Meconium Ileus Equivalent Research Articles

Notfälle bei erwachsenen Mukoviszidosepatienten

Cystic fibrosis is an inherited autosomal recessive metabolic disease caused by mutations on the CFTR gene. This leads to defective chloride channels on epithelial cell membranes and causes various disorders of the respiratory, gastrointestinal, and urogenital tracts.As a result, all exocrine glands produce a viscous secretion, leading to pulmonary symptoms such as chronic cough, secretion retention, recurring infections as well as bronchiectasis and obstructive lung emphysema. Gastrointestinal effects include exocrine and often also endocrine pancreatic insufficiency with chronic diarrhea and maldigestion syndrome as well as pancreoprivic diabetes mellitus; biliary cirrhosis occurs in 10% of cases. Additional effects include reduced fertility in women and infertility in men.Life-threatening complications include bleeding from the bronchial arteries, pneumothorax, and distal intestinal obstruction syndrome (DIOS), previously known as meconium ileus equivalent. Treatment requires rapid diagnosis and should be carried out in experienced centres, since the mortality rate can otherwise be up to 50%.

The ESPGHAN Cystic Fibrosis Working Group: Defining DIOS and Constipation in Cystic Fibrosis With a Multicenter Study on the Incidence, Characteristics, and Treatment of DIOS

In 2005 a group of pediatric gastroenterologists convened at the annual meeting of the European Society for Pediatric Gastroenterology and Nutrition (ESPGHAN) to reevaluate some important gastrointestinal manifestations of cystic fibrosis (CF). It had become evident that the key gastrointestinal symptoms of the disease, such as distal intestinal obstruction syndrome (DIOS), meconium ileus equivalent (MIE), and constipation, were ill-defined. Such a limitation could not only represent a bias for clinical studies evaluating the incidence and prevalence of these gastrointestinal manifestations in CF but also negatively influence the diagnosis and treatment of these manifestations of CF. The results of the activity of the ESPGHAN CF Working Group appear in this issue of JPGN, specifically in the article by Houwen et al (1). Cystic fibrosis is the most common fatal autosomal recessive disease among white populations, with a frequency of 1 in 2000 to 3000 live births. It is generally thought of as a lung disease because much of the associated morbidity rate and mortality rate is related to pulmonary complications (2). However, gastrointestinal complications also have become an increasingly important cause of the morbidity rate in part because of the improved life expectancy of patients with CF. The underlying pathophysiology of CF is related to abnormal chloride transport caused by mutations in the CF transmembrane conductance regulator gene (CFTR) located on chromosome 7. The mutations cause the production of abnormally tenacious mucus and secretions in the lungs, gut, pancreas, and hepatobiliary system. The gastrointestinal manifestations of CF can be broken down into 3 categories: intestinal, pancreatic, and hepatobiliary. One of the most common intestinal abnormalities is the DIOS, formerly known as “meconium ileus equivalent,” which is characterized by complete or partial obstruction of the bowel lumen by intestinal contents. Distal intestinal obstruction syndrome is caused by inspissated intestinal contents that completely or partially block the intestinal lumen, although a detailed understanding of this process remains unclear. In the last few years, different definitions of DIOS, MIE, and constipation in patients with CF have been used, but better definitions for the 3 conditions would now seem to be necessary for an improved assessment and management of these patients (3,4). The new definition proposed by the ESPGHAN CF Working Group for incomplete or impending DIOS is “a short history (days) of abdominal pain and/or distension and a faecal mass in ileo-caecum, but without signs of complete obstruction.” Constipation was defined as “abdominal pain and/or distension or a decline in the frequency of bowel movements in the last few weeks to months and/or increased consistency of stools in the last few weeks or months, while the symptoms are relieved by the use of laxatives.” Meconium ileus equivalent was identified with DIOS. The most important outcome of the consensus is that these specific definitions could avoid misdiagnosis between DIOS and constipation in patients with CF, as sometimes occurred in the past. The practical result of this update was immediately seen in the study because the impact of this review was evaluated in a questionnaire-based multicenter survey in which the new definitions were applied to a large number of patients younger than 18 years with CF, enrolled between 2001 and 2005 in 8 medical centers. Using the new criteria, the incidence of both DIOS and meconium ileus at birth resulted to be far higher than that reported in previous studies. It seems clear that the higher incidence of DIOS is mainly due to the newly established definitions, although new therapeutic approaches could have partly influenced the results of other recent studies. The authors have also reevaluated the correlation between DIOS and CFTR mutations, as organized into 5 classes (I–III severe mutations, IV–V mild mutations), comparing the CFTR genotype with the DIOS-positive or DIOS-negative phenotype. The great majority of the subjects with CF had a severe genotype, being homozygous for the most common mutation, ΔF508. The results of the present study seem to confirm the association between major CFTR dysfunction and DIOS, whereas the possible role of other genetic factors such as dysmotility and inflammatory status could be evaluated in the next clinical trials. In conclusion, the new definitions established by the consensus conference of the ESPGHAN CF Working Group are important for 2 main reasons: the epidemiology of the gastrointestinal complications of CF could be profoundly modified, and the new definitions will help the clinician perform a more specific diagnosis of MIE, DIOS, and constipation, thus improving not only the diagnostic accuracy but also the effectiveness of treatment.

