eMedicine Specialties > Pediatrics: General Medicine > Allergy & Immunology
Asplenia: Treatment & Medication
Updated: Aug 13, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
Once the diagnosis of anatomic or functional asplenia is confirmed, aggressive management is the key to decreasing the morbidity and mortality associated with this condition. Newborn diagnosis of sickle cell disease is essential because the first manifestation of the hemoglobinopathy in these infants may be an asplenia-related fatal bacteremia. Any episode of fever or signs of infection should be promptly and aggressively treated.
Medical care involves 4 key components: antibiotic prophylaxis, appropriate immunization, aggressive management of suspected infection, and parent education.
- Antibiotic prophylaxis
- Antibiotic prophylaxis should be initiated immediately upon the diagnosis of asplenia because these patients are at significant risk of pneumococcal infections. For children younger than 2 years, oral penicillin V may be given twice a day. Amoxicillin has also been recommended as an appropriate prophylactic antibiotic. Erythromycin is an alternate choice in patients who are allergic to penicillin.
- In general, antimicrobial prophylaxis should be considered for all children with asplenia or splenic dysfunction until age 5 years and for at least 1 year after surgical splenectomy. Some experts recommend continuing prophylaxis into adulthood, particularly for high-risk patients.
- Numerous controversies surround when to discontinue antimicrobial prophylaxis in asplenia and hyposplenia (if it should be discontinued at all). Arguments for cessation of prophylaxis include poor patient compliance and the development of resistant bacterial strains in patients on daily antibiotics. Those in favor of lifelong prophylaxis cite case reports of overwhelming postsplenectomy sepsis that occurs years after removal of the spleen. Currently, most guidelines leave the option open to continue lifelong prophylaxis based on the clinical circumstances of the individual patient.
- Immunization
- All patients should receive all standard childhood and adolescent immunizations at the recommended age. Most importantly, vaccinations against encapsulated organisms, including pneumococcal conjugate and/or polysaccharide, H influenzae type b conjugate, and meningococcal conjugate and/or polysaccharide vaccines, should be administered on the standard schedule.
- Approximately 80% of the pediatric pneumococcal bacteremias in the United States are caused by the 7 serotypes covered in the conjugate vaccine: 4, 6B, 9V, 14, 18C, 19F, and 23F. The conjugate vaccine has been effective in dramatically reducing the occurrence of invasive pneumococcal disease. In children younger than 2 years, the incidence of all invasive pneumococcal infections has decreased by 80% after conjugate vaccine was recommended in the routine childhood immunization schedule. Infections caused by vaccine and vaccine-related serotypes have decreased by 90% in older children and adults.
- The immunization schedule for pneumococcal conjugate vaccine (PCV7) consists of a primary series of 4 doses (0.5 mL each) at age 2, 4, 6, and 12-15 months. Catch-up immunization schedules are published regarding appropriate dosing schedules for children aged 5 years or younger. Administration of a single dose of PCV7 to children of any age is not contraindicated, especially for patients with asplenia or splenic dysfunction who are at high risk for invasive pneumococcal disease.
- The pneumococcal polysaccharide vaccine against 23 serotypes (PPV23) should be given after age 24 months for supplemental protection. PCV7 should be administered first, with administration of PPV23 at least 8 weeks after the last dose of PCV7. A booster dose 3-5 years after the first dose of PPV23 is appropriate.
- Patients should also receive quadrivalent meningococcal vaccine. Two licensed meningococcal vaccines are available in the United States against serotypes A, C, Y, and W-135, and another vaccine against serotype C is available in Europe. Meningococcal conjugate vaccine (MCV4) was licensed in 2005 for people aged 11-55 years; in 2007, the indication was expanded to include children aged 2 years or older with increased risk of invasive meningococcal disease. Meningococcal polysaccharide vaccine (MPSV4) is licensed for children aged 2 years and older and confers immunity for approximately 4 years. Because of its ability to induce a T-cell response, MCV4 is expected to confer a longer duration of protection than MPSV4; however, the exact duration of protective immunity from MCV4 is unknown. Immunization with MCV4 should be considered in adolescents 3 years after receiving MPSV4. Revaccination schedules with MCV4 are ongoing.
