• Journal of Biomedical Engineering Research
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• v.27 no.2
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• pp.78-82
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• 2006

The ventricular assist device(VAD) helps to reduce the overload against the patient's native heart(NH). The pulsatile VAD pumps out the ventricular blood to the aorta with pulsatile flow. If the VAD pulsates simultaneously with the NH, the ventricle of the NH could confronts abnormally elevated aortic pressure, and this could deteriorate the ventricle rather than assist to recover it. Thus counterpulsation algorithms to avoid copulsation have been adopted by many VADs, but these methods utilize electrocardiography or arterial pressure signals, which may have difficulties to acquire consistently for a long period. In this study, the copulsation estimation algorithm for the counterpulsation is developed using the VAD outlet pressure signal. The VAD outlet pressure signal is good to maintain for a long time and the sensor part could be integrated to the VAD as a built-in module. From the VAD outlet pressure signal and its pump rate information calculated with Fast Fourier Transform, pulse peaks by the VAD and the NH were extracted and the next copulsation time at which the VAD and the NH would pulsate simultaneously was estimated. This estimation algorithm was implemented by using PC MATLAB software and tested for various pump rate conditions with mock circulation system. For each condition, the copulsation time was estimated successfully. Consequently, the results showed the possibility to use the outlet cannula pressure signal in the copulsation estimation.

• Journal of Sensor Science and Technology
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• v.9 no.1
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• pp.51-60
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• 2000

This paper outlines the development of a non-pulsatile axial flow type blood pump control system. By utilizing blood pressure and heart rate, this system can assist the left ventricle in controlling blood pressure and blood volume. The system is comprised of a blood pump, signal sensor, signal interface, and signal-processing component. A control algorithm is also proposed which can control non-pulsatile, continuous blood flow in the human circulatory system. To facilitate the control required for non-pulsatile blood pump in a physiological system, an experimental control rule was developed utilizing ECG and blood pressure data, both of which are easily detectable variables in the body. The system was then tested using a mock-up circulation system and we found that it is possible that this systems could be temporarily used in clinic.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.164-175
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• 2001

Purpose : Alport syndrome is a hereditary nephrotic disease characterized by progressive nephrotic symptom, sensorineural hearing loss, ophthalmic abnormality, typical microscopic findings, and familial occurrence. In this study, we tried to find the risk factors related with its prognosis by taking a close observation on clinical symptoms of children with Alport syndrome reviewing retrospectively. Materials & methods : We chose children diagnosed as Alport syndrome in renal biopsy during 20 years(from 1980, Jan. until 1999, Dec.) who could receive follow up studies in tile department of pediatrics. They were divided into two groups by comparing renal function at the time of diagnosis and at current status. We compared several clinical aspects in them, and applied nonparametric test for statistical analysis. Results : The sex ratio(male:female) of 24 children was 3:1. The most common clinical symptom presented at their first visit was gross hematuria. Among those 24 children, 11 cases($46\%$) of progressing into chronic renal failure(Group II) were observed. Hypertension, proteinuria and edema were seen much frequently in group II. The level of serum protein, albumin, and creatinine clearance were decreased while BUN, creatinine were relatively increased. All the results were statistically significant. Conclusion Clinically significant risk factors related to prognosis in Alport syndrome were the presence of hypertension, edema, and proteinuria at the time of diagnosis. Also, the level of serum protein, albumin, BUN, creatinine, and glomerular filtration rate were proved to be important factors in predicting prognosis. We believe that studies on these possible risk factors would be of great help in treating and predicting prognosis of children suffering with Alport syndrome. (J Korean Soc Pediatr Nephrol 2001;5 : 164-75)

• Clinical and Experimental Pediatrics
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• v.53 no.1
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• pp.106-110
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• 2010

Hippocampal sclerosis (HS) is one of the most common features of intractable temporal lobe epilepsy. Generally it can be identified through brain magnetic resonance imaging (MRI) with high degree of sensitivity and specificity. Typical brain MRI findings of HS are hippocampal atrophy with hyperintense signal confined to the lesion. On the other hand cortical dysplasia exhibits blurring of the gray-white matter junction and abnormal white matter signal intensity. We present a case where preoperative brain MRI strongly suggested the presence of diffuse cortical dysplasia in the left temporal lobe but postoperative pathology revealed the temporal lesion to be unremarkable except for hippocampal sclerosis.

