严治家,规范家属的行为,同时强化对领导干部的监督和约束,严格考评机制,加大教育力度,传承传统优秀家风和红色基因,努力构建现代家风,从而积极营造风清气正的政治生态,切实推进全面从严治党。

  18. Period Cramps

    Science.gov (United States)

    ... Too Tall or Too Short All About Puberty Period Cramps KidsHealth > For Kids > Period Cramps Print A ... re a girl who gets them. What Are Period Cramps? Lots of girls experience cramps before or ...

  19. A severe autosomal-dominant periodic inflammatory disorder with renal AA amyloidosis and colchicine resistance associated to the MEFV H478Y variant in a Spanish kindred: an unusual familial Mediterranean fever phenotype or another MEFV-associated periodic inflammatory disorder?

    Science.gov (United States)

    Aldea, Anna; Campistol, Josep M; Arostegui, Juan I; Rius, Josefa; Maso, Montserrat; Vives, Jordi; Yagüe, Jordi

    2004-01-01

    Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurring short attacks of fever and serositis. Secondary AA amyloidosis is the worst complication of the disease and often determines the prognosis. The MEFV gene, on chromosome 16p13.3, is responsible for the disease and around 30 mutations have been reported to date. Colchicine is the standard FMF treatment today, and prevents both attacks and amyloid deposition in 95% of patients. Here we describe a three-generation Spanish kindred with five family members affected by a severe periodic inflammatory disorder associated with renal AA amyloidosis and colchicine unresponsiveness. Clinical diagnosis of definite FMF disease was made based on the Tel-Hashomer criteria set. Genetic analyses revealed that all subjects were heterozygous for the new H478Y MEFV variant, segregating with the disease. In addition, mutations in the TNFRSF1A and CIAS1/PYPAF1/NALP3 genes, related to the dominantly inherited autoinflammatory periodic syndromes, were ruled out. However, the dominant inheritance of the disease, the long fever episodes with a predominant joint involvement, and the resistance to colchicine in these patients raise the question of whether the periodic syndrome seen in this kindred is a true FMF disease with unusual manifestations or rather another MEFV-associated periodic syndrome. We conclude that the new H478Y MEFV mutation is the dominant pathological variant causing the inflammatory periodic syndrome in this kindred and that full-length analyses of the MEFV gene are needed to obtain an adequate diagnosis of patients with clinical suspicion of a hereditary periodic fever syndrome, especially those from non-ancestral populations.

  20. INFLUENCING FACTORS AFFECTING STRESS LEVEL OF FAMILY MEMBERS OF CERVICAL CANCER PATIENTS DURING PERIOPERATIVE PERIOD%宫颈癌围手术期患者家属应激水平影响因素分析

    Institute of Scientific and Technical Information of China (English)

    张慧芳; 颜春英

    2015-01-01

    目的::探讨宫颈癌围手术期患者家属应激水平的影响因素,为制定相关心理干预措施提供理论依据。方法:应用家属应激量表(RSS)、焦虑自评量表(SAS)和抑郁自评量表(SDS)调查90例宫颈癌围手术期患者家属的应激水平和焦虑、抑郁状况,并采用Pearson直线相关分析和Stepwise多元线性回归分析分析影响患者家属应激水平的因素。结果:宫颈癌围手术期患者家属应激反应水平较高,焦虑情况较轻,存在中至重度抑郁。直线相关分析显示,RSS及其三个因子评分与焦虑、抑郁均呈正相关关系(P<0.05)。多元线性回归分析表明,家属的民族、宗教信仰、家庭类型、焦虑、抑郁水平是影响其应激水平的因素(P<0.05)。结论:宫颈癌患者家属在围手术期同样存在严重的心理问题,医护人员应给予切实有效的心理指导,降低患者家属的心理应激水平。%Objective:To investigate the inlfuencing factors affecting stress level of family members of cervical cancer patients during perioperative period and provide theoretical basis for formulating psychological intervention measures. Methods:RSS scale, SAS scale and SDS scale were used to evaluate the stress level, anxiety and depression status of family members of cervical cancer patients during perioperative period; Pearson linear correlation analysis and Stepwise multiple linear regression analysis were applied to analyze the inlfuencing factors affecting stress level of family members of cervical cancer patients.Results:The stress level of family members of cervical cancer patients was higher; the anxiety status was lighter but the depression status was moderate to severe. Linear correlation analysis showed RSS and its’ 3 factors positively correlated with anxiety and depression status. Multiple linear regression analysis showed nation, religion, family type, anxiety level and depression level were

  1. Several Considerations about Carrying Forward and Passing on the Red Family Tradition in the New Period%新时期弘扬和传承红色家风的几点思考

    Institute of Scientific and Technical Information of China (English)

    欧阳慧; 熊轶欣; 廖昔君

    2016-01-01

    The red family tradition is a kind of family style formed by the old-generation proletariat revolution-ists and the outstanding communists of different periods during the long-term historical process of revolution, socialist building,opening-up and reform. It is embodied in the spiritual traits,group consciousness and politi-cal character in ideal and faith,morality and conduct,integrity and self-discipline,kinship and friendship,and so forth,becoming an important component of Chinese communists’spirit and good tradition. To carry forward and pass on the red family tradition and ask the party member leading cadres to hand on the spirit of the red family tradition has become a critical link for comprehensively strengthening the Party ’s self-discipline. We must correctly grasp the kennel of the red family tradition,proactively adapt to the development and change of family structure and interaction among family members in the modern society,and actively establish an effective and transparent evaluation and guarantee mechanism,so as to form a healthy and positive atmosphere in the whole society.%红色家风,是老一辈无产阶级革命家和各个时代的优秀共产党人,在长期革命实践、社会主义建设和改革开放历史进程中形成的家庭风尚,体现在理想信念、道德品行、廉洁自律、亲情友情等方面的精神风貌、群体意识和政治品格,成为中国共产党人精神和优良传统的重要组成部分。弘扬和传承红色家风,引导广大党员领导干部自觉传承红色家风的精神气脉,成为全面从严治党这一系统工程的关键一环。准确把握红色家风的内核要义,主动适应现代社会家庭结构和家庭成员交往方式的发展变化,积极建立高效透明的评价保障机制,从而引领全社会形成健康向上的良好风气。

  2. Randomized, placebo-controlled trials of dichlorphenamide in periodic paralysis.

    Science.gov (United States)

    Sansone, Valeria A; Burge, James; McDermott, Michael P; Smith, Patty C; Herr, Barbara; Tawil, Rabi; Pandya, Shree; Kissel, John; Ciafaloni, Emma; Shieh, Perry; Ralph, Jeffrey W; Amato, Antony; Cannon, Steve C; Trivedi, Jaya; Barohn, Richard; Crum, Brian; Mitsumoto, Hiroshi; Pestronk, Alan; Meola, Giovanni; Conwit, Robin; Hanna, Michael G; Griggs, Robert C

    2016-04-12

    To determine the short-term and long-term effects of dichlorphenamide (DCP) on attack frequency and quality of life in hyperkalemic (HYP) and hypokalemic (HOP) periodic paralysis. Two multicenter randomized, double-blind, placebo-controlled trials lasted 9 weeks (Class I evidence), followed by a 1-year extension phase in which all participants received DCP. Forty-four HOP and 21 HYP participants participated. The primary outcome variable was the average number of attacks per week over the final 8 weeks of the double-blind phase. The median attack rate was lower in HOP participants on DCP than in participants on placebo (0.3 vs 2.4, p = 0.02). The 9-week mean change in the Physical Component Summary score of the Short Form-36 was also better in HOP participants receiving DCP (treatment effect = 7.29 points, 95% confidence interval 2.26 to 12.32, p = 0.006). The median attack rate was also lower in HYP participants on DCP (0.9 vs 4.8) than in participants on placebo, but the difference in median attack rate was not significant (p = 0.10). There were no significant effects of DCP on muscle strength or muscle mass in either trial. The most common adverse events in both trials were paresthesia (47% DCP vs 14% placebo, both trials combined) and confusion (19% DCP vs 7% placebo, both trials combined). DCP is effective in reducing the attack frequency, is safe, and improves quality of life in HOP periodic paralysis. These studies provide Class I evidence that DCP significantly reduces attack frequency in HOP but lacked the precision to support either efficacy or lack of efficacy of DCP in HYP. © 2016 American Academy of Neurology.

  3. Randomized, placebo-controlled trials of dichlorphenamide in periodic paralysis

    Science.gov (United States)

    Burge, James; McDermott, Michael P.; Smith, Patty C.; Herr, Barbara; Tawil, Rabi; Pandya, Shree; Kissel, John; Ciafaloni, Emma; Shieh, Perry; Ralph, Jeffrey W.; Amato, Antony; Cannon, Steve C.; Trivedi, Jaya; Barohn, Richard; Crum, Brian; Mitsumoto, Hiroshi; Pestronk, Alan; Meola, Giovanni; Conwit, Robin; Hanna, Michael G.; Griggs, Robert C.

    2016-01-01

    Objective: To determine the short-term and long-term effects of dichlorphenamide (DCP) on attack frequency and quality of life in hyperkalemic (HYP) and hypokalemic (HOP) periodic paralysis. Methods: Two multicenter randomized, double-blind, placebo-controlled trials lasted 9 weeks (Class I evidence), followed by a 1-year extension phase in which all participants received DCP. Forty-four HOP and 21 HYP participants participated. The primary outcome variable was the average number of attacks per week over the final 8 weeks of the double-blind phase. Results: The median attack rate was lower in HOP participants on DCP than in participants on placebo (0.3 vs 2.4, p = 0.02). The 9-week mean change in the Physical Component Summary score of the Short Form–36 was also better in HOP participants receiving DCP (treatment effect = 7.29 points, 95% confidence interval 2.26 to 12.32, p = 0.006). The median attack rate was also lower in HYP participants on DCP (0.9 vs 4.8) than in participants on placebo, but the difference in median attack rate was not significant (p = 0.10). There were no significant effects of DCP on muscle strength or muscle mass in either trial. The most common adverse events in both trials were paresthesia (47% DCP vs 14% placebo, both trials combined) and confusion (19% DCP vs 7% placebo, both trials combined). Conclusions: DCP is effective in reducing the attack frequency, is safe, and improves quality of life in HOP periodic paralysis. Classification of evidence: These studies provide Class I evidence that DCP significantly reduces attack frequency in HOP but lacked the precision to support either efficacy or lack of efficacy of DCP in HYP. PMID:26865514

  4. Bumetanide prevents transient decreases in muscle force in murine hypokalemic periodic paralysis.

    Science.gov (United States)

    Wu, Fenfen; Mi, Wentao; Cannon, Stephen C

    2013-03-19

    To test the hypothesis that inhibition of the Na-K-2Cl transporter with bumetanide will reduce the susceptibility to decreases in muscle force in a mouse model of hypokalemic periodic paralysis (HypoPP). In vitro contraction tests were performed on soleus muscle isolated from mice with knock-in missense mutations that result in HypoPP (sodium channel NaV1.4-R669H) or hyperkalemic periodic paralysis (HyperPP; sodium channel NaV1.4-M1592V). Bumetanide prevented the development of weakness in 2 mM K(+) and also restored force during an established attack of HypoPP. Stimulation of the Na-K-2Cl transporter via induction of hyperosmolality exacerbated the weakness seen in low K(+) and was also prevented by bumetanide. Bumetanide was more efficacious than acetazolamide for preventing weakness in low K(+) conditions. Decreases in force in HyperPP muscle exposed to 10 mM K(+) were not prevented by treatment with bumetanide. The Na-K-2Cl inhibitor bumetanide was highly effective in preventing attacks of weakness in the NaV1.4-R669H mouse model of HypoPP and should be considered for management of patients with HypoPP due to sodium channel mutations. Dehydration may aggravate HypoPP by stimulating the Na-K-2Cl transporter.

  5. Cui Family Graveyard of the Sui and Tang period at Xiyue Village in Pingshan,Hebei%河北平山县西岳村隋唐崔氏墓

    Institute of Scientific and Technical Information of China (English)

    河北省文物研究所; 平山县博物馆

    2001-01-01

    In April-June 1998, three brick-chambered tombs of the Sui and Tang period were discovered to the north of Xiyue village in Lianghe township, Pingshan county, Hebei. Tomb M1 faces to the south and consists of a tomb-passage, a corridor and a chamber. It yielded a celadon jar and an epitaph. Tomb M2 is unclear in shape and contains an epitaph and 17 remaining funeral objects, such as terra-cotta tomb figurines, domestic animals and other models, tomb-guarding creatures and vessels.Tomb M3 is in the best condition and close to M1 in shape. Its funeral objects include an epitaph and more than 160 terra-cotta tomb figurines, tomb-guarding creatures, domestic animals and other models.The three unearthed epitaphs indicate that M1 belonged to Li Liyi of the Sui period, M2 to Li's husband Cui Zhongfang dying in the Tang period, M3 to Cui Zhongfang's son Cui Dashan who died in the Sui dynasty, and the whole burial ground was the graveyard of Cui, a noble family in Boling.

  6. On Cultural Connotation of Patrilineal Families in Yidi of Ninglang during the Period of Democratic Reform--An example of four families%民主改革前后宁蒗县宜底父系大家庭的文化内涵--以四户家庭情况为例

    Institute of Scientific and Technical Information of China (English)

    何林富

    2014-01-01

    The cardinal form of the family was patrilineal family where Yi Di of Ning Lang before the democratic of reforming. But there was close contacted about big maternal family. And being was a long period of time from the big maternal family to the patrilineal family. It was reflected in the marriage form in around of democratic of reforming. The patrilineal family was rich maternal culture. Because of some reason about historical factor and geographical environment and so on. It was reflected conflict of rights and status about men and women. The men were rights and status more and more powerful. And the women were rights and status more and more feeble. Finally, it was became not only harmonious but also contradiction situation.%在民主改革以前,云南省宁蒗县宜底主要的家庭形式是父系大家庭,但与母系大家庭有着千丝万缕的联系,即处在一个从母系大家庭向父系大家庭过渡的漫长时期。体现在民主改革前后宜底的婚姻家庭形态,由于地理环境、历史因素等各种原因,形成具有浓厚母系文化的父系大家庭。在父系大家庭的背后反映出男性与女性权利地位的冲突中,男性的权利地位的不断巩固,女性为曾经拥有的地位在最终的抵抗,形成宜底父系大家庭和谐而又充满矛盾的局面。

  7. Effect of cinesiotherapy on patients with Parkinson's disease in the hospital - family transition period%运动疗法对医院-家庭过渡期帕金森病患者的影响

    Institute of Scientific and Technical Information of China (English)

    尧慧燕; 石红婷; 董亚贤

    2016-01-01

    Objective:To explore the effect of cinesiotherapy on patients with Parkinson's disease in the hospital - family transition peri-od. Methods:104 patients with Parkinson's disease were randomly divided into the control group(n = 53)and the observation group(n =51). The conventional health education was given to the patients in the control group one day before discharge and the patients in the ob-servation group received cinesiotherapy for 3 months in the hospital - family transition period,the motor function and quality of life of the patients were compared between the two groups respectively on admission and in one and 3 months after discharge. Results:The motor func-tion and quality of life of the patients in the observation group were significantly better than those in the control group after the intervention (P < 0. 05). Conclusion:Implementation of cinesiotherapy is conducive to the improvement of the motor function and quality of life of the patients with Parkinson's disease in the hospital - family transition period.%目的:探讨运动疗法对医院-家庭过渡期帕金森病(PD)患者的影响。方法:将104例 PD 患者随机分为对照组53例和观察组51例,对照组出院前1 d 给予常规出院宣教,观察组接受为期3个月的医院-家庭过渡期运动疗法,比较两组入院时、出院1个月及3个月后运动功能和生活质量。结果:干预后观察组运动功能和生活质量明显优于对照组(P <0.05)。结论:在医院-家庭过渡期对 PD 患者实施运动疗法,有助于改善患者的运动功能和生活质量。

  8. 低钾周期性麻痹家系CACNA1S基因突变的研究%CACNA1S mutation in a Chinese hypokalemic periodic paralysis family

    Institute of Scientific and Technical Information of China (English)

    翟金盛; 李卫巍; 安丽梅; 李科; 周洋; 刘阳; 崔英霞; 李晓军; 夏欣一

    2012-01-01

    目的 低钾周期性麻痹(hypokalemic periodic paralysis,HoKPP)是一种由于离子通道功能异常引起的疾病,呈常染色体显性遗传.文中筛查HoKPP家系患者的基因突变位点,为产前基因诊断提供实验依据.方法 报告1个具有4代18例患者的HoKPP中国家系.提取HoKPP患者、家系中健康人以及100例无血缘正常对照血样中白细胞基因组DNA,应用PCR和DNA测序进行候选基因突变分析,包括骨骼肌二氢嘧啶敏感性钙通道α1亚单位(CACNA1S)基因和骨骼肌钠通道α亚单位基因(SCN4A).结果 分子遗传学研究显示该HoKPP家系所有患者CACNA1S基因外显子30上均存在杂合突变(G3716A),推测导致氨基酸序列改变(R1239H),家系中健康人以及无血缘正常对照组中均未见患者所携带的杂合突变位点(G3716A).结论 CACNA1S基因的R1239H突变是该HoKPP家系发病的分子遗传学基础,可行产前基因诊断预防患儿出生.%Objective Hypokalemic periodic paralysis ( HOKPP ) is an autosomal dominant disorder belonging to a group of muscle diseases involving the abnormal function of ion channels. This study aimed to screen gene mutations in a Chinese HOKPP family for prenatal gene diagnosis. Methods DNA was extracted from the blood samples of 18 affected individuals of a four-generation Chinese HOKPP family, unaffected relatives of the family and 100 normal controls. The disease-causing mutation was analyzed by poly-merase chain reaction ( PCR ) and DNA sequencing of candidate genes, including the al subunit of the dihydropyridine receptor calcium channel gene ( CACNA1S ) and skeletal muscle sodium channel gene ( SCN4A ). Results There was a heterozygous mutation ( G3716 A ) on exon 30 of the CACNA1S gene in all the patients of the family, resulting in a predicted change of amino acid sequence ( R1239H ), but no mutation was found on this site in the other healthy family members and 100 normal controls. Conclusion Mutation of the CACNA1S gene

  9. Mechanisms in hyperkalemic renal tubular acidosis.

    Science.gov (United States)

    Karet, Fiona E

    2009-02-01

    The form of renal tubular acidosis associated with hyperkalemia is usually attributable to real or apparent hypoaldosteronism. It is therefore a common feature in diabetes and a number of other conditions associated with underproduction of renin or aldosterone. In addition, the close relationship between potassium levels and ammonia production dictates that hyperkalemia per se can lead to acidosis. Here I describe the modern relationship between molecular function of the distal portion of the nephron, pathways of ammoniagenesis, and hyperkalemia.

  10. 姓氏、语言、相貌、性情、禁忌、教育——康乾时期燕行使眼中关于满族的几个问题%Family Name, Language, Appearance, Temperament, Taboo and Education --Yan Exercise about the Manchu in the Period of Kangxi and Qianlong Period

    Institute of Scientific and Technical Information of China (English)

    赵兴元

    2012-01-01

    Yan exercise came China and recorded what they observed and heard in the period of Kangxi and Qianlong. This text is valuable information to study the period of Manchu. In the eyes of Yan exercise, Manchu surname was difficult to grasp;Manchu family name was changed into Han surnames in this period. Second, the Manchu language and text once flourished, but it began to be replaced by Han language in the late of Qianlong period. General Manchu was "full length" appearance because of family, there are no uniform characteristics. Comparing with the Han, Manchu is the lack of cultural and soon-real, some of them were arrogant because of their privileges. Manchu bans to abuse dogs, prohibits killing crow and magpie, prohibits sitting west kang. Manchu attaches important to children education through establishment of schools to provide for studying, riding and shooting.%康乾时期,来到中国的燕行使记录了他们的所见所闻。这些文字是研究该时期满族的珍贵资料。在燕行使眼中,一、满族人的姓氏是个很难把握的问题;此时满姓正在向汉字姓改变。二、满语满文曾一度兴盛,但从乾隆晚期开始则出现被汉语言文字替代的迹象。三、一般满族人"丰伟长大",相貌因家族而异,没有统一特征。四、与汉族相比,满族人"少文"而"淳实",有时也因特权而有些霸道。五、满族禁止虐狗,禁止捕杀乌鸦、喜鹊,也禁止坐西炕。六、满族很重视子弟教育,通过设立各种学堂为子弟读书骑射提供条件。

  11. Irregular Periods

    Science.gov (United States)

    ... blood become too low or too high. Some women have irregular periods because their bodies produce too much androgen, which is a hormone that causes increased muscle mass, facial hair, and deepening of the voice in males and ...

  12. Period Pain

    Science.gov (United States)

    ... You may also have other symptoms, such as lower back pain, nausea, diarrhea, and headaches. Period pain is not ... Taking a hot bath Doing relaxation techniques, including yoga and meditation You might also try taking over- ...

  13. Reasons for Scholars and Literators Flourishing in the Pan's Family in the Thirteen-hong Merthants Period of Guangzhou%试论行商潘氏家族人才特盛的原因

    Institute of Scientific and Technical Information of China (English)

    郭展鹏

    2012-01-01

    In 1757, Guangzhou became the only city that could have foreign trade in China. In other words, all foreign transactions between China and other countries must be made via Guangzhou. At that time, the Cantonese economy was prosperous and the Thirteen-hong Merchants was the most important foreign business trade center in Guangzhou. The Thirteen-hong Merchants in Canton (Guangzhou) was set up as a professional trading institution that dealing with foreign trade in Qing dynasty. The Pan' s Family was one of the successful firms in the Thirteen-hong Merchants. The Pan' s enterprise operated as an import and export agent on a commission basis. They were doing well in foreign trade business. In addition, the Pan' s also had great contribution to the Cantonese cultural development. A great number of their descendants became scholars and literators. In this paper, the author wants to find out reasons why lots of talented people in Guangzhou at that period were mostly from the Pan' s Family.%广州“一口通商”时期,十三行行商名声享誉全国,尤以潘、卢、伍、叶四大家族为盛。潘家除了在商业上取得巨大成就外,在文化事业上也贡献良多,后代文人辈出。文章试从制度、社会、经济及家族观念几个方面考察,试图找出潘氏家族屡出人才的原因。

  14. Periodic behaviors

    CERN Document Server

    Napp, Diego; Shankar, Shiva

    2010-01-01

    This paper studies behaviors that are defined on a torus, or equivalently, behaviors defined in spaces of periodic functions, and establishes their basic properties analogous to classical results of Malgrange, Palamodov, Oberst et al. for behaviors on R^n. These properties - in particular the Nullstellensatz describing the Willems closure - are closely related to integral and rational points on affine algebraic varieties.

  15. PERIODIC BEHAVIORS

    NARCIS (Netherlands)

    Napp, Diego; Put, Marius van der; Shankar, Shiva

    2010-01-01

    This paper studies behaviors that are defined on a torus, or equivalently, behaviors defined in spaces of periodic functions, and establishes their basic properties analogous to classical results of Malgrange, Palamodov, Oberst et al. for behaviors on R(n). These properties-in particular the Nullste

  16. Assessing postpartum family functioning.

    Science.gov (United States)

    Midmer, D; Talbot, Y

    1988-09-01

    The birth of a child requires adaptation and reorganization within the family system in order to accommodate the new family member and to allow the family to continue in its psychosocial development. Knowledge of the normative and transitional changes required at this stage of family life will enhance family practitioners' understanding of some of the common concerns and complaints related to them by various family members during the postpartum period. The Family FIRO model represents a helpful conceptual framework to increase the family physician's understanding of the issues of inclusion, control, and intimacy that are highlighted during the transition to parenthood. The authors briefly present this model and discuss its application to postpartum adjustment and its implications for health-care professionals.

  17. Parálisis periódica hipocalémica familiar (PPHF: reporte de un caso y revisión del tema Periodic familial hypoaklemic paralysis: report of a case and review of the literature

    Directory of Open Access Journals (Sweden)

    José William Cornejo Ochoa

    2002-02-01

    Full Text Available La parálisis periódica hipocalémica familiar es una enfermedad que pertenece al grupo de las canalopatías. Consiste en la presentación de episodios de parálisis muscular progresivos en intensidad y frecuencia acompañada de hipocalemia. dos mutaciones explican la presencia de la enfermedad, la CACNA1S y la SCN4A, que afectan los canales de potasio mediados por calcio y los canales de sodio, respectivamente. Lo anterior repercute en la función de los canales para el potasio mediados por voltaje, llevando a una hipocalemia extracelular sostenida produciendo despolarización continua con parálisis. Los desencadenantes son el ejercicio, los carbohidratos, el frío, el estrés, entre otros, y las pruebas de provocación clínica se realizan con insulina, glucosa y ejercicio. La enfermedad se puede prevenir evitando estos factores y tiene alguna respuesta al tratamiento con acetazolamida. Se presenta el caso clínico de un niño que cumple con los criterios clínicos de paresia asociada a hipocalemia desencadenada por carbohidratos. Periodic hypokalemic familial paralysis (PHFP is a channel-mediated disease. Increasing focal or generalized muscular paralytic episodes are associated with low serum potassium levels. Two point mutations are described, CACNA1S and SCN4A. These mutations affect calcium and sodium mediated potassium channels. A continuous depolarization with low extracelular potassium promotes hypotonia. Known stressors are excercise, cold, stress and high carbohydrate intake. Control of stressors and azetazolamyde are the main treatment options. We describe one patient with clinical and laboratory characteristics of PHFP.

  18. Dissolved families

    DEFF Research Database (Denmark)

    Christoffersen, Mogens

    The situation in the family preceding a family separation is studied here, to identify risk factors for family dissolution. Information registers covering prospective statistics about health aspects, demographic variables, family violence, self-destructive behaviour, unemployment, and the spousal...

  19. Dissolved families

    DEFF Research Database (Denmark)

    Christoffersen, Mogens

    The situation in the family preceding a family separation is studied here, to identify risk factors for family dissolution. Information registers covering prospective statistics about health aspects, demographic variables, family violence, self-destructive behaviour, unemployment, and the spousal...

  20. Family Life

    Science.gov (United States)

    ... With Family and Friends > Family Life Request Permissions Family Life Approved by the Cancer.Net Editorial Board , ... your outlook on the future. Friends and adult family members The effects of cancer on your relationships ...

  1. 5. Natural Family Planning

    African Journals Online (AJOL)

    Sitwala

    influencing utilization of Natural Family Planning .... Because of the potential benefits of FP, it is important to any community ... Data was collected over a period of 4 months after approval ..... The DHMT to educate parents on the importance of.

  2. Numerical bifurcation of Hamiltonian relative periodic orbits

    DEFF Research Database (Denmark)

    Wulff, Claudia; Schilder, Frank

    2009-01-01

    that the family of choreographies rotating around the $e^2$-axis bifurcates to the family of rotating choreographies that connects to the Lagrange relative equilibrium. Moreover, we compute several relative period-doubling bifurcations and a turning point of the family of planar rotating choreographies, which...... to symmetry-breaking/symmetry-increasing pitchfork bifurcations or to period-doubling/period-halving bifurcations. We apply our methods to the family of rotating choreographies which bifurcate from the famous figure eight solution of the three-body problem as angular momentum is varied. We find...

  3. Period-doubling cascades galore

    OpenAIRE

    Sander, Evelyn; Yorke, James A.

