scholarly journals Lactic Acidosis in Children – A Varied Presentation

2016 ◽  
Vol 06 (03) ◽  
pp. 206-208
Author(s):  
Ira Shah
Keyword(s):  
Lactic Acidosis ◽  
Metabolic Disorders ◽  
Acid Oxidation ◽  
Urinary Organic Acid ◽  
Pyruvate Metabolism ◽  
Second Child ◽  
First Child ◽  
Acute Rhabdomyolysis ◽  
Lactic Acidemia ◽  
Serum Ammonia

AbstractPrimary lactic acidemias represent a family of disorders of pyruvate metabolism or defects in the respiratory chain. However, lactic acidosis may also be seen in metabolic disorders such as organic acidemias, urea cycle defects, and fatty acid oxidation defects, which can be easily excluded by serum ammonia estimation, urinary organic acid estimation, and quantification of plasma amino acids. The classical presentation of a patient with primary lactic acidemia is growth retardation, ataxia, stroke, and increased lactic acid levels in the blood and cerebrospinal fluid. Patients may also present with cerebral edema, acute rhabdomyolysis, cardiac arrhythmias, cardiomyopathy, coma, and neurodegeneration. We present three cases of lactic acidemia with varied presentation. The first child presented at 5 years with recurrent hematemesis with hypoglycemia. The second child presented at 11 years of age with recurrent episodes of unconsciousness. The third child presented at one and a half months with convulsions.

?Cerebral? lactic acidosis: defects in pyruvate metabolism with profound brain damage and minimal systemic acidosis

1988 ◽  
Vol 147 (1) ◽  
pp. 10-14 ◽  
Author(s):  
G. K. Brown ◽  
E. A. Haan ◽  
D. M. Kirby ◽  
R. D. Scholem ◽  
J. E. Wraith ◽  
...  
Keyword(s):  
Lactic Acidosis ◽  
Brain Damage ◽  
Pyruvate Metabolism

INHERITED METABOLIC DISORDERS AND HEART DISEASES

2019 ◽  
Author(s):  
Vjekoslav Krželj ◽  
Ivana Čulo Čagalj
Keyword(s):  
Metabolic Disorders ◽  
Metabolic Diseases ◽  
Technological Development ◽  
Heart Diseases ◽  
Glycogen Storage ◽  
Acid Oxidation ◽  
Lysosomal Storage ◽  
Glycogen Storage Diseases ◽  
Coronary Diseases ◽  
Inherited Metabolic Disorders

Inherited metabolic disorders can cause heart diseases, cardiomyopathy in particular, as well as cardiac arrhythmias, valvular and coronary diseases. More than 40 different inherited metabolic disorders can provoke cardiomyopathy, including lysosomal storage disorders, fatty acid oxidation defects, organic acidemias, amino acidopathies, glycogen storage diseases, congenital disorders of glycosylation as well as peroxisomal and mitochondrial disorders. If identified and diagnosed on time, some of congenital metabolic diseases could be successfully treated. It is important to assume them in cases when heart diseases are etiologically undefined. Rapid technological development has made it easier to establish the diagnosis of these diseases. This article will focus on common inherited metabolic disorders that cause heart diseases, as well as on diseases that might be possible to treat.

scholarly journals Tang-Nai-Kang Alleviates Pre-diabetes and Metabolic Disorders and Induces a Gene Expression Switch toward Fatty Acid Oxidation in SHR.Cg-Leprcp/NDmcr Rats

PLoS ONE ◽  
2015 ◽  
Vol 10 (4) ◽  
pp. e0122024 ◽  
Author(s):  
Linyi Li ◽  
Hisae Yoshitomi ◽  
Ying Wei ◽  
Lingling Qin ◽  
Jingxin Zhou ◽  
...  
Keyword(s):  
Gene Expression ◽  
Fatty Acid ◽  
Fatty Acid Oxidation ◽  
Metabolic Disorders ◽  
Acid Oxidation

scholarly journals AMPK as a mediator of hormonal signalling

2009 ◽  
Vol 44 (2) ◽  
pp. 87-97 ◽  
Author(s):  
Chung Thong Lim ◽  
Blerina Kola ◽  
Márta Korbonits
Keyword(s):  
Protein Synthesis ◽  
Fatty Acid ◽  
Metabolic Disorders ◽  
Energy Homeostasis ◽  
Metabolic Effects ◽  
Whole Body ◽  
Acid Oxidation ◽  
Treatment Of Obesity ◽  
Amp Activated Protein Kinase ◽  
Hormonal Signalling

