Multiple Myeloma: An Update 

Khalil Al-Farsi 

Abstract

Multiple myeloma is a rare, largely incurable malignant disease of plasma cells. Patients usually present with hypercalcemia, renal insufficiency, anemia and/or lytic bony lesions along with a monoclonal protein in the serum and/or urine in addition to an increase in the number of clonal plasma cells in the bone marrow. Patients with myeloma live on an average for five to seven years, with their survival dependent on the presence or absence of different prognostic markers. Treatment of younger fit patients is with induction therapy consisting of steroids with one or more novel anti-myeloma agents followed by high dose melphalan and autologous stem cell transplantation, while older and less fit patients are treated with melphalan-based combination chemotherapy. Supportive care is of paramount importance and includes the use of bisphosphonates, prophylactic antibiotics, thrombosis prophylaxis and the use of hematopoietic growth factors along with the treatment of complications of disease and its therapy. As more progress is being made and deeper responses are being attained, the disease might turn into a potentially curable one in the near future.

Does Cigarette Smoking Affect Seminal Fluid Parameters? A Comparative Study

Zakarya Bani Meri, Ibrahim Bani Irshid, Mohammad Migdadi, Ayat Bani Irshid, Somia A. Mhanna, MT 

Abstract

Objective: To study the effect of cigarette smoking on seminal fluid parameters, namely; volume, sperm concentration, and motility, as well as morphology, leukocyte infiltration, among males complaining of infertility.

Methods: Between August 2010 and July 2011, seminal fluid analysis was done for 1438 males who are partners of couples who visited the infertility clinic at Prince Rashid Ben Al Hassan Hospital (PRH) for infertility. The men who fit the inclusion criteria (n=960) were classified into two groups: group a (non-smokers; n=564) and  group B (smokers; n=396), which represents 41.25% of the study group. Seminal fluid was collected using masturbation after 3-5 days of abstinence then analyzed for volume, sperm count, sperm concentration, motility and morphology. In order to analyze whether the number of cigarettes smoked per day has an effect on the spermatogram; the smoking men were divided into two subgroups: the heavy smokers (n=266) and non-heavy smokers (n=130).

Results: A total of 960 adult males were enrolled. Their age ranged between 21 and 76 years, 564 were non-smokers with mean age of 36. 45±6.27 (Mean±SD). Three-hundred-and-ninety-six were smokers with a mean age of 34.35±4.25 (Mean±SD). There was a significant effect of smoking on the motility of sperms and the ratios of abnormality (p<0.005). Concentration appeared not to be affected by smoking. Furthermore, the group of  heavy smokers were found to have lower sperm concentrations and a higher percentage of abnormal sperms compared to the non-heavy smokers.

Conclusion: Cigarette smoking has a deleterious effect on some of the seminal fluid parameters (motility, morphology and leukocyte count) which in turn may result in male subfertility.

High-sensitivity C-reactive Protein and Tumor Necrosis Factor Alpha in Pseudoexfoliation Syndrome

Rana Sorkhabi,¹ Amir Ghorbanihaghjo,² Mohamadhossein Ahoor,¹  Mehriar Nahaei,¹ Nadereh Rashtchizadeh³

Abstract

Objectives: The purpose of the present study was to determine the alterations in high-sensitivity C-reactive protein and Tumor Necrosis factor alpha levels in the blood serum of pseudoexfoliation syndrome cases (a disease with similar risk factors as systemic endothelial dysfunction diseases) and to compare the results with healthy individuals.

Methods: High-sensitivity C-reactive protein and Tumor Necrosis factor alpha levels were determined in 30 cases with pseudoexfoliation syndrome and in 30 control patients of the same age and sex, by enzyme-linked immunosorbent assay.

Results: The levels of high- sensitivity C-reactive protein and Tumor Necrosis factor alpha in the blood serum of patients with pseudoexfoliation syndrome (3.95±0.88 mg/l, 3.32±0.99 pg/ml, respectively) were significantly higher than in the control group (2.51±0.79mg/l, 0.43±0.15 pg/ml, respectively) p=0.001, p=0.002.

Conclusion: The results suggest that increased levels of high- sensitivity C-reactive protein and Tumor Necrosis factor alpha, as  markers of inflammation and peripheral endothelial dysfunction in pseudoexfoliation syndrome, may be risk factors for systemic and ocular manifestations of pseudoexfoliation syndrome.

