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Familial Isolated Congenital Asplenia

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]

Most of these presented in the early years of life and only seven cases were diagnosed in the adult [ 1 ]. [omicsonline.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]

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]

Eyes

  • Anisocoria

    伝染性単核球症】【伝染性単核症】*infectious mononucleosis (略 IM) 【伝導聴覚障害】*conduction hearing impairment (殿) 【殿痛】*pygalgia 【殿部脂肪過多(症)】【殿部脂肪蓄積】*steatopygia (洞) 【洞炎】*sinusitis 【副鼻腔感染】*sinus infection (=洞感染) (導) 【導管乳頭腫】*ductal papilloma (瞳) 【瞳孔異常】*dyscoria 【瞳孔左右不同症】【瞳孔不同症】*anisocoria [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

Treatments can boost the immune system in many types of primary immunodeficiency disorders. Los tratamientos pueden estimular el sistema inmunitario en muchos tipos de trastornos de inmunodeficiencia primaria. [context.reverso.net]

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]

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]

True or false: All patients with primary immunodeficiency diseases require immunoglobulin replacement therapy to prevent infections. [context.reverso.net]

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]

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