Septicaemia and adrenal haemorrhage in congenital asplenia.

Gill, D. G.; Kara, Müjdat

doi:10.1136/adc.66.11.1366-b

Cited by 9 publications

(2 citation statements)

References 2 publications

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“…The same search using the ISI Web of Science database identified 1,549 publications, including 4 relevant publications not previously identified (69,73,163,169). In addition, all identified publications were searched for further relevant references, which identified another eight papers (63,121,129,165,189,209,215,220). Only cases in which the likely underlying etiology was described were included.…”

Section: Search Strategy and Resultsmentioning

confidence: 99%

Epidemiology, Etiology, Pathogenesis, and Diagnosis of Recurrent Bacterial Meningitis

2008

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SUMMARY Recurrent bacterial meningitis is a rare phenomenon and generally poses a considerable diagnostic challenge to the clinician. Ultimately, a structured approach and early diagnosis of any underlying pathology are crucial to prevent further episodes and improve the overall outcome for the affected individual. In this article, we are reviewing the existing literature on this topic over the last two decades, encompassing 363 cases of recurrent bacterial meningitis described in 144 publications. Of these cases, 214 (59%) were related to anatomical problems, 132 (36%) were related to immunodeficiencies, and 17 (5%) were related to parameningeal infections. The review includes a detailed discussion of the underlying pathologies and microbiological aspects as well as recommendations for appropriate diagnostic pathways for investigating this unusual entity.

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Section: Search Strategy and Resultsmentioning

confidence: 99%

Epidemiology, Etiology, Pathogenesis, and Diagnosis of Recurrent Bacterial Meningitis

2008

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“…Dyke [8] reported 5 cases of congenital asplenia associated with adrenal haemorrhage in 4 cases with overwhelming sepsis with Haemophilus influenza and death within 24 hrs. Gill [16] and Kanthan [21] reported an autopsy finding of pneumococcemia and acute bilateral adrenal haemorrhage with asplenia in a 4 yr old with sudden unexpected death. The diagnosis of functional asplenia in the context of pneumococcal sepsis can be confirmed by identification of Howell-Jolly bodies on a blood film or surface pits in the erythrocyte membrane using phase contrast microscopy and the absence of the spleen noted on ultrasound examination of the abdomen [15].…”

Section: Discussionmentioning

confidence: 99%

Familial isolated congenital asplenia: case report and literature review

et al. 2009

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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. The mode of inheritance is usually autosomal dominant, though sporadic cases are also reported. In affected individuals, the use of appropriate antibiotic prophylaxis and immunisations could save lives. In our report, we describe a family of three siblings with isolated congenital asplenia and unaffected parents, suggestive of recessive inheritance. The diagnosis in the proband was made post mortem following overwhelming pneumococcal sepsis. We also review the literature and compare the eight families previously reported with congenital isolated asplenia.

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Asplenia (Congenital Absence of the Spleen)

Al-Salem

2023

The Spleen

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Septicaemia and adrenal haemorrhage in congenital asplenia.

Cited by 9 publications

References 2 publications

Epidemiology, Etiology, Pathogenesis, and Diagnosis of Recurrent Bacterial Meningitis

Epidemiology, Etiology, Pathogenesis, and Diagnosis of Recurrent Bacterial Meningitis

Familial isolated congenital asplenia: case report and literature review

Asplenia (Congenital Absence of the Spleen)

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