Carnitine deficiency: Acute postpartum crisis

Angelini, C.; Govoni, E; Bragaglia, Mercedes M.; Vergani, L

doi:10.1002/ana.410040616

Cited by 49 publications

(14 citation statements)

References 18 publications

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“…Does lipid continue to accumulate in the muscles with an inverse ratio to carnitine concentrations, or is there a threshold value below which lipid-storage occurs? As illustrated in figure 4 the levels of muscle free-carnitine detected in haemodialysis patients fall within an intermediate range between those observed in carnitine deficiency myopathies (2.06 ±0.54 pmol/g NCP) [16][17][18][19][20][21][22][23] and those of our con- trols. Thus we suggest that a free-carnitine threshold value for lipid storage does exist and may correspond to the mean value observed in carnitine deficiency myopathies.…”

Section: J-----------------------------------------------------------mentioning

confidence: 75%

Plasma and Muscle Carnitine Levels in Haemodialysis Patients with Morphological-Ultrastructural Examination of Muscle Samples

Savica

Bellinghieri

Stefano

et al. 1983

Nephron

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The present study investigates 14 patients on intermittent haemodialysis. Pre-dialysis blood and muscle samples were taken for determining plasma free- and acetylcarnitine levels. The tissue fragments were used for light and electron microscopy studies. Our results support the findings of other investigators that patients on haemodialysis generally display decreased free- and acetylcarnitine levels both in plasma and skeletal muscle when compared with control values. Muscle carnitine deficiency was apparently more severe in the longer-term haemodialysis patients. Moreover, a significant correlation (p < 0.05) between plasma and muscle free-carnitine values was found. Morphologically no pathological alterations were observed in the muscle fibres in 13 of the patients. Light and electron microscopic studies of the muscle fibre of the 14th patient showed a typical nemaline myopathy with rod bodies in the cytoplasm. The muscle free-carnitine concentration in this patient was among the lowest of the group.

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Section: J-----------------------------------------------------------mentioning

confidence: 75%

Plasma and Muscle Carnitine Levels in Haemodialysis Patients with Morphological-Ultrastructural Examination of Muscle Samples

Savica

Bellinghieri

Stefano

et al. 1983

Nephron

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“…62 Plasma carnitine levels normally fall during pregnancy. 4 A deficiency of carnitine can cause a progressive myopathy associated with elevated serum CK levels and biopsy evidence of a necrotizing myopathy with abundant lipid storage droplets in muscle fibers. Women with carnitine-deficiency myopathy can deteriorate in the months postpartum; a 19-year-old woman with carnitine deficiency died within a year of delivery; another woman died within 2 years after delivery.…”

Section: Inherited Disorders Of Nerve and Muscle Complicating Pregnancymentioning

confidence: 99%

“…15,31 In other reports, weakness associated with carnitine deficiency is first evident in the postpartum months; two women were treated with supplemental carnitine, and one improved. 4,65 Cytochrome oxidase deficiency mitochondrial myopathy can lead to increased pain, fatigue, and reduced maternal mobility necessitating delivery by caesarian section. 117 However, Blake and Shaw advocated trying to avoid the rigors of caesarian section.…”

Section: Inherited Disorders Of Nerve and Muscle Complicating Pregnancymentioning

confidence: 99%

Neuromuscular disorders in pregnancy

Sax

Rosenbaum

2006

Muscle and Nerve

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Many neuromuscular conditions occur more frequently during pregnancy and include carpal tunnel syndrome, Bell's palsy, lumbosacral radiculopathy, meralgia paresthetica, intercostal neuralgia, and other compression neuropathies. Preexisting or coincident neuromuscular diseases, including demyelinating polyneuropathies, inflammatory muscle disease, myasthenia gravis, and inherited nerve and muscle disease present specific problems during pregnancy. We review management of neuromuscular disorders during pregnancy, labor, delivery, and the early postpartum period.

