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Meconium ileus
Published in Mark Davenport, James D. Geiger, Nigel J. Hall, Steven S. Rothenberg, Operative Pediatric Surgery, 2020
Robert J. Vandewalle, Frederick J. Rescorla
Gastrointestinal problems after the newborn period are relatively common in children with CF. These include intussusception, appendiceal distension with inspissated material and appendicitis, rectal prolapse, and gallbladder disease. In the early 1990s, colonic strictures were reported by several centers in association with high pancreatic enzyme replacement. One of the most common gastrointestinal disorders in children and adolescents with CF is distal intestinal obstruction syndrome. The obturator obstruction often occurs after an intercurrent illness in which the child has a decreased oral intake and stops taking the pancreatic enzyme supplement.
The abdomen
Published in Spencer W. Beasley, John Hutson, Mark Stringer, Sebastian K. King, Warwick J. Teague, Paediatric Surgical Diagnosis, 2018
Spencer W. Beasley, John Hutson, Mark Stringer, Sebastian K. King, Warwick J. Teague
Appendicitis may produce an inflammatory mass in the right iliac fossa. In the acute phase, the overlying guarding may make it difficult to palpate the mass, at least until the patient is anaesthetised. The mass is tender, has ill-defined margins and is immobile. It should be distinguished from a large ovarian cyst, which tends to be non-tender and mobile, and can be manipulated in and out of the pelvis. However, if an ovarian cyst has undergone torsion, it too may be difficult to palpate on account of overlying guarding and other signs of peritonism. In a child under 3 years of age, the possibility of an intussusception mass must be considered. In these children, there will be colicky pain and vomiting. In the older child, a relatively painless immobile mass is more suggestive of a lymphoma; other supporting features may include weight loss, fever, lymphadenopathy and hepatospleno- megaly. Less common causes include Meckel diverticulitis, Yersinia lymphadenitis, duplication cyst, lymphangioma, omental cyst, mesenteric cyst, omental infarction, tuberculosis or actinomycosis. In children with cystic fibrosis, severe constipation or distal intestinal obstruction syndrome from inspissated luminal gut content or appendiceal pathology may produce a palpable abdominal mass.
Respiratory, Including Cystic Fibrosis
Published in Timothy G Barrett, Anthony D Lander, Vin Diwakar, A Paediatric Vade-Mecum, 2002
Timothy G Barrett, Anthony D Lander, Vin Diwakar
Children are usually admitted for administration of IV antibiotics because of chest exacerbation, weight loss, as part of a ‘regular’ antipseudomonal antibiotic regime, or in preparation for an operation under general anaesthetic. Occasionally, inpatient management may be required for meconium ileus equivalent/distal intestinal obstruction syndrome, intractable feeding problems or a rarer complication of CF.
Acute and chronic non-pulmonary complications in adults with cystic fibrosis
Published in Expert Review of Respiratory Medicine, 2019
Lucile Regard, Clémence Martin, Guillaume Chassagnon, Pierre-Régis Burgel
Distal intestinal obstruction syndrome (DIOS) is a frequent complication in CF patients and is brought on by the accumulation of viscous fecal material and sticky mucous secretions in the distal ileum and cecum [27]. In a recent study, DIOS incidence in the adult CF population was estimated at 7.8 episodes/1000 patient-years [27]. DIOS diagnosis can be challenging because symptoms might not be specific (abdominal pain, nausea, vomiting). Differential diagnoses include constipation, appendicitis, appendicular abscess, mucocele of the appendix, intussusception, or malignancy. In 2011, an international consensus introduced the differentiation between Complete DIOS (C DIOS) and Impending DIOS (I DIOS) [28]. A CT scan is the gold standard for diagnosis and usually shows intestinal wall thickening, inspissated fecal material in the distal ileum, and small bowel dilation [29] (Figure 1(b)). Risk factors for DIOS include severe CFTR genotype, history of meconium ileus, PI, previous DIOS, CF-related liver disease (CFLD), CF-related diabetes (CFRD), airway colonization with P. aeruginosa, and dehydration [27,30]. Delayed diagnosis and treatment are both associated with significant morbidity [22]. For I DIOS, oral rehydration combined with oral osmotic laxatives (containing polyethylene glycol (PEG) or sodium meglumine diatrizoate [Gastrografin®]) is almost always sufficient.
Cystic fibrosis: a model for precision medicine
Published in Expert Review of Precision Medicine and Drug Development, 2018
Paul McNally, Catherine M. Greene
Several abdominal organs are affected in CF including the pancreas, gut, and liver. At birth, most newborns will have evidence of pancreatic exocrine insufficiency (PI) and develop malabsorption, however some infants, particularly those with mutations associated with residual CFTR function, will be pancreatic sufficient. Malabsorption is associated with malnutrition and growth failure, particularly with advancing age and lung disease. Impaired intestinal epithelial function, particularly in the setting of PI, can be associated with partial or complete bowel obstruction either in infancy (meconium ileus) or later in life (distal intestinal obstruction syndrome). Intestinal disease in CF is characterized by inflammation, dysbiosis, and dysmotility, and in some can lead to a significant impairment in quality of life [27]. Liver disease in CF is still poorly understood and associated with a heterogeneous collection of features including impairments in transaminases, abnormal ultrasound appearance and progressive focal and multilobar biliary cirrhosis [28,29]. Progressive liver disease in CF is associated with a higher mortality, but is hard to predict and has no treatment aside from liver transplant [30].
Tezacaftor and ivacaftor for the treatment of cystic fibrosis
Published in Expert Review of Respiratory Medicine, 2020
Sarah L. Paterson, Peter J. Barry, Alexander R. Horsley
VX-445 in combination with tez/iva has been reported in a phase 2 trial [59] to result in improvement in absolute ppFEV1 of 13.8% and 11% in Phe508del/MF heterozygotes and Phe508del homozygotes, respectively. In addition, a significant reduction in sweat chloride and improvement in CFQ-R scores were seen in both genotype groups, to a much higher degree than observed differences with dual therapy. Minor adverse respiratory events were common, with a profile not dissimilar to the preceding tez/iva trials. A 4% rate of serious adverse events was observed in the VX-445 triple therapy group, which consisted of pulmonary exacerbation and distal intestinal obstruction syndrome (DIOS). Three patients discontinued therapy.