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Reduced forced vital capacity

MedGen UID:
337630
Concept ID:
C1846678
Finding
Synonym: Decreased forced vital capacity
 
HPO: HP:0032341

Definition

An abnormal reduction in the amount of air a person can expel following maximal inspiration. [from HPO]

Conditions with this feature

Cystic fibrosis
MedGen UID:
41393
Concept ID:
C0010674
Disease or Syndrome
Cystic fibrosis (CF) is a multisystem disease affecting epithelia of the respiratory tract, exocrine pancreas, intestine, hepatobiliary system, and exocrine sweat glands. Morbidities include recurrent sinusitis and bronchitis, progressive obstructive pulmonary disease with bronchiectasis, exocrine pancreatic deficiency and malnutrition, pancreatitis, gastrointestinal manifestations (meconium ileus, rectal prolapse, distal intestinal obstructive syndrome), liver disease, diabetes, male infertility due to hypoplasia or aplasia of the vas deferens, and reduced fertility or infertility in some women. Pulmonary disease is the major cause of morbidity and mortality in CF.
Congenital myopathy with fiber type disproportion
MedGen UID:
108177
Concept ID:
C0546264
Disease or Syndrome
Congenital fiber-type disproportion is a condition that primarily affects skeletal muscles, which are muscles used for movement. People with this condition typically experience muscle weakness (myopathy), particularly in the muscles of the shoulders, upper arms, hips, and thighs. Weakness can also affect the muscles of the face and muscles that control eye movement (ophthalmoplegia), sometimes causing droopy eyelids (ptosis). Individuals with congenital fiber-type disproportion generally have a long face, a high arch in the roof of the mouth (high-arched palate), and crowded teeth.\n\nIndividuals with congenital fiber-type disproportion may have joint deformities (contractures) and an abnormally curved lower back (lordosis) or a spine that curves to the side (scoliosis). Approximately 30 percent of people with this disorder experience mild to severe breathing problems related to weakness of muscles needed for breathing. Some people who experience these breathing problems require use of a machine to help regulate their breathing at night (noninvasive mechanical ventilation), and occasionally during the day as well. About 30 percent of affected individuals have difficulty swallowing due to muscle weakness in the throat. Rarely, people with this condition have a weakened and enlarged heart muscle (dilated cardiomyopathy).\n\nThe severity of congenital fiber-type disproportion varies widely. It is estimated that up to 25 percent of affected individuals experience severe muscle weakness at birth and die in infancy or childhood. Others have only mild muscle weakness that becomes apparent in adulthood. Most often, the signs and symptoms of this condition appear by age 1. The first signs of this condition are usually decreased muscle tone (hypotonia) and muscle weakness. In most cases, muscle weakness does not worsen over time, and in some instances it may improve. Although motor skills such as standing and walking may be delayed, many affected children eventually learn to walk. These individuals often have less stamina than their peers, but they remain active. Rarely, people with this condition have a progressive decline in muscle strength over time. These individuals may lose the ability to walk and require wheelchair assistance.
Autosomal recessive limb-girdle muscular dystrophy type 2I
MedGen UID:
339580
Concept ID:
C1846672
Disease or Syndrome
MDGDC5 is an autosomal recessive muscular dystrophy characterized by variable age at onset, normal cognition, and no structural brain changes (Brockington et al., 2001). It is part of a group of similar disorders resulting from defective glycosylation of alpha-dystroglycan (DAG1; 128239), collectively known as 'dystroglycanopathies' (Mercuri et al., 2006). For a discussion of genetic heterogeneity of muscular dystrophy-dystroglycanopathy type C, see MDDGC1 (609308).
Myosclerosis
MedGen UID:
338098
Concept ID:
C1850671
Disease or Syndrome
