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LA
CONSULTA SEMANAL
NOVIEMBRE
2000
CONSULTA
Anemia
de células falciformes
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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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