    2009-01-01

    The appearance of numerous period-doubling cascades is among the most prominent features of {\\bf parametrized maps}, that is, smooth one-parameter families of maps $F:R \\times {\\mathfrak M} \\to {\\mathfrak M}$, where ${\\mathfrak M}$ is a smooth locally compact manifold without boundary, typically $R^N$. Each cascade has infinitely many period-doubling bifurcations, and it is typical to observe -- such as in all the examples we investigate here -- that whenever there are any cascades, there are...

  4. PERIODIC COMPLEMENTARY BINARY SEQUENCE PAIRS

    Institute of Scientific and Technical Information of China (English)

    XuChengqian; ZhaoXiaoqun

    2002-01-01

    A new set of binary sequences-Periodic Complementary Binary Sequence Pair (PCSP)is proposed .A new class of block design-Difference Family Pair (DFP)is also proposed .The relationship between PCSP and DFP,the properties and exising conditions of PCSP and the recursive constructions for PCSP are given.

  5. PERIODIC COMPLEMENTARY BINARY SEQUENCE PAIRS

    Institute of Scientific and Technical Information of China (English)

    Xu Chengqian; Zhao Xiaoqun

    2002-01-01

    A new set of binary sequences-Periodic Complementary Binary Sequence Pair (PCSP) is proposed. A new class of block design-Difference Family Pair (DFP) is also proposed.The relationship between PCSP and DFP, the properties and existing conditions of PCSP and the recursive constructions for PCSP are given.

  6. Family Therapy

    Science.gov (United States)

    ... may be credentialed by the American Association for Marriage and Family Therapy (AAMFT). Family therapy is often short term. ... challenging situations in a more effective way. References Marriage and family therapists: The friendly mental health professionals. American Association ...

  7. Familial hypertriglyceridemia

    Science.gov (United States)

    ... page: //medlineplus.gov/ency/article/000397.htm Familial hypertriglyceridemia To use the sharing features on this page, please enable JavaScript. Familial hypertriglyceridemia is a common disorder passed down through families. ...

  8. Family Meals

    Science.gov (United States)

    ... Teaching Kids to Be Smart About Social Media Family Meals KidsHealth > For Parents > Family Meals Print A ... even more important as kids get older. Making Family Meals Happen It can be a big challenge ...

  9. Family Arguments

    Science.gov (United States)

    ... Spread the Word Shop AAP Find a Pediatrician Family Life Medical Home Family Dynamics Adoption & Foster Care ... Life Listen Español Text Size Email Print Share Family Arguments Page Content Article Body We seem to ...

  10. Family History

    Science.gov (United States)

    Your family history includes health information about you and your close relatives. Families have many factors in common, including their genes, ... as heart disease, stroke, and cancer. Having a family member with a disease raises your risk, but ...

  11. Family control and financing decisions

    NARCIS (Netherlands)

    Croci, Ettore; Doukas, John A.; Gonenc, Halit

    2011-01-01

    This study uses a comprehensive European dataset to investigate the role of family control in corporate financing decisions during the period 1998-2008. We find that family firms have a preference for debt financing, a non-control-diluting security, and are more reluctant than non-family firms to ra

  12. INFLUENCING OF FAMILY AFFECTION ON MENOPAUSAL SYMPTOMS AND DEPRESSION OF PERIMENOPAUSE PERIOD OF RURAL AREAS WOMEN%婚姻家庭对农村妇女围绝经期综合症及抑郁的影响

    Institute of Scientific and Technical Information of China (English)

    赵晓军; 陈长香; 付丽; 马素慧; 岳静玲

    2011-01-01

    [Objective] To understand Symptoms of menopausal and depression and their influencing factors of rural areas women in their perimenopause period, so as to provide countermeasures. [Methods] With stratified random sampling, aged 45-55 years were selected from Heilongjiang, Hebei, shan'xi, Shanxi and Henan province. Basic information questionnaire, Kupperm an score sheet, self-rating depress ion scale (SDS) and AGAR were adopted. [Results] Among the 884 subjects, 41.18% of them had depression. 15.05% of them were suspected to have depression, and 26.13% of them were diagnosed with depression. 79.30% reported premenopausal syndromes The rate of depression in women with long-time work and high pressures, poor quality of marriage, illness partner, divorced, low family income, big children burden; , addicted to cigarette or alcohol, bland diet and poor sleeping quality was higher than that of Perimenopausal Women (P < 0.05 or P < 0.01) . The women with perimenopausal syndrome had significant higher rate of depression. [Conclusion] The incidence of menopausal symptoms and perimenopausal depression were higher and the influencing factors were complex, the problems should to be attach importance of interstitial texture.%[目的]了解农村妇女围绝经期综合症、抑郁症状及其影响因素,为采取应对措施提供依据. [方法]2010年1~3月选取黑龙江,河北,陕西,山西,河南5省45 ~55岁妇女,采用自编一般情况调查表、更年期Kupperman评分量表、抑郁评分量表(CES - D)进行调查. [结果]884名调查对象中,抑郁总发生率为41.18%,可疑抑郁者15.05%,肯定抑郁者26.13%;围绝经期综合征发生率为79.30%;婚姻质量差、伴侣患病、离异、全家经济收入低、子女负担重者等发生围绝经期抑郁症的频率或程度高,差异有统计学意义(P< 0.05或P<0.01);且围绝经期综合征程度超重,抑郁发生率越高,差异有统计学意义(P<0.01).[结论]农村妇女

  13. Research on the Transformation of Paradigm and the Consciousness of Problem:Chinese Rural Marriage and Family in the Collectivization Period%范式转换与问题意识:集体化时期中国农村婚姻家庭研究述评

    Institute of Scientific and Technical Information of China (English)

    李飞龙

    2012-01-01

    近些年,集体化时期的农村婚姻家庭研究逐渐成为当代史研究的热点之一,这很大程度上促进了乡村史、日常生活史、婚姻家庭等诸多研究领域的进展。范式转换和问题意识成为集体化时期中国农村婚姻家庭研究重点考察的对象,研究者将婚姻家庭研究与集体化时期的国家权力、社会变迁、集体化生产紧密联系起来,扩展了婚姻家庭史的研究领域与视角。不过,需要指出的是,在已有的研究成果中,范式研究的单一和问题意识的薄弱等问题仍十分突出。%In recent years,research on the rural marriage and family in the collectivization period has gradually become one of hot topics in the contemporary history,which has largely promoted the progress in researches of the rural history,history of daily life,marriage and family.The transformation of paradigm and consciousness of problem are becoming the focus of the object in Chinese rural marriage and family research of the collectivization period,in which researchers connect marriage and family with national power,social changes and collectivize production of that period,extending the study field and perspectives of marriage and family.However,it is still very prominent that the single paradigm and the weak consciousness of problem are common existing in the research results.

  14. Family Privilege

    Science.gov (United States)

    Seita, John R.

    2014-01-01

    Family privilege is defined as "strengths and supports gained through primary caring relationships." A generation ago, the typical family included two parents and a bevy of kids living under one roof. Now, every variation of blended caregiving qualifies as family. But over the long arc of human history, a real family was a…

  15. Family Privilege

    Science.gov (United States)

    Seita, John R.

    2014-01-01

    Family privilege is defined as "strengths and supports gained through primary caring relationships." A generation ago, the typical family included two parents and a bevy of kids living under one roof. Now, every variation of blended caregiving qualifies as family. But over the long arc of human history, a real family was a…

  16. Science.gov (United States)

    Lehmann-Horn, F.; Orth, M.; Kuhn, M.; Jurkat-Rott, K.

    2011-01-01

    We report a 4-generation Turkish family with 10 affected members presenting with myotonia and potassium- and exerciseinduced paralytic attacks. The clinical presentation was neither typical for the chloride channel myotonias Thomsen and Becker nor for the separate sodium channel myotonia entities potassiumaggravated myotonia, paramyotonia congenita, and hyperkalemic periodic paralysis. It is best described by a combination of potassium-aggravated myotonia and hyperkalemic periodic paralysis. We excluded exonic chloride channel mutations including CLCN1 exon deletion/duplication by MLPA. Instead we identified a novel p.N440K sodium channel mutation that is located at the inner end of segment S6 of repeat I. We discuss the genotype phenotype relation. PMID:22106717

  17. The effect of community maternal and newborn health family meetings on type of birth attendant and completeness of maternal and newborn care received during birth and the early postnatal period in rural Ethiopia.

    Science.gov (United States)

    Barry, Danika; Frew, Aynalem Hailemichael; Mohammed, Hajira; Desta, Binyam Fekadu; Tadesse, Lelisse; Aklilu, Yeshiwork; Biadgo, Abera; Buffington, Sandra Tebben; Sibley, Lynn M

    2014-01-01

    Maternal and newborn deaths occur predominantly in low-resource settings. Community-based packages of evidence-based interventions and skilled birth attendance can reduce these deaths. The Maternal and Newborn Health in Ethiopia Partnership (MaNHEP) used community-level health workers to conduct prenatal Community Maternal and Newborn Health family meetings to build skills and care-seeking behaviors among pregnant women and family caregivers. Baseline and endline surveys provided data on a random sample of women with a birth in the prior year. An intention-to-treat analysis, plausible net effect calculation, and dose-response analysis examined increases in completeness of care (mean percentage of 17 maternal and newborn health care elements performed) over time and by meeting participation. Regression models assessed the relationship between meeting participation, completeness of care, and use of skilled providers or health extension workers for birth care-controlling for sociodemographic and health service utilization factors. A 151% increase in care completeness occurred from baseline to endline. At endline, women who participated in 2 or more meetings had more complete care than women who participated in fewer than 2 meetings (89% vs 76% of care elements; P < .001). A positive dose-response relationship existed between the number of meetings attended and greater care completeness (P < .001). Women with any antenatal care were nearly 3 times more likely to have used a skilled provider or health extension worker for birth care. Women who had additionally attended 2 or more meetings with family members were over 5 times as likely to have used these providers, compared to women without antenatal care and who attended fewer than 2 meetings (odds ratio, 5.19; 95% confidence interval, 2.88-9.36; P < .001). MaNHEP's family meetings complemented routine antenatal care by engaging women and family caregivers in self-care and care-seeking, resulting in greater completeness

  18. Food preferences during complementary feeding period among ...

    African Journals Online (AJOL)

    Food preferences during complementary feeding period among nursing mothers ... transition between breastfeeding and family diets being mostly implicated. ... of nursing mothers attending the immunization clinic at a secondary health care ...

  19. Periodic words connected with the Fibonacci words

    Directory of Open Access Journals (Sweden)

    G. M. Barabash

    2016-06-01

    Full Text Available In this paper we introduce two families of periodic words (FLP-words of type 1 and FLP-words of type 2 that are connected with the Fibonacci words and investigated their properties.

  20. Dongchang Qian Family and Spectrum Continued Practice During the Historical Period:On the Value of Historical Materials of Genealogy%历史时期东昌钱族与谱续实践--兼谈族谱之史料研究价值

    Institute of Scientific and Technical Information of China (English)

    周嘉

    2016-01-01

    The Dongchang Qian clan was the descendant of Qianliu who was the king of the Wu and Yue country during the Five Dynasties.Ancestor began to settle down Dongchang in the early Ming Dynasty, rooted in western Shandong Province to thrive,and by clan self-construction,therefore abandoned after a giant clan.The newly founded Qian genealogy which being compiled during Qianlong period recorded the surname reproduction,family lineage,population changes,residence migration,marital status,and the status,role and achievements of the family members who had obtained from the political,economic and cultural life.It reflects directly the Dongchang Qian family archives during the historical period.So it is of great significance.Firstly,the article elaborates the history of Dongchang Qian family,and then focus on the analysis of its own building family genealogy compilation practices,and finally explore the historical value of the spectrum.%东昌钱族为五代时期吴越国王钱镠后裔,始迁祖自明初随军北征落籍此地繁衍生息。乾隆年间所修族谱《钱氏世传宗谱》,记述东昌钱族源流、世代繁衍、人口变动、婚姻网络与科举仕宦等情况,成为反映历史时期钱族在东昌发展脉络的家族档案资料。该谱具有重要的研究意义,既能弥补文献记载之不足,又能为区域社会文化研究提供“文本”史料。

  1. Familial gigantism

    NARCIS (Netherlands)

    W.W. de Herder (Wouter)

    2012-01-01

    textabstractFamilial GH-secreting tumors are seen in association with three separate hereditary clinical syndromes: multiple endocrine neoplasia type 1, Carney complex, and familial isolated pituitary adenomas.

  2. Familial gigantism

    Directory of Open Access Journals (Sweden)

    Wouter W. de Herder

    2012-01-01

    Full Text Available Familial GH-secreting tumors are seen in association with three separate hereditary clinical syndromes: multiple endocrine neoplasia type 1, Carney complex, and familial isolated pituitary adenomas.

  3. Familial dermographism.

    Science.gov (United States)

    Jedele, K B; Michels, V V

    1991-05-01

    Urticaria in response to various physical stimuli has been reported in sporadic and familial patterns. The most common of these physical urticarias, dermographism, is a localized urticarial response to stroking or scratching of the skin and has not been reported previously to be familial. A four-generation family with dermographism, probably inherited as an autosomal dominant trait, is presented along with a discussion of sporadic dermographism and other types of familial physical urticarias.

  4. Excavation of the Zhu Family Graveyard of the Former Qin Period in Wenlin Neighborhood of Xianyang City, Shaanxi%陕西咸阳市文林小区前秦朱氏家族墓的发掘

    Institute of Scientific and Technical Information of China (English)

    咸阳市文物考古研究所

    2005-01-01

    In 1999, the Xianyang Municipal Institute of Cultural Relics and Archaeology carried out excavation in an ancient cemetery within Wenlin Neighborhood, and revealed there 114 tombs, including 9 burials of the Former Qin period. These Former Qin graves are the same in structure, each consisting of a tomb passage, a corridor and a chamber. The corpses are buried in wooden coffins. Their funeral objects consist of pottery, bronzes, silver ware, iron artifacts, etc., numbering 167. The pottery includes male and female tomb-figurines, models of ox carts, horses, pigs, dogs, chickens, cooking stoves, wells, mills, granaries and treadle-operated tilt hammers for hulling rice, jars, bowls, small pots and discs. The bronzes embrace basins, bowls, mirrors, bracelets, hairpins, finger-rings, small bells, dui butt-end pieces and crossbows. The iron artifacts belong to the mirror, pick and scissors, and the silver ware includes hairpins, finger-rings and bracelets. In addition, there are gold hairpins, jade pieces, clay chickens, wuzhu and yanhuan coins, and the dated brick epitaphs coming from four tombs. Definite in date, these graves provide important material for studying the tomb shape and burial custom of the Former Qin period and the history of the Sixteen Kingdoms in the Guanzhong region. Moreover, they have great value to establishing the chronological sequence of burials in this region during the Han, Wei and Sixteen Kingdoms periods.

  5. Preliminary Study on the Composing Contents and Stylistic Innovations of Xiuning--- Based Fan Family's Genealogy in Wanli Period%万历《休宁范氏宗谱》编纂内容与体例初探

    Institute of Scientific and Technical Information of China (English)

    吕春阳

    2016-01-01

    徽州地区宗族社会历来十分重视纂修家谱,《休宁范氏宗谱》具有重视史实考证、注重家谱的教化功能特点,同时对徽州家谱体例结构的研究使得该谱成为一部具有代表性的家谱,其创立的家谱编修体例影响了后世两百多年徽州地区的修谱体例,对梳理明代徽州家谱体例的发展脉络,全面展现明代徽州家谱体例的继承和发展状况具有十分重要的作用。%Huizhou's lineage society always pays a lot of attention to composing genealogical trees .The Xiuning - based Fan Family's Genealogical Tree was featured in composition with the emphasis on textual research about history facts and on the edification effect of the genealogical tree .Meanwhile ,studies on the stylistic structure and starting - ending time of genealogical trees in Huizhou region have made the Fan family's genealogical tree a very representative one .This genealogical tree created a composing style that influenced later styles of genealogical tree in Huizhou for two hundred years ,so it has very important significance in terms of sorting out the development process of the styles of genealogical tree in Ming Dynasty and presenting an all - round status of the inheritance and development of the styles of genealogical trees in Huizhou region .

  6. Familial risk factors in autism.

    Science.gov (United States)

    Brimacombe, Michael; Xue Ming; Parikh, Amisha

    2007-05-01

    Familial history risk factors in relation to autism were examined in a cohort of 164 autistic children referred to The Autism Center at New Jersey Medical School-University of Medicine and Dentistry of New Jersey, Newark, over a 2-year period (2001-2003). Information related to familial history was obtained from each family and reviewed by a clinician. It is shown that these families carry a higher overall burden of psychiatric and developmental illnesses compared to reported national levels. These families also carry a relatively high incidence of medical disorders, independently of developmental and psychiatric disorders. This work supports the underlying presence of genetic factors in the etiology of autism.

  7. 择期手术患者家属候手术期焦虑、抑郁情绪的调查分析%Survey on Anxiety and Depression Status in Family Numbers of Patients Undergoing Selective Operation During the Waiting Period of Operation

    Institute of Scientific and Technical Information of China (English)

    刘成辉

    2011-01-01

    [Objective]To understand the anxiety and depression status in family numbers of patients un dergoing selective operation during the waiting period of operation, and to explore the related factors in order to providing the evidence for clinical care.[Methods]The family numbers of 50 patients undergoing selective operation in our hospital were investigated by using self-rating depression scale(SDS) and self-rating anxiety scale(SAS).[Results]The maximum, minimum and mean value of SAS of family numbers of operation pa tients in this group were 70, 25 and 25.34±9.35, respectively, while those of SDS were 69, 25 and 44.34±9.86, respectively.The statistical analysis showed there were significant differences in anxiety and depression of patients' family numbers among different source of medical cost, the relation with the patients, home address and illness degree of patients and age of family numbers, etc( P <0.05).[Conclusion]The anxious level in family numbers of operation patients during the waiting period of operation is high.Different source of reed ical cost, the relation with patients, home address, the illness degree of patients and the age of family numbers are the main factors influencing the anxious level of family numbers of patients undergoing selective operation.Besides nursing staff take the care and treatment for the patients, they should concern with the psychological condition of patients' family numbers and construct good family support system in order to promote the patients recovery.%[目的]了解和分析手术患者家属候手术期的焦虑与抑郁情绪状况.[方法]采用抑郁自评量表(SDS)和焦虑自评量表(SAS)对本院50例择期手术患者家属进行调查分析.[结果]本组手术患者家属SAS最大值70分,最小值25分,平均值(25.34±9.35)分,SDS最大值69分,最小值25分,平均值(44.34±9.86)分.医疗费用来源、与患者的关系、家庭住址、病情程度、家属年龄差异等的焦

  8. 先秦时期我国女性在婚姻家庭中的地位探析%The Status of Women in Marriage and Family in the Pre -Qin Period

    Institute of Scientific and Technical Information of China (English)

    汪莹

    2016-01-01

    从西周封建礼制的开始创建到春秋战国时期的朝代更替,女性受到了封建礼教的严重束缚和摧残,在婚姻中的地位不断跌落,从恋爱中的自由者,到婚姻的附属品,再到政治的牺牲品,直到妇权彻底丧失,最终成为了男性的附属品。%From the begin of Western Zhou Dynasty to the Spring and Autumn Period and the Warring States Period,women are severely fettered by the feudal ethical code and trample upon.They have plummeted in the position in marriage,from the love of freedom,to the marriage of accessories,and then to the victim of a political until women's rights to lose,which eventually became the accessories of men.

  9. Family Polymorphism

    DEFF Research Database (Denmark)

    Ernst, Erik

    2001-01-01

    safety and flexibility at the level of multi-object systems. We are granted the flexibility of using different families of kinds of objects, and we are guaranteed the safety of the combination. This paper highlights the inability of traditional polymorphism to handle multiple objects, and presents family...... polymorphism as a way to overcome this problem. Family polymorphism has been implemented in the programming language gbeta, a generalized version of Beta, and the source code of this implementation is available under GPL....

  10. My Family

    Institute of Scientific and Technical Information of China (English)

    2012-01-01

    Everyone has a family.We live in it and feel very warm.There are three persons in my family,my mother,father and I.We live together very happily and there are many interesting stories about my family. My father is a hard-working man.He works as a doctor.He always tries his best to help every,patient and make patients comfortable.But sonetimes he works so hard

  11. Family Polymorphism

    DEFF Research Database (Denmark)

    Ernst, Erik

    2001-01-01

    safety and flexibility at the level of multi-object systems. We are granted the flexibility of using different families of kinds of objects, and we are guaranteed the safety of the combination. This paper highlights the inability of traditional polymorphism to handle multiple objects, and presents family...... polymorphism as a way to overcome this problem. Family polymorphism has been implemented in the programming language gbeta, a generalized version of Beta, and the source code of this implementation is available under GPL....

  12. International Perspectives on Family Violence.

    Science.gov (United States)

    Gelles, Richard J.; Cornell, Claire Pedrick

    This paper reviews a sampling of the literature on child abuse, spouse abuse, and family violence from around the world. The 72 books, journal articles, newspaper articles, and legislative reports which are included cover the period 1960-1981. A section on where family violence research has been conducted includes studies on child abuse and…

  13. Family literacy

    DEFF Research Database (Denmark)

    Sehested, Caroline

    2012-01-01

    I Projekt familielæsning, der er et samarbejde mellem Nationalt Videncenter for Læsning og Hillerød Bibliotek, arbejder vi med at få kontakt til de familier, som biblioteket ellers aldrig ser som brugere og dermed også de børn, der vokser op i familier, for hvem bøger og oplæsningssituationer ikke...... er en selvfølgelig del af barndommen. Det, vi vil undersøge og ønsker at være med til at udvikle hos disse familier, er det, man kan kalde family literacy....

  14. Study of a family that overcomes poverty issues: family resilience?

    Directory of Open Access Journals (Sweden)

    María Ángela Mattar Yunes

    2015-09-01

    Full Text Available Generally, researches with families focus the difficulties and the negative aspects of family life by bringing up their maladjustments and failures. The interest in family resilience contributes to change this logic by demonstrating the healthy aspects of the family world. Nevertheless, the term resilience presents ideological controversies which are more severe when the discussion is about families and poverty. In order to diminish these contradictions this study adopted a systemic concept of resilience which refers to “those processes that make possible to overcome adversities”. A case study was realized with a low income family who lived in a “very poor” neighborhood in the deep south of Brazil. The methodological strategies to the formal investigation of the family were: life history of the family using the principles of reflexive interview, genograms and data analyses through the approach of the grounded theory. The results showed that the family lived a number of risk experiences such as adoption, privation of basic needs, migration and diseases. Among the indicators of their abilities of “overcoming adversities”, emerged the belief system as the core of the discourses. The family showed that they value the interpersonal relationships through intra and extra familiar interactions based in the patterns of help, learning, affection and solidarity. During the crisis the family gives meaning to the difficulties in order to maintaining the situation controlled through cohesion, open communication, mutual respect and getting support of the extended family/ social network. The pos-adversity period is perceived as benefic and transforming as the family feels stronger and with feelings of solidarity, which is a mark of this family. Their attitude in relation to the neighborhood is active in the sense of promoting the welfare of other families who live in the same social address. Would those above identified processes be adequate to

  15. Small Families

    Science.gov (United States)

    ... easier for both parents to combine careers with family life. The general stress level is lower because there often are fewer ... can help replace the missing ties. For many families, religious congregational ... such as youth and neighborhood activity centers also can fulfill these ...

  16. FAMILY PLATYSTOMATIDAE.

    Science.gov (United States)

    Wendt, Lisiane Dilli

    2016-06-14

    Platystomatidae (Signal Flies) are one of the largest families of Tephritoidea, with about 1200 species and four subfamilies, worldwide distributed. However, Platystomatidae are not well represented in the New World, and in the Neotropical Region only four genera and 26 species, belonging to Platystomatinae, are recorded. The family is a group understudied in Colombia and only one species is recorded to the country.

  17. An Introduction to the Periodic Law for General Chemistry Students.

    Science.gov (United States)

    Schlenker, Richard M.

    A brief introduction to the periodic table of the elements is presented. The periodic relationships are demonstrated through the use of seven tables which include a breakdown of the periodic table by groups or families, valence ring electron populations by period, electronic configurations of the elements, electron configurations of the elements…

  18. Periodic orbits for three and four co-orbital bodies

    Science.gov (United States)

    Verrier, P. E.; McInnes, C. R.

    2014-08-01

    We investigate the natural families of periodic orbits associated with the equilibrium configurations of the planar-restricted 1 + n-body problem for the case 2 ≤ n ≤ 4 equal-mass satellites. Such periodic orbits can be used to model both trojan exoplanetary systems and parking orbits for captured asteroids within the Solar system. For n = 2, there are two families of periodic orbits associated with the equilibria of the system: the well-known horseshoe and tadpole orbits. For n = 3, there are three families that emanate from the equilibrium configurations of the satellites, while for n = 4, there are six such families as well as numerous additional connecting families. The families of periodic orbits are all of the horseshoe or tadpole type, and several have regions of neutral linear stability.

  19. Periodic orbits for 3 and 4 co-orbital bodies

    CERN Document Server

    Verrier, Patricia

    2014-01-01

    We investigate the natural families of periodic orbits associated with the equilibrium configurations of the the planar restricted $1+n$ body problem for the case $2\\leq n \\leq 4$ equal mass satellites. Such periodic orbits can be used to model both trojan exoplanetary systems and parking orbits for captured asteroids within the solar system. For $n=2$ there are two families of periodic orbits associated with the equilibria of the system: the well known horseshoe and tadpole orbits. For $n=3$ there are three families that emanate from the equilibrium configurations of the satellites, while for $n=4$ there are six such families as well as numerous additional connecting families. The families of periodic orbits are all of the horseshoe or tadpole type, and several have regions of neutral linear stability.

  20. Normokalemic and hypokalemic periodic paralysis coexist in a family with a novel R1129Q mutation in the SCN4A gene%骨骼肌钠通道α1亚基基因R1129Q新突变导致正常钾和低钾性周期性瘫痪一家系

    Institute of Scientific and Technical Information of China (English)

    洪道俊; 栾兴华; 郑日亮; 陈彬; 张巍; 王朝霞; 袁云

    2009-01-01

    目的 报道1个骨骼肌钠通道α1亚基(SCN4A)基因新突变导致的正常钾和低钾性周期性瘫痪家系的临床和病理改变特点.方法 本家系为常染色体显性遗传,共有9例患者,男性4例,女性5例,发病年龄7~25岁.5例患者为正常钾性周期性瘫痪,其中4例伴随肌强直症状;3例患者为低钾性周期性瘫痪;1例发作时血钾浓度不详.对先证者进行左肱二头肌活体组织检查.先证者和7例家系患者、3名无症状家系成员以及50名健康人行SCN4A基因测序.结果 先证者的肌纤维出现轻度肥大和萎缩,伴随核内移和肌纤维内空泡,部分肌纤维内氧化酶分布异常.所有患者均存在SCN4A基因的R1129Q突变,3名无症状家系成员以及50名健康对照无此突变.结论 SCN4A基因R1129Q新突变在同一家系内可以导致低血钾性和正常血钾性周期性瘫痪共存.%Objective To describe the clinical and myopathological phenotype of periodic paralysis in a family associated with a novel mutation in the skeletal muscle sodium channel a subunit (SCN4A) gene.Methods Nine patients in a family presented periodic paralysis in an inheritance pattern of autosomal dominant.Except for 1 deceased patient,3 affected individualshad hypokalemic periodic paralysis and 5 showed normokalemic periodic paralysis.Among the patients with normakalemia,4 had muscle stiffness and myalgia;3 showed myotonia discharges in electromyogram explorations,and 1 had worsened weakness after potassium administration.A muscle biopsy was performed in the proband.SCN4A gene mutation screening was carried out in 8 patients.3 asymptomatic family members and 50 health controls.Results Muscle biopsy indicated vacuolar myopathy with abnormal distribution of oxidative enzyme activity.Genetic analysis revealed novel heterozygous missense mutation G3386A in exon 18 of SCN4A gene.The mutation substituted an arginine for a glutamine at the amino acid site1129(R1129Q

  1. Family Disruptions

    Science.gov (United States)

    ... stay angry, or avoid fights altogether? Your children model themselves on you. Departures and Returns Do you or your spouse frequently travel on business? These can be disruptive times for your child and for the family ...

