• Title/Summary/Keyword: deficiency of the kidney

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Heterogenous Clinical Manifestations in Adult Patients with Late Diagnosis of Glycogen Storage Disease type Ia (성인기에 진단된 당원병 제 Ia형의 다양한 임상 양상)

  • Kim, Yoo-Mi;Cheon, Chong Kun;Kim, Gu-Hwan;Yoo, Han-Wook
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.15 no.1
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    • pp.9-17
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    • 2015
  • Glycogen storage disease (GSD) type Ia is rare inborn metabolic disorder, caused by glucose-6-phosphatase deficiency. It characterized by hepatomegaly, hypoglycemia, lactic acidosis, hypertriglyceridemia, and hyperuricemia and it is usually manifested in the infantile period. In addition, it is also associated with growth failure, pubertal delay, anemia, platelet dysfunction, osteopenia, and pulmonary hypertension. Hepatocellular adenoma and renal dysfunction are frequent late complications. Delayed diagnosis and inappropriate therapy lead to many complications such as growth failure, osteoporosis, refractory gout, renal failure, hepatocellular carcinoma (HCC), and pulmonary hypertension. Here, two Korean sisters diagnosed with GSD Ia, aged 33 and 36 respectively, were described and compared to recent articles about four adults with late diagnosis of GSD Ia. One sister had typical manifestations of GSD Ia including short stature (height, 145 cm), multiple hepatic adenoma, chronic kidney disease stage IV, and severe osteoporosis, whereas the older sister had normal stature (162 cm), one tiny hepatic nodule, and normal renal function. Direct sequencing of G6PC in two sisters identified a homozygous splicing mutation, c.645G>T, which is a prevalent mutation in Korea. Interestingly, our cases and four adults from recent reports had asymptomatic mild hypoglycemia and various manifestations including renal failure, HCC, fatty liver, or uncontrolled hyperlipidemia. These adult cases represent not only heterogenous phenotype to genotype within family members with GSD Ia but also long-term complications such as gouty arthritis, renal failure, and osteoporosis in untreated adult GSD Ia patients. In addition, lactic academia and hypertriglyceridemia are good markers of GSD Ia to distinguish from metabolic disease.

Disorders of Potassium Metabolism (칼륨 대사 장애)

  • Lee, Joo-Hoon
    • Childhood Kidney Diseases
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    • v.14 no.2
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    • pp.132-142
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    • 2010
  • Hypokalemia usually reflects total body potassium deficiency, but less commonly results from transcellular potassium redistribution with normal body potassium stores. The differential diagnosis of hypokalemia includes pseudohypokalemia, cellular potassium redistribution, inadequate potassium intake, excessive cutaneous or gastrointestinal potassium loss, and renal potassium wasting. To discriminate excessive renal from extrarenal potassium losses as a cause for hypokalemia, urine potassium concentration or TTKG should be measured. Decreased values are indicative of extrarenal losses or inadequate intake. In contrast, excessive renal potassium losses are expected with increased values. Renal potassium wasting with normal or low blood pressure suggests hypokalemia associated with acidosis, vomiting, tubular disorders or increased renal potassium secretion. In hypokalemia associated with hypertension, plasam renin and aldosterone should be measured to differentiated among hyperreninemic hyperaldosteronism, primary hyperaldosteronism, and mineralocorticoid excess other than aldosterone or target organ activation. Hypokalemia may manifest as weakness, seizure, myalgia, rhabdomyolysis, constipation, ileus, arrhythmia, paresthesias, etc. Therapy for hypokalemia consists of treatment of underlying disease and potassium supplementation. The evaluation of hyperkalemia is also a multistep process. The differential diagnosis of hyperkalemia includes pseudohypokalemia, redistribution, and true hyperkalemia. True hyperkalemia associated with decreased glomerular filtration rate is associated with renal failure or increased body potassium contents. When glomerular filtration rate is above 15 mL/min/$1.73m^2$, plasma renin and aldosterone must be measured to differentiate hyporeninemic hypoaldosteronism, primary aldosteronism, disturbance of aldosterone action or target organ dysfunction. Hyperkalemia can cause arrhythmia, paresthesias, fatigue, etc. Therapy for hyperkalemia consists of administration of calcium gluconate, insulin, beta2 agonist, bicarbonate, furosemide, resin and dialysis. Potassium intake must be restricted and associated drugs should be withdrawn.

