A 24-year-old female who have had pseudoxanthoma elasticum for 14 years, showed brownish, umbilicated papules forming serpiginous pattern on the anterior neck, A skin biopsy specimen from the neck lesion revealed epidermal hyperplasia and perforating canal containing basophilic, necrotic materials and degenerated elastic fibers, also showed short, swollen and irregularly clumped elastic fibers in the lower dermis.
Basophils ; Biopsy ; Dermis ; Elastic Tissue ; Female ; Humans ; Hyperplasia ; Neck ; Pseudoxanthoma Elasticum* ; Skin ; Young Adult

Clinical survey was performed on 413 cases of twin pregnancies and their 816 twin babies who were born at IlSin Christian Hospital during 5years from January 1987 to December 1991. The result of study was as follows: 1) there was 413 twin pregnancies among 40,711 deliveries, so the incidence of twins was 1:98.6. Average male-female sex ratio of twins was 1.3:1, same sex pairs rate was 86% and different sex ratio was 14%. 2) The incidence of relation with maternal age was highest between 25 years old and 29 years old but the ratio of twin delivery to total delivery was 0.9% so, was not higher than other age group. 3) The incidence of relation with maternal parity was highest at first pregnancy but the ratio of twin delivery to total delivery was highest at second pregnancies. 4) Normal birth weight infants were 45.7%, low birth weight infants were 54.3% and very low birth weight infants was 9.3%. Fullt-erm infants were 63.7% and prematurity were 35.4%. 5) The incidence of feto-fetal transfusion was 7.3%. 6) Perinatal death rate was 76.3 and was more higher in second babies. The most common cause of perinatal death was prematurity, followed by respiratory complication, congenital anomaly, infection and asphyxia. Still-births were 8 cases.
Adult ; Asphyxia ; Birth Weight ; Female ; Humans ; Incidence ; Infant ; Infant, Low Birth Weight ; Infant, Newborn ; Infant, Very Low Birth Weight ; Maternal Age ; Mortality ; Parity ; Pregnancy ; Pregnancy, Twin ; Sex Ratio ; Twins*

A clinical observation was performed on 2,122 cases of premature infants who were admitted to Ilsin Christian during the past 5 years from Jan. 1986 to Dec. 1990. The results were as follows; 1) The incidence of prematurity was 5.7%, overall mortality rate was 15.8%, and the sex ratio of male to female was 1.5:1. 2) Concomitant maternal diseases were premature rupture of membrane 457 cases (21.5%), preeclampsia & eclampsia 424 cases (19.9%), multiple pregnancy 250 cases (11.7%) etc, in the order of frequency. 3) Prematurity was most prevalent among multipara over 3. 4) Incidence of prematurity according to maternal age was frequent in woman more than 35 years old. 5) Mortality of prematurity was higher inversely propotional to birth weight & gestational age. 6) Main causes of death were IRDS 45 cases (43%), congenital anomaly 72 cases (21.6%), sepsis 31 cases (9.3%), and unknown 84 cases (25.0%). 7) Clinical problems were hyperbilirubinemia 38.9%, hypoglycemia 28.6%, IRDS 18%, sepsis & other infections 8.1% etc, in the order of frequency. 8) Most deaths were seen within first 24hr (74.5%).
Adult ; Birth Weight ; Cause of Death ; Eclampsia ; Female ; Gestational Age ; Humans ; Hyperbilirubinemia ; Hypoglycemia ; Incidence ; Infant, Newborn ; Infant, Premature ; Male ; Maternal Age ; Membranes ; Mortality ; Pre-Eclampsia ; Pregnancy ; Pregnancy, Multiple ; Rupture ; Sepsis ; Sex Ratio

PURPOSE: To evaluate the diagnostic value of gross bloody stools as a predictor of intussusception. METHODS: We reviewed 159 cases retrospectively that had an ultrasound examination for diagnosis of intussusception from January 2001 to December 2003 at Ilsin Christian Hospital. We compared each symptom and assessed the diagnostic value of gross bloody stools in the patients with suspected intussusception. RESULTS: Thirty-six of the 159 patients had intussusception. Of 36 patients with intussusception, 19 (52.8%) children were male and 28(77.8%) children were younger than two years. The most common symptom was intermittent abdominal pain or irritability. But its frequency among the intussusception (24.2%) was lower than frequency of gross bloody stools(50.9%). The sensitivity, specificity, positive predictive value(PPV) and negative predictive value(NPV) of gross bloody stools in patients with suspected intussusception were as follows; sensitivity=77.8%(95% CI 60.8-89.9), specificity=78.0%(95% CI 69.7-85.0), PPV=50.9%(95% CI 37.1-64.6), NPV=92.3%(95% CI 85.4-96.6)(Diagnostic accuracy 78.0 %; 95% CI 70.7-84.2). CONCLUSION: It is useful for diagnosis of intussusception to confirm the presence of gross blood in stools.
Abdominal Pain ; Child ; Diagnosis ; Humans ; Intussusception* ; Male ; Retrospective Studies ; Sensitivity and Specificity ; Ultrasonography

