Steamed Ginger Extract Exerts Anti-inflammatory Effects in Helicobacter pylori-infected Gastric Epithelial Cells through Inhibition of NF-κB

Ginger (Zingiber officinale) has traditionally been used as a treatment for inflammatory diseases in the Asian region. Recently, anti-inflammatory effects of steamed ginger extract (GGE03) have been reported, but its association with Helicobacter pylori (H.pylori)-induced gastritis has not been investigated. The purpose of this study was to assess the anti-inflammatory activity of GGE03 in H. pylori-infected gastric epithelial cells. Our studies revealed that the GGE03 suppressed the growth of H. pylori. GGE03 markedly reduced the expression of the H. pylori-induced pro-inflammatory cytokines including interleukin (IL)-8, TNF-α, IL-6, inducible NOS (iNOS) and IFN-γ. We also demonstrated that GGE03 treatment inhibited the H. pylori-activated NF-κB signaling pathway. In addition, the treatment with GGE03 significantly attenuated nitric oxide production and myeloperoxidase activity in H. pylori-infected gastric epithelial cells. These anti-inflammatory effects of GGE03 were more effective than ginger extract. Finally, we investigated the minimum effective concentration of GGE03 to inhibit H. pylori-induced inflammation. Our findings suggest that GGE03 not only inhibits the growth of H. pylori, but also attenuates H. pylori-induced inflammation.

Trefoil Factor 1 Suppresses Epithelial-mesenchymal Transition through Inhibition of TGF-beta Signaling in Gastric Cancer Cells

Gastric cancer is a malignancy with high incidence and mortality worldwide. In gastric cancer, epithelial-mesenchymal transition (EMT) and metastasis further increase the mortality rate. Trefoil factor 1 (TFF1) has been reported as a protective factor in the gastric mucosa. In this study, TFF1 inhibited the migration and invasive capability of gastric cancer cells. Elevated TFF1 levels induced the expression of E-cadherin, the epithelial marker, and reduced the expression of N-cadherin, vimentin, Snail, Twist, Zinc finger E-box binding homeobox (ZEB) 1 and ZEB2, well-known repressors of E-cadherin expression. In addition, the expression of matrix metalloproteinase (MMP)-2, MMP-7 and MMP-9, which are major markers of cancer metastasis, was suppressed by TFF1. Upregulation of TFF1 inhibited TGF-β, a major signaling for EMT induction, and the phosphorylation of Smad2/3 activated by TGF-β in AGS cells. In conclusion, TFF1 inhibits EMT through suppression of TGF-β signaling in AGS cells, which might be used in therapeutic strategies for reducing metastatic potential and invasiveness of these cells.

Trefoil Factor 1 Suppresses Epithelial-mesenchymal Transition through Inhibition of TGF-beta Signaling in Gastric Cancer Cells

Gastric cancer is a malignancy with high incidence and mortality worldwide. In gastric cancer, epithelial-mesenchymal transition (EMT) and metastasis further increase the mortality rate. Trefoil factor 1 (TFF1) has been reported as a protective factor in the gastric mucosa. In this study, TFF1 inhibited the migration and invasive capability of gastric cancer cells. Elevated TFF1 levels induced the expression of E-cadherin, the epithelial marker, and reduced the expression of N-cadherin, vimentin, Snail, Twist, Zinc finger E-box binding homeobox (ZEB) 1 and ZEB2, well-known repressors of E-cadherin expression. In addition, the expression of matrix metalloproteinase (MMP)-2, MMP-7 and MMP-9, which are major markers of cancer metastasis, was suppressed by TFF1. Upregulation of TFF1 inhibited TGF-β, a major signaling for EMT induction, and the phosphorylation of Smad2/3 activated by TGF-β in AGS cells. In conclusion, TFF1 inhibits EMT through suppression of TGF-β signaling in AGS cells, which might be used in therapeutic strategies for reducing metastatic potential and invasiveness of these cells.

