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1.
Pakistan Journal of Medical Sciences. 2009; 25 (1): 74-78
in English | IMEMR | ID: emr-92377

ABSTRACT

Hydroxyurea has been used in thatassemia major [T.M.] and thalassemia intermedia patients since 1994 with some success in different centers. The objective was to evaluate the effect and possible side effects of hydroxyurea in these patients. This was a descriptive study done in 2007 after nine years of initiation of Hydroxyurea. Medical records of 1050 patients were reviewed. Patients who had received hydroxyurea for at Least three continuous months were enrolled into the study. Clinical and laboratory data during six months before and six month after starting hydroxyurea were compared. Two hundred ninety seven patients were enrolled of these two hundred forty eight [83.8%] were thalassemia major and forty eight [16.2%] were thatassemia intermedia. Dose of hydroxyurea was 15.5 +/- 6.4mg/kg /day and duration was 5.2 +/- 2 years [ranging 0.5-9 years]. Transfusions were completely stopped in one hundred eleven [44.7%] of thalassemia major patients with a mean Hb of l0g/dl. After 22.5 +/- 18 months, using Desferal was stopped in sixty six [26.6%] patients. The reasons for stopping hydroxiurea were "ineffectiveness" in 20%, "poor compliance" in 12.4% and side effects in 13.4% of patients. The side effects were nausea, palpitation, transient leucopenia and transient raising of creatinin. Hydroxyurea is effective and safe in thalassemia major and thalassemia intermedia patients and saves them from side effects of blood transfusions


Subject(s)
Humans , Male , Female , beta-Thalassemia/epidemiology , beta-Thalassemia , Thalassemia , Hydroxyurea , Hydroxyurea/administration & dosage , Blood Transfusion/adverse effects , Hematologic Diseases/genetics , Hematologic Diseases/drug therapy
2.
Saudi Medical Journal. 2009; 30 (6): 835-839
in English | IMEMR | ID: emr-92756

ABSTRACT

To investigate the knowledge, attitude, and practice of reproductive behavior in Iranian minor thalassemia couples in Ghaemshahr City, Mazandaran, Iran. This is a cross-sectional descriptive survey conducted in 2006. Birth rates from 1997-2005 and the number of newly registered patients from at risk couples was recorded. Tools for data collection were a valid questionnaire containing epidemiologic characteristics of couples, knowledge [20 questions], attitude 20 statements, and practice by studying the family file in health centers. Questionnaires were completed by husband and wife separately. Actual versus expected numbers of patients born in that period were compared. The data were analyzed using the Statistical Package for Social Science version 13.00, and p<0.05 was interpreted as significant. Of the 240 at risk couples, 100 were studied. Of them, 82% had good knowledge of thalassemia, and 68.5% had a positive attitude toward thalassemia prevention program. Correlations of knowledge with attitude were significant [p<0.001], and 50% of the couples had unfavorable practice including unplanned pregnancy, fetal abortion without prenatal diagnosis [PND], delivery without PND, and having a child affected by thalassemia major [TM]. Without PND, 4 TM patients were born. Ninety-eight episodes of unfavorable practice were reported. Meanwhile, the contraceptive method used by 12% of couples was unsafe. Suspected TM patients with no prevention program were 25; thus, the birth of 2 TM was prevented [92% reduction]. We achieved great success during the last 9 years in the region, and TM prevention program improved knowledge, attitude, and practice in high-risk couples and carrier families


Subject(s)
Humans , Male , Female , Reproductive Behavior , beta-Thalassemia , Family Characteristics , Cross-Sectional Studies
3.
Iranian Journal of Pediatrics. 2009; 19 (1): 5-10
in English | IMEMR | ID: emr-91411

ABSTRACT

Nocturnal enuresis is a common diagnosis in patients referred to pediatric and pediatric nephrology clinics. Nocturnal polyuria is an important patho-physiologic factor in enuresis. Hypercalciuria, with altering concentrating capacity of the kidneys, can affect children's response to desmopressin. This is a double blind clinical trial starting September 2007 to March 2008. One hundred and twenty four enuretic children, 76 [61.3%] males, 48 [38.7%] females, mean age 7.7 [ +/- 1.7], were evaluated by measuring random morning urinary calcium to creatinine ratio. Patients were divided into group 1 with a calcium to creatinine ratio equal to or more than 0.2 mg/mg, and group 2 with a ratio less than 0.2 [Hypercalciuric and non hypercalciuric respectively]. All patients received 10 to 40 mcg of nasal desmopressin at bed time. The response was defined as reduction in wet nights, a "full response" [greater than 90% reduction], "partial response" [50% to 90% reduction] and "no response" [less than 50% reduction]. Chi-square method was used to compare the responses and P<0.05 was considered statistically significant. Nineteen patients in group 1 [Hypercalciuric] and 105 patients in group 2 [Non hypercalciuric] were studied. Response to desmopressin was "full" in 47.4% in group 1 and 64.8% in group 2. 42.1% and 26.7% had "Partial response" in group1 and 2 respectively [P < 0.04]. Hypercalciuria can affect negatively the responsiveness to desmopressin therapy


Subject(s)
Humans , Male , Female , Nocturnal Enuresis/physiopathology , Hypercalcemia/diagnosis , Hypercalcemia/drug therapy , Deamino Arginine Vasopressin/administration & dosage , Deamino Arginine Vasopressin , Creatinine/urine , Creatinine , Chi-Square Distribution , Treatment Outcome
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