- The recommended vaccination schedule for H influenzae type b is a primary series of 3 doses given at age 2, 4, and 6 months or 2 doses given at age 2 and 4 months, depending on the particular conjugate vaccine product administered. A booster dose at age 12 months is recommended for all vaccine products. Children who are undergoing scheduled splenectomy after completion of their primary series and booster dose, may benefit from an additional dose of conjugate vaccine at least 7-10 days before surgery. Catch-up immunization schedules regarding H influenzae type b vaccine are published.
- Yearly influenza vaccine is also recommended to minimize the likelihood of secondary bacterial infections.
- Management of suspected infection
- The risk of serious bacterial infection is always present in these patients. Many patients have trivial symptoms yet rapidly develop fulminant sepsis and death within hours.
- All patients with impaired splenic function with suspected infection must be urgently and promptly evaluated. Obtain blood, urine, and, if indicated, cerebrospinal fluid (CSF) cultures. Initiate broad-spectrum intravenous antibiotics effective against S pneumoniae, H influenzae type b, and N meningitidis. Because of the fulminant nature of infections with these agents, intravenous antibiotics need to be initiated before bacteriological results are available. Second-generation or third-generation cephalosporins may be the initial choices. If multiple-drug resistance is a concern, vancomycin should be added to the regimen. In addition, many patients require supportive care with intravenous fluids, volume expanders, and pressor support.
- Because of the potential rapid progression of a serious bacterial infection, some experts recommend that asplenic patients have access to "stand-by" antibiotics, which can be initiated at the first sign of infection (fever, chills, or malaise). That the initiation of "stand-by" antibiotics is not a substitute for seeking immediate medical attention at the onset of an illness cannot be overemphasized.
- Patients with asplenia are at an increased risk of sepsis, shock, and meningitis secondary to Capnocytophaga canimorsus resulting from dog, cat, or rodent bites. The diagnosis may be made by means of Gram staining of the buffy coat, blood, and CSF cultures. Early treatment with penicillin is the therapy of choice, but cephalosporins, clindamycin, and erythromycin may also be used.
- Parent education
- The most important component in the treatment of these patients is parent education. Risks must be explained to all caretakers because they are an integral part of the management team. Seeking medical advice at the first sign of illness is crucial.
- Ongoing education must become a part of each physician-parent encounter so that the parents remain vigilant, which allows potentially serious infections to be identified early and managed aggressively. The child should wear a Medic Alert bracelet that reads "Asplenia" or "No Spleen." Written instructions should be given to the parents in a form that they can keep with them. For example, they can be given a wallet-sized card with the child's diagnosis and concise guidelines for early treatment and intervention.
Surgical Care
- Elective splenectomy for conditions such as hemolytic anemia are strongly discouraged before age 6 years and should be delayed as long as possible.
- Options to splenectomy should be considered when appropriate. These include partial splenectomy or embolization, conservative management of splenic trauma, and autotransplantation.
- To the author's knowledge, no data support the routine use of prophylactic antibiotics in the perioperative period.
- When surgical splenectomy is imminent, administration of pneumococcal, H influenzae type b, and meningococcal vaccines at least 2 weeks before splenectomy, if possible, is appropriate. If the immunizations are not received prior to surgery, some recommend immunization 14-21 days postsurgery because of enhanced immune response, compared with immediately postsurgery.
- Surgical splenectomy in patients with immunodeficiency should be avoided because of increased risk of invasive bacterial infections.
Activity
- No restrictions on activities are usually advised.
- Infections with H influenzae type b and pneumococcal and meningococcal bacteria are known to be increased among immunologically competent children and adults in daycare centers, college dormitories, military barracks, and other crowded facilities. Therefore, the risks of these situations should be explained to patients and their families.
Medication
The aim of medical therapy is to prevent invasive disease secondary to polysaccharide-encapsulated organisms, especially pneumococci. Penicillin and amoxicillin are currently the drugs of choice.
Antibiotics, prophylactic
These agents are used to prevent invasive bacterial disease. Antibiotic prophylaxis is given to patients before they undergo procedures that may cause bacteremia.
Penicillin V (V-Cillin K, Veetids)
Bactericidal b -lactam antibacterial antibiotic. Main activity is against gram-positive organisms such as streptococci, some gram-negative organisms, and anaerobes. Approximately 60% of PO dose is absorbed. Best taken on empty stomach. Some prefer amoxicillin because it is more bioavailable and less expensive. Preferred for children <2 y. PO susp (125 or 250 mg/mL) available.
