Screening for depression and anxiety disorder in children with headache / 소아과

PURPOSE: The purpose of this study was to investigate the importance of initial screening tests for depression and anxiety disorders in children with headache. In addition, this study evaluated whether the Children's Depression Inventory (CDI) and Revised Children's Manifest Anxiety Scale (RCMAS) are suitable for screening symptoms of depression and anxiety. METHODS: A retrospective chart review was conducted of 720 children aged 7-17 years who had visited a pediatric neurology clinic for headaches and were referred to a pediatric psychiatric clinic for psychiatric symptoms from January 2010 to December 2011. All patients completed the CDI and RCMAS. Among them, charts of patients with clinically significant total scores (cutoff>15) for psychiatric symptoms, as defined by the CDI and RCMAS scoring scales, were reviewed. RESULTS: Nineteen patients had headaches and clinically significant total scores for psychiatric symptoms. The mean age at headache diagnosis was 11.7 years, and 57% were male. Mean duration of headache was 11.5 months. Two point eight percent of the patients were diagnosed with psychiatric disorders including major depression (1.7%), generalized anxiety disorder (1.1%), and bipolar disorder (0.1%). Four patients (0.6%) were diagnosed with attention deficit/hyperactivity disorder (ADHD). Total mean CDI and RCMAS scores of patients referred to the psychiatric clinic were 18.8 and 22.2, respectively. There was no correlation between CDI or RCMAS total scores and headache frequency, duration, or severity. CONCLUSION: We recommend that all patients with headache should be screened for depression and anxiety by CDI and RCMAS scores.

Lower fat and better quality diet therapy for children with pharmacoresistant epilepsy / 소아과

The ketogenic diet (KD) is an established, effective, nonpharmacologic treatment for children with pharmacoresistant epilepsy. Although the KD is the most well-established dietary therapy for epilepsy, it is too restrictive and is associated with serious complications; therefore, alternative lower-fat diets, including a modified Atkins diet and low-glycemic index diet, have been developed. Recent ongoing clinical evidence suggests that other dietary therapies have an efficacy almost comparable to that of the KD. In addition, a diet rich in polyunsaturated fatty acids appears to increase the efficacy of diet therapy and reduce the complications of a high-fat diet. Here, we review the systematic information about lower-fat diets and better-quality dietary therapies and the current clinical status of each of these dietary approaches.

A Case of Developmental Regression with Hypsarrhythmia on EEG Treated with Pulse Methylprednisolone / 대한소아신경학회지

Infantile spasms (IS) is a type of epileptic encephalopathy with poor developmental outcomes. The goal of treatment is complete control of spasms, improvement of hypsarrhythmia, and rapid reversal of delayed development. Improvements in diagnosis can result in the selection of appropriate therapy, which can improve a patient's developmental outcomes. However, there is no consensus on the best initial treatment for IS. Here, we describe 6-month-old boy with severe periventricular leukomalacia, developmental regression, and hypsarrhythmic pattern on electroencephalography, who was successfully treated with intravenous methylprednisolone.

A Case of Epstein-Barr Virus-associated Brainstem Encephalitis Presenting with Sixth Cranial Nerve Palsy in a Child / 대한소아신경학회지

Brainstem encephalitis caused by Epstein-Barr virus (EBV) is rare in childhood. We describe a 14-month-old-boy who presented with limitation of eye movements with sixth cranial nerve palsy. Brainstem encephalitis caused by EBV was diagnosed, based on the neuroimaging and cerebrospinal fluid (CSF), serological examination for EBV infection. Brain magnetic resonance imaging (MRI) showed high signal intensity in T2 and low signal intensity in T1 in both pons and medulla. The result of EBV polymerase chain reaction (PCR) of the CSF was positive, and a serological test showed a convalescent phase of primary infection: positive for viral capsid antigen (VCA)-IgM, VCA-IgG and EBV nuclear antigen (EBNA)-IgG, negative for heterophil antibody. We suggest that EBV infection should be considered a differential diagnosis cause of pediatric brainstem encephalitis and emphasizes the need for immediate diagnosis and initiation of symptomatic and specific therapy.

