Coagulation Factor Deficiencies Research Articles

Rare bleeding disorders in Egyptian females presented with heavy menstrual bleeding: single-center study

BackgroundHeavy menstrual bleeding is an important health problem in women of reproductive age and is also one of the most common symptoms in women with bleeding disorders. Data about the frequency of rare bleeding disorders are limited, and population-based studies are lacking, so we aimed to determine the frequency of rare bleeding disorders among women presented with heavy menstrual periods that cannot be attributed to obvious problems.MethodsComplete blood count and bleeding profiles include activated partial thromboplastin time, prothrombin time, factor VIII activity assay, ristocetin cofactor activity, von Willebrand antigen assay, platelet aggregation tests and other factor assays in 100 out of 300 females presented with unexplained heavy menstrual period, pictorial bleeding assessment chart (PBAC) > 100 as a screening tool for heavy menstrual periods, and or International Society of Thrombosis and Hemostasis-Bleeding Assessment Tool (ISTH-BAT) > 6.ResultsA total of 300 women with heavy menstrual periods without an obvious explained cause were included in our study. Among them, we found 100 (30%) females with a mean PBAC of 234 ± 147 and mean ISTH-BAT of 9 ± 5 denoting HMB may be due to underlying bleeding disorders. Among them, the most common diagnosis was VWD in 30 (30%). Other disorders were as follows: 28 (28%) cases with clotting factor deficiencies, 24 (24%) cases were found to have platelet dysfunction, and in 18% of our studied cases, we did not find a clear cause of their bleeding disorders (unknown).ConclusionRare bleeding disorders are not uncommon and require comprehensive hemostatic evaluation as well as simple tools like PBAC and ISTH-BAT questionnaires for the identification of females presented with unexplained HMB.

New method to differentiate between lupus anticoagulants, progressive coagulation inhibitors and coagulation factor deficiencies in the mixing tests.

Mixing tests in activated partial thromboplastin time (APTT) are used for the differentiation between lupus anticoagulants (LA), coagulation inhibitors, and factor deficient samples with APTT prolongation. However, the indexes for the differentiation have not been established. The present study aimed to develop new mixing test indexes for the differentiation. Twenty-six LA-positive, 8 progressive coagulation factor VIII inhibitor, and 35 coagulation deficient samples were employed. APTT were measured for normal plasma, patient plasma, and mixing plasma prepared at a ratio of 1:1 proportion in both without incubation and 2 h-incubation. New two parameters named as ALD50 and mixture plasma-patient plasma after Warming change rate Subtraction (WaS) calculated from the clotting times of normal, 1:1 mixing and patient samples with/without 2 h-incubation were established. In the samples with WaS result of <10.2%, ALD50 of ≥87.8%, and < 87.8% were defined as LA and coagulation factor deficiency, respectively, and WaS of ≥10.2% defined progressive coagulation factor inhibitors. Sensitivity and specificity to LA were 80.8% and 93.0% for ALD50, and sensitivity and specificity to progressive coagulation factor inhibitor were 100.0% and 100.0% for WaS, respectively. The agreement between sample classification and WaS-ALD50 was 88.4% (61/69). ALD50 and WaS showed acceptable sensitivity and specificity to LA and progressive coagulation factor inhibitor, respectively. These indexes would be useful for the differentiation between LA, factor deficiency, and progressive coagulation factor inhibitor in the mixing tests.

The good, the bad and the ugly of pain in haemophilia: Recent evidence on the epidemiology, molecular mechanisms and knowledge gaps preventing optimal treatment.

