• Title/Summary/Keyword: Alkalosis

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A novel mutation of CLCNKB in a Korean patient of mixed phenotype of Bartter-Gitelman syndrome

  • Cho, Hee-Won;Lee, Sang Taek;Cho, Heeyeon;Cheong, Hae Il
    • Clinical and Experimental Pediatrics
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    • v.59 no.sup1
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    • pp.103-106
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    • 2016
  • Bartter syndrome (BS) is an inherited renal tubular disorder characterized by low or normal blood pressure, hypokalemic metabolic alkalosis, and hyperreninemic hyperaldosteronism. Type III BS is caused by loss-of-function mutations in CLCNKB encoding basolateral ClC-Kb. The clinical phenotype of patients with CLCNKB mutations has been known to be highly variable, and cases that are difficult to categorize as type III BS or other hereditary tubulopathies, such as Gitelman syndrome, have been rarely reported. We report a case of a 10-year-old Korean boy with atypical clinical findings caused by a novel CLCNKB mutation. The boy showed intermittent muscle cramps with laboratory findings of hypokalemia, severe hypomagnesemia, and nephrocalcinosis. These findings were not fully compatible with those observed in cases of BS or Gitelman syndrome. The CLCNKB mutation analysis revealed a heterozygous c.139G>A transition in exon 13 [p.Gly(GGG)465Glu(GAG)]. This change is not a known mutation; however, the clinical findings and in silico prediction results indicated that it is the underlying cause of his presentation.

A Case of Pseudo-Bartter's Syndrome Due to Hypertrophic Pyloric Stenosis (비후성 유문 협착증에 의한 가성 Bartter 증후군 1례)

  • Kim, Yoon Hee;Lee, Tae Ho;Kim, Hong Bae
    • Clinical and Experimental Pediatrics
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    • v.45 no.11
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    • pp.1430-1434
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    • 2002
  • We report a 3-month old boy admitted to our hospital with Bartter's syndrome like symptoms and laboratory findings, which were vomiting, failure to thrive, hypochloremic and hypokalemic metabolic alkalosis associated with hyperreninemia, hyperaldosteronism and normal blood pressure. However, the urine chloride level was low. Hypertrophic pyloric stenosis was diagnosed through abdominal ultrasonography. Fredet-Ramstedt operation was done after electrolyte correction. After surgery he made a good recovery and gained body weight. The electrolytes maintained within a normal limit without any potassium supplementations after surgery. Differential diagnosis from Bartter's syndrome was made on the basis of a decrease in urine chloride and the non-necessity for potassium supplementation after surgery. It is relatively rare for hypertrophic pyloric stenosis to induce pseudo-Bartter's syndrome. The importance of considering this diagnosis in such cases is discussed.

Changes of Total and Ionized Calcium following Cardiopulmonary Bypass (심폐관류에 따른 혈청칼슘의 변동)

  • 전상훈
    • Journal of Chest Surgery
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    • v.21 no.2
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    • pp.240-245
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    • 1988
  • This study was prospectively planned to realize the reduction of calcium ion in serum along with the cardiopulmonary bypass[CPB], to find out the cause of the reduction, and to verify the justification of the classical methods of calcium replacement. Nine patients with various open heart surgeries by CPB in 1987 wee selected at random. Calcium chloride was added as follows:: For each unit of ACD blood transfusion, 600mg of calcium chloride was added. In case of massive transfusion, 600 mg of calcium chloride was injected every 2 or 3 units of transfusion. On occasions such as weaning from CPB, or following defibrillation, or hypotension, weak myocardial contractility of the heart, calcium chloride was needed in an amount of 10 mg / kg. In ICU, calcium chloride was limited to use in low serum level or in emergency use. Total calcium decreased early bypass and progressively increased above the preoperative value during late bypass and three hours thereafter, Ionized calcium increased during late bypass and three hours following. Total and ionized calcium depicted similar patterns of change during open heart surgery. Decrease of the calcium at the early bypass was thought from reduction of total protein and alkalosis during bypass. Meanwhile, increase of both calciums during the end of surgery was presumably attributable to addition of calcium chloride in priming solution, injections of calcium chloride in the process of termination of bypass. We conclude that enough calcium was replaced by the classical methods of calcium supplement.

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Reninoma: a rare cause of curable hypertension

  • Kim, Ji Hye;Kim, Ji Hyun;Cho, Myung Hyun;Park, Eujin;Hyun, Hye Sun;Ahn, Yo Han;Kang, Hee Gyung;Moon, Kyung Chul;Ha, Il-Soo;Cheong, Hae Il
    • Clinical and Experimental Pediatrics
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    • v.62 no.4
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    • pp.144-147
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    • 2019
  • The most common type of refractory hypertension found in children is secondary hypertension, which is a potentially curable disease. Reninoma, a renin-secreting juxtaglomerular cell tumor, is a rare cause of severe hypertension that is usually diagnosed in adolescents and young adults. Surgical resection of the tumor completely cures the hypertension of patients with reninoma. The typical clinical presentation of reninoma includes hypokalemia, metabolic alkalosis, and features secondary to the increased activation of the renin-angiotensin system without renal artery stenosis. We report a case of reninoma in a female adolescent with a typical clinical presentation, in which surgical removal of the tumor completely cured hypertension. We discuss here the clinical features, imaging studies, and immunohistochemical examination of the tumor used to establish the diagnosis of reninoma and for the management of the condition.