HLA, CTLA-4 and TNF-beta Gene Polymorphisms and Disease Susceptibility in Korean Children with Graves' Disease / 대한내분비학회지

BACKGROUND: Graves' disease(GD) is an organ-specific autoimmune disorder that is inherited as a complex trait. At present three loci, namely the human leukocyte antigen(HLA), the cytotoxic T lymphocyte antigen-4(CTLA-4) and a thyroid stimulating hormone receptor(TSHR) are the only well-known genetic determinants for GD. To understand the mechanisms underlying the development of GD, we investigated the relationship of HLA alleles, polymorphisms of CTLA-4 gene and the tumor necrosis factor(TNF)-beta gene, with the disease susceptibility. METHODS: Fifty-two Korean children with GD(45 girls and 7 boys), and 119 healthy children, were investigated in this study. The HLA alleles were determined by a standard lymphocyte microtoxicity technique, ARMS-PCR(Amplification Refractory Mutation System-Polymerase Chain Reaction), PCR-SSP(Sequence Specific Primer) and PCR-SSOP(Sequence Specific Oliogonucleotide Probe) method. The CTLA-4 gene polymorphism was analyzed by PCR-SSCP(Single Strand Conformation Polymorphism), and the TNF-beta gene polymorphism by PCR-RFLP(Restriction Fragment Length Polymorphism). RESULTS: (1) The frequencies of HLA-A2, B46, DRB1*08 and DPB1*0202 were significantly increased, and those of HLA-A24, DQA1*01 and DQB1*05 were significantly decreased, in the GD patients compared to the control subjects. (2) A significant difference in the distributions of the AA, AG, and GG genotypes of the CTLA-4 exon 1 were observed between the GD patients and the control subjects, and a significant increase in the frequency of the G (alanine) allele was seen in the GD patients compared with the control subjects(84.6% vs 63.4%; RR=3.2; p or =45% compared with GD patients having TSHRAb <45%(37.5% vs 3.6%; RR=14.8; p<0.01). CONCLUSION: These data suggest that HLA-A2, B46, Cw*0102, DRB1*08 and DPB1*0202 are markers for disease susceptibility, and that HLA-A24, DQA1*01 and DQB1*05 are markers for disease protection, in Korean children with GD. This study showed that the CTLA-4 gene polymorphism was an additional marker of susceptibility in the GD patients, and was associated with exophthalmos, and that the TNF-beta gene polymorphism was associated with the TSHRAb activity.

Chromosomal Assay after In-vitro Irradiation of Lymphocytes in Ataxia Telangiectasia

BACKGROUND: Hypersensitivity to both cell-killing and chromosome-damaging effects of ionizing radiation is a consistent feature of cells from individuals with ataxia-telangiectasia (AT). This radiobiological behavior of AT cells is a component of genetic instability and may contribute to cancer risk. Also, heterozygotes for AT-mutated (ATM) genes have no clinical expressions of AT, but may become cancer prone with a moderate increase in in-vitro radiosensitivity. METHODS: We performed a chromosomal analysis on lymphocytes from 3 AT patients, 5 obligate AT carriers (siblings and parents of the patients), and 5 normal controls. RESULTS: Increases in chromosomal breakages after irradiation with 1 gray/min in cells from AT patients ranged from 0.65 to 0.83 rearrangements per metaphase, while in the carriers and controls the levels of breakage were between 0 and 0.15 per metaphase cells (P<0.05). CONCLUSIONS: These results are consistent with previously reported chromosomal radiosensitivity in AT patients. However, carriers do not show moderate radiosensitivity due to various technical factors such as the dose or distance of radiation. Although this research has some limitations due to the small numbers of patients, carriers and controls, this method may be an easy and useful diagnostic tool for AT patients in Korea. (J Korean Neurol Assoc 19(5):509~513, 2001)

A Case of 18 Ring Chromosome

A ring 18 chromosome(18r) karyotype is a rare disorder characterized by short stature, obesity, microcephaly, mental retardation, micropenis, cryptorchidism, hypertelorism, epicanthal folds, micrognathia, and small hands with short tapering fingers. A 22-month-old girl was referred to our department of pediatrics because of ptosis and delayed development. Karyotype revealed ring 18 and dicentric ring 18 chromosome(46,XX,r(18)[45]/45,XX,-18[7]/46,XX,dic r(18)[3]). Fluorescent in situ hybridization with 18 centromeric probe showed 18 ring chromosome with 1 signal and 18 dicentric ring chromosome with 2 signals. We report a case of a ring 18 chromosome diagnosed by karyotype and fluorescent in situ hybridization.

Role of Leptin in Puberty / 대한소아내분비학회지

No abstract available.

