1/63. Adenotonsillar hypertrophy: a precipitating factor of cerebrovascular accident in a child with sickle cell anemia.Cerebrovascular accident is one of the most serious complications of sickle cell anemia. The specific factors that predispose patients with sickle cell anemia to stroke are increased disease severity, higher baseline white blood cell count and lower baseline hematocrits. Likewise the presence of a co-existent alpha thalassemia trait and/or high fetal hemoglobin (HbF%) may reduce the risk. We report a child with sickle cell anemia and marked adenotonsillar hypertrophy resulting in obstructive sleep apnea syndrome. There was no other known risk factor for developing cerebrovascular accident in this child during her hospitalization for adenotonsillectomy.- - - - - - - - - - ranking = 1keywords = thalassemia (Clic here for more details about this article) |
2/63. Hodgkin's disease in a child with sickle cell disease treated with hydroxyurea.hydroxyurea (HU) is an oral drug that ameliorates the clinical course of sickle cell anemia by increasing the levels of fetal hemoglobin and decreasing the adhesion of red cells to endothelium. Although HU has minimal short-term toxicity, few data are available about the long-term safety and the potential risk for carcinogenesis or leukemogenesis. An 8-year-old child with sickle cell/beta 0-thalassemia who received HU treatment for painful crises is described. Six months after the initiation of the HU treatment he developed Hodgkin's disease, lymphocyte predominance subtype. Chemotherapy induced a complete remission. After discontinuation of chemotherapy the painful crises recurred and bone marrow transplantation was decided at the age of 12 years. Two years after the bone marrow transplantation, the child is in complete remission without painful crises. Although the authors suggest that the development of Hodgkin's disease is a coexisting event, questions arise about the safety of HU treatment in childhood.- - - - - - - - - - ranking = 1keywords = thalassemia (Clic here for more details about this article) |
3/63. 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.- - - - - - - - - - ranking = 1keywords = thalassemia (Clic here for more details about this article) |
4/63. An unusual etiology of hypertension in a 5-year-old boy.Ganglioneuromas are rare benign tumors of neural crest origin, arising from ganglia of the sympathetic nervous system and adrenal medulla. These masses are usually detected during the first 2 decades of life and are generally discovered incidentally. We present a 5-year-old boy with sickle beta-thalassemia whose hypertension is caused by a perihilar ganglioneuroma encasing the right renal artery and distorting the right renal vein. The tumor was resected and the child's blood pressure subsequently normalized.- - - - - - - - - - ranking = 1keywords = thalassemia (Clic here for more details about this article) |
5/63. liver transplantation in a patient with S(beta)o-thalassemia.BACKGROUND: patients presenting sickle cell disease may develop different types of hepatic complications. Intrahepatic cholestasis is a potentially fatal complication of the disease, and sometimes the only possible solution is transplantation. Postoperative transfusion management has not yet been well established. In this report, we describe the transfusional program of a patient presenting sickle cell disease and intrahepatic cholestasis who underwent liver transplantation 2 years ago. methods: Data were obtained from the chart and the blood bank records. RESULTS: The liver transplantation was performed successfully. Despite mild allograft dysfunction 3 months after surgery, secondary to intrahepatic sickling, the patient has been doing well with the transfusional management adopted (sickle-cell hemoglobin <20%). CONCLUSION: Sickle cell disease should not be a criterion for exclusion from liver transplantation. Regular transfusion with monitoring of sickle-cell hemoglobin is a very important measure to minimize the risk of intrahepatic sickling and possible rejection.- - - - - - - - - - ranking = 4keywords = thalassemia (Clic here for more details about this article) |
6/63. Sicklemia with multi-organ failure syndrome and thrombotic thrombocytopenic purpura.Acute multi-organ failure syndrome is a rare and life-threatening complication of patients with sickle cell disease. The syndrome appears to be reversed with prompt, aggressive exchange transfusion therapy. It has been attributed to widespread vascular occlusion due to micro-vascular red cell sickling. We present a case of severe multi-organ failure in a patient with sickle thalassemia and mild clinical course, who had clinical and laboratory features consistent with thrombotic thrombocytopenic purpura (TTP). The dramatic response to therapy with plasma exchange, a treatment often effective in TTP, suggests a similarity in pathophysiology of micro-vascular occlusion and multi-organ failure in sickle cell disease.- - - - - - - - - - ranking = 1keywords = thalassemia (Clic here for more details about this article) |