Haemophilia is an inherited, X-linked blood clotting disorder caused by the deficiency of coagulation factors VIII (FVIII, haemophilia A) or IX (FIX, haemophilia B). Spontaneous bleeds are common in severe forms of haemophilia and can also occur in moderate and mild haemophilia. Severe or repeated bleeding at a joint can evolve into chronic haemophilic arthropathy, with functional damage of the joint, disability, and intense chronic articular pain. Nonetheless, acute and chronic pain may emerge due to secondary conditions related to bleedings. This narrative review aims to critically discuss the most recent evidence about pain in haemophilia to give healthcare professionals a clear picture of current knowledge hence favouring the optimisation of clinical management of pain. Extensive literature search with the terms 'hemophilia' AND 'pain', focusing on the time window 2021-2023. Acute and chronic pain is a critical aspect of haemophilia at all ages. It should be considered a multifaceted phenomenon, with a positive role as an early emergency signal of a clinical event (haemarthrosis), and numerous detrimental aspects linked to its burden that heavily affects the health-related quality of life, with psychological and social consequences. Despite its prevalence and frequency in people with haemophilia, pain is often underestimated by healthcare professionals, leading to insufficient and inadequate treatment, also due to uncertainty linked to the presence of the coagulation disorder or arthritic flares.

Surgical Treatment of Coronary Heart Disease Against the Background of Coagulopathy of Unknown Etiology. Case Report

Coronary heart disease (CHD) is one of the most common cardiovascular diseases and one of the leading causes of death in the world. Diet is the first step in the prevention of CHD. Vegetarians have significantly lower risk of death from CHD, but also have changes in the hemostasis system due to a deficiency of blood clotting factors. Cardiosurgical intervention for patients with hemostatic disorders should be performed in specialized centers with the necessary support.&#x0D; Case description. A 73-year-old patient G. was hospitalized to the National Amosov Institute of Cardiovascular Surgery with angina pectoris, weakness and edema. From the past medical history it is known that the patient has been following a diet with complete exclusion of meat and dairy products for 20 years, rarely eats fish; in addition, he smoked for 20 years at a young age. After coronary angiography, the patient developed bleeding from the oral cavity of unknown origin, which resolved on its own. According to the examination, the patient was found to have a multivessel lesion of the coronary arteries, and cardiosurgical intervention was indicated: coronary bypass surgery. At a case conference with cardiovascular surgeons, anesthesiologist and cardiologist, a decision was made to perform salvage cardiac surgery. The predicted mortality risk was 7.0% by the EuroSCORE II. The early postoperative period was complicated by bleeding with subsequent revision of the pericardium to identify the source of bleeding. Coagulopathy of unknown origin was suspected. Replacement hemostatic therapy was started, which included massive blood transfusions. The pace and level of exudation significantly decreased, the operation was completed. After surgical interventions and hemotransfusions, thromboelastography was performed and plasma hypercoagulation was detected. The patient was discharged for continued cardiopulmonary rehabilitation, and outpatient treatment was prescribed.&#x0D; Conclusions. This case report describes successful treatment of multivessel coronary artery disease by bypass grafting in a vegetarian patient with a coagulation disorder. Studies of the effects of a vegetarian diet on the hemostatic system indicate that vegetarians may have lower concentrations of hemostatic factors and that thrombin time is also prolonged due to the nutritional guidance provided by this diet. In addition, timely detection of a deficiency of blood coagulation factors in the plasma of a patient with such history can facilitate the diagnosis of the hemostasis system.

Women and girls with inherited bleeding disorders: Focus on haemophilia carriers and heavy menstrual bleeding.

Raising awareness and improving recognition, accurate classification, and enhanced access to new treatments represent current key challenges for carriers of haemophilia. Women and girls carrying genes for haemophilia often experience significant bleeding and/or low factor levels. The bleeding associated with female haemophilia is frequently overlooked, has a weak correlation with factor levels, and manifests differently than in males, with heavy menstrual bleeding being a predominant symptom. Recent changes in terminology now allow the diagnosis of haemophilia in females with low factor levels and differentiate between symptomatic and asymptomatic carriers of the gene. Observations from real-world experiences and limited clinical trial data have highlighted the positive impact of various new haemophilia treatments for women and girls with clotting factor deficiencies. There is an urgent need for initiatives that increase their access to these treatments and encourage well-designed clinical trials focusing on female-specific outcomes. In women with inherited bleeding disorders, early recognition and optimal management of heavy menstrual bleeding are crucial. However, treatment options and guidance from high-quality clinical trials are currently insufficient. Menstrual health assessment should be a regular part of monitoring women and girls with inherited bleeding disorders throughout their lives, emphasizing the importance of gathering data to improve future management.

