• Korean Journal of Clinical Pharmacy
• /
• v.14 no.1
• /
• pp.11-23
• /
• 2004

Although most children with idiopathic nephrotic syndrome respond to corticosteroid therapy, many responders show steroid dependency and frequent relapse. In these children, one of the major problems is the serious side effects resulting from continuous steroid therapy. Thus, this study was conducted to assess the therapeutic efficacy and safety of six-month cyclosporine treatment with the low-dose deflazacort therapy in children with nephrotic syndrome. Thirty children with steroid dependence (SD), frequent relapse (FR) and steroid resistance (SR) were enrolled in this study. They were treated with 6-month oral cyclosporine $(Cypol-N^{(R)})$ plus the low-dose deflazacort $(Calcort^{(R)})$ therapy at Samsung Medical Center from September 2002. The dosage of cyclosporine was started at 5 mg/kg/day and was monthly adjusted to maintain clinical remission and/or a trough blood level, while deflazacort dosage was reduced gradually. Clinical evaluation and monitoring of cyclosporine toxicity were performed every $2\sim4$ weeks. Outcomes were compared to the latest sir-month period of steroid only therapy before cyclosporine treatment. Student's t-test and ANOVA were used for statistical analysis. Out of 28 children with SD and FR, 23 $(82.1\%)$ sustained remission, and 5 $(17.9\%)$ experienced 1 or 2 relapses during therapy. Out of 2 children with SR, 1 child sustained remission, and 1 child showed no response. The mean duration of remission and occurrence of relapse were significantly improved (p <.0001). In addition, the mean dosage of steroid was significantly reduced (p=.003). Although a number of adverse effects occurred in this study, they were not so serious as to necessitate discontinuation of the therapy. No nephrotoxicity was observed. Twenty out of the 28 children who had been in remission relapsed after withdrawal of cyclosporine. Fifteen of these children showed relapse within a month. These results demonstrated that the combination of cyclosporine with the low-dose deflazacort was efficient and safe in children with SD and FR during the six-month treatment. However, further studies are necessary in order to resolve the problem of high relapse rate after discontinuation of cyclosporine.

• Molecules and Cells
• /
• v.43 no.4
• /
• pp.408-418
• /
• 2020

The sinus node (SN) is located at the apex of the cardiac conduction system, and SN dysfunction (SND)-characterized by electrical remodeling-is generally attributed to idiopathic fibrosis or ischemic injuries in the SN. SND is associated with increased risk of cardiovascular disorders, including syncope, heart failure, and atrial arrhythmias, particularly atrial fibrillation. One of the histological SND hallmarks is degenerative atrial remodeling that is associated with conduction abnormalities and increased right atrial refractoriness. Although SND is frequently accompanied by increased fibrosis in the right atrium (RA), its molecular basis still remains elusive. Therefore, we investigated whether SND can induce significant molecular changes that account for the structural remodeling of RA. Towards this, we employed a rabbit model of experimental SND, and then compared the genome-wide RNA expression profiles in RA between SND-induced rabbits and sham-operated controls to identify the differentially expressed transcripts. The accompanying gene enrichment analysis revealed extensive pro-fibrotic changes within 7 days after the SN ablation, including activation of transforming growth factor-β (TGF-β) signaling and alterations in the levels of extracellular matrix components and their regulators. Importantly, our findings suggest that periostin, a matricellular factor that regulates the development of cardiac tissue, might play a key role in mediating TGF-β-signaling-induced aberrant atrial remodeling. In conclusion, the present study provides valuable information regarding the molecular signatures underlying SND-induced atrial remodeling, and indicates that periostin can be potentially used in the diagnosis of fibroproliferative cardiac dysfunctions.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.3 no.1
• /
• pp.63-67
• /
• 2000

Purpose: Biliary atresia, one of the major causes of neonatal cholestais, is an idiopathic, serious disorder, affecting the newborn that results in complete obstruction of biliary tract. Successful reestablishment of bile flow is dependent on early surgical intervention, early diagnosis is imperative. The authors evaluate the utility of Tc-99m-labeled diisoprpyliminodiacetic acid (DISIDA) hepatobiliary scintigraphy in the diagnosis of biliary atresia. Methods: From January, 1995 to August, 1999, total 60 patients with neonatal cholestasis underwent Tc-99m DISIDA hepatobiliary scintigraphy at Asan Medical Center. Results: The undelying causes of neonatal cholestasis were biliary atresia in 14, neonatal hepatitis in 33, intrahepatic bile duct paucity in 9, and total parenteral nutrition induced cholestasis in 4. All patient with biliary atresia were interpreted correctely in DISIDA hepatobiliary scintigraphy, showing 100% sensitivity. Of the 46 patients with neonatal hepatitis and other causes, 37 patients had intestinal radioactivity showing 80% specificity. Conclusion: Visualization of DISIDA in the intestinal tract indicates patency of the biliary ducts and excludes the diagnosis of biliary atresia. But the absence of intestinal excretion on the DISIDA hepatobiliary scintigraphy dose not necessarily indicate biliary atresia.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.41 no.4
• /
• pp.328-334
• /
• 2014

