Deferiprone (L1) as an adjuvant therapy for Plasmodium falciparum malaria.

BACKGROUND & OBJECTIVES: Mortality due to Plasmodium falciparum infection remains high in India, hence any modality of treatment which can improve the outcome of this disease is worth exploring. The present study was undertaken to see whether addition of an oral iron chelator, deferiprone (L1) to the conventional treatment regime for P. falciparum infection improves the clinical course and final outcome. METHODS: In this prospective, randomised double blind trial, 45 consecutive patients with P. falciparum infection were randomised into two groups. Patients in Group I (control group, 21 patients) received standard quinine and doxycycline therapy along with supportive therapy and placebo capsules for 10 days. Patients in Group II (24 patients) received the same treatment as Group I but in place of placebo capsule received deferiprone capsules 75 mg/kg/day in 12 hourly divided doses. The parameters evaluated included the time taken in resolution of parasitaemia, fever and coma, differences in final outcome i.e., death or other severe complications, and side effects and deferiprone tolerance. RESULTS: Four patients in Group I and two in Group II died (P > 0.05). The resolution of fever and coma was significantly faster in Group II (P < 0.05) and parasitaemia cleared 24 h earlier in this Group. The drug was well tolerated and had no side effects. INTERPRETATION & CONCLUSION: Deferiprone (L1) seems to be a promising agent as an adjuvant in the treatment for severe P. falciparum malaria infection.

Transfusion transmitted diseases in haemophilics from western India.

BACKGROUND & OBJECTIVES: Transfusion related human immunodeficiency virus (HIV), hepatitis B virus (HBV) and hepatitis C virus (HCV) infections have been a major cause for morbidity and mortality in the haemophilic population in the west. The prevalence of these markers of transfusion transmitted viral diseases in severe and moderate haemophilia patients was studied. METHODS: The seropositivity for these viral markers was evaluated in 400 haemophilics (323 severe and 77 moderate) in a 5-year survey starting from 1995. First 188 of these patients were also tested for HCV. Serological tests for HIV, HBsAg and HCV were done by third generation ELISA; positive samples were also confirmed by Western blot. RESULTS: Fifteen of the 400 patients were found to be HIV positive (3.8%), 24/400 were HBsAg positive (6%) and 45/188 (23.9%) were positive for HCV (28 for both non-structural and core antigen, 13 for core only and 4 for non-structural antigen only). The lowest age of HIV positivity was 12 yr and that of HCV positivity was 8 yr. INTERPRETATION & CONCLUSION: The above study shows a reduction in blood product related HIV transmission in severe and moderately affected haemophilics but more stringent policy for blood product usage, universal hepatitis C screening, hepatitis B vaccination and continuous awareness programmes for medical staff, general public and patients is needed to reduce the incidence of these diseases in haemophilics.

Evaluation of cardiac function in iron deficiency anemia before and after total dose iron therapy.

OBJECTIVE: To evaluate the cardiac function before and after the total dose iron therapy (TDI) and to correlate the myocardial function to the rise in haemoglobin after TDI in patients of iron deficiency anemia. METHODS: The study included 30 patients of iron deficiency anemia who presented to our institution in the last one year. There were 11 men and 19 women with the mean age of 30 years. Parameters compared before and after TDI infusion included clinical features, haemoglobin, electrocardiogram (ECG), treadmill stress test (TST) and 2 dimensional echocardiogram (2D echo). RESULTS: During the study period 30 patients (11 men and 19 women) were included for TDI. The mean haemoglobin level increased from 5 gm/dl to 5.7 gm/dl 4 days after TDI. The congestive cardiac failure disappeared in four out of eight patients after TDI. The mean heart rate on the ECG pretherapy was 102.66 +/- 14.9 and post therapy 93.4 +/- 14.9 (p = 0.011). The TST results showed improvement in effort tolerance in 17 out of 24 patients (p = 0.0012) and it improved much before there was a significant rise in haemoglobin. CONCLUSION: Impaired ventricular performance is observed in patients with iron deficiency anemia. After TDI the left ventricular function improved before there was a significant rise in haemoglobin level proving the theory that correction of the electrophysiological abnormalities of the heart in iron deficiency patient by TDI may be the result of correction of iron at the tissue level.

Pancreatic lymphoma masquerading as adenocarcinoma.

Primary pancreatic lymphoma is rare. We report a young man with primary lymphoma in the pancreatic head who presented with obstructive jaundice.

Spontaneous isolated lesser sac hematoma in a patient with hemophilia.

In patients with hemophilia, hematomas in the mesentery and bowel wall have been described uncommonly. The lesser sac is a rare site of spontaneous hemorrhage even in patients with bleeding diathesis; only a single case of isolated lesser sac hemorrhage has been reported in a hemophiliac patient. We report a similar case with no history of trauma. He recovered with administration of factor VIII concentrate.

Prenatal diagnosis of haemophilia: a preliminary report.

