Evaluation of the efficacy and safety of conversion from the tacrolimus capsule to tablet in stable liver transplant recipients with maintenance therapy: a 24-week, open-label, single-center, phase IV exploratory clinical study

Purpose@#The tablet form of tacrolimus is more convenient for drug ingestion than the capsule form. We examined the efficacy and safety of tacrolimus tablets and a satisfaction survey after formula conversion in liver transplant (LT) recipients. @*Methods@#This study was an open-label, prospective clinical trial for tacrolimus formula 1:1 conversion from capsule to tablet in 41 adult LT recipients with tacrolimus maintenance therapy of more than 1 month. The primary endpoint was incidence of biopsy-proven acute rejection (BPAR) within 24 weeks. Surveys 1 week before and 4 weeks after formula conversion were conducted for total daily dose of medication, number, scale of discomfort and satisfaction. @*Results@#The overall incidence of BPAR was 0% and there was no graft loss or patient death. The incidence of adverse effects was 34.1% (n = 14) after formula conversion. The most common severe adverse effect was abnormal liver function test (n = 5): biliary complications (n = 4) and alcoholic recidivism (n = 1). Total daily dose and number of tacrolimus doses were significantly lower after formula conversion (P < 0.05) without changes in trough level. According to survey analysis, there was no significant difference in discomfort and satisfaction scales from capsule to tablet conversion (P < 0.05). @*Conclusion@#The present study suggests that the new tablet formula can be a useful treatment option to maintain a consistent level of tacrolimus with a lower total daily dose and number in adult LT recipients.

Modified Charlson comorbidity index as a survival prediction tool for older patients after liver transplantation

Purpose@#An increasing number of older patients now undergo liver transplantation (LT). Although the overall outcomes in older patients are not different from those of younger patients, there is no tool to predict LT prognosis in older patients.We hypothesized that a modified Charlson comorbidity index (mCCI) and 5-factor modified frailty index (mFI-5) can predict outcomes in older patients after LT. @*Methods@#This retrospective study included 155 patients (aged >65 years) who underwent LT at Seoul National University Hospital. The recipients were subcategorized into 2 groups based on the mCCI score and mFI-5: the low (0–1) and high (2–5) mCCI groups, and low (≤0.4) and high (>0.4) mFI-5 groups. The independent effect of each variable on post-LT survival was determined using the mCCI subgroup, age at transplantation, sex, Child-Turcotte-Pugh score, model for end-stage liver disease (MELD) score, and mFI-5 subgroup. @*Results@#The high-mCCI group (41 patients) showed significantly lower 1- and 3-month and 1-, 3-, and 5-year survival than the low-mCCI group. Using the Cox regression model, the mCCI, sex, and MELD score remained significant. The mFI-5 was not a significant factor to predict patients’ survival. @*Conclusion@#The mCCI and MELD scores could be used to predict post-LT survival in older patients.

Recurrence in patients with totally necrotic nodules of hepatocellular carcinoma after liver transplantation: “totally” an inaccurate description

Purpose@#Total necrosis of hepatocellular carcinoma (HCC) achieved via locoregional treatment (LRT) is considered to indicate a lack of tumor viability. Nonetheless, there is insufficient evidence of recurrence after liver transplantation (LT) in patients with such a status. The aim of this study was to investigate the prognosis of patients diagnosed with totally necrotic nodules upon explant hepatectomy after LT. @*Methods@#We conducted a retrospective study of patients diagnosed with totally necrotic nodules after LT for HCC. A total of 165 patients with HCC who underwent living- or deceased-donor LT from 2000 to 2020 in our hospital were included. @*Results@#A total of 5 patients (3.0%) exhibited HCC recurrence during a median follow-up of 84 months (range, 4–243 months) after LT. The 5-year overall and recurrence-free survival rates of these patients were 92.8% and 92.2%, respectively. Four patients in the HCC-recurrence group (80.0%) died even after further treatment, including transarterial chemoembolization, surgery, and systemic treatment. Both univariate and multivariate analyses of clinicopathological factors identified a maximum diameter of the totally necrotic nodules of >5 cm as the only factor associated with tumor recurrence following LT (P = 0.005 and P = 0.009, respectively). @*Conclusion@#Total necrosis of HCC via LRT yielded excellent survival outcomes for patients undergoing LT. Nevertheless, patients with large tumors should be considered at high risk of recurrence after LT, suggesting the need for their active surveillance during the follow-up period.

