[The tale of two stones in an obstructed partial duplex kidney: case presentation].

Duplex renal systems is a common anomalies. Incidence rate of 0.8% in healthy adult population and 2-4% in patients investigated for urinary tract symptoms. Urolithiasis management for patients with anomalies is complex and require proper imaging and planning. We have a patient with a partial duplex collecting system presented with a right renal calculus in a non-functioning lower moiety and multiple distal ureteric calculi. Preoperative planning done and surgery performed with good outcome without any early and late complications.

[A rare case of bladder leiomyoma in pregnancy: a case report].

We present a rare case of leiomyoma of the urinary bladder that was diagnosed during pregnancy. The case of a 29-year-old woman primigravida at 13 weeks of pregnancy who presented with 6 months history of abdominal swelling which was gradually increasing in size. Computed tomography done revealed a large heterogenous mass(enhancing) with an area of non-enhancing (necrosis) suggestive of malignant ovarian tumor. The histological findings of the surgical specimen confirmed a leiomyoma of the urinary bladder. The clinical presentation, imaging findings, and management of this relatively rare benign tumor are discussed in this case report.

Female sexual dysfunction post radical cystectomy and urinary diversion.

OBJECTIVES: To evaluate the impact of radical cystectomy and urinary diversion on female sexual function. MATERIALS AND METHODS: A Medline search was conducted according to the PRISMA statement for all English full-text articles published between 1980 and 2016 and assessing female sexual function post radical cystectomy and urinary diversion. Eligible studies were subjected to critical analysis and revision. The primary outcomes were the reporting methods for female sexual dysfunction (FSD), manifestations of FSD, and factors associated with FSD, postoperative recoverability of FSD, and awareness level regarding FSD. RESULTS: From the resulting 117 articles, 11 studies were finally included in our systematic review, with a total of 361 women. Loss of sexual desire and orgasm disorders were the most frequently reported (49% and 39%). Dyspareunia and vaginal lubrication disorders were reported in 25% and 9.5%, respectively. The incidence of sexual dysfunction was 10% in 30 patients receiving genital- or nerve-sparing cystectomy vs. 59% receiving conventional cystectomy. CONCLUSION: Although female sexual function is an important predictor of health-related quality of life post radical cystectomy and urinary diversion, the available literature is not enough to provide proper information for surgeons and patients.

Laparoscopic radical nephrectomy with inferior vena cava thrombectomy: highlight of key surgical steps.

OBJECTIVE: Vascular involvement in the form of renal vein (RV) or inferior vena cava (IVC) thrombus can be seen in 4-10% of patients presented with RCC. In patients without presence of metastasis, surgical treatment in the form of radical nephrectomy remains the treatment of choice with 5-year survival rates of 45-70%. Open surgery is still the first treatment option of choice at the moment for RCC patients with IVC thrombus. MATERIALS AND METHODS: In our study, we are reporting a case of patient with RCC and level I IVC thrombus treated with laparoscopy. Our patient is a 72 years old man with underlying co-morbidity of hypertension and chronic kidney disease (CKD) presented with right-sided RCC. The CT scan done showed a large right renal upper pole tumor measuring 8.4x5.2cm with level I IVC thrombus (Figure-1). There were no regional lymphadenopathy and the staging scans were negative. RESULTS: The operative time was 124 minutes and blood loss was minimal. The patient was progressed to diet on POD 1 with bowel movement on POD 2. There was no significant change in the pre and post-operative glomerular filtration rate (GFR). The surgical drain was removed on POD2. The patient was discharged well on POD 5. There were no perioperative complications. The pathology was pT3bN0M0 Fuhrman grade II clear cell RCC. CONCLUSIONS: As a conclusion, laparoscopic radical nephrectomy and IVC thrombectomy is a complex and technically demanding surgery. With advancement of surgical skills as well as technology, more cases of minimally invasive laparoscopic radical nephrectomy and IVC thrombectomy can performed to improve the perioperative outcomes of carefully selected patients in a high volume center.

Effect of C-factor on microtensile bond strengths of low-shrinkage composites.

