• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.16 no.3
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• pp.195-199
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• 2013

Congenital chloride diarrhea (CLD) is a rare inherited autosomal recessive disorder. Mutations of the solute carrier family 26 member 3 gene cause profuse, chloride ion rich diarrhea, which results in hypochloremia, hyponatremia and metabolic alkalosis with dehydration. If a fetal ultrasound shows bowel dilatation suggestive of bowel obstruction, or if a neonate shows persistent diarrhea and metabolic alkalosis, CLD should be considered in the differential diagnosis. The severity of CLD varies, but early detection and early therapy can prevent complications including growth failure. We report a case of dizygotic twins affected by CLD who had been born to non-consanguineous parents. Both of them showed growth failure, but one of the twins experienced worse clinical course. He showed developmental delay, along with dehydration and severe electrolyte imbalance. He was diagnosed with CLD first at 6-month age, and then the other one was also diagnosed with CLD.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.27 no.2
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• pp.88-94
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• 2024

Purpose: Infantile hypertrophic pyloric stenosis (IHPS) is a common gastrointestinal disease in neonates and hypochloremia metabolic alkalosis is a typical laboratory finding in affected patients. This study aimed to analyze the clinical characteristics of infants with IHPS and evaluate the association of clinical and laboratory parameters with ultrasonographic findings. Methods: Infants diagnosed with IHPS between January 2017 and July 2022 were retrospectively evaluated. Results: A total of 67 patients were included in the study. The mean age at diagnosis was 40.5±19.59 days, and the mean symptom duration was 11.97±9.91 days. The mean pyloric muscle thickness and pyloric canal length were 4.87±1.05 mm and 19.6±3.46 mm, respectively. Hyponatremia and metabolic alkalosis were observed in five (7.5%) and 36 (53.7%) patients, respectively. Serum sodium (p=0.011), potassium (p=0.023), and chloride levels (p=0.015) were significantly lower in patients with high bicarbonate levels (≥30 mmol/L). Furthermore, pyloric canal length was significantly higher in patients with high bicarbonate levels (p=0.015). To assess metabolic alkalosis in IHPS patients, the area under the receiver operating characteristic curve of pyloric canal length was 0.910 and the optimal cutoff value of the pyloric canal length was 23.5 mm. Conclusion: We found a close association between laboratory and ultrasonographic findings of IHPS. Clinicians should give special consideration to patients with pyloric lengths exceeding 23.5 mm and appropriate fluid rehydration should be given to these patients.

• Journal of Korean Neurosurgical Society
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• v.30 no.5
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• pp.592-599
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• 2001

Objective : The anterior communicating artery(ACoA) is known to be the most frequent location of intracranial aneurysms, but the complex arterial anatomy of the ACoA region makes this aneurysm among the most difficult one to treat. In the treatment of ACoA aneurysms, the direction of aneurysmal fundus is known to be very important in the surgical tactics. All ACoA aneurysms in our series were classified according to its direction, and analyzed the clinical features in order to investigate the prognostic factors influencing upon the surgical outcome. Methods : The authors reviewed 236 cases of ruptured ACoA aneurysms that were operated from 1990 to 1997, were classified according to Pia's classification. Results : The incidence rate of the ACoA aneurysm was 35.1%(236/672). Ventral group was more common than dorsal group, especially in ventro-caudal projection subgroup(36.0%). Poor preoperative clinical grade(Hunt-Hess grade IV and V) patients were more common in dorsal group(13.1%) than ventral group(2.6%). Rebleeding and intracerebral hematoma were more commonly seen in ventral group. However, vasospasm, hydrocephalus, hyponatremia, and intraventricular hemorrhage were observed more frequently in dorsal group. Worse outcome was more common in dorsal group than ventral group, especially in dorso-caudal projection subgroup. Also, poor outcome was identified in patients with intracerebral hematoma, intraventricular hemorrhage, hyponatremia, and hypertension, although statistically insignificant. In cases with the A1 dominancy, there was no difference in surgical outcome between the right and left side approach. The higher the aneurysmal neck from the planum sphenoidale, the worse outcome via pterional approach. Conclusion : It seems that the preoperative clinical grade, aneurysmal direction, and the height of aneurysmal neck, especially in the pterional approach, would be the major prognostic factors, and that intracerebral hematoma, intraventricular hemorrhage, hyponatremia, hydrocephalus and the intraoperative aneurysmal rupture would be the minor prognostic factors.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.23 no.5
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• pp.472-483
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• 2020