Failure of medical treatment in an adult cystic fibrosis patient with meconium ileus equivalent

Meconium ileus equivalent is one of the lesser-known manifestations of cystic fibrosis. It manifests as distant small bowel obstruction caused by meconium-like stool plugs and occurs mostly in adult patients. With the improved overall survival of patients with cystic fibrosis, general surgeons may encounter this condition more often in the future. We treated a 19-year-old woman with cystic fibrosis who presented with complete distal small bowel obstruction. Medical therapy with Gastrografin and N-acetylcysteine failed to resolve the obstruction. At surgery, a meconium-like plug in the distal ileum was manually pushed into the colon with subsequent relief of symptoms. Meconium ileus equivalent should be considered and treated in cystic fibrosis patients presenting with small bowel obstruction.

Liver cirrhosis and portal hypertension in cystic fibrosis.

Liver disease is the second cause of death in cystic fibrosis. The most deleterious complication of liver disease is portal hypertension, which has an estimated prevalence of up to 8%. Portal hypertension may manifest itself by splenomegaly, hypersplenism, gastro-oesophageal bleeding and ascites. The aim of our study was to determine the prevalence, risk factors and invasive management of portal hypertension at our centre. One hundred and fifty patients with cystic fibrosis were followed up between 1975 and 2000 in the national cystic fibrosis centre in Israel. Forty patients (27%) had liver disease. All underwent clinical evaluation and laboratory and imaging studies. Portal hypertension was diagnosed in 10 patients (7%), of whom eight were male. The mean age at diagnosis was 11 years (range, 4-17 years). All had severe mutations of the cystic fibrosis transmembrane conductance regulator gene (the CFTR gene), pancreatic insufficiency, meconium ileus or distal intestinal obstruction syndrome and variceal bleeding. Seven patients underwent sclerotherapy to control acute bleeding. Four underwent portosystemic shunting (functioning up to 37 years). Two patients with severe lung and liver disease underwent transjugular intrahepatic portosystemic shunting, which provided bleeding control, but both died while waiting for lung/liver transplantation. One patient underwent liver transplantation due to liver failure and still had good liver and lung function 10 years later. Portal hypertension is more common among Israeli patients with cystic fibrosis. The unique genetic composition of our population may explain this phenomenon. Risk factors include male gender, pancreatic insufficiency, severe CFTR mutations, meconium ileus and meconium ileus equivalent. Sclerotherapy is the main option to control oesophageal variceal bleeding, while portosystemic shunts offer a prolonged alternative treatment for refractory bleeding. A transjugular intrahepatic portosystemic shunt and liver transplantation may also be effective, but further research is required in order to establish their role.

Intestinal button implantation for obstipation and fecal impaction in children

Chronic obstipation and fecal impaction in children can be difficult management problems for pediatricians. We describe a novel approach to the management of obstipation and fecal impaction: the implantation of the button gastrostomy device in the appendiceal stump or the terminal ileum. We report the procedure in two children. One child had pseudoobstruction syndrome complicated by recurrent obstipation; the other had cystic fibrosis complicated by recurrent obstruction from meconium ileus equivalent. Both children received a polyethylene glycol/electrolyte solution, with or without pancreatic enzymes, which was administered through the button. The children have remained essentially asymptomatic for at least 10 months. We believe that this is the first report of the use of a button gastrostomy device to successfully manage chronic obstipation and recurrent fecal impaction in children.

