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

OBJECTIVETo examine the number and function of circulating endothelial progenitor cells (EPCs) in children with cyanotic congenital heart diseases (CHD) and study their correlation with serum levels of vascular endothelial growth factor (VEGF) and stromal cell derived factor-1 (SDF-1).

METHODSFifteen children with tetralogy of Fallot (cyanotic group) and 15 age-and sex-matched children with ventricular septal defect (control group) were enrolled. Serum levels of VEGF and SDF-1 were measured using ELISA. Mononuclear cells were isolated from peripheral blood by Ficoll density gradient centrifugation and cultured in vitro. EPCs were identified by immunofluorescence and were counted under a microscope. Modified Boyden chamber assay and the MTT assay were used to measure the migration and proliferation capacities of EPCs. EPCs adhesion ability assay was performed by replating cells on fibronectin-coated dishes, and then adherent cells were counted. The correlations of serum levels of VEGF and SDF-1 with the number and function of circulating EPCs were assessed by linear regression analysis.

RESULTSSerum levels of VEGF (201.42+/-44.74 ng/L vs 113.56+/-35.62 ng/L; P<0.05) and SDF-1 (3.45+/-1.07 ng/L vs 1.05+/-0.99 ng/L; P<0.05) in the cyanotic group were higher than those in the control group. There was a positive correlation between serum levels of VEGF and SDF-1(r=0.675, P<0.01). The number of EPCs (*200 field) in the cyanotic group significantly increased compared with that of the control group (72.2+/-9.73 vs 51.2+/-3.83; P<0.01). The functional activities of EPCs, including proliferation, migration and adhesion capacities, were augmented in the cyanotic group compared with those in the control group. The increased number and function of EPCs and the increased serum levels of VEGF and SDF-1 were consistent in the cyanotic group, with a correlation coefficient of 0.8395, 0.5491, 0.6376 and 0.7392 respectively.

CONCLUSIONSThe number and functional activity of EPCs as well as serum levels of VEGF and SDF-1 increased in children with cyanotic CHD. Serum levels of VEGF and SDF-1 were correlated to the number and functional activity of EPCs. Serum VEGF and SDF-1 together with circulating EPCs may play important roles in the pathology and physiology in these patients.

Chemokine CXCL12 ; blood ; physiology ; Cyanosis ; blood ; Endothelial Cells ; cytology ; physiology ; Heart Defects, Congenital ; blood ; Humans ; Stem Cells ; physiology ; Vascular Endothelial Growth Factor A ; blood ; physiology

The popular term malignant hyperthermia syndrome (MHS) refers to a clinical syndrome classically observed during general anesthesia. It is characterized by a rapidly increasing .temperature and a high mortality rate. A case of MHS was experienced which developed 60 minutes after induction of general anesthesia with thiopental sodium, succinylcholine, halothane, N2O and 02. High fever, muscle rigidity and cyanosis developed and were followed by arrythmia and unstable blood pressure. Anesthesia was terminated and vigorous emergency treatment was attempted. The patient died about 6 hours after induction of anesthesia.
Anesthesia ; Anesthesia, General ; Arrhythmias, Cardiac ; Blood Pressure ; Cyanosis ; Emergency Treatment ; Fever ; Halothane ; Humans ; Malignant Hyperthermia* ; Mortality ; Muscle Rigidity ; Succinylcholine ; Thiopental

Cyanosis ; diagnosis ; Early Diagnosis ; Echocardiography ; Female ; Heart Defects, Congenital ; diagnosis ; Humans ; Infant, Newborn ; Oxygen ; blood ; Pulmonary Artery ; abnormalities ; Syndrome

BACKGROUNDCyanotic patients have potential growth retardation and malnutrition due to hypoxemia and other reasons. Ghrelin is a novel endogenous growth hormone secretagogue that has effects on growth and cardiovascular activities. The aim of this study was to evaluate the plasma level and myocardial expression of ghrelin and insulin-like growth factor-1 (IGF-1) using an immature piglet model of chronic cyanotic congenital heart defects with decreased pulmonary blood flow.

METHODSTwelve weanling Chinese piglets underwent procedures of main pulmonary artery-left atrium shunt with pulmonary artery banding or sham operation as control. Four weeks later, hemodynamic parameters were measured. Enzyme-linked immunosorbent assay for plasma ghrelin and IGF-1 level measurement were performed. Ventricular ghrelin and IGF-1 mRNA expressions were measured by quantitative real-time polymerase chain reaction.