Purpose: Sodium is an essential nutritional electrolyte that affects growth. A low serum sodium concentration in healthy premature infants beyond 2 weeks of life is called late-onset hyponatremia (LOH). Here, we investigated the association between LOH severity and growth outcomes in premature infants. Methods: Medical records of premature infants born at ≤32 weeks of gestation were reviewed. LOH was defined as a serum sodium level <135 mEq/L regardless of sodium replacement after 14 days of life. Cases were divided into two groups, <130 mEq/L (severe) and ≥130 mEq/L (mild). Characteristics and growth parameters were compared between the two groups. Results: A total of 102 premature infants with LOH were included. Gestational age ([GA] 27.7 vs. 29.5 weeks, p<0.001) and birth weight (1.04 vs. 1.34 kg, p<0.001) were significantly lower in the severe group. GA was a risk factor of severe LOH (odds ratio [OR], 1.328, p=0.022), and severe LOH affected the development of bronchopulmonary dysplasia (OR, 2.950, p=0.039) and led to a poor developmental outcome (OR, 9.339, p=0.049). Growth parameters at birth were lower in the severe group, and a lower GA and sepsis negatively affected changes in growth for 3 years after adjustment for time. However, severe LOH was not related to growth changes in premature infants. Conclusion: Severe LOH influenced the development of bronchopulmonary dysplasia and developmental outcomes. However, LOH severity did not affect the growth of premature infants beyond the neonatal period.

• Korean Journal of Psychosomatic Medicine
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• v.10 no.1
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• pp.55-60
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• 2002

Central pontine myelinolysis(CPM) and Extrapontine myelinolysis(EPM) are uncommon neurologic disorders associated with osmotic inequality between the extracellular fluid compartment and intracellular fluid compartment in the brain. Myelinolysis can occur in hyponatremia and after rapid correction of hyponatremia. It may be caused by various metabolic disturbances such as chronic alcoholism, malnourishment, cancer, chronic renal failure and organ transplantation. The authors reported a 43-year-old male patient who have received a kidney transplantation because of chronic renal failure due to diabetic nephropathy. The patient manifested psychotic symptoms such as delusion, loosened association, hallucination, inappropriate affect and aggressiveness as a sequele of CPM and EPM. He also showed neurocognitive impairment such as disorientation, memory impairment, decresed intelligence and aphasia. These manifestations are rare in CPM and EPM. We discuss the clinical features, diagnosis, course and management of the patient which may be clinically significant in the neuropsychiatric aspect especially at the consultation-liaison field.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.1
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• pp.66-70
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• 2014

X-linked adrenoleukodystrophy (ALD) is a uncommon metabolic disorder which derived by peroxismal ${\beta}$-oxidation and elevation of serum very long chain fatty acid (VLCFA). VLCFA is mainly accumulated in the myelin of the central nervous system and adrenal cortex, by which the expressed symptoms of this disease are mainly neurologic and endocrinologic (such as adrenal insufficiency). The mutations in the ABCD1 gene causes X-linked ALD, nevertheless its phenotypes and genotypes are poorly coordinated. We report the case of a 12-year-old boy with X-linked ALD who developed vomiting, fatigue and poor oral intake. Severe dehydration and hyponatremia were found in initial physical examination and laboratory test, but his motor/sensory nerve function and mental status were completely normal. We diagnosed ALD with diffuse high-intensity signal in both parietotemporal cerebellar white matter in brain MRI and elevated serum VLCFA. Later, we confirmed a novel c.1635-1G>A (IVS6-1G>A) mutations of the ABCD1 gene. With the discrepancy between its phenotypes and genotypes, various phenotypes could be seen in X-ALD patient. Careful examination and further studies for these patients will be needed.

• Childhood Kidney Diseases
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• v.8 no.1
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• pp.91-95
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• 2004

We report a 2-month-old boy who presented with severe hyponatremia and hyperkalemia secondary to ureteropelvic junction(VPJ) obstruction. By prenatal ultrasonography at 19 weeks of gestation, severe hydronephrosis was found which was confirmed postnatally Pyeloplasty was done on the 45th day of life, and fifteen days after pyeloplasty, non-bilious vomiting, decreased activity and dehydration developed. Severe hyponatremia and hyperkalemia were observed, as a result of elevated serum aldosterone and plasma renin activity. The anterior posterior pelvic diameter(APPD) and Society for Fetal Urology(SFU) grade measured showed no interval change before and after pyeloplasty. Pseudohypoaldosteronism was diagnosed, and 2M NaCl was administrated orally for 7 days. The electrolyte imbalance was corrected, and 8 weeks later, the elevated levels of aldosterone and plasma renin activity were normalized. The left hydronephrosis was improved at 5 months of age. We hereby report a transient pseudohypoaldosteronism secondary to UPJ obstruction with a review of the literature.