Long-term outcomes of liver transplantation using grafts from donors with active hepatitis B virus replication: a multicenter cohort study

Purpose@#Liver grafts from donors with HBV infection contributed to expanding the donor pool under the hepatitis B immunoglobulin and antiviral agents (nucleos(t)ide analogues) in the HBV-endemic area. We report long-term outcomes of liver transplantations (LTs) using grafts from donors with active or chronic HBV infection. @*Methods@#Overall, 2,260 LTs performed in 3 major hospitals in Seoul from January 2000 to April 2019 were assessed for inclusion. Twenty-six grafts (1.2%) were obtained from HBsAg (+), HBeAb (+), or HBcAb (+) donors, and recipient outcomes were retrospectively reviewed. Donor and recipient demographics and transplantation outcomes were analyzed. @*Results@#Sixteen deceased donor LTs were performed using active HBsAg (+) grafts. Ten other LTs were sourced from 10 living donors. There was no significant difference in survival in patients who received deceased donor LTs compared with that in those who underwent LT with non–hepatitis virus-infected grafts. Fourteen patients who were followed up for >5 years were stable, and no difference in hepatocellular carcinoma recurrence rate was observed 5 years after transplantation between transplants from donors with and those without HBV. @*Conclusion@#Considering long-term outcomes, liver grafts from donors with active HBV replication can be safely used for LT.

Pure laparoscopic versus open right donor hepatectomy including the middle hepatic vein: a comparison of outcomes and safety

Purpose@#Analyses on pure laparoscopy in donor hepatectomies, including the middle hepatic vein (MHV), are still scarce. This study aimed to compare the outcomes of donor right hepatectomy, including the MHV, when performed laparoscopically with conventional open surgery. @*Methods@#Data from living donors who underwent donor right hepatectomy between January 2012 and December 2020 were retrospectively analyzed. The intraoperative and postoperative complication rates of the pure laparoscopic donor right hepatectomy (PLDRH) with MHV inclusion (PLDRHM) group were compared with the conventional open donor right hepatectomy with MHV inclusion (CDRHM) group and the PLDRH without MHV inclusion [PLDRHM(–)] group. @*Results@#Compared to the CDRHM group, the PLDRHM group had a longer bench time (P < 0.001) and higher Δ%,calculated as [(preoperative value – postoperative value)/preoperative value] × 100, of AST (P < 0.001), ALT (P < 0.001), and total bilirubin (P = 0.023), but shorter hospital stay (P = 0.004) and a lower rate of complications (P = 0.005). Compared to the PLDRHM(–) group, the PLDRHM group had fewer male donors (P < 0.001) and a lower body mass index (P < 0.001), estimated total liver volume (P < 0.001), and real graft weight (P < 0.001). Results of laboratory changes, hospital stays, and complication rates were similar between the 2 groups. @*Conclusion@#PLDRH with the inclusion of the MHV in selected donors and recipients is feasible and safe when performed by surgeons experienced in laparoscopic surgery, with favorable complication rates compared to CDRHM and PLDRHM(–).

Impact of tumor size on hepatectomy outcomes in hepatocellular carcinoma: a nationwide propensity score matching analysis

Purpose@#The aim of this study was to compare surgical outcomes after liver resection for hepatocellular carcinoma (HCC) according to tumor size using a large, nationwide cancer registry-based cohort and propensity score matching. @*Methods@#From 2008 to 2015, a total of 12,139 patients were diagnosed with liver cancer and registered in the Korean Primary Liver Cancer Registry. Patients without distant metastasis who underwent hepatectomy as a primary treatment were selected. We performed 1:1 propensity score matching between the small (<5 cm), large (≥5 cm and <10 cm), and huge (≥10 cm) groups. @*Results@#Overall, 265 patients in the small and large groups were compared, and 64 patients each in the large and huge groups were compared. The overall and progression-free survival rates were significantly lower in the large group than in the small group (P < 0.001 and P < 0.001, respectively). Overall survival tended to be poorer in the huge group than in the large group (P = 0.051). The progression-free survival rate was significantly lower in the huge group than in the large group (P = 0.002). @*Conclusion@#Although primary liver resection can be considered even in patients with huge HCC, greater caution with careful screening for recurrence is needed.

