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At least 145 records · Page 8Linked to original sources

[Cyanosis in children caused by inherited methemoglobinemia due to deficiency of NADH-dependent methemoglobin reductase in erythrocytes].

Two cases of the congenital methemoglobinemia in children due to the deficiency of NADH-dependent methemoglobin reductase in erythrocytes. These children were referred to the Cardiological Ward at the Child Health Centre with suspected cyanotic heart defect. Cardiological examinations excluded heart defect but an increased blood methemoglobin level and decreased activity of NADH-dependent methemoglobin reductase were found, that caused methemoglobinemia. Methylene blue and vitamin C diminished cyanosis. These cases advocate inclusion of methemoglobinemia into differential diagnosis of cyanotic disorders especially if there is no evident pathology in cardio-vascular system.

Adolescent↗

[Atrial septal defect and cyanosis. Apropos of 6 cases related to abnormal drainage of the inferior vena cava into the left atrium].

The authors report six cases of atrial septal defect (ASD) associated with abnormal drainage of the inferior vena cava into the left atrium responsible for right-to-left shunting, without pulmonary hypertension. The abnormal drainage could be due either to an anatomical malposition of the inferior vena cava opening into the left atrium, or to an abnormal blood flow from this vein, normally located through a low ASD, under the influence of anatomical, mechanical and haemodynamic factors. Clinically, all patients presented with light cyanosis and with the usual signs of ASD. None of them had elevated pulmonary pressure. The lesion, suggested by clinical findings, was diagnosed either at angiography, which in four cases demonstrated an abnormal pulmonary venous return, or at colour-coded doppler echocardiogram, or at surgery. In every case, surgical correction consisted of closure of the often low-sited ADS by a patch which diverted the inferior vena cava into the right atrium and the abnormal venous return towards the left atrium. The short--and long-term results of surgery were excellent. The authors review the literature concerning this unusual association of ASD with an abnormal drainage of the inferior vena cava into the left atrium.

Adult↗

Cyanosis and cardiovascular depression in a neonate: complications of halothane anesthesia or phenylephrine eyedrops?

A 3-week-old patient underwent cataract extraction under halothane anesthesia. After induction of anesthesia and instillation of phenylephrine hydrochloride eyedrops, the patient experienced cyanosis and cardiovascular depression. Her condition improved over the next 10 minutes. The possible contributions of halothane and phenylephrine to this response are discussed.

Anesthesia↗

[Toxic methemoglobinemia following the postoperative ingestion of 4,4-diaminodiphenylsulfone (DADPS)--on the differential diagnosis of cyanosis].

Blood gas analysis devices routinely used in clinical practice measure pH, pCO2 and pO2 directly, while all other values they specify are calculated on the basis of nomograms. In the presence of higher concentrations of biologically useless forms of haemoglobin, calculation of the haemoglobin saturation (% O2 Hb) leads to a clinically relevant false assessment of oxygen availability. A case is described in which toxic methemoglobinaemia occurred postoperatively after resumption of long-term medication with 150 mg 4,4-diaminodiphenylsulfone (DADPS). Increasing methaemoglobin values up to maximal 17.7% at a total haemoglobin of 11.3 g/dl correlated with the symptoms of cyanosis and respiratory distress. This symptom complex, which is not atypical postoperatively, could not be clarified by differential diagnosis with the aid of cardiac output measurements and arterial blood gas analyses. The cardiac index amounted to 4.1 l/m2 at a pulmonary capillary wedge pressure of 8 mmHg; the arterial partial oxygen pressure was between 16 and 19 kPa. Only the differential determination of oxihaemoglobin, CO-haemoglobin and methaemoglobin with an oximeter yielded evidence of severe arterial hypoxaemia and made therapy possible.

Adult↗

Evaluation of tcPO2, beat-to-beat heart rate and respiration in neonates with episodes of cyanosis, bradycardia, or apnea.

Episodes of cyanosis, bradycardia, or apnea were studied in 26 newborns and prematures by continuous recording of tcPO2, beat-to-beat heart rate, and impedance pneumography. Hypoxemia related to therapeutic, diagnostic, or care procedures is very common and often is not accompanied by bradycardia or apnea. Idiopathic bradycardia lasting less than 30 seconds will not lead to hypoxemia. However, idiopathic bradycardia of more than 30 seconds' duration can lead to hypoxemia. Beat-to-beat cardiograms and transcutaneous oxygen measurement appear to be the most reliable methods for routine monitoring of these patients.

Apnea↗

The Rombo syndrome: a familial disorder with vermiculate atrophoderma, milia, hypotrichosis, trichoepitheliomas, basal cell carcinomas and peripheral vasodilation with cyanosis.

This hitherto unknown and dominantly inherited disorder is characterized by vermiculate atrophoderma, milia, hypotrichosis, trichoepitheliomas, basal cell carcinomas and peripheral vasodilation with cyanosis. It has been present in the family reported for at least four generations. The lesions become visible in late childhood and are most pronounced on the face. Basal cell carcinomas may develop around the age of 35. Histological observations during the early stage include irregularly distributed and atrophic hair follicles, milia, dilated dermal vessels, lack of elastin or elastin in clumps. After light irradiation a tendency to increased repair activity was observed both in epidermis and in the dermal fibroblasts. On exposure to cold the change in the skin temperature of the hands compared with that in the controls was insignificant. The response to adrenalin iontophoresis was weak.

Abnormalities, Multiple↗

[PFC syndrome (persistent fetal circulation). Central cyanosis in a full-term newborn infant without congenital heart defect].

