Neonatal hepatitis induced by alpha 1-antitrypsin: a transgenic mouse model.

Transgenic mouse lineages were established that carry the normal (M) or mutant (Z) alleles of the human alpha 1-antitrypsin (alpha 1-Pi) gene. All of the alpha 1-Pi transgenic mice expressed the human protein in the liver, cartilage, gut, kidneys, lymphoid macrophages, and thymus. The human M-allele protein was secreted normally into the serum. However, the human Z-allele protein accumulated in several cell types, but particularly in hepatocytes, and was found in serum in tenfold lower concentrations than the M-allele protein. Mice in one lineage carrying the mutant Z allele expressed high levels of human alpha 1-Pi RNA and displayed significant runting (50% of normal weight) in the neonatal period. This lineage was found to have alpha 1-Pi-induced liver pathology in the neonatal period, concomitant with the accumulation of human Z protein in diastase-resistant cytoplasmic globules that could be revealed in the Periodic acid-Schiff reaction (PAS). The phenotype of mice in the strain expressing high levels of the Z allele is remarkably similar to human neonatal hepatitis, and this strain may prove to be a useful animal model for studying this disease.

The safety of anaesthetising biochemically hyperthyroid patients undergoing thyroidectomy-a retrospective cohort study.

Traditionally it is recommended that hyperthyroid patients should be made euthyroid prior to thyroidectomy. However, several small observational studies have reported no increase in adverse events when hyperthyroid patients undergo thyroidectomy. The aim of this study was to assess outcomes following total thyroidectomy in patients who were biochemically hyperthyroid at the time of surgery compared to those who were euthyroid. One hundred and fifty-one eligible patients undergoing thyroidectomy for hyperthyroidism between January 2012 and February 2016 were identified, of whom 57 were hyperthyroid on perioperative blood tests and 94 were euthyroid (comparison group). Primary outcomes were 30-day mortality, increased length of postoperative hospital stay and intraoperative signs consistent with thyrotoxicosis (e.g. heart rate >100 per minute, systolic blood pressure >180 or <60 mmHg, or temperature >38°C). Secondary outcomes were intraoperative beta-blocker use and level of care required postoperatively. Thirty-day mortality was zero. The only significant difference between the two groups was a higher use of intraoperative beta-blockers amongst hyperthyroid patients (28.1% versus 8.5%, P=0.002). Our findings suggest that thyroidectomy for mild to moderate biochemical hyperthyroidism performed by an experienced thyroid surgeon and anaesthetist, is associated with increased intraoperative beta-blocker use but no statistical difference in mortality, length of postoperative stay or intraoperative signs consistent with thyrotoxicosis. While we still recommend attempting to achieve a euthyroid state whenever possible prior to thyroid surgery, mild to moderate degrees of residual biochemical hyperthyroidism when appropriately managed may not be associated with an increase in adverse outcomes.

Delayed recovery of severely "stunned" myocardium with the support of a left ventricular assist device after coronary artery bypass graft surgery.

A 53 year old man underwent repeat coronary artery bypass graft surgery after presenting with unstable angina. Because of intraoperative ischemia, the patient developed profound left ventricular dysfunction requiring placement of a left ventricular assist device and intraaortic balloon pump and catecholamine infusion. Serial radionuclide ventriculograms documented delayed recovery of the severely stunned myocardium with mechanical and pharmacologic support.

Outcome of the native lung after single lung transplant. Multiorgan Transplant Group.

Twenty-one long-term survivors of single lung transplant since 1987 have been followed from 7 to 81 months. Posttransplant complications unique to the native lung and their impact on patient outcome are reported. In 7 of 21 recipients of single lung transplant, clinical complications in the native lung developed, including infection, pulmonary infarction, and severe ventilation-perfusion mismatching. Impact on the patient has ranged from little effect (prolongation of hospital or ICU stay) to recurrent severe infections, the need for surgical intervention, and a possible contribution to the recurrence of original disease--giant cell interstitial pneumonitis. The remaining native lung can be a source of significant complications following single lung transplant. Pretransplant diagnoses other than uncomplicated idiopathic pulmonary fibrosis seem to be most frequently associated with compromise of function or risk of infection arising from the native lung.

Extracorporeal membrane oxygenation for early graft dysfunction in lung transplantation: a case report.

Although extracorporeal membrane oxygenation (ECMO) has become standard treatment for neonatal respiratory failure, the results of ECMO in adults have been less encouraging. With technical improvements in membrane oxygenators and revised ECMO protocols, there has been renewed interest in ECMO for adults with severe acute respiratory failure. However, few cases of ECMO as an adjunct to adult lung transplantation have been reported. We present the case of a 20-year-old woman with cystic fibrosis who underwent bilateral single lung transplantation. She had development of severe graft dysfunction in the immediate postoperative period requiring support with ECMO for stabilization.

