• Clinical and Experimental Pediatrics
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• v.53 no.1
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• pp.93-96
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• 2010

A 47-year-old male patient in whom atrial septal defect (ASD) had been diagnosed 15 years previously was admitted for cardiac catheterization. He had definite cyanotic lips and nail beds and severe pulmonary arterial hypertension (PAH). He had received medical treatment only for the last few years after being diagnosed with Eisenmenger syndrome. After cardiac catheterization, he received iloprost inhalation therapy pre and postoperation and was discharged after successful surgical closure of the ASD.

• Journal of Korean Critical Care Nursing
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• v.14 no.3
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• pp.128-140
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• 2021

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 collected from April to June 2021 via the electrical medical records of 95 patients with therapeutic hypothermia for 72 hours after a cardiac arrest admitted to an acute care unit at a tertiary hospital between January 1, 2016, and December 31, 2019. The data were analyzed using descriptive statistics and a t-test, Mann-Whitney U test, Chi-squared test, Fisher's exact test, and logistic regression using SPSS/WIN. Results : The incidence of peripheral cyanosis was 20%. In the peripheral cyanosis group, peripheral vascular disease, fibrinogen, vasopressor, infection, disseminated intravascular coagulation, acute physiology, chronic health evaluation II score on the second hospital day, nursing intervention, and mortality on the seventh hospital day were higher. The level of fibrinogen and use of vasopressors affected the occurrence of peripheral cyanosis. Conclusion : Considering the influencing variables, careful observation is necessary for patients with high fibrinogen levels and vasopressor use. These results provide basic data to recognize the need for nursing intervention for peripheral cyanosis and encourage nurses to deliver them during therapeutic hypothermia.

• Tuberculosis and Respiratory Diseases
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• v.46 no.3
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• pp.420-425
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• 1999

We report a case of hepatopulmonary syndrome defined as a triad of chronic liver disease, increased alveolar oxygen gradient on room air, and intrapulmonary arteriovenous shunting. Chest rediographs showed bilateral, basilar, medium sized reticulonodular opacities. High resolution CT scand showed multiple centrilobular nodules and branching structures in the subpleural lung that suggested dilatation of lung vessels with abnormally large number of visible terminal branches. $^{99m}Tc$-macroaggregated albumin(MAA) perfusion lung scan showed right to left shunt. Contrast echocardiography demonstrated intrapulmonary vascular shunt without intracardiac shunt.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.30 no.5
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• pp.422-427
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• 2004

Treacher Collins syndrome is inherited as an autosomal dominant trait with variable penetrance. It shows a marked variability even in the same family. This syndrome is developmental defect affecting the branchial arches. It is not usually associated with acute respiratory distress, but has symptoms of microtia, hypoplastic zygomatic bones, hypoplastic mandibular rami, and bilateral coloboma. It usually requires an emergency operation immediately after the birth. We experienced an infant with Treacher Collins syndrome who showed retrognathia, glossoptosis, microtia, and cleft palate. Intermittent cyanosis, depression of the chest, respiratory difficulty associated with airway obstruction, and swallowing difficulty were also observed. To relieve severe upper airway obstruction caused by retrognathia and glossoptosis, we simultaneously performed tongue-lip adhesion and subperiosteal release of the floor of the mouth. The respiratory and swallowing difficulties were relieved and the tongue repositioned anteriorly. We report the present case with a review of the literature.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.8 no.2
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• pp.252-256
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• 2005

The combination of Congenital central hypoventilation syndrome with Hirschsprung's disease, also known as Haddad syndrome, belongs to the family of diseases now designated as Neurocristopathies. We have experienced a case of Haddad syndrome in a male infant who presented with repetitive abdominal distension, bilious vomiting, and sleep apnea. Following colon study and rectal biopsy disclosed the absence of the ganglion cell. And the infant could not be weaned from mechanical ventilation since birth because of the absence of effective, spontaneous respiration during sleep. As he was diagnosed as Haddad syndrome, tracheostomy and ileostomy were performed consecutively. At the age of 4 months, he was relatively healthy but remained ventilator-dependent. We report the first Korean case of Haddad syndrome with a brief review of the related literature.