Limits of serum carcinoembryonic antigen and carbohydrate antigen 19-9 as the diagnosis of gallbladder cancer

Purpose@#Although serum CEA and CA 19-9 have been widely utilized for the diagnosis of gallbladder cancer (GBC), few studies have examined the diagnostic performance of them. This study aimed to investigate the diagnostic performance of these 2 biomarkers and demonstrate their clinical usefulness in diagnosing GBC. @*Methods@#Between January 2000 and March 2020, a total of 751 GBC patients and 2,310 normal controls were included. Serum CEA and CA 19-9 were measured preoperatively. Receiver operating characteristic curves were obtained, and the sensitivity and specificity of each biomarker were evaluated. @*Results@#In terms of differentiating GBC from the control, the sensitivity and specificity of serum CEA at 5 ng/mL was 12.1% and 99.1%, respectively, and those of serum CA 19-9 at 37 IU/mL were 28.7% and 94.5%, respectively. The optimal cutoff values of CEA and CA 19-9 were set to 2.1 ng/mL and 26 IU/mL in the receiver operating characteristic curves, respectively.The sensitivities of CEA and CA 19-9 at new cutoff values slightly increased but remained low (CEA, 42.9%; CA 19-9, 38.2%). When differentiating early-stage GBC from advanced tumor, the sensitivity and specificity, were 14.2% and 96.1% for CEA (cutoff value, 5 ng/mL) and 33.6% and 90.1% for CA 19-9 (cutoff value, 37 IU/mL), respectively. @*Conclusion@#Serum CEA and CA 19-9 levels are not suitable for screening GBC patients from controls. New promising biomarkers with higher sensitivity should be explored.

Incidence trends and associated factors of diabetes mellitus in Korean children and adolescents: a retrospective cohort study in Busan and Gyeongnam

PURPOSE: This study investigated the incidence trends and associated factors of type 1 (T1DM) and type 2 diabetes mellitus (T2DM) in children and adolescents under 15 years of age in Busan and Gyeongnam, Korea from 2001 to 2010. METHODS: Medical records of newly diagnosed diabetes patients (n=328; 160 males, 168 females) were collected in questionnaire form from 5 tertiary and 42 general hospitals in Busan and Gyeongnam. RESULTS: The average crude incidence rate of T1DM and T2DM was 2.01/100,000 (95% confidence interval [CI], 1.76-2.28) and 0.75/100,000 (95% CI, 0.60-0.92), respectively. The incidence rate ratio (IRR) of T1DM was 1.31 (95% CI, 1.01-1.69), and that of T2DM was 1.97 (95% CI, 1.25-3.11) in the latter half-decade (2006 to 2010) compared to the early half-decade (2001 to 2005). There were gradually increasing incidence trends in both T1DM and T2DM over the 10-year period (P for trend: T1DM, 0.0009; T2DM, <0.0001). Age-specific IRR was highest in the 10- to 14-year-old group, regardless of diabetes type. In particular, a rapid increase in incidence of T2DM occurred in the 10- to 14-year-old group. IRR for females was 1.07 (95% CI, 0.83-1.38) for T1DM and 1.56 (95% CI, 1.01-2.41) for T2DM. IRR for Busan (urban) was 1.41 (95% CI, 1.09-1.83) for T1DM and 1.49 (95% CI, 0.96-2.30) for T2DM. CONCLUSION: T1DM and T2DM incidence both increased over time in youth under age 15 living in Busan and Gyeongnam; in particular, the incidence of T2DM in adolescents increased more rapidly.