Hypertension after lung transplantation.

Hypertension is a recognized side effect of cyclosporine administration after kidney, heart, and bone-marrow transplantation. The incidence has not been previously reported after lung transplantation. We reviewed the incidence and potential causes of hypertension in recipients of single and double lung transplants. Twenty-one previously normotensive, stable recipients of lung transplants were reviewed retrospectively to determine the incidence of hypertension. Renal function as measured by blood urea nitrogen, creatinine, prednisone, and cyclosporine levels and dosages were determined at time of onset of hypertension. Hypertension developed in 14 of 21 previously normotensive patients (66%) followed from 4 to 64 months after transplantation (mean onset 11 months after transplantation). Renal function was diminished in all patients after transplantation. Neither the level of renal dysfunction nor cyclosporine dosage or level predicted the development of hypertension. The incidence of hypertension in lung transplant recipients was comparable to that reported in cyclosporine-treated kidney transplant patients (67%) and bone-marrow transplant patients (60%) but was less than that in heart transplant recipients (90%). Preserved cardiac innervation may explain the lower incidence of hypertension in lung compared with heart transplant recipients in the presence of comparable immunosuppressives and renal function. Time to onset of hypertension in lung transplant recipients is delayed compared with that in other organ transplants. This suggests that additional mechanisms as yet unexplored may be invoked.

Popliteal vein pseudoaneurysm: a case report.

This is a case report of an athletic, middle-aged man with a remote history of penetrating right leg trauma who developed a pulmonary embolism, thrombosis of the popliteal vein, a mass in the popliteal fossa, and acute ischemia of the right lower leg. At operation a thrombosed pseudoaneurysm was found arising from a defect in the popliteal vein with extrinsic compression of the popliteal artery. The defect was patched with autogenous vein and the long-term result was excellent. Pseudoaneurysm of the popliteal vein is a previously unreported entity. Popliteal venous aneurysms usually begin with phlebitic or pulmonary thromboembolic manifestations and present a diagnostic challenge. Venous aneurysms in general are uncommon pathologic entities with a diverse etiology; the current etiologic concepts and classification are briefly discussed in the text. A new differential diagnosis for masses in the popliteal fossa is introduced by this report.

Paraplegia associated with the use of oxidized cellulose in posterolateral thoracotomy incisions.

Three patients suffered permanent neurologic injury after oxidized regenerated cellulose migrated into the spinal canal after thoracotomy. These cases are reported to alert surgeons of this potential complication when oxidized cellulose is used to achieve hemostasis in the posterior angle of the thoracotomy incision.

Bio-Medicus centrifugal ventricular support for postcardiotomy cardiac failure: a review of 129 cases.

BACKGROUND: Bio-Medicus centrifugal ventricular support has been used widely for postcardiotomy cardiac failure. The purpose of this study was to review a large series of support patients examining complications and outcomes. METHODS: One hundred twenty-nine patients undergoing cardiac operations at The Methodist Hospital in Houston, Texas, were placed on Bio-Medicus centrifugal ventricular support for postcardiotomy cardiac failure; 102 were male and 27 were female with a mean age of 61.6 years. These patients were selected for ventricular support based solely on failure to be weaned from cardiopulmonary bypass or progressive postcardiotomy cardiac failure shortly after arrival in the intensive care unit. RESULTS: These patients experienced numerous complications including coagulopathy, renal insufficiency or failure, sepsis, neurologic deficits, ventricular failure, arrhythmias, and death; 56.3% of patients were weaned from mechanical support and 21.0% were discharged alive. Causes of death included ventricular failure (62.4%), arrhythmias (12.9%), triage (6.9%), perioperative myocardial infarction or cardiac arrest (5.0%), coagulopathy (4.0%), sepsis syndrome (4.0%), cardiac graft failure (3.0%), and device related (1.0%). The one device-related death was a venous cannula dislodgment in the intensive care unit with subsequent exsanguination. Device-related complications were seen in only 1.6% of patients. CONCLUSIONS: Bio-Medicus centrifugal ventricular support can be implemented rapidly and relatively easily. There are few device-related complications and the cost is relatively inexpensive compared with other assist systems. This series demonstrates that a substantial number of patients who experience reversible postcardiotomy myocardial injury will benefit from temporary centrifugal ventricular support.

Ionisation corrections in the determination of impurities in solids by means of the M.S.7 mass spectrometer.

Determinations on the M.S.7 mass spectrometer of a number of trace elements have been made on standard samples, whose composition had previously been determined by independent techniques and which had been tested for homogeneity. From the ratios of the apparent contents, as determined on the M.S.7, to the true contents, a correction factor is obtained. This factor depends largely on ionisation phenomena at the source, but also includes other errors. It has been shown that for a number of elements such factors depart significantly from unity and that the value for a particular impurity depends on the matrix element. The variation of the factors showed no direct connection with the electrical parameter of the source. Cooling of the electrodes produced a significant reduction in the magnitude of the correction factor in the cases of only very few elements. In the absence of independent standards of similar composition to the sample, any figures obtained by mass spectrometry must be treated with caution unless the ionisation factor for the particular impurity in the particular matrix is known. This is especially the case for certain elements (e.g., S, Zn, Pb) known to be liable to give high factors.

