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Showing papers in "Jcpsp-journal of The College of Physicians and Surgeons Pakistan in 2009"


Journal Article
Saeeda Baig1
TL;DR: There may be an influence of estrogen in the protection and defense of hepatic cells against the development of chronic liver disease and the male to female ratio increased during the reproductive years.
Abstract: Gender differences prevail in the infections caused by the Hepatitis B virus. Four hundred and seventy two patients with HBV infection were selected for the study. The frequency of hepatic infection in males was 79.5% (n=375) and in females 20.5% (n=97), with a male to female ratio of 3.8:1. Out of 472 patients, 49% had acute hepatitis, 26% were carriers, 18% had chronic hepatitis, 6% had cirrhosis and 3% patients had hepatocellular carcinoma. Male dominance was found to be consistent in all categories of patients. When the patients were divided into groups according to age, the male to female ratio increased during the reproductive years. There may be an influence of estrogen in the protection and defense of hepatic cells against the development of chronic liver disease.

74 citations


Journal Article
TL;DR: The very high maternal mortality ratio suggests lack of access of women to quality healthcare facilities and a majority of these women suffered first and second delays in their management, which could be due to their poor sociodemographic factors.
Abstract: OBJECTIVE: To describe the socio-demographic characteristics and the three delays of maternal mortality in a tertiary teaching hospital. STUDY DESIGN: Retrospective observational study. PLACE AND DURATION OF STUDY: Department of Obstetrics and Gynaecology Unit III Civil Hospital Karachi from April 2005 to May 2008. METHODOLOGY: One hundred and four consecutive maternal deaths were reviewed. Data regarding age parity sociodemographic characteristics booking status referral source cause of death and the three delays was collected on structured proformas analyzed by the statistical software SPSS version 13 and presented in the form of frequencies and percentages. RESULTS: The projected maternal mortality ratio was 1650/100000 live births. The mean age was 28+/-6.2 years and median parity was two. Seventy-one women (68%) were uneducated 65 (62.5%) belonged to lower socioeconomic class and 60 (58%) had received no antenatal care. Ninety-eight women (94%) had one or more delays with 70 (71%) having the first delay 73 (74%) having the second delay and 47 (48%) the third delay. The most frequent reasons for first second and third delays were lack of awareness in 88.5% women long distance in 39.7% women and difficulty in getting blood in 49% women respectively. CONCLUSION: The very high maternal mortality ratio suggests lack of access of women to quality healthcare facilities. A majority of these women suffered first and second delays in their management which could be due to their poor sociodemographic factors.

66 citations


Journal Article
TL;DR: It is recommended that all the patients suspected for dual infections should be treated concomitantly for dengue and malaria in malaria endemic areas.
Abstract: OBJECTIVE To find out clinical features, diagnostic techniques and management outcome of patients having dual dengue and malaria infection. STUDY DESIGN A case series. PLACE AND DURATION OF STUDY Medical Unit-III, Ward- 7, Jinnah Postgraduate Medical Centre, Karachi, from September 2007 to January 2008. METHODOLOGY Patients presented with fever of less than or equal to 10 days duration, severe body aches, rash and bleeding manifestations were included. Patients with obvious features of other diseases like typhoid, hereditary bleeding diathesis and hematological malignancies and only malarial parasite positive with high grade intermittent fever without rash and myalgia were excluded from the study. Diagnosis of dengue and malaria was based on history, clinical features, laboratory parameters and malarial parasite test by thin and thick films. Serological evaluation was done by dengue IgM and IgG by ELISA test kit. Patients were divided into three groups. Group A was dengue IgM positive plus MP positive, group B was dengue IgM positive and MP negative and group C was dengue IgM negative and MP negative and were clinically suspected dengue and malaria. The clinical manifestations and laboratory parameters of dual dengue and malaria positive patients were compared with malaria and dengue negative patients. RESULTS One hundred and fourteen patients were seen during the study period. Antibody titer (IgM) tested in all patients was found positive in 78 patients (69.64%). Among those 78 patients, 26 (23.21%) were concomitantly positive for malarial parasite (Group A). Plasmodium vivax was positive in 25 patients and falciparum in one patient. Fifty-two patients (46.42%) were dengue IgM positive and MP negative (Group B). Thirty four (30.35%) patients were MP and dengue IgM negative (Group C) but were strongly suspected for DHF and malaria on clinical and hematological basis. The hemoglobin of 34.61% of patients of group A, 5.76% of group B and 14.7% of group C were low, hematocrit level was also low in group A (92.3%), group B (15.38%) and group C (70.58%) patients. The platelet count was markedly low in 84.61% of patients of group A, 57.69% of group B and 94.11% of group C. Leukopenia was found in 34.61% of patients of group A, 78.84% in group B and 29.411% in group C. The liver function tests were deranged in all groups. CONCLUSION The frequency of dual dengue and malaria infection was 23.21%. The serology of the dengue and malaria showed negative results in 30.35%. The diagnosis of dual infections could be made on the basis of history, clinical examination supported by hematological results. It is recommended that all the patients suspected for dual infections should be treated concomitantly for dengue and malaria in malaria endemic areas.

54 citations


Journal Article
TL;DR: Serum zinc level was significantly lower in type-2 diabetics, whereas no significant difference was found in serum magnesium level when compared with control subjects, and there was no association of age, gender, glycemic status and duration of diabetes on the serum concentration of these trace elements intype-2 diabetic patients.
Abstract: Objective: To assess serum zinc and magnesium level in type-2 diabetic patients and the effect of age, gender, glycemic control and duration of diabetes on these trace elements in comparison with those of control subjects. Study Design: Non-interventional case control study. Place and Duration of Study: Department of Medicine, Liaquat University of Medical and Health Sciences, Jamshoro, Hyderabad, from October 2007 to March 2008. Methodology: There were 42 diabetic patients and 42 age matched non-diabetic (control) subjects included in this study. Serum zinc, serum magnesium and fasting blood sugar measured among the diabetic and control groups and association of both trace elements were assessed with glycemic status, age, gender and duration of diabetes using SPSS version 16.0 for analysis. Results: Serum zinc level was significantly lower (mean 2.03+0.39 mg/dL) in diabetic patients as compared to control subjects (4.84+4.217 mg/dL, p = < 0.001). No significant difference was found in serum magnesium level with mean of 22.67+24.5 mg/dL in diabetic patients as compared to controls (18.3+3.4 mg/dL, p = 0.26). Conclusion: Serum zinc level was significantly lower in type-2 diabetics, whereas no significant difference was found in serum magnesium level when compared with control subjects. There was no association of age, gender, glycemic status and duration of diabetes on the serum concentration of these trace elements in type-2 diabetic patients.