Epilepsy in Korean patients with Angelman syndrome / 소아과

PURPOSE: The aim of this study was to investigate the natural history of epilepsy and response to anti-epileptic drug treatment in patients with Angelman syndrome (AS) in Korea. METHODS: We retrospectively reviewed the clinical records of 14 patients diagnosed with epilepsy out of a total of 17 patients with a genetic diagnosis of AS. These patients were seen at the Department of Pediatric Neurology at Severance Children's Hospital from March 2005 to March 2011. RESULTS: Fourteen (9 males and 5 females) subjects (82.3%) were diagnosed with epilepsy in AS. The most common seizure types were generalized tonic-clonic (n=9, 27%) and myoclonic (n=9, 27%), followed by atonic (n=8, 24%), atypical absence (n=4, 12%) and complex partial seizure (n=3, 9%). The most commonly prescribed antiepileptic drug (AED) was valproic acid (VPA, n=12, 86%), followed by lamotrigine (LTG, n=9, 64%), and topiramate (n=8, 57%). According to questionnaires that determined whether each AED was efficacious or not, VPA had the highest response rate and LTG was associated with the highest rate of seizure exacerbation. Complete control of seizures was achieved in 6 patients. Partial control was achieved in 7 patients, while one patient was not controlled. CONCLUSION: Epilepsy is observed in the great majority of AS patients. It may have early onset and is often refractory to treatment. There are few reports about epilepsy in AS in Korea. This study will be helpful in understanding epilepsy in AS in Korea.

Clinical characteristics of vitamin D deficiency rickets in infants and preschool children / 소아과

PURPOSE: Vitamin D deficiency rickets is a significant public health problem that results from insufficient exposure to sunlight and inadequate vitamin D supplementation. The purpose of this study is to identify the clinical characteristics of vitamin D deficiency rickets in infants. METHODS: Data of 35 infants diagnosed as vitamin D deficiency rickets at Sanggye-Paik Hospital, Seoul, Korea, from March 2007 to May 2009 were reviewed. Children with plasma 25-hydroxyvitamin D levels <15 ng/mL and 15-30 ng/mL were considered to have vitamin D deficiency and vitamin D insufficiency, respectively. RESULTS: Thirty-five infants (22 boys, 13 girls) were diagnosed with rickets. Mean age at diagnosis was 7.4+/-7.1 months (range: 0.1-29.8 months). Eighteen infants (51%) were vitamin D deficient and seventeen infants (49%) were insufficient. Twenty-eight of all (80%) diagnosed as subclinical rickets. Twenty-nine infants (83%) were below the age of 12months. Twenty infants (57%) had breastfed and ten infants (29%) had iron deficiency anemia. Nine of breastfed infants (45%) were vitamin D deficient and ten of their mothers were vitamin D insufficient. Overall, radiographic evidence of rickets was present in 93% of the cases. Radiographic sign of rickets was evident even in vitamin D insufficient state. CONCLUSION: It is important for the clinician to screen for subclinical vitamin D deficiency rickets in inadequately supplemented infants by pairing 25-hydroxyvitamin D levels with wrist radiographs. A nationwide epidemiological study of vitamin D deficiency rickets must be conducted and evidence-based national guidelines must be defined to prevent rickets.

Risk Factors and Prognosis for Periventricular Leukomalacia According to Neuroimage in Preterm Infants