UNLABELLED: This study evaluated the microtensile bond strength (µ-TBS) of low-shrinkage composites with their corresponding adhesive systems, Filtek Silorane/Silorane adhesive (SIL, 3M ESPE AG, Seefeld, Germany) and Aelite LS/One-Step Plus (AL, BISCO Inc, Schaumburg, IL, USA) in cavities with different C-factors. Filtek Z250/Adper Single Bond Plus (Z, 3M ESPE, St Paul, MN, USA) was used as a control. METHOD: Standardized Class I cavities were prepared in extracted human molars after removing occlusal enamel. Cavities were assigned into six different C-factors by applying nail polish to four walls, three walls, two walls adjacent to each other, two walls opposite to each other, one wall, or no walls. Resin composites with their corresponding adhesive systems were applied according to manufacturer instructions. Specimens were sectioned to obtain four rectangular beams from the center of the restorations and µ-TBS was measured. Data were analyzed by Weibull survival analysis. Shrinkage stresses of the resin composites were determined after 30 minutes from the start of light-curing using a tensometer testing machine. Flexure elastic modulus was determined using standard procedures, in accordance with ISO 4049. Data for shrinkage stress and elastic modulus were analyzed by one-way analysis of variance followed by a Tukey multiple-comparisons test (p<0.05). RESULTS: µ-TBS of both SIL and AL were not affected by different C-factors; however, the bond strength of Z decreased significantly when the C-factor increased. Shrinkage stress results were 0.94 ± 0.1, 1.79 ± 0.18, and 2.14 ± 0.23 MPa for SIL, AL, and Z, respectively. The flexural modulus of both the SIL and the AL was significantly lower than that of Z. CONCLUSIONS: Increasing C-factor did not negatively affect the bond strength of low-shrinkage composites.

Hematopoietic Stem Cell Transplantation in Egypt: Challenges and Opportunities.

Hematopoietic stem cell transplantation (HSCT) is now an established treatment modality with definitive indications for many hematological disorders. However, HSCT requires tremendous resources, and it is increasingly challenging for transplantation experts to practice in the developing world and to reach a compromise between requirements and available resources. Based on 30 years of experience and 4256 transplants (60% allogeneic and 40% autologous), this article focuses on the challenges our HSCT program encountered since it started in 1989 and what opportunities we see to solve them. Since 1997, HSCT procedures increased dramatically with the opening of 15 HSCT units distributed all over Egypt.

Differential induction of altered gene expression by carcinogens at mutant alleles of a Drosophila locus with a transposable element.

Alterations in gene expression by carcinogens were analyzed on three unstable alleles of the white (w+) locus of Drosophilia melanogaster: white-crimson (wc); white-ivory 16 (wi16); and white-unstable 11 (wu11). Two of these alleles (wi16 and wu11) were spontaneous mutant derivatives of wc, which is known to harbor a transposable element. The compounds studied were dimethylnitrosamine, 7,12-dimethylbenz(a)anthracene, and aflatoxin B1. These carcinogens were topically applied on the early larval stages, and the genetic effects assayed were the alterations in eye color either to wild-type (w+) or to other w mutants, initiated both somatically and germinally, as well as the simultaneously induced X-chromosome recessive mutations. The tested compounds influenced the different unstable w alleles in a highly selective manner, both as a function of the inducing agent and the organization of the genome in the target cells. The same treatments raised the somatic reversions to w+ above the corresponding controls for wc and wi16, but not for wu11, whereas the simultaneous induction of other w mutant phenotypes occurred appreciably only with wc. Furthermore, these treatments gave high and variable somatic reversions to w+ with wi16, whereas the simultaneously induced germinal events were uniformly very low. The frequencies of altered expression at the unstable test loci, whether in the soma or germ line, were quantitatively uncorrelated with the mutagenic effects of the treatments in terms of the yield of X-chromosome recessive mutations assayed in the progeny of males emerging from the same treated larvae. There was also an association between the time of the induction of these alterations by the tested carcinogens in the soma and the cellular stage in genomic differentiation. Reversions to w+ were induced preferentially after the onset of genetic determination, whereas changes to the w mutant phenotypes occurred predominantly during the predetermination phases. The genetic properties of transposable elements and the manner of their response to carcinogens supported the hypothesis that nonviral cancer might arise from molecular processes similar to those involved in the evolution of retroviruses.

Genetic properties of N-alpha-acetoxymethyl-N-methylnitrosamine in relation to the metabolic activation of N,N-dimethylnitrosamine.

