BACKGROUND Multiple acyl-CoA dehydrogenase deficiency(MADD)is a disease of rare autosomal recessive disorder.There are three types of MADD.Type I is a neonatalonset form with congenital anomalies.Type II is a neonatal...BACKGROUND Multiple acyl-CoA dehydrogenase deficiency(MADD)is a disease of rare autosomal recessive disorder.There are three types of MADD.Type I is a neonatalonset form with congenital anomalies.Type II is a neonatal-onset form without congenital anomalies.Type III is considered to a milder form and usually responds to riboflavin.However,late-onset form could also be fatal and not responsive to treatments.CASE SUMMARY We report a severe case of a young man with onset type III MADD induced by drugs and strenuous exercise characterized by rhabdomyolysis and liver dysfunction.Urine analysis indicated 12 out of 70 kinds of organic acids like glutaric acid-2 were detected.Serum analysis in genetic metabolic diseases revealed 24 out of 43 tested items were abnormal,revealing the elevation of several acylcarnitines and the reduction of carnitine in the patient.By next generation sequencing technology for gene sequencing related to fatty acid oxidation and carnitine cycle defects,a rare ETFDH gene variant was identified:NM_004453:4:C.1448C>T(p.Pro483 Leu).The patient was diagnosed with lateonset GAII.He was not responsive to riboflavin and progressively worsened into multiple organ failure that finally led to death.CONCLUSION Type III MADD can also be fatal and not responsive to treatments.展开更多
The effect of zinc(Zn) deficiency and excessive bicarbonate on the allocation and exudation of organic acids in plant organs(root, stem, and leaf) and root exudates of two Moraceae plants(Broussonetia papyrifera and M...The effect of zinc(Zn) deficiency and excessive bicarbonate on the allocation and exudation of organic acids in plant organs(root, stem, and leaf) and root exudates of two Moraceae plants(Broussonetia papyrifera and Morus alba) were investigated. Two Moraceae plants were hydroponically grown and cultured in nutrient solution in four different treatments with 0.02 mM Zn or no Zn,combined with no or 10 mM bicarbonate. The variations of organic acids in different plant organs were similar to those of root exudates in the four treatments except B. papyrifera, which was in a treatment that was a combination of 0.02 mM Zn and no bicarbonate. The response characteristics in the production, translocation, and allocation of organic acids in the plant organs and root exudates varied with species and treatments. Organic acids in plant organs and root exudates increased under Zn-deficient conditions,excessive bicarbonate, or both. An increase of organic acids in the leaves resulted in an increase of root-exuded organic acids. B. papyrifera translocated more oxalate and citrate from the roots to the rhizosphere than M. alba under the dual influence of 10 mM bicarbonate and Zn deficiency. Organic acids of leaves may be derived from dark respiration and photorespiration. By comparison, organic acids in stems, roots, and root exudates may be derived from dark respiration and organic acid translocation from the leaves. These results provide evidence for the selective adaptation of plants to environments with low Zn levels or high bicarbonate levels such as a karst ecosystem.展开更多
In?the Canadian Prairies, many soils on organic farms are low in available P, and the only alternative is to use external sources to prevent P nutrient deficiency on these soils. A 3-year (2012 to 2014) field experime...In?the Canadian Prairies, many soils on organic farms are low in available P, and the only alternative is to use external sources to prevent P nutrient deficiency on these soils. A 3-year (2012 to 2014) field experiment was established in spring 2012 on a P-deficient soil near Kelvington, Saskatchewan, Canada, to determine the potential of organic amendments (alfalfa pellets, compost manure, thin stillage and distiller grain dry of wheat), inorganic amendments (rock phosphate granular, rock phosphate fine, wood ash and bone meal ash) and microbial inoculants/products (JumpStart®and MYKE®PRO), applied alone or in a combination with N and/or P commercial fertilizers, in preventing P deficiency and increasing seed yield, N and P uptake of barley. Compared to unfertilized control, N only treatment did not result in any significant increase in seed yield, while application of P alone increased seed yield significantly but to a lesser degree than when both N and P fertilizers were applied together in all 3 years. Rock phosphate did not result in any seed yield benefit, even when applied along with N fertilizer. Wood ash fine increased seed yield of barley significantly only in the presence of N fertilizer, with highest seed yield in the presence of both N + P fertilizers. Seed yield of barley increased moderately with alfalfa pellets, significantly with compost manure, and considerably with distiller grain dry of wheat, but highest seed yield was obtained from thin stillage, which was essentially similar to that obtained from the N + P fertilizer combination. There was no yield benefit from JumpStart or MykePro in any year and only slight benefit from bone meal ash in 2013. The addition of N fertilizer to MykePro or bone meal ash treatments increased seed yield, but highest yield was obtained when both N and P fertilizers were added, suggesting a lack of available P for optimum seed yield. With few exceptions, the response trends of total N and P uptake in seed + straw to the amendments studied were generally similar to those of seed yield. In conclusion, the organic amendment “thin stillage” provided balanced nutrition and produced yield and nutrient uptake of barley similar to balanced N + P fertilizer treatment, and it was closely followed by “distiller grain dry of wheat”, with moderate benefit from compost manure and some benefit from alfalfa pellets. In this extremely P-deficient soil, rock phosphate was not found effective in preventing P deficiency in barley, while wood ash and bone meal ash provided moderate increase in barley yield, with little yield benefit from JumpStart and MykePro, when other nutrients were not limiting in the soil.展开更多
