Hoi Soo Yoon

High Remission Rate of Chronic Immune Thrombocytopenia in Children: Result of 20-Year Follow-Up

Purpose This study examined the outcomes of children with chronic immune thrombocytopenia (ITP). Materials and Methods We retrospectively analyzed the medical records of all patients diagnosed with ITP from January 1992 to December 2011 at our institution. Results A total of 128 patients (64%) satisfied the criteria for newly diagnosed ITP, 31 (15%) for persistent ITP, and 41 (21%) for chronic ITP. The median age at diagnosis was 4.5 years (range, 1 month to 18 years). The median platelet count at diagnosis was 32×109/L. A comparison of the initial treatment data from 2001 to 2011 with those from 1992 to 2000 showed that the number of bone marrow examinations decreased, whereas observation increased. Chronic ITP presented at an older age than newly diagnosed and persistent ITP (6.6 years vs. 3.8 years vs. 4.1 years, respectively); however, the difference did not reach statistical significance (p=0.17). The probability of complete remission of chronic ITP was 50% and 76% at 2 and 5 years after diagnosis, respectively. Patients aged <1 year at diagnosis had a significantly better prognosis than did older patients (hazard ratio, 3.86; p=0.02). Conclusion Children with chronic ITP showed a high remission rate after long-term follow-up. This study suggests that invasive treatments such as splenectomy in children with chronic ITP can be delayed for 4 to 5 years if thrombocytopenia and therapeutic medication do not affect the quality of life.

Clinical Impact of Epileptiform Discharge in Children With Attention-Deficit/Hyperactivity Disorder (ADHD)

The aim of this study was to investigate the prevalence and clinical significance of epileptiform discharges in patients with attention-deficit/hyperactivity disorder (ADHD). The authors retrospectively reviewed 180 children who were diagnosed with ADHD and had an electroencephalography (EEG) recording. Epileptiform discharges were found in 29 (16.1%) of 180 patients with ADHD. Of these, 15 (8.3%) had generalized epileptiform discharges and 14 (7.7%) had focal epileptiform discharges. The focal epileptiform discharges were most prevalent from the frontal (5/14) and rolandic area (5/14). Among the 29 patients with epileptiform discharges and ADHD, 5 patients had previous history of epilepsy and 4 patients developed epilepsy later, whereas none of the normal EEG group developed epilepsy. The authors suggest that interictal epileptiform discharges appear to be associated with seizure occurrence in children with ADHD and might reflect maturational pathophysiology overlapping with epilepsy.

Should healthy children who will undergo minor surgery be screened for coagulation disorder

ABSTRACT The objective of this study is to analyze the major causes of abnormal findings seen in the preoperative coagulation tests of asymptomatic pediatric patients and to discuss the usefulness of coagulation tests prior to minor surgery. Among patients who received minor surgery in Kyung Hee University Medical Center from March 2008 to April 2015, a total of 7114 pediatric patients (ages 1–18) were included in our study. Of these, 226 (3.1%) were referred to the pediatrics hematology-oncology department because of abnormal preoperative coagulation tests. A review of the coagulation tests indicate the majority (n = 216, 95.5%) have prolonged activated partial thromboplastin time (aPTT), whereas a smaller number (n = 10, 4.5%) have prolonged prothrombin time international normalized ratio (PT INR). When the coagulation tests were repeated, 136 displayed abnormal findings again. Of these 136 patients, 128 patients underwent mixing tests, and 127 showed results of correction and were composed as follows: normal (n = 83), subnormal (n = 26), factor deficiency (n = 15), and lupus anticoagulant positive (n = 3). Breakdown by factor deficiencies was as follows: (i) factor XII (n = 9), (ii) factor IX (n = 2), (iii) factors XII and IX (n = 1), (iv) factor VIII (n = 1), (v) factor XI (n = 1), (vi) von Willebrand factor (vWF; n = 1), and (vii) factor V (n = 1). Each factor activity range was mild (21%–39%), so no patients received preoperative medications or clotting factors/blood products. Even in the presence of factor deficiencies, bleeding symptoms were mild and postoperative complications did not occur. These results suggest that coagulation tests may not be needed in healthy children and should be reserved for patients with positive bleeding and/or family history.

Consecutive occurrence of benign epilepsy with centro-temporal spike and childhood absence epilepsy: true coexistence or atypical evolution?

