Glucose-6-phosphate dehydrogenase(G6PD) deficiency is the most common X- linked inherited disorder and is estimated to affect 400 million people worldwide. But the incidence of this disease is very rare in far-east Asia, especially in Korea. Many drugs and infections cause hemolytic anemia in patients with G6PD deficiency. We experienced a case of G6PD deficiency with chronic hepatitis B. The diagnosis was made by clinical symptoms, laboratory data including serologic test and bone marrow findings. We report a case of G6PD with chronic hepatitis with a brief review of related literatures.
Anemia, Hemolytic ; Asia ; Bone Marrow ; Diagnosis ; Glucose-6-Phosphate* ; Glucosephosphate Dehydrogenase Deficiency ; Hepatitis B ; Hepatitis B, Chronic* ; Hepatitis, Chronic* ; Humans ; Incidence ; Korea ; Serologic Tests

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.
Adolescent ; Age Distribution ; Child ; Congenital Hypothyroidism ; Endocrine System Diseases ; Female ; Goiter ; Graves Disease ; Growth and Development ; Hashimoto Disease ; Humans ; Infant, Newborn ; Male ; Neonatal Screening ; Pediatrics ; Prevalence ; Puberty ; Thyroid Diseases* ; Thyroid Function Tests ; Thyroid Gland*

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.
Child* ; Growth Hormone* ; Human Growth Hormone ; Humans

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.
Adrenalectomy ; Adrenocortical Carcinoma* ; Adult ; Child ; Cushing Syndrome ; Feminization ; Hair ; Humans ; Hyperaldosteronism ; Magnetic Resonance Imaging ; Male ; Rare Diseases ; Virilism

The optic nerve do not easily sever because of its anatomic status in the orbit. The purpose of this study is to investigate electron microscopic findings in the proximal portion of the severed optic nerve which remained in the orbit for about 4.5 months. Thirty seven-year-old woman showed the protruded eyeball and deep skin laceration following traffic accident. Computerized tomography and MRI demonstrated the complete severance in her left optic nerve. Her eyeball containing the proximal portion of the severed optic nerve was enucleated at about 4.5 months after traffic accident. Eletron microscopic findings showed several macrophages, fibroblast, and dense collagen fibers, which mean the fibrosis histopathologically. The fibrosis was the main pathologic outcome in the proximal portion of the severed optic nerve. We suggest that the process of the fibrosis could result from the incorporation of the fibroblast within the meninges around the optic nerve and the proliferation of the fibroblast within fibrovascular septa of the pia mater which extend into the optic nerve.
Accidents, Traffic* ; Collagen ; Female ; Fibroblasts ; Fibrosis ; Gliosis ; Humans ; Lacerations ; Macrophages ; Magnetic Resonance Imaging ; Meninges ; Optic Nerve* ; Orbit ; Pia Mater ; Skin

BACKGROUND: There are many differences in the prevalence of syphilis according to the objects and districts. Recently, the incidence of syphilis increased in the world because of various factors. OBJECTIVE: The purpose of this study is to investigate the changes of the epidemiological, clinical characteristics, and stages of syphilis. METHODS: We selected 357 patients with reactive results on VDRL among 79,991 cases who visited in our hospital, between January 2002 and December 2007. We assessed the age, gender, skin lesion, serologic result, clinical stage, treatment history, and underlying disease. RESULTS: During the 7-year period under study, the reactive rate of serum VDRL test was 4.5% in 79,991 people (preoperation or admission examinees: 35.0%. physical examinees: 30.8%, skin lesion: 21.3%, partner's (+): 9.0%, pregnant women: 3.7%). The annual incidence of syphilis had increased from 3.5% in 2002 to 6.3% in 2007. On a total 357 sera with reactive results on VDRL, the symptomatic syphilis rate was 21.3%, increased from 13.6% in 2002 to 26.8% in 2007 (p=0.001). Statistically, there are differences in age distribution between both sexes. The male:female ratio was 1:3.3 in 0~19 years and 1.9:1 in 60~69 years (p=0.029). The incidence of symptomatic syphilis cases was 8 (66.7%) in 0~19 years, 22 (39.3%) in 20~29 years, while latent syphilis was 53 (74.6%) in 50~59 years and 35 (60.3%) in 60~69 years (p<0.001). CONCLUSION: This study suggests that the incidence of symptomatic syphilis may be increasing. Further observation, analysis, and continued vigilance in the general population are required.
Age Distribution ; Humans ; Incidence ; Prevalence ; Skin ; Syphilis ; Syphilis, Latent

