Keratinization and its Disorders

Shibani Shetty,¹ Gokul S.²

Abstract

Keratins are a diverse group of structural proteins that form thei ntermediate filament network responsible for maintaining the structural integrity of keratinocytes. In humans, there are around 30 keratin families divided into two groups, namely, acidic and basic keratins, which are arranged in pairs. They are expressed in a highly specific pattern related to the epithelial type and stage of cellular differentiation. A total of 54 functional genes exist which codes for these keratin families. The expression of specific keratin genes is regulated by the differentiation of epithelial cells within the stratifying squamous epithelium. Mutations in most of these genes are now associated with specific tissue fragility disorders which may manifest both in skin and mucosa depending on the expression pattern. The keratins and keratin-associated proteins are useful as differentiation markers because their expression is both region specific and differentiation specific. Antibodies to keratin are considered as important tissue differentiation markers and therefore are an integral aid in diagnostic pathology. The present review discusses the structure of keratin, the various types of keratin and their distribution and the disorders associated with keratinization with special emphasis on the disorders of the oralcavity. A brief note on the clinical significance of keratin is also mentioned.

A Five-year Review on the Etiology and Antimicrobial Susceptibility Pattern ofOtitis Media Pathogens in Jordanian Children

Mohammad Al-Shara

Abstract

Objective: This study aimed to identify the bacteriological agentsof otitis media in Jordanian children and to assess the in vitro susceptibility of these isolates to commonly used antibiotics.

Methods: Retrospective analysis of consecutive samples submitted for microbiological evaluation from outpatients children aged between 6 months and 15 years who were clinically diagnosed with otitis media and were treated at Princess Rahma Hospital in North Jordan between January 2005 and December 2009.

Results: A total of 724 isolates were recovered from cultures obtained from 863 children patients giving an isolation rate of 83.8%. The male and female isolate ratio was (1.26:1.0). The most common bacterial species isolated were S. aureus (59.9%) followed by Streptococcus pneumoniae (22.4%), Pseudomonas (7.7%), E .coli (5.9%), Klebsiella spp. (3.1%) and Proteus spp. (0.9%). The most of S. aureus isolates were susceptible to vancomycin (94.0%) and gentamicin (87.3%). Streptococcus organisms were susceptible in highest percentage to amoxicillin-clavulanic acid (87.1%). Gramnegative isolates were highly susceptible to ciprofloxacin (83.5%) and gentamicin (79.8%). Among all isolates, vancomycin was themost effective antimicrobial agent with susceptibility rate of 83.9%, whereas cefixime showed the lowest susceptibility rate of 39.7%.

Conclusions: S. aureus isolates were the most frequent bacteria isolated from otitis media and were highly sensitive to vancomycin and gentamicin, while gram-negative isolates were more sensitive tociprofloxacin and gentamicin.

Congenital Anomalies in Infant with Congenital Hypothyroidism

Zahra Razavi,¹ Alireza Yavarikia,² Saadat Torabian³

Abstract

Objective: Congenital hypothyroidism is characterized by inadequate thyroid hormone production in newborn infants. Many infants with CH have co-occurring congenital malformations. This is an investigation on the frequency and types of congenital anomalies in infants with congenital hypothyroidism born from May 2006-2010 in Hamadan, west province of Iran.

Methods: The Iranian neonatal screening program for congenital hypothyroidism was initiated in May 2005. This prospective descriptive study was conducted in infants diagnosed with congenital hypothyroidism being followed up in Pediatric Endocrinology Clinicof Besat Hospital, a tertiary care centre in Hamadan. Cases included all infants with congenital hypothyroidism diagnosed through newborn screening program or detected clinically. Anomalies were identified by clinical examination, echocardiography, and X-ray of the hip during the infant’s first year of life.

Results: A total of 150 infants with biochemically confirmed primary congenital hypothyroidism (72 females and 78 males) were recruited during the period between May 2006-2010. Overall, 30 (20%) infants had associated congenital anomalies. The most common type of anomaly was Down syndrome. Seven infants (3.1%) had congenital cardiac anomalies such as: ASD (n=3), VSD (n=2), PS (n =1), PDA (n=1). Three children (2.6%) had developmental displasia of the hip (n=3).

