Ocular albinism is a rare condition of abnormality in the cellular metabolism of the product of melanin in the ocular tissue. This disease is transmitted as an imtermediate sex linked recessive. This condition was first described by Nettleship in 1909. In addition, many authors described this condition. The fundamentalelinical symptoms and signs are lowered visual acuity, photophobia, nystagmus, the yellow-orange color of the fundus with the choroidal vessels perfectly visible, absence of the foveal reflex and iris that transilluminates well with scleral illumination. Recently, we experienced two cases of typical ocular albinism.
Albinism, Ocular* ; Choroid ; Iris ; Lighting ; Melanins ; Metabolism ; Photophobia ; Reflex ; Visual Acuity

BACKGROUND: This study examined hemodynamic variables, oxygen delivery, extraction, and consumption in response to acute progressive hypoxia and hypercarbia in the setting of apnea. METHODS: Apnea was induced in 9 healthy mongrel dogs by disconnecting animals from mechanical ventilation of 30 minutes with pure oxygen. Hemodynamic variables, oxygen transport, extraction, and consumption were rapidly and repeatedly measured using pulmonary arterial and arterial catheters until cardiac output was undetectable. RESULTS: The baseline PaO2, PaCO2, pH, base excess were 318 +/- 137 mm Hg, 36 +/- 3.5 mm Hg, 7.30 +/- 0.06, 6.81 +/- 2.65 mmol/l respectively. Hypercarbia and hypoxemia (76 +/- 33 mm Hg) was first noted at 1 and 4 minute respectively. Base excess was not changed. Indices of preload (PCWP and CVP) were increased early in the time course (p<0.05). In contrast, indices of afterload (SVR) increased later, just before cardiac decompensation began (p<0.05). No significant reduction of cardiac output, oxygen delivery, extraction, and consumption was detected just until abrupt cardiac decompensation started, 5 minute. CONCLUSIONS: These data suggest that the early increase in preload was primarily due to hypercarbia whereas the late increase in afterload was due to hypoxemia, but the main cause of acute cardiac decompensation was a critical decrease in arterial oxygen tension with some contribution of increased preload and afterload.
Animals ; Anoxia ; Apnea* ; Cardiac Output ; Catheters ; Dogs* ; Hemodynamics* ; Hydrogen-Ion Concentration ; Oxygen* ; Respiration, Artificial

It is well documented that rapid administration of solution containing dextrose results in marked hyperglycemia and osmotic diuresis. The maternal effect of hyperglycemia caused by rapid administration of dextrose solution may affect the fetus or the newborn baby as well. Therefore, there was a need to ascertain an appropriate IV solution for prehydration in cesarean section. The aim of this study was to determine an appropriate solution for cesarean section based on the results of the effects of matemal IV glucose solution on the newborn's glucose, electrolyte and insulin. 40 parturients scheduled for cesarem section were chosen at random. They were divided into two groups : one group received Hartmann's solution and the other group received 5% D/W. Patients' venous blood was drawn on the operating table prior to starting IV infusion for the measurement of blood sugar and electrolytes(Na, K, Cl) in various conditions of NPO. Then, intra-venous fluid was administered rapidly for prehydration about 20 ml/kg/hr(about 500 ml for 20 minutes) while anesthesia was induced and maintained with an endotracheal tube in place. The second blood samples were taken from matemal vein, umbilical vein and artery immediately after delivery for the measurement of glucose and electrolyte. At the same time insulin levels of umbilical veins were measured. The prehydrative glucose levels were 75.40+/-20.80 mg/dl in group 1 and 78.60+/-12.44 mg/dl in group 2. Both values were within normal range without significant hypoglycemia following the various times of NPO. Posthydration values of maternal glucose in group 2 with glucose infusion was 221.30+/-3.62 mg/dl. This was significantly higher than 72.20+/-7.11 mg/dl in group 1(p<0.05). At the time of delivery, the values of umbilical venous and arterial blood glucose in group 2 were significantly higher than in group 1(p<0.05). The values of insulin in umbilical venous blood in group 2 was 48.09+/-25.54 uU/ml. This was significantly higher than 6.06+/-2.06 uU/ml in group 1(p<0.05). Hydration using either solutions did not affect the value of electrolyte either in the mother or newborn baby. However, the rapid administration of glucose solution increased the maternal and umbilical glucoses as well as umbilical insulin proportionally. As the result of this study, prehydration with the solution containing glucose for cesarean section increased maternal and fetal glucoses as well as fetal insulin levels. Therefore, prehydration with any solution containing glucose is not recommended to prevent maternal and fetal hyperglycemia, and can result in delayed newbom hypoglycemia. It was concluded that prehydration with balanced electrolyte solution without dextrose seems to be safer to babies as well as mothers.
Anesthesia ; Arteries ; Blood Glucose ; Cesarean Section* ; Diuresis ; Female ; Fetus ; Glucose* ; Humans ; Hyperglycemia ; Hypoglycemia ; Infant, Newborn ; Insulin* ; Mothers ; Operating Tables ; Pregnancy ; Reference Values ; Umbilical Veins ; Veins

