A Comparative Analysis of Clinical Screening Test and Language Specific Test in Language Delay Children / 전남의대학술지

The aim of this study was to investigate the usefulness of a clinical screening test [the Korean Infant and Child Developmental Test (KICDT)] compared to language specific tests: the sequenced language scale for infant (SELSI) and the Preschool Receptive-Expressive language Scale (PRES) in children with delayed language development. A retrospective chart review was conducted on 615 children who visited the Department of Pediatrics at Chonbuk National University Hospital from January 2013 to December 2016. All patients were evaluated with KICDT as a clinical screening test and SELSI or PRES as a language specific test. Language Developmental Quotients (LDQs) from the KICDT were compared with the Receptive Language Quotient (RLQ) and expressive language quotient (ELQ) from the SELSI or PRES. The sensitivity, specificity and predictive values of LDQ of KICDT were calculated by comparing with SELSI/PRES. Language DQs from the KICDT were significantly correlated with the RLQ (r=0.706), ELQ (r=0.768), and total language quotient (TLQ) (r=0.766) from the SELSI/PRES (p<0.05). In cross tabulation, the patients belonging to the retardation groups in both KICDT and SELSI/PRES were 417 (67.8%). Otherwise, patients belonging to the normal group in KICDT but not in SELSI/PRES were 151 (24.6%). Sensitivity and specificity of LDQ of KICDT relative to SELSI/PRES were 72.3% and 92.2% respectively (p<0.05). Our data suggests that clinical screening tests alone, not cumbersome language specific tests, can determine language developmental delays in children.

IgG4-Related Lung Disease without Elevation of Serum IgG4 Level: A Case Report / 결핵및호흡기질환

Since IgG4-related pancreatitis was first reported in 2001, IgG4-related disease has been identified in other organs such as salivary gland, gallbladder, thyroid, retroperitoneum and kidney; but lung invasion is rare. A 63-year-old man presented with hemoptysis at the pulmonary clinic and chest computed tomography revealed about 4.1 cm irregular shaped mass with spiculated margin at the left upper lobe. Despite no elevation of serum IgG4 level, he was finally diagnosed as IgG4-related lung disease by transthoracic needle biopsy. After treatment with oral glucocorticoids, hemoptysis disappeared and the size of lung mass was decreased.

A Case of Acute Kidney Injury in A 62 Year Old Man Who Took Atractylodes Macrocephala Koidzumi

The drug inducing tubulointerstitial nephritis is a crucial cause of acute kidney injury. Use of herbal plants in disease treatment is generally practiced in Korea. Although there are a small number of case reports, tubulointerstitial nephritis caused by taking Atractylodes macrocephala Koidzumi in human has never been reported. A 62-year-old man visited our hospital with symptoms of systemic edema accompanied by reduced urine volume and dyspnea after taking herbal plant 1 week ago. Chest radiographs showed both sided pleural effusion. Blood test results showed elevation of serum blood urea nitrogen and creatinine level and urine test results showed hematuria and proteinuria. Renal biopsy result showed evidence of tubulointerstitial nephritis by tubulitis and inflammatory cell expansion in the interstitium was observed. The patient's renal function recovered after administration of active renal replacement treatment and conservative therapy such as supplementation of fluid and electrolytes, and there was no disease recurrence for 1 year. Therefore, this case proved that Atractylodes macrocephala Koidzumi could cause acute kidney injury by the drug induced tubulointerstitial nephritis in human.

Obstructive Jaundice Caused by Tuberculous Lymphadenopathies

Although pulmonary tuberculosis is known to be the most common type in tuberculosis, it actually can affect any organ system. However, abdominal type is very rare among the extra-pulmonary types, and obstructive jaundice caused by lymphadenopathies due to tuberculosis is especially uncommon manifestation even in endemic areas. Tuberculous lymphadenopathies can mimic lymphadenopathies by other metastatic tumors or lymphoma, thus early correct diagnosis is very important for avoiding unnecessary surgical interventions. Here, we reported two cases of obstructive jaundice caused by tuberculous lymphadenopathies. Both were treated with anti-tuberculosis medications and endoscopic retrograde biliary drainage without surgery.