Cost-Effectiveness Analysis of Etranacogene Dezaparvovec Versus Extended Half-Life Prophylaxis for Moderate-to-Severe Haemophilia B in Germany.

Haemophilia B is a rare genetic disease that is caused by a deficiency of coagulation factor IX (FIX) in the blood and leads to internal and external bleeding. Under the current standard of care, haemophilia is treated either prophylactically or on-demand via intravenous infusions of FIX. These treatment strategies impose a high burden on patients and health care systems as haemophilia B requires lifelong treatment, and FIX is costly. Etranacogene dezaparvovec (ED) is a gene therapy for haemophilia B that has been recently approved by the United States Food and Drug Administration and has received a recommendation for conditional marketing authorization by the European Medicines Agency. We aimed to examine the cost-effectiveness of ED versus extended half-life FIX (EHL-FIX) prophylaxis for moderate-to-severe haemophilia B from a German health care payer perspective. A microsimulation model was implemented in R. The model used data from the ED phase 3 clinical trial publication and further secondary data sources to simulate and compare patients receiving ED or EHL-FIX prophylaxis over a lifetime horizon, with the potential for ED patients to switch treatment to EHL-FIX prophylaxis when the effectiveness of ED waned. Primary outcomes of this analysis included discounted total costs, discounted quality-adjusted life years (QALYs), incremental cost-effectiveness, and the incremental net monetary benefit. The annual discount rate for costs and effects was 3%. Uncertainty was examined via probabilistic analysis and additional univariate sensitivity analyses. Probabilistic analysis indicated that patients treated with ED instead of EHL-FIX prophylaxis gained 0.50 QALYs and experienced cost savings of EUR 1,179,829 at a price of EUR 1,500,000 per ED treatment. ED was the dominant treatment strategy. At a willingness to pay of EUR 50,000/QALY, the incremental net monetary benefit amounted to EUR 1,204,840. Depending on the price, ED can save costs and improve health outcomes of haemophilia patients compared with EHL-FIX prophylaxis, making it a potentially cost-effective alternative. These results are uncertain due to a lack of evidence regarding the long-term effectiveness of ED.

Prophylactic Treatment of Children with Hemophilia in Sweden.

Hemophilia A/B are caused by deficiency or lack of coagulation factors VIII (FVIII) or factor IX (FIX), respectively, in plasma. A person with hemophilia develops bleeding in the joints and muscles at an early age, which, if left untreated, leads to early arthropathy. Preventive treatment can be achieved by regular (prophylactic) administration of FVIII/FIX. In 1958, this was implemented on a small scale in Sweden with FVIII in patients with severe hemophilia A, and in those with hemophilia B in 1972 when FIX became available. However, there were problems with human immunodeficiency virus and hepatitis infection from contaminated blood products. In the 1990s, recombinant FVIII and FIX concentrates were introduced. The major remaining problems then were the development of inhibitors, and the need for a venous route for the injections in very young children. High-titer inhibitors were treated by immune tolerance induction according to a modified model of the original Bonn high-dose protocol. A central venous line, i.e., Port-A-Cath, has enabled early prophylaxis in many children with poor venous access and has enabled the early start of home treatment with adequate injection frequency. Scoring systems for X-rays, magnetic resonance imaging, and function of joints were developed early in Sweden and have been widely disseminated worldwide, partly with modifications. Extended half-life products with half-life increased three to five times have been developed, which can provide superior bleed protection when dosed once-weekly and can maintain therapeutic trough levels when administered less frequently. The ultimate prophylaxis therapy in the future may be gene therapy.

How to investigate mild to moderate bleeding disorders and bleeding disorder of unknown cause.

