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Biomedical subjects

Philippe Lévy

Publications and source records attributed to Philippe Lévy.

32 records · Page 2Linked to original sources

[Frequency of CFTR gene mutations in idiopathic pancreatitis].

UNLABELLED: The prevalence of mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene in idiopathic chronic pancreatitis has been shown to be increased. The aim of this study was to prospectively analyze the frequency of such mutations in a homogeneous group of patients with idiopathic pancreatitis studied in a French Gastroenterology department. PATIENTS AND METHODS: Between April 1999 and December 2001, all patients with unexplained chronic or recurrent acute pancreatitis were studied. Other causes of pancreatitis were excluded and no patient had personal signs or family history compatible with cystic fibrosis. Following informed written consent, genetic analysis for CFTR was performed using an oligonucleotid ligation assay, on the 31 most frequently known mutations of the CFTR gene. A complementary analysis for variants in exons 9, 10 and 17a, thought to be implicated in atypical cystic fibrosis, was also performed using denaturing gradient gel electrophoresis. RESULTS: Idiopathic pancreatitis occurred in 64 patients (chronic, n=30; recurrent acute, n=34) with a median age of 36 years. Eighteen CFTR mutations or variants were detected in 16 patients (25%): DeltaF508 (n=7), L997F (n=2), E528E (n=4), 5T (n=5). Two patients were compound heterozygous. The frequency of DeltaF508 mutations was greater than that of the general population (10.9 vs 2.4%; P<0.001). Pancreatitis was diagnosed at a median age of 32 years in mutation-positive patients compared to 39 in mutation-negative patients. The prevalence of CFTR mutations was 35.5% in patients < or =35 years against 15.1% in those > 35 years (P=0.06). The clinical course of pancreatitis (severity and complication rates) was not altered by the presence of a mutation. CONCLUSION: One-quarter of all patients and one-third of those < or =35 years with idiopathic pancreatitis have at least one mutation of the CFTR gene. The presence of a CFTR mutation appears to predict the development of pancreatitis at an earlier age.

Acute Disease↗

[Are the causes similar for benign and severe forms of acute pancreatitis?].

UNLABELLED: The frequency of severe acute pancreatitis not due to alcohol or biliary causes is not well known. AIMS: To evaluate the distribution of causes responsible for benign and severe cases of acute pancreatitis in an effort to identify causes to search for in patients with severe acute pancreatitis. PATIENT: All patients hospitalized for acute pancreatitis between January 1994 and May 2001 with a good quality CT scan. METHODS: All patients had a complete, standardized evaluation to look for all possible causes of acute pancreatitis. The following severity criteria were retrospectively reviewed: maximal C-reactive protein level, Ranson's score, Balthazar's score, percentage of patients hospitalized in intensive care unit or a high-dependency unit, hospitalization duration, and local or general complications. RESULTS: One hundred thirty-nine patients were included. The cause of acute pancreatitis were: alcohol (34%), biliary (27%), obstructive (16%), miscellaneous (10%), unknown (9%), post endoscopic retrograde cholangio-pancreatography (4%). The studied severity factors did not differ with respect to the cause of acute pancreatitis with the exception of Balthazar's score. Non-alcoholic non-biliary causes were found in 19 (27%) of the 71 patients with severe necrotic acute pancreatitis (Balthazar > or =D) and 35 (51%) of the 68 patients with acute pancreatitis with Balthazar score or =D). For the other severity scores, the distribution of causes was similar. After exclusion of biliary and alcoholic causes, a careful search for other etiologies should be carried out in both benign and severe cases of acute pancreatitis.

Acute Disease↗

Macrocystic form of serous pancreatic cystadenoma.

OBJECTIVES: Macrocystic serous cystadenoma of the pancreas are benign lesions with sometimes difficult diagnostic issues. We aimed to describe clinicopathological and imaging features with cyst fluid analysis in a series of patients undergoing surgery for macrocystic serous cystadenoma. METHODS: Eight patients underwent pancreatic resection for a macrocystic lesion of the pancreas diagnosed on ultrasonography or CT. Endoscopic ultrasonography and preoperative fine-needle aspiration were performed in seven patients. Immunohistochemical analysis of the surgical specimen with antibodies to carcinoembryonic-antigen (CEA), carbohydrate antigen (CA) 19-9, estrogen receptor, and progesterone receptor antibodies was performed in all cases. RESULTS: Patients included seven women and one man, with a mean age of 48 yr. Lesions were incidentally discovered on ultrasonography in six patients and had a mean size of 3 cm (range, 1.5-5 cm). Endoscopic ultrasonography revealed millimetric cysts in three cases. In the seven aspirated cysts, cytological analysis was non-contributive, but biochemical analysis showed low content of CEA (< 5 ng/ml) and CA72.4 (< 40U/ml) in all but two. At histology, cysts were lined by clear cuboidal cells. They focally expressed CA19-9 but were negative for anti-CEA, antiestrogen receptor, and antiprogesterone receptor antibodies. Microscopic cysts in the wall of the lesions were demonstrated in five cases. CONCLUSIONS: Macrocystic serous cystadenoma is a particular variant of pancreatic serous cystadenoma. Endoscopic ultrasonography may be useful in detecting peripherally located millimetric cysts in unilocular lesions, and measurement of enzymes and tumor markers in cyst fluid may also contribute to the diagnosis showing low concentrations.

