Hwang, Il Tae;Kim, Kyung Hee;Hwang, Jin Soon;Shin, Choong Ho;Yang, Sei Won
Clinical and Experimental Pediatrics
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v.46
no.8
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pp.795-802
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2003
Purpose : We investigated the hormonal control of OB gene expression and leptin secretion in cultured human visceral adipose tissue. Methods : Visceral adipose tissues were cultured for up to 48 hrs in modified Eagle's medium with varying concentration of hormones : Control(no hormone), bovine insulin(100 nM), Dexamethasone(DEX, 100 nM), growth hormone(GH, 40 ng/mL), insulin+DEX(100 nM each), insulin+DEX+GH(100 nM insulin and DEX, 40 ng/mL GH). Quantitative analysis of leptin mRNA was performed by competitive reverse transcription polymerase chain reaction, and leptin secretion in culture medium was measured by IRMA using a commercial kit. Results : The addition of dexamethasone to the medium significantly increased OB gene expression and leptin secretion(P<0.05). Unlike dexamethasone, insulin did not affect OB gene expression and leptin secretion. Both insulin and dexamethasone, at high concentration, significantly stimulated leptin secretion compared with basal values(P<0.05). Leptin gene expression was not significantly increased by GH treatment alone, however GH, in combination with high concentrations of insulin and dexamethasone, attenuated the stimulatory effects of high concentrations of insulin and dexamethasone. Conclusion : Insulin cannot increase leptin secretion without the presence of dexamethasone. The mechanism suggested is that insulin may increase leptin secretion in cytoplasm only after dexamethasone increases the expression of OB gene. Further studies are necessary to elucidate the mechanism of the action of insulin on leptin secretion after increasing OB gene expression by dexamethasone.
Infantile hypertrophic pyloric stenosis(IHPS), which occurs three of 1,000 live births, is a major cause of nonbilious vomiting of early infancy but its etiology and pathogenesis are still obscure. The operation of pyloromyotomy as described by Ramstedt in 1912 remains the standard of care for the treatment of IHPS. From January 1993 to October 1996, 35 infants with IHPS were surgically treated and the following results were obtained. 1. Thirty-five patients comprised 32 males and 3 females, and the ratio of male to female was 10.7:1. 2. The most prevalent age group was between 2 weeks and 8 weeks. 3. Of 35 infants, first born babies were 23 cases(65.7%). 4. Breast feeding was in 23 cases(65.7%). 5. The body weight percentile at admission was lower than 50 percentile in all 35 cases. 6. Onset of symptoms was predominantly between 1week and 2 weeks in 11 cases(31.4%). 7. All had a history of nonbilious vomting, generally projectile in nature. 8. In the measured serum electrolytes, hypokalemia was noted in 9 cases(25.7%), hypochloremia was observed in 14 cases(40.0%). 9. In the preoperative ultrasonography, the average muscle thickness, diameter, and length of the pylorus were 6.2mm, 12.1mm, and 17.9mm, respectively. 10. Among 35 cases, significant sonographic criteria of IHPS, muscle thickness of 5mm or more, were noted in 26 cases(83.9%). 11. A total of seven associated anomalies were noted in six patients. 12. All 35 cases were treated with Fredet-Ramstedt pyloromyotomy. 13. There were postoperative complications of wound infection in 2 cases. Intermittent nonprojectile vomiting was presented in 8 cases(22.9%) after operation, but one of them was relieved in 13 days and the rest were relieved within one week by adjustment of oral intake.
Infantile hypertrophic pyloric stensosis(IHPS) occurs in three of 1000 live births, and is a major cause of nonbilious vomiting of early infancy. It's etiology and pathogenesis however are still obscure. The operation of pyloromyotomy described by Ramstedt in 1912 remains the standard treatment. From January 1990 to July 1997, 64 infants with IHPS were treated at the Department of Pediatric Sursery, Yeungnam University Hospital. The ratio of male to female was 7:1, and the most prevalent age ranged from 2 weeks to 8 weeks(81.2 %) of age. Fifty-seven infants were first born (57.8 percent). The body weight of all patients at admission was below the 50 percentile. Age of onset of symptoms was between 2 and 4weeks of age in 23 cases(35.9 %). All infants had a history of nonbilious vomting, generally projectile in nature. Hypokalemia was noted in 14 cases(21.9 %) and hypochloremia in 26 cases(40.6 %). In the preoperative ultrasonography, the average muscle thickness, diameter, and length of the pylorus were 6.3 mm, 12.3 mm, and 17.8 mm. A total of 13 associated anomalies were noted in 12 patients. All cases were treated with Fredet-Ramstedt pyloromyotomy. Postoperative wound infection occured in 3 cases. Thirteen cases(20.3 %) presented intermittent nonprojectile vomiting after operation. With control of oral intake vomiting subsided within one week in 63 patients, and in thirteen days in another.
