PURPOSE: The purpose of this study was to describe the clinical outcome after pancreatcetmy and its relationship with pathological appearances and clinical features in patients with persistent hyperinsulinemic hypoglycemia of infancy(PHHI). METHODS: Medical records of 10 patients(9 males and 1 female, mean age:40.4+/-1.5 months) who were diagnosed as PHHI and underwent pancreatectomy from 1988 to 2000 were reviewed. Clincal and biochemical data were recorded. Subjects were classified arbitrarily into early-onset or late-onset group according to age of onset. Pathologic appearance of pancreas was divided into 2 forms:diffuse or focal. The former had a focal pancreatic adenomatous hyperplasia and the latter was characterized by increased number of betacells with similar distribution seen in normal neonates. RESULTS: One patient had focal, and nine had diffuse lesions. After near-total pancreatectomy, 4 patients(40.0%) showed complete response, 4(40.0%) had persistent hypoglycemia, and 2(20.0%) developed diabetes mellitus. As neurological sequelae, 6 patients(60.0%) had persistent seizures, and 6(60.0%) had delayed motor and speech development. No clinical or biochemical factors related to postoperative outcome were found. CONCLUSION: This data indicate that early diagnosis of patients who present with hypoglycemic symptoms in infancy, especially early in life, and development of more effective therapy are warranted, because there is no clinical or biochemical factor predicting final outcome after near-total pancreatectomy and only 40% of patients with PHHI remained euglycemic after surgery with possible severe neurological sequelae.
Age of Onset ; Congenital Hyperinsulinism* ; Diabetes Mellitus ; Early Diagnosis ; Female ; Humans ; Hyperplasia ; Hypoglycemia ; Infant, Newborn ; Male ; Medical Records ; Pancreas ; Pancreatectomy* ; Seizures

Purpose : Shortness is the most frequent and quite disturbing characteristics of patients with Turner syndrome. The aim of this study was to evaluate the factors affecting final adult height(FAH) in these patients. METHODS : The study group was comprised of 19 patients who were diagnosed as Turner syndrome and attained FAH. We analyzed the influences of various factors on FAH in GH treated group with those in GH untreated group. Results : Nineteen patients were enrolled; thirteen received GH treatment and six did not. The mean duration of GH treatment was 24.3 months(range : 9 to 50 months), and the mean dosage of GH was 0.98+/-0.35IU/kg/wk in GH treated group. The mean growth velocity during GH treatment was 5.6+/-1.8 cm/yr, which was significantly higher than that during pretreatment period(P<0.05). In GH treated group, the mean chronological age, bone age, mean height, and height SD score at GH therapy were 13.7+/-1.7yr, 11.3+/-1.9yr, 129.7+/-7.9cm, and -4.1+/-1.1, respectively, which were not statistically different from those at diagnosis of GH untreated group. In GH treated group, the mean FAH and FAH SD score were 144.8+/-5.0cm, and -3.2+/-0.9, respectively, which showed no significant difference compared with those of GH untreated group. Analyzing the factor affecting FAH in all Turner girls of both groups together, parental height, chronological age, bone age, and bone age delay at diagnosis(or at the initiation of GH therapy) were not related to FAH. Height and height SD score at diagnosis(or at the initiation of therapy) were positively related to FAH(P<0.05, r=0.72). CONCLUSION : The results suggest that GH treatment dose not improve FAH in patients with Turner syndrome, despite increased growth velocity during GH treatment, which might come from intermittern GH therapy. This should be remained to be clarified with more Turner patients who attained FAH.
Adult* ; Diagnosis ; Female ; Growth Hormone ; Humans ; Parents ; Turner Syndrome*

