Comparison of treatment effects from total arch distalization using modified C-palatal plates versus maxillary premolar extraction in Class II patients with severe overjet.

INTRODUCTION: This study aimed to compare the skeletodental and soft tissue changes with total arch distalization using a modified C-palatal plate (MCPP) and maxillary first premolar extraction treatment in Class II malocclusion patients with severe overjet. SETTING AND SAMPLE POPULATION: The sample consisted of 46 adult patients who had Class II Division 1 malocclusion with severe overjet; 25 of them received non-extraction treatment with MCPPs (age, 22.5 ± 7.2 years), and 21 received maxillary first premolar extraction treatment (age 23.4 ± 6.5 years). METHOD: A total of 26 variables were measured on pre- and post-treatment lateral cephalograms. To evaluate the differences between pretreatment and post-treatment in each group, t tests and Wilcoxon rank-sum tests were used. To compare the amount of change between the two groups, MANOVA test was used. RESULTS: The overjet was significantly reduced in the MCPP and extraction groups by 4.8 mm and 5.4 mm, respectively. However, the two groups had no significant difference in the sagittal, vertical and angular changes of the maxillary incisors. In addition, regarding soft tissue changes, the MCPP and extraction groups showed an increased nasolabial angle of 7.5° and 9.4°, decreased upper lip to the true vertical line of 1.8 mm and 2.2 mm, respectively (P < .001). CONCLUSIONS: There was no significant difference in the skeletal changes between the MCPP and extraction groups, and the reduction in overjet was similar in the groups. These results suggest that MCPP might be a viable treatment option for total arch distalization in Class II malocclusion patients with severe overjet.

Leclercia adecarboxylata Bacteremia without a Focus in a Non-Immunosuppressed Patient.

BACKGROUND Leclercia adecarboxylata is a gram-negative rod, which is normally found in water and food. It is an emerging pathogen that affects immunocompromised patients, including patients with hematological malignancies or those receiving chemotherapy. Generally, L. adecarboxylata is considered a low-virulence pathogen with an excellent susceptibility profile, but some strains may be resistant to multiple antibiotics, such as b-lactams. Moreover, L. adecarboxylata is usually isolated as a part of polymicrobial cultures in immunocompetent individuals, but there have been cases where it was the only isolate. CASE REPORT A 74-year-old woman who was non-immunosuppressed and had multiple comorbidities was admitted with acute decompensated heart failure due to pneumonia. She was treated with multiple courses of antibiotics including amoxicillin-clavulanate and ciprofloxacin for pneumonia, but her infection worsened, and she had cardiopulmonary arrest. After resuscitation, she was stable for several days but suddenly became confused and hypotensive. The septic screen showed L. adecarboxylata bacteremia without a clear source, which was treated successfully with meropenem for 14 days. After the meropenem course, the patient developed diarrhea and was found to have severe Clostridium difficile infection. She did not respond to oral vancomycin and intravenous metronidazole and died. CONCLUSIONS This case illustrated an infection in a non-immunosuppressed individual by an organism that is considered an opportunistic pathogen, mainly affecting immunocompromised patients. The patient's blood culture grew L. adecarboxylata, which was sensitive to all antibiotics but resolved with meropenem treatment. Owing to increasing L. adecarboxylata infections, we recommend further studies to understand the organism's pathogenesis, risk factors, and resistance pattern.

Total maxillary arch distalization with modified C-palatal plates in adolescents: A long-term study using cone-beam computed tomography.

