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Insulin Therapy For Diabetic Ketoacidosis, Abdelaziz Elamin, Latif A. Khan
Insulin Therapy For Diabetic Ketoacidosis, Abdelaziz Elamin, Latif A. Khan
Saudi Medical Journal
No abstract provided.
Forensic Pathology, 2nd Edition, Mohamed K. Ahmed
Forensic Pathology, 2nd Edition, Mohamed K. Ahmed
Saudi Medical Journal
No abstract provided.
Impact Of A Mini-Clinic On Diabetic Care At A Primary Health Care Center In Southern Saudi Arabia, Yahia M. Al-Khaldi, Mohd Y. Khan
Impact Of A Mini-Clinic On Diabetic Care At A Primary Health Care Center In Southern Saudi Arabia, Yahia M. Al-Khaldi, Mohd Y. Khan
Saudi Medical Journal
OBJECTIVE: The aim of this study was to assess the impact of a mini-clinic on the quality of diabetic care at a Primary Health Care Center in Aseer region, Kingdom of Saudi Arabia. METHODS: All the files of diabetics in Wasat Abha Primary Health Care Center were reviewed at the end of 1997 for diabetic process based on a scoring system of 11 items. Diabetic outcomes were evaluated in accordance with Quality Assurance Protocol. Data of all the files was entered into and analyzed by Statistical Package for Social Sciences. Relevant statistical tests were used. RESULTS: Files of 198 patients …
Current Concepts In Gastric Cancer Surgery, Ingo K. Schumacher, Andreas Hunsicker, Pierre S. Youssef, Dietmar Lorenz
Current Concepts In Gastric Cancer Surgery, Ingo K. Schumacher, Andreas Hunsicker, Pierre S. Youssef, Dietmar Lorenz
Saudi Medical Journal
OBJECTIVE: Current problems in gastric cancer surgery concern the extent of gastric resection, the need for abdominal evisceration, the degree of lymphadenectomy, and an optimal preoperative tumor staging procedure. METHODS: A retrospective clinical trial of 284 patients who underwent surgery at Ernst-Moritz-Arndt-University, Greifswald, Germany for gastric cancer between 1987 and 1996. Main outcome measures consist of epidemiological parameters, data on type of surgery, histopathology, postoperative complications, mortality and cancer survival. Statistical analysis between groups was performed using Chi square test (perioperative risk factors, tumor localization, and surgical treatment) and Mann Whitney U tests (Lauren classification). Survival was calculated according to …
Eating Patterns And Prevalence Of Obesity Among Arabs Living In Ahwaz, Iran.
Eating Patterns And Prevalence Of Obesity Among Arabs Living In Ahwaz, Iran.
Saudi Medical Journal
No abstract provided.
Acute Eosinophilic Pneumonia Simulating Severe Community Acquired Pneumonia, Ahmed S. Bahammam
Acute Eosinophilic Pneumonia Simulating Severe Community Acquired Pneumonia, Ahmed S. Bahammam
Saudi Medical Journal
Idiopathic acute eosinophilic pneumonia is a recently described cause of acute respiratory failure. It usually affects young healthy individuals. Usually non-pulmonary organs are not involved. Bronchoalveolar lavage eosinophilia is required for diagnosis. This disease responds uniformly to a short course of corticosteroids and does not recur. We report a young man who presented with a 2-day history of acute respiratory failure simulating severe community acquired pneumonia and necessitating mechanical ventilation. The diagnosis was made based on the classical clinical presentation and bronchoalveolar lavage and peripheral blood eosinophilia. Acute eosinophilic pneumonia in our patient was associated with acute hepatitis and erythema …
Combined Brucella Melitensis And Streptococcal Viridans Endocarditis, Tarig S. Al-Khuwaitir, Bashir A. Wani, Suphia M. Sherbeeni
Combined Brucella Melitensis And Streptococcal Viridans Endocarditis, Tarig S. Al-Khuwaitir, Bashir A. Wani, Suphia M. Sherbeeni
Saudi Medical Journal
Infectious endocarditis is a potentially lethal inflammation of the hearts9 inner lining invaded by microorganisms. The mortality from this illness increases as the number of infective organisms rises to 2, due to involvement of the left side of the heart. These microorganisms usually arise from the patient9s own flora but can be acquired from the environment. Fever and heart murmurs are the principal clinical manifestations followed by a plethora of peripheral signs due to dissemination of microorganisms via the bloodstream. Echocardiographic imaging and sensitive culture techniques form the cornerstone of diagnosis. We report a patient with rheumatic heart disease who …
Utilization Of Emergency Services In A Community Hospital, Saima Siddiqui, Danny O. Ogbeide
Utilization Of Emergency Services In A Community Hospital, Saima Siddiqui, Danny O. Ogbeide
Saudi Medical Journal
