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Literature DB >> 8259877

Cystic fibrosis presenting as kwashiorkor with florid skin rash.

R J Phillips¹, C M Crock, M J Dillon, P T Clayton, A Curran, J I Harper.

Abstract

Two infants with a florid erythematous rash and generalised oedema, hypoalbuminaemia, and anaemia were found to have cystic fibrosis. This rare presentation is associated with false negative sweat tests, delays in diagnosis, and a considerable mortality. It is proposed that this presentation represents a manifestation of kwashiorkor secondary to malabsorption. The recognition that these infants have kwashiorkor provides some insight into the pathogenesis and management of their illness.

Entities: Disease Species

Mesh：

Year: 1993 PMID： 8259877 PMCID： PMC1029554 DOI： 10.1136/adc.69.4.446

Source DB: PubMed Journal: Arch Dis Child ISSN： 0003-9888 Impact factor: 3.791

18 in total

1. HYPOPROTEINEMIA AND EDEMA IN INFANTS WITH CYSTIC FIBROSIS OF THE PANCREAS.

Authors: D S FLEISHER; A M DIGEORGE; L A BARNESS; D CORNFELD
Journal: J Pediatr Date: 1964-03 Impact factor: 4.406

Review 2. Anemia associated with protein deficiency.

Authors: E B Adams
Journal: Semin Hematol Date: 1970-01 Impact factor: 3.851

3. Skin breakdown in kwashiorkor responds to zinc.

Authors: M H Golden; B E Golden; A A Jackson
Journal: Lancet Date: 1980-06-07 Impact factor: 79.321

Review 4. Transport proteins as indices of protein status.

Authors: M H Golden
Journal: Am J Clin Nutr Date: 1982-05 Impact factor: 7.045

5. The role of protein deficiency in the aetiology of kwashiorkor.

Authors: J Landman; A A Jackson
Journal: West Indian Med J Date: 1980-12 Impact factor: 0.171

6. Protein deficiency, energy deficiency, and the oedema of malnutrition.

Authors: M H Golden
Journal: Lancet Date: 1982-06-05 Impact factor: 79.321

7. Cystic fibrosis manifesting with acrodermatitis enteropathica-like eruption. Association with essential fatty acid and zinc deficiencies.

Authors: R C Hansen; R Lemen; B Revsin
Journal: Arch Dermatol Date: 1983-01

8. Albumin and nutritional oedema.

Authors: M H Golden; B E Golden; A A Jackson
Journal: Lancet Date: 1980-01-19 Impact factor: 79.321

9. Zinc and immunocompetence in protein-energy malnutrition.

Authors: M H Golden; P S Harland; B E Golden; A A Jackson
Journal: Lancet Date: 1978-06-10 Impact factor: 79.321

10. The incidence of anemia, hypoproteinemia, and edema in infants as presenting symptoms of cystic fibrosis: a retrospective survey of the frequency of this symptom complex in 130 patients with cystic fibrosis.

Authors: O H Nielsen; B F Larsen
Journal: J Pediatr Gastroenterol Nutr Date: 1982 Impact factor: 2.839

6 in total

Cystic fibrosis presenting as kwashiorkor with florid skin rash.

1. HYPOPROTEINEMIA AND EDEMA IN INFANTS WITH CYSTIC FIBROSIS OF THE PANCREAS.

Review 2. Anemia associated with protein deficiency.

3. Skin breakdown in kwashiorkor responds to zinc.

Review 4. Transport proteins as indices of protein status.

5. The role of protein deficiency in the aetiology of kwashiorkor.

6. Protein deficiency, energy deficiency, and the oedema of malnutrition.

7. Cystic fibrosis manifesting with acrodermatitis enteropathica-like eruption. Association with essential fatty acid and zinc deficiencies.

8. Albumin and nutritional oedema.

9. Zinc and immunocompetence in protein-energy malnutrition.

10. The incidence of anemia, hypoproteinemia, and edema in infants as presenting symptoms of cystic fibrosis: a retrospective survey of the frequency of this symptom complex in 130 patients with cystic fibrosis.

1. Myelodysplastic features in an infant with cystic fibrosis presenting with anaemia, oedema and failure to thrive.

Review 2. Neonatal erythroderma: differential diagnosis and management of the "red baby".

Review 3. Nutritional management of cystic fibrosis.

Review 4. Newborn screening for cystic fibrosis.

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

6. Hair depigmentation and dermatitis - an unexpected presentation of cystic fibrosis.