Image interpretation session. Cystic fibrosis with distal intestinal obstruction syndrome or meconium ileus equivalent.

Image interpretation session. Cystic fibrosis with distal intestinal obstruction syndrome or meconium ileus equivalent.

CT Monitoring of Therapy for Meconium Ileus

Meconium ileus equivalent is a common complication of cystic fibrosis in the postneonatal period. Because of the added risks of surgery in these patients, conservative management is preferred. We describe here a method of monitoring the conservative treatment of this condition using CT and suggest that its application will reduce the need for potentially dangerous surgical intervention.

The Age-Related Incidence of Meconium Ileus Equivalent in a Cystic Fibrosis Population

During the decade from 1976 to 1986, the age-related incidence of meconium ileus equivalent (MIE) was calculated on the basis of 240 Danish cystic fibrosis (CF) patients. In the first 5 years, the patients were given enteric-coated granules of pancreatic enzymes (Pancreatin, Rosco, Denmark), and a low fat diet was recommended. In the last 5 years, the Center recommended supplementation with acid-resistant, enteric-coated, encapsulated microspheres (Pancrease, Cilag, Birkerød, Denmark), and high-energy intake with a 40% fat content and no dietary restrictions. There was no difference in the incidence of MIE when these two 5-year periods were compared, and the overall incidence of MIE was low (5.4 MIEs/1,000 patient years). This may, at least in part, be due to the rather high intake of exocrine pancreas enzyme supplementation (EPES) (a mean intake of 0.9 capsules/kg/day). MIE occurred almost exclusively among patients greater than 15 years old and peaked in young adults aged 20-25 years (35.5 MIEs/1,000 patient years). The daily intake of EPES/kg of body weight declined significantly with age, and the patients who developed MIE received even less than average per day. Both of these points strengthen the view that a low enzyme dosage is likely to have an effect on the incidence of MIE.

The Age‐Related Incidence of Meconium Ileus Equivalent in a Cystic Fibrosis Population

SummaryDuring the decade from 1976 to 1986, the age‐related incidence of meconium ileus equivalent (MIE) was calculated on the basis of 240 Danish cystic fibrosis (CF) patients. In the first 5 years, the patients were given enteric‐coated granules of pancreatic enzymes (Pancreatin, Rosco, Denmark), and a low fat diet was recommended. In the last 5 years, the Center recommended supplementation with acid‐resistent, enteric‐coated, encapsulated microspheres (Pancrease, Cilag, Birkerφd, Denmark), and high‐energy intake with a 40% fat content and no dietary restrictions. There was no difference in the incidence of MIE when these two 5‐year periods were compared, and the overall incidence of MIE was low (5.4 MIEs/1.000 patient years). This may, at least in part, be due to the rather high intake of exocrine pancreas enzyme supplementation (EPES) (a mean intake of 0.9 capsules/kg/day). MIE occurred almost exclusively among patients >15 years old and peaked in young adults aged 20–25 years (35.5 MIEs/1.000 patient years). The daily intake of EPES/kg of body weight declined significantly with age, and the patients who developed MIE received even less than average per day. Both of these points strengthen the view that a low enzyme dosage is likely to have an effect on the incidence of MIE.

Appendicitis in cystic fibrosis: can morbidity be reduced?

The management of appendicitis in cystic fibrosis (CF) has been controversial, and most series report a mortality of over 30%. Difficulties are distinguishing appendicitis from meconium ileus equivalent (MIE) often cause diagnostic delay and advanced disease by the time surgery is undertaken. We report a series of 9 patients with appendicitis proven on histology in whom there was no mortality and minimal morbidity. Factors likely to reduce morbidity and mortality in appendicitis in children with CF include early diagnosis; preoperative intravenous antibiotics effective against both bowel and chest organisms; early appendicectomy; avoidance of right hemicolectomy, or drainage alone; thorough peritoneal irrigation with warm antibiotic saline solution; and continuous postoperative narcotic analgesia to allow early mobilisation and effective chest physiotherapy. Accurate early diagnosis is facilitated if these children are treated by respiratory paediatricians and paediatric surgeons in a centre familiar with the management of cystic fibrosis. Delay in appendicectomy worsens the underlying respiratory disease.

Acute hypomagnesaemia complicating the treatment of meconium ileus equivalent in cystic fibrosis.