INTRODUCTION: The aims of this study were to evaluate the long-term skeletodental effects, the volume of maxillary tuberosity, and airway space changes after maxillary molar distalization using modified C-palatal plate (MCPP) in adolescents with Class II malocclusion. METHODS: The sample consisted of 20 adolescent patients (MCPP group; mean age, 12.9 ± 1.0 year) who underwent bilateral distalization of their maxillary dentition and 20 subjects as a control group. In the MCPP group, cone-beam computed tomography images were taken before distalization, at the end of the treatment, and during retention with a minimum of a 3-year posttreatment follow up period. Repeated measures ANOVA followed by post-hoc analysis with the Bonferroni test were used to identify significant differences between time points. RESULTS: After the long-term observation period, sagittal skeletal and dental relationships were maintained (there were no significant changes in ANB, occlusal plane angle, and overjet postretention). The vertical skeletal dimension did not change during treatment and was stable at the long-term follow-up (the mandibular plane angle and ANS-Me were relatively well maintained). The volume of the maxillary tuberosity showed no significant change during long-term retention. However, the volume was significantly smaller in the treatment group than in the control group (P <0.0001). There were no significant airway space changes after distalization and the postretention period. In addition, there was no significant difference between the MCPP and control groups. CONCLUSIONS: Improved sagittal skeletal and dental relationships because of treatment were maintained in the long-term evaluation. There was no negative long-term effect on airway space associated with the maxillary arch distalization. Therefore, these findings might be beneficial for clinicians in diagnosis and treatment planning for Class II malocclusion in adolescents.

Perception Among Diabetic Patients of Telescreening for Diabetic Retinopathy.

Purpose: To evaluate the satisfaction of diabetic patients and its determinants of telescreening for diabetic retinopathy (DR) in Saudi Arabia. Methods: This cross-sectional survey was conducted in December 2018 in a diabetes center of Riyadh, Saudi Arabia. Ten questions were asked by the interviewer. A 5-point Likert scale was used to grade patient responses for each question. Rasch analysis was conducted to evaluate the response of the participants. The main outcome variable was the sum of the Logit values of the responses. The Rasch score was also compared among subgroups. Results: Two hundred (n = 200) diabetic patients were interviewed. The median Rasch score of client-perceived benefit of DR telescreening was +14.6 (25% quartile +3.09, minimum; -23.2, maximum; +35.7). A positive attitude of patients regarding DR telescreening was recorded in 159 (79.5%) participants. The perception of telescreening was better in younger diabetic patients than in older diabetic patients (Friedman p < 0.001). The perception was similar in both genders (Mann-Whitney p = 0.3). Diabetic patients from Riyadh and the southern region of Saudi Arabia had significantly more positive perception than those from north and eastern regions (Freedman p < 0.001). Conclusion: Diabetic patients have positive attitude toward tele-DR screening. Their cooperation is likely to be high if large scale tele-DR screening is implemented in the Kingdom.

Comparison of Digital Retinal Images and Information Tele-Transferred Through the Hala System to Manual Image Transfer of Diabetic Patients from Primary Health Care Centers in Riyadh.

Background: Diabetes is a significant problem in Saudi Arabia, and telemedicine in the form of tele-screening for diabetic retinopathy (DR) is recommended. Introduction: A comparison of the quality of images and information transferred through the Hala system of the Ministry of Health, Saudi Arabia, with images transferred in a survey at Primary Healthcare Centers in Riyadh, Saudi Arabia, was conducted. Methods: A review of health records was undertaken in 2017-2018. Digital retinal images of diabetics were tele-transferred from a diabetes center to the King Khaled Eye Specialist Hospital (KKESH) for review by medical retina specialists (Group I). Similarly, images of diabetic patients that were transferred from seven primary health clinics to KKESH during a survey were used as images for research (Group II). Additional information reviewed for completeness included patient demographics, risk factors of DR, and previous eye surgery. Results: There were 40 images of 40 patients transferred through the Hala system. There were 120 randomly selected images from survey files. There were significantly more poor images from the Hala system (12.5%) than the research survey system (2.4%). There was no significant difference in the severity of DR between groups. The median Rasch score for the images was -0.58 (interquartile range [IQR] -5.6, 2.6) in Group I and 19.7 (IQR 18.3, 19.7) in Group II. The image reader perceived that the quality of digital retinal images was significantly better in images transferred from the research survey compared with those transferred through the Hala DR tele-transfer system. (Mann-Whitney p < 0.001). Conclusions: Digital image capture and supplementary risk factor information require improvement to enhance DR screening using the Hala system.

