A high tuberculosis case-fatality rate in a setting of effective tuberculosis control: implications for acceptable treatment success rates.

Worldwide, the case-fatality rate of smear-positive pulmonary tuberculosis among persons on treatment is 3.8%. We assessed the case-fatality rate among such patients in Baltimore between January 1993 and June 1998. Tuberculosis incidence was < 17/100 000 population, and 99% of patients received directly observed therapy. Of 174 patients, 42 (24%) died on treatment. Patients who died were older (mean age 62 vs. 47 years; P < 0.001) and were more likely to have underlying medical conditions. In multivariate analyses, older age, diabetes mellitus, and renal failure were independently associated with an increased risk of death. With effective control, tuberculosis may become concentrated in older persons with chronic diseases and be associated with high case-fatality rates. In such settings, acceptable treatment success rates may need to be revised.     

Cardiac amyloidosis--a case report

A 75-year-old male patient presented with resistant congestive cardiac failure. Echocardiographic examination revealed normal left ventricular size with symmetrical hypertrophy and generalised hypokinesia. In addition, there was a characteristic granular sparkling appearance of the myocardium. Cardiac catheterization showed biventricular diastolic restriction with normal coronary arteries. Possibility of cardiac amyloid was considered, and the diagnosis was confirmed on rectal biopsy.     

Rapid Flow Cytometric Prenatal Diagnosis of Primary Immunodeficiency (PID) Disorders

Objectives

Primary Immunodeficiency diseases (PID) are a heterogeneous group of inherited disorders of immune system. Immunophenotypic evaluation of PIDs using flowcytometry provides important clues for diagnosis of these disorders, though confirmation requires identification of underlying molecular defects. Prenatal diagnosis (PND) forms an important component of management in families affected with severe PID. However, molecular diagnostic facilities for each of these diseases are not available and may not be possible to perform in all cases. In such scenario we opted for phenotypic prenatal diagnosis by cordocentesis for families with index case having immunophenotypically well characterized PID.

Methods

Normal reference ranges of lymphocyte subsets, CD 18/CD11 integrins on leukocytes, MHC class II expression and oxidative burst activity of fetal neutrophils at 18 weeks of gestation were previously established on 30 cord blood samples. PND was performed in 13 families with PIDs. Maternal contamination was ruled out by VNTR analysis.

Results

Out of 13 fetuses, nine were found to be unaffected (three cases with leukocyte adhesion deficiency (LAD-I), four cases with severe combined immunodeficiency diseases (SCID), one with X-linked agammaglobulinemia (XLA), and one with chronic granulomatous disease (CGD)] and three were found to be affected (one with T-B+NK-SCID, one with MHC class II deficiency and one with LAD-I). Diagnosis was confirmed by testing the cord blood samples after delivery and further follow-up of the children. In one family diagnosis could not be offered due to maternal contamination. No procedure related complications were observed.

Conclusion

Flowcytometry offers rapid and sensitive method for prenatal diagnosis and genetic counseling for selected phenotypically well characterized PID in cases where molecular diagnostic facilities are not available.     

Leiomyosarcoma of third part of duodenum with perforation.

Malignant stromal cell tumors of the duodenum present with pain or bleed. We report a patient with leiomyosarcoma of the third part of the duodenum that presented with perforation.