The Prevalence of Lewis, Lutheran, and P1 Antigens and Phenotypes in Southwestern Saudi Arabia.

BACKGROUND: Inherited hemoglobinopathies are common in Jazan Province, Saudi Arabia, and some patients may frequently require a blood transfusion. Therefore, the provision of compatible units using extended phenotypes is necessary to preclude the risk of alloimmunization. This study aimed to investigate the frequencies of the Lewis (LE), Lutheran (LU), and P1 antigens, as well as determine the prevalence of LE and LU phenotypes. METHODS: This study collected 150 blood samples from Saudi Arabian anonymous volunteering blood donors at Prince Muhammed bin Nasser Hospital in Jazan Province, Saudi Arabia. Serotyping was performed using antigen profile-II based on gel card technology to determine LE, LU, and P1 antigens. RESULTS: The prevalence of antigens was as follows: Lea (n = 37, 24.6%), Leb (n = 87, 58%), Lua (n = 6, 4%), Lub (n = 150, 100%), and P1 (n = 120, 80%). Regarding the LE phenotypes, Le (a+b-) was 24.7%, Le (a-b+) was 58%, and Le (a-b-) was 17.3%. The frequencies of only observed LU phenotypes Lu (a-b+) and Lu (a+b+) were 96% and 4%, respectively. CONCLUSIONS: In summary, this study reports LE, LU, and P1 antigen prevalence. Moreover, LE and LU phenotype frequencies were investigated. This study may help establish a national database of blood group antigens in Jazan Province, Saudi Arabia. Additionally, it may provide better transfusion practice to avoid the alloimmunization risk.

Depression and Quality of Life Among Caregivers of Pediatric Cancer Patients.

Background This study aimed to assess the prevalence of depression, depressive symptoms, and quality of life among caregivers of pediatric cancer patients and the associated risk factors. Methodology In total, 73 participants were recruited for this cross-sectional study in King Abdullah Specialist Children Hospital. Two self-administered questionnaires were used, the Patient Health Questionnaire 9 (PHQ9) and World Health Organization Quality of Life (WHOQOL), to assess the depressive symptoms as well as the prevalence of clinical depression and quality of life, respectively. Data were analyzed using SPSS (IBM Corp., Armonk, NY, USA) to assess the level of depression and quality of life and the associated factors using Fisher's exact and Mann-Whitney tests. Results It was found that 90.4% were females and 9.6% were males, with 49.3% being between the ages of 31 and 40. Regarding the level of depression, 47.80% had mild depression. There were no significant associations between the baseline characteristics and the level of depression. Gender was significantly associated with all four domains of quality of life, age was significant in physical health and environmental domains, duration of illness was significant only in the physical health domain, while education level was found to be not significantly associated with any of the domains. Conclusions This study found that nearly half of the participants had mild levels of depression, and the four domains of the WHOQOL were significantly affected by several risk factors. We recommend further research into this topic with larger sample sizes, as well as a follow-up assessment of caregivers for a more accurate representation of caregivers' depression and quality of life. We recommend that in addition to the assessment of pediatric cancer patients, caregivers must also be assessed due to the burden associated with the task of being a caregiver.

Effect of newly Developed Resin Cements and Thermocycling on the Strength of Porcelain Laminate Veneers.

AIM: The aim of this study was to determine the effect of different luting cements and accelerated artificial aging (AAA) in the fracture resistance of porcelain laminate veneers (PLVs). MATERIALS AND METHODS: A total of 80 disc-shaped specimens were prepared using computer-aided design/computer-aided milling technology from lithium disilicate glass-ceramic blocks. Specimens (0.5 mm thick, 10 mm diameter) were divided into eight groups of 10 specimens per group. The control groups consisted of specimens without cement and not subjected to AAA (CN group) and specimens prepared without cement but subjected to AAA (CW group). The experimental groups were subjected to AAA and cemented with Variolink Veneer, Variolink Esthetic LC, Variolink Esthetic DC, RelyX Unicem, RelyX Veneer, or RelyX Ultimate. Specimens were individually tested for biaxial flexure on a universal testing machine. One-way analysis of variance and the Tukey's post hoc test were used to compare the groups' significance statistically (α = 0.05). RESULTS: The loads to fracture (LTF) values in the CN group were higher than those in the CW and experimental groups. The lowest LTF value was in the CW group (31.5 ± 9.5 N) and the highest LTF value in the CN group (56.7 ± 10.6 N). Tukey's post hoc test demonstrated a statistically significant (p < 0.01) difference between the CN group and the other groups. CONCLUSION: Artificial aging had a significant effect on the LTF value of the tested specimens compared with the resin cements used. Cohesive failure within the PLVs was the most common mode of failure. CLINICAL SIGNIFICANCE: Fatigue strength of dental ceramics and moisture was shown to affect the mechanical properties of all-ceramic restorations. All-ceramic material is extremely sensitive to humidity and thermocycling.

