A unique pulmonary venous abnormality: with associated pulmonary arterial anomaly and arteriovenous malformation

Lead and cadmium were administered intraperitoneally, singly and jointly, to the mice. The levels of cadmium, copper, manganese, lead and zinc were determined in liver, kidney and brain by atomic absorption spectrophotometric technique and delta-aminolevulinic acid dehydratase (ALA-D) activity was determined in erythrocytes. The tissue levels of some of these metals were found significantly altered by cadmium and lead both, but cadmium was found to have no effect on blood on ALA-D activity.     

Behcet's disease with pulmonary vessel involvement

The case of systemic vasculitis with involvement of pulmonary vessels was described. 36-years white woman with cerebral vasculitis and recurrent uveitis 5 and 3 years ago, now was admitted to hospital because of the mouth ulceration and lesions in the chest x-ray. After lung cancer exclusion, aneurysm of pulmonary artery branch was confirmed by dynamic tomocomputer examination All mentioned above manifestations were diagnosed as Behcet disease. Patient was treated with prednison, cyclophosphamide and cyclosporine. Clinical effect was observed after corticotherapy, but no improvement in chest X-ray picture was obtained also after immunosuppression. Patient died because of pulmonary haemorrhage 7 years after first symptoms of vasculitis and 2 years after first massive haemorrhage.     

Treatment of a persistent postoperative chylothorax with somatostatin

Chylothorax is a rare but potentially serious complication of pediatric cardiac operations. We report the case of a 4-month-old boy who underwent a Senning procedure for correction of D-transposition of the great vessels. A persistent postoperative chylothorax developed, necessitating continuous drainage, despite conservative treatment over 3 weeks. Thereafter, continuous somatostatin infusion for 14 days led to the reduction and finally cessation of chyle production. This treatment allowed early enteral feeding and avoided further surgical intervention.     

Adams-Oliver syndrome: a case with juvenile chronic myelogenous leukemia and chylothorax

We report on a patient with Adams-Oliver syndrome and report new findings: a chylous pleural effusion and juvenile chronic myelogenous leukemia. Also, our patient had congenital heart disease, confirming that heart lesions are a manifestation in this syndrome. The major manifestations of this disorder are summarized. Included are cases not previously recognized as having Adams-Oliver syndrome identified in a literature survey. Distal limb deficiency is commonest with more frequent and more severe involvement of the lower limbs. Scalp defects are the second commonest manifestation, while an underlying skull defect is not infrequent. Cutis marmorata telangiectatica and dilated scalp veins are significant signs of this condition. This review highlights unresolved questions about Adams-Oliver syndrome.     

Hemodynamic pulmonary edema in dogs with acute and chronic lymphatic ligation

The effect of lymphatic ligation on relative lung water (g H2O/g dry lung) was studied in dogs. Raising left atrial pressure to 20 mmHg for 2 h in chronically lymphatic-ligated dogs (4 days) caused a significantly greater increase in relative lung water than the same hemodynamic challenge did in sham-operated and acutely lymphatic-ligated dogs. There was no significant difference in relative lung water between the acutely lymphatic-ligated and sham-operated dogs. At normal left atrial pressures, there was no significant difference in relative lung water between the sham-operated and chronically lymphatic-ligated dogs. Since the combined effects of chronic lymphatic ligation and left atrial hypertension is greater than the sum of the individual effects, it appears that chronic lymphatic ligation increases the susceptibility of the lung to hemodynamic edema, we suggest that chronic lymphatic ligation may have produced increases in the interstitial volume and protein mass that are undetectable by our technique. These increases, in turn, could lead to a reduction in tissue safety factors against hemodynamic pulmonary edema.     