Agranulocytosis Induced by Ethambutol in a Patient with Pulmonary Tuberculosis / 결핵및호흡기질환

We report a case of agranulocytosis caused by ethambutol in a 79-year-old man with pulmonary tuberculosis. He was referred for fever and skin rash developed on 21th day after antituberculosis drugs (isoniazid, rifampicin, ethambutol, and pyrazinamide) intake. Complete blood count at the time of diagnosis of pulmonary tuberculosis was normal. On the seventh admission day, agranulocytosis was developed with absolute neutrophil count of 70/microL. We discontinued all antituberculosis drugs, and then treated with granulocyte colony-stimulating factor. Three days later, the number of white blood cell returned to normal. We administered isoniazid, pyrazinamide, and ethambutol in order with an interval. However, fever and skin rash developed again when adding ethambutol, so we discontinued ethambutol. After these symptoms disappeared, we added rifampicin and ethambutol in order with an interval. However after administering ethambutol, neutropenia developed, so we discontinued ethambutol again. He was cured with isoniazid, rifampicin, and pyrazinamide for 9 months.

Clinical Characteristics of Acute Transverse Myelitis in Childhood / 대한소아신경학회지

PURPOSE: We performed this study to investigate clinical and radiological features, CSF findings, clinical courses and prognostic factors of acute transverse myelitis(ATM) in pediatric patients. METHODS: A retrospective review of the medical records was performed for 15 patients with ATM under the age of 16 years from January 1997 to January 2007. RESULTS: The mean age at onset was 9.3+/-4.4 years, and the male to female ratio was 1.5:1. During the initial phase, the common presenting symptoms were back pain, abdominal pain, leg pain and weakness. Motor deficits progressed in all patients and urinary difficulty was found in 13 patients(86.7%). CSF leukocytosis was found in four patients and protein was elevated in nine patients among the 12 patients who underwent CSF study. On spine MRI, high signal intensity in T2-weighted image was mainly identified at the cervicothoracic level in 11 patients(73.3 %). According to Paines scale, 11 patients(73.3%) had normal or good outcomes. Favorable outcomes were associated with shorter duration of maximal neurologic deficits, an early recovery, and shorter interval to independent walking. In this study, we described two patients suffering from relapsing myelits without any association with a systemic disease. One patient was diagnosed as idiopathic recurrent transverse myelitis(IRTM) and the other was re-diagnosed as multiple sclerosis(MS). CONCLUSION: The prognosis of ATM in childhood is better than that reported for adults. The clinical symptoms and the extent of lesions in MRI were not correlated with outcomes. However earlier recovery was significantly associated with favorable outcomes.

Effect of Hyperbilirubinemia on the Brainstem Auditory Evoked Response in Newborn Piglets

PURPOSE: We sought to know the effect of hyperbilirubinemia on brainstem auditory evoked response in newborn piglets. METHODS: To achieve the concentration of bilirubin above 20 mg/dL, we injected a bolus of 50 mg/kg of bilirubin intravenously over 30 minutes, followed by 30-40 mg/kg/ hr of bilirubin continuous intravenous infusion to 10 newborn piglets. Brainstem auditory evoked responses were obtained from these piglets at baseline, 1 hour, 2 hours, 3 hours and 4 hours after the exposure to hyperbilirubinemia. RESULTS: The mean amplitude of wave V was 0.33+/-0.03 microV at baseline, 0.32+/-0.04 microV at 1 hour, 0.33+/-0.05 microV at 2 hours, 0.23+/-0.04 microV at 3 hours and 0.26+/-0.05 microV at 4 hours of experiment and began to decrease after 3 hours of the exposure to hyperbilirubinemia. The latency of wave III was 4.06+/-0.08 ms at baseline, 3.95+/-0.09 ms at 1 hour, 4.05+/-0.10 ms at 2 hours, 4.05+/-0.09 ms at 3 hours, 4.12+/-0.11 ms at 4 hours of experiment and began to increase after 1 hour of the exposure to hyperbilirubinemia. CONCLUSION: Hyperbilirubinemia decreased the amplitude of wave V and increased the latency of wave III of brainstem auditory evoked responses in newborn piglets.