OBJECTIVE: Over utilization of emergency room services by patients with non-urgent complaints is a global problem. It results in a waste of resources, stress among the emergency room staff and an increase in waiting time for patients requiring attention. This study was carried out to establish the extent of inappropriate emergency room attendance in a Saudi community. METHODS: Data was collected from the emergency room register, regarding the age, sex, presenting complaints, time of presentation and disposal of patients, from January 1st 1999 to March 31st 1999 at Al-Kharj Military Hospital, Al-Kharj, Kingdom of Saudi Arabia. Patients were classified in …
A Modified Method Of Submental Oroendotracheal Intubation, Alagumba L. Nwoku, Saeed A. Al-Balawi, Saeed A. Al-Zahrani
A Modified Method Of Submental Oroendotracheal Intubation, Alagumba L. Nwoku, Saeed A. Al-Balawi, Saeed A. Al-Zahrani
Saudi Medical Journal
OBJECTIVE: The aim of this paper is to present a modification of submental intubation in the management of airway. METHODS: Following usual oroendotracheal intubation, the tube and cuff are exteriorized through a laterosubmental incision, and connected to the anesthetic machine. RESULTS: Ten patients comprising 8 panfacial fractures and 2 orthognathic surgical cases have successfully been managed with this approach. There were no complications, no morbidity, and the scar was inconspicuous and esthetically pleasing. CONCLUSION: The laterosubmental intubation technique is simple, efficacious in the management of severe maxillofacial injuries, orthognathic surgical cases with nasal obstruction and in plastic surgery for concomitant …
Urinary Tract Infection, Ahmed N. Ghanem
Long-Term Follow Up Of Carbonic Anhydrase Ii Deficiency Syndrome, Mohammad Awad, Abdullah A. Al-Ashwal, Nadia Sakati, Abbas A. Al-Abbad, Bassam S. Bin-Abbas
Long-Term Follow Up Of Carbonic Anhydrase Ii Deficiency Syndrome, Mohammad Awad, Abdullah A. Al-Ashwal, Nadia Sakati, Abbas A. Al-Abbad, Bassam S. Bin-Abbas
Saudi Medical Journal
OBJECTIVE: To describe the long term clinical, biochemical and radiological features of 35 Saudi Arabian children with carbonic anhydrase II deficiency syndrome who have been followed at King Faisal Specialist Hospital and Research Center, Riyadh since 1979. METHODS: The records of these patients were retrospectively evaluated. The diagnosis was based on the clinical and the radiological evidence of the disease. Carbonic anhydrase II level was measured in 9 patients. RESULTS: Clinically, these patients had typical facial features, growth failure and varying degrees of psychomotor retardation. Biochemically, all children had renal tubular acidosis that was of distal type in the majority …
Right Lower Quadrant Pain In Females. Is It Appendicitis Or Gynecological?, Eric A. Archibong, Mamdoh Eskandar, Adekunle A. Sobande, Oluwole G. Ajao
Right Lower Quadrant Pain In Females. Is It Appendicitis Or Gynecological?, Eric A. Archibong, Mamdoh Eskandar, Adekunle A. Sobande, Oluwole G. Ajao
Saudi Medical Journal
OBJECTIVE: To determine if a gynecological consultation is needed for patients who are labeled to have acute appendicitis. METHODS: A retrospective study carried out in Assir Central Hospital, Abha, Kingdom of Saudi Arabia. Fifteen female patients who were clinically diagnosed and operated upon for acute appendicitis were discovered intra-operatively to have unrelated gynecological lesions, are presented. RESULTS: Histopathological reports on the removed appendices revealed 80% "normal" and 20% "mildly inflamed". Seven (46.7%) of the patients had right ovarian cysts (one of them, bilateral); 4 (26.7%), corpus luteum cysts, and 4 had bilateral salpingitis, bilateral pyosalpinx, right ovarian cyst with bilateral …
The Diagnostic Challenge And Management Of Pulmonary Kaposi's Sarcoma In Renal Transplant Recipients, Ayman B. Krayem, Siraj O. Wali, Yaseen S. Samman
The Diagnostic Challenge And Management Of Pulmonary Kaposi's Sarcoma In Renal Transplant Recipients, Ayman B. Krayem, Siraj O. Wali, Yaseen S. Samman
Saudi Medical Journal
Kaposi9s sarcoma is a multicentric low grade tumor that usually begins with the development of violaceous skin lesions and is associated with the presence of human herpes virus 8. Kaposi9s sarcoma has been described in immunocompromised patients, particularly following renal transplantation, with cutaneous involvement being the most salient finding. Infectious and non-infectious pulmonary disorders in immunocompromised patients can simulate the radiological manifestations of pulmonary Kaposi9s sarcoma. This report highlights the dilemma in reaching an accurate diagnosis of pulmonary Kaposis sarcoma as a complication of immunosuppression post-renal transplant and reviews the management of immunosuppression related Kaposi9s sarcoma.
