We present a case of tacrolimus-induced encephalopathy after successful kidney transplantation. An 11-year-old girl presented with sudden onset of neurologic symptoms, hypertension, and psychiatric symptoms, with normal kidney function, after kidney transplantation. The symptoms improved after cessation of tacrolimus. Magnetic resonance imaging (MRI) showed acute infarction of the middle cerebral artery (MCA) territory in the right frontal lobe. Three days later, she had normal mental function and maintained normal blood pressure with left hemiparesis. Follow-up MRI was performed on D19, showing new infarct lesions at both cerebral hemispheres. Ten days later, MRI showed further improvement, but brain single photon emission computed tomography (SPECT) showed mild reduction of uptake in both the anterior cingulate gyrus and the left thalamus. One month after onset of symptoms, angiography showed complete resolution of stenosis. However, presenting as a mild fine motor disability of both hands and mild dysarthria, what had been atrophy at both centrum semiovale at 4 months now showed progression to encephalomalacia. There are two points of interest in this case. First, encephalopathy occurred after administration of tacrolimus and improved after discontinuation of the drug. Second, the development of right-side hemiplegia could not be explained by conventional MRI; but through diffusion tensor imaging (DTI) and diffusion tensor tractography (DTT) of white matter tract, visualization was possible.
Angiography ; Atrophy ; Blood Pressure ; Brain ; Cerebrum ; Child ; Constriction, Pathologic ; Diffusion ; Diffusion Tensor Imaging ; Dysarthria ; Encephalomalacia ; Follow-Up Studies ; Frontal Lobe ; Gyrus Cinguli ; Hand ; Hemiplegia ; Humans ; Hypertension ; Infarction ; Kidney ; Kidney Transplantation ; Magnetic Resonance Imaging ; Middle Cerebral Artery ; Neurologic Manifestations ; Paresis ; Tacrolimus ; Thalamus ; Tomography, Emission-Computed, Single-Photon

Bartter syndrome (BS) is a clinically and genetically heterogeneous inherited renal tube disorder characterized by renal salt wasting, hypokalemic metabolic alkalosis and normotensive hyperreninemic hyperaldosteronism. There have been several case reports of BS complicated by focal segmental glomerulosclerosis (FSGS). Here, we have reported the case of a BS patient who developed FSGS and subsequent end-stage renal disease (ESRD) and provided a brief literature review. The patient presented with classic BS at 3 months of age and developed proteinuria at 7 years. Renal biopsy performed at 11 years of age revealed a FSGS perihilar variant. Hemodialysis was initiated at 11 years of age, and kidney transplantation was performed at 16 years of age. The post-transplantation course has been uneventful for more than 3 years with complete disappearance of BS without the recurrence of FSGS. Genetic study revealed a homozygous p.Trp(TGG)610Stop(TGA) mutation in the CLCNKB gene. In summary, BS may be complicated by secondary FSGS due to the adaptive response to chronic salt-losing nephropathy, and FSGS may progress to ESRD in some patients. Renal transplantation in patients with BS and ESRD results in complete remission of BS.
Alkalosis ; Bartter Syndrome ; Biopsy ; Glomerulosclerosis, Focal Segmental ; Humans ; Hyperaldosteronism ; Kidney Failure, Chronic ; Kidney Transplantation ; Proteinuria ; Recurrence ; Renal Dialysis

