Erdheim-Chester disease: expanding the spectrum of cutaneous manifestations.

BACKGROUND: Erdheim-Chester disease (ECD) is a rare condition and there is limited information available regarding its cutaneous manifestations. OBJECTIVES: To describe the clinical and histopathological features of cutaneous involvement in ECD. METHODS: This study is a single-centre retrospective analysis of patients 18 years old and older with biopsy-proven diagnosis of ECD between 1 January 1990 and 1 April 2017. Patients from this cohort were screened for cutaneous manifestations, and BRAF c.1799T>A (p.V600E) mutational analysis was conducted in novel skin manifestations. Primary outcomes included cutaneous manifestations (morphology and topography of lesions) and BRAF mutation status in novel cutaneous findings. RESULTS: Of 71 patients with ECD, 15 patients (21%; median age 52 years) presented with cutaneous manifestations. The most common finding was the presence of xanthelasma-like lesions (n = 8). Two patients had nonfacial cutaneous xanthomas. Seven patients presented with nonxanthomatous cutaneous involvement, with the most common finding being subcutaneous nodules (n = 5). A single patient presented with granuloma annulare-like lesions. Another patient with mixed ECD and Langerhans cell histiocytosis presented with lightly scaling, pink-red macules. In three patients, the appearance of skin lesions was the first manifestation of the disease. Most patients presented with bone/extremity pain, weight loss and other constitutional symptoms at the time of diagnosis. The BRAF V600E mutation was not found in patients with panniculitis-like and granuloma annulare-like lesions. CONCLUSIONS: The most common presentation in ECD is the presence of periorbital xanthelasma-like lesions. Other presentations include nonfacial cutaneous xanthomas, panniculitis-like lesions and granuloma annulare-like lesions. Associated symptoms at presentation include bone/extremity pain and weight loss. What's already known about this topic? Erdheim-Chester disease is a rare form of non-Langerhans cell histiocytosis characterized by lipid-laden macrophage infiltration of tissue and subsequent fibrosis. Cutaneous involvement is found in approximately 25% of patients, with the majority presenting with periorbital xanthelasma-like lesions. What does this study add? We report two novel cutaneous findings: panniculitis-like lesions and granuloma annulare-like lesions. Associated bone/extremity pain and weight loss should raise suspicion for Erdheim-Chester disease.

Presentation, complications, and impact of concurrent malaria infection on anticancer therapy.

BACKGROUND: There is very limited data on the effects of malaria on on-going anticancer therapy. MATERIALS AND METHODS: We performed a retrospective analysis of adult solid tumor patients who contracted malaria while on active anticancer therapy. We noted their demographic profile, clinical course and the effects of malaria infection on their on-going anticancer therapy. Analysis was done with simple percentages. RESULTS: We analyzed 33 malarial episodes in 30 patients over 3 months. Plasmodium vivax was the most common type of infection (75%). Presenting symptoms included the typical triad of fever with chills and rigors. Malaria caused multiple complications, necessitating hospitalization in half of the patients and intensive care unit care in 1 of 8 patients. Common complications included thrombocytopenia (73%), anemia (67%), hyponatremia (66%), hepatic dysfunction (27%), and hypotension (12%). There were no deaths as a result of malaria. Malaria caused treatment delays with an average of 2.42 days per event. Plasmodium vivax caused more complications and therapy delays, average: 3.7 days per event, while non-vivax malaria caused an average of 0.5 days delay per event. There was a high level of resistance to chloroquine. CONCLUSION: Malaria is a significant problem in adult solid tumor patients, leading to multiple complications and therapy delays.

Combined effect of vacuum packaging and refrigerated storage on the chemical quality of paneer tikka.