Thyroid Diseases in Childhood / 대한소아내분비학회지

PURPOSE: Thyroid disease is the most common endocrine disease in childhood. Thyroid hormone has critical effects on growth and development, especially in childhood. We survey the prevalence, sex and age distribution, symptoms and thyroid function states of thyroid diseases in childhood. METHODS: Three hundred ninety one children who were diagnosed as having thyroid disease at department of pediatrics, St. Mary's hospital from Jan. 1987 to Dec. 1998 enrolled in this study. RESULTS: 1) The ratio between male and female patients was 1: 5.3. Age distribution was puberty, school age and infancy in their order of frequency. 2) The results of thyroid function tests showed normal function in 210 cases (53.7%), increased function in 95 cases(24.3%), and decreased function in 86 cases (22%). 3) Simple goiter, Graves disease, and chronic lymphocytic thyroiditis were the most common diseases in euthyroid, hyperthyroid, and acquired hypothyroid state, respectively. 4) The most common sign and symptom was goiter in euthyroid(100%), hyperthyroid(98.8%) and acquired hypothyroid state(96.2%). Forty of 68 cases(58.8%) with congenital hypothyroidism were detected by neonatal screening. CONCLUSION: Simple goiter, chronic lymphocytic thyroiditis and Graves disease were common acquired thyroid diseases in childhood, and goiter is the most common clinical manifestation in acquired thyroid disease. Recently, increasing number of congenital hypothyroidism was detected by neonatal screening test.

A Case of Autonomous Ovarian Cyst with Severe Vaginal Bleeding / 대한소아내분비학회지

Small ovarian cysts(<0.7 cm in diameter) are common in prepubertal children. Occasionally larger cysts occur in young girls with isosexual incomplete precocious puberty in the absence of LH and FSH elevation. The mechanism of production of these autonomous functioning cysts is unknown. A 3 year 6 month-old girl was referred to our department of pediatrics because of severe vaginal bleeding. On examination, she was tall and had breast enlargement of Tanner stage II. On hormonal study, FSH(0.38mIU/ml) was suppressed and estradiol(62.2pg/ml) was markedly elevated, and GnRH stimulation failed to evoke an increase in gonadotropin. Bone age was advanced. Pelvic ultrasonography showed both huge ovarian cysts. Brain MRI showed no abnormalities. We underwent both ovarian cystectomy and histological examination showed follicular cyst in left ovary and luteal cyst in right ovary. After ovarian cystectomy vaginal bleeding stopped and hormonal abnormalities were normalized. We report a case of autonomous ovarian cysts presenting severe vaginal bleeding treated with ovarian cystectomy.

Neonatal Cord Blood Leptin: Its Relationship to Birth Weight

PURPOSE: The purpose of this study was to correlate birth weight, skinfold thickness, gestational age, BMI and ponderal index of neonates and maternal status with cord serum leptin concentration. METHODS: Sixty newborns were enrolled in this study. Standard growth curves were used to categorize infants as appropriate(AGA), large(LGA), or small for gestational age(SGA). Gestational age, birth weight, length, skinfold thickness, and placental weight were measured. Maternal age, weight, and height were measured. Cord blood and maternal venous blood samples were collected, and serum was separated and stored at -20degrees C. Leptin was measured by radioimmunoassay. RESULTS: Mean leptin concentration in cord blood was 4.38+/-2.65ng/ml and closely related to size and adiposity at birth, but not to gestational age. Cord blood leptin level was significantly lower in SGA infants(1.64+/-0.58ng/ml) and higher in LGA infants(5.53+/-2.64ng/ml) than in AGA infants (4.99+/-2.37ng/ml)(P<0.05). Mean leptin concentration in maternal blood(13.01+/-8.28ng/ml) was significantly higher than in cord blood(P<0.001), and maternal obesity and serum leptin concentrations did not correlate with cord blood leptin concentrations. No gender differences were found in cord blood leptin. CONCLUSION: The leptin level of cord blood is highly correlated to the birth weight and the size of adipose tissue. Leptin may be responsible for the regulation of body mass of human neonates and can be used as a marker of fetal growth.