Intestinal pseudo-obstruction (IPO) is a rare and poorly understood manifestation of systemic lupus erythematosus (SLE), especially in children. The characteristic clinical feature of IPO is obstruction without an identifiable obstructive lesion. The authors a 13-year-old girl whose first symptom of SLE was IPO. The patient presented with a 3-day history of nausea, bilious vomiting, abdominal distention, and no bowel movement. Simple abdominal radiographs revealed mild dilatation with partial air-fluid levels in the small intestine. Abdominal CT and methylcellulose small bowel studies showed massive ascites, engorgement of the small mesenteric vessels, pleural effusion, and diffuse bowel wall thickening of the gastric antrum, duodenum. and jejunum. The delayed passage of contrast for 15 days after the methylcellulose small bowel studies was suggestive of decreased bowel motility. Laboratory findings were positive for ANA, anti-double-stranded DNA, anti-Smith and lymphopenia. After 10-day treatment with high-dose corticosteroids, the symptoms improved. IPO associated with SLE should be considered in the differential diagnosis for patients presenting with symptoms of intestinal obstruction. Early recognition of IPO in SLE and appropriate therapy are important for prevention of complications and unnecessary surgery. This case raises awareness among pediatricians that although rare, IPO can be the presenting symptom of SLE in children.
Adolescent ; Adrenal Cortex Hormones ; Ascites ; Child ; Diagnosis, Differential ; Dilatation ; DNA ; Duodenum ; Humans ; Intestinal Obstruction ; Intestinal Pseudo-Obstruction ; Intestine, Small ; Jejunum ; Lupus Erythematosus, Systemic ; Lymphopenia ; Methylcellulose ; Nausea ; Pleural Effusion ; Pyloric Antrum ; Unnecessary Procedures ; Vomiting

Glycogen storage disease (GSD) and mucopolysaccharidosis (MPS) are both independently inherited disorders. GSD is a member of a group of genetic disorders involving enzymes responsible for the synthesis and degradation of glycogen. GSD leads to abnormal tissue concentrations of glycogen, primarily in the liver, muscle, or both. MPS is a member of a group of inherited lysosomal storage diseases, which result from a deficiency in specific enzymatic activities and the accumulation of partially degraded acid mucopolysaccharides. A case of a 16-month-old boy who presented with hepatomegaly is reported. The liver was four finger-breadth-palpable. A laboratory study showed slightly increased serum AST and ALT levels. The liver biopsy showed microscopic features compatible with GSD. The liver glycogen content was 9.3% which was increased in comparison with the reference limit, but the glucose-6-phosphatase activity was within the normal limit. These findings suggested GSD other than type I. Bony abnormalities on skeletal radiographs, including an anterior beak and hook-shaped vertebrae, were seen. The mucopolysaccharide concentration in the urine was increased and the plasma iduronate sulfatase activity was low, which fulfilled the diagnosis criteria for Hunter syndrome (MPS type II). To the best of the authors' knowledge, this is the first case of GSD and Hunter syndrome being identified at the same time.
Animals ; Beak ; Biopsy ; Glucose-6-Phosphatase ; Glycogen ; Glycogen Storage Disease ; Glycosaminoglycans ; Hepatomegaly ; Humans ; Iduronate Sulfatase ; Infant ; Liver ; Liver Glycogen ; Lysosomal Storage Diseases ; Mucopolysaccharidoses ; Mucopolysaccharidosis II ; Muscles ; Plasma ; Spine

Hallermann-Streiff syndrome is a rare disease. Approximately 150 cases have been reported, including 6 cases in Korea. The authors experienced a case of Hallermann-Streiff syndrome in a 6-year-old female with aphakia. The syndrome is characterized by a bird-like face, dental abnormalities, hypotrichosis, atrophy of the skin, bilateral microphthalmia, and proportionate dwarfism. A brief review of the literature was conducted.
Aphakia ; Atrophy ; Child ; Dwarfism ; Female ; Hallermann's Syndrome ; Humans ; Hypotrichosis ; Korea ; Microphthalmos ; Rare Diseases ; Skin