Evaluation Of Commonly Used Tribal And Traditional Remedies In Saudi Arabia, Baker H. Al-Awamy
Evaluation Of Commonly Used Tribal And Traditional Remedies In Saudi Arabia, Baker H. Al-Awamy
Saudi Medical Journal
There are several compounds used in traditional prescriptions and as cosmetics, among several tribes in the Kingdom of Saudi Arabia. Nothing pertinent in the literature exists regarding the components and health hazards of these compounds. There are no health education programs to warn the community especially high-risk populations (such as sickle cell anemia and diabetes mellitus patients) of the hazards involved in the use of the traditional preparations. Tradition and folk medicine continue to be used in many developing countries, including the Kingdom of Saudi Arabia. For physicians who are not accustomed to dealing with this entity, particularly when the …
Pulmonary Changes In Liver Transplant Candidates With Hepatitis C Cirrhosis, Mohamed S. Al-Moamary, Tanja Gorka, Ibrahim H. Al-Traif, Hamdan H. Al-Jahdali, Abdullah A. Al-Shimemeri, Bander Al-Kanway, Abdulmajed A. Abdulkareeem, Abdulmajed A. Abdulkareeem
Pulmonary Changes In Liver Transplant Candidates With Hepatitis C Cirrhosis, Mohamed S. Al-Moamary, Tanja Gorka, Ibrahim H. Al-Traif, Hamdan H. Al-Jahdali, Abdullah A. Al-Shimemeri, Bander Al-Kanway, Abdulmajed A. Abdulkareeem, Abdulmajed A. Abdulkareeem
Saudi Medical Journal
OBJECTIVE: Several studies have shown that pulmonary abnormalities are common in patients with end-stage liver disease. However, most of these studies were conducted on patients with heterogeneous etiologies. Therefore, we studied these changes in a homogenous group of hepatitis C cirrhotic patients who were potential candidates for liver transplantation. METHODS: The charts of 81 patients from King Fahad National Guard Hospital, Riyadh, Kingdom of Saudi Arabia with hepatitis C cirrhosis who were evaluated for liver transplantation were reviewed. The following data was retrieved: echocardiography with micro-bubble study, arterial blood gases, and pulmonary function tests of 81 candidates and reviewed over …
Role Of Computerized Tomography In Diagnosis Of Atypical Gall Bladder And Common Bile Duct Stones, Tareq K. El-Husseini, Ibrahim A. Al-Shebrien
Role Of Computerized Tomography In Diagnosis Of Atypical Gall Bladder And Common Bile Duct Stones, Tareq K. El-Husseini, Ibrahim A. Al-Shebrien
Saudi Medical Journal
OBJECTIVE: To assess the value of computerized tomography as an adjuvant to ultrasound in the diagnosis of atypical gallbladder and common duct stone disease. METHODS: Real time ultrasound scanning for the gallbladder and common duct was performed in the routine manner. High resolution computerized tomography images were subsequently obtained for the region of interest. RESULTS: Computerized tomography resolved undetermined results as follows: 1. Non shadowing gallbladder debris (6 points), 2. Focal gallbladder wall thickening (2 points), 3. Stone obscured by calcified gallbladder wall (3 points), 4. Non visualized gallbladder double arc shadow (4 points), 5 and 6. Impacted gallbladder neck …
An Evaluation Of Theophylline Dosing. Suggested By The Saudi National Protocol For Children With Severe Asthma, Samira I. Islam, Ahmed S. Ali, Nadia M. Fida
An Evaluation Of Theophylline Dosing. Suggested By The Saudi National Protocol For Children With Severe Asthma, Samira I. Islam, Ahmed S. Ali, Nadia M. Fida
Saudi Medical Journal
OBJECTIVE: To evaluate the impact of Saudi national protocol on the diagnosis and management of asthma for optimizing theophylline use in children with severe asthma. To also review theophylline clearance and provide guidelines for individualization of doses. METHODS: Theophylline level was estimated in blood samples of 66 children with severe asthma who were admitted to King Abdulaziz University Hospital during the period 1998-1999. The theophylline doses given to these patients was reviewed and patients were categorized into 2 groups: Group one had received doses recommended by Saudi national protocol ± 10%, group 2 had received doses <90% of that recommended by Saudi national protocol. The distribution