Neurocognitive Disorders in HIV-positive Patients.

Human immunodeficiency viruses (HIV) associated neurocognitive disorders (HAND) encompasses a group of syndromes of various degrees of impairment in cognition and daily functioning of HIV positive individuals. Although the widespread use of highly active antiretroviral therapy (HAART) has drastically reduced the prevalence of severe form of HAND, like HIV associated dementia (HAD), the prevalence of HAND and associated morbidity remains high. OBJECTIVES: (1) To know the prevalence of HAND in HIV-infected patients of a multi-ethnic population. (2) To describe various types of neurocognitive impairment among patients of HAND and study the factors affecting HAND. STUDY DESIGN: This study was a cross-sectional descriptive study conducted on 250 HIV-positive patients in outpatient department (OPD) of a tertiary care center in Mumbai, conducted over a period of 12 months. Patients with HIV-1 attending the OPD and having a minimal formal education of 4 years were included. Patients with concomitant delirium, any known central nervous system (CNS) disorder, any psychiatric disorder, and pregnant females were excluded. Outcome measures-the test batteries used were (1) International HIV Dementia Scale (IHDS) and (2) Addenbrookes cognitive examination-revised (ACE-R) scale. RESULTS: Of 250 subjects studied, 55.6% (139) were males and 44.4 % (111) were females. The mean age of study population was 39.42 years. The mean years of education were 8.32 years. The mean duration of infection (diagnosis of HIV-positive state) was 64.49 months and the mean duration of HAART intake in our patients was 52.30 months. The mean cluster of differentiation 4 (CD4) counts of our subjects were 527.13 per cumm [standard deviation (SD) of 234.13]. The mean nadir CD4 counts were 224.35 per cumm (SD of 115.09). Using the ACE-R scale, the prevalence of HAND was 71.60%, of which 37.20% had an asymptomatic neurological impairment, 29.60% had mild cognitive dysfunction, and 4.80% had HAD. Memory, verbal fluency and visuospatial abilities were the most affected domains on the ACE-R and memory recall and psychomotor speed were affected more on the IHDS. The prevalence of HAND was more with increasing age (p = 0.020), lesser education (p < 0.00) and lesser nadir CD4 counts (p < 0.00). However, it was not affected by the duration of the disease and the current CD4 counts (p > 0.05). CONCLUSION: Human immunodeficiency viruses (HIV) associated neurocognitive disorders HAND is common in HIV-positive patients, most of whom are asymptomatic. Older patients with less education and severe disease, having lower nadir counts are at the highest risk of HAND. Memory, verbal fluency, and visuospatial abilities were the most commonly affected domains.

Snake Bite Envenomation in a Tertiary Care Centre.

Background: In India, it is estimated that up to 20,000 people die annually from snake bites. The present study was carried to out to estimate the snake bite related epidemiology, predictors of severity, relationship between type of snake, clinical severity, complications, outcome and usage pattern of polyvalent anti snake venom (ASV) in a tertiary care center. Methods: All indoor patients admitted in our institute with definitive history of bite by a snake, with or without presence of fang marks, Evidence of cellulitis, acute onset of neurotoxicity or bleeding diathesis were serially recruited in the study. Results: The majority of cases were in the range of 21- 40 years (54.7%). There were 82.8% males (53/64), 17.2% females (11/64) and 60.9% (39/64) bites were during day time. Upper limb bites were seen in 34% (22/64) of the patients and lower limb bites in 54% (35/64), and axial body bites in 6%. There were 43.8% (28/64) vasculotoxic bites, 34.4% (22/64) neurotoxic bites and 20.3% (14/64) non-poisonous bites. Viper was the most common (9%) identified snake, followed by krait (5%). References from Rural Health Centers were 57.8% (57/64), 11% were from Primary health centers and rest from private sector. Anti snake venom (ASV) was received by 68.75% (44/64) patients before reaching tertiary care. Local swelling was present in 90.6% (58/64) patients, Systemic bleeding was seen in 35.9% (23/64), and Neuromuscular weakness in 35.9% (23/64) patients. Complications like Respiratory paralysis developed in 18.75% (12/64), Acute kidney injury in 12% (8/64), DIC in 9% (6/64), and hepatic involvement in 7% (5/64) of snake bite patients. Blood transfusion was required in 20.3% (13/64) p<0.001), 18.75% (12/64) required Mechanical ventilation (p=0.001), 4 received hemodailysis and 4 required ionotropic support (p<0.001). Improvement was seen in 57.8% (37/64), morbidity during hospital stay was seen in 39% (25/64) and 2 patients expired (3%). ASV was received within 4 hours in 67% (42/64) patients, 22.5% (14/64) received ASV between 4 to 24 hours and remaining after 24 hours (p=0.016). Total ASV requierment was 24.05 vials in patients who improved and 34.4vials in patients in Morbid group and 29.0 vials in mortality group (p>0.05). The SSS score amongst improved was 4.76 ± 2.46 whereas among morbid, it was 8.48 ± 1.75 and amongst expired, it was 8.5 ± 0.707 (p<0.05). Conclusions: Patients requiring various supportive treatments like blood transfusion, Inotropes, Haemodialysis and Mechanical ventilation, had a statistically significant correlation with poor outcome. Early administration of ASV that is within 4 hours was, associated with better outcome. The total amount of ASV (in vials) had no a significant correlation with outcome. Snakebite Severity Score correlates significantly with early recovery in vasculotoxic snake bites (p=0.03).

Dyskeratosis congenita with portal hypertension of unknown etiology.

Dyskeratosis Congenita (DKC) is a rare progressive bone marrow disorder associated with multi-systemic involvement. It is also characterized by triad of abnormal skin pigmentation, nail dystrophy and mucosal leukoplakia. Liver cirrhosis and portal hypertension are said to be uncommon among these patients. We hereby report a case of an adult male who presented with pancytopenia, abnormal skin pigmentation, nail dystrophy and mucosal leukoplakia. Skin biopsies along with clinical features confirmed the case. Imaging studies were reported as suggestive of and portal hypertension. Liver biopsy done but non-conclusive. Patient's one son and one daughter also had similar skin pigmentation.

Renal tubular acidosis due to Wilson's disease presenting as metabolic bone disease.

Two sisters presented with lower limb deformity and difficulty in walking without support. Both had short stature; however, neurodevelopment and secondary sexual characters were normal. Abdominal examination revealed splenomegaly and ophthalmic examination showed presence of Kayser-Fleischer (K-F) rings. Diagnosis of Wilson's disease was confirmed with low serum copper and ceruloplasmin levels. Further investigations revealed urinary acidification defect with hypercalciuria pointing towards distal renal tubular acidosis. Both patients were started on copper chelation therapy and showed gradual radiographic improvement in osteopaenia.