The role of thoracic endovascular aortic repair (tevar) of thoracic aortic diseases in patients with connective tissue disorders - a literature review

Abstract Objective: To review the currently available literature to define the role of thoracic endovascular aortic repair (TEVAR) in patients with connective tissue disorders (CTD). Methods: A comprehensive electronic database search was performed in PubMed, SCOPUS, Embase, Google scholar, and OVID to identify all the articles that reported on outcomes of utilizing TEVAR in patients with CTD during elective and emergency settings. The search was not limited to time or language of the published study. Results: All the relevant studies have been summarized in its correspondence section. The outcomes were analyzed in narrative format. The role of TEVAR has been elaborated as per each study. Currently, there is limited large cohort size studies outlining the use of TEVAR in patients with CTD. The use of endovascular repair in patients with CTD is limited due to progressive aortic dilatations and high possibility of further reinterventions at later stage of life. Conclusion: Open repair remains the gold standard method of intervention in young patients with progressive CTD, especially in the setting of acute type A aortic dissection. However, TEVAR can be sought as a reliable alternative in emergency setting of diseases involving the descending thoracic aorta; yet the long-term data needs to be published to support such practice.

Evaluation of antimicrobial activities of Harmine, Harmaline, Nicotine and their complexes

Harmine, Harmaline, Nicotine and its various complexes synthesized have been characterized by physical, spectral and analytical methods and curtained for in-vitro antimicrobial activity against different bacterial and fungal species at two different concentrations i.e.100micro g/100micro l and 200micro g/100micro l dose level respectively. Analysis showed that Nicotine, Zinc-Nico, Cd-Nico, Hg-Nico, Ni-Nico, Cu-Nico, Co-Nico, Harmine, and Harmaline having conc. of 100ug/ 100ul had antibacterial activity on zero, 5, 4, 10, zero, 5, 7, zero, zero strain of bacteria having an average of zero [SD=0.0000], 15.2000 [SD=1.30384], 18.2500 [SD=3.30404], 20.2000 [SD=1.39841], zero [SD=0.0000], 14.6000 [SD=0.89443], 15.8571 [SD=1.34519], zero [SD=0.0000], zero [SD=0.0000] respectively. Zinc [II] chloride, Cadmium [II] Iodide, Mercury [II] chloride, Nickel [II] chloride, Copper [II] chloride, Cobalt [II] chloride, Mercury [II] chloride, Mercury [II] harmine, Mercury [II] harmaline at 100ug/100ul is valid for 7, 8, 9, 2, 7, 8, 9, 10, 8 strains of bacteria with an average of 7.1429 [SD=1.06904], 10.0000 [SD=5.01427], 14.8889 [SD=6.00925], 6.0000 [SD=0.0000], 8.5714 [SD=4.27618], 8.2500 [SD=0.88641], 14.8889 [SD=6.00925], 18.6000 [SD=2.45855], 18.5000 [SD=1.85164] respectively. The above given compounds at the conc. of 200 micro g/100ul is valid for 10, 9, 10, 8, 8, 10, 10, 10, 10 strains of bacteria with an average of 8.1 [SD=1.66333], 11.7778 [SD=5.28625], 16.1000 [SD=6.36745], 6.5000 [SD=0.92582], 9.7500 [SD=4.43203], 9.9000 [SD=2.76687], 16.1000 [SD=6.36745], 22.0000 [SD=2.44949], 20.4000 [SD=2.75681] respectively. The above given compounds at conc. of 200 micro g/100ul showed antibacterial action on 3, 8, 8, 10, 3, 9, 8, zero, 3 strains of bacteria with an average of 14[SD=0.000], 16.8750 [SD=1.35620], 18.2500 [SD=3.45378], 22.7000 [SD=1.82878], 14.3333 [SD=0.57735], 16.7778 [SD=1.71594], zero [SD=0.000], 12.0000 [SD=1.00000] respectively. Hence according to the average value of the zone of inhibition, maximum antibacterial activity at 100-200ug/100ul is of Hg-Nico and Mercury salt; Mercury [II] harmine having an average of 20.2000 [SD=1.39841]-22.7000 [SD=1.82878] and 18.6000 [SD=2.45855]-22.0000 [SD=2.44949]. Minimum antibacterial activity at 100-200ug/100ul is Nicotine100, Nicotine-Nico100, Harmine 100,Harmaline 100, Harmine 200 having zero average [SD=0.000]