Gingival fibromatosis is a rare oral condition that is characterized by proliferative fibrous overgrowth of the attached gingiva, the marginal gingiva, and the interdental papilla, typically presenting in the growth period. A case of a 27-month-old girl with a generalized severe gingival overgrowth is described herein. The patient had no known systemic disease, but enlarged gingival tissue had gradually covered her teeth. The excess gingival tissue was removed by conventional gingivectomy, which involved extraction of the retentive primary teeth under general anesthesia when she was 5 years old. Post surgical follow-up at 18 months after the surgery demonstrated no recurrence. Resectional surgery of the enlarged gingival tissue is the treatment choice for gingival fibromatosis, although there is a high risk of recurrence. More frequent professional follow-ups and oral hygiene instruction might be required. A delay in the surgical treatment may have significant consequences for the patient, such as primary dentition retention and consequent delay in the eruption of the permanent teeth, difficulties in mastication and phonation, malpositioning of the teeth, and psychological problems. Early surgical treatment should be performed according to the severity of enlargement.

• Neonatal Medicine
• /
• v.18 no.1
• /
• pp.124-129
• /
• 2011

Purpose: To evaluate the effect of oral sildenafil therapy in neonates with persistent pulmonary hypertension of the newborn (PPHN) Methods: We conducted a retrospective review of 32 neonates ${\geq}$35 weeks' gestation and fraction of inspired oxygen ($FiO_2$) 1.0 with PPHN. The first dose (0.5 mg/kg) of oral sildenafil was started and 1 mg/kg was given every 6 hour thereafter. Mean airway pressure (MAP), $FiO_2$, oxygenation index (OI), mean arterial blood pressure (MBP) were documented before and 6, 12, 24, and 48 hours after sildenafil. For adverse effects, gastrointestinal symptoms, brain ultrasound, funduscopy and auditory brainstem response results were evaluated. Results: The underlying diseases of PPHN (n=32) were meconium aspiration syndrome (n=9), respiratory distress syndrome (n=8), pneumonia (n=3), and idiopathic (n=12). Thirty-one neonates survived; 3 neonates were transferred for inhaled nitric oxide (iNO) and all of them survived. In 28 infants, $FiO_2$ and OI improved significantly by 6 hours and MAP improved significantly by 48 hours after initiation of sildenafil. There were no clinically significant adverse effects of sildenafil. Conclusion: Sildenafil may be an effective and safe agent for near-term and term neonates with PPHN, providing significant improvement in oxygenation, and thus may be especially useful in the treatment of PPHN in hospitals without iNO.

• Clinical and Experimental Pediatrics
• /
• v.46 no.1
• /
• pp.51-55
• /
• 2003

Purpose : We intended to find out the role of transesophageal atrial pacing in evaluation of infants and children with palpitation of unknown origin. Methods : We tried transesophageal atrial pacing study in 69 infants and children with palpitation, in whom tachycardia wasn't documented in electrocardiogram and reviewed retrospectively the records of transesophageal pacing and medical records of theses patients to find out the induction rate of tachycardia by transesophageal atrial pacing and the possible mechanism of tachycardia if induced. Results : In 70.1% infants and children with palpitation, tachycardia was induced, so we could conclude that tachycaridia was the cause of palpitation in these cases. In most cases, tachycardia was induced by rapid atrial pacing, and in 21% by using isoproterenol. Tachycardia induction rate was higher in <6-year-old children than ${\geq}6$-year-old children(P<0.05). In cases of the induced, we reviewed the mechanism of tachycardia. Of these induced tachycardias, 53.2% is atrioventricular reentry tachycardia, 34.0% were atrioventricular nodal reentry tachycardia, and 12.8% were idiopathic left ventricular tachycardia. Invasive electrophysiologic study was done to 10 patients of those induced. The results of electrophysiologic study and transesophageal pacing and recording were the same except for one patient. Conclusion : Transesophageal atrial pacing and recording is a less invasive, safe and useful method to find out the cause of palpitation and the mechanism of tachycardia in infants and children.

