PURPOSE: The reported clinical manifestations in Turner syndrome were different by karyotypes, countries and races. So, We evaluated the physical findings & medical problems in patient with Turner syndrome according to karyotype. METHODS: We examined prospectively 53 cases that diagnosed as Turner syndrome by chromosome study at the Pediatric Endocrine Clinic, YUMC from May 1999 to Aug 1999. According to the karyotype, they were divided into 3 groups:45, X(22 cases, 41.5%), mosaicism(18 cases, 34%) and structural aberration group(13 cases, 24.5%), and 53 cases were reevaluated by prepared protocol of 22 abnormal physical findings and 4 medical problems. Chi-square test was used for statistic analysis. RESULTS: 1)In case of 45, X group, they had short stature(100%), cubitus valgus(95.5%), widely spaced nipples(86.4%), shield chest(72.7%), low posterior hair line(68.2%), in case of mosaicism group, they had short stature(100%), cubitus valgus(72.2%), high arched palate(66.7%), widely spaced nipples(61.1%), short neck(61.1%) and in case of structural aberration group, they had short stature(100%), high arched palate (92.3%), widely spaced nipples(92.3%), low posterior hair line(76.9%), shield chest (76.9%), in frequency order. Short stature was the most common finding in Turner syndrome. The incidence of high arched palate was significantly higher in structural aberration group(P<0.05). 2)In case of 45, X group, they had otitis media(63.6%), thyroid problem(4.5%) and no renal problem and cardiac problem, in case of mosaicism group, they had otitis media(38.9%), thyroid problem(5.6%) and no renal problem and cardiac porblem and in case of structural aberration group, they had otitis media(61.5%), thyroid problem(15.4%), renal problem(7.7%) and cardiac problem(7.7%) in frequency order, they had no statistical difference in among three groups. CONCLUSION: In case of 45, X group, the incidence of cubitus valgus, webbed neck were more common findings and of high arched palate, micrognathia were less common findings compared to other group. In case of mosaicism group, the incidence of micrognathia, epicanthal fold were more common findings and wide spaced nipples, antimongoloid fissure were less common findings compared to other group. In case of structural aberration group, the incidence of high arched palate is most common finding among 3 group(P<0.05) and pigmented nevi and epicanthal fold were less common findings compared to other group. The incidence of medical problems such as otitis media and thyroid problem were than other foreign country reports.
Continental Population Groups ; Hair ; Humans ; Incidence ; Karyotype* ; Mosaicism ; Neck ; Nevus, Pigmented ; Nipples ; Otitis ; Otitis Media ; Palate ; Prospective Studies ; Thorax ; Thyroid Gland ; Turner Syndrome*

PURPOSE: Neonatal adrenal hemorrhage is not rare disease which can be caused by such risk factors as sepsis, large baby, birth trauma and asphyxia. The clinical manifestations include jaundice, anemia, abdominal mass and differentiation from neuroblastoma, renal vein thrombosis and adrenal abscess is needed. Through the clinical assessment of presenting features, we hope that this study be of any help to early detection and proper management of neonatal adrenal hemorrhage. METHODS: The 16 subjects out of neonates admitted to our hospital from July 1991 to June 1997 were diagnosed as neonatal adrenal hemorrhage. The risk factors, clinical manifestations, diagnostic methods and prognosis of neonatal adrenal hemorrhage were evaluated in these 16 cases. RESULTS: 1) Among 16 cases, males were 10 (62.5%) and females were 6 (37.5%). Mean birth weight was 3.601.08kg and mean gestational age was 39.82+1.08 week, and all were fullterm babies. In modes of delivery, vaginal deliveries were 12 cases (75.0%) and cesarean sections were 4 cases (25.0%). 2) 13 cases (81.2%) were involved in right side, 2 cases (12.5%) in left side and 1 case (6.3%) bilaterally. 3) The risk factors include sepsis in 6 cases (37.5%), large baby in 5 cases (31.3%), birth trauma in 5 cases<31.3%) and asphyxia in 3 cases (18.8%). 4) The clinical manifestations include jaundice in 7 cases (43.8%), anemia in 7 cases (43.8%), fever in 6 cases (37.5%) and abdominal mass in 3 cases (18.8%). 5) In the time of diagnosis, until 7 days of birth were 9 cases (56.2%), 8-14 days were 3 cases (18.8%), 15-21 days were 2 cases (12.5%) and 22-28 days were 2 cases (12.5%). Follow-up studies were done in 13 cases (81.2%), and tha lesions all decreased without any specific complications or sequelae. CONCLUSION: Neonatal adrenal hemorrhage can be diagnosed by abdominal ultrasono- gram in the presence of suggestive manifestations of jaundice, anemia, fever and abdominal mass, with relatively good prognosis. Differentiation from other conditions as well as avoidance of unnecessary explorations can be achieved by serial follow-up examinations of abdominal ultrasonogram.
Abscess ; Anemia ; Asphyxia ; Birth Weight ; Cesarean Section ; Diagnosis ; Female ; Fever ; Follow-Up Studies ; Gestational Age ; Hemorrhage ; Hope ; Humans ; Infant, Newborn ; Jaundice ; Male ; Neuroblastoma ; Parturition ; Pregnancy ; Prognosis ; Rare Diseases ; Renal Veins ; Risk Factors ; Sepsis ; Thrombosis ; Ultrasonography

