The sella turcica is often the key to intracranial diseases and some endocrine disorders. It is significanly enlarged in hypothyroidism, pituitary tumors, some types of increased intracranial pressure and decreased in size in hypopituitarism. Estimation of the sellar size by naked eye is not scientific and moreover very difficult, especially in the borderline sella, an everyday problem of radiologist and pediatrician. It would be very convenient in evaluating the size of the sellar turcica if there are reliable standards. However normal values of the sella turcica for children have not been published in Korea yet. Means+/-2 S.D. of the area and volume of the sella turcica were obtained from skull films taken antero-posterior and lateral projections with focus-film distance of 36 inches in 517 Korean children(Boys, 293; Girls, 224). The area was calculated using the formula : A=LxD, and the volume was studied using the formula : V=1/2 (LxDxW) where A is area ; V, volume ; L, length ; D, depth and W, width of the sella turcica (Fig. 1,2,3). The results obtained are presented in Tables 2~5 and Figures 4~7. By the data, sella turcica area above 70mm2 in infants (0~1 year of age), 90mm2 in preschool children(2~5 years of age), 100mm2 in schoolchildren (6~11 years of age) and 140mm2 in adolescents(12~14 years of age) and/or sellar turcica volume above 450mm2 in infants, 600mm2 in preschool children, 700mm2 in school children and 1,000mm2 in adolescents can be regarded abnormally large sella whereas sella turcica area below 10mm2 in infants, 30mm2 in preschool children, 40mm2 in school children and 50mm2 in adolescents and/or sella turcica volume below 60mm2 in infants, 150mm2 in preschool children, 300mm2 in schoolchildren and 450mm2 in adolescents can be said abnormally small sella. Separate plottings of the volume of the sella turcica for boys and girls show a rapid increase in size during the first two years of life, gradual deceleration in growth rate thereafter and pre-pubertal acceleration of growth in girls by 12 years of age and in boys by 14 years of age. The sella turcica of boys tends to be larger than that of girls from the infancy to 10 years and girls tend to be larger than that of boys between the ages of 11 and 14 years because of the earlier pre-adolescent growth acceleration in girls compared with boys(Fig. 8). An application of these normal values for the sella turcica should help the pediatrician, radiologist, neurologist and neurosurgeon in judging the size of the sellar turcica for children.
Acceleration ; Adolescent ; Child* ; Child, Preschool ; Deceleration ; Female ; Humans ; Hypopituitarism ; Hypothyroidism ; Infant ; Intracranial Pressure ; Korea ; Pituitary Neoplasms ; Reference Values ; Sella Turcica* ; Skull

No abstract available.
Humans ; Neurogenic Bowel* ; Spinal Cord Injuries*

Hereditary fructose intolerance (HFI) is a carbohydrate metabolic disease of autosomal recessive inheritance. The basic deficit is deficiency of aldolase B, the enzyme catalyzing catabolism of fructose-1-phosphate, which is found only in intestinal mucosa, liver and kidney. Its main symptoms are abdominal pain, vomiting, hypoglycemia, and severe liver disease following the ingestion of fructose. Neurologic impairment is not typical in HFI, but it can occur in the acute phase of the disease. Neurologic impairment is related to the acute hepatic toxicity of fructose (hypoglycemia, abnormal coagulation, cardiovascular collapse). The 7 year-old German girl admitted because of generalized tonic clonic seizure. She had the first seizure at the age of 2, and was diagnosed as Lennox-Gastaut syndrome. Thereafter, frequent morning and midnight seizures were developed following indigestion of milk, sweety cake and cookies. Her family history was unknown because she was adopted from India at the 4 months of age. She showed developmental delay. After the ingestion of fructose, the patient experienced hypoglycemic episode within 60-90 minutes of the intake. Based on this finding, she was diagnosed as HFI. With fructose free diet, the patient became free of seizure even without the anticonvulsant, and improved in growth and development.
Abdominal Pain ; Child ; Diet ; Dyspepsia ; Eating ; Female ; Fructose ; Fructose Intolerance* ; Fructose-Bisphosphate Aldolase ; Growth and Development ; Humans ; Hypoglycemia ; India ; Intestinal Mucosa ; Kidney ; Liver ; Liver Diseases ; Metabolic Diseases ; Metabolism ; Milk ; Seizures ; Vomiting ; Wills

Fifteen patients, 15 eyes with medically uncontrollable glaucomas and poor surgical prognoses underwent one-stage, single plate Molteno implatation without postoperative adjunctive systemic antifibrosis therapy. The Molteno implant procedures were successful in 12 (80%) of the 15 eyes. The visual acuity was improved in 2 eyes (13%), but worsened in 2 eyes (13%). Complications included hyphema in 5 eyes (33%), choroidal detachment in 4 eyes (26%), shallow anterior chamber in 3 eyes (20%), tube-iris touch in one eye (6%).
Anterior Chamber ; Choroid ; Glaucoma* ; Humans ; Hyphema ; Prognosis ; Visual Acuity