Clinical Course of Childhood Onset Pseudoprecocious Puberty due to Autonomous Ovarian Cyst / 대한소아내분비학회지

PURPOSE: There are few reports about the natural history of patients with pseudoprecocious puberty due to autonomous ovarian cyst. We reviewed the clinical course of 7 patients who had autonomous ovarian cysts and signs of precocious puberty. METHODS: We retrospectively evaluated 7 children, aged 2.8 to 7.9 years, who were diagnosed with pseudoprecocious puberty due to autonomous ovarian cysts from November 2005 to May 2011. The follow-up durations ranged from 0.5 to 6.3 years. RESULTS: Four out of 7 patients showed elevated serum estrogen levels and all revealed prepubertal response of gonadotropin to GnRH stimulation at diagnosis. The size of the cysts was from 1.7 to 4.6 cm on pelvic ultrasound examination. After 1 to 3 months, the ovarian cysts disappeared in all patients. Three of the girls developed relapsing signs of precocious puberty (vaginal bleeding and breast budding). Two of them showed an increase in growth velocity and bone age due to recurrent ovarian cysts, and one of them was converted to true precocious puberty. CONCLUSION: In our cases, all patients with autonomous ovarian cysts resolved spontaneously. However, some showed frequent recurrence of ovarian cysts, and needed a longer follow up because of the possibility of conversion to true precocious puberty and signs of McCune-Albright syndrome.

Clinical Significance of Cone-shaped Epiphysis and Brachymesophalangia of the Fifth Middle Phalanx in Korean Children with Normal Short Stature / 대한소아내분비학회지

PURPOSE:The cone-shaped epiphyses mid-5 (CSE-5) and brachymesophalagia-5 (BMP-5) are common osseous anomalies. Those are thought to be normal variants. We evaluated the frequency of CSE-5 and BMP-5 and the influence of them on adult height in Korean children with normal short stature. METHODS:We retrospectively reviewed medical records of 322 normal short stature children. Lengths of the fourth (MP-4) and fifth middle phalanx (MP-5) and widths of MP-5 of all children were measured. Two indicies for BMP-5 were used. Index 1 was based upon the ratio of the width to the length of the MP-5. Index 2 was based upon the ratio of the lengths of MP-5 to MP-4. CSE-5 was assessed by visual inspection only. We assessed several clinical parameters as follows; advanced skeletal maturation, z-scores of height, target height (THz) and predicted adult height (PAHz) according to CSE-5 and/or BMP-5. Results:Of the 322 children, 23.6% had BMP-5 (male 19.5%, female 27.4%), 23.6% had CSE-5 (male 13.0%, female 33.3%). The children with CSE-5 and/or BMP-5 were more advanced skeletal maturation than normal fifth finger (0.07+/-1.09 yrs vs -0.23+/-1.34 yrs, P=0.049), lower PAHz (-1.13+/-1.09 vs -0.71+/-1.28, P=0.008), lower PAHz- THz (-0.53+/-1.07 vs -0.14+/-1.30, P=0.013). In male subjects, the PAHz had weak correlation with index 1 (r=-0.26, P=0.001) and index 2 (r=0.27, P=0.001). CONCLUSION:This study suggests that BMP-5 and CSE-5 in Korean children with short stature are one contributable factor for adult height.

Readmission in Neonatal Period among the Normal Full-term Neonates / 대한주산의학회잡지

PURPOSE:This study was conducted to analyse the characteristics of the full-term neonates admitted in neonatal period and to evaluate the relationship between readmission and perinatal, maternal factors. METHODS:We studied retrospectively the neonatal readmissions who were born in Il Sin Christian Hospital during the 18-months period (January 1, 2007 to June 30, 2008). RESULTS:After discharge, 415 neonates were readmitted during the first month of life. The most common cause for readmission was jaundice (82.7%). Among them, 211 (61.5%) neonates were fully breast feeding and 119 (34.7%) were mixed feeding infants. The second cause was rota enteritis (6.3%). Others included dehydration (4.1%), suspected sepsis (3.1%), other enteritis (1.7%), pneumonia (0.7%), cardiac problem (0.5%), meningitis (0.5%), urinary tract infection (0.2%), and other urinary problem (0.2%). There were no significant relationships between readmission and neonatal, maternal factors. CONCLUSION:While newborns discharged from hospital are at risk for readmission, but relationship among neonatal, maternal, and other factors are not proven. Education of risk factors for jaundice to parents should be done and prevention of hospital acquired infection is important. Further research should include relationships between neonatal, maternal factors and the other factors influencing neonatal readmission.