Clinical Manifestations and Surgical Outcome of Medically Refractory Epilepsy in Childhood / 대한소아신경학회지

PURPOSE: The cognitive and psychosocial morbidity of medically refractory epilepsy is considerable. Epilepsy surgery can play a important role in these patients. We investigated the clinical manifestations and the surgical outcome of our patients with medically refractory epilepsy. METHODS: We studied 27 patients under 15 years old who had epilepsy surgery at Samsung Medical Center between March 1995 and December 2001 retrospectively. RESULTS: The median age at first unprovoked seizure was 6 years, the median age at surgery was 11.02 years and duration of follow-up was 14 months to 7 years 2 months. Complex partial seizure was the most common type of seizures. Interictal and ictal discharges were lateralized in 66.7% and 81.5%, respectively. MRI showed abnormal findings in 23 patients and the cortical dysplasia was the most common pathologic finding. As per surgical outcomes, 77.8% of the patients became seizure free. Among patients with temporal lobe epilepsy(TLE), seizure free rate was 92.3% whereas patients with extratemporal lobe epilepsy(ETLE) showed 64.3% seizure free rate. All patients with TLE with focal lesions became seizure free. Patients with focal lesions which were concordant with findings of EEG, SPECT, and PET showed excellent surgical outcomes. However, patients with no focal lesion in MRI and discordant preoperative examinations showed poor outcomes. CONCLUSION: The results of epilepsy surgery in our patients were quite satisfactory. Good surgical outcome can be expected when the decision of surgery is made based on a good correlation among clinical, neuroradiological, and other preoperative examinations.

Surgically Treated Intractable Child Epilepsy with Focal Cortical Dysplasia : Clinical and Electroencephalographic Findings

PURPOSE: Widespread use of MRI now gives us increased insights into the different expressions of cortical malformations. We carried out this study to characterize the clinical and EEG features of focal cortical dysplasia(FCD) which also caused intractable epilepsy requiring surgical treatment. METHODS: A retrospective analysis was conducted in 8 children. The history of seizures, imaging studies, electroencephalographic findings, pathologic results, and surgical outcomes were reviewed. RESULTS: The onset ages of clinical seizure were from 4 days after birth to 36 months. The fre quency of seizures were from multiple times a day to a few times a month. Five out of 8 FCD patients had global developmental delays with more degrees of delay in language. Only 2 out of 8 patients showed clinical partial seizures only. The scalp EEG disclosed localized interictal epileptiform activity in all 8 patients and localized continuous slow waves in 7 out of 8 patients. Ictal scalp EEG onset demonstrated a predominantly localized EEG seizure activity in 6 patients. Ictal invasive EEG findings were variable in each patient and the patterns of ictal EEG were demonstrated. Five out of 8 patients became seizure-free. One patient had one episode of seizure during the 16 months follow up period. Two patients did not have significant relief from seizures. CONCLUSION: The patients with FCD had varied spectrums of clinical manifestation, MRI and EEG findings. Five out of 8 FCD patients had complete relief from seizures, one patient has had one short, simple, partial seizure since surgery, but 2 patients with FCD in the frontal area did not experience significant relief from seizures.

The Clinical Features of Acute Disseminated Encephalomyelitis in Children / 대한소아신경학회지

PURPOSE: Acute disseminated encephalomyelitis(ADEM) is an immunologically mediated demyelinating disease of the central nervous system. We investigated the clinical features and outcomes of children with ADEM presenting with different neurologic symptoms and clinical courses. METHODS: Fifteen cases(male 10 and female 5) of ADEM who were diagnosed in the department of pediatrics, Samsung Medical Center from July 1997 to April 2001 were reviewed. RESULTS: The ages at initial presentations were from 1 year 5 months to 14 year 8 months. The presence of preceding events was present in all patients. The most common presenting symptom was seizure(60%), followed by altered consciousness(40%), ataxia(33 %), cranial nerve palsy, headache, quadriparesis, visual disturbance, and tremor. Brain magnetic resonance imaging showed relatively symmetrical, multifocal hyperintense lesions on T2-weighted image. One out of 4 brain CT showed an abnormal finding and remaining 3 cases showed abnormal findings in MRI only. Two patients had normal MRI findings at the initial phase of the illness but later revealed MRI findings compatible with ADEM. Thirteen patients were managed on high dose methylprednisolone therapy and/or intravenous immunoglobulin. After treatments, all patients revealed rapid clinical recovery. Relapse occurred in four patients(26%) 3 weeks to 1 year 6 months after developing their first illness. Among them, two patients were diagnosed as multiple sclerosis later. CONCLUSION: We experienced 15 children with the initial diagnosis of ADEM. Even though immunosuppressive drugs were effective to eliminate their presenting symptoms in most patients, there was 26% of relapse rate with 2 patients with later diagnosis of multiple sclerosis during long-term follow-up suggesting heterogeneity existed with regard to etiology and clinical courses of ADEM.