Keywords: Pseudoexfoliation syndrome; Tumor necrosis factor alpha; High sensitivity C-reactive protein.

Anthropometric Analysis of Palpebral Fissure Dimensions and its Position in South Indian Ethnic Adults

P. Vasanthakumar,¹ Pramod Kumar,² Mohandas Rao¹ 

Abstract

Objectives: The morphology and anatomical relationship of periorbital features vary according to age, sex and ethnicity. Standard database regarding periorbital region is available for other ethnic groups. Since there is no data available in the literature, specifically for south Indian ethnic adults, the present study was carried out to determine the normal average values for South Indian ethnic population related to gender.

Methods: Anthropometric measurements of both eyes were done on standardized frontal view photograph of 200 South Indian ethnic adults aged 18 to 26 years. Parameters included were palpebral fissure width (PFW), palpebral fissure height (PFH), palpebral fissure inclination (PFI), outercanthal distance (OCD), interpupillary distance (IPD), intercanthal distance (ICD) and comparisons were made between the genders using the independent t test.

Results: Significant sexual dimorphism was noted in the given parameters. Palpebral fissure width (male: 31.08 mm; female: 29.90 mm), palpebral fissure inclination (male: 5.053°; female: 6.102°), outercanthal distance (male: 95.55 mm; female: 92.44 mm) and interpupillary distance (male: 66.72 mm; female: 62.59 mm). The palpebral fissure height (male: 11.30 mm; female: 11.58 mm) and intercanthal distance (male: 34.27 mm; female: 33.41 mm) showed no significant sexual differences.

Conclusion: Statistically significant differences were found between South Indian ethnic males and female in certain key parameters. The present study suggests that ethnicity and gender should be considered in orbital surgery. To individualize the treatment planning and diagnosis, it is important for the surgeons to have knowledge of these local norms.

Keywords: Aesthetics; Anthropometry; Database; Orbital; Palpebral fissure.

Maternal Perceptions of Antenatal Care Provision at a Tertiary Level Hospital, Riyadh

Aneela Kamil, Ezzeldin Khorshid

Abstract

Objective: Report Maternal perceptions of antenatal care provision and identify deficiencies in the current model of care provision.

Methods: A survey was conducted to record maternal views about quality of antenatal consultations provided at a tertiary level hospital. Trained nurses and female health workers interviewed the patients attending antenatal visits during the month of July 2009. A standard questionnaire was use to enter the responses. Data were entered into the statistical package for social sciences (SPSS) for appropriate statistical analysis of the results. Data analysis generated two groups. One group had up to 4 antenatal visits and the other group had more than 4 visits.

Results: Responses were obtained for 244 patients. Chi-square test was applied for the comparison of variables between the two groups. Significantly higher number of women preferred to follow the 4 visit care plan (n=118/244 vs. n=103/244, respectively; p=0.004). Patient satisfaction was also significantly higher among women in the 4 visit group (n=112/244 vs. n=90/244; p=0.04). More than 50% of patients said that they did not receive any information about the process of labor, breast-feeding or contraception.

Conclusion: Women included in the study did not want frequent visits to antenatal clinic. Efforts should be made to provide information about labor, breast-feeding and contraception.

Keywords: Antenatal; Care provision; Maternal; Views.

Outcome of Management of Otosclerosis by Stapedotomy Compared to Stapedectomy in a Jordanian Population

Hassan Al-Husban

Abstract

Objective: To study the success results rates and complications of stapedotomy compared to stapedectomy in the operative management of otosclerosis.

Methods: This is a retrospective study of 50 patients who were treated surgically for otosclerosis. The medical records of these patients were reviewed for the type of operation performed, complications and the serial pure tune audiometries pre- and postoperatively for at least one year. Patients with conductive hearing loss not due to otosclerosis were excluded from this study. The medical records of equal number of patients (25 patients with stapedotomy and 25 patients with stapedectomy) were analyzed for hearing improvement or loss, postoperative nausea, vomiting, vertigo, nystagmus, perilymph fistula, reparative granuloma, labyrinthitis, tinnitus and perforation of the tympanic membrane. All operations were performed by the senior consultant-otologists of our department. Stapes superstructures were removed by crural scissors and stapes footplate was perforated by microdrill.