Paneer tikka, a popular dish for vegetarians, is a tongue tingling favourite of Indian gourmets. It is a perishable commodity which requires more than five hours for its preparation. The shelf life of paneer tikka is hardly one day at room temperature which impedes its proper marketing. In order to enhance the shelf life of paneer tikka, the product was vacuum packed in two high barrier packages, viz., LLDPE(*)/BA(†)/Nylon-6/BA/LDPE(‡) (110 µ, (*)linear low density polyethylene, (†)binding agent, (‡)low density polyethylene) and metallized polyester/LDPE (20/75 µ) along with LDPE (100 µ) as control, and stored at 3 ± 1 °C. The stored samples were evaluated at 0, 10, 20, 30 and 40 day(s) for changes in chemical characteristics, namely moisture, pH, titratable acidity, free fatty acids content, tyrosine content and water activity. The analysis of variance of the data revealed that type of packages had significant influence on all the above parameters excluding water activity during storage of paneer tikka.

A study to evaluate the impact of examination stress on recurrent aphthous ulceration in professional college students in Jaipur district.

AIM: Recurrent aphthous ulcers (RAU) are one of the most common and poorly understood mucosal disorders. Most of the literature suggests that stress has a causal role in RAU and it is estimated that at least 1 in 5 individuals is afflicted with RAU. Review of literature reveals that nutritional and stress factors may be of paramount importance in the occurrence and severity of RAU. To test this hypothesis we have derived a study to explore definitive relationship between nutrition, stress and RAU in professional undergraduate students. METHODS: A total of 80 undergraduate students were selected randomly for the study from professional colleges. On evaluation 50 subjects were identified to be RAU positive and the rest 30 never had ulcers. All the subjects were evaluated during their university examinations and the 50 RAU positive subjects were later evaluated for the stress levels after the examinations. The major variables that were compared were simplified oral hygiene index, body mass index, hemoglobin percentage, mid upper arm circumference and malnutrition universal screening tool, Hamilton anxiety rating scale and general health questionnaire. RESULTS: The stress level was measured using Hamilton anxiety scale (HAS) and a highly significant difference was determined between during exams and post exam period in the students who tested positive for RAU. The General Health Questionnaire (GHQ) also appeared to be a sensitive instrument to determine the stress levels and statistically significant differences were recorded in the RAU positive students during exams and post exam period. General health is also poor during exams as compared after exams. The hematological factor as denoted by HB measurement showed significant statistical difference between patients who had RAU (present and history) and those who did not. Nutritional indicator (Malnutrition Universal Screening Tool) MUST also showed that it was a sensitive measure for detecting nutritional compromise and the statistically significant difference was shown between RAU positive and negative. The BMI, OHI-S and MUAC had no statistical differences shown in our analysis. CONCLUSION: A number of conditions and diseases have been shown to lead to the onset of RAU. However, RAU can occur in the absence of any diagnosable disease or physical condition. According to our evaluation, stress emerged as having a causal role on RAU, along with hematinic deficiencies and poor nutritional status in professional undergraduate college students.

Comparative study of thyrotoxic periodic paralysis from idiopathic hypokalemic periodic paralysis: An experience from India.

OBJECTIVE: There is paucity of reports on thyrotoxic periodic paralysis (TPP) from India. We report the patients with TPP and compare them with idiopathic hypokalemic periodic paralysis (IHPP). MATERIALS AND METHODS: Patients with hypokalemic periodic paralysis (HPP) treated during the past 11 years were evaluated retrospectively. Their demographic parameters, family history, clinical features, precipitating factors, severity of weakness, laboratory parameters and rapidity of recovery were recorded. The demographic, clinical and laboratory parameters of TPP and IHPP were compared. RESULTS: During the study period, we managed 52 patients with HPP; nine (17.3%) of whom had TPP and 27 (52%) had IHPP. The demographic, precipitating factors, number of attacks and severity of limb weakness were similar between the TPP and IHPP groups, except in the IHPP group, bulbar weakness was present in four and respiratory paralysis in six, needing artificial ventilation in two patients. Serum potassium was significantly lower in TPP (2.21 ± 0.49) compared with IHPP (2.67 ± 0.59, P = 0.04). Four patients with TPP had subclinical thyrotoxicosis and two had subclinical hyperthyroidism. Rebound hyperkalemia occurred in both TPP and IHPP (three versus eight patients). The recovery was faster in IHPP (26.7 ± 15.4 h) compared with TPP (34.0 ± 14.0 h), but was statistically insignificant. CONCLUSION: TPP constitutes 17.3% of HPP, and absence of clinical features of thyrotoxicosis and subclinical hyperthyroidism in TPP is not uncommon. Clinical features, demographic profile and rebound hyperkalemia are similar in both TPP and IHPP. The serum potassium level is significantly low in the TPP compared with the IHPP group.

