Journal of the Korean Academy of Child and Adolescent Psychiatry
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v.4
no.1
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pp.173-178
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1993
Narcolepsy's age at onset is reported to be relatively homogeneous, occuring usually after the onset of puberty, although most cases are diagnosed when the patients are in their late teens to late 20s. It is very unusual for a patient to develop narcolepsy before 15 years of age or after 30 years of age. A 11-year old boy who has developed excessive daytime sleepiness since age of 7 and has all the four major features of narcolepsy by the time of evaluation is presented. On polysomnographic examination, the patient showed two sleep onset REM periods in the three latency test of the multiple sleep latency test and the nocturnal polysomnogram. In addition, the findings of typing HLA class I and II of the patient's family are presented. Reports of pediatric narcolepsy previously reported are reviewed.
Adenotonsillar hypertrophy is the leading cause of childhood obstructive sleep apnea. Obstructive sleep apnea syndrome in childhood, however, can occur from various causes such as obesity or craniofacial abnormalities. Childhood obstructive sleep apnea syndrome can be accompanied by enuresis, parasomnias and behavior problems. For patients with the symptoms of snoring and apnea, obstructive sleep apnea should be suspected and diagnosed properly. In addition, the evaluation of complications and proper treatment are indispensable. When the cause of childhood obstructive sleep apnea is adenotonsillar hypertrophy, symptoms can be improved by surgical methods. If the cause is other than adenotonsillar hypertrophy, such as obesity, it should be treated with other therapeutic modalities, like nasal continuous positive airway pressure (nCPAP), weight reduction and modification of life style. This paper reports a case of nCPAP used to manage severe sleep apnea when it was not resolved after adenoidectomy and tonsillectomy. Differential diagnosis of narcolepsy in a case with excessive daytime sleepiness and reflections on accompanying enuresis and parasomnia were also described.
Mycoplasma pneumoniae (MP) is the most common causative agent of community-acquired pneumonia in school-aged children. An 8-year-old boy who had been diagnosed with autism looked severely ill when he presented to our hospital due to dyspnea and lethargy. He had fever and cough 7 days prior to hospitalization. He had signs and symptoms of severe respiratory distress. The percutaneous oxygen saturation was 88% at high oxygen supply. Chest radiography showed diffusely increased opacity with moderate pleural effusion. He was intubated immediately and admitted to the intensive care unit. Under the clinical impression of mycoplasmal pneumonia, intravenous clarithromycin was started. Laboratory findings showed leukocytosis, hepatitis, decreased renal function, and presence of serum MP immunoglobulin (Ig) M (+) IgG (+) and sputum MP polymerase chain reaction (+). On hospital day 2, the patient developed multiple organ failure with acute respiratory distress syndrome (ARDS). Veno-venous extracorporeal membrane oxygenation (ECMO) was performed with continuous renal replacement therapy (CRRT) and was weaned successfully. This is the first reported case of an ARDS due to MP infection complicated by multiple organ failure that was successfully treated with ECMO and CRRT in South Korea.
Purpose: This study was designed to investigate rotavirus infection by comparing the clinical characteristics in neonates and infants. Methods: We enrolled 104 neonates and 250 infants wiht gastroenteritis and a rotazyme test positive reaction at the Soonchunhyang University Bucheon Hospital from February 2001 to January 2003. Results: The seasonal peaks of infection in infants occurred from February to June. However, in neonates, it occurred from October to December due to nursery outbreaks. Diarrhea, vomiting, fever and convulsions were significant symptoms in infants; however, metabolic acidosis with dehydration, jaundice, irritability, apnea, bloody stool, gastric residual, grunting, poor oral intake, lethargy as well as fever and diarrhea were more common in the neonates. Upper respiratory infection, pneumonia and bronchitis were present in the infants; however, necrotizing enterocolitis was more commonly observed in the in neonates. Among the patients with rotaviral infection, formula feeding was more popular than breast milk feeding in both the neonates and infants; however, this finding was not statistically significant. Conclusion: Rotavirus can be a significant pathogen in neonates as well as infants. Neonates suffering from fever, poor oral intake, lethargy and apnea should be investigated for rotaviral infection. A new vaccine, rotaviral specific immunoglobulin and treatment guidelines are needed for eradicating rotavirus infection. Further studies on isolation, infection pathway, immune response and treatment of rotavirus are needed.