Adult
250 mg PO bid
Pediatric
<5 years: 125 mg PO bid
>5 years: Administer as in adults
Probenecid may increase effectiveness by decreasing clearance; tetracyclines are bacteriostatic, decreasing effectiveness when administered concurrently
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Caution in renal impairment; rash commonly observed; anaphylactic shock, erythema nodosum, and interstitial nephritis less common; possible cross-reactivity with cephalosporin allergy
Erythromycin (EES, E-Mycin, Eryc)
Used for those with penicillin hypersensitivity. Limited activity against H influenzae. Bacteriostatic antibiotic that acts mainly by inhibiting protein synthesis. Administer >1-2 h pc. PO susp, chewable tab, and enteric-coated tab available.
Adult
250 mg PO bid
Pediatric
Administer as in adults
Inhibits CYP3A4 isoenzymes and decreases terfenadine, cisapride, and astemizole clearance, which may result in serious cardiac arrhythmias; may also increase toxicity of theophylline, digoxin, carbamazepine, triazolam, midazolam, and cyclosporine; may potentiate anticoagulant effects of warfarin; coadministration with lovastatin and simvastatin increases risk of rhabdomyolysis
Documented hypersensitivity; hepatic impairment
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Caution in liver disease; estolate formulation may cause cholestatic jaundice; GI adverse effects common (give doses pc); discontinue if nausea, vomiting, malaise, abdominal colic, or fever occur
Amoxicillin (Amoxil, Trimox)
Superior bioavailability and stability to gastric acid and has broader spectrum of activity than penicillin. Somewhat less active than that of penicillin against Streptococcus pneumococcus. Penicillin-resistant strains also resistant to amoxicillin, but higher doses may be effective. More effective against gram-negative organisms (eg, N meningitidis, H influenzae) than penicillin, thus, may provide better prophylaxis in children <2 y.
Susp (125, 200, 250, or 400 mg/5 mL) and pediatric drops (50 mg/mL) available.
Adult
250 mg PO bid
Pediatric
<5 years: 125 mg PO bid
>5 years: Administer as in adults
May reduce effectiveness of PO contraceptives; probenecid increases serum concentration
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Caution in cephalosporin allergy; dose adjustments may be necessary in renal failure; carefully evaluate rash to differentiate nonallergic ampicillin rash from hypersensitivity reaction
Vaccines
Active immunization increases resistance to infection. Vaccines consist of microorganisms or cellular components that act as antigens. The administration of the vaccine stimulates the production of antibodies with specific protective properties.
Because of the increased problem of penicillin resistance in S pneumoniae, prevention with PCV7 in children or PPV23 in children and adults is mandatory. Similarly, immunizations with the conjugated H influenzae type b vaccine and the meningococcal conjugated or polysaccharide vaccine are essential.
Pneumococcal 7-valent conjugate vaccine (Prevnar)
Sterile solution of saccharides of capsular antigens of S pneumoniae serotypes 4, 6B, 9V, 14, 18C, 19F, and 23F individually conjugated to diphtheria CRM197 protein. These 7 serotypes responsible for >80% of invasive pneumococcal disease in children <6 y in the United States. Accounts for 74% of penicillin-nonsusceptible S pneumoniae (PNSP) and 100% of pneumococci with high-level penicillin resistance. First dose recommended at age 2 mo but can be given in patient as young as 6 wk. Preferred sites of IM injection include the anterolateral aspect of the thigh in infants or the deltoid muscle of the upper arm in toddlers and young children. Do not inject in gluteal area or areas where a major nerve trunk or blood vessel may be present.
Three 0.5-mL doses for infants aged 7-11 mo (4 wk apart; third dose after first birthday), 2 doses for 12-23 mo (2 mo apart), 1 dose for >24 mo through 9 y. Minor illnesses such as a mild upper respiratory tract infection with or without low-grade fever are generally not contraindications.
Adult
Not established
Pediatric
3 doses of 0.5 mL each at >2-mo intervals, followed by a fourth dose of 0.5 mL at age 12-15 mo; recommended dosing interval is 4-8 wk; administer fourth dose 2 mo or longer after third dose
Effects may decrease with immunosuppressive agents (immunosuppressive doses of corticosteroids, antimetabolites, alkylating agents, cytotoxic agents); may increase effects of anticoagulant therapy; globulin preparations may interfere with immune response and reduce effectiveness (do not administer within 3 mo of vaccine)
Documented hypersensitivity; severe or moderate febrile illness; infants or children with thrombocytopenia or coagulation disorder contraindicating IM injection (unless benefits outweigh risks)
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
For IM use only, do not administer IV under any circumstances; take special care to prevent injection into or near a blood vessel or nerve; caution in patients with possible history of latex sensitivity (packaging contains dry natural rubber); does not replace 23-valent pneumococcal polysaccharide vaccination in children >24 mo with sickle cell disease, asplenia, HIV infection, chronic illness, or immunocompromise; caution in coagulation disorders
Pneumococcal vaccine (Pneumovax-23, Pnu-Imune 23)
Polyvalent vaccine used for prophylaxis against infection with S pneumoniae. Used in populations with increased risk for pneumococcal pneumonia (eg, >55 y, chronic infection, asplenia, immunocompromise).