Collagen VI-related dystrophies (COL6-RDs) represent a continuum of overlapping clinical phenotypes with Bethlem muscular dystrophy at the milder end, Ullrich congenital muscular dystrophy (UCMD) at the more severe end, and a phenotype in between UCMD and Bethlem muscular dystrophy, referred to as intermediate COL6-RD. Bethlem muscular dystrophy is characterized by a combination of proximal muscle weakness and joint contractures. Hypotonia and delayed motor milestones occur in early childhood; mild hypotonia and weakness may be present congenitally. By adulthood, there is evidence of proximal weakness and contractures of the elbows, Achilles tendons, and long finger flexors. The progression of weakness is slow, and more than two thirds of affected individuals older than age 50 years remain independently ambulatory indoors, while relying on supportive means for mobility outdoors. Respiratory involvement is not a consistent feature. UCMD is characterized by congenital weakness, hypotonia, proximal joint contractures, and striking hyperlaxity of distal joints. Decreased fetal movements are frequently reported. Some affected children acquire the ability to walk independently; however, progression of the disease results in a loss of ambulation by age ten to eleven years. Early and severe respiratory insufficiency occurs in all individuals, resulting in the need for nocturnal noninvasive ventilation (NIV) in the form of bilevel positive airway pressure (BiPAP) by age 11 years. Intermediate COL6-RD is characterized by independent ambulation past age 11 years and respiratory insufficiency that is later in onset than in UCMD and results in the need for NIV in the form of BiPAP by the late teens to early 20s. In contrast to individuals with Bethlem muscular dystrophy, those with intermediate COL6-RD typically do not achieve the ability to run, jump, or climb stairs without use of a railing.
Surfactant metabolism dysfunction, pulmonary, 2
MedGen UID:
410078
Concept ID:
C1970470
Disease or Syndrome
Pulmonary surfactant metabolism dysfunction-2 (SMDP2) is a rare autosomal dominant disease associated with progressive respiratory insufficiency and lung disease with a variable clinical course. The pathophysiology of the disorder is postulated to involve intracellular accumulation of a structurally defective SPC protein (Thomas et al., 2002). For a general phenotypic description and a discussion of genetic heterogeneity of pulmonary surfactant metabolism dysfunction, see SMDP1 (265120).
Surfactant metabolism dysfunction, pulmonary, 4
MedGen UID:
393858
Concept ID:
C2677877
Disease or Syndrome
Pulmonary alveolar proteinosis (PAP) is a rare lung disorder in which surfactant-derived lipoproteins accumulate excessively within pulmonary alveoli, causing severe respiratory distress. Three forms of PAP have been described: hereditary (usually congenital), secondary, and acquired. Hereditary PAP is associated with mutations in the CSF2RA gene or in genes encoding surfactant proteins. Secondary PAP develops in conditions in which there are reduced numbers or functional impairment of alveolar macrophages and is associated with inhalation of inorganic dust (silica) or toxic fumes, hematologic malignancies, pharmacologic immunosuppression, infections, and impaired CSF2RB (138960) expression. Acquired PAP (610910), the most common form, usually occurs in adults and is caused by neutralizing autoantibodies to CSF2 (138960) (Martinez-Moczygemba et al., 2008). For a general phenotypic description and a discussion of genetic heterogeneity of congenital pulmonary surfactant metabolism dysfunction, see SMDP1 (265120).
Myofibrillar myopathy 6
MedGen UID:
414119
Concept ID:
C2751831
Disease or Syndrome
Myofibrillar myopathy-6 is an autosomal dominant severe neuromuscular disorder characterized by onset in the first decade of rapidly progressive generalized and proximal muscle weakness, respiratory insufficiency, cardiomyopathy, and skeletal deformities related to muscle weakness. Muscle biopsy shows fiber-type grouping, disruption of the Z lines, and filamentous inclusions, and sural nerve biopsy shows a neuropathy, often with giant axonal neurons. Most patients are severely affected by the second decade and need cardiac transplant, ventilation, and/or a wheelchair (summary by Jaffer et al., 2012). For a phenotypic description and a discussion of genetic heterogeneity of myofibrillar myopathy (MFM), see MFM1 (601419).
Spondylocostal dysostosis 4, autosomal recessive