7/63. colonic pseudo-obstruction in sickle cell disease.A young Arab woman with sickle cell-beta0-thalassemia disease developed acute colonic pseudo-obstruction that became chronic but showed some response to hydroxyurea. There was no evidence of microvascular or macrovascular occlusion. We also report the case of an Arab man with sickle cell anemia who presented with acute colonic pseudo-obstruction from which he recovered completely within a few days. Although the development of pseudo-obstruction in these two cases seems to have been a complication of sickle cell disease, its pathogenesis remains unclear. There are several reports of ischemic and inflammatory disorders of the colon complicating sickle cell disease; however, these two cases represent the first descriptions of large-bowel pseudo-obstruction in this hemoglobinopathy [corrected].- - - - - - - - - - ranking = 1keywords = thalassemia (Clic here for more details about this article) |
8/63. Acute renal failure following deferoxamine overdose.A 17-year-old patient with sickle cell-beta thalassemia undergoing treatment with home iron chelation therapy inadvertently received ten times the recommended dose of intravenous deferoxamine. Acute renal failure (ARF) developed within hours. Immediate treatment with high-efficiency hemodialysis resulted in the prompt return of renal function after only one hemodialysis session. No long-term nephrotoxic effects of the deferoxamine overdose developed after more than 1 year of follow-up. Children with sickle cell disease who are on intravenous deferoxamine and their parents should be cautioned about the possibility of ARF with overdose due to malfunction of the pump and/or inadequate monitoring during treatment. ARF, should it occur in such children, appears to respond well to treatment with high-efficiency hemodialysis.- - - - - - - - - - ranking = 1keywords = thalassemia (Clic here for more details about this article) |
9/63. 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.- - - - - - - - - - ranking = 10keywords = thalassemia (Clic here for more details about this article) |
10/63. Surgical management of children with hemoglobinopathies.Sickle hemoglobinopathies include sickle cell disease, sickle-C disease, and sickle-beta thalassemia. patients with these disorders commonly suffer a multitude of destructive events to vital organs, especially to the central nervous system, the spleen, the kidney, the lung, and the heart as a result of microvascular plugging by the sickled erythrocytes. Thoughtful preparation for anesthesia and operation, especially when directed by experienced individuals, can greatly reduce the hazard of inducing the sickle crises that formerly plagued individuals with sickle hemoglobinopathies who faced major operations under general anesthesia. The patient must be free of any acute illness, especially one involving the respiratory system. Adequate hydration preoperatively combined with avoiding perioperative hypoxia, hypothermia, and acidosis, the triggers for sickling, will go far toward avoiding sickle-induced complications. Modern transfusion therapy, consisting of multiple small transfusions of Hb A erythrocytes administered over several weeks prior to the operation, not only corrects the chronic anemia but suppresses erythropoiesis of cells containing Hb S in the patient's bone marrow and leaves him or her with a majority of cells containing Hb A. This provides a safety net in case a sickle-inducing insult occurs despite the best efforts to avoid one. Individuals with sickle hemoglobinopathies may require any of the operations common to all children, for example, herniorrhaphy, appendectomy, tonsillectomy, and circumcision, but a significant number will develop calcium bilirubinate cholelithiasis and possibly cholecystitis as a result of the continual increased load of bile salts resulting from the shortened lifespan of the cells containing Hb S. Also, although most individuals with Hb S will gradually suffer splenic infarction by late childhood, a significant number of infants will experience acute splenic sequestration crisis, a life-threatening entity, the recurrence of which is prevented by splenectomy. Several publications have demonstrated that such surgical procedures can be performed in large numbers of patients with sickle hemoglobinopathies without deaths and with minimal morbidity.- - - - - - - - - - ranking = 1keywords = thalassemia (Clic here for more details about this article) |
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