  2. Familial hypercholesterolemia

    Science.gov (United States)

    ... hypercholesterolemia or early heart attacks High level of LDL cholesterol in either or both parents People from families ... called fibroblasts to see how the body absorbs LDL cholesterol Genetic test for the defect associated with this ...

  3. Family matters

    DEFF Research Database (Denmark)

    Kieffer-Kristensen, Rikke; Siersma, Volkert Dirk; Teasdale, Thomas William

    2013-01-01

    OBJECTIVES: To relate illness and family factors to emotional and behavioural problems in school-age children (7–14 years old) of parents with acquired brain injury and their healthy spouses. PARTICIPANTS, MATERIALS/METHODS: Members of 35 families in which a parent had been diagnosed with acquired...... brain injury participated. Family and brain injury characteristics were reported by the ill and healthy parents. Children self-reported post-traumatic stress symptoms (PSS) using the Child Impact of Events revised (CRIES). Emotional and behavioural problems among the children were also identified...... by the parents using the Achenbach’s Child Behaviour Checklist (CBCL). RESULTS: The family stress variables relating to the healthy spouse in all six comparisons were significant (p

  4. Families in the Military

    Science.gov (United States)

    ... Contents Facts For Families Guide - View by Topic Chinese Facts for Families Guide Facts For Families Guide - Search Spanish Facts for Families Guide Facts for Families - Vietnamese Military Families No. 88; updated March 2017 Global conflict ...

  5. Inverse periodic shadowing properties

    CERN Document Server

    Osipov, Alexey V

    2011-01-01

    We consider inverse periodic shadowing properties of discrete dynamical systems generated by diffeomorphisms of closed smooth manifolds. We show that the $C^1$-interior of the set of all diffeomorphisms having so-called inverse periodic shadowing property coincides with the set of $\\Omega$-stable diffeomorphisms. The equivalence of Lipschitz inverse periodic shadowing property and hyperbolicity of the closure of all periodic points is proved. Besides, we prove that the set of all diffeomorphisms that have Lipschitz inverse periodic shadowing property and whose periodic points are dense in the nonwandering set coincides with the set of Axiom A diffeomorphisms.

  6. Your First Period

    Science.gov (United States)

    ... Events Advocacy For Patients About ACOG Your First Period (Especially for Teens) Home For Patients Search FAQs ... Teens) FAQ049, May 2015 PDF Format Your First Period (Especially for Teens) Especially For Teens What is ...

  7. Middle Helladic Period

    DEFF Research Database (Denmark)

    Sarri, Kalliopi

    1999-01-01

    The Middle Bronze Age on Mainland Greece is also known as the Middle Helladic period. The chronological framework of this period extends from the beginnings of the second millenium - roughly 1900 - until 1550 BC, that is until the beginnings of the Mycenaean period. The Middle Helladic period...... is considered as the dark period of the cultural decline. The remains of the material culture reveal a clear retrogression while the information available on the social stratification and economy are so few and problematic in interpretation that this period is considered as the "Middle Age of Greek Prehistory......". About 1900 BC, the period during which the first palaces of Crete were being built, Mainland Greece was entering a long period of decline during which economic features changed radically. A large number of metals and imported products became particularly rare while composite forms of economic...

  8. ON PERIODIC DYNAMICAL SYSTEMS

    Institute of Scientific and Technical Information of China (English)

    LU WENLIAN; CHEN TIANPING

    2004-01-01

    The authors investigate the existence and the global stability of periodic solution for dynamical systems with periodic interconnections, inputs and self-inhibitions. The model is very general, the conditions are quite weak and the results obtained are universal.

  9. Community College Periodicals.

    Science.gov (United States)

    Pederson, Eldor O.

    Drawing from an examination of community college periodicals, their availability and characteristics, the academic affiliations of contributing authors, and the topics of their articles, this paper discusses the minor role which community college periodicals appear to play. A list of 35 periodicals dealing primary with community college education…

  10. Few period quasisymmetric stellarators

    Energy Technology Data Exchange (ETDEWEB)

    Isaev, M.Y.; Mikhailov, M.I.; Shafranov, V.D.; Subbotin, A.A. [Russian Research Centre `Kurchatov Institute`, Moscow (Russian Federation); Cooper, W.A. [Ecole Polytechnique Federale, Lausanne (Switzerland). Centre de Recherche en Physique des Plasma (CRPP); Medvedev, S.Y. [Keldysh Inst. of Applied Mathematics, Russian Academy of Sciences, Moscow (Russian Federation)

    1997-06-01

    The results of plasma equilibrium and local stability investigations in two and four-period quasisymmetric stellarators are presented. A near-axis approximation is used for 2-period systems and the 3D codes VMEC and TERPSICHORE are used for four-periods devices to optimise the configurations. (author) 4 figs., 8 refs.

  11. The Periodic Pyramid

    Science.gov (United States)

    Hennigan, Jennifer N.; Grubbs, W. Tandy

    2013-01-01

    The chemical elements present in the modern periodic table are arranged in terms of atomic numbers and chemical periodicity. Periodicity arises from quantum mechanical limitations on how many electrons can occupy various shells and subshells of an atom. The shell model of the atom predicts that a maximum of 2, 8, 18, and 32 electrons can occupy…

  12. Family planning Indonesia.

    Science.gov (United States)

    Singarimbun, M

    1968-06-01

    The growth of family planning activities in Indonesia in the Postwar period is traced; and future prospects for family planning are assessed. Transmigration projects initiated by the Dutch and supported by President Sukarno after Indonesian independence as a means of decreasing population pressure on the island of Java, are identified as the only official response to the population problem until 1965. In the face of the government's opposition to the idea of birth control as a population control measure, the activities of the Indonesian Planned Parenthood Association (IPPA) after its founding in 1957 were limited to advising mothers on spacing of their children for health reasons. Statements made in support of a national family planning program by government officials at a 1967 IPPA Congress and on other occasions are noted. The major components of an approved national family planning program to start in 1969 are described. However, the government's policy as of late 1967 and early 1968 is characterized as one of mainly benevolent encouragement and help to voluntary organizations. The chief impediment to family planning in Indonesia is said to be a lack of motivation and the force of traditional values that favor large families. On the positive side are: 1) Studies showing considerable interest in birth control by the rural population; 2) A long history of traditional birth control practices; 3) The absence of outright opposition by religious groups to the principle of family planning. However, financial costs, the need for the training of personnel, and a general unawareness of the magnitude of the task lying ahead constitute other formidable obstacles.

  13. Family Literacy

    Directory of Open Access Journals (Sweden)

    Livija Knaflič

    1999-12-01

    Full Text Available Research in child and adult literacy demonstrates that the achievement and the level of literacy that children attain at school is connected with the social and cultural characteristics and the level of literacy of the child's family. This intergenerational transfer of the level of literacy has motivated the search for different ways of improving the level of literacy.The concept of family literacy is based on the assumption that a higher level of parent literacy means that the children may achieve the same, and it also offers better schooling prospects. Family literacy programmes help fami­lies to develop different activities, in­cluding reading and writing skills, both in their community and in everyday life.

  14. The post-suicide family and the family physician.

    Science.gov (United States)

    Fisher, J V; Barnett, B L; Collins, J

    1976-06-01

    It is estimated that there are 750,000 people each year who are intimately affected by suicide. Prominent among these are the family survivors and their family physician. This paper offers a time frame which divides the period following the suicide into three phases: Immediate (the first ten days after the suicide); Intermediate (after the first ten days through the first year); and Extended (from the first year until restitution occurs). It identifies the chief emotional reactions which occur in each phase, explores their psychodynamic origins, and proposes suggestions for appropriate management during each of the three periods. The goal of this plan of management is to enable the family physician to function in a supportive emphatic, and restorative manner for the post-suicide family.

  15. Family Structure and Family Processes in Mexican American Families

    OpenAIRE

    Zeiders, Katharine H.; Roosa, Mark W.; Tein, Jenn-Yun

    2011-01-01

    Despite increases in single-parent families among Mexican Americans (MA), few studies have examined the association of family structure and family adjustment. Utilizing a diverse sample of 738 Mexican American families (21.7% single parent), the current study examined differences across family structure on early adolescent outcomes, family functioning, and parent-child relationship variables. Results revealed that early adolescents in single parent families reported greater school misconduct,...

  16. 六朝后期及隋唐三吴地区的道教信仰--以栝州叶氏家族为例%The Religious Beliefs of Three Wu Areas in the Late Period of the Six Dynastiesand Sui and Tang Dynasties---Taking the Guazhou Yip Family for Example

    Institute of Scientific and Technical Information of China (English)

    王大良

    2013-01-01

    六朝时期,三吴地区的宗教信仰有些受外来移民的影响,有些则是由自身激发、转化、延续、继承而来,其中道教文化信仰的源头便可一直追溯到春秋时期。此后又潜流涌动,到齐梁之际,在其西南部边陲地区孕育诞生了直到隋唐时期还有广泛影响的著名道教世家栝州叶氏家族。叶氏家族在六朝及隋唐的发展从一个侧面说明,三吴地区经济、文化发展受到内外双重因素的推动,如果单纯强调一个方面而忽视另一方面,便很难反映当时的历史全貌。%In the six dynasties period, the religious beliefs in Three Wu areas were influenced by some immigrants, while others were inspired, transformed, extended and inherited by itself, in which the source of Taoist cultural beliefs can be traced back to the Spring and Autumn Period. After that, undercurrent surging to the Qi and Liang dynasty, it gave birth to the famous Taoist family Guazhou Yip family, which had wide-ran-ging implications in southwestern border region until the Sui and Tang dynasties. The development of Yip family in the Sui and Tang Dynasties had indicated from a side note that economic and cultural development in the Three Wu areas has come from double factors both inside and outside. If we just simply focus on one aspect and ignoring the other aspect, it would be difficult to fully reflect the historical panorama.

  17. Hereditary periodic fever and reactive amyloidosis.

    NARCIS (Netherlands)

    Hilst, J.C.H. van der; Simon, A.; Drenth, J.P.H.

    2005-01-01

    Hereditary periodic fever syndromes (HPF) are a group of diseases characterised by recurrences of fever and inflammation separated by symptom-free intervals. Familial Mediterranean fever (FMF) is the most frequent entity within this group of disorders which further consists of

  18. Molecular and pathophysiologic mechanisms of hyperkalemic metabolic acidosis.

    OpenAIRE

    DuBose, T D

    2000-01-01

    In summary, hyperkalemia may have a dramatic impact on ammonium production and excretion. Chronic hyperkalemia decreases ammonium production in the proximal tubule and whole kidney, inhibits absorption of NH4+ in the mTALH, reduces medullary interstitial concentrations of NH4+ and NH3, and decreases entry of NH4+ and NH3 into the medullary collecting duct. The potential for development of a hyperchloremic metabolic acidosis is greatly augmented when renal insufficiency with associated reducti...

  19. Family Circle

    Institute of Scientific and Technical Information of China (English)

    2006-01-01

    Foster care is conducive to giving orphaned children a better life For most children living in orphanages, having a real home is just a pipe dream. Although they may be well looked after, receive a good education and proper nutrition, the love and care that come from being part of a real family just aren't there.

  20. My Family

    Institute of Scientific and Technical Information of China (English)

    刘才来

    2002-01-01

    There are four people in my family. They are grandma, father,mother and I. Now we all live in Wuhan. They are from different places. My grandma comes from Sichuan. She likes hot(辣4的) meat very much. She doesn't like bread or noodles at all. She likes vegetables a little. My father is from Guang Zhou.

  1. My Family

    Institute of Scientific and Technical Information of China (English)

    李梅

    2012-01-01

    There are four people in my family--my parents, my brother and I. My name is Li Mei. I'm fifteen years old. I am of medium height and build. I like English very much. It's very interesting. I can play the piano very well. It makes me feel very happy.

  2. Finding Family

    Institute of Scientific and Technical Information of China (English)

    LIU YUNYUN

    2010-01-01

    @@ It took 14 years--and just two min-utes-for an adopted Chinese girl to find her biological family. July 21 this year marked the first anniversary of Haley Butler's finding of her biological parents in Maanshan in east China's Anhui Province.

  3. FAMILY RICHARDIIDAE.

    Science.gov (United States)

    Wendt, Lisiane Dilli; Ale-Rocha, Rosaly

    2016-06-14

    Richardiidae are a family of "acalyptrate" Diptera represented by ca. 180 species distributed in the New World, mostly in the Neotropical region. The species that occur in Colombia have received little attention from taxonomists, and the great majority of them are known only from their type localities. Currently, 14 genera and 23 species are known to occur in the country.

  4. Family Genericity

    DEFF Research Database (Denmark)

    Ernst, Erik

    2006-01-01

    Type abstraction in object-oriented languages embody two techniques, each with its own strenghts and weaknesses. The first technique is extension, yielding abstraction mechanisms with good support for gradual specification. The prime example is inheritance. The second technique is functional abst...... the result as family genericity. The presented language design has been implemented....

  5. Finding Family

    Institute of Scientific and Technical Information of China (English)

    2010-01-01

    Discovering her birth parents was an exciting adventure for a 15-year-old girl It took 14 years-and just two minutes-for an adopted Chinese girl to find her biological family.July 21 this year marked the first

  6. Familial hypercholesterolaemia

    DEFF Research Database (Denmark)

    Versmissen, Jorie; Vongpromek, Ranitha; Yahya, Reyhana

    2016-01-01

    cholesterol efflux capacity between male familial hypercholesterolaemia (FH) patients with and without CHD relative to their non-FH brothers, and examined HDL constituents including sphingosine-1-phosphate (S1P) and its carrier apolipoprotein M (apoM). RESULTS: Seven FH patients were asymptomatic and six had...

  7. Family Genericity

    DEFF Research Database (Denmark)

    Ernst, Erik

    2006-01-01

    Type abstraction in object-oriented languages embody two techniques, each with its own strenghts and weaknesses. The first technique is extension, yielding abstraction mechanisms with good support for gradual specification. The prime example is inheritance. The second technique is functional abst...... the result as family genericity. The presented language design has been implemented....

  8. The attitudes of women towards family planning and related factors in the postpartum periodDoğum sonu dönemdeki kadınların aile planlaması konusundaki tutumları ve ilişkili faktörler

    Directory of Open Access Journals (Sweden)

    Nergiz Eryılmaz

    2016-08-01

    Full Text Available An early second pregnancy after birth may negatively influence the health of the first child and mother. Most of the women want to delay or prevent future pregnancies during the postpartum period. The postpartum is a very important period of preventing unwanted pregnancies. In postpartum period, use of contraceptive is connected with women’s cultural and religious believes, partner’s attitudes and previous contraceptive experiences. Family planning training in prenatal and postnatal period is considered as a standard care. The aim of the study is to determine the attitudes of women towards family planning and related factors in the postpartum period. This study is done as a descriptive research in the postpartum period. The samples consist of 272 women in the postpartum period. "Family Planning Attitude Scale" (FPAS and personal information questionnaire prepared by the researchers have been used to collect the data. In the analysis of data; Number, percentage, mean and standard deviation, Mann-Whitney U and Kruskal-Wallis test were used for the analysis of the data. It was found out that mean age of the postpartum women was 26.30±5.60 years and mean marriage duration (year was 5.84±4.92. It was noted that 72.8% of women planned to last pregnancy and 52,6% of them don’t want to do pregnancy again. It was determined that 47,8% of women’s source of information about family planning (FP was the health professional and 76,1% of them received counseling about FP. 94,9% of postpartum women want to use contraceptive method until they decide a new pregnancy .Significant differences were detected between postpartum women; education  (Z=-3.320; p:0.001, employment status (Z=-4.741; p:0.000, social security (Z=-4.569; p:0.000, family type (Z=-4.086; p:0.000, perception of economic status (KW=8402; p:0.015 getting counseling service about family planning  (Z=2.841; p=0.004, and using family planning in the postnatal period. (Z=-4.741; p:0

  9. Periodicity of chaotic solutions

    CERN Document Server

    Berezowski, Marek

    2016-01-01

    The scope of the paper is the analysis of the impact of flow reversal on the dynamics of cascades of reactors. Periodic and chaotic oscillations occur in the analyzed system. There is a dependence between the oscillation period of the state variable of the system without flow reversal and the recurrence period of windows of chaos in the steady-state diagram of the system with flow reversal.

  10. Bott periodicity of inclusions

    CERN Document Server

    Mare, Augustin-Liviu

    2011-01-01

    When looking at Bott's original proof of his periodicity theorem for the stable homotopy groups of the orthogonal and unitary groups, one sees in the background a differential geometric periodicity phenomenon. We show that this geometric phenomenon extends to the standard inclusion of the orthogonal group into the unitary group. This explains the periodicity of the group homomorphisms between stable homotopy groups which are induced by the inclusion map. Standard inclusions between other classical Riemannian symmetric spaces are also discussed.

  11. Painful menstrual periods

    Science.gov (United States)

    Menstruation - painful; Dysmenorrhea; Periods - painful; Cramps - menstrual; Menstrual cramps ... related activities for a few days during each menstrual cycle. Painful menstruation is the leading cause of ...

  12. Hypokalemic periodic paralysis

    National Research Council Canada - National Science Library

    Abbas, Haider; Kothari, Nikhil; Bogra, Jaishri

    2012-01-01

    Hypokalemic periodic paralysis is a rare genetic disorder characterized by recurrent attacks of skeletal muscle weakness with associated hypokalemia which is precipitated by stress, cold, carbohydrate...

  13. Learning about Familial Hypercholesterolemia

    Science.gov (United States)

    ... terms used on this page Learning About Familial Hypercholesterolemia What is familial hypercholesterolemia? What are the symptoms ... Additional Resources About Familial Hypercholesterolemia What is familial hypercholesterolemia? Familial hypercholesterolemia is an inherited condition that causes ...

  14. Natural Family Planning

    Science.gov (United States)

    ... Sex and Birth Control Birth Control Natural Family Planning Natural Family Planning Birth ControlPrevention and WellnessSex and Birth Control Share Natural Family Planning Natural Family PlanningWhat is natural family planning?Natural ...

  15. [Family violence].

    Science.gov (United States)

    Manoudi, F; Chagh, R; Es-soussi, M; Asri, F; Tazi, I

    2013-09-01

    Family violence is a serious public health problem, the scale of which is seriously increasing in Morocco. Although it has existed for a long time, we ignore the real characteristics of this plague in our country; our work consisted in an epidemiological approach of family violence in Marrakech during 2006. After elaborating a questionnaire, which allows the study of the demographic and social profile of the families, the study of violence exercised in the family and the evaluation of the depression in the women, we led an inquiry amongst 265 women. Analysis of the results obtained has allowed us to underline the following characteristics: 16.6% of the women in our sample had been physically beaten; the young age is a risk factor; the age range most affected by violence is in women between the ages of 30 and 40 and which represent 39% of the battered women; domestic violence touches all the social, economic and cultural classes: in our study, 63% of the women having undergone violence were housewives, 25% were managers and 3% senior executives; family problems were the most important cause of violence in our study, representing 32.32%. Requests for money was the cause in 11.3% of the cases, and imposed sexual relations were found in 6.8% of the cases; alcoholism is an aggravating factor of family violence; 27.3% of the spouses who assaulted their wives were drunk; 52% of the assaulted women were victims of violence in childhood and 36% had been witness to their father's violence; in 63.6% of the cases of violence, the children were witnesses, and in 25% of the cases the children were victims of violence at the same time as their mothers; 50% of the women victims of violence did not react, while 38.6% left home, and 9.1 filed for divorce. Thirty-two percent of the assaulted woman had been traumatised by the aggression; the association of depression and violence was very high, 343% of the battered women in our study suffered from severe depression. This work

  16. Second Period Access Report

    DEFF Research Database (Denmark)

    Maisondieu, Christophe; Giebhardt, Jochen; Tetu, Amelie

    The work described in this publication has received support from the European Community - Research Infrastructure Action under the FP7 “Capacities” Specific Programme through grant agreement number 262552, MaRINET. Project Periodic Report. 2nd Period: October 2012 – March 2014 inclusive....

  17. The Living Periodic Table

    Science.gov (United States)

    Nahlik, Mary Schrodt

    2005-01-01

    To help make the abstract world of chemistry more concrete eighth-grade students, the author has them create a living periodic table that can be displayed in the classroom or hallway. This display includes information about the elements arranged in the traditional periodic table format, but also includes visual real-world representations of the…

  18. Latent Period of Relaxation.

    Science.gov (United States)

    Kobayashi, M; Irisawa, H

    1961-10-27

    The latent period of relaxation of molluscan myocardium due to anodal current is much longer than that of contraction. Although the rate and the grade of relaxation are intimately related to both the stimulus condition and the muscle tension, the latent period of relaxation remains constant, except when the temperature of the bathing fluid is changed.

  19. On some periodicity effects

    DEFF Research Database (Denmark)

    Sorokin, Sergey V.

    2015-01-01

    The talk is concerned with the modelling of wave propagation in and vibration of periodic elastic structures. Although analysis of wave-guide properties of infinite periodic structures is a well establish research subject, some issues have not yet been fully addressed in the literature. The aim o...

  20. The Living Periodic Table

    Science.gov (United States)

    Nahlik, Mary Schrodt

    2005-01-01

    To help make the abstract world of chemistry more concrete eighth-grade students, the author has them create a living periodic table that can be displayed in the classroom or hallway. This display includes information about the elements arranged in the traditional periodic table format, but also includes visual real-world representations of the…

  1. Periodic Time Series Models

    NARCIS (Netherlands)

    Ph.H.B.F. Franses (Philip Hans); R. Paap (Richard)

    2004-01-01

    textabstractThis book considers periodic time series models for seasonal data, characterized by parameters that differ across the seasons, and focuses on their usefulness for out-of-sample forecasting. Providing an up-to-date survey of the recent developments in periodic time series, the book

  2. My family

    Institute of Scientific and Technical Information of China (English)

    陈君怡

    2011-01-01

    I have a happy fam_ly.Lookthis is my family photo.This is mydad.He often wears a pair Ofglasses(戴着一副眼镜I_This ismy mum.She is very pretty.Sheloves me very much.The little girlin a red blouse is me.I’m smiling(微笑).I love my family.

  3. [Familial hypercholesterolemia].

    Science.gov (United States)

    Turpin, G; Bruckert, E

    1999-12-01

    Familial hypercholesterolemia is characterized by a high plasma LDL-cholesterol level. The low-density particles are the end-product of the triglyceride-rich particles, i.e. VLDL, synthetized by the liver. These triglyceride-rich particles are subsequently transformed into intermediate density lipoprotein by the lipoprotein lipase and LDL after further triglyceride hydrolysis by the hepatic lipase. The LDL particles are taken up in all cells by the mean of the LDL receptor. A large body of evidence (including experimental, clinical, epidemiological data as well as the results of large trial with lipid lowering drugs) has accumulated to establish that these particles are one of the major causative factor of atherosclerosis and its complications. Two different mechanisms may be at work in the familial hypercholesterolemia: a mutation in the LDL receptor or a single mutation in the apolipoprotein B100. Specific therapeutic intervention should be undertaken to decrease the risk to develop cardiovascular disease, mainly coronary heart disease. The therapeutic intervention includes both a diet low in saturated fatty acids and cholesterol and statins which are now the first line therapy. Fibrates are proposed to those who do not tolerate statins and LDL-apheresis is associated to statin in the rare homozygous familial hypercholesterolemia.

  4. Periods and Nori motives

    CERN Document Server

    Huber, Annette

    2017-01-01

    This book casts the theory of periods of algebraic varieties in the natural setting of Madhav Nori’s abelian category of mixed motives. It develops Nori’s approach to mixed motives from scratch, thereby filling an important gap in the literature, and then explains the connection of mixed motives to periods, including a detailed account of the theory of period numbers in the sense of Kontsevich-Zagier and their structural properties. Period numbers are central to number theory and algebraic geometry, and also play an important role in other fields such as mathematical physics. There are long-standing conjectures about their transcendence properties, best understood in the language of cohomology of algebraic varieties or, more generally, motives. Readers of this book will discover that Nori’s unconditional construction of an abelian category of motives (over fields embeddable into the complex numbers) is particularly well suited for this purpose. Notably, Kontsevich's formal period algebra represents a to...

  5. Hypokalemic periodic paralysis: Three rare secondary causes

    Directory of Open Access Journals (Sweden)

    Prasanna Eswaradass Venkatesan

    2015-01-01

    Full Text Available Periodic paralysis is a rare neuromuscular disorder, related to a defect in muscle ion channels, characterized by episodes of painless muscle weakness, which may be precipitated by heavy exercise, fasting, or high-carbohydrate meals. Hypokalemic periodic paralysis may be familial (primary or secondary. Here, we report three cases of secondary causes of hypokalemic periodic paralysis. On evaluation, case 1 had distal renal tubular acidosis (RTA due to Sjogren′s syndrome, case 2 had drug induced proximal RTA (Fanconi′s syndrome and case 3 had thyrotoxicosis. Clinician must be aware of causes of secondary PP as recognition and diagnosis can completely prevent further attacks of periodic paralysis. Each of the above case is rare, but completely treatable if diagnosed. Low dose steroids with bicarbonate replacement in case 1, stopping tenofovir in case 2 and carbimazole therapy in case 3 prevented further attacks of periodic paralysis and cardiopulmonary complications.

  6. The Main Mode of Rural Elder Care in the Period of Social Transition:Social Insurance or Family Security%社会转型期农村养老的主体模式:社会保险抑或家庭保障

    Institute of Scientific and Technical Information of China (English)

    刘玮; 张正军

    2013-01-01

    Since the 1980 s the social pension insurance system has been the core issue in the construction of China’ s rural elder care system, but the reform of rural social pension insurance hasn’ t reached the anticipated goals over the past 20 years. The changes of rural family structure in the period of social transition bring the dual influence on the function of home-based elder care, exhibiting the structural characteristics. Compared with the social elder care, the home-based elder care has more advantages in terms of economic support, life care and spiritual comfort due to its characteristics of low cost and elaborateness and so on. Currently and even for a long period of social transition, the home-based elder care is supposed to be the main mode of rural elder care in Chi-na. The construction of the family support plan supported by the government is indispensable to stabilize and ex-tend the function of home-based elder care while the social pension insurance should also be established in a long run.%20世纪80年代以来,制度性社会养老保险成为构建中国农村养老体系的核心议题,但20多年来的农村社会养老保险改革目前尚未实现预期目标。社会转型期农村家庭结构的变迁对家庭养老功能带来双重影响,表现出结构性特征。与社会养老相比,家庭养老的低成本、精准化等特征使其在经济扶持、生活照料、精神慰藉方面仍具优势。在目前甚至较长期的社会转型期,家庭养老应当成为我国农村养老的主体模式。构建政府支持的家庭支持计划对于稳定、拓展家庭养老功能不可或缺,而社会养老保险值得长期建立。

  7. Mean-periodic functions

    Directory of Open Access Journals (Sweden)

    Carlos A. Berenstein

    1980-01-01

    Full Text Available We show that any mean-periodic function f can be represented in terms of exponential-polynomial solutions of the same convolution equation f satisfies, i.e., u∗f=0(μ∈E′(ℝn. This extends to n-variables the work of L. Schwartz on mean-periodicity and also extends L. Ehrenpreis' work on partial differential equations with constant coefficients to arbitrary convolutors. We also answer a number of open questions about mean-periodic functions of one variable. The basic ingredient is our work on interpolation by entire functions in one and several complex variables.

