Report on the accident at Windscale No. 1 Pile on 10 October 1957.

A serious fire developed in the core of a nuclear reactor at Windscale Works, Sellafield, northwest England, which led to the release of significant quantities of radioactive material into the environment during 10-11th October, 1957. In 1957, Windscale Works was operated by the United Kingdom Atomic Energy Authority (UKAEA), and on 15th October it was announced by the Chairman of the UKAEA that a Committee of Inquiry had been established under the chairmanship of Sir William Penney to conduct an investigation into the accident. The Committee sat at Windscale Works during 17-25th October, interviewed 37 people (some more than once), and examined 73 technical exhibits. The Committee reported to the Chairman of the UKAEA on 26th October. The Report of the Committee formed the technical basis of a UK Government White Paper (Cmnd. 302) published on 8th November, 1957, but the Penney Report itself was not published, and was only made public (at what is now The National Archives, TNA, Kew) in January, 1988. The original Report of the Committee of Inquiry is reproduced here from a copy of the Report supplied by TNA from TNA File AB 86/25.

A randomized, controlled trial comparing thoracoscopy and limited thoracotomy for lung biopsy in interstitial lung disease.

BACKGROUND: Lung biopsies are frequently needed to diagnose diffuse interstitial lung diseases. A prospective randomized, controlled trial comparing limited thoracotomy (open lung biopsy) and thoracoscopy for lung biopsy was done. METHODS: Ambulatory patients with a clinical diagnosis of diffuse interstitial lung disease were randomized to thoracoscopy or limited thoracotomy. Data on postoperative pain, narcotic requirements, operating room time, adequacy of biopsy, duration of chest tube drainage, length of hospital stay, spirometry, and complications were collected. RESULTS: A total of 42 randomized patients underwent lung biopsy (thoracoscopy 20, thoracotomy 22). The two study groups were comparable with respect to age, gender, corticosteroid use, and preoperative spirometry. Visual analog scale pain scores were nearly identical in the two groups (p = 0.397). Total morphine dose was 50.8 +/- 27.3 mg in the thoracoscopy group and 52.5 +/- 25.6 mg in the thoracotomy group (p = 0.86). Spirometry (FEV1) values in the two groups were not significantly different on postoperative days 1, 2, 14, and 28 (p = 0.665). Duration of operation was similar in both groups (thoracoscopy 40 +/- 30 minutes, thoracotomy 37 +/- 15 minutes; p = 0.67). The thoracoscopy and thoracotomy groups had equivalent duration of chest tube drainage (thoracoscopy 38 +/- 28 hours, thoracotomy 31 +/- 26 hours; p = 0.47) and length of hospital stay (thoracoscopy 77 +/- 82 hours, thoracotomy 69 +/- 55 hours; p = 0.72). Definitive pathologic diagnoses were made in all patients. CONCLUSIONS: There is no clinical or statistical difference in outcomes for thoracoscopic and thoracotomy approaches. Both thoracoscopy and thoracotomy are acceptable procedures for diagnostic lung biopsy in diffuse interstitial lung disease.

Exogenous lipid pneumonia related to smoking weed oil following cadaveric renal transplantation.

A 30-year-old female presented shortly after cadaveric renal transplantation with respiratory distress typical of a bacterial infection. Following initial improvement, she developed progressive respiratory failure, initially felt to be secondary to cytomegalovirus infection. Two bronchoalveolar lavages were nondiagnostic, and an open lung biopsy was performed, which revealed a pulmonary alveolar proteinosis (PAP) reaction and exogenous lipid pneumonia (ELP). The ELP was considered to be secondary to the use of marijuana, in the form of weed oil, that was smoked daily for over 10 years and stopped just before renal transplantation. This is the first description of both PAP and ELP following renal transplantation, and the first description of ELP related to smoking weed oil. Physicians should be aware of the different forms of marijuana available and of their potential medical complications.

Laser resection of a pedunculated tracheal adenoma.

We report a case of tracheal adenoma presenting as hemoptysis and reversible airflow obstruction in an ex-smoker. A questionable defect in the tracheal air shadow on a posteroanterior chest radiograph was shown on CT to be a pedunculated, mid-tracheal tumor. Two-stage bronchoscopic laser resection resulted in an apparently normal tracheal mucosa. Results of postresection spirometry were normal.

Pulmonary vasculopathy in idiopathic spontaneous pneumothorax in young subjects.

