Sporadic case of warts, hypogammaglobulinemia, immunodeficiency, and myelokathexis syndrome

The term WHIM syndrome (WHIMS) is an acronym describing a rare primary immunodeficiency disorder characterized by warts, hypogammaglobulinemia, immunodeficiency, and myelokathexis, the unusual association of neutropenia with bone marrow myeloid hypercellularity. WHIMS was recently associated with mutations in the gene encoding the chemokine receptor CXCR4 and as such is the first disease ascribed to abnormalities of chemokine signaling. We report a sporadic case of WHIMS in a woman presenting with recurrent infections and human papilloma virus-related genital dysplasia.     

Abdominal epilepsy

Abdominal epilepsy (AE) is a rather uncommon clinical entity in children that might create diagnostic confusion especially when it lacks the typical manifestations of an epileptic seizure. We report the case of a young boy having apparently unexplained episodes of paroxysmal abdominal symptoms with no other suggestion of an underlying epileptic disorder. The case also explains how the clinical presentation can be misleading unless a high index of suspicion is maintained to reach the ultimate diagnosis.     

Effect of school policy on tobacco use by school personnel in Bihar, India

This study examined the relationship between school tobacco policies and tobacco use prevalence among school personnel. Two subsets of schools were identified in Bihar, India: Federal Schools (with a tobacco policy), and State schools (without a tobacco policy). Stratified probability samples of 50 schools each were selected. The survey was conducted through an anonymous, self-administered questionnaire. School personnel from State Schools (non-policy schools) reported significantly higher daily cigarette smoking and daily current smokeless tobacco use compared to personnel in Federal schools (policy schools). Teachers in State schools did not teach about health consequences of tobacco, and they had not received training for such teaching. Extent of teaching about health consequences of tobacco varied across topics for teachers in Federal schools. They received negligible training, but more than 35% reported access to teaching materials. More than one-half the personnel from Federal schools knew about their school's policy prohibiting tobacco use among students and school personnel, and about policy enforcement. Personnel in State schools did not know about tobacco control policy in their schools. All school personnel in both types of schools were near unanimous in supporting policy prohibiting tobacco use in schools. The study demonstrated an association between enacting a school policy regarding tobacco use and school personnel's use of tobacco, curricular teaching, and practical training of students. Findings suggest that more extensive introduction of comprehensive school policies may help reduce tobacco use among school personnel.     

School policy and tobacco use by students in Bihar, India

The association between school tobacco policies and tobacco use prevalence among students were examined. A two stage cluster sample design with probability proportional to the enrolment in grades VIII-X was used. Comparison was made between schools with a tobacco policy (Federal schools) and schools without a policy (State schools). Stratified probability samples of 50 schools each were selected. SUDAAN and the C-sample procedure in Epi-Info was used for statistical analysis. Students from State schools (without tobacco policy) reported significantly higher ever and current any tobacco use, current smokeless tobacco use and current smoking compared to Federal schools (with tobacco policy) both in rural and urban areas. Classroom teaching on the harmful effects of tobacco was significantly higher (17-24 times) in Federal schools than State schools both in rural and urban areas. Parental tobacco use was similar for students in Federal and State schools. Students attending state schools were more likely than students attending Federal schools to have friends who smoke or chew tobacco. These findings suggest that the wider introduction of comprehensive school policies may help to reduce adolescent tobacco use.     

Analysis of the natural killer mediated immune response in metastatic renal cell carcinoma patients

Metastatic renal cell carcinomas (MRCC) are considered as immunogeneic tumors on the basis of the clinical responses observed in patients treated by IL-2. However, renal cell carcinoma patients are also characterized by alterations of the immune response that may compromise the immunotherapeutic approaches. In our study, we have studied the phenotype and the functional capacities of peripheral NK cells in a panel of neprectomized metastatic renal cell carcinoma patients. NK cells were harvested by negative immunoselection from fresh peripheral blood samples. In most of MRCC patients analysed (23/28), the expression of NCR (NKp46 and NKp30) was similar to that of donors. Lytic capacities by activated immunoselected NK cells from MRCC patients assessed against K562 and 3 renal tumor cell lines were in the range of that observed in NK cells from normal donors. HLA-I- renal tumor cells UOK23 were killed with a good efficiency, whereas HLA-I renal tumor cells were more resistant. Although LFA-1/ICAM-1 interaction potentiates RCC cell lysis, HLA-I/NKR interaction clearly decreased RCC cell susceptibility to NK cells. In addition, proliferation of NK cells from MRCC patients in response to cytokines was altered.     

Family functioning in the caregivers of patients with dementia

BACKGROUND: Caregiver burden has been extensively studied in the dementia population. The marital relationship has been suggested as a mediational model through which variables influence the caregiver and contribute to the experience of burden or reward. OBJECTIVES: This study examines family functioning, caregiver burden and reward and quality of life in 38 family members caring for a relative with dementia. METHODS: Caregivers of out-patients with dementia completed self report questionnaires. RESULTS: 63% of caregivers were female with a mean age of 62 years. Patient mean age was 73 years. The average number of caregiving years was 3.1. Caregivers were more likely to be spouses (61%) than children (29%) or other relatives (11%). Despite the fact that caregivers reported that their relatives were moderately disabled, they perceived more reward than burden. Caregivers who reported poor family functioning had higher ratings of strain and burden. Family functioning in these caregivers was poorest in the dimensions of affective responsiveness, problem solving and communication but it was also impaired in roles and affective involvement. CONCLUSIONS: Assessing a family's functioning may be an important factor in the care of the dementia patient and his/her family.     

Cellular schwannoma of the paranasal sinuses: initial report of a case

Cellular schwannomas are a benign variant of classic schwannomas. Their histologic appearance closely mimics that of malignant peripheral nerve-sheath tumors, which are high-grade sarcomas. In this article, we describe what to our knowledge is the first reported case of a cellular schwannoma of the paranasal sinuses and only the 33rd reported case of any schwannoma at this site. We also discuss the histology and management of cellular schwannomas and review the pertinent literature.     

The COX-2 inhibitors: new analgesic and anti-inflammatory drugs

Selective COX-2 inhibitors offer a therapeutic alternative to the conventional nonselective NSAIDs. Rofecoxib has been demonstrated to be a valuable therapeutic agent in the management of acute orofacial pain. Selective COX-2 inhibitors are also indicated in patients who are likely to undergo surgery or invasive procedures in the near future because these drugs do not prolong the bleeding time. The efficacy of these drugs in the management of chronic orofacial pain is yet to be evaluated. The pharmacoeconomic impact of COX-2 inhibitors must also be considered, as the cost of selective COX-2 inhibitors is considerably higher than the other commonly used NSAIDs. Although it is clear that COX-2 inhibitors offer some advantages over the nonselective NSAIDs in terms of a lower risk of GI toxicity with long-term use, the effects following short-term use are still unclear. Until more data are available, COX-2 inhibitors should be avoided or used with the same caution as for conventional NSAIDs in patients with compromised renal and cardiac function.