• Clinical and Experimental Pediatrics
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• v.52 no.9
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• pp.971-975
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• 2009

Seizures in the neonatal period are common and frequently indicate serious underlying brain injury. Neonatal seizures continue to present a diagnostic and therapeutic challenge to pediatricians because recognition and classification of neonatal seizures remain problematic, particularly when clinicians rely only on clinical criteria. Neonatal seizures can permanently disrupt neuronal development, induce synaptic reorganization, alter plasticity, and "prime" the brain to increased damage from seizures later in life. Since neonatal seizures predict an increased risk for later epilepsy and other neurological sequelae, accurate diagnoses are needed for aggressive antiepileptic drug use. The present review summarizes the treatment and prognosis of neonatal seizures.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.245-250
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• 2005

The syndrome of reversible posterior leukoencephalopathy syndrome(RPLS) is characterized clinically by acute neurologic signs such as headache, vomiting, confusion, seizures, and visual abnormalities. Radiologically, abnormalities consistent with reversible white matter edema in the occipital and parietal lobes are characteristic. RPLS has often been associated with various systemic disorders, such as hypertensive encefhalopathy, eclampsia, and the use of intravenous or intrathecal immunosuppressive drugs. We report a case of RPLS that occurred after intravenous steroid pulse therapy and treatment with oral cyclophosphamide in a child with nephrotic syndrome, and we emphasize the importance of early recognition of RPLS in the treatment of nephrotic syndrome and appropriate management tn prevent Permanent neurologic disability. (J Korean Soc Pediatr Nephrol 2005;9:245-250)

• Clinical and Experimental Pediatrics
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• v.58 no.1
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• pp.8-14
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• 2015

In patients with a language developmental delay, it is necessary to make a differential diagnosis for autism spectrum disorders (ASDs), specific language impairment, and mental retardation. It is important that pediatricians recognize the signs and symptoms of ASDs, as many patients with language developmental delays are ultimately diagnosed with ASDs. Pediatricians play an important role in the early recognition of ASDs, because they are usually the first point of contact for children with ASDs. A revision of the diagnostic criteria of ASDs was proposed in the Diagnostic and Statistical Manual of Mental Disorders, fifth edition (DSM-5) that was released in May 2013. The autism spectrum describes a range of conditions classified as neurodevelopmental disorders in the fifth edition of the DSM. The new diagnostic criteria encompasses previous elements from the diagnosis of autistic disorder, Asperger disorder, childhood disintegrative disorder, and pervasive developmental disorder-not otherwise specified. An additional change to the DSM includes synthesizing the section on social and communication deficits into one domain. In ASD patients, the appropriate behavioral therapies and rehabilitation treatments significantly affect the prognosis. Therefore, this makes early diagnosis and treatment very important. In conclusion, pediatricians need to be able to recognize the signs and symptoms of ASDs and be attentive to them in order to make an early diagnosis and provide treatment.

• Clinical and Experimental Pediatrics
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• v.64 no.10
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• pp.497-503
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• 2021

Reading disability (dyslexia) refers to an unexpected difficulty with reading for an individual who has the intelligence to be a much better reader. Dyslexia is most commonly caused by a difficulty in phonological processing (the appreciation of the individual sounds of spoken language), which affects the ability of an individual to speak, read, and spell. In this paper, I describe reading disabilities by focusing on their underlying neurobiological mechanisms. Neurobiological studies using functional brain imaging have uncovered the reading pathways, brain regions involved in reading, and neurobiological abnormalities of dyslexia. The reading pathway is in the order of visual analysis, letter recognition, word recognition, meaning (semantics), phonological processing, and speech production. According to functional neuroimaging studies, the important areas of the brain related to reading include the inferior frontal cortex (Broca's area), the midtemporal lobe region, the inferior parieto-temporal area, and the left occipitotemporal region (visual word form area). Interventions for dyslexia can affect reading ability by causing changes in brain function and structure. An accurate diagnosis and timely specialized intervention are important in children with dyslexia. In cases in which national infant development screening tests have been conducted, as in Korea, if language developmental delay and early predictors of dyslexia are detected, careful observation of the progression to dyslexia and early intervention should be made.

• Clinical and Experimental Pediatrics
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• v.50 no.5
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• pp.430-435
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• 2007

Even though we drink and excrete water without recognition, the amount and the composition of body fluid remain constant everyday. Maintenance of a normal osmolality is under the control of water balance which is regulated by vasopressin despite sodium concentration is the dominant determinant of plasma osmolality. The increased plasma osmolality (hypernatremia) can be normalized by the concentration of urine, which is the other way of gaining free water than drinking water, while the low plasma osmolality (hyponatremia) can be normalized by the dilution of urine which is the only regulated way of free water excretion. On the other hand, volume status depends on the control of sodium balance which is regulated mainly by renin-angiotensin-aldosterone system, through which volume depletion can be restored by enhancing sodium retention and concomitant water reabsorption. This review focuses on the urine concentration and dilution mechanism mediated by vasopressin and the associated disorders; diabetes insipidus and syndrome of inappropriate antidiuretic hormone secretion.

