Presentation of a Mullerian Duct Cyst in a Male Child with Prolonged Fever

The Mullerian duct cyst (MDC) occurs due to incomplete focal regression and saccular dilatation of the paramesonephric duct. It is usually found asymptomatic in adults, but it also shows symptoms such as ejaculatory impairment, hematospermia, pelvic pain, urinary retention, and urinary tract infections. MDCs are rare in children. Transrectal ultrasonography, computed tomography, and magnetic resonance imaging are useful for diagnosis. Treatment is based on age, clinical symptoms as well as size and location of the cyst. Here, we report a case of a 19-month-old boy with an infectious MDC that presented with fever. He has improved with antibiotic treatment, not surgical resection.

Presentation of a Mullerian Duct Cyst in a Male Child with Prolonged Fever

The Mullerian duct cyst (MDC) occurs due to incomplete focal regression and saccular dilatation of the paramesonephric duct. It is usually found asymptomatic in adults, but it also shows symptoms such as ejaculatory impairment, hematospermia, pelvic pain, urinary retention, and urinary tract infections. MDCs are rare in children. Transrectal ultrasonography, computed tomography, and magnetic resonance imaging are useful for diagnosis. Treatment is based on age, clinical symptoms as well as size and location of the cyst. Here, we report a case of a 19-month-old boy with an infectious MDC that presented with fever. He has improved with antibiotic treatment, not surgical resection.

The relationship between serum adiponectin and inflammatory cytokines in obese Korean juveniles / 소아과

PURPOSE: Obesity is related to systemic inflammatory processes causing cardiovascular complications. Intercellular adhesion molecule-1 (ICAM-1), CD40 ligand (CD40L), P-selectin are newly described mediators of inflammation and have a significant effect in atherosclerosis. Adiponectin has shown anti-inflammatory effects in adults. The aim of this study was to evaluate the relationship between adiponectin and inflammatory mediators in children and adolescents. METHODS: Fifty children or adolescents, twenty two with a body mass index (BMI) over 95th percentile, and twenty eight with a BMI below 75th percentile were included in the study. Serum soluble ICAM-1 (sICAM-1), P-selectin, CD40L, lipid profiles, aspartate aminotransferase, alanine aminotransferase, glucose and insulin were measured to evaluate associations with adiponectin. Comparison of these variables was performed between the obese and the nonobese group. RESULTS: We found a adiponectin to be significant lower and sICAM-1 significant higher in the obese group compared to the nonobese group, but there were no significant differences in P-selectin and soluble CD40L. Adiponectin was negatively associated with ICAM-1 and P-selectin in the obese group. CONCLUSION: Negative associations of adiponectin with ICAM-1 and P-selectin in obese children and adolescents suggest that serum adiponectin level may represent the inflammatory status.

Effectiveness and Tolerability of Fosphenytoin for Pediatric Status Epilepticus

PURPOSE: The study was aimed to investigate the effectiveness and tolerability of intravenous fosphenytoin (fPHT) in the treatment of pediatric status epilepticus (SE). METHODS: Medical-records of patients who received intravenous (IV) fPHT for the treatment of SE were retrospectively reviewed and their clinical data were analyzed regarding age, gender, seizure types, underlying neurologic status, use of other anticonvulsants, loading dose, response and adverse events of IV fPHT. RESULTS: Twenty patients (12 boys and 8 girls) were included in the study. The mean age at administration of IV fPHT was 3.98 years (range 0-18.6 years). Of the 20 patients, 15 patients had no underlying neurological conditions, but five patients were on anticonvulsants. IV fPHT terminated the seizures in 15 of the 20 patients (75%). No adverse events occurred during or after the infusions. CONCLUSION: This study demonstrated that IV fPHT was as effective as phenytoin and was well-tolerated in the treatment of pediatric SE. IV fPHT can be considered as a substitute for phenytoin in the management of pediatric SE.