Incidence, clinical distribution, and patient characteristics of childhood cancer in Saudi Arabia: A population-based analysis.

PURPOSE: Information regarding the incidence and patterns of childhood malignancies is disproportionately overrepresented by high-income countries, representing mainly the Caucasian population. There is a need to evaluate and disseminate information for other ethnicities, particularly from the Middle East. METHODS: Data from the National Cancer Registry, Saudi Arabia (SA-NCR), for pediatric patients (age 0-14 years) diagnosed between 2005 and 2009 and for similar patients at our institution during the same period were analyzed. Population numbers reported in the 2007 national census were used to calculate the annual incidence of childhood cancer. RESULTS: Data from SA-NCR on 3885 patients were included in this analysis. The median age was 5.58 years, and 57.3% were males. The annual age-specific cancer incidence rate (ASR) for children in SA is 99.83 per million population; ASR per million for lymphoid leukemia is 25.75, 12.05 for brain tumors, and 9.82 for Hodgkin lymphoma. Of all childhood cancers in SA, 35% were treated at our institution. The five-year overall survival for these 1350 patients is 74.6% (median follow-up 7.52 years [95% confidence interval: 7.36-7.68]). Significant differences in the distribution of childhood malignancy subtypes were evident compared with other countries. CONCLUSION: We have reported differences in the cancer ASR and cancer subtype distribution for children in SA as compared with the worldwide incidence and with other populations. This paper provides a comprehensive epidemiological overview of childhood cancer in SA, which could be extrapolated to other regional Arab populations.

Dietary products consumption in relation to serum 25-hydroxyvitamin D and selenium level in Saudi children and adults.

Vitamin D deficiency is a global health threat that has been associated with several chronic diseases. Selenium is an essential trace element because of role in major metabolic processes, immune function, thyroid hormone metabolism, male infertility, neoplasms and cardiovascular disease. We aimed to investigate for the first time in the Saudi population the association between vitamin D and selenium status with various dietary products consumption. A total of 259 children and 95 adults were included in this cross-sectional study. We estimated the consumption frequencies of various dietary food products using a qualitative food frequency questionnaire (FFQ) and also measured serum levels of 25-hydroxyvitamin D and selenium. Associations between variables of interest were assessed. Vitamin D deficiency and insufficiency were observed in 80% of the boys, 90% of the girls, 64% of men and 50% of women. Modest associations were found between mean serum 25 (OH) D concentration and consumption frequencies of fresh milk in children (r=0.11; P<0.05), more specifically in girls (r=0.12; P<0.05), and to the overall consumption of dairy products in women (r=0.12; P<0.05). Vitamin D status was also inversely associated with selenium in adults (r=-0.43; P<0.05). There was a significant correlation between delta changes of serum selenium, triglycerides and HDL levels (P-values <0.05). Vitamin D and selenium levels are modestly associated with dietary products consumption. Changes in selenium levels were associated with increased serum triglyceride levels, indicating a potential biomarker for cardiovascular risk and dyslipidemia. The widespread vitamin D deficiency observed in the present study highlight the need for adequate fortification of dairy products.

Inflammatory and bone turnover markers in relation to PTH and vitamin D status among saudi postmenopausal women with and without osteoporosis.

