• Childhood Kidney Diseases
• /
• v.26 no.2
• /
• pp.101-106
• /
• 2022

Nephrotic syndrome (NS) is a hypercoagulable state in which children are at risk of venous thromboembolism. A higher risk has been reported in children with steroid-resistant NS than in those with steroid-sensitive NS. The mortality rate of cerebral venous sinus thrombosis (CVST) is approximately 10% and generally results from cerebral herniation in the acute phase and an underlying disorder in the chronic phase. Our patient initially manifested as a child with massive proteinuria and generalized edema. He was treated with albumin replacement and diuretics, angiotensin-converting enzyme inhibitor, and deflazacort. Non-contrast computed tomography showed areas of hyperattenuation in the superior sagittal sinus when he complained of severe headache and vomiting. Subsequent magnetic resonance imaging revealed empty delta signs in the superior sagittal, lateral transverse, and sigmoid sinuses, suggesting acute CVST. Immediate anticoagulation therapy was started with unfractionated heparin, antithrombin III replacement, and continuous antiproteinuric treatment. The current report describes a life-threatening CVST in a child with steroid-dependent NS, initially diagnosed by contrast non-enhanced computed tomography and subsequently confirmed by contrast-enhanced magnetic resonance imaging, followed by magnetic resonance venography for recanalization, addressing successful treatment.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.35 no.2
• /
• pp.351-356
• /
• 2008

Cerebral palsy, a range of non-progressive syndromes of posture and motor impairment, is a common cause of disability in childhood. Self-injurious behavior(SIB) is deliberate harm to the body without suicidal intend, often involving repetitive actions that cause tissue damage. One of the most common orofacial self-injurious behavior is chewing tongue, lip or oral mucosa. This kind of SIB in children is not common in normal children. High occurrence rates are observered in cases of syndromatic, mentally retarded children, and children with congenital disease. Various methods such as behavior modification, behavior control by drugs, body restraints, application of dental appliance, surgery and extraction of teeth have been suggested to control those self-injurious behavior. Using mouthguard as one of dental applainaces is the most conservative and appropriate method in terms of reducing oral self-injurious habits and protection of tissue. This case report describes a child with cerebral palsy who presented with self-injurious ulceration of lip and buccal mucosa. A modified mouthguard was effective in preventing self-injurious oral trauma in a child with cerebral palsy.

• Childhood Kidney Diseases
• /
• v.24 no.1
• /
• pp.42-46
• /
• 2020

Disturbances in water and salt balances are relatively common in children after brain tumor surgery. However, the coexistence of different diseases of water and sodium homeostasis is challenging to diagnose and treat. The coexistence of combined central diabetes insipidus (CDI) and cerebral salt wasting syndrome (CSWS) is rare and may impede accurate diagnosis. Herein, we report the case of an 18-year-old girl who underwent surgery for a germinoma and who presented prolonged coexistence of CDI and CSWS. The patient was diagnosed with panhypopituitarism with CDI at presentation and was treated with hydrocortisone, levothyroxine, and desmopressin. Postoperatively, she developed polyuria of more than 3L/day, with a maximum daily urine output of 7.2 L/day. Her serum sodium level decreased from 148 to 131 mEq/L. Polyuria was treated with desmopressin at incremental doses, and hyponatremia was managed with fluid replacement. At 2 months after surgery, she presented with hyponatremia-induced seizure. Polyuria and hyponatremia combined with natriuresis indicated CSWS. Treatment with fludrocortisone were initiated; then, her electrolyte level gradually normalized. CSWS is self-limiting and generally resolves within 2 weeks. However, the patient in this study still required treatment with vasopressin and fludrocortisone at 16-months after surgery. Hyponatremia in a patient with CDI may be erroneously interpreted as inadequate CDI control or syndrome of inappropriate antidiuretic hormone secretion, leading to inappropriate treatment. The identification of the potential combination of CDI and CSWS is important for early diagnosis and treatment.