  8. Setting the Periodic Table.

    Science.gov (United States)

    Saturnelli, Annette

    1985-01-01

    Examines problems resulting from different forms of the periodic table, indicating that New York State schools use a form reflecting the International Union of Pure and Applied Chemistry's 1984 recommendations. Other formats used and reasons for standardization are discussed. (DH)

  9. The Periodic Table CD.

    Science.gov (United States)

    Banks, Alton J.; Holmes, Jon L.

    1995-01-01

    Describes the characteristics of the digitized version of The Periodic Table Videodisc. Provides details about the organization of information and access to the data via Macintosh and Windows computers. (DDR)

  10. Extremal periodic wave profiles

    Directory of Open Access Journals (Sweden)

    E. van Groesen

    2007-01-01

    Full Text Available As a contribution to deterministic investigations into extreme fluid surface waves, in this paper wave profiles of prescribed period that have maximal crest height will be investigated. As constraints the values of the momentum and energy integrals are used in a simplified description with the KdV model. The result is that at the boundary of the feasible region in the momentum-energy plane, the only possible profiles are the well known cnoidal wave profiles. Inside the feasible region the extremal profiles of maximal crest height are "cornered" cnoidal profiles: cnoidal profiles of larger period, cut-off and periodically continued with the prescribed period so that at the maximal crest height a corner results.

  11. On semi-periods

    CERN Document Server

    Avram, A C; Jancic, D; Uni, H; Uni, Humboldt

    1995-01-01

    The periods of the three-form on a Calabi-Yau manifold are found as solutions of the Picard-Fuchs equations; however, the toric varietal method leads to a generalized hypergeometric system of equations which has more solutions than just the periods. This same extended set of equations can be derived from symmetry considerations. Semi-periods are solutions of this extended system. They are obtained by integration of the three-form over chains; these chains can be used to construct cycles which, when integrated over, give periods. In simple examples we are able to obtain the complete set of solutions for the extended system. We also conjecture that a certain modification of the method will generate the full space of solutions in general.

  12. Setting the Periodic Table.

    Science.gov (United States)

    Saturnelli, Annette

    1985-01-01

    Examines problems resulting from different forms of the periodic table, indicating that New York State schools use a form reflecting the International Union of Pure and Applied Chemistry's 1984 recommendations. Other formats used and reasons for standardization are discussed. (DH)

  13. The Periodic Table CD.

    Science.gov (United States)

    Banks, Alton J.; Holmes, Jon L.

    1995-01-01

    Describes the characteristics of the digitized version of The Periodic Table Videodisc. Provides details about the organization of information and access to the data via Macintosh and Windows computers. (DDR)

  14. Optimal periodic control

    CERN Document Server

    Colonius, Fritz

    1988-01-01

    This research monograph deals with optimal periodic control problems for systems governed by ordinary and functional differential equations of retarded type. Particular attention is given to the problem of local properness, i.e. whether system performance can be improved by introducing periodic motions. Using either Ekeland's Variational Principle or optimization theory in Banach spaces, necessary optimality conditions are proved. In particular, complete proofs of second-order conditions are included and the result is used for various versions of the optimal periodic control problem. Furthermore a scenario for local properness (related to Hopf bifurcation) is drawn up, giving hints as to where to look for optimal periodic solutions. The book provides mathematically rigorous proofs for results which are potentially of importance in chemical engineering and aerospace engineering.

  15. Hypokalemic periodic paralysis

    Science.gov (United States)

    ... Rott K, Rudel R, Lehmann-Horn F. Muscle channelopathies: myotor and periodic paralyses. In: Darras BT, Jones ... Elsevier; 2015:chap 38. Kerchner GA, Ptacek LJ. Channelopathies. In: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy ...

  16. A calcium channel mutant mouse model of hypokalemic periodic paralysis

    National Research Council Canada - National Science Library

    Wu, Fenfen; Mi, Wentao; Hernández-Ochoa, Erick O; Burns, Dennis K; Fu, Yu; Gray, Hillery F; Struyk, Arie F; Schneider, Martin F; Cannon, Stephen C

    2012-01-01

    Hypokalemic periodic paralysis (HypoPP) is a familial skeletal muscle disorder that presents with recurrent episodes of severe weakness lasting hours to days associated with reduced serum potassium (K+...

  17. Historically defined autobiographical periods

    DEFF Research Database (Denmark)

    Brown, Norman R.; Hansen, Tia G. B.; Lee, Peter J.;

    2012-01-01

    The chapter reviews a research programme that has demonstrated the existence of historically defined autobiographical periods and identified the conditions that bring them about. Data from four samples of World War II-generation adults show that historically defined autobiographical periods endure...... over time and theoretical implications are discussed, notably by introducing a new approach to autobiographical memory, Transition Theory, which assumes that autobiographical memory is organized by transitional events that can be selfinitiated or externally imposed - historically defined...

  18. Embryonic development period and the prevalence of avian blood parasites.

    OpenAIRE

    Ricklefs, R E

    1992-01-01

    Variation in prevalence of avian hematozoa is related to taxonomic affiliation at the level of the family or subfamily but not of the genus within families. Prevalence is comparatively insensitive to the influences of habitat and season; however, temperate species have higher incidences of infection than tropical species belonging to the same families. Among taxa of nonraptorial altricial landbirds, hematozoan prevalence is inversely related to the length of the incubation period but shows li...

  19. Time-Periodic Einstein--Klein--Gordon Bifurcations of Kerr

    CERN Document Server

    Chodosh, Otis

    2015-01-01

    We construct one-parameter families of solutions to the Einstein--Klein--Gordon equations bifurcating off the Kerr solution such that the underlying family of spacetimes are each an asymptotically flat, stationary, axisymmetric, black hole spacetime, and such that the corresponding scalar fields are non-zero and time-periodic. An immediate corollary is that for these Klein--Gordon masses, the Kerr family is not asymptotically stable as a solution to the Einstein--Klein--Gordon equations.

  20. Periodic orbits of planets in binary systems

    Science.gov (United States)

    Voyatzis, G.

    2017-03-01

    Periodic solutions of the three body problem are very important for understanding its dynamics either in a theoretical framework or in various applications in celestial mechanics. In this paper we discuss the computation and continuation of periodic orbits for planetary systems. The study is restricted to coplanar motion. Starting from known results of two-planet systems around single stars, we perform continuation of solutions with respect to the mass and approach periodic orbits of single planets in two-star systems. Also, families of periodic solutions can be computed for fixed masses of the primaries. When they are linearly stable, we can conclude about the existence of phase space domains of long-term orbital stability.

  1. Roles within the Family

    Science.gov (United States)

    ... Life Listen Español Text Size Email Print Share Roles Within the Family Page Content Article Body Families ... family unit, and which rights, privileges, obligations, and roles are assigned to each family member. In most ...

  2. Periodic Ising Correlations

    CERN Document Server

    Hystad, Grethe

    2010-01-01

    In this paper, we first rework B. Kaufman's 1949 paper, "Crystal Statistics. II. Partition Function Evaluated by Spinor Analysis", by using representation theory. Our approach leads to a simpler and more direct way of deriving the spectrum of the transfer matrix for the finite periodic Ising model. We then determine formulas for the spin correlation functions that depend on the matrix elements of the induced rotation associated with the spin operator in a basis of eigenvectors for the transfer matrix. The representation of the spin matrix elements is obtained by considering the spin operator as an intertwining map. We exhibit the "new" elements V+ and V- in the Bugrij-Lisovyy formula as part of a holomorphic factorization of the periodic and anti-periodic summability kernels on the spectral curve associated with the induced rotation for the transfer matrix.

  3. Thyrotoxic periodic paralysis

    Directory of Open Access Journals (Sweden)

    Rojith Karandode Balakrishnan

    2011-01-01

    Full Text Available This article aims at highlighting the importance of suspecting thyrotoxicosis in cases of recurrent periodic flaccid paralysis; especially in Asian men to facilitate early diagnosis of the former condition. A case report of a 28 year old male patient with recurrent periodic flaccid paralysis has been presented. Hypokalemia secondary to thyrotoxicosis was diagnosed as the cause of the paralysis. The patient was given oral potassium intervention over 24 hours. The patient showed complete recovery after the medical intervention and was discharged after 24 hours with no residual paralysis. Thyrotoxic periodic paralysis (TPP is a complication of thyrotoxicosis, more common amongst males in Asia. It presents as acute flaccid paralysis in a case of hyperthyroidism with associated hypokalemia. The features of thyrotoxicosis may be subtle or absent. Thus, in cases of recurrent or acute flaccid muscle paralysis, it is important to consider thyrotoxicosis as one of the possible causes, and take measures accordingly.

  4. Hyperthyroid hypokalemic periodic paralysis.

    Science.gov (United States)

    Neki, N S

    2016-01-01

    Hyperthyroid periodic paralysis (HPP) is a rare life threatening complication of hyperthyroidism commonly occurring in young Asian males but sporadically found in other races. It is characterised by hypokalemia and acute onset paraparesis with prevalence of one in one hundred thousand (1 in 100000). The symptoms resolve promptly with potassium supplementation. Nonselective beta blockers like propranol can also be used to ameliorate and prevent subsequent paralytic attack. We report a case of 22 year old male presenting with hyperthyroid periodic paralysis (HPP) having very low serum potassium level.

  5. Integrating Family Resilience and Family Stress Theory.

    Science.gov (United States)

    Patterson, Joan M.

    2002-01-01

    The construct, family resilience, is defined differently by practitioners and researchers. This study tries to clarify the concept of family resilience. The foundation is family stress and coping theory, particularly the stress models that emphasize adaptation processes in families exposed to major adversities. (JDM)

  6. NEW EXACTLY SOLVABLE SUPERSYMMETRIC PERIODIC POTENTIALS

    Institute of Scientific and Technical Information of China (English)

    LIU KE-JIA; HE LI; ZHOU GUO-LI; WU YU-JIAO

    2001-01-01

    Using the formalism of supersymmetric quantum mechanics, we give an exact solution for a family of onedimensional periodic potentials, which are the supersymmetric partners of the potential proportional to the trigonometric function cos(2x) such that the Schrodinger equation for this potential is named the Mathieu equation mathematically.We show that the new potentials are distinctly different from their original ones. However, both have the same energy band structure. All the potentials obtained in this paper are free of singularities.

  7. Families' Selection of Children's Literature Books

    Science.gov (United States)

    Saracho, Olivia N.; Spodek, Bernard

    2010-01-01

    This study examined families' choices of children's literature books for joint story reading. Teachers, parents, and their children from five kindergarten classrooms participated in the study. Over a 4 months period, family members joined other parents twice a week to learn and practice story reading techniques. They selected children's literature…

  8. Hereditary periodic fever syndromes

    NARCIS (Netherlands)

    McDermott, MF; Frenkel, J

    Hereditary periodic fever syndromes are defined by recurrent attacks of generalised inflammation for which no infectious or auto-immune cause can be identified. For most of these disorders, the molecular basis has recently been elucidated. This has opened the prospect of novel therapeutic

  9. Periodic Table of Students.

    Science.gov (United States)

    Johnson, Mike

    1998-01-01

    Presents an exercise in which an eighth-grade science teacher decorated the classroom with a periodic table of students. Student photographs were arranged according to similarities into vertical columns. Students were each assigned an atomic number according to their placement in the table. The table is then used to teach students about…

  10. A Modern Periodic Table.

    Science.gov (United States)

    Herrenden-Harker, B. D.

    1997-01-01

    Presents a modern Periodic Table based on the electron distribution in the outermost shell and the order of filling of the sublevels within the shells. Enables a student to read off directly the electronic configuration of the element and the order in which filling occurs. (JRH)

  11. Oscillations following periodic reinforcement.

    Science.gov (United States)

    Monteiro, Tiago; Machado, Armando

    2009-06-01

    Three experiments examined behavior in extinction following periodic reinforcement. During the first phase of Experiment 1, four groups of pigeons were exposed to fixed interval (FI 16s or FI 48s) or variable interval (VI 16s or VI 48s) reinforcement schedules. Next, during the second phase, each session started with reinforcement trials and ended with an extinction segment. Experiment 2 was similar except that the extinction segment was considerably longer. Experiment 3 replaced the FI schedules with a peak procedure, with FI trials interspersed with non-food peak interval (PI) trials that were four times longer. One group of pigeons was exposed to FI 20s PI 80s trials, and another to FI 40s PI 160s trials. Results showed that, during the extinction segment, most pigeons trained with FI schedules, but not with VI schedules, displayed pause-peck oscillations with a period close to, but slightly greater than the FI parameter. These oscillations did not start immediately after the onset of extinction. Comparing the oscillations from Experiments 1 and 2 suggested that the alternation of reconditioning and re-extinction increases the reliability and earlier onset of the oscillations. In Experiment 3 the pigeons exhibited well-defined pause-peck cycles since the onset of extinction. These cycles had periods close to twice the value of the FI and lasted for long intervals of time. We discuss some hypotheses concerning the processes underlying behavioral oscillations following periodic reinforcement.

  12. Periodically poled silicon

    Science.gov (United States)

    Hon, Nick K.; Tsia, Kevin K.; Solli, Daniel R.; Khurgin, Jacob B.; Jalali, Bahram

    2010-02-01

    Bulk centrosymmetric silicon lacks second-order optical nonlinearity χ(2) - a foundational component of nonlinear optics. Here, we propose a new class of photonic device which enables χ(2) as well as quasi-phase matching based on periodic stress fields in silicon - periodically-poled silicon (PePSi). This concept adds the periodic poling capability to silicon photonics, and allows the excellent crystal quality and advanced manufacturing capabilities of silicon to be harnessed for devices based on χ(2)) effects. The concept can also be simply achieved by having periodic arrangement of stressed thin films along a silicon waveguide. As an example of the utility, we present simulations showing that mid-wave infrared radiation can be efficiently generated through difference frequency generation from near-infrared with a conversion efficiency of 50% based on χ(2) values measurements for strained silicon reported in the literature [Jacobson et al. Nature 441, 199 (2006)]. The use of PePSi for frequency conversion can also be extended to terahertz generation. With integrated piezoelectric material, dynamically control of χ(2)nonlinearity in PePSi waveguide may also be achieved. The successful realization of PePSi based devices depends on the strength of the stress induced χ(2) in silicon. Presently, there exists a significant discrepancy in the literature between the theoretical and experimentally measured values. We present a simple theoretical model that produces result consistent with prior theoretical works and use this model to identify possible reasons for this discrepancy.

  13. Treatment for periodic paralysis

    NARCIS (Netherlands)

    Sansone, [No Value; Meola, G.; Links, T. P.; Panzeri, M.; Rose, M. R.

    2008-01-01

    Background Primary periodic paralyses are rare inherited muscle diseases characterised by episodes of flaccid weakness affecting one or more limbs, lasting several hours to several days, caused by mutations in skeletal muscle channel genes. Objectives The objective of this review was to systematical

  14. A Modern Periodic Table.

    Science.gov (United States)

    Herrenden-Harker, B. D.

    1997-01-01

    Presents a modern Periodic Table based on the electron distribution in the outermost shell and the order of filling of the sublevels within the shells. Enables a student to read off directly the electronic configuration of the element and the order in which filling occurs. (JRH)

  15. Periodic Table of Students.

    Science.gov (United States)

    Johnson, Mike

    1998-01-01

    Presents an exercise in which an eighth-grade science teacher decorated the classroom with a periodic table of students. Student photographs were arranged according to similarities into vertical columns. Students were each assigned an atomic number according to their placement in the table. The table is then used to teach students about…

  16. Scheduling: Seven Period Day

    Science.gov (United States)

    Williamson, Ronald

    2010-01-01

    Driven by stable or declining financial resources many school districts are considering the costs and benefits of a seven-period day. While there is limited evidence that any particular scheduling model has a greater impact on student learning than any other, it is clear that the school schedule is a tool that can significantly impact teacher…

  17. Treatment for periodic paralysis

    NARCIS (Netherlands)

    Sansone, [No Value; Meola, G.; Links, T. P.; Panzeri, M.; Rose, M. R.

    2008-01-01

    Background Primary periodic paralyses are rare inherited muscle diseases characterised by episodes of flaccid weakness affecting one or more limbs, lasting several hours to several days, caused by mutations in skeletal muscle channel genes. Objectives The objective of this review was to systematical

  18. Chaotic period doubling

    NARCIS (Netherlands)

    Chandramouli, V. V. M. S.; Martens, M.; De Melo, W.; Tresser, C. P.

    2009-01-01

    The period doubling renormalization operator was introduced by Feigenbaum and by Coullet and Tresser in the 1970s to study the asymptotic small-scale geometry of the attractor of one-dimensional systems that are at the transition from simple to chaotic dynamics. This geometry turns out not to depend

  19. FAMILY BUSINESS AND DEVELOPMENT OF HOME WORK

    Directory of Open Access Journals (Sweden)

    Shuhrat Oblokulovich Kuvandikov

    2016-02-01

    Full Text Available The work we consider importance of a family business in conditions of modern market relations. The place of a family entrepreneurship of the market economy was substantiated, proposals on uplifting the effectiveness of the interrelations between the family entrepreneurship and financial institutions were given.Based on a comparative analysis of existing scientific literature determinations concepts of “family business “ and “household”, developed new definitions, and also made some specific adjustments to the economic functions of the family farm. Justified theoretically place the family farm in the market system, analyzed the activity of households in rural areas and develop ways to improve its efficiency.The main factors were developed using the methods of correlation and regression analysis forecast figures for the period 2015-2017 years and evidence-based recommendations on the prospects for the development of family businesses.

  20. Family Matters

    Directory of Open Access Journals (Sweden)

    Isabel de Riquer

    2011-04-01

    Full Text Available The scene is at the court of James I of Aragon in the mid-13th c., the place is the royal palace of Barcelona or any of the crown's other possessions, and the dramatis personae include the heir to the throne, prince Peire (future king Peire the Great, and the court's most famous troubadour, Cerverí de Girona (fl. 1259-85. Author of the largest corpus of any Occitan troubadour (114 poems, Cerverì distinguishes himself by the surprises and challenges he presents to his audience: an alba (the most openly erotic genre to the Virgin Mary, the Cobla in sis lengatges (Cobla in Six Languages, the apparently nonsensical Vers estrayn. Cerverì borrows equally from the folk-inspired Galician-Portuguese poetry and from the French tradition, including the chanson de malmariée, where a young woman bemoans being sold off by her family to an old man (gilos, "Jealous" and separated from her youthful doulz amis, some even praying for the death of their husband. Both within that tradition and among Cerverì's three chansons de malmariée, the Gelosesca stands out as "especially determined" to lose her husband, using every "solution" (prayer, black magic, potion or experimenta.

  1. Confusion in the Periodic Table of the Elements.

    Science.gov (United States)

    Fernelius, W. C.; Powell, W. H.

    1982-01-01

    Discusses long (expanded), short (condensed), and pyramidal periodic table formats and documents events leading to a periodic table in which subgroups (families) are designated with the letters A and B, suggesting that this format is confusing for those consulting the table. (JN)

  2. Confusion in the Periodic Table of the Elements.

    Science.gov (United States)

    Fernelius, W. C.; Powell, W. H.

    1982-01-01

    Discusses long (expanded), short (condensed), and pyramidal periodic table formats and documents events leading to a periodic table in which subgroups (families) are designated with the letters A and B, suggesting that this format is confusing for those consulting the table. (JN)

  3. Thyrotoxic periodic paralysis

    Energy Technology Data Exchange (ETDEWEB)

    Ferreiro, J.E.; Arguelles, D.J.; Rams, H. Jr.

    1986-01-01

    A case of thyrotoxic periodic paralysis is reported in a Hispanic man with an unusual recurrence six weeks after radioactive iodine treatment. Thyrotoxic periodic paralysis has now been well characterized in the literature: it occurs primarily in Orientals with an overwhelming male preponderance and a higher association of specific HLA antigens. Clinical manifestations include onset after high carbohydrate ingestion or heavy exertion, with progressive symmetric weakness leading to flaccid paralysis of the extremities and other muscle groups, lasting several hours. If hypokalemia is present, potassium administration may help abort the attack. Although propranolol can be efficacious in preventing further episodes, the only definitive treatment is establishing a euthyroid state. The pathophysiology is still controversial, but reflects altered potassium and calcium dynamics as well as certain morphologic characteristics within the muscle unit itself.

  4. Cells anticipate periodic events

    Science.gov (United States)

    Nakagaki, Toshiyuki

    2009-03-01

    We show that an amoeboid organism can anticipate the timing of periodic events. The plasmodium of the true slime mold Physarum polycephalum moves rapidly under favourable conditions, but stops moving when transferred to less-favourable conditions. Plasmodia exposed to unfavourable conditions, presented in three consecutive pulses at constant intervals, reduced their locomotive speed in response to each episode. When subsequently subjected to favourable conditions, the plasmodia spontaneously reduced their locomotive speed at the time point when the next unfavourable episode would have occurred. This implied anticipation of impending environmental change. After this behaviour had been evoked several times, the locomotion of the plasmodia returned to normal; however, the anticipatory response could subsequently be induced by a single unfavourable pulse, implying recall of the memorized periodicity. We explored the mechanisms underlying these behaviours from a dynamical systems perspective. Our results hint at the cellular origins of primitive intelligence and imply that simple dynamics might be sufficient to explain its emergence.

  5. Microtransitions and the dynamics of family functioning.

    Science.gov (United States)

    Everri, Marina; Fruggeri, Laura; Molinari, Luisa

    2014-03-01

    This paper presents a qualitative observational study aimed at exploring microtransitions in the relational dynamics of family functioning when the children are adolescents. Three concurrent levels were considered central for family functioning in this period: the acknowledgment of emerging competences, the redefinition of the power structure, and the regulation of interpersonal distances. Twenty-eight non-clinical Italian families with at least one adolescent child were interviewed and video-recorded in their homes. A stance-taking process analysis was carried out on the family interactive sequences arising in the course of the interviews. This analysis was based on the stances taken by all family members in relation to their reciprocal evaluations, positions, and alignments, which allowed us to point out the interlocking of competences, power and distances. Out of all the possible theoretical combinations of these three dimensions, we identified four forms of interaction. In two forms, the emerging changes were not incorporated in the families' interactive repertoires by either reconfirming family stability or resisting family changes. In these ways of interacting competences, power, and distances were not reorganized. In the other two forms, instead, family microtransitions were observable in the extent to which family members either explored family changes or legitimated family reorganizations. In these processes, they could redefine and readdress their ways of interacting.

  6. Bibliography of the family Braconidae (Hymenoptera: Ichneumonoidea) (1964-2003)

    NARCIS (Netherlands)

    Ghahari, H.; Yu, D.S.; Achterberg, van C.

    2006-01-01

    A bibliography of the family Braconidae/Hymenoptera: Ichneumonidae is given for the period 1964-2003. It is an addition to Shenefelt's bibliography (1965), which covers the period 1785-1963. In total 10,436 references are listed.

  7. Correlation between family interaction and adolescents’ attitudes

    Directory of Open Access Journals (Sweden)

    Rozana Petani

    2011-10-01

    Full Text Available The impact of the family on the individual is very powerful and life-long. The family differs from other factors because the individual spends the most time in the family environment. In his family the child makes his first steps, utters the first words, starts gaining knowledge, learns his first value, forms attitudes and forms the foundation for the later development as a complete personality. Parents are the first and most responsible educators of their child, and upbringing is the most important and probably the hardest task of every parent. The period of adolescence is very important in every person’s life, especially because of the process of separation and individuation. This is a transitional period from childhood to maturity with the greatest dynamics in the development of the individual. In this period adolescents change and mature. They are able to make independent decisions. Relationships with their parents play an important role in shaping their behavior. The paper observes the family through family interaction, which consists of the following dimensions: satisfaction with their own families, acceptance and rejection by the mother and father. The aim of this study was to examine whether dimensions of parental behavior and satisfaction with their own families, perceived by adolescents, are associated with adolescents’ attitudes to some aspects of family life. The study was conducted on 862 students of the final year of high school, of whom 385 were male and 477 female. The results showed a statistically significant correlation between the assessed family interaction dimensions and adolescents’ attitudes to family life, with the exception of the attitude to work. The results obtained can be used in organizing programs focusing on preparation of young people for parental and marital roles and strengthening the skills required later in their family lives for constructive solving of the problems they will encounter.

  8. Thyrotoxic hypokalemic periodic paralysis in a Hispanic male.

    Science.gov (United States)

    Zumo, Lawrence A.; Terzian, Christian; Brannan, Timothy

    2002-01-01

    We report a case of a Hispanic male presenting with acute onset of bilateral lower extremity weakness, without any antecedent viral or bacterial illness, dietary changes, infiltrative orbitopathy, diffuse goiter, infiltrative dermopathy, and family history of periodic paralysis, who was later found to have Graves' disease. This demonstrates a rare case of periodic paralysis as the initial presentation of hyperthyroidism. Thyrotoxic hypokalemic periodic paralysis is common in Asian and Hispanic individuals and uncommon in whites and African Americans. PMID:12069220

  9. Family and family therapy in Russia.

    Science.gov (United States)

    Bebtschuk, Marina; Smirnova, Daria; Khayretdinov, Oleg

    2012-04-01

    This article represents the information about family and family therapy in the context of culture, traditions and contemporary changes of social situations in Russia. The legislation of family rights are mentioned within items about marriage and family in the Constitution, Civil Code and Family Code of the Russian Federation which has changed during recent years. The definition of family and description of family structure are given through the prism of the current demographic situation, dynamics of statistics of marriage and divorce rates, mental disorders, disabilities and such phenomena as social abandonment. The actual curriculum, teaching of family therapy and its disadvantages, system of continuous education, supervision and initiatives of the Institute of Integrative Family Therapy in improvement of preparing of specialists who can provide qualified psychosocial assistance for the family according to the actual needs of society are noted. The directions of state and private practice of family counselling and therapy both for psychiatric patients and medical patients, for adults and children in a family systemic approach are highlighted with an indication of the spectrum of techniques and methods used by Russian professionals. The main obstacles and perspectives of development of family therapy in Russia are summarized.

  10. The postanesthetic period. Complications.

    Science.gov (United States)

    Malamed, S F

    1987-01-01

    Postanesthetic complications can occur even in the best of circumstances. Proper preparation of the staff, aggressive monitoring of the recovering patient, and early recognition and management of the complications are essential if the outcome is to be successful. In reviewing postanesthetic complications, two factors are present in the overwhelming majority of situations--hypoxia and hypercarbia--often the direct result of inadequate monitoring during the postanesthetic period. The anesthetic procedure is not over once the anesthetic agents are discontinued. The skillful anesthetist is aware of the possibilities of postoperative complications and prevents problems by employing enhanced monitoring techniques during the recovery phase.