PURPOSE: The objective of this study was to determine the factors affecting the time to recurrence after radical nephrectomy for localized renal cell carcinoma. MATERIALS AND METHODS: We retrospectively evaluated 321 patients who received radical nephrectomies for localized renal cell carcinoma (pT1a-pT2b N0M0). Of 29 patients with disease recurrence, 9 had recurrence more than 5 years after radical nephrectomy. We evaluated the clinicopathological factors, with the use of a retrospective study design. RESULTS: Tumor necrosis was statistically different between the late recurrence group and the recurrence free group (Fisher exact test, p=0.046). Hematuria at diagnosis (chi-square test, p=0.045) was statistically significant in early recurrence. In the univariate logistic regression analysis, tumor necrosis (odds ratio [OR], 4.629; 95% confidence interval [CI], 1.106 to 19.379; p=0.036) and pT stage>1 (OR, 7.232; 95% CI, 1.727 to 30.280; p=0.007) were risk factors of late recurrence. In the multivariable logistic regression analysis, pT stage>1 (OR, 7.143; 5% CI 1.706 to 29.912, p=0.007) was associated with late recurrence. Regarding early recurrence, initial symptoms at diagnosis and pathologic T stage>1 were statistically significant in both univariate and multivariable logistic regression analysis. In terms of recurrence site, patients with late recurrence tended to have unusual metastasis sites other than lung, liver or bone (chi-square test, p=0.012). CONCLUSIONS: These data suggest that tumor necrosis may affect late disease recurrence. Patients with initial symptoms and hematuria at diagnosis are vulnerable to recurrence in a shorter period after nephrectomy. Patients with late recurrence showed a tendency to have unusual metastasis site other than lung, liver or bone.
Carcinoma, Renal Cell* ; Disease-Free Survival ; Hematuria ; Humans ; Liver ; Logistic Models ; Lung ; Necrosis ; Neoplasm Metastasis ; Nephrectomy* ; Recurrence* ; Retrospective Studies ; Risk Factors

PURPOSE: Although eosinophilia is a common laboratory finding in many neonatal intensive care units (ICUs), its causative mechanisms remain obscure. We aimed to determine the causes of eosinophilia in the neonatal ICU environment. METHODS: Serial eosinophil counts were determined weekly for 288 hospitalized, appropriately grown neonates. Infants were divided into four groups according to gestational age, and the incidence and etiologic factors of eosinophilia were retrospectively studied. RESULTS: Absolute eosinophilia (>700/mm3) was documented in 18% (52/288) of neonates. Twenty-two infants (42.3%) exhibited mild eosinophilia (700-999 cells/mm3), 27 (51.9%) exhibited moderate eosinophilia (1,000-2,999 cells/mm3), and 3 (5.8%) exhibited severe eosinophilia (>3,000 cells/mm3). Of the 288 infants studied, 54 suffered sepsis. Thirty of these 54 infants (55.6%) showed eosinophilia, and 22 out of the remaining 234 infants (9%) without sepsis showed eosinophilia, indicating that eosinophilia was more prevalent in the sepsis group (P <0.05). All 5 infants suffering from bronchopulmonary dysplasia showed eosinophilia, and 47 out of the remaining 283 infants (16.7%) without bronchopulmonary dysplasia showed eosinophilia. Thus, eosinophilia was more prevalent in the bronchopulmonary dysplasia group (P <0.05). Furthermore, increased prevalence of eosinophilia was associated with respiratory distress syndrome, ventilator use, blood transfusion, and total parenteral nutrition (P <0.05). CONCLUSION: Our results suggest that eosinophilia is influenced by sepsis and bronchopulmonary dysplasia, although it can also occur idiopathically at birth. Moreover, the potential role of eosinophils in conditions such as wound healing and fibrosis in sepsis or chronic lung disease may be a cause of eosinophilia.
Blood Transfusion ; Bronchopulmonary Dysplasia ; Eosinophilia ; Eosinophils ; Fibrosis ; Gestational Age ; Humans ; Incidence ; Infant ; Infant, Newborn ; Intensive Care Units, Neonatal ; Intensive Care, Neonatal ; Lung Diseases ; Parenteral Nutrition, Total ; Parturition ; Prevalence ; Retrospective Studies ; Sepsis ; Stress, Psychological ; Ventilators, Mechanical ; Wound Healing