48 citations


Journal Article
TL;DR: Thrombocytopenia was a common haematological finding in patients with Plasmodium infection particularly marked in vivax species infection.
Abstract: Objective: To determine the frequency of thrombocytopenia in Malarial Parasite (MP) positive patients. Study Design: A cross-sectional study. Place and Duration of Study: Medical Unit-III, Ward-7, JPMC, Karachi, from June to October 2006. Methodology: One hundred twenty four MP positive cases were included in the study. Infections with both Plasmodium falciparum and Plasmodium vivax species were included. Complete blood picture with platelet count was obtained in all patients. Thrombocytopenia was defined as platelets count of < 150,000/cmm. Results: Among 124 patients of MP positive, 100 (80.6%) were found to have thrombocytopenia. Over all 64 patients had Plasmodium falciparum, while 60 patients were having Plasmodium vivax infection. The frequency of thrombocytopenia was 71.87% (n=46) in falciparum and 93.33% (n=56) in vivax infection. Conclusion: Thrombocytopenia was a common haematological finding in patients with Plasmodium infection particularly marked in vivax species nfection.

45 citations


Journal Article
TL;DR: Hepatic dysfunction in acute P.falciparum malaria ranged from mild elevation of liver enzymes to acute hepatitis (ALT>10 times of normal level), which indicates severe illness with high frequency of complication and mortality rates.
Abstract: UNLABELLED To evaluate the frequency and severity of jaundice with hepatic dysfunction in Plasmodium (P.) falciparum malaria in adult patients admitted in the hospital. STUDY DESIGN Descriptive study. PLACE AND DURATION OF STUDY The Infectious Diseases Unit and Medical Wards at Rashid Hospital, Dubai, United Arab Emirates, from January 2005 to December 2007. METHODOLOGY This study included 105 adult patients who fulfilled the inclusion criteria. The diagnosis of P.falciparum malaria was confirmed by examination of thin and thick film stained with Leishman's stain. Other laboratory investigations included full blood count, liver function tests, blood urea, electrolytes, serum creatinine, reticulocyte count, blood sugar, viral hepatitis serology and coagulation profile. Patient with significant clinical/biochemical hepatic dysfunction were also subjected to ultrasonic examination of abdomen. RESULTS On clinical examination, 23% patients were found to be jaundiced. Serum alanine amino transferase (ALT) level was above the reference range in 67.6%, but in only 11.4%, ALT was more than 3 times of normal level. Serum bilirubin was found to be higher than normal level in 81%, however, only in 23% of the patients, Serum bilirubin was >3mg/dl. Predominantly conjugated hyperbilirubinemia was observed in patients with high ALT. There was no significant change in serum albumin and prothrombin time. In comparison to normal bilirubin level, the patient with bilirubin >3mg/dl had high frequency of raised ALT 87.5% vs. 45% (p .05). Overall, 5 (4.7%) patients died and mortality rate was high among the patients with bilirubin level>3mg/dl than with normal bilirubin level 4 (16.6%) vs 1 (5%). CONCLUSION Hepatic dysfunction in acute P.falciparum malaria ranged from mild elevation of liver enzymes to acute hepatitis (ALT>10 times of normal level). It indicates severe illness with high frequency of complication and mortality rates.

44 citations


Journal Article
TL;DR: This study showed that for every 7 women who survived life threatening complications, one died, however, the underlying disease processes for near-miss and mortalities were almost same.
Abstract: Objective To determine the frequency of near-miss cases, nature of near-miss events and mortality among obstetric patients. Study design Cross-sectional, observational study. Place and duration of study The study was conducted in the Obstetric Unit of Fatima Hospital, Baqai Medical University, from January 2006 to December 2006. Methodology Near-miss case definition was based on validated specific criteria comprising of five diagnostic features: haemorrhage, hypertensive disorders in pregnancy, dystocia, infection and anemia. The main outcome measures were frequency and characteristics of near-miss cases, total hospital stay, high dependency unit/ICU stay and development of multiple organ dysfunction. Near-miss events and maternal deaths were described with respect to disease profiles. Mortality indices were determined for various disease processes to appreciate the standard of care provided for near-miss cases. The maternal death to near-miss ratio was calculated. Results There were 868 deliveries in the year 2006, 44 near-miss cases, 47 near-miss events and 6 maternal deaths. The maternal death to near-miss ratio was 1:7. The most common type of near-miss events were obstetrical haemorrhage, anemia and dystocia responsible for 51%, 21.2% and 14.8% respectively. Severe hypertensive disorders in pregnancy and infections accounted for 8.5% and 4.2% of near-miss events. Postpartum haemorrhage was responsible for 83.3% and infection for 16.6% of the maternal deaths. The mortality index was higher for infections (33.3%) than for haemorrhage (17.2%). Organ system dysfunction/failure was diagnosed in 18.1% of near-miss cases. Conclusion This study showed that for every 7 women who survived life threatening complications, one died. However, the underlying disease processes for near-miss and mortalities were almost same. Evaluation of the circumstances surrounding near miss cases could act as proxy for maternal deaths in the studied population.

42 citations


Journal Article
TL;DR: A high percentage of ESBL-producers among clinical isolates of K. pneumoniae is demonstrated and a high rate of multidrug resistance is observed and continued infection control measures and prudent use of antimicrobial agents are essential.
Abstract: The aim of this descriptive study was to determine the prevalence of Klebsiella pneumoniae from different clinical specimens, their antibiotic susceptibility pattern and the extended-spectrum β-lactamase (ESBL) production among such isolates at Armed Forces Hospital, Al-Kharaj, Saudi Arabia, from November, 2004 to October, 2007. A total of 328 isolates of K. pneumoniae recovered from 8152 different specimens were studied for ESBL production and antimicrobial susceptibility. The prevalence of K. pneumoniae was 4% (328/8152). We observed a high percentage of K. pneumoniae resistant to ampicillin (100%) and tetracycline (92%). The prevalence of ESBL-producing K. pneumoniae was found to be 10.4% (34/328). The ESBL-producing strains were mostly from aspirates (25%) followed by sputum (20%) and blood (18.2%). These ESBLproducing isolates were highly resistant to gentamicin and ciprofloxacin. All isolates were susceptible to imipenem. Our findings demonstrate a high percentage of ESBL-producers among clinical isolates of K. pneumoniae and a high rate of multidrug resistance. Continued infection control measures and prudent use of antimicrobial agents are essential in reducing the spread of multi-resistant ESBL-producing K. pneumoniae.