Data gathered from the 1987 National Nutrition Survey in the Philippines provided the opportunity to study the interactions among micronutrient deficiencies and undernutrition in different age groups as basis for prog...Data gathered from the 1987 National Nutrition Survey in the Philippines provided the opportunity to study the interactions among micronutrient deficiencies and undernutrition in different age groups as basis for program targeting. A randomly selected set of 50% of the households (3,200) covered by the national survey served as source of subjects. Results showed that there was a greater proportion of anemia among the undernourished (as judged by weight for age in children and weight for height in adults) (66.0%)than among the adequately nourished (54.6%) (P <0.01 ). However, the observed differencesin the proportion of serum vitamin A deficiency and of goiter among the undernourished compared to the adequately nourished were not significant. Also not significant were the observed higher prevalence of anemia among subjects with acceptable serum vitamin A levels for both adequately nourished and undernourished, and the higher prevalence of vitamin A deficiency among the non-anemics. Again there were no significant differences in the prevalence of anemia among goitrous and non-goitrous subjects, as well as the prevalence of goiter among anemic and non-anemic subjects. Neither were there significant differences in the prevalence of vitamin A deficiency among goitrous and non-goitrous subjects, but there were significant differences in the prevalence of goiter among vitamin A deficient and non-vitamin A defjcient subjects among the 7-14 years old and among pregnant and lactating women.The study concludes that at the national level, there is apparently an interaction between anemia and protein-energy undernutrition and possibly also between goiter and vitamin A deficiency in the high-risk age groups, but between anemia on the one hand and goiter and vitamin A deficiency in the other, perhaps because of clustering in the latter conditions not found in anemia and general undernutrition. These findings may be useful in targeting communities with high prevalence of micronutrient deficiencies by using prevalence of underweight and goiter as indicators for high prevalence of anemia and vitamin A deficiency, respectively展开更多
文摘BACKGROUND Multiple acyl-CoA dehydrogenase deficiency(MADD)is a disease of rare autosomal recessive disorder.There are three types of MADD.Type I is a neonatalonset form with congenital anomalies.Type II is a neonatal-onset form without congenital anomalies.Type III is considered to a milder form and usually responds to riboflavin.However,late-onset form could also be fatal and not responsive to treatments.CASE SUMMARY We report a severe case of a young man with onset type III MADD induced by drugs and strenuous exercise characterized by rhabdomyolysis and liver dysfunction.Urine analysis indicated 12 out of 70 kinds of organic acids like glutaric acid-2 were detected.Serum analysis in genetic metabolic diseases revealed 24 out of 43 tested items were abnormal,revealing the elevation of several acylcarnitines and the reduction of carnitine in the patient.By next generation sequencing technology for gene sequencing related to fatty acid oxidation and carnitine cycle defects,a rare ETFDH gene variant was identified:NM_004453:4:C.1448C>T(p.Pro483 Leu).The patient was diagnosed with lateonset GAII.He was not responsive to riboflavin and progressively worsened into multiple organ failure that finally led to death.CONCLUSION Type III MADD can also be fatal and not responsive to treatments.
基金funded by the National Key Basic Research Program of China under Grant No. 2013CB956701the National Natural Science Foundation of China under Grant No. 31070365Funded by talents introduction of Anqing Normal University (No. 14000100032)
文摘The effect of zinc(Zn) deficiency and excessive bicarbonate on the allocation and exudation of organic acids in plant organs(root, stem, and leaf) and root exudates of two Moraceae plants(Broussonetia papyrifera and Morus alba) were investigated. Two Moraceae plants were hydroponically grown and cultured in nutrient solution in four different treatments with 0.02 mM Zn or no Zn,combined with no or 10 mM bicarbonate. The variations of organic acids in different plant organs were similar to those of root exudates in the four treatments except B. papyrifera, which was in a treatment that was a combination of 0.02 mM Zn and no bicarbonate. The response characteristics in the production, translocation, and allocation of organic acids in the plant organs and root exudates varied with species and treatments. Organic acids in plant organs and root exudates increased under Zn-deficient conditions,excessive bicarbonate, or both. An increase of organic acids in the leaves resulted in an increase of root-exuded organic acids. B. papyrifera translocated more oxalate and citrate from the roots to the rhizosphere than M. alba under the dual influence of 10 mM bicarbonate and Zn deficiency. Organic acids of leaves may be derived from dark respiration and photorespiration. By comparison, organic acids in stems, roots, and root exudates may be derived from dark respiration and organic acid translocation from the leaves. These results provide evidence for the selective adaptation of plants to environments with low Zn levels or high bicarbonate levels such as a karst ecosystem.