Purpose: Ischemic enterocolitis (IEC) is the total or partial infarction of the intestine in the absence of occlusion of a major mesenteric blood vessel. The purpose of this study was to evaluate the clinical features of IEC in children. Methods: A clinical analysis of 6 patients with IEC who were admitted to the Department of Pediatrics at Pusan National University Hospital, between 1996 and 2005 was conducted retrospectively. Patients were diagnosed with IEC based on clinical characteristics, including radiologic, endoscopic, histopathologic, and intraoperative findings. Results: Four boys and 2 girls between the age of 6 weeks and 6 years were included in this study. Most of the patients were born at term and had a birth weight that was appropriate for their gestational age. The major symptoms of IEC observed included hematochezia or hematemesis (5 cases), vomiting, diarrhea, abdominal pain or irritability (4 cases), as well as abdominal distension and fever (3 cases). IEC occurred in thecolon in 5 cases (2 descending colon, 1 descending and sigmoid colon, 1 sigmoid colon, 1 whole colon) and the duodenal bulb and gastric antrum in 1 case each. The type of the lesions observed includedulcera, which were found in 3 cases, perforation, which was pbserved in 2 cases, necrotic patches, which were observed in 2 cases, stricture, which was observedin 1 cases, and massive membranous desquamation of the epithelium, which was observed in 1 case. Two of the patients received surgical treatment and the remaining four were treated conservatively. None of the patients died. Conclusion: The presentation of IEC varies, and the findings of this study will be helpful in managing patients with IEC.
Purpose: The ultrasonography (USG) and computed tomography (CT) are popular diagnostic tools for the diagnosis of acute appendicitis in children, but there are many debates about their clinical significance. The purpose of this study is to clarify the clinical significance of USG, CT and follow-up CT performed subsequently to USG, especially in perforated acute appendicitis in children. Methods: We have reviewed 419 cases of surgically confirmed acute appendicitis in children under the age of sixteen, who had been treated in Inje University Ilsan Paik Hospital from March 2002 to February 2006. All the clinical data including the results of USG and CT were collected and analyzed. Results: Sensitivity, specificity, positive and negative predictive values of USG were 98.7%, 96.8%, 98.1%, 97.8% in non-perforation group and 90.8%, 100%, 100%, 81.9% in perforation group. Those of CT were 96.4%, 100%, 100%, 96.5% and 86.6%, 100%, 100%, 87.5% respectively. Those of follow-up CT after USG were 100%, 100%, 100%, 100% and 87.5%, 100%, 100%, 92.0% respectively. The duration of using antibiotics in seven patients showed positive correlation with the interval between two imaging studies (r=0.0472, p=0.019). There was no statistical significance of correlation when these imaging studies performed within 30 hours together. Conclusion: In most of the cases, single choice between USG and CT would be enough to diagnose the acute appendicitis in children. But, it may be helpful to perform CT as early as possible subsequently to USG when there is discrepancy between initial USG and clinical impression.
In order to determine the dose-response relationship of ethanol on blood pressure and renal function, 2 doses of ethanol were intubated into albino rats. For a direct measurement of arterial blood pressure, a polyethylene catheter(PE 10) was implanted in the abdominal aorta, and the other end of the catheter was pulled out of the back of the neck. The experiment was conducted after the rats recovered from the surgery. After emptying their bladders, the rats were placed in a metabolism cage. Mean arterial pressure (MAP) was measured and arterial blood samples were collected through the catheter. Following the collection of the control urine sample, 1 ml of 10 g% (low dose), or 30 g% (high dose) of ethanol/100 g BW was intubated. 1 ml of water/100 g BW was intubated into the control group. MAP and blood samples were taken every hour, and urine samples were collected every 90 min for 3 hours. Blood alcohol concentrations reached a peak at 1 hour (low dose: $105.0{\pm}7.5$, high dose: $214.7{\pm}20.2\;mg%$) and decreased linearly thereafter. Following alcohol ingestion, MAP began to decrease at 15 min and remained at a significantly low level thoughout the 3 hours experimental period(low dose: $112{\pm}2{\rightarrow}102{\pm}4$, high dose: $117{\pm}2{\rightarrow}100{\pm}8\;mmHg$). Urine Flow increased markedly during the first 90 min of ethanol ingestion (low dose: $0.88{\pm}0.20{\rightarrow}1.04{\pm}0.22$, high dose: $0.56{\pm}0.11{\rightarrow}1.35{\pm}0.18\;ml/1.5\;hr$) and decreased during the second 90 min period(low dose: $0.25{\pm}0.06$, high dose: $0.22{\pm}0.06\;ml/1.5\;hr$). Urine flow of the control group decreased gradually during the experiment $(0.88{\pm}0.10{\longrightarrow}0.59{\pm}0.09{\rightarrow}0.45{\pm}0.09\;ml/1.5\;hr)$. These results indicate that the blood-pressure-lowering and diuretic effects of ethanol are dose-related: higher doses of ethanol produce a greater decrease in blood pressure and greater diuresis.
