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Breaking Free from Your Fetal Chains: A Case-Based Review of the Literature on Gamma Chain Variant Hemoglobinopathies

Varughese PM, Thomas S, Nampoothiri S, Bendapudi P

Methemoglobinemia is a dyshemoglobinemia characterized by cyanosis and reduced oxygen saturation with increased methemoglobin values. The etiology may be congenital or acquired, with the latter being more common. We report...
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Newborn Periventricular Nodular Heterotopia with Persistent Feeding Cyanosis and Apneic Spell: A Case Report

Hong SJ, Park JE, Sohn YB, Suh YA, Lee JH, Park MS

Periventricular nodular heterotopia (PNH) is a neuronal migration disorder that occurs during early brain development. Patients with PNH may be asymptomatic and have normal intelligence; however, PNH is also known...
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Incidence of Peripheral Cyanosis in Patients with Therapeutic Hypothermia after Cardiac Arrest in the Acute Care Unit of a Tertiary General Hospital

Bang SY, Yi YH

Purpose : This study aimed to identify the incidence of peripheral cyanosis and the characteristics and clinical results of patients with therapeutic hypothermia after a cardiac arrest. Methods : Data were...
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Reopening of the Left Superior Vena Cava in Right Superior Vena Cava Obstruction

Yubbu P, Latiff HA, Leong MC

A 5-year-old child developed progressive cyanosis after a Warden procedure. Cardiac catheterization and CT scan revealed right superior vena cava (SVC) obstruction, reopening of a previously obliterated left superior caval...
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Successful Dual-Patch Closure of a Fistula between the Right Pulmonary Artery and the Left Atrium

Joshi AS, Aironi BD

A fistula between the right pulmonary artery and the left atrium is a very rare congenital anomaly, for which there is no definitive embryogenetic explanation. Patients present with cyanosis or...
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The First Korean Family with Hemoglobin-M Milwaukee-2 Leading to Hereditary Methemoglobinemia

Kim DS, Baek HJ, Kim BR, Yoon BA, Lee JH, Kook H

Hemoglobin M (HbM) is a group of abnormal hemoglobin variants that form methemoglobin, which leads to cyanosis and hemolytic anemia. HbM-Milwaukee-2 is a rare variant caused by the point mutation...
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Anomalous Origin of the Right Subclavian Artery in a Patient with D-transposition of the Great Arteries

Park H, Song J, Huh J, Kang IS, Jun TG, Yang JH

This case report concerns a young patient with an extremely rare combination of d-transposition of the great arteries (d-TGA) and anomalous origin of the right subclavian artery. In our patient,...
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Long-Term Results after Surgical Treatment of Ebstein's Anomaly: a 30-year Experience

Kim MS, Lim HG, Kim WH, Lee JR, Kim YJ

BACKGROUND AND OBJECTIVES: The aim of the study is to evaluate the long-term results after a surgical repair of Ebstein's anomaly. SUBJECTS AND METHODS: Forty-eight patients with Ebstein's anomaly who underwent...
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Ruptured Tricuspid Valve Papillary Muscle in a Neonate with Intractable Persistent Fetal Circulation

Yoon JK, Kim HR, Kwon HW, Kwon BS, Kim GB, Bae EJ, Noh CI, Kim WH

Unguarded tricuspid regurgitation (TR) due to a flail tricuspid leaflet is a rare condition of newborn cyanosis. A high perinatal mortality has been associated with this fatal condition. But, there...
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Atrial Septal Defect with Normal Pulmonary Arterial Pressure in Adult Cyanotic Patient

Kim IC, Kim H, Lee JE, Yoon HJ, Kim JB, Kim JH

A 22-year-old male presented with recurrent stroke, central cyanosis, and dyspnea. Transesophageal echocardiography and cardiac catheterization revealed bidirectional shunt flow through atrial septal defect (ASD) without pulmonary arterial hypertension. The...
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Clinical presentations of critical cardiac defects in the newborn: Decision making and initial management

Lee JY

The risk of mortality and morbidity of patients with congenital heart defects (CHDs) is highest during neonatal period and increases when diagnosis and proper management are delayed. Neonates with critical...
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Pulmonary arteriovenous malformation manifesting with perioral cyanosis and dyspnea on exertion: A case report

