PURPOSE: The pharmacologic effect of atropine on HPS can be considered to control pyloric muscle spasm. Therefore, we studied the effects of intravenous atropine sulfate on the clinical course of HPS, and periodically observed the ultrasonographic appearance of the pyloric muscles after atropine treatment. METHODS:From April 1998 to May 1999, 14 infants who were diagnosed with HPS were treated with intravenous atropine sulfate. Intravenous atropine sulfate was administered at an initial dose of 0.04mg/kg/day, which was divided into 8 equal doses. The daily dose was increased by 0.01 mg/kg/day until vomiting was controlled for an entire day while infants received unrestricted oral feeding. Ultrasonographic examinations were performed during hospitalization and repeated at least every 2 months until normalization of pyloric muscles was confirmed. RESULTS: Intravenous atropine was effective in 12 of 14 infants with HPS and the conditions of 9 of them improved. Two infants who were not free from vomiting despite a week of intravenous atropine sulfate treatment underwent pyloromyotomy. A series of ultrasonographic examinations were done after vomiting had improved with intravenous atropine sulfate. The ultrasonographic findings showed good passage of gastric contents through pyloric canals despite thickening of the pyloric muscles. CONCLUSION: Intravenous administration of atropine sulfate is an effective therapy for HPS and can be an alternative to pyloromyotomy. (J Korean Pediatr Soc 2000;43:763-768)
Administration, Intravenous ; Atropine* ; Hospitalization ; Humans ; Infant ; Muscles ; Pyloric Stenosis, Hypertrophic* ; Spasm ; Vomiting

A oral inotropic agent, denopamine(TA-064, (-)-alpha-(3,4-dimethoxyphenethyl aminomethyl)-4-hydroxybenzylalcohol), was shown to have strong positive inotropic effect in experimental animals. To determine effects of denopamine on the left ventricular9LV) function and clinical features in patients with severe chronic heart failure who were treated with conventional regimens, denopamine(5mg 3 times per day for 4 weeks) was administered orally to 28 patients with chronic heart failure(22 dilated cariomyopathy, 6 ischemic heart disease) and systolic time interval, 2-D and Doppler echocardiognaphy were performed to evaluate LV function. Denopamine had no effect on LV dimension, volume and fractional shortening, and produced modest increase of ejection fraction and cardiac index, and modest decrease of PEP/LVET and cardiothoracic ratio in the cheat X-ray(PA view). The stroke volume was significantly increased (32.8+/-10.0 to 36.4+/-11.1 ml, p<0.05). Systolic BP was decreased (131.3+/-32.7 to 123.2+/-20.8 mmHg, p<0.05) with little change in diastolic BP and heart rate. Subjective symptoms and NYHA functional class were improved (71.4%, 67.9%, respectively). No adverse effect and ECG abnormality were noted. In conclusion, the addition of denopamine to conventional therapy improved clinical symptoms and caused modest increase of LV contractile function in patients with severe chronic heart failure without significant untoward effects. Therefore, the addinion of denopamine to conventional therapy may be an effective and safe method for the treatment of chronic congestive heart failure.
Animals ; Electrocardiography ; Heart Failure* ; Heart Rate ; Heart* ; Humans ; Stroke Volume ; Systole ; Ventricular Function, Left*

We report a very rare case of congenital adrenal agenesis presented with adrenal insufficiency in a 4-day-old female newborn. She was admitted with darkish skin color and seizure. Her external genitalia was normal. Elevated serum level of adrenocorticotropic hormone and increased plasma renin activity were observed. Plasma cortisol level and aldosterone level were decreased. Pelvic ultrasonography revealed bilateral agenesis of adrenal glands. Six exons of the steroidogenic factor-1 (SF-1, NR5A1) gene and their intronic flanking sequences were normal. Now, she is continuously receiving replacement doses of glucocorticoids and mineralocorticoids under adrenal insufficiency. Her growth and development are completely normal. We propose that when a patient presents with 46, XY disorder of sex development or normal female genitalia with adrenal insufficiency, SF-1 gene mutation study should be included in the differential diagnosis.
Adrenal Glands ; Adrenal Insufficiency ; Adrenocorticotropic Hormone ; Aldosterone ; Diagnosis, Differential ; Exons ; Female ; Genitalia ; Genitalia, Female ; Glucocorticoids ; Growth and Development ; Humans ; Hydrocortisone ; Infant, Newborn ; Introns ; Mineralocorticoids ; Plasma ; Renin ; Seizures ; Sexual Development ; Skin

