Unusual bilateral renal histiocytosis. Extranodal variant of Rosai-Dorfman disease.

A 37-year-old Bangladeshi presented with large bilateral masses involving the hilus of the kidneys. No lymphadenopathy was noted. Nephrectomy was performed. Histopathologically, it revealed a lymphohistiocytic and plasma cell inflammatory tumoral proliferation with characteristic lymphophagocytosis by the S100-positive-CD1-negative histiocytes. Extranodal presentation of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) should be entertained in the differential diagnosis of bilateral masses involving the kidneys.     

Deletion of a coordinate regulator of type 2 cytokine expression in mice

Mechanisms that underlie the patterning of cytokine expression in T helper (T(H)) cell subsets remain incompletely defined. An evolutionarily conserved approximately 400-bp noncoding sequence in the intergenic region between the genes Il4 and Il13, designated conserved noncoding sequence 1 (CNS-1), was deleted in mice. The capacity to develop T(H)2 cells was compromised in vitro and in vivo in the absence of CNS-1. Despite the profound effect in T cells, mast cells from CNS-1(-/-) mice maintained their capacity to produce interleukin 4. A T cell-specific element critical for the optimal expression of type 2 cytokines may represent the evolution of a regulatory sequence exploited by adaptive immunity.     

Effectiveness of Preventive Therapy for Persons Exposed at Home to Drug-Resistant Tuberculosis,Karachi, Pakistan

In Karachi, Pakistan, a South Asian megacity with a high prevalence of tuberculosis (TB) and low HIV prevalence, we assessed the effectiveness of fluoroquinolone-based preventive therapy for drug-resistant (DR) TB exposure. During February 2016–March 2017, high-risk household contacts of DR TB patients began a 6-month course of preventive therapy with a fluoroquinolone-based, 2-drug regimen. We assessed effectiveness in this cohort by comparing the rate and risk for TB disease over 2 years to the rates and risks reported in the literature. Of 172 participants, TB occurred in 2 persons over 336 person-years of observation. TB disease incidence rate observed in the cohort was 6.0/1,000 person-years. The incidence rate ratio ranged from 0.29 (95% CI 0.04–1.3) to 0.50 (95% CI 0.06–2.8), with a pooled estimate of 0.35 (95% CI 0.14–0.87). Overall, fluoroquinolone-based preventive therapy for DR TB exposure reduced risk for TB disease by 65%.     

Community-Based Assessment of Knowledge,Attitude, Practices and Risk Factors Regarding COVID-19 Among Pakistanis Residents During a Recent Outbreak: A Cross-Sectional Survey

Exceptional precautionary measures have been adopted to stop the transmission and control of COVID-19 through the world and Pakistan is facing lockdown in this scenario. Public loyalty to precautionary measures is affected by their knowledge, attitude, risk factors and practices (KAP) towards COVID-19. The present study was conducted among the Pakistani residents to observe the knowledge, attitude, practices and risk factors towards COVID-19 outbreak in Pakistan. A questionnaire was designed, and a cross-sectional survey was conducted among participants of the study area. Participants were asked the questions regarding knowledge, attitude, practices and risk factors towards COVID-19. Data were analyzed by SPSS and t/F test and correlation was applied among the knowledge, attitude, risk factors and practices. A total of 1060 questionnaires were received. 1004 were included while 56 were excluded. The highest representation was from Punjab province (65.6%), female (63%) and age group of 21–30 years (62.1%). Most participants were single (85%), Muslim (99.4%), Urdu speaking (45.6%) and were graduates (51.5%). Most of the participants were students (52.9%) and were from economically middle-class families (40.8%). The knowledge was positively correlated with attitude and practices whereas negatively correlated with risk factors (P?<?0.05). The attitude was negatively correlated with risk factor and positively correlated with practices. The risk factors and practices were positively correlated with each other. Health education program to improve the COVID-19 knowledge, attitude, practices and risk factors should be initiated to combat current health challenge.

     

A novel assay for lysosomal pepstatin-insensitive proteinase and its application for the diagnosis of late-infantile neuronal ceroid lipofuscinosis

A highly sensitive assay for mammalian lysosomal pepstatin-insensitive proteinase (LPIP) is described using a synthetic peptide substrate coupled to aminotrifluoromethyl coumarin (AFC). LPIP is an endocarboxyl proteinase which has specific sequence requirements of Phe-Phe around the carboxyl terminal. This HPLC based assay can detect patients suffering from late-infantile neuronal ceroid lipofuscinosis (LINCL) and also heterozygote carriers in cultured lymphoid cells and skin fibroblasts. None of the patients analyzed had detectable enzyme activity confirming the defective gene product, while carriers had about 50% activity when compared with the normal controls. Neurological controls comprised of patients with other neurodegenerative disorders have LPIP activities similar to normal controls. LPIP activity is also detectable in amniocytes and chorionic villi. Thus the assay reported can also be used for prenatal diagnosis of LINCL.     

Is preoperative donation of autologous blood rational for radical retropubic prostatectomy?

PURPOSE: We investigated differences in the rate of homologous blood transfusion and the degree of anemia to determine whether it is rational to have patients donate autologous blood before radical prostatectomy. MATERIALS AND METHODS: We retrospectively reviewed the charts of 221 consecutive men who underwent radical retropubic prostatectomy performed by 1 surgeon in a 14-month period. About half of the patients donated autologous blood preoperatively. We evaluated perioperative hemoglobin, and the rate of autologous and homologous transfusion. RESULTS: The groups did not significantly differ in terms of demographic data, co-morbid conditions, clinical variables or hospitalization. Preoperatively mean hemoglobin plus or minus standard deviation was 13.4 +/- 1 and 14.7 +/- 1 gm./dl. in patients who did and did not donate blood, while homologous transfusion was required in 1 (1%) and 4 (3.5%), respectively (p = 0. 18). At hospital discharge anemia was more prevalent in nondonors. Of the men who did versus did not donate blood hemoglobin was less than 10 and less than 9 gm./dl. in 8.4% versus 34% (p <0.0001), and 12.5% versus 0% (p <0.0004), respectively. CONCLUSIONS: Our retrospective review of a cohort of patients who underwent radical retropubic prostatectomy showed no difference in homologous blood transfusion based on preoperative autologous donation status. Autologous donors had lower hemoglobin preoperatively, a higher rate of transfused units and higher hemoglobin at hospital discharge. Preoperative donation of autologous blood may not decrease the need for homologous transfusion in healthy patients undergoing radical retropubic prostatectomy.