of theophylline levels in blood samples of each group was estimated. Theophylline steady state level was used to estimate theophylline clearance using a standard pharmacokinetic equation. RESULTS: Out of the total samples from group one, 70% of theophylline levels were within therapeutic range, while only 10% of the total samples of group 2 were within therapeutic range. The mean theophylline clearance in children (1-8 years) was estimated as 0.092 ± 0.023 and was found significantly higher than the mean theophylline clearance (0.069 ± 0.014) which is observed in older children (9-13 years). CONCLUSION: Saudi national protocol theophylline dose guidelines had a favorable impact on the optimization of theophylline use in children with severe asthma. Guidelines that ensure accurate adjustment of doses on individual basis in view of drug level were suggested.
Screening For Hypertension. Assessing The Knowledge, Attitudes And Practice Of Primary Health Care Physicians In Riyadh, Saudi Arabia, Ahmad S. Al-Khashman
Screening For Hypertension. Assessing The Knowledge, Attitudes And Practice Of Primary Health Care Physicians In Riyadh, Saudi Arabia, Ahmad S. Al-Khashman
Saudi Medical Journal
OBJECTIVE: The aim of this study is to assess the performance of primary health care physicians in screening people for hypertension in Riyadh, Kingdom of Saudi Arabia, by assessing their knowledge, attitudes and practice. METHODS: A cross-sectional descriptive study including 20 primary health care centers were selected randomly in Riyadh city during September 1996. The participation of 107 primary health care working doctors in these centers were invited to complete a 38 item questionnaire which contained essential knowledge regarding hypertension, screening for hypertension, their attitudes and practice in screening for hypertension. RESULTS: One 3rd of the doctors knew the correct …
The Magnitude Of Osteoporosis In Middle Aged Women, Raeda J. Al-Qutob, Salah M. Mawajdeh, Allam A. Khalil, Amy B. Schmidt, Akram O. Hannak, Basel K. Masri
The Magnitude Of Osteoporosis In Middle Aged Women, Raeda J. Al-Qutob, Salah M. Mawajdeh, Allam A. Khalil, Amy B. Schmidt, Akram O. Hannak, Basel K. Masri
Saudi Medical Journal
OBJECTIVE: This study aims to increase the body of knowledge regarding osteoporosis in the Jordanian context by investigating the magnitude of osteoporosis amongst perimenopausal women, as well as the risk factors associated with the disease. METHODS: Two hundred and fifty one women between the ages of 40 to 60 years were randomly selected from the Orthopedics and Rheumatology out patient clinics at the Major Teaching Hospital in Amman, Jordan. A structured questionnaire, which focused upon risk factors and preventive measures for osteoporosis, was administered by a trained staff nurse to all women. Index women were then referred to a private …
Pityriasis Lichenoides Et Varioliformis Acuta In Pregnancy, Mamdoh A. Eskandar
Pityriasis Lichenoides Et Varioliformis Acuta In Pregnancy, Mamdoh A. Eskandar
Saudi Medical Journal
Pityriasis lichenoides et varioliformis acuta is an uncommon disease, especially during pregnancy. In review of the obstetric literature, there was no report of pityriasis lichenoides et varioliformis acuta during pregnancy. A 25-year-old female was seen at 24 weeks of gestation for consultation about a cutaneous disease. She was admitted at 30 weeks of gestation because of threatened premature labor, and some active cutaneous papules presented themselves at that time. After the treatment, cutaneous papules remitted. But at 35 weeks of gestation, she had spontaneous labor. Both the mother and infant were doing well at 5 months postpartum. If pityriasis lichenoides …
Lingual Granuloma Gravidarum, Othman M. Elmustafa, Ahmed A. Badie
Lingual Granuloma Gravidarum, Othman M. Elmustafa, Ahmed A. Badie
Saudi Medical Journal
Granuloma gravidarum or pregnancy tumor is a benign lesion of the gingival mucosa which occurs during pregnancy. It is a pyogenic granuloma. This case report presents the clinical, histopathological features and the treatment of a grauloma gravidarum in a Sudanese patient in the puerperium.