Conclusion: The overall frequency of Down syndrome, cardiac malformation and other birth defect was high in infants with CH. This reinforces the need to examine all infants with congenital hypothyroidism for the presence of associated congenital anomalies.

Immunohistochemical Expression of Ki-67, PCNA and CD34 in Astrocytomas: A Clinicopathological Study

Mohammed Kassim Chaloob,¹ Hussam Hasson Ali,² Ban Jumaa Qasim,³ Ahmed Salahaldeen Mohammed⁴

Abstract

Objectives: To assess the immunohistochemical expression of Ki-67 and Proliferating Cell Nuclear Antigen (PCNA) as proliferative markers to study proliferative activity and CD34 as an endothelial cell marker in order to study vascular proliferation in astrocytomas in correlation with some clinicopathological parameters (age, gender, site of the tumor, and tumor grade).

Methods: A retrospective study wherein a total of 51 formalin-fixed paraffin-embedded brain astrocytoma excisional biopsies covering the period of June 2009 to February 2011 were retrieved from the archival materials of the Specialized Surgical Hospital inMedical City in Baghdad, Iraq. The histopathological diagnosis had been revised and all cases were stained by immunohistochemical technique with Ki-67, PCNA, and CD34 tumor markers. Values were considered statistically significant when p<0.05.

Results: Fibrillary astrocytoma (WHO grade II) was found to be the most common type among astrocytic tumors with the peak ageincidence of astrocytomas found in the second and fifth decades of life, and a slight male predominance had been identified. There was asignificant correlation between the age of the patients and the gradeof the tumor, Ki-67 and PCNA labeling indices, and microvessel density (MVD) detected by CD34 (p<0.05). There was a highly significant correlation between Ki-67 and PCNA labeling indices in astrocytomas (p<0.001).

Conclusion: A significant correlation was found between Ki-67, PCNA labeling indices, and MVD (microvessel density) detected by CD34, and between the clinicopathological variables of astrocytomas (age and grade of tumor). Hence, Ki-67 and PCNA, as markers for proliferation, and MVD as a marker of angiogenesis, could be used as ancillary methods in the differentiation of borderline grades of astrocytomas.

General and Gender Characteristics of Type 2 Diabetes Mellitus Among theYounger and Older Age Groups

Samir Burhanaldin Al-Mukhtar,¹ Nabeel Najib Fadhil,² Bassam Edward Hanna³

Abstract

Objectives: To study the characteristics of cardiovascular risk factors in regard to age (before and after 60) and gender. Many reports refer to the higher prevalence of cardiovascular risk factors among the younger type 2 diabetics in comparison with the older population.

Methods: The study included 462 randomly recruited type 2 diabetic subjects (above and below 60 years) attending Al-Zahrawi Private Hospital in Mosul City-Iraq, during the period from June to August 2011. They were analyzed in regard to age, duration of diabetes, smoking, socio economic status, anthropometric indices, blood pressure, fasting plasma glucose, glycated hemoglobin A1c and serum lipids. Data were analyzed using chi-square and unpaired Z test.

Results: Duration of diabetes, diastolic blood pressure, glycated hemoglobin A1c, fasting plasma glucose, serum lipids, number of hypercholesterolemic patients, number of patients having unfavorable total cholesterol/HDL ratio (≥5) and positive family history of coronary heart disease were all significantly higher in the younger diabetics. In addition, younger diabetic females were distinguished by a larger number of hypertensive patients, higher level of systolic blood pressure, higher means of body mass index, total cholesterol and LDL, and larger number of patients having low HDL-C (<1 mmol/L). The younger diabetic males were distinct bya larger number of smokers, number of smoked cigarettes/day, and longer duration of smoking. All parameters ranged between p<0.05 and p<0.005.

Conclusion: Cardiovascular risk factors were significantly higher among younger type 2 diabetics (<60 years), particularly females.