Postmenopausal osteoporosis is characterized by rapid bone loss occurring in the post-menopausal period. The bone loss predominantly involves the trabecular bone and is brought about by an imbalance between the bone remodeling process which can be influenced by factors that could cause or contribute to osteoporosis. Pro-inflammatory cytokines (Il-1ß, Il-6, IL-8 and TNF-α) have been implicated in the regulation of bone cells and play a critical role in bone remodeling. They act both directly and indirectly to increase bone resorption, and/or inhibit bone formation. The aim of the study is to determine whether pro-inflammatory cytokines correlate with bone turnover markers (BTM) in a cohort of Saudi post-menopausal women with or without osteoporosis and which BTMs will correlate with PTH and Vitamin D for use in osteoporosis diagnosis. The study is composed of 100 post-menopausal patients and 100 controls aged around 50 years. Serum concentrations of pro-inflammatory and BTMs as well as PTH and vitamin D were determined by ELISA, Luminex and electrochemiluminescence. Serum calcium, phosphorus, glucose, and lipid profile were measured by using a chemical analyzer. There was a significant increase in the levels of pro-inflammatory cytokines, PTH, CTx, and glucose. A significantly lower vitamin D and osteocalcin levels were observed in subjects with osteoporosis than those without. No significant differences were recorded in the circulating lipid profile between groups. The present study proved that the pro-inflammatory cytokines accelerate the bone loss in postmenopausal women.

Inflammatory and bone turnover markers in relation to PTH and vitamin D status among Saudi postmenopausal women with and without osteoporosis.

Postmenopausal osteoporosis is characterized by rapid bone loss occurring in the post-menopausal period. The bone loss predominantly involves the trabecular bone and is brought about by an imbalance between the bone remodeling process which can be influenced by factors that could cause or contribute to osteoporosis. Pro-inflammatory cytokines (Il-1ß, Il-6, IL-8 and TNF-α) have been implicated in the regulation of bone cells and play a critical role in bone remodeling. They act both directly and indirectly to increase bone resorption, and/or inhibit bone formation. The aim of the study is to determine whether pro-inflammatory cytokines correlate with bone turnover markers (BTM) in a cohort of Saudi post-menopausal women with or without osteoporosis and which BTMs will correlate with PTH and Vitamin D for use in osteoporosis diagnosis. The study is composed of 100 post-menopausal patients and 100 controls aged 50 years and above. Serum concentrations of pro-inflammatory and BTMs as well as PTH and vitamin D were determined by ELISA, Luminex and electrochemiluminescence. Serum calcium, phosphorus, glucose, and lipid profile were measured by using a chemical analyzer. There was a significant increase in the levels of pro-inflammatory cytokines, PTH, CTx, and glucose. A significantly lower vitamin D and osteocalcin levels were observed in subjects with osteoporosis than those without. No significant differences were recorded in the circulating lipid profile between groups. The present study proved that the pro-inflammatory cytokines accelerate the bone loss in postmenopausal women.

Glaucoma after open globe injury at a tertiary care university hospital in Central Saudi Arabia. Cumulative incidence and risk factors.

OBJECTIVE: To investigate the incidence and risk factors of glaucoma after open globe injury (OGI). METHODS: The medical records of all patients admitted with the diagnosis of OGI at the Department of Ophthalmology, King Abdulaziz University Hospital, Riyadh, Kingdom of Saudi Arabia and had undergone primary repair from January 1996 to December 2011 were retrospectively reviewed. RESULTS: For the 15-year study period, 775 patients who underwent repair of an OGI were identified. The overall risk of post-traumatic glaucoma was 5.3% (41/775) with a mean +/- SD follow-up period of 12+/-6.5 months. Univariate analysis revealed that Zone II injury (p=0.027), penetrating ocular injury (p=0.0008), lens injury (p=0.011), vitreous hemorrhage (p=0.002), and presence of intraocular foreign body (p<0.0001) were significantly associated with glaucoma. Age of more than 18 years was critical (p=0.054). Following logistic regression, penetrating ocular injury (p=0.019), lens injury (p=0.002), and vitreous hemorrhage were significant (p=0.037). CONCLUSION: Glaucoma after OGI is not uncommon. Zone II injury, penetrating ocular injury, lens injury, presence of vitreous hemorrhage, and presence of an intraocular foreign body were significant risk factors for developing post-traumatic glaucoma.

