Cystic Fibrosis Study Questioned

PEDIATRICS ◽  
1984 ◽  
Vol 74 (1) ◽  
pp. 169-169
Author(s):  
JOANN ATER

In Reply.— Wagener and colleagues make a valid point that increase in plasma volume can be an appropriate response to hypoxia, without the development of polycythemia. This probably is a factor contributing to the inappropriately low hemoglobin values we found in our patients with cystic fibrosis. However, as in anything in medicine, there is rarely a single reason for any clinical observation. Our finding that some of our patients with low iron stores, as reflected by low serum ferritin values, had a significant increase in hemoglobin values with oral iron therapy, provides good evidence that iron deficiency may be one factor limiting the development of polycythemia in these patients.

PEDIATRICS ◽  
1983 ◽  
Vol 71 (5) ◽  
pp. 810-814
Author(s):  
Joann L. Ater ◽  
John J. Herbst ◽  
Stephen A. Landaw ◽  
Richard T. O'Brien

Significant alterations in hemotologic function in cystic fibrosis are suggested by the observation that polycythemia is uncommon, even among cyanotic patients. To elucidate those factors that influence hematologic equilibrium, 39 stable patients with cystic fibrosis were evalulated with regard to hemoglobin, hematocrit, RBC indices, reticulocyte count, serum iron and total iron binding capacity, serum ferritin, vitamin E, and carboxyhemoglobin levels. Hemoglobin concentrations were below the 50th percentile for age in 90% of the patients, including the 23% who were cyanotic. Serum ferritin levels were below the mean for age in 85% and below 12 ng/mL in 33% of patients. Vitamin E levels were less than 5 µg/dL in 33%, indicating deficiency. Carboxyhemoglobin values were elevated in 64% of the patients. These data indicate that relative anemia is common in cystic fibrosis and suggest that iron and vitamin E deficiency may contribute to that anemia. Twenty-two patients with cystic fibrosis were then given 2 weeks of oral iron therapy followed by two to three additional weeks of iron and vitamin E. This therapeutic trial resulted in an increase in mean hemoglobin concentration from 13.87 to 14.50 g/dL (P < 0.01) associated with a significant increase in levels of serum ferritin (P < 0.001). The increase in hemoglobin occurred primarily during the second 2 weeks when patients were receiving both iron and vitamin E. However, we were unable to document evidence of increased hemolysis when patients were receiving iron therapy alone. This response to oral iron therapy is confirmation that iron deficiency contributes to the failure of some patients with cystic fibrosis to compensate hemotologically for hypoxia.


Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 5149-5149
Author(s):  
John Adamson ◽  
Zhu Li ◽  
Paul Miller ◽  
Annamaria Kausz