40 citations


Journal Article
TL;DR: Most of the patients were satisfied with their experience with spinal anaesthesia although there was a higher frequency of postoperative backache.
Abstract: Objective To determine the level of satisfaction in terms of intra-operative pain and postoperative nausea, vomiting and backache among patients receiving spinal anaesthesia for caesarean section. Study design Cross-sectional survey. Place and duration of study Department of Anaesthesia and Intensive Care at the Combined Military Hospital, Bahawalpur, from August 2005 to June 2007. Methodology A total of 246 pregnant patients undergoing caesarean section under spinal anaesthesia were surveyed. Postoperatively, a questionnaire was given to the patients to score their satisfaction on a four-point visual analogue scale regarding pain during surgery, Postoperative Nausea and Vomiting (PONV), and postoperative backache. The average of response to the questions in each of these three areas was taken as the Fundamental Area Score (FAS) and the average of all these individual area scores was taken as the Patient Satisfaction Score (PSS). Results There was high satisfaction score for PONV (98.17%); but the satisfaction regarding intra-operative pain / discomfort and postoperative backache was low as shown by a decrease in satisfaction score in these areas (74.09% and 76.83%). Patient's overall level of satisfaction with spinal anaesthesia was 81.40%. Patients (53.66%) would opt for spinal anaesthesia in future, if required, 90 (36.59%) would not and 8 patients (9.8%) were not sure. Conclusion Most of the patients were satisfied with their experience with spinal anaesthesia although there was a higher frequency of postoperative backache.

37 citations


Journal Article
TL;DR: Pregnancy-related ARF was associated with poor outcome and antepartum and postpartum haemorrhage were the most common cause of ARF in pregnancy.
Abstract: OBJECTIVE To determine the etiology and outcome of Acute Renal Failure (ARF) in pregnancy. STUDY DESIGN A case series. PLACE AND DURATION OF STUDY Nephrology Department of the Jinnah Postgraduate Medical Centre, Karachi, from August 2007 to July 2008. METHODOLOGY Pregnant women who were healthy previously and had developed ARF, diagnosed on oliguria (urine output 2 mg%) were included in the study. Percutaneous renal biopsy was performed for delayed recovery, i.e. after three weeks. Patients were followed up for a period of 6 months. Percentages were calculated for qualitative variables i.e. causes of ARF, mortality, morbidity and outcome in form of complete recovery, partial recovery, demise and non-recovery. RESULTS A total of 43 patients with pregnancy-related ARF were included in the study. The puerperal group comprised 36 patients (83.7%). Haemorrhage was the etiology for ARF in 25 (58.1%), antepartum haemorrhage APH in 8 (18.6%) and postpartum haemorrhage PPH in 16 (37.2%) of patients. In 12 (27.9%), puerperal sepsis was the etiological factor, while 4 (9.3%) patients had DIC on presentation. Pre-eclampsia, eclampsia and HELLP syndrome accounted for 5 (11.6%). While 1 (2.3%) was diagnosed with hemolytic uremic syndrome and another one was diagnosed as ARF secondary to hypotension produced by hyperemesis gravidarum. Renal biopsy was performed in 31 patients showing that 10 had acute cortical necrosis and 21 had acute tubular necrosis. Maternal mortality was 16.2% (n=7). Of the 36 (83.7%) surviving patients, 18 (41.4%) had complete recovery of renal function; 12 (27.9%) had partial recovery; and 6 (13.9%) required chronic dialysis. CONCLUSION Pregnancy-related ARF was associated with poor outcome. Antepartum and postpartum haemorrhage were the most common cause of ARF in pregnancy.

35 citations


Journal Article
TL;DR: The high levels of plasma homocysteine could indicate a reason for mass micronutrient supplementation to prevent the high incidence of cardiovascular disease observed in Pakistani population.
Abstract: Objective: To determine the plasma/serum levels of homocysteine, and vitamins folate, B6 and B12, in Pakistani healthy adults. Study Design: Cross-sectional study. Place and Duration of Study: The Aga Khan University, from October 2006 to April 2008. Methodology: Fasting levels of plasma/serum folic acid, pyridoxal phosphate (PLP), vitamin B12 and homocysteine were determined in 290 apparently healthy hospital personnel from institutions in two cities of Pakistan. Spearman correlation test and linear regression analysis was conducted. Results: There were 219 males and 71 females with mean age of 46±10.5 years and mean body mass index of 23.5 ±3.8. Mean plasma homocysteine levels in Pakistani normal adults were found to be 17.95±8.4 μmol/l. Mean concentrations of plasma/serum folate, vitamin B12 and PLP were found to be 5±3.9 ng/ml, 522±296 pg/ml and 21.6±14 nmol/l, respectively. Serum/plasma levels of folate, vitamin B12 and PLP were negatively correlated with plasma homocysteine (rho coefficient=-0.367, p<0.001; -0.173, p=0.004; -0.185, p=0.002, respectively). Serum folate and plasma PLP levels were inversely related with plasma homocysteine, adjusted for gender, age, smoking and body mass index (p<0.001 and p=0.003, respectively). Percent deficiency values of folate, vitamin B6 and vitamin B12 were 39.7%, 52.8% and 6.6% respectively. Conclusion: The high levels of plasma homocysteine could indicate a reason for mass micronutrient supplementation to prevent the high incidence of cardiovascular disease observed in Pakistani population.