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.

Extramedullary myeloid tumors in children: the limited value of local treatment.

PURPOSE: To determine the incidence of extramedullary tumors (EMT) in Saudi Arabian children with acute myeloid leukemia, the factors associated with these tumors and the impact of local treatment on local tumor control, complete remission and survival rates. PATIENTS AND METHODS: One hundred children, median age 6 years, who received their primary treatment for acute myeloid leukemia at King Faisal Specialist Hospital and Research Center, from 1983 to 1997 were studied. EMT at diagnosis occurred in 18 (18%) patients at 25 sites. Meningeal leukemia, hepatosplenomegaly, lymph node enlargement, gingival hypertrophy, and cutaneous infiltration were not included in the definition of EMT. With these exclusions, children with EMT were younger than those without EMT (median age, 3.5 v. 7.5 years) and were more likely to have meningeal leukemia at diagnosis (33% v. 10%). The t(8;21) translocation was associated with a 47% EMT incidence compared with 23% without the translocation. Local radiation treatment was given to 16 of 25 (64%) EMT sites. RESULTS: The overall 5-year survival rate for all patients was 28%, and this was not significantly influenced by the drug regimen used, meningeal leukemia at diagnosis, the presence of the (8;21) translocation, M4 and M5 morphology combined, or EMT at diagnosis. Significant differences were observed in the 5-year survival rates for patients who underwent allogeneic bone marrow transplantation (52%; N = 37) and those who attained complete remission (CR) but did not undergo transplantation (21%; N = 44) and those who did not achieve complete remission with initial therapy (5%; N = 19). Systemic and local EMT CR was achieved in 17 of 18 patients with EMT, including 12 patients who underwent radiation treatment and 5 of 6 of those who did not. Isolated relapse was not seen at an EMT site and was not noted at any later stage of the disease. CONCLUSIONS: Permanent local control at sites of EMT was achieved in all patients who attained a bone marrow CR, whether or not the site was irradiated. Local radiation treatment of an EMT site did not appear to contribute to overall CR and survival rates. The use of radiation treatment should be conservative and limited to patients in whom there is a real and immediate threat to vision or renal function or when the spinal cord is compromised.

Hypocalcemia due to hypoparathyroidism in beta-thalassemia major patients.

BACKGROUND: This is a retrospective analysis of case records of AA(2)-thalassemia major patients who developed hypoparathyroidism (HPT). The objective of this study was to assess the prevalence of hypocalcemia and hypoparathyroidism in AA(2)-thalassemia major patients being followed at King Khalid University Hospital (KKUH), Riyadh, Saudi Arabia. PATIENTS AND METHODS: Diagnosis was based on low serum calcium (S/Ca), high serum phosphate (Po4), normal serum magnesium and alkaline phosphatase, and low serum parathyroid hormone levels. Other parameters analyzed included age, sex, serum ferritin levels, age of onset of HPT, any symptoms of hypocalcemia, and presence of other complications in these patients. RESULTS: Out of 40 patients, eight (20%) were diagnosed to have HPT. The mean age at diagnosis was 13.6 years (range 11-16 years), mean serum calcium was 1.88 mmol/L (range 1.58-2.04), mean serum ferritin was 7490 AA(1/4)g/L (range 2000-23,064) and mean serum phosphate was 1.88 mmol/L (range 1.50-2.73). Serum parathyroid hormone (PTH) levels were low in most of the patients. Only two patients (25%) had mild symptoms of hypocalcemia. Growth retardation was present in all patients, while four patients had liver dysfunction, two had diabetes mellitus and two had cardiac dysfunction. CONCLUSION: HPT due to iron overload may develop in a significant number of thalassemia major patients, especially when chelation therapy is not optimal, therefore, all thalassemics should be carefully watched for this complication from early in their second decade.

Epstein-Barr virus-associated leiomyosarcoma of the thyroid in a child with congenital immunodeficiency: a case report.