Postoperative complications in primary definitive management of hard and soft tissues in mandibular gunshot injuries; a review of 47 cases

Background: Firearm injuries cause significant morbidity and mortality among its victims. The type and extent of tissue damage vary from simple small size wound to a large soft tissue or skeletal defect. The selection of the appropriate surgical technique is as important as the timing because incorrect selection or improper application of surgical techniques may also lead to infection, sequestration, wound dehiscence, graft rejection, facial deformity and subsequent re-visional operations

Objective: The present study, carried out at Oral and Maxillofacial Surgery department, King Edward Medical University/ Mayo Hospital, Lahore, was aimed at highlighting the frequency of Postoperative complications in primary definitive management of hard and soft tissues in mandibular gunshot injuries

Study design: Descriptive case series

Setting: Department of Oral and Maxillofacial Surgery, King Edward Medical University/ Mayo Hospital, Lahore, from November 2010 to November 2012; including 6 months follow up

Patients and methods: The study was conducted on 47 patients. All patients suffered gunshot injuries to the mandible and had soft and hard tissue defects at a single site. Patients ranged in age from 17 to 54 years with a mean age of 32.31 years. Males dominated in the study and were n=37 and females n=10. All patients were treated definitively in the first operation by addressing the hard and soft tissue defects

Results: All had single site fracture with angle of the mandible being most common 27 [57.44%]. For reconstruction of soft tissue defect local advancement by undermining and primary closure was carried out in 35 [74.47%] patients, buccal pad of fat in 4 [8.51%] patient, deltopectoral flap in 5 [10.64%] patients and skin graft in 3 [6.38%] patient. For hard tissue reconstruction, nonvascularized bone graft was given in all cases i.e. n=47 [100%]. Iliac crest bone graft was given in 34 [72.34%] patients, rib graft in 6 [12.77%] patients and symphyseal outer cortex bone graft in 7 [14.89%] patients. Post-operative complications were noted in terms of infection, plate exposure, plate fracture and malocclusion which appeared to be 9 [19.1%], 5 [10.6%], 2 [4.3%], 5 [10.6%] respectively

Conclusion: All patients in this series required surgical intervention for treatment of their facial gunshot wounds. Primary definitive hard and soft tissue management can be considered in patients suffering from gunshot injuries to the mandible. Although post-operative complications can result at higher rates in such injuries yet properly selected surgical techniques and post-operative infection control can produce excellent desired esthetic and functional results

Male preponderance of Ziqun Nafas Sho'bi among 0-14 years age group in Padrayanpura Bangalore an epidemiological study

The present study is a one-time observational cross sectional, field survey based study undertaken to know the prevalence of Ziqun Nafas Sho'bi in Padrayanpura, Bangalore. The sample size was estimated as 555 subjects based on known 15.32% prevalence of Ziqun Nafas Sho'bi in Bangalore. Detail of the population and houses of the area were taken from the Bangalore Municipal Corporation. Face to face interview was performed using a structured pre test questionnaire. In the study population the prevalence of asthma among males in age group of 0-14 years was 42.3%, among 15-29 years group it was 11.95%, among 30-44 years it was 12.12%, among 45-60 years it was 15.15%, and among >60 years age group was 20.83%. P<0.000.1, Chi-Square test showed that the findings were statistically highly significant. It is concluded from the study that the prevalence of asthma is increasing day by day in Padrayanpura, the field area of NIUM, Bangalore, and it is more prevalent among males in 0-14 years age group as compared to other age groups

Frequency of catheter related blood stream infections due to indwelling temporary double lumen catheter with respect to duration of catheterization in hemodialysis patients