Efficacy and Tolerability of Rufinamide for Intractable Generalized Epilepsies

PURPOSE: The purpose of the study was to evaluate the efficacy and safety of rufinamide for intractable generalized epilepsies. METHODS: Eighteen patients with intractable generalized epilepsies were included in the study. Their medical records were retrospectively reviewed. Rufinamide was administered as an add-on treatment for intractable epilepsies. The initial administered dose was 10 mg/kg/day, which was subsequently titrated up to 30-50 mg/kg/day. The effectiveness was assessed by comparing the frequency of seizures after the treatment. The difference in number of seizures during 4 weeks was compared before and after reaching the final dose. RESULTS: The study population consisted of 13 males and 5 females (mean age 13.6+/-6.2 years, range 3.3-29.2 years). The responder rate (> or =50% in seizure frequency) was 39% and the seizure free rate was 11%. Retention rate was 44% and the reasons for withdrawal was adverse events (6/18 patients, 33%), aggravation of seizures (4/18 patients, 22%), and ineffectiveness (2/18 patients, 11%). Adverse events included hyperactivity, somnolence, ataxia and polyhidrosis. Adverse events and seizure aggravation occurred even at the starting dose of rufinamide treatment. CONCLUSION: Rufinamide can be used as an efficacious and safe adjunctive anticonvulsant for patients with intractable generalized epilepsy.

Clinical Characteristics of Post-traumatic Seizures in Children

PURPOSE: Post-traumatic seizures (PTS) are well-recognized complications from head injuries and children are particularly more vulnerable to them. The aim of this study was to investigate the clinical characteristics of PTS in children and the findings of several diagnostic tools and to determine the role of prophylactic anticonvulsants. METHODS: We retrospectively reviewed the medical records of patient under 18 years of age who presented with seizures after traumatic brain injuries. Data analyzed included patient's demographics, clinical presentations, radiological and electroencephalographic findings, management and outcomes. RESULTS: Thirty one patients with PTS were included in the study and consisted of 13 males and 18 females. A mean age of the accident was 3.2 years (4 months-6.8 years) and a mean duration of follow-up was 26.0 months (12 months-54 months). Twenty one patients (67.7%) developed seizures within 24 hours after injury. Focal radiological findings were observed in 83.8% and described as subdural or epidural hematoma (25.8%), intraparenchymal hemorrhage (19.3%) and intracerebral parenchymal lesions (51.6%). Electroecephalographic findings included background abnormalities in 32.2% and interictal epileptiform discharges in 45.1%. All patients were treated with anticonvulsants for a certain period of time and a mean duration of treatment was 12.5 weeks (4-40 weeks). Eight patients (25.8%) developed subsequent seizures during follow-up period and 2 patients (6.5%) were diagnosed afterward with post-traumatic epilepsy. CONCLUSION: PTS generally take a benign clinical course, but subsequent seizures including epileptic seizures can occur in minor proportion. In these cases, radiological and electroencephalographic findings are helpful in prediction of clinical course of PTS.

A Case of Idiopathic CD4+ T-lymphocytopenia Associated with Autoimmune Hemolytic Anemia / 대한혈액학회지

Idiopathic CD4+ T-lymphocytopenia (ICL) is defined by the CDC as depressed numbers of circulating CD4+ T-lymphocytes (<300 cells/microliter or <20% of the total T cells) on more than one determination, with the absence of HIV infection and other known causes of immunodeficiency. The clinical spectrum of ICL ranges from asymptomatic laboratory abnormalities to severe opportunistic infections that mimic the clinical course of human immunodeficiency virus (HIV) infected patients. There are a few reports of ICL associated with different diseases such as Sjogren's syndrome, pulmonary sarcoidosis, Down syndrome or non-Hodgkin's lymphoma. We describe here a 5-year-old male patient with a three-year history of recurrent otitis media and pulmonary infection, and he was without any risk factors for HIV infection; this patient presented with autoimmune hemolytic anemia and was ultimately found to have idiopathic CD4+ T-lymphocytopenia.