• The Journal of Korean Physical Therapy
• /
• v.11 no.1
• /
• pp.23-43
• /
• 1999

A study on severity of cerebro-vascular accident patients in Korea was conduced in order to determine the risk factors affecting the severity of stroke patients. This study was performed by interviewing a total of 477 hospitalized and ambulatory patient of CVA in Seoul, Taejon, and Taegu and Pusan areas from April 1, 1998 through June 30, 1998. The results are as follows; 1. Two hundred and sixty seven $(56\%)$ at Four hundred and seventy seven subjects were manes, md two hundred and ten $(44\%)$ were females. $28.3\%$ of the subjects' ages were 50's and $27.3\%$ were 60's and $18.5\%$ were 70's. $22.6\%$ of the subjects' occupations were home makers, $17.8\%$ were farming. $66.4\%$ of the subjects spent their childhood in urban areas and $33.1\%$in rural areas. $41.7\%$ of the subjects became violin of CVA in Spring, $35.0\%$ in Winter. 2. On physical and mental conditions of the subjects at the occurrence of CVA, $28.7\%$ of the subjects were engaged in physical activities. $22.6\%$ were in a rest stale $19.5\%$ were in sleeping and $18.9\%$ were mentally shocked. $79.4\%$ of the male subjects and $14.8\%$ of female subjects smoked cigarettes. $82\%$ of male subjects drank coffee. $81.1\%$ of the subjects did not exercise regularly. $45.9\%$ of the subjects had the systolic blood pressures in the range of 160 to 199 mmHg and $5.6\%$ of the subjects had hypertension before the occurrence of stroke and $11.7\%$ had diabetes. 3. Of the types of strokes, cerebral hemorrhage was the highest $(49.1\%)$. cerebral infarction was the second $(41.1\%)$. Severe strokes were found in cerebral hemorrhage cases $(52.0\%)$ and cerebral infarction cases $(40.1\%).\;50.9\%$. of the male subjects were moderate cases, $50.9\%$ were severe cases. In females, moderate cases were $72.4\%$, severe cases $15.2\%$. $37.5\%$ of the subjects who had preceding diseases were severe cases, and $15.6\%$ of the subjects without preceding diseases were severe cases. $50.7\%$ of the subjects whose family members had strokes had severe strokes. $34\%$ of the subjects sleeping less than 6 hours a day, $42.4\%$ of the subjects with irregular eating habits, $33.3\%$ of the subjects who liked meat, and $42.3\%$ of the subjects who liked salty foods had severe strokes. $35.9\%$ of the subjects with hot temper, $27.6\%$ of the subjects with moderate temper and $14.5\%$ of subjects with mild temper were severe cases. 4. The correlation coefficient between obesity and blood pressure was 0.094.

• Clinical and Experimental Pediatrics
• /
• v.48 no.11
• /
• pp.1232-1238
• /
• 2005

Purpose : Though more than 15 years have passed after introduction of Lorenzo's oil therapy in X-linked adrenoleukodystrophy(ALD), its efficacy is still fully not known. In patients who already have neurologic symptoms, most reports indicate that the neurologic disability continues to increase. We evaluated its efficacy in X-linked ALD patients. Methods : Four patients who were treated with Lorenzo's oil for at least 12 months were enrolled from 1996 to 2003. During treatment, changes of neurologic symptoms, brain magnetic resonance imaging(MRI) findings, and serum saturated very long-chain fatty acid(C26:0) were assessed. Results : Two patients with childhood cerebral ALD had progression of neurologic symptoms and MRI lesions during treatment. One asymptomatic patient developed childhood cerebral ALD after six year treatment of Lorenzo's oil. One "Addison only" patient remained neurologically intact after three years. During Lorenzo's oil therapy, serum C26:0 levels which had increased at diagnosis decreased in all four patients. Conclusion : Treatment with Lorenzo's oil did not prevent disease progression in childhood cerebral ALD patients who were already symptomatic. Long term follow up will be needed in asymptomatic ALD including "Addison only" to reveal the efficacy of Lorenzo's oil.

