Severe Clostridium difficile–associated colitis in young patients with cystic fibrosis

doi:10.1016/S0022-3476(98)70511-6

The Journal of Pediatrics

Volume 132, Issue 1, January 1998, Pages 177-179

https://doi.org/10.1016/S0022-3476(98)70511-6 Get rights and content

Abstract

We report four patients with cystic fibrosis and fulminant Clostridium difficile–associated colitis: two died, and one required hemicolectomy. Three of four patients carried the N1303K mutation. Severe and fatal C. difficile colitis can occur in cystic fibrosis patients, possibly with a genotype-specific predilection (i.e., N1303K/other). Because cystic fibrosis patients may have a wide spectrum of gastrointestinal symptoms, disease caused by C. difficile must be considered when these patients have acute abdominal pain, diarrhea, or severe leukocytosis. (J Pediatr 1998;132:177-9)

Section snippets

Patients

Four children with CF from three geographically distinct centers (total population of 244 patients, age range 8 months to 51 years, median age 12 years), had CDAC over a 13-month period (Table). All patients had significant pulmonary disease and pancreatic insufficiency. One patient received immunosuppressive therapy after lung transplantation.

Clinical Diagnosis of CDAC

CD-associated disease was clinically defined as a diarrheal syndrome, usually with crampy abdominal pain and tenderness in a patient with previous or

Epidemiologic Investigations

At the time of CDAC onset, no other cases were recognized in the hospital and no diarrheal diseases were noted in home or school contacts. Despite efforts to identify an epidemiologic factor common to all four (antibiotics, other drugs, type and dose of pancreatic enzyme replacement, demography including socioeconomic status), no link between these patients was found. Three of the four patients received ceftazidime and an aminoglycoside. These antibiotics are used widely in CF, and it is

Discussion

Four children with CF had severe CDAC; each had crampy abdominal pain, fever, and diarrhea. Laboratory examinations and various imaging modalities were also characteristic (Table). Sigmoidoscopy, when done, was diagnostic and should be performed early in any CF patient with suspected CDAC.

The manifestations of CD infection in children may range from asymptomatic colonization or mild self-limited diarrhea to toxic megacolon and severe colitis.2, 3 In CF patients, crampy abdominal pain and loose

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Cited by (36)