Prevalence of carotid calcification detected on panoramic radiographs in a Saudi population from a training institute in Central Saudi Arabia.

OBJECTIVE: To determine the prevalence of carotid artery calcification as detected on panoramic radiographs in a Saudi population. METHODS: This cross sectional study was conducted at the College of Dentistry, King Saud University, Saudi Arabia between March 2008 and January 2009. Five hundred and fifty-five panoramic radiographs of Saudi patients aged 30 years and above was collected from the patient charts. The panoramic radiographs were examined by 3 trained and calibrated examiners to detect any carotid artery calcification. RESULTS: Carotid artery calcification was noted in 28 (5%) of the 555 radiographs. Among these, 21 images were obtained from men (3.75%) and 7 from women (1.25%). CONCLUSION: Dentists should be aware of the techniques to detect carotid artery calcification on the widely used panoramic radiographs. Consequently, information on a life-threatening condition could be provided to the patient, and a precautionary treatment could be suggested.

Fracture resistance of teeth obturated with RealSeal using two different chelating agents: an in vitro study.

OBJECTIVES: The aim of this study was to evaluate the fracture resistance of endodontically treated teeth filled with a resin-based obturation material using two different chelating agents. METHODS AND MATERIALS: Forty extracted single-canal human teeth were prepared, instrumented, and randomly divided into three groups: Group 1 (n=15) received a final flush with 10 ml of neutralized 17% EDTA, followed by 10 ml of sodium hypochlorite (NaOCl), then obturated using lateral condensation with RealSeal. Group 2 (n=15) received a final flush of BioPure MTAD, followed by 10 ml of NaOCl, then obturated using lateral condensation with RealSeal. Group 3, the control group (n=10), was instrumented but not obturated, then the root canal opening was sealed with a temporary filling material. The specimens were stored in 100% humidity for 10 days, mounted in polyester resin, and loaded to failure. RESULTS: The ANOVA revealed a significant difference between the control group and the experimental groups, although there was no statistically significant difference between Group 1 and Group 2 (p=0.05). The MTAD group displayed higher mean fracture load values than the EDTA group. CONCLUSIONS: It can be concluded that filling the root canals with RealSeal increased the in vitro resistance to fracture of single-canal extracted human teeth when compared to instrumented and unobturated teeth. Teeth treated with MTAD demonstrated high fracture-resistance values when compared to teeth treated with 17% EDTA, but they were not statistically significant. CLINICAL SIGNIFICANCE: Filling the root canals with RealSeal with increased bonding to roots will increase resistance to fracture of these teeth.

Penetrating keratoplasty for trachomatous corneal scarring.

PURPOSE: To evaluate graft survival and visual outcome after penetrating keratoplasty (PKP) for trachomatous corneal scarring. METHODS: A retrospective review was conducted on all cases of PKP performed at King Khaled Eye Specialist Hospital between January 1, 1997, and December 31, 2001, for trachomatous corneal scarring. RESULTS: This study included 127 eyes. The mean age at the time of surgery was 64.7 years (range, 40-90 years). The mean follow-up was 1266 days (range, 91-3423 days). At the most recent visit, 102 (80.2%) grafts were clear, and 25 (19.7%) had failed. Kaplan-Meier graft survival was 98.3% at 1 year, 85.9% at 2 years, 83.2% at 3 years, 80.2% at 4 years, and 76.6% at 5 years. Major postoperative complications included worsening of glaucoma (27.6%), endothelial rejection (17.3%), and bacterial keratitis (8.7%). Visual acuity improved in 107 (84.3%) eyes, remained the same in 12 (9.5%) eyes, and worsened in 8 (6.3%) eyes. Final visual acuity of 20/160 or better was obtained in 67 (56.7%) eyes. CONCLUSIONS: Treating trachomatous corneal scarring with PKP can be associated with a good prognosis for graft survival and improved vision in carefully selected cases with mild or well-controlled ocular surface disease and absent or previously surgically corrected eyelid abnormalities.

