A Clinico-Pathological Study of Hemophilia in Rural Set up of Karnataka

Parthiban, Raja; Kaler, Amrit Kaur; Sangeeta, M; Hanagavadi, Suresh; Sashikala, P; Shariff, Shameem

doi:10.9734/bjmmr/2015/12810

Cited by 5 publications

(6 citation statements)

References 13 publications

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“…Most common clinical presentation in present study is haemarthrosis (72%). Haemarthrosis as most common clinical manifestation is also seen in study done by Karim et al 12 Percentage of patients of mild, moderate and severe haemophilia in this study is corresponding to study done by Sadaria 24 et al, Nikethan et al, 13 Parthiban et al, 16 Raina et al 23,[25][26][27][28] In study done by R.K.Nigam 14 et al, percentage of mild, moderate and severe was 32.38%, 36.22% and 31.4% respectively. The difference in percentage of mild, moderate and severe cases between present study and in study done by R. K. Nigam et al 14 appears due to difference in study design.…”

Section: Type Of Haemophilia Number Of Patients Percentagesupporting

confidence: 83%

Clinico-Haematological Profile of Haemophilia in Patients Attending Gandhi Medical College and Associated Hamidia Hospital, Bhopal

Nigam¹,

Sharma²,

Choudhary³

et al. 2020

jemds

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BACKGROUND Haemophilia A and haemophilia B are the commonest form of haemophilia encountered and they result from a defect in Factor VIII and Factor IX gene respectively. This hinders the process of haemostasis and predisposing haemophiliacs to spontaneous or post traumatic bleeding. We wanted to study the clinico-haematological profile of patients with haemophilia. METHODS This observational study was conducted in Gandhi Medical College and Associated Hamidia Hospital, Bhopal, during the period of March 2017 to June 2018. After clinical evaluation, patients were subjected to a battery of coagulation tests (Bleeding Time, Prothrombin Time, Activated Partial Thromboplastin Time, Correction Studies and whenever possible, Specific Coagulation Factor Assay). The results were analysed. RESULTS During the study period, 100 patients of haemophilia were studied. Majority of patients were of haemophilia A (89%). Most common age group was 6-15 years (49%) and mean age was 19.02±12.58 years. Most common age of onset was <1 year (62%). Positive family history was present in 57% of cases. 52% patients had severe haemophilia. Most common presentation was haemarthrosis & knee joint was the most common joint involved. APTT was prolonged in all cases. CONCLUSIONS Haemophiliacs are distributed worldwide and have heterogeneous presentation depending upon disease severity. Knowledge of the spectrum of presentation of haemophilia in the population helps in early diagnosis and management planning. Promotion of regular availability of factor concentrate, establishing comprehensive care center and positive public awareness along with good haematology laboratory will help in achieving outcome comparable to that of developed countries.

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Section: Type Of Haemophilia Number Of Patients Percentagesupporting

confidence: 83%

Clinico-Haematological Profile of Haemophilia in Patients Attending Gandhi Medical College and Associated Hamidia Hospital, Bhopal

Nigam¹,

Sharma²,

Choudhary³

et al. 2020

jemds

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“…Our findings of severity of haemophilia are also in correlation with Agarwal et al 13 The family history was present among more than half of the patients in this study. Similar percentage was reported by Parthiban et al, 15 in which 52.2% of the patients had family history.…”

Section: Discussionsupporting

confidence: 89%

“…Kulkarni et al states that majority (81%) of their haemophilia patients belonged to Haemophilia A and only 19% were having haemophilia B. 14 Majority of haemophilia patients in our study had severe factor deficiency, which is similar to the findings reported by Parthiban et al 15 in which both haemophilia A and B showed 66% of cases with severe factor deficiency, 26% with moderate and 8% with mild deficiency. Our findings of severity of haemophilia are also in correlation with Agarwal et al 13 The family history was present among more than half of the patients in this study.…”

Section: Discussionsupporting

confidence: 89%

Clinical Profile of Haemophilia in Children in a Tertiary Care Centre

Varghese¹,

Padmakumar²

2017

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BACKGROUNDHaemophilia A and haemophilia B are the most common and serious congenital coagulation factor deficiencies. The clinical findings in haemophilia A and haemophilia B are identical. Majority of children with haemophilia show increased bleeding tendency by around one year. The hallmark of haemophilic bleeding is haemarthrosis. MATERIALS AND METHODSThis is an observational study conducted at Government Medical College, Kottayam. Study population included all children with haemophilia less than 18 years of age attending the haemophilia clinic. RESULTSTotal 40 children with haemophilia were enrolled in the study. Factor VIII deficiency was observed in 30 (75%) patients (Haemophilia A), while 10 (25%) patients were deficient in Factor IX (Haemophilia B). Majority of haemophilia patients in our study had severe deficiency. CONCLUSIONHaemophilia A was more in our study. Bruises and ecchymoses are the most common initial presentation.

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“…In a study, out of the 50 cases diagnosed with haemophilia based on the laboratory tests, a majority of 82% were haemophilia A and rest was haemophilia B. [ 24 ]…”

Section: Discussionmentioning

confidence: 99%

“…Sixty-one out of 80 patients with Hemophila A had severe deficiency, which amounted to 76% of the total 80 whose factor levels were assessed. We also noticed severe deficiency more common than moderate and mild deficiency and the similar data was reported by Parthiban et al .,[ 24 ] Agarwal et al .,[ 26 ] Hazewinke et al . [ 28 ] in South Africa, Kim et al .…”

Section: Discussionmentioning

confidence: 99%

Rare but not Abdicated

Rathaur

Vigneshwar

Imran

et al. 2021

Journal of Family Medicine and Primary Care

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Background: Haemophilia is one of the bleeding disorders, which is inherited, in an xlinked recessive pattern. The diagnosis is by estimation of factor levels of 8 and 9. Timebound treatment for people living with Haemophilia (PWH) is factor replacement during bleeding manifestation. The prevalence of Haemophilia was mostly underestimated, and it is more so in hilly terrains like the state of Uttarakhand. Materials and Method: This is a crosssectional study by compiling the data of PWH visiting the tertiary care centre for Haemophilia in Uttarakhand. We collected data from the patients with bleeding disorder reporting to the Haemophilia centre from July 2017 to December 2018. In this manuscript, we try to describe the pattern of Haemophilia and the degree of severity and incidence of inhibitors among the sample population of PWH who represent the population of Uttarakhand. The magnitude of problems faced by PWH from this hilly terrain to assess basic treatment in case of emergency is also being depicted. Result: We reported Haemophilia A contributing about 80% of the PWH in our centre. Average distance a PWH has to travel to obtain treatment was about 131.5 km (SD ± 83.7 km). Incidence of inhibitors was about 5%. Conclusion: We infer from our study that Hemophilia A is more common than Hemophilia B. Through this manuscript we hope to spread awareness of the Haemophilia care that is ongoing, the role of prophylaxis therapy and the future role of primary care physicians that may change the care of PWH in future.

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A Clinico-Pathological Study of Hemophilia in Rural Set up of Karnataka

Cited by 5 publications

References 13 publications

Clinico-Haematological Profile of Haemophilia in Patients Attending Gandhi Medical College and Associated Hamidia Hospital, Bhopal

Clinico-Haematological Profile of Haemophilia in Patients Attending Gandhi Medical College and Associated Hamidia Hospital, Bhopal

Clinical Profile of Haemophilia in Children in a Tertiary Care Centre

Rare but not Abdicated

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