A clinical profile of hemophilia patients and assessment of their quality of life in Western Uttar Pradesh, India: An observational study

Mishra, Saurabh; Kumar, Sanjeev; Panwar, Ajay; Bhagchandani, Deepak; Aneja, Girish Kumar; Verma, Neetu; Kumar, Pradeep

doi:10.4103/0975-2870.182501

Cited by 13 publications

(4 citation statements)

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“…A total of 80.20%, meaning 81 patients, had severe hemophilia A, data which are similar to those found in the international literature [7][8][9]; 17.82% had moderate hemophilia, and only 1.98%, 2 patients, were mild cases.…”

Section: Resultssupporting

confidence: 86%

A Perspective on the Quality of Life of Hemophilia A Patients in Romania—A Study on 100 Patients

Guran

Ioniţă

Haţegan

et al. 2022

JVD

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Hemophilia A is an X-linked coagulopathy, where there is a deficit in the production of the coagulation factor VIII. Even though there is a higher incidence of hemophilia A than of hemophilia B, it is still considered a rare disease, as its incidence is of 1 in 10,000 people born. We have applied three questionnaires regarding quality of life: Haem-A-QoL, Haemo-SYM and EQ-5D-5L to 101 adult patients with hemophilia A, which were separated into two groups: on-demand and prophylactic treatment. The results showed a relatively young lot, with medium and high education, but with a sedentary lifestyle and are pensioners. They also seem to have moderate mobility issues but, overall, a good quality of life. The quality of life in our studied lot is relatively good, but a more diverse lot is advised.

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Section: Resultssupporting

confidence: 86%

A Perspective on the Quality of Life of Hemophilia A Patients in Romania—A Study on 100 Patients

Guran

Ioniţă

Haţegan

et al. 2022

JVD

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“…The majority of PwH in our study who had affected joints (70.18%) had the knee as a target joint, followed by the elbow (19.30%) and the ankle (10.53%). This is a higher proportion than reported in the observational study of 71 PwH in Western Uttar Pradesh by Mishra et al, who found the knee as the target joint among 57.1% of participants [23] . Of the four PwH with inhibitors in our study, two had both the ankle and the elbow as target joints, and one had the knee, ankle and elbow as target joints.…”

Section: Discussioncontrasting

confidence: 54%

Clinical and radiological assessment of joints in people with haemophilia in Assam, Northeast India: a cross-sectional study

Dhal,

Dutta,

Das

et al. 2023

The Journal of Haemophilia Practice

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Background Despite the availability of factor replacement therapy, including prophylaxis, to treat and prevent bleeding, haemophilic arthropathy continues to be the most common complication of haemophilia and significantly impairs the quality of life in people with haemophilia (PwH). Regular periodic assessment of joint status in PwH is essential to identify early arthropathic changes and prevent the development or progression of haemophilic arthropathy. Kinematic and kinetic assessment are preferable and MRI is the gold standard for diagnosing haemophilic arthropathy, but availability is limited in developing countries. HJHS and ultrasound in conjunction with HEAD-US have been shown to effective in assessing changes indicating arthropathy. Aim This study is designed to identify which radiological assessment tool(s) works best when integrated with clinical assessment tools to examine joint health in PwH, with the aim of establishing a cheap, effective, multimodal approach to joint assessment in clinical practice in low resource settings which can then help to guide treatment. Methods A cross-sectional observational study was carried out among PwH attending a clinic in the department of medicine at Assam Medical College and Hospital, Northeast India, over a one-year time period. The elbow, knee and ankle joints of participants were assessed clinically and functionally using HJHS and FISH and radiologically by Pettersson (X-ray) and HEAD-US (ultrasound) scoring. The resulting data was analysed statistically to ascertain correlation between these parameters. Results Sixty-seven PwH were enrolled in the study, with a mean age of 21.69±8.24 years (median 21 years); 38 (57.72%) had severe factor deficiency. The majority (71.70%) presented with joint bleeds; 40 (59.70%) had the knee as a target joint, followed by the elbow (11; 16.41%) and ankle (6; 8.95%). Mean ABR (annual bleed rate), AJBR (annual joint bleed rate) and ATJBR (annual target joint bleed rate) values had a positive correlation with increased severity of factor deficiency (p <0.001). FISH score increased with factor activity level; HJHS, HEAD-US and Pettersson scores decreased with factor activity level. Significant positive correlation was established between Pettersson score, age and ABR. The degree of association was greatest between HJHS and HEAD-US at 60.62%; between HJHS and Pettersson it was 57.74%. Statistically significant negative correlation was established between FISH and HEAD-US. Conclusion A combined multimodal approach using the HJHS, FISH and HEAD-US scoring systems can provide a cheap, quick, more reliable and accurate option for assessing haemophilic joints, with minimal inter and intra observer reliability. This can support the optimisation of management of joint damage in PwH in resource-constrained settings.

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“…In our study, 58.33% of the patients had only one target joint while 41.67% of patients had two or more target joints. Mishra et al [28] found that the most common target joint was the knee (57.1% of cases). About 76% of patients had swollen joints resulting in restricted joint mobility.…”

Section: Discussionmentioning

confidence: 99%

Association between the levels of serum vitamin D and trace elements and joint health in children with hemophilia

Mahmoud,

Sharaf,

Bayomy

et al. 2023

Voprosy gematologii/onkologii i immunopatologii v pediatrii

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Hemophilia is defined as X-linked recessive bleeding disorder. Recurrent bleeding episodes lead to hemarthrosis.Objectives: to investigate the levels of serum 25(OH) D and trace elements in children with hemophilia A and B and to identify the possible association of these factors with Hemophilia Joint Health Score (HJHS). This case-control study was conducted among children with hemophilia A and B. A total of 48 cases were recruited from the hematology units at the Menoufia University Hospital (n = 36) and Sohag University Hospital (n = 12) from December 2020 to February 2022. Forty healthy controls were matched to cases on age, sex and socioeconomic status. Serum zinc and magnesium levels in the hemophilia patients were significantly lower than in the controls, while serum alkaline phosphatase levels in the cases were significantly higher than in the controls. Informed consent was obtained from all the children's parents and ethical approval was acquired from the ethical committee (ID: 5/2020PEDI38), Faculty of Medicine, Menoufia University. The levels of phosphorus and calcium were the same in two groups. Serum 25(OH) D levels were deficient in 85.4% of the cases and insufficient in 14.6%. None of the hemophilia patients had sufficient levels of serum 25(OH) D. There was no significant correlation between HJHS and the levels of serum trace elements but there was a significant positive correlation between HJHS and annualized bleeding rate and a significant negative correlation between HJHS and serum vitamin D. There was no significant difference regarding the demographic data except for weight and body mass index. The patients had significantly higher weight and body mass index compared to the control group. The levels of serum vitamin D and trace elements were decreased in hemophilia patients, and these low values were associated with the worst joint health.

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A clinical profile of hemophilia patients and assessment of their quality of life in Western Uttar Pradesh, India: An observational study

Cited by 13 publications

References 1 publication

A Perspective on the Quality of Life of Hemophilia A Patients in Romania—A Study on 100 Patients

A Perspective on the Quality of Life of Hemophilia A Patients in Romania—A Study on 100 Patients

Clinical and radiological assessment of joints in people with haemophilia in Assam, Northeast India: a cross-sectional study

Association between the levels of serum vitamin D and trace elements and joint health in children with hemophilia

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