11/24. Congenital pulmonary arteriovenous fistula: a rare cause of cyanosis in childhood.Two children (both females) aged 15 months and 4 years are described as very rare cases of central cyanosis in childhood being caused by a congenital pulmonary arteriovenous fistula. The initial diagnosis was made based on cyanosis and chest radiographs, with normal physical, ECG, and radiological findings of the heart. They had no family history of the Rendu-Weber-Osler syndrome. The patients underwent cardiac catheterization and pulmonary angiography, where the diagnosis was confirmed. After the surgery, both were symptom-free, and had no evidence of the disease.- - - - - - - - - - ranking = 1keywords = chest (Clic here for more details about this article) |
12/24. Hypoxia and cyanosis in alpha-1-antitrypsin deficiency. Cirrhosis as an unusual etiologic factor.A patient with type PiZZ alpha-1-antitrypsin deficiency was found to have severe hypoxia despite normal pulmonary function testing and a normal chest radiograph. A nuclear medicine ventilation-perfusion study revealed a right-to-left shunt. Computed tomography showed minimal bleb formation, no diffuse changes, and hepatic changes of cirrhosis with portal hypertension. No nodular pulmonary masses or enlarged peripheral pulmonary vessels were found. The diagnosis of diffuse intrapulmonic arteriovenous shunts ("pulmonary spiders of cirrhosis") was suggested and then confirmed with a dynamic radionuclide flow study.- - - - - - - - - - ranking = 1keywords = chest (Clic here for more details about this article) |
13/24. Sudden and unexpected death between 1 and 5 years.Of a population of 9856 children followed up from birth, 9251 of whom underwent 24 hour tape recordings of electrocardiograms and abdominal wall breathing movements during early infancy, five died suddenly and unexpectedly at home at ages ranging from 16 months to 4 years. Postmortem examination, including full histological and microbiological investigations. failed to identify abnormalities ordinarily associated with death in all five cases. Two of the children were known to have had frequent cyanotic episodes and died during these events. In the three remaining cases there was no previous history of cyanotic or apnoeic episodes. The death of one of these three children was seen by his parents and the clinical features suggested that apnoea rather than a cardiac arrhythmia was the primary mechanism for his death. As in infancy, sudden and unexpected death for which no adequate cause is found at necropsy seems to constitute a major component of mortality between 1 and 5 years.- - - - - - - - - - ranking = 52.970458645553keywords = breathing (Clic here for more details about this article) |
14/24. Respiratory complications of metatropic dwarfism.Two infants with clinical and radiologic features of metatropic dwarfism presented in the neonatal period with episodes of cyanosis. Diagnostic studies to determine the etiology of these spells, including electrocardiogram, electroencephalogram, arterial blood gases, and metabolic and sepsis studies, were unremarkable. Chest roentgenograms revealed the characteristic long, narrow thoracic cage with no evidence of parenchymal disease. Cervical spine stability evaluation, pulmonary function studies, and chest impedance monitoring with qualitative air flow thermistor studies and transcutaneous oxygen monitoring were carried out. Both patients demonstrated a significant increase in resistance of the respiratory system following passive maneuvering of the head from a neutral position, suggestive of hypopharyngeal air flow obstruction. Obstructive sleep apnea resulting in cyanosis was documented in both patients. All other studies failed to yield a cause for the episodes of cyanosis. Our investigation failed to alter the clinical course which resulted in respiratory arrest and death by 7 months of age. A table is presented for the differentiation of skeletal dysplasias presenting in the perinatal period.- - - - - - - - - - ranking = 1keywords = chest (Clic here for more details about this article) |
15/24. Traumatic pulmonary artery--left atrial fistula: an unusual case of cyanosis in an adult.Eighteen months after sustaining a stab wound to the left upper chest, a 59-year-old man presented with cyanosis and extertional dyspnea. Arterial desaturation due to a central 22 per cent right-to-left shunt was present. A selective pulmonary arteriogram demonstrated a fistula between the main pulmonary artery and the left atrium. At operation the fistula was closed. A laceration of the pulmonic valve and healed pericarditis were present. Marked symptomatic improvement followed the operation, but a murmur of pulmonic valvular regurgitation persisted. The fistula and laceration of the pulmonic valve were probably traumatic in origin.- - - - - - - - - - ranking = 1keywords = chest (Clic here for more details about this article) |