• Journal of Biomedical Engineering Research
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• v.26 no.6
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• pp.393-398
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• 2005

An electromechanical type is the most useful mechanism in the various pumping mechanisms. It, however, requires a movement converting system including a ball screw, a helical cam, or a solenoid-beam spring, which makes the device complex and may lessen reliability. Thus, the authors have hypothesized that an electromagnetic actuator mechanism can eliminate the movement converting system and that thereby enhance the mechanical reliability and operative simplicity of an electro­pneumatic pump. The purpose of this study was to show a novel application of electromagnetic actuator mechanism in pulsatile pump and to provide preliminary data for further evaluations. The electromagnetic actuator consists of stators with a single winding excitation coil and movers with a high energy density neodymium-iron-boron permanent magnet. A 0.5mm diameter wire was used for the excitation coil, and 1000 turns were wound onto the stators core with parallel. A prototype of extracorporeal electro-pneumatic pump was constructed, and the pump performance tests were performed using a mock system to evaluate the efficiency of the electromagnetic actuator mechanism. When forward and backward electric currents were supplied to the excitation coil, the mover effectively moved back and forth. The nominal stroke length of the actuator was 10mm. The actuator dimension was 120mm in diameter and 65mm in height with a mass of 1.4kg. The prototype pump unit was 150mm in diameter, 150mm in thickness and 4.5kg in weight. The maximum force output was 70N at input current of 4.5A and the maximum pump rate was 150 beats per minute. The maximum output was 2.0 L/minute at a rate of 80bpm when the afterload was 100mmHg. The electromagnetic actuator mechanism was successfully applied to construct the prototype of extracorporeal electro­pneumatic pump. The authors provide the above results as a preliminary data for further studies.

• Clinical and Experimental Pediatrics
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• v.49 no.2
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• pp.187-191
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• 2006

Purpose : Malformation of cortical development(MCD) constitutes an important etiology of intractable epilepsy and is considered an indication for surgical treatments, though their efficacy is limited and variable depending on MCD's location or distribution. Ketogenic diets are widely known to be effective, but as little study has been made concerning their efficacy on epilepsy with MCD, we evaluated the efficacy of ketogenic diets on MCD patients compared with that of epileptic surgery, which is more invasive. Methods : We performed retrospective studies and analyse on 30 patients with MCD diagnosed by brain MRI and treated with ketogenic diets for intractable epilepsy since 1998, checking decreases in their seizure episodes after starting the diets. Results : Cortical dysplasia was observed in 24(80.0 percent) patients as the most common type of MCD. Also, MCD was observed in unilateral hemisphere most commonly, in 23(76.7 percent) patients; it was observed in both hemispheres in 7(23.3 percent) patients. Nine(30.0 percent) out of 30 patients became seizure-free after starting ketogenic diets, and 14(46.7 percent) patients experienced 50 percent seizure reductions as well. Age of starting the diet or the duration of epilepsy period before starting showed no statistical relationship with the efficacy of the diet. Though the younger the patient and the longer the treatment the more effective the diet seemed to be, there was no statistical correlation between them. The location of MCD showed no significance neither. Conclusion : Considering various limits and invasiveness of surgical treatment, a ketogenic diet could be a good tool in treating children with intractable epilepsy with MCD.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.23 no.1
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• pp.25-30
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• 2023

Leigh syndrome is a rare progressive neurodegenerative mitochondrial disorder with clinical and genetic heterogeneity. Recently, balletic IARS2 variants have been identified in a number of patients presenting broad clinical phenotypes from Leigh and West syndrome to a rare syndrome CAGSSS characterized by cataracts, growth hormone deficiency, sensory neuropathy, sensorineural hearing loss, and skeletal dysplasia syndrome (OMIM#616007). We describe a child with Korean Leigh syndrome with urologic manifestations resulting from a compound heterozygote mutation in IARS2. A 5-year-old girl visited the emergency room with a complaint of abdominal pain accompanied by abdominal distension. Abdominal-pelvic CT showed a markedly distended urinary bladder without definite obstructive lesions. She was diagnosed with neurogenic bladder dysfunction based on a urodynamic study. She had global delayed development due to neurologic regression after 6 months of age and a history of bilateral cataract surgery at the age of 2 years. Her brain magnetic resonance imaging showed symmetrically increased signal intensities in the bilateral putamen and caudate nuclei with diffuse cerebral atrophy. No gene variants were identified through whole-mitochondrial genome analysis. Whole exome sequencing was performed for diagnosis, and compound heterozygous pathogenic variants were identified in IARS2: c.2446C>T (p. Arg816Ter) and c.2450G>A (p. Arg817His). To the best of our knowledge, this is the first case report of bladder dysfunction manifestation in a patient with IARS2-related Leigh syndrome. Thus, it broadens the clinical and genetic spectrum of IARS2-associated diseases.