  11. Periodic Mesoporous Organosilica Nanorice

    Directory of Open Access Journals (Sweden)

    Mohanty Paritosh

    2008-01-01

    Full Text Available Abstract A periodic mesoporous organosilica (PMO with nanorice morphology was successfully synthesized by a template assisted sol–gel method using a chain-type precursor. The PMO is composed of D and T sites in the ratio 1:2. The obtained mesoporous nanorice has a surface area of 753 m2 g−1, one-dimensional channels, and a narrow pore size distribution centered at 4.3 nm. The nanorice particles have a length of ca. 600 nm and width of ca. 200 nm.

  12. Periods of Hecke characters

    CERN Document Server

    Schappacher, Norbert

    1988-01-01

    The starting point of this Lecture Notes volume is Deligne's theorem about absolute Hodge cycles on abelian varieties. Its applications to the theory of motives with complex multiplication are systematically reviewed. In particular, algebraic relations between values of the gamma function, the so-called formula of Chowla and Selberg and its generalization and Shimura's monomial relations among periods of CM abelian varieties are all presented in a unified way, namely as the analytic reflections of arithmetic identities beetween Hecke characters, with gamma values corresponding to Jacobi sums. The last chapter contains a special case in which Deligne's theorem does not apply.

  13. Evidence-based provisional clinical classification criteria for autoinflammatory periodic fevers

    NARCIS (Netherlands)

    Federici, Silvia; Sormani, Maria Pia; Ozen, Seza; Lachmann, Helen J; Amaryan, Gayane; Woo, Patricia; Koné-Paut, Isabelle; Dewarrat, Natacha; Cantarini, Luca; Insalaco, Antonella; Uziel, Yosef; Rigante, Donato; Quartier, Pierre; Demirkaya, Erkan; Herlin, Troels; Meini, Antonella; Fabio, Giovanna; Kallinich, Tilmann; Martino, Silvana; Butbul, Aviel Yonatan; Olivieri, Alma; Kuemmerle-Deschner, Jasmin; Neven, Benedicte; Simon, Anna; Ozdogan, Huri; Touitou, Isabelle; Frenkel, Joost; Hofer, Michael; Martini, Alberto; Ruperto, Nicolino; Gattorno, Marco

    2015-01-01

    The objective of this work was to develop and validate a set of clinical criteria for the classification of patients affected by periodic fevers. Patients with inherited periodic fevers (familial Mediterranean fever (FMF); mevalonate kinase deficiency (MKD); tumour necrosis factor

  14. Bequeathing Family Continuity.

    Science.gov (United States)

    Spanier, Graham B.

    1989-01-01

    Notes that many children who experience abuse, family disruption, or poverty reach adulthood with a strong commitment to family life. Questions whether changes in American families are indicators of pathology, deterioration, and instability; and asks how dysfunctional families transmit commitment to the concept of family to succeeding generations.…

  15. Family Reading Night

    Science.gov (United States)

    Hutchins, Darcy; Greenfeld, Marsha; Epstein, Joyce

    2007-01-01

    This book offers clear and practical guidelines to help engage families in student success. It shows families how to conduct a successful Family Reading Night at their school. Family Night themes include Scary Stories, Books We Love, Reading Olympics, Dr. Seuss, and other themes. Family reading nights invite parents to come to school with their…

  16. Family Factors in Small Family Business Growth

    National Research Council Canada - National Science Library

    Jim Cater; Marilyn Young

    2016-01-01

    .... We suggest that while all three patterns of small family business growth may lead to individual company success and sustainability, businesses with units in multiple trade areas may be most adept at managing the six family factors.

  17. Periodic truss structures

    Science.gov (United States)

    Zok, Frank W.; Latture, Ryan M.; Begley, Matthew R.

    2016-11-01

    Despite the recognition of the enormous potential of periodic trusses for use in a broad range of technologies, there are no widely-accepted descriptors of their structure. The terminology has been based loosely either on geometry of polyhedra or of point lattices: neither of which, on its own, has an appropriate structure to fully define periodic trusses. The present article lays out a system for classification of truss structure types. The system employs concepts from crystallography and geometry to describe nodal locations and connectivity of struts. Through a series of illustrative examples of progressively increasing complexity, a rational taxonomy of truss structure is developed. Its conceptual evolution begins with elementary cubic trusses, increasing in complexity with non-cubic and compound trusses as well as supertrusses, and, finally, with complex trusses. The conventions and terminology adopted to define truss structure yield concise yet unambiguous descriptions of structure types and of specific (finite) trusses. The utility of the taxonomy is demonstrated by bringing into alignment a disparate set of ad hoc and incomplete truss designations previously employed in a broad range of science and engineering fields. Additionally, the merits of a particular compound truss (comprising two interpenetrating elementary trusses) is shown to be superior to the octet truss for applications requiring high stiffness and elastic isotropy. By systematically stepping through and analyzing the finite number of structure types identified through the present classification system, optimal structures for prescribed mechanical and functional requirements are expected to be ascertained in an expeditious manner.

  18. Consuming Family Dinner Time

    National Research Council Canada - National Science Library

    Kelli Gutierrez; Linda Price; Eric Arnould

    2008-01-01

      Our study examines whether and how family dinners are embedded in the centrifugal and centripetal dynamics of everyday family life, the diversity of meanings they have for families and the intentions...

  19. Family Adjustment to Aphasia

    Science.gov (United States)

    ... Public / Speech, Language and Swallowing / Disorders and Diseases Family Adjustment to Aphasia Richard S. was a senior manager ... It also presents a great challenge to the family. There may be tension among family members and ...

  20. Improving Family Communications

    Science.gov (United States)

    ... Spread the Word Shop AAP Find a Pediatrician Family Life Medical Home Family Dynamics Adoption & Foster Care ... Listen Español Text Size Email Print Share Improving Family Communications Page Content Article Body How can I ...

  1. Normal Functioning Family

    Science.gov (United States)

    ... Spread the Word Shop AAP Find a Pediatrician Family Life Medical Home Family Dynamics Adoption & Foster Care ... Español Text Size Email Print Share Normal Functioning Family Page Content Article Body Is there any way ...

  2. Family Activities for Fitness

    Science.gov (United States)

    Grosse, Susan J.

    2009-01-01

    This article discusses how families can increase family togetherness and improve physical fitness. The author provides easy ways to implement family friendly activities for improving and maintaining physical health. These activities include: walking, backyard games, and fitness challenges.

  3. When Periodicities Enforce Aperiodicity

    Science.gov (United States)

    Bédaride, Nicolas; Fernique, Thomas

    2015-05-01

    Non-periodic tilings and local rules are commonly used to model the long range aperiodic order of quasicrystals and the finite-range energetic interactions that stabilize them. This paper focuses on planar rhombus tilings, which are tilings of the Euclidean plane, which can be seen as an approximation of a real plane embedded in a higher dimensional space. Our main result is a characterization of the existence of local rules for such tilings when the embedding space is four-dimensional. The proof is an interplay of algebra and geometry that makes use of the rational dependencies between the coordinates of the embedded plane. We also apply this result to some cases in a higher dimensional embedding space, notably tilings with n-fold rotational symmetry.

  4. Periods and Superstring Amplitudes

    CERN Document Server

    Stieberger, S

    2016-01-01

    Scattering amplitudes which describe the interaction of physical states play an important role in determining physical observables. In string theory the physical states are given by vibrations of open and closed strings and their interactions are described (at the leading order in perturbation theory) by a world-sheet given by the topology of a disk or sphere, respectively. Formally, for scattering of N strings this leads to N-3-dimensional iterated real integrals along the compactified real axis or N-3-dimensional complex sphere integrals, respectively. As a consequence the physical observables are described by periods on M_{0,N} - the moduli space of Riemann spheres of N ordered marked points. The mathematical structure of these string amplitudes share many recent advances in arithmetic algebraic geometry and number theory like multiple zeta values, single-valued multiple zeta values, Drinfeld, Deligne associators, Hopf algebra and Lie algebra structures related to Grothendiecks Galois theory. We review the...

  5. [Family reunification, family-formation migration, and return migration of Turks and Moroccans].

    Science.gov (United States)

    De Beer, J; Kuijper, H; Noordam, R

    1991-01-01

    The authors analyze migration patterns to the Netherlands from Turkey and Morocco for the period 1977-1989. Three distinct phases emerge: labor migration, family-reunification migration, and family-formation migration. The rise in migration from Turkey and Morocco during the second half of the 1980s is attributed to family-formation migration, which is defined as a member of the second generation marrying a partner from the country of origin. (SUMMARY IN ENG)

  6. Branding a family business

    OpenAIRE

    Pohjola, Matti

    2016-01-01

    This master’s thesis main object was to understand better the very little researched topic: branding a family business. The main aim was to seek the values used behind family business that are the family values used in the brand and how the branding has been implemented in a family company. A qualitative method was chosen for this research for an interpretative analysis of the subject. Five family companies were chosen for the interviews. All these family companies are known Fi...

  7. Family Health History and Diabetes

    Science.gov (United States)

    ... Diabetes Diabetes Risk Test Family Health History Quiz Family Health History Quiz Family health history is an ... health problems. Four Questions You Should Ask Your Family About Diabetes & Family Health History Knowing your family ...

  8. Periodic Orbits in Rotating Second Degree and Order Gravity Fields

    Institute of Scientific and Technical Information of China (English)

    Wei-Duo Hu; Daniel J.Scheeres

    2008-01-01

    Periodic orbits in an arbitrary 2nd degree and order uniformly rotating gravity field are studied. We investigate the four equilibrium points in this gravity field. We see that close relation exists between the stability of these equilibria and the existence and stability of their nearby periodic orbits. We check the periodic orbits with non-zero periods. In our searching procedure for these periodic orbits, we remove the two unity eigenvalues from the state transition matrix to find a robust, non-singular linear map to solve for the periodic orbits. The algorithm converges well, especially for stable periodic orbits. Using the searching procedure, which is relatively automatic, we find five basic families of periodic orbits in the rotating second degree and order gravity field for planar motion, and discuss their existence and stability at different central body rotation rates.

  9. Families and family therapy in Hong Kong.

    Science.gov (United States)

    Tse, Samson; Ng, Roger M K; Tonsing, Kareen N; Ran, Maosheng

    2012-04-01

    Family therapy views humans not as separate entities, but as embedded in a network of relationships, highlighting the reciprocal influences of one's behaviours on one another. This article gives an overview of family demographics and the implementation of family therapy in Hong Kong. We start with a review of the family demographics in Hong Kong and brief notes on families in mainland China. Demographics show that the landscape has changed markedly in the past decade, with more cross-border marriages, an increased divorce rate, and an ageing overall population - all of which could mean that there is increasing demand for professional family therapy interventions. However, only a limited number of professionals are practising the systems-based approach in Hong Kong. Some possible reasons as to why family therapy is not well disseminated and practised are discussed. These reasons include a lack of mental health policy to support family therapy, a lack of systematic family therapy training, and a shortage of skilled professionals. Furthermore, challenges in applying the western model in Chinese culture are also outlined. We conclude that more future research is warranted to investigate how family therapy can be adapted for Chinese families.

  10. Family psychology and family therapy in Japan.

    Science.gov (United States)

    Kameguchi, K; Murphy-Shigematsu, S

    2001-01-01

    The development of family psychology and family therapy in Japan has occurred mostly since the 1980s. This development was originally activated by the major social issue in contemporary Japan of school refusal, in which more than 127,000 children either overtly refuse to or claim that they cannot go to school. From a family perspective, this problem is analyzed as it relates to the confusion that children experience from unbalanced and unclear boundaries in family relations or "membranes." An approach to family therapy that adapts systems theory and integrates a clay sculpting medium has been developed to work with Japanese families confronting this problem. The design and implementation of preventative family psychology programs applied at the community level are also an important part of the future development in these fields.

  11. Family doctors' involvement with families in Estonia

    Directory of Open Access Journals (Sweden)

    Lember Margus

    2004-10-01

    Full Text Available Abstract Background Family doctors should care for individuals in the context of their family. Family has a powerful influence on health and illness and family interventions have been shown to improve health outcomes for a variety of health problems. The aim of the study was to investigate the Estonian family doctors' (FD attitudes to the patients' family-related issues in their work: to explore the degree of FDs involvement in family matters, their preparedness for management of family-related issues and their self-assessment of the ability to manage different family-related problems. Methods A random sample (n = 236 of all FDs in Estonia was investigated using a postal questionnaire. Altogether 151 FDs responded to the questionnaire (response rate 64%, while five of them were excluded as they did not actually work as FDs. Results Of the respondents, 90% thought that in managing the health problems of patients FDs should communicate and cooperate with family members. Although most of the family doctors agreed that modifying of the health damaging risk factors (smoking, alcohol and drug abuse of their patients and families is their task, one third of them felt that dealing with these problems is ineffective, or perceived themselves as poorly prepared or having too little time for such activities. Of the respondents, 58% (n = 83 were of the opinion that they could modify also relationship problems. Conclusions Estonian family doctors are favourably disposed to involvement in family-related problems, however, they need some additional training, especially in the field of relationship management.

  12. [Childhood periodic syndromes].

    Science.gov (United States)

    Cuvellier, J-C; Lépine, A

    2010-01-01

    This review focuses on the so-called "periodic syndromes of childhood that are precursors to migraine", as included in the Second Edition of the International Classification of Headache Disorders. Three periodic syndromes of childhood are included in the Second Edition of the International Classification of Headache Disorders: abdominal migraine, cyclic vomiting syndrome and benign paroxysmal vertigo, and a fourth, benign paroxysmal torticollis is presented in the Appendix. The key clinical features of this group of disorders are the episodic pattern and intervals of complete health. Episodes of benign paroxysmal torticollis begin between 2 and 8 months of age. Attacks are characterized by an abnormal inclination and/or rotation of the head to one side, due to cervical dystonia. They usually resolve by 5 years. Benign paroxysmal vertigo presents as sudden attacks of vertigo, accompanied by inability to stand without support, and lasting seconds to minutes. Age at onset is between 2 and 4 years, and the symptoms disappear by the age of 5. Cyclic vomiting syndrome is characterized in young infants and children by repeated stereotyped episodes of pernicious vomiting, at times to the point of dehydration, and impacting quality of life. Mean age of onset is 5 years. Abdominal migraine remains a controversial issue and presents in childhood with repeated stereotyped episodes of unexplained abdominal pain, nausea and vomiting occurring in the absence of headache. Mean age of onset is 7 years. Both cyclic vomiting syndrome and abdominal migraine are noted for the absence of pathognomonic clinical features but also for the large number of other conditions to be considered in their differential diagnoses. Diagnostic criteria, such as those of the Second Edition of the International Classification of Headache Disorders and the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, have made diagnostic approach and management easier. Their diagnosis

  13. Differentiating PFAPA syndrome from monogenic periodic fevers.

    Science.gov (United States)

    Gattorno, Marco; Caorsi, Roberta; Meini, Antonella; Cattalini, Marco; Federici, Silvia; Zulian, Francesco; Cortis, Elisabetta; Calcagno, Giuseppina; Tommasini, Alberto; Consolini, Rita; Simonini, Gabriele; Pelagatti, Maria Antonietta; Baldi, Maurizia; Ceccherini, Isabella; Plebani, Alessandro; Frenkel, Joost; Sormani, Maria Pia; Martini, Alberto

    2009-10-01

    To analyze whether there were clinical differences between genetically positive and negative patients fulfilling periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome criteria and to test the accuracy of the Gaslini diagnostic score for identifying patients with PFAPA syndrome with higher probabilities of carrying relevant mutations in genes associated with periodic fevers. Complete clinical and genetic information was available for 393 children with periodic fever; 82 had positive genetic test results, 75 had incomplete genetic test results, and 236 had negative results for MVK, TNFRSF1A, and MEFV mutations. Current diagnostic criteria for PFAPA syndrome were applied. Of 393 children, 210 satisfied PFAPA syndrome criteria; 43 carried diagnostic mutations (mevalonate kinase deficiency: n = 33; tumor necrosis factor receptor-associated periodic syndrome: n = 3; familial Mediterranean fever: n = 7), 37 displayed low-penetrance mutations or incomplete genotypes, and 130 demonstrated negative genetic testing results. Genetically positive patients had higher frequencies of abdominal pain and diarrhea (P < .001), vomiting (P = .006), and cutaneous rash and arthralgia (P = .01). Genetically negative patients had a higher frequency of exudative pharyngitis (P = .010). Genetically undetermined patients showed the same pattern of symptom frequency as genetically negative patients. The Gaslini diagnostic score was able to identify 91% of genetically positive patients correctly, with a global accuracy of 66%. The Gaslini diagnostic score represents a useful tool to identify patients meeting PFAPA syndrome criteria and at low risk of carrying relevant mutations in genes associated with periodic fevers.

  14. Familial congenital heart disease in Bandung, Indonesia

    Directory of Open Access Journals (Sweden)

    Sri Endah Rahayuningsih

    2013-01-01

    Full Text Available Background Congenital heart disease (CHD may occur in several members of a family. Studies have shown that familial genetic factor play a role in CHD.Objective To identify familial recurrences of CHD in families with at least one member treated for CHD in Dr. Hasan Sadikin Hospital, Bandung Indonesia.Methods In this descriptive study, subjects were CHD patients hospitalized or treated from January 2005 to December 2011. We constructed family pedigrees for five families.Results During the study period, there were 1,779 patients with CHD. We found 5 families with 12 familial CHD cases, consisting of 8 boys and 4 girls. Defects observed in these 12 patients were tetralogy of Fallot, transposition of the great arteries, persistent ductus arteriosus, ventricular septal defect, tricuspid atresia, pulmonary stenosis, and dilated cardiomyopathy. Persistent ductus arteriosus was the most frequently observed defect (4 out of 12 subjects. None of the families had a history of consanguinity. The recurrence risk of CHD among siblings was calculated to be 0.67%, and the recurrence risk of CHD among cousins was 0.16%.Conclusion Familial CHD may indicate the need for genetic counseling and further pedigree analysis.

  15. Familial congenital heart disease in Bandung, Indonesia

    Directory of Open Access Journals (Sweden)

    Sri Endah Rahayuningsih

    2013-03-01

    Full Text Available Background Congenital heart disease (CHD may occur in several members of a family. Studies have shown that familial genetic factor play a role in CHD. Objective To identify familial recurrences of CHD in families with at least one member treated for CHD in Dr. Hasan Sadikin Hospital, Bandung Indonesia. Methods In this descriptive study, subjects were CHD patients hospitalized or treated from January 2005 to December 2011. We constructed family pedigrees for five families. Results During the study period, there were 1,779 patients with CHD. We found 5 families with 12 familial CHD cases, consisting of 8 boys and 4 girls. Defects observed in these 12 patients were tetralogy of Fallot, transposition of the great arteries, persistent ductus arteriosus, ventricular septal defect, tricuspid atresia, pulmonary stenosis, and dilated cardiomyopathy. Persistent ductus arteriosus was the most frequently observed defect (4 out of 12 subjects. None of the families had a history of consanguinity. The recurrence risk of CHD among siblings was calculated to be 0.67%, and the recurrence risk of CHD among cousins was 0.16%. Conclusion Familial CHD may indicate the need for genetic counseling and further pedigree analysis. [Paediatr Indones. 2013;53:173-6.

  16. 3D periodic orbits in the restricted four body problem

    Science.gov (United States)

    Baltagiannis, A.; Papadakis, K.

    2013-09-01

    One big body (Sun) of mass m1 and two other small bodies of masses m2 and m3 correspondingly, move in circular orbits keeping an equilateral triangle configuration, about the center of mass of the system fixed at the origin of the coordinate system. A massless particle is moving under the Newtonian gravitational attraction of the primaries and does not affect the motion of the three bodies. Using the vertical-critical orbits of planar families of symmetric periodic orbits as starting points, we determine and present in this paper, families of three-dimensional periodic solutions of the problem. Characteristic curves of the 3D-families which emanate from the plane are presented. The stability of every three-dimensional periodic orbit which numerically calculated is also studied.

  17. Periodic 2-graphs arising from subshifts

    CERN Document Server

    Pask, David; Weaver, Natasha

    2009-01-01

    Higher-rank graphs were introduced by Kumjian and Pask to provide models for higher-rank Cuntz-Krieger algebras. In a previous paper, we constructed 2-graphs whose path spaces are rank-two subshifts of finite type, and showed that this construction yields aperiodic 2-graphs whose $C^*$-algebras are simple and are not ordinary graph algebras. Here we show that the construction also gives a family of periodic 2-graphs which we call \\emph{domino graphs}. We investigate the combinatorial structure of domino graphs, finding interesting points of contact with the existing combinatorial literature, and prove a structure theorem for the $C^*$-algebras of domino graphs.

  18. Nekhoroshev theorem for the periodic Toda lattice.

    Science.gov (United States)

    Henrici, Andreas; Kappeler, Thomas

    2009-09-01

    The periodic Toda lattice with N sites is globally symplectomorphic to a two parameter family of N-1 coupled harmonic oscillators. The action variables fill out the whole positive quadrant of R(N-1). We prove that in the interior of the positive quadrant as well as in a neighborhood of the origin, the Toda Hamiltonian is strictly convex and therefore Nekhoroshev's theorem applies on (almost) all parts of phase space (2000 Mathematics Subject Classification: 37J35, 37J40, 70H06).

  19. Competitiveness of Family Businesses

    NARCIS (Netherlands)

    M.A.A.M. Leenders (Mark); E. Waarts (Eric)

    2001-01-01

    textabstractThe purpose of this study is to systematically examine the advantages and disadvantages of different types of family businesses. We distinguish four different types of family businesses based on their family and business orientation: (1) House of Business, (2) Family Money Machine, (3) F

  20. Families in Transition .

    Science.gov (United States)

    Bundy, Michael L., Ed.; Gumaer, James, Ed.

    1984-01-01

    Focuses on disrupted families and the role of the school counselor in helping children adjust. Describes characteristics of healthy families, and discusses the transition to the blended family, effects of divorce groups on children's classroom behavior, counseling children in stepfamilies, single-parent families, and parenting strengths of single…

  1. Competitiveness of Family Businesses

    NARCIS (Netherlands)

    M.A.A.M. Leenders (Mark); E. Waarts (Eric)

    2001-01-01

    textabstractThe purpose of this study is to systematically examine the advantages and disadvantages of different types of family businesses. We distinguish four different types of family businesses based on their family and business orientation: (1) House of Business, (2) Family Money Machine, (3) F

  2. Near periodicity in dynamical systems

    Institute of Scientific and Technical Information of China (English)

    陈文成

    1995-01-01

    The notion of near periodicity is shown to be equivalent to that of weak near periodicity in dynamical systems. A sufficient condition for the positive near periodicity of a point in dynamical systems is given. The structure of nearly periodic dynamical systems is discussed, and a condition is proved to be necessary and sufficient for a dynamical system on a local compact space to be positively nearly periodic.

  3. Periodicity-dependent stiffness of periodic hydrophilic-hydrophobic heteropolymers

    Science.gov (United States)

    Chowdhury, Debashish; Stauffer, Dietrich; Strey, Reinhard

    1999-08-01

    From extensive Monte Carlo simulations of a Larson model of perfectly periodic heteropolymers (PHP) in water, a striking stiffening is observed as the period of the alternating hydrophobic and hydrophilic blocks is shortened. At short period and low temperature needlelike conformations are the stable conformations. As temperature is increased thermal fluctuations induce kinks and bends. At large periods compact oligomeric globules are observed. From the generalized Larson prescription, originally developed for modeling surfactant molecules in aqueous solutions, we find that the shorter the period is the more stretched the PHP is. This novel effect is expected to stimulate polymer synthesis and trigger research on the rheology of aqueous periodic heteropolymer solutions.

  4. THE WAVELET TRANSFORM OF PERIODIC FUNCTION AND NONSTATIONARY PERIODIC FUNCTION

    Institute of Scientific and Technical Information of China (English)

    刘海峰; 周炜星; 王辅臣; 龚欣; 于遵宏

    2002-01-01

    Some properties of the wavelet transform of trigonometric function, periodic function and nonstationary periodic function have been investigated. The results show that the peak height and width in wavelet energy spectrum of a periodic function are in proportion to its period. At the same time, a new equation, which can truly reconstruct a trigonometric function with only one scale wavelet coefficient, is presented. The reconstructed wave shape of a periodic function with the equation is better than any term of its Fourier series. And the reconstructed wave shape of a class of nonstationary periodic function with this equation agrees well with the function.

  5. Periodicity of extinctions in the geologic past.

    Science.gov (United States)

    Raup, D M; Sepkoski, J J

    1984-02-01

    The temporal distribution of the major extinctions over the past 250 million years has been investigated statistically using various forms of time series analysis. The analyzed record is based on variation in extinction intensity for fossil families of marine vertebrates, invertebrates, and protozoans and contains 12 extinction events. The 12 events show a statistically significant periodicity (P less than 0.01) with a mean interval between events of 26 million years. Two of the events coincide with extinctions that have been previously linked to meteorite impacts (terminal Cretaceous and Late Eocene). Although the causes of the periodicity are unknown, it is possible that they are related to extraterrestrial forces (solar, solar system, or galactic).

  6. Three Classes of Newtonian Three-Body Planar Periodic Orbits

    CERN Document Server

    Šuvakov, Milovan

    2013-01-01

    We present the results of a numerical search for periodic orbits of three equal masses moving in a plane under the influence of Newtonian gravity, with zero angular momentum. A topological method is used to classify periodic three-body orbits into families, which fall into four classes, with all three previously known families belonging to one class. The classes are defined by the orbits geometric and algebraic symmetries. In each class we present a few orbits initial conditions, 15 in all; 13 of these correspond to distinct orbits.

  7. Persistence in periodic and almost periodic Lotka-Volterra systems.

    Science.gov (United States)

    Gopalsamy, K

    1984-01-01

    It is shown that a strongly self-regulating (or resource limited) Lotka-Volterra population system can "persist" in a periodic or almost periodic environment if and only if the system tracks the environmental variations.

  8. Photolysis of Periodate and Periodic Acid in Aqueous Solution

    DEFF Research Database (Denmark)

    Sehested, Knud; Kläning, U. K.

    1978-01-01

    The photochemistry of periodate and periodic acid in aqueous solution was studied (i) by quantum yield measurements at low light intensity (ii) by flash photolysis, and (iii) by photolysis of glassy samples at 77 K. The photochemical studies were supplemented with pulse radiolysis studies...... of aqueous periodate solutions and with kinetic studies using stopped-flow technique. In strongly alkaline solution the photodecomposition of periodate proceeds via formation of O– and IVI. At pH

  9. Effects of Green House nursing homes on residents' families.

    Science.gov (United States)

    Lum, Terry Y; Kane, Rosalie A; Cutler, Lois J; Yu, Tzy-Chyi

    2008-01-01

    A longitudinal quasi-experimental study with two comparison groups was conducted to test the effects of a Green House (GH) nursing home program on residents' family members. The GHs are individual residences, each serving 10 elders, where certified nursing assistant (CNA)-level resident assistants form primary relationships with residents and family, family is encouraged to visits, and professionals adapted their roles to support the model. GH family were somewhat less involved in providing assistance to their residents although family contact did not differ among the settings at any time period. GH family were more satisfied with their resident's care and with their own experience as family members, and had no greater family burden. Issues in studying family outcomes are discussed as well as implications for roles of various personnel, including social service and activities staff in a GH model.