• Korean Journal of Veterinary Research
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• v.60 no.3
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• pp.133-137
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• 2020

Diarrhea is the most common cause of death in calves, and remains a major health challenge. Although there are many studies on the related pathogens, the understanding of the clinicopathological changes is limited. This study aimed to identify the pathogens and observe the clinicopathological changes in electrolytes and acute phase proteins (APPs) associated with diarrhea. Blood samples and fecal samples were collected from 141 calves for the determination of APPs, electrolyte and acid-base status and identification of enteropathogens, respectively. Single or co-infections with enteropathogens, including virus (bovine viral diarrhea virus, coronavirus, and rotavirus), Eimeria, Cryptosporidium, and Escherichia coli K99 were detected in both non-diarrheic and diarrheic calves. Levels of APPs such as serum amyloid A, haptoglobin and fibrinogen were comparable between diarrheic and non-diarrheic calves. Hypoglycemia, high blood urea, electrolytes and acid-base imbalance (hyponatremia, hypochloremia, and decreased bicarbonate), and strong ion difference (SID) acidosis showed a significant association in diarrheic calves (p < 0.01). Particularly, significant hyponatremia, bicarbonate loss, SID acidosis, hypoglycemia, and elevated blood urea nitrogen were found in rotavirus-infected calves. Monitoring the clinicopathological parameters of APPs and electrolyte levels could be vital in the clinical management of diarrheic calves.

• Korean Journal of Exercise Nutrition
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• v.25 no.3
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• pp.16-22
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• 2021

[Purpose] Exercise-associated hyponatremia (EAH) is a well-known condition among endurance athletes at low altitudes. The incidence of EAH during ultramarathons at high altitudes warrants further investigation. This prospective observational study was conducted on the participants of the Leadville Trail 100 run, a 161-km race held at a high altitude (2,800 m-3,840 m). [Methods] Venous blood samples were collected before and immediately after the race. The participants completed an electronic survey after the race. Our main outcome measure was the post-race serum sodium ([Na⁺]) level. [Results] Of the 672 athletes who started the race, 351 (52%) successfully completed the event within the 30-hour cut-off. Post-race blood samples were collected from 84 runners (66 finishers). Both pre- and post-race blood samples were collected from 37 participants. Twenty percent of the post-race participants had EAH. Only one post-race participant had a [Na⁺] level of <130 mmol/L. All participants with EAH were asymptomatic. One participant had an abnormal pre-race [Na⁺] level (134 mmol/L). Female participants had a significantly higher rate of EAH than male participants (40% vs. 16%; p=0.039). Age, body mass index, weight changes, race completion status, nonsteroidal anti-inflammatory drug use, and urine specific gravity were not associated with the development of EAH. Lower postrace [Na⁺] levels were associated with higher serum creatine kinase values (R²=0.1, p<0.005). [Conclusion] High altitude (3,840 m peak) does not appear to enhance the incidence of EAH after an ultramarathon footrace. This suggests that ambient temperature (low temperatures reduce risk), sex (female predilection), endurance running, and overhydration are more prominent risk factors for EAH than high altitude.

• Journal of Veterinary Clinics
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• v.18 no.1
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• pp.74-77
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• 2001

A ten-year-old, male cat presented with recent loss of body weight, depression, vomiting, anorexia, polydipsia, and polyuria. General physical findings included depression, weakness, severe dehydration and a strong acetone odor on the breath. A complete blood count and serum biochemical profiles were leukocytosis, hyperglycemia (286 mg/dl), hypokalemia (2.6 mEq/L), hyponatremia, and high serum fructosamine (600 $\mu$mol/L). In blood gas analysis the cat had acidosis (pH 7.127, p$CO_2$26.7 mmHg). In urinalysis glycosuria and ketouria were appeared. On the basis of clinical signs, serum chemistry, blood gas analysis and urinalysis, diabetic ketoacidosis was diagnosed. Treatment included subcutaneous administration of protamine zinc insulin (0.75 U/head) and intravenous administration of 0.9% saline. Potassium phosphate and sodium bicarbonate was added to the fluids. Serum fructosamine for assessment of glycemic control was measured on occasion calls. On day 296, the patient improved clinically and did not experience any problems resulting from diabetic ketoacidosis.