UDP-glucuronosyltransferase 1A1 Gene Polymorphism in Severe Neonatal Hyperbilirubinemia

PURPOSE: TATA box mutation/polymorphism in the promoter region of the bilirubin uridinediphosphoglucuronate glucuronosyltransferase 1A1 (UGT-1A1) gene is known to be an etiology of hyperbilirubinemia. This study examined if a TATA box mutation/polymorphism in UGT-1A1 gene promoter could be associated with the development of severe early neonatal jaundice in Korean infants. METHODS: Thirty-nine neonatal jaundice patients and 40 controlled infants were analyzed for UGT-1A1 promoter genotypes by using DNA sequencing. RESULTS: The homozygote for (TA)7TAA mutation was not found in this study. Comparison of the prevalence of UGT-1A1 promoter (TA)7TAA heterozygotes revealed no difference between the group with jaundice and the controlled group (15.4% vs. 10%). The peak bilirubin level was higher and the onset of jaundice was earlier in the jaundice group with (TA)7TAA heterozygote compared to the jaundice group without (TA)7TAA heterozygote (23.2+/-1.0 mg/dL vs. 19.7+/-2.4 mg/dL, P=0.004, 5.0+/-1.5 days vs. 8.3+/-4.1 days, P= 0.057). CONCLUSION: The results of this study showed that TATA box polymorphism in UGT-1A1 gene promoter did not increase the prevalence of severe early neonatal jaundice in Korean infants.

Copper Deficient Anemia in Severe Protein-EnergyMalnutrition due to Child Abuse / 대한소아소화기영양학회지

An unconscious 5-year-old girl was admitted to the Intensive Care Unit. She was neglected by her parents; she suffered from inadequate nutritional, medical and emotional care. The girl appeared to be emotionally detached, dehydrated and malnourished; she had edematous extremities, moderately bruised skin and brittle coarse hair. Laboratory testing showed electrolyte imbalance, anemia, pneumonia, copper deficiency, and liver dysfunction in addition to severe protein-energy malnutrition (PEM). Medical intervention was followed by improvement of most of the symptoms. During the rehabilitation phase, the patient showed a voracious appetite and gained weight too fast. The liver became enlarged and the patient developed a mild fever due to excessive nutrition. The microcytic anemia with severe PEM did not responded to iron supplementation possibly due to the copper deficiency. Addition of copper without zinc and iron helped to improve the anemia. The patient was discharged to a childcare center where she received cognitive and psychosocial therapy.

A Study of Serum Adiponectin and Insulin Resistance in Children and Adolescents / 대한소아내분비학회지

PURPOSE: Currently many studies have confirmed the relationship between serum adiponectin and its influence on insulin resistance in adults. But physiologic insulin resistance due to changes in sex hormones exists during puberty, which prohibits us from knowing the influence of adiponectin to insulin resistance in this population. In this study, we investigated the influence of adiponectin on insulin resistance in children and adolescents. METHODS: 18 obese children, from the OPD were selected. From All subjects, blood was sampled after overnight fasting, adiponectin, lipid profiles, AST, ALT, fasting glucose and insulin levels were measured to evaluate correlations with adiponectin. The group were divided into subgroups by BMI, sex and HOMA-IR values. All variables were compared for differences and relationships. RESULTS: Obese groups showed decrease in adiponectin and showed increased insulin. HOMA-IR values were increased in the obese group. In all study participants, adiponectin showed significant correlation with triglyceride and HDL. But adiponectin showed no significant correlation with HOMA-IR, insulin and glucose. In the obese group adiponectin showed no correlation with HOMA-IR, insulin and glucose. HOMA-IR showed significant correlation with BMI, body weight and age. There were no sexual differences in adiponectin, insulin and HOMA-IR values. Insulin resistant group showed decrease in adiponectin but had no statistical significance. CONCLUSION: Decreased adiponectin levels and increased insulin resistance were observed in obese children but adiponectin showed no correlation with insulin resistance in children and adolescence. Factors other than adiponectin may play a more significant role in insulin resistance in this age group.

