This report a case of a ten-year-old female with progressive cyanosis and dyspnea on exertion. Clinical and laboratory work up ruled out a cardiac and pulmonary pathology warranting further investigation for possible hemoglobinopathies. Enzyme assay showed deficiency in cytochrome b5 reductase seen in patients with congenital methemoglobinemia. Ascorbic acid at 200mg daily afforded gradual improvement in cyanosis.
Methemoglobinemia ; Cyanosis

No abstract available.
Child* ; Cyanosis* ; Humans

No abstract available.
Arteriovenous Fistula* ; Cyanosis*

The simultaneous bilateral spontaneous pneumothorax is very dangerous, so the chest drain insertion should be done urgency. Contrary to the unilteral pneumothorax, the patient with simultaneous bilateral pneumothorax complains a severe respiratory distress, cyanosis and chest pain without the tension pneumothorax. Now, we present two cases of the simultaneous bilateral pneumothorax. The prompt insertion of chest drains relieved symptoms. One case is resolved by the bilateral thoracotomy, the other by the insertion of chest drains only.
Chest Pain ; Cyanosis ; Humans ; Pneumothorax* ; Thoracotomy ; Thorax

Authors experienced a case of a huge intrathoracic teratoma in the right uppet mediastinum in male 7 months of age who had dyspnes of 1 month of duration, which was aggravated with cough and fever, and cyanosis 2 days prior to admission. He was relieved dramatically by open chest surgery, resecting the tumor measured 8X5X5 cm. The review of literatures was made.
Cough ; Cyanosis ; Fever ; Humans ; Male ; Mediastinum ; Teratoma* ; Thorax

Tricuspid regurgitation(TR) with normal heart structure during fetal life is a frequent functional phenomenon with a prevalence of six to seven percent. Isolated severe congenital TR, however, is an uncommon cause of neonatal congestive heart failure, cyanosis and massive cardiomegaly. We experienced a case of severe transient neonatal TR presented as massive cardiomegaly during fetal life which was normalized in the postnatal period.
Cardiomegaly* ; Cyanosis ; Heart ; Heart Failure ; Prevalence ; Tricuspid Valve Insufficiency*

Congenital methemoglobinemia is caused by NADH-methemoglobin reductase deficiency in more than half of the total reported cases. NADH-methemoglobin reductase deficiency is an uncommon hereditary disorder producing methemoglobinemia and cyanosis in the homozygous subject. A majority of the patients born with these abnormalities have only a cosmetic defect-asymptomatic cyanosis. Congenital methemoglobinemia due to NADH-methemoglobin reductase deficiency is an autosomal recessive disorder and classified into 4 types according to the pathophysiology of the disorder. In type I, the deficiency of NADH-methemoglobin reductase is restricted to erythrocytes of patients with mild cyanosis, and 7 missence mutations have been reported in the case of type I. We report the first Korean pediatric case of type I congenital methemoglobinemia due to NADH- methemoglobin reductase deficiency with a review of the literature.
Cyanosis ; Cytochrome-B(5) Reductase ; Erythrocytes ; Humans ; Methemoglobinemia* ; Oxidoreductases*

Choanal atresia is the congenital failure of one or both posterior nasal apertures to communicate with the nasopharynx. Coexisting congenital anomalies are 20% to 50% of patients. Bilateral choanal atresia almost always presents respiratory distress, sucking difficulty and cyanosis relieved by crying in the newborn. Bilateral choanal atresia in newborns and infants carries significant morbidity and mortality, therefore, prompt correction is required. Athelia is the absence of the nipple-areola complex. It is a rare entity that can be either congenital or acquired. Congenital athelia is always associated with amastia and a syndrome. We report a case of choanal atresia associated athelia, in term baby.
Choanal Atresia* ; Crying ; Cyanosis ; Humans ; Infant ; Infant, Newborn* ; Mortality ; Nasopharynx

PURPOSE: The adverse reactions of prostaglandin E1(PGE1) are troublesome in the preoperative management of critical patients with ductus dependent congenital heart disease, and a preparation with less adverse reactions is preferable. The effects of Lipo PGE1, a new preparation of PGE1 contained in lipid microspheres, were compared with those of conventional PGE1(PGE1-CD). METHODS: Lipo PGE1 was infused at a rate of 5 ng/kg/min in 19 patients, PGE1-CD at a rate between 10 and 50 ng/kg/min in 15 patients. The effects of drugs were assessed in terms of clinical response rate and overall safety. RESULTS: Clinically, both treatment were effective in relieving cyanosis and hypoxemia except in patients already having either a closed ductus or severe hypoxemia and acidosis. The increments of PaO2 1 hour after infusion were 10.9 and 6.2 mmHg (p>0.1), respectively and those 4 hours postinfusion were 16.0 and 7.8 mmHg(p<0.05), respectively. Even though there was no significant difference in clinical response rate(78.9 vs 60.0%, p>0.1), the mean dose of Lipo PGE1 at appearance of response was about 1/5 of that of PGE1-CD in overall patients and also in patients with ductus dependent pulmonary circulation(6.7 vs 31.7 ng/kg/min, p<0.005). The adverse reactions occurred in 52.6% of the patients given Lipo PGE1, while it was 86.7% in those administered PGE1-CD(p<0.05). The adverse reactions in Lipo PGE1 group was much less severe than that in PGE1-CD group. There was a significant difference in overall safety between the two drugs(84.2 vs 40%, p<0.01). As the incidence of the adverse reactions increased at dose over 5 ng/kg/min, the initial dose of 5 ng/kg/min seemed to be appropriate for Lipo PGE1. CONCLUSIONS: Lipo PGE1 was effective at a lower dose than was PGE1-CD, and was associated with fewer or less severe adverse reactions, and is therefore judged to be more suitable for clinical use than conventional PGE1-CD.
Acidosis ; Alprostadil ; Anoxia ; Cyanosis ; Heart Defects, Congenital* ; Humans ; Incidence ; Microspheres

Methemoglobin (MetHb) is an oxidation product of hemoglobin in which the sixth coordination position of ferric iron is bound to a water molecule or to a hydroxyl group. The most common cause of acquired MetHb-emia is accidental poisoning which usually is the result of ingestion of water containing nitrates or food containing nitrite, and sometimes the inhalation or ingestion of butyl or amyl nitrite used as an aphrodisiac. We herein report a case of MetHb-emia after ingestion of an aphrodisiac, later identified as dapsone by gas chromatograph/mass selective detector (GC/MSD). A 24-year old male was admitted due to cyanosis after ingestion of a drug purchased as an aphrodisiac. On arterial blood gas analysis, pH was 7.32, PaCO2 26.8 mmHg, PaO2 75.6 mmHg, and bicarbonate 13.9 mmol/L. Initial pulse oxymetry was 89%. With 3 liter of nasal oxygen supplement, oxygen saturation was increased to 90-92%, but cyanosis did not disappear. Despite continuous supplement of oxygen, cyanosis was not improved. On the fifth hospital day, MetHb was 24.9%. Methylene blue was administered (2 mg/kg intravenously) and the patient rapidly improved. We proved the composition of aphrodisiac as dapsone by the method of GC/MSD.
Administration, Oral ; Adult ; Antidotes/therapeutic use ; Aphrodisiacs/adverse effects* ; Case Report ; Cyanosis/drug therapy ; Cyanosis/chemically induced ; Cyanosis/blood ; Dapsone/adverse effects* ; Human ; Male ; Methemoglobinemia/drug therapy ; Methemoglobinemia/chemically induced* ; Methylene Blue/therapeutic use