Adult
0.5 mL IM/SC once
Pediatric
<2 years: Contraindicated (antibody response poor in this age group)
>2 years: 0.5 mL IM/SC; repeat dose after 3-5 y in high-risk children (eg, those with functional or anatomic asplenia, those with conditions associated with rapid antibody decline after initial vaccination)
Immunosuppressive agents (large amounts of corticosteroids, antimetabolites, alkylating agents, cytotoxic agents) may reduce effectiveness; therapy with immunoglobulin preparations likely to block active immunity induced with pneumococcal vaccination, withhold for 3 mo after discontinuation of immunoglobulin therapy
Documented hypersensitivity to any component; severe or even a moderate febrile illness; age <2 y; thrombocytopenia or any coagulation disorder contraindicating IM injection unless potential benefits clearly outweigh risks
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
May cause arthralgia, fever, urticaria, Guillain-Barré syndrome (rare)
Haemophilus influenzae type b vaccines (HibTITER, ActHIB, PedvaxHIB)
For routine immunization of children against invasive diseases caused by H influenzae type b. Decreases nasopharyngeal colonization. The CDC Advisory Committee on Immunization Practices (ACIP) recommends that all children routinely receive one of the conjugate vaccines licensed for use in infants beginning at age 2 mo.
Adult
Not indicated
Pediatric
Regimens vary depending on product; one example for HibTITER follows.
2-6 months: 0.5 mL IM q2mo for 3 doses
7-11 months: If previously unvaccinated, 0.5 mL IM q2mo for 2 doses
12-14 months: If previously unvaccinated, 0.5 mL IM once
Booster dose: All children, 0.5 mL at age 15 mo or at least 2 mo after last dose of series; if aged 15-71 mo and previously unvaccinated, 0.5 mL IM given only once
Corticosteroids or cyclosporine may inhibit full immunologic response
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Delay immunization if febrile illness evident; may cause erythema, swelling, or tenderness; cause-effect relationship with observed postvaccination Guillain-Barré syndrome not established
Meningococcal vaccine (Menomune)
Capsular polysaccharide antigens (groups A, C, Y, and W-135) of N meningitidis. Used for active immunization against invasive meningococcal disease caused by inclusive serogroups. May be used to prevent and control outbreaks of serogroup C meningococcal disease according to CDC guidelines. Routine vaccination is recommended for high-risk groups (eg, deficiencies in late complement components [C3, C5-C-9], functional or actual asplenia, laboratory or industrial exposure to N meningitidis aerosols, travelers or residents of hyperendemic areas). The vaccine induces antibody response for serogroup A in individuals as young as 3 mo, but it is poorly immunogenic for serogroup C in recipients who are younger than 18-24 mo.
For information concerning geographic areas in which vaccination is recommended, contact the CDC at (404)-332-4559.
Adult
0.5 mL SC
Pediatric
Administer as in adults
Coadministration with whole-cell pertussis or whole-cell typhoid vaccines may increase endotoxin content; immunosuppressive drugs may interfere with immune response
Documented hypersensitivity; avoid during course of acute illness
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Deficiencies in late complement components (C3, C5-C9); do not administer IV/IM/ID; functional or actual asplenia; persons with laboratory or industrial exposure to N meningitidis aerosols; travelers to and residents of hyperendemic areas such as sub-Saharan Africa
Meningococcal conjugate vaccine (Menactra)
Capsular polysaccharide antigens (groups A, C, Y, and W-135) of N meningitidis individually conjugated to diphtheria toxoid proteins. Used for active immunization in individuals aged 2-55 years for the prevention of invasive meningococcal disease caused by inclusive serogroups. Routine vaccination also recommended for high-risk groups (eg, those with deficiencies in late complement components [C3, C5-C-9], functional or anatomic asplenia, properdin deficiencies, and travelers or residents of hyperendemic areas).
