Combined tricuspid atresia- AV septal defect-a rare congenital cardiac abnormality.

INTRODUCTION: Congenital heart diseases (CHDs) are one of the most common birth defects worldwide with a prevalence of 1%. CHDs can be classified into cyanotic and acyanotic diseases based on the presence or absence of the characteristic bluish discoloration of skin and mucus membranes. A subset of cyanotic diseases is single ventricle malformations. This group of disorders comprises 1% of all CHDs. A remarkable yet rare and underreported entity of single ventricle malformations is combined tricuspid atresia (TA) and atrioventricular (AV) septal defect which is characterized by the anatomical features of both entities. Combined TA-AV septal malformation was first anatomically described in 1953 and further explored through echocardiography and cardiac catheterization in 1987 and then 1991. Since then, no studies have been documented in the literature prompting us to share our findings. METHODS: Herein we are describing a rare and underreported cardiac lesion based of a retrospective revision of medical charts at the American University of Beirut Medical Center (AUBMC) Children's Heart Center, a tertiary medical center in the Middle East RESULTS: Out of 200 cases with confirmed single ventricle physiology, we identified a few patients with characteristics of combined TA-AV septal defect. Our patients exhibited characteristic echocardiographic findings of primum ASD, VSD, atretic RAVV, and clefted LAVV. CONCLUSION: In short, TA-AV septal defect is a rare, underreported congenital malformation. Tracking our patients' clinical profiles will help improve our understanding of the prognosis of this entity. Our findings may also improve treatment modalities since replacing the left-sided valve is often overlooked if the defect is inaccurately diagnosed. In addition, such findings can help shed light on the embryological development of the rarely encountered variation of AV septal malformation.

Age specific histologic types of carcinoma breast in Malaysians.

The influence of age on various histological types of breast cancer at both age <50 years and >50 years to simulate menopause was studied retrospective from 2002 to 2004 in Malaysia. One hundred and fifty five cases were reviewed. Ninety two cases recorded at age <50 years, 60 (65.2%, 95%CI: 54.6 - 74.8%) were infiltrating ductal carcinoma in 11(12.0%, 95% CI: 6.1-20.4%), ductal carcinoma in situ (DCIS) in 9 (9.8%, 95% CI: 4.6-17.8%), medullary carcinoma in 6 (6.5%, 85%CI: 2.4-13.6%), invasive lobular carcinoma in 4 (4.3%, 95%CI: 1.2-10.8%), mucinous carcinoma and poorly differentiated carcinoma in 2 cases (2.2%, 95%CI: 0.3-7.6%). At >50 years of age, 63 cases were recorded. Forty seven (74.6%, 95%CI: 62.0- 84.7%) cases were of infiltrating ductal carcinoma, ductal carcinoma in situ in 9 (14.3%, 95%CI: 6.7-25.4%), mucinous carcinoma in 5 (7.9%, 95%CI: 2.6-17.6%), medullary carcinoma (8.5%) and papillary carcinoma in 1 case each (1.6%, 95%CI: 0.0-8.5%). Infiltrating ductal carcinoma was the commonest histology at both age <50 years and >50 years.

Hereditary spherocytosis in a malay patient with chronic haemolysis.

This case report describes a 35-year-old lady who presented with generalized weakness and lethargy of two weeks duration and jaundice of more than 20 years duration. Her initial workup was suggestive of haemolysis and blood film showed a leucoerythoblastic picture with moderate microspherocytes. She was finally diagnosed as a case of hereditary spherocytosis after ruling out other possible causes of chronic haemolysis and supported by an abnormal osmotic fragility test, although family members refused for screening. Hereditory spherocytosis is uncommon in Malay population and presentation with jaundice of 20 years duration with leucoerythroblastic picture on blood film were interesting features in this case. Patient is being followed closely for need of splenectomy in near future as per severity of haemolysis and currently being managed with folic acid supplement.

Hereditary spherocytosis in a Malay patient with chronic haemolysis

This case report describes a 35-year-old lady who presented with generalized weakness and lethargy of two weeks duration and jaundice of more than 20 years duration. Her initial workup was suggestive of haemolysis and blood film showed a leucoerythoblastic picture with moderate microspherocytes. She was finally diagnosed as a case of hereditary spherocytosis after ruling out other possible causes of chronic haemolysis and supported by an abnormal osmotic fragility test, although family members refused for screening. Hereditory spherocytosis is uncommon in Malay population and presentation with jaundice of 20 years duration with leucoerythroblastic picture on blood film were interesting features in this case. Patient is being followed closely for need of splenectomy in near future as per severity of haemolysis and currently being managed with folic acid supplement.

Pathogenesis and treatment of gastric carcinoma: "an up-date with brief review".

Gastric cancer is one of the most common cancers and most frequent causes of cancer-related deaths in the world. The overall survival rate is 15-20%. Although the incidence is declining, its prognosis remains poor. The etiological factors and pathogenesis of gastric cancer are not yet fully understood. The integrated research in molecular pathology clarified the details of genetic and epigenetic abnormalities of cancer-related genes in the course of development and progression of gastric cancer. Although epidemiological evidences indicate that environmental factors play a major role in the carcinogenesis, the role of immunological, genetic and immunogenetic factors are thought to contribute to etiopathogenesis of gastric carcinoma. In addition to better understanding of pathogenesis of gastric cancer, the incidence, diagnostic studies and the therapeutic options have also undergone important changes in the last decade. There is ongoing debate regarding the role of adjuvant treatment. In advanced disease, palliation of symptoms, rather than cure, is the primary goal of patient management. Several combination therapies have been developed and have been examined in phase III trials; however, in most cases, they have failed to demonstrate a survival advantage over the reference arm. This review summarizes the newer concepts of molecular biology on gastric carcinogenesis and the new important recommendations for the management of patient with gastric carcinoma.

Cancer treatment - objectives and quality of life issues.

The first aim of cancer treatment is to acheive a cure, and when cure is not possible, a good palliation (life prolongation and relief of sufferings) is warranted. This article highlights the aim of cancer treatment and also attempts to assess the issues of quality of life experienced as a result of the disease and its treatment. Palliative therapy should be less intensive than radical treatment and should cause less morbidity than disease itself. It must be effective, completed in a short time and should be tolerable. It is also essential for a physician to give a clear explanation of illness to the patient and realistic advice regarding the likely outcome of therapy and the long and short term morbidities which may occur. The patient may opt for a palliative treatment with a reduced chance of cure but a better quality of life than accepting a radical treatment with a potentially higher degree of morbidity. Quality of life in oncology practice should be seen as a process and as a part of this process it seems sensible to pursue several different lines of questionnaire development rather than constructing one 'perfect" quality of life instrument.