Background: Temporary hemodialysis catheters are an important means of gaining immediate vascular access. Catheter related blood stream infection [CRBSI] is the most important complication limiting its use. Frequency of CRBSI increases with duration of catheterization

Objective: To find the frequency of catheter related blood stream infections due to indwelling temporary double lumen catheter with duration of insertion in hemodialysis patients

Materials and methods: This is a hospital based cross-sectional study which includes 40 renal failure patients requiring dialysis with temporary hemodialysis catheter who were found to have CRBSI. The frequency of catheter related blood stream infections due to temporary double lumen catheter in relation to duration of insertion in hemodialysis patients was calculated

Results: Mean duration of catheterization till CRBSI developed was 16.97+/-8.56 days, although, it varied from 7 to 37 days. The frequency of CRBSI at 1[st] week, 2[nd] week, 3[rd] week, 4[th] week and more than 4 weeks was 4 [10%], 14 [35%], 11 [27.5%], 4 [10%] and 7 [17.5%] respectively. A sub-group analysis of frequency of blood stream infections with respect to duration of catheterization amongst diabetics against non-diabetics, internal jugular [IJ] against subclavian [SC] vein site, optimal against sub-optimal hygienic conditions, males against females and amongst various age groups was conducted and did not show any statistically significant difference. [p value> 0.05]

Conclusion: The frequency of CRBSI in temporary hemodialysis catheters increases with duration of catheterization and is maximum at 2nd week post catheterization. We recommend duration of catheterization should be limited to less than two weeks in hemodialysis population

Assessment of Cedrus deodara root oil on the histopathological changes in the gastrointestinal tissues in rats

The present study was designed to investigate the effect of Cedrus deodara root oil on the histopathology of different gastrointestinal organs of Wistar rats. This oil was used traditionally as an anti-ulcer agent in the Indus Unic System and extracted from the plant root by destructive distillation method. A total of 90 rats were taken and divided into groups A, B and C, each comprising of 30 animals. The animals of group B and C were given 0.5 ml/kg and 2.5 ml/kg of C. deodara oil respectively while group A served as control and administered vehicle only. The treatment was given to the animals ones only for 24 hours. All animals were sacrificed and the organs like esophagus, stomach and ileum were taken out. Tissue processing and staining procedure was then carried out for any pathological changes in the animal tissues during microscopic examination. The results indicated that Cedurs deodara root oil at both doses 0.5ml/ kg and 2.5 ml/kg exhibited some adverse effects such as erosion of epithelium, edema on sub-mucosal and mucosal layers, congestion of blood vessels as well as presence of inflammatory cells on esophagus, stomach and ileum were seen. Moreover shortening of villi was also seen at both doses. A study conducted on mammalian toxicity previously on rats revealed that the C. deodara root oil used is not very toxic and comes under least toxic group as standardized by toxicologists. Based on the results obtained it was concluded that C. deodara root oil produced some adverse changes in the tissues of GIT when given at 0.5 ml/kg and 2.5 ml/kg doses but the effects were not lethal therapeutically at this dose LC[50] 16.5 ml/kg. The plant oil showed some toxicity and needs further detailed studies to assess its potential toxicity and therapeutic status before using this material as drug

Urological complications in ureteric stenting live related renal transplantation

To determine the frequency and types of post-transplant urological complications in live-related kidney transplantation with reference to the impact of JJ ureteric stent. Case series. Shaikh Zayed Postgraduate Medical Institute and National Institute of Kidney Diseases, Lahore, from June 2006 to July 2010. Consecutive renal transplantations, donors being alive were relatives, reterospectively reviewed. All patients underwent extravesical ureteroneocystostomy and all, except one were stented. From the retrieved clinical records, the frequency and types of various minor and major urological complications and their management was studied. All the complications were managed according to standard guidelines. The overall incidence of urological complications among transplant recipients was 11.9%, observed in 12 patients. The complications were urinary leakage in 2 [2%] and clot retention, ureterovesical junction obstruction and wound infection in one [1%] patient each. Urinary tract infection was observed in 7 [6.9%] patients. Urinary tract infection was the most common urologic complication in the studied cases. The technique of stented extravesical ureteronecystostomy had a low rate of urological complications in this series. Other factors which may reduce the urological complications are preserving adventitia, fat and blood supply of ureter by delicate dissection during donor nephrectomy and prevent kinking and twisting of ureter are important factors in reducing the post-transplant urological complications

