Cases reported "Hemoglobin C Disease"

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1/32. Outcome of twin pregnancies in patients with haemoglobinopathies--case reports.

    pregnancy in patients with haemoglobinopathy is associated with increased risk of maternal and perinatal morbidities and mortalities. Multiple pregnancy is potentially more hazardous than singleton pregnancy. There is a dearth of information concerning multiple pregnancies in patients with haemoglobinopathy. Four of such patients seen in the obstetric service of the University College Hospital, Ibadan are presented here and discussed. Increased surveillance and elective caesarean delivery are suggested in the management of these patients. ( info)

2/32. Hemoglobinopathy with prolonged bactermia. A report of two cases.

    Prolonged escherichia coli bacteremia occurred as a complication of pyelonephritis in two patients with abnormal hemoglobins (SC and SS), despite "appropriate" antibiotic therapy. Careful investigation in each case failed to account for the persistent sepsis. Pyogenic arthritis ultimately developed in both patients. ( info)

3/32. urea resolves gross hematuria in a 15 year old with hemoglobin C trait.

    hematuria is a rare complication seen in patients with hemoglobin C trait. We report a 15-year-old African-American female with hemoglobin C trait, who presented with persistent hematuria. None of the urological, serological or histological workups revealed any other pathology. hematuria failed to respond to all conventional modalities used in the treatment of the same condition seen in sickling hemoglobinopathies. This case is the first known case of persistent hematuria in a pediatric patient with hemoglobin C trait, which resolved with intravenous urea administration. ( info)

4/32. Spontaneous regression (autoinfarction) of proliferative sickle retinopathy.

    Of 45 patients with proliferative sickle retinopathy in stages III, IV, and V, nine patients (eight with hemoglobin sc disease, one with sickle cell thalassemia) showed spontaneous regression (autoinfarction) of retinal sea fans. One mechanism involved in autoinfarction of neovascular tissue is progressive, centripetal retraction of the anterior vascular arcade of the peripheral retina. In addition, vitreous traction on feeder vessels may result in sluggish blood flow and occlusion of these vessels, or may tear the sea fan completely away from its feeder vessels. In view of the many incidences of vitreous hemorrhages that occur in patients with proliferative retinopathy, however, we recommend treatment of neovascularization rather than prolonged observation. ( info)

5/32. Haemoglobin C/alpha thalassaemia: haematological and biosynthetic studies.

    A family with genes for haemoglobin C (Hb C) and alpha thalassaemia was studied. The mother had Hb-C trait. The father also had Hb-C trait but in addition displayed microcytosis, elevated Hb-F levels and a concentration of Hb-C less than usual for heterozygotes. The proband was homozygous for Hb-C but had Hb-F levels far exceeding those present in Hb-C disease. Biosynthetic studies of globin synthesis in both father and daughter showed a deficit of alpha chains relative to non-alpha chains, confirming the presence of alpha thalassaemia. The coexistence of alpha thalassaemia influences the level of mutant haemoglobin in haemoglobinopathies in which Hb C is present, in a fashion similar to that observed in sickle-cell trait. ( info)

6/32. Multiple major retinal vascular occlusions in sickle cell haemoglobin C disease.

    A case of multiple occlusions of different sized arterioles involving the central area of the fundus has been reported in a patient suffering from sickle cell haemoglobin C disease (SC). ( info)

7/32. Migratory white-without-pressure retinal lesions.

    Nine patients had white-without-pressure retinal lesions. These lesions were seen in areas of vitreoretinal adhesions. The configuration and location of these lesions changed over variable periods of time. The cause of this pecular migration is unknown, but may be related to separation and re-creation of vitreoretinal adhesions. The migratory nature of white-without-pressure areas has not, to our knowledge, previously been reported in the literature. ( info)

8/32. Sickle cell syndromes. I. Hemoglobin SC-alpha-thalassemia.

    Hematologic and globin synthesis studies were performed in a black American family in which the genes for alpha-thalassemia and hemoglobins (Hb) S and C were segregating. The following distribution of these abnormalities was found: father, sickle cell trait alpha-thalassemia; mother, HbC trait alpha-thalassemia, propositus, HbSC alpha-thalassemia; older sibling, alpha-thalassemia trait; and younger sibling, hemoglobin h disease. The child with HbSC-alpha-thalassemia demonstrated more severe anemia and a more hemolytic picture than is typical of HbSC disease. Her erythrocytes exhibited decreased osmotic fragility in comparison with HbSC erythrocytes, but had an indistinguishable oxygen equilibrium curve and 2, 3-diphosphoglycerate (2, 3-DPG) level. Erythrocyte sickling in the patient, however, was significantly reduced, with less than 35% sickle forms observed at nearly complete oxygen desaturation. The sibling with hemoglobin h disease exhibited 26% Bart's (gamma4) hemoglobin at birth, a level comparable with that seen in infants with HbH disease in Far Eastern populations. At age 5 months typical findings of mild hemoglobin h disease appeared, with HbH making up 6.5% of the total hemoglobin. ( info)

9/32. Severe proliferative retinopathy as the only sign of sickle cell hemoglobin c disease.

    A 48-year-old black woman developed severe bilateral hypoxic proliferative retinopathy without other clinical manifestations. The hemoglobin level was 10.6 to 11.5 g/100 ml, reticulocyte level was 2.2%, targeted and sickled red blood cells were seen on blood smears, and hemoglobins S and C were demonstrated by electrophoresis. glucose tolerance test was normal. The development of neovascular proliferation, vitreous hemorrhage, and retinal detachment unassociated with other clinical symptoms is unusual in sickle cell hemoglobin C disease. ( info)

10/32. Red cell osmotic fragility studies in hemoglobin C-beta thalassemia: osmotically resistant microspherocytes.

    Typically certain features of red cell morphology predict the results of osmotic fragility testing. Microspherocytes generally have increased and target cells decreased fragility. Blood smears in homozygous hemoglobin c disease show an interesting admixture of microspherocytes and target cells. Yet osmotic fragility studies generally show only reduced fragility and no population of fragile cells to correspond with the spherocytes. The present study demonstrates that the red cells of patients with hemoglobin C-beta thalassemia share many characteristics with hemoglobin C red cells, including the decreased osmotic fragility of all cells despite the presence of both spherocytes and target cells. These paradoxically osmotically resistant spherocytes probably arise because of cellular dehydration due to a K-Cl transport system which may be activated by binding of hemoglobin C to the red cell membrane. ( info)
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