Journal Article
TL;DR: Postnatal depression was found in almost 1/3rd of the study participants and the preponderance of them suffered from moderate or severe depression, and they were young and came from a background of socio-economic adversity.
Abstract: Objective: To determine the frequency and associated sociodemographic, obstetric and medical factors for postnatal depression in an outpatient sample.Study Design: A descriptive, cross-sectional study.Place and Duration of Study: Department of Obstetrics and Gynaecology, Benazir Bhutto Hospital (formerly Rawalpindi General Hospital), Rawalpindi, between June 2006 and February 2007.Methodology: The sample consisted of women who were in the puerperal period (6 weeks postpartum). They were screened with the help of Edinburgh Postnatal Depression Scale (Urdu version) and the severity of depression was rated with the Hamilton Rating Scale for Depression.Results: A total of 51 participants, or 33.1% of a sample of 154 women suffered from postnatal depression, the majority of whom were either moderately or severely depressed. The demographic profile of depressed patients showed that they were young (mean age around 25 years), had a low level of education (below the matriculate level) and came from the lower socioeconomic class. They had small families comprising of fewer than 3 children, were married for less than 5 years and the majority were from extended families (living with in-laws).Conclusion: Postnatal depression was found in almost 1/3rd of the study participants and the pre-ponderance of them suffered from moderate or severe depression. They were young and came from a background of socio-economic adversity. Since postnatal depression had adverse consequences for the mother and her newborn baby, there was an urgent need to direct more attention to this problem, in particular towards its early detection, so that morbidity could be reduced in this group of women.

Journal Article
TL;DR: Epidural steroid injections in acute symptoms of sciatica are considered to be a better option compared to conservative treatment.
Abstract: Objective To determine the difference in short- and long-term pain improvement between lumbar Epidural Steroid Injections (ESIs) and conservative management in patients with lumbar radiculopathy. Study design Quasi-experimental study. Place and duration of study The Postgraduate Medical Institute of Hayatabad Medical Complex, Peshawar, from April 2005 to March 2007. Methodology Fifty elective patients fulfilling the inclusion criteria were randomly divided into two groups. Patients in the steroid group were treated with 80 mg of methylprednisolone injected in combination with 3 ml of 2% plain xylocaine and 3 ml of normal saline in the lumbar epidural space, while patients in the conservative group were treated with bed rest, non-steroidal anti-inflammatory agents, muscle relaxants, and opioids. All the 50 patients in the two groups were regularly assessed at 2 weeks, 1 month, 3 months and 6 months of periods for pain score by the Visual Analogue Scale (VAS), patients satisfaction score and any unwanted side effects. Results A marked improvement of the pain score and patients satisfaction score were noticed in the steroid group. Less significant improvement was seen in the conservative group during the initial period i.e 2 weeks and 1 month (p 0.05). Conclusion Epidural steroid injections in acute symptoms of sciatica are considered to be a better option compared to conservative treatment.

Journal Article
TL;DR: CML presented at a younger age in the chronic phase, as determined by a study to determine the frequency of three phases of chronic myeloid leukaemia at first presentation.
Abstract: Objective To determine the frequency of three phases of chronic myeloid leukaemia at first presentation. Study design Case series. Place and duration of study Department of Oncology, Combined Military Hospital (CMH), Rawalpindi, from June 2006 to December 2007. Methodology Forty-five patients of either gender with Chronic Myeloid Leukaemia (CML) at their first presentation in outpatient department were included in the study by consecutive sampling technique. All patients were diagnosed on blood complete picture and bone marrow examination including aspiration, trephine and cytogenetics at Armed Forces Institute of Pathology (AFIP). Each phase was defined on the basis of World Health Organization (WHO) criteria. Results Out of 45, there were 31 (68.9%) male and 14 (31.1%) female patients. The mean age of presentation was 37.9 years. The pattern of presentation revealed 35 (77.8%) in Chronic Phase (CP), 7 (15.5%) in Accelerated Phase (AP) and 3 (6.7%) in Blast Crisis (BC). Philadelphia chromosome was detected in 39 (86.7%) cases on culture method. Splenomegaly was observed in 37 (82.2%) patients. The mean total leukocyte count, platelet count, haemoglobin and marrow blast were 214.3 x 10(9)/L, 551.4 x 10(9)/L, 9.94 g/dl and 9.3% respectively. Conclusion CML presented at a younger age in the chronic phase.

Journal Article
TL;DR: Various factors leading to domestic violence were identified among the husbands of women subjected to violence during pregnancy and the differences between the two groups, with regard to neonatal outcome (except depression), did not reach statistical significance.
Abstract: Objective: To determine the causes and type of domestic violence endured by pregnant women and their effect on pregnancy, in terms of maternal and neonatal outcome Study Design: Cohort study Place and Duration of Study: The study was conducted in Sir Syed Trust Hospital, Karachi, from March 2007 to February 2008 Methodology: All the antenatal patients were screened for domestic violence, using a self-made questionnaire and abuse assessment screen All the pregnant patients, who had language barrier or those who declined to be interviewed were excluded All the pregnant patients with positive response, according to the abuse assessment screen were included in the group A, while every 4th patient with negative response was taken as control SPSS 10 was used for statistical analysis Logistic regression analysis was performed to determine the effect of domestic violence on delivery outcome Relative risk was also calculated Statistical significance was taken at p (2) 005 Results: Four hundred and ten (n=410) antenatal patients were interviewed The factors associated with domestic violence included addiction in 32 (39%), allegedly aggressive nature of husband in 21 (256%) and unemployment of husband in 6 (731%) cases (p=0001) Fifty two per cent women had been victims to more than one form of violence Antenatal complications were not observed in a statistically significant number in either group Depression was identified in 2560% of group A and in 365% of group B (p=0001; RR=201; 95%CI=158-256) Conclusion: Various factors leading to domestic violence were identified among the husbands of women subjected to violence during pregnancy The differences between the two groups, with regard to neonatal outcome (except depression), did not reach statistical significance However, depression was significantly higher in the women enduring violence Language: en

Journal ArticleDOI
TL;DR: Diltiazem may be the first-line treatment for chemical sphincterotomy for the chronic anal fissure and caused fewer side-effects particularly headache than glyceryl trinitrate ointment.
Abstract: OBJECTIVE To compare the symptomatic relief, healing and side-effects of topical diltiazem (DTZ) and glyceryl trinitrate in the treatment of chronic anal fissure. STUDY DESIGN Randomized controlled trial. PLACE AND DURATION OF STUDY The Surgical Outpatient Department of Civil Hospital, Karachi, from March 2006 to February 2007. METHODOLOGY Patients with chronic anal fissure were included in the study and randomized to two groups. One group was administered topical 2% diltiazem hydrochloride and other was given 0.2% glyceryl trinitrate (GTN), perianally twice daily for 8 weeks. Patients with anal fissure due to other diseases like inflammatory bowel disease, malignancy, sexually transmitted diseases, previous treatment with local ointment or surgery; patients who required anal surgery for any concurrent disease like hemorrhoids, pregnant women and patients with significant cardiovascular conditions were excluded. There were four follow-up sessions during the course of treatment. Healing and side-effects were recorded. Analysis was done by SPSS version 10 on intention-to-treat basis. Chi-square was used where appropriate. RESULTS Eighty patients with symptomatic chronic anal fissure were included in the study and equally divided into two groups. After 8 weeks of treatment healing occurred in 31 of 40 patients treated with diltiazem and 33 of 40 patients treated with GTN (p = 0.576). There were less side-effects with DTZ (n=13) than with GTN (n=29, p < 0.001]. In particular, headache occurred more commonly with GTN (n=27) than with DTZ (n=9, p < 0.0001). CONCLUSION Diltiazem hydrochloride and glyceryl trinitrate were equally effective in healing chronic anal fissure. Diltiazem caused fewer side-effects particularly headache than glyceryl trinitrate ointment. Diltiazem may be the first-line treatment for chemical sphincterotomy for the chronic anal fissure.