  10. Periodic feedback stabilization for linear periodic evolution equations

    CERN Document Server

    Wang, Gengsheng

    2016-01-01

    This book introduces a number of recent advances regarding periodic feedback stabilization for linear and time periodic evolution equations. First, it presents selected connections between linear quadratic optimal control theory and feedback stabilization theory for linear periodic evolution equations. Secondly, it identifies several criteria for the periodic feedback stabilization from the perspective of geometry, algebra and analyses respectively. Next, it describes several ways to design periodic feedback laws. Lastly, the book introduces readers to key methods for designing the control machines. Given its coverage and scope, it offers a helpful guide for graduate students and researchers in the areas of control theory and applied mathematics.

  11. Family Therapy for the "Truncated" Nuclear Family.

    Science.gov (United States)

    Zuk, Gerald H.

    1980-01-01

    The truncated nuclear family consists of a two-generation group in which conflict has produced a polarization of values. The single-parent family is at special risk. Go-between process enables the therapist to depolarize sharply conflicted values and reduce pathogenic relating. (Author)

  12. Strengthening Family Practices for Latino Families

    Science.gov (United States)

    Chartier, Karen G.; Negroni, Lirio K.; Hesselbrock, Michie N.

    2010-01-01

    This study examined the effectiveness of a culturally adapted Strengthening Families Program (SFP) for Latinos to reduce risks for alcohol and drug use in children. Latino families, predominantly Puerto Rican, with a 9- to 12-year-old child and a parent(s) with a substance abuse problem participated in the study. Pre- and post-tests were conducted…

  13. Putting the "family" back into family therapy.

    Science.gov (United States)

    Breunlin, Douglas C; Jacobsen, Elizabeth

    2014-09-01

    In this article, we examine the field of family therapy by drawing a distinction between two forms of practice: Whole Family Therapy (WFT), defined as treating the whole family, and Relational Family Therapy (RFT), defined as working with a subsystem of the family or an individual while retaining a systemic lens. Our thesis is that the practice of WFT has been in decline for some time and steps must be taken to keep it from becoming a defunct practice. We consider the trajectory of WFT and RFT throughout the development of family therapy through reference to the people, the literature, training, and practice patterns associated with family therapy. We remind the reader of the many benefits of WFT and suggest that today WFT is likely to be practiced in conjunction with RFT and individual therapy. Since training of family therapists today is largely located in degree-granting programs, we identify constraints to including WFT in such programs. We conclude by offering suggestions that can enhance a program's ability to train students in WFT. © 2014 FPI, Inc.

  14. Strengthening Family Practices for Latino Families

    Science.gov (United States)

    Chartier, Karen G.; Negroni, Lirio K.; Hesselbrock, Michie N.

    2010-01-01

    This study examined the effectiveness of a culturally adapted Strengthening Families Program (SFP) for Latinos to reduce risks for alcohol and drug use in children. Latino families, predominantly Puerto Rican, with a 9- to 12-year-old child and a parent(s) with a substance abuse problem participated in the study. Pre- and post-tests were conducted…

  15. Knowledge transfer in family business successions

    DEFF Research Database (Denmark)

    Boyd, Britta; Royer, Susanne; Pei, Rong

    2015-01-01

    businesses to the success of transferring strategically valuable knowledge in different business environments to the next generation. Design/methodology/approach – Building on the contingency model of family business succession (Royer et al., 2008) knowledge transfer in family businesses from different...... are compared with each other and conclusions for both cases are drawn. Implications for theory and practice as well as avenues for future research are sketched. Originality/value – The focus of the current study is to gain more insight into long-lived family businesses by comparing two cases over a period......Purpose – Knowledge often is the fundament for strategic competitive advantage. Thus, it is highly relevant to understand better how knowledge is transferred from one generation to the next in family businesses. The purpose of this paper is to link the competitive advantage realisation in family...

  16. The redoubtable ecological periodic table

    Science.gov (United States)

    Ecological periodic tables are repositories of reliable information on quantitative, predictably recurring (periodic) habitat–community patterns and their uncertainty, scaling and transferability. Their reliability derives from their grounding in sound ecological principle...

  17. The redoubtable ecological periodic table

    Science.gov (United States)

    Ecological periodic tables are repositories of reliable information on quantitative, predictably recurring (periodic) habitat–community patterns and their uncertainty, scaling and transferability. Their reliability derives from their grounding in sound ecological principle...

  18. Doubly Resonant Optical Periodic Structure.

    Science.gov (United States)

    Alagappan, G; Png, C E

    2016-02-08

    Periodic structures are well known in various branches of physics for their ability to provide a stopband. In this article, using optical periodic structures we showed that, when a second periodicity--very closed to the original periodicity is introduced, large number of states appears in the stopband corresponding to the first periodicity. In the limit where the two periods matches, we have a continuum of states, and the original stopband completely disappears. This intriguing phenomena is uncovered by noticing that, regardless of the proximities of the two periodicities, there is an array of spatial points where the dielectric functions corresponding to the two periodicities interfere destructively. These spatial points mimic photonic atoms by satisfying the standards equations of quantum harmonic oscillators, and exhibit lossless, atom-like dispersions.

  19. Periodic review and continuous ordering

    OpenAIRE

    Prak, Dennis R.J.; Teunter, Ruud; Riezebos, Jan

    2014-01-01

    There exist many inventory control studies that consider either continuous review & continuous ordering, or periodic review & periodic ordering. Mixtures of the two are hardly ever studied. However, the model with periodic review and continuous ordering is highly relevant in practice, as information on the actual inventory level is not always up to date while making ordering decisions. This paper will therefore consider this case of periodic review and continuous ordering. Assuming zero fixed...

  20. Periodic review and continuous ordering

    NARCIS (Netherlands)

    Prak, Dennis; Teunter, Ruud; Riezebos, Jan

    2015-01-01

    Many inventory control studies consider either continuous review and continuous ordering, or periodic review and periodic ordering. Mixtures of the two are hardly ever studied. However, the model with periodic review and continuous ordering is highly relevant in practice, as information on the actua

  1. Periodic review and continuous ordering

    NARCIS (Netherlands)

    Prak, Dennis R.J.; Teunter, Ruud; Riezebos, Jan

    2014-01-01

    There exist many inventory control studies that consider either continuous review & continuous ordering, or periodic review & periodic ordering. Mixtures of the two are hardly ever studied. However, the model with periodic review and continuous ordering is highly relevant in practice, as information

  2. Periodic review and continuous ordering

    NARCIS (Netherlands)

    Prak, Dennis R.J.; Teunter, Ruud; Riezebos, Jan

    2014-01-01

    There exist many inventory control studies that consider either continuous review & continuous ordering, or periodic review & periodic ordering. Mixtures of the two are hardly ever studied. However, the model with periodic review and continuous ordering is highly relevant in practice, as information

  3. Periodic review and continuous ordering

    NARCIS (Netherlands)

    Prak, Dennis; Teunter, Ruud; Riezebos, Jan

    2015-01-01

    Many inventory control studies consider either continuous review and continuous ordering, or periodic review and periodic ordering. Mixtures of the two are hardly ever studied. However, the model with periodic review and continuous ordering is highly relevant in practice, as information on the

  4. Family ethnic socialization and ethnic identity: a family-driven, youth-driven, or reciprocal process?

    Science.gov (United States)

    Umaña-Taylor, Adriana J; Zeiders, Katharine H; Updegraff, Kimberly A

    2013-02-01

    The current study examined the longitudinal associations between family ethnic socialization and youths' ethnic identity among a sample of Mexican-origin youth (N = 178, Mage = 18.17, SD = .46). Findings from multiple-group cross lagged panel models over a 2-year period indicated that for U.S.-born youth with immigrant parents, the process appeared to be family driven: Youths' perceptions of family ethnic socialization in late adolescence were associated with significantly greater ethnic identity exploration and resolution in emerging adulthood, while youths' ethnic identity during late adolescence did not significantly predict youths' future perceptions of family ethnic socialization. Conversely, for U.S.-born youth with U.S. born parents, youths' ethnic identity significantly predicted their future perceptions of family ethnic socialization but perceptions of family ethnic socialization did not predict future levels of youths' ethnic identity, suggesting a youth-driven process. Findings were consistent for males and females.

  5. Midy's Theorem for Periodic Decimals

    OpenAIRE

    Lewittes, Joseph

    2006-01-01

    The decimal expansion of 1/7 is 0.142857142857..., the block 142857 repeating forever. We call 142857 the period and its length is 6 = 2x3. If the period is broken into 2 pieces each of length 3 which are then added, the result is 142 + 857 = 999; similarly 14 + 28 + 57 = 99. Other periodic decimals show the same phenomenon while others do not. The general question then arises: Let a/N be a fraction with denominator prime to 10, having decimal expansion with period length dk, if the period is...

  6. Periodic orbits around areostationary points in the Martian gravity field

    CERN Document Server

    Liu, Xiaodong; Ma, Xingrui

    2012-01-01

    This study investigates the problem of areostationary orbits around Mars in the three-dimensional space. Areostationary orbits are expected to be used to establish a future telecommunication network for the exploration of Mars. However, no artificial satellites have been placed in these orbits thus far. In this paper, the characteristics of the Martian gravity field are presented, and areostationary points and their linear stability are calculated. By taking linearized solutions in the planar case as the initial guesses and utilizing the Levenberg-Marquardt method, families of periodic orbits around areostationary points are shown to exist. Short-period orbits and long-period orbits are found around linearly stable areostationary points, and only short-period orbits are found around unstable areostationary points. Vertical periodic orbits around both linearly stable and unstable areostationary points are also examined. Satellites in these periodic orbits could depart from areostationary points by a few degree...

  7. Nonparametric Inference for Periodic Sequences

    KAUST Repository

    Sun, Ying

    2012-02-01

    This article proposes a nonparametric method for estimating the period and values of a periodic sequence when the data are evenly spaced in time. The period is estimated by a "leave-out-one-cycle" version of cross-validation (CV) and complements the periodogram, a widely used tool for period estimation. The CV method is computationally simple and implicitly penalizes multiples of the smallest period, leading to a "virtually" consistent estimator of integer periods. This estimator is investigated both theoretically and by simulation.We also propose a nonparametric test of the null hypothesis that the data have constantmean against the alternative that the sequence of means is periodic. Finally, our methodology is demonstrated on three well-known time series: the sunspots and lynx trapping data, and the El Niño series of sea surface temperatures. © 2012 American Statistical Association and the American Society for Quality.

  8. Choosing a Family Doctor

    Science.gov (United States)

    ... nav nav, .header-9#header-section #main-nav, #overlay-menu nav, #mobile-menu, #one-page-nav li . ... editorial staff Home Your Health Resources Doctor Visit Preparation Choosing a Family Doctor Choosing a Family Doctor ...

  9. Loosely coupled class families

    DEFF Research Database (Denmark)

    Ernst, Erik

    2001-01-01

    are expressed using virtual classes seem to be very tightly coupled internally. While clients have achieved the freedom to dynamically use one or the other family, it seems that any given family contains a xed set of classes and we will need to create an entire family of its own just in order to replace one......Families of mutually dependent classes that may be accessed polymor- phically provide an advanced tool for separation of concerns, in that it enables client code to use a group of instances of related classes safely without depending on the exact classes involved. However, class families which...... of the members with another class. This paper shows how to express class families in such a manner that the classes in these families can be used in many dierent combinations, still enabling family polymorphism and ensuring type safety....

  10. Familial lipoprotein lipase deficiency

    Science.gov (United States)

    ... page: //medlineplus.gov/ency/article/000408.htm Familial lipoprotein lipase deficiency To use the sharing features on this page, please enable JavaScript. Familial lipoprotein lipase deficiency is a group of rare genetic ...

  11. Familial Mediterranean fever

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/000363.htm Familial Mediterranean fever To use the sharing features on this page, please enable JavaScript. Familial Mediterranean fever (FMF) is a rare disorder passed down through ...

  12. Importance of Family Routines

    Science.gov (United States)

    ... Structure Your Child's Nighttime Routine . Weekends: Weekends are good times for family togetherness. You might go grocery shopping as a family, visit museums and zoos, do chores​ that everyone participates in, go on ...

  13. Family Practice Consultation.

    African Journals Online (AJOL)

    gauge the patients' opin:on on the family doctors' role regardirg prayer ... family practice consultation arc presented. ... patients' expectation of prayer and ..... New developments and frequently asked questions covering - high and low BMI, ...

  14. MSUD Family Support Group

    Science.gov (United States)

    ... Group The MSUD Family Support Group is a non-profit 501 (c)(3) organization for those with MSUD ... Family Support Group is a 501(c)(3) non-profit organization with no paid staff. Funds are needed ...

  15. National Military Family Association

    Science.gov (United States)

    ... 931.6632 info@MilitaryFamily.org © 2016 - National Military Family Association Twitter Facebook Pinterest Instagram Charity Navigator Four Star Charity GuideStar Exchange Better Business Bureau Charity Watch Independent Charity of America nonprofit ...

  16. Familial Episodic Amaurosis

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2004-08-01

    Full Text Available A family with a stereotyped unilateral or bilateral transient visual loss, that recurred many times daily and was associated with childhood epilepsy and familial hemiplegic migraine, is reported from University Hospitals, Geneva, Switzerland.

  17. IGSF9 Family Proteins

    DEFF Research Database (Denmark)

    Hansen, Maria; Walmod, Peter Schledermann

    2013-01-01

    The Drosophila protein Turtle and the vertebrate proteins immunoglobulin superfamily (IgSF), member 9 (IGSF9/Dasm1) and IGSF9B are members of an evolutionarily ancient protein family. A bioinformatics analysis of the protein family revealed that invertebrates contain only a single IGSF9 family gene......, the longest isoforms of the proteins have the same general organization as the neural cell adhesion molecule family of cell adhesion molecule proteins, and like this family of proteins, IGSF9 family members are expressed in the nervous system. A review of the literature revealed that Drosophila Turtle...... facilitates homophilic cell adhesion. Moreover, IGSF9 family proteins have been implicated in the outgrowth and branching of neurites, axon guidance, synapse maturation, self-avoidance, and tiling. However, despite the few published studies on IGSF9 family proteins, reports on the functions of both Turtle...

  18. Family Caregiver Alliance

    Science.gov (United States)

    ... on your schedule. Look for our launch soon! FAMILY CARE NAVIGATOR ─ Click on Your State AL AK ... County Smart Patients Caregivers Community In partnership with Family Caregiver Alliance Learn more Caregiver Research Veterans suffer ...

  19. Periodic Fever: A Review on Clinical, Management and Guideline for Iranian Patients - Part II

    OpenAIRE

    Ahmadinejad, Zahra; Mansouri, Sedigeh; Ziaee, Vahid; Aghighi, Yahya; Moradinejad, Mohammad-Hassan; Fereshteh-Mehregan, Fatemeh

    2014-01-01

    Periodic fever syndromes are a group of diseases characterized by episodes of fever with healthy intervals between febrile episodes. In the first part of this paper, we presented a guideline for approaching patients with periodic fever and reviewed two common disorders with periodic fever in Iranian patients including familial Mediterranean fever (FMF) and periodic fever syndromes except for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA). In this part, we revi...

  20. Positive periodic solutions of delayed periodic Lotka-Volterra systems

    Energy Technology Data Exchange (ETDEWEB)

    Lin Wei [Laboratory of Nonlinear Mathematics Science, Institute of Mathematics, Fudan University, Shanghai 200433 (China)]. E-mail: weilin@fudan.edu.cn; Chen Tianping [Laboratory of Nonlinear Mathematics Science, Institute of Mathematics, Fudan University, Shanghai 200433 (China)]. E-mail: tchen@fudan.edu.cn

    2005-01-17

    In this Letter, for a general class of delayed periodic Lotka-Volterra systems, we prove some new results on the existence of positive periodic solutions by Schauder's fixed point theorem. The global asymptotical stability of positive periodic solutions is discussed further, and conditions for exponential convergence are given. The conditions we obtained are weaker than the previously known ones and can be easily reduced to several special cases.

  1. Family and Enterprise

    OpenAIRE

    Fletcher, Denise Elaine

    2006-01-01

    This chapter provides a review of the different approaches that have been utilised to examine the link between family and enterprise. Definitional issues are addressed, as are rationalist and ‘systems’ ways of evaluating the relationship between family and enterprise. Criticism is made of the effects of rationalist thinking on studies of family businesses which have tended to create duality and polarity in our understanding of the family-enterprise relationship. It is argued that a developm...

  2. Family Obligations in Denmark

    DEFF Research Database (Denmark)

    Koch-Nielsen, Inger

    How is the balance in obligations between the Family and the Danish Welfare State? Can we observe a trend to shift the responsibility back to the family? This booklet intends to sketch the legal framework around the division of responsibilities between the Family and the state and to analyse...... to what extent and where the unit of rights and obliagations is the individual and where it is the family or household....

  3. Family Obligations in Denmark

    DEFF Research Database (Denmark)

    Koch-Nielsen, Inger

    How is the balance in obligations between the Family and the Danish Welfare State? Can we observe a trend to shift the responsibility back to the family? This booklet intends to sketch the legal framework around the division of responsibilities between the Family and the state and to analyse...... to what extent and where the unit of rights and obliagations is the individual and where it is the family or household....

  4. Periodic Hamiltonian hierarchies and non-uniqueness of superpotentials

    Indian Academy of Sciences (India)

    PARTHA MANDAL; ABHIJIT BANERJEE

    2017-01-01

    In this article, a family of periodic quantum Hamiltonians, that is subject to a closure condition is considered. In the context of the factorization method, we address the question of non-uniqueness of the governing superpotentials and study an alternative factorization to generate new hierarchies of potentials.

  5. Recognition of resonance type in periodically forced oscillators

    NARCIS (Netherlands)

    Broer, H.W.; Holtman, S.J.; Vegter, G.

    2010-01-01

    This paper deals with families of periodically forced oscillators undergoing a Hopf-Neimarck-Sacker bifurcation. The interest is in the corresponding resonance sets, regions in parameter space for which subharmonics occur. It is a classical result that the local geometry of these sets in the non-deg

  6. Strengths of Remarried Families.

    Science.gov (United States)

    Knaub, Patricia Kain; And Others

    1984-01-01

    Focuses on remarried families' (N=80) perceptions of family strengths, marital satisfaction, and adjustment to the remarried situation. Results indicated that although most would like to make some changes, scores on the measurements used were high. A supportive environment was the most important predictor of family strength and success. (JAC)

  7. Family systems and fertility

    NARCIS (Netherlands)

    Moenkediek, Bastian

    2016-01-01

    This thesis studies the role of regional family organization principles, so called family systems, for explaining fertility behaviours in different parts of Europe. Studying family systems and its impact on fertility is important, because many aspects of societal life, such as the organization of we

  8. Families in Transition.

    Science.gov (United States)

    Britton, Patti O., Ed.; McGee, Michael, Ed.

    1987-01-01

    This issue of "Emphasis" deals with families in transition, providing some model programs for the new family and some historical perspectives on how families have developed over time. Articles include: (1) "Nostalgia on the Right" (Nancy Theriot); (2) "Heart to Heart" (Nancy Harrington-MacLennan); (3) "The Media Get the Message" (Janet Alyn); (4)…

  9. Books in the Family.

    Science.gov (United States)

    Swinger, Alice K.

    1989-01-01

    Opportunities for parents to encourage reading in the family are noted and ways to enhance the reading experience are discussed, including writing letters to book characters, singing combined with reading aloud, supplementing school subjects with enjoyable reading, sharing books at family gatherings, and using family experiences for book…

  10. Single Mothers "Do" Family

    Science.gov (United States)

    Nelson, Margaret K.

    2006-01-01

    This paper explores how single mothers both incorporate others into family life (e.g., when they ask others to care for their children) and simultaneously "do families" in a manner that holds out a vision of a "traditional" family structure. Drawing on research with White, rural single mothers, the author explores the manner in which these women…

  11. Exponential dichotomy of nonautonomous periodic systems in terms of the boundedness of certain periodic Cauchy problems

    Directory of Open Access Journals (Sweden)

    Dhaou Lassoued

    2013-04-01

    Full Text Available We prove that a family of $q$-periodic continuous matrix valued function ${A(t}_{tin mathbb{R}}$ has an exponential dichotomy with a projector $P$ if and only if $int_0^t e^{imu s}U(t,sPds$ is bounded uniformly with respect to the parameter $mu$ and the solution of the Cauchy operator Problem $$displaylines{ dot{Y}(t=-Y(tA(t+ e^{i mu t}(I-P ,quad tgeq s cr Y(s=0, }$$ has a limit in $mathcal{L}(mathbb{C}^n$ as s tends to $-infty$ which is bounded uniformly with respect to the parameter $mu$. Here, ${ U(t,s: t, sinmathbb{R}}$ is the evolution family generated by ${A(t}_{tin mathbb{R}}$, $mu$ is a real number and q is a fixed positive number.

  12. [Periodic breathing with periodic oxygen variation in infancy].

    Science.gov (United States)

    von Czettritz, G; Bax, R T; Eckardt, T; Springer, S; Emmrich, P

    1996-01-01

    Oxycardiorespirographies, recording arterial oxygen saturation (SaO2), breathing movements, heart rate and ECG with a mean recording time of 22.3 hours, were performed on 85 preterm (mean postconceptional age: 38 weeks) and 81 term infants (mean postconceptional age 42.4 weeks). 83% of the preterm infants showed periodic breathing (PB), in 97% of them this was accompanied by periodic variations of arterial oxygen saturation (PVO). Periodic breathing occurred in 61% of the term infants, 84% of them showed PVO during periodic breathing. The mean variation of oxygen saturation was between 92.8 and 96.8% (+/- 1.7) for preterm and between 92.9 and 96.0% (+/- 2.2) for term infants. In some infants the peak to peak amplitude of the SaO2 cycles was up to 22%, sometimes a further fall of SaO2 occurred. There was a strong correlation of the PVO both at the beginning and end of the episode as well as with the PB-cycle periodicity itself. The fall of the oxygen saturation occurred 3.1 to 7.8 s after the beginning of the first apnea of an episode of periodic breathing, the minimum SaO2 was reached approximately 4.2 to 8.6 s later. This periodic rapid fall of SaO2 from a high oxygenation level cannot be explained by the apneas of a rather short duration during periodic breathing. It is discussed that PVO during periodic breathing may be caused by an ideopathic right to left shunting across fetal circulation pathways which occurs intermittently and periodically. This mechanism could-via patterns of reaction exhibited during the fetal and neonatal time period-lead to acute hypoxemia, as found in apparently life threatening events (ALTE) and as postulated in sudden infant death (SID).

  13. The ''EVA'' trial: evaluation of the efficacy of diagnostic methods (mammography, ultrasound, MRI) in the secondary and tertiary prevention of familial breast cancer. Preliminary results after the first half of the study period; Die ''EVA''-Studie: Evaluierung der Leistungsfaehigkeit diagnostischer Verfahren (Mammographie, Sonographie, MRT) zur sekundaeren und tertiaeren Praevention des familiaeren Mammakarzinoms - Zwischenergebnisse nach der ersten Haelfte der Foerderungsperiode

    Energy Technology Data Exchange (ETDEWEB)

    Kuhl, C.K.; Schrading, S.; Morakkabati, N.; Leutner, C.; Schild, H. [Radiologische Universitaetsklinik, Bonn (Germany); Weigel, S.; Tombach, B.; Nordhoff, D.; Heindel, W. [Inst. fuer Klinische Radiologie der Univ. Muenster, Muenster (Germany); Nuessle-Kuegele, K.; Arand, B.; Rieber, A.; Brambs, H.J. [Abt. fuer Roentgendiagnostik der Univ. Ulm, Ulm (Germany); Sittek, H.; Reiser, M. [Inst. fuer Klinische Radiologie des Klinikums der Univ. Muenchen-Grosshadern, Muenchen (Germany)

    2005-06-01

    Purpose: to investigate the respective diagnostic accuracies of the different breast imaging modalities, i. e., mammography (Mx), high-frequency breast ultrasound (US), and dynamic contrast-enhanced breast (MRI) regarding the early diagnosis of familial (hereditary) breast cancer. Materials and methods: a prospective, non-randomized controlled clinical multi-center trial is performed at 4 academic tertiary care centers in Germany (Ulm, Muenchen/Grosshadern, Muenster and Bonn) for a total period of 4 years, sponsored by the German Cancer Aid. The protocol consists of semiannual clinical visits and breast ultrasound, and annual bilateral two-view Mx, US and MRI. Imaging studies were first analyzed independently, then Mx was read in conjunction with US, followed by Mx combined with MRI, and finally, all three imaging modalities were read in synopsis. We present the concept and first results of this trial. Results: so far, 748 screening rounds are available for analysis in 613 women. A total of 12 breast cancers have been identified, with 11/12 cases in the pTis or pT1/N0 stage. The mean size of detected invasive cancers was 7 mm. A total of 19 benign lesions were biopsied due to false-positive imaging diagnoses. The breast cancer detection rates were: Mx: 5/12 (42%), US 3/12 (25%), MRI 10/12 (83%), and the positive predictive values: Mx 5/17 (29%), US 3/15 (30%), and MRI 10/23 (43%). Conclusion: the preliminary data suggest that early diagnosis of familial breast cancer is feasible by intensified surveillance, in particular with the addition of MRI. (orig.)

  14. Return times at periodic points in random dynamics

    Science.gov (United States)

    Haydn, Nicolai; Todd, Mike

    2017-01-01

    We prove a quenched limiting law for random measures on subshifts at periodic points. We consider a family of measures {≤ft\\{{{μω}\\right\\}}ω \\in Ω } , where the ‘driving space’ Ω is equipped with a probability measure which is invariant under a transformation θ. We assume that the fibred measures {μω} satisfy a generalised invariance property and are ψ-mixing. We then show that for almost every ω the return times to cylinders A n at periodic points are in the limit compound Poisson distributed for a parameter ϑ which is given by the escape rate at the periodic point.

  15. Family traditions and generations.

    Science.gov (United States)

    Schneiderman, Gerald; Barrera, Maru

    2009-01-01

    Currently, traditional family values that have been passed down through generations appear to be at risk. This has significant implications for the stability and health of individuals, families, and communities. This article explores selected issues related to intergenerational transmission of family values and cultural beliefs, with particular reference to Western culture and values that are rooted in Jewish and Christian traditions. It also examines family values and parenting styles as they influence the developing perspective of children and the family's adaptation to a changing world.

  16. Repelling periodic points of given periods of rational functions

    Institute of Scientific and Technical Information of China (English)

    CHANG Jianming; FANG Mingliang

    2006-01-01

    Let R(z) be a rational function of degree d ≥ 2. Then R(z) has at least one repelling periodic point of given period k ≥ 2, unless k = 4 and d=2, or k= 3 and d ≤ 3, or k=2 and d≤8. Examples show that all exceptional cases occur.