Thyroid hormones are crucial for development of the central nervous system. Congenital hypothyroidism (CH) is the most common preventable disease resulting in mental retardation. A neonatal screening test (NST) can detect a mild form of CH that can be treated at an early age. Generally after 3 years of age, when most of the brain has matured, clinicians consider reevaluation of thyroid function for CH patients that have been identified with a normal thyroid gland at a normal position. This report presents three CH patients that developed normally, with persistent goiter despite thyroid hormone supplements. The patients’ initial thyroid-stimulating hormone (TSH) level after NST was 47, 157, and 57 mIU/L, respectively. Levothyroxine administration began at 1 or 2 months of age and was terminated after reevaluation at the age of 3, 15, and 5 years, respectively. However, 1 or 2 years later, they all resumed their medication due to increased TSH level coupled with newly developed or enlarged goiter. They all showed dual oxidase maturation factor 2 (DUOXA2) gene mutation: a homozygous mutation with DUOXA2 (c.413dupA; p.Tyr138*) in case 1, a presumed compound heterozygotic mutation with DUOXA2 (p.Tyr138*/p.Tyr246*) in case 2, and heterozygous mutations with DUOXA2 (c.738C>G; p.Tyr246*) and TPO (c.2268dupT; p.Glu757*) in case 3. When goiter persists or is newly developed despite a maintained euthyroid status, for those with transient CH history, follow-up to assess the thyroid function is recommended for at least 1 or 2 years, and genetic testing would be helpful. This study presents the first clinical cases of DUOXA2 mutation in Korea.

PURPOSE: We evaluated restoration of range of motion(ROM) after manipulation under intravenous anesthesia in patients whose maximum flexion was less than 90 degrees at 2 weeks after total knee arthroplasty(TKA). MATERIALS AND METHODS: 136 knees of 108 patients who received TKA between January 1996 and December 2000 and followed up for more than 2 years were analyzed. The forceful manipulation underwent for 16 knees because whose maximum flexion was less than 90 degrees after 2 weeks of TKA. Manipulation were done for 12 of 16 cases at 2 weeks after TKA and 4 cases after more than 3 months. RESULTS: 12 knees which were manipulated 2 weeks after TKA had a mean flexion 103+/-10.2 degrees , 111+/-9.6 degrees and 116+/-11.4 degrees at immediate post-operation, 3 months and 2 years after TKA. In 4 knees manipulated more than 3 months after TKA, a mean flexion was 100+/-10.4 degrees , 102+/-11.5 degrees and 96+/-11.4 degrees at immediate post-operation, 3 months after manipulation and 2 years after TKA. 120 knees with no manipulation had a mean flexion 115+/-13.7 degrees at last follow-up. There was no difference of a mean flexion at last follow-up between group manipulation within 2weeks after TKA and group with no manipulation. CONCLUSION: Manipulation under intravenous anesthesia performed 2 weeks after TKA if patients had less than 90 degrees of flexion increased flexion of the operated knee without any serious complication.
Anesthesia, Intravenous ; Arthroplasty* ; Follow-Up Studies ; Humans ; Knee*

OBJECTIVE: The purpose of our study is to evaluate the success rate and feasibility of intraoperative neuromonitoring (IONM) focusing on transcranial motor evoked potential (TcMEP) monitoring for patients with preoperative motor weakness in spine surgery. METHODS: Between November 2011 and December 2013, TcMEP and somatosensory evoked potential (SSEP) monitoring were attempted in 130 consecutive patients undergoing spine surgeries for cervical or thoracic cord lesions. Patients ranged in age from 14 to 81 years (mean±standard deviation, 56.7±14.8 years), and 84 patients were male. The success rates of both SSEP and MEPs monitoring were assessed according to the preoperative Medical Research Council (MRC) and Nurick grades. RESULTS: TcMEP was recorded successfully in 0%, 28.6%, 72.3%, and 100% of patients with MRC grades 1, 2, 3, 4, and 5, respectively. SSEP was obtained from 0%, 37.5%, 21.5%, 61.4%, and 85.4% of patients with MRC grades 1, 2, 3, 4, and 5, respectively. TcMEP was recorded successfully in 84% of patients with Nurick grades 1-3 and 26% of patients with Nurick grades 4-5. SSEPs were recorded successfully in 76.3% of patients with Nurick grades 1-3 and 24% of patients with grades 4-5. CONCLUSION: IONM during spine surgery may be useless in patients with MRC grades 1-2, applicable MRC grade 3, and useful MRC grades 4-5. MRC grade 3 is a critical point of indication for application of MEPs. In unmonitorable cases with MRC grade 3, increasing stimulus intensity or facilitation techniques may be considered to improve the usefulness of TcMEP.
Evoked Potentials, Motor ; Evoked Potentials, Somatosensory ; Humans ; Male ; Spine*