Migratory insertion of [B(C6F5)2] into C-H bonds: CO promoted transfer of the boryl fragment.

The reaction of (eta(5)-C5H5)Fe(CO)2B(C6F5)2 with CO has been shown to proceed via ligand substitution at the metal with accompanying transfer of the boryl fragment (via C-H insertion) to the Cp ring, thereby generating the zwitterion [eta(5)-C5H4B(C6F5)2H]Fe(CO)3 in quantitative yield.

Reactivity of the bis(pentafluorophenyl)boranes ClB(C6F5)2 and [HB(C6F5)2]n towards late transition metal reagents.

The reactivities of the highly electrophilic boranes ClB(C(6)F(5))(2) (1) and [HB(C(6)F(5))(2)](n) (2) towards a range of organometallic reagents featuring metals from Groups 7-10 have been investigated. Salt elimination chemistry is observed 1 between and the nucleophilic anions eta(5)-C(5)R(5))Fe(CO)(2)](-)(R = H or Me) and [Mn(CO)(5)](-), leading to the generation of the novel boryl complexes (eta(5)-C(5)R(5))Fe(CO)(2)B(C(6)F(5))(2)[R = H (3) or Me (4)] and (OC)(5)MnB(C(6)F(5))(2) (5). Such systems are designed to probe the extent to which the strongly sigma-donor boryl ligand can also act as a pi-acceptor; a variety of spectroscopic, structural and computational probes imply that even with such strongly electron withdrawing boryl substituents, the pi component of the metal-boron linkage is a relatively minor one. Similar reactivity is observed towards the hydridomanganese anion [(eta(5)-C(5)H(4)Me)Mn(CO)(2)H](-), generating a thermally labile product identified spectroscopically as (eta(5)-C(5)H(4)Me)Mn(CO)(2)(H)B(C(6)F(5))(2) (6). Boranes 1 and 2 display different patterns of reactivity towards low-valent platinum and rhodium complexes than those demonstrated previously for less electrophilic reagents. Thus, reaction of 1 with (Ph(3)P)(2)Pt(H(2)C=CH(2)) ultimately generates EtB(C(6)F(5))(2) (10) as the major boron-containing product, together with cis-(Ph(3)P)(2)PtCl(2) and trans-(Ph(3)P)(2)Pt(C(6)F(5))Cl (9). The cationic platinum hydride [(Ph(3)P)(3)PtH](+) is identified as an intermediate in the reaction pathway. Reaction of with [(Ph(3)P)(2)Rh(mu-Cl)](2), in toluene on the other hand, appears to proceed via ligand abstraction with both Ph(3)P.HB(C(6)F(5))(2) (11) and the arene rhodium(I) cation [(Ph(3)P)(2)Rh(eta(6)-C(6)H(5)Me)](+) (14) ultimately being formed.

Localised Ewing sarcoma/PNET of bone--prognostic factors and international data comparison.

BACKGROUND: To determine if the distribution of prognostic factors accounted for the differences when the outcome for localised Ewing Sarcoma/PNET bone in Saudi Arabia was compared with results from countries with well developed health care systems. PROCEDURE: Retrospective analysis was undertaken of 163 consecutive patients of all ages, treated with radical intent at KFSHRC from 1975 to 1998. Standard chemotherapy was commenced in all patients. The local treatment modality was resection +/- radiation in 30% and radiation treatment alone in 67%. Size data were available for 51 patients treated from 1994 to 1998, inclusive. One third of these patients had tumors with volume >500 ml. RESULTS: Three year survival significantly increased with the year of diagnosis, 1975-1988 45%; 1989-1993 55%; and 1993-1998 63% (P = 0.006). Favorable prognostic factors were age < or =14 (P = 0.07); site, distal extremity, and skull (P = 0.08); and volume < or = 200 ml (P = 0.06). Secondary prognostic factors were response to induction chemotherapy, both histological, 100% necrosis, (P = 0.04) and clinical CR+PR, (P = 0.02). From 1994 to 1998, 3 year survival for tumors in the distal extremity and skull was 80% and for small tumors, < 200 ml, at any site was 82%. In comparison, the 3 year survival for patients with tumors at any other sites was 60%, and for tumors >200 ml, 55%. CONCLUSIONS: Overall survival progressively improved. From 1994 to 1998 the survival of patients with small tumors and/or favorable sites was similar to the best reported results. It was not possible to compare results by tumor size for large tumors, > 500 ml, due to the absence of data from elsewhere. A better staging system is required for the international comparison of results.

