LA CONSULTA SEMANAL

 

NOVIEMBRE 2000

 

 

CONSULTA

Anemia de cÚlulas falciformes

 

Am Fam Physician 2000 Sep 15;62(6):1309-14 [Texto completo]

Sickle cell disease in childhood: Part II. Diagnosis and treatment of major complications and recent advances in treatment.

Wethers DL

St. Luke's-Roosevelt Hospital Center, New York, New York, USA.

Treatment advances over the past 25 years have significantly decreased morbidity and mortality in children with sickle cell disease. Aggressive management of fever, early diagnosis of acute chest syndrome, judicious use of transfusions and proper treatment of pain can improve quality of life and prognosis for these children. Prophylactic hydroxyurea therapy has been shown to reduce the incidence and severity of pain crises in adults with sickle cell disease and has been effective in limited studies conducted in children. Research into stem cell transplantation provides hope that a cure for sickle cell disease may be possible.

Publication Types:

  Review

  Review, tutorial

 

Am Fam Physician 2000 Sep 1;62(5):1013-20, 1027-8 [Texto completo]

Sickle cell disease in childhood: Part I. Laboratory diagnosis, pathophysiology and health maintenance.

Wethers DL

St Luke's--Roosevelt Hospital Center, New York, New York, USA.

Over the past 25 years, morbidity and mortality have decreased significantly in children with sickle cell disease, and screening tests are now available to diagnose the disease in newborns. The incidence of sepsis caused by pneumococcal and Haemophilus influenzae infections has declined because of the prophylactic administration of penicillin soon after birth and the timely administration of pneumococcal and H. influenzae type b vaccines. Optimal nutrition can maximize growth in children with sickle cell disease, and timely screening can identify complications such as retinal damage and chronic renal involvement, thereby ensuring prompt treatment. Family physicians and parents who have been educated about sickle cell disease can detect acute, life-threatening complications such as splenic sequestration crisis and acute chest syndrome at their onset, thereby allowing treatment to be instituted without delay.
Publication Types:

  Review

  Review, tutorial

 

N Engl J Med 2000 Jun 22;342(25):1910-2

Blockade of adhesion of sickle cells to endothelium by monoclonal antibodies.

Hebbel RP

University of Minnesota Medical School, Minneapolis 55455, USA.

Publication Types:

  Review

  Review, tutorial

 

Pediatr Clin North Am 2000 Jun;47(3):699-710

The management of pain in sickle cell disease.

Yaster M, Kost-Byerly S, Maxwell LG

Department of Anesthesiology, Johns Hopkins Medical Institutions, Baltimore, Maryland, USA. myaster@jhmi.edu

The pain of vaso-occlusive crisis in patients with sickle cell disease is excruciating, incapacitating, and sometimes refractory to even the most advanced analgesic treatments. A comprehensive, multimodal approach to therapy that includes education, cognitive therapies, anti-inflammatory drugs, opioids, and psychostimulant adjuvant drugs has been presented. Until a cure for the underlying disease is found, these are the best approaches available. The authors hope that future research will find even better modalities of analgesic care.

Publication Types:

  Review

  Review, tutorial

 

Am Fam Physician 2000 Mar 1;61(5):1349-56, 1363-4 [Texto completo]

Approach to the vaso-occlusive crisis in adults with sickle cell disease.

Yale SH, Nagib N, Guthrie T

Marshfield Clinic, Wisconsin, USA.

The vaso-occlusive crisis, or sickle cell crisis, is a common painful complication of sickle cell disease in adolescents and adults. Acute episodes of severe pain (crises) are the primary reason that these patients seek medical care in hospital emergency departments. Frequently, however, the pain is incompletely treated. Despite advances in pain management, physicians are often reluctant to give patients adequate dosages of narcotic analgesics because of concerns about addiction, tolerance and side effects. It is important to recognize a pain crisis early, correct the inciting causes, control pain, maintain euvolemia and, when necessary, administer adequate hemoglobin to decrease the hemoglobin S level. The family physician and the hematologist must work together to treat acute pain episodes promptly and effectively, manage the long-term sequelae of chronic pain and prevent future vaso-occlusive crises.

Publication Types:

  Review

  Review, tutorial

 

Postgrad Med 2000 Jan;107(1):215-8, 221-2 [Texto completo]

Acute chest syndrome in sickle cell disease. Crucial considerations in adolescents and adults.