A Delphi Study For Treatment Standardization of Coldness in hands and Feet (수족냉증 치료 표준화를 위한 델파이 연구)

  • Sun, Seung-Ho;Go, Ho-Yeon;Ko, Seungwoo;Cho, Yoon-Young;Shin, Ji-Hye;Kim, Tae-Hoon;Choi, Dong-Jun;Lee, Jin-Moo;Jang, Jun-Bok;Jung, Ki-Yong;Song, Yun-Kyung;Go, Seong-Gyu;Choi, You-Kyung;Lim, Eun-Mee;Park, Jong-Hyeong;Jeon, Chan-Yong
    • Journal of Society of Preventive Korean Medicine
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    • v.19 no.3
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    • pp.131-140
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    • 2015
  • Objective : The purpose of this study is to figure out the treatment of coldness in hands and Feet by using Delphi survey. Method : This study was approved by institutional review boards at Kyung Hee University. Self recording type delphi survey were sent by e-mail to 55 Korean medicine specialists (19 gynecologist, 36 internal medical doctors) who treat coldness in hands and Feet. And replies were analyzed. As this study is cross sectional study, simple descriptive analysis was applied. Results & Conclusion : Herbal medicine, acupuncture and moxibustion were mainly applied and extract (granule), pharmacopuncture, bee venom pharmacopuncture and cupping therapy were also added case by case. Treatment was done twice a weak for 4 months. Cupping therapy was done at GB21 and SI11. Bee venom pharmacopuncture and Kidney yang deficiency pharmacopuncture were applied at ST36, GB21, CV4 and LR3. As extract (granule) Ijungtang, Dangguisayeokgaosuyusaenggangtang and Dangguisayeoktang were mainly used and as herbal medicine formula Palmijihwangwon, Bojungikgitang, Ijungtang, Danggwisayeok-gaosuyusaenggangtang and Danggwisayeoktang were mainly prescribed. Comprehensive treatment of acupuncture, moxibustion and herbal medicine was considered to have more effective and more rapid result than single treatment of herbal medicine have.

Effects of Ca Supplementation on Mineral Metabolism during Pregnancy and Lactation of Calcium-Deficient Young Adult Rats (칼슘섭취 부족 흰쥐에서 임신, 수유기 동안 칼슘보충 수준이 무기질 대사에 미치는 영향)

  • 박미나;김은애;이연숙
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.33 no.6
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    • pp.958-964
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    • 2004
  • This study examined the effects of maternal calcium intake levels on mineral metabolism during pregnancy and lactation. Five week-old female Sprague-Dawley rats were fed normal Ca (NCa, 0.5%) and low Ca (LCa, 0.15%) diets for 5 weeks during preconception. The low Ca intake group was divided into three groups and fed low Ca (0.15%), normal Ca (0.5%) and high Ca (1.5%) diets for 6 weeks during pregnancy and lactation. All of the rats were mated with normal male rats. The control group was consistently fed a normal Ca diet for 11 weeks. Pups were adjusted to 10 per dam. After weaning, dams and their weaned rats were sacrificed. We measured total protein, albumin, alkaline phosphatase and mineral content in serum and weight, length, breaking force, ash and mineral content in bone and kidney. There was no significant difference in weight gain and food intake among the groups. Serum total protein and albumin were in the normal range but slightly decreased with a low Ca diet during preconception. Calcium supplementation above normal level increased bone weight, Ca content and breaking force in femur of maternal and their weaned rats. However, high maternal Ca intake significantly decreased Mg and Fe contents in maternal serum and bone and Mg and Zn contents in serum of weaned rats. Also, it induced Ca, P and Mg deposit in maternal kidney. Our experimental results suggest that Ca deficiency during preconception may deteriorate bone status of mother and their offsprings. Moreover, high Ca supplementation during pregnancy and lactation may lead to disturb mineral metabolism in bone and other tissues.