Purpose: We designed this retrospective study to establish the incidence of diagnosic delay in children diagnosed with acute appendicitis and to identify associated factors with delayed diagnosis and its impact on the clinical course. Methods: All cases of children under 15 years of age who underwent appendectomy from 1996 to 2001 at Gangneung Asan Hospital were reviewed. We reviewed signs and symptoms, type of health professional first contacted, the advice given by the health professional and a history of appendicitis in first degree relatives. Diagnostic period is the time elapsed between first complaints and definitive diagnosis. Delay was defined as diagnostic period exceeded the 48 hours. Postoperative course and complications were also reviewed. Results: Incidence of diagnostic delay differed by whether diarrhea and fecalith on X-ray were present. Also children whose parents were advised to observe them at home were more likely to have a diagnostic delay. In almost half of the cases in delayed group, initial diagnosis was not acute appendicitis but gastroenteritis. The perforation rate in non-delayed group was 22%, whereas 87% in delayed group. The delayed group showed a higher number of postoperative complication and a longer hospitalization period. Conclusions: Diarrhea with abdominal pain and fever in children should not be dismissed as gastroenteritis, respiratory infections or other common disorders. Our study suggests that physicians have a responsibility to prevent diagnostic delay and resultant perforation of acute appendicitis in children by having a high index of suspicion about acute appendicitis.
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.
A 9-year old, castrated male Pekingese dog weighing 6.9 kg was referred with one week history of dyspnea, depression, yellow vomiting and abdominal distension. Refractory right lung field opacity was diagnosed as spontaneous right cranial lung lobe torsion (LLT) with collapsed middle lobe on the computed tomography. Four days after diagnosis, right fourth intercostal thoracotomy was performed under propofol-remifentanil based total intravenous anesthesia (TIVA). Right cranial and middle lung lobes were twisted together in a clockwise direction. ENDOPATH-ETS-FLEX-45, an articulating endoscopic linear cutting stapler was applied at the hilus and there were no complications after resection. The patient recovered favorably and has been doing well up to one year follow up. Lung lobe torsion is an uncommon condition in small breed dogs, this case report suggests serial lung lobe torsion of cranial lobe followed by middle lobe in small breed dog, and immediately after diagnosis surgical intervention is beneficial to prevent progression of LLT. Furthermore, using self cutting stapling device and TIVA technique will be helpful for lung lobectomy in dogs.
Purpose: Ingested foreign bodies present a common clinical problem. It is well known that most of them pass uninterrupted through the gastrointestinal tract. We evaluated the role of endoscopy and Foley catheter for removal of foreign bodies in the gastrointestinal tract. Methods: We investigated retrospectively 60 cases with foreign bodies in the gastrointestinal tract. They had been treated at Wonju Christian Hospital, Yonsei University of Korea, from January, 1996 through December, 1999. Results: The age of the patients ranged from 7 months to 13 years. Patients under 5 years were 57 cases (97%) and there was no significant difference in sex (M : F=1.07 : 1). 45 cases of the patients had no symptom. The most common foreign bodies were coins (43 cases). The most common location was esophagus (31 cases). The number of foreign body removal using flexible endoscopy and Foley catheter was 22 (36.7%) and 18 (30.0%) cases, respectively. In 18 cases (30.0%), foreign bodies passed spontaneously. Only 1 case (1.7%), curtain pin impaction at ileocecal region, required surgery. Conclusion: Early foreign body removal from esophagus and stomach is recommended to lessen the morbidity and complication. Fluoroscopic foley catheter technique and flexible endoscopy for removal of esophageal foreign bodies in children is safe and effective.
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