The authors have experienced with three cases of Leber's hereditary optic atrophy one family which is a relatively rare condition characterized by acute or subacute failrure of central vision presenting as a retrobulbar neuritis or optic atrophy typically inypung males in late teens or in the early twenties, though the age range is very wide. The literature relating to Leber's hereditary optic atrophy was briefly reviewed.
Adolescent ; Humans ; Male ; Optic Atrophy ; Optic Atrophy, Hereditary, Leber* ; Optic Neuritis

Recently several reports have described the usefullness of meperidine as a sole agent for spinal anesthesia. In this study, 50 mg meperidine mixed with l5-25 mg lidocaine(group 1, n= 11), 25 mg meperidine mixed with 40~50 mg lidocaine(group 2, n= l0) and 65~75 mg lidocaine only (group 3, n=9) were respectively injected into the subarachnoid space for elective cesarean section. The results were as follows: 1) Hypotension over 20% decrease in systolic blood pressure was found in 7 out of 11 cases in group 1,5 out of 10 cases in group 2 and S out of 9 cases in group 3. The dosage of ephedrine used for hypotension was not signifieantly different amoung the three groups. 2) The time from subarachnoid injection to Bromage scale 0 of group l was 77.3+/-20.7 minutes which was significantly shorter than 125.0+21.5 minutes of group 3(p<0.05). 3) The duration from the administration of the drug until the development of severe pain postoperatively was checked as 483.6+/-157.9 minutes in group 1, 424.0+/-109.4 minutes in group 2 which were significantly longer than 55.5+/-25 minutes in group 3(p<0.05). 4) Nausea/vomiting were occured 5/0 out of l1 cases in group 1, 4/1 out of 10 cases but none in group 3. 5) Complaints about pain during the operation occured in 5 out of 9 cases in group 3, but none in group 2 and 3. 6) The Apgar score and umbilical cord blood gas analyses were not significantly different amoung the three groups. The results of this study clear1y indicate that combining meperidine with hyperbaric spinal lidocaine for cesarean section is a safe and effective method of improving intraoperative pain relief. Furthermore, it provides adquate prolonged postoperative analgesia.
Analgesia ; Anesthesia, Spinal* ; Apgar Score ; Blood Pressure ; Cesarean Section* ; Ephedrine ; Female ; Fetal Blood ; Hypotension ; Lidocaine* ; Meperidine* ; Pregnancy ; Subarachnoid Space