A comparative study on iron deficiency anemia based on feeding patterns of nine-month-old infants / 소아과

PURPOSE: This study was conducted to evaluate the red cell indices and frequency of iron deficiency anemia based on the feeding patterns of nine-month-old infants. METHODS: Blood tests were performed on 253 nine-month-old infants, who visited Il Sin Christian Hospital for health checkups from January to December 2007. Their parents answered telephonic questions regarding their feeding patterns and weaning foods. RESULTS: Three infants groups were created according to feeding patterns before they started weaning foods. One group was exclusively breast-fed (48.6%), another had mixed feeding (27.3%), and the third had artificial milk feeding (24.1%). Red cell indices (hemoglobin, hematocrit, MCV, MCH) of the breast-fed group were comparatively lower than those of the other two groups (P<0.05). Twenty-five infants (9.9%) were diagnosed with iron-deficiency anemia. According to feeding patterns, the frequency of anemia was highest in the breast-fed group. Six infants who started weaning foods before six months of age (113 infants) were diagnosed with iron-deficiency anemia (5.3%), and nineteen who started after six months of age (140 infants) were diagnosed with iron-deficiency anemia (13.6%). CONCLUSION: When nine-month old infants visit hospitals for health check-ups, pediatricians must consider their feeding pattern and weaning foods histories, and then recommend screening blood tests for iron-deficiency anemia.

Antigen Presenting Cells Appearing in Injured Rat Brain Detected by Immunohistochemistry Method

PURPOSE: Traumatic brain injury is a multifaceted injury that involves direct mechanical damage, intraparenchymal and subarachnoid hemorrhage, breakdown of the blood- brain barrier, excitotoxicity, and ischemia. Despite the dozens of previous investigations, the information about its pathogenic mechanism is still limited. The aim of this study was to reveal the appearance of antigen presenting cells in the cerebral cortex of rats after cauterization. METHODS: A total of 18 male Sprague-Dawley rats weighing 300 g and 2 months old on the average were used throughout the experiment. The frontal bones were exposed by elevating the skin and craniectomies were performed adjacent to the central suture, midway between lambda and bregma. Cauterizing injury was then created by battery-operated small vessel cauterizers to the left frontal cortex. The rats were sacrificed on the 1st, 4th, 7th and 14th days after the surgery(n=3, each time), and three rats were sacrificed as normal controls. Serial brain cryosections were made by cryostat. For immunohistochemistry, brain tissue sections were allowed to react with mouse anti-rat MHC class II antibody(1:500) and mouse anti-rat ED2 antibody(1:200). Also, brain tissues were routinely stained by H-E, and then microscopic observation and cell counts were performed. RESULTS: 1) MHC class II positive dendritic cells were absent in normal cerebral cortex parenchyme, but were found 28 times more in number in injured rats on the 7th day after cauterization. 2) ED2 positive macrophages were absent in normal cerebral cortex parenchyme, and were found 16 times more in number in injured rats on the 7th day after cauterization. 3) The number of MHC class II positive dendritic cells were smaller in number than that of ED2 positive macrophages 6 hours and 1st day later after cauterization, but it was higher in number on the 4th, 7th and 14th days. 4) The number of MHC class II positive dendritic cells were higher in number than that of ED2 positive macrophages around blood vessels and peripheral regions in the injured brain. 5) MHC class II positive dendritic cells were usually aggregated. CONCLUSION: It can be suggested that the increase in number of two kinds of antigen- presenting cells affect cell-mediated immune responses and phagocytosis.

