Pulmonary agenesis is a very rare anomaly. It is defined as total absence of the pulmonary parenchyma, vascular structures, and bronchi beyond the carina. We experienced a case of right pulmonary agenesis in association with other congenital defects who died at 1 day of age. The other defects included: esophageal atresia, tracheoesophageal fistula, cardiac malformation, anal atresia and a malformed left thumb. The cardiac malformations were a type of Pentalogy of Fallot, composed of right ventricular hypertrophy, ventricular septal defect, an overiding of aorta, pulmonary atresia, and an atrial septal defect. Hand roentgenograms of the malformed left thumb showed an unarticulated metacarpopharyngeal joint. This unique combination of anomalies is extremely rare.

No abstract available.
Hyperthyroidism*

Primary hyperparathyroidism is a rare endocrine disease in children. It involves bone and joint, urinary tract, gastrointestinal tract and cardiovascular system. The main cause of these involvement is high level of PTH in serum, resulting in hypercalcemia.An 11 years old male patient who had complained of limping gait since last 18 months, showed typical laboratory and radiological findings of primary hyperparathyroidism. At the ultrasonography, computed tomography and radionuclide scanning, a well defined mass(10 X 15mm) was found on the posterior aspect of the right thyroid lobe. The mass was confirmed histologically as adenoma of parathyroid gland. The patient was successfully treated with subtotal parathyroidectomy and temporal administration of calcium and vitamin D.We report this case of primary hyperparathyroidism with brief review of the literatures.
Adenoma ; Calcium ; Cardiovascular System ; Child ; Endocrine System Diseases ; Gait ; Gastrointestinal Tract ; Humans ; Hyperparathyroidism, Primary ; Joints ; Male ; Parathyroid Glands ; Parathyroidectomy ; Thyroid Gland ; Ultrasonography ; Urinary Tract ; Vitamins

PURPOSE: We evaluated clinical results and incidence of exposures of orbital implants according to operative methods (enucleation or evisceration) and types of orbital implants. METHODS: We retrospectively reviewed the charts of 222 patients who underwent hydroxy apatite or Medpor(R) implantation after enucleation or evisceration and were followed up for more than 3 months from July, 1992 to November, 2001 at department of ophthalmology, Busan Paik hospital. RESULTS: In 222 eyes of 222 patients, who underwent hydroxyapatite and Medpor(R) implantaion, the exposure of implants occurred in 21 eyes (9.5%). In these cases, the exposure of implants occurred in 16 (12.8%) of 125 eyes with hydroxyapatite implantation and 5 (5.2%) of 97 eyes with Medpor(R) implantation. The exposure of implants occurred in 6 (7.1%) of 84 eyes after enucleation and in 15(10.8%) of 138 eyes after evisceration. The exposure of implants occurred in 12 (12.2%) of 98 eyes that used standard evisceration and in 3 (7.5%) of 40 eyes that used modified evisceration. CONCLUSIONS: The exposure of Medpor(R) implants was less than that of hydroxyapatite implants. But, it was influenced by types of orbital implants as well as operator and operative skill. There are few differences of exposure rate between the modified evisceration and enucleation.
Busan ; Durapatite ; Humans ; Incidence* ; Ophthalmology ; Orbit* ; Orbital Implants* ; Retrospective Studies

PURPOSE:Autonomic neuropathy in adult diabetics is known to be common and poor in prognosis. Cardiac autonomic neuropathy increases sudden cardiac death and is present before apparant cardiac symptoms. Subclinical diabetic peripheral neuropathy is common with type 1 diabetes. In children, few studies for subclinical peripheral neuropathy and cardiac autonomic neuropathy were reported but the results were inconsistent. The authors investigated subclinical cardiac and peripheral neuropaties and evaluated correlation between nerve conduction velocity(NCV) and cardiac autonomic function(CAF) test in type 1 diabetic children and adolescents METHODS:Over 5 years from 1997 to 2002, 27 IDDM without clinical neuropathy were registered. Total 57 CAF and NCV were tested every 2 or 3 years. Duration of diabetes, average HbA1c during 1 year prior to the test, and urinary microalbumin excretion for 24 hours were assessed. Diabetic autonomic nervous function was evaluated by cardiovascular reflex test:falling systolic blood pressure in response to standing, heart rate changes in response to standing, beat-to-beat rate variation during deep breathing, RR interval change during and after Valsalva maneuver. Subclinical peripheral neuropathy was evaluated by NCV in both upper and lower extremities. Correlation of parameters according to NCV abnormality and microalbuminuria were analyzed. RESULTS:The age, duration of diabetes and microalbuminuria were not statistically different between children with normal and abnormal NCV. The level of HbA1c was associated with NCV abnormality. In a multivariate logistic model for NCV abnormality, level of HbA1c showed statistical significance after controlling the effects of age, duration of diabetes, and urinary microalbumin level, and the odd ratio was 1.532. The degree of CAF abnormality was marginally significant in the logistic model. Children with microalbuminuria showed older age and longer duration of diabetes than those without microalbuminuria. However, mean HbA1c level was not significantly different between the two groups. Degree of CAF abnormality was not significantly associated with microalbuminuria, either. In a general linear model including urinary albumin level as the dependent variable, none of independent variables was statistically significant. CONCLUSION: Therapy against neuropathy should be considered in patients with high HbA1c and abnormal CAF test even without clinically apparent neuropathy.
Adolescent ; Adult ; Blood Pressure ; Child* ; Death, Sudden, Cardiac ; Diabetes Mellitus, Type 1 ; Diabetic Neuropathies ; Heart Rate ; Humans ; Linear Models ; Logistic Models ; Lower Extremity ; Neural Conduction ; Peripheral Nervous System Diseases ; Prognosis ; Reflex ; Respiration ; Valsalva Maneuver