PURPOSE: Bladder irritative symptoms such as urinary urgency or urge incontinence could be worse even after proper treatments, if accompanying the unstable bladder with lower urinary tract obstruction or female urinary incontinence was not found before treatments. We performed walking cystometrography to detect unstable bladder which could be masked by cystometrography in sitting position. MATERIALS AND METHODS: We evaluated 133 patients who complained of urinary urgency or urge incontinence. Walking cystometrography (Group 2) was done for pa tients who did not show unstable bladder at cystometrography in sitting position (Group 1). Bladder volume at first desire to void, cystometric maximal bladder capacity, and occurrence of unstable bladder of both groups were compared. RESULTS: In cystometrography performed in sitting position (Group 1), unstable bladder was found in 48 (36.1%) out of 133 patients. Among the rest 85 patients, 23 patients (27.1%) showed unstable bladder in walking cystometrography (Group 2). Conse quently, 71 (53.4%) out of 133 patients showed unstable bladder in both groups. Com paring the bladder capacity of these two measuring methods, we could observe that bladder volume at first desire to void and maximal bladder capacity of walking state were smaller than those of sitting position from 187.4 +/- 36.9ml (Group 1) to 138.5 +/- 31.6ml (Group 2), 413.6 +/- 42.5ml (Group 1) to 342.8 +/- 43.2ml (Group 2), respectively (p <0.05). CONCLUSIONS: We expect that the diagnosis of unstable bladder through walking cysto metrography is meaningful when the method is applied to the patients who have urinary urgency or urge incontinence but seem to be normal by conventional cystometrography.
Diagnosis* ; Female ; Humans ; Masks ; Urinary Bladder ; Urinary Incontinence ; Urinary Incontinence, Urge* ; Urinary Tract ; Walking*

A case of massive transvaginal eversion of urinary bladder through a large vesicovaginal fistula is reported. It has developed by spillage of hyprochloride on prolapse uteri and accompanied with rectovaginal fistula. Satisfactory surgical repair of the fistula was obtained by reduction of bladder and urethral reconstruction using anterior bladder wall.
Fistula ; Prolapse ; Rectovaginal Fistula ; Urinary Bladder* ; Uterus ; Vesicovaginal Fistula

In 1991, the Ministry of Health & Social affairs adopted a nationwide service program for neonatal screening of phenylketonuria, galactosemia, maple syrup urine disease, homocystinuria, histidinemia & congenital hypothyroidism for newborns delivered from low class pregnant women registered in health centers. Government decreased the test items from six to two, PKU & congenital hypothyroidism to increase test numbers with same budget from 1995. Government decided to test PKU & hypothyroidism for all newborns from 1997. 78 laboratories wanted to participate for neonatal screening test in 1999. Government didn't decide laboratory center for a certain district and placed responsibility on free competition. Government are planning to test 573,000 newborns from 1998, Government decided to screen 6 items PKU, congenital hypothyroidism, maple syrup urine disese, homocystinuria, galactosemia and congenital adrenal hyperplasia from 2006. 17 laboratores are participating now. The cost of screening test is supported by both the federal government and local government on a 40-60 basis. In case a patient with an inherited metabolic disease is diagnosed by screening of government program, special milk is provided at government's expense. Interlaboratory quality control was started 6 times a year from 1994. According to the government project, 3,707,773 newborns were screened. 86 PKU, 718 congenital hypothyroidism were detected. So incidence of PKU is 1/43,114 and congenital hypothyroidism is 1/4,612. Maeil dairy company produced new special formula for PKU, MMA and PA, MSUD, urea cycle disorder, homocystinuria, isovaleric acidemia from Oct. 1999. The cost benefit of performing screening procedures coupled with treatment has been estimated to be as high as 1.77 times in PKU, 11.11 times in congenital hypothyroidism than cost without screening. We are trying to increase the budget to test all newborns for Tandem mass sereening & Wilson disease from 2008. Now it is a very important problem to decrease laboratory numbers of neonatal screening in Korea. So we are considering 4-5 central laboratories which cover all newborns and are equipped with tandem mass spectrometer & enzyme immunoassay for TSH, 17OHP & enzyme colorimetric assay for galactose.
Acer ; Adrenal Hyperplasia, Congenital ; Budgets ; Congenital Hypothyroidism ; Cost-Benefit Analysis ; Federal Government ; Female ; Galactose ; Galactosemias ; Government Programs ; Hepatolenticular Degeneration ; Homocystinuria ; Humans ; Hypothyroidism ; Immunoenzyme Techniques ; Incidence ; Infant, Newborn* ; Korea* ; Local Government ; Maple Syrup Urine Disease ; Mass Screening* ; Metabolic Diseases* ; Milk ; Neonatal Screening ; Phenylketonurias ; Pregnant Women ; Quality Control ; Urea Cycle Disorders, Inborn