Diaphragmatic paralysis pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

The exact pathogenesis of [disease name] is not fully understood.

OR

It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].

OR

[Pathogen name] is usually transmitted via the [transmission route] route to the human host.

OR

Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.

OR


[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].

OR

The progression to [disease name] usually involves the [molecular pathway].

OR

The pathophysiology of [disease/malignancy] depends on the histological subtype.

Pathophysiology

The main muscles of inspiration:

  • Scalenes
  • External intercostals,
  • Sternomastoids.

The muscles of expiration:

  • Internal intercostals
  • Muscles of the abdominal wall
    • Rectus abdominus
    • Internal and external obliques
    • Transversus abdominus

Pathogenesis

  • The diaphragm is the musculo-fibrous membrane. It has two parts: non-contractile central fibrous  and peripheral muscular components. [1][2][3]
  • Peripheral muscular section has two fibers:
    • Type 1: slow and fatigue resistant fibers: play roles in low intensity, continual cycle of breathing
    • Type 2: fast fibers: play roles in rapid and intense situations such as:
      • Talking
      • Singing,
      • Sneezing,
      • Defecation
      • Acute hyperventilation

The diaphragm create negative intrathoracic pressure and facilitates movement of air into the lungs. It is innervated by cervical nerve roots ( C3-C5 ) via the phrenic nerves.[4][5]

Diaphragmatic paralyses can be unilateral or bilateral according to involvemnet of one or two leaflets of diaphragm.

Diaphragmatic paralysis is an uncommon cause of dyspnea.

It is understood that diaphragmatic paralysis is the result of paralysis of ( C3-C5 ).

In the case of unilateral diaphragm paralysis, it is compensated by other hemidiaphragm or accessory muscles of respiration.

In bilateral diaphragmatic paralysis, accessory muscles do all of the work of breathing and finally it may lead to ventilatory failure.

Genetics

Early diaphragmatic paralysis may be one of the manifestations od genetic neuromuscular disorders such as spinal muscular atrophy (Werdnig-Hoffmann disease) and acid maltase deficiency.[6]

  • [Disease name] is transmitted in [mode of genetic transmission] pattern.
  • Genes involved in the pathogenesis of [disease name] include [gene1], [gene2], and [gene3].
  • The development of [disease name] is the result of multiple genetic mutations.

Associated Conditions

Gross Pathology

  • On gross pathology, there are no characteristic findings of diaphragmatic paralysis

Microscopic Pathology

  • On microscopic histopathological analysis, There are no characteristic findings of sialolithiasis.

References

  1. Mizuno M (1991). "Human respiratory muscles: fibre morphology and capillary supply". Eur. Respir. J. 4 (5): 587–601. PMID 1936230.
  2. Sánchez J, Medrano G, Debesse B, Riquet M, Derenne JP (1985). "Muscle fibre types in costal and crural diaphragm in normal men and in patients with moderate chronic respiratory disease". Bull Eur Physiopathol Respir. 21 (4): 351–6. PMID 4041660.
  3. Roussos C, Macklem PT (1982). "The respiratory muscles". N. Engl. J. Med. 307 (13): 786–97. doi:10.1056/NEJM198209233071304. PMID 7050712.
  4. Lieberman DA, Faulkner JA, Craig AB, Maxwell LC (1973). "Performance and histochemical composition of guinea pig and human diaphragm". J Appl Physiol. 34 (2): 233–7. doi:10.1152/jappl.1973.34.2.233. PMID 4265565.
  5. Fell SC (1998). "Surgical anatomy of the diaphragm and the phrenic nerve". Chest Surg. Clin. N. Am. 8 (2): 281–94. PMID 9619305.
  6. Sivan Y, Galvis A (1990). "Early diaphragmatic paralysis. In infants with genetic disorders". Clin Pediatr (Phila). 29 (3): 169–71. doi:10.1177/000992289002900305. PMID 2407409.

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