Acute Effects of Total Body Irradiation on Serum Leptin Concentration in Children with Acute Leukemia Undergoing Bone Marrow Transplantation / 대한소아내분비학회지

urpose:Adipocyte specific hormone, leptin is thought to regulate appetite and body weight, and serum level of leptin correlate with body fat content not only in adulthood but also in childhood. Adults who received cranial irradiation for childhood acute lymphoblastic leukemia are prone to hypothalamic damage in the form of growth hormone deficiency and leptin insensitivity. The purpose of this study is to evaluate the effect of total body irradiation on serum leptin concentration at short- term follow-up. METHODS:Twenty-four acute leukemia patients undergoing bone marrow transplantation(BMT) were enrolled in this study. They were divided into total body irradiation(TBI) group(n=13) and non total body irradiation(NTBI) group(n=11). Body mass index and leptin concentration at 1 week before BMT and 1 day, 1 week and 4 weeks after BMT were checked. Serum leptin concentration was measured by RIA method. RESULTS:Leptin concentration was well correlated with BMI(r=0.689, P<0.05). BMI were 17.96+/-2.48, 17.58+/-2.39, 17.39+/-2.30, 17.74+/-2.31 in TBI group and 16.91+/-1.88, 16.53+/-1.91, 16.50+/-1.96, 16.78+/-1.82 in NTBI group at 1 week before BMT and 1 day, 1 week and 4 weeks after BMT. Lepin concentrations were 4.31+/-3.57ng/ml, 3.22+/-2.87ng/ml, 3.78+/-4.41ng/ml, 3.46+/-2.96ng/ml in TBI group and 3.46+/-1.84ng/ml, 2.60+/-1.11ng/ml, 2.71+/-1.61ng/ml, 3.61+/-3.27ng/ml in NTBI group at 1 week before BMT and 1 day, 1 week and 4 weeks after BMT. BMI was different at each time period(P<0.05) but not different between two groups. Lepin concentration was not different between two groups and at each time period. CONCLUSION: Serum leptin concentration was not changed, but BMI was significantly decreased by total body irradiation at short-term follow-up.

Serum Leptin Levels in Children with Obesity

PURPOSE: The identification of the ob gene and its adipocyte-specific protein leptin has provided the first physiologic links to the regulatory system controlling body weight. In adults, elevations of serum leptin concentrations correlated closely with the percentage of body fat. This study investigated whether leptin concentrations were elevated in obese children, and the relationship between leptin concentrations and gender and pubertal stage. METHODS: The obese group was composed of 84 children (56 boys and 28 girls) with mean age 9.9 +/- 1.7 years. All obese children had an obesity index greater than 20. Twenty-five normal children (19 boys and 6 girls) with mean age 9.5 +/- 3.1 years constituted the control group. All in children of the control group had an obesity index less than 20. We measured height, weight, blood pressure, midarm circumference, triceps skinfold thickness, waist and hip circumference in all subjects. Body mass index (BMI) was calculated by weight in kilograms divided by the square of height in meters. Arm fat was calculated from the measured midarm circumference and triceps skinfold thickness according to the formulas provided by Must et al. We measured serum AST/ ALT, total cholesterol, HDL-cholesterol, LDL-cholesterol and triglyceride concentrations in all subjects. Radioimmunoassay for serum leptin was performed on a blood sample collected from each child after an overnight fast. RESULTS: The mean serum concentration of leptin in the obese group (14.5 +/- 7.0ng/ml) was significantly higher than that in the control group (4.7 +/- 3.1ng/ml)(P<0.05). Serum leptin concentrations significantly correlated with obesity index (r=0.54), BMI (r=0.50), and arm fat (r=0.46)(P<0.05). Girls had significantly higher leptin level than boys (P<0.05). Tanner stage had no effect on leptin concentration. CONCLUSION: As in adults, obese children have high concentrations of serum leptin, which highly correlates with obesity index, arm fat and BMI. Increased adipose tissue in children is associated directly with serum leptin concentration. Compared to boys, girls had higher leptin concentrations.

Two Cases of Thyroid Tumor after Radiation Therapy of Primary Malignancy / 대한소아내분비학회지

The thyroid gland is highly sensitive to the carcinogenic effects of ionizing radiation. The development of benign thyroid nodules, thyroid carcinoma and thyroid dysfunction following radiotherapy during childhood has been documented. Recently we experienced two cases of thyroid tumor after irradiation. One is a 14-year-old boy with thyroid follicular carcinoma who had received mantle irradiation(3,000cGy) eight years previously for Hodgkin' disease. The other is a 15-year- old boy with thyroid follicular adenoma who had received craniospinal irradiation (3,600cGy) seven years previously for CNS relapse of acute lymphoblastic leukemia. We recommend frequent sonographic evaluation and early suppression of thyroid stimulation in an attempt to arrest the development of neoplastic changes in long term survivors of childhood malignancies who received radiation therapy.