Results: Out of 25 patients with stapedotomy, 22 (88%) developed complete closure (≤10 dB) of the air-bone gap on pure tune audiometry; in two patients (8%), the air-bone gap improved to less 20 dB, and recurrent conductive hearing loss (due piston slipping) in one patient (4%). On the other hand, in patients with stapedectomy, only 16 patients (64%) developed complete closure of the air-bone gap (≤10 dB) on pure tune audiometry, and in four patients (16%) the air-bone gap improved to less 20 dB, while recurrent conductive hearing loss occured in three patients (12%), one patient developed complete sensorineural hearing loss (4%), there was one case of fluctuating hearing loss due to reparative granuloma (4%), perilymph fistula was reported in one case (4%) and no cases of facial palsy or perforation of the tympanic membrane were recorded.

Conclusion: The results of this study clearly show that stapedotomy gives better hearing results and fewer complications than stapedectomy.

Keywords: Otosclerosis; Stapedectomy; Stapedotomy.

Incidence of Low Birth Weight in Mazandaran Province, Northern Iran

Mohammad Khorshidi, Peyvand Nooshirvanpour, Siamak Najafi

Abstract

Objectives: Low Birth Weight (LBW) has an important role in the mortality and morbidity of neonates and the incidence of LBW may vary across different environments. The aim of this study was to determine the incidence and some related factors of LBW in Mazandaran province, Northern Iran.

Methods: In this longitudinal study, all live births that were born in 5 maternity hospitals in Mazandaran province, north of Iran in 2011, were evaluated. Data including birth weight, sex, maternal age, gestational age, living location, number of pregnancy and delivery, as well as multiple births were recorded in medical files. LBW neonates were compared with neonates whose birth weight was more than 2,500 gram (control group).

Results: Out of 3792 infants, 2.9% (CI 95%: 2.3-3.3) were of low birth weight. Sixty percent of the infants in the LBW group and 8.3% in the control group were preterm, (p<0.001). In the LBW group, 22.2% were multiple births, while 0.5% in control group were multiple births (p<0.001), but other variable distributions such as sex, living location and mother's age did not differ between the LBW and the control groups (p>0.05).

Conclusion: Results show that the incidence of LBW in Mazandaran was low and prevention of preterm labor, educational intervention programs for high risk can be effective in the prevention of low birth weight.

Keywords: Low birth weight; Neonate; Incidence.

Vitamin D Deficiency in Patients with Systemic Lupus Erythematosus

Suzan M. Attar and Aisha M. Siddiqui

Abstract

Objectives: Hypovitaminosis D is common in the general population. Many studies that have been conducted to show the association between vitamin D deficiency and systemic lupus erythematosus (SLE) reveal that deficiencies in vitamin D are common in this group of patients. Our aim was to study the relationship between 25(OH)D and disease activity in patients with SLE.

Methods: Retrospective cohort study of patients with SLE who were followed up at King Abdulaziz University Hospital, Jeddah, from January 2007 to November 2010. Demographic and clinical data were recorded and the 25(OH)D levels of the patients were measured. Chi square tests, Student’s t-test, ANOVA and Pearson tests were used for data analysis. ANOVA test was followed by Bonferroni correction. A p-value <0.05 was considered significant.

Results: Ninety-five patients with SLE were enrolled in the study. The levels of 25(OH)D were significantly lower in patients with active SLE (n=41; 43%) than in those with inactive disease (n=54; 57%; p=0.04). The mean (SD) levels were 22.3 (14) nmol/L for patients with active disease against 25.0 (14) nmol/L for patients with inactive SLE. No correlation was detected between 25(OH) D levels and disease activity score evaluated by SLEDAI-2K. By Pearson correlation, a significant negative correlation existed between 25(OH) D and anti ds-DNA (r=-0.38; p<0.001); a positive correlation existed between 25(OH)D levels and C4 (r=0.25; p=0.25). By chi square testing, azathioprine treatment (OR=3.5), low C4 (OR= 2.23), low C3 (OR=1.92), and active disease (OR=1.6) were associated with 25(OH)D deficiency in SLE patients.

Conclusion: Vitamin D deficiency is frequent in patients with SLE. Patients with SLE have a higher risk of developing 25(OH)D deficiency in the presence of low serum C3 and C4 levels, and high anti-dsDNA levels.