BACKGROUND: Haemophilia A is the most common congenital bleeding disorder seen in man, affecting one in 5000 to 10,000 males. Because of the large size and heterogeneity of mutations in the factor VIII gene, direct detection of mutations is not practically feasible, except the recently detected intron 22 inversions. Hence, the indirect method of gene tracking using various polymorphic markers is the method of choice. Using this approach, we have performed antenatal diagnosis in four haemophilia A families. METHODS: The four families included 21 subjects who were used for gene-tracking analysis. Two families had a positive history with more than one member affected, while the remaining two families had a negative history with only one affected son. In all four families, the propositi and their affected relatives had severe haemophilia A with factor VIII:C less than 1%. All were negative for inhibitors. The polymorphic markers used were IVS 18 Bcl I, IVS 19 Hind III and the extragenic DXS 52 St 14 of the factor VIII gene. Prior to polymorphism analysis, the sex of the foetus was determined using Y chromosome-specific primers. All the analyses were carried out by polymerase chain reaction. RESULTS: Antenatal diagnosis in the four families showed three normal male foetuses and one normal female foetus. Two families provided evidence with only IVS 18 Bcl I and St 14 markers. One family provided information with only intron 19 Hind III marker. The fourth family provided information with all three markers. The coagulation parameters were almost in agreement with the results of DNA analysis. CONCLUSION: All three polymorphic markers yielded information. This suggests that these three markers can be effectively used in the antenatal diagnosis of haemophilia A in Indian families.

Plasma cell leukaemia--a report of two cases.

Two cases of plasma cell leukaemia--a rare form of leukaemia are described. Both cases presented with anaemia and hepatosplenomegaly. Investigations revealed leucocytosis with increased plasma cells (> 20%). Skeletal survey revealed a few osteolytic lesions in both cases.

Diabetes in acromegaly--a study of 34 cases.

The incidence and severity of diabetes mellitus (DM) in 34 patients with acromegaly were studied. The incidence was higher (55.9%) than any other series reported. Nineteen had DM, of which 10 were symptomatic. Only one had diabetic retinopathy. Glucose tolerance was improved after successful treatment of acromegaly.

Hemostasis in acquired hemophilia--role of intracavitary instillation of EACA.

An 82 year old man developed antibodies against coagulation factor VIII:C without any apparent cause. Bleeding from the soft tissue cavity could not be controlled by factor VIII:C concentrates, immunosuppression with steroids and intravenous immunoglobulin therapy in the standard dosages. However, a single injection of Epsilon Aminocaproic Acid (EACA) instilled into the cavity under aseptic precautions achieved lasting hemostasis with resultant wound healing.

Effect of low dose recombinant human omega erythropoietin (rHuEPO) on anaemia in patients on hemodialysis.

The effect of low dose rHuEPO therapy in ESRD patients on regular dialysis therapy was assessed in a prospective study in 22 patients. Routine hematological and biochemical tests, bone marrow aspiration, serum iron and ferritin studies were performed. The quality of life was also assessed. rHuEPO was administered in a dose of 25 units/kg i.v. post dialysis 3 times a week for 8 weeks, followed by 36 units/kg for further 4 weeks. Significant rise (p = 0.0001) in Hb & PCV with rise in reticulocyte count (0.016) was noted. Serum ferritin was a better index of iron status of the body. Significantly improved anemia and quality of life of ESRD patients on hemodialysis was seen in 95% of the patients.

Outbreak of kala-azar in Bombay.

A chance diagnosis of kala-azar in a patient referred from Acworth Leprosy Home in Bombay was followed up, resulting in an investigation of a total of 25 patients (inpatients and residents) for the presence of the disease. 30.3% of the patients investigated were found to be suffering from the disease. This confirms the earlier suspicion that Bombay and especially the Acworth Leprosy Home is an endemic area for kala-azar.

Use of aminocaproic acid (ACA) in extra-amniotic MTP in patients on anti-coagulant therapy.

A case of rheumatic heart disease (RHD) with prosthetic mitral valve endocarditis receiving anticoagulation with heparin, underwent medical termination of pregnancy in a second trimester. The following report entails the use of aminocaproic acid (ACA) in preventing excessive bleeding during and after the procedure, while the patient continued to receive anticoagulant therapy.

Hereditary spherocytosis: experience of 145 cases.

145 patients were diagnosed to have hereditary spherocytosis (HS) over a period of 25 years. Jaundice (66.9%), fever (65.5%), weakness (44.8%), and abdominal pain (35.8%) were the commonest complaints. 94.5% had splenomegaly (JP-17 cm) and 71.7% had hepatomegaly (JP-6 cm). Spherocytes were detected in the peripheral smears of all patients at presentation on careful examination. 67 patients had been investigated elsewhere and spherocytes missed in 86.6%. Gall stones were seen in 20 of the 54 patients investigated. Family history suggestive of HS was available in only 16.6% of cases, whereas examination and investigations revealed HS in almost all families. Splenectomy was done in all symptomatic patients. In the 39 patients followed up for 1-9 years after splenectomy.