Pharmacological agents such as hydroxyurea (HU) have been known to cause induction of fetal hemoglobin and possibly may alleviate the symptoms in thalassemia intermedia patients. Thirty-seven patients with beta-thalassemia intermedia were enrolled to assess response to HU therapy. Major response was defined as transfusion independence or hemoglobin rise of more than 20 g/l and minor response as rise in hemoglobin of 10-20 g/l or reduction in transfusion frequency by 50%. The median age was 10 years (range: 4-50 years) and median follow-up was 12 months (range: 4-36 months). Twenty-six patients (70.2%) showed response to HU therapy. Seventeen patients (45.9%) were major responders, and nine patients (24.3%) showed minor response. There was no correlation of response with beta-thalassemia mutation or XmnI polymorphism; however, the presence of alpha(3.7) deletion was associated with major response in three patients. Mean fetal hemoglobin (HbF) levels rose on HU therapy. Older age, low baseline F cell percent, and low baseline HbF levels (below 10%) were predictors of poor response. Response was evident within 1 month of starting HU therapy in the majority of responders. Thus, a short trial of HU therapy can predict durable response.

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A 25-year autopsy study (1972-1997) of acute poisoning deaths from a tertiary care hospital in northern India (Postgraduate Institute of Medical Education and Research, Chandigarh) revealed a steep increase in the incidence of acute poisoning since 1987. The majority (68%) of subjects were between the ages of 14 and 30 years, and there was a male preponderance (69%). The main victims were students and unemployed youths, followed by agricultural workers and domestic workers. The proportion of urban victims increased from 45% in the period from 1972 to 1977 to 72% in the period from 1992 to 1997. The proportion of suicidal deaths increased from 34% in the period from 1972 to 1977 to 77% in the period from 1992 to 1997, whereas accidental deaths decreased from 63% to 17% in the same period. Barbiturates (37%) and copper sulfate (22%) were the most common poisons causing mortality between 1972 and 1977; organophosphates (46%) became the most common between 1977 and 1982. Since 1982, aluminum phosphide (65%) has been the most common poison.

IntroductionInherited bleeding disorders are characterized by the absence or reduced level of clotting factors, and the clinical manifestations vary according to the type and magnitude of the deficient factor.AimTo study the clinical presentation of the rare inherited coagulation factor disorders in a tertiary care hospital and to compare the data from those reported in other populations.MethodsSixty-seven patients, who presented to the Department of Hematology, All India Institute of Medical Sciences, New Delhi, were evaluated retrospectively from 2005 to 2011. The tests performed included platelet count, prothrombin time (PT), activated partial thromboplastin time (aPTT), thrombin time (TT), factors assay and clot solubility test in 5 M urea. Factor XI assays were aPTT based while factors V, VII and X assays were PT based.ResultsMale to female ratio was 2:1. The median age of onset of the first episode of bleeding was at 6 months (range, from birth to 20 years) whereas the median age of presentation to our hospital was 9 years (range, 2 months to 54 years). The most common deficient factor was factor X (43%), followed by factor XIII (27%) and factor VII (10%).ConclusionThere is a wide gap between the initial manifestation of the bleeding disorders and first presentation to the tertiary care hospital for assessment and treatment. Factor X deficiency is the most common among these rare coagulation disorders in our population, whereas factor VII deficiency is more common in Iranian and North American population.

Inherited factor VII deficiency is the commonest autosomally inherited factor deficiency with marked variation in the age of presentation and clinical symptoms. The laboratory results in form of PT and factor VII levels do not correlate with the severity of clinical presentation. A comprehensive evaluation to exclude acquired causes of factor VII deficiency, e.g. obesity, liver diseases, vitamin K deficiency and acquired inhibitors is required before labeling it as inherited in the absence of family history and molecular studies.