A bleeding tendency is one of the most common complaints observed by hematologists. It is challenging to differentiate a clinically insignificant bleeding from a bleeding phenotype that requires hemostatic evaluation and medical intervention. A thorough review of personal and familial history, objective assessment of bleeding severity using a bleeding assessment tool, and a focused physical examination are critical to correctly identifying suspected patients with mild to moderate bleeding disorders (MBDs). A basic laboratory work-up should be performed in all patients referred for a bleeding tendency. If a hemostatic abnormality is found such as evidence of von Willebrand disease, a platelet function disorder, or a coagulation factor deficiency, more extensive testing should be performed to further characterize the bleeding disorder. Conversely, if all results are normal the patient is considered to have bleeding disorder of unknown cause (BDUC). For patients with BDUC, further evaluation may include non-routine testing to look for rare bleeding disorders not detected by routine hemostasis tests, such as thrombomodulin-associated coagulopathy, tissue factor pathway inhibitor-related bleeding disorder, hyperfibrinolytic-bleeding disorders or impaired tissue factor production. In this review, we summarize the stepwise diagnostic procedure in MBDs and provide some insights into the biological features of BDUC.

Changing hemostatic management in post-partum hemorrhage.

Early and fast assessment of hemostasis during postpartum hemorrhage (PPH) is essential to allow early characterization of coagulopathy, estimate bleeding severity and improve outcome. During PPH, fibrinogen decrease occurs earlier than other coagulation factors deficiency and hypofibrinogenemia is an early marker of PPH severity of progression. With good evidence in the context of PPH, point-of-care viscoelastic (VET) hemostatic assays have been shown to provide rapid assessment of hemostatic disorders, low fibrinogen levels, and allow VET-guided fibrinogen replacement. Further studies are needed to define the thresholds for the other coagulation parameters.

Causal Effects of COVID-19 on the Risk of Thrombosis: A Two-Sample Mendel Randomization Study.

Coronavirus disease 2019 (COVID-19) and thrombosis are linked, but the biomolecular mechanism is unclear. We aimed to investigate the causal relationship between COVID-19 and thrombotic biomarkers. We used two-sample Mendelian randomization (MR) to assess the effect of COVID-19 on 20 thrombotic biomarkers. We estimated causality using inverse variance weighting with multiplicative random effect, and performed sensitivity analysis using weighted median, MR-Egger regression and MR Pleiotropy Residual Sum and Outlier (MR-PRESSO) methods. All the results were examined by false discovery rate (FDR) with the Benjamin and Hochberg method for this correction to minimize false positives. We used R language for the analysis. All COVID-19 classes showed lower levels of tissue factor pathway inhibitor (TFPI) and interleukin-1 receptor type 1 (IL-1R1). COVID-19 significantly reduced TFPI (odds ratio [OR] = 0.639, 95% confidence interval [CI]: 0.435-0.938) and IL-1R1 (OR = 0.603, 95% CI = 0.417-0.872), nearly doubling the odds. We also found that COVID-19 lowered multiple coagulation factor deficiency protein 2 and increased C-C motif chemokine 3. Hospitalized COVID-19 cases had less plasminogen activator, tissue type (tPA) and P-selectin glycoprotein ligand 1 (PSGL-1), while severe cases had higher mean platelet volume (MPV) and lower platelet count. These changes in TFPI, tPA, IL-1R1, MPV, and platelet count suggested a higher risk of thrombosis. Decreased PSGL-1 indicated a lower risk of thrombosis. TFPI, IL-1R, and seven other indicators provide causal clues of the pathogenesis of COVID-19 and thrombosis. This study demonstrated that COVID-19 causally influences thrombosis at the biomolecular level.