• Clinical and Experimental Pediatrics
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• v.52 no.9
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• pp.964-970
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• 2009

Neonatal seizures are generally not only brief and subtle but also not easily recognized and are usually untreated. In sick neonates, seizures are frequently not manifested clinically but are detected only by electroencephalography (subclinical EEG seizures). This phenomenon of electroclinical dissociation is fairly common in neonates. On the other hand, neonates frequently show clinical behaviors such as stiffening, apnea, or autonomic manifestations that mimic seizures, which is usually associated with underlying encephalopathy and non-epileptic seizures. Therefore, it might be difficult to confirm the diagnosis of neonatal seizures. Early recognition of neonatal seizures is important to minimize poor neurodevelopmental outcomes, including cognitive, behavioral, and learning disabilities, as well as the development of postnatal epilepsy. EEG is a reliable tool in the determination of neonatal seizures. Continuous EEG monitoring is essential for the identification of seizures, evaluation of treatment efficacy, and prediction of the neurodevelopmental outcome. However, there is not yet a wide consensus on the optimal "standard" lead montage for the continuous EEG monitoring.

• Clinical and Experimental Pediatrics
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• v.49 no.2
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• pp.117-128
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• 2006

Non-traumatic acute abdominal pain in children presents a diagnostic dilemma. Numerous disorders can cause abdominal pain. Although many etiologies are benign, some require a rapid diagnosis and treatment in order to minimize morbidity. This review concentrates on the clinical office evaluation of acute abdominal pain in infants and children and details the clinical guideline for the diagnostic approach to imaging and the salient clinical features of some of the conditions. The clinical outcomes of children presenting with acute abdominal pain and the risk factors of the high risk underlying diseases would be provided for the diagnostic algorithm.

• Clinical and Experimental Pediatrics
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• v.55 no.10
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• pp.367-370
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• 2012

Urinary tract infection (UTI) is a common bacterial illness in children. Acute pyelonephritis in children may lead to renal scarring with the risk of later hypertension, preeclampsia during pregnancy, proteinuria, and renal insufficiency. Until now, vesicoureteral reflux (VUR) has been considered the most important risk factor for post-UTI renal scar formation in children. VUR predisposes children with UTI to pyelonephritis, and both are associated with renal scarring. However, reflux nephropathy is not always acquired; rather, it reflects reflux-associated congenital dysplastic kidneys. The viewpoint that chronic kidney disease results from renal maldevelopment-associated VUR has led to questioning the utility of any regimen directed at identifying or treating VUR. Despite the recognition that underlying renal anomalies may be the cause of renal scarring that was previously attributed to infection, the prevention of renal scarring remains the goal of all therapies for childhood UTI. Therefore, children at high risk of renal scar formation after UTI should be treated and investigated until a large clinical study and basic research give us more information.

• Clinical and Experimental Pediatrics
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• v.60 no.11
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• pp.344-352
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• 2017

Arrhythmias in the neonatal period are not uncommon, and may occur in neonates with a normal heart or in those with structural heart disease. Neonatal arrhythmias are classified as either benign or nonbenign. Benign arrhythmias include sinus arrhythmia, premature atrial contraction, premature ventricular contraction, and junctional rhythm; these arrhythmias have no clinical significance and do not need therapy. Supraventricular tachycardia, ventricular tachycardia, atrioventricular conduction abnormalities, and genetic arrhythmia such as congenital long-QT syndrome are classified as nonbenign arrhythmias. Although most neonatal arrhythmias are asymptomatic and rarely life-threatening, the prognosis depends on the early recognition and proper management of the condition in some serious cases. Precise diagnosis with risk stratification of patients with nonbenign neonatal arrhythmia is needed to reduce morbidity and mortality. In this article, I review the current understanding of the common clinical presentation, etiology, natural history, and management of neonatal arrhythmias in the absence of an underlying congenital heart disease.

• Clinical and Experimental Pediatrics
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• v.58 no.9
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• pp.317-324
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• 2015

Kabuki syndrome (KS) is a rare syndrome characterized by multiple congenital anomalies and mental retardation. Other characteristics include a peculiar facial gestalt, short stature, skeletal and visceral abnormalities, cardiac anomalies, and immunological defects. Whole exome sequencing has uncovered the genetic basis of KS. Prior to 2013, there was no molecular genetic information about KS in Korean patients. More recently, direct Sanger sequencing and exome sequencing revealed KMT2D variants in 11 Korean patients and a KDM6A variant in one Korean patient. The high detection rate of KMT2D and KDM6A mutations (92.3%) is expected owing to the strict criteria used to establish a clinical diagnosis. Increased awareness and understanding of KS among clinicians is important for diagnosis and management of KS and for primary care of KS patients. Because mutation detection rates rely on the accuracy of the clinical diagnosis and the inclusion or exclusion of atypical cases, recognition of KS will facilitate the identification of novel mutations. A brief review of KS is provided, highlighting the clinical and genetic characteristics of patients with KS.