Journal Article
TL;DR: There was no significant difference between treatment groups in prevention of esophageal variceal rebleeding and difference in mortality rates was also not significant.
Abstract: Objective: To compare the efficacy of propranolol, propranolol with nitrate, band ligation, and band ligation with propranolol and nitrate for the prevention of esophageal variceal rebleeding. Study Design: A prospective randomized trial. Place and Duration of Study: Department of Gastroenterology and Hepatology, Sheikh Zayed Hospital, Lahore, from November 2003 to July 2005. Methodology: One hundred and sixty cirrhotic patients with esophageal variceal bleeding were randomized to four treatment groups (propranolol, propranolol plus isosorbide mononitrate, band ligation, band ligation plus propranolol and nitrate) with 40 patients in each group. Patients were followed for 6 months after the enrolment of last patient. Primary end points were recurrence of esophageal variceal bleeding and death. Treatment complications were noted. Results: Four treatment groups were comparable regarding baseline characteristics. Esophageal variceal rebleeding occurred in 22% patients in band ligation plus drugs group, 26% patients in drug combination group, 31% patients in banding group and 38% patients in propranolol group (p=0.41). Difference in mortality rates was also not significant. Conclusion: There was no significant difference between treatment groups in prevention of esophageal variceal rebleeding.

Journal Article
TL;DR: There was no significant association of risk factors with HBV and HCV infection, thus necessitating the need of universal antenatal screening, however, large scale epidemiological/multicentric studies are required to determine the cost-effectiveness of the screening and prevention of vertical transmission.
Abstract: OBJECTIVE To determine the positive yield of universal antenatal screening of hepatitis B and C and its association with known risk factors. STUDY DESIGN Cross-sectional, analytical study. PLACE AND DURATION OF STUDY The study was conducted from January to December 2006 at Zainab Panjwani Memorial Hospital, Karachi. METHODOLOGY All pregnant women were screened for hepatitis B surface antigen (HBsAg) and hepatitis C virus (HCV) antibodies during antenatal period irrespective of the presence of known risk factors. Carriers i.e. women whose HBV or HCV infection positive status was already laboratory confirmed on first antenatal visit were excluded. Screening was performed by Acu-check one strip test (chromatographic immunoassay) in serum. Women screened positive were interviewed, a questionnaire was filled inquiring about the history of any risk factors predisposing to these infections. Frequency of positive cases was determined in those with risk factors and those without. The proportion was compared using Chi-square test. RESULTS Of the 2592 pregnant women screened during the study period, 09 (0.34%) were HBsAg positive and 18 (0.69%) were HCV antibodies positive. None was found to be a carrier of both viruses. There were 1004 primigravidas, who had no recallable risk factor, and of those, 02 (0.19%) and 09 (0.89%) were HBV and HCV carriers respectively. No significant association of risk factors was seen in both HBV (p=0.310) and HCV (p=0.328). CONCLUSION There was no significant association of risk factors with HBV and HCV infection, thus necessitating the need of universal antenatal screening. However, large scale epidemiological/multicentric studies are required to determine the cost-effectiveness of the screening and prevention of vertical transmission.

Journal Article
TL;DR: The frequency of super infection of hepatitis C and D was found to be highest in HBV cirrhosis patients compared to patients having chronic liver disease (non-cirrhotics and carriers), and genotype D of hepatitis B virus was found dominant in all hepatitis B related complex liver disorders.
Abstract: Objective: To determine the frequency of super infection of hepatitis C and D in patients with hepatitis B related complex liver disorders and the distribution of HBV genotypes in these patients. Study Design: Descriptive study. Place and Duration of Study: The Gastroenterology Unit of PMRC in JPMC, Karachi, from July 2006 to June 2007. Methodology: All patients registered for HBV associated infections were selected. Blood was drawn from 180 patients who fulfilled the inclusion criteria. Those with an incomplete test profile were excluded. All clinical conditions were investigated through liver function tests, coagulation profile, and findings at abdominal ultrasonography, upper gastrointestinal endoscopy and liver biopsy. Liver cirrhosis and hepatocellular carcinoma (HCC) were diagnosed either on the basis of histology, or on a combination of radiological, endoscopic and laboratory data. Hepatitis B virus DNA was extracted from serum, and subjected to a nested PCR using the type specific primers for HBV genotype. Descriptive statistics were used for frequency and mean determination. Results: The 129 patients finally selected for statistical analysis included 108 (84%) males and 21 (16%) females. The age ranged from 6- 68 years (mean=31.5 ±12.39 years). There were 70 (54.2%) patients of non-cirrhotic, chronic hepatitis (CLD), 38 (29.4%) carriers, 12 (9.3%) cirrhotics and 9 (6.9%) HCC patients. Among the 129 patients, 45 (34.9%) were positive for double infection with HDV. These included 35 CLD cases, 7 cirrhotic and 3 carriers, 4 (3.1%) patients were positive for double infection with HCV including one with CLD, 2 with cirrhosis and one with HCC. Triple infection with HBV/HDV/HCV was present in 4 (3.1%) patients who had CLD. Approximately 59% (n=76) patients were not coinfected, though 9 had developed HCC. The genotype distribution of HBV was observed as D in 98 (76%) patients, A in 24 (18.6%), and AD mix in 7 (5.4%). Genotypes B, C, E or F were not found. Accordingly, genotype D strains were the predominant strains among all categories. Conclusion: The frequency of super infection of hepatitis C and D was found to be highest in HBV cirrhosis patients compared to patients having chronic liver disease (non-cirrhotics) and carriers. Genotype D of hepatitis B virus was found dominant in all hepatitis B related complex liver disorders.