Meconium ileus equivalent (MIE) is a common and often recurrent complication in adolescent and adult patients with cystic fibrosis (CF). MIE is characterized by partial or complete bowel obstruction, resulting from abnormally viscid mucofaeculant material in the terminal ileum and right colon. Patients present with recurrent abdominal pain, intestinal obstruction, and/or a palpable faecal mass. Conventional treatment consists of the oral and rectal administration of the mucolytic agent N-acetylcysteine, and hypertonic solutions of sodium diatrizoate. We describe the occurrence of acute decreases in plasma magnesium in all of seven patients treated with this regimen with marked hypomagnesaemia (less than 0.70 mmol/l) in four of the seven patients. No changes in plasma sodium, potassium, or calcium were observed.

Cystic fibrosis in adolescents and adults.

Three hundred and sixteen patients with cystic fibrosis were seen at the Brompton Hospital during 1965-83; 178 (56.3%) of them were male and 136 female, and their ages ranged from 12 to 51 years. Most patients presented in infancy with respiratory symptoms and malabsorption, but 19 (6%) were diagnosed in adult life, three in their 30s. Pulmonary disease was almost universal (99.7%), being responsible for 97% of all deaths and three quarters of hospital admissions. All patients had developed a productive cough by the age of 21 and over half before the age of 5. Many complained of wheezing, but reversible airflow obstruction was present in only 40% of those tested. Minor haemoptysis was very common (62%), but major episodes less so (10%). Pneumothorax was seen in 61 cases (19%), and was often recurrent. Some irreversible airflow obstruction was present in all patients with pulmonary disease. Two patients have been followed for over 20 years without showing appreciable decline in lung function. Thirty five patients (11%) had no symptoms of malabsorption. Acute meconium ileus equivalent was seen in 16% and a chronic partial obstruction with episodic symptoms in a further 19%. Diabetes mellitus developed in 36 patients, 13 of whom were insulin dependent. Hepatomegaly was common (29%), often occurring without abnormal results in biochemical tests of liver function; only 1% of patients developed portal hypertension with varices and ascites. Skin reactions to at least one common allergen, including Aspergillus fumigatus, were positive in 70%, but very few patients suffered from hay fever or eczema. One hundred and twenty one patients have died, 97% from infection or other pulmonary complications, and 195 were alive in December 1983 (mean age 23 years). Seventy eight per cent of patients were in full time education or full or part time employment, or were housewives, and only 41 were unemployed for reasons for health. Many patients are married and 10 women have borne children. Most patients were admitted to hospital only three or four times during the period of follow up and 50 individuals (16%) have never been in hospital at all. The improvement in prognosis and quality of life for adults with cystic fibrosis should encourage a positive attitude in those who care for them.

Granulomatous appendiceal disease in cystic fibrosis

Abdominal pain due to acute appendicitis in patients with cystic fibrosis is uncommon. A review of 572 patients with cystic fibrosis from the cystic fibrosis clinic of the Children's Hospital of Pittsburgh from 1959 to 1983 disclosed only 4 in whom the diagnosis of acute appendicitis lead to appendectomy. In 3 of these the diagnosis was confirmed at operation. In 1 child, laparotomy for a persistent asymptomatic abdominal mass disclosed a huge cecal fecaloma originating in the appendiceal lumen. Histological examination of the appendix established the diagnosis of cystic fibrosis. Large, symptomatic appendiceal granulomas were noted in 6 other children; these simulated abdominal tumors in 2. In 3 others there was smoldering infection and abscess with sepsis and in 1 a colocystic fistula. Such chronic granulomas, acute appendicitis, meconium ileus, and nonsurgical abdominal pain in cystic fibrosis can best be differentiated by careful clinical examination before and after treatment for meconium ileus equivalent. The granulomas involve not only the appendix but also the cecum and ascending colon. Contrast X-ray studies are of little value in diagnosis. The appendix in cystic fibrosis undergoes changes that are diagnostic even in the newborn; the granulomas described here appear to represent a progression of these changes.

