Chronic granulomatous disease - Revision history

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Text replacement - " species]]" to "]]"

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	*Recurrent infections		*Recurrent infections
	**Catalase-positive organisms, including [[Staphylococcus aureus]], [[Burkholderia cepacia]], [[Serratia marcescens]], [[Nocardia ~~species~~]], and [[Aspergillus ~~species~~]]		**Catalase-positive organisms, including [[Staphylococcus aureus]], [[Burkholderia cepacia]], [[Serratia marcescens]], [[Nocardia]], and [[Aspergillus]]
	**CGD is the highest-risk group for [[invasive aspergillosis]]		**CGD is the highest-risk group for [[invasive aspergillosis]]
	*Granuloma formation, often in the GI and GU tracts and may precede other clinical symptoms		*Granuloma formation, often in the GI and GU tracts and may precede other clinical symptoms

Aidan: /* Diagnosis */

2021-11-09T17:59:19Z

Diagnosis

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	==Diagnosis==		==Diagnosis==

	*The diagnosis is confirmed with the ~~dihyohodamine~~-123 (DHR-123) test		*The diagnosis is confirmed with the dihydrohodamine-123 (DHR-123) test

	==Management==		==Management==

Aidan: /* Differential Diagnosis */

2020-08-15T19:41:49Z

Differential Diagnosis

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	*[[Inflammatory bowel disease]]		*[[Inflammatory bowel disease]]
	*[[Cystic fibrosis]]		*[[Cystic fibrosis]]
			*[[Sarcoidosis]], which will cause granuloma formation but shouldn't increase risk of infection
	*[[Sarcoidosis]]

	==Diagnosis==		==Diagnosis==

Aidan: /* Clinical Manifestations */

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Clinical Manifestations

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	==Clinical Manifestations==		==Clinical Manifestations==

	*Recurrent infections ~~with catalase-positive organisms, including [[Staphylococcus aureus]]~~		*Recurrent infections
			**Catalase-positive organisms, including [[Staphylococcus aureus]], [[Burkholderia cepacia]], [[Serratia marcescens]], [[Nocardia species]], and [[Aspergillus species]]
	*~~Highest~~-risk group for [[invasive aspergillosis]]		**CGD is the highest-risk group for [[invasive aspergillosis]]
			*Granuloma formation, often in the GI and GU tracts and may precede other clinical symptoms

	== Differential Diagnosis ==		==Differential Diagnosis==

	* Other [[Primary immunodeficiency\|primary immunodeficiencies]]		*Other [[Primary immunodeficiency\|primary immunodeficiencies]]
	** [[MPO deficiency]], which will have an abnormal DHR test but largely asymptomatic unless concurrent [[diabetes mellitus]]		**[[MPO deficiency]], which will have an abnormal DHR test but largely asymptomatic unless concurrent [[diabetes mellitus]]
	** [[Hyper-IgE syndrome]], though ''Aspergillus'' will be more typically pulmonary		**[[Hyper-IgE syndrome]], though ''Aspergillus'' will be more typically pulmonary
	** IRAK4/MyD88 deficiency, which won't have fungal infections and improves with age		**IRAK4/MyD88 deficiency, which won't have fungal infections and improves with age
	** Humoral immunodeficiencies, such as [[CVID]] or [[Bruton tyrosine kinase deficiency]], though they tend to have more infections with encapsulated organisms and will have abnormal quantitative immunoglobulins		**Humoral immunodeficiencies, such as [[CVID]] or [[Bruton tyrosine kinase deficiency]], though they tend to have more infections with encapsulated organisms and will have abnormal quantitative immunoglobulins
	* [[Inflammatory bowel disease]]		*[[Inflammatory bowel disease]]
	* [[Cystic fibrosis]]		*[[Cystic fibrosis]]
	* [[Sarcoidosis]]		*[[Sarcoidosis]]

	== Diagnosis ==		==Diagnosis==

	* The diagnosis is confirmed with the dihyohodamine-123 (DHR-123) test		*The diagnosis is confirmed with the dihyohodamine-123 (DHR-123) test

	==Management==		==Management==
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	**Possibly also [[interferon-γ]]		**Possibly also [[interferon-γ]]