MedGen UID:
462292
Concept ID:
C3150942
Disease or Syndrome
Spondylocostal dysostosis (SCDO), defined radiographically as multiple segmentation defects of the vertebrae (M-SDV) in combination with abnormalities of the ribs, is characterized clinically by: a short trunk in proportion to height; short neck; non-progressive mild scoliosis in most affected individuals, and occasionally, more significant scoliosis. Respiratory function in neonates may be compromised by reduced size of the thorax. By age two years lung growth may improve sufficiently to support relatively normal growth and development; however, even then life-threatening complications can occur, especially pulmonary hypertension in children with severely restricted lung capacity from birth. Males with SCDO appear to be at increased risk for inguinal hernia.
Congenital myopathy 10b, mild variant
MedGen UID:
762102
Concept ID:
C3541476
Disease or Syndrome
Congenital myopathy-10B (CMYP10B) is an autosomal recessive skeletal muscle disorder characterized by infantile- or childhood-onset myopathy, areflexia, dysphagia, and respiratory distress that usually requires nocturnal ventilation. Other common features include facial and neck muscle weakness, feeding difficulties, contractures, scoliosis, high-arched palate, hyporeflexia, and difficulties walking. The disorder is slowly progressive and most patients follow a chronic course. Muscle biopsy shows variable findings, including type 1 fiber predominance, minicore lesions, and myofibrillar disorganization (Boyden et al., 2012; Harris et al., 2018). Patients with missense mutations affecting conserved cysteine residues in the EGF-like domain show the mild variant phenotype (CMYP10B) with later onset of respiratory failure and minicores on muscle biopsy, whereas patients with more damaging mutations, including nonsense or frameshift null mutations, show the severe variant phenotype (CMYP10A) (Croci et al., 2022). For a discussion of genetic heterogeneity of congenital myopathy, see CMYP1A (117000).
Pulmonary fibrosis and/or bone marrow failure, Telomere-related, 3
MedGen UID:
901644
Concept ID:
C4225346
Disease or Syndrome
Dyskeratosis congenita and related telomere biology disorders (DC/TBD) are caused by impaired telomere maintenance resulting in short or very short telomeres. The phenotypic spectrum of telomere biology disorders is broad and includes individuals with classic dyskeratosis congenita (DC) as well as those with very short telomeres and an isolated physical finding. Classic DC is characterized by a triad of dysplastic nails, lacy reticular pigmentation of the upper chest and/or neck, and oral leukoplakia, although this may not be present in all individuals. People with DC/TBD are at increased risk for progressive bone marrow failure (BMF), myelodysplastic syndrome or acute myelogenous leukemia, solid tumors (usually squamous cell carcinoma of the head/neck or anogenital cancer), and pulmonary fibrosis. Other findings can include eye abnormalities (epiphora, blepharitis, sparse eyelashes, ectropion, entropion, trichiasis), taurodontism, liver disease, gastrointestinal telangiectasias, and avascular necrosis of the hips or shoulders. Although most persons with DC/TBD have normal psychomotor development and normal neurologic function, significant developmental delay is present in both forms; additional findings include cerebellar hypoplasia (Hoyeraal Hreidarsson syndrome) and bilateral exudative retinopathy and intracranial calcifications (Revesz syndrome and Coats plus syndrome). Onset and progression of manifestations of DC/TBD vary: at the mild end of the spectrum are those who have only minimal physical findings with normal bone marrow function, and at the severe end are those who have the diagnostic triad and early-onset BMF.
Pulmonary fibrosis and/or bone marrow failure, Telomere-related, 4
MedGen UID:
903928
Concept ID:
C4225347
Disease or Syndrome