Purpose: We proposed a new classification of pediatric intussusception based on clinical and radiologic findings. Methods: Data from 88 consecutive patients with intussusception were reviewed. We retrospectively analyzed six factors; patient age, sites of intussusception, symptoms, therapeutic methods, existence of enlarged mesenteric lymph nodes, and ultrasonographic (US) findings from clinical records. Results: 1) There was one neonatal case (1.1%), the others (98.9%) were infants and children. 2) These 87 infant and child cases consisted of 14 cases (16.1%) of small bowel intussusception (SBI) and 73 cases (83.9%) of ileo-colic intussusception (ICI). Of the 14 SBI cases, 12 cases were symptomatic and 2 cases were asymptomatic. The symptomatic group comprised 8 transient cases (66.7%), 3 operative cases (25.0%), and 1 enema-reduction case (8.3%). Two asymptomatic cases were incidentally captured by computed tomography. Of the 73 ICI cases, 19 cases (26.0%) required operation, and 54 (74.0%) enema-reduction. 3) When transient SBI cases were compared with operated SBI cases, enema-reduced and operated ICI cases, the age ($38.0{\pm}22.9$ months) of transient SBI cases were significantly higher than those of the others (p=0.003). Mean mass size ($20.8{\pm}2.7mm$) in transient SBI was significantly smaller than in the others (p=0.0001). 4) No correlation was found between the existence of enlarged mesenteric lymph nodes and therapeutic method or concomitant illness. 5) Most of the target types observed by US were in transient SBI cases, the remainder were in the enema-reduced ICI cases. In terms of the doughnuts type, all 8 cases (34.8%) with an external hypoechoic rim thickness of >8.9mm were treated surgically. Conclusion: Pediatric intussusception may be classified based on clinical and radiologic findings, which are likely to indicate appropriate therapies.
Purpose : Salmonellosis including typhoid fever is still prevalent in Korea. Recently it has been reported that the incidence of salmonella gastroenteritis is increasing with a reduction of typhoid fever. We studied the clinical and laboratory features of salmonellosis. Methods : We evaluated the clinical records of 83 patients with salmonellosis who had been confirmed by stool culture from 1994 to 1999. Results : Annual incidence of salmonellosis showed an increasing tendency during recent 4 years. Seasonally, summer(45.8%) was the most prevalent, followed by fall(32.5%). Male to female ratio was 1.4 : 1. In age distribution, 64 cases(77.1%) were under 5 year-old, and 18 cases(21.7%) were younger than 1 year-old. Clinical features included diarrhea(96.4%), fever(91.6%), vomiting(49.4%), bloody stool(42.1%), abdominal pain(40.1%) and tenesmus(12.0%). In serogroups, there was no group A and group B, group C, group D(including 2 cases of S.typhi) and group E were in 41.0%(34 cases), 3.6%(3 csaes), 51.8%(43cases) and 3.6%(3 cases), respectively. In Widal test, 5 cases(13.5%) and 1 case(2.7%) of group B, C and E(total 37 cases) were observed the O titer above 1 : 80 and 1 : 320, respectively. However, in 36 cases of group D, 19 cases(52.7%) and 9 cases(25.0%) were above the O titer 1 : 80 and 1 : 320, respectively. Antibiotics resistant rates to ampicillin, trimethoprim/sulfamethoxazole and chloramphenicol were 23.2%, 10.1% and 51.4%, respectively. Conclusion : Salmonellosis has become a common cause of gastroenteritis in children, especially under the pre-school age. Isolation of salmonella is neccessary for accurate diagnosis of gastroenteritis and typhoid fever. A careful attention of the use of antibiotics is needed to reduce the muilti-drug resistant strains.
Purpose : Methylmalonic aciduria (MMA) and propionic aciduria (PA) are inborn errors in the catabolism of branched-chain amino acids. The study was undertaken to investigate the genotypes and clinical features of Korean patients with MMA and PA. Methods : This study examined 12 patients with MMA and eight with PA. We analyzed various clinical features, laboratory findings, treatments, and neuro-developmental outcomes. Diagnoses were based on the presence of characteristic compounds detected by amino acid analysis in serum and organic acid analysis in urine. Mutation analysis was performed in the genes of MUT, MMAA, MMAB, and MMACHC for MMA and PCCA and PCCB for PA. Results : Among the 20 patients, six patients were diagnosed before one month of age and nine patients were diagnosed after the newborn period. Five patients were diagnosed via a neonatal screening test. Patients with early-onset forms had more severe illness at presentation and generally poor outcomes. A favorable outcome was obtained in 55% patients; most of them were of a late-onset type or diagnosed by neonatal mass screening test without symptoms. Genotypes were confirmed in all patients with MMA. We detected 11 different mutations by MUT gene analysis in 10 patients, and three different mutations in MMACHC genes in two patients. PCCA and PCCB gene mutations were identified in 14 of the 16 alleles, in eight patients with PA. Conclusion : Organic aciduria is a fatal disease; however, better outcomes are expected whenever early diagnosis and prompt management are made possible. Mutation analysis is useful for confirming diagnoses and planning management strategies.
