In Reply: Pathogenesis of Hyposthenuria in Sickle Cell Anemia

Hans G. Keitel

doi:10.1542/peds.26.6.1051a

In Reply: Pathogenesis of Hyposthenuria in Sickle Cell Anemia

PEDIATRICS ◽

10.1542/peds.26.6.1051a ◽

1960 ◽

Vol 26 (6) ◽

pp. 1051-1052

Author(s):

Hans G. Keitel

Keyword(s):

Sickle Cell ◽

Sickle Cell Anemia ◽

Normal Level ◽

Urinary Concentration ◽

Urea Excretion ◽

Low Level ◽

Concentration Of Urine

In reply to the question raised by Dr. Morgan, it is now well established that urea plays an important part in the concentration of urine. Starting at a very low level of urea excretion, the urinary concentration maxima (as obtained during prolonged fluid deprivation, or following sustained vasopressin stimulation) is less than normal. When the rate of urea excretion is increased to the normal level, the maximal urinary concentration ability increases, but when the rate of urea excreted increases markedly, the urinary concentration maxima once again decreases.

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PATHOGENESIS OF HYPOSTHENURIA IN PERSONS WITH SICKLE CELL ANEMIA OR THE SICKLE CELL TRAIT

PEDIATRICS ◽

10.1542/peds.26.2.249 ◽

1960 ◽

Vol 26 (2) ◽

pp. 249-254

Author(s):

L. Schlitt ◽

H. G. Keitel

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Sickle Cell Anemia ◽

Renal Damage ◽

Sickle Cell Trait ◽

Urinary Concentration ◽

Cell Disease ◽

Renal Vessels

Hyposthenuria was investigated in subjects with sickle cell trait and in patients with sickle cell anemia. The following were observed: 1) in subjects with sickle cell trait both normal and reduced maxima of urinary concentration are found, whereas all untreated patients with sickle cell anemia over 6 months of age have hyposthenuria; 2) hyposthenuria becomes increasingly more severe with advancing age in both sickle cell anemia and sickle cell trait; 3) in a 6-month-old patient with sickle cell anemia and hyposthenuria, the maxima of urinary concentration returned to normal after two transfusions of normal erythrocytes. Reasons are presented for favoring the hypothesis that hyposthenuria in sickle cell disease is due to renal damage, possibly from intravascular sickling of erythrocytes in renal vessels or from the presence of "free" circulating S-hemoglobin.

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Low-level laser therapy of leg ulcer in sickle cell anemia

Revista Brasileira de Hematologia e Hemoterapia ◽

10.5581/1516-8484.20120018 ◽

2012 ◽

Vol 34 (1) ◽

pp. 65-66 ◽

Cited By ~ 3

Author(s):

Claudia Regina Bonini-Domingos ◽

Flavia Mariana Valente

Keyword(s):

Laser Therapy ◽

Sickle Cell ◽

Sickle Cell Anemia ◽

Leg Ulcer ◽

Low Level Laser Therapy ◽

Low Level ◽

Low Level Laser ◽

Level Laser

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Assessment of Salivary Zink Level and its impact on Periodontal Health among Children and Adolescence with Sickle Cell Anemia

10.21203/rs.3.rs-821539/v1 ◽

2021 ◽

Author(s):

Rogyia Eltayeb Hassan ◽

Lamis AbdelGadir Kaddam

Keyword(s):

Sickle Cell ◽

Sickle Cell Anemia ◽

Serum Zinc ◽

Zinc Level ◽

Control Group ◽

P Value ◽

Periodontal Health ◽

Low Level ◽

The Mean ◽

The Impact

Abstract Background: Sickle cell anemia (SCA) is the most common inherited disorder around the world. It affects red blood cells resulting in severe hemolysis and painful crisis with multi-organ damage. Sickle cell anemia patients showed an impaired antioxidant status due to a reduced antioxidant defense mechanism that depends indirectly on zinc micronutrient. Several studies revealed a low level of serum zinc among sickle patients. However, none of them investigates the impact of SCA on salivary zinc level. This study aims to measure zinc level in saliva and its influence on the periodontal health of Sudanese children with SCA in Khartoum state. Up to our knowledge, this the first study conducted to measure salivary Zink level and its impact on periodontal health among sickle patients Subjects and Methods: Cross-sectional study was conducted among children and adolescents aged 5-18 years old. Sixty individuals were involved, 30 with SCA compared to 30 healthy controls groups. An Atomic Absorption spectrophotometer quantified the salivary level of zinc. Periodontal examinations were assessed in SCA children using the periodontal community index.Results: The mean ages in the sickle cell anemic group were 10.37 ± 4.13 years old, while the mean ages of subjects in the control group were 9.37 ± 3.6 years (P.Value: 0.169). Salivary zinc level was significantly lower among SCA patients (P.Value <0.001) than in the healthy control group. None of the participants were diagnosed with periodontitis. The less salivary zinc level was significantly higher in SCA with standard pocket depth and no clinical attachment loss than patients diagnosed with gingivitis ( P. Value:0.025).Conclusions: Sickle patients have a low level of zinc in saliva, which may contribute to some manifestations of sickle cell disease and may make them more susceptible to periodontal disease in the future. We recommend conducting more research on oral and periodontal health among children affected with SCA to improve their quality of life.

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The orientation of sickle hemoglobin fibers within fascicles

Proceedings, annual meeting, Electron Microscopy Society of America ◽

10.1017/s0424820100104066 ◽

1988 ◽

Vol 46 ◽

pp. 400-401

Author(s):

Christopher A. Miller ◽

Bridget Carragher ◽

William A. McDade ◽

Robert Josephs

Keyword(s):

Sickle Cell ◽

Sickle Cell Anemia ◽

Relative Orientation ◽

Unit Cell ◽

Red Cell ◽

High Ph ◽

Sickle Hemoglobin ◽

Polar Crystal ◽

Helical Configuration

Highly ordered bundles of deoxyhemoglobin S (HbS) fibers, termed fascicles, are intermediates in the high pH crystallization pathway of HbS. These fibers consist of 7 Wishner-Love double strands in a helical configuration. Since each double strand has a polarity, the odd number of double strands in the fiber imparts a net polarity to the structure. HbS crystals have a unit cell containing two double strands, one of each polarity, resulting in a net polarity of zero. Therefore a rearrangement of the double strands must occur to form a non-polar crystal from the polar fibers. To determine the role of fascicles as an intermediate in the crystallization pathway it is important to understand the relative orientation of fibers within fascicles. Furthermore, an understanding of fascicle structure may have implications for the design of potential sickling inhibitors, since it is bundles of fibers which cause the red cell distortion responsible for the vaso-occlusive complications characteristic of sickle cell anemia.

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