Blood vessel and lymphatic vessel invasion in resected nonsmall cell lung carcinoma. Correlation with TNM stage and disease free and overall survival

BACKGROUND: The objective of this prospective study was to assess in 96 patients with resected nonsmall cell lung carcinoma (NSCLC) the prevalence of both blood and lymphatic vessel invasion (BVI and LVI) according to stage, as well as their prognostic value for disease free and overall survival. METHODS: BVI and LVI were evaluated by hematoxylin and eosin stains on surgical specimens after resection. Associations among variables were tested by Fisher's exact test or the chi-square test; prognostic values on time-failure data were analyzed by the log rank test and the multivariate Cox model. RESULTS: BVI was present in 52% of NSCLC cases and LVI in 59%. Venous but not arterial vascular invasion correlated with the T factor and pTNM, whereas LVI correlated with the N factor and pTNM. In univariate analysis, LVI but not BVI was associated with a short disease free interval (P = 0.0007) and poor survival (P = 0.0001). The estimated relative risk of death in patients with LVI was 3.2 compared with patients without LVI. In multivariate analysis, LVI and pTNM were additional predictors for poor disease free and overall survival. In this series, BVI had no prognostic value. CONCLUSIONS: The prevalence of BVI and LVI appeared high in patients with NSCLC, especially those with advanced pTNM stages. LVI was predictive of poor outcome, both time to recurrence and death.     

Clipping of the thoracic duct with video-assisted thoracic surgery in the treatment of chylothorax after pulmonary resection

We describe a procedure for video-assisted thoracoscopic clipping of the thoracic duct to treat postoperative chylothorax. This technique was successfully performed on a 62-year-old man who developed chylothorax following right lower lobectomy and partial resection of the 11th and 12th vertebral bodies for squamous cell lung cancer. Because conservative therapy for 7 days failed to reduce the amount of pleural effusion, we performed thoracoscopic examination of the thoracic duct and found a site leaking chylous fluid. The thoracic duct was successfully and easily clipped resulting in complete elimination of the effusion in 2 days. Generally, chylothorax complicating pulmonary resection has been managed by medical treatment first, followed by surgical intervention in case that fail to respond to initial therapy. The newly designed video-assisted thoracic surgery procedure reduces the trauma, shortens the drainage period and hospital stay, and provides better exposure of the thoracic duct. We believe that this procedure can be carried out shortly after the occurrence of chylothorax.     

The changes in lymphocytes subpopulations of a patient with postoperative chylothorax

A case of 64-year-old male who developed chylorrhea at 2 days post coronary artery by-pass grafting, is reported. He was managed conservatively for 3 weeks. But chylothorax was not improved, he was treated operatively. Analysis of his lymphocyte subpopulations in peripheral blood were performed during the course of chylothorax. Lymphocytepenia became apparent and subpopulation of T cell were decreased gradually. The subpopulation of CD 4(+) cell decreased, while the subpopulation of CD 8(+) increased. The CD 4(+) cell/CD 8(+) cell ratio decreased consequently till 7th day after 2nd operation. Although the replenishment of nutritional deficiencies using TPN allows prolonged conservative management for chylothorax patient, the deterioration in cellular immunocompetence can not be prevented at present. It is necessary to take great care about infection for chylothorax patient.     

Neonates with congenital heart disease, Part II: Congenital cardiac defects with increased pulmonary blood flow

Generally, cardiac lesions with increased pulmonary blood flow demonstrate cardiomegaly, increased pulmonary vascular markings, and pulmonary congestion on the chest x-ray. These findings occur as a result of the following: 1. A left-to-right shunt or mixing lesion in which excess volume of blood flow causes dilation of cardiac chambers, resulting in the appearance of cardiomegaly, and in which increased pulmonary artery blood flow causes increased pulmonary vascular markings 2. Obstruction of blood flow that produces pulmonary venous hypertension and resultant pulmonary edema The next article in this series will address cardiac lesions with decreased pulmonary blood flow.     

Blepharophimosis: a causally heterogeneous malformation frequently associated with developmental disabilities

The effects of loxiglumide (CAS 107097-80-3, CR 1505), a novel cholecystokinin-A(CCK-A) receptor antagonist, on pancreatic exocrine secretion stimulated by exogenously administered CCK-8 were examined in conscious dogs with chronic pancreatic fistula. Pancreatic exocrine secretion in dogs was significantly increased by intravenous infusion of CCK-8 at a dose of 0.06 microgram/kg/h. Loxiglumide inhibited CCK-8-augmented outputs of pancreatic protein, trypsin and amylase at intravenous doses of 1, 3, 10 mg/kg/h (p < 0.05 or 0.01), and inhibited pancreatic juice volume at a dose of 10 mg/kg/h (p < 0.05). These results demonstrated that the selective CCK-A antagonist loxiglumide inhibited the increase of pancreatic exocrine secretion stimulated by CCK-8 based on selective blockade of receptor binding of CCK in dogs.     