PURPOSE: It is widely known that allergic diseases progress through an allergic march. However, there have not been any recent reports in Korea on how the diseases progress. METHODS: Parents who visited one of the Pediatric Allergy Clinics of four university hospitals in Seoul with a child under the age of 6 years during the period from May 1-30, 2006 were asked to complete a questionnaire. RESULTS: A total of 229 parents answered the questionnaire about their children, of which 122 were male and 107 were female. The most common allergic disease before 2 years of age was atopic dermatitis (79%). Meanwhile, in 2- to 4-year age group, allergic rhinitis (38%) and asthma (37%) were predominant. The most common allergic disease in the 4- to 6-year age group was asthma (72%), followed by allergic rhinitis (64%). Seventy-three percent of the children had a family history of allergic disease, most often in the fathers (39%). Among patients with allergic rhinitis, 50% had experienced asthma in their earlier days and 30% had experienced atopic dermatitis. In addition, 57% of the children with asthma had suffered from atopic dermatitis. Parents believed that asthma was the most serious allergic disease. CONCLUSION: In the present study, allergic disease showed a tendency to march from atopic dermatitis to asthma and then to allergic rhinitis. Early diagnosis and treatment of atopic dermatitis is, therefore, considered important for prevention of the allergic march.
Asthma ; Child ; Dermatitis, Atopic ; Early Diagnosis ; Fathers ; Female ; Hospitals, University ; Humans ; Hypersensitivity ; Korea ; Male ; Parents ; Surveys and Questionnaires ; Rhinitis ; Rhinitis, Allergic, Perennial

PURPOSE: There has been considerable disagreement regarding the most appropriate dosage of gonadotropin-releasing hormone agonist in cases of central precocious puberty. The aim of this study was to determine the appropriate dosage for suppression of the puberty in girls with central precocious or early puberty. METHODS: Twenty-two girls with early puberty were randomly subjected to 3 types of dosages of leuprolide acetate for at least 6 months. The number of cases in groups 1, 2, and 3 were 7, 7, and 8, and dosages were 70, 90, and 110 microgram/ kg/-month, respectively. Height, weight, bone age, Tanner stage of breast development, and serum levels of LH, FSH, estradiol, and progesterone were measured before treatment and after 6 months of treatment. The number of cases of puberty suppression was compared using a modified puberty suppression score with a nonparametric chi-square test. RESULTS: There were no significant differences of chronologic and bone ages among the groups. There was a significant decrease in height SDS gain after 6 months in group 3 (P<0.05) compared with groups 1 and 2. Serum levels of LH, FSH, estradiol and progesterone were all significantly decreased after treatment in all 3 groups (P<0.05). The number of cases of puberty suppression in each group were 4 (57%), 5 (71%), and 8 (100%). There was a significantly increased proportion of suppression of puberty in group 3 (P<0.05). CONCLUSION: It was necessary to use a higher dose of gonadotropin-releasing hormone agonist to suppress early puberty in girls; however further longitudinal study will be needed for their prognosis of final adult height.
Adult ; Breast ; Estradiol ; Gonadotropin-Releasing Hormone ; Humans ; Leuprolide ; Longitudinal Studies ; Progesterone ; Prognosis ; Puberty ; Puberty, Precocious

PURPOSE: This study was designed to investigate normal domestic values for the diameter of the left main coronary artery (LCA), the left anterior descending coronary artery (LAD) and the right coronary artery (RCA). These data are necessary to define dilatation of coronary arteries in Kawasaki disease cases. METHODS: Study subjects were 43 normal healthy children whose ages ranged from 3 months to 6 years. They children visited Konkuk University hospital for echocardiograph examination between March 2005 and November 2007. Measurements of coronary arterial diameters at each branch were done by off-line analyses of recorded images. Simple regression analysis of each the measurements were performed using the body size (body surface area, etc.) as the independent variable. RESULTS: Body surface area was significantly related to the diameters of LCA (r(2)=0.20, P=0.0038), of LAD (r(2)=0.41, P< 0.0001), and of RCA (r(2)=0.30, P=0.0002). In the regression model, the estimates of the y-intercept were 1.703, 1.058, and 1.007; the estimates of the regression coefficient were 0.971, 1.175, and 1.177; and the estimates of the standard deviation were 0.315, 0.221, and 0.282 with respect to the three coronary arteries. CONCLUSION: A the linear regression model of the diameters of three coronary arteries adjusted for body surface area was produced. With these results, the Z-score calculation of the diameter of three coronary arteries, based on normal domestic data, will be possible.
Aged ; Body Size ; Body Surface Area ; Child ; Coronary Vessels ; Dilatation ; Humans ; Linear Models ; Mucocutaneous Lymph Node Syndrome