Keywords: Vitamin D; Systemic lupus erythematosus; Disease activity; 25(OH)D deficiency.

Breaking Bad News Issues: A Survey Among Physicians

Abdulrahman A. Al-Mohaimeed and Fawzy K. Sharaf 

Abstract

Objective: The aim of this study was to explore the physicians' perspectives and practices in relation to breaking bad news (BBN) to patients.

Methods: A quantitative survey was performed in the Qassim Region from January to July 2011. A cross-sectional study was conducted using a questionnaire administered to all practicing physicians working in both hospitals and Primary Healthcare centers in the Qassim Region. Anonymity was maintained throughout. The target groups received a self-administered questionnaire with a covering letter introducing the study and explaining their rights.

Results: A total of 458 physicians participated in the study. Physicians with higher qualifications had lower total scores of the mean in BBN skills. The majority (70%) preferred to discuss information with close relatives rather than the patients. In case of serious diseases, only 32% said that they would inform the patient’s family without the patient’s consent. More than 90% of our study sample did not avoid telling their patients the bad news; however, physicians working in Primary Healthcare centers were less reserved.

Conclusion: Although most of the participating physicians were keen to help their patients, they lacked the essential knowledge and skills for breaking bad news. Thus, they are in need of specific training in this regard.

Mutation in the PCSK9 Gene in Omani Arab Subjects with Autosomal Dominant Hypercholesterolemia and its Effect on PCSK9 Protein Structure

Khalid Al-Waili,¹ Ward Al-Muna Al-Zidi,¹  Abdul Rahim Al-Abri,¹ Khalid Al-Rasadi,¹ Hilal Ali Al-Sabti,² Karna Shah,³ Abdullah Al-Futaisi,⁴ Ibrahim Al-Zakwani,⁵ Yajnavalka Banerjee¹

Abstract

Proprotein convertase subtilisin/kexin type (PCSK9) is a crucial protein in LDL cholesterol (LDL-C) metabolism by virtue of its pivotal role in the degradation of the LDL receptor. Mutations in the PCSK9 gene have previously been found to segregate with autosomal dominant familial hypercholesterolemia (ADFH). In this study, DNA sequencing of the 12 exons of the PCSK9 gene has been performed for two patients with a clinical diagnosis of familial hypercholesterolemia where mutation in the LDL-receptor gene hasn't been excluded. One missense mutation was detected in the exon 9 PCSK9 gene in the two ADFH patients. The patients were found to be heterozygote for Ile474Val (SNP rs562556). Using an array of in silico tools, we have investigated the effect of the above mutation on different structural levels of the PCSK9 protein. Although, the mutation has already been reported in the literature for other populations, to the best of our knowledge this is the first report of a mutation in the PCSK9 gene from the Arab population, including the Omani population.

Keywords: Mutation; Autosomal dominant hypercholesterolemia; PCSK9 protien.

Infantile Systemic Hyalinosis: A Case Report with a Novel Mutation

Siham Al Sinani,¹ Fathyia Al Murshedy,² Reem Abdwani¹

Abstract

Infantile Systemic Hyalinosis (ISH) (OMIM 236490) is a rare, progressive and fatal autosomal recessive disorder characterized by multiple subcutaneous skin nodules, gingival hypertrophy, osteopenia, joint contractures, failure to thrive, diarrhea with protein losing enteropathy, and frequent infections. There is diffuse deposition of hyaline material in the skin, gastrointestinal tract, muscle and endocrine glands. It is caused by mutations in the ANTXR2 (also known as CMG2) gene, which encodes a trans-membranous protein involved in endothelial development and basement membrane-extracellular matrix assembly. We describe a child with classical features of ISH presenting in infancy with severe chronic debilitating pain and progressive joint contractures. The diagnosis was confirmed by molecular DNA sequencing of ANTXR2 gene which revealed a novel homozygous mutation not previously reported; 79 bp deletion of the entire exon 11 (c.867_945del, p.E289DfsX22). Although this is the first reported case of ISH in Oman, we believe that the disease is under-diagnosed since children affected with this lethal disease pass away early in infancy prior to establishing a final diagnosis.

Keywords: Infantile systemic hyalinosis; Joint contractures; Skin thickness.