Usefulness of echocardiographic findings in the early diagnosis of Kawasaki disease / 소아과

PURPOSE: The early diagnosis of Kawasaki disease (KD) is difficult sometimes, especially in atypical or incomplete cases presenting 4 or less principal clinical features without coronary arterial lesions (coronary arterial ectasia or aneurysm). The authors investigated the incidence of echocardiographic abnormalities in patients with KD to discover whether abnormal echocardiographic findings might be helpful in the early diagnosis of KD. METHODS: Echocardiography was done in the acute stage of 103 patients with KD (Kawasaki group) and 40 patients with other acute febrile illnesses (control group). Abnormal echocardiographic findings were classified into 4 categories and defined as follows; 1) significant pericardial effusion, 2) significant valvular dysfunctions, 3) left ventricular systolic dysfunction, 4) coronary arterial ectasia or aneurysm. RESULTS: In the Kawasaki group, significant pericardial effusion was present in 24 patients (23.3 percent), significant valvular dysfunctions in 30 patients (29.1 percent), left ventricular systolic dysfunction in 10 patients (9.7 percent), and coronary arterial lesions in 27 patients (26.2 percent). In the control group, significant pericardial effusion was present in only one patient (2.5 percent). The number of patients with any one of echocardiographic abnormalities was 57 (55.3 percent) in Kawasaki group and one (2.5 percent) in control group. The sensitivity of echocardiography in the diagnosis of KD was 55.3 percent, the specificity 97.5 percent, the positive predictive value 98.3 percent, and the negative predictive value 45.9 percent. CONCLUSION: If abnormal echocardiographic findings, even if other than coronary arterial lesions, are confirmed in patients in whom KD is suspected, it seems desirable to inifiate specific treatment for KD.

A Case of Gastric Outlet Obstruction Complicated by Peptic Ulcer Secondary to Non-Steroidal Anti-Inflammatory Drug (NSAID) / 대한소아소화기영양학회지

Peptic ulcer disease complicated with gastric outlet obstruction is rare in children. Even though NSAIDs have been reported to cause various adverse events, they are still regarded as safe and, therefore, widely utilized in children. In the past, pediatric patients who were at risk of seizure due to high fever, were treated with dipyrone (Metamizole sodium) injection which inhibits cyclooxygenase-1 and inhibit prostaglandin, to weaken the gastro-duodenal defensive mechanism. A case of an infant with multiple esophagogatroduodenal ulcers and bleeding caused by NSAID complicated with gastric outlet obstruction is reported in this paper.

Anesthesia for Removal of Traeheobronchial Foreign Eodies in Children - Report of 54 Cases / 대한마취과학회지

Trachobronchial aspiration of foreign bodies is one cause of fatal acciderts in children. Serious complications and death of these children is avoided by early diagnocsis and early removal of foreign bodies. The purpose of this report is to analyze the alteration of anesthetic method for removal of tracheobronchial foreign bodies in 54 children(62 anesthesia) experienced in St. Mary's hospital, Catholic Medical College, seoul, Korea, From Jan. 1, 1963 to Sep. 30, 1980. The removal of tracheobronchail foreign bodies for all cases was done under general anesthesia. Results were as follows: 1) Most tracheobronchial foreign bodies appeared in the 1~3 year old group (24 cases among 54 children). 2) The kinds of foreign bodies were foods and metals. Most were peanuts and beans, which were not visible on chest X-ray films. These foreign bodies can cause atelectasis and emphysema in 24 hours, which was evidenced by chest X-ray. 3) During bronchoscopy, the chest was compressed manually after mask ventilation. Recently 1005 oxygen was inhaled through a thin plastic catheter lodged side the bronchescope. intravenous ketamine with 100% oxygen through thin catheter allowed safe anesthesia for bronchoscopic procedure. 4) Anesthetic time required for bronchoscopic procedure was 31~60 minutes on the average. 5) Pulse rate was increased in 39 cases during bronchoscopic procedure.