Answer to the letter to the editor “Quantitative bone ultrasound in pediatric patients with cystic fibrosis”

Cystic fibrosis (CF) is a common autosomal recessive genetic disorder where a deletion mutation and subsequent downstream alteration in transmembrane regulator proteins results in increased mucus viscosity. CF manifests clinically with chronic multisystem inflammation and recurrent infections. Nearly all children with CF have chronic sinusitis, and a large majority will have concurrent sinonasal polyposis. Chronic sinusitis and sinonasal polyposis in pediatric patients with CF can be managed conservatively initially, though most will fail medical management and require surgical intervention. Unfortunately, symptom resolution is marginal and polyp recurrence rates are high. Currently, no cure exists for CF and the mainstay of treatment is to provide symptomatic relief, and minimize disease morbidity.

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Population Pharmacokinetics of Tobramycin Inhalation Solution in Pediatric Patients With Cystic Fibrosis

Journal of Pharmaceutical Sciences ◽

10.1016/j.xphs.2017.06.010 ◽

2017 ◽

Vol 106 (11) ◽

pp. 3402-3409 ◽

Cited By ~ 4

Author(s):

Xinting Wang ◽

Stephan Koehne-Voss ◽

SivaNaga S. Anumolu ◽

Jing Yu

Keyword(s):

Cystic Fibrosis ◽

Population Pharmacokinetics ◽

Pediatric Patients

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Nutrition Management of Pediatric Patients Who Have Cystic Fibrosis

Pediatric Clinics of North America ◽

10.1016/j.pcl.2009.06.008 ◽

2009 ◽

Vol 56 (5) ◽

pp. 1123-1141 ◽

Cited By ~ 19

Author(s):

Suzanne H. Michel ◽

Asim Maqbool ◽

Maria D. Hanna ◽

Maria Mascarenhas

Keyword(s):

Cystic Fibrosis ◽

Pediatric Patients ◽

Nutrition Management

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Letter to the Editor

PEDIATRICS ◽

10.1542/peds.49.4.630b ◽

1972 ◽

Vol 49 (4) ◽

pp. 630-631

Author(s):

Henry Levison ◽

Norman Aspin

Keyword(s):

Cystic Fibrosis ◽

Disease Process ◽

Bronchial Tree ◽

Aerosol Therapy ◽

Letter To The Editor

Many physicians do not accept the hypothesis put forward in Dr. Chadwick's letter but rather they still accept the statement by the Committee on Therapy regarding the treatment of cystic fibrosis. This document states, "the aim of nebulization therapy in cystic fibrosis is to deposit particles of water or water-containing medication in the bronchial tree at the site of the disease process" and further goes on to state "nebulization or aerosol therapy must not be confused with humidification."

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Increased Plasma YKL-40 Level and Chitotriosidase Activity in Cystic Fibrosis Patients

10.21203/rs.3.rs-501880/v1 ◽

2021 ◽

Author(s):

Dilara Bal Topcu ◽

Gökcen Tugcu ◽

Berrin Er ◽

Sanem Eryilmaz Polat ◽

Mina Hizal ◽

...

Keyword(s):

Cystic Fibrosis ◽

Pediatric Patients ◽

Adult Patients ◽

Enzyme Linked Immunosorbent Assay ◽

Healthy Children ◽

Plasma Samples ◽

Stable Period ◽

Valuable Marker ◽

Chitotriosidase Activity ◽

Relevant Factors

Abstract Background We investigated plasma YKL-40 levels and chitotriosidase (CHIT1) activity in patients with cystic fibrosis (CF) lung disease and evaluated clinically relevant factors that may affect their levels. Methods Plasma samples were obtained from pediatric (n = 19) and adult patients (n = 15) during exacerbation, discharge and stable period of the disease. YKL-40 levels and chitotriosidase activity were measured by enzyme-linked immunosorbent assay and fluorometric assay, respectively. Data were compared with healthy children and adults of similar age. Results YKL-40 levels of pediatric and adult CF patients at all periods were significantly higher than controls (p < 0.001 and p < 0.05). CHIT1 activities of adult patients at all periods were significantly higher compared to controls (p < 0.05). On the other hand, CHIT1 activities of pediatric CF patients were similar with controls. YKL-40 levels of exacerbation period of adult CF patients were negatively correlated with % FVC (r= -0.800, p = 0.014) and % FEV1 (r= -0.735, p = 0.008). YKL-40 levels in the exacerbation period of pediatric CF patients were negatively correlated with % FVC (r= -0.697, p = 0.0082) and % FEV1 (r= -0.720, p = 0.006). Conclusions CHIT1 activity may be a valuable marker of chronic inflammation in adult CF patients who suffer from CF for a longer period of time compared to pediatric patients. Increased YKL-40 levels in both pediatric and adult patients compared to controls may point to a role in between CF pathology. Furthermore, as YKL-40 levels are correlated with FEV1 and FVC in patients, it may be useful for the monitoring of pulmonary function in CF patient.

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Letter to the Editor

PEDIATRICS ◽

10.1542/peds.43.5.905b ◽

1969 ◽

Vol 43 (5) ◽

pp. 905-906

Author(s):

Aubrey Milunsky

Keyword(s):

Cystic Fibrosis ◽

Bone Marrow ◽

Clinical Presentation ◽

Pancreatic Insufficiency ◽

Chloride Concentration ◽

Letter To The Editor ◽

Patient Reported ◽

Sweat Sample ◽

Chloride Concentrations

The patient reported in the foregoing letter is of particular interest in view of the recent observations on patients with tnisomy 21 and cystic fibrosis. The multiple possibilities that could explain the clinical presentation have no doubt been considered by the authors. However, the description of "hypoplastic thrombocytopenia" and later pancytopenia in this patient, associated with pancreatic insufficiency, leads to the serious consideration of Shwachman's syndrome (pancreatic insufficiency and bone marrow dysfunction). The wide discrepancy between the sodium and chloride concentrations in the sweat reported in their letter indicates that evaporation of water may have occurred from the sweat sample, leading to an apparently higher sodium and chloride concentration.

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