Hemorrhagic Cardioembolic Stroke Secondary to a Left Ventricular Thrombus: a Therapeutic Dilemma

Khalil Al-Farsi,¹ Aftab A. Siddiqui,² Yasser W. Sharef,² Ali K. Al-Belushi,² Hakeem Al-Hashim,²

Mohammed Al-Ghailani,² William J Johnsto²

Abstract

Cardiogenic embolism is a major cause of stroke and often leads to significant morbidity and mortality. Despite the recent advances in our understanding of the pathophysiology of stroke and its risk factors, diagnosis and therapy; some case scenarios still present a real challenge for the treating physicians. We report a case of a 50 year old male patient presenting with multi-territory cerebral infarctions due to a left ventricular mobile thrombus complicated with hemorrhagic transformation at the time of presentation. Gradual introduction of anticoagulation coupled with a multidisciplinary team approach advocating careful daily clinical assessment of the patient and regular echocardiographic and neuroimaging studies have resulted in a better management and achievement of therapeutic goals.

Keywords: Stroke; Cardioembolism; Mobile thrombus; Hemorrhagic transformation; Echocardiography; Multidisciplinary approach.

Neoadjuvant Chemotherapy and Radical Surgery in Locally Advanced Cervical Cancer During Pregnancy:  Case Report and Review of Literature

Zohreh Yousefi,¹ Azam Hoseini Hoshyar,¹ Sima Kadkhodayan,¹ Maliheh Hasanzade,¹

Mahmoud reza Kalantari,² Mansoureh Mottaghi¹

Abstract

For pregnant patients with cervical cancer, treatment recommendations are individualized and dependent on the stage of the disease, gestational age at the time of diagnosis, and the patient's desire as to the cosntinuation of the pregnancy. The aim of this study is to describe the outcome of neoadjuvant chemotherapy with radical surgery and pelvic lymphadenectomy in a woman with cervical cancer who wished to maintain her pregnancy.  This is a report of a 26-week pregnant woman with locally advanced cervical cancer stage I_b2 (FIGO) who was successfully treated with neoadjuvant chemotherapy Paclitaxel plus platinum, followed by C/S and radical surgery. Her neonate was healthy and had no abnormalities. This case was the first cervical cancer during pregnancy that was treated using this method at the  tumor clinic, Mashhad University of Medical Sciences, Iran. Neoadjuvant chemotherapy is an effort to allow time for the fetal to reach viability by preventing the progression of the disease.

Keywords: Locally advanced cervical cancer; Neoadjuvant chemotherapy; Pregnancy; Radical surgery.

A Case of Congenital Syndromic Hydrocephalus: A Subtype of ‘Game-Friedman-Paradice Syndrome'

Tapan Kumar Jana,¹  Hironmoy Roy,²  Susmita Giri (Jana)³

Abstract

Human hydrocephalus is a disorder of abnormality in CSF flow or resorption, which has been classified in pertinent literature as congenital and acquired. Congenital hydrocephalus can present as an isolated phenomenon which is common; or with associated anomalies affecting other organs, disturbing physiology or presenting as a syndrome. This report describes a case with congenital foetal hydrocephalus, hypoplastic lungs with super-numery lobations and large left lobe of liver compared to right. Thus far, a review of the literature indicates that this case can be postulated as a subtype of Game-Friedman-Paradice syndrome.

Keywords: Congenital hydrocephalus; Supernumery pulmonary lobations; Game-Friedman-Paradice syndrome.

Primary Malignant Melanoma of the Esophagus

Asit Arora, Amit Javed, Anil K. Agarwal 

Abstract

Primary malignant melanoma of the esophagus is an extremely rare neoplasm arising from the esophageal mucosal melanocytes. We herein describe a patient of primary malignant melanoma of the esophagus, who was managed by thoracolaparoscopic esophagectomy.

Keywords: Melanoma, Primary malignant melanoma, Esophagus, Esophagectomy.