The Microbiology Of Nasal Nostril Area In Healthy Muslim Prayers, Khalifa E. Sharquie, Jamal R. Al-Rawi, Sarmad A. Al-Hashimi, Zuhair I. Al-Ani
The Microbiology Of Nasal Nostril Area In Healthy Muslim Prayers, Khalifa E. Sharquie, Jamal R. Al-Rawi, Sarmad A. Al-Hashimi, Zuhair I. Al-Ani
Saudi Medical Journal
No abstract provided.
Cushing's Syndrome And Adrenal Suppression From Percutaneous Absorption Of Clobetasol Propionate In Infants, Mathew P. Cherian, Mohammad Abduljabbar
Cushing's Syndrome And Adrenal Suppression From Percutaneous Absorption Of Clobetasol Propionate In Infants, Mathew P. Cherian, Mohammad Abduljabbar
Saudi Medical Journal
No abstract provided.
Use Of Nucleic Acid Testing For Blood Donor Screening Of Human Immunodeficiency Virus And Hepatitis C Virus In The Saudi Population, Javed Akhter, George T. Roberts, Angela Perry, Julie S. Gaucher, Peter A. Howman
Use Of Nucleic Acid Testing For Blood Donor Screening Of Human Immunodeficiency Virus And Hepatitis C Virus In The Saudi Population, Javed Akhter, George T. Roberts, Angela Perry, Julie S. Gaucher, Peter A. Howman
Saudi Medical Journal
OBJECTIVE: To determine the risk of transfusion associated infection for human immunodeficiency virus and Hepatitis C virus using nucleic acid testing. METHODS: During March 1998, 400 donor blood samples from the Saudi population that were negative by serology were further tested for human immunodeficiency virus 1 and 2 and Hepatitis C virus using nucleic acid testing. RESULTS: A total of 400 were tested by nucleic acid testing, 381 of these were negative, 4 were indeterminate but were found to be negative on repeat testing and one seronegative sample was found to be positive for Hepatitis C virus. CONCLUSION: Due to …
Acute Splenic Sequestration Crisis In Children With Sickle Cell Disease, Zakaria M. Al-Hawsawi, Ghousia A. Ismail
Acute Splenic Sequestration Crisis In Children With Sickle Cell Disease, Zakaria M. Al-Hawsawi, Ghousia A. Ismail
Saudi Medical Journal
OBJECTIVE: The aim of this study was to demonstrate the clinical experience with acute splenic sequestration crisis in children with sickle cell disease, followed in Madina Maternity & Children9s Hospital, Madina Al-Munawara, Kingdom of Saudi Arabia. METHODS: A retrospective review of hospital case notes of all children with acute splenic sequestration in sickle cell disease, was carried out in the Pediatric Hematology unit at Madina Maternity & Children9s Hospital between 1993 through to 2000. RESULTS: One hundred and twenty children with sickle cell disease were registered and followed in Madina Maternity & Children9s Hospital. Out of these, 8 had acute …
The Protective Effect Of Garlic Oil On Hepatotoxicity Induced By Acetaminophen In Mice And Comparison With N-Acetylcysteine, Heybatuallah Kalantari, Majeed Salehi
The Protective Effect Of Garlic Oil On Hepatotoxicity Induced By Acetaminophen In Mice And Comparison With N-Acetylcysteine, Heybatuallah Kalantari, Majeed Salehi
Saudi Medical Journal
OBJECTIVE: The aim and purpose of this study was to find out the protective effect of garlic oil in liver toxicity induced by acetaminophen overdose and the comparison of its effect with N-acetylcysteine in albino male mice (18-22g). METHODS: This study was undertaken during the period from January 1999 through to August 2000, at the School of Pharmacy, Ahwaz University of Medical Sciences, Ahwaz, Iran. All animals were fasted over night and were divided into 8 groups. Each group consisted of 10 mice. Garlic oil was administered intraperitoneally in doses of 100mg/kg, 200mg/kg and 500mg/kg. Immediately after this, a toxic …