Extracorporeal shock wave lithotripsy; outcome in pediatric urolithiasis

[1] To determine the efficacy of extra-corporeal shock wave lithotripsy with reference to stone size, site and radiodensity in children. [2] To determine acute early complications during and following extra-corporeal shock wave lithotripsy in children. Analytical case series study. Fifty patients of either sex below the age fourteen [14 years] having renal, ureteric and bladder stones between 5-20 mm in size along with their long axis presented to department of urology Shaikh Zayed Hospital Lahore during the period of one year extending from 02-02-2008 to 02-02-2009 were included in this study. Children with serum creatinine level greater than or equal to 3mg/dl, obstruction distal to the stone, patient with active bleeding disorders, uncorrected hypertension, patient unfit for general anaesthesia, untreated urinary tract infection and patients with gross anatomical anomaly were excluded from study. All the patients were given shock waves under intravenous sedation or general anaesthesia in a standard manner on out door basis. All the children were evaluated for stone clearance and early complications at first 24-72 hours. At the end of three months ESWL treatment was considered successful, if the patients were stone free or had residual fragments 4 mm or less in size. Out of the fifty patients, 40 [80%] were males and 10 [20%] females with male to female ratio of 4.1. The age range of patients was 2-14 years [mean +/- SD 9.24 +/- 3.48 years]. Thirty three patients [66%] had renal stones, 7 [14%] ureteric and 10 patients [20%] had bladder stones. Single successful treatment session was noted in 22 patients [44%], 18 patients [36%] received two sessions and 10 patients [20%] required three sessions for successful stone fragmentation. Seventeen patients did not show stone clearance even after three sessions within three months follow-up. Twenty one patients [42%] felt pain after lithotripsy session, and they were given injectable analgesia and the pain settled, haematuria in 17 patients [34%], impacted stone in five [10%], ureteric colic and urinary tract infection in three, three cases respectively and only one case developed steinstrasse. Twenty patients [40%] developed minor complications of anaesthesia, like nausea, vomiting which relieved with injectable antiemetics

Live renal donors; comparison of CT angiographyand per operative findings

To compare the findings of helical computed tomographic angiography and intra-operative findings in live related donors. To evaluate the accuracy of helical computed tomography with advanced 3D techniques in depicting the renal vasculature, parenchymal and anatomy of collecting system. Sheikh Zayed Post Graduate Medical Institute and National Institute of kidney diseases Lahore. Between June 2006 to May 2009 eighty potential donors underwent CT angiogram as a part of their preoperative workup. We retrospectively studied the CT angiogram and compared the finding with the surgical findings. The results were reviewed with radiologists to determine the discrepancy in discordant cases. The accuracy of CT angiography was 93.40% to predict number of vessels. Five arteries and one vein was missed, this disconcordant comprised 7.59% during initial CT interpretation. The overall concordance between CT angiography and operative findings in delineating the arterial anatomy was found in 74 [93.67%] and venous in 78 [98.73%] donors. All CT scans demonstrated normal collecting system except one, which showed a dilated right pelvicalical system and ureter. Simple renal cysts about the size of 2-4 cm were found in the four left kidneys. CT scan supplied additional important anatomical information including kidney size and the presence of nephrolithiasis. Helical CT angiography is very specific for arterial and venous anatomy as well as other anatomical and functional details. It provides all the information required by a surgeon. It can become the single imaging modality for preoperative assessment of potential donors in place of conventional angiography and intravenous urography. CT angiography is minimally invasive and cost effective

Copper sulphate toxicity in a young male complicated by methemoglobinemia, rhabdomyolysis and renal failure