A comparison of traditional and quantitative analysis of acid-base and electrolyte imbalance in 87 cats

  • Chun, Daseul;Yu, DoHyeon
    • Korean Journal of Veterinary Research
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    • v.61 no.4
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    • pp.40.1-40.6
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    • 2021
  • Acid-base disorder is a common problem in veterinary emergency and critical care. Traditional methods, as well as the Stewart method based on strong ion difference concepts and the Fencl-Stewart method, can be used to analyze the underlying causes. On the other hand, there are insufficient comparative study data on these methods in cats. From 2018 to 2020, 327 acid-base analysis data were collected from 69 sick and 18 healthy cats. The three most well-known methods (traditional method, Stewart method, and Fencl-Stewart method) were used to analyze the acid-base status. The frequency of acid-base imbalances and the degree of variation according to the disease were also evaluated. In the traditional acid-base analysis, 5/69 (7.2%) cats showed a normal acid-base status, and 23.2% and 40.6% of the simple and mixed disorders, respectively. The Fencl-Stewart method showed changes in both the acidotic and alkalotic processes in 64/69 (92.8%), whereas all cats showed an abnormal status in the Fencl-Stewart method (semiquantitative approach). The frequencies of the different acid-base imbalances were identified according to the analysis method. These findings can assist in analyzing the underlying causes of acid-base imbalance and developing the appropriate treatment.

A case of chronic licorice intoxication-induced apparent mineralocorticoid excess syndrome (만성 감초 중독으로 유발된 미네랄코르티코이드 과잉증후군 1예)

  • Young Jae Lim;Ji Eun Kim
    • Journal of The Korean Society of Clinical Toxicology
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    • v.21 no.2
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    • pp.151-155
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    • 2023
  • Licorice is a perennial herb belonging to the legume family that mainly grows in northeastern China, Mongolia, Siberia, and other regions. It is used in traditional medicine in the form of dried roots in the East and the West. The main active component of licorice, glycyrrhizin, is known to produce mineralocorticoid effects when consumed chronically, which can lead to apparent mineralocorticoid excess syndrome. Herein, we present the case of a 72-year-old woman who was admitted to the emergency room with severe generalized weakness and difficulty keeping her neck upright, which had developed after daily consumption of licorice-infused water for the past 2 months. Blood tests revealed metabolic alkalosis and severe hypokalemia, and an electrocardiogram showed ventricular bigeminy. The patient was treated with daily potassium and spironolactone supplements, leading to a significant improvement in muscle strength after a week. One week later, the patient was discharged, showing rare ventricular premature contractions on electrocardiography, but with no specific complaints. Chronic licorice ingestion leading to hypokalemia and muscle weakness can be life-threatening, necessitating the discontinuation of the causative agent, close monitoring, and cautious supplementation of potassium and spironolactone as treatment.

Hyperventilation due to Incision & Drainage under Inadequate Psychosedation & Local Anesthesia in Advanced Odontogenic Infectious Lesion (진행성 치성 감염병소에서 부적절한 진정요법과 국소마취 시행하 절개 배농술에 따른 과환기증)

  • Oh, Ji-Hyeon;Son, Jeong-Seog;Yoo, Jae-Ha;Kim, Jong-Bae
    • Journal of The Korean Dental Society of Anesthesiology
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    • v.14 no.1
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    • pp.63-71
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    • 2014
  • Extension of advanced odontogenic infection from deep neck fascial spaces into the mediastinum is heralded by chest pain, dyspnea, fever, and radiographic demonstration of mediastinal widening. The critical care should be done in a team approach by multiple medical and dental departments, such as, oral & maxillofacial surgery, otolaryngology, anesthesiology, chest surgery, and infection medicine. Especially, fluid & drug therapy, adequate incision & drainage and systemic supportive psychosedation care are important. But, acute hyperventilation can be produced by several distinct causes: severe anxiety, respiratory alkalosis, increased blood catecholamine levels, and a decrease in the level of the ionized calcium in the blood. The orofacial fears about acute pain, trismus, dysphagia, swelling and oral surgical treatment lead to the severe anxiety and increased blood catecholamine level by stress. Therefore, the most dental patient should be cared gently as the stress reduction protocol. In spite of the care, hyperventilation was occurred during psychosedation and local anesthesia for incision and drainage of the masticatory fascial space abscess with deep neck infection & mediastinitis. We suggest that the dental patient with advanced odontogenic infection must be attention for the manifestation of hyperventilation, especially in the medically compromised conditions.

