Growth status in children with cystic fibrosis based on the National Cystic Fibrosis Patient Registry data: Evaluation of various criteria used to identify malnutrition

doi:10.1016/S0022-3476(98)70024-1

The Journal of Pediatrics

Volume 132, Issue 3, March 1998, Pages 478-485

https://doi.org/10.1016/S0022-3476(98)70024-1 Get rights and content

Abstract

Objectives: The objectives of this study were to determine growth status and to identify malnutrition with various anthropometric indicators in children with cystic fibrosis (CF) based on cross-sectional analysis of the 1993 National CF Patient Registry data. Methods: Heights and weights of 13,116 children with CF were evaluated with percentile, percent of reference median, Z-score, and percent ideal weight-for-height based on National Center for Health Statistics/Centers for Disease Control growth references. Malnutrition was defined by four criteria: (1) height-for-age <5th percentile (“stunting”) or weight-for-age <5th percentile (“wasting”) (2) height-for-age <90% of reference median or weight-for-age <80% of reference median, (3) height-for-age <5th percentile or percent ideal weight-for-height <85%, and (4) height-for-age <90% of reference median or weight-for-height <85% of reference median. Results: Mean and median height- and weight-for-age were found to be at the 30th and 20th percentiles in children with CF. Malnutrition (height- or weight-for-age <5th percentile) was particularly pronounced in infants (47%) and adolescents (34%) and patients with newly diagnosed CF (44%). A significant sex difference (p < 0.01) in the occurrence of stunting (height-for-age <5th percentile) was observed during adolescence: boys 11 to 14 years of age showed lower occurrence of stunting (19%) compared with girls (29%), whereas the opposite trend was observed at 15 to 18 years (34% in male patients vs 28% in female patients). Conclusion: Twenty percent of all children in the 1993 National CF Patient Registry were <5th percentile for height- or weight-for-age. A significant discrepancy was found when different criteria were used to distinguish “stunting” versus “wasting” in malnourished children with CF. (J Pediatr 1998;132:478-85)

Section snippets

Study Population

The heights and weights of 13,116 children (age range 0 to 18 years) from 114 CFF-accredited CF centers for the year 1993 were obtained from the National CF Patient Registry.⁴ This pediatric CF population constituted 68% of all patients with CF registered at the National CF Patient Registry for the year 1993.

Computation of Growth Percentiles, Z-scores, and Percent Ideal Weight-for-height

Height-for-age percentile, height-for-age Z-score, height-for-age percent of reference median, weight-for-age percentile, weight-for-age Z-score, weight-for-age percent of reference median,

Patient Characteristics

Of the 13,116 children maintained in the 1993 National CF Patient Registry, 52.8% were male. Most (95.6%) were white, 3.6% were black, and 0.8% were other races. The age distribution of this pediatric CF population indicated that approximately 40% of the children with CF were infants (<1 year) or adolescents (11 to 18 years). These children have higher energy and nutrient requirements for growth and therefore may be at higher risk for malnutrition than children at other ages.

Growth Status as Assessed by HAP and WAP

The 5th, 50th, and

Discussion

Overall, our results confirm and extend previous findings that children with CF have subnormal growth at all ages as reflected by their low mean HAP and WAP (~30th percentile) and median HAP and WAP (~20th percentile) compared with the NCHS/CDC reference population. In addition, 20% of the children with CF were below the 5th percentile for HAP or WAP during the year 1993. The occurrence of “stunting” (HAP <5th percentile) and “wasting” (IWH <85%) and the combination of “stunting and wasting”