Perinatal outcomes according To chorionicity in Twin Gestations

PURPOSE: The purpose of this study was to assess the natural history and perinatal outcomes of twin gestations according to chorionicity. METHODS: We retrospectively reviewed the medical records of 99 monochorionic (MC) and 206 dichorionic (DC) twin gestations delivered at Il Sin Christian Hospital in Busan between January 2002 and December 2007. The incidences of twin-to-twin transfusion syndrome (TTS) and selective intrauterine growth restriction (sIUGR), as well as perinatal morbidity and mortality, were evaluated. RESULTS: MC twins had a lower gestational age (35.7 vs. 36.6 weeks, P=0.03) at birth and a higher incidence of intrauterine fetal loss (10% vs. 1.5%, P<0.001) than DC twins. The incidence of intrauterine fetal loss was higher in MC sIUGR than in DC sIUGR (19% vs. 2.5%, P=0.025) twins. The number of admissions to the neonatal intensive care unit (NICU; 31% vs. 16%, P=0.042), and the incidence of periventricular leukomalacia (7% vs. 0%, P=0.031), and respiratory distress syndrome with surfactant treatment (27% vs. 11%, P=0.049) were higher in MC than DC twins. The incidences of sIUGR and TTS were 21 and 9% among the MC twins. The incidences of intrauterine fetal loss were higher in MC twins with TTS [6 of 9 (67%)] or sIUGR [4 of 21 (19%)] than uncomplicated MC twins (P<0.001). The frequency of admission to the NICU (P=0.001), the length of hospital stay (P=0.033), the prevalence of periventricular leukomalacia (P=0.011), and intraventricular hemorrhage (P=0.007) were also higher in MC with TTS or sIUGR than in uncomplicated MC twins. CONCLUSION: The incidence of neonatal complications was higher in MC twins, especially those gestations complicated by TTS or sIUGR.

Factors influencing birth weight premature infants / 소아과

PURPOSE: The purpose of this study was to identify factors influencing premature infants who are small for their gestational ago. METHODS: The medical records of 1,010 premature infants of 26 to 35 weeks of gestational age born at Il-Sin Christian Hospital, Busan from January 2000 to August 2006 were reviewed. We collected data on gestational age, birth weight, infant gender, birth order, maternal age and previous abortion history and analyzed the factors influencing premature infants who were small for their gestational ago at birth. RESULTS: In our study more female than male (P=0.042) in premature infants who were small for their gestational ago were born from mothers aged younger than 20 or older than 35 (P=0.041). But association between smallness for gestational age and birth order or maternal previous abortion history was statistically insignificant (P=0.228, P=0.129). CONCLUSION: Considering the association of birth weight and the survival rate of premature infants, it is thought that maternal age had an influence on the survival rate of premature infants. Social and political support to lower the teenager pregnancy and older pregnancy is expected to increase the survival rate of premature infants and the birth of healthy normal neonates.

Insulin Sensitivity in Prepubertal Children born with Low Birth Weight / 대한소아내분비학회지

PURPOSE: We aimed to determine whether prepubertal children born with low birth weight (LBW) have decreased insulin sensitivity, compared with children born with normal birth weight and to know a relationship between insulin sensitivity and adiponectin concentration in those children. METHODS: LBW (n=33) was defined as a birth weight less than 2.5 kg and control (n=20) was defined as a birth weight between 2.5 kg and 4.0 kg. Height and weight were measured for calculation of BMI. Fasting blood samples were taken for the measurements of glucose, insulin, lipid and adiponectin concentration. Body fat mass and abdominal fat ratio were measured. HOMA-IR and QUICKI were calculated, as a mean of insulin sensitivity. RESULTS: Children with LBW showed significantly higher levels of QUICKI and adiponectin than control group (P-1) were more resistant to insulin than shorter children with LBW (height SDS6 yr) children with LBW. In children with LBW, age was negatively correlated with QUICKI adjusted by BMI percentile (r=-0.373, P<0.05). CONCLUSION: These findings suggest that the levels of adiponectin increase to compensate for the diminished insulin sensitivity in younger children with LBW and this phenomenon is faded away with aging. Additionally, the results suggest that LBW children with better postnatal growth have a tendency to have insulin resistance.

Factors Affecting Final Adult Height in Patients with Turner Syndrome / 소아과

PURPOSE: Short stature is one of the characteristic features of Turner syndrome. We investigated the factors affecting final adult height(FAH) in patients with Turner syndrome. METHODS: The study group was comprised of 60 patients who were diagnosed with Turner syndrome by chromosomal study and clinical phenotypes and attained FAH. Data were obtained from retrospective review of the medical records. We analyzed the factors influencing FAH in growth hormone(GH) treated and GH untreated groups. RESULTS: Sixty patients were enrolled; 48 patients received GH treatment, and 12 patients did not. Mean duration of GH treatment was 35.8 months(range 4 to 120 months), and mean dosage of GH was 0.8+/-0.2 IU/kg/wk in GH treated group. Mean growth velocity was 5.6+/-2.0 cm/yr, which was significantly higher than that during pretreatment period. In the GH treated group, mean chronological age, bone age, mean height, and height standard deviation(SD) score at GH treatment were 12.2+/-2.7 yr, 10.3+/-2.5 yr, 127.5+/-10.1 cm and -3.1+/-1.1, respectively. In the GH treated group, the mean FAH and SD score of FAH were 146.9+/-5.8 cm and -2.7+/-1.2, respectively, which showed significant differences compared with those of the GH untreated group. Analyzing the factors affecting FAH in GH-treated patients, only the SD score of height at the time of treatment was significantly related to FAH. CONCLUSION: GH treatment leads to an increment in FAH in patients with Turner syndrome. Average FAH gain was as much as 5.8 cm. SD score of height at the time of GH treatment was the only factor influencing FAH.

