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

We describe a case of fine needle aspiration cytology of metastatic nasopharyngeal carcinoma in the lymph node of the neck presenting a predominantly spindle cell pattern. A 36 year-old male patient complained of dysesthesia on the right face and a palpable neck mass. Fine needle aspiration was done on the neck mass. Tumor cells were present in syncytial groups or singly with mainly spindle shaped nuclei, vesicular chromatin, thin and regular nuclear membrane, occasional prominent nucleoli and a few foci of cellular cohesiveness. The cytoplasm was scant and pale with ill-defined borders. Mature lymphocytes were present in the background of aspirates and within the tumor cell clusters. Histologically, the tumor of nasopharynx showed several areas of spindle cell pattern. Because the tumor cells showed a predominantly spindle shape with vesicular nuclear chromatin, the differential diagnosis of spindle cell sarcoma or granuloma of epithelioid cells were considered, but the characteristic morphology of the nuclei with vesicular chromatin and prominent nucleoli, and cellular cohesiveness were important in making the diagnosis of nasopharyngeal carcinoma. The possibility of metastatic carcinoma should always be considered in fine needle aspiration cytology of the lymph node in the neck because the incidence of metastatic carcinoma, particularly of the nasopharyngeal carcinoma in the lymph nodes of the neck is relatively high.
Child ; Male ; Female ; Humans ; Diagnosis, Differential ; Incidence ; Neoplasm Metastasis

It has been known that childhood bipolar disorder is rare, especially combined with attention deficit hyperactivity disorder (ADHD). But, recent studies reported that childhood bipolar is more common than our guess. Many symptoms of childhood mania overlapped with ADHD, so it is difficult to differentiate from ADHD. It also often reported that psychostimulants, medication for ADHD, induce manic symptoms. Clinically, childhood mania has several characteristics. It is frequently chronic, mixed type, rapid cycling and often refractory to treatment. So it is important to diagnose, differentiate, treat childhood bipolar disorder and ADHD, but there is no report about comorbidity, treatment, clinical characteristics about that. We report a patient combined ADHD, mental retardation and childhood bipolar disorder.
Attention Deficit Disorder with Hyperactivity* ; Bipolar Disorder* ; Child* ; Comorbidity ; Humans ; Intellectual Disability*

Herpes simplex esophagitis can occur in those with normal immune function, but is more often seen in those who are immunocompromised. In one series, 5 percent of post-kidney transplant recipients had herpes esophagitis. We experienced a case of herpes simplex esophagitis, following renal transplantation in a 9 year old male. He complained of epigastric pain, nausea and blood-tinged vomiting. Endoscopic examination showed volcano ulcer, mucosal friability and multiple confluent ulcers covered by whitish exudates on elevated margin in the middle and lower esophagus. Microscopic findings revealed multinucleated giant cells, margination of chromatin, intense nonspecific inflammation and strong positive for herpes simplex virus immunohistochemical staining. Esophageal lesions and symptoms improved after acyclovir therapy.
Acyclovir ; Child* ; Chromatin ; Esophagitis ; Esophagus ; Exudates and Transudates ; Giant Cells ; Herpes Simplex* ; Humans ; Inflammation ; Kidney Transplantation ; Male ; Nausea ; Simplexvirus* ; Transplantation ; Ulcer ; Vomiting

We present a family of FSP with review of literatures. Seeligmuller (1876) and Strumpell (1880) were the first to describe familial case of spastic paraplegia characterized by progressive weakness and spasticity of the lower limbs with little or no involvement of the upper extremities. This syndrome is heterogenous in inheritance, age of onset, severity and associated signs. A pure type without complications has been named "Strumpell's FSP". We have recently experienced a case of 17 years old male who had bilateral weakness and stiffness of lower extremities, and gait disturbance with family history. Differential diagnosis, particularly with familial amyotrophic lateral sclerosis, is discussed.
Adolescent ; Age of Onset ; Amyotrophic Lateral Sclerosis ; Diagnosis, Differential ; Gait ; Humans ; Lower Extremity ; Male ; Muscle Spasticity* ; Paraplegia* ; Upper Extremity ; Wills