Estrogen, progesterone and HER2 receptor expression in breast tumors of patients, and their usage of HER2-targeted therapy, in a tertiary care centre in India.

BACKGROUND: This study was undertaken to document the pattern of expression of estrogen (ER), progesterone (PR) and human epidermal growth factor receptor-2 (HER2) and the usage of HER2-targeted therapy in a large tertiary care hospital in India in the year 2008. MATERIALS AND METHODS: The histopathology reports of all breast cancer patients registered in the hospital in 2008 were extracted from the electronic medical record system. All the cases were immunohistochemically evaluated for estrogen and progesterone receptor status (ER and PR), and c-erbB-2 protein (HER2) expression using standard immunoperoxidase method. The use of HER2-targeted therapies was evaluated by extracting relevant information from the database of the hospital pharmacy and case charts of patients enrolled in ongoing approved trials. RESULTS: A total of 2001 new patients of invasive breast cancers with available pathology reports were registered in the hospital in the year 2008. ER and/or PR expression was positive in tumors of 1025 (51.2%) patients. HER2 3+ expression by immunohistochemistry (IHC) was found in 335 (16.7%) and HER2 2+ in 163 (8.1%). The triple negative phenotype was found in 596 (29.8%) patients. An estimated 441 patients were eligible to receive HER2-targeted therapy based on their HER2 status. Of these 38 (8.6%) patients received some form of HER2-targeted therapy; 20 patients (4.5%) as part of ongoing clinical trials and 18 (4.1%) as part of routine care. CONCLUSIONS: The overwhelming majority of patients eligible for HER2-targeted therapy in our institution are unable to receive it because of financial constraints and limited access to health insurance. There is a higher fraction of patients with the triple negative phenotype compared to the Western population.

Effect of MAP on microbiological quality of Mozzarella cheese stored in different packages at 7 ± 1 °C.

Mozzarella cheese was placed in two types of packaging materials (Cryovac, P1 and linear low density polyethylene/BA/Nylon-6/BA/Low density polyethylene, P2) under 5 different atmospheres, air (A1), vacuum (A2), 100% CO2 (A3), 100% N2 (A4) and mixture of 50% N2 and 50% CO2 (A5). The product was evaluated periodically for microbiological quality. Among the different gases studied, A3 showed minimum microbial count during storage, thus proving superior followed by A5, A4, A2 and A1.

Molecular characterization of MHC class II region in guinea fowl.

1. The MHC class II gene was amplified, cloned and sequenced in guinea fowl. 2. The NumeMHC II sequence of 754 nucleotides included complete exon 1 (91 nt), exon 2 (270 nt), exon 3 (282 nt) and exon 4 (110 nt). 3. The size of ß(1) and ß(2), domains were 89 and 93 amino acids, respectively in guinea fowl. 4. High amino acid variability (38·2%) was observed within guinea fowl in ß(1) domain, while in ß(2) domain, amino acid variability (6·3%) was low. 5. Among poultry species, the percent amino acid identity between guinea fowl and chicken, quail, pheasant and duck was 38·8, 42·2, 44·4 and 58·8 in ß(1) domain; and 13·8, 17·0, 13·8 and 27·6 in ß(2) domain, respectively. 6. Sequence alignment with mammalian and avian MHC showed that many of the conserved features of MHC class II glycoprotein was conserved in guinea fowl. 7. Within-species genetic distances (Poisson correction) based on cumulative amino acid variability in ß(1) domain and ß(2) domains was 0·141 in guinea fowl. 8. Guinea fowl showed low and similar genetic distances with all the poultry species (0·255-0·268) except duck (0·456). 9. Guinea fowl made separate branch within the major cluster having chicken, quail and pheasant, showing equal distance from these poultry species, whereas duck MHC II clustered separately.

