Edema, Anemia, Hypoproteinemia, and Acrodermatitis Enteropathica

Muñiz, Antonio E.; Bartle, Sam; Foster, Robert J.

doi:10.1097/01.pec.0000113881.10140.50

Cited by 27 publications

(27 citation statements)

References 23 publications

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“…2,[4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] The dermatitis typically presents as erythematous, scaling papules that later progress to extensive coalescing, desquamating plaques. The eruption is usually first noted in the perineum anywhere from several days to 15 months after birth, with subsequent spread to the perioral region, extremities, and trunk.…”

Section: Commentmentioning

confidence: 99%

“…32 Anemia is also found in both states and likely arises because of decreased globin synthesis secondary to inadequate protein. 15 Hepatic steatosis (fatty liver) may result from a number of malnutrition-related deficits. When reported, hepatic enzyme levels were elevated in nearly all cases of CF dermatitis, and fatty liver was diagnosed in 5 of 6 liver biopsy or autopsy findings.…”

Section: Commentmentioning

confidence: 99%

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Cystic Fibrosis Presenting With Dermatitis

Wenk¹,

Higgins²,

Greer³

2010

Arch Dermatol

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Background Patients with cystic fibrosis classically present with evidence of pulmonary disease, exocrine pancreatic insufficiency, and high sweat chloride concentrations. Dermatitis as an initial manifestation of the disease is uncommon and has been attributed to multiple nutritional deficiencies. Observation We describe the case of a 3-month-old female infant with cystic fibrosis presenting with dermatitis in the setting of protein-energy malnutrition. A review of the laboratory study results in this case and others showed that a deficiency in zinc, essential fatty acids, and protein likely contributes to the development of the rash seen in cystic fibrosis. Conclusions Given the frequent delay in diagnosis, as well as the increased morbidity and mortality associated with protein-energy malnutrition in these patients, it is important to consider cystic fibrosis as a possible diagnosis in any infant presenting with a rash and other signs of malnutrition. The relative contribution of specific nutritional deficiencies and the degree to which they influence and interact with each other in producing the dermatitis remain unclear, although they may all affect a common underlying metabolic pathway

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Section: Commentmentioning

confidence: 99%

Section: Commentmentioning

confidence: 99%

Cystic Fibrosis Presenting With Dermatitis

Wenk¹,

Higgins²,

Greer³

2010

Arch Dermatol

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“…Up to 10% of patients with CF presented with hypoproteinemia, delayed development, anemia, and edema due to the kwashiorkor caused by pancreatic insufficiency. Cutaneous symptoms as a presenting sign in CF are rare [1,5,6,12,15]. The skin eruption is usually seen between the 3rd to 7th months of age and is seen about a month before edema and severe malnutrition.…”

Section: Discussionmentioning

confidence: 97%

A 4-month-old boy with acrodermatitis enteropathica-like symptoms

et al. 2008

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“…One symptom secondary to malabsorption may be pursued rather than the whole picture interpreted: edema and anemia with referral to a child nephrologist; hyperinflation, increased liver span and difficulty feeding with referral to the pediatric cardiologist; hematomas with referral to the child oncologist; perianal and perioral rash with referral to the dermatologist, and anemia and hemolysis with referral to the pediatric hematologist. All these situations have been reported [11] . In addition, children are often not acutely ill.…”

Section: Symptoms Leading To the Diagnosis Of Cfmentioning

confidence: 90%

Diagnostic Procedures, Clinical Characteristics and Counseling in Cystic Fibrosis

Boeck

2006

Ann Nestlé [Engl]

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Most patients with the eventual diagnosis of cystic fibrosis (CF) present in the first months of life with classical symptoms such as meconium ileus, failure to thrive and recurrent or persistent cough. Disease manifestations are obvious, progressive and concern different organ systems. In these patients the standard diagnostic test is the sweat test. A sweat chloride concentration of >60 mmol/l confirms the clinically suspected diagnosis. Since the discovery of the CFTR (CF transmembrane conductance regulator) gene, it has become obvious that the clinical spectrum is broad. Several categories of patients have been proven to carry two CFTR mutations: patients with classic CF; patients with milder symptoms and disease onset during adolescence or even adulthood; patients with a single clinical feature, e.g. recurrent pancreatitis, sclerosing cholangitis, ‘idiopathic’ bronchiectasis, and male infertility. The latter two categories are described as atypical or non-classic CF. It is very important that these patients know that they do not suffer from classic CF, but that they have a risk factor for developing a CF-like disease that warrants intermittent follow-up by physicians familiar with the very wide disease spectrum. In patients with classic CF, chronic progressive bacterial sinopulmonary infection and inflammation with intermittent exacerbations are the most typical disease manifestations. Ninety percent of patients have pancreatic insufficiency. Postpubertal men are sterile. During their lifetime CF patients may develop disease manifestations and complications in the sinuses, lung, pancreas, liver, intestine, esophagus, bone, joints, etc. The complex mix of possible disease manifestations makes each patient unique. As they become older, the number of complications tends to increase and their physical condition tends to worsen. Many young adult CF patients have decreased exercise tolerance because of advanced lung disease; 20% have CF-related diabetes mellitus. Classic CF is a life-shortening disorder and, even with the most intense treatment and follow-up, the current median survival is around 34 years. This puts an enormous psychological stress on patients and families. Counseling to prevent disease recurrence in siblings and in the extended family is important and complex.

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Edema, Anemia, Hypoproteinemia, and Acrodermatitis Enteropathica

Cited by 27 publications

References 23 publications

Cystic Fibrosis Presenting With Dermatitis

Cystic Fibrosis Presenting With Dermatitis

A 4-month-old boy with acrodermatitis enteropathica-like symptoms

Diagnostic Procedures, Clinical Characteristics and Counseling in Cystic Fibrosis

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