Pattern of Headache in School Going Children Attending Specialized Clinic in aTertiary Care Hospital in Bangladesh

Md Azharul Hoque,¹ Kazi Mohibur Rahman,¹ Badrul Haque,¹ Rajib Nayan Chowdhury,¹ Sharif Uddin Khan,¹ ATM Hasibul Hasan,² Md Badrul Alam Mondol,¹ Monsur Habib,¹ Quazi Deen Mohammad¹

Abstract

Objective: To determine the pattern of headache and its associated symptoms in school going children.

Methods: The data of all the school going children attending the Headache Clinic in the Dept. of Neurology, Dhaka Medical College Hospital were retrospectively reviewed. A total of 1021 patients from October 1996 to September 2011 were selected. Data were collected through a predesigned questionnaire containing information on age, sex, social status, clinical features, opthalmoscopic findings, management, and in selected cases imaging results.

Result: The mean age of headache in school children was 12.6±1.08 years with relatively older age of presentation among girls. The sex ratio was 1.64:1 in favor of girls at older age. Tension type headache (71.1%) was the most common form of headache, followed by migraine (18.4%) and mixed headache (6.7%). Though the girls had more frequent headache of both tension type (59.4%) and migraine (68.1%) variety, the latter was significantly associated in girls (p<0.001). Headache was of moderate severity in 53.3%, whereas severe headache was experienced by 19.9% of the children. The children commonly had nausea and/or vomiting (47.2%), as well as photophobia (24.7%) with headache. Mentalstress (34%) and sunlight (30.9%) were common triggering factors whereas a sound sleep relieved headache in the majority (59.4%). Paracetamol (83.3%) and nor tryptyline (62.8%) were the most commonly prescribed drug taken by them.

Conclusion: Headache is a major health problem in school children, apart from other common health issues at this age. With increasing age, the girls more commonly suffer not only from migraine but also with other chronic headache. The direct causal association is yet to be determined.

Synthesis and Anti-hyperlipidemic Activity of 3H-benzo [4, 5] thieno [2, 3-d] [1, 2, 3] triazin-4-ones: Possible Mechanism of Altered Lipid Metabolism

Gollapalle L. Viswanatha,¹ Janaki Priyadarshini B,¹ Shylaja Hanumanthappa,² Srinath Rangappa,¹ Saravanan Janardhanan³

Abstract

Objectives: The present study was aimed to evaluate the anti-hyperlipidemic activity of newly synthesized tricyclic benzothieno 1, 2, 3-triazine derivatives namely CP-1 (3-(methyl)-5,6,7,8-tetrahydro,3H-benzo[4,5] thieno [2,3-d][1,2,3] triazin-4-one), CP-2 (3-(ethyl)- 5,6,7,8-tetrahydro,3H-benzo[4,5] thieno[2,3-d][1,2,3] triazin-4-one) and CP-6 (3-(2-chloro phenyl)-5,6,7,8-tetrahydro,3H-benzo[4,5] thieno [2,3-d][1,2,3] triazin-4-one) against dexamethasone and Triton WR-1339-induced hyper-lipidemia in rats.

Methods: Anti-hyperlipidemic activity of the test compounds were evaluated against dexamethasone (10 mg/kg, subcutaneous [s.c.]) and Triton WR-1339 (200 mg/kg, intraperitoneal [i.p]) induced hyperlipidemia in rats.

Results: Administration of single dose of Triton WR-1339 (200 mg/kg i.p) and dexamethasone (10 mg/kg s.c.) for 8 consecutive days to adult wistar rats caused severe hyperlipidemia characterized by marked increase in serum cholesterol, LDL-C, VLDL-C and triglyceride levels along with an increase in atherogenic index. Serum HDL-C levels were decreased significantly compare to normal control. Pretreatment with Atorvastatin (10 mg/kg, p.o.), CP-1 (25 & 50 mg/kg), CP-2 (25 & 50 mg/kg) and CP-6 (25 & 50mg/kg) showed significant and dose-dependent protection against dexamethasone and Triton WR-1339-induced hyperlipidemia in rats by maintaining serum total cholesterol, LDL-C, VLDL-C and HDL-C levels within the normal range. Also, a significant decrease in atherogenic index was observed. The anti-hyperlipidemic effect of CP-6 was comparable with reference standard Atorvastatin. Furthermore, CP-6 was found to be more potent than CP-1 and CP-2.