A genetic study was undertaken in Drosophila with N-alpha-acetoxymethyl-N-methylnitrosamine, a precursor of the alpha-hydroxymethyl derivative of N,N-dimethylnitrosamine, to assess the role of alpha-carbon oxidation in toxicological activation. Genetic activity was measured for the whole testicular tissue with respect to the general response of the X chromosome (recessive lethals and visibles), as well as certain specific genic sites, including representatives of the RNA genes. The biological activity of the acetoxy compound proved to be considerably higher than that of the parent amine with respect to both cytotoxicity and mutagenicity. At low and equitoxic molarities of the 2 agents (0.1 to 1.0 and 1.0 to 10.0 mM, respectively), dose-dependence for all the investigated genetic functions followed identical patterns, which were best described by quadratic dose curves. However, the regression coefficients for the acetoxy derivative were at least 1 order of magnitude higher than the corresponding values for the amine, indicating a consistent level of mutagenic activation as a result of the alpha-acetoxymethyl substitution. At mutagenically equivalent doses, the 2 compounds gave statistically comparable frequencies of mosaicism among corresponding mutational classes and equal ribosomal DNA selectivity indices, indicating identical molecular mechanisms of mutagenesis. The higher mutagenicity of N-alpha-acetoxymethyl-N-methylnitrosamine compared to N,N-dimethylnitrosamine was paralleled by its higher carcinogenicity which would suggest that the same effective metabolites might be involved in both processes.

Mutagenicity of N-alpha-acetoxyethyl-N-ethylnitrosamine and N,N-diethylnitrosamine in relation to the mechanisms of metabolic activation of dialkylnitrosamines.

The mutagenicities of N-alpha-acetoxyethyl-N-ethylnitrosamine and N,N-diethylnitrosamine were compared in Drosophila for an assessment of the role of alpha-carbon ethyl oxidation in the toxicological activation of the amine. The relative genetic potencies of the two compounds were deduced from regression studies of the mutation frequencies on molar dose for the whole testicular tissue with respect to the induction of the overall X-chromosome recessives (lethals and visibles), representatives of the RNA genes (especially ribosomal DNA), and six specific euchromatic loci. Genetic activity per unit molar dose was invariably higher for the acetoxy compound than for the parent amine, but the two agents produced comparable mosaicism among corresponding mutational classes. This indicated that the same mutagenic reactive species were generated from the two agents, but more readily from the acetoxy derivative, which accorded with the hypothesis of nitrosamine metabolic activation through alpha-carbon alkyl hydroxylation. The differentials for the higher activity of the acetoxy compound varied between mutational classes: 20-fold for the X-chromosome recessives collectively or per locus, but only 5-fold for the specific ribosomal DNA deletions. These relationships indicated that the mutagenicities of these compounds were not entirely an outcome of DNA ethylation but were readily explicable on the basis of the additional generation of difunctional nitrosaldehydic metabolites, which were largely responsible for the induction of the ribosomal DNA deletions through DNA protein cross-link-age. The higher mutagenicity of the acetoxy compound relative to the parent amine on ribosomal DNA was in part countered by its much lower selectivity index for these genes. The possible relevance of these genetic features to the carcinogenicities of these compounds was considered.

Impact of CD34 subsets on engraftment kinetics in allogeneic peripheral blood stem cell transplantation.

Our objective was to evaluate, probably for the first time, the impact of CD34 subsets on engraftment kinetics in allogeneic PBSC transplantation (PBSCT). PBSC graft components were analyzed in 62 cases for the absolute count/kg of total CD34+ and the following subsets: DR- and +, CD71+/-, CD38+/-, CD33+/- and CD61+/-. Time to ANC >0.5 and >1 x 10(9)/l and platelets >20 and >50 x 10(9)/l was reported. The median value for each parameter was used to discriminate rapid from slow engraftment. Four parameters showed significant predictive power of early neutrophil engraftment, namely CD34+ /DR- (P = 0.002), CD34+/38- (P = 0.02), CD34+/CD61- (P = 0.04) and total CD34+ cell dose (P = 0.04). Four parameters showed significant predictive power of early platelet engraftment, namely CD34+/CD61+ (P = 0.02), CD34+ /CD38- and total CD34+ cell dose (P = 0.04) and CD34+ /CD71- (P = 0.05). Comparing patients who received > to those who received < the threshold dose(s), only CD34+ /CD38- lost its significance for neutrophil engraftment; and only CD34+ /CD61+ retained its significance for platelet engraftment (P = 0.03); furthermore, the former group required significantly fewer platelet transfusions (P = 0.018). We concluded that in allogeneic PBSCT, the best predictor of early neutrophil engraftment is the absolute CD34+ /DR- and for early platelet engraftment is the absolute CD34+ /CD61+ cell dose.

Peripheral blood vs bone marrow as a source for allogeneic hematopoietic stem cell transplantation.

