A Comparative Cross-sectional Study of the Development of Hepatocellular Carcinoma in Patients with Liver Cirrhosis Caused by Hepatitis B Virus, Alcohol, or Combination of Hepatitis B Virus and Alcohol / 대한소화기학회지

BACKGROUND/AIMS: Alcohol may be a cocarcinogen in patients with chronic viral hepatitis. We investigated the effect of alcohol on the development of hepatocellular carcinoma (HCC) in liver cirrhosis (LC) caused by hepatitis B virus (HBV). METHODS: All patients with LC or HCC associated with HBV or alcohol, admitted between March 2001 and June 2005, were included. Patients were divided into three groups according to the etiology of LC: Alcohol (AL), HBV, or HBV+alcohol (HBV+AL). Age and laboratory data at the enrollment of study were analyzed. The logistic regression coefficiency for the prevalence of HCC was calculated by using variables such as age, gender, serologic markers, and etiology of LC. RESULTS: In LC patients (n=342), the proportions of AL, HBV, and HBV+AL groups were 44%, 39%, and 17%, respectively. The proportions of HCC in AL, HBV and HBV+AL groups were 17%, 55%, and 76%, respectively. Age at the diagnosis of HCC was younger in HBV+AL than in AL group (p=0.036). In logistic regression analysis for the risk factor of HCC, odds ratio of age was 1.056 (p<0.001). Odds ratios of HBV and HBV+AL group comparing AL were 8.449 (p<0.001) and 17.609 (p<0.001), respectively. Therefore, old age and chronic alcohol intake in patients with HBsAg were the risk factors of HCC. CONCLUSIONS: Chronic alcohol intake may be an additive factor for the development of HCC in patient with LC caused by HBV. However, a prospective cohort study is needed to confirm these findings.

A Case of Diabetic Nephropathy without Microalbuminuria in Type 1 Diabetes / 대한소아내분비학회지

Diabetes is a rapidly increasing heath care problem all over the world due to increased prevalence during past decade. Diabetic nephropathy develops in 25-30% of patients with type 1 diabetes and is the leading cause of end stage renal disease. Diabetic nephropathy is characterized by persistent proteinuria, decline in renal function, hypertension and increased cardiovascular morbidity and mortality. Early detection of diabetic nephropathy risk is an important goal because early diagnosis and treatment prevent advanced renal damage and other diabetic complications. Increased urinary albumin excretion rate is widely accepted as the first clinical sign of diabetic nephropathy. However, reduced glomerular filtration or hypertension could be the first manifestation in some diabetic patients. We need improved markers and predictors of diabetic nephropathy risk. We report a case of diabetic nephropathy and decreased glomerular filtration rate (GFR) without microalbuminuria occcured in type 1 diabetic patient.

Successful Long Term Antifungal Agent Therapy for Renal Candidiasis without Surgical Drainage in a Very Low Birth Weight Infant

Improved survival rate of premature infants requiring intensive care lead into an increased risk for nosocomial infections such as disseminated fungal infection. Neonatal candida sepsis has become one of the most important causes of neonatal morbidity and mortality. The most common site of end organ involvement in premature infants with candidemia is the kidney. But no consensus has been reached concerning the treatment of candidemia in the newborn. We recently experienced a case of premature infant who was diagnosed as renal candidiasis with microabscess formation due to Candida Albicans and patient was treated successfully with long term liposomal amphotericin B and fluconazole therapy without surgical drainage.

Spontaneous Ileal Perforation in Very Low Birth Weight Infant without Evidence of Necrotizing Enterocolitis

Ongoing advances in the treatment of very-low-birth-weight infants have confronted us with a growing number of infants susceptible to acquired illnesses of the gastrointestinal tract. Although necrotizing enterocolitis has been regarded as the major cause of gastrointestinal perforation in preterm infants, the incidence of spontaneous perforation occurred in an apparently normal bowel is increasing. The risk factors for spontaneous intestinal perforation include prematurity, twin pregnancies, perinatal asphyxia, prior use of umbilical artery catheter, use of indomethacin and/or steroid, and bacterial or fungal sepsis. We report a case of spontaneous ileal perforation occurred in very low birth weight infant who was successfully treated with emergency operation.