『황제내경소문(黃帝內經素問)·칠편대론(七篇大論)』 왕빙 주본(注本)을 통(通)한 운기학설(運氣學說) 관(關)한 연구(硏究)

  • Kim, Gi-Uk;Park, Hyeon-Guk
    • The Journal of Dong Guk Oriental Medicine
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    • v.4
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    • pp.109-140
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    • 1995
  • As we considered in the main subjects, investigations on the theory of 'Doctrine on five elements' motion and six kinds of natural factors(運氣學說)' through 'Wang Bing's Commentary(王氷 注本)' of 'The seven great chapters in The Yellow Emperor's Internal Classic Su Wen' ("黃帝內經素問 七篇大論") are as follows. (1) In The seven great chapters("七篇大論")' Wang Bing supplement theory and in the academic aspects as a interpreter, judging from 'forget(亡)' character. expressed in the 'The missing chapters("素問遺篇")', 'Bonbyung-ron("本病論")' and 'Jabeob-ron(刺法論)', 'The seven great chapters("七篇大論")' must be supplementary work by Wang Bing. Besides, he quoted such forty books as medical books, taoist books, confucianist books, miscellaneous books, etc in the commentary and the contents quoted in the 'Su Wen(素問)' and 'Ling Shu("靈樞")' scripture nearly occupy in the book. As a method of interpreting scripiure as scripture, he edited the order of 'Internal Classic("內經")' ascended from the ancient time and when he compensated for commentary, with exhaustive scholarly mind and by observing the natural phenomena practically and writing the pathology and the methods of treatment. We knew that the book is combined with the study of 'Doctrine on five elements motion and six kinds of natural factors(運氣學說)' (2) When we compare, analyze the similar phrase of 'The seven great chapters in The Yellow Emperor's Internal Classic Su Wen'("黃帝內經素問ㆍ七篇大論") through 'Wang Bing's Commentary(王氷 注本)', he tells abouts organized 'five elements(五行)' and 'heaven's regularly movement(天道運行)' rather than 'Emyangengsangdae-ron("陰陽應象大論")' in 'The seven great chapters("七篇大論")'. Also the 'Ohanunhangdae-ron("五運行大論")' because the repeated sentences with 'Emyangengsangdae-ron("陰陽應象大論")' is long they are omitted. And in the 'Youkmijidae-ron("六微旨大論")', 'Cheonjin ideology(天眞四象)' based on the 'Sanggocheonjin- ron("上古天眞論")', 'Sagijosindae-ron("四氣調神大論")' is written and in the 'Gigoupyondae-ron("氣交變大論")', the syndrome and symptom are explained in detail rather than 'Janggibeobsi-ron("藏氣法時論")', 'Okgijinjang-ron ("玉機眞藏論")' and in the 'Osangieongdae-ron("五常政大論")', the concept of 'five element(五行)' of the 'Gemgwejineon-ron("金櫃眞言論")' is expanded to 'the five elements' motion concept(五運槪念)' and in the 'Youkwonjeonggidae-ron("六元正紀大論")', explanations of 'The five elements' motion and six kinds of natural factors(運氣)' function are mentioned mainly and instead systematic pathology is not revealed rather than 'Emyangengsangdae-ron("陰陽應象大論")'. And in the 'Jijinyodae-ron("至眞要大論")', explanations of the change of atmosphere which correspond to treatment principle by 'The three Yin and Yang(三陰三陽)' as a progressed concepts are revealed. Therefore there are much similarity between the phrase of 'Emyangengsangdae-ron("陰陽應象大論")' and 'chapters of addition(補缺之篇)'. Generally, the doctrine which 'The seven great chapters("七篇大論")' are added by Wang Bing(王氷) is supported because there are more profound concepts rather than the other chapter in 'The seven great chapters("七篇大論")'. (3) When we study Wang Bing's(王氷) 'Pattern on five elements motion and six kinds of natural factors(運氣格局)' in 'The seven great chapter("七篇大論")', in the 'Cheonwongi-dae-ron("天元紀大論")', With 