Late Post Traumatic Right-sided Diaphragmatic Hernia Presenting with Acute Intestinal Obstruction and Strangulation: A Rare Presentation

Shahbaz Habib Faridi,¹ Amjad Ali Rizvi,¹ Bushra Siddiqui,² Mohd Khalid³

We report a case of 16 year old male, who was presented to Jawaharlal Nehru Medical College, AMU, Aligarh, India as an emergency case with complaints of severe abdominal pain, difficulty in breathing and bilious vomiting. He was diagnosed as a case of late post traumatic right side diaphragmatic hernia with acute intestinal obstruction. He was successfully treated surgically by laparotomy and the defect was closed with polypropylene suture. The patient was followed for 3 months after operation and there was no complication. 

Keywords: Late Diaphragmatic Hernia, Acute Intestinal Obstruction, Strangulation.

Anesthetic Management of a Patient with Severe Post-tracheostomy Stenosis

Mahfouz M. AbdulKader, Rashid M. Mohammad, Khan S. Mohammed, Murugan L.

Anesthetic management of cases of post-tracheostomy stenosis is challenging for most of the anesthesiologists. We are describing a case of severe post-tracheostomy stenosis and its management using Hunsaker Mon-Jet laser resistant tube. 

Keywords: Tracheostomy; Tracheal stenosis; Laser; Insufflation anesthesia.

Pneumothoraces in a Neonatal Tertiary Care Unit: Case Series

Rehan Ali, Shakeel Ahmed, Maqbool Qadir, Prem Maheshwari, Rehan Khan

Abstract

Objective: Neonatal pneumothoraces are associated with high mortality. Prompt recognition to minimize its complications is paramount for ultimate outcome of these babies.

Methods: A retrospective case series study was carried out at Aga khan University Hospital, from January 2010 to December 2010 to determine the etiology and outcome of neonates with  pneumothorax in a neonatal tertiary care unit.

Results: Ten neonates diagnosed radiologically with pneumothoraces were included. M: F ratio was 1:2.3. Birth weight ranged from 1750-3600 grams with a mean of 2100 grams. The occurrence of pneumothoraces was 50% on the left side, 20% on right, and 30% were bilateral. Primary etiology included pneumonia and sepsis (30%), hyaline membrane disease (20%), meconium aspiration syndrome (20%) and congenital diaphragmatic hernia (10%). Spontaneous pneumothoraces were present in 20% of cases. In our study, the incidence of neonatal pneumothoraces was 2.5/1000 births compared to 10-15/1000 in Denmark, 10-20/1000 in Turkey and 6.3/1000 from Vermont Oxford Group. Despite the small number of cases, one incidental finding was the occurrence of pneumothorax, which declined in elective cesarean section after 37 weeks gestation i.e., 1.3 of 1000 births. Mortality was 60% determined mainly by the primary etiology and other co-morbid conditions.

Conclusion: The study showed a higher number of mortality cases (60%). Although, it was difficult to draw a conclusion from the limited number of cases, there may be a benefit on neonatal respiratory outcome to be obtained by better selection of mothers and by waiting until 37 weeks before performing elective cesarean section. Adequate clinician training in soft ventilation strategies will reduce the occurrence of pneumothoraces.

Keywords: Neonates; Pneumothoraces; Pneumothorax.

Celiac Disease in Oman: A Tertiary Centre Experience

Tawfiq Taki Al-Lawati, Hashim Sadiq Al-Musawi

Abstract

Objective: To describe the frequency of encounter of celiac disease in Royal Hospital, Muscat, Oman.

Methods: Retrospective study of records of all adult and pediatric patients in Royal Hospital from the period of 1/4/2006 to 31/3/2012. Data regarding symptoms, anthropometry of the patients, haemoglobuin levels, liver and thyroid functions were retrieved. Diagnosis of celiac disease was established based on combination of serological detection of anti tissues transglutaminase (tTG) or anti endomysial antibodies (EMA) with duodenal biopsy.

Results: Only 9 children were identified in the hospital during the period of study. Two children were identified by screening protocol for Insulin Dependent Diabetes Melitus (IDDM) and one child from short stature workup. Six children presented with abdominal pain and diarrhea. Four children were severely wasted and stunted. No adult patients were identified with celiac disease. Anaemia was noted in 3 children and none had deranged thyroid functions.

Conclusion: Celiac disease is infrequently encountered in Royal Hospital and might be under diagnosed. The low rate of celiac disease in children with IDDM might indicate a different genetic composition. Awareness about celiac disease and family screening should be implemented in Oman.

Keywords: Celiac Disease; Arab Population; Oman.