Cardiovascular effects of microgravity: evolution of understanding.

The understanding of cardiovascular effects of spaceflight has evolved throughout the course of the American manned spaceflight program. Originally descriptive in nature, the present understanding is based on empiric measurements of vascular volume, cardiac output, vascular reflexes, and peripheral and central autonomic control. More detailed understanding of cardiovascular effects has allowed us to separate those symptoms from symptoms caused by musculoskeletal or neurovestibular abnormalities.

Poorly coordinated children: a survey of 95 cases.

Screening of 717 5-year-olds and 757 7-year-olds, found 55 of the former and 77 of the latter possibly to be poorly coordinated. Further diagnostic testing with the McCarthy Motor Scales confirmed the problem in a total of 95 children, a prevalence of 6.4%. Neurological examination showed 43% of the 5-year-olds and 21% of the 7-year-olds to have choreiform movements. Of the total 95, proprioception was abnormal in 40%, but abnormal muscle tone present in only 4%. An increased prevalence of hearing loss and obesity and a history of developmental delays was found. Low birth weights, prematurity, post-maturity and perinatal problems were significantly associated with poor coordination. Socioeconomic status was not a significant factor. The difficulties of testing and measuring poor coordination and the need for more precise measures are discussed. Follow-up of at risk children at age 5 with tests of motor coordination is recommended.

Relative enterotoxigenicity of coliform bacteria.

The enterotoxigenicity of 12 strains of coliform bacteria (Enterobacter cloacae, Klebsiella pneumoniae, and Escherichia coli) isolated from the gastrointestinal (GI) tract of persons with either acute diarrhea or tropical sprue and of 13 strains of the same species isolated from urine (GU) cultures was determined. Fractions of heat-labile (LT) and heat-stable (ST) toxins of each strain were separated by ultrafiltration, and the effect of graded concentrations (range, 100 microgram-10 pg/ml) on water transport was assessed by in vivo perfusion in the rat jejunum. Enterotoxigenic activity was defined as inducement of net secretion of water. All 12 of the GI strains and six of 13 GU strains elaborated enterotoxins, but there was a millionfold quantitative difference in the potency of the toxins produced. All of the GI strains produced one or both forms of toxin, which had a minimal effective concentration of as low as 0.1-10 ng/ml, whereas the GU strains produced toxins of weak or, rarely, of intermediate potency.

Neurilemmoma of the frontal-ethmoid sinus simulating a mucocele. A case report.

A 48-year-old black female had a 1-year history of increasing proptosis and headache. Sinus films and CT scan demonstrated an expansive soft tissue mass in the right frontal-ethmoid sinus with lateral displacement of the medial orbital wall, consistent with a mucocele. Resection revealed a solid tumor which was identified pathologically as a neurilemmoma. To our knowledge, this is the first published report of a frontal-ethmoid neurilemmoma presenting solely with proptosis, and also mimicking a mucocele.

Enzyme-linked immunosorbent assays for virulence properties of Campylobacter jejuni clinical isolates.

To evaluate the capacity of enzyme-linked immunosorbent assays (ELISAs) to identify pathogenic strains among clinical fecal isolates of Campylobacter jejuni, 40 consecutively obtained strains from 39 sick patients and 1 asymptomatic person were tested by respective ELISAs for enterotoxin production in culture filtrates and for the invasive virulence antigen of bacterial cells. Of the 40 strains, 14 produced the enterotoxin; 15 strains, two of which were also enterotoxigenic, were invasive; and 11 strains had no detectable virulence property. The presence or absence of these virulence properties was confirmed by the demonstration that viable cells of all 12 randomly selected enterotoxigenic or invasive strains tested, but none of 9 nonpathogenic strains tested, caused fluid secretion in rat ligated ileal loops. All 12 patients examined who were infected with an invasive strain had grossly or microscopically evident blood cells in their stools or both, whereas none of those infected with an enterotoxigenic strain had overtly bloody diarrhea, and only 1 of 8 patients examined had microscopically evident blood cells in the stool. Twelve of the invasive, five of the enterotoxigenic, and three of the nonpathogenic strains also produced small amounts of cytotoxin, but there was no correlation between cytotoxin production and an abnormal response in rat ligated ileal loops. These observations show that enterotoxin production or invasiveness or both can be detected by ELISAs in three-fourths of C. jejuni fecal isolates and that there is usually a relationship between the specific pathogenic property of the infecting strain and the clinical mainfestations.