'Cheonjin ideology(天眞思想)' and the concepts of 'Owang(旺)'${\cdot}$'Sang(相)'${\cdot}$'Sa(死)'${\cdot}$'Su(囚)'${\cdot}$'Hu(休)' and 'Cheonbu(天符)'${\cdot}$'Sehwoi(歲會)' are measured time-spacially to the concept of 'Three Sum(三合)' the concept of 'Taeulcheonbu(太乙天符)' is explained. In the 'Ounhangdae-ron("五運行大論")', 'The calender Signs five Sum(天干五合)' is compared to the concepts of 'couples(夫婦)', 'weak-strong(柔强)' and in the 'Youkmijidae-ron("六微旨大論")', 'the relationship of obedience and disobedience(順逆關係)' which conform to the 'energy status(氣位)' change and 'monarch-minister(君相)' position is mentioned. In the 'Gikyobyeondae-ron("氣交變大論")', the concept of 'Sang-duk(相得)', 'Pyungsang(平常)' is emphasized but concrete measurement is mentioned. In the 'Osangieongdae-ron("五常政大論")', the detailed explanation with twenty three 'systemic of the five elements' motion(五運體系)' form and 'rountine-contrary treatment(正治. 反治)' with 'chill-fever-warm-cold(寒${\cdot}$${\cdot}$${\cdot}$凉)' are mentioned according to the 'analyse and differentiate pathological conditions in accordance with the eight principal syndromes(八綱辨證)'. In the 'Youkwonjeonggidae-ron("六元正紀大論")', Wang Bing of doesn't mention the concepts of 'Jungwun(中運)' that is seen in the original classic. In the new corrective edition, as the concepts of 'Jungwun, Dongcheonbu, Dongsehae and Taeulcheonbu(中運, 同天符, 同歲會, 太乙天符)' is appeared, Wang Bing seems to only use the concepts of 'Daewun, Juwun, and Gaekwun(大運, 主運, 客運)'. In the 'Jijinyodaeron("至眞要大論")', Wang Bing added detailed commentary to pathology and treatment doctrine by explaining the numerous appearances of 'Sebo, sufficiency, deficiency(歲步, 有餘, 不足)' and in the relation of 'victory-defeat(勝復)', he argued clearly that it is not mechanical estimation. (4) When we observe the Wang Bing's originality on the study of 'the theory of Doctrine on five elements' motion and six kinds of natural factors(運氣學說)', he emphasized 'The idea of Jeongindogi and Health preserving(全眞導氣${\cdot}$養生思想)' by adding 'Wang Bing's Commentary(王氷 注本)' of 'The seven great chapters("七篇大論")' and explained clearly 'The theory of Doctrine on five elements' motion and six kinds of natural factors(運氣學說)' and simpled and expanded the meaning of 'man, as a microcosm, is connected with the macrocosm(天人相應)' and with 'Atmosphere theory(大氣論)' also explained the meaning of 'rising and falling mechanism(升降氣機)'. In the sentence of 'By examining the pathology, take care of your health(審察病機 無失氣宜)'. he explained the meaning of pathology of 'heart-kidney-water-fire(心腎水火)' and suggested the doctrine and management of prescription. In the estimation and treatment, by suggesting 'asthenia and sthenia(虛實)' two method's estimation, 'contrary treatment(反治)' and treatment principals of 'falling heart fire tonifyng kidney water(降心火益腎水)', 'two class of chill and fever(寒熱二綱)' were demonstrated. There are 'inside and outside in the illness and so inner and outer in the treatment(病有中外 治有表囊)'. This sentence suggests concertedly. 'two class of superfies and interior(表囊二綱)' conforming to the position of disease. Therefore Wang Bing as an excellent theorist and introduced 'Cheoniin ideology(天眞思想)' as a clinician and realized the medical science. With these accomplishes mainly written in 'The theory of Doctrine on five elements' motion and six kinds of natural factors(運氣學說)' of 'The seven great chapters("七篇大論")', he interpreted the ancient medical scriptures and expanded the meaning of scriptures and conclusively contributed to the development of the study 'Korean Oriental Medicine(韓醫學)'.