ПРОЯВЛЕНИЕ ГИПЕРКОАГУЛЯЦИОННОГО СИНДРОМА У ПАЦИЕНТКИ С МНОЖЕСТВЕННОЙ МИОМОЙ МАТКИ БОЛЬШИХ РАЗМЕРОВ

When analyzing the features of the manifestation of hypercoagulation syndrome in a patient with multiple large uterine fibroids, it was revealed that in this clinical case the patient had one of the variants of the syndrome Gardner, which can be confirmed by the fact that the patient’s history revealed the presence of osteoma of the clavicle and radius, a gastric polyp, which required surgical interventions. In addition, multiple uterine fibroids, reaching large sizes in combination with the hyperplastic process of the endometrium, can also be manifestations of hyperplastic syndrome in this clinical case. The presence of hyperplastic syndrome could lead the patient to an undiagnosed state of hypercoagulation, the so-called hypercoagulation syndrome, which, even with minimal blood loss, turned into a deficiency of blood coagulation factors. As a result of this pathology, despite observing the correct technique for performing the operation, intra-abdominal bleeding occurred in the early postoperative period, due to a deficiency of blood coagulation factors. The complication was diagnosed in a timely manner, the correct management tactics were chosen, consisting of both surgical hemostasis and correction of blood coagulation factors, which contributed to the rapid relief of this complication and the timely discharge of the patient. In addition, a careful analysis of this clinical case revealed that the patient had one of the variants of Gardner's syndrome. Despite the benign course of this disease, the patient was given recommendations for further management tactics, including mandatory observation by a gastroenterologist, fibrogastrodudenoscopy and colonoscopy over time for the timely detection of polyps.

Bleeding Disorder of Unknown Cause: A Diagnosis of Exclusion.

Patients with an unexplained mild to moderate bleeding tendency are diagnosed with bleeding disorder of unknown cause (BDUC), a classification reached after ruling out other mild to moderate bleeding disorders (MBD) including von Willebrand disease (VWD), platelet function defects (PFDs), coagulation factor deficiencies (CFDs), and non-hemostatic causes for bleeding. This review outlines our diagnostic approach to BDUC, a diagnosis of exclusion, drawing on current guidelines and insights from the Vienna Bleeding Biobank (VIBB). According to guidelines, we diagnose VWD based on VWF antigen and/or activity levels ≤50 IU/dL, with repeated VWF testing if VWF levels are <80 IU/dL. This has been introduced in our clinical routine after our findings of diagnostically relevant fluctuations of VWF levels in a high proportion of MBD patients. PFDs are identified through repeated abnormalities in light transmission aggregometry (LTA), flow cytometric mepacrine fluorescence, and glycoprotein expression analysis. Nevertheless, we experience diagnostic challenges with regard to reproducibility and unspecific alterations of LTA. For factor (F) VIII and FIX deficiency, a cutoff of 50% is utilized to ensure detection of mild hemophilia A or B. We apply established cutoffs for other rare CFD being aware that these do not clearly reflect the causal role of the bleeding tendency. Investigations into very rare bleeding disorders due to hyperfibrinolysis or increase in natural anticoagulants are limited to cases with a notable family history or distinct bleeding phenotypes considering cost-effectiveness. While the pathogenesis of BDUC remains unknown, further explorations of this intriguing area may reveal new mechanisms and therapeutic targets.

Perioperative Considerations in Management of the Severely Bleeding Coagulopathic Patient

(Anesthesiology. 2023; 138:535–560) This clinical review explored coagulopathy in severely bleeding patients after major surgery (eg, cardiac surgery with cardiopulmonary bypass or CPB), serious trauma, and postpartum hemorrhage. There are a multitude of surgical and nonsurgical factors, or a mix of both, that can cause perioperative bleeding in patients. This includes fibrinolysis, metabolic acidosis, platelet impairment, thrombocytopenia, coagulation factor deficiency, activation of inflammatory pathways, hemodilution, hypothermia, and hypovolemia. Bleeding patients can often have previous hemostatic issues because of oral anticoagulants or antiplatelet therapy.

Clot Waveform Analysis: From Hypercoagulability to Hypocoagulability - A Review.

Prothrombin time (PT) and activated partial thromboplastin time (aPTT) are coagulative screening tests used for the diagnosis of several pathologic conditions, such as liver failure, coagulation factor deficiencies, anti-phospholipid antibodies (lupus anticoagulant), and factor VIII inhibitors. A new test was developed several years ago to detect the amount of thrombin generated during plasma clotting, using low tissue factor concentrations and fluorogenic substrates, and it has since been used successfully in conditions ranging from hypocoagulable to hypercoagulable states. However, the test is expensive and difficult to perform in nonspecialized laboratories, and efforts have thus been made to find an economic and easily implementable test suitable for routine use, even in nonspecialist laboratories. To evaluate clot waveform analysis (CWA) of PT and aPTT, aiming to show the dynamics of clot formation; that is, the "hidden" features of both tests. CWA can be implemented by using an automated coagulometer with dedicated software. The aim of this review was to evaluate whether CWA is able to detect both hypercoagulative and hypocoagulative states. Using MedLine, we searched and retrieved articles relating to CWA. We only considered articles published in English, but with no limits in terms of article type, publication year, or geography. CWA was shown to be a reliable test in patients with both hypercoagulable and hypocoagulable states. It represents a simple and inexpensive global test that can easily provide information on the behavior of the coagulation system. Both the first and second derivatives are computed by using dedicated software implemented with an on-board algorithm in a routine automated coagulometer.