Journal Article
TL;DR: The most common factors in the study group were previous history of tuberculosis, repeated courses of ATT, prescribed by different clinicians and unsupervised treatment by a GP during the initial course of ATT.
Abstract: Objective To determine the frequency of common risk factors for multi-drug resistant tuberculosis in patients presented at a tertiary care hospital, Peshawar. Study design Cross-sectional, observational study. Place and duration of study Pulmonology Department, Khyber Teaching Hospital, Peshawar, from December 2006 to October 2007. Methodology Patients with positive AFB culture and sensitivity results and found resistant to both rifampicin and isoniazid with or with resistance to other first line anti-tuberculosis drugs, were recruited from both Pulmonology Ward and Outpatient Department (OPD). Informed verbal consent was taken and a questionnaire administered to all participants of the study. Information regarding demographics, education status, occupation, monthly household income, AFB C/S, details of past history of tuberculosis and family history of TB or Multi-Drug Resistant (MDR-TB) was recorded. Data was analyzed on SPSS version 11. Results A total of 30 patients of MDR-TB were interviewed. Male (n=17) and female (n=13) ratio was 1.3:1. Mean age was 34.2+/-15.3 years. Ninety-two percent female and 52.9% male were uneducated. In 56.7%, monthly income was less than 5,000 rupees and in 40% between 5,000-10,000 rupees. All patients had previous history of Antituberculous Treatment (ATT), in which 20% had undertaken ATT course once, 53.3% twice and 26.7% thrice in the past. In the study group, 13 (43.3%) patients had not completed their first ATT course and 11 of them were receiving ATT from a general practitioner (GP) at that time. Seven (23.3%) patients had family history of TB but no one had documented MDR-TB in the family. Resistance to RH was present in all patients; moreover, 56.7% had resistance to RHEZ+S. Conclusion The most common factors in the study group were previous history of tuberculosis, repeated courses of ATT, prescribed by different clinicians and unsupervised treatment by a GP during the initial course of ATT.

Journal Article
TL;DR: The Urdu version of CBCL and SDQ are both equally valid assessment tools to be used for both clinical and research purpose in Pakistani settings, where Urdu is widely spoken and understood.
Abstract: Objective: To compare CBCL (Child Behaviour Check Llist) Urdu, with the validated Urdu version of Strengths and Difficulties Questionnaire (SDQ) used as “gold standard” among school children in Karachi, Pakistan, and to develop local cutoffs for CBCL using SDQ as a gold standard. Study Design: A cross-sectional study. Place and Duration of Study: Schools of Karachi metropolitan area from January to December 2006. Methodology: The Strengths and Difficulties Questionnaire (SDQ) and Child Behaviour Check List (CBCL) was completed by parents of 5-11 years old primary school children in Karachi. Appropriate cutoff points for total problem, internalizing and externalizing scales were obtained for CBCL. Results: A total of 556 parents filled out both the SDQ Urdu version as well as CBCL. Scores from the parent rated total SDQ scores were highly correlated with the total CBCL scores (r=0.589). The local cutoffs derived for CBCL were considerably lower than USA norms. Slightly higher cutoff for males was found as compared to females for the total CBCL scores. Conclusion: Like the original English version, the Urdu version of CBCL and SDQ are both equally valid assessment tools to be used for both clinical and research purpose in Pakistani settings, where Urdu is widely spoken and understood.

Journal Article
TL;DR: Having the equal effectiveness, ease of monitoring and cost-effectiveness, single loading dose of magnesium sulphate is preferable over the standard regime in the management of pre-eclampsia as a prophylactic measure for prevention of seizure.
Abstract: Objective To determine the efficacy of single dose of magnesium sulphate versus the standard Pritchard regime in the management of pre-eclampsia Study design Quasi-experimental study Place and duration of study Jinnah Postgraduate Medical Centre, Karachi, from January 2004 to January 2006 Methodology All women with severe pre-eclampsia and impending eclampsia were included in the study Patients with pregnancy induced hypertension and mild to moderate pre-eclampsia were excluded From the 100 women included in the study, after matching for age, parity and gestational age, 50 were given only bolus dose of magnesium sulphate and 50 were given the standard regime They were observed for one week for the number of convulsions Fisher's exact test and Chi-square test were used to analyze results Results There was no significant difference in the two groups in term of occurrence of seizures, one patient developed fit with Pritchard regimen The rate of caesarean section was lower in group A, 12% versus 30% in group B (p=005) There was no significant difference in perinatal outcome in either group (82% live births in group A versus 72% amongst group B (p=02) Few side effects like vomiting, dizziness and irritation at the site of injection were observed when standard treatment was used Single dose treatment was also found to be cost-effective costing Pak Rs 45 (US $ 056) as compared to Pak Rs 195 (US $ 24) in control group No maternal death was observed in either group Conclusion Having the equal effectiveness, ease of monitoring and cost-effectiveness, single loading dose of magnesium sulphate is preferable over the standard regime in the management of pre-eclampsia as a prophylactic measure for prevention of seizure

Journal Article
TL;DR: A significant percentage of patients having falciparum malaria with jaundice fulfill the criteria for malarial hepatopathy, and should be considered in patients presenting with acute febrile illness withJaundice so that specific treatment can be given.
Abstract: Objective: To evaluate the clinical, biochemical and sonographic changes in patients with falciparum malaria and jaundice. Study Design: A case series. Place and Duration of Study: This study was conducted at Medical Unit-I (Ward 5), Jinnah Postgraduate Medical Centre, Karachi, from January 2006 to November 2007. Methodology: A total of 62 adult patients, regardless of age and gender, with peripheral blood film evidence of falciparum malaria, who had jaundice, were included. Any patient with evidence of infection with Plasmodium vivax or other causes of liver disease (e.g. viral hepatitis, cirrhosis, portal hypertension, amoebic liver abscess, unexplained hepatomegaly, ascites, history of alcoholism, taking hepatotoxic drugs, past history of jaundice) was excluded on the basis of history, relevant clinical examination and investigations. Results: Age of the patients ranged from 13-48 years (mean 26.04±8.33). All patients were febrile and icteric, with pallor in 67.7%, hepatomegaly in 30.6%, splenomegaly in 70.9% and impaired consciousness in 20%. Serum bilirubin levels ranged from 3 to 24 mg%. Thirty two (51.6%) had serum bilirubin 3-6 mg%, 20 (32.2%) had 6-10 mg% and 10 (16.1%) had >10 mg%. ALT levels ranged from 20-870 IU/L and AST levels 24-1210 IU/L respectively. INR ranged from 1-1.3. Twenty eight patients (45%) had predominantly conjugated or mixed hyperbilirubinemia and serum transaminases were more than three times normal. Ultrasonography revealed hepatomegaly with decreased echogenicity in 22 (35.4%), splenomegaly in 48 (77.4%) and both hepatomegaly and splenomegaly in 16 (25.8%). Gallbladder wall thickness was increased in 5 (8.06%) patients. There was no evidence of biliary dilatation. Conclusion: A significant percentage of patients having falciparum malaria with jaundice fulfill the criteria for malarial hepatopathy. It should be considered in patients presenting with acute febrile illness with jaundice so that specific treatment can be given.