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“…The patient after carnitine supplementation did not regain full functionality but neither did she become any worse. Three more case reports by Boudin et al [17], and Cornelio et al [18] and Angelini et al [16] reported necrotising myopathies during pregnancy and post-partum. In the first two cases by Boudin and Cornelio, no therapy was effective and death ensued within 1 and 2 years, respectively.…”

mentioning

confidence: 95%

“…There is increasing evidence that these antibodies activate interferon-alpha (IFNalpha) in plasmatocytic dendritic cells that could be very important in facilitating immune-mediated processes that induce myopathy [7,13,14]. The main treatment for necrotising immune-mediated myopathies has so far been methylprednisolone with good results, discontinuation of the statin use where applicable [1,3,6] and carnitine supplementation, where there was deficiency [15][16][17][18].…”

mentioning

confidence: 99%

Acute treatment-resistant post-partum necrotising myopathy with anti-Ro52/TRIM21 antibodies treated with rituximab

Psarelis

Polydorou²,

Corsava

et al. 2015

Rheumatol Int

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examination of the upper and lower limbs revealed proximal muscle weakness MRC grade 3/5 in both upper and lower limbs. Due to her rapid decline (advancing muscle weakness on a daily basis), a neurological evaluation and a magnetic resonance imaging (MRI) were requested to exclude possible multiple sclerosis. The MRI brain did not show any relevant findings. The MRI spine revealed some congenital abnormalities. An immunological screen showed positive anti-Ro52/TRIM21 antibodies but negative anti-Jo1, anti-PL-7, anti-PL-12, anti-EJ, anti-OJ, anti-KS, anti-Zo, anti-Ha, anti-Mi-2, anti-MDA5, anti-155/140, anti-140, anti-SAE, anti-SRP, anti-HMGCR (anti 200/100), PM-Scl100, Ku and PM.Scl75 antibodies. Her blood tests showed elevated creatinine phosphokinase (CPK) at 38,040 U/L (normal range 136-145 U/L), lactic dehydrogenase (LDH) at 1,500 U/L (normal range 135-214 U/L), aspartate aminotransferase (AST) at 448 U/L and alanine aminotransferase (ALT) 225 U/L, whilst gamma-glutamyl transpeptidase (γGT), bilirubin and alkaline phosphatase were within normal range. Muscle carnitine levels were normal. The muscle biopsy showed acute non-inflammatory necrotising myopathy. In Fig. 1 below, we can see the initial patient presentation and the histological samples from the muscle biopsy.An electromyography study (EMG) was also conducted. The deltoid, rectus femoris, vastus lateralis, vastus medialis, tibialis anterior and soleus muscles were examined on both sides. The findings were consistent with polymyositis. Whole-body CT scan did not show any abnormal tumour growths. The patient was initially treated with high-dose IV methylprednisolone 1 g for 3 days and showed some minor initial improvement. When she was put on oral steroids, she rapidly deteriorated with more pronounced muscle weakness and dysphagia. Her deterioration was so rapid that she was transferred to the intensive care unit. Additional Sir, a 29-year-old lady presented 2 months post-partum with a 2-week history of hoarse voice, lethargy, progressively worsening proximal myopathy, peri-orbital oedema, rash on her upper chest, hands and face. Initial clinical presentation was that of dermatomyositis and the patient's general condition declined within a week of hospital admission with generalised paralysis and inability to breathe on her own. The patient was not expected to live.The patient had a 5-year history Hashimoto's thyroiditis, for which she was treated with 50 mcg thyroxine once daily. She had no history of smoking or alcohol consumption, nor any known allergies. She had a known thalassaemia trait. She had conceived through in vitro fertilisation treatment and had delivered with a caesarian section, epidural anaesthesia, a healthy baby girl at 38 weeks of gestation. This was a first and only pregnancy. She did not have any other past significant medical history nor any family history of note.Her thyroid peroxidase antibodies were elevated at 62.9 IU/ml, and her thyroglobulin antibodies were within normal range. Her respiratory, gastrointestinal, genitourina...

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Carnitine deficiency: Acute postpartum crisis

Cited by 49 publications

References 18 publications

Plasma and Muscle Carnitine Levels in Haemodialysis Patients with Morphological-Ultrastructural Examination of Muscle Samples

Plasma and Muscle Carnitine Levels in Haemodialysis Patients with Morphological-Ultrastructural Examination of Muscle Samples

Neuromuscular disorders in pregnancy

Acute treatment-resistant post-partum necrotising myopathy with anti-Ro52/TRIM21 antibodies treated with rituximab

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