The Clinical Manifestations and Results of Medical and Surgical Treatment of Epileptic Children with Neuronal Migration Disorders / 대한소아신경학회지

PURPOSE: The objective of this study is to investige the clinical features, natural histories, and results of medical and surgical treatment of NMD in patients who were diagnosed during childhood. METHODS: We performed a retrospective analysis of medical records of 57 patients with NMD who were newly diagnosed by MRI or pathologically in epilepsy children since March 1993 to June 2000. RESULTS: These 57 patients with NMD consisted of 26 with cortical dysplasia, 9 with lissencephaly, 7 with polymicrogyria, 6 with schizencephaly, 4 with hemimegalencephaly, 3 with heterotopias, and 2 with double cortex. Clinically, 94.7% of these patients showed seizures, 33.3% with developmental delay, 21.1% mental retardation, 15.8% cerebral palsy, and 7.0% attention deficit hyperactivity disorder. Their response to antiepileptic drugs was good to 31 patients (75.6%), moderate to 3 (7.3%), and poor to 7 (17.1%). Twelve patients were completely seizure-free after receiving medication for at least 15 months. Seventeen patients tolerated with monotherapy with antiepileptic drugs. Fourteen patients underwent surgical resection. The results of operation were highly correlated with the complete removal of epileptic focus. Six patients who underwent complete resection were seizure-free after operation. On the other hand, Eight patients who had incomplete resection of the epileptic focus showed poor outcome. CONCLUSION: Most of previous reports suggested that NMD is associated with refractory to medical treatments, and early surgical operation has been recommended. Our study demonstrates remarkably good responses of NMD patients with medical treatment only.

Congenital Urinary Tract Anomalies Associated with Urinary Tract Infection in Infants and Children

We report 4 cases of malignant thymoma which were composed of 2 cases of invasive thymoma and 2 cases of thymic carcinoma. The cytologic findings of invasive thymoma were similar to those of benign thymoma. The distinctive cytologic features of thymic carcinoma were necrotic background, irregular clusters and individually scattered arrangement of anaplastic epithelial cells, and some scattered mature small lymphocytes. These findings may be found in the Hodgkin's lymphoma, seminoma, and metastatic squamous cell carcinoma, undifferentiated carcinoma, and large cell carcinoma of the lung. But, the feature of irregular clustering of anaplastic epithelial cell having scanty cytoplasm was different from Hodgkin's lymphoma and seminoma. Clinical and radiologic findings as well as cytologic finding were helpful in differential diagnosis of thymic carcinoma from metastatic carcinoma.

Clinical characteristics of acute viral lower respiratory tract infections in hospitalized children in Seoul, 1996-1998

This study was performed to investigate the etiologic agents, age distribution, clinical manifestations and seasonal occurrence of acute viral lower respiratory tract infections in children. We confirmed viral etiologies using nasopharyngeal aspirates in 237 patients of the ages of 15 years or younger who were hospitalized for acute lower respiratory tract infection (ALRI) from March 1996 to February 1998 at Samsung Seoul Hospital, Seoul, Korea. The overall isolation rate was 22.1%. The viral pathogens identified were adenovirus (12.7%), influenza virus type A (21.1%), -type B (13.9%), parainfluenza virus type 1 (13.5%), -type 2 (1.3%), -type 3 (16.0%) and respiratory syncytial virus (21.5%). The occurrence of ALRIs was highest in the first year of life, although parainfluenza virus type 1 infection occurred predominantly in the second year of life and influenza virus caused illnesses in all age groups. The specific viruses are frequently associated with specific clinical syndromes of ALRI. The respiratory agents and associated syndromes frequently have characteristic seasonal patterns. This study will help us to estimate the etiologic agents of ALRI, and establish a program for the prevention and treatment. An annual nationwide survey is necessary to understand the viral epidemiology associated with respiratory illnesses in Korea.

