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Query: UMLS:C0240066 (iron deficiency)
 7,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 47-year-old man with sickle cell anemia, chronic cor pulmonale, and congestive heart failure died following a short illness. A diagnosis of iron deficiency was established during life by usually accepted criteria including a low serum ferritin concentration. Autopsy showed no stainable iron in the bone marrow, liver, and the heart. Marked deposits of iron were seen in the kidneys and the atrophic spleen. These findings suggest that the serum ferritin concentration may not reflect the metabolically sequestered stores of iron in the spleen and the kidneys.
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PMID:Serum ferritin and sequestered stores of body iron. 619 14

Two 19-year-old men with sickle cell anemia and hypogonadism had hypothalamic dysfunction that responded to oral clomiphene therapy. The patients had no nutritional deficiencies or anatomic lesions known to result in the hypogonadism associated with sickle cell anemia. Their sickle cell disease was characterized by infrequent crises, severe hemolytic anemia, urinary iron loss, and iron deficiency. Partial hypothalamic hypogonadism was shown by low levels of testosterone, low to low-normal levels of luteinizing hormone and follicle-stimulating hormone, and a nearly normal rise in gonadotropin levels in response to exogenous gonadotropin releasing hormone. Treatment with oral clomiphene raised luteinizing hormone, follicle-stimulating hormone, and testosterone levels to normal, and induced puberty in both patients. Treatment was discontinued in one patient because of the onset of priapism, but was continued for 10 months without side effects in the other. Severe hypogonadism in patients with sickle cell anemia should be thoroughly evaluated and clomiphene therapy considered in patients with hypothalamic dysfunction.
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PMID:Clomiphene-responsive hypogonadism in sickle cell anemia. 641 55

In a patient with sickle cell anemia, iron deficiency was accompanied by hypochromic, microcytic RBCs, absence of bone marrow iron, and a low serum ferritin level. The mean corpuscular hemoglobin concentration (MCHC) was decreased (27.6 g/dL) and was associated with an extreme scarcity of sickled erythrocytes in blood smears. Iron therapy resulted in reticulocytosis and an increase in sickled erythrocytes. In vitro studies demonstrated a decrease in sickling of erythrocytes as a function of oxygen saturation of the blood when the patient was iron deficient. The whole blood oxygen dissociation curve showed a substantial decrease in oxygen pressure necessary to produce 50% saturation of hemoglobin at pH 7.4 and 37 degrees C (P50), indicating an increased oxygen affinity. These data suggest that a reduction of the MCHC induced by iron deficiency may ameliorate sickling.
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PMID:Iron deficiency and sickle cell anemia. 667 16

Iron status was investigated in 60 adults with sickle cell anemia as determined by stainable iron in bone marrow aspirates, serum ferritin, serum iron, and the total iron-binding capacity. Seventeen patients (28%) were found to have absent bone marrow iron. There was an excellent inverse correlation between serum ferritin and the serum transferrin (p less than 0.001), and a significant positive correlation between serum ferritin and transferrin saturation (p less than 0.005), bone marrow iron (p less than (p less than 0.001), and history of prior blood transfusion (p = 0.005). Results of complete radiologic examination of the gastrointestinal tract and proctoscopy were negative in the 17 patients in the iron-deficient group. The high incidence of iron deficiency may be related to excessive urinary losses of iron. Our data indicate that serum ferritin values of less than 30 ng/ml are diagnostic of iron deficiency in patients with sickle cell anemia, with a high degree of specificity (98.7%). However the sensitivity of the test at a serum ferritin level of 30 ng/ml is only 32%. The diagnostic evaluation and the management of sickle cell anemia in iron-deficient patients needs to be better defined.
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PMID:Iron stores in adults with sickle cell anemia. 671 57

Thirty-seven patients with SCD were studied: 24 were diagnosed as homozygous Hb S on the basis of their haematological findings, and alpha:non-alpha globin chain ratios were found to be balanced in all. Thirteen patients were thought to have alpha or beta thalassaemia interaction with Hb S on the basis of low MCV and MCH, family history and/or presence of Hb A on electrophoresis. Six of them had abnormal alpha:non-alpha ratio (one had a ratio of 0.72 suggestive of alpha thalassaemia, and five had ratios between 1.4 and 1.9, compatible with beta thalassaemia interaction). The remaining seven patients with microcytosis had balanced globin chain synthesis and five were found to be iron deficient. Five additional patients (3 with Hb SS and 2 with Hb S/beta thalassaemia) had lower than normal serum ferritin concentration. The analysis of case histories disclosed that peptic ulceration, recurrent epistaxis and multiple pregnancies could account for iron loss in seven patients. These findings indicate that iron deficiency may be common in SCD and should be excluded as a cause of microcytosis. Microcytosis, in the absence of conclusive family studies and/or presence of Hb A on electrophoresis, is an unreliable indicator of alpha or beta thalassaemia interaction with Hb S.
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PMID:Iron deficiency in sickle cell anaemia. 688 17

