Ewing's tumour of rib presenting as chest mass

A case of Ewing's tumour of rib presenting as chest mass is reported. The role of computed tomography and chest ultrasound in evaluating such patients is discussed along with a brief review of literature.     

von Hippel-Lindau disease affecting 43 members of a single kindred

We present a 6-generation kindred of over 221 members, 43 of whom were affected with von Hippel-Lindau (vHL) disease. Through a simple screening protocol, we diagnosed vHL retrospectively in 15 cases, and for the first time in 28, 11 of whom were presymptomatic. We found many complications of vHL in previously diagnosed relatives and in new cases. This study has demonstrated the utility and benefit of preventive surveillance in those known to have vHL, and of presymptomatic screening for affected relatives in families with vHL. The features of vHL were reviewed in our 43 cases and 511 cases from the medical literature. The patterns, frequencies, and ages of onset for each lesion were compared. Renal malignancies caused almost as much mortality in vHL as CNS malignancies. This family was exceptional for absence of pheochromocytoma and erythrocythemia, for more renal and pancreatic cysts and malignancies, and for slightly fewer eye or CNS lesions. Bilateral renal adenocarcinomata were found presymptomatically in five young subjects, who had bilateral nephrectomy and hemodialysis. Three survived long-term after renal transplants. Five relatives had pancreatic malignancies, which are definite although uncommon manifestations of vHL. Recommendations are made for family screening, which was economical and effective. Bayesian calculations help to predict risks for genetic counseling. The molecular basis of vHL may soon be found, since it has been linked to DNA markers on the short arm of chromosome 3.     

The persistence of the corpus luteum of pregnancy into lactation in the marsupial bandicoot, Isoodon macrourus

The bandicoot is unique among marsupials in possessing a corpus luteum of pregnancy which continues to secrete progesterone during lactation. Different factors which may influence the activity of this corpus luteum were examined. There was no correlation between the number of pouch young and the plasma progesterone concentration. Similarly, bromocriptine did not appear to cause a dramatic decrease in plasma progesterone early in lactation, however, it may induce premature regression of the corpus luteum late in lactation. Corpora lutea were not observed in the ovaries of two out of six bandicoots treated with bromocriptine late in lactation. Although the corpus luteum may influence ovarian activity early in lactation, and corpus luteum does not appear to influence reproduction late in lactation. Removal of corpora lutea at Day 30 of lactation caused an abrupt decline in plasma progesterone; however, the birth of the subsequent litter occurred on Days 58 and 61 (N = 2), births normally occurring on Day 60.     

Vasoactive intestinal peptide inhibits luteinizing hormone secretion: the inhibition is not mediated by dopamine

Vasoactive intestinal peptide (VIP) is a neuropeptide that is present in the hypothalamus and is probably a neuroendocrine regulator. The effect of VIP on pulsatile LH secretion in the long-term ovariectomized rat was re-examined in the light of earlier conflicting reports. VIP or saline was infused into the third ventricle at the rat of 15 microliters/h and blood was sampled frequently before and during the infusion. VIP at 3.5 nmol/h significantly depressed mean LH levels (p less than 0.05) and lowered pulse frequency (p less than 0.05), but had no effect on LH pulse amplitude (p greater than 0.05). VIP at lower levels was not consistently effective, and intraventricular saline was without influence. We examined indirectly whether the site of action of VIP (3.5 nmol/h) was the brain or pituitary by injecting various doses of gonadotropin-releasing hormone (GnRH; 0.5-4.0 ng/100 g BW i.v.) during VIP-induced inhibition of LH secretion and in saline-infused controls. VIP did not alter the response of the pituitary to GnRH or the slope of the GnRH-LH dose-response curve (p greater than 0.05). We conclude that the inhibitory action of VIP on pulsatile LH secretion is probably exerted in the hypothalamus. To test the hypothesis that dopamine mediates the inhibitory effects of VIP (3.5 nmol/h), animals were pretreated with the dopamine receptor blocking agent pimozide (1.26 mg/kg) in an attempt to block the actions of VIP. Pimozide did not affect the response of LH to VIP infusion (p greater than 0.05). We conclude that dopamine is not a likely mediator of the action of VIP.     