16/24. A polygraphic study of one case of primary alveolar hypoventilation (Ondine's curse).We report here on a case of primary alveolar hypoventilation in a 9 yr old child. From the age of 8 years, the patient has suffered from episodes of bronchopneumonia associated with severe respiratory insufficiency and lethargy. After recovery, cyanosis developed during the night and, later on, during the day. On two occasions, serious respiratory depression followed ketamine sedation for cardiac catheterization and total anaesthesia for cerebral angiography. Pulmonary function tests showed normal volumes and normal mechanics of breathing; blood gas analysis revealed a slight hypercapnic acidosis and hypoxia. The ventilatory response to CO2 was virtually absent, whereas voluntary hyperventilation normalized blood gas values. A polygraphic recording during sleep showed a marked worsening of hypoventilation, which occurred soon after falling asleep and continued throughout all sleep stages; sporadic central apnoeas, at times prolonged, were recorded only during light sleep. The patient, now 14 yr old, is maintained in satisfactory condition with low flow nocturnal oxygen administration combined with the use of a body respirator during sleep twice a week.- - - - - - - - - - ranking = 52.970458645553keywords = breathing (Clic here for more details about this article) |
17/24. Right pulmonary artery to left atrium communication. An unusual cause of cyanosis in the newborn.A one-day-old newborn infant presented with intense cyanosis, a continuous murmur, and mild congestive heart failure. The chest roentgenogram showed an abnormal right-heart border, and the echocardiogram demonstrated enlargement of the left ventricle and left atrium. cardiac catheterization and angiography demonstrated a right pulmonary artery to left atrium communication. The infant responded favorably to medical management and is asymptomatic with the exception of mild cyanosis with crying. Right pulmonary artery to left atrium communication is a rare but potentially correctable cause of cyanosis in the newborn.- - - - - - - - - - ranking = 1keywords = chest (Clic here for more details about this article) |
18/24. Neonatal coronary thrombosis.We have described a case of coronary thrombosis in a neonate. The diagnosis should be considered in a term infant who presents with a shock-like state with poor perfusion soon after birth. The chest roentgenogram may vary from a normal pattern to generalized cardiomegaly with pulmonary congestion. The electrocardiogram may show a pattern varying from severe right ventricular hypertrophy to anterior left myocardial injury. prognosis is usually poor, and the infant may die within 24 hours after delivery. The etiology of the thrombus formation remains obscure.- - - - - - - - - - ranking = 1keywords = chest (Clic here for more details about this article) |
19/24. Direct communication of the right pulmonary artery with the left atrium in an infant.An 8-month-old Japanese girl with cyanosis and evidence of a round shadow connected with the right cardiac border on the chest x-ray film was diagnosed as a case of direct communication of the right pulmonary artery with the left atrium (RPA-LA communication) following cardiac catheterization and angiocardiography. She had a history of transient congestive heart failure in the early neonatal period. A secundum atrial septal defect and a two-lobed right lung were also present. The patient became asymptomatic after a successful ligation of the anomalous connecting vessel.- - - - - - - - - - ranking = 1keywords = chest (Clic here for more details about this article) |
20/24. Echocardiographic diagnosis of pulmonary embolism in childhood.pulmonary embolism is a rare problem in the pediatric age group. As in adults, symptoms include tachypnea, chest discomfort, and hypoxia, but the index of suspicion in making this diagnosis in children is low. This report confirms the usefulness of two-dimensional and Doppler echocardiography in the diagnosis of pulmonary embolism, regardless of patient age. Causes of hypercoagulable states in children are also briefly discussed.- - - - - - - - - - ranking = 1keywords = chest (Clic here for more details about this article) |
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