Locoregional failure of rectal cancer is a troublesome problem and a major cause of morbidity and mortality following curative surgery. The mesorectum has been regarded as an important site in local failure after surgery of rectal cancer. Total mesorectal excision (TME) has been raised by some colorectal surgeons to prevent early local recurrence. This study was performed to ascertain the incidence of metastatic lymph nodes in the distal mesorectum (DMR) of the colorectal cancer patient. We also examined the clinicopathologic risk factors of distal mesorectal metastasis. Eight of 53 patients had positive metastatic lymph nodes in DMR. Twenty-seven patients were Dukes B and 26 patients were Dukes C stage. Out of 26 Dukes C patients, 8 patients (30.8%) had metastatic lymph nodes in the DMR. However, there was no significant difference in risk factors between DMR positive and DMR negative patients with Dukes C stage. In conclusion, the incidence of metastatic lymph nodes in DMR was about 30.8%, therefore the mesorectum especially the DMR should be removed completely by total mesorectal excision to eradicate the metastatic lymph nodes which may cause local recurrence.
Aged ; Female ; Human ; *Lymphatic Metastasis ; Male ; Middle Age ; Neoplasm Invasiveness ; Postoperative Period ; Rectal Neoplasms/pathology/*surgery ; Rectum/*surgery

Background: Personal socio-economic abilities are crucial as it affects health inequalities. These multidimensional inequalities across the regions have been structured and fixed. This study aimed to analyze health vulnerabilities by regional cluster and identify regional health disparities of self-rated health, using nationally representative cross-sectional data. Methods: This study used personal and regional data. Data from the Community Health Survey 2021 were analyzed. K-means cluster analysis was applied to 250 si-gun-gu using administrative regional data. The clusters were based on three areas: physical environment, health-related behaviors and biological factors, and the psychosocial environment through the conceptual framework for action on the social determinants of health. And binary logistic regression analyses were conducted to examine the differences in self-rated health status by the regional clusters, controlling human biology, environment, lifestyle, and healthcare organization factors. Results: The most vulnerable group was group 3, the moderate vulnerable group was group 1, and the least vulnerable group was group 2. The group 2 was more likely to have high self-rated health status than the moderate vulnerable group (odds ratio [OR], 1.023; p<0.001). And the group 3 showed low self-rated health status than the moderate vulnerable group (OR, 0.775; p<0.001). However, the moderate vulnerable group had significantly higher self-rated health status than the most vulnerable group (group 2:OR, 1.023; p<0.001; group 3: OR, 0.775; p<0.001). Conclusion These results demonstrate that community members’ health status is influenced by regional determinants of health and individual levels. And these contribute to understanding the importance of specific and differentiated interventions like locally tailored support programs considering both individual and regional health determinants.

Cantú syndrome (CS, OMIM 239850) is a very rare autosomal dominantly inherited genetic disease characterized by congenital hypertrichosis, neonatal macrosomia, a distinct facial features such as macrocephaly, and cardiac defects. Since the first description by Cantú et al. in 1982, about 50 cases have been reported to date. Recently, two causative genes for CS has been found by using exome sequencing analyses: ABCC9 and KCNJ8 . Most cases of clinically diagnosed CS have resulted from de novo mutations in ABCC9. In this study, we report three independent Korean children with CS resulting from de novo ABCC9 mutations. Our patients had common clinical findings such as congenital hypertrichosis, distinctive facial features. One of them showed severe pulmonary hypertension and hypertrophic cardiomyopathy, which require medical treatment. And, two patients had a history of patent ductus arteriosus. Although two of our patients had shown early motor developmental delay, it was gradually improved during follow-up periods. Although CS is quite rare, there are the concerns about development of various cardiac problems in the lifetime. Therefore, an accurate diagnosis followed by appropriate management and genetic counseling should be provided to CS patients.
Cardiomyopathy, Hypertrophic ; Child ; Databases, Genetic ; Diagnosis ; Ductus Arteriosus, Patent ; Exome ; Follow-Up Studies ; Genetic Counseling ; Humans ; Hypertension, Pulmonary ; Hypertrichosis ; Megalencephaly