A Study on the Incidence and Risk Factors of Nosocomial Sepsis in Very Low Birth Weight Infants

PURPOSE: Although advances in neonatal intensive care have led to improved survival of very low birth weight(VLBW) infants, nosocomial sepsis continues to be an important cause of morbidity and death among these infants. Our study was carried out to estimate the incidence of nosocomial sepsis and to identify the attributable risk factors for sepsis. METHODS: The results of blood cultures taken from 182 infants with their birth weights less than 1,500 gram who were admitted to the NICU at Samsung Medical Center from October 1994 to December 1997 were retrospectively reviewed. A multiple logistic regression was performed to identify which factors were independently associated with sepsis. RESULTS: Of 166 infants who survived beyond 3 days, 57(34.3%) had nosocomial sepsis(positive blood culture at age greater than 3 days and antibiotic therapy for more than 5 days). The incidence of nosocomial sepsis was 1.8/100 hospital days and the interval between admission and onset of sepsis was 18.4+17.8 days(meanSD). Coagulase negative staphylococci(35.3%) were the most common organism in nosocomial sepsis. On multiple logistic regression analysis, several risk factors appeared to be independently associated with sepsis. The risk of nosocomial sepsis rose with decreasing gestational age, with increasing ventilator duration, UAC(umbilical venous catheter) duration, PCVC(percutanous central venous catheter) duration, nasal prong duration. And infants with nosocomial sepsis were more likely to be outborn, resuscitated at delivery room and to have bronchopulmonary dysplasia. CONCLUSION: Directing quality improvement efforts toward decreasing exposure to invasive vascular catheter and reducing ventilator days may decrease the incidence of nosocomial sepsis in VLBW infants.

Clinical Manifestations of Dysphagia in Children

PURPOSE: In children, dysphagia is a common cause of recurrent pneumonia and malnutrition and can be fatal. However, till now no studies were performed concerning the clinical investigation of dysphagia in Korea. METHODS: In this study we evaluated the clinical manifestations of dysphagia in children with an age range between 1 month and 12 years by reviewing the clinical records, and we classified the dysphagia by videofluoroesophagography. RESULTS: The etiologies of dysphagia demonstrated as follows; developmental delay in 7 patients, treacheoesophageal fistula in 2 patients, and ischemic encephalopathy, encephalitis, brain tumor, subepiglottic stenosis, bronchiolitis, and Williams syndrome in 1 patient, respectively. The presenting symptoms of dysphagia revealed as follows; choking in 7(46%) patients, nasal regurgitation in 2(13.3%) patients, hypersensitive gag reflex in 2(13.3%) patients and drooling, foreign body sensation, and feeding refusal in 1 patient, respectively. The complications of dysphagia were malnutrition in 12(80%) patients, which was followed by aspiration pneumonia in 8(53.3%) patients. On videofluoroesophagography, 5 patients showed oral phase dysphagia, Seven and two patients showed pharyngeal and esophageal phase dysphagia, respectively. Two patients presented normal deglutition on videofluoroesophagography. Total of 8 patients required gastrostomy because of recurrent aspiration and poor weight gain. In 7 patients, however, dysphagia improved with rehabilitation therapy only. CONCLUSION: Our data demonstrated that the complications of dysphagia in childhood were not uncommon, and suggested that diagnostic workup including videofluoroesophagography were helpful in classifying the feeding difficulties of these children. Gastrostomy and rehabilitation offered effective therapeutic possibilities.