We read with great interest the recent review of benign epilepsy with centro-temporal spikes (BECTS) and childhood absence epilepsy (CAE) by Verrotti et al. [1]. BECTS and CAE are the most common epilepsy syndromes in childhood and share certain clinical features: similar age at onset, overall good prognosis, and genetic predisposition. Several studies have reported the coexistence of the two syndromes, and recently, Verrotti et al. analyzed 19 cases reported to have clinical features of CAE and BECTS. Among the total 19 cases, three cases had features of both syndromes contemporarily; three cases experienced absence seizures initially followed by BECTS; and the other 13 cases experienced BECTS at onset and had absence seizures later. Here, we would like to consider the patients who presented with BECTS at onset and absence seizures later. Of the 13 patients, seven had typical or simple absence seizures and the other six had atypical absence seizures, atonic absences, absence seizure with fluttering, or eyelid myoclonia. According to inclusion and exclusion criteria for CAE proposed by Panayiotopoulos, these feature are not compatible with CAE [2]. Atypical absence seizures are differentiated from absence seizures in the frequency of spikes and wave discharges, refractoriness to medical therapy, and severely abnormal cognitive and neurodevelopmental outcome. Both typical and atypical absence epilepsy are thought to be generated by a rhythmogenic interplay between the cortex and the thalamus. However, it is hypothesized that atypical absence seizures engage different neuronal networks within the thalamo-cortical circuitry, as compared to typical absence seizures [3]. In some patients with BECTS, atypical evolution occur during the natural course or medical treatment. Atypical features at onset, such as frequent spikes or spike-wave discharges, or early age at onset, are considered risk factors for, and atypical evolution of, BECTS [4]. We suggest that some of the 13 patients with BECTS who later developed CAE might be examples of atypical evolution of BECTS rather than a true coincidence of two syndromes. Notably, three of 13 patients have experienced atypical absence seizures after the administration of antiepileptic drugs, which improved after drug withdrawal. Several authors have reported children with BECTS who developed CAE after admiration of antiepileptic drugs such as oxcarbazepine, phenobarbital, and lamotrigine [5–7]. This implies that the evolution to atypical BECTS was triggered by certain antiepileptic drugs in prone subjects. Similarly, five of the six patients in Dimova et al. were treated with carbamazepine, and they also suggested the possibility of aggravation of BECTS by the drugs [8]. Obviously, it is possible CAE and BECTS can simply coexist. The three patients with contemporary onset of CAE and BECTS in this review are such cases, in whom neurocognitive function is normal and they have good responses to drugs. However, when CAE and BECTS occur consecutively in one patient, they should be distinguished from atypical evolution of BECTS. Especially in these cases, the effect of antiepileptic drug should be considered.

Burst Suppression Patterns on an Electroencephalogram of an Infant with Congenital Central Hypoventilation

We read with great interest the recent case report by Hong et al 1 describing an infant with congenital central hypoventilation syndrome who displayed burst suppression patterns on the electroencephalogram (EEG). The patient in the report developed apnea at the age of 2 days, and had spasms during sleep when she was age 1 month. The EEG performed at that time showed a typical burst suppression (BS) pattern. As the authors noted in the case study, BS patterns on the EEG imply severe central nervous system dysfunctions including coma, cerebral anoxia or childhood epileptic encephalopathy. Early infantile epileptic encephalopathy (EIEE, Ohtahara syndrome) and early myoclonic epilepsy (EME) are representative examples of conditions associated with the BS pattern. These syndromes share several clinical manifestations and are characterized by the early onset of seizures, BS patterns on the EEG, and poor prognosis with severe psychomotor retardation. 2 However, the etiologiesandseizuretypesaresomewhat different between EIEE and EME. The majority of EIEE patients have brain malformations, and many cases of EME are associated with metabolic disorders such as nonketotic hyperglycinemia. EIEE cases typically present with tonic seizures, which is similar to the patient in the cited report, while EME is likely to be associated with myoclonic seizures. However, the BS pattern that presented only during sleep and normal waking EEG in this patientsuggestsEMEratherthanEIEE.Althoughtheclinicalseizures mimicked EIEE in this patient, the EEG findings more favored EME. It is often difficult to clearly differentiate between the two syndromes.