• Clinical and Experimental Pediatrics
• /
• v.50 no.6
• /
• pp.536-542
• /
• 2007

Purpose : We investigated the effects of restricted fluid in the first 7 days of life on the risk of bronchopulmonary dysplasia (BPD) or patent ductus arteriosus (PDA) in very low birth weight (VLBW) infants. Methods : Eighty three VLBW infants who lived more than 28 days were selected. The amount of daily maintenance fluid was determined by calculation of insensible water loss (IWL) and urine output (UO). Seventy to 80 percent of calculated amount was given to the ventilated infants. Subjects were grouped into low (<25th%), moderate (25-75th%), and high (>75th%) fluid groups for the first 24 hours, 3 days and 7 days. Chi square tests analyzed proportions of subjects with or without morbidities across fluid groups. Multivariate logistic regression was used to analyze the effect of fluid intake on BPD or PDA, controlling for factors that are significantly associated with BPD or PDA by univariate analysis. Results : Rates of BPD and PDA were not significantly associated with fluid groups on each time period. The result was the same after controlling for factors that are significantly associated with BPD or PDA by univariate analysis. For the first 3 and 7 days, fluid intakes were positively related with maximal weight loss, urine output and mechanical ventilation duration. Conclusion : In VLBW infants, when given based on needs reflected from IWL and UO versus intake, relatively low fluid intakes in the first week of life do not decrease the risk of BPD or PDA, and vice versa. We suggest that calculation of daily fluid based on IWL and UO is appropriate for VLBW infants.

• Journal of Korean Neurosurgical Society
• /
• v.30 no.5
• /
• pp.611-621
• /
• 2001

Objective : We analyzed the clinical and endocrinological results of the transsphenoidal microsurgery for ACTH secreting pituitary adenomas. Marerials and Methods : From October 1995 to August 2000, 18 patients underwent transsphenoidal microsurgery for Cushing's disease. We analyzed the surgical results of 17 patients, one patient who was previously operated from other hospital was excluded. Age of the patients were 18 to 61 years old(mean 37.7), male to female ratio was 1 : 3.3, and follow-up period was 3 to 50 months(mean 20.3). The selection of candidates for transsphenoidal exploration was based on endocrinologic criteria. Magnetic resonance imaging was the preferred radiologic test. Selective inferior petrosal sinus sampling of adrenocorticotropic hormone futher refined the diagnosis when endocrinologic and radiologic procedures were not definitive. Results : Results of the preoperative endocrinological test were : level of serum ACTH 29.4 to $225{\mu}g/dL$(mean $93.88{\mu}g/dL$) ; serum cortisol 11.9 to $47.5{\mu}g/dL$(mean $27.49{\mu}g/dL$) ; 24-hour urine free cortisol 235 to $1019{\mu}g/day$(mean $571.0{\mu}g/day$). Inferior petrosal sinus sampling for ACTH was performed in 11 patients and all were confirmed by Cushing's disease and we could predict the laterality of the tumor in 9 of 11 patients. We performed transsphenoidal selective adenomectomy in 5 patients, adenomectomy and subtotal hypophysectomy in 2 patients, adenomectomy and partial hypophysectomy in 9 patients, and in the remaining one patient, hemihypophysectomy followed by total hypophysectomy due to remission failure. Fifteen of 17 patients(88.2%) showed endocrinological remission. Glucocorticoid replacement therapy was performed in all the patients who showed remission for 1 to 24 months(mean 5.9 months), and 6 patients received steroid over 6 months. Conclusion : We conclude that the direct demonstration of a tumor in the pituitary gland by MRI is the most important and definitive diagnostic tool and the location of a mass should be confirmed with increased level of ACTH by the inferior petrosal sinus sampling. Transsphenoidal microsurgery is effective treatment modality for Cushing's disease and the immediate postoperative evaluation of the surgical resection of the tumor is very important. The patients should show hypocortisolism, decreased, subnormal serum ACTH and cortisol levels and 24-hours urine free cortisol. We performed 18 transsphenoidal microsurgery for Cushing's disease in 17 patients and 15 patients(88.2%) showed endocrinological remission.