Purpose: The objectives of this study were to describe the needs and knowledge for hospice palliative care, to identify the caregiving burden among cancer caregivers, and to determine factors that influence the needs for hospice palliative care. Methods: This study was designed with a descriptive correlation study with structured questionnaires. Data were collected from 162 caregivers of patients with cancer from September 2018 to March 2019 in a university-affiliated hospital in Seoul, Korea. Cancer patients’ needs, knowledge of hospice palliative care, and caregiving burden were measured. Data analysis was performed with descriptive statistics, independent t-test, one-way ANOVA, Pearson’s correlation coefficient, and multiple linear regression using the SPSS/WIN 25.0 program. Results: The mean needs score was 90.32±17.12, the correct answers rate of knowledge items was 47.5%, and the mean score of caregiving burden was 32.53±13.94. In the regression model, knowledge, caregiving burden, age, and relationship with the patient were identified as the influencing factors of the needs of caregivers (F=6.98, p<. 001) and showed a variance of 16%. Conclusion This study highlights the unmet needs for early palliative care. Policies and social support should be established to alleviate the caregiving burden, enhance knowledge, and reduce the need for hospice palliative care. These factors are essential not only for caregivers but also for patients with cancer.

Acute simultaneous rupture of the anterior cruciate ligament (ACL) and patellar tendon is a rare injury. We present a case report of a 32-year-old male patient with ruptured ACL and ipsilateral patellar tendon rupture sustained while playing baseball. Surgery was performed on the patellar tendon and the ACL simultaneously. The clinical and radiological outcomes of the treatment were successful. We present this case with a review of the literatures.
Adult ; Anterior Cruciate Ligament ; Baseball ; Humans ; Male ; Patellar Ligament ; Rupture

Popliteal artery injury is a very rare complication of anterior cruciate ligament (ACL) reconstruction. The authors experienced a case of popliteal arterial pseudoaneurysm after re-revision of ACL reconstruction using Rigidfix for femoral tunnel fixation. Pseudoaneurysm was detected in knee magnetic resonance imaging, which caused pain, limit of motion, common peroneal nerve palsy, leg swelling and symptoms similar to compartment syndrome. After excision and re-anastomosis of the popliteal artery using a greater saphenous vein graft, all symptoms were resolved within 3 months except for common peroneal nerve palsy. So we report on this case with a review of the literature.
Aneurysm, False ; Anterior Cruciate Ligament ; Compartment Syndromes ; Knee ; Leg ; Magnetic Resonance Imaging ; Paralysis ; Peroneal Nerve ; Popliteal Artery ; Saphenous Vein ; Transplants