The subject of ocular injuries is of great importance in military surgery, not only for scientific and human reasons but also from an economic standpoint. The authors analysed 59 cases of perforating ocular injuries who were admitted to the Department of Ophthalmology, C.A.F.G.H. The results from these analyses were as follow: 1. The incidence of ocular perforating injuries was 16.4% of all patients who were admitted to the Department of Ophthalmology during last 15 months and was 45% of ocular injuries. 2. The incidence of perforating injuries that involved in one eye was 84.8% and involved in both eye was 15.2%. 3. The perforating ocular injuries were most common in spring(52.5%) and secondly in summer(28.8%). 4. The ocular perforation occured most frequently in the cornea(55.8%), followed by corneosclera(23.5%) and sclera(20.6%). 5. The most common cause of ocular perforating injuries was land mine(28.8%), followed by grenade(23.7%), gun(20.3%), bullet(10.2%) in frequency. 6. The incidence of multiple ocular perforations was relatively higher in land mine, grenade and blast injuries, and single perforations were observed more common in gun, bullet and secondary missile. 7. Other ocular manifestations accompanied by the ocular perforations were hyphema(60%), uveal prolapse(60%), followed by lid laceration, burn, conjunctival laceration, etc. 8. Prolapsed uveal tissue occured the highest frequency in the corneoscleral perforations, on the other ha.nd, intraocular foreign bodies in scleral perforations and traumatic cataracts in the corneal perforations. 9. The location of intraocular foreign bodies was most common in vitreous and outer coat(66.7%), followed by anterior chamber, lens and retroorbital space. 10. Final visual acuity over 20/200 accounted for 47.4% in the corneal perforations, 42.9% in the scleral perforations, 12.5% in the corneoscleral perforations.
Anterior Chamber ; Blast Injuries ; Burns ; Cataract ; Corneal Perforation ; Foreign Bodies ; Humans ; Incidence ; Lacerations ; Military Personnel ; Ophthalmology ; Visual Acuity

BACKGROUND: Stellate ganglion block (SGB) is the most common nerve block procedure in pain clinics. To evaluate changes in the hemodynamics and peripheral blood flow on the affected extremity after SGB, SGB was performed unilaterally one at a time on the right and left stellate ganglions by injecting 1% mepivacaine 10 ml without epinephrine in a designated healthy man. METHODS: SGB was repeated 16 times in one subject (right side SGB: 8, left side SGB: 8) by the same clinician. The mean arterial pressure (MAP), heart rate (HR), cardiac index (CI), systemic vascular resistance index (SVRI) and peripheral blood flow were measured in the supine position before (control), and 3, 6, 9, 12, and 15 minutes after SGB using thoracic electrical bioimpedence (Bioz system A-10043, Cardiodynamics, USA), sphygomanometer, and flow meter. RESULTS: The values after SGB including MAP, HR, CI, and SVRI increased slightly compared to the control value. However, peripheral blood flow increased significantly (p < 0.05). The SGB did not affect systematic hemodynamics and the comparison between left and right SGB in hemodynamic changes were not clinically significant. Following SGB, ptosis (100%), nasal stiffness (100%), skin temperature elevation (100%), hoarseness (100%), numbness (81%), dizziness (25%), and swallowing difficulty (25%) were observed. CONCLUSIONS: We concluded that SGB showed to be a hemodynamically safe clinical technique.
Arterial Pressure ; Deglutition ; Dizziness ; Epinephrine ; Extremities ; Heart Rate ; Hemodynamics* ; Hoarseness ; Hypesthesia ; Mepivacaine ; Nerve Block ; Pain Clinics ; Skin Temperature ; Stellate Ganglion* ; Supine Position ; Vascular Resistance

BACKGROUND: A right thoracoscopic thoracic sympathicotomy involves the removal of T2 and T3 sympathetic chains. Since part of the sympathetic fibers to the heart traverse these two ganglia, we examined the hemodynamic changes during a thoracoscopic thoracic sympathicotomy in primary hyperhidrosis. METHODS: Noninvasive cardiac output monitoring was done on the both side of the neck and chest. A physiograph for measuring of continuous blood flow was taken from the right index finger and a thermometer was placed in the right palm. Following endotracheal intubation was done with double lumen endotracheal tube, anesthesia was maintained with isoflurane. Sympathicotomies were done for T2-3 during one lung ventilation. Heart rate (HR), mean arterial pressure (MAP), systemic vascular resistance index (SVRI), cardiac index (CI), accelerated contractility index (ACI), end-diastolic index (EDI), and temperature were recorded at arrival, before sympathicotomy, after sympathicotomy at 1, 2, 3, 4 and 5 minuets. The blood flow of the right index finger was recorded before and after the sympathicotomy. RESULTS: Concurrent with initiation of the sympathicotomy, MAP and SVRI were reduced, but the CI was elevated. It was accompanied with right palmar temperature elevation and an increase in the blood flow of the right index finger. CONCLUSIONS: A thoracoscopic thoracic sympathicotomy reduces MAP and SVRI and elevates CI, palmar temperature, and blood flow. We concluded that the hemodynamic changes during a thoracoscopic thoracic sympathicotomy seems to be the peripheral vasodilatation.
Adrenergic Fibers ; Anesthesia ; Arterial Pressure ; Cardiac Output ; Fingers ; Ganglia ; Heart ; Heart Rate ; Hemodynamics* ; Hyperhidrosis* ; Intubation, Intratracheal ; Isoflurane ; Neck ; One-Lung Ventilation ; Thermometers ; Thorax ; Vascular Resistance ; Vasodilation