Overt iron deficiency was diagnosed in four patients with sickle cell disease. Three patients had homozygous SS and one had hemoglobin SC disease. The cause in each case was proved or suspected blood loss. Iron repletion was accompanied by increases in the blood hemoglobin and hematocrit levels, erythrocyte mean corpuscular volume, and mean corpuscular hemoglobin concentration (MCHC) and by change in the RBC morphologic characteristics from hypochromic microcytic to normochromic normocytic. The diagnosis of iron deficiency was confirmed by the finding of a low serum ferritin level, a high serum total iron-binding capacity, or both. Two patients who had had no painful crises while they were iron deficient began having crises again, and another patient had painful crises for the first time after the blood values improved. Whether a lowered MCHC is beneficial to patients with sickle cell diseases is an important but unanswered question.
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PMID:Overt iron deficiency in sickle cell disease. 711 79

The amount of trapped plasma in the microhematocrit red blood cell column of samples from 25 normal individuals and 102 patients was investigated. The mean value for the normal individuals was 1.53%, and the mean values for the samples from the patient groups ranged from 1.41% to 1.82%. These groups included patients with sickle cell disease, iron deficiency, and hereditary spherocytosis. There was an inverse correlation between trapped plasma and the MCH in the samples from patients with iron-deficiency (MCH less than or equal to 25.0 pg). These findings have relevance to the determination of the PCV and derived red blood cell indices.
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PMID:Trapped plasma in the microhematocrit. 713 20

A cohort study of sickle cell disease from birth has allowed observations on the disease without the symptomatic selection inherent in previous series. The development of haematological indices from birth to 6 years in male and female infants with homozygous sickle cell (SS) disease is presented and compared with values in age and sex matched controls with a normal haemoglobin (AA) genotype previously presented elsewhere. In SS disease total haemoglobin levels fell rapidly from birth to a plateau at 3-6 months before falling again to 15 months after which no age related change occurred. Mean cell haemoglobin concentration fell from birth to lowest values at 15-18 months before increasing to reach the level present at birth by the age of 5 years. Red cell counts fell rapidly after birth to a plateau at 2 months, increased slightly to 6 months and then fell steadily throughout the remaining period of the study. The men cell volume and mean cell haemoglobin also fell rapidly after birth reaching the lowest values by 6 months and then increased progressively. Female patients showed significantly higher MCV from 4 to 8 months and significantly higher haemoglobin levels from 15 months to 4 1/2 years. Compared to AA controls, SS patients manifested significantly lower levels of haemoglobin from 2 weeks, and red cell counts from 1 month, and significantly higher levels of MCHC from 4 months to 3 years, MCV from 8 months to 5 years, and serum iron levels from 1 to 4 years. Children with SS disease were partially protected from iron deficiency in early childhood, perhaps by increased intestinal absorption of iron, and the associated increase in intracellular haemoglobin concentration might be disadvantageous during this high risk period.
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PMID:The development of haematological changes in homozygous sickle cell disease: a cohort study from birth to 6 years. 727 16

We determined the prevalence and optimal methods for laboratory diagnosis of iron deficiency anemia in patients with sickle cell disease. Laboratory investigations of 38 nontransfused and 32 transfused patients included transferrin saturation, serum ferritin, mean corpuscular volume (MCV), and free erythrocyte protoporphyrin (FEP). Response to iron supplementation confirmed the diagnosis of iron deficiency anemia in 16% of the nontransfused patients. None of the transfused patients were iron deficient. All iron-deficient patients (mean age 2.4 yr) had a low MCV, serum ferritin less than 25 ng/ml, transferrin saturation less than 15%, and FEP less than 90 micrograms/dl RBC. Following therapy, all parameters improved and the hemoglobin concentration increased greater than 2 g/dl. A serum ferritin below 25 ng/ml was the most reliable screening test for iron deficiency. There were 13% false positive results with transferrin saturation, 3% with MCV, and 62% with FEP. FEP values correlated strongly with reticulocyte counts. The high FEP was in part due to protoporphyrin IX and not completely due to zinc protoporphyrin, which is elevated in iron deficiency. We conclude that iron deficiency anemia is a potential problem in young nontransfused sickle cell patients. Serum ferritin below 25 ng/ml and low MCV are the most useful screening tests.
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PMID:The diagnosis of iron deficiency anemia in sickle cell disease. 729 5

Iron studies were performed on 25 children with homozygous sickle cell disease. The majority (80%) of patients had never been transfused. Surprisingly, the results showed that all had low serum iron and low transferrin saturation. Three children had no marrow iron stores while the rest had diminished amounts of iron. This may be an important finding in view of recent efforts at fortifying common salt with iron. The exact effects of iron deficiency on sickle cell disease are not known and a controlled trial is called for.
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PMID:Iron deficiency in sicle cell disease. 737 58


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