Liver Injury After Oral and Rectal Administration of N-Acetylcysteine for Meconium Ileus Equivalent in a Patient With Cystic Fibrosis

N-acetylcysteine (NAC) is a mucolytic agent (Mucomyst, Mead-Johnson) that is often used for the treatment of meconium ileus equivalent, a complication of cystic fibrosis.1-3 No serious adverse reactions to nonparenterally administered NAC have been observed, and there have been no reports of significant hepatotoxicity. We describe a patient in whom severe hepatic dysfunction occurred on two occasions after the administration of NAC for the treatment of meconium ileus equivalent. CASE REPORT A 3-year-old, 12.5-kg, white boy with cystic fibrosis was admitted for treatment of a fractured left femur. His medications included theophylline, metaproterenol, cefaclor, nebulized isoethanine, and pancreatic enzyme replacement with meals.

Gastrografin in acute meconium ileus equivalent.

Twenty-five (37%) patients with cystic fibrosis attending our clinic have experienced acute meconium ileus equivalent. In one year 37 of 40 episodes were treated with single dose oral Gastrografin with an 81% success rate, 75% being treated as outpatients. Patients found this treatment preferable to other recommended treatment.

Constipation and Meconium Ileus Equivalent in Patients With Cystic Fibrosis

Constipation and its complications, particularly meconium ileus equivalent, may become management problems in patients with cystic fibrosis. The medical records of 168 patients with cystic fibrosis were reviewed for the prevalence of constipation and meconium ileus equivalent. Of 168 patients, 54 (32%) had experienced at least one episode of constipation which responded to oral or rectal laxative therapy. In 16 of the study group (9%) meconium ileus equivalent developed. Patients younger than 5 years of age had a lower prevalence and those older than 30 years of age had a much higher prevalence of both conditions. Those with prolonged histories of inadequately controlled steatorrhea appeared to be at higher risk for the eventual development of meconium ileus equivalent. Recurrences and complications of constipation may be avoided by instituting early and aggressive therapy.

Intra-abdominal complications of cystic fibrosis

In recent years patients with cystic fibrosis (CF) have experienced longterm survival and have demonstrated a number of intra-abdominal complications. This report evaluates the intra-abdominal complications seen in 69 of 189 children with cystic fibrosis from 1972 to 1983. Forty-one patients were boys and twenty-eight girls. Complications occurred in 36 neonates, with meconium ileus (MI) noted in 33 and giant cystic meconium peritonitis (GCMP) in 3. Meconium ileus equivalent occurred in seven older children presenting with bowel obstruction. In addition, rectal prolapse occurred in 12, inguinal hernia in 10, intussusception in 3, cholelithiasis in 3, GE reflux in 4, stress ulcer in 1 and appendicitis in 1. Three infants with GCMP survived resection and enterostomy. Infants with MI were divided into simple (15) or complicated (18) cases. Nonoperative therapy using gastrografin enema was successful in three of eight with simple MI. Operative enterotomy and irrigation was successful in three cases while resection and enterostomy was done in nine. MI was complicated by atresia, volvulus and/or perforation in 18 cases requiring resection and anastomosis or enterostomy. Survival for MI was 86% compared to 36% in 25 MI patients treated in the previous two decades. Meconium ileus equivalent was successfully managed using gastrografin enema in five of seven children. Only 3 of 12 children with rectal prolapse required repair. Two cases of intussusception were reduced while one required resection. Three of 10 children had hernia recurrence due to chronic pulmonary problems.(ABSTRACT TRUNCATED AT 250 WORDS)

The pathology of meconium ileus equivalent

The pathology of meconium ileus equivalent

The pathology of meconium ileus equivalent.

The pathology of meconium ileus equivalent.

The meconium ileus equivalent in mucoviscidosis

Meconium ileus equivalent (MIE) complicating cystic fibrosis of the pancreas (CF) increases in frequently with increasing age of patients. In the present paper the course of 11 children and adolescents with MIE diagnosed and treated at the University Dept. of Paediatrics in Zürich during the last 15 years, i.e. 9% of 120 CF patients, is analyzed. 9 were successfully managed by medical treatment alone, 1 three months old infant was treated surgically with no later relapse and a 26 years old patient with a chronic deleterious course leading to opiate dependency could be relieved only by ileostomy. 5 patients experienced only 1 episode of MIE, 2- two to three and in 4 a chronic refractory course with multiple episodes was probably due to an inadequate medical management. The evaluation of the events in our patients and of the available data from the literature allows the following conclusions: MIE is a preventable condition in CF patients; a rigorous medical treatment of exocrine pancreatic insufficiency supplemented with mucolytic agents orally can usually prevent MIE and relieve an established MIE; surgical treatments is indicated only in desperate situations.