In this randomized prospective study, we included 30 patients with different hematological diseases (acute myeloid leukemia, acute lymphoblastic leukemia, chronic myeloid leukemia, myelodysplastic syndrome or severe aplastic anemia) to compare peripheral blood stem cells (PBSC) (15 patients; mean age 23) and bone marrow (BM) (15 patients; mean age 21.8) as a source for allogeneic transplantation regarding the tempo of hematopoietic recovery and the incidence of acute graft-versus-host disease (GVHD). In the BM group, the median nucleated cell count harvested was 1.3 x 10(10), while in the PBSC group, the aphereses contained a median of 4.4 x 10(6) CD34+/kg recipient weight. PBSC transplantation (PBSCT) was associated with faster hematopoietic reconstitution measured as absolute neutrophil count (ANC) >0.5 x 10(9)/l (log-rank P value <0.0018) and platelet count >25 x 10(9)/l (log-rank P value <0.0098). Seven patients (46.7%) in the BM group vs only one patient (6.7%) in the PBSC group developed acute GVHD (P = 0.013). Therefore, we conclude that PBSCT is associated with faster hematopoietic recovery and the incidence of acute GVHD does not exceed that seen with BMT.

Mutagenic selectivity of carcinogenic nitroso compounds. II. N,N-dimethylnitrosamine.

The genetic properties in the hepatocarcinogen N,N-dimethylnitrosamine (DMN) were examined in Drosophila for the assessment of the role of dose, cellular metabolism and genic target in its mutagenicity. Genetic activity was assayed with respect to the induction of the non-specific X-chromosome recessives (lethals and visibles) relative to the effects on specific genic sites, especially rDNA, which yields bobbed (bb) mutations. Dosses and germ cell types, which indicated that DMN induced at least some multiple-hit mutagenic events. The genetic activity of DMN was favoured by cellular metabolism for all mutational classes, as was indicated by the progressive increase in mutational classes, as was indicated by the progressive increase in mutation yield during spermatogenesis--from the metabolically inert mature sperm to the actively metabolizing spermatocyte and spermatogonia. The role of DNA methylation in the mutagenicity of DMN was deduced from quantitative assays for its genetic activity relative to the methylating nitrosamide--N-methyl-N-nitrosourethane (MNUr)--over the same dose range (1-10 mM) and on identical cell types and genic targets. In the metabolically inert cells (mature sperm), the two compounds were equally active with respect to the non-specific effects (X-recessives), but MNUr, but the two compounds were equally effective on rDNA. These results could not be entirely interpreted by the methylation hypothesis and indicated that a DMN aldehydic metabolite, structurally analogous to MNUr, might be responsible for the induction of the rDNA mutations. The rDNA selectivity index of DMN was significantly lower than for MNUr, which paralleled their relative carcinogenic verstilities. However, DMN was comparatively more effective on the tRNA genes, a feature which might be associated with its oncogenic specificity.

Mutagenic selectivity of carcinogenic nitroso compounds 2. n-methyl-N-nitrosourethane.

The mutagenicity of the carcinogen methylnitrosourethane (MNUr) was examined in Drosophila with a view to the determination of its activity on heterochromatic loci (especially rDNA) relative to those in the euchromatin. Assays were made of the yield of rDNA mutations (bobbed: bb) relative to other X-chromosome recessive lethals and visibles [X(l + v)] in the same male germ cells after treatment with different doses (1-10 mM) and at various stages in spermatogenesis. Dose dependence followed the same pattern for all genic loci and germ cell stages. In all instances, the regression of mutation frequency on injected molar dose was approximately linear, but could better be described by a quadratic dose curve. In contrast, the mutagenicity pattern during spermatogenesis varied according to the target genes. The response of the euchromatic loci reached a peak among the earlier germ cells (probably the spermatocytes), whereas that for the heterochromatic sites (including rDNA) was maximal in mature sperm. Mutagenic selectivity for rDNA with MNUr, as indicated by the percentage bb/X-mutations, was among the highest for the intrinsically reactive carcinogens (alkylating and arylating agents). This correlates with the strong carcinogenicity of MNUr and adds further support to the concept that rDNA mutations might well be a crucial step in cancer initiation.

Mutagenic selectivity of carcinogenic nitroso compounds: III. N,alpha-acetoxymethyl-N-methylnitrosamine.