Effect of Herbal Medicines Including Licorice on Serum Potassium Levels (감초가 포함된 한약 복용 시 혈중 포타슘 수치의 저하에 영향을 미치는 요인)

  • Bae, Jeong-Hwa;Kim, Jung-Tae;Chung, Sung-Hyun
    • Korean Journal of Clinical Pharmacy
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    • v.22 no.2
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    • pp.153-159
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    • 2012
  • Licorice is a very useful herbal medicine frequently prescribed, but glycyrrhizin, one of its components, can cause pseudoaldosteronism presenting hypokalemia, metabolic alkalosis and hypertension as a result of prolonging a biological half-life of systemic steroid by strongly inhibiting the enzyme $11{\beta}$-hydroxysteroid dehydrogenase type 2. The risk factors for this side effect are still unclear. Here, 479 hospitalized patients taking herbal medicines including licorice for more than five days were recruited to analyze their serum potassium levels, under following parameters ; age and gender of patient, dose and period of licorice, disease state such as hypertension and with or without diuretics. They were divided into the non-occurrence group (over 3.5 mEq/L) and the occurrence group (under 3.5 mEq/L) of hypokalemia based on serum potassium levels during hospitalization. The average ages of the non-occurrence group and the occurrence group were $52.6{\pm}17.8$ and $68{\pm}10.5$, respectively (p < 0.001). But there were no noticeable differences in the daily dose of licorice, days of taking, and total dose between groups. Regression analysis showed that odd ratio of age (>60) and taking diuretics inducing hypokalemia was 3.5 (95%CI 1.5-8.1), 4.6 (95%CI 2.0-10.9) that indicates significant correlation with hypokalemia. Based upon this analysis, it is imperative to monitor regularly the risk of pseudoaldosteronism or hypokalemia not only when administered licorice of high dose for long period but also when combined it with diuretics causing hypokalemia to the elderly patients over 60 year old.

Clinical Experience of Open Heart Surgery Under The Extracorporeal Circulation With Partial Hemodilution: Operation 16 Cases (혈희석 체외순환에 의한 개심수술: 16례 수술 경험)

  • 유회성
    • Journal of Chest Surgery
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    • v.10 no.2
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    • pp.299-314
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    • 1977
  • Clinical experience on 16 cases of open heart surgery under the extracorporeal circulation with mild or moderate hypothermia and partial hemodilution technique at the National Medical Center during the period from June 1976 to October 1977. Nine of sixteen were congenital heart disease and seven were acquired heart disease. The age of the patient ranged between 6 and 48 years. The body weight varied from 18.5kg to 60kg and body surface area 0. 79-1.70m2. The average priming volume of pump oxygenator was 2080 ml, which was consisted fresh ACD blood, buffered Hartmann`s solution, Mannitol, 50% dextrose in water and Vit. C. The average hemodilution rate was 27%. The average flow 2.3 L/min/m2 or 80 ml/min and the duration of perfusion varied from 31 min to 270 min with average of 107 min. The perfusion was carried out under the mild or moderate hypothermia using core cooling alone in 10 cases, core cooling and local myocardial cooling with $0-4^{\circ}C$ physiologic saline in 2 cases. From a hemodynamic point of view, the blood pressure dropped down around 80 mmHg after the initiation of perfusion follwed by increase to safety level and stable during the perfusion. The central venous pressure remained within normal limits. In most cases, hemoglobin and hematocrit decreased during and after the perfusion. Hemogiobin level was decreased, average of 20.6 %, hematocrit 18.6%, pletelets 55% postoperatively. Plasma hemoglobin increased moderately, from preperfusion average valve of 7.79 mg % to post-perfusion value of 54.7 mg %. Electrolytes changes during cardiopulmonary bypass showed definite hypokalemia but changes of Na, Ca were not definite. Arterial blood gas analysis during cardiopulmonary bypass suggested that the metabolic acidosis which was accompanied by respiratory alkalosis which was corrected postoperatively. As the opera tive complication, transient hemoglobinuria in 4 cases and neurological signs in 2 cases were all cured. There were 2 death cases and operative mortality rate was 12.5%.

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Hyperventilation During Local Anesthesia in Acute Odontogenic Infectious Lesion - Report of two cases - (급성 치성감염 병소에서 국소마취 중 유발된 과환기 -증례 보고-)

  • Yoo, Jae-Ha;Kim, Hyun-Sil;Baek, Sung-Hum;Yoo, Tae-Min;Lee, Ji-Woong;Chung, Won-Gyun
    • Journal of The Korean Dental Society of Anesthesiology
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    • v.2 no.2 s.3
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    • pp.107-113
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    • 2002
  • Hyperventilation is defined as ventilation in excess of that required to maintain normal blood $PaO_2$ and $PaCO_2$. It is produced by several distinct causes: anxiety, respiratory alkalosis, increased blood catecholamine levels, and a decrease in the level of the ionized calcium in the blood. The dental fears about acute pain, needle, drill and dental surgery lead to the severe anxiety and increased blood catecholamine level. Therefore, the most dental patient should be cared gently as the stress reduction protocol. In spite of the gentle care, two cases of hyperventilation were occurred during local anesthesia for incision and drainage of acute odontogenic infectious lesions. We suggest that the dental patients with acute odontogenic infection must be attention for the manifestation of hyperventilation, especially in the medically compromised conditions.

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