• Journal of Oral Medicine and Pain
• /
• v.35 no.2
• /
• pp.155-163
• /
• 2010

Dermatomyositis (DM) is an idiopathic inflammatory connective tissue disorder and a systemic autonomic immune disease which shows a progressive muscle weakness and characteristic rash. It is identified by a characteristic rash accompanying, or more often preceding muscle weakness. Pathognomonic skin lesions are a blue-purple discoloration on the upper eyelids with edema (heliotropic rash), a flat red rash on the face and upper trunk, and erythema of the knuckles with a raised violaceous scaly eruption (Gottron's papule). The myopathy represents inflammatory and degenerative changes primarily affecting proximal muscles. DM often involves GI tract and respiratory system with as risk of 15-25% internal malignancy. It's managed with sun protection since muscle weakness as well as a rash could be aggravated by sun exposure. Systemic corticosteroid is an initial therapy and other immunosuppressive agent has been used as alternatives. Facial muscles are unaffected and masticatory muscles are rarely affected in DM. We present trismus close to muscle contracture in a patient with DM. Therefore, it needs continuous mouth-opening exercise to prevent progressive muscle contracture and to ensure normal mouth opening.

• Journal of Oral Medicine and Pain
• /
• v.32 no.4
• /
• pp.449-453
• /
• 2007

Trigeminal neuralgia is defined as "a sudden, usually unilateral, brief stabbing recurrent pain in the distribution of one or more branches of the fifth cranial nerve" by the International Association for the Study of Pain(IASP). Trigeminal neuralgia is classified as an idiopathic trigeminal neuralgia with no apparent cause and a symptomatic trigeminal neuralgia which is caused by a structural lesion such as brain tumor. Over 80% of the tumors are meningioma, acoustic neuroma, and epidermoid tumors. Symptomatic trigeminal neuralgia can not be excluded even if old-aged patient does not have abnormal neurologic sign and symptom, and good response to pharmacotherapy. Therefore, initial examinations such as MRI or CT are essential to exclude symptomatic trigeminal neuralgia. When compared with CT, MRI, especially gadolinium enhanced MRI, has an increased sensitivity in the detection of intracranial lesions. The most effective medical treatment of trigeminal neuralgia is carbamazepine. The most common side effects of carbamazepine include drowsiness, dizziness, unsteadiness, nausea, anorexia. Hepatotoxicity, bone marrow depression are the most feared side effect of carbamazepine therapy but occurs rarely. It require periodic complete blood cell counts as well as hepatic and renal function tests. It has been recommended that complete blood cell counts is done every 2 weeks for the first 2months and then quaterly thereafter. Oxcarbazepine can be used if neutropenia occurs.

• Clinical and Experimental Pediatrics
• /
• v.50 no.3
• /
• pp.272-276
• /
• 2007

Purpose : Bell's palsy is defined as an idiopathic facial nerve paralysis of sudden onset. In spite of intensive clinical and experimental investigation, there is still uncertainty in the incidence, etiology, and preferred mode of treatment in children. The objective of this study was to analyze clinical outcome and prognosis of children with Bell' palsy. Methods : We analyzed 61 cases of Bell's palsy diagnosed at the Department of Wonkwang University Hospital from January 1998 to July 2006. The inclusion criteria were any children with acute isolated unilateral lower motor neuron type of facial nerve palsy. The clinical findings and investigations were reviewed including age, sex, affected site, seasonal incidence and result of steroid treatment. Chi-square and Fisher's exact test was used to compare clinical outcome between duration of complete recovery and age. Results : There was no difference in incidence according to sex or age. Incidence was higher in summer and winter. There was no difference in complete recovery rate and duration between steroid treated group and control group. In the group of children younger than 6 years, duration of complete recovery was shorter than older children. Conclusion : We found increasing the incidence of Bell's palsy in summer and winter. Children younger than 6 years had shorter duration in complete recovery.

• Journal of Yeungnam Medical Science
• /
• v.25 no.1
• /
• pp.19-30
• /
• 2008

Osteoporosis, a disease characterized by low bone mass and microarchitectural deterioration of bone tissue leading to enhanced bone fragility and fracture risk, is a major public health problem. The diagnostic methods for osteoporosis include simple radiography, bone scan, DXA (Dual energy X-ray Absortiometry) and biochemical markers of bone turnover. Optimal treatment and prevention of osteoporosis require modification of risk factors, particularly smoking cessation, adequate physical activity, and attention to diet, in addition to pharmacologic intervention. The estrogens and raloxifene both prevent bone loss in postmenopausal women, and the estrogens probably also decrease the risk of first fracture. There is good evidence that raloxifene prevents further fractures in postmenopausal women who already have had fractures and some evidence that estrogen does as well. Bisphosphonate prevents bone loss and reduces fractures in healthy and osteoporotic postmenopausal women and in osteoporotic men as well. Risedronate is more potent and has fewer side effects than alendronate and reduces the incidence of fractures in osteoporotic women. Calcitonin increases bone mineral density in early postmenopausal women and men with idiopathic osteoporosis, and also reduces the risk of new fractures in osteoporotic women. All of the agents discussed above prevent bone resorption, whereas teriparatide and strontium increase bone formation and are effective in the treatment of osteoporotic women and men. New avenues for targeting osteoporosis will emerge as our knowledge of the regulatory mechanisms of bone remodeling increases, although issues of tissue specificity may remain to be addressed.