Dyskeratosis congenita and related telomere biology disorders (DC/TBD) are caused by impaired telomere maintenance resulting in short or very short telomeres. The phenotypic spectrum of telomere biology disorders is broad and includes individuals with classic dyskeratosis congenita (DC) as well as those with very short telomeres and an isolated physical finding. Classic DC is characterized by a triad of dysplastic nails, lacy reticular pigmentation of the upper chest and/or neck, and oral leukoplakia, although this may not be present in all individuals. People with DC/TBD are at increased risk for progressive bone marrow failure (BMF), myelodysplastic syndrome or acute myelogenous leukemia, solid tumors (usually squamous cell carcinoma of the head/neck or anogenital cancer), and pulmonary fibrosis. Other findings can include eye abnormalities (epiphora, blepharitis, sparse eyelashes, ectropion, entropion, trichiasis), taurodontism, liver disease, gastrointestinal telangiectasias, and avascular necrosis of the hips or shoulders. Although most persons with DC/TBD have normal psychomotor development and normal neurologic function, significant developmental delay is present in both forms; additional findings include cerebellar hypoplasia (Hoyeraal Hreidarsson syndrome) and bilateral exudative retinopathy and intracranial calcifications (Revesz syndrome and Coats plus syndrome). Onset and progression of manifestations of DC/TBD vary: at the mild end of the spectrum are those who have only minimal physical findings with normal bone marrow function, and at the severe end are those who have the diagnostic triad and early-onset BMF.
Autosomal recessive limb-girdle muscular dystrophy type 2R1
MedGen UID:
934627
Concept ID:
C4310660
Disease or Syndrome
Autosomal recessive limb-girdle muscular dystrophy-21 (LGMDR21) is characterized by progressive limb-girdle weakness with age of onset ranging from congenital to adult. Muscle imaging shows a specific and selective pattern of fatty muscle degeneration (summary by Servian-Morilla et al., 2020). For a discussion of genetic heterogeneity of autosomal recessive LGMD, see LGMDR1 (253600).
Congenital muscular dystrophy-respiratory failure-skin abnormalities-joint hyperlaxity syndrome
MedGen UID:
934703
Concept ID:
C4310736
Disease or Syndrome
A rare congenital muscular dystrophy characterised by neonatal hypotonia, life-threatening respiratory failure and feeding difficulties, furthermore by delayed motor development, severe muscle weakness predominantly affecting axial muscles (leading to poor head control, rigid cervical spine, and severe scoliosis), generalised joint laxity with no or mild contractures, as well as dry skin with follicular hyperkeratosis. Serum creatine kinase is normal or slightly elevated. Muscle biopsy shows fibre size variability, rounded fibres with mild increase of endomysial connective tissue and adipose replacement, abundant minicore lesions, increase of centrally located nuclei, angular fibres and cap lesions.
Autosomal recessive limb-girdle muscular dystrophy type 2U
MedGen UID:
1683417
Concept ID:
C5190987
Disease or Syndrome
A rare subtype of autosomal recessive limb-girdle muscular dystrophy disorder with characteristics of infantile to childhood-onset of slowly progressive, principally proximal shoulder and/or pelvic-girdle muscular weakness that typically presents with positive Gowers'' sign and is associated with elevated creatine kinase levels, hyporeflexia, joint and achilles tendon contractures and muscle hypertrophy usually of the thighs, calves and/or tongue. Other highly variable features include cerebellar, cardiac and ocular abnormalities.
Oculopharyngodistal myopathy 1
MedGen UID:
1684682
Concept ID:
C5231388
Disease or Syndrome
Oculopharyngodistal myopathy-1 (OPDM1) is an autosomal dominant disorder characterized by adult-onset ptosis, external ophthalmoplegia, facial muscle weakness, distal limb muscle weakness and atrophy, and pharyngeal involvement, resulting in dysphagia and dysarthria. Skeletal muscle biopsy shows myopathic changes with rimmed vacuoles. There are variable manifestations of the disorder regarding muscle involvement and severity (summary by Ishiura et al., 2019). Genetic Heterogeneity of Oculopharyngodistal Myopathy See also OPDM2 (618940), caused by trinucleotide repeat expansion in the GIPC1 gene (605072) on chromosome 19p13; OPDM3 (619473), caused by trinucleotide repeat expansion in the NOTCH2NLC gene (618025) on chromosome 1q21; and OPDM4 (619790), caused by trinucleotide repeat expansion in the RILPL1 gene (614092) on chromosome 12q24. Oculopharyngeal muscular dystrophy (OPMD; 164300) is a similar disorder with overlapping features. It is caused by a similar heterozygous trinucleotide repeat expansion in the PABPN1 gene (602279) (summary by Durmus et al., 2011).
