Infantile Osteomyelitis Secondary to Malignant Osteopetrosis

Osteopetrosis (OP) is a rare congenital disorder (present at birth) in which the bones become over dense. This results from an imbalance between the formation of bone and the resorption of the bone. Thickening of the bones which become abnormally dense due to an inherited defect in bone resorption, the process in which old bone is broken down and removed so that new bone can be added to the skeleton. Osteoclasts are the cells responsible for bone resorption. In osteopetrosis the osteoclasts do not perform normally. This flaw in bone resorption results in bones that are abnormally dense, yet are fragile and easily broken. Osteopetrosis is also known as Albers–Schonberg disease, generalized congenital osteosclerosis, ivory bones, marble bones, osteosclerosis fragilis generalisata. In this article, we have described about the diagnosis and medical and surgical management of osteopetrosis reported case to our hospital.     

Facial Swelling with Atypical Mycobacterial Infection in Immuno-Competent Patient

A middle aged immune-competent woman presented with a facial swelling, which was excised. The histopathology showed it to be a non-caseating granuloma. PCR confirmed that the swelling was caused by mycobacterium other than tuberculosis (MOTT) infection. No relapse was seen on follow up even without chemotherapy.     

Depressive symptoms and smoking among Hong Kong Chinese adolescents

AIMS: To examine associations among depressive symptoms, smoking, smoking trajectories and quitting smoking in Hong Kong. DESIGN: Prospective longitudinal design, with wave 1 at baseline (T1) and wave 2 (T2) 12 months later. SETTING AND PARTICIPANTS: Form 1 (equivalent to 7th grade in the United States) students, mean age = 12.7 years, n = 1894. MEASUREMENTS: Self-reported smoking status, attempts to quit and depressive symptoms. FINDINGS: At both waves, current as well as ex-smokers had higher depressive symptoms than never smokers. T1 smoking predicted T2 depressive symptoms among those with low baseline depressive symptoms. Depressive symptoms at T1 predicted smoking at T2 among non-smokers at T1. Trajectories were defined by separating participants who were never smokers at both waves ('non-smokers'), those who smoked at both waves ('persistent smokers'), those who smoked at one time but were not smoking at either wave ('past smokers), and those who had never smoked at T1 but reported smoking a year later ('new smokers'). Persistent, past and new smokers had higher depressive symptoms at both waves than non-smokers. Smokers who reported not wanting or trying to quit and those who had been unsuccessful at quitting had higher depressive symptoms at T2 than those who successfully quit. CONCLUSION: Our results suggest that depressive symptoms promote tobacco use in Asian adolescents by making it more likely that an adolescent will begin smoking and less likely that she or he will quit. These findings elucidate risk factors in Hong Kong for two important public health concerns for adolescents: smoking and depression.     

Frailty assessment and impact of frailty on outcomes after transcatheter aortic valve replacement

Introduction: Certain frail patients fail to achieve adequate functional or mortality benefit despite successful transcatheter aortic valve replacement (TAVR). Therefore, frailty assessment methods are becoming an important tool to identify and intervene on this high-risk patient subset for improving clinical outcomes.

Areas covered: The authors provide an overview of frailty and frailty assessment tools being used in clinical practice and discuss the impact of frailty on the cardiac patients, particularly among the TAVR population.

Expert commentary: Available evidence suggests that frailty assessment is critical for identifying patients at high risk of morbidity and mortality after TAVR procedures. However, there is lack of consensus for the best methodology to determine frailty and its optimal management in TAVR populations. Although, physical exercise is a commonly employed intervention to reduce frailty, a greater attention towards improving nutrition may convey more benefit than either intervention alone. Ongoing studies are investigating the benefits of a multicomponent approach to improve clinical outcomes in frail patients undergoing TAVR.     

Keratoglobus: An experience at a tertiary eye care center in India

Context:

This study was carried out as a part of an internal audit and is the largest series of patients having keratoglobus, published in the literature. Poor visual acuity of the patients indicates the blinding nature of the disease.

Aims:

We report our experience with patients having keratoglobus at a tertiary eye care center in India.

Settings and Design:

Retrospective study.

Materials and Methods:

We analyzed adults and pediatric patients (<16 years) with keratoglobus, seen during 2008–2012. The age, gender, consanguinity, presenting ocular signs, ocular and systemic associations, visual acuity, corneal topography, and surgeries were documented.

Results:

Forty-eight patients (mean age 22 ± 15 years, 31 males) having keratoglobus were analyzed. 21 patients (42 eyes) were <16 years. Twelve eyes (16 events) had positive history of trauma. The presenting clinical signs were corneal scars/scars of tear repair (15 eyes), hydrops, healed and acute (14 eyes) and corneal or globe rupture (9 eyes). Best-corrected visual acuity was >20/40 in 6/42 (14.3%) pediatric eyes and 15/53 (28.30%) adults. Visual acuity ranging from counting of fingers to no light perception was noted in 20/53 (37.74%) adults and 21/42 (50%) pediatric patients; 13/20 (65%) with blue sclera and 8/22 eyes (36.37%) without blue sclera. Vernal keratoconjunctivitis was present in one pediatric patient. Choroidal osteoma, retinitis pigmentosa, and retinal detachment were present in adults. Surgeries performed were corneal tear repair (5 eyes), tissue adhesive application (2 eyes), descematopexy (4 eyes) and penetrating keratoplasty (PK - 8 eyes: Three had post-PK glaucoma, graft failure-one eye, 4 patients wore scleral lens - prosthetic replacement of the ocular surface ecosystem).