PURPOSE: To investigate the relation of the gastric epithelial cell proliferation, apoptosis and genotypes of H. pylori in children. METHODS: Histologic grading by updated Sydney system, PCNA immunostaining, TUNEL method and the genotypes (cagA, picB and iceA) by PCR were performed in H. pylori positive (N=20) and negative (N=20) gastric biopsy specimens. RESULTS: PCNA index was significantly different between H. pylori positive children (77.4+/-13.12) and H. pylori negative children (52.3+/-12.20) (p=0.000). There were positive correlations between PCNA index and H. pylori density (r=0.624, p=0.000), polymorphonuclear neutrophil activity (r=0.460, p=0.005) and chronic inflammation (r=0.433, p=0.009). Apoptosis index of H. pylori positive children (0.70+/-0.411) was significantly higher than of H. pylori negative children (0.14+/-0.201) (p=0.000). Positive correlations between apoptosis index and H. pylori density (r=0.691, p=0.000), polymorphonuclear neutrophil activity (r=0.585, p=0.000) and chronic inflammation (r=0.535, p=0.001) were noted. As PCNA index increased, apoptosis index significantly increased (r=0.527, p=0.001). The positive rates of genotypes were cagA 90%, picB 75%, iceA1 60% and iceA2 15%, respectively. There were no significant correlations between the status of the genotypes and PCNA index, apoptosis index, the endoscopic findings and the histologic findings. CONCLUSION: PCNA index and apoptosis index in H. pylori positive children were higher than in H. pylori negative children but were not related to H. pylori genotypes. This study suggested that correlatively increased gastric epithelial cell proliferation and apoptosis are important to pathogenesis of H. pylori infection in children.
Apoptosis* ; Biopsy ; Child* ; Epithelial Cells* ; Genotype* ; Helicobacter pylori* ; Helicobacter* ; Humans ; In Situ Nick-End Labeling ; Inflammation ; Neutrophils ; Polymerase Chain Reaction ; Proliferating Cell Nuclear Antigen

PURPOSE: The purpose of this study is to detect viral coproantigens in children who were hospitalized with acute diarrhea and to compare its association with clinical symptoms. METHODS: Seventy-four stool samples were collected from children admitted to Ewha Mokdong Hospital from March 1996 to December 1999. The samples were frozen and analyzed for rotavirus, adenovirus, enterovirus, astrovirus, and calicivirus by enzyme immunoassay (EIA) with monoclonal antibody. 53 stool samples were collected from patients with diarrhea (diarrheal group) and 21 stool samples from patients hospitalized for reasons other than diarrhea (control group). Clinical features and laboratory findings were reviewed in both groups. RESULTS: Among 74 stool samples, virus antigens were detected in 60 samples. Of the 60 virus-positive stool samples, 47 enterovirus, 26 rotavirus, 16 adenovirus, 11 astrovirus, and 11 calicivirus antigens were detected by EIA. Of the 60 virus-positive stool samples, 28 samples have one viral antigen, 30 samples have 2 or more viral antigens, and 2 samples showed a simultaneous infection of Salmonella group B and enterovirus. There was no relationship between the detected virus and clinical features. CONCLUSION: In this study, viral coproantigen and clinical symptoms were not associated. In the future, further larger scale studies are necessary.
Adenoviridae ; Antigens, Viral* ; Child ; Child, Hospitalized* ; Diarrhea ; Enterovirus ; Humans ; Immunoenzyme Techniques ; Rotavirus ; Salmonella