Jessner's lymphocytic infiltration of the skin (JLIS) is a well-known but poorly understood disorder. Some doubt still exists about whether it is a distinct disease or a variant of lupus erythematosus or, less commonly, polymorphous light eruption, cutaneous lymphoid hyperplasia. An effective therapy is still unavailable for JLIS. We report a patient with JLIS which was successfully treated with dapsone and intermittent systemic glucocorticoid therapy.
Dapsone ; Humans ; Hyperplasia ; Light ; Skin

BACKGROUND: Telogen effluvium (TE) is a form of nonscarring alopecia, characterized by diffuse scalp hair thinning in response to some form of physiologic stress. It is commonly described in women subsequent to childbirth. OBJECTIVE: The purpose of this study was to evaluate the prevalence of TE after childbirth. METHODS: Medical records from January 2006 to June 2007 of 240 postpartum patients from our obstetric department were reviewed. Telephone interviews with TE patients were conducted to investigate the initial occurrence time, restoration time, and treatment history. RESULTS: Prevalence of TE according to parity is higher in multiparas than primiparas (p=0.026). In addition, the prevalence of TE after a cesarean section delivery is higher than that after non-surgical delivery (p=0.014). Prevalence of TE with respect to weight gain is higher in the groups displaying agreater increase in weight (p=0.014). Finally, prevalence of TE according to gestational age is higher in groups giving birth after 38 weeks gestational age (p=0.015). CONCLUSION: Prevalence of telogen effluvium may be associated with multiparity, cesarean section, marked weight gain, and high gestational age.
Alopecia ; Cesarean Section ; Female ; Gestational Age ; Hair ; Humans ; Interviews as Topic ; Medical Records ; Parity ; Parturition ; Postpartum Period ; Pregnancy ; Prevalence ; Scalp ; Weight Gain

Previously we reported that THI 52 inhibits tumor necrosis factor (TNF)-alpha mRNA expression in mouse peritoneal macrophages exposed to LPS plus IFN-gamma. In the present study, the effects of THI 52 on vascular reactivity ex vivo, and iNOS protein expression (rat lung) were investigated in LPS-treated rats. Treatment of THI 52 concentration-dependently reduced not only serum nitrite production but also the expression of iNOS protein in rat lung tissues. Thoracic aorta taken from LPS injected rat for 8 h ex vivo resulted in suppression of vasoconstrictor effects to phenylephrine (PE), which was restored by THI 52 (20 mg/kg) 30 min prior to LPS. When measured iNOS activity, treatment of THI 52 concentration-dependently reduced the enzyme activity in RAW 264.7 cells activated with LPS plus IFN-gamma. Likewise, iNOS activity was significantly reduced in lung tissues taken those rats that were injected THI 52 prior to LPS injection compared with LPS injection alone. These results strongly suggest that THI 52 can suppress iNOS gene expression induced by LPS, and restore the vascular contractility to PE. Thus, THI 52, a new synthetic isoquinoline alkaloid, may be beneficial in inflammatory disorders where production of NO is excessed by iNOS expression.
Animals ; Aorta, Thoracic ; Gene Expression* ; Lung* ; Macrophages, Peritoneal ; Mice ; Nitric Oxide Synthase Type II* ; Phenylephrine ; Rats* ; RNA, Messenger ; Tumor Necrosis Factor-alpha

Among the several rotenoids, amorphigenin is isolated from the leaves of Amopha Fruticosa and it is known that has anti-proliferative effects and anti-cnacer effects in many cell types. The main aim of this study was to investigate the effects of amorphigenin on osteoclast differentiation in vitro and on LPS treated inflammatory bone loss model in vivo. We show here that amorphigenin inhibited RANKL-induced osteoclast differentiation from bone marrow macrophages in a dose dependent manner without cellular toxicity. Anti-osteoclastogenic properties of amorphigenin were based on a down-regulation of c-fos and NFATc1. Amorphigenin markedly inhibited RANKL-induced p38 and NF-kappaB pathways, but other pathways were not affected. Micro-CT analysis of the femurs showed that amorphigenin protected the LPS-induced bone loss. We concluded that amorphigenin can prevent inflammation-induced bone loss. Thus we expect that amorphigenin could be a treatment option for bone erosion caused by inflammation.
Bone Marrow ; Down-Regulation ; Femur ; Inflammation ; Macrophages ; NF-kappa B ; Osteoclasts ; Osteoporosis ; Rotenone ; T-Lymphocytes