BACKGROUND: We have encountered instances where young subjects with idiopathic spontaneous pneumothorax have been needlessly referred for investigation of pulmonary hypertension because surgical pathologists have misinterpreted the significance of medial hypertrophy and intimal fibrosis of muscular pulmonary arteries in lung resection specimens. METHODS: We reviewed 20 cases of idiopathic spontaneous pneumothorax and determined the prevalence and severity of medial and intimal lesions in the pulmonary arteries and pulmonary veins. We correlated the vascular changes with inflammation and fibrosis in the lung. RESULTS: Pulmonary artery medial hypertrophy was seen in 15% of cases, pulmonary artery intimal fibrosis in 90% of cases, and pulmonary vein intimal fibrosis in 80% of cases. In 95% of cases, the lung showed some fibrosis and chronic inflammation. There was a significant positive correlation between pulmonary artery medial thickness and lung fibrosis and inflammation scores. CONCLUSIONS: Pulmonary artery medial hypertrophy and intimal fibrosis of pulmonary arteries and pulmonary veins are commonly seen in resected lung tissue from patients with idiopathic spontaneous pneumothorax. The vascular lesions are probably secondary to chronic inflammation and fibrosis in the adjacent lung. They are not clinically significant and do not represent hypertensive pulmonary vascular disease.

Pulmonary vascular response of the coati to chronic hypoxia.

The unusually muscular pulmonary arteries normally present in cattle and swine residing at low altitude are associated with a rapid development of severe pulmonary hypertension when those animals are moved to high altitude. Because these species lack collateral ventilation, they appear to have an increased dependence on hypoxic vasoconstriction to maintain normal ventilation-perfusion balance, which, in turn, maintains thickened arterial walls. The only other species known to lack collateral ventilation is the coati, which, similarly, has thick-walled pulmonary arteries. We tested the hypothesis that coatis will develop severe high-altitude pulmonary hypertension by exposing six of these animals (Nasua narica) to a simulated altitude of 4,900 m for 6 wk. After the exposure, pulmonary arterial pressures were hardly elevated, right ventricular hypertrophy was minimal, there was no muscularization of pulmonary arterioles, and, most surprising of all, there was a decrease in medial thickness of muscular pulmonary arteries. These unexpected results break a consistent cross-species pattern in which animals with thick muscular pulmonary arteries at low altitude develop severe pulmonary hypertension at high altitude.

Interferon-gamma and interleukin-2 release by lymphocytes derived from the blood, mesenteric lymph nodes and intestines of normal sheep and those affected with paratuberculosis (Johne's disease).

This study sought to determine if T-cell cytokine responses to mycobacterial infections in sheep were similar to those in other species and if such responses correlated with prevailing gut pathology. Lymphocytes were isolated from the blood (PBL), mesenteric lymph nodes (MLN) and ileal lamina propria (LPL) of control sheep and of sheep with clinical Johne's disease due to infection with Mycobacterium avium ssp. paratuberculosis (M.a. paratuberculosis). These animals had previously been categorised into two groups exhibiting either the 'tuberculoid' (paucibacillary) form of lesion or the 'lepromatous' (multibacillary) form. Lymphocytes were examined for their capacity, following stimulation with johnin-PPD, to release interferon-gamma (IFN-gamma) and interleukin 2 (IL-2) characteristic of the Th1 subset of MHC Class II-restricted CD4+ (helper) T-cells in other species. The expression of the two cytokines appeared related to the type of histological lesion observed. Antigen-stimulated lymphocytes from the tuberculoid group exhibited greater release of IFN-gamma and IL-2 than lymphocytes from the lepromatous group suggesting a Th1-type of response in the former in which expression of IFN-gamma by PBL showed a significant positive correlation with that expressed by MLN and LPL. Lymphocytes from animals with lepromatous lesions released lesser mycobacterium-induced IFN-gamma and IL-2 indicating a diminished role for a Th1 subset in this group of sheep. Differences in cytokine expression were much more apparent with lymphocytes which were derived from MLN.

Spontaneous duodenal-caval fistula with embolization of intestinal contents.

We describe the second example of a duodenal-caval fistula in a patient with peptic ulcer disease. This condition was complicated by embolization of intestinal contents to the lung, with abundant intravascular mucin. This is the first histological documentation of intravascular mucin apart from amniotic fluid embolism. It is well known that mucin may activate coagulation. We propose that intravascular mucin activated the coagulation cascade in our patient, causing disseminated intravascular coagulation and adult respiratory distress syndrome.

A study of immunological responses of sheep clinically-affected with paratuberculosis (Johne's disease). The relationship of blood, mesenteric lymph node and intestinal lymphocyte responses to gross and microscopic pathology.

Nineteen adult sheep diagnosed as having clinical paratuberculosis (Johne's disease) and 16 unaffected controls were examined in this study. Animals were tested for the presence of circulating antibodies of Mycobacterium avium ssp. paratuberculosis (M. a. paratuberculosis) and lymphocytes derived from the blood, mesenteric lymph nodes and intestines were examined for cell-mediated immune (CMI) responses to Johnin pure protein derivative (Johnin-PPD: J-PPD). Bacteriological examinations were carried out on faeces and tissues and any mycobacterial isolates identified as M. a. paratuberculosis (IS900+) or M. avium ssp. silvaticum (M. a. silvaticum) (IS901+) by polymerase chain reaction (PCR). Full necropsy and histopathological studies were performed and diseased animals were categorised on the basis of having a lepromatous or tuberculoid form of intestinal pathology. Unaffected control sheep were generally antibody-negative and demonstrated varying CMI responses to J-PPD. Clinically-affected animals were almost always antibody-positive with variable CMI responses. A correlation was observed between the histological lesion type in the intestine and the cellular immune response. Tuberculoid-type lesions were associated with strong CMI responses in lymphocytes derived from the peripheral blood, mesenteric lymph node and intestine, whereas lepromatous-type lesions were associated with weak CMI responses in all tissues examined.