• Journal of Society of Preventive Korean Medicine
• /
• v.3 no.1
• /
• pp.55-65
• /
• 1999

From January 1994 to April 1997, there was 15 survivals who was admitted to the department of internal medicine, Wonkwang Oriental Medicine Hospital in Cheun-ju, after ingestion of paraquat, and treated with Oriental and western medicine therapy. For the patients, I investigated clinical symptoms, gastroduodenoscopy, intake by oral and parenteral route, and output by urine and stool, serum ALP, AST. ALT, Bilirubin, BUN, Creatinine level and urine analysis. On admission day, the LFT level was as follows. The serum mean ALP, AST, ALT, Total Bilirubin and Direct Bilirubin was $10.05{\pm}2.75\;KAU$, $66.67{\pm}9.88\;IU／L$, $43.80{\pm}7.74\;IU／L$, $1.89{\pm}1.22\;mg／dl$ and $1.10{\pm}1.14\;mg／dl$ respectively. After that day, administered Gamdutang and checked the mean LFT level regullary. Until the 3rd day, the mean ALP, AST, ALT, Total Bilirubin and Direct Bilirubin level was $11.01{\pm}3.16\;KAU$, $56.47{\pm}7.19\;IU／L$, $59.00{\pm}7.57\;IU／L$, $2.54{\pm}1.78\;mg／dl$, $1.64{\pm}1.59\;mg／dl$ respectively. From 4th day to 7th day, the mean ALP; AST, ALT, Total Bilirubin and Direct Bilirubin level was $12.51{\pm}3.49\;KAU$, $77.85{\pm}7.17\;IU／L$, $58.00{\pm}9.09\;IU／L$, $2.54{\pm}1.97\;mg／dl$, and $1.80{\pm}1.81\;mg／dl$ respectively. From 8th day to 10th day, the mean ALP, AST, ALT, Total Bilirubin and Direct Bilirubin level was $12.43{\pm}3.14\;KAU$, $41.13{\pm}6.49\;IU／L$, $50.40{\pm}7.17\;IU／L$, $1.66{\pm}1.90\;mg／dl$ and $1.14{\pm}1.50\;mg／dl$ respectively. From 11th day to 14th day, the mean ALP, AST, ALT, Total Bilirubin and Direct Bilirubin level was $12.30{\pm}3.25\;KAU$, $31.07{\pm}3.85\;IU／L$, $43.33{\pm}5.49\;IU／L$, $1.62{\pm}1.95\;mg／dl$, $1.17{\pm}1.71\;mg／dl$ respectvely. On admission day, the mean RFT level as follows. Serum BUN and Creatinine level was $28.73{\pm}5.19\;mg／dl$ and $1.82{\pm}1.27\;mg／dl$ respectively. After that day, administered Gamdutang and checked the mean RFT level regullary. Until the 3rd day, the mean BUN and Creatinine level was $32.12{\pm}5.65\;mg／dl$ and $2.31{\pm}0.45\;mg／dl$ respectively. From 4th day to 7th day, the mean BUN and Creatinine level was $31.07{\pm}5.47\;mg／dl$ and $1.92{\pm}0.79\;mg／dl$ respectively. From 7th day to 10th day, the mean BUN and Creatinine level was $17.47{\pm}3.57\;mg／dl$ and $1.33{\pm}0.59\;mg／dl$ respectively. From 11th day to 14th day, the mean BUN and Creatinine level was $11.93{\pm}3.16\;mg／dl$, $1.27{\pm}0.38\;mg／dl$ respectively.

• Childhood Kidney Diseases
• /
• v.10 no.1
• /
• pp.18-26
• /
• 2006

Purpose : Empirical antimicrobial treatment is indicated before bacteriological results are available for young children with febrile UTI to minimize renal scarring. To ensure appropriate therapy, knowledge of the prevalence of causative organisms and their susceptibility patterns to antimicrobials is mandatory. We performed a retrospective analysis investigating the local prevalence and resistance patterns of uropathogens, primarily E. coli, isolated from community-acquired UTIs. Methods : A total of 103 positive urine cultures from children with febrile UTI collected at Bundang CHA General Hospital from February 2004 to February 2005 were analyzed. Inclusion criteria were fever higher than $37.5^{\circ}C$, significant bacteriuria with single strain growth of at least 10s colony forming units/mL urine, and leukocyturia >5/HPF. Results : E. coli(89.3%) was the leading uropathogen followed by Enterococcus spp.(3.9%) Klebsiella spp.(2.9%), Citrobctcter spp.(1.9%) and Enterobacter spp.(1.9%). E. coli strains revealed a low proportion of antimicrobial susceptibility to ampicillin(AMP; 27.2%) ampicillinsulbactam(AMS; 34.8%) and trimethoprim-sulfamethoxazole(SXT; 65.2%). Susceptibility patterns to cephalosporins were as follows; cefazolin(1st generation; 91.3%), cefoxitin(2nd; 100%), ceftriaxone(3rd; 97.8%) and cefepime(4th; 97.8%). Three E. coli isolates produced ex tended - spectrum beta-lactamase(ESBL). Conclusion : Empirical treatment with AMP, AMS and SXT, which are commonly used in pediatric clinics, is not recommended for childhood UTI due to high incidence of resistance. The high level of susceptibility to cephalosporins makes these drugs reasonable alternatives. However the emergence of ESBL-producers, even though they are quite few, may have an impact on cephalosporin treatment in the future. (J Korean Soc Pediatr Nephrol 2006;10:18-26)