RESULTSFour weeks after surgical procedure, the cyanotic model produced lower arterial oxygen tension ((68.73 ± 15.09) mmHg), arterial oxygen saturation ((82.35 ± 8.63)%), and higher arterial carbon dioxide tension ((51.83 ± 6.12) mmHg), hematocrit ((42.67 ± 3.83)%) and hemoglobin concentration ((138.17 ± 16.73) g/L) than the control piglets ((194.08 ± 98.79) mmHg, (96.43 ± 7.91)%, (36.9 ± 4.73) mmHg, (31.17 ± 3.71)%, (109.83 ± 13.75) g/L) (all P < 0.05). Plasma ghrelin level was significantly higher in the cyanotic model group in comparison to the control (P = 0.004), and the plasma IGF-1 level was significantly lower than control (P = 0.030). Compared with control animals, the expression of ghrelin mRNAs in the ventricular myocardium was significantly decreased in the cyanotic model group (P = 0.000), and the expression of IGF-1 mRNAs was elevated (P = 0.001).

CONCLUSIONSChronic cyanotic congenital heart defects model was successfully established. Plasma ghrelin level and myocardial IGF-1 mRNA expression were significantly up-regulated, while plasma IGF-1 level and myocardial ghrelin mRNA expression were down-regulated in the chronic cyanotic immature piglets. The ghrelin system may be an important part of the network regulating cardiac performance.

Animals ; Cyanosis ; blood ; metabolism ; physiopathology ; Female ; Ghrelin ; blood ; metabolism ; Heart Defects, Congenital ; blood ; metabolism ; physiopathology ; Insulin-Like Growth Factor I ; genetics ; metabolism ; Male ; Pulmonary Circulation ; physiology ; Swine

OBJECTIVEA prospective study was conducted to probe into the relationship between arterial oxygen partial pressure (PaO2) and brain injury during cardiopulmonary bypass (CPB) in infants with cyanotic congenital heart disease (CHD).

METHODEnrolled in the study were 45 cyanotic infants, who were less than three years old and underwent corrective cardiac surgery from August 1(st), 2010 to January 31(st), 2011 at Guangdong General Hospital. All the infants had a pulse oxygen saturation (SpO2) lower than 85% and were randomly allocated into three groups by a specific computer program. In controlled group 1 (G1 group), PaO2 levels were controlled at 80 - 120 mm Hg (1 mm Hg = 0.133 kPa) during CPB; in controlled group 2 (G2 group), PaO2 levels at 120 - 200 mm Hg during CPB; while in uncontrolled group (G3 group), PaO2 levels were at 200 - 400 mm Hg during CPB. Blood samples were collected just before starting CPB, at the end of CPB, and at 3 h, 5 h, and 24 h after CPB (T1, T2, T3, T4, T5) for the determination of serum concentrations of protein S100β, neuron specific enolase (NSE), and adrenomedullin (ADM) by ELISA.

RESULTProtein S100β rose significantly after starting CPB. In group G3, it reached a peak of (699 ± 139) ng/L by the end of CPB, significantly higher than those in groups G1 and G2 [(528 ± 163) ng/L and (585 ± 155) ng/L], and was positively correlated with PaO2 levels (r = 0.526, P < 0.01). NSE levels of group G1 were continuously rising after starting CPB and reached significantly high levels at 3 h or 5 h after CPB [(12.2 ± 3.4) µg/L and (12.3 ± 3.7) µg/L], while those of group G2 rose significantly during CPB [(10.9 ± 4.8) µg/L] and even higher at 3 h or 5 h after CPB [(12.6 ± 5.1) µg/L and (13.2 ± 5.4) µg/L]. NSE levels of group G3 rose significantly during CPB and maintained at a high level [(12.2 ± 5.7) µg/L] afterwards. There was no significant difference in serum ADM concentrations among different time points in each group and among these three groups. All the infants were discharged from the hospital without any obvious nervous symptom and sign.

CONCLUSIONHigh PaO2 during CPB in infants with CHD might cause an increase of serum protein S100β and NSE, indicating that brain injury might become worse with a higher PaO2 and might be positively correlated with PaO2 during CPB.

Cardiopulmonary Bypass ; Child, Preschool ; Cyanosis ; Female ; Heart Defects, Congenital ; blood ; physiopathology ; surgery ; Humans ; Infant ; Male ; Nerve Growth Factors ; blood ; Oximetry ; Oxygen ; blood ; Partial Pressure ; Phosphopyruvate Hydratase ; blood ; Prospective Studies ; S100 Calcium Binding Protein beta Subunit ; S100 Proteins ; blood ; Serum