A Clinical Study of Aseptic Meningitis in the Busan Area in 2002

PURPOSE: There was a outbreak of aseptic meningitis in Busan, 2002. We report the clinical features and causative viruses. METHODS: Two hundred seventy six children with aseptic meningitis who were admitted to the Department of Pediatrics, Maryknoll Hospital between January and December, 2002 were included. CSF, stool and throat swab viral cultures were done in 244 of these children. RESULTS: The male to female ratio was 1.4 : 1. Age of patients varied from five months to fourteen years old. Average age was 5.7+/-6.2 years old and mostly between one and four years.(41.3%) Monthly distribution revealed that the number of patients increased from May to July. The main symptoms were fever, headache and vomiting in this order. Respiratory tract infection symptom was associated from June to July and headache and nausea without fever were characteristically observed in children more than 10 years old from November to December. In peripheral blood examination, leukocytosis(WBC>10,000/mm3) showed in 34.8%, ESR was increased in 56.1%, and CRP was positive value in 61%. Therefore differential diagnosis was difficult through peripheral blood examination. CSF findings revealed mean leukocyte count 86.5+/-180.2/mm3, protein 41.7+/-32.9 mg/dL, glucose 56.4+/-9.9 mg/dL. Median hospitalized period was 4.7+/-7.2 days and compared with non-tapping group, hospitalized period was shorter and subsidance of symptoms was faster, therefore antibiotics injection period was shorter in the spinal tapping group. Virus was isolated in 31 cases of 244. The causative agents were echovirus 6, echovirus 9, echovirus 25, coxsakie virus B3, B4. CONCLUSION: There was an epidemics of aseptic meningitis in Busan, 2002; the causative agent was echovirus 6, 9, 25, coxsakie virus B3, B4.

Transcatheter Closure of Atrial Septal Defect Using Amplatzer Septal Occluder

BACKGROUND AND OBJECTIVES: Transcatheter occlusion (TCO) may be an alternative method for the surgical closure of a secundum atrial septal defect (ASD) below 20 mm in diameter. We performed this study in order to evaluate the safety and feasibility of an Amplatzer septal occluder for closing ASD bigger than 20 mm in diameter percutaneously. SUBJECTS AND METHODS: Thirty three of 39 patients presenting with ASD were included in this study (3 patients with a large defect over 32mm and 3 with multiple defects were excluded). The median age was 8.6 years (2.2 - 54) and median weight was 27 kg (10.7 - 85). The mean defect size was 15+/-3 mm as measured by transthoracic echocardiogram, 17+/-5 mm by transesophageal echocardiogram, and 21+/-6 (11 - 32) mm by balloon stretched diameter. The balloon stretched diameter was larger than 20 mm in 20 of 33 patients. The mean Qp/Qs was 2.3+/-0.7. The mean device size was 22+/-6 mm and the mean fluoroscopic time was 13+/-7 min. RESULTS: The device was successfully implanted in 29 of 33 patients. The 4 patients in which implantation failed showed a left disc protrusion into the right atrium. Three of these patients were treated surgically, and one underwent a successful second attempt of TCO 12 months after the first trial. Complete closure was obtained in 30 patients in follow-up. The complications encountered included;cobra-shaped deformity of the device (3), transient AV block (Wenckebach) (1), embolization of the radioopaque marker into the left atrial appendage (1), failure in the first device (1), and mild mitral regurgitation at 3 months follow-up due to device protrusion into the mitral valve (1). CONCLUSION: The Amplatzer septal occluder appears to be a promising device for TCO of ASD up to 32 mm in diameter, however, long-term follow-up in a large number of patients is warranted.

A Case of Congenital Nasal Pyriform Aperture Stenosis

The cause of congenital nasal pyriform aperture stenosis is unclear. The development of the facial skeleton occurs between the fifth and the eighth week of gestation. It is at this stage that the stenosis occurs due to overgrowth of the ossification of the maxilla. Infants are obligate nasal breathers. Incomplete and milder forms of nasal airway obstruction may be present with mild respiratory distress, cyanosis, respiratory failure, asphyxia and eventual death. The diagnosis is done by CT, which demonstrates marked narrowing of the nasal inlet. Mild stenosis can be managed conservatively with humidification and topical decongestants but if conservative treatment fails, surgical intervention is candidate. We report a case of congenital nasal pyriform aperture stenosis. The patient, a 2-day old male neonate, had cyanosis during feeding and noisy breathing relieved by crying. His symptoms and signs were improved with surgical intervention without development disturbances.