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Hypersensitive Large Intestine Syndrome in Hyungsang Medicine (과민성대장증후군의 형상의학적 고찰 -동의보감(東醫寶鑑)을 중심으로-)

  • Choi Byung-Tae;Choi Yung-Hyun;Han Jin-Soo;Lee Yong-Tae
    • Journal of Physiology & Pathology in Korean Medicine
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    • v.19 no.5
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    • pp.1129-1136
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    • 2005
  • The writer reports the conclusions gained from study about the cause of the hypersensitive large intestine syndrome with Dongeuibogam as the central figure through researching the disharmony among Body Essence, Vital Energy, Mentality, and Blood, mutual action of five viscera and six bowels, and external shapes. The hypersensitive large intestine syndrome is generally chronic and recurred in many cases, so it is more efficacious than symptomatic to treat according to find the contradictions of individual shapes. The shapes and cases suffering frequently the hypersensitive large intestine syndrome are Gi-kwa and Sin-kwa, having a long nose, having a bruised spot on Triple warmer, man with inclined mouth, Taeeum type, man with congested fluids, man with colic symptoms. The hypersensitive large intestine syndrome in Oriental medicine is recognized of diarrhea, constipation, abdominal pain, abdominal distention and fullness caused by seven emotions. In Dongeuibogam it can be found out the similarity in depressive symptoms due to disorder of Gi, stagnation of Gi, dysphasia due to disorder of Gi, diarrhea due to disorder of Gi, fullness of due to Gi, diarrhea due to phlegm-retention, retention of undigested food, immoderate drinking, hypo-function of the spleen, or deficiency, abdominal pain from colic symptom, and difficulty in defecation and urination, internal injury, diarrhea due to weakness and fatigue. If the Jung, Gi, Sin, and Hyul composed the human body is broken harmony, the function of large intestinal transmission would be fallen, so similar symptoms like the hypersensitive large intestine syndrome are gotten. Especially Gi-kwa suffers diarrhea, constipation abdominal pain, and abdominal distention and fullness due to depressive symptoms from disorder of Seven emotions or Seven Gi. And Sin-kwa suffers from the hypersensitive large intestine syndrome due to emotional restlessness having an influence on rhythmic movement of abdomen. Examining between five viscera and six bowels and the hypersensitive large intestine syndrome, Liver cannot disperse well having influence on mutual relation of Liver-Large intestine, Heart reduces the function of defecation and urination not to control the seven emotions, Lung having exterior and interior relation with intestine has an influence on primordial energy and let the main symptoms occur, Spleen circulating the body fluid let the main symptoms occur due to malfunction of circulation, Kidney locating in lower part of the body has deep connection with intestine, so let the disorder. Urinary bladder is connected with intestine in moisture metabolism, Stomach is connected in receive and transmission, Small intestine is connected in absorption and excretion, from small intestine pain disturbing the abdominal movement, Samcho managing the catharsis of lower heater if declined its function causes the hypersensitive large intestine syndrome. The colic symptoms of Front private parts which disorder in lower abdomen give rise to abdominal pains, difficulty in defecation and urination due to Cold are similar to the hypersensitive large intestine syndrome. The treatments of applying the shapes of colic syndrome advocated by Master Park can be efficacious cure in clinic. Researching after the clinical cases of Master Park advocating Hyungsang medicine, we came to know that plenty of prescriptions of internal injury are applied and take good effects.

A Study on the External Treatment of Dysmenorrhea using the Method of applying Herb-medicine at the acupoints (월경통(月經痛)의 약물혈위첩부치료법(藥物穴位貼敷治療法)에 대한 고찰(考察))

  • Lim, Eun-Mee
    • The Journal of Korean Medicine
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    • v.16 no.2 s.30
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    • pp.134-148
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    • 1995
  • Dysmenorrhea is probably the most common of all Gynecologic disorders. In the oriental medicine the etiology of Dysmenorrhea is very various, but its mechanism is just that the block of the flow makes the pain. So its treatment is removing the pain by promoting menstrual flow, promoting the flow of qi and by warming channel and activating blood flow. In the various treatments of dysmenorrhea, the method of applying drug at the acupoints which could be combined with feeding herb-medicine by oral was studied. The results obtained here were as follows; 1. In the treatments of dysmenorrhea, the method of applying drug at the acupoints was mainly applied to the type dued to stagnancy of qi and blood stasis, menorrhalgia dued to to cold and dampness, and primary dysmenorrhea. 2. The acupoint used in this treatment was Shin-gwol$(CV_8)$, the umbilicus. 3. The drugs used in this treatment were almost same as oral herb-medicine mainly to activate the blood flow and remove the blood stasis and to promote the flow of qi by warming the channel and remove the pain. 4. The duration of the treatment is, from 3 days before menstration till its period or a few days after it., usually concentrated on fore-postmenstration. 5. The effect of this treatment was reported as excellent. It is more effective to the type of stagnancy of qi and blood stasis, cold and dampness than dued to deficiency of both qi and blood, and dued to the impaired liver-kidney essence. 6. The method of applying drug at the acupoint was as an external treatment, easy, economical, and had no pain and side effect. 7. In the treatments of dysmenorrhea, the method of applying drug at the acupoint could be used as emergency treatment and symptomatic treatment in fore-postmenstration.