PURPOSE: The purpose of this study was to evaluate the immunogenicity of the booster immunization with pneumococcal conjugate vaccine in Korean children. METHODS: Thirty-nine children aged 12-23 months who visited Kangnam CHA Hospital between September 2006 and December 2006 were enrolled. The children were divided into primary and booster groups depending on their vaccination status for the 7-valent pneumococcal conjugate vaccine. The anti-pneumococcal antibody levels of each serotype included in the vaccine (4, 6B, 9V, 14, 18C, 19F, 23F) were determined by third-generation ELISA. RESULTS: The geometric mean titer (GMT) of antibodies to each pneumococcal serotype in the booster group was higher than in the primary group (P<.05). The percentage of subjects with pneumococcal antibodies > or =0.35 microgram/mL was 90.5-100% for all serotypes in both the primary and booster groups. The percentage of subjects with pneumococcal antibodies > or =1.0 g/mL in the booster group was 94.4-100%, which was higher than the primary group except for serotypes 6B and 14 (P<.05). The percentage of subjects with pneumococcal antibodies > or =5.0 microgram/mL in the booster group was 50.0-94.4% which was higher than the primary group for all serotypes (P<0.05). CONCLUSION: The immunogenicity of a booster dose of the pneumococcal conjugate vaccine in Korean children was high and the immunogenicity of a primary series was also relatively high. To determine the feasibility of the introduction of the pneumococcal conjugate vaccine and the appropriate schedule for Korean children, further prospective investigation of the immunogenicity of the booster immunization is needed.
Aged ; Antibodies ; Appointments and Schedules ; Child ; Enzyme-Linked Immunosorbent Assay ; Humans ; Immunization ; Immunization, Secondary ; Vaccination

PURPOSE: This study was undertaken to identify factors that influence 17-OHP levels in preterm infants and to suggest a reasonable follow-up schedule of screening for congenital adrenal hyperplasia (CAH) in preterm infants. METHODS: The 17-OHP concentrations in filter paper blood spots of 427 preterm infants were obtained. The effects of gestational age (GA), systemic diseases, and antenatal dexamethasone on screening and follow-up 17-OHP values were investigated. RESULTS: The screening 17-OHP values were markedly variable (range: 0.1-143.3 ng/mL). The screening 17-OHP levels were negatively correlated with GA (r=-0.535, P<0.01). In infants with GA <32 weeks, the screening 17-OHP levels were significantly higher in sick infants or infant with hypotension than in healthy infants. The screening values of prenatal dexamethasone-treated infants had a tendency to be low. In infants with initial 17-OHP values > or =20 ng/mL, the intervals until rescreening 17-OHP <10 ng/mL or serum 17-OHP <20 ng/mL were negatively correlated with GA (r=-0.541, P<0.01) and were prolonged in infants with bronchopulmonary dysplasia (P<0.01). None of the preterm infants were confirmatively diagnosed with CAH. CONCLUSION: The 17-OHP values of preterm infants were influenced by GA, prenatal dexamethasone, and postnatal diseases. Because the 17-OHP vlues of preterm infants were markedly variable, a follow-up schedule should be developed considering both 17-OHP values and clinical status.
Adrenal Hyperplasia, Congenital ; Appointments and Schedules ; Bronchopulmonary Dysplasia ; Dexamethasone ; Follow-Up Studies ; Gestational Age ; Humans ; Hypotension ; Infant ; Infant, Newborn ; Infant, Premature ; Mass Screening ; Neonatal Screening