In a case report the main features of the PFC-syndrome (persistent fetal circulation) are demonstrated: 1. general central cyanosis, 2. pulmonary hypertension, 3. right-to-left shunting via foramen ovale and (or) ductus arteriosus. At postmortem analysis in some of these patients the muscular thickness of the pulmonary vessels was found to be increased: primary PFC-syndrome. Hypoxia, shock, and many pulmonary disturbances of the newborn can produce the clinical picture of "persistent fetal circulation": secondary PFC-syndrome. Under adequate therapy with O2-insufflation, vasodilatory drugs, and if necessary mechanical ventilation the prognosis in the majority of cases is good.

Cyanosis↗

[Central cyanosis in a newborn infant due to a prominence of the eustachian valve].

A 3-day-old neonate with severe central cyanosis was found to have a right to left shunt across a secundum atrial septal defect, due to prominence of the Eustachian valve. Two-dimensional echocardiography with Doppler and color Doppler allowed a precise morphological and pathophysiological diagnosis. Cardiac catheterization and angiocardiography confirmed the diagnosis. The patient is asymptomatic 12 months after surgical excision of the Eustachian valve and closure of the atrial septal defect, performed at two months of age.

Angiocardiography↗

[Another reason for cyanosis--methemoglobinemia].

Intraoperative cyanosis is an utmost emergency for anesthesiologist. If the patient has adequate control ventilation, and normal cardiac pulmonary physiology, then methemoglobinemia must be considered. Reported here is a normal female with dark color lip on the second day after her second parturition and was undergoing tubal ligation. Twenty minutes after induction of general anesthesia and endotracheal intubation, dark blood at the incision site was noted by the operator. After emergent check up of the anesthesia machine, tubings, breathing sound and arterial blood gas, there was only one suspicion left. Methemoglobinemia was confirmed by the hematological examination. Methemoglobinemia is a product from the oxidation of the iron atom in the heme ring when oxygen dissociates from it. This process exists in nature, but can also be induced by nitrate or nitrite-containing drugs or foods or benzene-like organic compounds. Methemoglobinemia can be differentiated from normal hemoglobin by mass spectrometry. If acute illness develops, patients should be treated with methylene blue. Otherwise ascorbic acid will do. This case is reported to remind all anesthesia personnel about one of the rare but serious hemoglobinopathy.

Adult↗

Cytochrome b5 reductase deficiency, an uncommon cause of cyanosis.

A patient with cyanosis due to methaemoglobinaemia caused by cytochrome b5 reductase deficiency is described. Investigation of his family confirmed transmission of this disorder as an autosomal recessive trait. The consequences of this rare condition are discussed.

Aged↗

[Symmetrical cyanosis circumscripta e lipomate].

We report the case of a 78-year-old woman with a symmetrical type of cyanosis circumscripta e lipomate above both knees. Clinical symptoms, pathogenesis and possible treatments are presented.

Aged↗

[Pulmonary arteriovenous fistula. A rare cause of dyspnea and cyanosis in an adult].

The authors report the case of a pulmonary arteriovenous fistula presenting with dyspnoea and cyanosis in a young adult. The malformation was situated on the proximal pulmonary vessels and was cured by surgical correction. The diagnostic value of the clinical signs and complementary investigations of this rare conditions, often detected during adulthood, is discussed. The functional tolerance of the condition is often variable. Oxygen desaturation of arterial blood indicates a right-to-left shunt. Pulmonary angiography enables visualisation of the lesions and guides surgical management. The prevalence of complications of this condition is not well known, but the potential severity is an argument for therapeutic intervention. Embolisation techniques are developing but there is a risk of a systemic embolism. Surgery is the reference for the treatment of proximal fistulae, its curative effect being immediate and definitive.

Adolescent↗

[Right-to-left interatrial shunt with normal pulmonary pressures after pneumonectomy. Apropos of a case with severe cyanosis following left pneumonectomy].

Atrial septal defects usually give rise to left-to right in the absence of obstruction of the pulmonary outflow tract or pulmonary hypertension. The authors report a case of atrial septal defect with a right-to-left shunt despite normal pulmonary pressures at catheterisation in a 56 year-old-man who had undergone left pneumonectomy 6 months previously. The shunt was responsible for major arterial desaturation aggravated by the left lateral or dorsal decubitus position. Surgical closure of the defect resulted in cure with disappearance of cyanosis and normalisation of blood gases. The physiopathological mechanisms of these right-to-left shunts with normal pulmonary pressures are discussed with reference to previously reported cases in the literature.

Blood Gas Analysis↗

[Chronic central cyanosis in children; always an indication for further diagnosis].

A girl and a boy, both aged 4 years, had displayed a blue discolouration of the skin for several years. In the girl, electrocardiography and roentgenography of the chest revealed no abnormalities; in the boy, the cardiac murmur was attributed to an insignificant ventricular septal defect. Further examinations were performed only when the children developed sleeping problems and decrease of exercise tolerance, respectively. In both, a right-left shunt was discovered caused by a direct communication between the right pulmonary artery and the left atrium, and tetralogy of Fallot, respectively. Both patients' condition improved after operation. Chronic central cyanosis in a child constitutes an indication for consultation of a paediatric cardiologist.

Arteriovenous Fistula↗

[A case of two-chambered right ventricle complicating severe cyanosis due to tricuspid regurgitation and patent foramen ovale].

A 63-year-old female of two-chambered right ventricle (TCRV) associated with tricuspid regurgitation (TR), patent foramen ovale (PFO) and small ventricular septal defect (VSD) underwent corrective surgery successfully. She suffered severe heart failure and cyanosis with 47.7% of right-to-left shunt through PFO. The VSD was so small that no significant shunt was shown in catheterization data. Operative findings suggested that TR was caused by elongation of the chorda as a consequence of long-term pressure load of right ventricular inflow chamber. Among reported cases of TCRV, the present case is the oldest one who underwent corrective surgery successfully.

Female↗