Imaging changes in the pancreas in cystic fibrosis: a retrospective evaluation of 55 cases seen over a period of 9 years.

BACKGROUND: Pathologic changes of the pancreas have been observed as early as the recognition of the disease termed initially "cystic fibrosis of the pancreas". Atrophy of the gland and its fatty infiltration were considered as usual features. The aim of this study was to follow-up the evolution of cystic fibrosis pancreas and to define its successive stages in correlation with the clinical, biochemical, and imaging findings. METHODS: Fifty-five patients were followed up during 9 years. The patients' genetic backgrounds were systematically performed. Blood lipase levels were analyzed systematically at each consultation of the patients and in the event of bouts of abdominal pains. Imaging using mainly echograms and tomodensitometric scans were regularly performed: echograms every 6 months, and tomodensitometric scans every 1 to 2 years. Magnetic resonance imaging was performed in four patients. RESULTS: Five groups of patients were identified on the basis of tomodensitometric scan findings: normal pancreas (n = 4), incomplete lipomatosis of the pancreas (n = 9), complete lipomatosis of the pancreas (n = 23), cystic pancreas (n = 5), macrocystic pancreas (n = 1), atrophic pancreas (n = 13). Pancreas exocrine function was not correlated with findings. Forty episodes of pancreatitis were observed in seven patients. They had bouts of abdominal pain and elevation of lipase levels. Five of these patients were composite heterozygotes (D508/other). Incomplete lipomatosis represents an intermediate stage leading toward complete lipomatosis or toward atrophy after pancreatitis. CONCLUSIONS: Studies of pancreatic function should be performed routinely in cystic fibrosis, especially in pancreatic sufficiency or in patients with normal pancreas images. Acute pancreatitis should be diagnosed and properly identified to be differentiated from other acute abdominal syndromes occurring in cystic fibrosis.

[Tuberculous pneumopathy in the course of cystic fibrosis]. / Pneumopathie tuberculeuse au cours d'une mucoviscidose.

BACKGROUND: Tuberculosis is rarely seen in patients with cystic fibrosis. CASE REPORT: A 14-year old female adolescent, regularly followed for a well-tolerated form of cystic fibrosis, developed an acute respiratory infection with consolidation of the left inferior lobe, and no response to the usual antibiotic treatment of cystic fibrosis. Mycobacterium tuberculosis was found in aspirate by fibroscopy, on Loewenstein medium. No familial or social infection contact were identified. Antituberculous chemotherapy with three drugs brought about a prompt improvement of sytemic signs, weight gain, resolution of pulmonary foci and sedation of biological findings referable to inflammation. CONCLUSION: This case report reminds us that tuberculosis may occur in cystic fibrosis patients. Loewenstein cultures should routinely be made when faced with an unexplainable worsening of the condition.

Liver cirrhosis in cystic fibrosis--therapeutic implications and long term follow up.

Experience gained from liver studies in 450 patients with cystic fibrosis, seen in a 38 year period from 1964 to 1992, is surveyed. Of these, 31 (7%) showed findings that indicated multilobular cirrhosis. There was a slight but not significant male predominance: 19 males against 12 females. Liver disease had its onset during childhood in most cases. The natural course of liver disease and of cirrhosis is protracted. All patients were routinely evaluated by way of: (i) clinical examination, (ii) biochemical studies and specifically estimation of transaminases and gamma glutamyltransferase, and (iii) liver imaging, ultrasonography, and computed tomography. The study aimed to detect early liver disease, that is multilobular cirrhosis and its complications, with a view to optimal introduction of treatment with ursodeoxycholic acid as this drug shows promise for preventing or stabilising the cirrhotic process. Effects of surgical treatment on portal hypertension are surveyed. These include portacaval shunting, partial splenectomy (considered the procedure of choice), liver transplant in the event of liver failure, or a triple transplant (liver, lungs, and heart) if necessary. One triple transplant was successfully performed in a boy of 10 years with a 2 year follow up.

[Anomalies of the sperm, vas deferens and epididymis in cystic fibrosis]. / Anomalies du sperme, des déférents et de l'épididyme dans la mucoviscidose.

Ten adult patients with cystic fibrosis presented with lesions of the genital tract at different levels. Bilateral lesions of the vas deferens were constant and resulted in azoospermia in 9 cases; the fertile patient had a son. The sperm of these 10 patients was compared to that of 5 adult controls. A decrease in sperm volume, pH and fructose and an increase in citric acid and acid phosphatase were observed in the cystic fibrosis group. It is concluded that all subjects with azoospermia should be investigated for cystic fibrosis by means of the sweat test.