Copper sulphate is a compound prepared by the action of sulphuric acid on copper II. Copper sulphate is widely used as fungicide, herbicide and for photography. In a human being, it can lead to anemia. Medical literature is lacking regarding accidental or suicidal poisoning cases of copper sulphate in Pakistan. We present a case of accidental ingestion of copper sulphate resulting in severe acute toxicity, which was successfully managed by intensive supportive measures and Dimercaprol [BAL]

spectrum of intradural spinal tumors

To assess the spectrum of clinical, radiological and histological features of patients with intradural spinal tumors. This descriptive study was carried out in Department of Neurosurgery Lady Reading Hospital Peshawar, from April 2003 to March 2009. Medical records of patients with spinal tumors were reviewed and patients operated for intradural spinal tumors were identified. A total of 312 patients, out of 525 cases of spinal tumors, with different intradural spinal tumors were considered in this study. Their clinical features, radiological reports, peroperative findings and histological reports were analyzed in different aspects. There were total of 312 patients with age range from 2 years to 74 years, with median age of 38 years. Out of these 187 were males and 125 were female, overall male to female ratio of 1.5: 1. Backache, leg weakness, parasthesia and poor sphincters were the main clinical features. MRI spine [274 cases] was the main diagnostic tool along with plain X-rays and X-ray myelography in limited cases [35 cases]. CT myelogram was done only in 3 cases. The common site of involvement was dorsal spine followed by lumber and cervical spines respectively in 185, 80 and 47 cases. Histological report was suggestive of Neurofibroma in 166 Meningioma in 96, Ependymoma in 20, Dermoid in 12, Astrocytoma in 7, Hemangioblastom and Tuberculoma in 3 cases each and Hydatid cyst in one case. Neurofibroma and meningioma constituted majority of cases belonging to intradural extramedulary group, while ependymoma and astrocytoma were common intramedullary tumors. Third and 5[th] decade of life was the common age group for both Intramedulary and extramedulary tumors. Intramedulary lesions were common in 3[rd] decade of life

Additional chromosomal abnormalities in Philadelphia-positive chronic myeloid leukemia

The emergence of non-random chromosomal abnormalities is a well-recognized occurrence in chronic myeloid leukemia [CML] and detection of these abnormalities is important in prognostic stratification. The frequency and types of additional chromosomal abnormalities in CML patients has not been determined in our region. We conducted a descriptive, prospective study of additional chromosomal abnormalities in patients with an established diagnosis of Philadelphia-positive CML from May 2001 to June 2007. Cytogenetic studies were repeated every three months with the conventional G-banding technique and described according to the international system for Human Cytogenetic Nomenclature. All patients received imatinib mesylate. In 219 patients with Philadelphia-positive CML, 34 [15.5%] [median age, 38 years] developed 51 additional chromosomal abnormalities. Five cases had variant translocations prior to starting imatinib; the remaining 29 cases acquired chromosomal abnormalities after starting imatinib, including 8 cases that received prior interferon-alfa. Twenty-one patients were in chronic phase, 10 in accelerated phase and 3 were in blast crisis. Trisomy 8 was the most frequent abnormality followed by random chromosomal abnormalities and variants of the Philadelphia chromosome. The overall frequency of additional chromosomal abnormalities was similar to that in previous reports. Early identification of these abnormalities may help in adapting to a more appropriate therapeutic approach

analysis of one thousand chest radiographs at a secondary care center

To find the workload of radiology department consisting of chest x-ray, clinical notes writing trends, and, percentage of radiographs showing abnormalities, One Thousand Chest X-Rays were studied at the department of radiology, CMH Murree from April 2002 to August 2002. Chest x-ray made up 28% of the workload. Three major symptoms of patients were cough [7%], Fever [6%] and chest pain [5%]. Only 9.2% films had significant findings. Pulmonary Tuberculosis [2.1%] cardiomegaly [2.1%] consolidation [1.4%] and pleural effusion [.8%] were the major radiological findings. X-ray chest constitutes the major bulk of radiographic workload at our institution and Tuberculosis was the commonest radiological finding