A male baby with intrauterine growth retardation had a short neck, small hands and feet, hypospadia, both grade I hydronephrosis, type II atrial septal defect, and moderate valvular pulmonary stenosis. The routine chromosome and banding analyses revealed a 46,XY,rec(8)del(8)(p21)dup(8) (q24.1)inv(8)(p21q24.1)pat chromosome constitution. His mother has normal chromosomes, but the father had 46,XY,inv(8)(p21q24.1). Also his uncle had an inv(8) chromosome constitution. We used lymphocytes and examined 40 mitotic cells. All mitotic cells showed deletion of 8p21-->pter and duplication of 8q24.1-->qter. Because 8p21 involves secretion of macrophage and lymphocyte against cancer cells, long-term follow-up for cancer will be needed.
Chromosome Deletion ; Chromosomes, Human, Pair 8 ; Constitution and Bylaws ; Fathers ; Female ; Fetal Growth Retardation ; Foot ; Hand ; Heart Septal Defects, Atrial ; Humans ; Hydronephrosis ; Hypospadias ; Lymphocytes ; Macrophages ; Male ; Mothers ; Neck ; Pulmonary Valve Stenosis ; Trisomy

A neonate may incur liver injury during spontaneous delivery. The liver is the organ most likely to be injured during the birth process. Breech presentation and manipulation are the most common causes of hepatic trauma. However, hepatic hemorrhage can occur in any infant, regardless of size and the type of delivery. Infants with subcapsular hemorrhage are usually asymptomatic at birth, and gross hepatic rupture from these lesions is unusual. In patient with primary rupture, major bleeding takes place immediately, explaining the high percentage of neonatal deaths with massive liver rupture. If subcapsular hemorrhage remain intracapsular with spontaneous resolution, the only treatment needed in subcapsular hemorrhage may be transfusion. We experienced one case of neonatal subcapsular hemorrhage with persistent hyperbilirubinemia. We report this case with the brief review of related literature.
Breech Presentation ; Female ; Hematoma* ; Hemorrhage ; Humans ; Hyperbilirubinemia ; Hyperbilirubinemia, Neonatal* ; Infant ; Infant, Newborn ; Liver ; Parturition ; Pregnancy ; Rupture

Transient hyperammonemia of the newborn is an overwhelming disease manifestated by hyperammonemic coma in ill premature infant. This recognized metabolic disorder is chiefly characterized by severe hyperammonemia in the postnatal period, a comatous state, absence of abnormal organic aciduria, normal activity of urea cycle enzymes and, usually, complete recovery. The etiology is unknown. Infant had mild respiratory distress that progressed within 48 hours to deep coma requiring ventilatory assistance and had marked hyperammonemia. The degree of neurologic impairment and developmental delay in this disorder depends on the duration of the hyperammonemic coma. So, treatment of hyperammonemia should be initiated promptly and continued vigorously. We report of a preterm infant (34+5 weeks of gestation) presenting with respiratory distress, seizure, coma, and marked elevated plasma ammonia level.
Ammonia ; Coma ; Humans ; Hyperammonemia* ; Infant ; Infant, Newborn* ; Infant, Premature ; Plasma ; Seizures ; Urea

PURPOSE: We evaluared the risk factors, clinical characteristic, diagnosis and treatment of neonatal systemic fungal infection in 28 cases in order to find ways to prevent development of and to improve the prognosis of neonatal systemic fungal infection METHODS: From November 1994 to August 1996, 28 premature infants who were diagnosed as systemic fungal infection at Ilsin Christian Hospital NICU were analyzed retrospectively. RESULTS: 1) The mean gestational age was 30.7 weeks(27-35.5weeks) and the mean birth weight was 1528g(975-2980g). 2) The risk factors associated with the development of neonatal systemic fungal infection included long-term use of broad-spectrum antibiotics, prolonged hyperalimentation, prolonged use of aminophylline and steroid, and endotracheal intubation. 3) The most common presenting clinical manifestations were temperature instability, feeding intolerance, and apnea. 4) In laboratory studies, blood, urine, and CSF culture positivity was 92.8%, 92.8%, and 10.7%, respectively and renal and cranial sonogram were helpful to evaluate the renal and CNS involvement. 5) The principal treatment was intravenous amphotericin B administration for 4 weeks. The side effects, such as renal toxicity, hepatotoxicity, hypokalemia, and vomiting, developed but were reversible. 6) The mortality rate was 7.1%, and ventriculo-peritoneal shunts was performed in 3 cases who had fungal meningitis for the management of postmeningitic hydrocephalus. CONCLUSION: For was beformedin the diagnosis of neonatal systemic fungal infection is very difficult and the mortality is high, we recommend that antifungal therapy be initiated in clinically ill infants who are not responsive to antibiotic therapy and have negative culture findings and have some of the risk factors associated with systemic fungal infection.
Aminophylline ; Amphotericin B ; Anti-Bacterial Agents ; Apnea ; Birth Weight ; Diagnosis ; Gestational Age ; Humans ; Hydrocephalus ; Hypokalemia ; Infant ; Infant, Newborn ; Infant, Premature ; Intubation, Intratracheal ; Meningitis, Fungal ; Mortality ; Prognosis ; Retrospective Studies ; Risk Factors ; Ventriculoperitoneal Shunt ; Vomiting