We report an unusual case of multifocal leiomyosarcoma involving the thyroid gland, liver, and right lung in a child with congenital immunodeficiency disease. The smooth muscle nature of these neoplasms was confirmed by immunohistochemistry and electron microscopic studies. In situ hybridization showed large amounts of Epstein-Barr virus messenger RNA within the tumor cells. Although Epstein-Barr virus-associated smooth muscle tumors have been reported in children with AIDS and after organ transplantation, we are unaware of any case report in congenital immunodeficiency disease.

Piracetam for the treatment of sickle cell disease in children- a double blind test.

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Laparoscopic cholecystectomy: The treatment of choice for cholelithiasis in infancy and childhood.

Twelve consecutive laparoscopic cholecystectomies (LC) were performed between January 1994 and October 1996 at King Khalid University Hospital. In all patients the indication for cholecystectomy was symptomatic gallstones. Among the 12 children, six had sickle cell disease. The operating time ranged between 65 and 135 minutes (mean=897plusmn;21.06). There was no major morbidity or mortality. The average duration of postoperative parenteral analgesia (pethidine hydrochloride) required was 0.47+/-0.19 day (ranged between 0.3 and one day). The average postoperative stay was 1.67+/-0.44 days (ranged between 1 and 2.5 days). In conclusion, LC is safe, effective and the preferred approach for cholelithiasis in children, with the advantages of short postoperative analgesia requirement, shorter hospitalization, and therefore, an early return to normal daily activities.

Hepatitis C virus infection in two groups of paediatric patients: one maintained on haemodialysis and the other on continuous ambulatory peritoneal dialysis.

Seropositivity to hepatitis C virus (HCV) was evaluated in three groups of Saudi children. One group (n = 18) was maintained on haemodialysis and another group (n = 21) on continuous ambulatory peritoneal dialysis (CAPD). The third group were community-based normal controls. The prevalence of antibody to HCV (anti-HCV) in children on haemodialysis (11.2%) was significantly higher than that in the control group (n = 220) (1.4%; p = 0.056). There was no significant difference in the prevalence of anti-HCV between children on CAPD (4.8%) and the control group (1.4%; p = 0.244). Among ten children on haemodialysis who were anti-HCV-negative 4 years earlier, two seroconverted and the seroconversion was not due to transfused blood but was most likely due to environmental contamination. This is the first report on the prevalence of anti-HCV in children maintained on CAPD. The results of the study emphasize the need for separate dialysis machines for anti-HCV-positive patients. It seems that CAPD therapy might reduce transmission of HCV but a large number of CAPD patients will need to be studied to confirm superiority to haemodialysis in this respect.

Hepatitis E virus infection in patients from Saudi Arabia with sickle cell anaemia and beta-thalassemia major: possible transmission by blood transfusion.

The seroprevalence of antibodies against hepatitis E virus (HEV) and hepatitis C virus (HCV) was investigated in Saudi children with sickle cell anaemia (SCA) (50 patients: 28 boys, 22 girls; age range 2-14 years) and beta-thalassemia major (28 patients: 12 boys, 16 girls; age range 2-12 years). The SCA patients were from the Gizan area (South) while the thalassemics were from the Riyadh area (Central province). The prevalence of hepatitis E virus antibody (HEVAb) in patients with SCA (18.0%) and in those with beta-thalassemia major (10.7%) was higher than in the control groups (5.5% and 2.8%) but this did not reach the level of statistical significance. In contrast to the situation with HEV, hepatitis C virus antibody (HCVAb) positivity was significantly higher in patients with SCA (16.0%) and in thalassemics (57.1%) than in the respective control groups. Although the difference in HEV seropositivity between beta-thalassemia major, SCA patients and their respective controls is not statistically significant, the possibility of blood-borne HEV in the Saudi population cannot be excluded. Further investigations using HEV-specific polymerase chain reaction techniques are required to confirm whether transmission of HEV through blood preparations or transfusion is possible.

Hereditary bleeding disorders in Riyadh, Saudi Arabia.

The result of an eight-year retrospective analysis of patients with hereditary bleeding disorders (HBD) at King Khalid University Hospital, Riyadh, is presented. One hundred and sixty-eight patients referred for investigation for suspected bleeding disorders had bleeding symptoms which fulfilled the criteria for HBD and were categorized as follows: 1) coagulation factor deficiencies: 41 patients had hemophilia A, while 16 had hemophila B; two patients each had factors XI and XII deficiency; four patients each had factors V and VIII deficiency and one patient had factor VII deficiency. There were two patients with dysfibrinogenemias and one with afibrinogenemia. 2) Von Willerbrand's disease was the second most common cause of HBD-25 patients were encountered in 15 different families. 3) Qualitative platelet disorders consisted of Glanzmann's thrombasthenia, with 18 patients, Bernard-Soulier disease, with five patients, and other qualitative platelet disorders, with 33 patients. 4) In 14 patients who presented with a history of bleeding, the only abnormality noted was prolongation of the bleeding time and normal coagulation and platelet function, and no definitive diagnoses could be established. The distribution of hereditary bleeding disorders obtained in this study resembles what has already been established in Western countries, with the exception of an increase of platelet disorders, mostly due to the increased rate of consanguinity in the community.

