Presentation
Definition A rare primary immunodeficiency and life-threatening condition, often presenting with pneumococcal sepsis. Most affected individuals die of severe bacterial infections in early childhood. [uniprot.org]
[email protected] Abstract Congenital asplenia is a rare life-threatening condition, often presenting with pneumococcal sepsis. It may arise as part of situs abnormalities or result from an unrelated specific defect of spleen development. [ncbi.nlm.nih.gov]
Most of these presented in the early years of life and only seven cases were diagnosed in the adult [ 1 ]. [omicsonline.org]
Asplenia is also present in Stormorken syndrome (thrombocytopenia and miosis). [emedicine.medscape.com]
Amine Post to Citeulike Two classes of micromechanics-based models of void enlargement are presented succinctly with their fundamental hypotheses and synopsis of derivation highlighted. [authormapper.com]
Entire Body System
- Wound Infection
【爪引抜癖】*onychotillomania (=爪甲損傷癖) 【爪甲肥大症】【爪肥大症】*onychauxis 【爪甲離床症】【爪離床症】*onycholysis *onychoschizia (=爪甲剥離症) 【爪白癬】*nail ringworm *tinea unguium[L] 【爪壁炎】*naronychia (騒) 【騒音耳痛】*odynacusis (双) 【双角子宮】*bicornate uterus (藻) 【藻菌症】*phycomycosis (創) 【創傷感染[症]】*wound [medo.jp]
Workup
Although it is generally a benign condition, it can radiographically mimic malignancy and result in extensive workup and invasive procedures. Congenital asplenia is most often found in association with other developmental anomalies. [emedicine.medscape.com]
Treatment
Retrieved 18 June 2018. linked from https://www.lls.org/leukemia/chronic-lymphocytic-leukemia/treatment/splenectomy ^ Lowenbraun, Stanley (January 1971). [en.wikipedia.org]
It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. Our staff consists of biologists and biochemists that are not trained to give medical advice . [uniprot.org]
Six months after the treatment, the patient has reported no further episodes of gastrointestinal bleeding and his hemoglobin has remained stable. [unboundmedicine.com]
For instance, penicillin remains the drug of choice for the treatment of infection with C. canimorsus. 2, 5 TABLE 4 Empiric Treatment of Possible Overwhelming Postsplenectomy Infection The rightsholder did not grant rights to reproduce this item in electronic [aafp.org]
Despite inten-sive treatment, the patient died within 24 h. Autopsyrevealed a nearly completely involuted spleen (51.5 g;normal 4150 g). [dokumen.tips]
Prognosis
Because the prognosis for these patients must be optimistic, we recommend early documentation of splenic function in children suspected of having the asplenia syndrome, prophylactic antibiotics, and parent education. [ncbi.nlm.nih.gov]
Eur J Pediatr (2010) 169:315–318 Prognosis - Isolated congenital asplenia (ICA) Congenital isolated asplenia may be a minor form of situs abnormality or a separate entity due to a specific defect of spleen development. [checkorphan.org]
Etiology
Skeletal dysmorphology is seen and severe prenatal and postnatal growth failure usually leads to death by one year of age, FCCX is most likely etiologically heterogeneous but RPS20 may be implicated in some cases Mutation In biology, a mutation is the [wikivisually.com]
Epidemiology
Classification (Orphanet): - Rare abdominal surgical disease - Rare developmental defect during embryogenesis - Rare genetic disease - Rare immune disease Classification (ICD10): - Congenital malformations, deformations and chromosomal abnormalities - Epidemiological [csbg.cnb.csic.es]
Prevention
"Guidelines for the prevention and treatment of infection in patients with an absent or dysfunctional spleen. Working Party of the British Committee for Standards in Haematology Clinical Haematology Task Force". [en.wikipedia.org]
Prevention and management ofoverwhelming postsplenectomy infectionan update. [dokumen.tips]
Waghorn DJ (2001) Overwhelming infection in asplenic patients: Current best practice preventive measures are not being followed. J Clin Pathol 54: 214-218. [omicsonline.org]
Science. doi:10.1126/science.1234864 Prevention - Isolated congenital asplenia (ICA) It is already known that congenital isolated asplenia is a rare condition, which is heterogeneous in its presentation. [checkorphan.org]
Guidelines for the prevention and treatment of infection in patients with an absent or dysfunctional spleen. BMJ. 1996;312:430–4. 6. Advisory Committee on Immunization Practices (ACIP). Prevention of pneumococcal disease. [aafp.org]