	== Further Reading ==		==Further Reading==

	* Considerations in the Diagnosis of Chronic Granulomatous Disease. ''J Pediatric Infect Dis Soc''. 2018;7(S1):S6-S11. doi: [https://doi.org/10.1093/jpids/piy007 10.1093/jpids/piy007]		*Considerations in the Diagnosis of Chronic Granulomatous Disease. ''J Pediatric Infect Dis Soc''. 2018;7(S1):S6-S11. doi: [https://doi.org/10.1093/jpids/piy007 10.1093/jpids/piy007]

	[[Category:Pediatrics]]		[[Category:Pediatrics]]

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added DDx

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	== Background ==		==Background==

	* [[Primary immunodeficiency]] of neutrophils leading to recurrent infections with [[catalase-positive]] organisms		*[[Primary immunodeficiency]] of neutrophils leading to recurrent infections with [[catalase-positive]] organisms

	=== Pathophysiology ===		===Pathophysiology===

	* Defect in NADPH oxidase		*Defect in NADPH oxidase
	* Therefore unable to generate superoxide radicals required by phagocytes to kill pathogens		*Therefore unable to generate superoxide radicals required by phagocytes to kill pathogens

	== Clinical Manifestations ==		==Clinical Manifestations==

	* Recurrent infections with catalase-positive organisms, including [[Staphylococcus aureus]]		*Recurrent infections with catalase-positive organisms, including [[Staphylococcus aureus]]
	* Highest-risk group for [[invasive aspergillosis]]		*Highest-risk group for [[invasive aspergillosis]]

	== ~~Management~~ ==		== Differential Diagnosis ==

			* Other [[Primary immunodeficiency\|primary immunodeficiencies]]
⚫	* Treat intercurrent infections
			** [[MPO deficiency]], which will have an abnormal DHR test but largely asymptomatic unless concurrent [[diabetes mellitus]]
⚫	* Prophylaxis with:
			** [[Hyper-IgE syndrome]], though ''Aspergillus'' will be more typically pulmonary
⚫	** [[TMP-SMX]]
			** IRAK4/MyD88 deficiency, which won't have fungal infections and improves with age
⚫	** [[Itraconazole]]
			** Humoral immunodeficiencies, such as [[CVID]] or [[Bruton tyrosine kinase deficiency]], though they tend to have more infections with encapsulated organisms and will have abnormal quantitative immunoglobulins
⚫	** Possibly also [[interferon-γ]]
			* [[Inflammatory bowel disease]]
			* [[Cystic fibrosis]]
			* [[Sarcoidosis]]

			== Diagnosis ==

			* The diagnosis is confirmed with the dihyohodamine-123 (DHR-123) test

			==Management==

		⚫	*Treat intercurrent infections
		⚫	*Prophylaxis with:
		⚫	**[[TMP-SMX]]
		⚫	**[[Itraconazole]]
		⚫	**Possibly also [[interferon-γ]]

			== Further Reading ==

			* Considerations in the Diagnosis of Chronic Granulomatous Disease. ''J Pediatric Infect Dis Soc''. 2018;7(S1):S6-S11. doi: [https://doi.org/10.1093/jpids/piy007 10.1093/jpids/piy007]

	[[Category:Pediatrics]]		[[Category:Pediatrics]]

Aidan: Created page with "== Background == * Primary immunodeficiency of neutrophils leading to recurrent infections with catalase-positive organisms === Pathophysiology === * Defect in NADP..."

2020-08-15T19:32:05Z

Created page with "== Background == * Primary immunodeficiency of neutrophils leading to recurrent infections with catalase-positive organisms === Pathophysiology === * Defect in NADP..."

New page

== Background ==

* [[Primary immunodeficiency]] of neutrophils leading to recurrent infections with [[catalase-positive]] organisms

=== Pathophysiology ===

* Defect in NADPH oxidase
* Therefore unable to generate superoxide radicals required by phagocytes to kill pathogens

== Clinical Manifestations ==

* Recurrent infections with catalase-positive organisms, including [[Staphylococcus aureus]]
* Highest-risk group for [[invasive aspergillosis]]

== Management ==

* Treat intercurrent infections
* Prophylaxis with:
** [[TMP-SMX]]
** [[Itraconazole]]
** Possibly also [[interferon-γ]]

[[Category:Pediatrics]]
[[Category:Infectious diseases]]
[[Category:Primary immunodeficiencies]]
[[Category:Immunocompromised hosts]]