• The Korean Journal of Nuclear Medicine
• /
• v.28 no.1
• /
• pp.22-30
• /
• 1994

Encephalo-duro-arterio-synangiosis (EDAS) is a relatively new surgical procedure for treatment of childhood moyamoya disease. We assessed regional cerebral perfusion in moyamoya patients before (1.3 mo) and after (6.8 mo) EDAS with $^{99m}Tc$-HMPAO brain SPECT. A total of 21 EDAS operations in 17 moyamoya patients was included. Preoperative CT or MRI showed cerebral infarction in 14 patients and carotid angiography showed Suzuki grade I to V stenosis in 6%, 9%, 62%, 12% and 12% of the hemispheres respectively. Preoperative SPECT showed regional hypoperfusion in all patients, bilateral frontal and temporal lobes being the most frequently involved site. $4{\times}4$ pixel sized ROIs were applied on the frontotemporal cortex in 3 slice averaged transverse tomographic images. An index of regional perfusion was measured as: PI (%)=average F-T activity/average cerebellar activity${\times}100$ Pre-EDAS ipsilateral PI ranged from 23.7 to 98.4% (mean: $74.3{\pm}17%$) and increased significantly after operation ($81.4{\pm}17%$, p<0.001). Individual post-EDAS PI improved in 15/21 cases, showed no significant change in 5 and was slightly aggravated in 1. The amount of clinical improvement (${\Delta}CI$) was graded with a scale of 0 to 4 based on frequency and severity of TIA attacks. When patients were grouped according to pre-EDAS PI, group II (PI 70-89) showed a significantly higher ${\Delta}CI$ (3.3) compared to group I (PI< 70, 1.57) or group III (PI >90, 0.5) (P< 0.001). The amount of perfusion improvement (${\Delta}PI$) showed significant correlation with ${\Delta}CI$ (r=0.42, p=0.04). ${\Delta}PI$ did not, however, correlate with the amount of neovascularization assessed angiographically in 8 patients. Serial HMPAO SPECT is an useful noninvasive study for assessing perfusion improvement after EDAS in childhood moyamoya patients.

• Clinical and Experimental Pediatrics
• /
• v.64 no.7
• /
• pp.313-321
• /
• 2021

Here we describe the neurodevelopmental outcomes of very low birth weight (VLBW) infants (birth weight ≤1,500 g) at 3 years of age in the Neonatal Research Network of Japan (NRNJ) database in the past decade and review the methodological issues identified in follow-up studies. The follow-up protocol for children at 3 years of chronological age in the NRNJ consists of physical and comprehensive neurodevelopmental assessments in each participating center. Neurodevelopmental impairment (NDI)-moderate to severe neurological disability-is defined as cerebral palsy (CP) with a Gross Motor Function Classification System score ≥2, visual impairment such as uni- or bilateral blindness, hearing impairment requiring hearing amplification, or cognitive impairment with a developmental quotient (DQ) of Kyoto Scale of Psychological Development score <70 or judgment as delayed by pediatricians. We used death or NDI as an unfavorable outcome in all study subjects and NDI in survivors using number of assessed infants as the denominator. Follow-up data were collected from 49% of survivors in the database. Infants with follow-up data had lower birth weights and were of younger gestational age than those without follow-up data. Mortality rates of 40,728 VLBW infants born between 2003 and 2012 were 8.2% before discharge and 0.7% after discharge. The impairment rates in the assessed infants were 7.1% for CP, 1.8% for blindness, 0.9% for hearing impairment, 15.9% for a DQ <70, and 19.1% for NDI. The mortality or NDI rate in all study subjects, including infants without follow-up data, was 17.4%, while that in the subjects with outcome data was 32.5%. The NRNJ follow-up study results suggested that children born with a VLBW remained at high risk of NDI in early childhood. It is important to establish a network follow-up protocol and complete assessments with fewer dropouts to enable clarification of the outcomes of registered infants.

