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Biomedical subjects

A Iturrioz Mata

Publications and source records attributed to A Iturrioz Mata.

5 recordsLinked to original sources

[Cystic adenomatoid malformation: late diagnosis of a congenital anomaly].

Cystic adenomatoid malformation (CAM) is a congenital lung malformation characterized by adenomatoid proliferation of alveoli and terminal bronchioli. It is usually diagnosed perinatally, but some silent cases are discovered during childhood or adulthood. We present the case of a 9-year-old girl who presented with chest pain. Chest X-ray showed a cystic image in the upper lobe of the left lung, which was confirmed by computed tomography and magnetic resonance angiography. Lobectomy of the affected segment was performed and histological examination confirmed the diagnosis of CAM type I.

Age Factors↗

[Idiopathic purpura fulminans with transient protein S deficiency].

Idiopathic purpura fulminans produces rapidly progressive hemorrhagic necrosis of the skin with disseminated intravascular coagulation in individuals without known abnormalities of the protein C pathway or acute infections. The disease mainly affects children and in 90 % of cases is preceded by a benign infection. Its pathogenesis involves a temporary autoimmune protein S deficiency that provokes a state of hypercoagulability. We present the case of a previously healthy 2-year-old boy with hemorrhagic skin lesions characteristic of purpura fulminans and disseminated intravascular coagulation without sepsis. Severe, temporary protein S deficiency was confirmed. The patient received daily replacement therapy with fresh frozen plasma for 12 days and anticoagulation with heparin for 3 months. Evolution was favorable. Although the other parameters returned to normal, protein S remained low for 50 days despite treatment. The patient has made a complete recovery.

Child, Preschool↗

[Use of nitric oxide in a newborn child with pulmonary cystic adenomatoid malformation].

Nitric oxide (NO) inhalation therapy relaxes preconstricted pulmonary blood vessels without causing concomitant systemic hypotension and increases oxygen uptake into the blood. NO inhalation is a new treatment for various disorders of neonates (respiratory distress syndrome, persistent pulmonary hypertension, congenital heart disease and congenital diaphragmatic hernia). There is no references of its use in congenital cystic adenomatoid malformation (CCAM). We report a case of a newborn of 33 week's gestation. The infant underwent resection of the CCAM that had occupied the right middle lobe. At the end of the operation, arterial blood gases at a fractional inspired oxygen concentration (FiO2) of 1.0 reveled acidosis and severe hypoxemia probably due to persistent pulmonary hypertension. NO therapy was used for 16 hours with decreased oxygen need and increase of arterial blood oxygen data. According to the extent of the adenomatoid lesion, likely due to the compression of the surrounding tissue, these patients at times postoperatively develop difficulty in oxygenation and ventilation and the changes are similar to those patients with congenital diaphragmatic hernia. The use of NO in these disorders are successfull.

Cystic Adenomatoid Malformation of Lung, Congenita↗