Metastatic Ewing sarcoma/PNET of bone at diagnosis: prognostic factors--a report from Saudi Arabia.

BACKGROUND: To evaluate outcome and prognostic factors in Saudi Arabian patients with metastatic Ewing sarcoma and PNET of bone (PMES) at diagnosis. PROCEDURE: Ninety-nine of 304 (33%) consecutive patients with Ewing sarcoma and PNET of bone registered at our centre from 1975 to 1998, had metastatic disease at registration and 93 were available for analysis. The maximum x-axis diameter of the primary tumor was used as the measure of primary tumor size. Usually a trial of systemic treatment was undertaken before a decision was made on local treatment. Standard chemotherapy regimens were used in all treated patients. Forty-one (44%) patients did not receive radical local treatment due to an inadequate response to chemotherapy, or a decision not to undertake more than palliative treatment. Radical treatment of the primary site was radiation alone 41 (79%), resection alone 7 (13%), and resection and radiation 4 (8%). RESULTS: The 5-year survival rates were 9% for all 93 evaluable patients, 16% for 52 patients who received chemotherapy and radical local treatment, 0% for 41 patients who received lesser treatment, 19% for 43 patients with lung metastases alone, and 0% (P = 0.002) for 50 patients with other sites involved. For 60 patients with imaging data, 5-year survivals were 34 and 0% when the maximum transverse diameter of the primary tumor was < 10 cm (N = 20) and > or = 10 cm (N = 40), respectively. CONCLUSIONS: Small primary tumor size and the presence of lung metastases alone were the only significant favorable prognostic factors. Earlier diagnosis will be the basis for better results.

Bone marrow transplantation from matched siblings in patients with fanconi anemia utilizing low-dose cyclophosphamide, thoracoabdominal radiation and antithymocyte globulin.

Nineteen patients with Fanconi anemia (FA) and bone marrow failure underwent bone marrow transplantation (BMT) from matched siblings. Median age at BMT was 8.7 years. Conditioning consisted of low-dose cyclophosphamide (CY 5 mg/kg x 4 days) and thoracoabdominal irradiation (TAI 400 cGy). Graft-versus-host disease (GVHD) prophylaxis was cyclosporin A (CsA) in 13 patients and CsA plus methotrexate in 6 patients. Antithymocyte globulin (ATG) was added in the pretransplant as well as the post-transplant period. All patients received high-dose acyclovir from day 2 pre-BMT to day 28 post BMT, and intravenous immunoglobulins (IVIG), 500 mg/kg weekly from day 7 pre-BMT to day 90 post BMT. No fungal prophylaxis was given. All patients engrafted, (median, 14 days for an absolute neutrophil count > or =0.5 x 10(9)/l; median, 37 days for platelet count > or =20 x 10(9)/l). Fourteen (74%) patients are alive with sustained engraftment and are transfusion independent. Three (16.6%) patients developed acute GVHD; none developed chronic GVHD. Five (26%) patients developed invasive fungal infections, and two (10%) developed fatal CMV disease. We believe the addition of ATG may have contributed to the increased incidence of severe life-threatening fungal and viral infections in our series.