Manifestaciones Pulmonares

G Model

YPRRV-785; No. of Pages 6
Paediatric Respiratory Reviews xxx (2011) xxx–xxx

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Paediatric Respiratory Reviews

Mini-Symposium: Pulmonary Complications of Paediatric Systemic Disorders

Pulmonary Complications of Endocrine and Metabolic Disorders
Carlos E. Milla *, Jacquelyn Zirbes
Center for Excellence in Pulmonary Biology, StanfordUniversity Medical School, Palo Alto, CA, USA

EDUCATIONAL AIMS The reader will become familiar with how to: a. b. c. d. e. Identify key factors on how endocrine and metabolic disorders affect the respiratory system. Recognise disorders of other bodily systems and the impact on the respiratory system Appreciate the specific pulmonary involvement of liposomal storage disorders Distinguish thepulmonary manifestations of patients with diabetes Describe the pulmonary phenotype associated with specific endocrine disorders

A R T I C L E I N F O

S U M M A R Y

Article history: Available online xxx Keywords: diabetes pituitary dysfunction pseudohypoaldosteronism hypothyroidism hyperthyroidism hypoparathyroidism lysosomal storage disorders

There are many important respiratorymanifestations of endocrine and metabolic diseases in children. Acute and chronic pulmonary infections are the most common respiratory abnormalities in patients with diabetes mellitus, although cardiogenic and non-cardiogenic pulmonary oedema are also possible. Pseudohypoaldosteronism type 1 may be indistinguishable from cystic fibrosis (CF) unless serum aldosterone, plasma renin activity, and urinaryelectrolytes are measured and mutation analysis rules out CF. Hypo- and hyperthyroidism may alter lung function and affect the central respiratory drive. The thyroid hormone plays an essential role in lung development, surfactant synthesis, and lung defence. Complications of hypoparathyroidism are largely due to hypocalcaemia. Laryngospasm can lead to stridor and airway obstruction. Ovarian tumours,benign or malignant, may present with unilateral or bilateral pleural effusions. Metabolic storage disorders, primarily as a consequence of lysosomal dysfunction from enzymatic deficiencies, constitute a diverse group of rare conditions that can have profound effects on the respiratory system. ß 2011 Elsevier Ltd. All rights reserved.

INTRODUCTION Although hormones and growth factors influencedevelopment of lung tissue, particularly in the perinatal period and first few years of life, no direct significant derangements in lung function or respiratory morbidity are associated with the most prevalent endocrine conditions in paediatric patients. During late stages of lung development, a number of hormones, including corticosteroids, growth, and thyroid hormones have been shown to stimulatedifferentiation of type II alveolar epithelial cells and produce architectural rearrangements in the lung connective tissue to promote gas exchange. Glucocorticoids in the perinatal period delay Clara cell differentiation, increase surfactant proteins mRNA, and stimulate pulmonary cytochrome P450. These effects translate

into the known developmental changes induced by glucocorticoids that have led totheir antenatal use as a preventative measure in premature births. This article provides a review of how endocrine and metabolic disorders affect the respiratory system. Emphasis is placed on the pulmonary manifestations of diabetes, pseudohypoaldosteronism, hypothyroidism, hyperthyroidism, hypoparathyroidism, and metabolic diseases such as lysosomal storage disorders. Obesity-hypoventilationsyndrome and the respiratory complications of obesity will not be addressed within this article. SPECIFIC DISORDERS Diabetes Pulmonary complications, outside from infections, are not a major problem for children with diabetes, even when the disease has progressed to a point where other complications are present.1 Many mechanisms have been proposed for an increased risk of

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