Pseudohypoaldosteronism type 1 is a genetic renal tubular disease of salt wasting, presenting in young infants. Tubular unresponsiveness to elevated endogenous and exogenous aldosterone is the suggested pathogenetic mechanism. Oral sodium chloride supplementation relieve the clinical symptoms and electrolyte distrubances. We experienced 2 cases of PHA type 1 in 38-day and 45-day old male infants who were presented with failure to thrive, vomiting and/or dehydration. Laboratory data showed hyponatremia, hyperkalemia, hypochloremia and metabolic acidosis. Renal and adrenal functions were normal. Plasma renin activity and plasma aldosterone concentration were markedly elevated. Under the diagnosis of pseudohypoaldosteronism type 1, oral supplementation of NaCl and/or kayexalate improved the clinical states of the patients.
Acidosis ; Aldosterone ; Dehydration ; Diagnosis ; Failure to Thrive ; Humans ; Hyperkalemia ; Hyponatremia ; Infant ; Male ; Plasma ; Pseudohypoaldosteronism* ; Renin ; Sodium Chloride ; Vomiting

BACKGROUND: Decreased heart rate variability(HRV) has been shown to be an independent predictor of poor outcome after acute myocardial infarction, Spectral analysis of HRV has recently been shown to be a reliable noninvasive test for quantiative assessment of cardiovascular autonomic regulatory response. We estimated the reproducibility and circadian rhythm of HRV parameters in patients with acute myocardial infarction. METHODS: Three consecutive 24-hour ambulatory electrocardiographic recordings were made in 24 patients with acute myocardial infarction, with 10~14 days after the acute attack, and analyzed for HRV parameters of both frequency and time domains. Parameters of frequency domain include low frequency(LF) and high frequency(HF) components. Parameters of time domain include sdRR, SDANN, SD, pNN50, rMSSD, HRV index. RESULTS: Mean total, low frequency and high frequency amplitude spectral densities were 33msec, 19msec and 11msec, respectively. Mean values of sdRR, SDANN, SD, rMSSD, pNN50 and HRV index were 103msec, 90msec, 47msec, 28msec, 7% and 32, respectively. Coefficients of variation(CV) of total amplitude spectral density, and low and high frequency components were 7%, 9% and 12%, respectively. CV of most HRV parameters of time domain were also around 10% except rMSSD and pNN50. Heart rate increased in the morning to be sustained during the day time and decreased in the night. Heart rate variability of high frequency component decreased in the early morning to be sustained during day time with increase in the night. CONCLUSIONS: We conclude that in the predischarge period of acute myocardial infarction, HRV parameters of frequency and time domains are reproducible and there are some morning fall of cardiac vagal activity.
Circadian Rhythm ; Electrocardiography ; Heart Rate* ; Heart* ; Humans ; Myocardial Infarction*

A 25-year-old man had been intermittently treated with topical steroid ointment for his eczematous lesions on the scrotum. One year later, a few hard nontender nodules were found with surrounding thickened lichenified skin on the previous lesions. Histopathologic examination of the skin revealed moderate acanthosis of the epidermis and multiple scattered calcified materials in the upper demis and within the hair follicle. We present this case, being considered a dystrophic calcinosis cutis due to local injury.
Adult ; Calcinosis* ; Epidermis ; Hair Follicle ; Humans ; Lupus Erythematosus, Discoid* ; Scrotum ; Skin

No abstract available.
Diazepam* ; Seizures, Febrile*

No abstract available.
Sympathectomy

Mandibular fractures in infants are rare. This case report describes management of a mandibular fracture in an 11-month-old infant using a microplate and screws with open reduction. The surgical treatment was successful. Because the bone fragments were displaced and only the primary incisors had erupted, conservative treatment, such as an acrylic splint and circummandibular wiring, was not recommended. Nine weeks after surgery, the microplate was removed. The results showed complete clinical and radiological bone healing with normal eruption of deciduous teeth.
Fracture Fixation ; Humans ; Incisor ; Infant ; Mandibular Fractures ; Splints ; Tooth, Deciduous

PURPOSE: The purpose of this study was to determine the utility of serum prealbumin as a biochemical marker for nutritional adequacy in neonates. METHODS: From March 1998 to May 1999, 71 fullterm (54 AGA, 9 LGA, 8 SGA) and 46 preterm neonates were enrolled. The correlations among prealbumin, albumin, birth weight and gestational age were obtained. Serum concentrations of albumin and prealbumin according to calory and protein intake were also serially measured in 30 fullterm and 29 preterm neonates on days 0, 3 and 7 of life. RESULTS: Serum prealbumin concentrations at birth were 10.2+/-2.6mg/dL in fullterm AGA, 12.1+/-3.3mg/dL in fullterm LGA, 8.3+/-1.2mg/dL in fullterm SGA and 8.8+/-2.4mg/dL in preterm neonates. Serum prealbumin concentration was significantly correlated with birth weight, gestational age and serum albumin level (P<0.01). In the neonates, prealbumin concentrations on day 3 of life were significantly lower than initial concentrations, and then they rised on the day 7 of life (P< 0.05). Serial serum prealbumin level was significantly correlated with body weight, calory intake and protein intake (P<0.01). When caloric and protein intakes were higher than 100kcal/kg/d and 2g/kg/d, respectively, there were significant differences in the changes of prealbumin concentrations on days 0, 3, and 7 of life, compared with those in neonates with lower intake. CONCLUSION: Serum prealbumin concentration could be used in the early recognition of changes in nutritional state in neonates.
Biomarkers* ; Birth Weight ; Body Weight ; Gestational Age ; Humans ; Infant, Newborn* ; Parturition ; Prealbumin* ; Serum Albumin