文摘In?the Canadian Prairies, many soils on organic farms are low in available P, and the only alternative is to use external sources to prevent P nutrient deficiency on these soils. A 3-year (2012 to 2014) field experiment was established in spring 2012 on a P-deficient soil near Kelvington, Saskatchewan, Canada, to determine the potential of organic amendments (alfalfa pellets, compost manure, thin stillage and distiller grain dry of wheat), inorganic amendments (rock phosphate granular, rock phosphate fine, wood ash and bone meal ash) and microbial inoculants/products (JumpStart®and MYKE®PRO), applied alone or in a combination with N and/or P commercial fertilizers, in preventing P deficiency and increasing seed yield, N and P uptake of barley. Compared to unfertilized control, N only treatment did not result in any significant increase in seed yield, while application of P alone increased seed yield significantly but to a lesser degree than when both N and P fertilizers were applied together in all 3 years. Rock phosphate did not result in any seed yield benefit, even when applied along with N fertilizer. Wood ash fine increased seed yield of barley significantly only in the presence of N fertilizer, with highest seed yield in the presence of both N + P fertilizers. Seed yield of barley increased moderately with alfalfa pellets, significantly with compost manure, and considerably with distiller grain dry of wheat, but highest seed yield was obtained from thin stillage, which was essentially similar to that obtained from the N + P fertilizer combination. There was no yield benefit from JumpStart or MykePro in any year and only slight benefit from bone meal ash in 2013. The addition of N fertilizer to MykePro or bone meal ash treatments increased seed yield, but highest yield was obtained when both N and P fertilizers were added, suggesting a lack of available P for optimum seed yield. With few exceptions, the response trends of total N and P uptake in seed + straw to the amendments studied were generally similar to those of seed yield. In conclusion, the organic amendment “thin stillage” provided balanced nutrition and produced yield and nutrient uptake of barley similar to balanced N + P fertilizer treatment, and it was closely followed by “distiller grain dry of wheat”, with moderate benefit from compost manure and some benefit from alfalfa pellets. In this extremely P-deficient soil, rock phosphate was not found effective in preventing P deficiency in barley, while wood ash and bone meal ash provided moderate increase in barley yield, with little yield benefit from JumpStart and MykePro, when other nutrients were not limiting in the soil.
文摘Data gathered from the 1987 National Nutrition Survey in the Philippines provided the opportunity to study the interactions among micronutrient deficiencies and undernutrition in different age groups as basis for program targeting. A randomly selected set of 50% of the households (3,200) covered by the national survey served as source of subjects. Results showed that there was a greater proportion of anemia among the undernourished (as judged by weight for age in children and weight for height in adults) (66.0%)than among the adequately nourished (54.6%) (P <0.01 ). However, the observed differencesin the proportion of serum vitamin A deficiency and of goiter among the undernourished compared to the adequately nourished were not significant. Also not significant were the observed higher prevalence of anemia among subjects with acceptable serum vitamin A levels for both adequately nourished and undernourished, and the higher prevalence of vitamin A deficiency among the non-anemics. Again there were no significant differences in the prevalence of anemia among goitrous and non-goitrous subjects, as well as the prevalence of goiter among anemic and non-anemic subjects. Neither were there significant differences in the prevalence of vitamin A deficiency among goitrous and non-goitrous subjects, but there were significant differences in the prevalence of goiter among vitamin A deficient and non-vitamin A defjcient subjects among the 7-14 years old and among pregnant and lactating women.The study concludes that at the national level, there is apparently an interaction between anemia and protein-energy undernutrition and possibly also between goiter and vitamin A deficiency in the high-risk age groups, but between anemia on the one hand and goiter and vitamin A deficiency in the other, perhaps because of clustering in the latter conditions not found in anemia and general undernutrition. These findings may be useful in targeting communities with high prevalence of micronutrient deficiencies by using prevalence of underweight and goiter as indicators for high prevalence of anemia and vitamin A deficiency, respectively