Abstract Abstract 5149 BACKGROUND Iron deficiency anemia (IDA) is associated with reduced physical functioning, cardiovascular disease, and poor quality of life. The measurement of body iron stores is essential to the management of IDA, and the indices most commonly used to assess iron status are transferrin saturation (TSAT) and serum ferritin. Unfortunately, serum ferritin is not a reliable indicator of iron status, particularly in patients with chronic kidney disease (CKD), because it is an acute phase reactant and may be elevated in patients with iron deficiency in the presence of inflammation. Recent clinical trials have shown that patients with iron indices above a strict definition of iron deficiency (TSAT >15%, serum ferritin >100 ng/mL), do have a significant increase in hemoglobin (Hgb) when treated with iron. These results are consistent with recent changes to the National Cancer Comprehensive Network (NCCN) guidelines, which have expanded the definition of functional iron deficiency (relative iron deficiency) to include a serum ferritin <800 ng/mL; previously, the serum ferritin threshold was <300 ng/mL. Additionally, for patients who meet this expanded definition of functional iron deficiency (TSAT <20%, ferritin <800 ng/mL), it is now recommended that iron replacement therapy be considered in addition to erythropoiesis-stimulating agent (ESA) therapy. Ferumoxytol (Feraheme®) Injection, a novel IV iron therapeutic agent, is indicated for the treatment of IDA in adult patients with CKD. Ferumoxytol is composed of an iron oxide with a unique carbohydrate coating (polyglucose sorbitol carboxymethylether), is isotonic, has a neutral pH, and evidence of lower free iron than other IV irons. Ferumoxytol is administered as two IV injections of 510 mg (17 mL) 3 to 8 days apart for a total cumulative dose of 1.02 g; each IV injection can be administered at a rate up to 1 mL/sec, allowing for administration of a 510 mg dose in less than 1 minute. METHODS Data were combined from 2 identically designed and executed Phase III randomized, active-controlled, open-label studies conducted in 606 patients with CKD stages 1–5 not on dialysis. Patients were randomly assigned in a 3:1 ratio to receive a course of either 1.02 g IV ferumoxytol (n=453) administered as 2 doses of 510 mg each within 5±3 days or 200 mg of oral elemental iron (n=153) daily for 21 days. The main IDA inclusion criteria included a Hgb ≤11.0 g/dL, TSAT ≤30%, and serum ferritin ≤600 ng/mL. The mean baseline Hgb was approximately 10 g/dL, and ESAs were use by approximately 40% of patients. To further evaluate the relationship between baseline markers of iron stores and response to iron therapy, data from these trials were summarized by baseline TSAT and serum ferritin levels. RESULTS Overall, results from these two pooled trials show that ferumoxytol resulted in a statistically significant greater mean increase in Hgb relative to oral iron. When evaluated across the baseline iron indices examined, statistically significant (p<0.05) increases in Hgb at Day 35 were observed following ferumoxytol administration, even for subjects with baseline iron indices above levels traditionally used to define iron deficiency. Additionally, at each level of baseline iron indices, ferumoxytol produced a larger change in Hgb relative to oral iron. These data suggest that patients with CKD not on dialysis with a wide range of iron indices at baseline respond to IV iron therapy with an increase in Hgb. Additionally, ferumoxytol consistently resulted in larger increases in Hgb relative to oral iron across all levels of baseline iron indices examined. Disclosures: Adamson: VA Medical Center MC 111E: Honoraria, Membership on an entity's Board of Directors or advisory committees. Li:AMAG Pharmaceuticals, Inc.: Employment. Miller:AMAG Pharmaceuticals, Inc.: Employment. Kausz:AMAG Pharmaceuticals, Inc.: Employment.


1975 ◽  
Vol 48 (6) ◽  
pp. 529-532 ◽  
Author(s):  
L. R. I. Baker ◽  
W. R. Cattell ◽  
J. A. Child ◽  
E. Savdie

1. Marrow-iron stores were absent or reduced in twenty-three of thirty-nine patients studied within 52 months of starting maintenance haemodialysis. 2. Oral iron was given to twelve patients (group I) with absent or reduced, and to eleven patients (group II) with normal or increased marrow-iron stores. 3. A significant increase in mean haemoglobin concentration and marrow iron was observed in group I. No significant change in mean haemoglobin concentration or marrow iron occurred in group II. Mean haemoglobin concentration after treatment was significantly higher in group I than in group II. 4. The four patients who had normal or increased marrow iron and who received no oral iron all suffered a fall in haemoglobin concentration, and three of them showed a reduction in marrow iron. 5. These findings indicate that continuous oral iron therapy should be given to all patients on maintenance dialysis to correct or prevent iron deficiency.


PEDIATRICS ◽  
1984 ◽  
Vol 74 (1) ◽  
pp. 169-169
Author(s):  
JEFFREY S. WAGENER ◽  
LYNN M. TAUSSIG ◽  
JAMES J. CORRIGAN

To the Editor.— Ater and associates1 reported that patients with cystic fibrosis (CF) increase their hemoglobin values while receiving oral iron therapy, and they suggested that this indicates a relative iron deficiency which helps to explain why hypoxic patients with CF do not develop polycythemia. Similar data have been reported by Nickerson and colleagues.2 However, neither study related hematologic response to degree of hypoxia and both studies failed to recognize that polycythemia is not a necessary response to chronic hypoxia.


1993 ◽  
Vol 33 (6) ◽  
pp. 661-661
Author(s):  
Helena U Suzuki ◽  
Mauro B Morais ◽  
Jose N Corral ◽  
Ulisses Fagundes-Neto ◽  
Nelson L Machado

2021 ◽  
pp. 1-8
Author(s):  
Kriti Puri ◽  
Joseph A. Spinner ◽  
Jacquelyn M. Powers ◽  
Susan W. Denfield ◽  
Hari P. Tunuguntla ◽  
...  