Development of transfusion-related acute lung injury (TRALI), a non-cardiogenic pulmonary edema, after blood transfusion, is a rare but potentially leading cause of mortality from blood transfusion. We report on a case of TRALI in a 51-year male with acute calculous cholecystitis and liver cirrhosis. As preoperative treatment, he was given ten units of fresh frozen plasma (FFP) for 3 days before the operation. During the transfusion of the 10th unit of FFP, he experienced a sudden onset of hemoptysis, tachypnea, tachycardia, and cyanosis. Bilateral pulmonary infiltration not observed on the chest X-ray at the visit was newly developed. There was no evidence of volume overload but severe hypoxemia. Blood transfusion was stopped and he recovered fully after 8 days of oxygen therapy through a nasal cannula. Although HLA and HNA antibodies were not detected in the donor's blood, HLA antibodies (A2, B57, B58) were detected in the patient's blood. We reported this meaningful case of TRALI that occurred after transfusion of only fresh frozen plasma which did not contain human leukocyte antibody in a patient with HLA antibody.
Acute Lung Injury* ; Anoxia ; Antibodies ; Blood Transfusion ; Catheters ; Cholecystitis ; Cyanosis ; Hemoptysis ; Humans ; Leukocytes ; Liver Cirrhosis ; Male ; Mortality ; Oxygen ; Plasma* ; Pulmonary Edema ; Tachycardia ; Tachypnea ; Thorax

The hepatopulmonary syndrome is defined as the triad of liver disease, an increased alveolar-arterial gradient while breathing room air, and evidence of intrapulmonary dilatation. Other cardiopulmonary abnormalities(such as pleural effusion or decreased lung volumes) are common and may coexist in patients with the hepatopulmonary syndrome. An abnormal dilatation of intrapulmonary capillaries is evidenced by Tc 99m-MAA perfusion scan, contrast-enhanced echocardiography, and pulmonary angiography. We have experienced a case of hepatopulmonary syndrome in a fi7 year old woman with liver cirrhosis who complained of severe dyspnea and cyanosis. Her arterial blood gas analysis (ABGA) showed severe hypoxemia(PaO2 59mmHg), suggesting the hepatopulmonary syndrome, which was confirmed with the contrast-enhanced echocardiography. It showed delayc4 opacification of left side chambers during agitated saline injection', thus, intrapulmonary shunt was confirmed. So we report a case of hepatopulmonary syndrome with a rewiew of literature.
Angiography ; Blood Gas Analysis ; Capillaries ; Cyanosis ; Dihydroergotamine ; Dilatation ; Dyspnea ; Echocardiography ; Female ; Hepatopulmonary Syndrome* ; Humans ; Liver Cirrhosis* ; Liver Diseases ; Liver* ; Lung ; Perfusion ; Pleural Effusion ; Respiration

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 reduced oxygen affinity are an exceptionally uncommon cause of cyanosis in children. Hemoglobin-M (Hb-M) is rapidly auto-oxidized into the met-form resulting in the loss of its O2-binding ability. This hemoglobinopathy is inherited in an autosomal dominant pattern and is characterized by marked cyanosis. Hb-M affects the anesthetic management because of the anomalous absorption spectrum of Hb-M in standard pulse oximetry. Sufficient O2 delivery should be maintained by keeping a high FiO2 and intermittently checking the O2 delivery state using arterial blood gas analysis. We reported our experience of the anesthetic management of a patient with hemoglobin M.
Absorption ; Blood Gas Analysis ; Child ; Cyanosis ; Heart Diseases ; Hemoglobin M ; Hemoglobinopathies ; Hemoglobins, Abnormal ; Humans ; Lung Diseases ; Methemoglobin ; Methemoglobinemia ; Oximetry ; Oxygen ; Sulfhemoglobinemia

This is a report of a successful, rapid two-stage arterial switch operation in an infant with dextrotransposition of great arteries with an intact ventricular septum beyond the appropriate time for arterial switch operation. A 4-month-old female infant was admitted due to severe cyanosis, respiratory insufficiency, and frequent diarrhea secondary to ischemic colitis. Echocardiographic examination presented marked leftward deviation of the interventricular septum, and left and right ventricular pressures as measured by cardiac catheterization were 40/4 mm Hg and 85/2 mm Hg, respectively. Fifteen days after a preparatory operation (pulmonary artery band with modified right Blalock-Taussig shunt), left ventricular-right ventricular systolic pressure ratio increased to 105/90. Arterial switch operation associated with the division of the right Blalock-Taussig shunt and the removal of pulmonary artery band was performed. After the operation, the infant showed normal cardiac function with no postoperative complication.
Arteries* ; Blood Pressure ; Cardiac Catheterization ; Cardiac Catheters ; Colitis, Ischemic ; Cyanosis ; Diarrhea ; Echocardiography ; Female ; Humans ; Infant ; Postoperative Complications ; Pulmonary Artery ; Respiratory Insufficiency ; Ventricular Pressure ; Ventricular Septum*