A novel treatment for chronic pancreatitis

Background Hereditary pancreatitis is an important cause of chronic pancreatitis, which may result in endocrine and exocrine failure. This may necessitate simultaneous pancreas and kidney transplant (SPK). Bladder drainage of the exocrine secretions may cause problems. Aim To report one such case and its surgical correction. Methods A 20-year-old male with insulin-dependent diabetes mellitus secondary to idiopathic chronic pancreatitis had a SPK with bladder drainage. Urological and metabolic complications secondary to the drainage of pancreatic secretions, rich in proteolytic enzymes required convertion from bladder to enteric drainage. Results He was able to discontinue his pancreatic enzyme supplements, ceased to have steatorrhoea and gained weight. He was referred to the €pean Registry of Hereditary Pancreatitis and Familial Pancreatic Cancer (€PAC), hereditary pancreatitis was confirmed by genetic analysis. Conclusion Enteric-drained pancreas transplantation is a successful treatment for exocrine as well as endocrine pancreatic failure and should be considered as a treatment option in patients with chronic pancreatitis.     

Twenty year follow-up of a cohort based in general practices in 24 British towns

BACKGROUND: A national prospective study of cardiovascular disease (CVD) was set up in 1978 to explain the reasons for the marked geographical variation in CVD rates in Great Britain. A total of 7735 males, aged 40-59 years (born between 1919 and 1939) randomly selected from one general practice age-sex register in each of 24 towns, responded to a screening invitation from their general practitioner (GP) and were examined in 1978-1980. We describe the methods used and the contact maintained after following a cohort for 20 years. METHODS: The established system of patient registration with a GP was used for tracing and maintaining contact with a low-mobility cohort through local area health authority networks and the National Health Service Central Register. RESULTS: By 31 December 1997, there were 1856 recorded deaths (14 known to have occurred abroad), 66 emigrations/ living overseas/lost from follow-up. In addition, 1500 study subjects had registered with new GPs who, every 2 years, provided information on both fatal and non-fatal cardiovascular events to complement information supplied by the original practices. Information was obtained on all cardiovascular events and deaths for 99.5 per cent of the surviving sample. Questionnaires mailed to surviving subjects 5 years after recruitment (1983-1985) and again in November 1992 and 1996 were returned by 98, 90 and 88 per cent, respectively, providing information on lifestyle changes, new cardiovascular symptoms and new diagnoses. CONCLUSION: Using the NHSCR framework for primary care registration procedures, maximum follow-up has been maintained. Brief and structured enquiry forms have gained and maintained co-operation from subjects and their GPs with considerable success. Mortality reporting from dual sources identified 5 per cent more cases.     

Effects of positive and negative pressure ventilation on cerebral blood volume of newborn infants

The effects of intermittent positive airway and continuous negative extrathoracic pressure ventilation on cerebral blood volume in preterm infants were studied using near infrared spectroscopy. In 12 infants continuous negative extrathoracic pressure caused a median decrease in cerebral blood volume of 0.14ml/100ml brain (95% confidence intervals (CI) 0.035–0.280) compared with no respiratory support. Oxygenated and deoxygenated haemoglobin also decreased, implying increased venous drainage as the main effect. In 17 infants intermittent positive pressure ventilation also caused a median reduction in cerebral blood volume of 0.06 ml/100 ml brain (95% CI 0.010–0.115) compared with endotracheal positive airway pressure. Deoxygenated haemoglobin increased by 0.07 ml/100 ml brain (95% CI 0.010–0.100) while oxygenated haemoglobin decreased by O.lOml/lOOml brain (95% CI 0.005–0.175). The increase in deoxygenated haemoglobin implies decreased venous drainage and the decrease in oxygenated haemoglobin implies that other factors may also be significant. Heart rate, blood pressure and oxygen saturation were monitored continuously and remained stable.     

Reduction in the minimum candidate interval in the dominant-intermediate form of Charcot-Marie-Tooth neuropathy to D19S586 to D19S432

As part of an on-going genomic screen of unlinked Charcot-Marie-Tooth disease type 2 (CMT2) families, we identified 11 regions in the genome with lod scores ≥1.0. One of these regions was near the recently identified CMTDI1 locus on 19q. We show evidence of linkage of DUK 1118 to this region and our data reduce the minimum candidate interval for CMTDI1 to the 9-cM interval spanned by D19S586 and D19S432.We also demonstrate that five additional CMT2 families are unlinked to 19q markers, providing further evidence of CMT2 heterogeneity. Electronic Publication     

Paraneoplastic chorea associated with CRMP-5 neuronal antibody and lung carcinoma

Paraneoplastic chorea is described in 16 patients: 11 with limited small-cell carcinoma, 2 with lung cancer revealed by imaging, 1 with renal cell carcinoma, and 1 with lymphoma. All had CRMP-5-IgG; 6 also had ANNA-1 (anti-Hu), including 1 without evident cancer. Chorea was the initial and most prominent symptom in 11 patients, asymmetric or unilateral in 5 patients, and part of a multifocal syndrome in 14 patients. Basal ganglia abnormalities were revealed by magnetic resonance imaging and at autopsy (as perivascular inflammation and microglial activation). Four patients improved with chemotherapy, and 2 improved with intravenous methylprednisolone.     

Immediate ambulation following diagnostic coronary angiography procedures utilizing a vascular closure device (The Closer)

OBJECTIVE: We investigated the safety and efficacy of The Closer, a suture-mediated vascular closure device, to facilitate immediate ambulation after diagnostic coronary angiography. METHODS AND RESULTS: We identified 487 non-consecutive patients who were eligible for an immediate ambulation protocol following vascular closure after diagnostic coronary angiography. A total of 434 patients (89%) were allowed immediate ambulation (mean time, 6.3 +/- 2.4 minutes) and 34 patients (7%) were treated with intermediate duration bed rest (mean time, 105.2 +/- 55.3 minutes). Of the 34 patients treated with intermediate duration bed rest, ten had minor bleeding from the arterial access tract requiring 2 5 minutes of light compression and 24 were delayed secondary to physician preference. Nineteen patients (4%) failed to achieve hemostasis with The Closer. Outpatients were followed up at 24 hours, and inpatients were followed up the next morning. Four patients (0.8%) suffered recurrent femoral artery bleeds after initially successful vascular closure. Three recurrent bleeds occurred during the observation period in-hospital and one occurred 6 days after device deployment. At follow-up, no patients developed the following: hematoma > 4 cm, ipsilateral retroperitoneal bleed, arterio-venous fistula, pseudoaneurysm, access site infection or loss of distal pulses. No patients had lower extremity ischemia or required blood transfusion. CONCLUSION: Use of The Closer after diagnostic angiography with subsequent immediate ambulation is safe and effective for most patients. Overall, hemostasis was achieved in 96% of patients, with 89% of our patients able to ambulate immediately and 7% able to ambulate after intermediate duration bed rest.