Dietary Lycopene: Its Properties and Anticarcinogenic Effects.

Lycopene is the principal pigment of the carotenoids naturally found in tomatoes and is important not only because of the color it imparts but also because of the recognized health benefits associated with its presence. Red tomatoes typically contain about 95% of their lycopene as the all-trans isomer, the most stable form. In tangerine tomatoes, on the other hand, the lycopene is present as tetra-cis-lycopene, a geometric isomer of all-trans lycopene. Lycopene is a major component found in blood serum. This carotenoid has been extensively studied for its antioxidant and cancer-preventing properties. Prevention of heart disease has been shown to be another antioxidant role played by lycopene because it reduces the accumulation of platelets that eventually lead to blood clots, heart attacks, and strokes. In contrast to many other food phytonutrients whose effects have only been studied in animals, lycopene from tomatoes has been repeatedly studied in humans and found to be protective against several cancers, which now include colorectal, prostate, breast, lung, and pancreatic cancers. This review outlines the background information dealing with lycopene and presents the most comprehensive and current understanding of its potential functional role in human health.

Disappearing saccular intracranial aneurysms: do they really disappear?

SUMMARY: Evolution and natural history of cerebral aneurysms is a dynamic process. Spontaneous regression in size or complete disappearance of an aneurysm is a known phenomenon, more commonly noted in giant intracranial aneurysms. However, reappearance or regrowth of such aneurysms is rare with few anecdotal reports. We report a series of four cases including one giant aneurysm, which either disappeared or regressed on sequential angiograms. Regrowth or reappearance of two of these previously disappeared or regressed aneurysms was noted and endovascularly treated while the other two cases are being followed up. The decision to follow up was crucial considering the nature of the aneurysms to change in morphology under the influence of various hemodynamic factors.

Acute respiratory failure due to hypokalaemic muscular paralysis from renal tubular acidosis.

We report a case of hypokalaemic quadriplegia with acute respiratory failure and life-threatening cardiac arrhythmias in a 26-year-old woman who was diagnosed to have distal renal tubular acidosis. She had persistent metabolic acidosis with severe hypokalaemia and required mechanical ventilation and potassium replacement. The anaesthetic implications of renal tubular acidosis are also discussed.

Photosensitization of liposomal membranes by hematoporphyrin derivative.

The putative tumor-localizing and -photosensitizing fraction of hematoporphyrin derivative, the fastest migrating fraction of hematoporphyrin derivative separated by polyacrylamide gel filtration (HPD-A), photosensitized lipid peroxidation and membrane lysis in egg phosphatidylcholine liposomes. The rate of membrane damage was approximately 4-fold faster in oxygen compared to anoxia, with evidence for the involvement of singlet oxygen. The diffusion of HPD-A into small liposomes led to a shift of the Soret band from 363 nm in buffer to 398 nm accompanied by 4-fold enhancement of the fluorescence. The presence of human serum albumin retarded the diffusion of HPD-A into small liposomes, which is attributed to partial complexing of the HPD-A. A different effect of serum albumin was the protection of large liposomes from photosensitized lysis by incorporated HPD-A. This protection is attributed to scavenging of singlet oxygen, as evidenced by oxidation of tryptophan in the protein.