Surgery in Haemophilia: experience from a centre in India

Ghosh,; Jijina,; Pathare, Anil; Mohanty,

doi:10.1046/j.1365-2516.1998.00148.x

Cited by 23 publications

(29 citation statements)

References 13 publications

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“…All our patients except one developed type I inhibitors and only one patient, who developed an inhibitor to FVIII postoperatively and then lost it, developed a type II inhibitor. As many of our patients with severe haemophilia received very little factor concentrate, concern has been voiced that these patients may develop inhibitors in their postoperative period, making this a turbulent time with attendant complications that we may be ill‐equipped to handle because of the nonavailability of FEIBA ® or other alternatives in sufficient quantities [21,22]. Although we have not encountered any such patients before, the present case of postoperative inhibitor development shows it can happen.…”

Section: Discussionmentioning

confidence: 82%

Development of inhibitors in patients with haemophilia from India

et al. 2001

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Four hundred and seven patients (352 haemophilia A and 55 haemophilia B) were investigated for the presence of factor VIII and IX inhibitors. Twenty-four out of 292 severe and two out of 36 moderate haemophilia A patients showed the presence of inhibitors. The mean age at development of inhibitors was 17.7 years (range 6-52 years). In 12 patients the inhibitors were detected due to suboptimal response to factor replacement therapy (symptomatic) and in the remaining 14 patients the inhibitors were detected during the routine screening of the patients' samples for inhibitors. They had, however, responded well to the usual doses of factor concentrates and there was no suspicion in these patients that they had developed an inhibitor (asymptomatic). There were two families in which the inhibitors were detected in more than one family member. The level of inhibitors in symptomatic patients ranged from 2.2 Bethesda units (BU) mL(-1) to 460.6 BU mL(-1), and in asymptomatic patients it ranged from 0.8 BU mL(-1) to 3.2 BU mL(-1). The inhibitors persisted in all patients except one, who developed an inhibitor postoperatively for a brief period of 3 months. All these patients were followed up from first factor exposure and were tested for inhibitors at least twice a year. The mean number of exposure days before they developed inhibitors was 47.5 exposure days (range 17-98 exposure days). No inhibitors appeared after more than 100 exposure days in any of the patients. When 50 consecutive patients were investigated for intron 22 inversions of the factor VIII gene, 17 patients were found to be positive for inversions (10 proximal inversion; seven distal inversion) out of whom four patients developed inhibitors, three patients belonging to the same family. Out of 35 haemophilia B patients, only one patient developed an inhibitor. The overall prevalence of inhibitors was thus 8.2%, which is similar to the reports from western countries, prior to the introduction of highly purified factor concentrate therapy.

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Section: Discussionmentioning

confidence: 82%

Development of inhibitors in patients with haemophilia from India

et al. 2001

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“…Briefly, the factor replacement protocol was as follows: 100% correction for the first 3–4 days followed by 50–60% for subsequent 7–8 days. If the patient needed surgical intervention then the factor replacement was given as per our protocol [5]. All the patients were put on dilantin or valproic acid as an anticonvulsant for a period of 2 years after the episode.…”

Section: Methodsmentioning

confidence: 99%

Intracranial haemorrhage in severe haemophilia: prevalence and outcome in a developing country

Ghosh¹,

Nair

Jijina

et al. 2005

Haemophilia

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Intracranial haemorrhage (ICH) is a common cause of morbidity and mortality in haemophilic patients all over the world. From 1995 to 2004, we have investigated 37 patients with 43 episodes of ICH at our Comprehensive Haemophilia Care Center from a total of 600 registered patients. Diagnosis of ICH in the patients was confirmed by clinical, haematological and computed tomographic imaging data. Three patients died despite replacement therapy while one child who had a ventriculo-atrial shunt for acute hydrocephalus also died before further intervention. One of the four patients who died also had severe aplastic anaemia for 6 years in addition to severe haemophilia. Detailed history obtained from 143 families with haemophilia attending the Genetic Diagnosis Clinic at our Center showed a positive history of cerebral bleed in 39 episodes in 37 patients. Sixteen families gave a history of death in the family of haemophilic patients due to ICH, while in the remaining 21 families, the patients had survived the episode after treatment elsewhere. However, the ICH was not confirmed by image data in these cases. The treatment protocols were also not available in these cases. Conservative factor replacement therapy 100% correction for 3 days followed by 50-60% correction for 7 days) coupled with the epsilon amino caproic acid, the antifibrinolytic agent at least for 30 days led to a mortality (10.8%) similar to that of the western countries and almost no morbidity. Surgery was not required in any of these patients except in one elderly patient with HIV infection on antiretroviral therapy.

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“…These data show that compared with the usual quantities of factor concentrates, lower doses, aimed at maintaining 20–40% levels in the first three to five post‐operative days followed by 10–20% levels during the subsequent days, can reduce factor usage to about 300 IU kg −1 per procedure even with intermittent infusion protocols [18]. These findings have been reconfirmed at a few other centres [19, S. Apte, India; T. Shamsi, Pakistan; personal communications], but they need to be more widely assessed. Our evaluation of factor replacement by continuous infusion for surgery suggests that it may not be possible to reduce the total dose any further even by maintaining 30% factor level during the first three to five post‐operative days and 20% level over the next 2–3 days followed by 10% factor level subsequently [20].…”

Section: Surgery In Haemophiliamentioning

confidence: 69%

Factor replacement therapy in haemophilia – are there models for developing countries?

Srivastava

2003

Haemophilia

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Successful models for factor replacement in severe haemophilia involves prophylactic or on-demand administration of large quantities (1500-9000 IU kg-1 year-1) of very high purity factor concentrates starting early in life. The prohibitive cost of these protocols make them completely impractical in developing countries where the quantity of factor used for replacement therapy is much lower and varies considerably (25-500 IU kg-1 year-1). At this level of treatment, as some joint damage is inevitable, the aim of therapy shifts to preventing disability and preserving reasonable joint function rather than perfect architecture. There are no carefully recorded data on long-term outcome of musculo-skeletal function on these doses. Appropriate studies are needed to document such data. With regard to products for factor replacement, economic compulsions lead to the use a variety of factor concentrates and blood-bank products for replacement therapy. Most patients in the developing world do not have access to adequate replacement therapy. Among the rest, some get limited quantities of plasma-derived concentrates while others use cryoprecipitate, fresh frozen plasma or even whole blood. Since the superiority of virus-inactivated purified factor concentrates in achieving the aims of replacement therapy is well established, the aim should be to provide this for all people with haemophilia. This can be achieved by production of such concentrates locally or by importing them. Different models are possible depending on the circumstances in each country.

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Surgery in Haemophilia: experience from a centre in India

Cited by 23 publications

References 13 publications

Development of inhibitors in patients with haemophilia from India

Development of inhibitors in patients with haemophilia from India

Intracranial haemorrhage in severe haemophilia: prevalence and outcome in a developing country

Factor replacement therapy in haemophilia – are there models for developing countries?

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