  17. From Periodic Properties to a Periodic Table Arrangement

    Science.gov (United States)

    Besalú, Emili

    2013-01-01

    A periodic table is constructed from the consideration of periodic properties and the application of the principal components analysis technique. This procedure is useful for objects classification and data reduction and has been used in the field of chemistry for many applications, such as lanthanides, molecules, or conformers classification.…

  18. From Periodic Properties to a Periodic Table Arrangement

    Science.gov (United States)

    Besalú, Emili

    2013-01-01

    A periodic table is constructed from the consideration of periodic properties and the application of the principal components analysis technique. This procedure is useful for objects classification and data reduction and has been used in the field of chemistry for many applications, such as lanthanides, molecules, or conformers classification.…

  19. On the Period-Amplitude and Amplitude-Period Relationships

    Science.gov (United States)

    Wilson, Robert M.; Hathaway, David H.

    2008-01-01

    Examined are Period-Amplitude and Amplitude-Period relationships based on the cyclic behavior of the 12-month moving averages of monthly mean sunspot numbers for cycles 0.23, both in terms of Fisher's exact tests for 2x2 contingency tables and linear regression analyses. Concerning the Period-Amplitude relationship (same cycle), because cycle 23's maximum amplitude is known to be 120.8, the inferred regressions (90-percent prediction intervals) suggest that its period will be 131 +/- 24 months (using all cycles) or 131 +/- 18 months (ignoring cycles 2 and 4, which have the extremes of period, 108 and 164 months, respectively). Because cycle 23 has already persisted for 142 months (May 1996 through February 2008), based on the latter prediction, it should end before September 2008. Concerning the Amplitude-Period relationship (following cycle maximum amplitude versus preceding cycle period), because cycle 23's period is known to be at least 142 months, the inferred regressions (90-percent prediction intervals) suggest that cycle 24's maximum amplitude will be about less than or equal to 96.1 +/- 55.0 (using all cycle pairs) or less than or equal to 91.0 +/- 36.7 (ignoring statistical outlier cycle pairs). Hence, cycle 24's maximum amplitude is expected to be less than 151, perhaps even less than 128, unless cycle pair 23/24 proves to be a statistical outlier.

  20. The concept of the family and its function

    Directory of Open Access Journals (Sweden)

    Denada Keçiçi

    2016-07-01

    Full Text Available The family in the broadest sense includes family in the strict sense and people of the opposite gender. There is no accepted definition of the notion of family, since its very concept has undergone continuous changes in time and space (Omari, 2010, 28. If we refer to historical periods, we see that the concept of family has been different compared to its contemporary meaning. Originally a family relationship was based on consanguinity. The family in the narrow sense: derives from marriage, members are united because of the legal ties of marriage (husband and wife, and blood (parent-child. Unlike the sociological concept in the legal sense, family represents the group of persons among which the legal provisions predict rights and obligations. The family is normally a group of people related by marriage; extramarital cohabitation, or gender related, among whom the law or they themselves determine their rights and obligations.

  1. Asteroid family ages

    CERN Document Server

    Spoto, Federica; Knezevic, Zoran

    2015-01-01

    A new family classification, based on a catalog of proper elements with $\\sim 384,000$ numbered asteroids and on new methods is available. For the $45$ dynamical families with $>250$ members identified in this classification, we present an attempt to obtain statistically significant ages: we succeeded in computing ages for $37$ collisional families. We used a rigorous method, including a least squares fit of the two sides of a V-shape plot in the proper semimajor axis, inverse diameter plane to determine the corresponding slopes, an advanced error model for the uncertainties of asteroid diameters, an iterative outlier rejection scheme and quality control. The best available Yarkovsky measurement was used to estimate a calibration of the Yarkovsky effect for each family. The results are presented separately for the families originated in fragmentation or cratering events, for the young, compact families and for the truncated, one-sided families. For all the computed ages the corresponding uncertainties are pro...

  2. Inside the Family Firm

    DEFF Research Database (Denmark)

    Bennedsen, Morten; Nielsen, Kasper; Pérez-González, Francisco

    2005-01-01

    This paper uses a unique dataset from Denmark to investigate (1) the role of family characteristics in corporate decision making, and (2) the consequences of these decisions on firm performance. We focus on the decision to appoint either a family or an external chief executive officer (CEO). We...... show that a departing CEO's family characteristics have a strong predictive power in explaining CEO succession decisions: family CEOs are more frequently selected the larger the size of the family, the higher the ratio of male children and when the departing CEOs had only had one spouse. We...... then analyze the impact of family successions on performance. We overcome endogeneity and omitted variables problems of previous papers in the literature by using the gender of a departing CEO's first-born child as an instrumental variable (IV) for family successions. This is a plausible IV as male first...

  3. Inside the Family Firm

    DEFF Research Database (Denmark)

    Bennedsen, Morten; Nielsen, Kasper; Pérez-González, Francisco

    2005-01-01

    This paper uses a unique dataset from Denmark to investigate (1) the role of family characteristics in corporate decision making, and (2) the consequences of these decisions on firm performance. We focus on the decision to appoint either a family or an external chief executive officer (CEO). We...... show that a departing CEO's family characteristics have a strong predictive power in explaining CEO succession decisions: family CEOs are more frequently selected the larger the size of the family, the higher the ratio of male children and when the departing CEOs had only had one spouse. We...... then analyze the impact of family successions on performance. We overcome endogeneity and omitted variables problems of previous papers in the literature by using the gender of a departing CEO's first-born child as an instrumental variable (IV) for family successions. This is a plausible IV as male first...

  4. Wartime Stress: Family Adjustment to Loss

    Science.gov (United States)

    1981-07-31

    behaviors are merely normal coping with an abnormal situation, then the reunion/reintegration period may be even more difficult for all family members. THE...Avenue Philadelphia PA 19122 New Haven CT 06520 (Distribution, p. 4) Dr. Irwin G. Sarason University of Washington Department of Pychology Seattle WA 98]95

  5. Latino Periodicals: A Selection Guide.

    Science.gov (United States)

    Guerena, Salvador, Ed.; Pisano, Vivian M., Ed.

    This guide is a collection development tool of national scope for librarians who need in-depth coverage of Latino periodicals suitable for public, school, and academic libraries. Periodicals evaluated include general interest and popular magazines and newspapers that appeal to Spanish-speaking, bilingual, and English-speaking library patrons and…

  6. Family governance practices and teambuilding : Paradox of the enterprising family

    NARCIS (Netherlands)

    Berent-Braun, M.M.; Uhlaner, L.M.

    2012-01-01

    This paper explores the relationship between family governance practices and financial performance of the business and family assets of business-owning families. A business-owning family that shares a focus on preserving and growing wealth as a family is defined as the enterprising family. Results o

  7. 24 CFR 982.515 - Family share: Family responsibility.

    Science.gov (United States)

    2010-04-01

    ... 24 Housing and Urban Development 4 2010-04-01 2010-04-01 false Family share: Family responsibility... Assistance Payment § 982.515 Family share: Family responsibility. (a) The family share is calculated by subtracting the amount of the housing assistance payment from the gross rent. (b) The family rent to owner is...

  8. Opening Doors: Understanding School and Family Influences on Family Involvement

    Science.gov (United States)

    Carlisle, Erin; Stanley, Lindsey; Kemple, Kristen Mary

    2005-01-01

    Family involvement in schooling can benefit young children, teachers, and families. Family involvement in schools can be influenced by both school-related and family-related factors. School-related factors include teachers' attitudes toward families, and school and teacher expectations. Family-related factors include ethnicity, prior school…

  9. Family governance practices and teambuilding : Paradox of the enterprising family

    NARCIS (Netherlands)

    Berent-Braun, M.M.; Uhlaner, L.M.

    2012-01-01

    This paper explores the relationship between family governance practices and financial performance of the business and family assets of business-owning families. A business-owning family that shares a focus on preserving and growing wealth as a family is defined as the enterprising family. Results o

  10. Family governance practices and teambuilding : Paradox of the enterprising family

    NARCIS (Netherlands)

    Berent-Braun, M.M.; Uhlaner, L.M.

    2012-01-01

    This paper explores the relationship between family governance practices and financial performance of the business and family assets of business-owning families. A business-owning family that shares a focus on preserving and growing wealth as a family is defined as the enterprising family. Results

  11. Steady periodic gravity waves with surface tension

    CERN Document Server

    Walsh, Samuel

    2009-01-01

    In this paper we consider two-dimensional, stratified, steady water waves propagating over an impermeable flat bed and with a free surface. The motion is assumed to be driven by capillarity (that is, surface tension) on the surface and a gravitational force acting on the body of the fluid. We prove the existence of global continua of classical solutions that are periodic and traveling. This is accomplished by first constructing a 1-parameter family of laminar flow solutions, $\\mathcal{T}$, then applying bifurcation theory methods to obtain local curves of small amplitude solutions branching from $\\mathcal{T}$ at an eigenvalue of the linearized problem. Each solution curve is then continued globally by means of a degree theoretic theorem in the spirit of Rabinowitz. Finally, we complement the degree theoretic picture by proving an alternate global bifurcation theorem via the analytic continuation method of Dancer.

  12. Family dynamics and family psychotherapy of psychosomatic.

    Science.gov (United States)

    Wirsching, M; Stierlin, H

    1979-01-01

    Family therapy of psychosomatic disorders is oftern difficult and comparable to the therapy of psychotic patients. Nonetheless, the results published today by authors such as Minuchin and Selvini and our own experiences are promising indeed. We have found that what seemed to be a deep-rooted psychic structure changed rapidly and enduringly if the relationship field changed. Amelioration of symptoms is in many cases easily attained if they are understood in their function within a relational system. Also, we regard the system or family approach as a chance for medical practice. The general practioner who usually deals with family systems has, in our view, an ideal position to bring about change if he uses his authority and trust properly. He has to obtain a positive, not pathology-oriented view and should use family and social resources in spite of engaging in an often fruitless and endless contact with the designated patient, which only serves to maintain and even to increase the homeostatic lock of the family system.

  13. Geometrical families of mechanically stable granular packings

    Science.gov (United States)

    Gao, Guo-Jie; Blawzdziewicz, Jerzy; O'Hern, Corey S.

    2009-12-01

    We enumerate and classify nearly all of the possible mechanically stable (MS) packings of bidipserse mixtures of frictionless disks in small sheared systems. We find that MS packings form continuous geometrical families, where each family is defined by its particular network of particle contacts. We also monitor the dynamics of MS packings along geometrical families by applying quasistatic simple shear strain at zero pressure. For small numbers of particles (N16 , we observe an increase in the period and random splittings of the trajectories caused by bifurcations in configuration space. We argue that the ratio of the splitting and contraction rates in large systems will determine the distribution of MS-packing geometrical families visited in steady state. This work is part of our long-term research program to develop a master-equation formalism to describe macroscopic slowly driven granular systems in terms of collections of small subsystems.

  14. Fewer Babies in Poor Families Are Overweight: CDC

    Science.gov (United States)

    ... percent in that period. The WIC program helps low-income pregnant women, new mothers and children up to ... for Disease Control and Prevention. And children in low-income families are at particular risk of both a ...

  15. Family psychology: Past and future reflections on the field.

    Science.gov (United States)

    Parke, Ross D

    2017-04-01

    Prominent issues in the field of family psychology during my term as editor (1998-2003) of this journal were briefly noted, including a focus on marital issues, divorce, remarriage and family conflict. Parenting, attachment and parent-child relationships were also significant topics in this period. Special sections of the journal focused on cultural variations, families and the law, families and religion, and family routines and rituals. Several neglected issues that need more attention in the future were noted. These include the need to recognize the embeddedness of families in socioecological contexts, the importance of monitoring the impact of secular changes on families, and the value and limitations of viewing family psychology as a separate field. Other topics for a future agenda include the challenge of defining "family" in the midst of changing family forms, the effects of technological change on families, and the challenges of integrating biological research into the family psychology agenda. A multilevel bio-social approach to family research was recommended. (PsycINFO Database Record (c) 2017 APA, all rights reserved).

  16. Optical Periodicity Analysis of 3C 446 using Period04

    Indian Academy of Sciences (India)

    Fei Guo; Hao Jing Zhang

    2014-09-01

    All the data of the blazar 3C446 at 8, 4.8, 14 and 22 GHz, presented in publications from 1977 to 2006, have been compiled to generate light curves. The light curves show violent activity of 3C446. Using Period04 analysis method, we have found that there is a period of 7.2 yr, which is consistent with the results that we found using wavelet analysis method. We get the instability region as = 123.83.

  17. Secular trends in family dinner frequency among adolescents

    OpenAIRE

    Walton, Kathryn; Kleinman, Ken P.; Rifas-Shiman, Sheryl L; Horton, Nicholas J; Matthew W. Gillman; Field, Alison E.; S. Bryn Austin; Neumark-Sztainer, Dianne; Haines, Jess

    2016-01-01

    Background: Eating meals, particularly dinner, with family members has been found to be associated with improved dietary intake, lower prevalence of disordered eating behaviors, lower levels of substance abuse, and improved academic outcomes among adolescents. Limited research has examined how the frequency of family meals has changed over time. The objective of this study was to examine secular trends in family dinner frequency over a 12-year period using a large, nation-wide sample of adole...

  18. Familial pituitary tumor syndromes.

    Science.gov (United States)

    Elston, Marianne S; McDonald, Kerrie L; Clifton-Bligh, Roderick J; Robinson, Bruce G

    2009-08-01

    The vast majority of pituitary tumors are benign and occur sporadically; however, they can still result in significant morbidity and even premature mortality through mass effects and hormone dysfunction. The etiology of sporadic tumors is still poorly understood; by contrast, advances have been made in our understanding of familial pituitary adenoma syndromes in the past decade. Currently, four genes are known to be associated with familial pituitary tumor syndromes: MEN1, CDKN1B, PRKAR1A and AIP. The first three genes are associated with a variety of extrapituitary pathologies, for example, primary hyperparathyroidism with multiple endocrine neoplasia type 1, which might aid identification of these syndromes. By contrast, AIP mutations seem to occur in the setting of isolated familial pituitary adenomas, particularly of the growth-hormone-secreting subtype. Awareness and identification of familial pituitary tumor syndromes is important because of potential associated pathologies and important implications for family members. Here, we review the current knowledge of familial pituitary tumor syndromes.

  19. Advancing family psychology.

    Science.gov (United States)

    Fiese, Barbara H

    2016-02-01

    To realize the broad and complex nature of the field of family psychology, I have slightly revised the mission statement of the Journal of Family Psychology (JFP) to capture contemporary scholarship in family psychology and to advance systems perspectives in this top-tier scientific journal. Over the next 6 years, I hope that authors will consider JFP as an outlet for their best work in the following areas: (1) JFP addresses societal challenges faced by families today; (2) JFP publishes important studies on what makes couple and family relationships work; (3) JFP is a leader in publishing reports that use cutting-edge sophisticated approaches to research design and data analysis; and (4) JFP imparts knowledge about effective therapy and prevention programs relevant to couples and families. The journal is also expanding its publication rate to eight issues per year.

  20. Separation anxiety in families with emerging adults.

    Science.gov (United States)

    Kins, Evie; Soenens, Bart; Beyers, Wim

    2013-06-01

    In several developmental theories, separation anxiety has been identified as an important feature of close interpersonal relationships. Most often, separation anxiety has been examined in the context of mother-child dyads in infancy. Increasingly, however, it is recognized that separation anxiety is also relevant in other relationships (e.g., the father-child relationship) and in later developmental periods (e.g., adolescence and emerging adulthood). The present study aimed to investigate separation anxiety at the family level in families with emerging adults. By using the Social Relations Model, we aimed to determine the extent to which the actor, the partner, their specific relationships, and the family contribute to separation anxiety in dyadic family relationships. A total of 119 Belgian two-parent families with an emerging adult participated in a round-robin design, in which family members reported on their feelings of separation anxiety toward each other. Findings showed that separation anxiety can be represented as a personal characteristic (i.e., an actor effect) and as a specific feature of the mother-child dyad. Further, findings indicate that separation anxiety is also characteristic of the father-mother marital relationship and of the family climate as a whole. Implications for the meaning of separation anxiety and clinical practice are discussed.

  1. KSF of family business

    OpenAIRE

    Boroš, Petr

    2014-01-01

    Family business is a business in which family members have a large stake in ownership and also a deciding vote on business operation. This thesis deals with what makes the family businesses thriving. The research is conducted using the Good to Great framework by Jim Collins. Based mainly on quantitative and qualitative survey of 8 Czech companies of various fields and sizes, it was discovered that there are some links between the companies' success. These findings yield a set of recommendatio...

  2. Family in contemporary society

    OpenAIRE

    Rabije Murati

    2016-01-01

    The family is part of social change and, as such changes and transform into steps with modern trends of society. Family function in a given society is structured according to the overall changes that occur in all areas of social life, not neglecting family life. The contemporary conditions impose requirements that must be met to move forward with the times that follow. In particular, should highlight the social changes that are related to the growth and advancement of the educational and prof...

  3. Homeless Families' Education Networks: An Examination of Access and Mobilization

    Science.gov (United States)

    Miller, Peter M.

    2011-01-01

    Purpose: This study sought deeper understanding of how sheltered families accessed and mobilized educationally related relationships and resources during periods of homelessness. Such work is posited to be especially relevant considering that there is a growing crisis of family homelessness in the United States and school- and community-based…

  4. Exploring Conditions to Enhance Student/Host Family Interaction Abroad

    Science.gov (United States)

    Knight, Susan M.; Schmidt-Rinehart, Barbara C.

    2010-01-01

    This study investigates the role of task-based learning in the study abroad experience in order to enhance interaction with the host family. Tasks were incorporated into a Family Interaction Journal and implemented under four evolving, though different, conditions over a 5-year period. The conditions were: (1) home campus administered/student…

  5. Rethinking Family Patriarchy and Women's Positions in Presocialist China

    Science.gov (United States)

    Zuo, Jiping

    2009-01-01

    Grounded in the patrilineal family system in presocialist China, this study explores the intersection between generation and gender dimensions of family patriarchy in influencing marital power relations. Data come from the life stories of 80 elderly married individuals, collected during the 2000-2001 period in the city of Beijing. Patterns of male…

  6. Bidders' gains and family control of private target firms

    NARCIS (Netherlands)

    Gonenc, Halit; Hermes, Niels; van Sinderen, Erik

    2013-01-01

    This paper examines the announcement returns of bidders acquiring private firms owned by families versus the returns of bidders acquiring non-family controlled private firms. The sample consists of 391 acquisitions of private targets in seven continental European countries for the period 1997-2008.

  7. Homeless Families' Education Networks: An Examination of Access and Mobilization

    Science.gov (United States)

    Miller, Peter M.

    2011-01-01

    Purpose: This study sought deeper understanding of how sheltered families accessed and mobilized educationally related relationships and resources during periods of homelessness. Such work is posited to be especially relevant considering that there is a growing crisis of family homelessness in the United States and school- and community-based…

  8. The Patterns and Costs of Services Use among Homeless Families

    Science.gov (United States)

    Culhane, Dennis P.; Park, Jung Min; Metraux, Stephen

    2011-01-01

    This study examines families' use of behavioral health hospitalization and foster care placement before, during, and after shelter use, comparing families based on shelter pattern and type of housing exit. Results show that inpatient and foster care services use drops in the homelessness period, but rebounds after exit, regardless of pattern of…

  9. The Periodic Table in Croatia

    Directory of Open Access Journals (Sweden)

    Raos, N.

    2011-12-01

    Full Text Available The Croatian (Yugoslav Academy of Sciences and Arts was the first academy to elect D. I. Mendeleev as its honorary member (1882, whereas the periodic table of the elements has been taught regularly at the Zagreb University since 1888. The early interest of Croatian chemists in the periodic table should be attributed primarily to their pan-Slavic attitude, particularly as proof that Slavic people were able to produce "their own Newtons" (M. V. Lomonosov and D. I. Mendeleev. Such enthusiastic views, however, did not help in analyzing the contribution of Mendeleev and other scientists to the discovery and development of the periodic table of the elements.

  10. Periodic insolation variations on Mars.

    Science.gov (United States)

    Murray, B C; Ward, W R; Yeung, S C

    1973-05-11

    Previously unrecognized insolation variations on Mars are a consequence of periodic variations in eccentricity, first established by the theory of Brouwer and Van Woerkom (1950). Such annual insolation variations, characterized by both 95,000-year and 2,000,000-year periodicities, may actually be recorded in newly discovered layered deposits in the polar regions of Mars. An additional north-south variation in seasonal insolation, but not average annual insolation, exists with 51,000-year and 2,000,000-year periodicities.

  11. The Hoffmeister asteroid family

    Science.gov (United States)

    Carruba, V.; Novaković, B.; Aljbaae, S.

    2017-03-01

    The Hoffmeister family is a C-type group located in the central main belt. Dynamically, it is important because of its interaction with the ν1C nodal secular resonance with Ceres, which significantly increases the dispersion in inclination of family members at a lower semimajor axis. As an effect, the distribution of inclination values of the Hoffmeister family at a semimajor axis lower than its centre is significantly leptokurtic, and this can be used to set constraints on the terminal ejection velocity field of the family at the time it was produced. By performing an analysis of the time behaviour of the kurtosis of the vW component of the ejection velocity field [γ2(vW)], as obtained from Gauss' equations, for different fictitious Hoffmeister families with different values of the ejection velocity field, we were able to exclude that the Hoffmeister family should be older than 335 Myr. Constraints from the currently observed inclination distribution of the Hoffmeister family suggest that its terminal ejection velocity parameter VEJ should be lower than 25 m s-1. Results of a Yarko-YORP Monte Carlo method to family dating, combined with other constraints from inclinations and γ2(vW), indicate that the Hoffmeister family should be 220^{+60}_{-40} Myr old, with an ejection parameter VEJ = 20 ± 5 m s-1.

  12. Family in contemporary society

    Directory of Open Access Journals (Sweden)

    Rabije Murati

    2016-01-01

    Full Text Available The family is part of social change and, as such changes and transform into steps with modern trends of society. Family function in a given society is structured according to the overall changes that occur in all areas of social life, not neglecting family life. The contemporary conditions impose requirements that must be met to move forward with the times that follow. In particular, should highlight the social changes that are related to the growth and advancement of the educational and professional standards, which will increase the overall impact on the family and its function. If you're looking for full responsibility of parents in the upbringing of children then it is necessary to see the conditions in which the family lives. For normal education and the rights of children with special meaning the number of members in the (quantity family. The tendency to a higher standard of economic life, a small number of children in the family and it is more than obvious that fewer family members or less have greater opportunity for parents to pay more attention to their children. One of the main roles of family, no matter where they are located in the city, village, developed or developing countries, by all means participate, intermediates and transfers the moral, social and other values in modern life.

  13. Saturating Sperner families

    CERN Document Server

    Gerbner, Dániel; Lemons, Nathan; Pálvölgyi, Dömötör; Palmer, Cory; Patkós, Balázs

    2011-01-01

    A family $\\cF \\subseteq 2^{[n]}$ saturates the monotone decreasing property $\\cP$ if $\\cF$ satisfies $\\cP$ and one cannot add any set to $\\cF$ such that property $\\cP$ is still satisfied by the resulting family. We address the problem of finding the minimum size of a family saturating the $k$-Sperner property and the minimum size of a family that saturates the Sperner property and that consists only of $l$-sets and $(l+1)$-sets.

  14. The DLGAP family

    DEFF Research Database (Denmark)

    Rasmussen, Andreas H; Rasmussen, Hanne B; Silahtaroglu, Asli

    2017-01-01

    downstream signalling in the neuron. The postsynaptic density, a highly specialized matrix, which is attached to the postsynaptic membrane, controls this downstream signalling. The postsynaptic density also resets the synapse after each synaptic firing. It is composed of numerous proteins including a family...... in the postsynapse, the DLGAP family seems to play a vital role in synaptic scaling by regulating the turnover of both ionotropic and metabotropic glutamate receptors in response to synaptic activity. DLGAP family has been directly linked to a variety of psychological and neurological disorders. In this review we...... focus on the direct and indirect role of DLGAP family on schizophrenia as well as other brain diseases....

  15. Communication in family businesses : Relationships between family and non-family managers

    OpenAIRE

    Grytsaieva, Tetiana; Strandberg, Johan

    2016-01-01

    Problem: Family firms often comprise of a complex web of relationships between family and non-family managers that are active within the business. Family enterprises are also known for their closed communication and decision-making practices. It often occurs that families do not include non-family managers into important business-related discussions and do not consult their decisions with managers from outside of the family. At the same time, research in the area of family business defines th...

  16. 75 FR 1301 - Periodic Reporting

    Science.gov (United States)

    2010-01-11

    ... From the Federal Register Online via the Government Publishing Office POSTAL REGULATORY COMMISSION 39 CFR Part 3050 Periodic Reporting AGENCY: Postal Regulatory Commission. ACTION: Proposed rule; notice of availability of rulemaking petition. SUMMARY: The Postal Service has proposed adjustments to...

  17. Measuring Scars of Periodic Orbits

    CERN Document Server

    Kaplan, L

    1999-01-01

    The phenomenon of periodic orbit scarring of eigenstates of classically chaotic systems is attracting increasing attention. Scarring is one of the most important ``corrections'' to the ideal random eigenstates suggested by random matrix theory. This paper discusses measures of scars and in so doing also tries to clarify the concepts and effects of eigenfunction scarring. We propose a new, universal scar measure which takes into account an entire periodic orbit and the linearized dynamics in its vicinity. This measure is tuned to pick out those structures which are induced in quantum eigenstates by unstable periodic orbits and their manifolds. It gives enhanced scarring strength as measured by eigenstate overlaps and inverse participation ratios, especially for longer orbits. We also discuss off-resonance scars which appear naturally on either side of an unstable periodic orbit.

  18. Autism: a "critical period" disorder?

    Science.gov (United States)

    LeBlanc, Jocelyn J; Fagiolini, Michela

    2011-01-01

    Cortical circuits in the brain are refined by experience during critical periods early in postnatal life. Critical periods are regulated by the balance of excitatory and inhibitory (E/I) neurotransmission in the brain during development. There is now increasing evidence of E/I imbalance in autism, a complex genetic neurodevelopmental disorder diagnosed by abnormal socialization, impaired communication, and repetitive behaviors or restricted interests. The underlying cause is still largely unknown and there is no fully effective treatment or cure. We propose that alteration of the expression and/or timing of critical period circuit refinement in primary sensory brain areas may significantly contribute to autistic phenotypes, including cognitive and behavioral impairments. Dissection of the cellular and molecular mechanisms governing well-established critical periods represents a powerful tool to identify new potential therapeutic targets to restore normal plasticity and function in affected neuronal circuits.

  19. Periodicities in photospheric magnetic flux

    Institute of Scientific and Technical Information of China (English)

    SONG; Wenbin; WANG; Jingxiu

    2006-01-01

    Magnetic field plays an important role in solar structure and activity. In principle, the determination of magnetic flux would provide the best general-purpose index of solar activity. Currently, the periodicity studies corresponding to photospheric magnetic flux (PMF) are very few possibly due to the absence of a uniform flux sequence. In this paper, by using 383 NSO/Kitt Peak magnetic synoptic charts we reconstruct a flux sequence from February 1975 to August 2003 and perform a relatively systemic periodicity analysis with two methods of the Scargle periodogram and the Morlet wavelet transform. As a result, four periods are found at around 1050, 500, 300 and 160 days. We analyze these periods' temporal variabilities in detail and discuss their respective origins briefly.

  20. Family Learning Center Workshops: A Series for Growth and Getting Along Together. The Des Moines Family Learning Project.