Journal Article
TL;DR: Isolated LPA origin stenosis and MPA hypoplasia were the most common abnormalities and significant associated cardiac lesions were present in one-third of the patients and included PDA, additional muscular VSD, coronary artery abnormalities and MAPCA.
Abstract: Objective: To determine pulmonary artery variations and other associated cardiac defects in patients with Tetralogy of Fallot (TOF). Study Design: Cross-sectional, descriptive study. Place and Duration of Study: The Children’s Hospital and the Institute of Child Health, Lahore, from April 2006 to October 2007. Methodology: All patients with TOF, who underwent cardiac catheterization during this period, were included. Standard cine-angiograms were recorded and pulmonary artery sizing was done using z-scoring. Results: A total of 216 patients with TOF were catheterized. Pulmonary Artery (PA) abnormalities were present in 84 (38.9%) patients. The commonest abnormality was isolated Left Pulmonary Artery (LPA) stenosis (n=27, 32.14%) followed by isolated hypoplasia of Main Pulmonary Artery (MPA) (n=18, 21.43%) and supra-valvular stenosis in (n=11, 13.1%) patients. LPA was absent in one patient, while 2 patients had both absent right and left PA with segmental branch pulmonary arteries originating directly from MPA. Associated cardiac lesions included right aortic arch in 34 (15%), additional muscular VSD vary in 13 (5.5%), Patent Ductus Arteriosus (PDA) in 11 (6%) and Major Aortopulmonary Collateral Arteries (MAPCA) in 2 (1.9%) patients. Significant coronary artery abnormality was present in 10 (4.6%) children. Conclusion: Pulmonary artery abnormalities were present in 38.9% of patients with TOF. Isolated LPA origin stenosis and MPA hypoplasia were the most common abnormalities. Significant associated cardiac lesions were present in one-third of the patients and included PDA, additional muscular VSD, coronary artery abnormalities and MAPCA.

Journal Article
TL;DR: Low-grade inflammation exists in Diabetes mellitus and it is positively related with dyslipidemia (except for HDL cholesterol) in diabetics.
Abstract: OBJECTIVE To measure the levels of inflammatory markers (serum ferritin and high sensitivity C-reactive protein) and dyslipidemia in diabetics and to find a correlation between these inflammatory markers and dyslipidemia STUDY DESIGN Comparative study PLACE AND DURATION OF STUDY Department of Biochemistry and Molecular Biology, Army Medical College, Rawalpindi, from March 2007 to February 2008 METHODOLOGY The study included 30 known type-2 diabetic patients randomly inducted from diabetic clinics of Rawalpindi Healthy volunteers (n=30) having blood glucose less than 6 mmol/L were inducted as the comparison group Fasting blood samples of diabetics and controls were analyzed for glucose, glycated hemoglobin (HbA1c), lipid profile, high sensitivity C-reactive protein (hs-CRP) and serum ferritin RESULTS The diabetic subjects had significantly higher levels of glucose, HbA1c, total cholesterol, LDL cholesterol, triglycerides, hs-CRP and ferritin as compared to normal subjects (p 005 r=-010) CONCLUSION Low-grade inflammation exists in Diabetes mellitus and it is positively related with dyslipidemia (except for HDL cholesterol) in diabetics

Journal Article
TL;DR: For preseptal cellulitis, insect bite was the most common cause in group I and trauma was the leading cause in groups II and traumaWas the leadingCause in group II, and sinusitis was the important cause in Group I and Sinusitis in Group II.
Abstract: Objective To estimate the frequency and major risk factors of preseptal and orbital cellulitis. Study design A cross-sectional analytical study. Place and duration of study The Khyber Institute of Ophthalmic Medical Sciences, Hayatabad Medical Complex, Peshawar from July 2003 to December 2006. Methodology All consecutive patients between ages 6 and 40 years, admitted to the institute with the diagnosis of preseptal and orbital cellulitis were included in the study. The patients were divided into two groups. Group I included patients with ages 6-16 years and group II with ages 17-40 years. The clinical features, diagnosis and risk factors were entered on a specially-designed proforma. The risk factors included were trauma, insect bite, localized or systemic infection and postsurgical. Odd ratio and p-values were calculated for potential risk factors. Results The frequency of orbital cellulitis was 0.1% of total admission. Out of 26 patients, 42.30% patients were in group I and 57 in group II. In group I, insect bite was the most common risk factor identified in 40% of patients with preseptal cellulitis and trauma as a common cause in 50% with orbital cellulitis. In group II, trauma was the leading cause in 50% of patients with preseptal cellulitis and sinusitis as a common cause in 18.1% with those of orbital cellulitis. In both groups the p-values were found insignificant (p>0.5). Complications included cicatricial ectropion in 44.4% and orbital abscess in 41.1%. Conclusion For preseptal cellulitis, insect bite was the most common cause in group I and trauma was the leading cause in group II. For orbital cellulitis, trauma was important cause in group I and sinusitis in group II.