Abstract Introduction: Iron deficiency is associated with worse outcomes in children and adults with systolic heart failure. While oral iron replacement has been shown to be ineffective in adults with heart failure, its efficacy in children with heart failure is unknown. We hypothesised that oral iron would be ineffective in replenishing iron stores in ≥50% of children with heart failure. Methods: We performed a single-centre retrospective cohort study of patients aged ≤21 years with systolic heart failure and iron deficiency who received oral iron between 01/2013 and 04/2019. Iron deficiency was defined as ≥2 of the following: serum iron <50 mcg/dL, serum ferritin <20 ng/mL, transferrin >300 ng/mL, transferrin saturation <15%. Iron studies and haematologic indices pre- and post-iron therapy were compared using paired-samples Wilcoxon test. Results: Fifty-one children with systolic heart failure and iron deficiency (median age 11 years, 49% female) met inclusion criteria. Heart failure aetiologies included cardiomyopathy (51%), congenital heart disease (37%), and history of heart transplantation with graft dysfunction (12%). Median dose of oral iron therapy was 2.9 mg/kg/day of elemental iron, prescribed for a median duration of 96 days. Follow-up iron testing was available for 20 patients, of whom 55% (11/20) remained iron deficient despite oral iron therapy. Conclusions: This is the first report on the efficacy of oral iron therapy in children with heart failure. Over half of the children with heart failure did not respond to oral iron and remained iron deficient.


Author(s):  
Manal Mahmoud Atia ◽  
Rasha Mohamed Gama ◽  
Mohamed Attia Saad ◽  
Mohammed Amr Hamam

Greater prevalence of iron deficiency (ID) has been observed in overweight and obese children and adolescents. Hepcidin acts as a key regulator of iron metabolism. Hepcidin synthesis increases in response inflammatory cytokines especially Interleukin-6 (IL-6). Considering that obesity represents a low grade chronic inflammatory state, a high concentration of hepcidin has been found in obese children. Elevated hepcidin level in obese children is associated with diminished response to oral iron therapy. Lactoferrin is an iron-binding multifunctional glycoprotein and has strong capacity to modulate the inflammatory response by its capacity to reduce pro-inflammatory cytokine expression in vivo, including IL-6 and hepcidin. Aim of the Work: To compare the efficacy of lactoferrin versus oral iron therapy in treatment of obese children and adolescents with iron deficiency anemia and the effect of therapy on serum hepcidin and interleukin 6 levels. Methodology: This prospective randomized clinical trial was conducted on 40 obese children and adolescents aged between 6 –18 years suffering from iron deficiency anemia (IDA). They were equally randomized into one of 2 groups. Group A received regular oral lactoferrin in a dose of 100 mg/day. Group B received regular oral iron supplementation (Ferric hydroxide polymaltose) in a dose of 6 mg elemental iron/kg /day.Baseline investigations included complete blood count (CBC), iron profile (Serum ferritin, serum iron, total iron binding capacity (TIBC), transferrin saturation), serum Interleukin 6, and serum hepcidin. Reevaluation of CBC was done monthly while iron status parameters, serum IL-6 and serum hepcidin were reevaluated after 3 months of receiving regular therapy. Results: Significant elevations in hemoglobin, MCV, MCH, Serum ferritin, serum iron and transferrin saturation with lactoferrin therapy compared to oral iron therapy. Significantly Lower TIBC after 3 months of lactoferrin therapy while the decrease in TIBC was insignificant in the iron therapy group.Lower serum hepcidin and IL6 after 3 months of lactoferrin therapy with no significant change in serum hepcidin and IL6 after iron therapy. Conclusion: This study clearly demonstrated the superiority of lactoferrin over iron use as oral in the treatment of iron deficiency anemia in obese children not only for the better response of hematological and iron status parameters and less gastrointestinal side effects but also for its effect on decreasing inflammatory biomarkers as hepcidin and IL6.


2020 ◽  
Vol 143 (5) ◽  
pp. 496-499
Author(s):  
Attilio Di Girolamo ◽  
Marcello Albanesi ◽  
Filomena Loconte ◽  
Danilo Di Bona ◽  
Maria Filomena Caiaffa ◽  
...  

Iron deficiency is the main cause of anemia in both sexes, with women being more commonly affected. Iron therapy is currently considered an effective and safe remedy to replenish the iron storages. Iron can be administrated both orally and intravenously. In particular, intravenous (IV) iron therapy is widely used when oral iron preparations are either not tolerated or ineffective. Indeed, IV iron improves iron stores more rapidly. Two main immunological responses have been described for iron hypersensitivity reactions (HSRs): IgE-mediated allergy and complement activation-related pseudo-allergy. Here, we report 3 cases of adult patients with iron allergy, who were successfully treated with two different desensitization procedures, respectively. Analysis of these cases demonstrates that, in the presence of HSRs to iron products, desensitization is an effective and safe procedure that prevents treatment discontinuation and hence allows therapeutic target achievement.


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