Mycobacterium avium complex infection in an immunocompetent young adult related to hot tub exposure.

An unusual case of Mycobacterium avium complex infection occurred in a young adult with no preexisting disease and no evidence of immunodeficiency. There was diffuse interstitial involvement of the lungs which suggested an active alveolitis. Diagnosis required open-lung biopsy. Restriction fragment length polymorphism analysis and multilocus enzyme electrophoresis indicated that the source of the infection was a hot tub. The infection proved to be exceptionally responsive to treatment, and there was complete resolution with a four-drug regimen.

Diffuse smooth muscle proliferation of the lungs with severe pulmonary hypertension.

We describe the second example of a distinctive clinicopathologic syndrome of severe pulmonary hypertension and interstitial lung disease caused by diffuse smooth muscle proliferation of the lungs (DSMPL) occurring in a 44-year-old man of normal intelligence with no cutaneous or neurological lesions. The smooth muscle proliferation involved the walls of bronchioles, alveoli, small pulmonary arteries, and pulmonary veins. DSMPL is distinct from pulmonary lymphangioleiomyomatosis and tuberous sclerosis, and does not appear to be neoplastic or reactive. The lesions are probably best regarded as multiple hamartomas or "native benign nodular smooth muscle proliferations."

Structural and pathologic changes in the lung vasculature in chronic liver disease.

The hepatopulmonary syndrome results from erythrocytes bypassing the lung without oxygenation. In addition to ventilation-perfusion mismatching, the hypoxemia may result from portapulmonary shunting, passage around alveoli through pleural and hilar blood vessels, and intrapulmonary vascular dilatations. Dilated vascular channels between arteries and veins on the pleural surface are seen more often than dilated intrapulmonary capillaries in chronic liver disease. These anastomoses appear grossly as vascular "spider nevi" on the pleura. Portal vein-to-pulmonary vein anastomoses could produce arterial hypoxemia because the deoxygenated portal venous blood can mix with oxygenated pulmonary venous blood. There is an association of esophageal varices with the hepatopulmonary syndrome and anastomoses between the portal veins and pulmonary veins have been found in both animals and humans. As portal pressures increase, the mediastinal veins enlarge, enhancing the chance that they may penetrate the pleura and drain into pulmonary veins. Direct splenic injections in patients, however, suggest that this shunt pathway is uncommon and small. Pulmonary artery injection studies have demonstrated dilated intrapulmonary vascular segments in humans and animals. Dilation of capillaries may allow a more rapid flow through the lung and the greater distance between the erythrocyte and alveolar wall may make it more difficult to oxygenate rapidly passing erythrocytes. Pulmonary capillary dilation can explain the abnormalities of the perfusion lung scan and contrast echocardiogram that portapulmonary shunting cannot. Pulmonary hypertension may occur in chronic liver disease even without arterial hypoxemia, but it is rare. The prevalence of hypertensive pulmonary vascular disease in patients with cirrhosis of the liver is less than 1%, although a higher percentage (2%) has been found when patients with portal hypertension were studied by cardiac catheterization. The hypertensive pulmonary vascular disease (pulmonary arteriopathy with plexiform lesions) that occurs in patients with liver disease appears identical to that encountered in patients with congenital cardiac shunts and unexplained (primary) pulmonary hypertension.

Collateral ventilation and pulmonary arterial smooth muscle in the coati.

Collateral ventilation can participate in ventilation-perfusion regulation by shifting normoxic gas into hypoxic lung regions. In species lacking collateral pathways, such as cattle and swine, ventilation-perfusion balance must rely heavily on hypoxic vasoconstriction, which may explain why their muscular pulmonary arteries are much thicker than those of other animal species. The presence of these unusually muscular vessels in turn may account for the vigorous pressor response to acute hypoxia in these species. The only other species known to lack collateral ventilation is the coati. To determine whether coatis fit the pulmonary circulatory pattern of cattle and swine, we measured pulmonary arterial wall dimensions and pulmonary vascular responsiveness to acute airway hypoxia in 11 adult coatis. Hypoxia caused impressive pulmonary arterial hypertension [normoxia = 17 +/- 1 (SE) Torr, hypoxia = 40 +/- 2 Torr, cardiac output unchanged]. The medial thickness of muscular pulmonary arteries (50-300 microns) was 17.1 +/- 1.8% (SD) of external diameter, a thickness unprecedented in normotensive adult mammals. We conclude that coatis fit the pattern of other species lacking collateral ventilation, since they have thick-walled pulmonary arteries and a vigorous pressor response to hypoxia.