To evaluate the influencing factors on pulmonary venous flow(PVF) pattern, we studied the relationship between PVF and left ventricular ejection fracton(EF), mitral annulus motion(MAM) and transmitral flow using pulsed doppler echocardiography in patients with dilated cardiomyopathy(DCMP), acute myocardioal infarction(AMI), left ventricular hypertrophy(LVH) and atrial fibrillation(AE). Ther results were as follows : 1) In the normal controls(13 cases), two forward flow during ventricular systole(VS) and diastole(VD) and one retrograde flow during atrial systole(AS) were observed. The peak velocity of VS, VD and AS flow was 45.9cm/s, 42.8cm/s and -18.3cm/sec, respectively. The peak VS/VD ratio was 1.1. 2) In patients with DCMP(11 cases), (a) compared to the noraml subjects, the peak velocity of VS flow and VS/VD ratio were were significantly reduced(p<0.005 and p<0.001, respectively) and were positively correlated with ejection fraction(r=0.8 and r=0.7, respectively) (b) in 2 DCMP cases with severe mitral regurgitation, systolic retrograde flow was observed in the pulmonary vein instead of forward VS flow. 3) In 12 AMI cases and 7 LVH cases with normal or slightly diminished left ventricular systolic function but with abnormal diastolic function. (a) the peak velocity of VS flow and peak VS/VD ratio were significantly increased(r<0.005 and p<0.01, respectively). (b) the peak velocity of VD flow is positively correlated with transmitral E/A ratio(r=0.8) and the peak VS/VD ratio was positively correlated with transmitral pressure half time(r=0.8). (c) the peak velocity of retrograde AS flow was significantly increased(p<0.001). (d) there was no correlation between doppler parameters of PVF and left ventricular ejection fraction. 4) In patients with atrial fibrillation(10 cases), VS flow was markedly diminished or absent and only VD flow was observed. Also, retrograde AS flow was not observed. These findings suggest that the pattern of PVF is influnced by LVEF, MAM, transmitral inflow and atrial contraction. However, main contributary factors in determining the pattern of PVF in each disease are diverse according to its main pathophysiology.
Deoxycytidine Monophosphate ; Echocardiography, Doppler, Pulsed ; Heart Diseases* ; Heart* ; Humans ; Mitral Valve Insufficiency ; Pulmonary Veins ; Stroke Volume

PURPOSE: About 10% of girls with Turner syndrome may have autoimmune thyroid disease(AIT), but the disease's pathophysiology has not yet been elucidated. Accordingly, this study was performed to observe whether the pathogenesis of AIT in children with Turner syndrome and without Turner syndrome correlate with special loci of DQ and chain in HLA. METHODS: Blood samples were drawn from children with and without Turner syndrome. Thyroid antibodies(anti-thyroglobulin and anti-microsomal antibody) were measured from the samples to determine AIT. DNAs were extracted with the DNA extraction kit and processed in PCR reaction for amplification of exon 2 region of HLA-DQA1 and -DQB1, and then eluted again. The eluted PCR products were sequenced directly with an automatic sequencer. The sequences were compared with those of normal control. RESULTS: There was a signficant increase in frequencies of HLA DQA1*0301(P<0.05) and HLA DQB1*0601 but without statistical significance(P=0.06) in normal children with AIT, compared with those in control group. There was signficantly but slightly increased frequency of HLA DQA1*0104, 0105 and DQB1*0202 in the group of children with Turner syndrome who had AIT than in control group. The frequency of the marker chromosome(45,X/46,XX+mar) increased in children with Turner syndrome who had AIT, compared with these in children with Turner syndrome who did not have AIT. Children with Turner syndrome who had spontaneous puberty had higher a incidence rate of AIT than those who did not have spontaneous puberty(P<0.01). CONCLUSION: The results suggest that HLA DQA1*0301 and HLA DQB1*0601 play a role in the pathogenesis of AIT in children without Turner syndrome, but not in children with Turner syndrome. Additionally, there seem to be other factors participating in the pathogenesis of AIT in children with Turner syndrome, such as chromosomal karyotype and spontaneous puberty. Therefore, the factors participitating in the pathogenesis of AIT in children with Turner syndrome remain to be elucidated with further study.
Adolescent ; Child* ; DNA ; Exons ; Female ; Humans ; Incidence ; Karyotype ; Polymerase Chain Reaction ; Puberty ; Thyroid Diseases* ; Thyroid Gland* ; Turner Syndrome*