Yale SH, Nagib N, Guthrie T

Marshfield Clinic, Department of Internal Medicine, WI 54449, USA.yales@mfldclin.edu

Rapid recognition of acute chest syndrome is essential in patients with sickle cell disease. The condition can be particularly severe in adolescents and adults and often leads to death. In this article, the authors review the challenges of evaluating the syndrome and outline current treatment and supportive care.

 

Blood 2000 Jan 15;95(2):365-7

Introduction: anti-adhesion therapy in sickle cell disease.

Harlan JM

Division of Hematology, University of Washington, Seattle, WA.

Publication Types:

  Comment

  Review

  Review, tutorial

Comments:

  Comment on: Blood 2000 Jan 15;95(2):368-74

 

N Engl J Med 1999 Apr 1;340(13):1021-30

Management of sickle cell disease.

Steinberg MH

G.V. (Sonny) Montgomery Veterans Affairs Medical Center, Jackson, MS 39216, USA. mhs@fiona.umsmed.edu

Publication Types:

  Review

  Review, tutorial

 

Blood 1999 Mar 15;93(6):1787-9

Induction of fetal hemoglobin in sickle cell disease.

Bunn HF

Division of Hematology, Brigham and Women's Hospital, Boston, MA, USA.

Publication Types:

  Comment

  Review

  Review, tutorial

Comments:

  Comment on: Blood 1999 Mar 15;93(6):1790-7

 

J Am Soc Nephrol 1999 Jan;10(1):187-92 [Texto completo]

Sickle cell nephropathy.

Saborio P, Scheinman JI

Department of Pediatrics, Virginia Commonwealth University's Medical College of Virginia Campus, Richmond, USA.

Publication Types:

  Review

  Review, tutorial

 

J R Coll Surg Edinb 1998 Apr;43(2):73-9 [Texto completo]

Sickle cell disease and the general surgeon.

Meshikhes AW, al-Faraj AA

Department of Surgery, Dammam Central Hospital, Eastern Province, Saudi Arabia.

Sickle cell disease (SCD) is a common genetic disorder which represents a major medical problem in certain parts of the world. It is characterized by chronic haemolytic anaemia and vaso-occlusive crises, which can lead to widespread vascular occlusion by sickled red blood cells leading to multiple organ infarctions. In this respect, SCD can be considered as a multisystem disease presenting, to various surgical disciplines, a wide variety of surgical ailments. As surgical management of these patients is associated with high morbidity and mortality, close collaboration between medical and surgical services is essential. Greater population mobility makes recognition of the surgical implications of this disease of paramount importance in surgical practice. This article reviews surgical problems with which SCD patients may present to general surgeons and discusses their management.

Publication Types:

  Review

  Review, tutorial

 

Am J Hematol 1998 May;58(1):61-6

New concepts in assessing sickle cell disease severity.

Schnog JJ, Lard LR, Rojer RA, Van der Dijs FP, Muskiet FA, Duits AJ

Department of Internal Medicine, St. Elisabeth Hospital, Curacao, Netherlands Antilles.

Vasoocclusion leads to pain, chronic organ damage, and a decreased life expectancy in patients with sickle cell disease. Therapeutic options for sickle cell disease have usually been evaluated according to their capacity for reducing the frequency of vasoocclusive crises requiring clinical attention. However, the frequency of vasoocclusive crises is not representative for the rate of accumulating organ damage in most sickle cell patients. This implies that the frequency of vasoocclusive crises needn't correlate with disease severity and, although being of importance, cannot solely serve as a parameter of treatment efficacy. Therefore, additional new objective parameters are needed to effectively study the vasoocclusive process in sickle cell disease. Several studies show that intricate adhesive interactions between (red) blood cells, plasma components, and endothelium play a crucial role in the pathophysiology of sickle cell vasoocclusion, offering new potential parameters to effectively assess disease severity as well as new therapeutical targets in the near future. Whether these adhesive mechanisms involve the causes or the effects of vasoocclusion will be determined if their inhibition, by interventive measures, results in therapeutic benefits.

Publication Types:

  Review

  Review, tutorial

 

BMJ 1997 Sep 13;315(7109):656-60 [Texto completo]

Management of patients with sickle cell disease.

Davies SC, Oni L

Imperial College, School of Medicine, Central Middlesex Hospital, London.

