Objectives To examine the relationship between 12 macro-and micro-nutrients and the risk of recurrent calcium stone formation by comparing the diets of a large outpatient clinic-based group of patients who had formed calcium-based urinary tract calculi with that of a population-based control group matched for age, gender and body mass index. Patients, subjects and methods The dietary intake of 500 patients (cases) randomly selected from the adult population attending an outpatient renal-stone clinic and being evaluated and/or treated for biochemically or radiologically diagnosed calcium-based upper urinary tract calculi were compared with those of 500 control subjects selected to match for age, sex and body mass index from a strati®ed probability sample of 2212 adults (not institutionalized) living in the same geographical area. Results Comparing the mean nutritional intakes showed a statistically higher consumption of energy, carbohydrates, sodium, ®bre, vitamin C, fat and folic acid among cases than in controls. The intake of calcium, alcohol and vitamin A was signi®cantly higher among the controls. There were no signi®cant differences in the intake of protein, niacin or iron. The results of these comparisons varied when the groups were strati®ed by sex, age and body mass index. Conclusions Dietary risk factors for calcium-based urinary tract calculi are many and complex, and a detailed consideration of sex, age and body mass index is important in interpreting such data. While it is dif®cult to draw ®rm conclusions about causes and effects of individual nutrients from the available data, this study indicates a possibly more important role for dietary fat in stone formation than has been previously recognized. This relationship needs to be further explored in relation to urinary risk factors, as it may be possible to advise patients to reduce dietary fat as a prophylactic measure for stone formation. As dietary fat has been associated with cardiovascular diseases and possibly cancer, an overall recommendation to these patients for a low dietary fat intake may be easier to follow.

Hepatic veno-occlusive disease (VOD) is one of the most common and important regimen-related toxicities observed after hematopoietic stem cell transplantation (HSCT). There are no universally accepted preventative or therapeutic approaches for VOD. We prospectively evaluated the safety and efficacy of a short course of methylprednisolone (MP) in 48 patients undergoing allogeneic HSCT who were diagnosed with hepatic VOD. MP was administered at a dose of 0.5 mg/kg i.v. every 12 h for a total of 14 doses, and then discontinued without taper. Thirty (63%) patients responded with a reduction in total serum bilirubin of 50% or more after 10 days of treatment. In univariate analysis, non-responders had a higher total bilirubin at the start of MP therapy, more weight gain, evidence of fungal infection and platelet refractoriness. High SGPT and early engraftment were significant factors among responders. Twenty-five of the 30 responders survived up to day þ 100, whereas all but three non-responders died within 100 days post-HSCT, for a probability of survival of 58% among responders and 10% for non-responders. Prospective comparative studies are needed to confirm the observed encouraging outcome of MP therapy for VOD.

Different types of mutations have been reported in patients with hemophilia A. Although about half of all severe factor VIII deficiencies are caused by gene rearrangements (inversions) involving intron 22 in F8, other mutations such as point mutation, large deletions and insertions had been reported. We report the result of the first molecular testing for or F8 mutations from Saudi Arabia. A cohort of 22 men with hemophilia A was studied for F8 mutations. All patients were tested for factor VIII coagulant activity and inhibitors. Peripheral blood samples were used for DNA extraction followed by PCR detection of intron 22 inversion and all samples tested negative were screened for other F8 mutations. The patient's age ranged between 4 and 37 years. All patients except two siblings had severe hemophilia A. Only two patients out of 22 developed inhibitors with no obvious relation to the genotype. F8 Intron 22 inversion was detected in 10 patients (50%) of severe cases. Additionally, five point mutations and one deletion/insertion involving different exons were detected. All identified mutations were associated with severe phenotype except for one, which was associated with mild phenotype of hemophilia. This is the first report of molecular genotype of hemophilia A in the Saudi population and one of the few for Arab population. We had confirmed the incidence of Inversion 22 in severe hemophilia. We are reporting two novel mutations in F8, which can be used for carrier detection and prenatal genetic diagnosis (PGD).

These results suggest that the MEA is sensitive for the detection of Glanzmann thrombasthenia. Furthermore, MEA may also be able to differentiate between the subtypes of Glanzmann thrombasthenia.

Introduction: The use of a Myeloablative (MA) regimen followed bypost-transplantation high dose Cyclophosphamide (PT-CY) has been adopted to overcome the increased relapse risk following nonmyeloablative conditioning regimen and unmanipulated Haploidentical Bone Marrow Transplantation, in patients with high-risk hematological malignancies, with acceptable TRM and risk of GVHD. We added ATG to our Myeloablative regimen with PT-CY after noticing significant incidence of grade II-IV aGVHD with the first few cases. Here we compare the outcomes of the 12 patients who received ATG to the outcomes of the first 11 patients who were transplanted without ATG. Patients and Methods: our haploidentical program was started in 2013 as a phase I/II prospective clinical trial. After reviewing the first 11 cases enrolled on the trial (MA regimen with PT-CY, without ATG), we noticed significant incidence of high grade aGVHD (54.55%). We amended our protocol in early 2015. Rabbit ATG was added at a dose of 3 mg/kg (1.5 mg/kg day -3 and day -2) to our conditioning regimen [thiotepa (5mg/kg/day on day -8 and -7), busulfan (3.2mg/kg/day IV day-6, -5 and -4), fludarabine (50mg/m2/day on day -6, -5 and -4 )] or [TBI 1000 cGy (200cGy twice a day on days -10, -9 and one dose on day -8), fludarabine (30mg/m2/day on Days -7, -6, -5 and -4)]. Graft-versus-host disease (GVHD) prophylaxis consisted of PT-CY (50mg/kg/day) on days +3 and +5, cyclosporine (starting day +4), and mycophenolate mofetil (starting day +1). Table 1 summarizes the disease-type and disease-status at transplant. Patient characteristics were comparable at baseline between the 2 groups. Results (Table 2): We enrolled patients with high risk hematologic malignancies in need for SCT but have no matched donor (MSD, MUD). Despite the small sample size, our results showed a statistically significant difference in the rate of acute GVHD between the 2 groups (p= 0.0161) in favor of the ATG group, chronic GVHD was more frequent in the non-ATG group however the difference did not reach statistical significance probably due to the small sample size. There was no statistically-significant difference in the risk of relapse, CMV reactivation or Hemorrhagic cystitis between the 2 groups. However, there was a trend of higher relapse rate (33.3% vs 18.18%), a higher rate of Hemorrhagic cystitis (50% vs 18.18%) and a higher rate of CMV reactivation (100% vs 81.82%) in the ATG group. The cumulative incidence of TRM at day 100 was in favor of the ATG group (figure 1), with a trend toward a better DFS in these patients 6 months post-transplant (figure 2). To be noted the follow up period was shorter for the ATG group because ATG was added later on. Figure 3 shows the survival for ATG vs non ATG group. Conclusion: The use of ATG with myeloablative Haplo conditioning can significantly reduce the risk of acute GVHD and early TRM. We have seen more relapses, higher rate of CMV reactivation, and hemorrhagic cystitis with the addition of ATG but these did not reach statistical significance probably due to the small sample size. A lower dose of ATG might be the way to go to strike a careful balance and improve the outcomes of myeloablative haploidentical transplant. Disclosures No relevant conflicts of interest to declare.