Ciliary dyskinesia, primary, 42
MedGen UID:
1684665
Concept ID:
C5231464
Disease or Syndrome
Primary ciliary dyskinesia-42 (CILD42) is an autosomal recessive disorder characterized by a defect in motile cilia and ciliary clearance resulting in the onset of respiratory insufficiency soon after birth, and associated with recurrent upper and lower respiratory infections with chronic progressive lung disease. Other more variable features may include infertility and mild hydrocephalus. Patients with this form of the disorder do not have situs abnormalities. The disorder is considered to be a type of ciliopathy known as 'reduced generation of multiple motile cilia' (RGMC) (summary by Boon et al., 2014). For a discussion of genetic heterogeneity of primary ciliary dyskinesia, CILD1 (244400).
Ciliary dyskinesia, primary, 44
MedGen UID:
1716408
Concept ID:
C5394063
Disease or Syndrome
Primary ciliary dyskinesia-44 (CILD44) is an autosomal recessive disorder characterized by recurrent sinopulmonary infections resulting from defective mucociliary clearance. Affected individuals have onset of symptoms in infancy or early childhood, and the repetitive nature of the disorder results in bronchiectasis. Although respiratory epithelial cell motile cilia are shorter than normal and overall ciliary motion is decreased, nasal nitric oxide, radial ciliary structure, and ciliary beat frequency are normal. In addition, patients do not have situs inversus (summary by Chivukula et al., 2020). For a phenotypic description and a discussion of genetic heterogeneity of primary ciliary dyskinesia, see CILD1 (244400).
Mitochondrial DNA depletion syndrome 18
MedGen UID:
1713890
Concept ID:
C5394140
Disease or Syndrome
Mitochondrial DNA depletion syndrome-18 (MTDPS18) is an autosomal recessive neuromuscular disorder characterized by early-onset progressive weakness and atrophy of the distal limb muscles, resulting in loss of ambulation as well as atrophy of the intrinsic hand muscles with clawed hands. Affected individuals may also develop scoliosis and have hypo- or hyperreflexia and decreased pulmonary vital capacity. Examination of skeletal muscle shows neurogenic atrophy and combined mitochondrial oxidative phosphorylation deficiency associated with mtDNA depletion. The clinical phenotype is reminiscent of spinal muscular atrophy (see SMA, 253300) and the metabolic profile is reminiscent of 2-aminoadipic 2-oxoadipic aciduria (AMOXAD; 204750), which is caused by mutation in the DHTKD1 gene (614984) (summary by Boczonadi et al., 2018). For a discussion of genetic heterogeneity of autosomal recessive mtDNA depletion syndromes, see MTDPS1 (603041).
Myofibrillar myopathy 11
MedGen UID:
1782465
Concept ID:
C5543038
Disease or Syndrome
Myofibrillar myopathy-11 (MFM11) is an autosomal recessive skeletal muscle disorder characterized by onset of slowly progressive proximal muscle weakness in the first decade of life. Some patients may present at birth with hypotonia and feeding difficulties, whereas others present later in mid-childhood. Although most patients show delayed walking at 2 to 3 years, all remain ambulatory into adulthood. More variable features may include decreased respiratory forced vital capacity, variable cardiac features, and calf hypertrophy. Skeletal muscle biopsy shows myopathic changes with variation in fiber size, type 1 fiber predominance, centralized nuclei, eccentrically placed core-like lesions, and distortion of the myofibrillary pattern with Z-line streaming and abnormal myofibrillar aggregates or inclusions (summary by Donkervoort et al., 2020). For a phenotypic description and a discussion of genetic heterogeneity of myofibrillar myopathy, see MFM1 (601419).
Ciliary dyskinesia, primary, 46
MedGen UID:
1780196
Concept ID:
C5543646
Disease or Syndrome
Primary ciliary dyskinesia-46 (CILD46) is characterized by recurrent sinus and respiratory infections, with reduced pulmonary function and uncoordinated beating of respiratory cilia. No situs abnormalities have been observed (Edelbusch et al., 2017). For a general phenotypic description and a discussion of genetic heterogeneity of primary ciliary dyskinesia, see CILD1 (244400).