Publication Types:

  Review

  Review, tutorial

Comments:

  Comment in: BMJ 1998 Mar 21;316(7135):934; discussion 934-5

  Comment in: BMJ 1998 Mar 21;316(7135):935

  Comment in: BMJ 1998 Mar 21;316(7135):935; discussion 936

  Comment in: BMJ 1998 Mar 21;316(7135):936

 

Paediatr Nurs 1997 Jun;9(5):29-34; quiz 35-6

Sickle cell anaemia.

Thomas VN, Westerdale N

Department of Nursing Studies, King's College, London University.

Publication Types:

  Review

  Review, tutorial

 

Lancet 1997 Sep 6;350(9079):725-30 [Texto completo]

Sickle-cell disease.

Serjeant GR

MRC Laboratories (Jamaica) University of West Indies, Mona, Kingston, Jamaica.

Publication Types:

  Review

  Review, tutorial

Comments:

  Comment in: Lancet 1997 Dec 6;350(9092):1710

 

N Engl J Med 1997 Sep 11;337(11):762-9

Pathogenesis and treatment of sickle cell disease.

Bunn HF

Division of Hematology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA 02115, USA.

Publication Types:

  Review

  Review, tutorial

 

BMJ 1997 May 3;314(7090):1333-6 [Texto completo]

ABC of clinical haematology. Haematological emergencies.

Frewin R, Henson A, Provan D

Southampton University Hospitals NHS Trust.

Publication Types:

  Review

  Review, tutorial

 

BMJ 1997 Feb 15;314(7079):492-6 [Texto completo]

ABC of clinical haematology. The hereditary anaemias.

Weatherall DJ

Institute of Molecular Medicine, University of Oxford, John Radcliffe Hospital.

Publication Types:

  Review

  Review, tutorial

 

Hematol Oncol Clin North Am 1996 Dec;10(6):1265-73

Continuing care for adult patients with sickle cell disease.

Koshy M, Dorn L

Division of Hematology/Oncology, University of Illinois at Chicago, USA.

Continuing services for patients with sickle cell disease focuses on holistic care. Participation in preventive medicine principles of health management, avoidance of risk behaviors and seeking appropriate counseling for education, employment, healthy lifestyles, and productive living are beneficial to the patient.

Publication Types:

  Review

  Review, tutorial

 

Hematol Oncol Clin North Am 1996 Dec;10(6):1255-64

The adolescent with sickle cell anemia.

Kinney TR, Ware RE

Duke-UNC Comprehensive Sickle Cell Center, Durham, North Carolina, USA.

Adolescence is a time of intense change and turmoil. Helping patients with sickle cell disease have a smooth transition from the pediatric to adult health care environment is an important and meaningful experience. Facilitating the patient's transition, however, takes time and effort. Pediatricians must compile
accurate medical summaries transmitting the details and nuances of the patient's history and care. Effort must be expended to ensure that details related to alloimmunization are not omitted. The pediatrician must be careful that the transfer does not create feelings of rejection and abandonment in the patient and the family. Physicians accepting the patient in transfer must devote time to educating and counseling the new patient. A relationship of trust and respect must be built. The physician should work with the patient to explore feelings of distrust and to uncover any concerns and fears, which should be dealt with proactively to avoid major conflicts later. Working closely with the adolescent patient can have many rewards, including helping the patient through a difficult period of adjustment. Helping the patient negotiate this difficult period can have many positive consequences. It is essential that both the pediatrician and internist work closely with the patient and family during the transfer process. Failure to do so can have disastrous consequences. When the collaboration is successful, however, the rewards for patients, families, and providers are great.

Publication Types:

  Review

  Review, tutorial

 

Hematol Oncol Clin North Am 1996 Dec;10(6):1241-53

Pathophysiology of sickle cell anemia.

Bookchin RM, Lew VL

Department of Medicine, Albert Einstein College of Medicine, Bronx, New York, USA.

The anemia results from the markedly shortened circulatory survival of SS cells, together with a limited erythropoietic response. Both independent properties of Hb S-polymerization of the deoxy-Hb and instability of the oxy-Hb-contribute to  early red cell destruction by effects on the Hb and on the red cell membranes. The erythroid response is limited mainly by the low oxygen affinity of SS cells, caused by the polymer and the increased 2,3-DPG. But the worst culprits in these processes are the dense, dehydrated SS cells (including the ISCs), most of which are formed rapidly from non-Hb F-reticulocytes by cation transport mechanisms triggered by polymerization. Since the clinical consequences of microvascular occlusion far exceed those of anemia per se, measures to lessen the anemia must also inhibit polymerization and sickling.

Publication Types:

  Review

  Review, tutorial

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