A comparative genetic study was undertaken on the testicular tissue of Drosophila with N-alpha-acetoxymethol-N-METHYLNITROSAMINE (AcODMN) and its unsubstituted parent N,N-dimethylnitrosamine (DMN), to assess the role of intracellular metabolism on their mutagenicities. The relative genetic potencies of the two compounds were deduced from regression studies of the dose effect on the metabolically inert sperm and the metabolizing early germ cells (spermatocytes and spermatogonia) with respect to the induction of the non-specific X-chromosome recessives (lethals and visibles) and the specific effects on representatives of the RNA genes, especially rDNA. Genetic activity per unit molar dose was invariably higher for the acetoxy derivative as compared to the parent amine, but the differential in this respect varied significantly for various mutational classes and as a function of the metabolic level in the target cells. The induction of point-mutations (X-recessives) increased with the level of intracellular metabolism with bothe compounds and this was more pronounced with the parent amine, which was in accordance with the DNA methylation mechanism. In contrast, the yield of the specific rDNA deletions was not markedly enhanced with the increased metabolic activity in the early germ cells, especially with the acetoxy derivative. The induction of these deletions could not, therefore, be explained on the basis of DNA methylation, but was reconcilable with the posible generation of a nitroso-aldehydic metabolite, which could effect DNA-protein cross-linkage within the genic nucleoproteins. The two test compounds gave comparable frequencies of mosaicism among corresponding mutations and the same rDNA selectivity index, which indicated identical molecular mechanisms of mutagenesis. The higher genetic potency of the acetoxy derivative as compared to the parent amine would thus be indicative of its greater yield of the same mutagenic metabolites. Carcinogenicity studies with the two compounds paralleled the mutagenicity results, which whould suggest that the same molecular mechanisms could well be responsible for the initiation of both phenomena.

Mutagenicity of hair dye components relative to the carcinogen benzidine in Drosophila melanogaster.

A comparative assay was undertaken in Drosophila melanogaster for the assessment of the mutagenic efficiency of the hair dye components m-toluene-diamine (m-TD) and 4-nitro-o-phenylenediamine (4-NOPD) relative to the aromatic amine human carcinogen benzidine (Bzd). The compounds were injected at equimolar dose ranges (5-20 mM) around the testes of adult males and their mutagenicities were measured separately on the various stages of spermatogenesis. Genetic activity was simultaneously assayed with respect to the overall induction of the X-chromosome recessives (lethals and visibles) relative to the specific effects on rDNA (expressed as bobbed mutations). All compounds exerted decisive mutagenicity both on the X-chromosome and the RNA genes, although their activities on the different genic sites varied between compounds and as a function of cell stage, but not in response to changes in dose, within the investigated molarity range. The mutagenicities and selectivities of the test compounds for rDNA gradually decreased in the order Bzd greater than m-TD greater than 4-NOPD, which correlated with the evidence-so far-about their carcinogenicities.

The MR P-type transposable elements and the genetic activities of mutagens and carcinogens in Drosophila melanogaster. I. N,N-Dimethylnitrosamine (DMN).

A technique was developed for the assay of the genetic activities of carcinogens in both the soma and germ line in the course of early larval development and to assess the extent of their modification through the introduction into the genome of the MR IInd autosome P-type transposable elements. The influence of MR on genotoxicity for a given treatment was indicated by the relative frequencies of non-MR (Cy) to MR-carrying sibs emerging within the same cultures. The viable genetic changes in the soma were classified as recombinational or mutational events on the basis of the comparative yields of mosaic sectors in females heterozygous for the markers y w sn carried in standard order (XS) or multiply inverted (XIn) X-chromosomes. The results with this technique are here described for the carcinogen DMN. In the absence of MR, the topical application of DMN induced no larval lethality up to the highest tested doses (20 mM), but raised the yields of the somatic sectors for all the test markers in the emerging females in accordance with a linear dose fit. Comparison of the XS and XIn data indicated that somatic mutagenesis by DMN entailed the induction of recombinational and mutational events in roughly equal proportions. The introduction of MR into the genome, whether patro- or matroclinously, resulted in dramatic and proportionately equivalent enhancements in the activities of DMN, both with respect to the induction of larval lethality and somatic sectoring. These activities increased exponentially with dose, following a 4th-degree polynomial course up to 10 mM, when larval lethality approached 100%. At lower dose levels, the yields of all sector types in the viable females also followed comparable polynomial curves at different heights, except for y sn in the XIn series, where sector recovery remained at the control level throughout the examined dose range. Analysis of the sector size distribution for eye and bristle mosaicism in the XS control and DMN-treated series gave statistically comparable mean values, irrespective of the presence or absence of MR, indicating that DMN alone, or in conjunction with the P elements, did not alter the timing of aberrant clone initiation. In contrast, estimates of the genetic induction events in the somatic primordia with 5 mM DMN indicated greatly increased response in the presence of MR, which was in excess of 20-fold for the mutational changes involving the sn locus.(ABSTRACT TRUNCATED AT 400 WORDS)

Genetic activities of 4-chloromethylbiphenyl, the 4-hydroxy derivative and benzyl chloride in the soma and germ line of Drosophila melanogaster.