AMP-activated protein kinase (AMPK) is a key molecular player in energy homeostasis at both cellular and whole-body levels. AMPK has been shown to mediate the metabolic effects of hormones such as leptin, ghrelin, adiponectin, glucocorticoids and insulin as well as cannabinoids. Generally, activated AMPK stimulates catabolic pathways (glycolysis, fatty acid oxidation and mitochondrial biogenesis) and inhibits anabolic pathways (gluconeogenesis, glycogen, fatty acid and protein synthesis), and has a direct appetite-regulating effect in the hypothalamus. Drugs that activate AMPK, namely metformin and thiazolidinediones, are often used to treat metabolic disorders. Thus, AMPK is now recognised as a potential target for the treatment of obesity and associated co-morbidities.

SURVIVAL OF A ONE-POUND FEMALE INFANT BORN IN 1847

PEDIATRICS ◽  
1980 ◽  
Vol 66 (2) ◽  
pp. 253-253
Author(s):  
T. E. C.
Keyword(s):  
Premature Infants ◽  
Literature Search ◽  
Survival Rates ◽  
Newborn Infants ◽  
Female Infant ◽  
Second Child ◽  
The Past ◽  
First Child ◽  
Accepted Practice

After a thorough literature search of the survival rates of premature infants, I believe the infant described below was the smallest to survive until this century.1 I am mindful that the reported birth weights in the past may have been inaccurate because the weighing of newborn infants was not an accepted practice prior to this century. 2 Mrs. A. (aged 30) weaned her first child on the 17th of November 1846, a fortnight after which (1st December) she menstruated naturally. Two days after the catamenia disappeared (7th December), she conceived, having the same sensations post coitu which she felt at her previous conception. At four months she quickened. She was delivered (by a midwife) of her second child, a female, on the 14th of May 1847-on the hundred and fifty-eighth day of gestation. The child had only rudimentary nails, and almost no hair, except a little, of slightly reddish colour, at the lower part of the back of the head. It weighed one pound, and measured eleven inches. It was merely wrapped up at first, laid in a box about a foot long, used by the father (who is a slater) for carrying nails, and set on the kitchen fender, before the fire, to keep it warm. It came on very well, and was subsequently treated very much the same as other children, except perhaps, that it was a little more looked after than usual, being considered a curiosity. She is still of small make but is quite healthy, and takes her food well.

Intergenerational transmission of ethnic identity in mixed native couples in Finland

2019 ◽  
Vol 63 (1) ◽  
pp. 103-123
Author(s):  
Ognjen Obućina ◽  
Jan Saarela
Keyword(s):  
Ethnic Identity ◽  
Parental Education ◽  
Positive Association ◽  
Individual Characteristics ◽  
Parental Education Level ◽  
Second Child ◽  
Ethnic Affiliation ◽  
First Child ◽  
Strong Positive Association ◽  
Using Data

The aim of this study is to analyse the factors determining the ethnic affiliation of children born to interethnic native couples in Finland, using data from couples with one Finnish-speaking and one Swedish-speaking partner, between 1988 and 2014. In addition to individual characteristics of each partner and contextual factors, we also consider the role of couple characteristics. We look at the affiliation of the first child, as well as the combined affiliation of the first two children, in order to analyse how often children from the same parents are affiliated to different ethnicities. Around 60% of first- and second-born children of interethnic couples born between 1988 and 2014 were affiliated to the Swedish-speaking minority. The affiliation of the second child seldom differs from that of the first. Children of mixed couples with a Swedish mother are more likely to be affiliated to the Swedish-speaking community. Boys are more likely than girls to be affiliated to the father’s community, and vice versa. In line with our expectations based on ethnic awareness, preference for cultural plurality and parental aspirations, the multivariate analysis shows a strong positive association between parental education level and the likelihood of the first child being Swedish speaking. The analysis also indicates that bargaining is not an important strategy when choosing a child’s ethnic identity.

