Critical care issues: portopulmonary hypertension.
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Biomedical subjects
Publications and source records attributed to M S Mandell.
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BACKGROUND: Living donor liver transplantation has gained wide acceptance as an alternative for children with end-stage liver disease. The standard left lateral segment used in this operation does not provide adequate parenchymal mass to broaden its application to larger children or adults. METHODS: We report two cases of adult to adult living donor liver transplantation using a right hepatic lobe in patients with chronic liver disease. RESULTS: Both recipients experienced excellent initial graft function and have normal liver function 4 and 9 months postoperatively. Both donors are alive and well and returned to normal life 4 weeks postoperatively. CONCLUSIONS: Our initial experience suggests that this technique is a safe and reliable option for adults with chronic end-stage liver disease. A conservative application of this procedure in the adult population could significantly reduce the mortality on the adult waiting list.
UNLABELLED: Alterations in cerebral blood flow (CBF) are implicated in the etiology of portal-systemic encephalopathy. We hypothesized that CO2 reactivity of the cerebral circulation may be impaired in subjects with chronic liver disease (CLD) who also had subclinical portal-systemic encephalopathy (SPSE). We compared the relationship between PETCO2 and cerebral blood flow velocity in 10 patients with CLD with those of 10 healthy control subjects. Middle cerebral artery mean blood flow velocity (MCAMFV) was measured using transcranial Doppler during rest, hyperventilation, and hypoventilation. The degree of SPSE was quantified by using psychometric testing. Patients with CLD had poorer psychometric test scores compared with control subjects. Patients with CLD had lower PETCO2, MCAMFV, and blood pressure values and higher heart rates, differing from control subjects in all ventilation states. However, CO2 reactivity, the rate of change in MCAMFV to changes in ventilation (expressed as percent change in CBF velocity per mm Hg change in PETCO2) was similar for both groups (4.6% +/- 0.6% vs 4.2% +/- 0.5% for patients with CLD versus control subjects, P = 0.15). IMPLICATIONS: Psychometric test scores in patients with chronic liver disease revealed subclinical impairment compared with control subjects. Transcranial Doppler measurements of middle cerebral artery blood flow with varying PETCO2 were conducted, but the CO2 response of patients with liver disease was within the range of control subjects.
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Early tracheal extubation has been safely performed after large operative procedures, questioning the need for routine postoperative ventilation. Because immediate postoperative tracheal extubation of liver transplantation patients has not been previously reported, we performed preliminary studies at two institutions to evaluate potential risk and cost benefit. At the University of Colorado (UC), extubation criteria were derived from the retrospective analysis of patients who were ventilated less than 8 h and experienced an intensive care unit stay less than 48 h in 1994. Preoperative criteria for age, severity of illness, and absence of encephalopathy and coexistent disease were used in a subsequent prospective study in 1995. Donor graft function, blood use, hemodynamic stability, and alveolar-arterial oxygen gradient served as intraoperative criteria. Cost of intensive care services was compared for the 1994 ventilated patients and the 1995 patients whose tracheas were extubated immediately postoperatively. At the second institution, University of California at San Francisco (UCSF), patients were tracheally extubated immediately postoperatively, based on clinical judgment by the anesthesiologist. A retrospective analysis was then completed. Sixteen of 67 patients at UC and 25 of 106 patients at UCSF were tracheally extubated. There were no reintubations at UC, while 2 of 25 patients at UCSF required reintubation. Prior encephalopathy, poor donor liver function, and an increased alveolar-arterial oxygen gradient were present in the patients who suffered perioperative respiratory failure. Seventeen of 25 patients at UCSF did not have all criteria used at UC but did not require reintubation. Wider limits on age and severity of illness did not preclude successful extubation. Cost analysis at UC showed a significant reduction in intensive care unit services and associated cost for extubated patients. We conclude that immediate postoperative tracheal extubation of selected liver transplantation patients is safe and cost effective.
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Pulmonary hypertension develops in approximately 2% of patients with portal hypertension. Diagnosis is often difficult and requires a high degree of clinical suspicion. Treatment of patients with portal and pulmonary hypertension is limited, and mean survival following diagnosis is approximately 15 months. The effect of liver transplantation on the natural history of disease is discussed.
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Physicians who encounter a patient with a genetic disorder are under the same legal obligation to that patient as they are to each patient whom they treat. They are required to possess sufficient knowledge, training, and skill with respect to the particular subject to provide proper medical services in a competent manner. Physicians are required to act reasonably in accordance with the information that they acquire. The courts have held that reasonable conduct includes properly evaluating and diagnosing the medical condition, adequately informing the patient of the consequences of the diagnosis, and alerting other potentially inflicted individuals. The best advice to the practicing dermatologist who encounters a patient with a genetic disorder is that he or she maintain a high standard of medical care, combined with personal respect for each individual patient, which calls for both a sensitive awareness of the profound psychologic implications of the disorder and a thorough, meaningful dialogue encompassing informed consent.
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