Conclusions:

About 50% of pediatric eyes (65% having blue sclera) had no functional vision. Trivial trauma was responsible for corneal rupture indicating need for protective glasses. About 50% patients had post-PK glaucoma though grafts were clear.     

Evidence for activation of cellular immune responses in patients with acute hepatitis E.

INTRODUCTION: Although acute hepatitis E virus (HEV) infection is known to induce IgM and IgG humoral host immune responses, little is known about occurrence of cellular responses in this infection. We looked for evidence of lymphocyte sensitization to HEV peptides in patients with acute HEV infection. METHODS: peripheral blood lymphocytes were obtained from patients with acute hepatitis E and healthy controls. Proliferation of these lymphocytes in the presence of each of seven peptides with amino acid sequences corresponding to open reading frames 2 and 3 proteins of HEV (3 and 4 peptides, respectively) were studied; no peptide was added to control wells. Proliferative responses with stimulation indices exceeding 3.0 were taken as positive. RESULTS: More patients showed reactivity to two or more HEV peptides than did controls (11/21 vs 5/22, p<0.05). Reactivity to one peptide corresponding to open reading frame 2 of HEV was more frequent in patients than in controls (7/21 vs 1/22, p<0.05). CONCLUSION: Our results show that lymphocytes of patients with acute hepatitis E show sensitization to HEV peptides. This may have significance in understanding the pathogenetic mechanisms of liver injury in this infection.     

High-dose chemotherapy and stem cell transplantation for patients with stage IV breast cancer without clinically evident disease: correlation of CD34+ selection to clinical outcome

Forty-five patients with metastatic breast cancer without clinically evident disease were treated with thiotepa 750 mg/m2, mitoxantrone 40 mg/m2 and carboplatin 1000 mg/m2 followed by stem cell transplantation to determine the safety and efficacy of CD34+ selection of peripheral blood stem cells. Of these, 15 patients' (group I) stem cells were processed through Baxter Isolex 300 device for CD34+ selection, whereas 30 patients (group II) received unmanipulated stem cells. Toxicity, progression-free survival and survival were compared between these two groups. There was no difference in transfusion requirements, white cell count and platelet recovery and non-hematologic toxicity between the two groups. The survival of patients in group I was 27 months compared to 38 months in group II (P = 0.8). The progression-free survival was 12 months and 13.5 months for group I and group II patients, respectively (P = 0.6). Our results indicate that while there is no adverse effect, there is also no significant advantage of CD34+ selection in terms of progression-free survival and survival in patients with metastatic breast cancer without clinically evident disease. Bone Marrow Transplantation (2000).     

Associations Between Spirituality,Mindfulness, and Psychological Symptoms Among Advanced Lung Cancer Patients and Their Spousal Caregivers

ContextPatients with metastatic lung cancer and their spousal caregivers are at high risk of psychological symptoms. Mindfulness may improve psychological symptoms via spiritual well-being (SW); yet, this mediation model has not been examined in a dyadic context.ObjectivesWe examined the mediating role of two dimensions of SW (meaning/peace and faith) in the mindfulness-symptoms link in Stage IV lung cancer patients and their spousal caregivers.MethodsWe examined the actor-partner interdependence model of mediation using multivariate multilevel modeling with 78 couples. Four actor-partner interdependence model of mediation analyses were conducted to examine one predictor (mindfulness) × two mediators (meaning/peace and faith) × two psychological symptoms (depressive symptoms and cancer distress). We also tested four alternative models in which mindfulness mediates the associations between SW and psychological symptoms.ResultsThe alternative model (SW → mindfulness → psychological symptoms) was preferred than the original model (mindfulness → SW → psychological symptoms). For patients, meaning/peace was directly associated with their own psychological symptoms, whereas faith was only indirectly associated with their own psychological symptoms via mindfulness. For spouses, meaning/peace was both directly and indirectly associated with their own psychological symptoms, whereas faith was only directly associated with their own depressive symptoms (but not cancer distress). Moreover, spouses' faith was indirectly associated with patients' psychological symptoms through patients' mindfulness.ConclusionSW is associated with patients' and spouses' psychological symptoms both directly and indirectly through mindfulness. Thus, interventions that target SW, particularly meaning and peace, along with mindfulness may be beneficial to the psychological management of patients facing a terminal disease and their spousal caregivers.     

T Cell-Epstein-Barr Virus–Associated Hemophagocytic Lymphohistiocytosis (HLH) Occurs in Non-Asians and Is Associated with a T Cell Activation State that Is Comparable to Primary HLH

Journal of Clinical Immunology - T cell-Epstein-Barr virus–associated hemophagocytic lymphohistiocytosis (T cell-EBV-HLH) is prevalent in East Asia and has poor prognosis. Understanding of...     

26‐Year‐Old Man with a Cerebellar Mass

Medulloblastoma accounts for only 1% of all adult CNS tumors. Recently, we have encountered a case of medulloblastoma in a 26 year old man with divergent differentiation including myoblastic, glial, osteoblastic and chondroblastic differentiation. Here we present the results of immunohistochemical staining using several specific antibodies with a discussion and a review of literature.