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Anomalous coronary arteries in the bis-diamine-treated embryos are induced by the disruption of epicardial-mesenchymal transformation and subsequent poor development of coronary vasculature. Incomplete hatching of the coronary ostium is associated with abnormal truncal division.

SUMMARY Plasma fibrin/fibrinogen degradation product (FDP) determinations and CT scans were performed in 26 patients on the first, third, and seventh day after head injury. Plasma FDP concentrations were increased in the patients with cerebral contusion shown on CT scans, while the plasma FDP values were normal in the patients with various sized epidural haematomas. Furthermore, the plasma FDP elevation appeared to be more marked and more prolonged in the patients with severe contusion than in those with mild contusion. These findings suggest that the degree of the increase in plasma FDP is proportional to the amount of brain tissue destruction.Coagulation abnormalities after severe head injury have been reported with increasing frequency over the past ten years. Among these abnormalities, disseminated intravascular coagulation (DIC) is the most severe complication and often results in poor outcome. Coagulation abnormalities not so severe as disseminated intravascular coagulation are also found in head injured patients. Fibrin/fibrinogen degradation product (FDP) measurement is one of the most popular coagulation tests, and increased FDP in the plasma indicates accelerated fibrinolysis. We studied the serial plasma FDP values in 26 head injured patients classified into six groups on the basis of serial CT findings. Clinical materials and methodsThe 26 patients studied were admitted within 6 hours of head injury. In all patients the injuries were caused by traffic accident. Age distribution ranged from 10 to 68 years. There were 18 men and eight women. CT scans were carried out as soon as possible after admission, and consequently all of the initial CT scans were performed within 12 hours of the head injury. In addition to the CT scanning, plasma FDP concentration was measured by the method of FDPL (Latex agglutination test).' The patients who had other severe trauma (haemothorax, major bone fracture, etc) were excluded from this study. CT scans were normal in six patients. Subdural effusion was observed in three patients, epidural haematoma in five and cerebral contusion in 12. Of all patients with epidural haematoma, two had a large haematoma showing marked mass effect on CT scans. Craniotomy was performed for evacuation of haematoma in these patients with large epidural haematoma. Of all patients with cerebral contusion, seven had mild and localised contusion and five had severe and extensive contusion. All of the five patients with severe contusion underwent decompressive craniectomy. The other patients were managed only with medical decompression (glycerol, steroids), mild dehydration, and sedation. Serial FDP measurement and CT scans were routinely performed on Days 3 and 7 after injury in all but one patient who died on Day 4. We divided all 26 patients into six groups according to CT findings: normal, subdural effusion, small epidural haematoma, large epidural haematoma, mild contusion, and severe contusion. In each group the relationships between CT findings and FDP values were studied serially. Furtherm...

We describe a Japanese girl with Bernard-Soulier syndrome and 22q11.2 microdeletion. She had viral infections and recurrent thrombocytopenia and hemorrhagic diathesis after cardiac surgery. As congenital heart defects and abnormal immunity are the most common clinical manifestations associated with 22q11.2 deletion, patients with this association may have a greater risk of developing a severe bleeding disorder.