An analysis of 8 cases of factor X deficiency

Shah, Nilam; Patel, Ashwin

doi:10.1007/s12288-008-0018-7

Cited by 4 publications

(11 citation statements)

References 6 publications

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“…Patients with mild disease (FX activity 0.05‐0.1 IU/mL) are mainly asymptomatic or present with mucocutaneous bleeding . Heterozygote carriers with FX activity 0.1‐0.3 IU/mL may present with minor mucocutaneous bleeding after trauma or surgery . During pregnancy, mild or moderate phenotypes appear to be improved, and severe phenotypes unlikely do so.…”

Section: Introductionmentioning

confidence: 93%

“…Flow diagram of literature review TA B L E 2 Obstetric and gynaecological bleeding episodes in women with FX deficiency severity of FX deficiency was not mentioned 3,[32][33][34]. In the case series, 63 patients had FX coagulant activity ≤0.01 IU/mL, 95 patients >0.01 IU/mL (range 0.01-0.26) and in the rest FX level was not reported.Heavy menstrual bleeding was a common bleeding symptom reported in 72/284 (25%) in total, 14/30 (47%) in case reports,3,7,9,14,15,17,19,22,23,[25][26][27][35][36][37] and 58/254 (23%) in 11 case series38,39 (Table 2). Semi-objective assessment of HMB in terms of pictorial blood assessment chart was not reported.…”

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confidence: 99%

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Congenital Factor X deficiency in women: A systematic review of the literature

Spiliopoulos

Kadir

2019

Haemophilia

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Factor X deficiency (FXD) is a rare autosomal recessive bleeding disorder with a variable phenotypic severity. In women, heavy menstrual bleeding (HMB), recurrent ovulation bleeding with haemoperitoneum and bleeding complications in pregnancy such as retroplacental haematoma and postpartum haemorrhage have been reported. The aim of this review was to examine gynaecological problems and obstetric complications in women with congenital FXD. A total number of 49 relevant articles were identified, including 332 women, dating from 1960 to 2018. Heavy menstrual bleeding was reported in 72/284 (25%) women in total, 14/30 (47%) in case reports and 58/254 (23%) in 11 case series, 64% and 10% required blood products and blood transfusion, respectively. Haemoperitoneum from ovulation bleeding or ruptured haemorrhagic ovarian cyst requiring blood transfusion occurred in 8/322 (2.4%) women, six required surgical intervention, including oophorectomy in two. 31 pregnancies were reported in 19 women. There were four miscarriages (including a late miscarriage at 21 weeks). There was a high rate of preterm birth and neonatal death occurring in eight (30%) and three (11%) of pregnancies reaching viability stage. Postpartum haemorrhage (PPH) occurred in six (22%) of deliveries, one requiring hysterectomy. In conclusion, women with FXD are at an increased risk of heavy bleeding during menstruation and ovulation as well as adverse pregnancy outcome and postpartum haemorrhage. Collaboration in a multidisciplinary team including an obstetrician/gynaecologist, a perinatologist and a haematologist is necessary for the prevention and management of these complications.

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Section: Introductionmentioning

confidence: 93%

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confidence: 99%

Congenital Factor X deficiency in women: A systematic review of the literature

Spiliopoulos

Kadir

2019

Haemophilia

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“…[ 2 ] The bleeding manifestations are variable and their severity depends on functional activity of FX and genetic mutation in a given patient. [ 3 ] Most common manifestations include mucocutaneous bleeds such as cutaneous bruising, epistaxis, gum bleeds and menorrhagia in females. [ 4 ] Severe bleeding events such as hemarthrosis, intracranial and gastrointestinal bleeding, as seen in hemophilia, generally occur in those with severe (FX activity <1%) disease, but are uncommon in moderately severe (FX activity between 1% and 5%) disease.…”

Section: Introductionmentioning

confidence: 99%

“…Heterozygote carriers (FX activity between 10% and 30%) rarely can have minor mucocutaneous bleeding manifestations, after trauma or surgery. [ 3 ] Here we report the rare occurrence of life-threatening massive spontaneous intraperitoneal bleeding with hypovolemic shock, most probably following rupture of an ovarian cyst in a 25-year-old woman with FX deficiency carrier state (FX activity: 26%).…”

Section: Introductionmentioning

confidence: 99%

Near fatal spontaneous intraperitoneal bleeding: A rare manifestation in a congenital factor X deficiency carrier

Vinod

Babu

Kaaviya

et al. 2015

Indian Journal of Critical Care Medicine

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Congenital factor X (FX) deficiency is a rare coagulation disorder of autosomal recessive inheritance, characterized by bleeding of variable severity. Bleeding severity generally correlates with the level of FX functional activity and severe bleeding usually occurs in moderate and severe deficiency, when FX coagulant activity is <5%. FX activity above 10% is infrequently associated with severe bleeding. Here we report the rare occurrence of life-threatening massive spontaneous intraperitoneal bleeding with hypovolemic shock, resulting from spontaneous rupture of an ovarian luteal cyst in a 25-year-old FX deficiency carrier woman, with a FX activity of 26%. She was managed successfully conservatively, with fresh frozen plasma and packed red blood cell transfusions and she showed gradual improvement. The case is being reported to discuss the diagnosis and management of this rare inherited coagulation disorder.

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“…Reports of intraparenchymal hemorrhage have been relatively more common [8,9,10,11,12]. Subdural hematomas have been reported in congenital factor X deficiency [5,9,13]. Fujimoto et al [14] documented an infant who had intraventricular and epidural hematomas.…”

Section: Introductionmentioning

confidence: 99%

Factor X Deficiency Presenting with Bilateral Chronic Subdural Hematoma

Şentürk¹,

Güzel²,

Bayrak³

et al. 2010

Pediatr Neurosurg

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Factor X deficiency is a rare coagulation defect that can result in several hemorrhagic manifestations including central nervous system hematomas in infants and children. In this case report, we present computed tomography (CT) and magnetic resonance (MR) imaging findings of bilateral chronic subdural hematomas due to factor X deficiency. Cranial CT and MR imaging in a hypoactive 7-month-old male infant with right hemiparesis revealed bilateral chronic subdural hematomas at different stages. Laboratory findings showed a severe factor X deficiency, with a level of 0.7%. After fresh frozen plasma replacement, the patient was operated and the large hematoma on the left side evacuated. The patient recovered uneventfully and remained asymptomatic during the 1-year follow-up.

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An analysis of 8 cases of factor X deficiency

Abstract: Background Factor X defi ciency is a rare coagulation defect. There are occasional reports of factor X defi ciency from India. Diffi culty in accurate diagnosis and non-availability of ideal treatment is discussed.

Cited by 4 publications

References 6 publications

Congenital Factor X deficiency in women: A systematic review of the literature

Congenital Factor X deficiency in women: A systematic review of the literature

Near fatal spontaneous intraperitoneal bleeding: A rare manifestation in a congenital factor X deficiency carrier

Factor X Deficiency Presenting with Bilateral Chronic Subdural Hematoma

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