Myasthenic syndrome, congenital, 7B, presynaptic, autosomal recessive
MedGen UID:
1794157
Concept ID:
C5561947
Disease or Syndrome
Autosomal recessive presynaptic congenital myasthenic syndrome-7B (CMS7B) is characterized by severe generalized muscle weakness apparent from birth; decreased fetal movements may be apparent in utero. Affected infants have generalized hypotonia with poor cry and feeding, head lag, and facial muscle weakness with ptosis. Some patients may have respiratory involvement. Electrophysiologic studies show decreased compound muscle action potentials (CMAPs) and a decremental response to repetitive nerve stimulation. Treatment with 3,4-diaminopyridine and pyridostigmine may result in clinical improvement (summary by Bauche et al., 2020).
Muscular dystrophy, congenital hearing loss, and ovarian insufficiency syndrome
MedGen UID:
1794190
Concept ID:
C5561980
Disease or Syndrome
Muscular dystrophy, congenital hearing loss, and ovarian insufficiency syndrome (MDHLO) is an autosomal recessive systemic disorder characterized by progressive muscle weakness, sensorineural hearing loss, and endocrine abnormalities, mainly primary amenorrhea due to ovarian insufficiency. Features of the disorder appear soon after birth, although endocrine anomalies are not noted until puberty. The severity of the phenotype is variable: some patients may lose ambulation and have significant respiratory insufficiency, whereas others retain the ability to walk (Foley et al., 2020).
Muscular dystrophy, limb-girdle, autosomal recessive 27
MedGen UID:
1794212
Concept ID:
C5562002
Disease or Syndrome
Autosomal recessive limb-girdle muscular dystrophy-27 (LGMDR27) is characterized by progressive muscle weakness primarily affecting the lower limbs and resulting in walking difficulty or loss of ambulation. The age at onset is highly variable, from infancy to young adulthood. Patients with infantile onset may have a more severe disease course with rapid progression. Upper limb involvement and distal muscle weakness may also occur. Additional more variable features include neck muscle weakness, scoliosis, and joint contractures. Less common features include impaired intellectual development or speech delay, cardiomyopathy, and cardiac arrhythmia. Muscle biopsy shows nonspecific dystrophic changes (Coppens et al., 2021). For a discussion of genetic heterogeneity of autosomal recessive limb-girdle muscular dystrophy, see LGMDR1 (253600).
Congenital myopathy 15
MedGen UID:
1824046
Concept ID:
C5774273
Disease or Syndrome
Congenital myopathy-15 (CMYP15) is a skeletal muscle disorder characterized by symptom onset soon after birth. Affected infants are hypotonic and have severe respiratory insufficiency and feeding problems, sometimes requiring mechanical ventilation or tube feeding. The disorder is unique in that there is gradual improvement of the severe muscle weakness with time, although forced vital capacity remains decreased. Additional features include facial weakness, scoliosis, joint contractures, and persistent ptosis or external ophthalmoplegia (van de Locht et al., 2021). For a discussion of genetic heterogeneity of congenital myopathy, see CMYP1A (117000).
Nemaline myopathy 5B, autosomal recessive, childhood-onset
MedGen UID:
1841181
Concept ID:
C5830545
Disease or Syndrome
Autosomal recessive childhood-onset nemaline myopathy-5B (NEM5B) is a skeletal muscle disorder in which patients usually present with proximal muscle weakness of the lower and upper limbs in a limb-girdle distribution, resulting in gait abnormalities; however, most remain ambulatory even into late adulthood. Some affected individuals show delayed motor development. There is axial weakness and atrophy of the paraspinal muscles, along with kyphosis, scoliosis, and rigid spine, as well as variable limitations of the large joints. Most patients develop restrictive respiratory insufficiency with decreased forced vital capacity; some need noninvasive ventilation. Serum creatine kinase may be elevated. Muscle biopsy can show variable features, including nemaline rods, multiminicore lesions, endomysial fibrosis, and myofibrillar changes (Pellerin et al., 2020; Lee et al., 2022). For a discussion of genetic heterogeneity of nemaline myopathy, see NEM2 (256030).

