Life tables for cystic fibrosis.
The past 5 years have seen a continuing improvement in life expectancy in cystic fibrosis.
Biomedical subjects
Publications and source records attributed to M J Robinson.
The past 5 years have seen a continuing improvement in life expectancy in cystic fibrosis.
The impairment of mucociliary transport by dry air breathing and the restoration of function with subsequent humidification of inspired air were investigated in anesthetized dogs. Tracheal mucous velocity was measured by a cinebronchofiberscopic technique. The breathing of dry air through an uncuffed endotracheal tube produced almost complete cessation of the flow of tracheal mucus after 3 h. Subsequent breathing of air at 38 degrees C with 100% relative humidity restored tracheal mucous velocity to control values by the end of and additional 3 h. Histologic examination of the trachea at the end of the 3-h dry air breathing period revealed focal areas of sloughing of the ciliated epithelium and submucosal inflammation. Although morphometry was not employed, the inflammatory changes appeared to have progressed during 3 h of breathing fully humidified air subsequent to the dry air breathing period. These findings were consistent with previous reports that the inflammatory response to injury of the tracheobronchial mucosa might be delayed and that the mucociliary transport system has a great deal of functional reserve. We found that an artificial heat and moisture exchanger placed on the proximal end of an endotracheal tube partially protects against the suppression of tracheal mucous velocity caused by dry air breathing.
The echocardiographic features of a patient with severe aortic regurgitation due to a "floppy" aortic valve are presented. The salient abnormality observed was marked fluttering of the aortic valve cusps in diastole. Fluttering of the anterior leaflet of the mitral valve and left ventricular enlargement were noted. The diagnosis of a "floppy" aortic valve was substantiated at surgery.
Observations were made on 26 infants delivered by elective caesarean section under general anaesthesia. A standard anaesthetic technique was employed using a methohexitone, relaxant, nitrous oxide-oxygen sequence with regulated ventilation and the administration of papaveretum after clamping the umbilical cord. In 12 patients the induction dose of methohexitone was 1.4mg/kg and in 14 it was reduced to 1.0 mg/kg. There were no significant differences between the two groups in the clinical status of the mothers, in operative technique and timing, or in the value of PO(2), PCO(2), and pH in the umbilical cord venous blood.The infants whose mothers received the lower dose of methohexitone were in better condition, as assessed by the number needing assisted ventilation, the time taken to establish regular respiration, the Apgar score, and the "Apgar minus colour" score.
Among 69 patients with PVT, 338 variceal bleeding episodes occurred. Only two patients died from bleeding, and both lived in remote communities and were inaccessible to medical care. Fifty-three children underwent 164 operations for the management of PVT. Once operative management was undertaken, subsequent operations frequently were necessary. Nonoperative measures controlled acute variceal hemorrhage in most instances during the past 10 years. Almost all patients who underwent splenectomy alone, variceal ligation, gastric division, splenic transposition, or makeshift shunts subsequently rebled. These operations are rarely indicated in the current management of children with PVT. Portal venography is essential to define the portal venous circulation before a shunt operation is attempted. Cavomesenteric or central splenorenal shunts prevented further bleeding in eight of 15 patients and are the most reliable operations to control bleeding in patients with PVT. Emergency operation is rarely necessary to control bleeding. Sixteen patients (average age 14.6 years) with PVT did not undergo any operations, and are alive. Each of the six patients with PVT who died from complications of portal hypertension did so within nine months of an operation. Four of these patients had previous splenectomy and died with sepsis as one of the major factors. Bleeding episodes became less frequent as the patients increased in age. Patients who underwent shunts under unfavorable circumstances or who received various other operations to treat portal hypertension appeared to have a higher risk of morbidity and mortality than those managed nonoperatively.
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