Finding the relevance of antimicrobial stewardship for cystic fibrosis
2020, Journal of Cystic Fibrosis
Antimicrobials have undoubtedly improved the lives of people with CF, but important antimicrobial-related toxicities and the emergence of antimicrobial-resistant bacteria associated with their use must be considered. Antimicrobial stewardship (AMS) is advocated across the spectrum of healthcare to promote the appropriate use of antimicrobials to preserve their current effectiveness and to optimise treatment, and it is clear that AMS strategies are applicable to and can benefit both non-CF and CF populations. This perspective explores the definition and components of an AMS program, the current evidence for AMS, and the reasons why AMS is a challenging concept in the provision of CF care. We also discuss the elements of CF care which align with AMS programs and principles and propose research priorities for AMS in CF.
Complicated Clostridium difficile colitis in children with cystic fibrosis: Association with gastric acid suppression?
2014, Journal of Cystic Fibrosis
Citation Excerpt :
Other risk factors for C. difficile infection common in the CF population include gastrostomy and GI tract surgery [19]. One of the oldest studies in a hospital setting showed a high asymptomatic (32%) carriage rate of toxigenic strains of C. difficile in patients with CF who were on systemic antibiotics [14]. The rate was 17% in non-CF control subjects who were on antibiotics.
Patients with cystic fibrosis (CF) have several risk factors for Clostridium difficile colonization such as frequent hospitalization and exposure to a broad array of antibiotics utilized for the control, eradication, and prophylaxis of respiratory pathogens. However, despite this high rate of colonization, the occurrence of C. difficile infection (CDI) in CF is rare. We report three children with CF who presented with severe community-associated CDI. All three children had complicated courses and one died. These children were in good health without significant morbidities, and were not frequently hospitalized nor did they receive frequent antibiotic courses. The occurrence of 3 severe cases within a 15-month period prompted us to report these cases and review the literature in regard to CDI. We reviewed the CF GI tract as possible risk factors for a high rate of C. difficile colonization in individuals with CF. Since a high percentage of individuals with CF are on gastric acid blocking agents, we also focused on gastric acid suppression as a potential risk factor for CDI.
Pseudomembranous colitis in cystic fibrosis
2013, Paediatric Respiratory Reviews
Citation Excerpt :
The chloride secretory defect may result in unresponsiveness of CF enterocyte to C.difficile toxin similar to cholera toxin.8 Rivlin el al have reported 4 young CF patients with severe C.difficile associated colitis of which 3 patients had N1303K mutation suggesting possible genotype specific predilection to severe disease.4 The use of proton pump inhibitors are not associated with increased risk of C. difficile infection in paediatric CF patients.9
Cystic fibrosis (CF) patients may require frequent courses of antibiotics and repeated hospital admissions. Although children with CF have high carriage rate for C.difficile, they rarely develop colitis. Pseudomembranous colitis is more common in adult post lung transplant CF patients. Although rare, paseudomembranous colitis should be considered in CF patients presenting with abdominal symptoms even in the absence of diarrhoea.
Recurrent Clostridium difficile colitis in cystic fibrosis: An emerging problem
2013, Journal of Cystic Fibrosis
Citation Excerpt :
Several risk factors have been shown to be associated with CDAD in patients with CF including recent antibiotic use, impaired immunity and decreased gastric acidity. A genotype link (N1303K mutation) that may increase susceptibility to CDAD has been described between CF and C. difficile [6]. However, despite the frequent antibiotic use in patients with CF, the incidence of CDAD remains relatively low.
To examine the incidence of recurrent Clostridium difficile infection in patients with cystic fibrosis (CF), including patients who had undergone lung transplantation, and review clinical findings in hospitalized patients with C. difficile colitis.
A retrospective chart review was performed to examine the clinical presentation and management of patients with cystic fibrosis (CF) who received care at the University of Wisconsin Hospital and Clinics (UWHC) from 1994 to 2011 and were prospectively identified with C. difficile colitis.
Ten cases of C. difficile associated disease (CDAD) occurred in patients with CF followed by our Adult CF Center over a period of 17 years, and 4 patients were bilateral lung transplant recipients. Two of the lung transplant recipients had recurrent CDAD that lead to fulminant pancolitis, surgical intervention, and shock. Two patients in the non-transplant group experienced recurrent C. difficile infection that led to fulminant pancolitis with associated systemic inflammatory response syndrome and required colectomy.
C. difficile colitis can cause life threatening illness in patients with CF, and symptoms may be subtle and/or atypical and lead to significant delay in diagnosis. Patients with recurrent C. difficile colitis are at high risk of fatal outcome, and empiric therapy should be considered for patients with previous C. difficile colitis even in the absence of disease when broad-spectrum antibiotics are given to treat bacterial infection.
Nonpulmonary Manifestations of Cystic Fibrosis
2012, Kendig and Chernick's Disorders of the Respiratory Tract in Children
Nonpulmonary Manifestations of Cystic Fibrosis
2006, Kendig's Disorders of the Respiratory Tract in Children

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^☆: From the Department of Pediatrics, Carmel Medical Center, Haifa, The B. Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Department of Pediatrics, Sheba Medical Center, Tel Hashomer, Department of Pediatrics, Shaare Zedek Medical Center, Jerusalem, Israel.

^☆☆: Reprint requests: Joseph Rivlin, MD, Department of Pediatrics, Carmel Medical Center, 7 Michal Street, Haifa 34362, Israel.

^★: 9/22/82115

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The Journal of Pediatrics

Abstract

Section snippets

Patients

Clinical Diagnosis of CDAC

Epidemiologic Investigations

Discussion

References (11)

Escherichia coli heat-stable enterotoxin-mediated colonic C1 secretion is absent in cystic fibrosis

Gastroenterology

Clostridium difficile colitis

N Engl J Med

Clostridium difficile and gastroenteritis: How strong is the association in children?

Pediatr Infect Dis

Role of Clostridium difficile in childhood diarrhea

Pediatr Infect Dis J

Antibiotic-associated colitis and cystic fibrosis

Dig Dis Sci

Cited by (36)

Finding the relevance of antimicrobial stewardship for cystic fibrosis

Complicated Clostridium difficile colitis in children with cystic fibrosis: Association with gastric acid suppression?

Pseudomembranous colitis in cystic fibrosis

Recurrent Clostridium difficile colitis in cystic fibrosis: An emerging problem

Nonpulmonary Manifestations of Cystic Fibrosis

Nonpulmonary Manifestations of Cystic Fibrosis

Severe Clostridium difficile–associated colitis in young patients with cystic fibrosis☆,☆☆,★

Abstract

Section snippets

Patients

Clinical Diagnosis of CDAC

Epidemiologic Investigations

Discussion

Gastroenterology

Clostridium difficile colitis

N Engl J Med

Clostridium difficile and gastroenteritis: How strong is the association in children?

Pediatr Infect Dis

Role of Clostridium difficile in childhood diarrhea

Pediatr Infect Dis J

Antibiotic-associated colitis and cystic fibrosis

Dig Dis Sci