No abstract available.
Goldenhar Syndrome*

No abstract available.
Scalp* ; Skin* ; Transplants*

Polydactyly is one of the most common congenital anomalies of the limb, however its surgical treatment varies from simple excision to reconstruction. The purpose of this study is to present out classification of polydactyly in which the type of surgery was concerned primarily. Two hundred and thirty five digits(134 fingers and 101 toes) in 188 patients were operated from 1980 to 1992. We divided the polydactyly into two types-the simple type, in which the extradigit arises from only one digit, and the complex type in which the extradigit connects more than two adjacent main digits. The simple type was subdivided into joint type(type I), in that the extradigit has its own joint in its origine; epiphyseal type(type II), the extradigit share common epiphysis with main digit; and hypoplastic type(type III), the extradigit is connected only by soft tissue to the main digit. The epiphyseal type(type II) was further divided into subtype A(type IIA), in that the origin seems to be directly derived from the epiphysis; and subtype B(type IIB), which resemble an osteochondroma. Type III and type IIB can be treated by simple excision, however the type I and type IIB can be treated by arthroplasty with or without osteotomy as well as excision of extradigit. Result of surgical treatment in 1 digit of the simple form, which siginifies the extradigit arising from only one digit, are good in 193 digits(91%), fair 14(6.6%), and poor 5(2.4%) after an average follow-up period of 20 months. Our principles in the surgical treatment of polydactyly was treatment according to the type, and early treatment.
Arthroplasty ; Classification ; Epiphyses ; Extremities ; Fingers ; Follow-Up Studies ; Humans ; Joints ; Osteochondroma ; Osteotomy ; Polydactyly

No abstract available.
Facial Nerve* ; Paralysis* ; Transplants*

No abstract available.
Congenital Abnormalities*

Acardiac twinning affects 1 in 100 monozygotic twin pregnancies and 1 in 35,000 pregnancies overall. This condition is characterized by the absence or rudimentary development of fetal heart, and associated with various anomaly. The presence of an acardiac twin requires the normal (or "pump") twin to provide circulation for itself, as well as the acardiac sibling. The acardiac malformations are uniformly fatal in the affected twin, and mortality in the co-twin is as high as 55%. The principal perinatal problems associated with acardiac twinning are pump-twin congestive heart failure, maternal hydramnios, and preterm delivery. We recently experienced a case of acardius anceps associated with a normal male infant, so present with a brief review of the literature.
Fetal Heart ; Heart Failure ; Humans ; Infant ; Male ; Mortality ; Polyhydramnios ; Pregnancy ; Siblings ; Twins, Monozygotic

No abstract available.
Anemia, Megaloblastic* ; Humans ; Megaloblasts* ; Methotrexate*

The acute respiratory distress syndrome (ARDS) is a disorder of diffuse lung injury secondary to a wide variety of clinical insults including sepsis. It is manifested by impaired oxygenation, pulmonary edema, decreased static and dynamic compliance of lung, and increased airway resistance. Reduced lung compliance together with increased airway resistance is a hallmark of the syndrome and has generally been considered as the primary abnormality of lung mechanics. The study was designed to reproduce an ARDS condition in an animal model with acute lung injury and to determine whether these changes are reversed by inhalation of aerosolized bronchodilator such as salbutamol or nicorandil. Six adult mongrel dogs were exposed to intravenous oleic acid (0.08 ml/kg) mixed into a same amount of pure enthanol. We defined a condition of PaO2 less than 60 mmHg in FiO2 0.6 as an acute diffuse lung injury. Lung mechanics were deteriorated after oleic acid injection, from 27.33+/-2.73 to 17.50+/-.47 cmH2O/l/sec in compliance and from 14.55+/-.46 to 26.33+/-.02 ml/cmH2O in airway resistance. Salbutamol or Nicorandil inhalation significantly improved lung compliance to 30.57+/-11.30 or 36.33+/-.00 cmH2O/l/sec and airway resistance to 16.77+/-.47 or 19.55+/-.45 ml/cmH2O. From above data, we carefully concluded that compromized lung mechanics in adult respiratory distress syndrome could be effectively managed by inhalation of aerolized bronchodilator.
Acute Lung Injury ; Adult* ; Airway Resistance ; Albuterol ; Animals ; Compliance ; Dogs ; Humans ; Inhalation* ; Lung Compliance ; Lung Injury ; Lung* ; Mechanics* ; Models, Animal ; Nicorandil ; Oleic Acid ; Oxygen ; Pulmonary Edema ; Respiratory Distress Syndrome, Adult ; Sepsis