Kim YK, Kim JW, Lee G, Han MY

Pulmonary arteriovenous malformations (PAVMs) are direct communications between pulmonary arteries and pulmonary veins, resulting in right-to-left shunts that may cause cyanosis, dyspnea, and digital clubbing. Neurological complications such as intrace...
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A case of congenital vallecular cyst associated with gastroesophageal reflux presenting with stridor, feeding cyanosis, and failure to thrive

Yang MA, Kang MJ, Hong J, Shin SH, Kim SD, Kim EK, Kim HS, Choi JH, Kwon TK, Kim IO

Vallecular cyst is an uncommon but potentially dangerous condition causing stridor and has been associated with sudden airway obstruction resulting in death due to its anatomical location in neonates. It...
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Clinical Usefulness of Esophagopraphy in Term Infant with Oxygen Desaturation during and after Feeding

Yu HJ, Cho KY, Kim JY, Park EA

  • KMID: 2099106
  • J Korean Soc Neonatol.
  • 2006 Nov;13(2):209-215.
PURPOSE:Transient desaturation in fullterms related to feeding may be ignored. But those who are presenting significant cyanosis during feeding without any underlying disease, esophagogastric or laryngopharyngeal reflux must be regarded....
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Anesthetic Implication of Hemoglobin-M: A case report

Huh IY, Choi KT, Cheon MY, Park PH

Cyanosis in children is most often caused by pulmonary disease, or cyanotic heart disease but is rarely caused by hematological problems such as methemoglobinemia and sulfhemoglobinemia. Abnormal hemoglobins with a...
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A Case of Congenital Laryngeal Atresia with Single Umbilical Artery Who Required a Tracheotomy

Wi HS, Baek HS, Oh JW, Yum MK, Kim YJ, Moon SJ, Tae K, Kim CR

  • KMID: 2278633
  • Korean J Pediatr.
  • 2005 May;48(5):557-560.
Congenital laryngeal atresia is a rare cause of airway obstruction that is almost always lethal within a few minutes after birth. Therefore congenital laryngeal atresia should be diagnosed in the...
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Surgical Outcome of Tetralogy of Fallot in Adolt: Implication of Preoperative Cyanosis

Yun TJ, Kim SH, Park SI, Park JJ, Song H, Lee JW, Seo DM, Song MG, Song JM, Kang DH, Song JK, Jang WS, Kim YH, Ko JK, Park IS

  • KMID: 1878440
  • Korean J Thorac Cardiovasc Surg.
  • 2005 Apr;38(4):271-276.
BACKGROUND: We analysed differences in operative methods and postoperative outcome according to the severity of preoperative cyanosis in adult ToF (Tetralogy of Fallot) patients. MATERIAL AND METHOD: From August 1989...
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Congenital Pulmonary Vein Stenosis Manifested by Severe Cyanosis in Infancy

Cho JJ, Park WS, Kang IS, Jun TG, Jung MJ

  • KMID: 2073212
  • Korean J Pediatr.
  • 2004 Oct;47(10):1114-1118.
Congenital pulmonary vein stenosis(CPVS) with anatomically normal connection, a rare anomaly, usually leads to progressive pulmonary hypertension, cardiac failure in infancy, and death if untreated. Most are combined with other...
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The Influence of the Preoperative Chronic Cyanosis on the Cardiac Troponin I in the Pediatric Cardiac Operation

Song JY, Lee SK, Lee JY, Kim SJ, Shim WS

  • KMID: 2335620
  • J Korean Pediatr Soc.
  • 2002 Apr;45(4):498-504.
PURPOSE: The purpose of this study was to evaluate the perioperative myocardial damage in pediatric congenital heart disease with the cardiac specific protein of cardiac troponin I(cTpn-I). METHODS: All 25 pediatric...
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A Case of Congenital Nasal Pyriform Aperture Stenosis

Oh HS, Kim SH, Kim GH, Lee HS, Jang IH, Kim JH

  • KMID: 1605951
  • J Korean Pediatr Soc.
  • 2001 Jan;44(1):99-102.
The cause of congenital nasal pyriform aperture stenosis is unclear. The development of the facial skeleton occurs between the fifth and the eighth week of gestation. It is at this...
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