Two Cases of Infantile Hemangioendothelioma of the Liver in Neonates

Infantile hemangioendothelioma(IHE) of the liver is a rare benign vascular tumor that presents most commonly in infants before the age of 6 months. IHE presents as abdominal mass, cutaneous hemangiomas, unexplained jaundice, bleeding disorders, or congestive heart failure. Death often results from congestive heart failure despite appropriate treatment with digoxin and diuretics. IHE also is associated with Kasabach-Merritt syndrome, anemia, intraperitoneal hemorrhage secondary to rupture, consumptive coagulopathy and vascular malformation involving brain, skin, gut, and other organs. Although children with asymptomatic lesions may experience spontaneous regression within a year, symptomatic lesions shoud be treated aggressively because this disease can progress rapidly and may be fatal. Treatment options are divided into medical treatment, interventional therapy including embolization, and surgical resection. Corticosteroid may hasten involution by inhibiting proliferation of endothelial and smooth muscle cells, and this trial is warranted in most cases before invasive procedures are used. If steroid therapy is unsuccessful, early definitive treatment using embolization or ligation of the hepatic artery, resectional surgery, and orthotopic liver transplantation shoud be considered. We experienced symptomatic IHE in two neonates. In the first case, she showed respiratory failure and consumptive coagulopathy, and symptoms were aggravated despite steroid therapy, so a left lobectomy was performed. In the second case, he presented high output cardiac failure, and was successfully treated by the coil embolization of left hepatic artery. This coil embolization of hepatic artery for treating IHE was the first case in Korea we know of.

Comparison of Brainstem Auditory Evoked Response in Normal Infants, Male and Female, Right and Left Ear

PURPOSE: Brainstem auditory evoked response(BAER) is used as screening test for hearing disorders, damages of the central nervous system and congenital anomalies. We studied the difference values according to gender and stimulation sites in normal full-term infants. METHODS: We performed BAER in 38 male and 28 female normal full-term infants, delivered in the Gil Medical Center, Gachen Medical School, from March to July 1996, aged one to seven days. Amplitude I, V, V/I and latency I, III, V and interpeak latency(IPL) I-III, III-V, I-V were measured at 90, 60, 45, 30 dB. Data were analyzed between both sex and between both ears with Student t-test. RESULTS: There were no significant difference in male and female group with the same side's stimulation. At 90 dB, amplitude I of left ear stimulation was significantly higher than right in male and female. Amplitude V/I of right ear stimulation was significantly higher than left ear stimulation in total only. At 90 dB, latency I of right was significantly longer than left in male and female. Latency III of right was longer significantly in total only. IPL I-III, I-V was significantly longer in left than right in male and total. At 60 dB intensity, all data except latency I in total, showed no significant difference. CONCLUSIONS: Interpreting BAER, stimulation site and intensity should be considered. and further studies will be needed for the evaluation of the difference between left and right ear.

Incontinentia Pigmenti in a Mother and her Daughter

Incontinentia pigmenti (IP) is a rare multisystemic ectodermal disorder, which is characterized by vesicular, verrucous, and pigmented cutaneous lesions, and is frequently associated with various developmental defects of the eye, CNS, teeth, hair, and nail. It is regarded as an X-linked dominant genetic disorder. We recently experienced a case with IP, who presented with irregular, reticular, and slate-gray to brown colored pigmentation on the whole body at birth. Skin lesions were much improved by 6 month of age. The mother of this infant had the history of same cutaneous lesions in her neonatal period, suggesting that these lesions had familial tendency.

A Case of Saldino-Noonan Type Short Rib-Polydactyly Syndrome

Short rib-polydactyly syndrome (SRPS) is a rare type of skeletal dysplasia characterized by short limb dysplasia, thoracic hypoplasia, polydactyly and multiple visceral anomalies. It is transmitted as a autosomal recessive trait. There have been 4 classic types of SRPS, of which Saldino-Noonan type is the most common, and is characterized by very narrow thorax, short limb, postaxial polydactyly, striking metaphyseal dysplasia of tubular bones, pelvic abnormalities, hypoplasia of iliac bones, flat acetabulae, and abnormalities of urogenital, anorectal and cardiovascular system. We report a newborn infant who had typical features of Saldino-Noonan type SRPS, clinically and radiologically and had compatible autopsy findings.