[Alpha-1-antitrypsin and cystic fibrosis (author's transl)]. / Alpha-1-antitrypsine et mucoviscidose.

A methodical study of the alpha-1-antitrypsin serum level and the Pi type in 69 CF children, 156 CF heterozygote parents and 200 blood donors from Paris, has been made. Although the PiZ allele frequency was found to be higher in the CF population, especially in fathers, than in the reference population, the difference is not statistically relevant. Therefore there does not seem to be a relation between cystic fibrosis and the Pi alleles system.

[Successive courses of intensive therapy in cystic fibrosis (author's transl)]. / Cures intensives d'antibiotiques dans les mucoviscidoses évoluées.

Twenty-four patients aged from 3 to 30 years and suffering from advanced cystic fibrosis received several courses of intensive antibiotic therapy combined with prolonged respiratory physiotherapy with postural drainage. Antibiotics included carbenicillin 500 mg/kg/day and aminoglycosides in high dosage (6 mg/kg/day). These treatments were administered to prevent aggravation of chronic bronchitis and to suppress acute exacerbations. Tolerance was satisfactory, and all patients felt better, at least during the first courses. Improvement was also observed in the main respiratory function parameters. In a total of 95 courses only two side-effects (severe dizziness and giant urticaria) were recorded. Intensive courses of combined antibiotic therapy associated with physiotherapy constitute a new way of treating severe, progressive cystic fibrosis. The patients are made comfortable, and their survival time is increased by several years.

[Alpha-1-antitrypsin and cystic fibrosis (author's transl)]. / Alpha-1-antitrypsine et mucoviscidose.

A methodical study of the alpha-1-antitrypsin serum level and the Pi type in 69 CF children, 156 CF heterozygote parents and 200 blood donors from Paris, has been made. Although the PiZ allele frequency was found to be higher in the CF population, especially in fathers, than in the reference population, the difference is not statistically relevant. Therefore there does not seem to be a relation between cystic fibrosis and the Pi alleles system.

[Biliary lithiasis in cystic fibrosis. Study of the bile salts in the serum and in the bile in one case (author's transl)]. / Lithiase biliaire au cours de la mucoviscidose. Etude des sels biliaires dans le sérum et la bile chez une malade.

Biliary lithiasis was discovered in a girl aged 11 suffering from Cystic Fibrosis and who underwent partial small bowel resection for meconium ileus. A cholecystectomy was performed at 15. Examination of the stones showed that they were composed of cholesterol and chemical examination of the bile showed an increase of phospholipids and a decrease in bile salts. There was also a modification of the ratio (Formula: see text) in the serum and the bile. All those factors contribute to the insolubility of cholesterol and to the formation of lithiasis. For all those reasons, the authors recommend a regular radiologic surveillance of the gallbladder in Cystic Fibrosis.

[Coprologic anomaly in mucoviscidosis. Presence of albumin in low concentration in the feces]. / Une anomalie coprologique dans la mucoviscidose. Presence d'albumine--à faible concentration--dans les selles

With an immunological technique, presence of albumin was searched in the stools of 47 patients with cystic fibrosis and 54 controls. Albumin was found in 93% of cystic fibrosis, at a mean level of 90 mg/100 g of fresh stools. The test was negative in the 54 healthy controls. It was positive in one case of congenita IgA deficiency and in one untreated case of Hirschprung's disease. When found in the stools, albumin may give a useful additional diagnostic element.

Additional data on hepatic function tests in cystic fibrosis.

Fifty cystic fibrosis (CF) patients, of whom 9 had multilobular cirrhosis, were observed regularly for a period of 3 years and various liver function tests, indicating cytolysis, cholestasis and cellular insufficiency were performed. Immunoglobulin and prothrombin were assayed. In 9 patients with cirrhosis, the tests were generally abnormal. Two distinct biochemical patterns of cirrhosis were distinguished, one clearly cholestatic and the other of a more cellular type. The distinction was made on the basis of the IgA : Transferrin ratio and of gamma-glutamyl-transpeptidase levels. In the non-cirrhotic patients, a temporary increase of cytolysis and cholestasis was observed in 50% of the cases.