Clinical Manifestations of Dysphagia in Children

PURPOSE: In children, dysphagia is a common cause of recurrent pneumonia and malnutrition and can be fatal. However, till now no studies were performed concerning the clinical investigation of dysphagia in Korea. METHODS: In this study we evaluated the clinical manifestations of dysphagia in children with an age range between 1 month and 12 years by reviewing the clinical records, and we classified the dysphagia by videofluoroesophagography. RESULTS: The etiologies of dysphagia demonstrated as follows; developmental delay in 7 patients, treacheoesophageal fistula in 2 patients, and ischemic encephalopathy, encephalitis, brain tumor, subepiglottic stenosis, bronchiolitis, and Williams syndrome in 1 patient, respectively. The presenting symptoms of dysphagia revealed as follows; choking in 7(46%) patients, nasal regurgitation in 2(13.3%) patients, hypersensitive gag reflex in 2(13.3%) patients and drooling, foreign body sensation, and feeding refusal in 1 patient, respectively. The complications of dysphagia were malnutrition in 12(80%) patients, which was followed by aspiration pneumonia in 8(53.3%) patients. On videofluoroesophagography, 5 patients showed oral phase dysphagia, Seven and two patients showed pharyngeal and esophageal phase dysphagia, respectively. Two patients presented normal deglutition on videofluoroesophagography. Total of 8 patients required gastrostomy because of recurrent aspiration and poor weight gain. In 7 patients, however, dysphagia improved with rehabilitation therapy only. CONCLUSION: Our data demonstrated that the complications of dysphagia in childhood were not uncommon, and suggested that diagnostic workup including videofluoroesophagography were helpful in classifying the feeding difficulties of these children. Gastrostomy and rehabilitation offered effective therapeutic possibilities.

Neuroradiological Findings in a Case of Menke's Disease / 대한소아신경학회지

Menkes disease is an X-linked recessively inherited neurodegenerative disorder of copper metabolism leading to death in early childhood. This disease is characterized by low serum, liver and brain Cu levels, whereas the Cu content in nearly all other organs is increased. The main clinical features are irregular kinky hair, characteristic face, progresssive degeneration of the central nervous system, bone changes, arterial rupture and thrombosis, and instability of temperature. We have experienced a case of Menkes disease in a eight months old male patient, who manifested by seizure, developmental delay, hypotonia, characteristic hair and face along with low levels of serum copper and ceruloplasmin. We report the characteristic findings of cranial MRI and MR angiography in this Menke's patient. In cranial MRI, he showed progressive brain atrophy with subdural hemorrhage and effusion. MR angiography revealed tortuosity of vessels clearly obviating necessity of more invasive conventional arteriography in diagnosis of this disease. This study suggested that cranial MRI and especially MR angiography might be helpful in making early diagnosis of this disease.

A case of Iatrogenic Esophageal Perforation in a Premature Infant

Esophageal perforation, a well-known entity in adults, is relatively uncommon in infants and children. Aggressive management in neonatal intensive care units have led to increased survival of premature infants and very sick babies. But it have led to unusual complication like iatrogenic perforation. Perforation of the esophagus or pharynx may occur during replacement of endotracheal or nasogastric tubes in the newborn infant. We recently experienced a case of esophageal perforation presenting with pneumomediastinum in premature infant, which was induced by gastric tube insertion and managed medically. We think that prevention is more important. So, careful visualization of vocal cord during intubation, gentle laryngoscopy, and the use of a soft rubber catheter for oral suction are needed.

Hemolytic-Uremic Syndrome Associated with Bloody Diarrhea

The authors analysed 2,653 cases of transthoracic fine needle aspiration cytology of the lung to evaluate the diagnostic accuracy and its limitation. A comparison was made between the original cytologic and the final histologic diagnoses on 1,149 cases from 1,074 patients. A diagnosis of malignancy was established in 38.3% benign in 48.1%, atypical lesion in 2.3%, and inadequate one in 11.9% of the cases. Statistical data on cytologic diagnoses were as follows; specificity 98.9%: sensitivity of procedure, 76.8%: sensitivity of diagnosis, 95.5%: false positive 5 cases: false negative 18 cases: predictive value for malignancy, 98.8%: predictive value for benign lesion, 79.5%: overall diagnostic efficiency, 87.5%: typing accuracy in malignant tumor, 80%.