Journal Article
TL;DR: Her-2/neu, ER andPR expression is comparable to the West with inverse association with ER and PR and positive association with lymph node metastases and no association was seen between tumour size and tumour grade.
Abstract: Objective: To determine the morphological profile and association of HER-2/neu expression with histological prognostic markers and hormonal receptor expression in female breast carcinoma in Northern Pakistan. Study Design: Cross-sectional, observational study. Place and Duration of Study: Department of Histopathology, Armed Forces Institute of Pathology, Rawalpindi, from January 2004 to May 2007. Methodology: A total of 535 patients of primary operable female breast carcinoma with hormonal profile and biological status were included in the study. Patient’s age, microscopic tumour size, tumour grade, lymph node status, Estrogen Receptor (ER), Progesterone Receptor (PR) and joint ER/PR status were evaluated and their association was determined with HER-2/neu expression using the x2 test for univariate analysis. Results: Out of 535 cases, there were 481 (89.9%) cases of infiltrating ductal carcinoma with mean age of 48 years and mean tumour size of 4.4 cm. Tumour grade II was seen in 68% cases and lymph node metastases were present in 65% cases. HER-2/neu expression was seen in 31% cases, while ER and PR expression was seen in 72.3% and 62.6% respectively. ER and PR showed inverse association with HER-2/neu while positive association was seen with lymph node metastases (p <0.05). No association was seen between tumour size and tumour grade. Joint ER and PR expression also showed a higher number (73.5%) of HER-2/neu negative cases. Conclusion: HER-2/neu, ER and PR expression is comparable to the West with inverse association with ER and PR and positive association with lymph node metastases. Since HER-2/neu expression is variable in carcinomas with different tumour characteristics, it is not possible to predict hormone receptor expression in all the cases.

Journal Article
TL;DR: The main indication of EUS and pathology of mediastinal and celiac nodes were metastatic malignancy and tuberculosis and Pancreatic adenocarcinoma was another common cause for asking EUS.
Abstract: Objective To determine common indications for requesting Endoscopic Ultrasound (EUS) and to describe the diagnosis made after endoscopic ultrasound/Fine-Needle Aspiration and Cytology (FNAC) during two years at a tertiary gastrointestinal unit. Study design Cross-sectional descriptive study. Place and duration of study The study was carried out in Gastrointestinal Department of Military Hospital, Rawalpindi, from March 2006 to February 2008. Methodology One hundred and eighty nine patients who underwent EUS during study period at Military Hospital were included in the study. Patients too ill (hypoxemic/hypotensive) to undergo procedure safely and those with complete esophageal blockage at upper end by tumour not allowing scope/EUS probe to advance beyond were excluded. EUS was done with Olympus Exera EUS 160, linear or radial scope, as required. EUS findings were recorded against indications as enlarged lymph nodes, tumour, staging, normal or incomplete. Fine-Needle Aspiration (FNA) was done as per findings on EUS using 21-22 G needle. An on-site cytopathologist made the provisional cytopathological diagnosis. Final cytology/histopathology report was given after review of slides by consultant histopathologists at Armed Forces Institute of Pathology (AFIP), Rawalpindi, and were documented as tuberculosis, malignancy, chronic pancreatitis or reactive hyperplasia. Data was analyzed for documentation of patients' age, gender, common indications, findings on EUS/FNAC, using SPSS version 10. Percentages and frequencies were calculated for the presence of these above-mentioned variables. Results Of the 189 patients, 145 (77%) were male and 44 (23%) female. Age was 18-80 years (mean 49 years). Major indications for referral were lymphadenopathy in 92 (49%), suspected growth pancreas in 57 (28%), growth of stomach in 20 (11%) and a heterogeneous group included esophageal, liver, retroperitoneal masses, rectal and other pathologies. Findings on EUS included lymphadenopathy in 76, mostly in sub-carina and AP window. Mass in pancreas was seen in 36, followed by stomach tumour in 17 and esophagus in 9. FNAC was done in 142 out of 189 patients. Final diagnosis out of 67 FNAC/histopathology of lymph nodes were tuberculosis in 26 and malignant lesions in 23. These included metastatic adenocarcinoma in 8, lymphoproliferative disorder in 7, metastatic squamous cell carcinoma in 5, small cell carcinoma in 2 and anaplastic in 1. Pancreatic tumours were adenocarcinoma in 16, poorly differentiated in 3 and neuroendocrine in 2. Stomach tumours were found in 11, and included lymphomas 5, GIST 3, carcinoids 2, metastatic choriocarcinoma 1 and adenocarcinoma in 1. Therapeutically, 3 celiac blocks and one pancreatic pseudocyst drainage was done. Conclusion The main indication of EUS and pathology of mediastinal and celiac nodes were metastatic malignancy and tuberculosis. Pancreatic adenocarcinoma was another common cause for asking EUS.

Journal Article
TL;DR: First premolars in all the quadrants were the most frequently involved teeth in NCCLs, and a weak positive correlation was found between age and N CCLs.
Abstract: Objective: To determine factors associated with Non-Carious Cervical Lesions (NCCLs) and the teeth most commonly involved in such lesions. Study Design: Cross-sectional study. Place and Duration of Study: Department of Operative Dentistry, Section of Dentistry at Aga Khan University Hospital, Karachi, Pakistan, from December 2005 to January 2006. Methodology: A total number of 95 patients with 671 teeth were evaluated using a pre-coded questionnaire. Subject of evaluation was teeth. Patients with symptomatic or asymptomatic NCCLs and permanent dentition were included. Patients exhibiting active, untreatable periodontal disease, rampant uncontrolled caries, xerostomia, primary dentition, patients undergoing orthodontic treatment or bleaching procedure were excluded from the study. Data analysis was done using Spearman’s correlation, Mann Whitney test and Kruskal-Wallis test. Results: Mean age of patients was 50.3 years (r=0.22, p=0.028); males (73%) had more NCCLs than females (23%). Majority (45.3%) of them brushed their teeth twice a day, with medium type of brush (48.4%) and horizontal (73.7%) brushing technique. Most of the patients were non-bruxists (90.5%), with Angles Class 1 occlusion (48.4%) and canine guidance (50.5%). Majority (74.7%) of the patients did not have sensitivity. Conclusion: First premolars in all the quadrants were the most frequently involved teeth in NCCLs. More males had NCCLs. Middle aged patients were more involved. A weak positive correlation was found between age and NCCLs. No association was observed between hand used and site of NCCLs, between wear facets and NCCL, Excursive guidance and NCCL, Angles classification and NCCL.

Journal Article
TL;DR: The different clinical features in two affected individuals of different families with particular reference to characteristic findings of this syndrome are described.
Abstract: Crouzon syndrome is an autosomal dominant condition characterized by craniosynostosis with associated dentofacial anomalies. This report describes the different clinical features in two affected individuals of different families with particular reference to characteristic findings of this syndrome.