• Clinical and Experimental Pediatrics
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• v.45 no.10
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• pp.1298-1301
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• 2002

Systemic capillary leak syndrome(SCLS) is a rare disorder of unknown etiology, which is characterized by recurrent attacks of hypotension, hemoconcentration, and hypoalbuminemia. Urinary or enteric loss of protein is not demonstrated. It is often associated with monoclonal gammopathy, but does not manifest multiple myeloma. Since Clarkson et al. described the first case in a 34-year-old woman, about 50 cases have been reported in the literature. However, most of the cases were of adult age, and the mean age of onset in the reported cases was 42.6 years. In literature review, we could refer only one pediatric case of SCLC by Foeldvari et al. in 1995. We report another pediatric case of SCLS.

• Journal of Veterinary Clinics
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• v.32 no.4
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• pp.343-346
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• 2015

A 4-year-old castrated male Dachshund was presented with a chronic history of dyspnea and cyanosis. Complete blood count revealed marked polycythemia with a hematocrit of 79.7%. Thoracic radiographs showed mild right-sided cardiac enlargement. Echocardiography showed a large ventricular septal defect in the left ventricular outflow tract region just proximal to the aortic valve. On Doppler echocardiography, right-to-left shunt flow through the defect was found and confirmed by contrast echocardiography. Based on these diagnostic findings and clinical signs, the dog was diagnosed as right-to-left shunting ventricular septal defect. Phlebotomy and oxygen supplement were performed, but the dog died the day after presentation.

• Clinical and Experimental Pediatrics
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• v.45 no.3
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• pp.383-389
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• 2002

We experienced a case of partial DiGeorge syndrome in a $35^{＋5}$ week premature female infant presented with micrognathia, fish-shaped mouth, beaked nose, nasal regurgitation, obstructive sleep apnea, velopharyngeal insufficiency and late onset hypocalcemic seizures. The chromosome 22q11 microdeletion was found by the FISH method. The lab findings showed serum calcium level of 4.4 mg/dL, ionized calcium level of 0.49 mg/dL, phosphorous level of 7.5 mg/dL, magnesium level of 1.3 mg/dL and PTH-RIA level of <1 pq/mL. Initial treatment was done with 10% calcium gluconate infusion and magnesium sulfate followed by oral calcium gluconate and low phosphorousformula milk feeding. The serum calcium level was normalized in 6 days. Nasal regurgitation, desaturation with obstructive sleep apnea continued. T-cell functions & numbers(CD 3, CD 4, CD 8)were decreased but Ig G/A/M levels were normal. No visible signs of thymus shadow were seen in either chest X-ray & chest MRI. Electrocardiography and echocardiography showed normal heart. Kidney ultrasonographby showed right side mild hydronephrosis. Neurosonography was normal but EEG showed electrical partial seizure. Hearing assessment by BERA showed mild to moderate hearing impairment. Velopharyngoplasty is scheduled for further treatment. A brief review of literature was made.

• Neonatal Medicine
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• v.18 no.2
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• pp.395-398
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• 2011

Sick sinus syndrome (SSS) is a disorder characterized by sinus node dysfunction. Although the condition is most common in the elderly, it can occur in children including neonates and its recognition and treatment are important. The diagnosis of SSS is based on the presence of sinus bradycardia, sinus arrest or exit block, combinations of sinoatrial and atrioventricular nodal conduction disturbances, and atrial tachyarrhythmias documented in the Holter recordings. In most children with SSS, previous history of congenital heart malformation or cardiac surgery is noted. SSS is also seen in the children including neonates without heart disease or other contributing factors, however SSS is most often idiopathic. The treatment of SSS depends on the basic rhythm problem, but generally involves the placement of a cardiac pacemaker. We report a case of SSS in extremely low birth weight infant without congenital heart disease and suggest that the treatment system is necessary for preterm infants with SSS.

• Korean Journal of Veterinary Research
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• v.34 no.1
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• pp.77-83
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• 1994

Three viral strains, causing CPE in porcine alveolar macrophage cell, were isolated from aborted fetus, serum from young pig showing blue-ear sign and lung of suspected pig, respectively. The differential diagnostic results showed no characteristics of Aujeszky's disease virus(ADV), hog cholera virus (HCV), Japanese encephalitis virus(JEV), porcine parvovirus(PPV) and encephalomyocarditis virus (EMCV). However, positive reactions were demonstrated by IFA using monospecific porcine antibodies against Lelystad virus. When the paired sera of experimentally inoculated swine with one of isolate, KPRRSV-l were tested by IPMA, the result indicated that the isolate was related to United States isolate than European LV.