Adult
<55 years: 0.5 mL IM once
>55 years: Not established
Pediatric
<2 years: Not established
>2 years: Administer as in adults
Administration of immunoglobulin within 1 mo or concurrent administration with immunosuppressive agents may inhibit full immunologic response; coadministration with whole-cell pertussis or whole-cell typhoid vaccines may increase endotoxin content
Known hypersensitivity to any component of Menactra vaccine including diphtheria toxoid or a life-threatening reaction after previous administration of a vaccine containing similar components; known history of Guillain-Barré syndrome; known hypersensitivity to dry natural rubber latex
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Risks include hemorrhage, local pain, headache, or fatigue; Guillain-Barré syndrome has been reported in a temporal relationship following administration of Menactra vaccine; an evaluation of postmarketing adverse events suggests a potential for an increased risk of Guillain-Barré syndrome following Menactra vaccination; persons previously diagnosed with Guillain-Barré syndrome should not receive Menactra vaccine; the stopper of the vial contains dry natural rubber latex, which may cause allergic reactions in latex-sensitive individuals
Influenza virus vaccine
Indicated for active immunization to prevent influenza a and b viruses. Induces antibodies following administration specific to virus strains contained in vaccine. Influenza vaccine contents are determined annually by the US Public Health Service. Typically, 3 live, attenuated virus strains are included in the formulation each year, which antigenically represent the influenza strains likely to circulate the next flu season.
Adult
0.5 mL IM for 1 dose each year prior to flu season
Pediatric
<6 months: Not established
6-35 months: 0.25 mL IM once; administer second dose 4 wk after first dose for vaccine-naïve children
3-8 years: 0.5 mL IM once; administer second dose 4 wk after first dose for vaccine-naïve children
>8 years: 0.5 mL IM for 1 dose each year prior to flu season
Fluviron: <4 years: Not established
Fluarix: <18 years: Not established
Immunosuppressive therapy (eg, high-dose corticosteroids, chemotherapy) may reduce antibody response
Documented hypersensitivity to vaccine contents including thimerosal, eggs, egg products, or chicken protein; history of Guillain-Barré syndrome; history of neurologic symptoms following vaccination
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Defer vaccination with acute febrile illnesses or neurological findings until symptoms have abated; may cause soreness at injection site, fever, malaise, and myalgia
More on Asplenia |
| Overview: Asplenia |
| Differential Diagnoses & Workup: Asplenia |
 Treatment & Medication: Asplenia |
| Follow-up: Asplenia |
| Multimedia: Asplenia |
| References |
| « Previous Page | Next Page » |
References
Fremont RD, Rice TW. Splenosis: a review. South Med J. Jun 2007;100(6):589-93. [Medline].
Leahy RT, Philip RK, Gibbons RJ, et al. Asplenia in ATR-X Syndrome: a second report. Am J Med Genet A. 2005;15;139 (1):37-9. [Medline].
Casey B, Devoto M, Jones KL, Ballabio A. Mapping a gene for familial situs abnormalities to human chromosome Xq24-q27.1. Nat Genet. Dec 1993;5(4):403-7. [Medline].
US Preventive Services Task Force. Screening for sickle cell disease in newborns: recommendation statement. Am Fam Physician. May 1 2008;77(9):1300-2. [Medline].
Committee on Infectious Diseases American Academy of Pediatrics. Red Book: 2006 Report of the Committee on Infectious Diseases. 27th Ed. 2006.
Elsayes KM, Narra VR, Mukundan G, et al. MR imaging of the spleen: spectrum of abnormalities. Radiographics. 2005;Jul-Aug;25(4):967-82. [Medline]. [Full Text].
Garson A, Bricker JT, McNamara DG. The Science and Practice of Pediatric Cardiology. Vol 3. 1990:1288-97.
Gill DG, Kara M, Moore L. Archives of diseases in childhood. Vol 66. 1991:1366.
Gorg C, Eickhorn M, Zugmaier G. The small spleen: sonographic patterns of functional hyposplenia or asplenia. J Clin Ultrasound. 2003;Mar-Apr;31(3):152-5. [Medline].
Harrod VL, Howard TA, Zimmerman SA, Dertinger SD, Ware RE. Quantitative analysis of Howell-Jolly bodies in children with sickle cell disease. Exp Hematol. Feb 2007;35(2):179-83. [Medline].