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Nutritional and Tissue Specificity of IGF-I and IGFBP-2 Gene Expression in Growing Chickens - A Review -

  • Kita, K.;Nagao, K.;Okumura, J.
    • Asian-Australasian Journal of Animal Sciences
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    • v.18 no.5
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    • pp.747-754
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    • 2005
  • Nutritional regulation of gene expression associated with growth and feeding behavior in avian species can become an important technique to improve poultry production according to the supply of nutrients in the diet. Insulin-like growth factor-I (IGF-I) found in chickens has been characterized to be a 70 amino acid polypeptide and plays an important role in growth and metabolism. Although it is been well known that IGF-I is highly associated with embryonic development and post-hatching growth, changes in the distribution of IGF-I gene expression throughout early- to late-embryogenesis have not been studied so far. We revealed that the developmental pattern of IGF-I gene expression during embryogenesis differed among various tissues. No bands of IGF-I mRNA were detected in embryonic liver at 7 days of incubation, and thereafter the amount of hepatic IGF-I mRNA was increased from 14 to 20 days of incubation. In eyes, a peak in IGF-I mRNA levels occurred at mid-embryogenesis, but by contrast, IGF-I mRNA was barely detectable in the heart throughout all incubation periods. In the muscle, no significant difference in IGF-I gene expression was observed during different stages of embryogenesis. After hatching, hepatic IGF-I gene expression as well as plasma IGF-I concentration increases rapidly with age, reaches a peak before sexual maturity, and then declines. The IGF-I gene expression is very sensitive to changes in nutritional conditions. Food-restriction and fasting decreased hepatic IGF-I gene expression and refeeding restored IGF-I gene expression to the level of fed chickens. Dietary protein is also a very strong factor in changing hepatic IGF-I gene expression. Refeeding with dietary protein alone successfully restored hepatic IGF-I gene expression of fasted chickens to the level of fed controls. In most circumstances, IGF-I makes a complex with specific high-affinity IGF-binding proteins (IGFBPs). So far, four different IGFBPs have been identified in avian species and the major IGFBP in chicken plasma has been reported to be IGFBP-2. We studied the relationship between nutritional status and IGFBP-2 gene expression in various tissues of young chickens. In the liver of fed chickens, almost no IGFBP-2 mRNA was detected. However, fasting markedly increased hepatic IGFBP-2 gene expression, and the level was reduced after refeeding. In the gizzard of well-fed young chickens, IGFBP-2 gene expression was detected and fasting significantly elevated gizzard IGFBP-2 mRNA levels to about double that of fed controls. After refeeding, gizzard IGFBP-2 gene expression decreased similar to hepatic IGFBP-2 gene expression. In the brain, IGFBP-2 mRNA was observed in fed chickens and had significantly decreased by fasting. In the kidney, IGFBP-2 gene expression was observed but not influenced by fasting and refeeding. Recently, we have demonstrated in vivo that gizzard and hepatic IGFBP-2 gene expression in fasted chickens was rapidly reduced by intravenous administration of insulin, as indicated that in young chickens the reduction in gizzard and hepatic IGFBP-2 gene expression in vivo stimulated by malnutrition may be, in part, regulated by means of the increase in plasma insulin concentration via an insulin-response element. The influence of dietary protein source (isolated soybean protein vs. casein) and the supplementation of essential amino acids on gizzard IGFBP-2 gene expression was examined. In both soybean protein and casein diet groups, the deficiency of essential amino acids stimulated chickens to increase gizzard IGFBP-2 gene expression. Although amino acid supplementation of a soybean protein diet significantly decreased gizzard IGFBP-2 mRNA levels, a similar reduction was not observed in chickens fed a casein diet supplemented with amino acids. This overview of nutritional regulation of IGF-I and IGFBP-2 gene expression in young chickens would serve for the establishment of the supply of nutrients to diets to improve poultry production.