PURPOSE: Among the surgeries that can be performed for patients with acute peptic ulcer perforation, the frequency of performing laparoscopic surgeries is gradually increasing and it is being reported that laparoscopic surgeries have many advantages over laparotomies. So we will examined if laparoscopic surgery has advantages as compared to open surgery for appropriately selected patients. METHODS: The subjects were 15 patients who were diagnosed with peptic ulcer perforation and they were operated on by a single surgeon between September 2007 and January 2009 at the National Medical Center. The subjects were divided into an LG and an OG. Statistical analyses were performed using two-tailed students T-tests. RESULTS: There were 6 patients in the LG and 9 patients in the OG. All the patients in the two groups were male. The time until fist flatulence was 1.6 days in the LG and 2.6 days in the OG. The time until the first oral intake was 3 days in the LG and 3.4 days in the OG. The time during which pain control was necessary was 2.6 (range: 2~4) days in the LG and 3.3 (range: 2~4) days in the OG. The number of days of the hospital stay after surgery was 5.6 days (range: 4~7) in the LG and 10 days (range: 6~26) in the OG. There were 2 cases of complications in the OG only, and all of them were wound infections. CONCLUSION: It is thought that laparoscopic surgeries can be performed relatively easily and safely for patients with peptic ulcer perforation by selecting appropriate patients.
Flatulence ; Humans ; Laparoscopy ; Laparotomy ; Length of Stay ; Male ; Peptic Ulcer ; Peptic Ulcer Perforation

PURPOSE: Amenorrhea and short stature are serious complications in patients with congenital adrenal hyperplasia of 21-hydroxylase deficiency(CAH). We analysed several factors influencing the presence of menarche and final adult height in these patients. METHODS: We retrospectively reviewed medical records of 24 female patients with CAH. We analysed possible factors affecting the menarche and final adult height, such as clinical type, predicted adult height, age at treatment, age at onset of puberty, BMI, and plasma levels of DHEA and 17-OHP. RESULTS: The occurrence rate of menarche were significantly higher in patients with the simple virilizing form rather than the salt losing form(P<0.05). No significant differences were found in age at onset of puberty, BMI, and plasma levels of DHEA and 17-OHP between menarche group and amenorrhea group. The age at treatment was younger in amenorrhea group(P<0.05). There were no significant differences in the proportion of patients who attained predicted adult height between two clinical types. No significant differences were found in age at treatment, age at onset of puberty, BMI, and plasma levels of DHEA and 17-OHP between the group of patients who attained predicted adult height and the group of patients who didn't. Final adult height did not show any correlation with age at treatment, age at onset of puberty, BMI, and plasma levels of DHEA and 17-OHP but showed significant correlation with mid-parental height(r=0.426, P=0.01). CONCLUSION: Our data suggest that the presence of menarche in patients with CAH depends on the degree of prenatal exposure to adrenal androgen regardless of the degree of postnatal control and age at onset of puberty. Additionally, it seems likely that mid-parental height determines the final adult height.
Adolescent ; Adrenal Hyperplasia, Congenital* ; Adult* ; Amenorrhea ; Dehydroepiandrosterone ; Female ; Female* ; Humans ; Medical Records ; Menarche* ; Plasma ; Puberty ; Retrospective Studies ; Steroid 21-Hydroxylase*