BACKGROUND: Quinidine appeared to increase serum digoxin levels when given with quinidine. Therefore elevated serum digoxin concentrations and clinical toxicity have been reported in patient receiving quinidine. Currently, Bayesian method which estimates the most probable parameters of the drug for each patient from population parameters data is useful approach for adjusting digoxin dosage. To increase the accuracy of Bayesian method, it is desirable to use population parameters of Korean. Therefore we evaluated the effect of quinidine on digoxin clearance in Korea. METHOD: Patient's records from 19 adult cardiac disease without CHF having normal renal and liver function from Seoul National University of Hospital respectively wre evaluated. Digoxin pharmacokinetic parameters, CL and Vd, were obtained from serum concentration of digoxin of single and combined therapy at each steady-state by using bayesian method. RESULTS: This study show that quinidine reduced the total body clearance of digoxin from 2.39+/-0.17 to 1.51+/-0.08ml/min/kg(p<0.05) and reduced the digoxin volume of distribution from 8.57+/-0.29 to 4.98+/-0.19L/kg(p<0.05). This results show that digoxin dosage reduced to 40-50% in Korean, if quinidine therapy is initiated.
Adult ; Bayes Theorem ; Digoxin* ; Drug Interactions ; Heart Diseases ; Humans ; Korea ; Liver ; Pharmacokinetics ; Quinidine* ; Seoul

To determine the perinatal mortality and morbidity of fetuses with absent end-diastolic velocities (AEDV) of the umbilical artery in severe pre-eclampsia and eclampsia, the outcome of 5 fetuses with AEDV was compared with that of 35 fetuses with positive end-diastolic velocities (PEDV). The study population comprised 38 cases of severe pre-eclampsia and 2 cases of eclampsia with structurally normal singletons, who had had umbilical artery Doppler velocimetry weekly from admission to delivery. The Doppler velocimetry result was not used for the clinical management. Perinatal death and neonatal morbidity from both groups were further examined in gestational age category to control the influence of preterm births. The incidence of AEDV of the umbilical artery Doppler velocimetry in severe pre-eclampsia and eclampsia was 12.5% (5/40). The AEDV group had a significantly higher incidence than the PEDV group in terms of ceasarean section due to fetal distress (60% : 17%), Apgar score < 7 at 5 minutes (60% : 14%), perinatal death (25% : 0%) and assisted mechanical ventilation (67% : 9%) both at 32-36 weeks. Time intervals from the detection of AEDV to delivery of live neonates varied from the day to 15 days. In conclusion, AEDV in the umbilical artery might be of clinical value in routine surveillance of pregnancies complicated by severe pre-eclampsia and eclampsia, and predict hypoxic fetal condition which needs operative interventions before or during labor and mechanical ventilation after birth.
Apgar Score ; Cesarean Section ; Dystocia ; Eclampsia* ; Female ; Fetal Distress ; Fetus ; Gestational Age ; Humans ; Incidence ; Infant, Newborn ; Maternal Death ; Mortality ; Parturition ; Parturition* ; Perinatal Mortality ; Pre-Eclampsia* ; Pregnancy ; Premature Birth ; Respiration, Artificial ; Rheology ; Trial of Labor ; Umbilical Arteries* ; Uterine Rupture

Urinary cytology has become an essential element in the diagnosis and management of transitional cell carcinoma(TCC) of the urinary tract. It has the advantage of being noninvasive, inexpensive, and easily accessible. Besides that it can even detect malignancy when unsuspected at cystoscopy. We report a retrospective review of urine cytology in the diagnosis of 83 TCC cases that underwent 295 cytologic evaluation. All patients had biopsy-proven TCC of the bladder, ureter and renal pelvis. The overall inci- dence of the positive cytology cases was 66.2%. To define the cytologic features of tumor cells, we tried to use three cytologic gradings such as "grade 1", "grade 2", and "grade 3" according to the cytologic degree of anaplastic neoplastic cells. These cytologic gades of TCC were relatively well correlated with the histologic grade and tumor invasiveness. This result suggests that the recognition of characteristic cellular features of TCC can suspect the histologic grade and tumor stage. The false negative TCC cases were 78.9%. They showed severe inflammatory or bloody background and a few neoplastic cells. Therefore, a cautious approach for accurate interpretation, personal experience, and proper fixation and processing could expand the role of urinary cytology.
Cystoscopy ; Diagnosis ; Humans ; Kidney Pelvis ; Nocturnal Enuresis* ; Retrospective Studies ; Risk Factors* ; Thyroid Gland ; Ureter ; Urinary Bladder ; Urinary Tract