No abstract available.
Actinomycosis*

PURPOSE: The human fetus is primarily dependent on the placenta for its nutrition. However, as the fetus matures, it swallows increasing amounts of amniotic fluid, which contributes to the growth of fetus. Accordingly fetuses with congenital obstruction of the gut at high level have a reduced capacity for intestinal absorption of amniotic fluid. We undertook a study to investigate the effect of congenital gut obstruction on fetal growth. METHOD: A retrospective review of the records of all patients presenting congenital gut obstruction over 6-year period (from 1992 to 1997) in Chung-ang Gil hospital was performed. Patients with a complete proximal obstruction were included in group A; patients with incomplete or lower obstruction were included in group B. RESULT: 1) The ratio of male to female was 1.5:1. The mean birth weight and gestational age were 2.89+/-0.60kg and 38.7+/-0.20weeks. The mean birth weight and gestational age in group A were 2.68+/-0.69kg and 37.8+/-0.25weeks. The mean birth weight and gesnal age in group B were 2.980.54kg and 39.1+0.17weeks. There was significant difference between group A and B (P<0.01). 2) Significant differences were found between group A and B in prematurity and growth retardation rate (P<0.01). No significant difference was found between group A and B in associated anomaly rate (P>0.05). 3) In group A, 3 (42.8%) of 7 patients with associated anomalies had IUGR, whereas 8 (40.0%) of 20 patients without associated anomalies had IUGR (P>0.05). The corresponding figures for group B were 23.0% and 14.8%, respectively (P>0.05). CONCLUSION: Congenital gut obstruction causes IUGR by reducing intestinal absorption of amniotic fluid and the effect of IUGR is more pronounced as the obstruction is proximal to jejunum rather than distal to it.
Amniotic Fluid ; Birth Weight ; Female ; Fetal Development* ; Fetal Growth Retardation ; Fetus ; Gestational Age ; Humans ; Intestinal Absorption ; Jejunum ; Male ; Placenta ; Retrospective Studies ; Swallows

Mastocytosis is rare disorder characterized by the overproliferation and accumulation of tissue mast cells and spontaneous regression before adolescence in childhood, almost. Clinical features are vary and depend on local accumulation of mast cells in different organs and the effects of their mediators. Mast cell disease is commonly involved in the skin, and occasionally gastrointestinal tract, bone marrow, liver, spleen and lymphoid organs as well. Mastocytosis occurs equally in persons of each gender and it affects all age groups. The incidence is unknown and familial tendency is unusual. We are reporting a case of mastocytosis confirmed by skin biopsy who was visited for skin lesions that skin rashes at 10 days after birth, change to bullar at 3 month later.
Adolescent ; Biopsy ; Bone Marrow ; Exanthema ; Female* ; Gastrointestinal Tract ; Humans ; Incidence ; Infant* ; Liver ; Mast Cells ; Mastocytosis* ; Parturition ; Skin ; Spleen

We report a rare case of gastric adenocarcinoma with enteroblastic differentiation (GAED) that was treated with endoscopic submucosal dissection followed by additional distal gastrectomy with lymph node dissection. A 67-year-old man underwent endoscopic submucosal dissection for a gastric lesion, which was diagnosed as GAED with submucosal and lymphatic invasion. Histologically, GAED is characterized by a tubulopapillary growth pattern and clear cells that resemble those of the primitive fetal gut. Immunohistochemically, GAED variably expresses oncofetal proteins such as glypican-3, alpha-fetoprotein, and spalt-like transcription factor 4. Despite negative margins, additional gastrectomy with lymph node dissection was performed due to submucosal and lymphatic invasion.No residual tumor or metastasis was detected, and the patient remained disease-free for 2 years before dying from causes unrelated to GAED. Given its aggressive nature, frequent lymphovascular invasion, and high metastatic potential, clinicians should recognize the histopathological diagnosis of this rare tumor and its propensity for aggressiveness.

PURPOSE: The manner of rectal bleeding of auto-amputated polyps (AP) is similar to juvenile polyps (JP) or Meckel's diverticula (MD). We conducted this study to characterize the clinical spectrum of AP. METHODS: Fourteen patients were enrolled this study who were diagnosed AP due to painless rectal bleeding. The clinical data of AP was assessed and then compared with the clinical data of JP and MD retrospectively. RESULTS: The prevalence of AP was 10.4% (14/135) and high in younger patients compared with that of JP (p=0.042 below 2 years). Whereas JP was more common in patients aged 2 to 5 years (p=0.005). Male was predominant in AP (p=0.008 AP vs JP). The manner of rectal bleeding in AP group was sudden and transient. There was no significant difference in time interval between onset of rectal bleeding and diagnosis between the 3 groups. However AP was diagnosed in 9 patients (64.3%) within 7 days after onset of rectal bleeding, but JP was diagnosed in 5 patients (4.1%) in the same period (p<0.001). All of AP were located in the rectum and the sigmoid colon. The mean hemoglobin was 11.3+/-1.5 g/dL in AP, 11.8+/-1.3 g/dL in JP, and 8.4+/-1.2 g/dL in MD (p<0.001, only significant in MD). CONCLUSION: AP may be considered in male older than 1 year with transient and sudden onset or increase of painless rectal bleeding without drop of hemoglobin level.
Aged ; Colon, Sigmoid ; Diverticulum ; Hemoglobins ; Hemorrhage ; Humans ; Male ; Meckel Diverticulum ; Polyps ; Prevalence ; Rectum