The genetic status of cagA, vacA subtype, iceA1, and babA, and the relationship to gastroduodenal diseases were assessed in Helicobacter pylori isolates in Korea. Seventy-six strains of H. pylori were isolated from the antrum and the corpus of 41 adult patients (22 with peptic ulcer and 19 with gastritis). The cagA, iceA1, and babA genes were assessed by polymerase chain reaction and the vacA subtypes were determined by reverse hybridization-line probe assay. The positive rates of 349-bp cagA, 208-bp cagA, iceA1, and babA genes were 97.4%, 96.1%, 84.2%, and 36.1%, respectively. The vacA s1a, s1b, s1c, and s2 variants were detected in 11.8%, 3.9%, 80.4%, and 1.3%, respectively. m1 (78.9%) is more prevalent than m2 (5.3%). The most common vacA genotype was s1c/m1 (61.9%), and 14 isolates (18.4%) contained mixed vacA genotypes from a single biopsy specimen. Twenty-one (60%) of 35 patients were infected with more than two strains of different cagA, iceA1, babA, and vacA genotypes. None of cagA, iceA1, babA, and vacA s1/m1 were associated with peptic ulcer. In conclusion, most H. pylori isolates in Korea carry cagA, iceA1, and vacA s1c/m1 genes, and reside with multiple strains. These genes do not correlate with the peptic ulcer in the Korean patients.
Adult ; Aged ; Bacterial Proteins/*genetics ; Female ; Genotype ; Helicobacter pylori/*classification/genetics/pathogenicity ; Human ; Male ; Middle Age ; Peptic Ulcer/*etiology/microbiology

OBJECTIVE: To investigate the change of prognosis of the preterm infants delivered in Chosun University Hospital. METHODS: A study was conducted for 236 newborns who were born at Chosun University hospital from January 1. 2000 to December 31, 2002. The data were collected by review of the hospital record. RESULTS: 1) The survival rate of the preterm infants who weigh below 999 gm was 2% and 68.4% in 1,000-1,499 gm of infants 90.9% in 1,500-1,999 gm of infants and 92.7% in 2,000-2,499 gm of infants respectively. 2) The survival rate of the preterm infants who born 25% at 25-26 weeks, 50% at 27-28 weeks, 80% at 29-30 weeks, 88.2% at 31-32 weeks, 94.4% at 33-34 weeks and 95% after 35 wks respectively. 3) The cause of preterm delivery were premature rupture of membranes (41.7%), preterm labor (23.7%), pregnancy induced hypertension (10.9%), multiple pregnancy (8.3%) etc. 4) The perinatal complications of the preterm infants were hyperbilirubinemia (27.5%), respiratory distress syndrome (24.2%), sepsis (11.4%) etc. CONCLUSION: To increase the survival rates of preterm infants, the preterm labor should be controlled by 27 week's gestation and the birth weight of 1,000 gm minimally.
Birth Weight ; Epidemiologic Studies* ; Female ; Hospital Records ; Humans ; Hyperbilirubinemia ; Hypertension, Pregnancy-Induced ; Infant ; Infant, Newborn ; Infant, Premature ; Membranes ; Obstetric Labor, Premature ; Pregnancy ; Pregnancy, Multiple ; Premature Birth* ; Prognosis ; Rupture ; Sepsis ; Survival Rate

PURPOSE: To evaluate whether the Breast Imaging Reporting and Data System (BI-RADS) MRI lexicon could reflect the genomic information of breast cancers and to suggest intuitive imaging features as biomarkers. METHODS: Matched breast MRI data from The Cancer Imaging Archive and gene expression profile from The Cancer Genome Atlas of 70 invasive breast cancers were analyzed. Magnetic resonance images were reviewed according to the BI-RADS MRI lexicon of mass morphology. The cancers were divided into 2 groups of gene clustering by gene set enrichment an alysis. Clinicopathologic and imaging characteristics were compared between the 2 groups. RESULTS: The luminal subtype was predominant in the group 1 gene set and the triple-negative subtype was predominant in the group 2 gene set (55 of 56, 98.2% vs. 9 of 14, 64.3%). Internal enhancement descriptors were different between the 2 groups; heterogeneity was most frequent in group 1 (27 of 56, 48.2%) and rim enhancement was dominant in group 2 (10 of 14, 71.4%). In group 1, the gene sets related to mammary gland development were overexpressed whereas the gene sets related to mitotic cell division were overexpressed in group 2. CONCLUSION: We identified intuitive imaging features of breast MRI associated with distinct gene expression profiles using the standard imaging variables of BI-RADS. The internal enhancement pattern on MRI might reflect specific gene expression profiles of breast cancers, which can be recognized by visual distinction.