Profile Of Smoking Amongst Health Staff In A Primary Care Unit At A General Hospital In Riyadh, Saudi Arabia, Saima Siddiqui, Danny O. Ogbeide
Profile Of Smoking Amongst Health Staff In A Primary Care Unit At A General Hospital In Riyadh, Saudi Arabia, Saima Siddiqui, Danny O. Ogbeide
Saudi Medical Journal
OBJECTIVE: Smoking is a major health problem among healthcare personnel as in other parts of the community. The objective of this study was to find out the prevalence of smoking along with influencing factors related to smoking among our staff in Alkharj Military Hospital. METHODS: A self-administered questionnaire study. EPI-INFO statistical package was used for statistical analysis by calculating x2 and p value
Empirical Treatment Of Uncomplicated Urinary Tract Infection By Community Pharmacist In The Eastern Province Of Saudi Arabia, Mastour S. Al-Ghamdi
Empirical Treatment Of Uncomplicated Urinary Tract Infection By Community Pharmacist In The Eastern Province Of Saudi Arabia, Mastour S. Al-Ghamdi
Saudi Medical Journal
OBJECTIVE: It is a well known phenomenon in the Kingdom of Saudi Arabia that prescription drugs are dispensed over the counter in the community pharmacies. The aim of this study is to document the attitude of community pharmacists to fulfill the concept of pharmaceutical care and to evaluate how they manage a case of acute uncomplicated lower urinary tract infection. METHODS: Eighty-eight community pharmacists in the Eastern province of Saudi Arabia were presented with a patient claiming urinary tract infection and seeking medical treatment. RESULTS: Only one attendant pharmacist refused to dispense medications without prescription. Fifteen others (17%) dispensed urinary …
Prevention Of Postpartum Hemorrhage, Safety And Efficacy, Adnan A. Abu-Omar
Prevention Of Postpartum Hemorrhage, Safety And Efficacy, Adnan A. Abu-Omar
Saudi Medical Journal
OBJECTIVE: This study was carried out to describe the safety and efficacy of intramuscular syntometrine (oxytocin plus ergometrine) compared to intravenous oxytocin for prevention of postpartum hemorrhage, and the difference between administration at the end of the 2nd stage of labor compared with that after delivery of the placenta. METHODS: A prospective study was carried out at Prince Zaid Ben Al-Hussein Hospital, Tafilah, Jordan. Two thousand one hundred and sixty one women delivering singletons during 12 consecutive months were included in our study. Women received either intramuscular syntometrine (oxytocin plus ergometrine) or oxytocin alone. The drugs were used either before …
A New Autosomal Recessive Syndrome. Early Onset Of Pancytopenia, Distinct Facial Features, Growth Retardation And Developmental Delay, Fatma S. Al-Batniji, Madeha A. Mahmoud, Rizgullah H. Al-Asiri, Abdulrahman F. Al-Swaid, Abdulrahman M. Al-Marshedy
A New Autosomal Recessive Syndrome. Early Onset Of Pancytopenia, Distinct Facial Features, Growth Retardation And Developmental Delay, Fatma S. Al-Batniji, Madeha A. Mahmoud, Rizgullah H. Al-Asiri, Abdulrahman F. Al-Swaid, Abdulrahman M. Al-Marshedy
Saudi Medical Journal
The association of dysmorphic features and failure of one or more bone marrow cell lines is well known. Examples are Fanconi9s anemia and Diamond-Blackfan anemia. This report describes 3 similarly affected children from consanguineous parents, all showing low birth weight, severe growth retardation, distinct facial features, microcephaly, mental retardation and onset of severe pancytopenia in infancy without increased chromosomal breakage. We conclude that these cases represent a new familial autosomal recessive bone marrow failure syndrome.