• Korean Journal of Psychosomatic Medicine
• /
• v.31 no.2
• /
• pp.63-71
• /
• 2023

Urinary incontinence (UI), affecting 3%-11% of males and 25%-45% of females globally, is expected to rise with an aging population. It significantly impacts mental health, causing depression, stress, and reduced quality of life. UI can exacerbate psychiatric conditions, affecting treatment compliance and effectiveness. It is categorized into transient and chronic types. Transient UI, often reversible, is caused by factors summarized in the acronym DIAPPERS: Delirium, Infection, Atrophic urethritis/vaginitis, Psychological disorders, Pharmaceuticals, Excess urine output, Restricted mobility, Stool impaction. Chronic UI includes stress, urge, mixed, overflow, functional, and persistent incontinence. Drug-induced UI, a transient form, is frequently seen in psychiatric treatment. Antipsychotics, antidepressants, and other psychiatric medications can cause UI through various mechanisms like affecting bladder muscle tone, altering nerve reflexes, and inducing other conditions like diabetes or epilepsy. Specific drugs like lithium and valproic acid have also been linked to UI, though mechanisms are not always clear. Managing UI in psychiatric patients requires careful monitoring of urinary symptoms and judicious medication management. If a drug is identified as the cause, options include discontinuing, reducing, or adjusting the dosage. In cases where medication continuation is necessary, additional treatments like desmopressin, oxybutynin, trihexyphenidyl, or amitriptyline may be considered.

• Journal of Genetic Medicine
• /
• v.16 no.2
• /
• pp.85-89
• /
• 2019

Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome is a rare autosomal recessive urea cycle disorder. HHH is caused by a deficiency of the mitochondrial ornithine transporter protein, which is encoded by the solute carrier family 25, member 15 (SLC25A15) gene. Recently, government supported Korean newborn screening has been expanded to include a tandem mass spectrometry (MS/MS) measurement of ornithine level. We report a case of a neonate with HHH syndrome showing a normal MS/MS measurement of ornithine level. A female newborn was admitted to neonatal intensive unit due to familial history of HHH syndrome. Her parents were consanguineous Parkistani couple. The subject's older sister was diagnosed with HHH syndrome at age of 30 months based on altered mental status and liver dysfunction. Even though the subject displayed normal ammonia and ornithine levels based on MS/MS analysis, a molecular test confirmed the diagnosis of HHH syndrome. At 1 month of age, amino acid analysis of blood and urine showed high levels of ornithine and homocitrulline. After 11 months of follow up, she showed normal growth and development, whereas affected sister showed progressive cognitive impairment despite no further hyperammonemia after protein restriction and standard therapy. Our report is in agreement with a previous Canadian study, which showed that neonatal samples from HHH syndrome patients demonstrate normal ornithine levels despite having known mutations. Considering the delayed rise of ornithine in affected patients, genetic testing, and repetitive metabolic testing is needed to prevent patient loss in high risk patients.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.16 no.2
• /
• pp.57-61
• /
• 2016

Isovaleric acidemia (IVA) is an autosomal recessively inherited organic acid disorder due to a defect of the enzyme isovaleryl-CoA dehydrogenase in the leucine metabolic pathway. Deficiency of this enzyme results in the accumulation of derivatives of isovaleryl-CoA. In acute illness in IVA, isovaleric acid and its derivatives accumulate and profound metabolic acidosis with ketosis, characteristic pungent body odor, hypoglycemia, and hyperammonemia can be developed. Additionally, recurrent vomiting, failure to thrive, developmental delay, epilepsy and mental retardation are chronic presenting symptoms and signs for IVA. On the result of newborn screening for inherited metabolic disorders, increased levels of isovalerylcarnitine (C5) are shown. However, C5 elevation can be accompanied with short/branched-chain acyl-CoA dehydrogenase (SBCAD) and therapy with certain antibiotics containing pivalic acid. Quantitative measurement of organic acids in urine and acylcarnitine profiles in plasma are necessary to differential diagnosis. Molecular genetic analysis of the IVD gene for IVA and ACADSB is also helpful to confirm IVA and SBCAD deficiency, respectively. Considering that IVA can be associated with significant morbidity and mortality at acute presentation of metabolic crisis, early diagnosis prior to the onset of symptoms by newborn screening enable to introduction of early treatment and prevention of acute and chronic complications.

