Hereditary spherocytosis: Difference between revisions

Jump to navigation Jump to search
← Older editNewer edit →
VisualWikitext
Line 46: Line 46:


== [[hereditary spherocytosis surgery|Surgery]] ==
== [[hereditary spherocytosis surgery|Surgery]] ==
* Splenectomy is very effective in reducing hemolysis, leading to significant prolongation of the red cell life span.<ref>{{Cite journal
| author = [[P. H. B. Bolton-Maggs]], [[R. F. Stevens]], [[N. J. Dodd]], [[G. Lamont]], [[P. Tittensor]] & [[M.-J. King]]
| title = Guidelines for the diagnosis and management of hereditary spherocytosis
| journal = [[British journal of haematology]]
| volume = 126
| issue = 4
| pages = 455–474
| year = 2004
| month = August
| doi = 10.1111/j.1365-2141.2004.05052.x
| pmid = 15287938
}}</ref><ref name="CasalePerrotta2014">{{cite journal|last1=Casale|first1=Maddalena|last2=Perrotta|first2=Silverio|title=Splenectomy for hereditary spherocytosis: complete, partial or not at all?|journal=Expert Review of Hematology|volume=4|issue=6|year=2014|pages=627–635|issn=1747-4086|doi=10.1586/ehm.11.51}}</ref>
* Patients should be selected for splenectomy on the basis of their clinical symptoms and presence of complications such as gallstones, not simply on the basis of diagnosis alone.
* Following splenectomy, the clinical manifestations and complications (anemia & gallstones) are much reduced in severe hereditary spherocytosis and abolished in milder cases, but at the risk of increased life threatening sepsis from encapsulated organisms, particularly streptococcus pneumoniae.<ref name="Bolton-MaggsStevens2004">{{cite journal|last1=Bolton-Maggs|first1=P. H. B.|last2=Stevens|first2=R. F.|last3=Dodd|first3=N. J.|last4=Lamont|first4=G.|last5=Tittensor|first5=P.|last6=King|first6=M.-J.|title=Guidelines for the diagnosis and management of hereditary spherocytosis|journal=British Journal of Haematology|volume=126|issue=4|year=2004|pages=455–474|issn=0007-1048|doi=10.1111/j.1365-2141.2004.05052.x}}</ref><ref>{{Cite journal
| author = [[Sayeeda Huq]], [[Mark A. C. Pietroni]], [[Hafizur Rahman]] & [[Mohammad Tariqul Alam]]
| title = Hereditary spherocytosis
| journal = [[Journal of health, population, and nutrition]]
| volume = 28
| issue = 1
| pages = 107–109
| year = 2010
| month = February
| pmid = 20214092
}}</ref>
* Children or young adults with mild hereditary spherocytosis who also has gallstones are likely to benefit from combined splenectomy and cholecystectomy in terms of life expectancy.<ref name="Bolton-MaggsStevens2004">{{cite journal|last1=Bolton-Maggs|first1=P. H. B.|last2=Stevens|first2=R. F.|last3=Dodd|first3=N. J.|last4=Lamont|first4=G.|last5=Tittensor|first5=P.|last6=King|first6=M.-J.|title=Guidelines for the diagnosis and management of hereditary spherocytosis|journal=British Journal of Haematology|volume=126|issue=4|year=2004|pages=455–474|issn=0007-1048|doi=10.1111/j.1365-2141.2004.05052.x}}</ref>


==[[hereditary spherocytosis primary prevention|Primary Prevention]] ==
==[[hereditary spherocytosis primary prevention|Primary Prevention]] ==

Revision as of 17:08, 28 November 2018

Template:DiseaseDisorder infobox

Hereditary spherocytosis Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Hereditary spherocytosis from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

Chest X Ray

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Hereditary spherocytosis On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Hereditary spherocytosis

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Hereditary spherocytosis

CDC on Hereditary spherocytosis

Hereditary spherocytosis in the news

Blogs on Hereditary spherocytosis

Directions to Hospitals Treating Hereditary spherocytosis

Risk calculators and risk factors for Hereditary spherocytosis

For patient information click here

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Zahir Ali Shaikh, MD[2]

Overview

Hereditary spherocytosis is a genetically-transmitted form of spherocytosis, an auto-hemolytic anemia characterized by the production of red blood cells that are sphere-shaped rather than donut-shaped, and therefore more prone to hemolysis.

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Hereditary Spherocytosis from Other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications, and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

Treatment

Medical Therapy

Surgery

Primary Prevention

  • The administration of vaccines (pneumococcal, haemophilus influenzae type b, meningococcal group C and influenza) is indicated 2 to 3 weeks before splenectomy.[1]
  • Postsplenectomy patients should also be given 1mg of folic acid daily for preventing secondary folic acid deficiency and oral penicillin (penicillin V) for preventing secondary infection until reaching adulthood.

Secondary Prevention

Case Studies

Case #1

Related Chapters

Template:Otheruses4

External links

References

  1. ↑ Sayeeda Huq, Mark A. C. Pietroni, Hafizur Rahman & Mohammad Tariqul Alam (2010). "Hereditary spherocytosis". Journal of health, population, and nutrition. 28 (1): 107–109. PMID 20214092. Unknown parameter |month= ignored (help)

he:ספרוציטוזיס תורשתי sr:Сфероцитоза

Template:WikiDoc Sources

Retrieved from "https://www.wikidoc.org/index.php?title=Hereditary_spherocytosis&oldid=1505145"