LOW FERTILITY INTENTION IN TEHRAN, IRAN: THE ROLE OF ATTITUDES, NORMS AND PERCEIVED BEHAVIOURAL CONTROL

2016 ◽  
Vol 49 (3) ◽  
pp. 292-308 ◽  
Author(s):  
Amir Erfani
Keyword(s):  
Fertility Intentions ◽  
Policy Implications ◽  
Low Fertility ◽  
Second Child ◽  
Normative Pressure ◽  
Factors Influencing ◽  
First Child ◽  
Using Data ◽  
Perceived Constraints

SummaryPersistent low fertility rates are an increasing concern for countries with low fertility like Iran. Informed by the Theory of Planned Behaviour, this study examined the immediate factors influencing fertility intentions, using data from the 2012 Tehran Survey of Fertility Intentions. The findings show that more than half of young married adults in Tehran intend to have no more children. The multivariate analysis results indicate that individuals who view childbearing as being detrimental to their personal life, feel less normative pressure to have a/another child, and believe their childbearing decision is not contingent on the presence of economic resources required for childbearing, are more likely to want no (more) children or to be unsure rather than to want a/another child. Attitudes and normative pressure are dominant factors influencing the intention to have a first child, while the intention to have a second child is mainly affected by attitudes and perceived constraints. The policy implications of the results are discussed.

scholarly journals The High Incidence of Valproate Hepatotoxicity in Infants May Relate to Familial Metabolic Defects

1990 ◽  
Vol 17 (2) ◽  
pp. 145-148 ◽  
Author(s):  
R.E. Appleton ◽  
K. Farrell ◽  
D.A. Applegarth ◽  
J.E. Dimmick ◽  
L.T.K. Wong ◽  
...  
Keyword(s):  
Valproic Acid ◽  
Fatty Acid ◽  
Developmental Delay ◽  
Hepatic Failure ◽  
Metabolic Disorders ◽  
Acid Oxidation ◽  
Toxic Metabolite ◽  
Metabolic Defects ◽  
Intractable Seizures ◽  
High Incidence

ABSTRACT:The incidence of fatal hepatic failure associated with valproic acid (VPA) therapy is highest in children under the age of three years, particularly in those with developmental delay. The pathogenesis of VPA hepatotoxicity is unclear but may relate to the accumulation of a toxic metabolite of VPA which impairs fatty-acid oxidation. We describe two unrelated infants with developmental delay who developed hepatic failure while receiving VPA. Siblings of both children subsequently developed hepatic steatosis and intractable seizures without being exposed to VPA. This suggests that that the two children who developed liver failure when receiving VPA may have had a familial metabolic disorder. Familial metabolic disorders may account partly for the higher incidence of fatal hepatotoxicity described in infants receiving VPA.

scholarly journals Fertility in Real Generations of Russian Women: Trends and Regional Differences

2019 ◽  
Vol 12 (2) ◽  
pp. 59-69
Author(s):  
V. N. Arkhangelskiy
Keyword(s):  
Regional Differences ◽  
Birth Rates ◽  
Second Child ◽  
Younger Women ◽  
Fertility Trends ◽  
Number Of Children ◽  
First Child ◽  
Generational Changes ◽  
The Difference ◽  
Families With Children

The subject of the research is fertility trends in real generations of women in Russia. The relevance of the research stems from the fact that the majority of works devoted to the analysis of the fertility trends and the possible impact of demographic policies thereon in Russia are based on the use of calendar birth rates (total, special, age, total coefficient, etc.) subject to timing fluctuations, e.g. earlier childbirth due to favorable circumstances. The influence of this factor can be bypassed by using birth rates for real generations. The purpose of the paper was to analyze the dynamics of generational changes in birth rates and their regional differences. The results of the analysis showed that after a significant reduction in the average number of children born in the generations of women of the 1960s — early 1970s, the value slightly increased for women of the mid- and late 1970s. and would probably be somewhat higher for women born in the 1980s. The proportion of women who gave birth to at least one child is decreasing hampering the increase in the average number of children born in real generations. On the contrary, an increase in the proportion of women who gave birth to the second and third child contributes to this increase. While the share of those who gave birth to the second child among women who gave birth to the first child in the generations of the late 1970s, despite a significant increase, is lower than among women of the mid‑1950s, the proportion of those who gave birth to the third child among women who gave birth to the second child, is higher than in older generations. The increase in the proportion of women in the generations of the late 1970s who gave birth to the second and third children is to some extent due to more active measures for supporting families with children that are largely focused on supporting second and subsequent births of children. If the implementation of measures for supporting families with children in the 1980s helped to smooth out the difference in the average age of the mother who gave birth to the second and first child from 4.53 years in the generation of women born in 1950 up to 3.31 years in the generation of 1963, then in younger women the difference tended to increase and now it is the maximum for women of 1975. (5.91 years). It is concluded that the policy of encouraging child bearing needs to be continued.

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