Staphylococcal scalded-skin syndrome complicating wound infection in a preterm infant with postoperative chylothorax

The course of infection in a 3-week-old premature newborn suffering from extensive dermatitis with flaccid blisters is described. Staphylococcus aureus was recovered from a local wound infection around a chest tube inserted to drain a postoperative chylothorax. The strain isolated tested positive for the eta gene for exfoliative toxin A, the causative agent of staphylococcal scalded-skin syndrome (SSSS). In this case, prematurity and loss of chylus with consecutive lymphopenia may have contributed to development of SSSS.     

Congenital lobar emphysema resulting from bronchial sling around a normal right main pulmonary artery

A 5-month-old female infant was transferred to our institution for evaluation of recurrent upper respiratory tract symptoms and chest x-ray films showing hyperaeration of the right upper lobe. Preoperative studies including chest fluoroscopy, lung scan, and bronchoscopy suggested congenital lobar emphysema. At thoracotomy, an anomalous right upper lobe bronchus which bifurcated around the main pulmonary artery in a sling fashion was found. This anatomic abnormality has not been described previously and is a unique cause of congenital lobar emphysema.     

Ectopic ACTH secretion: a heterogeneous entity

OBJECTIVES: ACTH-secreting non-pituitary tumors are a rare cause of Cushing's disease. We report the clinical course, prognostic aspects and molecular analysis data in three patients for whom the diagnosis was confirmed but who had variable clinical features and laboratory results. CASE REPORTS: Patient n degree 1 had severe hypercorticism which rapidly progressed to death 13 months after diagnosis. In patient n degree 2, signs of hypercorticism severe, leading to death 5 years after discovery of the causal carcinoid tumor. Patient n degree 3 had moderate hypercorticism and has survived for more than 25 years. DISCUSSION: These 3 ectopic tumors are representative examples of a wide range of possible ACTH-secreting ectopic tumors. In highly malignant poorly-differentiated tumors such as small-cell anaplastic carcinomas, ACTH production is aberrant and poorly controlled, and thus not particularly effective. At the other extreme, typical benign bronchial carcinomas have a high degree of neuroendocrine differentiation and secrete ACTH in a well-controlled manner difficult to distinguish from corticotropic adenomas, further exaggerating the diagnostic pitfalls.     

Congenital Becker''s nevus with a familial association

Coprecipitates were prepared using different ratios of flutamide with polyvinyl pyrrolidine (PVP), polyethylene glycol (PEG) 4000 and 6000. Drug solubility in carrier solutions, thin layer chromatography (TLC), differential scanning calorimetry (DSC), infra-red spectroscopy (IR), uniformity of drug content, drug dissolution from its respective systems and effect of aging on the physico-chemical parameters of stored flutamide-polymer system were studied. PEG 6000 was found to be the most efficient polymer in increasing both the solubility and the release rate of flutamide. Interaction was found to be complete in certain ratios of drug/polymer systems. The dissolution pattern of flutamide from all the prepared systems appeared to fit a first order mechanism. Physico-chemical parameters of flutamide/carrier systems were not influenced by storage.     

Severe hyperchloriduria-hyperkaliuria: a new congenital renal tubular abnormality?

A female infant, aged 5 weeks, had metabolic alkalosis associated with severe electrolyte disturbances. In addition to findings typically seen in patients with Bartter syndrome or hyperprostaglandin E syndrome, she had massive urinary excretion of prostaglandins E2 and E-M, normal calcium metabolism, hyperphosphaturia, and severe hyperchloriduria and hyperkaliuria with limited response to indomethacin. These findings may represent a new congenital renal tubular abnormality.