Conclusion: These findings suggest that CP-1, CP-2 and CP-6 possess significant anti-hyperlipidemic activity against experimental animal models of hyperlipidemia.

Staff Perception of Relative Importance of Quality Dimensions for Patients atTertiary Public Services in Oman

Ismail Alrashdi,¹ Ahmed Al Qasmi²

Abstract

Objective: This research attempted to explore the public healthcare providers understanding the quality dimensions and patient priorities in Oman. It also addresses the issue of risks confronting health professionals in management without “a customer focused” approach.

Methods: A descriptive study was carried out using a self-administered questionnaire distributed around two tertiary public hospitals. A total of 838 respondents from several specialties and levels of hierarchy participated in the study. The data was analyzed to compare the perception of two groups; the group of junior and frontline staff, as well as of managers and senior staff involved in management.

Results: The results showed that 61% of the junior and frontline staff, and 68.3% of the senior staff and managers think that cure or improvement in overall health is the single most importantquality dimension in healthcare. Both groups perceive that technical dimensions have greater importance (to patients) over interpersonal aspects such as communication with the exception of dignity and respect. There was no significant difference between the perception of the managers and senior staff vis-à-vis the perception of junior and frontline staff on the importance of technical dimensions and the interpersonal aspects of service quality. Despite the proven contribution of empathy to patient satisfaction, it was ranked by both groups as the least important among the dimensions examined.

Conclusion: The findings of this research are therefore informative of the need to implement strategies that deal effectively with such attitudes and create the platform and programs that reinforcethe culture of good quality service amongst healthcare providers, managers in particular, and to improve patient satisfaction.

Continuing Professional Development Evaluation: Two Rapid Review Courses inNephrology and Rheumatology

Abdullah Shehab,¹ Asim Elnour,² Shirina Al Sowaidi,³ Abdishakur Abdulle³

Abstract

Objectives: Continuing professional development (CPD) is anovel approach to increase professional knowledge and skills. The aim of this study is to explore participants’ characteristics and to understand participants’ views on two rapid review courses (RRCs) as part of CPD program, and to assess healthcare providers’ views about the use of internet for accessing medical information.

Methods: Data were collected from 150 participants who attended an RRC in Nephrology and Rheumatology as part of an ongoing CME program.

Results: Participants’ response rate was 92% and 84.4% in Nephrology and Rheumatology RRCs, respectively. Participants’ Mean Age±SD were 39±2.1 and 41±2.1 years in the Nephrology and Rheumatology courses, respectively. Demographic variables, i.e., age, gender, and specialization showed a significant (p<0.01) impact on the learning objectives of the program. Further, participants reported that the course material had a significant (p<0.02) impacton their knowledge. Finding new medical information was the primary motive to search the internet among all participants. About half of the subjects reported knowledge of their preferred medical education sites and had access at their clinical setup. Barriers to internet use included lack of specific information, difficulty to download contents, and excessive material. Professional association websites, online journals, and CME programs were the most frequently searched sources of information. Most of the subjects reported significant (p<0.02) barriers to find medical resources on the internet and to adequately utilize the currently available medical search engines available in the healthcare system.

Conclusion: A discipline specific and integrated CPD programmay have provided dual benefit such as accredited CME hours and a significant change in the participants’ knowledge. There is a need to increase Internet accessibility and capacity in the current healthcare facilities. Future CPD studies should include measuring participants’ knowledge and skills “pre and post” course delivery, and a detailed evaluation of the course program.

Cleidocranial Dysplasia: A Rare Cause of Disproportionate Severe Short Stature

Manzoor Ahmad Bhat, Bashir Ahmad Laway, Suhail Mantoo, Khalid Choudry, Suman Kotwal,Shahnaz Ahmad Mir

Abstract

Skeletal dysplasia is an uncommon cause of short stature in children. An 11-year-old girl was evaluated for severe short stature in a tertiarycare hospital. Clinical examination revealed severe disproportionate short stature and classical triad of multiple supernumerary teeth, and complete absence of clavicles and open sagittal sutures and fontanelles. Skeletal survey confirmed these findings, in addition toother features associated with the syndrome.

