Emergencies caused by pheochromocytoma, neuroblastoma, or ganglioneuroma.

Pheochromocytoma may lead to important emergency situations, ranging from cardiovascular emergencies to acute abdomen and multiorgan failure. It is vital to think about this disease in any emergency situation when conventional therapy fails to achieve control or symptoms occur that do not fit the initial diagnosis. The importance of keeping this diagnosis in minds is underscored by the fact that, in 50% of pheochromocytoma patients, the diagnosis is initially overlooked. Two other tumors of the sympathetic nervous system, neuroblastoma and ganglioneuroma, are less commonly associated with emergency conditions. If they occur, they are often linked to catecholamine excess, paraneoplastic phenomena, or local tumor mass effect.     

To what extent does IQ 'explain' socio-economic variations in function?

Background  

The aims of this study were to examine the extent to which higher intellectual abilities protect higher socio-economic groups from functional decline and to examine whether the contribution of intellectual abilities is independent of childhood deprivation and low birth weight and other socio-economic and developmental factors in early life.     

Doctor's delay in HIV infection caused by blood transfusion]

Transfusion-associated symptomatic HIV infection in four patients led to death in two patients and to development of serious neurological sequelae in a third patient who also transmitted HIV infection to his spouse. The tardy diagnosis of HIV-associated disease in all cases can be ascribed to ignoring the earlier blood transfusion as a possible cause of HIV infection. This was due partly to advanced age of the patients and partly to lack of familiarity of the attending physicians with HIV-associated problems. This resulted in a substantial doctor's delay.     

Fracture of the thoracic spine with paralysis and esophageal perforation

A 17-year-old young man presented with a highly unstable fracture dislocation of the third and fourth thoracic vertebrae with neurological deficit, in which the fractured spine had perforated the thoracic esophagus. Open reduction and internal fixation of the spinal fractures in combination with aggressive treatment of the mediastinitis caused by esophageal perforation, consisting of two re-thoracotomies, was performed. Two years after the accident, the patient had recovered well. The neurological deficit had recovered, and there were no difficulties with swallowing.     

Discordant repeat size and phenotype in Kennedy syndrome

Previous reports in the literature have described correlation of increasing repeat length with severity of the phenotype, in Kennedy syndrome. We describe male siblings with different repeat lengths, with lack of expression of the phenotype in the sibling with the longer repeat length. The phenotype was identical to motor neurone disease. There is variability of expression in Kennedy syndrome and repeat length even in siblings cannot be taken as a conclusive indicator of severity. CAG repeat length cannot be used to predict the natural history of Kennedy disease. The diagnosis of Kennedy syndrome should be considered in male patients presenting with atypical motor neurone disease.     

INTERFERONS IN ONCOLOGY

SUMMARY The interferons are natural glycoproteins secreted in response to various stimuli, including viral infection. They have antiviral, antiproliferative and immunomodulatory effects on different target cell populations. Since recombinant human interferons have become available, they have been tested in a wide range of malignancies. They are well established in the treatment of hairy cell leukaemia, chronic myelogenous leukaemia and multiple myeloma. Although they have documented activity against lymphoma, melanoma, renal cell cancer and carcinoid tumours, their role in the treatment of these tumours is less clear. In the common solid tumours, such as lung cancer and colorectal cancer, the use of interferons remains experimental. Here we will summarise their practice applications in oncology, using randomised studies where available to establish their place in multi-modality treatment. We will not discuss their use as antiviral or immunomodulating agents in viral and autoimmune diseases, multiple sclerosis or after organ transplantation.