Neurophysiologic and intellectual evaluation of beta-thalassemia patients.

In order to detect involvement of the central and peripheral nervous system in beta-thalassemic patients, 32 children and young adults (mean age 14.5 +/- 6.4 years) participated in a systematic neurophysiologic and intellectual prospective study. All patients were in a regular transfusion program, receiving subcutaneous desferrioxamine chelation and maintaining a mean serum ferritin level of 2,101.56 +/- 986.32 ng/ml. Study patients underwent neurophysiologic evaluation consisting of brainstem auditory, visual and somatosensory evoked potential examination (BAEP, VEP, SEP) as well as motor and sensory nerve conduction velocity studies (MCV, SCV). Additionally, the verbal, performance and total IQ were assessed in patients under 16 years of age using the Weschler Intelligence Scale for Children (WISC-III). The incidence of abnormal BAEP, VEP, SEP and NCVs was 0, 3.12, 3.12 and 18.75%, respectively, findings comparative to or better than previously reported. On the contrary, the prevalence of abnormal total IQ score was considerably high (36.4%), not correlating, however, to any of the parameters assessed (age, sex, ferritin level, BAEP, VEP, SEP, NCV). Factors associated with chronic illness, rather than the disease per se, could play a potential role in the development of cognitive dysfunction in beta-thalassemia patients.

Superior sagittal sinus thrombosis in steroid-resistant nephrotic syndrome.

An 8(1/2)-year-old-female child with steroid-resistant nephrotic syndrome developed sagittal sinus thrombosis while on pulse therapy with corticosteroids, presenting with recurrent vomiting, headache, and impaired consciousness. The diagnosis was established by cranial computed tomography, magnetic resonance imaging, and magnetic resonance angiography. She gradually recovered without neurologic sequelae while being treated with low-molecular-weight heparin (2 mg/kg/day). Sagittal sinus thrombosis consists of a rare and probably underdiagnosed complication of childhood nephrotic syndrome.

Radiotherapy plus either transdermal fentanyl or paracetamol and codeine for painful bone metastases: a randomised study of pain relief and quality of life.

OBJECTIVE: To compare the effects of providing analgesia with either transdermal fentanyl (TTS-fentanyl) or paracetamol and codeine (P/C) in addition to radiotherapy in patients with metastatic bone pain. METHODS: In a prospective study, 26 patients with radiologically confirmed bony metastases received radiotherapy (R/T). They were randomised to receive either 500 mg paracetamol and 30 mg codeine four times per day (P/C group), or transdermal fentanyl patches delivering 25 microg fentanyl/h (TTS-fentanyl group). Pain was assessed using visual analogue pain ratings (VAS) and the Greek Brief Pain Inventory (G-BPI) questionnaire administered before R/T and after 3 months. RESULTS: Data were available from 24 eligible patients. Use of TTS-fentanyl was associated with significantly superior pain relief. Mean VAS fell from 7.0 to 1.1 with TTS-fentanyl and from 8.3 to 4.3 with P/C, p< 0.01. The TTS-fentanyl group also showed significantly greater improvements of important G-BPI domains including global quality of life, pain, and physical, cognitive, and role functioning, than the P/C group (p< 0.01). Four patients receiving TTS-fentanyl and three receiving P/C reported severe nausea/vomiting. CONCLUSIONS: Transdermal fentanyl combined with R/T was more effective in reducing metastatic bone pain and resulted in greater improvements in quality of life than paracetamol and codeine.