PURPOSE: The aim of this study was to determine the risk factors, clinical characteristics and prognosis for the development of periventricular leukomalacia (PVL) in preterm infants according to the extent and site of the PVL. METHODS: The medical records of infants (under 36 weeks of gestational age) delivered from January 1999 to December 2008 were reviewed. Twenty-five preterm infants with were PVL were diagnosed by brain magnetic resonance imaging (MRI) and an addition 50 preterm infants with no brain lesions were enrolled in this study. The perinatal and neonatal risk factors for the development of PVL was determine in these infants. Mental and Psychomotor Developmental Indices (MDI, PDI) were assessed by a clinical psychologist using the Bayley Scales of Infant Development II. We compared the differences of the clinical characteristics and prognosis according to brain MRI findings. RESULTS: Maternal fever, young maternal age, extended oxygen use, hypotension within the first week of birth, use of inotropics within the first week of birth, and respiratory distress syndrome were the risk factors associated with PVL (P<0.05). In the multivariate analysis, maternal fever and extended oxygen use were statistically significant independent risk factors (P<0.05). The mean MDI and PDI scores of the PVL group (74.4+/-27.8 and 58.0+/-17.7) were significantly lower than those of the control group (103.5+/-8.9 and 101.7+/-16.1, P<0.05). CONCLUSION: Maternal fever and extended oxygen use were independent risk factors for PVL. We should pay attention to infants who had the risk factors and follow them up closely by brain imaging study and Bayley Scales of Infant Development II.

Fatal and Near-fatal Cases of Lower Respiratory Tract Infection with Human Rhinovirus Group A / 소아알레르기및호흡기학회지

Human rhinoviruses (HRV) mostly cause mild and self-limiting upper respiratory tract infections. We report 2 infants with acute respiratory failure requiring mechanical ventilation, 1 of whom deteriorated to death. The causal pathogen was HRV group A confirmed by multiplex-PCR. In Korea, this is the first report of severe respiratory failure caused by HRV group A during the same season.

Obesity Related Glomerulopathy Progressed to Chronic Renal Failure

Obesity-related glomerulopathy (ORG) is a secondary form of focal and segmental glomerulosclerosis (FSGS) manifesting as proteinuria and progressive renal dysfunction that results from maladaptive glomerular response to increasing adiposity. Reports of ORG progressing to end stage renal diseases in rare in the pediatric population. We report a 9-year-old boy with obesity (body mass index 35 kg/m2) who was diagnosed with ORG presenting with proteinuria. He was diagnosed with obesity-related glomerulopathy based on the laboratory, urinary, and kidney biopsy finding. In spite of treatment with angiotensin-converting enzyme (ACE) inhibitor and/or, angiotensin-receptor blocking agent, the degree or amount of proteinuria increased and renal function declined continuously. His BMI did not decrease and eventually progressed to chronic renal failure. Consequently, obese patients should be monitored for proteinuria, which may be the first manifestation of FSGS, a lesion that may be associated with serious renal sequelae.

Urinary Protein and Enzyme Excretion of Spot Urine in Children with Vesicoureteral Reflux

PURPOSE: The aim of this study was to evaluate the clinical usefulness of measurement of beta2 microglobulin (beta2 MG), N-acetyl-beta-D-glucosaminidase (NAG) of spot urine samples as indices of renal tubular damage and microalbumin of spot urine samples as a parameter of glomerular damage in children with vesicoureteral reflux (VUR) or renal defects. METHODS: We studied 91 children with previous UTI. The children were classified as 62 children without VUR and renal defects (group I), 10 children with VUR, without renal defects (group II), and 19 children with VUR and renal defects (group III). Patients having VUR were separated according to the degree of VUR (mild VUR: VUR grade I-III, severe VUR: VUR grade IV-V). Urinary excretion of beta2 microglobulin (beta2 MG), microalbumin, N-acetyl-beta-D-glucosaminidase (NAG), creatinine were measured in samples of morning urine specimens. Children with VUR or renal defects detected by voiding cystourethrography (VCUG) and DMSA renal scan were investigated. RESULTS: Microalbumin/Cr ratio of spot urine was significantly increased in group III compared group I (42.3+/-27.2 mg/gCr vs 25.2+/-10.9 mg/gCr, P<0.05). NAG/Cr ratio of spot urine was significantly increased in group II compared group I (3.70+/-23.4 mg/gCr vs 18.7+/-12.7 mg/gCr, P<0.05). There was no statistically significant difference of beta2 MG/Cr ratio among three groups. CONCLUSION: Urinary microalbumin excretion of morning urine sample may be a simple and reliable clinical indicators for early identification of renal damage in children with VUR and renal defects. Urinary microalbumin excretion may be useful marker to predict the the severity of VUR.