Piracetam is useful in the treatment of children with sickle cell disease.

The management of children suffering from sickle cell disease [sickle cell anaemia (SCA) and sickle cell beta degree-thalassaemia (S beta degree-thal.)] has been the concern of all clinicians caring for these patients. Several agents have been tried for treatment, often limited by toxic side effects. Piracetam (2-oxo-l-pyrrolidine acetamide, Nootropyl), a cyclic derivative of gamma-amino butyrate, used for the treatment of psychosenescent syndromes with no known side effects, was considered as a possible therapeutic agent for sickle cell disease. Interest was focused on the use of piracetam when it was shown that it had an antisickling effect, both in vivo and in vitro. We initiated multicentre double-blind investigations in two groups of children suffering from sickle cell disease ranging in age from 3-6 to 6-12 years. The total number of patients included in the study were 87 (SCA = 79 and Hb S beta degree-thal. = 8) in 13 centres in 10 different regions of Saudi Arabia. Coded boxes of the drugs were received from the company (UCB) and were administered as intravenous infusion during crises and orally during the follow-up, for a period of up to 1 year. After decoding the code at the end of the study, the patients were grouped into those receiving placebo (n = 39), i.e. controls, or piracetam (n = 48), i.e. study cases. In terms of age, weight, height and severity index, number of blood transfusions received and number of hospitalization, both groups were statistically homogenous. Data analysis showed that the clinical severity of the disease, the number of crises, the extent of hospitalization and the blood transfusion requirements significantly decreased during piracetam treatment (p < 0.001), though no statistically significant changes occurred in the placebo group. However, in the levels of the haematological and biochemical parameters no significant changes were documented in both groups. In addition, the improvement in the clinical presentation of the disease continued even several months after discontinuation of the drug in the majority of the children, as judged from the low severity index value. Though our results point to the recommendation that piracetam can be used for the treatment of children suffering from sickle cell disease, both SCA and S beta degree-thal, it is advisable to conduct long-term and close follow-up treatment programmes using piracetam to establish its therapeutic value particularly in adults and to ascertain that there are no long-term toxic side effects.

Acute viral hepatitis, glucose-6-phosphate dehydrogenase deficiency and prolonged acute renal failure: a case report.

A 10-year-old boy with glucose-6-phosphate dehydrogenase deficiency developed acute renal failure during the icteric phase of non-fulminant hepatitis A infection. He needed peritoneal dialysis for 54 days. Acute tubular necrosis was confirmed by percutaneous renal biopsy. He had complete recovery of his renal function when he was discharged.

Infection with hepatitis viruses (B and C) and human retroviruses (HTLV-1 and HIV) in Saudi children receiving cycled cancer chemotherapy.

Serological markers of hepatitis B virus (HBV), hepatitis C virus (HCV), human T-cell lymphotropic virus type 1 (HTLV-1), and human immunodeficiency viruses (HIV-1 and HIV-2) were studied in 53 Saudi children (31 males, 22 females; 1-12 years of age) receiving cycled cancer chemotherapy and in 168 healthy Saudi children taken as control. Exposure to HBV in the patients was similar to that in the control (6 per cent HBsAg in patients v. 7 per cent in the control; 19 per cent exposure rate in patients v. 20 per cent in control). None of our patients was vaccinated against HBV prior to chemotherapy. The fact that among the 10 HBV exposed patients five patients were anti-HBs-positive is in favour of vaccinating Saudi oncology patients against HBV prior to chemotherapy. In contrast to the situation with HBV the prevalence of anti-HCV in the patients (11 per cent) was significantly higher than that in the control (1 per cent) (P = 0.003). None of our patients or the control were anti-HTLV-1 or anti-HIV-positive. The results of this study stress the need for an awareness of HCV problem in Saudi oncology patients. Strict measures of screening blood donors for all blood-borne viruses and, in particular, for HCV in addition to the use of disposable equipment in management of cancer patients are items that should be implemented as soon as possible.