Katcher AL. Familial asplenia, other malformations, and sudden death. Pediatrics. 1980;65(3):633-635. [Medline].
Kevy SV, Tefft M, Vawier GF, Rosen FS. Hereditary splenic hypoplasia. Pediatrics. Nov 1968;42(5):752-7. [Medline].
Lane PA, O'Connell JL, Lear JL, et al. Functional asplenia in hemoglobin SC disease. Blood. Apr 15 1995;85(8):2238-44. [Medline].
Lin XZ, Chang TM, Tsai HM, et al. Liver, spleen and tumor volume measured by personal computer. Hepatogastroenterology. Mar-Apr 1999;46(26):838-42. [Medline].
Lindor NM, Smithson WA, Ahumada CA, et al. Asplenia in two father-son pairs. Am J Med Genet. Mar 13 1995;56(1):10-1. [Medline].
Long WA. Ivemark syndrome (cardiosplenic or heterotaxy syndrome). In: Fetal and Neonatal Cardiology. 1990:616-19.
Mebius RE, Kraal G. Structure and function of the spleen. Nat Rev Immunol. Aug 2005;5(8):606-16. [Medline].
Melles DC, de Marie S. Prevention of infections in hyposplenic and asplenic patients: an update. Neth J Med. Feb 2004;62(2):45-52. [Medline].
Moller JH, Neal WA. Congenital cardiac anomalies. In: Fetal, Neonatal, and Infant Cardiac Disease. 1990:767-71.
Morgan MS. Prophylaxis should be considered even for trivial animal bites. BMJ. May 10 1997;314(7091):1413. [Medline].
Pearson HA. The spleen and disturbances of splenic function. Hematology. 1993;2:1058-9.
Price VE, Blanchette VS, Ford-Jones EL. The prevention and management of infections in children with asplenia or hyposplenia. Infect Dis Clin North Am. Sep 2007;21(3):697-710, viii-ix. [Medline].
Price VE, Dutta S, Blanchette VS, et al. The prevention and treatment of bacterial infections in children with asplenia or hyposplenia: practice considerations at the Hospital for Sick Children, Toronto. Pediatr Blood Cancer. 2006;May 1;46(5):597-603. [Medline].
Rose V, Izukawa T, Moes CA. Syndromes of asplenia and polysplenia. A review of cardiac and non- cardiac malformations in 60 cases with special reference to diagnosis and prognosis. Br Heart J. Aug 1975;37(8):840-52. [Medline].
Stiehm ER. Immunodeficiency disorders. In: Immunologic Disorders in Infants and Children. 4th ed. 1996:326-327.
Tham KT, Teague MW, Howard CA, Chen SY. A simple splenic reticuloendothelial function test: counting erythrocytes with argyrophilic inclusions. Am J Clin Pathol. May 1996;105(5):548-52. [Medline].
Tribioli C, Lufkin T. The murine Bapx1 homeobox gene plays a critical role in embryonic development of the axial skeleton and spleen. Development. Dec 1999;126(24):5699-711. [Medline].
Waldman JD, Rosenthal A, Smith AL, et al. Sepsis and congenital asplenia. J Pediatr. Apr 1977;90(4):555-9. [Medline].
Wang JK, Hsieh KH. Immunologic study of the asplenia syndrome. Pediatr Infect Dis J. Nov 1991;10(11):819-22. [Medline].
Further Reading
Keywords
asplenia, hypoplasia, splenic hypoplasia, absent spleen, nonfunctional spleen, autosplenectomy, hyposplenia, splen, Ivemark syndrome, asplenia syndrome, functional asplenia, congenital asplenia, bacterial sepsis, polysplenia, Klebsiella species, Escherichia coli, Streptococcus pneumoniae, Haemophilus influenzae type b, Neisseria meningitidis, malaria, babesiosis, endocardial cushion defects, pulmonary atresia, pulmonary stenosis, transposition of the great vessels, total anomalous pulmonary venous return
double-outlet right ventricle, atrioventricular canal defects, splenosis, respiratory distress, Pearson syndrome, pancreatic insufficiency, sideroblastic anemia, Stormorken syndrome, thrombocytopenia, miosis, Smith-Fineman-Myers syndrome, mental retardation, short stature, cryptorchidism, ATR-X syndrome, thalassemia, Fanconi anemia, Hodgkin disease, systemic lupus erythematous, SLE, rheumatoid arthritis, inflammatory bowel disease, graft versus host disease, nephrotic syndrome
