Fetal hydrocephalus and neonatal stroke as the first presentation of protein C deficiency

Ichiyama, Masako; Ohga, Shouichi; Ochiai, Masayuki; Fukushima, Kotaro; Ishimura, Masataka; Torio, Michiko; Urata, Michiyo; Hotta, Taeko; Kang, Dongchon; Hara, Toshiro

doi:10.1016/j.braindev.2015.07.004

Cited by 21 publications

(23 citation statements)

References 10 publications

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“…Proteins S and Z are cofactors of protein C in the inactivation of coagulation factors. Interestingly, protein C deficiency was associated with neonatal hemorrhagic stroke [9] . In this way, statins affect many organs through pathways involving vitamin K 2 -dependent processes as well as vitamin K 2 -independent processes ( fig.…”

Section: Statins Inhibit Several Biochemical Steps Leading To Accelementioning

confidence: 99%

Medicines and Vegetable Oils as Hidden Causes of Cardiovascular Disease and Diabetes

Okuyama

Langsjoen²,

Ohara

et al. 2016

Pharmacology

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Background: Positive associations have been observed between cardiovascular disease (CVD) and type 2 diabetes mellitus (DM), but their causal relationship has not been clarified. Nevertheless, guidelines from relevant medical societies recommend using cholesterol lowering medication (statin) for both types of patients. Medicines with several different action mechanisms have been developed, and the effectiveness of different lifestyle modifications has been studied extensively for the prevention of DM, which was successful in improving clinical marker status in relatively short-term treatments, but none have been shown to be effective in improving long-term outcomes (mortality from CVD and all causes). Summary: Statin-induced suppression of prenyl intermediates in the cholesterol biosynthetic pathway has been linked to stimulated atherosclerosis and heart failure. On the other hand, certain types of vegetable oil and hydrogenated oil shortened the survival of stroke-prone spontaneously hypertensive rats by decreasing platelet number, increasing hemorrhagic tendency and damaging kidney functions, which could not be accounted for by their fatty acid and phytosterol compositions. These vegetable oils and medicines such as statin and warfarin share, in part, a common mechanism to inhibit vitamin K₂-dependent processes, which was interpreted to lead to increased onset of CVD, DM, chronic kidney disease, bone fracture and even mental disorder. Impaired vitamin K₂-dependent processes by some types of vegetable oils and medicines, but not plasma high low density lipoprotein cholesterol, were proposed as the cause of CVD, DM and other lifestyle-related diseases. High n-6/n-3 fatty acid ratio of ingested foods, but not animal fats, was emphasized to be another risk factor for many of the diseases described above. Key Messages: To date, no randomized controlled trials (RCTs) have been performed to prove the above interpretation. However, the opposite types of RCT trials have been performed by increasing the intake of high-linoleic vegetable oils and reducing that of animal fats, which resulted in increased CVD and all-cause mortality. The amounts of these vegetable oils to exhibit adverse effects in animal studies are not huge (<6 energy %), which should not be overlooked nor disregarded.

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Section: Statins Inhibit Several Biochemical Steps Leading To Accelementioning

confidence: 99%

Medicines and Vegetable Oils as Hidden Causes of Cardiovascular Disease and Diabetes

Okuyama

Langsjoen²,

Ohara

et al. 2016

Pharmacology

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“…12 Homozygous PC deficiency may be fatal if not treated. 2, [11][12][13] Other severe complications of PC deficiency-renal vein thrombosis, vitreal vein thrombosis (leading to blindness), and hydrocephalus-have been described frequently in infants with homozygous PC deficiency. 2,13 The risk of VTE with PC deficiency varies in individual patients and families and is likely related to both the degree of deficiency as well as the presence of other acquired or inherited risk factors for thrombosis.…”

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confidence: 99%

Protein C Deficiency

Dinarvand

Moser

2019

Archives of Pathology &Amp; Laboratory Medicine

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Protein C (PC) deficiency is a heritable or acquired risk factor for thrombophilia, with presentations varying from asymptomatic to venous thromboembolism to neonatal purpura fulminans, a life-threatening disorder. Hereditary PC deficiency is caused by mutation in the PC ( PROC) gene located on chromosome 2q14.3. Heterozygous and acquired PC deficiencies are more common than homozygous deficiency. The recommended initial laboratory test measures PC activity using either clot-based or chromogenic methods. There are numerous potential interferences in PC activity testing that may result in either false-positive (falsely low activity) or false-negative (falsely normal or elevated activity) results. In the present review, we discuss common clinical presentations; laboratory testing, with a focus on potential assay interferences; treatment options; and prognosis in patients with PC deficiency.

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“…We previously experienced a case of fetal ventriculomegaly and neonatal stroke associated with a PC deficiency. 3 Saxonhouse 11 and Will 8 published the guidelines on the management of neonatal thrombosis. The primary goal of early intervention for the neonatal thrombosis in those guidelines was to prevent to prevent organ ischemia and amputation of extremities.…”

Section: Discussionmentioning

confidence: 99%

“… 1 PC deficiency is a major critical thrombophilia of the newborns who presents with purpura fulminans, intracranial thromboembolism, 2 and fetal hydrocephalous. 3 Newborn infants are at the highest risk of thrombosis throughout childhood even without having the inherited thrombophilias. 4 5 The incidence of embolism has been increasingly diagnosed in infants and children, as a complication of sepsis, cancer, congenital heart disease, therapy-related events of drugs, and intravenous catheters.…”

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confidence: 99%

Transient Hemi-Lower Limb Ischemia in the Newborn: Arterial Thrombosis or Persistent Sciatic Artery?

Kirino

Ochiai

Ichiyama

et al. 2017

AJP Rep

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Neonatal thromboembolism occurs with various predispositions and triggers. Early diagnosis of the thrombosis is challenging and essential for the therapeutic interventions. We herein report two newborns who presented with transient hemi-lower limb ischemia due to (1) arterial thrombosis or (2) a persistent sciatic artery (PSA). The patient with arterial thrombosis showed elevations of fibrin degradation product and D-dimer and received antithrombin and heparin intravenously. The patient with PSA was immediately assessed by a contrast-enhanced computed tomography because of a transient ischemic episode with no evidence of hypercoagulability. Newborns suspected of having arterial thrombosis may need urgent surgical intervention along with thrombolytic and anticoagulant therapy to prevent organ ischemia and amputation of extremities. Conversely, some PSA cases have reportedly been treated conservatively. This vascular anomaly was previously reported as a cause of lower limb ischemia only in a newborn. PSA is a critical differential diagnosis of neonatal arterial thrombosis that needs urgent therapeutic intervention.

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Fetal hydrocephalus and neonatal stroke as the first presentation of protein C deficiency

Cited by 21 publications

References 10 publications

Medicines and Vegetable Oils as Hidden Causes of Cardiovascular Disease and Diabetes

Medicines and Vegetable Oils as Hidden Causes of Cardiovascular Disease and Diabetes

Protein C Deficiency

Transient Hemi-Lower Limb Ischemia in the Newborn: Arterial Thrombosis or Persistent Sciatic Artery?

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