Foley's catheter in management of subglottic and tracheal injuries

In patients of Sub glottis and tracheal stenosis adequate lumen is achieved by endoscopic diathermization or open surgical techniques. However it is difficult to maintain patency of the created lumen due to formation of granulation and fibrous tissues. Many prosthetic devices like Laryngeal stents, Montgomery T tubes are available which are inserted and fixed after attaining an adequate lumen during a surgical procedure. Purpose of this study was to design a cheap and easily available prosthetic device for such patients. It is a descriptive case series study of ten cases. A specially tailored stent of silicone lined Foley's catheter, which is freely available and economically priced was used for Subglottic and Tracheal stenosis. Good air way was achieved after one year with no decanulation difficulty in 9 cases [90%] with an unsatisfactory result [10%]. After removing the folly's catheter Tracheostomy tube was corked for one month to observe the patency of newly formed airway in the Subglottic and upper tracheal region. When no difficulty in air way was observed it was finally taken out and the Tracheostomy was allowed to close by itself. Silicone lined specially tailored stent of Foley's catheter is a good cheap alternate addition in prosthetic devices for restoration lumen in Subglottic and Tracheal stenosis

Contributing factors of rickets among children at hyderabad

To determine the factors contributing to nutritional rickets among the children presenting at Isra University Hospital Hyderabad, Sindh - Pakistan. A case control study. The Pediatrics Department of Isra University Hospital Hyderabad from August 2004 to August 2005. Children less than 5 years of age of both sexes were included in study. Sixty children with rickets and 60 control children matched for age and social characteristics over study period were studied. Diagnosis was made on clinical, radiological and biochemical parameters. A specially designed questionnaire was administered, for patients and mothers of control subjects to assess the role of social, nutritional and other related factors in the pathogenesis of nutritional rickets. Biochemical investigations included estimation of serum calcium, serum phosphorus and alkaline phosphates. At the time of diagnosis, mean bodyweights of the patients and controls were 9.30 and 10.17kg respectively. Mean heights at the time of diagnosis were 60.08 and 62.38 cm for the patients and the controls respectively. Mean serum calcium and serum phosphate were significantly lower in the patients compared with the controls. Alkaline phosphates were higher among the patients. The weaning diet was started at the age of 6 months only in 30% of cases of rickets, compared with 52% of controls who started at the 6 months of age. Only 41% of mothers of children with rickets had normal nutritional status and 75% of control mothers had normal nutritional status. Most of the children with rickets 75% were completely wrap during infancy compared to controls; only 20% wrapped. There are a lot of contributing factors for nutritional rickets. However, several factors seem to make more important contribution. Among these, lack of exposure to sunlight, prolonged breast-feeding without supplementation and inadequate complementary feeding practices are important. Maternal health and education is important as it can influence all of the above factors

utility of red cell discriminant function [DF] in screening for trait

This study was conducted to determine the frequency of minor in adult males at a tertiary referral center by using discriminant function [DF] and to compare mean MCV [mean corpuscular volume] and mean RBC [red blood cell] count in minor and non thalassaemia minor group. This was an observational cross-sectional study. Place and Duration of Study: This study was conducted at the Clinical Laboratory, Agha Khan University Hospital from 1[st] August 2003 to 31st October 2003. Blood samples were taken from normal individuals in EDTA and were run on Coulter STKS. The indices that were taken into consideration were haemoglobin, RBC count and MCV, DF was calculated by formula as proposed by England and Fraser. Discriminant Function = MCV- [5xHaemoglobin]-RBC-3.4. If the value was less than zero; it was interpreted as suspected thalassaemia minor. Mean MCV and mean RBC count in thalassaemia minor and iron deficiency anaemia were calculated and compared. A total of 1270 individuals were included in the study. In 70 individuals the red cell indices were found to be low their DF was calculated and thalassaemia minor was suspected in 49 individuals and this was confirmed with haemoglobin electrophoresis in 25 patients. Mean MCV was almost the same in both thalassaemia minor and iron deficiency anaemia. However, mean RBC count was relatively higher in case of thalassaemia minor. Discriminant function can be a good screening tool for evaluating patients with low red cell indices. However further and confirmation with haemoglobin electrophoresis is required for labeling a patient as having thalassaemia minor