    Science.gov (United States)

    Miller, Maureen

    Developed over a two-and-a-half-year period at Des Moines Family Learning Center, this series of 30-week workshops allows parents to share problems and advice, becoming familiar with the ways in which other parents handle family situations. The series is divided into the following three sections: self-esteem, misbehavior, and emotions; creativity,…

  1. Period Integrals and Tautological Systems

    CERN Document Server

    Lian, Bong H; Yau, Shing-Tung

    2011-01-01

    We study period integrals of CY hypersurfaces in a partial flag variety. We construct a holonomic system of differential equations which govern the period integrals. By means of representation theory, a set of generators of the system can be described explicitly. The results are also generalized to CY complete intersections. The construction of these new systems of differential equations have lead us to the notion of a tautological system.

  2. Periodic billiard trajectories in polyhedra

    CERN Document Server

    Bedaride, Nicolas

    2011-01-01

    We consider the billiard map inside a polyhedron. We give a condition for the stability of the periodic trajectories. We apply this result to the case of the tetrahedron. We deduce the existence of an open set of tetrahedra which have a periodic orbit of length four (generalization of Fagnano's orbit for triangles), moreover we can study completly the orbit of points along this coding.

  3. Compact attractors for time-periodic age-structured population models

    Directory of Open Access Journals (Sweden)

    Pierre Magal

    2001-10-01

    Full Text Available In this paper we investigate the existence of compact attractors for time-periodic age-structured models. So doing we investigate the eventual compactness of a class of abstract non-autonomous semiflow (non necessarily periodic. We apply this result to non-autonomous age-structured models. In the time periodic case, we obtain the existence of a periodic family of compact subsets that is invariant by the semiflow, and attract the solutions of the system.

  4. Paralysis periodica paramyotonica caused by SCN4A Arg1448Cys mutation.

    Science.gov (United States)

    Hsu, Wei-Chih; Huang, Yung-Chuan; Wang, Chung-Wei; Hsueh, Chia-Hsiang; Lai, Ling-Ping; Yeh, Jiann-Horng

    2006-06-01

    Paralysis periodica paramyotonica is an overlapping disease that shares the features of paramyotonia characteristic of paramyotonia congenita (PC) and periodic paralysis characteristic of hyperkalemic periodic paralysis. We report the case of a 23-year-old man with paralysis periodica paramyotonica. His father and a younger brother also exhibited a similar phenotype. A SCN4A Arg1448Cys mutation was detected in this family. The affected family members exhibited marked shifts in compound muscle action potential amplitudes on exercise test, and muscle weakness could be induced by potassium loading and cold exposure. This case demonstrates that SCN4A Arg1448Cys can produce paralysis periodica paramyotonica. Other genetic or environmental factors may modulate the manifestation of SCN4A Arg1448Cys mutation.

  5. Paralysis Periodica Paramyotonica Caused by SCN4A Arg1448Cys Mutation

    Directory of Open Access Journals (Sweden)

    Wei-Chih Hsu

    2006-01-01

    Full Text Available Paralysis periodica paramyotonica is an overlapping disease that shares the features of paramyotonia characteristic of paramyotonia congenita (PC and periodic paralysis characteristic of hyperkalemic periodic paralysis. We report the case of a 23-year-old man with paralysis periodica paramyotonica. His father and a younger brother also exhibited a similar phenotype. A SCN4A Arg1448Cys mutation was detected in this family. The affected family members exhibited marked shifts in compound muscle action potential amplitudes on exercise test, and muscle weakness could be induced by potassium loading and cold exposure. This case demonstrates that SCN4A Arg1448Cys can produce paralysis periodica paramyotonica. Other genetic or environmental factors may modulate the manifestation of SCN4A Arg1448Cys mutation.

  6. Jupiter and Saturn Rotation Periods

    CERN Document Server

    Helled, Ravit; Anderson, John D

    2009-01-01

    Anderson & Schubert (2007, Science,317,1384) proposed that Saturn's rotation period can be ascertained by minimizing the dynamic heights of the 100 mbar isosurface with respect to the geoid; they derived a rotation period of 10h 32m 35s. We investigate the same approach for Jupiter to see if the Jovian rotation period is predicted by minimizing the dynamical heights of its isobaric (1 bar pressure level) surface using zonal wind data. A rotation period of 9h 54m 29s is found. Further, we investigate the minimization method by fitting Pioneer and Voyager occultation radii for both Jupiter and Saturn. Rotation periods of 9h 55m 30s and 10h 32m 35s are found to minimize the dynamical heights for Jupiter and Saturn, respectively. Though there is no dynamical principle requiring the minimization of the dynamical heights of an isobaric surface, the successful application of the method to Jupiter lends support to its relevance for Saturn. We derive Jupiter and Saturn rotation periods using equilibrium theory in ...

  7. The Rafita asteroid family

    Science.gov (United States)

    Aljbaae, S.; Carruba, V.; Masiero, J. R.; Domingos, R. C.; Huaman, M.

    2017-01-01

    The Rafita asteroid family is an S-type group located in the middle main belt, on the right side of the 3J:-1A mean-motion resonance. The proximity of this resonance to the family left side in semi-major axis caused many former family members to be lost. As a consequence, the family shape in the (a, 1/D) domain is quite asymmetrical, with a preponderance of objects on the right side of the distribution. The Rafita family is also characterized by a leptokurtic distribution in inclination, which allows the use of methods of family age estimation recently introduced for other leptokurtic families such as Astrid, Hansa, Gallia, and Barcelona. In this work we propose a new method based on the behavior of an asymmetry coefficient function of the distribution in the (a, 1/D) plane to date incomplete asteroid families such as Rafita. By monitoring the time behavior of this coefficient for asteroids simulating the initial conditions at the time of the family formation, we were able to estimate that the Rafita family should have an age of 490 ± 200 Myr, in good agreement with results from independent methods such as Monte Carlo simulations of Yarkovsky and Yorp dynamical induced evolution and the time behaviour of the kurtosis of the sin (i) distribution. Asteroids from the Rafita family can reach orbits similar to 8% of the currently known near Earth objects. ≃1% of the simulated objects are present in NEO-space during the final 10 Myr of the simulation, and thus would be comparable to objects in the present-day NEO population.

  8. Resilience in families with a child with cancer.

    Science.gov (United States)

    Greeff, Abraham Petrus; Vansteenwegen, Alfons; Geldhof, Annelies

    2014-10-01

    The aim of this study was to identify and explore resilience factors associated with family adaption after a child had been diagnosed with cancer. Using a cross-sectional survey research design, parents (n = 26), and children (n = 25) from the same families independently completed six self-report questionnaires, as well as responded to an open-ended question about those qualities that helped their family through the period following the diagnosis. The most significant results came from the children's data. According to these results, connectedness within the family, the experience of control over life events, family routines, positive, and supportive communication, redefinition of crisis situations, and lastly, a passive appraisal of crisis situations, were positively linked to better family adaptation. The identified factors should be strengthened and developed in families finding themselves in a similar situation.

  9. Family functioning and the adolescent mother: a systems approach.

    Science.gov (United States)

    Nathanson, M; Baird, A; Jemail, J

    1986-01-01

    This study examines unwanted adolescent pregnancy and early childbearing within the context of the family system. Fifty pregnant adolescents and their families were interviewed prenatally and again during the postpartum period. Utilizing the concepts of structural family theory and therapy as described by Minuchin, certain characteristics of family style and structure of organization were rated. These family variables were then related to aspects of the adolescent mother's adaptation postpartum. Boundaries, in terms of degree of intrusiveness and differentiation, were related to such variables as whether the adolescent is maintained in the household and to her continuing relationship with the baby's father. Similarly, the family's style of dealing with conflict was related to the relationship between the adolescent parents, among other outcome variables. Implications of the findings both for working with these families and for further research are discussed, and issues are raised about hypothesized relationships between independent and dependent variables which were not borne out.

  10. Introducing the World Health Organization Postpartum Family Planning Compendium.

    Science.gov (United States)

    Sonalkar, Sarita; Gaffield, Mary E

    2017-01-01

    The postpartum period offers multiple opportunities for healthcare providers to assist with family planning decision making. However, there are also many changing factors during the first year after delivery that can affect family planning choices. Given that several different documents have addressed WHO guidance on postpartum family planning, the electronic WHO Postpartum Family Planning Compendium (http://srhr.org/postpartumfp) has been introduced. This resource integrates essential guidance on postpartum family planning for clinicians, program managers, and policy makers. The development of the Compendium included consultations with family planning experts, key international stakeholders, and web developers. Once the website had been created, user testing by family planning experts allowed for improvements to be made before the official launch. Future directions are adaptation of the website into a mobile application that can be more easily integrated to low-resource settings, and translation of the content into French and Spanish.

  11. Moving family-centered care forward: Bereaved fathers' perspectives.

    Science.gov (United States)

    Davies, Betty; Baird, Jennifer; Gudmundsdottir, Maria

    2013-05-01

    This paper describes the key behaviors of "excellent" pediatric healthcare providers - a term used by fathers of children with complex, life-threatening illness to describe providers who consistently and effectively engage in family-centered care for children and their families. Using interview data from a multi-site grounded theory study of 60 fathers with a deceased child, five behaviors were identified: getting to know the family as individuals, talking about non-healthcare related topics, connecting in a human-human relationship, including parents as team members, and applying specialized knowledge to help the family. These behaviors are consistent with the goals of family-centered care, but they are inconsistently practiced, resulting in less-than-optimal care for children and their families during periods of crisis and vulnerability. A renewed focus on relationship building and interactions with families is needed, as well as a re-evaluation of the training of pediatric healthcare providers.

  12. Rotational properties of Maria asteroid family

    CERN Document Server

    Kim, Myung-Jin; Moon, Hong-Kyu; Byun, Yong-Ik; Brosch, Noah; Kaplan, Murat; Kaynar, Suleyman; Uysal, Omer; Guzel, Eda; Behrend, Raoul; Yoon, Joh-Na; Mottola, Stefano; Hellmich, Stephan; Hinse, Tobias C; Eker, Zeki; Park, Jang-Hyun

    2013-01-01

    Maria family is regarded as an old-type (~3 +/- 1 Gyr) asteroid family which has experienced substantial collisional and dynamical evolution in the Main-belt. It is located nearby the 3:1 Jupter mean motion resonance area that supplies Near-Earth asteroids (NEAs) to the inner Solar System. We carried out observations of Maria family asteroids during 134 nights from 2008 July to 2013 May, and derived synodic rotational periods for 51 objects, including newly obtained periods of 34 asteroids. We found that there is a significant excess of fast and slow rotators in observed rotation rate distribution. The two-sample Kolmogorov-Smirnov test confirms that the spin rate distribution is not consistent with a Maxwellian at a 92% confidence level. From correlations among rotational periods, amplitudes of lightcurves, and sizes, we conclude that the rotational properties of Maria family asteroids have been changed considerably by non-gravitational forces such as the YORP effect. Using a lightcurve inversion method (Kaa...

  13. Closure Issues with Families.

    Science.gov (United States)

    Craig, Steven E.; Bischof, Gary H.

    Closure of the counseling relationship constitutes both an ending and a beginning. Although closure signifies the ending of the present counseling relationship, many family counselors conceptualize closure as the start of a working relationship between counselor and family that may be summoned in future times of crisis or during a difficult life…

  14. Selecting Family Interventions.

    Science.gov (United States)

    Watts, Richard E.

    Just as counseling approaches designed for individuals have their theory-specific techniques, family counseling approaches also have theory-specific interventions and strategies. Whatever presenting problem the family brings to counseling, one or more of four essential components (communication, problem solving, roles and boundaries) is typically…

  15. Assessment of Troubled Families.

    Science.gov (United States)

    Combs-Orme, Terri; Thomas, Katherine H.

    1997-01-01

    Tests the utility of four standardized instruments used in assessing 105 families that sought services in a juvenile corrections setting for their teenage children. Results demonstrate that parents and adolescents can complete standardized assessment instruments and that the information provided can help in understanding distressed families. (RJM)

  16. Family Reunion Health Guide

    Science.gov (United States)

    ... be a sign of kidney disease. Encourage your family to keep their blood pressure at or below the number set by their ... Share NKDEP brochures about diabetes and high blood pressure. Encourage family members to take the information with them when ...

  17. Privacy in the family

    NARCIS (Netherlands)

    Newell, Bryce; Metoyer, Cheryl A.; Moore, Adam D.

    2015-01-01

    Popular access to increasingly sophisticated forms of electronic surveillance technologies has altered the dynamics of family relationships. Monitoring, mediated and facilitated by practices of both covert and overt electronic surveillance, has changed the nature of privacy within the family. In thi

  18. Narrative Family Therapy.

    Science.gov (United States)

    Walsh, William M.; Keenan, Robert

    1997-01-01

    States that narrative family therapy is informed by social constructionism and postmodern worldviews, and is a relatively significant departure from mainstream psychotherapy. Discusses the use of narrative family therapy. Uses the story of Adam and Eve in the Garden of Eden as an example. (MKA)

  19. Marriage or Family Therapy.

    Science.gov (United States)

    Haley, Jay

    1984-01-01

    Reviews the differences between family therapy and marriage counseling in terms of professional organization, theory, and practice. Suggests that training in marriage therapy does not appear adequate for family therapy. The goal of the therapy field should be more consensus in theory and a single profession of therapists. (JAC)

  20. Family Report 2011

    NARCIS (Netherlands)

    Freek Bucx

    2011-01-01

    Original title: Gezinsrapport 2011. Between 2007 and 2010 the Netherlands had its first ever Minister for Youth and Family. The position of the family in modern society is a subject of considerable debate, not just at political and policy level, but also in society itself. Voices are frequently hea

  1. Familie og arv

    DEFF Research Database (Denmark)

    Nielsen, Linda

    1995-01-01

    Familie og arv, familie, arv, børn, ægteskab, ægtefælle, skilsmisse, formuefællesskab, forældremyndighed, fælleseje, særråden, særeje, død, uskiftet bo, underholdspligt, samliv, tvangsarv, deling...

  2. Golden Matrix Families

    Science.gov (United States)

    Fontaine, Anne; Hurley, Susan

    2011-01-01

    This student research project explores the properties of a family of matrices of zeros and ones that arises from the study of the diagonal lengths in a regular polygon. There is one family for each n greater than 2. A series of exercises guides the student to discover the eigenvalues and eigenvectors of the matrices, which leads in turn to…

  3. Internationalization of Family Businesses

    DEFF Research Database (Denmark)

    Boyd, Britta; Hollensen, Svend; Goto, Toshio

    2010-01-01

    -depth qualitative interviews reveal the driving forces on both sides and show how the psychic distance can be reduced between the different parent firms including the joint venture (JV) child. The purpose of this study is to compare equal split or equity joint ventures of non-family and family firms regarding...

  4. America's Family Time Famine.

    Science.gov (United States)

    Mattox, Jr., William R.

    1990-01-01

    Parents spend increasingly less time with their children because of the pressures of dual careers and single parenthood. Economic pressures and social values have affected sharing of family time. Studies show both parents and children consider spending time together the most important element in improving family life. (BC)

  5. Marriage or Family Therapy.

    Science.gov (United States)

    Haley, Jay

    1984-01-01

    Reviews the differences between family therapy and marriage counseling in terms of professional organization, theory, and practice. Suggests that training in marriage therapy does not appear adequate for family therapy. The goal of the therapy field should be more consensus in theory and a single profession of therapists. (JAC)

  6. The Family Victivallaceae

    NARCIS (Netherlands)

    Plugge, C.M.; Zoetendal, E.G.

    2014-01-01

    The family Victivallaceae is a member of the order Victivallales and holds only two cultured representatives: Victivallis vadensis strain CelloT and the uncharacterized strain NML 080035. They are Gram-negative, nonmotile, and anaerobic. Within the family as well as the order, cloned 16S rRNA gene s

  7. The family Cyclobacteriaceae

    Digital Repository Service at National Institute of Oceanography (India)

    AnilKumar, P.; Srinivas, T.N.R.

    , Rhodonellum, and Shivajiella Phylogenetic analysis based on 16S rRNA gene sequences clearly clustered all the genera into the family Cyclobacteriaceae Members of the family are known to have a wide range of morphological properties like cell shape, size...

  8. Why the Family?

    NARCIS (Netherlands)

    Ferracioli, L.

    2015-01-01

    Among the most pressing philosophical questions occupying those interested in the ethics of the family is why should parents, as opposed to charity workers or state officials, raise children? In their recent Family Values, Brighouse and Swift have further articulated and strengthen their own

  9. Changing Families, Changing Workplaces

    Science.gov (United States)

    Bianchi, Suzanne M.

    2011-01-01

    American families and workplaces have both changed dramatically over the past half-century. Paid work by women has increased sharply, as has family instability. Education-related inequality in work hours and income has grown. These changes, says Suzanne Bianchi, pose differing work-life issues for parents at different points along the income…

  10. Rotational properties of the Maria asteroid family

    Science.gov (United States)

    Kim, M.; Choi, Y.; Moon, H.; Byun, Y.; Brosch, N.; Kaplan, M.; Kaynar, S.; Uysal, O.; Guzel, E.; Behrend, R.; Yoon, J.; Mottola, S.; Hellmich, S.; Hinse, T.; Eker, Z.; Park, J.

    2014-07-01

    Introduction: The Maria family is regarded as an old-type (˜3 ± 1 Gyr) [1] asteroid family which has experienced substantial collisional and dynamical evolution in the main belt. It is located near the 3:1 Jupiter mean-motion resonance area that supplies near-Earth asteroids (NEAs) to the inner Solar System. Observations: We carried out observations of Maria family asteroids in 134 nights from July 2008 to May 2013 using 0.5-m to 2-m class telescopes at seven observatories in the northern hemisphere, and derived synodic rotational periods for 51 objects, including new periods for 34 asteroids [2]. Results: We found that there is a significant excess of fast and slow rotators in the observed rotation-rate distribution. From the correlations among rotational periods, the amplitudes of lightcurves, and the sizes, we conclude that the rotational properties of the Maria family asteroids have been changed considerably by non-gravitational forces such as the YORP effect. Using the lightcurve inversion method [3,4], we successfully determined pole orientations for 13 Maria members, and found an excess of prograde spins over retrograde spins with a ratio (N_p/N_r) of 3. This implies that the retrograde rotators could have been ejected by the 3:1 resonance into the inner Solar System since the formation of the Maria family. We estimate that approximately 37 to 75 Maria family asteroids larger than 1 km have entered the near-Earth space as per 100 Myr [2].

  11. Familial malignant melanoma

    Energy Technology Data Exchange (ETDEWEB)

    Kopf, A.W.; Hellman, L.J.; Rogers, G.S.; Gross, D.F.; Rigel, D.S.; Friedman, R.J.; Levenstein, M.; Brown, J.; Golomb, F.M.; Roses, D.F.; Gumport, S.L.

    1986-10-10

    Characteristics associated with familial compared with nonfamilial malignant melanoma were assessed. These data were obtained from consecutive prospectively completed questionnaires on 1169 cases of cutaneous malignant melanoma. Of these, 69 patients indicated a positive family history for this cancer. Among the various clinical and histological variables compared, those that significantly correlated with the familial occurrence of malignant melanoma include younger age at first diagnosis, smaller diameter of the lesion, lower Clark level, decreased frequency of nonmelanoma skin cancer, and reduced prevalence of noncutaneous cancer. Increased awareness of malignant melanoma among family members could account for some of these observations. Identification of the familial variety of malignant melanoma has practical implications concerning early detection and prompt intervention.

  12. Genetics of familial melanoma

    DEFF Research Database (Denmark)

    Aoude, Lauren G; Wadt, Karin A W; Pritchard, Antonia L

    2015-01-01

    Twenty years ago, the first familial melanoma susceptibility gene, CDKN2A, was identified. Two years later, another high-penetrance gene, CDK4, was found to be responsible for melanoma development in some families. Progress in identifying new familial melanoma genes was subsequently slow; however......, with the advent of next-generation sequencing, a small number of new high-penetrance genes have recently been uncovered. This approach has identified the lineage-specific oncogene MITF as a susceptibility gene both in melanoma families and in the general population, as well as the discovery of telomere...... maintenance as a key pathway underlying melanoma predisposition. Given these rapid recent advances, this approach seems likely to continue to pay dividends. Here, we review the currently known familial melanoma genes, providing evidence that most additionally confer risk to other cancers, indicating...

  13. Maintenance of family networks

    DEFF Research Database (Denmark)

    marsico, giuseppina; Chaudhary, N; Valsiner, Jaan

    2015-01-01

    Families are social units that expand in time (across generations) and space (as a geographically distributed sub-structures of wider kinship networks). Understanding of intergenerational family relations thus requires conceptualization of communication processes that take place within a small...... collective of persons linked with one another by a flexible social network. Within such networks, Peripheral Communication Patterns set the stage for direct everyday life activities within the family context. Peripheral Communication Patterns are conditions where one family network member (A) communicates...... manifestly with another member (B) with the aim of bringing the communicative message to the third member (C) who is present but is not explicitly designated as the manifest addressee of the intended message. Inclusion of physically non-present members of the family network (elders living elsewhere, deceased...

  14. Negotiating Family Tracking

    DEFF Research Database (Denmark)

    Sonne Damkjær, Maja; Albrechtslund, Anders; Bøge, Ask Risom

    This presentation explores the question: What motivates the use of tracking technologies in families, and how does the use transform the relations between parent and child? The purpose is to investigate why tracking technologies are used in families and how these technologies potentially change...... the relation between parents and children. The use of tracking technologies in families implicate negotiations about the boundaries of trust and intimacy in parent-child relations which can lead to strategies of resistance or modification (Fotel and Thomsen, 2004; Rooney, 2010; Steeves and Jones, 2010......). In the presentation, we report from a qualitative study that focuses on intergenerational relations. The study draws on empirical data from workshops with Danish families as well as individual and group interviews. We aim to gain insights about the sharing habits and negotiations in intimate family relations...

  15. Fighting for the family

    DEFF Research Database (Denmark)

    Heiselberg, Maj Hedegaard

    2016-01-01

    The article explores how military deployment affects the everyday lives of Danish soldiers’ families. By approaching the challenges faced by soldiers and their partners from an anthropological perspective of the family, the article provides new insights into the social consequences of military...... deployment and the processes of militarization at home. Drawing on ethnographic examples from recent fieldwork among women, children, and soldiers at different stages of deployment, the article demonstrates how soldiers and their families attempt to live up to ideals about parenthood and family by creating...... ‘relational spaces’ that allow them to preserve intimate relationships despite geographical distances. Besides dealing with the practical responsibilities of everyday life, soldiers’ partners also fight to maintain the sense of closeness associated with normative ideals about family relations and a ‘good...

  16. Work-family conflict, family-supportive supervisor behaviors (FSSB), and sleep outcomes.

    Science.gov (United States)

    Crain, Tori L; Hammer, Leslie B; Bodner, Todd; Kossek, Ellen Ernst; Moen, Phyllis; Lilienthal, Richard; Buxton, Orfeu M

    2014-04-01

    Although critical to health and well-being, relatively little research has been conducted in the organizational literature on linkages between the work-family interface and sleep. Drawing on conservation of resources theory, we use a sample of 623 information technology workers to examine the relationships between work-family conflict, family-supportive supervisor behaviors (FSSB), and sleep quality and quantity. Validated wrist actigraphy methods were used to collect objective sleep quality and quantity data over a 1 week period of time, and survey methods were used to collect information on self-reported work-family conflict, FSSB, and sleep quality and quantity. Results demonstrated that the combination of predictors (i.e., work-to-family conflict, family-to-work conflict, FSSB) was significantly related to both objective and self-report measures of sleep quantity and quality. Future research should further examine the work-family interface to sleep link and make use of interventions targeting the work-family interface as a means for improving sleep health.

  17. Family health and family physician’s influence on prevention of psychoactive substances abuse

    Directory of Open Access Journals (Sweden)

    Lapčević Mirjana

    2010-01-01

    Full Text Available The family, as the basic social unit, has a decisive role in the health and disease of its members. It is the primary unit where health needs are formed and solved. By its own resources the family independently resolves about 75% of the total health requirements. In the paper the authors study family characteristics which influence family health and diseases, indicators of family health and the scale of life values. Also, the study evaluates social factors, communication and the influence of the usage of psychoactive substances on family health and the quality of family life. To form the personality of a child three factors are most significant: love, the feeling of safety and the presence of harmonious relationship between the parents. Life harmony in a family also depends on the quality of structural components of the personality and the interaction of motivation of its members. Early childhood determines the future personality of the adult person. At that period, habits and partially attitudes are formed. In harmonious family relationships the parents are the role model to children. Verbal and non-verbal communication enrich the relationship among people and enable efforts in supporting understanding, compassion and care for others by mutual agreement. On the scale of life values of Serbian citizens health holds the first position. Immediately following the health issue is good relationship in the family. As healthcare is not only the task of healthcare services, but also of each individual, family and the society as a whole, it is on healthcare personnel to educate the citizens how to preserve and improve their own health and the health of their family by a continual healthcare and education. Above all, this concerns avoidance of bad habits, such as smoking, immoderate alcohol consumption, narcotic abuse, physical inactivity, hypercaloric nutrition, etc. Also, it is significant to make an early recognition of disease symptoms and to turn for

  18. [Family health and family physician's influence on prevention psychoactive substances abuse].

    Science.gov (United States)

    Lapčević, Mirjana; Dimitrijević, Ivan

    2010-01-01

    The family, as the basic social unit, has a decisive role in the health and disease of its members. It is the primary unit where health needs are formed and solved. By its own resources the family independently resolves about 75% of the total health requirements. In the paper the authors study family characteristics which influence family health and diseases, indicators of family health and the scale of life values. Also, the study evaluates social factors, communication and the influence of the usage of psychoactive substances on family health and the quality of family life. To form the personality of a child three factors are most significant: love, the feeling of safety and the presence of harmonious relationship between the parents. Life harmony in a family also depends on the quality of structural components of the personality and the interaction of motivation of its members. Early childhood determines the future personality of the adult person. At that period, habits and partially attitudes are formed. In harmonious family relationships the parents are the role model to children. Verbal and non-verbal communication enrich the relationship among people and enable efforts in supporting understanding, compassion and care for others by mutual agreement. On the scale of life values of Serbian citizens health holds the first position. Immediately following the health issue is good relationship in the family. As healthcare is not only the task of healthcare services, but also of each individual, family and the society as a whole, it is on healthcare personnel to educate the citizens how to preserve and improve their own health and the health of their family by a continual healthcare and education. Above all, this concerns avoidance of bad habits, such as smoking, immoderate alcohol consumption, narcotic abuse, physical inactivity, hypercaloric nutrition, etc. Also, it is significant to make an early recognition of disease symptoms and to turn for help to the chosen

  19. Family Reconstruction: The Family within-a Group Experience.

    Science.gov (United States)

    Satir, Virginia; And Others

    1988-01-01

    Presents Virginia Satir's Family Reconstruction, a group therapy experience that blends and extends her 1982 process therapy and 1983 conjoint family therapy approaches. Describes the family reconstruction process, the review of family reconstruction data in the pregroup interview, the family reconstruction process in the counseling group, and the…

  20. Family Reconstruction: The Family within-a Group Experience.

    Science.gov (United States)

    Satir, Virginia; And Others

    1988-01-01

    Presents Virginia Satir's Family Reconstruction, a group therapy experience that blends and extends her 1982 process therapy and 1983 conjoint family therapy approaches. Describes the family reconstruction process, the review of family reconstruction data in the pregroup interview, the family reconstruction process in the counseling group, and the…