No abstract available.
Humans ; Thyroid Diseases* ; Thyroid Gland*

PURPOSE: The aim of this study was to evaluate the association between birth characteristics based on birth weight for gestational age, timing of menarche and adult height in girls born small for gestational age (SGA). METHODS: Thirty subjects born from 1988 through 1999 whose birth weight below 10th percentile for their gestational ages were recruited based on hospital chart reviews. The age of each subject at menarche was recorded, and the subject's heights and bone age were assessed. RESULTS: The adult height of the girls born SGA was significantly lower than the normal adult height and slightly shorter than their target height. The adult height was associated with the birth weight for gestational age. The age at menarche was not different with the menarche timing of the general population. CONCLUSION: More attention may need to be paid to the growth outcomes of SGA patients whose birth weight is lower for their gestational age than other SGA subjects.
Adult ; Birth Weight ; Body Height ; Female ; Gestational Age ; Humans ; Menarche ; Parturition

PURPOSE: The aim of this study was to evaluate the association between birth characteristics based on birth weight for gestational age, timing of menarche and adult height in girls born small for gestational age (SGA). METHODS: Thirty subjects born from 1988 through 1999 whose birth weight below 10th percentile for their gestational ages were recruited based on hospital chart reviews. The age of each subject at menarche was recorded, and the subject's heights and bone age were assessed. RESULTS: The adult height of the girls born SGA was significantly lower than the normal adult height and slightly shorter than their target height. The adult height was associated with the birth weight for gestational age. The age at menarche was not different with the menarche timing of the general population. CONCLUSION: More attention may need to be paid to the growth outcomes of SGA patients whose birth weight is lower for their gestational age than other SGA subjects.
Adult ; Birth Weight ; Body Height ; Female ; Gestational Age ; Humans ; Menarche ; Parturition

BACKGROUND: The automation system for blood typing and antibody screening has been developed and is now used widely. In this study, we evaluated the economic effectiveness between automation system QWALYS-3 (DIAGAST, Loos Cedex, France) and manual testing. METHODS: Clinical samples from March 2012 were used for comparison of the costs and TAT for ABO-RhD blood typing and antibody screening. The costs included those of materials (reagents and consumables), labor, and equipment depreciation. TAT was analyzed for either blood typing only for one, 16, and 32 samples or blood typing and antibody screening for the same number of samples. RESULTS: The blood typing TAT for one, 16, and 32 samples was 4.5, 35.1, and 70.1 minutes by manual and 24.0, 36.0, and 38.1 minutes by automated system. Both blood typing and antibody screening TAT for one, 16, and 32 samples was 27.5, 75.0, and 129.9 minutes by manual and 45.0, 52.0, and 54.0 minutes by automation. CONCLUSION: The blood automation system reduced TAT only for the batch test, therefore, when using the automation system, blood bank test size and emergency situation should be considered.
Automation* ; Blood Banks* ; Blood Grouping and Crossmatching* ; Depreciation ; Emergencies ; Mass Screening*

Restless legs syndrome (RLS) is a neurogenic disorder with the patients having a sensation of discomfort and an urge to move continuously. These symptoms can get worse during night and cause sleep disturbance. These symptoms can be misdiagnosed as lower leg pain of a spinal origin and the treatment can be wrongly focused on this. This treatment for an unproven state of symptoms can place clinicians in a difficult situation. We experienced RLS associated with spondylolisthesis and spinal stenosis, and we originally misdiagnosed the patient and wrongly treated the patient operatively with spinal fusion and posterior instrumentation. After an insufficient result, we diagnosed the patient with having RLS with the help of the neurology department and rehabilitation medical department. In one other case we diagnosed a RLS patient with the help of a neurologist and the patients had arrived an our department for total knee arthroplasty and spinal root block. We report on these cases so other orthopedic surgeons will not make same mistakes.
Arthroplasty ; Humans ; Hypogonadism ; Knee ; Leg ; Mitochondrial Diseases ; Neurology ; Ophthalmoplegia ; Orthopedics ; Restless Legs Syndrome ; Sensation ; Spinal Fusion ; Spinal Nerve Roots ; Spinal Stenosis ; Spondylolisthesis