BACKGROUND: Although idiopathic cardiomyopathies(i-CMP) are very important in all age groups, the epidemiology of i-CMP in children has not been well defined. A retrospective study in Korean children was performed in 1998 to obtain basic data on i-CMP. MATERIAL AND METHOD: The medical records of all patients aged birth to 15 years from the hospitals where pediatric cardiologists worked were reviewed to obtain information on i-CMP. Pediatric cardiologists from a total of 22 hospitals were participated in reviewing the medical records of their patients and filling up the protocol. The data were pooled to the study committee and reviewed. RESULTS: Of the 278 cases with i-CMP, there were dilated cardiomyopathy (d-CMP) in 182 (65.4%): hypertrophic cardiomyopathy (h-CMP) in 74 (26.6%): restrictive cardiomyopathy (r-CMP) and unclassified in 17 (6.1%) and 5 (1.9%) each. The average annual occurrence of new cases as a whole was 2.65 per 100,000 (95% CI: 1.5-3.7): d-CMP, 1.73/100,000/year (95% CI: 0.73-2.73): h-CMP, 0.71/100,000/year (95% CI: 0.35-1.07): r-CMP, 0.16/100,000/year (95% CI: 0.02-0.3). The median age at the time of diagnosis was 11 months in d-CMP: 3.0 years in h-CMP: 6.9 years in r-CMP. The survival rate in d-CMP was 76% at 1 year, 72.5% at 2 year, 70% at 5 year. There was no difference in survival rate according to age (in d-CMP, between children less than 2 years of age and over 2 years of age (74% vs. 79% at 1 year: 67% vs. 76% at 5 year, p=NS): in h-CMP, between children less than 1 year of age and over 1 year of age (84% vs. 96% at 1 year: 63% vs. 81% at 5 year, p=NS)). R-CMP showed the worst survival rate (72% at 1 year, 30.2% at 5 year). CONCLUSION: In spite of the inherent defects of retrospective analysis, this study provides the useful epidemiological data in children with i-CMP. However, more systemic approach is needed to define the nature of the i-CMP in children.
Cardiomyopathies* ; Cardiomyopathy, Dilated ; Cardiomyopathy, Hypertrophic ; Cardiomyopathy, Restrictive ; Child* ; Diagnosis ; Epidemiology ; Humans ; Medical Records ; Parturition ; Retrospective Studies ; Survival Rate

Intramural duodenal hematoma is an uncommon condition, which usually develops after blunt abdominal trauma. It is also reported as a complication of anticoagulant therapy, blood dyscrasia, pancreatic disease, and diagnostic and therapeutic endoscopy. The typical clinical pictures of intramural duodenal hematoma consist of upper abdominal pain, vomiting, fever, and hematochezia, and it is rarely accompanied by intestinal obstruction, peritonitis, and pancreatitis as its complication. We report a case of intramural duodenal hematoma extended to peritoneal cavity, and accompanied by acute pancreatitis following therapeutic endoscopy for duodenal ulcer bleeding in a 32-year-old man who was on maintenance of anti-coagulation therapy after valvular heart surgery.
Acute Disease ; Adult ; Diagnosis, Differential ; Duodenal Diseases/*diagnosis/pathology/surgery ; Duodenal Ulcer/*complications ; Hematoma/*diagnosis/pathology/surgery ; *Hemostasis, Endoscopic ; Humans ; Male ; Pancreatitis/complications/*diagnosis ; Peptic Ulcer Hemorrhage/*therapy ; Postoperative Complications ; Tomography, X-Ray Computed