Association Between Low Bone Mineral Density in Hemophilia Patients and Musculoskeletal Function Impairments

Background: Hemophilia A (HA) and B (HB) are inherited bleeding disorders due to the partial or total deficiency of coagulation factor (F) VIII or FIX, respectively. It is associated with low bone mineral density (BMD). Method: The current study was conducted on sixty-eight Iraqi patients with hemophilia to assess BMD using a DEXA scan enrolled from the National Center of Hematology / Mustansiriyah University from July 2020 to September 2021. Various medical history characteristics were recorded as (age, type of hemophilia, severity, number and site of bleeding, smoking, and alcohol intake) as well as Functional Independence Score in Haemophilia (FISH) was used to assess musculoskeletal functions and Hepatitis C virus (HCV)/Human immunodeficiency virus (HIV) seropositivity were recorded. Results: The results found that only 39.7% of patients have positive DEXA scans characterized by low BMD (abbreviated as DX+) and 60.3 % with normal BMD (abbreviated as DX0). The average Functional Independence (FISH) score in DX+ patients is significantly lower than those in the DX0 group, and about 33.3% of patients in the DX+ group have limitation of movement (LOM), which is significantly higher than of patients in DX0 group and 70.4% of patients in DX+ group are presented with seropositive viral infection, which is significantly higher than 39% of patients in DX0 group. Conclusion: It can be concluded that hemophilic patients with seropositive viral infection and severe LOM may be at higher risk of developing low BMD.

Combined factor VIII and IX deficiency: An exceptional case

Familial multiple coagulation factor deficiencies are a group of rare inherited disorders that are characterized by the simultaneous decrease in the levels of two or more coagulation factors. Common synchronous deficiencies are factors VIII and IX and combined deficiency of Vitamin K-dependent coagulation factors (factors II, VII, IX, and X). Here, we report a case of synchronous dual deficiency of factor VIII and IX, which is an extremely rare occurrence and no case report has been mentioned so far to the best of our knowledge. Recognizing such a dual deficiency is very important for proper management of patients.

Successful management of a pregnancy complicated by ollier disease and coagulation factors deficiency – A rare case report

Successful management of a pregnancy complicated by ollier disease and coagulation factors deficiency – A rare case report

Evaluation of the Effect of Regular Administration of Coagulant Factors on the Joint Pain Level and Dimensions of the Quality of Life of the Patients Suffering from Some Bleeding Disorders (BDs)

Background: Blood coagulation is a process that prevents blood loss. Lack of coagulation factors (inherited or acquired) disrupts this path. The prevalence of coagulant deficiency including VIII and XIII factors in Sistan and Baluchistan province is higher than in other parts of Iran. This study aimed to investigate the condition of patients suffering from some bleeding disorders (BDs) in terms of regular coagulant takes and their effects on quality of life.&#x0D; Methods: Our cross-sectional study was descriptive-analytical. Thirty-five BD patients suffering the blood coagulant deficiency enrolled, and were sorted into 5 groups.&#x0D; Results: The mean (±SD) age in the hemophilia A and B groups were (16.25 ± 8.15), and (8.14 ± 3.9), and other coagulation factor deficiencies including VWF, XIII, and IX were (15.1 ± 7.8), (15.5 ± 2.4), and IX (19.0 ± 11.5) respectively. The quality of life in group A (81.3 ± 14.9) compared to groups B (50 ± 0.25) and IX (47.5 ± 24.1) had a significant difference (p value &lt; 0.05). In addition, there was a significant difference in group XIII (79.2 ± 29.2) compared to groups B (50 ± 0.25) and IX (47.5 ± 24.1) (p value &lt; 0.05). Generally, the quality of life was higher in women (10 people) (72.5 ± 26.2) than in men (26 people) (61.1 ± 24.5). Furthermore, the Visual analog scale for pain in group A (1.3 ± 0.4) had a significant difference compared to group B (3.5 ± 0.43), IX (7.8 ± 0.9), XIII (0.4 ± 4.3) and VWF (3.2 ± 0.38) (p value &lt; 0.05).&#x0D; Conclusion: In sum, patients with hemophilia A and coagulation factor XIII deficiency had low levels of joint pain and a high level of quality of life dimensions.