The genetic activities of 4CMB, 4HMB and BC were assayed as regards the induction of somatic alterations in gene expression on an unstable w+ locus with an intragenic TE and all the simultaneously induced germinal mutations on the X-chromosome carrying this locus. The compounds were applied topically in solution at equimolar doses on late embryos and newly hatched larvae. The somatic events were scored as aberrantly pigmented eye sectors in the emerging adult males and the germinal mutations in their F2 progeny, according to the Muller-5 technique. The somatic events were expressed as red or while mosaic eye sectors; the former could be an outcome of the repression or deletion of the zeste-regulatory proximal subunits of w+ locus, and the latter generally attributable to deletions (w-) within its structural part. All 3 compounds were effective in the induction of red sectors at the higher tested doses (0.5-2.0 mM) and the level of this activity was virtually the same for 4CMB and 4HMB, but was 2-fold higher for BC. In contrast, the frequencies of the simultaneously scored white sectors were not raised significantly above the controls with 4CMB, but showed decisive increases above this level with both 4HMB and BC. The germinal X-chromosome mutations (recessive lethals and visibles) were only induced at the highest tested dose (2.0 mM), and their frequencies were virtually the same for all 3 compounds reaching a common level of about 0.6%, which is some 3-fold the normal control level for the test system. Specific-locus mutability at the TE w+ was suggestively positive only with BC.

Spectrophotometric determination of cimetidine in the presence of its acid-induced degradation products.

Cimetidine has been determined in the presence of its acid-induced degradation products using a second derivative (D2-) spectrophotometric method (method I) or a colorimetric method (method II). The former is based on D2-value measurement at 216 nm, whilst the latter depends on charge-transfer complexation with dichlorophenol-indophenol. The two methods are proved to be stability indicating, since plots of log C% versus time were linear. The application to cimetidine determination in tablets and ampoules gave good results.

Reduced intensity conditioning is effective for hematopoietic SCT in dyskeratosis congenita-related BM failure.

BM failure (BMF) is a major and frequent complication of dyskeratosis congenita (DKC). Allogeneic hematopoietic SCT (allo-HSCT) represents the only curative treatment for BMF associated with this condition. Transplant-related morbidity/mortality is common especially after myeloablative conditioning regimens. Herein, we report nine cases of patients with DKC who received an allo-SCT at five different member centers within the Eastern Mediterranean Blood and Marrow Transplantation Registry. Between October 1992 and February 2011, nine DKC patients (male, 7 and female, 2), with a median age at transplantation of 19.1 (4.9-31.1) years, underwent an allo-HSCT from HLA-matched, morphologically normal-related donors (100%). Preparative regimens varied according to different centers, but was reduced intensity conditioning (RIC) in eight patients. Graft source was unstimulated BM in five cases (56%) and G-CSF-mobilized PBSCs in four (44%) cases. The median stem cell dose was 6.79 (2.06-12.4) × 10(6) cells/kg body weight. GVHD prophylaxis consisted of CsA in all nine cases; MTX or mycophenolate mofetil were added in five (56%) and two (22%) cases, respectively. Anti-thymocyte globulin was administered at various doses and scheduled in four (44%) cases. Median time-to-neutrophil engraftment was 21 (17-27) days. In one case, late graft failure was noted at 10.4 months post allo-HSCT. Only one patient developed grade II acute GVHD (11%). Extensive chronic GVHD was reported in one case, whereas limited chronic GVHD occurred in another four cases. At a median follow-up of 61 (0.8-212) months, seven (78%) patients were still alive and transfusion independent. One patient died of metastatic gastric adenocarcinoma and graft failure was the cause of death in another patient. This study suggests that RIC preparative regimens are successful in inducing hematopoietic cell engraftment in patients with BMF from DKC. Owing to the limited sample size, the use of registry data and heterogeneity of preparative as well as GVHD prophylaxis regimens reported in this series, we are unable to recommend a particular regimen to be considered as the standard for patients with this disease.