A Case of Reversible Posterior Leukoencephalopathy Involving in the Spinal Cord with Pheochromocytoma

Reversible posterior leukoencephalopathy with hypertension is characterized by an acute and severe rise in blood pressure with headache, altered mental status, cortical visual disturbance, seizure and transient edematous changes in neuroimaging. The most common abnormality in neuroimaging is presumed edema involving the cortical and subcortical white matter predominant in the posterior region of the cerebral hemisphere and rarely the cerebellum and the brain stem, but not in the spinal cord. We experienced a case of 10-year-old girl with hypertensive encephalopathy involving the brainstem and the spinal cord.

A Case of Hyperinsulinism/hyperammonemia Syndrome / 대한소아내분비학회지

The hyperinsulinism/hyperammonemia (HI/HA) syndrome is a form of congenital hyperinsulinism. The children with HI/HA syndrome present recurrent symptomatic hypoglycemia and asymptomatic, persistent hyperammonemia, caused by mutations of the GLUD1 encoding the mitochondrial enzyme, glutamate dehydrogenase (GDH). The mutations impair sensitivity to the inhibition of GTP (guanosine triphosphate), which results in stimulation of insulin secretion from pancreatic beta-cells and increased rates of ammonia production. Leucine is known to mediate the insulin secretion. We report HI/HA syndrome with a 12-month-old male who had intermittent hypoglycemia. We revealed characteristic clinical findings of hypoglycemia induced by oral administration of protein in this patient who had mutations of GLUD1 (S445L).

Final Adult Height and Its Influencing Factors in Patients with Congenital Adrenal Hyperplasia Caused by 21-Hydroxylase Deficiency / 대한소아내분비학회지

PURPOSE: We evaluated adult final height and factors influencing adult height outcome in patients with congenital adrenal hyperplasia (CAH) caused by 21-hydroxylase deficiency. METHOD: We retrospectively reviewed medical records of 20 male and 22 female patients with 21- hydroxylase deficiency who reached adult final height from 1980 to 2004. We obtained final adult height and analysed possible factors influencing final adult height such as urine 17-ketosteroid (17- KS), plasma 17-OH-progesterone (17-OHP), plasma renin activity, pubertal initiaton age, glucocorticoid dose, age at diagnosis and effect of growth hormone or gonadotropin releasing hormone analogue. RESULT: Mean FH (final height) SDS-MPH (midparental height) SDS were -1.89+/-1.0 in male simple virilization (SV) and -0.83+/-0.8 in female SV patients, -2.27+/-1.3 in male salt wasting (SW) and -1.12+/-1.1 in female SW patients. Mean final adult height and mean FH SDS-MPH SDS were not different between SV and SW patients, but mean FH SDS-MPH SDS in males was significantly lower than that of females (-2.12+/-1.2 vs 0.95+/-0.9, P<0.05). In SW mean FH SDS-MPH SDS were correlated positively with pubertal height gain (r=0.484. P<0.05) and correlated negatively with pubertal initiation age (r=-0.334, P<0.05). but mean FH SDS-MPH SDS were not correlated with age at diagnosis, glucocorticoid dose, urine 17-KS level, plasma 17-OHP level, plasma renin activity, treatment with growth hormone or gonadotropin releasing hormone analogue in both SW and SV. CONCLUSION: Mean final height and mean FH SDS-MPH SDS in 21-hydroxylase deficiency were below the mean for the general population. In SW, adult final height was correlated with pubertal initiation age and pubertal height gain. Therefore it should be important to control early onset of puberty and make improvement pubertal height gain during puberty.