Association of tuberculosis and M. tuberculosis-specific antibody levels with HLA

In the search for HLA-linked immune response genes that control susceptibility to tuberculosis, we performed HLA typing and measured antibody titers to well-defined Mycobacterium tuberculosis antigenic determinants in 101 patients with sputum smear-positive pulmonary tuberculosis and 64 healthy controls from Surabaya, Indonesia. HLA-DR2 and DQw1 were associated with sputum smear-positive pulmonary tuberculosis (attributable risk = 36% and 39%, respectively), while DQw3 was associated even more strongly with the control group (preventive fraction = 57%). Antibody titers to the TB71 and TB72 epitopes of the 38-kDa protein, present only on tubercle bacilli, were strongly associated with DR2 (Pcorr = .001 and .024, respectively). The association of both the disease and the antibody response to the 38-kDa antigen of M. tuberculosis with Class II HLA genes HLA-DR2 indicates that Ir-gene-mediated regulation of the immune response to this antigen may be of pathogenic significance for the development of sputum smear-positive tuberculosis.     

Molecular cloning and expression of Mycobacterium tuberculosis strain Aoyama B peptide antigen genes in Escherichia coli--a gene encoding 15 kD antigen (AT 01)

A genomic library of Mycobacterium tuberculosis strain Aoyama B in Escherichia coli K-12 was constructed by cloning Sau 3A I cleaved M. tuberculosis Aoyama B chromosomal DNA into pUC18, pUC181 or pUC182. Clones reacting with anti-PPDs-rabbit-serum were screened by immunoblotting among 3 x 10(4) clones. Seven clones were selected; designating pAT 01, pAT 101, pAT 102, pAT 103, pAT 104, pAT 105 and pAT 201. On Western blotting, they were shown to produce 15 kD (pAT 01, pAT 101, pAT 105), 18 kD (pAT 103) and 60 kD (pAT 102, pAT 201) peptide antigens. Restriction endonuclease map of each of the above clones was composed, and putative coding frames of anti-PPDs-rabbit-serum reactive peptides were deduced by analysing deletion derivatives. Nucleotide sequence of pAT 01, encoding for 15 kD peptide antigen was analyzed, and a Hinf I-Hinf I fragment in pAT 01 was subcloned into pUC 18 lambda CPL 1 to determine the direction of its reading frame. The origin of promoter that drives cloned mycobacterial genes in E. coli was discussed.     

The utility of the presence or absence of chest pain in patients with suspected acute myocardial infarction

In 422 patients admitted from the emergency department (ED) for suspected acute myocardial infarction, the hypothesis that chest pain that persists on arrival in the ED or recurs during the initial ED evaluation is a useful predictor of acute myocardial infarction (AMI) and complications of coronary ischemia was tested. Compared with patients whose chest pain spontaneously ceased before arrival in the ED, patients whose chest pain persisted or recurred during the initial ED evaluation had a 2.3 times greater risk of interventions (P less than .001), a 1.7 times greater risk of complications (P = .045), a 3.8 times greater risk of life-threatening complications (P = .04), and a 2.4 times greater risk of AMI (P = .005). A third group of patients with suspected AMI never experienced chest pain. This group of patients who never experienced chest pain had a three times higher risk of death (P = .02) compared with patients whose chest pain persisted or recurred in the ED, and a 2.1 times greater risk of intervention (P = .01), a 5.2 times greater risk of life-threatening complication (P = .015), and a 7.9 times greater risk of death (P = .025) compared with patients whose chest pain resolved before arrival in the ED. It was concluded that patients with chest pain that resolves spontaneously before arrival to the ED have a better in-hospital prognosis than any other group.     

Portal thrombosis: Percutaneous transhepatic treatment with urokinase — A case report

We present a case report of a patient suffering from portal and superior mesenteric vein thrombosis secondary to splenectomy. No surgical procedure could be performed due to the extension of thrombus.Local fibrinolysis treatment with urokinase through a percutaneous transhepatic approach was decided upon, and this procedure had a successful patient outcome.