Purpose: Following up the cases of cow's milk-sensitive enteropathy (CMSE), We observed the development of clinical tolerance with cow's milk and other foods. We investigated the clinical outcome of CMSE. Methods: We reviewed the clinical records of patients who had been admitted and diagnosed as CMSE by responses to cow's milk challenge and elimination test and the findings of small intestinal biopsy at Department of Pediatrics, Taegu Catholic University Hospital from March 1992 to March 1997. All of them were being fed with protein hydrolysate before 6 months old, and tried cow's milk and other foods challenge test at following each two month. Twenty-one cases of them returned to be followed. The age at admission was $30.7{\pm}8.8$ (18~47) days old and at survey was $43.4{\pm}23.7$ (16~84) months old. Results: 1) Although the body weight at birth of the patients was 25~75 percentile, all on admission was below 3 percentile. The body weight on interview was 25~75 percentile. 2) The development of clinical tolerance in cow's milk was observed at 16~24 months of age and the tolerance rate was 61% at 12 months of age, 90% at 16 months of age. The development of clinical tolerance in other foods was observed at 10~24 months of age and the tolerance rate was 33% at 12 months of age, 80% at 18 months of age. 3) Adverse reactions after challenge test with cow's milk were observed at 19 cases, manifestated as vomiting (31%), diarrhea (31%), irritability or lethargy (21%), skin rash (10%), and abdominal distention (5%). 4) Comparing serum IgE and milk RAST positive group on admission (5 cases) and negative group on admission (16 cases), there was no significant difference at the age of tolerance in cow's milk (p>0.05), the age of tolerance in other foods (p>0.05), allergy history in family, and the incidence of other allergic diseases. 5) The history of family allergy was observed in 3 cases (14%) in 21 patients and 3 cases (14%) showed rhinitis, urticaria or asthma through a follow-up interview. Conclusion: The development of clinical tolerance in cow's and other foods was sharply increased at 12 months of age and most of all tolerated within 24 months of age. CMSE is a temporary disorder of infancy.
Purpose : The aims of this study were to verify the incidence of hypohidrosis and to determine the predictive value of noninvasive indicator test ($Neurocheck^{TM}$) for sweating after administration of topiramate in newly diagnosed pediatric epileptic patients. Methods : A total of 46 epileptic patients (22 boys; 24 girls) on topiramate treatment were evaluated in this study at the Department of Pediatrics, Chonbuk National University Hospital, from October 2004 to July 2005. We measured sweating functions using a noninvasive sweating test ($Neurocheck^{TM}$) before topiramate medication, and after 3 months when topiramate reached its target dosage. We performed a direct questionnaire survey for the hypohidrosis related symptoms during topiramate treatment. Results : The mean age was $7.8{\pm}3.2year$. The mean dosage of topiramate was $4.5{\pm}0.8mg/kg/day$. Among the patients, there were 40 complex partial seizures, one simple partial seizure, two partial seizures with secondarily generalization, two generalized seizures, and one Lennox-Gastaut syndrome case. Of the 46 epileptic patients, 17 patients (37.0 percent) experienced hypohidrosis and hypohidrosis related symptoms, 12 (26.1 percent) had facial flushing, four (8.7 percent) had heat intolerance, one (2.2 percent) had lethargy, but no one had anhidrosis. Among the 17 patients, the mild group numbered 12 and the severe group totalled five. Hypohidrosis by $Neurocheck^{TM}$ was diagnosed in 16 patients. The overall measures of agreement between $Neurocheck^{TM}$ and the survey was 76.5 percent. The specificity of this test was 89.7 percent. Patients who showed a time delay after medication, especially over 3 minutes, were seen only in the severe group. Conclusion : $Neurocheck^{TM}$ could be clinically useful to detect and predict topiramate induced hypohidrosis in pediatric epileptic patients. We recommend that patients who show a delay over 3 minutes in $Neurocheck^{TM}$ test after topiramate initiation should be monitored for hypohydrosis.
Purpose : Cerebral edema in meningitis is a potentially complication. Hypertonic saline of various concentrations are frequently used to treat cerebral edemas in meningitis. We analyzed the safety and efficacy of osmotic therapy in cerebral edema by comparison of various hypertonic saline concentrations and mannitol. Methods : The medical records of 42 patients, who were followed up in the Department of Pediatrics, Busan Medical Center, from Jan. 2002 to Oct. 2005, were analyzed retrospectively. We measured intracranial pressure, mean flow velocity, and various laboratory parameters. Results : In cerebral edema developed in meningitis, intracranial pressure and symptoms were improved in treatment of hypertonic saline and mannitol. Serial bolus infusion of 3 percent hypertonic saline resulted in the best outcome. There was not a statistically considerable difference on the mean values of the intracranial pressure gap. On transcranial doppler, mean flow velocity was increased and pulsatilty index was decreased. Laboratory findings (osmolarity, Na, Cl, pH, lactic acid, Ca) were diffenent during the treatment period as opposed to K, Hb, bicarbonate, base excess. There was not a specific form of hypertonic saline used in meningitis treatment with cerebral edema. Conclusion : The therapy for cerebral edema in meningitis remains largely empirical. Serial bolus infusion of 3 percent hypertonic saline is better than other hypertonic salines. Various concentrations and different infusion methods of hypertonic saline statistically does not influence the result of treatment. More research aimed at improving cerebral edema treatment is needed to identify new, effective forms of treatment.
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