Late-onset vitamin K deficiency bleeding: a preventable yet prevailing cause of intracranial hemorrhage in young infants—an ambispective descriptive study

BackgroundVitamin K deficiency bleeding (VKDB) may present as intracranial hemorrhage (ICH) in young infants, which results in severe morbidity and mortality. This study aims to determine the clinical presentation, risk factors, and outcome of children with VKDB.MethodsThis was an ambispective descriptive study conducted from January 2015 to August 2023 in southern India. Infants from 8 days to 6 months of age, diagnosed as VKDB based on bleeding with elevated PIVKA (protein induced by vitamin K absence) and prolonged prothrombin time, which is rapidly corrected 24 h after administration of vitamin K, were included. Infants with bleeding due to other causes, such as inherited clotting factor deficiency, cholestasis, and sepsis, were excluded. A structured data collection proforma was used to collect data.ResultsOut of 62 cases, 28 were excluded due to various causes, and 34 were analyzed. The median age at symptom onset was 54 days, and males were 22 (64.7%). Term gestation was 25 (73.5%), and low birth weight was 13 (38.2%), and 8 (23.5%) required NICU admission. All received vitamin K prophylaxis at birth. Thirty-three (97%) were exclusively breastfed. The most common presenting complaints were poor activity and convulsion. A total of 97% of children had ICH, and 5 (14.7%) had skin bleed. Twenty-three (67.6%) infants had complete recovery, 6 (17.6%) had sequelae (2 had hemiparesis, 2 monoparesis, and 2 quadriplegia), and 5 (14.7%) children expired.ConclusionVitamin K deficiency commonly presents as intracranial hemorrhage in infants. Exclusive breastfeeding is a significant risk factor for vitamin K deficiency, followed by low birth weight and prematurity.

Assessment of oral health awareness and hygiene practice in hemophilic patients in Indore: A cross-sectional study

: Haemophilia is a genetic X linked recessive bleeding disorder characterized by a deficiency or absence of certain clotting factors in the blood, primarily factors VIII (haemophilia A) or factor IX (haemophilia B). Dental caries and periodontal diseases are two main oral diseases affecting majority of population, but their effects are enhanced in hemophiliacs and medically compromised patients. There is a lack of epidemiological studies regarding oral health and oral hygiene of hemophilic patient especially in central India. This study therefore, aimed to evaluate the oral hygiene awareness and hygiene practices of patients with hemophilia in Indore district. This cross-sectional study was conducted on 30 hemophilic patients who were selected via snow ball sampling technique. The patients who were ready to participate voluntarily in the study were included. A study questionnaire in Hindi and English was given to the patients to obtain their knowledge about oral hygiene and dental health awareness.: Thirty hemophilic patients participated in the study with a mean age of 20 ±15 years. All the participants were male. Although hemophilic patients were conscious of oral hygiene, the study found most of them had poor dental practices. The majority of participants (56.67%) reported of using soft-bristled brushes. Only 56.67% of the participants reported cleaning their mouths after meals and having seen gum bleeding. The majority of hemophilia patients (46.15%) reported using Factor VIII, while some also used Tranexamic acid (15.39%).: Oral hygiene is crucial for everyone, but it holds particular importance for individuals with hemophilia. Good oral hygiene reduces the need for invasive dental treatments, minimizing potential complications.