Growth of Children with Hypochondroplasia Treated with Growth Hormone for Two Years / 대한소아내분비학회지

PURPOSE:Hypochondroplasia is a skeletal dysplasia characterized by poor childhood growth and an inadequate pubertal growth spurt. Final height attainment of hypochondroplasia has been reported to range between 120 and 152cm. Increased availability of growth hormone with the introduction of recombinant human growth hormone has allowed for clinical trials in a number of growth hormone sufficient children with growth problems. The purpose of this study was to assess the growth promoting effect of human growth hormone in children with hypochondroplasia. METHODS:Five patients with hypochondroplasia diagnosed by clinical and radiological findings between 1993 and 1997 at our hospital was aged 3 and 1/2 -11 and 1/2 years. Each patients continuously received human growth hormone 0.6-0.7U/Kg/week, intramuscularly or subcutaneously in 6-7 divided dose for 2 years. Standard auxologic assessment was carried out every 3 month interval in the first year after commencement of therapy and then same assessment was 6 monthly. Bone age was assessed 6 monthly using Gleurich-Pyle method. RESULTS:Mean height velocity of pretreatment and year 1 and 2 of GH treatment were 3.9+/-0.7, 6.5+/-1.8 and 5.7+/-1.5cm/year, respectively. Mean height standard deviation score for chronological age of pretreatment and year 1 and 2 of GH treatment were -2.7+/-0.3, -2.4+/-0.3 and -2.2+/-0.4, respectively. The increase in the height velocity diminishes over the subsequent year. The increment of bone age after GH treatment were same as the increments of chronological age. CONCLUSION: Short-term GH therapy increases the height velocity of children with hypochondroplasia, but the effect of GH therapy on final height remains unknown.

Serum Neopterin Concentration in Children with Graves' Disease / 대한소아내분비학회지

PURPOSE:Neopterin is a marker of activation of the T-lymphocyte/monocyte axis. We measured serum neopterin concentration to investigate whether serum neopterin levels are increased in children with Graves' disease and whether serum neopterin measurement can be used as a marker of disease activity in Graves disease. METHODS:Twenty children with Graves' disease(3 boys and 17 girls) and 15 healthy children(7 boys and 8 girls) are enrolled in this study. Serum neopterin concentrations are measured by radioimmunoassay. RESULTS:Neopterin concentration in children with Graves' disease(1.59+/-1.25ng/ml) is not higher than that of healthy children(1.51+/-0.73ng/ml). Neopterin concentration is not influenced by thyroid function and remission state. CONCLUSION: Serum neopterin level in children with Graves' disease can not be used as a marker of activity.

A Case of Transient Neonatal Hypothyroidism Due to Maternal TSH-Binding Inhibitor Immunoglobulins / 대한소아내분비학회지

Recognition of transient forms of neonatal hypothyroidism is very important to prevent the complications of congenital hypothyroidism. Transplacental passage of TSH-binding inhibitory immunoglobulins(TBII) may result in transient congenital hypothyroidism. Transient neonatal hypothyroidism was found in a daughter of 25-yr-old mother who was receiving levothyroxine for primary hypothyroidism due to Hashimoto's thyroiditis. The neonate was treated with thyroxine which was discontinued at 24 months of age. Thyroid scanning during the neonatal period failed to identify functional thyroid tissue, suggesting thyroid agenesis, whereas thyroid scan performed on subsequent follow-up revealed a normal gland. Sequential measurements of serum autoantibodies directed towards the TSH receptor were made in the patient and her mother. High titers of blocking antibodies were present in the mother(TBII, 82.1%) and newborn(TBII, 85.5%) at 19 days after birth. The levels remained persistently high in the mother, whereas they declined and undetectable in the patient at 23 months of age. The above laboratory and clinical data were compatible with blocking nature of TBII, resulting in transient neonatal hypothyroidism and an athyreotic appearance on scan. The TBII measurement can be a useful predictor of neonatal hypothyroidism as well as confirming the nature of the disease in newborn.