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Poor growth has long been a characteristic feature of cystic fibrosis (CF) and is significantly linked to lung function and overall health status. Improvements in pulmonary and nutrition care for patients with cystic fibrosis (CF) have resulted in better growth outcomes; however, height gains have not paralleled the improvements in weight in children with CF, and patients with more severe CF mutations remain significantly more affected. Many factors affect the growth hormone-IGF-1 axis and the growth plate of the long bones, including the chronic inflammatory state associated with CF. There are also increasing data on the direct effects of CFTR on bone and implications for CFTR modulators in attaining optimal growth. Treatments aimed at improving growth in CF are also reviewed here.
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In children with cystic fibrosis (CF), recovery from growth faltering within 2 years of diagnosis (Responders) is associated with better growth and less lung disease at age 6 years. This study examined whether these benefits are sustained through 12 years of age.
Longitudinal growth from 76 children with CF enrolled in the Wisconsin CF Neonatal Screening Project was examined and categorized into 5 groups: R₁₂, R₆, and R₂, representing Responders who maintained growth improvement to age 12, 6, and 2 years, respectively, and I₆ and N_6, representing Non-responders whose growth did and did not improve during ages 2–6 years, respectively. Lung disease was evaluated by % predicted forced expiratory volume in one second (FEV₁) and chest radiograph (CXR) scores.
Sixty-two percent were Responders. Within this group, 47% were R₁₂, 28% were R₆, and 25% were R₂. Among Non-responders, 76% were N₆. CF children with meconium ileus (MI) had worse lung function and CXR scores compared to other CF children. Among 53 children with pancreatic insufficiency without MI, R₁₂ had significantly better FEV₁ (97–99% predicted) and CXR scores during ages 6–12 years than N₆ (89–93% predicted). Both R₆ and R₂ experienced a decline in FEV₁ by ages 10–12 years.
Early growth recovery in CF is critical, as malnutrition during infancy tends to persist and catch-up growth after age 2 years is difficult. The longer adequate growth was maintained after early growth recovery, the better the pulmonary outcomes at age 12 years.
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MicroRNAs are involved in multiple pathophysiological networks and in the pathogenesis of a broad spectrum of human disorders, including cancer and inflammatory diseases.
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Children with inflammatory conditions may also be at risk of developing insulin resistance as a result of the inflammatory process and concurrent therapy.
Chronic inflammation may lead to a continuum of abnormalities in the Growth hormone/Insulin-like growth factor 1 (GH/IGF-I) axis, including relative GH insufficiency, GH/IGF-I resistance due to down regulation of GH and IGF-I receptors, changes in GH and IGF-I bioavailability due to modifications of binding proteins, and/or impaired GH/IGF-I signaling.
The aim of this review is first to summarize the current knowledge concerning microRNAs involved in inflammation in the most relevant chronic inflammatory diseases in childhood, second to provide new insights into miRNA regulation of growth and insulin sensitivity mediated by the inflammatory processes. We evaluated single microRNAs involved in inflammation in the single conditions mentioned above and verified which had validated and predicted targets within the GH receptor, IGF-I type 1 receptor and insulin receptor interactomes.
The findings show a new link among inflammation, growth and insulin sensitivity mediated by miRNAs that warrants further research in the future.
A smartphone-based chloridometer for point-of-care diagnostics of cystic fibrosis
2017, Biosensors and Bioelectronics
Chloride in sweat is an important diagnostic marker for cystic fibrosis (CF), but the implementation of point-of-care systems for diagnosis is hindered by the prohibitive costs of existing chloride sensors. To enable low cost diagnostic solutions, we recently established a citrate-derived synthesis platform for the development of new fluorescence sensors with high selectivity for chloride. As a next step, we herein designed a smartphone operated chloridometer that optimizes the analytical performance of the citrate-derived sensor materials for the detection of chloride in sweat. The sensor material demonstrated a wide linear range of 0.8–200 mM chloride and a diffusion-limited response time; sweat chloride levels corresponded to measurable changes in fluorescence emission that was captured by a smartphone. Clinical validation was performed with sweat from individuals with and without CF, demonstrating convenient sweat diagnostics with reliable detection of cystic fibrosis. To our knowledge, this is the first clinical study of a smartphone-based chloride sensor, paving the way for point-of-care diagnostic systems for CF.
Nutrition and cystic fibrosis
2017, Nutrition in the Prevention and Treatment of Disease
Cystic fibrosis (CF) is a life-shortening common genetic disorder characterized by malnutrition and lung disease. The primary care of CF focuses on optimizing nutritional status and preventing pulmonary deterioration. Malnutrition is prevalent and closely associated with both pulmonary status and survival. The major causes of malnutrition in CF include increased losses from maldigestion/malabsorption primarily due to pancreatic insufficiency; increased requirement from increased energy expenditure associated with pulmonary infection; and decreased intake from poor appetite associated with CF complications. Nutritional therapy for CF patients focuses on a high-caloric, high-fat diet combined with pancreatic enzyme therapy, with the goal of promoting normal growth for children and normal weight status for adults. Guidelines for nutritional assessment and management of CF patients are presented in this chapter.
Endocrine Disorders in Cystic Fibrosis
2016, Pediatric Clinics of North America
Citation Excerpt :
Individuals with symptoms that might be due to hypoglycemia may benefit from point-of-care (glucometer) testing at the time of symptoms. Reduced linear growth has been reported in several studies in CF (reviewed by Wong and colleagus23) with a prevalence of short stature (defined by height z score <−2 SDs below the Centers for Disease Control mean) found in approximately 20% of all people with CF in 1993.24 A clear likely contributor to poor growth is fat and micronutrient malabsorption, which may not always be completely treated using pancreatic enzyme replacement therapy.

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^☆: From the Departments of Pediatrics and Biostatistics, University of Wisconsin School of Medicine, Madison, Wisconsin; the Medical Department, Cystic Fibrosis Foundation, Bethesda, Maryland; and the Department of Pediatrics/Pulmonology, Tulane University School of Medicine, New Orleans, Louisiana.

^☆☆: Supported by National Institutes of Health grant R01 DK34108.

^★: Reprint requests: Hui-Chuan Lai, PhD, RD, Department of Pediatrics/Biostatistics, UW-Madison, K6/428 Clinical Science Center, 600 Highland Ave., Madison, WI 53792.

^★★: 9/21/84157

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Growth status in children with cystic fibrosis based on the National Cystic Fibrosis Patient Registry data: Evaluation of various criteria used to identify malnutrition☆,☆☆,★,★★

Abstract

Section snippets

Study Population

Computation of Growth Percentiles, Z-scores, and Percent Ideal Weight-for-height

Patient Characteristics

Growth Status as Assessed by HAP and WAP

Discussion

J Pediatr

J Clin Epidemiol

Nutr Res

Am J Clin Nutr

Am J Clin Nutr

Am J Clin Nutr

Am J Clin Nutr

Am J Clin Nutr

Relative underweight in cystic fibrosis and its prognostic value

Acta Paediatr Scand

Chronic undernutrition/growth retardation in cystic fibrosis

Clin Gastroenterol

Prediction of mortality in patients with cystic fibrosis

N Engl J Med

Relationship between clinical parameters and linear growth in children with cystic fibrosis

Am J Human Biol

Early nutritional therapy in cystic fibrosis

Pediatr Pulmonol Suppl

The assessment of nutritional status of the community (WHO Mono graphs Series 63)