Cauterization in unani system of medicine a review

In Unani system of medicine, treatment of a patient is performed by administering medicines [Ilaj bil-Dawa], by adopting suitable technique of interference in six-essentials of life [Regimental therapy] and by using manual art and surgical instruments [Ilaj bil-Yad/Ilaj bil-Hadid]. Since ancient time Ilaj bil-Yad consists of treatment of fractured bones, reduction of dislocated bones, incision of body tissues, amputation and excision of diseased body parts, cauterization of diseased body parts and stitching of incised and damaged tissues. In Arabic literature, Ilaj bil-Yad is also termed as Ilaj bil-Hadid [treatment by surgical instrument] or Sana'at ul-Yad [Manual Art or Operative Procedure]. Cauterization is reckoned in the category of manual treatment and it is performed either by fire [Hot metal] or by strong corrosive medicine. Now it is being realized that if cauterization is performed/applied as a common surgical procedure in the contemporary medical practise, it may provide successful treatment for a large number of diseases

Hairy Cell Leukemia: clinical presentation and long term follow up after treatment with 2-Chlorodeoxyadenosine [2-CdA]

The aim of the study was to the clinical features and long term follow up after treatment with Cladarbine in a tertiary care hospital. Seven patients with hairy cell leukemia were diagnosed between January 1990 till December 2003. Diagnosis in all the patients was established by bone marrow aspirates and trephine biopsy along with TRAP. In two patients the diagnosis was supplemented by flowcytometry and in another two patients by splenectomy. Six patients were male while one was female. Mean age was 47.7 years [range 36-64]. Most common presenting features were pallor and weakness [n=5]. All patients had splenomegaly. Blood count at presentation revealed that one patient had bicytopenia, two had isolated thrombocytopenia, and three had pancytopenia. Treatment responses were evaluable in seven patients. Complete response was seen in six patients [85.7%]. One patient died after two months due to sepsis while 3 [50%] patients relapsed. Those who relapsed received another course of CDA and have maintained remission with a median duration of response of 48 months [20-48]. From this small series we can conclude that CDA is an effective treatment for HCL and even it works very well in relapsed cases.

Freequency of FAB subtypes i acute myeloid leukemia patients at Aga Khan University Hospital Karachi

Acute myeloid leukemia [AML] is a heterogeneous disease. Therefore, various parameters are needed to classify this disease into subtypes, so that specific treatment approaches can be utilized effectively. The commonly used method for diagnosis and classification is based on FAB criteria using morphology and cytochemical stains. For sonic of the categories, imnninophenotyping is necessary. The aim of present study is to determine the frequency of various sub types in acute myeloid leukemia using FAB criteria in our population. This will aid in the correct diagnosis of acute leukemia and hence proper management of the patients. Materials and This is descriptive case control study conducted at Aga Khan University Hospital from January 1999 to December 2000. The total number of subjects was 116 that included both adults and children. The patients were diagnosed on the basis of bone marrow morphology using FAB classification. Cytochemistry was done in all cases, while immunophenotyping was considered only in those cases that were found to be problematic. Among 116 patients, 70 were males and 46 were females with male to female ratio 1.5:1. The age ranged between 6 months to 85 years with a mean age of 32 years. AML-M4 was the predominant French American-British [FAB] subtype [36.2%] followed by M2 [30.25%], M3 [10.4%], M1 [7.7%]. M5a [3.5%], M5b [2.5%] and M6 [0.8%]. Conclusions: The most common FAB subtype observed in our study was Acute myclomonocytic leukemia [M4] which is in accordance with studies reported from Saudia Arabia and a previous study reported from our institution. However, other national and international studies have reported Myeloblastic Leukemia with maturation [M2] as the predominant subtype of AML