BACKGROUND: Brief episode of coronary artery occlusion (i.e., ischemic preconditioning) makes the heart more resistant to injury from a subsequent ischemic insult. Although a great deal of effort has been made in studying ischemic preconditioning, the underlying mechanism of ischemic preconditioning and its effect on hypothermic insult has not been elucidated. This study was performed to see whether ischemic preconditioning protects against the depression of cardiac contractility induced by hypothermic cardioplegic arrest/reperfusion. And recently, adenosine was known to have some correlation with the mechanism of preconditioning. If so, does this effect remain after the blockade of adenosine receptor by 8-phenyl theophylline? METHOD: Twenty-four Sprague-Dawley rat weighed 250-350g were used and divided into three groups. Rat hearts were removed rapidly, and each isolated heart paced with a rate of 180/min was perused by modified Krebs-Hensleit buffer(KHB) solution on a Langendorff apparatus far an hour. After obtaining baseline data including left ventricular pressure(LVP), dp/dt, and coronary flow, cardiac arrest was induced by perfusion of 0degrees C crystalloid cardioplegic(St Thomas) solution. After that, all hearts were stored in the same St Thomas solution at salute temperature far 2 hours. In group I (control group), the hear was reperfused by KHB solution. In group II(preconditioning group), the heart was subjected to two 2-minute episode of global ischemia followed by 5 minute reperfusion with KHB solution(preconditioning) before cardiac arrest. In group III(phenyl theophylline group), the heart was subjected to preconditioning procedure and 8-phenyl theophylline at 10muM in concentration was added to KHB solution at time of reperfusion. Observing parameter was obtained in each group at 10, 20, 40 and 60 minutes after starting reperfusion and compared statistically by use of one way ANOVA test(STASTICA, release 4.5). P-value less than 0.05 was considered significant. RESULTS: Although depressed LVP, dp/dt, and Coronary flow were seen in all groups during the reperfusion period, the preconditioned group showed more effective recovery of LVP than that of the control group, especially at 10, 20 and 40 minutes(p<.05). We failed to demonstrate the difference between the phenyl theophylline group and the control group(p=NS). CONCLUSION: These results suggest that ischemic preconditioning has protective effect on recovery state of hypothermic cardioplegic arrest/reperfusion. Its protective effect was limited during early reperfusion stage and was blocked by adenosine blocker.
Adenosine ; Animals ; Coronary Vessels ; Depression ; Heart Arrest ; Heart* ; Ischemia ; Ischemic Preconditioning* ; Myocardium* ; Perfusion ; Rats ; Rats, Sprague-Dawley ; Receptors, Purinergic P1 ; Reperfusion ; Theophylline

Cutaneous smooth muscle hamartomas are benign proliferations of smooth muscle bundles within the dermis. They can be congenital or acquired, and most cases are congenital. Congenital smooth muscle hamartomas (CSMHs) usually manifest at birth as well-circumscribed, frequently hypertrichotic, hyperpigmented or skin-colored patches or plaques on trunk or extremities. We report a case of CSMH in a 10 year-old girl, who showed a localized skin-colored patch showing prominent follicular papules on the lateral aspect of her right upper arm, which were found at birth. There was no hypertrichosis and the pseudo-Darier sign was negative. This patchy follicular variant is the less common clinical type of the disease.
Arm ; Dermis ; Extremities ; Female ; Hamartoma* ; Humans ; Hypertrichosis ; Muscle, Smooth* ; Parturition