Adult↗

[Acinar cystic transformation of the pancreas (or acinar cell cystadenoma), a rare and recently described entity].

We report a case of cystic acinar transformation (also known as acinar cell cystadenoma) of the pancreas. A 40-year old woman presented 3 episodes of acute pancreatitis that revealed a multicystic lesion of the ventral part of the head of the pancreas. A Whipple resection was performed. Gross examination of the specimen revealed a well-demarcated multiloculated cyst measuring 4 x 2 cm without communication with the excretory ducts. The cystic spaces contained a non-mucinous fluid. Histological analysis showed numerous various sized cysts that seemed to develop from microcystic structures in the surrounding acinar tissue. The cysts were lined by normal appearing acinar cells, with no atypia, sometimes flattened. Acinar cystic transformation is a rare lesion of the pancreas whose histogenesis is unknown. It should be distinguished from other lesions such as IPMT in order to discuss a surveillance strategy instead of surgical procedure.

Acute Disease↗

[Intraductal papillary mucinous tumor with pancreatobiliary and pancreatodigestive fistulae: a case report].

Pancreatobiliary or pancreatodigestive fistulae are rare during the course of intraductal papillary mucinous tumors. The mechanism of the fistula is either malignant involvement or mechanical. We report the case of an 81-year-old patient with intraductal papillary mucinous tumor complicated by fistulae between the main pancreatic duct and common bile duct, duodenum and stomach. These fistulae were treated endoscopically and radiologically by several metallic stents.

Adenocarcinoma, Mucinous↗

[Jejunal vipoma].

We report here case of a 57-year-old woman presenting with a metastatic vipoma revealed by secretory diarrhea and severe ionic disorders successfully treated by somatostatin administration. The primitive tumour, located in the jejunum, was identified peroperatively. Both the primitive lesion and the liver metastases were resected at the same time. Early tumour relapse occurred and was unsuccessfully treated by systemic chemotherapy (5-fluorouracil, streptozotocin and doxorubin) and chemoembolization. Finally, a liver transplantation was proposed.

Female↗

[Cholangiocarcinoma due to postradiation therapy cholangitis and chronic pancreatitis].

There are no reports of cholangiocarcinoma complicating post radiotherapy cholangitis. We report the case of a 40 year old patient who had undergone thoracoabdominal radiotherapy for Hodgkin's disease, 22 years ago. This radiotherapy was complicated, many years later, by chronic pancreatitis and cholangitis. Recurrent angiocholangitis led us to schedule a biliodigestive anastomosis. During surgery, cholangiocarcinoma was discovered. The patient died from his carcinoma some months later.

Adult↗

Estimation of the prevalence and incidence of chronic pancreatitis and its complications.

UNLABELLED: Incidence and prevalence of chronic pancreatitis (CP) are poorly known and prospective nationwide epidemiologic estimation has never been performed. AIMS: To estimate prospectively national incidence and prevalence of patients attending gastroenterologists for CP in France. PATIENTS AND METHODS: Study was proposed to all of the French gastroenterologists (N=3215) of whom 753 accepted to participate (24% private, 40% hospital and 36% both). Were included all patients suffering from proved or suspected CP, from 04-2003 to 07-2003. Certain diagnostic criteria were pancreatic calcifications, ductal or histological abnormalities. For all of non-responder gastroenterologists, a tracking system was used (mail or by phone). RESULTS: A total of 456 gastroenterologists returned at least 1 case on 1748 patients. Median patient age was 51 years; sex-ratio was 5.07. Median duration between the first CP sign and the inclusion was 41 months. CP cause was alcoholism (84%), hereditary (1%), cystic fibrosis (1%), idiopathic (9%), other (6%). CP diagnosis was certain in 77%: calcifications (85%), ductal abnormalities (57%), and histology (8%). CP symptoms were: chronic abdominal pain (53%), acute pancreatitis episodes (67%), pseudocysts (40%), bi-liary tract compression (21%), diabetes mellitus (32%), pancreatic exocrine insufficiency (36%). Maximal annual incidence was 4,646 (crude annual incidence: 7.7 per 100,000; 12.9 in male; 2.6 in female) and prevalence was 15,832 cases (crude prevalence: 26.4 per 100,000; 43.8 in male; 9.0 in female). CONCLUSION: New CP patients attending gastroenterologists are about 5,000 a year. CP prevalence is about 16,000 patients (in France: 60,400,000 inhabitants). Frequency of main complications is close to hospital series, confirming that results issued from these centers are not or a few biased.

Adolescent↗