Successful Treatment of Cerebral Toxoplasmosis with Clindamycin: A Case Report

Deepak Madi,¹ Basavaprabhu Achappa,¹ Satish Rao,¹ John T. Ramapuram,¹ Soundarya Mahalingam²

Abstract

Toxoplasmosis is caused by infection with the obligate intracellular parasite Toxoplasma gondii. Toxoplasmosis is generally a late complication of HIV infection and usually occurs in patients with CD4 + T-cell counts below 200/μl. Co-trimoxazole (trimethoprim plus sulfamethoxazole) is the most common drug used in India for the treatment of AIDS-associated cerebral toxoplasmosis. Other alternative drugs used for the treatment of cerebral toxoplasmosis are clindamycin plus pyrimethamine and clarithromycin with pyrimethamine.

A 30-year-old male known case of retroviral disease presented to Kasturba Medical College, India, with complaints of fever, headache and vomiting. Computed tomography scan of his brain showed irregular ring enhancing lesion in the right basal ganglia. Toxoplasma serology revealed raised IgG antibody levels. Based on the CT features and serology, diagnosis of cerebral toxoplasmosis was made. He was treated with clindamycin alone as he had historyof sulfonamide allergy. The patient was symptomatically better after 48 hours. After 21 days, repeat CT of brain was done which was normal. The patient showed good clinical improvement within 48 hours and the lesion resolved completely within 3 weeks. The authors recommend using clindamycin without pyrimethamine in resource poor settings and in patients who do not tolerate sulfa drugs.

No More Milk in Milk-Alkali Syndrome: A Case Report

Ali AlMusawi,¹ Shurooq AlHawaj,² Mohammed Al-Mousawi,³ Tareq Dashti⁴

Abstract

This is a case of Milk-AlKali syndrome in a patient who presented with the classical triad of hypercalcemia, metabolic alkalosis and renal impairment. The source of calcium was over-the-counter-calcium-containing antacid (Tums®). Milk-alkali syndrome was first recognized secondary to treatment of peptic ulcer disease with milk and absorbable alkali. Its incidence fell after the introduction of H2-blocker and proton pump inhibitor. However, it is one of the leading causes of hypercalcemia now adays because of the wide availability, increased marketing and use of calcium carbonate especially in osteoporosis prevention and treatment. The demographics of milk-alkali syndrome have changed compared to when it was initially described. The presentation could be acute, subacute or chronic. Early diagnosis, discounting calcium supplement and intravenous hydration are the mainstay of MAS management.

Cylindrical Cell Papilloma (Oncocytic Schneiderian Papilloma); Clinicopathological Study of Five Cases

Abdulla Darwish,¹ Amal Al-Abdulla²

Objective: To study the clinicopathological findings of cylindrical cell papilloma (oncocytic schneiderian papilloma) diagnosed among patients at Bahrain Defense Force Hospital with review of literature.

Methods: All cases of cylindrical cell papilloma were retrieved from the pathology register from 2006-2010. The medical records of the cases were studied for age, sex, clinical presentation, and imaging reports along with the histopathological findings.

Results: Five cases were found and showed male to female ratio 4:1. Their ages were between 36-71 years with average age of 55 years. The main symptom found was unilateral nasal obstruction mainly involving the left side. All papillomas were removed by endoscopic sinus surgery. Four patients had regular followup with complete recovery, while only one case was lost for followup.

Conclusion: Cylindrical cell papilloma is a variant of sinonasal schneiderian papilloma. Although most are benign complete excision is the treatment of choice to prevent tumour recurrence as a proportion exhibit unpredictable biological behavior. This study is an important reminder for both clinicians and pathologists to recognize this tumor and to differentiate it from other tumours and rhinosporidiosis. Regular followup is an important strategy for complete recovery and tumour-free surveillance.

Keywords: Oncocytic schneiderian papilloma; Cylindrical cell papilloma; Sinonasal tumours; Inverted papilloma; Rhinosporidiosis.