Treatment of Tachycardia by Radiofrequency Catheter Ablation in Children and Adolescents

PURPOSE: RFCA has been proven to be an effective and safe tool for treating different kinds of tachycardia in adults. This study was designed to analyze the efficacy of this method in children and adolescents. METHODS: Seventy-eight patients referred to Gachon Medical School, Gil Hospital for ablation of supraventricular(SVT) and ventricular tachycardia(VT) between January 1997 and February 1999 were included in this study. An electrophysiologic study was performed in the same session to assure the diagnosis, and meet the appropriate criteria for ablation. All patients had regular follow-up at our center. RESULTS: Mean age of the patients was 9.9 years. Thirty-seven patients had Wolff-Parkinson- White syndrome, atrioventricular nodal reentrant tachycardiain in 12 patients, atrial flutter in 11 patients, atrial tachycardia in 9 patients, Mahaim tachycardia in 2 patients, nodoventricular tachycardia in one patients and VT in 6 patients was found. Seventy-seven patients were treated successfully(98.7%), 75 patients were treated in a single procedure, but 2 patients needed a second attempt. RFCA treatment failed in only one patient with nodoventricular tachycardia. In three of the 77 successfully treated patients(3.9%), tachycardia recurred after 1-3 months but could be treated by a further intervention. The duration of follow up after RFCA was from 2 to 27 months. CONCLUSION: RFCA is a highly effective method in the treatment of SVT and VT in children and adolescents. The major benefit of this technique is its potential as a cure for a chronic disease. RFCA can be used as the primary treatment for SVT and VT.

A Case of Improved Adrenal Insufficiency with Corticostreoid Treatment, Secondary to Adrenal Hemorrhage

The large size and vascularity of the neonatal adrenal glands are vulnerable to traumatic and asphyxial injuries. This condition varies in presentation, which the most common is an abdominal mass alone or mass with jaundice and anemia. Some infants show signs of adrenal insufficiency. Abdomial ultrasonogram is the most valuable diagnostic tool. To result in adrenal insuffiency, hemorrhage must involve both adrenals and at least 90% of the adrenocortical tissue must be destroyed. To affect infant may show signs of hypovolemic shock, electrolyte imbalance and metabolic acidosis. Treatment for adrenal insufficiency must be immediate and vigorous, and consists of intravenous glucose, fluid, and electolyte replacement. And conservative treatment failure is candidate for steroid replacement. We experienced a case of bilateral hemorrhage with adrenal insufficiency, who improved with hydrocortisone.

A Case of Candida Endocarditis with Vegetation on the Tricuspid Valve in a Preterm Infnat

Fungal endocarditis is a rare disease in infants, but it has been reported with increasing frequency among premature infants requiring neonatal intensive care. Congenital heart disease, pro- longed intravenous catheterization, the use of intravenous alimentation, broad-spectrum antibiotics administration and narcotic addiction are risk factors. Candida endocarditis is an unusual but severe complication of systemic candidiasis. Its occurrence has been related to the placement of a central venous catheter with its tip close to or within the right atrium and persistent candidemia. Embolization to major blood vessels is a common complication of Candida endocarditis and repeated pulmonary emboli are suspected to be cause of the repeated episodes of circulatory shock which finally leads to death. Thus, early diagnosis of Candida invasion and prevention of Candida endocarditis are essential for survival. We report a case of Candida endocarditis which was successfully managed with surgical removal and antifungal therapy. (J Korean Pediatr Soc 2000;43:832 836)

A Case of Unilateral Diaphragmatic Paralysis with Brachial Plexus Palsy in the Neonate

The diaphragm is especially important in sustaining minute ventilation in the neonate. Consequently, diaphragmatic paralysis is not tolerated well by the neonate and often results in prolonged respiratory failure. We experienced a case of unilateral diaphragmatic paralysis with the brachial plexus palsy in a male newborn infant who presented with apnea and cyanosis due to birth asphyxia. After endotracheal intubation, mechanical ventilaton was started. At two weeks after therapy, chest X-ray showed atelectasis and elevation of the right hemidiaphragm. The diagnosis of unilateral diaphragmatic paralysis was confirmed by real-time ultrasonography. At 4 weeks old, after several unsuccessful attempts at weaning from ventilatory support, right hemidiaphragm was plicated. On the second postoperative day, he could be weaned from ventiatory support without difficulty. He was doing well at follow-up 3 months later.

Magnetic Resonance Angiography in One Case of Hydraencephaly

In hydranencephaly, the cerebral hemispheres are absent or represented by membranous sacs with remnants of frontal, temporal or occipital cortex dispersed over the membrane. The brain stem is relatively intact. The cause of hydranencephaly is unknown, but bilateral occlusion of the internal carotid arteries during early fetal development can explain most of the pathologic abnormalities. We evaluated a case of hydranencephaly by magnetic resonance (MR) angiography. MR angiography shows both common, external carotid and vertebrobasilar arteries with no delineation of both internal carotid arteries from their origins. A brief review of the related literature was given on this subject.