Professional guidelines

PubMed

Liu J, Maleche-Obimbo E, Shanthikumar S, Graham SM
Pediatr Pulmonol 2023 May;58(5):1344-1354. Epub 2023 Mar 21 doi: 10.1002/ppul.26365. PMID: 36811157
Johnson SR, Fransen J, Khanna D, Baron M, van den Hoogen F, Medsger TA Jr, Peschken CA, Carreira PE, Riemekasten G, Tyndall A, Matucci-Cerinic M, Pope JE
Arthritis Care Res (Hoboken) 2012 Mar;64(3):358-67. doi: 10.1002/acr.20684. PMID: 22052658Free PMC Article
Levine TD
Arthritis Rheum 2005 Feb;52(2):601-7. doi: 10.1002/art.20849. PMID: 15692974

Recent clinical studies

Etiology

Brokamp G, Hurst L, Hartog L, Vilson F, Reynolds J, Elsheikh BH, Arnold WD
Am J Phys Med Rehabil 2023 May 1;102(5):427-432. Epub 2022 Oct 1 doi: 10.1097/PHM.0000000000002114. PMID: 36228186
Hsan S, Lakhdar N, Harrabi I, Zaouali M, Burney P, Denguezli M
BMC Pulm Med 2022 Jul 11;22(1):267. doi: 10.1186/s12890-022-02062-3. PMID: 35818049Free PMC Article
Masood IR, Detterich J, Cerrone D, Lewinter K, Shah P, Kato R, Sabati A
Pediatr Cardiol 2022 Jan;43(1):54-61. Epub 2021 Aug 7 doi: 10.1007/s00246-021-02692-0. PMID: 34365518
Higbee DH, Granell R, Sanderson E, Davey Smith G, Dodd JW
Eur Respir J 2021 Sep;58(3) Epub 2021 Sep 9 doi: 10.1183/13993003.03196-2020. PMID: 33574079
Obaseki DO, Erhabor GE, Awopeju OF, Adewole OO, Adeniyi BO, Buist EAS, Burney PG
Ann Am Thorac Soc 2017 May;14(5):714-721. doi: 10.1513/AnnalsATS.201608-598OC. PMID: 28244800Free PMC Article

Diagnosis

Miller RL, Rivera J, Lichtiger L, Govindarajulu US, Jung KH, Lovinsky-Desir S, Perera F, Balcer Whaley S, Newman M, Grant TL, McCormack M, Perzanowski M, Matsui EC
Environ Res 2023 Dec 15;239(Pt 1):117342. Epub 2023 Oct 9 doi: 10.1016/j.envres.2023.117342. PMID: 37813137Free PMC Article
Kelly CR, Saw JL, Thapa P, Mandrekar J, Naddaf E
Muscle Nerve 2022 Apr;65(4):415-421. Epub 2022 Jan 25 doi: 10.1002/mus.27493. PMID: 35020192
Verboeket SO, Wit FW, Kirk GD, Drummond MB, van Steenwijk RP, van Zoest RA, Nellen JF, Schim van der Loeff MF, Reiss P; AGEhIV Study Group
J Infect Dis 2019 Apr 8;219(8):1274-1284. doi: 10.1093/infdis/jiy653. PMID: 30418599
Obaseki DO, Erhabor GE, Awopeju OF, Adewole OO, Adeniyi BO, Buist EAS, Burney PG
Ann Am Thorac Soc 2017 May;14(5):714-721. doi: 10.1513/AnnalsATS.201608-598OC. PMID: 28244800Free PMC Article
Cooksley NA, Atkinson D, Marks GB, Toelle BG, Reeve D, Johns DP, Abramson MJ, Burton DL, James AL, Wood-Baker R, Walters EH, Buist AS, Maguire GP
Respirology 2015 Jul;20(5):766-74. Epub 2015 Feb 22 doi: 10.1111/resp.12482. PMID: 25704620

