Brainstem encephalitis with Kikuchi-Fujimoto disease

A case of young woman is described who developed clinical and MRI features of brainstem encephalitis in the setting of fever and cervical lymphadenopathy. Lymph node biopsy revealed histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease), which may reflect host response to an unspecified immune insult

Rhinocerebral zygomycosis in Pakistan: clinical spectrum, management, and outcome

OBJECTIVE: To study the disease spectrum and salient management features of 36 patients with histopathologically-confirmed rhinocerebral zygomycosis seen at our academic center over a 16-year period. METHODS: Retrospective review of patients admitted to the Aga Khan University Hospital in Karachi, Pakistan from January 1991 to December 2006 with histopathologically-confirmed zygomycosis of the head and neck. RESULTS: Mean patient age was 40 +/- 5.0 years (range, 34-63 years), and 23 (64%) patients were male. Thirty-two (89%) patients were referred from clinical services other than otolaryngology. Underlying predisposing conditions included diabetes mellitus (21 patients), haematologic diseases (9), and renal failure (6). Twenty (55%) patients had limited sinonasal disease, ten (28%) had orbital involvement, and six (17%) had intracranial extension. All patients underwent rigid nasal endoscopy and biopsy, and black necrotic tissue was seen in 22 (61%) instances warranting endoscopic or open surgical debridement. Four of 6 patients undergoing open surgery required orbital exenteration. Overall patient survival was 56% (20/36 patients). Diabetic patients had improved survival (17/21, or 81%) compared to patients with haematologic disorders (3/9, or 33%) (p = 0.001). All six patients with intracerebral disease died. Eighteen of the 22 (82%) patients treated with surgery plus amphotericin B survived vs. two of 14 (14%) receiving amphotericin B alone (p \u3c 0.001). CONCLUSIONS: In rhinocerebral zygomycosis, an aggressive, multidisciplinary, diagnostic and therapeutic approach that utilizes CT or MRI staging, and combines endoscopic or open surgical debridement with amphotericin B-based antifungal therapy offers the best chance of recovery

Clinical profile and outcome of infective endocarditis at the Aga Khan University Hospital

Background: The spectrum of infective endocarditis (IE) is significantly different in developed and developing countries. The present study was conducted to study the clinical profile and outcome of infective endocarditis in Pakistan.Methods: A descriptive cross-sectional study with review of medical records for 188 patients admitted to our teaching hospital with a diagnosis of IE from January 1988 to December 2001. One hundred fifty-nine subjects fulfilled the modified Duke diagnostic criteria.Results: Definite IE was found in 59.7% (95/159) patients, while the rest had possible IE. One-third of subjects had acute IE 55/159 (34.5%). Subacute IE was found in 62% (99/159) and Nosocomial IE in 3% of the cases. Eighty six (54%) were classified as having culturenegative endocarditis and 73 (46%) as culture positive. Ninty four patients (59%) had an underlying predisposing factor including congenital heart disease (31%) and rheumatic heart disease (21%). The most frequently isolated organisms were streptococci (52%) and followed by staphylococci (29%). Fourteen (8.1%) patients had right-sided cardiac involvement. Using univariate analysis, patients with heart failure, neurologic or renal complications, septicemia, nosocomial endocarditis, and prosthetic valve endocarditis were at increased risk of death (p ≤ 0.05), however no individual microorganism, or specific site, size, or morphology of vegetation seen on echocardiogram were significantly associated with death. Thirty-seven (23%) patients died of endocarditis or its complications.Conclusion: Endocarditis continues to be an important contributor to morbidity and mortality in Pakistan, especially in young adults. Our patients differ from the west in terms of epidemiology, predisposing factors, microbiology, complications, and outcom

Infective endocarditis in patients with congenitally malformed hearts: characterization of the syndrome in a developing country

Cardiac surgery for correction or palliation of congenital cardiac disease in infancy and childhood remains a privilege that is rarely accessible to two-thirds of the world’s population. This imbalance has created a unique spectrum of illness in patients with underlying congenital cardiac disease and complicating infective endocarditis in developing countries, including Pakistan. In this study, we characterize endocarditis as seen in such patients presenting in Karachi. We reviewed retrospectively patients admitted to Aga Khan University with underlying congenitally malformed hearts and endocarditis between 1991 and 2004. We identified 48 patients with endocarditis according to the modified Duke Criterions, with just over half the cases (54%) classified as definite endocarditis. Of the patients, 23 (49%) patients were more than 16 years old. Uncorrected left-to-right-shunts, tetralogy of Fallot, and congenital mitral valvar disease were the most common underlying defects. Patients with cyanotic defects, particularly of the complex type, were underrepresented (4%). Only 11 (22.9%) of the patients had a previous palliative or corrective surgery. In one-third of the patients (16), streptococcal species were identified as the microbiologic cause of endocarditis, and 22 (45.8%) had culture-negative endocarditis. In contrast, Staphylococcus aureus and enterococci caused endocarditis in only one patient each. There were no differences in mortality or complications between cyanotic and acyanotic congenital defects. Surgery was performed in nine (18.7%) patients with endocarditis, and of these, 13 (27.1%) died. In contrast to the developed world, endocarditis in the developing countries, such as Pakistan, complicates uncorrected left-to-right shunts and tetralogy of Fallot, probably because patients with complex cyanotic defects fail to survive long after birth due to the lack of available surgery. Almost half of patients had culture-negative endocarditis, likely related to several factors

Massive Fluid Requirements and an Unusual BUN/Creatinine Ratio for Pre-Renal Failure in Patients with Cholera

Background: Cholera is an important infectious cause of secretory diarrhea. The primary symptom of infection is the sudden onset of watery diarrhea with subsequent volume depletion causing renal insufficiency. The objective of this research is to study the level of dehydration at presentation and subsequent fluid management in Patients with cholera. Methods: This study was conducted on 191 Patients of Cholera admitted at a tertiary care hospital in Karachi, Pakistan during the period of 5 years. Medical charts were evaluated retrospectively for initial hydration status, baseline lab investigations on admission and discharge and fluid therapy given to all the Patients while their stay in the hospital and the data was analyzed on SPSS 15.0. Results: Out of the 191 Patients, 83(43%) were males and 108 (57%) were females with mean age of 42.3 years (SD+/-18.34). The average duration of symptoms was 3.75 days (SD+/-2.04). Of 191 Patients, 175 (92.1%) presented with dehydration, 80 (42.3%) were given Ringer\u27s Lactate (R/L) + Normal Saline (N/S), 45 (24%) Patients were given R/L + N/S + Oral Rehydration Therapy (ORS), 27 (14.3%) of the Patients were kept on R/L only and remaining were given various combinations of R/L, N/S, ORS and Dextrose Saline (D/S). On admission mean Blood Urea Nitrogen (BUN) was 24.54 (SD+/-16.6), mean creatinine was 2.47 (SD+/-2.35) and mean BUN/Creatinine ratio was 11.63 (SD+/-5.7). Conclusion: Aggressive fluid rehydration remains the cornerstone of management of cholera. Instead of presenting with a classical BUN/Creatinine ratio of \u3e20:1, Patients with pre-renal failure in cholera may present with a BUN/Creatinine ratio of \u3c15:1