Immunodeficiency diseases: Head and neck manifestations

Harris, Jeffrey P.; South, Mary Ann

doi:10.1002/hed.2890050206

Cited by 10 publications

(26 citation statements)

References 21 publications

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“…Bei Nasenabstrichuntersuchungen wurden überwiegend Haemophilus influenzae, Streptococcus pneumoniae und Staphylococcus aureus, aber auch Mycoplasma pneumoniae gefunden [1,3,8,9].…”

Section: Auswirkungen Der Krankheitunclassified

“…In der Literatur finden sich nur sehr wenige Untersuchungen zu morphologischen Veränderungen der Nasenschleimhaut bei Immunmangelzuständen [1,8]. Insbesondere war das Fehlen von Plasmazellen aufgefallen [1,2].…”

Section: Auswirkungen Der Krankheitunclassified

“…Ursächlich besteht eine Mutation der Bruton-Tyrosinkinase [11]. Bei der quantitativen Immunglobulinbestimmung finden sich meistens IgM-Konzentrationen von unter 10 I.E./ml und Absenkungen des IgM-und IgA-Spiegels unter 10% [13].Aufgrund des Antikörperman-gels durch fehlende Plasmazellen kommt es zu chronisch rezidivierenden, bakteriellen Infekten des oberen und unteren Respirationstrakts [3,8,11,13]. Durch verspätete Diagnosestellung und ineffiziente Therapie können sich chronische Lungenerkrankungen wie Bronchiektasien, Emphyseme und Lungenfibrosen entwickeln [11].…”

Section: Diskussionunclassified

“…Die Patienten benötigen eine lebenslange Substitution durch intramuskuläre oder intravenöse Injektion von Immunglobulinen in dreiwöchigen Abständen und bei Infekten eine suffizi- ente Antibiotikatherapie [1,8,14]. Durch regelmäßige Immunglobulinsubstitution können zumindest schwere Pneumonien verhindert werden [3].…”

Section: Therapieunclassified

“…Bei hartnäckigen Verläufen von chronischer Rhinosinusitis sollte eine operative Sanierung der Nasennebenhöhlen erfolgen. Ob in jedem Fall einer chronischen Sinusitis eine operative Therapie erforderlich ist, wird kontrovers diskutiert [3,8].…”

Section: Therapieunclassified

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Chronische Rhinosinusitis bei Hypogammaglobulinämie Eine morphologische Untersuchung

Knipping

Holzhausen

Salim

et al. 2002

HNO

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Primary hypogammaglobulinemia (Bruton's disease) is a rare X-linked infantile immunodeficiency syndrome due to a B-cell defect. The patients suffer from acute and recurrent bacterial infections with chronic rhinosinusitis and chronic lung disorders. Immunoglobulin replacement therapy and antibiotics do not suffice in some cases, making sinus surgery to advance the drainage necessary. A 25-year-old man with hypogammaglobulinemia was treated with functional endoscopic sinus surgery and mucotomy of the turbinates. Tissue samples of the inferior turbinates were taken for histological and electron-microscopic examination. Immuno-electron-microscopic methods were carried out with antibodies against substance P and calcitonin gene-related peptide (CGRP). Morphological investigations to better understand pathophysiological changes in hypogammaglobulinemia are rare. Pathological changes in the glands and venous vessels could be demonstrated. A rich neural supply and participation of neuropeptides such as substance P and CGRP could play a role in the unspecific defense via neurogenetic inflammation in these cases.

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“…Bei Nasenabstrichuntersuchungen wurden überwiegend Haemophilus influenzae, Streptococcus pneumoniae und Staphylococcus aureus, aber auch Mycoplasma pneumoniae gefunden [1,3,8,9].…”

Section: Auswirkungen Der Krankheitunclassified

Section: Diskussionunclassified

Section: Therapieunclassified

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Chronische Rhinosinusitis bei Hypogammaglobulinämie Eine morphologische Untersuchung

Knipping

Holzhausen

Salim

et al. 2002

HNO

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Organ‐based clues for diagnosis of inborn errors of immunity: A practical guide for clinicians

Mohammadi

Yadegar

Mardani

et al. 2023

Immunity Inflam & Disease

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Inborn errors of immunity (IEI) comprise a group of about 490 genetic disorders that lead to aberrant functioning or the development of distinct immune system components. So far, a broad spectrum of IEI‐related manifestations has been noted in the literature. Due to overlapping signs and symptoms of IEI, physicians face challenges in appropriately diagnosing and managing affected individuals. The last decade has witnesses improving in the molecular diagnosis of IEI patients. As a result, it can be the mainstay of diagnostic algorithms, prognosis, and possibly therapeutic interventions in patients with IEI. Furthermore, reviewing IEI clinical complications demonstrates that the manifestations and severity of the symptoms depend on the involved gene that causes the disease and its penetrance. Although several diagnostic criteria have been used for IEI, not every patient can be explored in the same way. As a result of the failure to consider IEI diagnosis and the variety of diagnostic capabilities and laboratory facilities in different regions, undiagnosed patients are increasing. On the other hand, early diagnosis is an almost essential element in improving the quality of life in IEI patients. Since there is no appropriate guideline for IEI diagnosis in different organs, focusing on the clues in the patient's chief complaint and physical exams can help physicians narrow their differential diagnosis. This article aims to provide a practical guide for IEI diagnosis based on the involved organ. We hope to assist clinicians in keeping IEI diagnosis in mind and minimizing possible related complications due to delayed diagnosis.

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Otitis media as a presenting complaint in childhood immunodeficiency diseases

Wilson

Hogan²

2008

Curr Allergy Asthma Rep

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Otitis media is one of the most common childhood infections and may result from a variety of underlying problems. Suspicion of immunodeficiency should increase when ear infections are frequent; suppurative; unresponsive to antibiotics; caused by unusual organisms; or seen in the context of other frequent infections, severe eczema, or failure to thrive. Humoral immune deficiencies, particularly with an inability to make antibody to encapsulated organisms, are the immunodeficiencies most likely to cause increased otitis media. Immune system evaluation should concentrate on humoral immunodeficiency disorders, but the presenting history and physical findings also should be considered when designing the work-up. Treating the underlying immune deficiency is usually necessary to adequately control the ear infections.

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Immunodeficiency diseases: Head and neck manifestations

Cited by 10 publications

References 21 publications

Chronische Rhinosinusitis bei Hypogammaglobulinämie Eine morphologische Untersuchung

Chronische Rhinosinusitis bei Hypogammaglobulinämie Eine morphologische Untersuchung

Organ‐based clues for diagnosis of inborn errors of immunity: A practical guide for clinicians

Otitis media as a presenting complaint in childhood immunodeficiency diseases

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