Therapy

Liu J, Maleche-Obimbo E, Shanthikumar S, Graham SM
Pediatr Pulmonol 2023 May;58(5):1344-1354. Epub 2023 Mar 21 doi: 10.1002/ppul.26365. PMID: 36811157
Vehar SJ, Yadav R, Mukhopadhyay S, Nathani A, Tolle LB
Am J Clin Pathol 2023 Feb 1;159(2):146-157. doi: 10.1093/ajcp/aqac144. PMID: 36495281Free PMC Article
Higbee DH, Granell R, Sanderson E, Davey Smith G, Dodd JW
Eur Respir J 2021 Sep;58(3) Epub 2021 Sep 9 doi: 10.1183/13993003.03196-2020. PMID: 33574079
Verboeket SO, Wit FW, Kirk GD, Drummond MB, van Steenwijk RP, van Zoest RA, Nellen JF, Schim van der Loeff MF, Reiss P; AGEhIV Study Group
J Infect Dis 2019 Apr 8;219(8):1274-1284. doi: 10.1093/infdis/jiy653. PMID: 30418599
Cooksley NA, Atkinson D, Marks GB, Toelle BG, Reeve D, Johns DP, Abramson MJ, Burton DL, James AL, Wood-Baker R, Walters EH, Buist AS, Maguire GP
Respirology 2015 Jul;20(5):766-74. Epub 2015 Feb 22 doi: 10.1111/resp.12482. PMID: 25704620

Prognosis

Hock J, Willinger L, Pozza RD, Ewert P, Hager A
Cardiol Young 2023 Feb;33(2):169-181. Epub 2023 Jan 5 doi: 10.1017/S1047951122004103. PMID: 36601957
Saad HB
BMC Pulm Med 2022 Nov 11;22(1):413. doi: 10.1186/s12890-022-02218-1. PMID: 36368991Free PMC Article
Masood IR, Detterich J, Cerrone D, Lewinter K, Shah P, Kato R, Sabati A
Pediatr Cardiol 2022 Jan;43(1):54-61. Epub 2021 Aug 7 doi: 10.1007/s00246-021-02692-0. PMID: 34365518
Kim Y, Lee H, Son TO, Jang H, Cho SH, Kim SE, Kim SJ, Lee JS, Kim JP, Jung YH, Lockhart SN, Kim HJ, Na DL, Park HY, Seo SW
Neuroimage Clin 2020;25:102140. Epub 2019 Dec 24 doi: 10.1016/j.nicl.2019.102140. PMID: 31896465Free PMC Article
Cohen KE, Buelow MW, Dixon J, Brazauskas R, Cohen SB, Earing MG, Ginde S
Congenit Heart Dis 2017 Jul;12(4):435-440. Epub 2017 May 2 doi: 10.1111/chd.12470. PMID: 28464509

Clinical prediction guides

Masood IR, Detterich J, Cerrone D, Lewinter K, Shah P, Kato R, Sabati A
Pediatr Cardiol 2022 Jan;43(1):54-61. Epub 2021 Aug 7 doi: 10.1007/s00246-021-02692-0. PMID: 34365518
Higbee DH, Granell R, Sanderson E, Davey Smith G, Dodd JW
Eur Respir J 2021 Sep;58(3) Epub 2021 Sep 9 doi: 10.1183/13993003.03196-2020. PMID: 33574079
Kim Y, Lee H, Son TO, Jang H, Cho SH, Kim SE, Kim SJ, Lee JS, Kim JP, Jung YH, Lockhart SN, Kim HJ, Na DL, Park HY, Seo SW
Neuroimage Clin 2020;25:102140. Epub 2019 Dec 24 doi: 10.1016/j.nicl.2019.102140. PMID: 31896465Free PMC Article
Verboeket SO, Wit FW, Kirk GD, Drummond MB, van Steenwijk RP, van Zoest RA, Nellen JF, Schim van der Loeff MF, Reiss P; AGEhIV Study Group
J Infect Dis 2019 Apr 8;219(8):1274-1284. doi: 10.1093/infdis/jiy653. PMID: 30418599
Cohen KE, Buelow MW, Dixon J, Brazauskas R, Cohen SB, Earing MG, Ginde S
Congenit Heart Dis 2017 Jul;12(4):435-440. Epub 2017 May 2 doi: 10.1111/chd.12470. PMID: 28464509

Recent systematic reviews

Hock J, Willinger L, Pozza RD, Ewert P, Hager A
Cardiol Young 2023 Feb;33(2):169-181. Epub 2023 Jan 5 doi: 10.1017/S1047951122004103. PMID: 36601957
Lee JH, Yim JJ, Park J
Respir Res 2022 Sep 6;23(1):233. doi: 10.1186/s12931-022-02163-x. PMID: 36068582Free PMC Article

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