• Journal of Yeungnam Medical Science
• /
• v.2 no.1
• /
• pp.103-111
• /
• 1985

Childhood epilepsy which has high prevalence rate and inception rate is one of the commonest problem encountered in pediatrician. In contrast with epilepsy of adult, in childhood epilepsy, more variable and varying manifestations are found because the factors of age, growth and development exert their influences in the manifestations and the courses of childhood epilepsy. Moreover epileptic children have associated problems such as physical and mental handicaps, psychologicaldisorders and learning disability. For these reasons pediatrician who deals with epileptic children experiences difficulties in making diagnosis and managing them. In order to improve understanding and management of childhood epilepsy, authors reviewed 103 cases of epileptic patients seen at pediatric department of Yeungnam University Hospital retrospectively. The patients were classified according to the type of epileptic seizure. Suspected causes of epilepsy, associated conditions of epileptic patients, age incidence and the findings of brain CT were reviewed. Large numbers of epileptic patients (61.2%) developed their first seizures under the age of 5. The most frequent type of epileptic seizure was generalized ionic-clonic, tonic, clonic seizure (49.5%), followed by simple partial seizure with secondary generalization (17.5%), simple partial seizure (7.8%), a typical absence (5.8%) and unclassified seizure (5.8%). In 83.5% of patients, we could not find specific cause of it, but in 16.5% of cases, history of neonatal hypoxia (4.9%), meningitis (3.9%), prematurity (1.9%), small for gestational age (1.0%), CO poisoning (1.0%), encephalopathy (1.0%), DPT vaccination (1.0%), cerebrovascular accident (1.0%) and neonatal jaundice (1.0%) were found, 30 cases of patients had associated diseases such as mental retardation, hyperactivity, delayed motor milestones or their combinations. The major abnormal findings of brain CT performed in 42 cases were cortical atrophy, cerebral infarction, hydrocephalus and brain swelling. This review stressed better designed classification of epilepsy is needed and with promotion of medical care, prevention of epilepsy is possible in some cases. Also it is stressed that childhood epilepsy requires multidisplinary therapy and brain CT is helpful in the evaluation of epilepsy with limitation in therapeutic aspects.

• Journal of Genetic Medicine
• /
• v.10 no.1
• /
• pp.43-46
• /
• 2013

Adrenoleukodystrophy (ALD) is an X-linked disorder which has diverse constellation of clinical pictures, ranging from the severe childhood cerebral form to adrenocortical insufficiency without neurological manifestations. This disorder is caused by the mutations in the ABCD1 gene encoding the adrenoleukodystrophy protein (ALDP), a transporter in the peroxisome membrane. ALD in most cases is inherited from one parent. Here, we report an incidentally identified sporadic case with ALD after traffic accident. He had adrenocortical insufficiency as well as abnormal findings in brain image. Genetic testing of ABCD1 gene revealed a previously reported mutation. With the description of clinical features of ALD in this patient, we discussed the difficulty in determining an appropriate therapeutic option for ALD patients with minimal neurological manifestation.

• The Journal of Pediatrics of Korean Medicine
• /
• v.14 no.2
• /
• pp.141-147
• /
• 2000

Moyamoya is a chronic cerebrovascular disease chracterized by progressive stenosis or occlusion of the terminal parts of both internal carotid arteries with telangiectatic vascular network of collateral circuration at the base of the brain and leptomeningeal arteries. The etiology and pathophysiology of this disease are still unknown. The clinical course in those whose first symptoms occur in childhood is different from those in whom symptoms develop in adult life. The term moyamoya disease should be resserved for those cases in which the chracteristic angiographic pattern is idiopathic; moayamoya syndrome is used when the underlying condition is known. we have experienced a case of moyamoya syndrome in a 5-year-2-month-old boy who presented right-sided hemiparesis. A cerebral angiogram revealed occlusion of abnomal collateral network. Moyamoya disease is applicable to stroke of an infant from oriental medicine point of view, and The symptoms is similar to adult stroke, we have treated adult stroke patint with herb medicine and acupuncutre and physical treatment. The acute stage of stroke is applied to the external treatment(標治), and The recovery stage is applied to the basic treament(本治).