PURPOSE: Nebulized selective beta2-adrenoreceptor agonists have been widely used in acute asthma and selectively in acute bronchiolitis. However, nebulized salbutamol have been reported to cause arterial oxygen desaturation in some of the acute bronchiolitis and severe asthma patients. This may be the results of a paradoxical bronchoconstriction linked to acidic and hyper-osmolar nebulized salbutamol solution and etc. We assessed the changes in arterial oxygen saturation by percutaneous pulse oxymeter during and after nebulization of salbutamol solution and compared the effect of 100% oxygen with the compressed air as a driving gas. METHODS: This study was performed in 80 mild to moderate wheezy children(bronchiolitis 51, asthma 29) who were admitted to Pediatrics department of of EWHA from January 1992 to October 1993. The study children are randomly assigned to be nebulized by compressed air or 6l/min of 100% oxygen as a driving gas. The arterial oxygen saturation, wheeze score, retraction score, and heart rate were recorded before nebulization, post-nebulization, 5, 10, 15 and 30minutes. RESULTS: 1) Arterial oxygen saturation decreased significantly at post-nebulization five minutes only in bronchiolitis, treated with salbutamol nebulization without oxygen(p<0.01). Salbutamol nebulization, with oxygen (6l/min) has not changed arterial oxygen saturation both in bronchiolitis and asthma(p>0.05). 2) Wheeze score decreased significantly at post-nebulization 5-30minutes in asthma but not in bronchiolitis whether nebulized salbutamol with or without oxygen(p<0.05). 3) Retraction score increased significantly at post-nebulization 0-5minutes or unchanged in bronchiolitis but significantly decreased in asthma at 10-30minutes(p<0.05). 4) There were no significant change in heart rate and respiratory rate in both groups(p>0.05). CONCLUSIONS: Significant fall in arterial oxygen saturation was noted only in bronchiolitis treated with salbutamol nebulization without oxygen. In bronchiolitis, oxygen (6l/min) is better than compressed air as a driving gas during salbutamol nebulization to prevent hypoxemia.
Albuterol* ; Anoxia ; Asthma ; Bronchiolitis ; Bronchoconstriction ; Child* ; Compressed Air ; Heart Rate ; Humans ; Infant* ; Oxygen* ; Pediatrics ; Respiratory Rate

PURPOSE: We investigate the risk factors of recurrence after first intussusception. METHODS: We have studied the relationship of the clinical features and ultrasonographic findings and the risk of recurrent intussusception in 97 patients with reduced intussusception including 22 recurrent intussusception who admitted at the department of pediatrics at Ewha Womans University Mok-dong hospital from September 1993 to Feburary 1996. RESULTS: 1) The overall recurrence rate of intussusception in this study was 16.9%. 2) The interval between the first and second attack was within 24 hours in 53.8%. 3) There were no stastistically significant difference in age and sex, antecedent illness, clinical symptoms and sings, type of intussusception and radiologic findings on simple abdomen. 4) There were stastistically significant difference in the average duration of symptom above 12 hours, infolding lymph node in target sign, outer hypoechoic diameter above 8mm, swelling on ileocecal valve and terminal ileum after reduction(wall thickness> 3mm) on ultrasonographic findings and number of trial of reduction more than 3 times between the nonrecurrent and recurrent groups. 5) In stepwise logistic regression analysis, hypoechoic outer rim thickness above 8mm in target sign, swelling of ileocecal valve and terminal ileum after reduction on ultrasonographic findings and number of trial of reduction more than 3 times contributed significantly to the prediction of recurrence of intussusception. CONCLUSIONS: Recurrent intussusception can be predicted by hypoechoic outer rim thickness above 8mm in target sign, swelling of ileocecal valve and terminal ileum after reduction on ultrasonographic findings and number of trial of reduction more than 3 times.
Abdomen ; Female ; Humans ; Ileocecal Valve ; Ileum ; Intussusception* ; Logistic Models ; Lymph Nodes ; Pediatrics ; Recurrence ; Risk Factors* ; Ultrasonography*

Treating diseases in the field of neurosurgery has progressed concomitantly with technical advances. Here, as a surgical armamentarium for the treatment of cerebral aneurysms, the history and present status of the keyhole approach and the use of neuroendoscopy are reviewed, including our clinical data. The major significance of keyhole approach is to expose an essential space toward a target, and to minimize brain exposure and retraction. Among several kinds of keyhole approaches, representative keyhole approaches for anterior circulation aneurysms include superciliary and lateral supraorbital, frontolateral, mini-pterional and mini-interhemispheric approaches. Because only a fixed and limited approach angle toward a target is permitted via the keyhole, however, specialized surgical devices and preoperative planning are very important. Neuroendoscopy has helped to widen the indications of keyhole approaches because it can supply illumination and visualization of structures beyond the straight line of microscopic view. In addition, endoscopic indocyanine green fluorescence angiography is useful to detect and correct any compromise of the perforators and parent arteries, and incomplete clipping. The authors think that keyhole approach and neuroendoscopy are just an intermediate step and robotic neurosurgery would be realized in the near future.
Aneurysm ; Arteries ; Brain ; Fluorescein Angiography ; Humans ; Indocyanine Green ; Intracranial Aneurysm* ; Lighting ; Neuroendoscopy* ; Neurosurgery ; Parents