Fatema Jawad  ( 7/6, Rimpa Plaza, M. A. Jinnah Road, Karachi. )

PRIMARY MEDIASTINAL LYMPIIOMA AN UNUSUAL CAUSE OF TAMPONADE Beg, M.H., Reyazuddin, Ansari, M.M., Aziz, M. Pak. Pediat. J., 1989; 13: 37-39.
A case of mediastinal lymphoma presenting as cardiac temponade is presented. The patient was a 10 years old boy with difficulty in breathing, chest pain, and fever of 10 days duration following a trivial blunt chest trauma due to fall from the cot. Physical examination revealed a respiratory rate of 60 per minute, pulse rate of 120 per minute, pulses paradoxus, engorged neck veins and blood pressure 100/60 mmHg. Chest examination showed fea­tures of a right sided pleural effusion with a tracheal and mediastinal shift to the left side. Laboratory tests and ECG were normal. Xray chest demonstrated right sided pleural effusion. Pus was obtained byaspirationwhichwas drained by tube thoracostomy connected to an underwater seal. As the pulsus paradoxus and engorged neck veins persisted, a diagnosis of cardiac tamponade was considered. A right anterolateral thoracotomy was performed and a large tumour of rubbery consistency was found engulfmg the heart. This was partially excised to relieve the constriction and a biopsy performed which gave a ding­nosis of lymphocytic predominant lymphoma. A combination of chemotherapy and radiotherapy was given post- operatively to which a good response was Lymphoma is usually encountered as involvement of the lymph nodes. Mediastinal lymphoma can cause supe­rior venacava obstruction. Empyema with cardiac tam­ponade detected in the present case has not beenreported earlier. A chest roentgenogram is the most important diag­aostic method. Exploratory thoracotomy or medias­tinotomy confirms the diagnosis. Radiotherapy and chemotherapy are the primary mode of treatment.
POST B.C.G. LYMPHADENITIS CONFIRMATION BY CYTOLOGY AND BACTERIOLOGY. Shakoor, K.A., Bughio, G.M. Pak. Paed. J., 1989; 13: 19-23.
BCG Vaccination is used routinely in the developing countries.It is not established whether complete immunity against tuberculosis is conferred by this vaccine or not. A large number of infants also develop regional lyrn­phadenopathy. A pilot study was undertaken at the National Institute of Child Health Laboratory JPMC, Karachi where 50 cases with regional lymphadenopathy were subjected to fme needle Aspiration Cytology and Bacteriology, The time of vaccination and appearance of lymphadenopathy was recorded. The needle aspirate was obtained with 22 gauge spinal or ordinary disposable needle and smeared on glass slides, Two smears each were stained with field stain and Ziel Neilson stain. Some of the aspirate was saved for AFB culture. A peripheral blood examination, ESR and chest Xraywas done on all children. Of the 50 infants, 35 were vaccinated at birth whereas 15 received BCG at a later date. Lymphadenopatbywas noted in less than 3 months in30 patients and 20 babies developed it after 3 months. 48 infants had involvement of the axilary nodes and only 2 had superaclavicular lymphadenopathy. There were no systemic complications. The stains gave a positive result for AFB in 29 cases. BCG vaccination’s usefulness has become questionable and its use has been given up in many western countries. The cause of com­plications of BCG vaccination has been attributed to either subcutaneous injection of the vaccine instead of intrader­mally or a high dose of the vaccine. It was concluded from the presented study, that BCG vaccination is not justified in children who are not exposed to tuberculosis and that the protective advantage of BCG may be much less than the serious side effects. If BCG has to be given then the technique and dose of vaccine should be carefully fol­lowed.
WISKOTT-ALDRICH SYNDROME-A CASE REPORT. Rirvi, All., Iqbal, I. Pak. Paed. J., 1989; 13 : 55-57.
A nine year old boy was admitted in the Paediatric Ward Nishtar Hospital, Multan with the symptoms of early bruising and prolonged bleeding after minor injuries since 7 years and eczema on various parts of the body since 3 years. There was a history of passing fresh blood in stool and epistaxis. The parents were healthy and unrelated to each other. 3 of his siblings had died due to excessive bleeding per rectum. Physical examination revealed pallor, bruises and eczema with healed scars. Systemic examination was normal. Laboratory tests gave a Hb of lOG, TLC-13000, urine and stool were packed with red cells on microscopy, Hess’ Test was strongly positive, bleeding time 16 minutes, clotting timeS minutes and platelet count 20,000/cm. Bone marrow smear showed normal erythrocytic and granulocytic series with megakaryocytes normal in number but non-budding and inactive. Immunoglobulins were normal. A combination of thrombocytopenia, eczema, repeated infections and a strong family history of bleeding confirmed the diagnosis of Wiskott-Aldrich Syndrome. This syndrome is an X-Iinked recessive disorder. Throm­bocytopenia is due to a shorter life span of platelets. Immunodeficiency is not well understood. A mild dysfunc­tion of T-cells has been noted in the initial stages. Treatment is supportive and prognosis is not good and survival beyond 10 years is rare.
SOME BIOCHEMICAL INDICES OF DIABETIC VASCULAR DIS. EASE. Fayyazuddin, Obaidullah, S., Ahined, I. Pakistan J. Med. Res., 1988; 27:81-85.
A study was carried out to compare the levels of serum cholesterol, triglycerides, beta lipoproteins and fibrinogen between 86 diabetic patients and 20 normal controls. This was correlated with the incidence of diabetic vascular complications. All the 86 diabetics were of the Non-Insulin Dependent Group and the ages ranged between 25 and 80 years. Detailed history was recorded and complete physical examination conducted. ECG was performed and clinical assessment of the grade of retinopathy, cardiovascular status and presence or ab­sence of nephropathy was made. Blood samples were withdrawn after an overnight fast and cholesterol was estimated by the method of Abell et al., triglycerides by Giegel et at., beta lipoproteins by Fried and fibrinogen was tested according to Parfentger et altechnique. It was noted that the vascular complications were directly proportional to the duration of diabetes with the highest frequency being in subjects with diabetes for more than 10 years. Plasma fibrinogen levels were found to be higher in diabetics with retinopathy, nephropathy, neuropathy and cardiovascular disease as compared to diabetics without complications. Serum cholesterol was significantly higher in diabetic patients with cardiovascular disease. Triglyceride levels were much higher in all diabetics as compared to normal controls. Beta lipoproteins were also raised in diabetics with one or more complications. It was apparent that the values of these tests were found raised in diabetics with complications. it is not known if the values reflect the cause or effect of vascular disease. A high triglye level in diabetics is attributed to the impaired removal of VLDL which in turn is due to a deficiency of the enzyme lipoprotein lipase. The increased serum beta-lipoproteins in diabetics could be due to an excess of FFA. These free fatty acids stimulate hepatic lipoprotein synthesis. Also there is a defective removal of lipoprotein from the plasma again due to deficiency of lipoprotein lipase. Fibrinogen levels rise in diabetics due to microangiopathic processes. This in turn favours the development of atheroma and intravas­cular thrombosis. It was concluded that elevated lipids could explain some features of diabetic vascular disease. The raised triglycerides are associated with retinal venous dilation.