The natural history of hereditary pancreatitis: a national series.

BACKGROUND AND AIMS: The prevalence and natural history of hereditary pancreatitis (HP) remain poorly documented. The aims of this study were to assess genetic, epidemiological, clinical and morphological characteristics of HP in an extensive national survey. METHODS: A cohort comprising all HP patients was constituted by contacting all gastroenterologists and paediatricians (response rate 84%) and genetics laboratories (response rate 100%) in France (60,200,000 inhabitants). Inclusion criteria were the presence of mutation in the cationic trypsingen gene (PRSS1 gene), or chronic pancreatitis in at least two first-degree relatives, or three second-degree relatives, in the absence of precipitating factors for pancreatitis. RESULTS: 78 families and 200 patients were included (181 alive, 6673 person-years, males 53%, alcoholism 5%, smoking 34%). The prevalence was 0.3/100,000 inhabitants. PRSS1 mutations were detected in 68% (R122H 78%, N29I 12%, others 10%). Penetrance was 93%. Median age at first symptom, diagnosis and date of last news, were 10 (range 1-73), 19 (1-80) and 30 (1-84) years, respectively. HP was responsible for pancreatic pain (83%), acute pancreatitis (69%), pseudocysts (23%), cholestasis (3%), pancreatic calcifications (61%), exocrine pancreatic insufficiency (34%, median age of occurrence 29 years), diabetes mellitus (26%, median age of occurrence 38 years) and pancreatic adenocarcinoma (5%, median age 55 years). No differences in clinical and morphological data according to genetic status were observed. 19 patients died, including 10 directly from HP (8 from pancreatic adenocarcinoma). CONCLUSION: The prevalence of HP in France is at least 0.3/100,000. PRSS1 gene mutations are found in 2/3 with a 93% penetrance. Mutation type is not correlated with clinical/morphological expression. Pancreatic adenocarcinoma is the cause of nearly half the deaths.

Percutaneous ethanol injection vs. resection in patients with small single hepatocellular carcinoma: a retrospective case-control study with cost analysis.

BACKGROUND: Percutaneous ethanol injection and hepatic resection are the most widely used curative therapeutic options for patients with compensated liver disease and small hepatocellular carcinoma. AIM: To compare percutaneous ethanol injection and hepatic resection in a selected group of consecutive French patients with a single hepatocellular carcinoma, smaller than or equal to 50 mm, in terms of survival, recurrence rate of malignancy and direct costs. METHODS: The analysis of two contemporary cohorts of Child-Pugh A or B patients with a single hepatocellular carcinoma of < or = 50 mm treated by percutaneous ethanol injection (n=55) or hepatic resection (n=50). RESULTS: Long-term survival was not significantly different between the two groups when the size of hepatocellular carcinoma was less than 30 mm. However, the survival of patients with hepatocellular carcinoma larger than 30 mm was higher after hepatic resection than after percutaneous ethanol injection (P=0.044). The cumulative direct costs were significantly higher in patients treated by hepatic resection than in those treated by percutaneous ethanol injection regardless of the tumour size. The calculated costs per month of survival in patients treated with percutaneous ethanol injection and hepatic resection were 999 vs. 3865 euros, respectively (P < 0.001). CONCLUSIONS: Percutaneous ethanol injection is more cost effective than hepatic resection in patients with a single hepatocellular carcinoma smaller than 30 mm. However, in patients with a larger tumour, long-term survival is higher after hepatic resection.

Left ventricular outflow tract obstruction and hemolytic anemia after mitral valve repair with a Duran ring.

We report a case of mitral valve repair with a Duran ring that was complicated by left ventricular outflow tract obstruction, mitral regurgitation, and hemolytic anemia. A 59-year-old man with severe mitral valve regurgitation underwent mitral valve repair, including a Duran ring annuloplasty. Postoperatively, left ventricular outflow tract obstruction developed and echocardiography revealed severe systolic anterior motion of the mitral valve. The patient then underwent mitral valve replacement with a 29-mm St. Jude valve.

Isolation and identification of a new diuretic peptide from the tobacco hornworm, Manduca sexta.

A 30-amino acid diuretic peptide was isolated from the corpora cardiaca-corpora allata complexes and, separately, from medial neurosecretory cells of the Sphingid moth, Manduca sexta. The peptide was found to have the following sequence, determined by automated Edman degradation and mass spectrometry: SFSVNPAVDILQHRYMEKV AQNNRNFLNRV-NH2. We have named the peptide Mas-DP II. The peptide was synthesized and shown to possess diuretic activity in decapitated moths. Mas-DP II is related by sequence homology to a 41-amino acid diuretic peptide identified previously from M. sexta, and it belongs to the family of corticotropin releasing factor-like peptides.

Early complications of heart transplantation.

In cyclosporine-treated cardiac allograft recipients, rejection and infection are two principal early complications. The following report describes our approach to the diagnosis and management of rejection. Infectious complications are discussed elsewhere in this journal. Lymphoproliferative disorders have not been reported in our series of transplant recipients. Other early complications particularly related to cyclosporine immuno-suppressive therapy include systemic hypertension, renal insufficiency, hepatic toxicity, and pancreatitis. Each of these is illustrated by a representative group or patient profile.

Fatal embolism in mitral valve prolapse.

The postmortem demonstration of fatal cerebral embolism arising from a thrombus detected at the base of a prolapsing and myxomatous mitral valve is reported. The clinical demonstration of recurrent atrial fibrillation in this patient lends support to the suggested use of anticoagulant therapy in similar patients.

Takayasu's arteritis: surgical considerations.

Takayasu's arteritis, the nonspecific occlusive disease originally believed to affect young Oriental women exclusively, was identified in a 19-year-old man who had severe involvement of the aortic arch and its branches. Successful surgical treatment was accomplished with a bypass from the ascending aorta to the supraceliac abdominal aorta using a tube graft. The right vertebral artery, as the single patent vessel reaching the head, was revascularized distal to the stenosis with a tube graft that extended from the aortic graft. Revascularization of ischemic organ systems and body areas may be accomplished in most patients with Takayasu's arteritis using the bypass concept and fabric conduit grafts.

Electrocardiographic pseudo-infarction pattern: appearance with a large posterior pericardial effusion after cardiac surgery.

Two patients with a large posterior pericardial effusion after cardiac surgery presented with electrocardiographic precordial Q waves without evidence of myocardial infarction. Resolution of the pericardial fluid resulted in the disappearance of the Q waves. Changes in conductivity and orientation of the heart within the pericardial space, along with a decrease in QRS voltage, could lead to the loss of initial R waves in the precordial leads, eventually resulting in a QS complex. Care should be taken in interpreting the electrocardiogram after cardiac surgery in patients with a large posterior pericardial effusion. The clinical course along with serial electrocardiographic and echocardiographic tracings should be helpful in identifying this false infarction pattern.