• Childhood Kidney Diseases
• /
• v.13 no.1
• /
• pp.84-91
• /
• 2009

We experienced a female neonate with congenital nephrotic syndrome (CNS) associated with congenital diaphragmatic hernia (CDH). Because of the rare combination of two conditions, we report this case with literature review. CDH was found immediately after birth and emergency operation was done for hernia repair. But on the next day, generalized edema and oliguria(0.59 mL/kg/hour) was found and her blood chemistry showed hypoalbuminemia (1.6 g/dL), increased BUN (27.7 mg/dL) and serum creatinine( 1.8 mg/dL) along with heavy proteinuria (4+). We started albumin infusion with a bolus of intravenous furosemide. We suspected the neonate had congenital nephrotic syndrome and her 24hr urine protein was 1,816 mg/day. In spite of immunosuppressive therapy, the nephrotic syndrome and renal failure progressed. We started peritoneal dialysis on the day of life 22 but it was not satisfactory. She was complicated by intracranial hemorrhage and multi-organ failure and expired at 34 days of age. Kidney necropsy was performed which showed diffuse mesangial sclerosis (DMS). Her chromosome study revealed 46, XX and her gene study revealed a heterozygous missense mutation, Arg366His, in Wilms tumor suppressor gene (WT1). This case deserves attention on account of the 4th case of CNS with CDH revealing the Arg366His mutation in the WT1 gene and G the 1st case of early onset renal failure without male pseudohermaphroditism and Wilms tumor with CNS, CDH and the Arg366His mutation in the WT1 gene. So, this report gives support to the hypothesis that Arg366His mutation in the WT1 gene can result in CNS and CDH.

• Clinical and Experimental Pediatrics
• /
• v.50 no.12
• /
• pp.1231-1240
• /
• 2007

Purpose : Bartter syndrome is a renal tubular defect in electrolyte transport characterized by hypokalemia, metabolic alkalosis and other clinical signs and symptoms. The aims of this study were to analyze the clinical manifestations and the short- and long-term outcomes of Bartter syndrome. Methods : We retrospectively reviewed clinical history, laboratory finding of blood and urine, renal ultrasonography, and hearing tests of five patients who were diagnosed and managed with Bartter syndrome at Asan Medical Center from April 1992 to May 2007. We also evaluated height and body weight periodically after institution of therapy. Results : All patients had poor oral intake, failure to thrive and polyuria. Three of them had maternal history of polyhydramnios and premature delivery. The mean age at diagnosis was 11.8 months. All children presented with hypokalemia, metabolic alkalosis, hyperreninemia. Their blood pressures were normal. One patient had nephrocalcinosis on renal ultrasonography and all of them had normal result in hearing tests. After treatment with indomethacin or other prostaglandin inhibitors and potassium supplementation,their clinical features improved with catch-up growth and improvement in the development during long-term follow-up. Conclusion : We emphasize that early diagnosis and proper treatment in patient with Bartter syndrome are related to better prognosis.

• Clinical and Experimental Pediatrics
• /
• v.46 no.3
• /
• pp.295-301
• /
• 2003

Glutaric aciduria type 1(GA1) is an autosomal recessive disorder of the lysine, hydroxylysine and tryptophan metabolism caused by the deficiency of mitochondrial glutaryl-CoA dehydrogenase. This disease is characterized by macrocephaly at birth or shortly after birth and various neurologic symptoms. Between the first weeks and the 4-5th year of life, intercurrent illness such as viral infections, gastroenteritis, or even routine immunizations can trigger acute encephalopathy, causing injury to caudate nucleus and putamen. But intellectual functions are well preserved until late in the disease course. We report a one-month-old male infant with macrocephaly and hypotonia. In brain MRI, there was frontotemporal atrophy(widening of sylvian cistern). In metabolic investigation, there were high glutarylcarnitine level in tandem mass spectrometry and high glutarate in urine organic acid analysis, GA1 was confirmed by absent glutaryl-CoA dehydrogenase activity in fibroblast culture. He was managed with lysine free milk and carnitine and riboflavin. He developed well without a metabolic crisis. If there is macrocephaly in an infant with neuroradiologic sign of frontotemporal atrophy, GA1 should have a high priority in the differential diagnosis. Because current therapy can prevent brain degeneration in more than 90% of affected infants who are treated prospectively, recognition of this disorder before the brain has been injured is essential for treatment.