Sexual Precocity:Sex Incidence and Etiology / 대한소아내분비학회지

PURPOSE:Differential diagnosis of sexual precocity has been aided by new imaging techniques and endocrine evaluation. Sexual precocity, defined as the appearance of secondary sexual characteristics before the age of 8 years in girls and 9 years in boys, was studied to evaluate the sex incidence and etiology of sexual precocity. METHODS:A total 95 children(87 girls and 8 boys) who were diagnosed with sexual precocity at St. Mary's Hospital, Catholic University Medical College from 1984 to 1996, were studied. All underwent standard anthropometric measures. They were assinged to diagnostic categories on the basis of clinical assessment, endocrine evaluation, radiologic imaging, and pelvic ultrasonography. RESULTS: 1) The most common cause of sexual precocity was premature thelarche, 50 cases(52.6%) of all children. 2) Central precocious puberty was noted in 7 girls and 3 boys(female to male ratio 2.3:1). The causes were not identified in 6 cases(85.7%) in girls, but the causes were not found in 1 cases(33.3%) in boys. The idiopathic central precocious puberty occurs at least two fold more frequently in girls than in boys. 3) Incomplete precocious puberty was noted in 29 girls and 5 boys(female to male ratio 5.8:1). In the causes of 29 girls with incomplete precocious puberty, congenital adrenal hyperplasia were found in 23 cases, autonomous functional ovarian cyst in 5 cases and McCune-Albright syndrome in 1 case. In the causes of 5 boys with incomplete precocious puberty, virilizing adrenal cortical tumor were found in 3 cases, congenital adrenal hyperplasia in 1 case, and hepatoblastoma in 1 case. CONCLUSION: Sexual precocity occurs at least ten fold more frequently in girls than in boys. Premature thelarche is the most common form of sexual precocity.

A Case of Virilizing Adrenal Cortical Carcinoma / 대한소아내분비학회지

Adrenal cortical carcinoma is a rare disease both in adults and in children. Most of these tumors are functional, especially in children, producing endocrine syndromes such as virilization, Cushing syndrome, hyperaldosteronism or feminization. We experienced a case of adrenal cortical carcinoma in a 7yr old boy who showed features of virilization such as rapid growth rate, penile enlargement, and pubic hair. This case was diagnosed with typical hormonal findings and abdominal MRI and confirmed by pathologic findings. He was successfully treated by total left adrenalectomy and has been followed up without problem over eight months.

A Case of von Gierke Disease

von Gierke disease (type Ia glycogen storage disease) is an inherited disease associated with accumulation of glycogen in the liver, kidney, intestine and erythrocytes due to the defect of glucose-6-phosphatase activity. Hepatomegaly, doll face, anemia, bleeding tendency and increased susceptability to infection are common features observed during infancy. Hypoglycemia especially fasting hypoglycemia is typical metabolic derangement in this disease, followed by metabolic acidosis, lactic acidemia, hyperlipidemia, hyperuricemia, and platelets dysfunction. We experienced a case of von Gierke disease in 6 month-old boy with doll face, hepatomegaly, fasting hypoglycemia, acidosis, anemia, hyperlipidemia, hyperuricemia, and acetonuria. Diagnosis was confirmed by light- and electron microscopic examination of liver biopsy specimen, which revealed hepatocytes filled with dense pools of glycogen and many lipid droplets. Cornstarch dietary therapy for him had favorable responses showing improvement of hypoglycemia, other metabolic derangements, and regression of hepatomegaly.

A Case of von Gierke Disease

von Gierke disease (type Ia glycogen storage disease) is an inherited disease associated with accumulation of glycogen in the liver, kidney, intestine and erythrocytes due to the defect of glucose-6-phosphatase activity. Hepatomegaly, doll face, anemia, bleeding tendency and increased susceptability to infection are common features observed during infancy. Hypoglycemia especially fasting hypoglycemia is typical metabolic derangement in this disease, followed by metabolic acidosis, lactic acidemia, hyperlipidemia, hyperuricemia, and platelets dysfunction. We experienced a case of von Gierke disease in 6 month-old boy with doll face, hepatomegaly, fasting hypoglycemia, acidosis, anemia, hyperlipidemia, hyperuricemia, and acetonuria. Diagnosis was confirmed by light- and electron microscopic examination of liver biopsy specimen, which revealed hepatocytes filled with dense pools of glycogen and many lipid droplets. Cornstarch dietary therapy for him had favorable responses showing improvement of hypoglycemia, other metabolic derangements, and regression of hepatomegaly.