The author observed clinical study and response of -interferon treatment of 43 cases of chronic hepatitis who were admitted to Department of Pediatrics Pusan National University College of Medicine during the period of 4 year 6 months from Jenuary, 1988 to Jun, 1992. The results were as follows: 1) Of 43 patients with chronic hepatitis, CPH was 17 cases (39.5%) and CAH was 26 cases (60.5%). 2) Of 43 patients, chronic hepatitis caused by HBV was 37 cases (86.0%) 2 cases were by Wilson's disease, 1 case was by glycogen storage disease and in the 3 cases, the cause was undetermined and CPH by HBV and CAH were 14 cases (82.4%), 23 cases (88.5%) respectively. 3) A peak frequency (46.5%) of chronic hepatitis occurred between 6 to age and male predominated in a propotion of 3.5:1. 4) On the clinical manifestation, jaundice and hepatomegaly was higher frequency on CAH than on CPH. 5) On the laboratory findings, CAH had higher serum ALT and direct bilirubin level than CPH. 6) On effect of -interferon treatment for patients of CAH, serum ALT and AST levels normalized in 81.8% of patients, negative conversion of HBeAg was 38.5%, Anti-HBe serocoversion was 7.7%, negative conversion of HBsAg was 7.7% and the clearnce of HBV-DNA from seum showed 60.0%. 7) The HBeAg seroconversion (28.6%) on children of HBsAg(+) mother showed lower reponse than HBeAg seroconversion (50.0%) of HBsAg(-) mother after -interferon treatment for patients of CAH.
Bilirubin ; Busan ; Child* ; Glycogen Storage Disease ; Hepatitis B e Antigens ; Hepatitis B Surface Antigens ; Hepatitis B, Chronic* ; Hepatitis, Chronic* ; Hepatolenticular Degeneration ; Hepatomegaly ; Humans ; Jaundice ; Male ; Mothers ; Pediatrics

Morphological changes of hepatic microcirculation, especially in the peribiliary plexus, in cirrhotic livers of rats induced by repeated intraperitoneal injections of N-diethyinitrosamine (DEN) (100mg/kg/week) were studied by scanning electron microscopy. Control rats were treated with saline. The livers were perfused with saline and injected with methyl-methacrylated resin (Mercox CL-2B) through the thoracic aorta. Diffuse nodular changes mimicking human cirrhosis were seen in the livers six weeks after injections of DEN. The cirrhotic livers showed an increase of vascular channels composed mainly of venous branches around the regenerating nodules and increased arterioloportal anastonloses. Peribiliary plexi of the cirrhotic livers had more vessels than those of the controls. Many dilated veins and ramificating portal vein branches were also Present. Direct connections between peribiliary plexi and sinusoids or between peribiliary plexi and portal veins were increased in the cirrhotic liver. These results suggest that the peribiliary plexi in experimentally induced cirrhotic liver might play a role in a collateral circulation under a state of portal hypertension.
Humans ; Rats ; Animals

The incidence of conjoined twins is so rare that few anesthesiologists have an opportunity of managing them. Especially in Korea, there are only a few reports describing the anesthetic management for surgical separation of newborn conjoined twins. We experienced the successful anesthetic management for surgical separation of thoraco-xiphopagus conjoined twins without any particular problems. After applying the noninvasive monitors (ECG, pulse oximeter), one of the twins (twinA) with congenital heart disease was administered with intravenous ketamine for induction of anesthesia and intubated without neuromuscular blocker. Anesthesia was maintained with N2O-O2 and hand ventilation using Mapleson D breathing circuit. After maintaining airway of the twinA, the twinB was intubated and maintained with the same manner. Eighteen days after the separation procedure, the twinA with congenital heart disease died and the other one, twinB has been alive with normal growth and development.
Anesthesia ; Growth and Development ; Hand ; Heart Defects, Congenital ; Humans ; Incidence ; Infant, Newborn ; Ketamine ; Korea ; Neuromuscular Blockade ; Respiration ; Twins, Conjoined* ; Ventilation

Purpose: To report a rare case of primary eyelid tuberculosis treated using systemic anti-tuberculosis medications.Case summary: A 30-year-old male presented to our hospital with an upper eyelid mass in the left eye for 1 month. An atypical cystic mass, pale yellow in color with a smooth surface, was observed on the medial side of the left upper eyelid. There were no associated ophthalmic or systemic symptoms. Excisional biopsy of the mass was performed under local anesthesia and histopathological examination revealed chronic granulomatous inflammation with central Langhans-type giant cells. Acid-fast bacilli smear was negative, but polymerase chain reaction for Mycobacterium tuberculosis was positive. There were no significant findings in blood tests and chest radiographs. The patient was diagnosed with primary eyelid tuberculosis and treated with systemic anti-tuberculosis medications for 6 months. There were no complications or recurrences during 1 year of follow-up. Conclusions Although eyelid tuberculosis is rare, early diagnosis through biopsy is important when a granulomatous tuberculosis lesion is suspected. Delayed diagnosis can lead to serious cosmetic and functional complications. Appropriate systemic anti-tuberculosis medications are required to treat these cases.

A case report of a perforated choledochal cyst with bile peritonitis occurring in a 13-month old girl is presented. The perfortion was identified on the right lateral aspect of the common bile duct. And T-tube drainage was instituted. Episodes of ascending cholangitis followed pooling of the stagnant bile in the biliary system. Reoperation was recommended but in vain.
Bile* ; Biliary Tract ; Cholangitis ; Choledochal Cyst* ; Common Bile Duct ; Drainage ; Female ; Humans ; Infant* ; Peritonitis* ; Reoperation