A Case of Alagille Syndrome

Alagille syndrome is characterized by chronic cholestasis, posterior embryotoxon, skeletal abnormalities, cardiovascular abnormalities, and a typical face with prominent forehead and pointed chin. Its histological feature includes paucity of interlobular bile ducts. We experienced a 49-day-old female infant presenting with frequent upper respiratory tract infection and persistent jaundice. She had a typical face and chronic cholestasis. Echocardiograms revealed peripheral pulmonary stenosis. The histological examination of liver revealed paucity of interlobular bile ducts.

Clinical Significance of MRI in Hypoxic-Ischemic Encephalopathy During Neonatal Period

PURPOSE: Hypoxic-ischemic encephalopathy is a major neurologic problem and one of the most important perinatal causes of neurological morbidity. Evaluation of the presence, extent, and subsequent evolution of hypoxic-ischemic lesions may be very important. We studied the significance of imaging modality through the analysis of brain MRI findings of hypoxic-ischemic encephalopathy during neonatal period and comparison with findings of brain ultrasonography. METHODS: We analysed the forty-seven infants retrospectively who were diagnosed as hypoxic-ischemic encephalopathy and underwent brain MRI and ultrasonography from Jan. 1992 to May 1996. RESULTS: 1) The mean gestational age and birth weight of the twenty-seven infants who were premature were 32.8+/-2.08weeks and 1.97+/-0.44kg respectively. The mean gestational age and birth weight of twenty infants who were fullterm were 39.3+/-1.04weeks and 2.98+/- 0.93kg respectively. The primary hypoxic-ischemic insults occurred during antenatal, intrapartum and postnatal period. 2) The findings of brain MRI were classified into periventricular leukomalacia, encephalomalacia, basal ganglia lesion, focal parenchymal hemorrhage, ventriculomegaly without other lesion and normal finding. 3) Three infants among twenty-five infants with periventricular leukomalacia, four infants among seven infants with basal ganglia lesion and six infants among seven infants with focal parenchymal hemorrhage were not diagnosed by brain ultrasonography. 4) All of ten infants with encephalomalacia and four infants with ventriculomegaly without other lesion were diagnosed by brain ultrasonography. CONCLUSIONS: MRI can diagnose the hypoxic-ischemic lesions which would not be possible by brain ultrasonography. Therefore MRI is the imaging modality of choice for diagnosis in infants with hypoxic-ischemic encephalopathy. We believe that the benefits of MRI outweigh its somewhat higher cost, lack of portability and monitoring difficulties.

Clinical Significance of MRI in Hypoxic-Ischemic Encephalopathy During Neonatal Period

PURPOSE: Hypoxic-ischemic encephalopathy is a major neurologic problem and one of the most important perinatal causes of neurological morbidity. Evaluation of the presence, extent, and subsequent evolution of hypoxic-ischemic lesions may be very important. We studied the significance of imaging modality through the analysis of brain MRI findings of hypoxic-ischemic encephalopathy during neonatal period and comparison with findings of brain ultrasonography. METHODS: We analysed the forty-seven infants retrospectively who were diagnosed as hypoxic-ischemic encephalopathy and underwent brain MRI and ultrasonography from Jan. 1992 to May 1996. RESULTS: 1) The mean gestational age and birth weight of the twenty-seven infants who were premature were 32.8+/-2.08weeks and 1.97+/-0.44kg respectively. The mean gestational age and birth weight of twenty infants who were fullterm were 39.3+/-1.04weeks and 2.98+/- 0.93kg respectively. The primary hypoxic-ischemic insults occurred during antenatal, intrapartum and postnatal period. 2) The findings of brain MRI were classified into periventricular leukomalacia, encephalomalacia, basal ganglia lesion, focal parenchymal hemorrhage, ventriculomegaly without other lesion and normal finding. 3) Three infants among twenty-five infants with periventricular leukomalacia, four infants among seven infants with basal ganglia lesion and six infants among seven infants with focal parenchymal hemorrhage were not diagnosed by brain ultrasonography. 4) All of ten infants with encephalomalacia and four infants with ventriculomegaly without other lesion were diagnosed by brain ultrasonography. CONCLUSIONS: MRI can diagnose the hypoxic-ischemic lesions which would not be possible by brain ultrasonography. Therefore MRI is the imaging modality of choice for diagnosis in infants with hypoxic-ischemic encephalopathy. We believe that the benefits of MRI outweigh its somewhat higher cost, lack of portability and monitoring difficulties.

Doxapram in the Treatment of Idiopathic Apnea of Prematyurity Unresponsive to Aminophyline

No abstract available.