A 5-month-old boy had erythematous nodules over the left side of his trunk with a segmental arrangement since birth. Histopathologic examination revealed sheets of foamy histiocytes infiltrating the dermis and subcutaneous fat, admixed with multinucleated giant cells and lymphocytes, making this an unusual case of juvenile xanthogranuloma appearing in a segmental distribution.
A 3-month-old female patient with a giant ulcerated nodule over the back since birth was diagnosed as congenital giant juvenile xanthogranuloma (JXG) based on clinical and histopathological examination. Congenital giant JXG with ulceration at birth is a rare presentation of JXG and commonly misdiagnosed. This case emphasizes the importance of being aware of the myriad presentations of JXG in order to make a correct diagnosis and avoid unnecessary investigations or treatment.
Traumatic brain injury (TBI) remains one of the leading causes of morbidity and mortality amongst civilians and military personnel globally. Despite advances in our knowledge of the complex pathophysiology of TBI, the underlying mechanisms are yet to be fully elucidated. While initial brain insult involves acute and irreversible primary damage to the parenchyma, the ensuing secondary brain injuries often progress slowly over months to years, hence providing a window for therapeutic interventions. To date, hallmark events during delayed secondary CNS damage include Wallerian degeneration of axons, mitochondrial dysfunction, excitotoxicity, oxidative stress and apoptotic cell death of neurons and glia. Extensive research has been directed to the identification of druggable targets associated with these processes. Furthermore, tremendous effort has been put forth to improve the bioavailability of therapeutics to CNS by devising strategies for efficient, specific and controlled delivery of bioactive agents to cellular targets. Here, we give an overview of the pathophysiology of TBI and the underlying molecular mechanisms, followed by an update on novel therapeutic targets and agents. Recent development of various approaches of drug delivery to the CNS is also discussed.
Lipoatrophic panniculitis is a rare condition affecting mainly children, often associated with connective tissue disease. We report a healthy 12-month-old girl with no clinical or laboratory features of connective tissue disease who presented with the progressive appearance of annular atrophic plaques beginning at the left arm. A histopathological analysis revealed lobular panniculitis, with fat necrosis and an associated inflammatory infiltrate supporting the diagnosis of lipoatrophic panniculitis. Lipoatrophic panniculitis should be considered in infants and young children with clinical features of panniculitis and fat atrophy even without clinical or serologic evidence of connective tissue disease.
Temporal bone squamous cell carcinoma (TBSCC) is rare and poses difficulties in diagnosing, staging and management. We describe a case series with six patients who were diagnosed TBSCC, from January 2009 to June 2014, with median age of 62 years old. All patients presented with blood-stain discharge and external auditory canal mass, showing that these findings should highly alert the diagnosis of TBSCC. Three patients staged T3 and another three with T4 disease. High-resolution CT (HRCT) temporal findings were noted to be different from intraoperative findings and therefore we conclude that MRI should be done to look for middle ear involvement or other soft tissue invasion for more accurate staging. Lateral temporal bone resection (LTBR) and parotidectomy was done for four patients with or without neck dissection. Patients with positive margin, perineural invasion or parotid and glenoid involvement carry poorer prognosis and postoperative radiotherapy may improve the survival rate. One patient had successful tumor resection via piecemeal removal approach in contrast with the recommended en bloc resection shows that with negative margin achieved, piecemeal removal approach can be a good option for patients with T2-3 disease. In general, T4 tumor has dismal outcome regardless of surgery or radiotherapy given.
Atopic eczema (AE) is a common chronic inflammatory skin disorder in children, with emollients and topical corticosteroids (TCSs) commonly prescribed as treatment. There is no published guidance on the correct order of application of emollient and TCS in children with AE.
The potential significance of the previously reported papaya (Carica papaya L.) beta-galactosidase/galactanase (beta-d-galactoside galactohydrolase; EC 184.108.40.206) isoforms, beta-gal I, II and III, as softening enzymes during ripening was evaluated for hydrolysis of pectins while still structurally attached to unripe fruit cell wall, and hemicelluloses that were already solubilized in 4 M alkali. The enzymes were capable of differentially hydrolyzing the cell wall as evidenced by increased pectin solubility, pectin depolymerization, and degradation of the alkali-soluble hemicelluloses (ASH). This enzyme catalyzed in vitro changes to the cell walls reflecting in part the changes that occur in situ during ripening. beta-Galactosidase II was most effective in hydrolyzing pectin, followed by beta-gal III and I. The reverse appeared to be true with respect to the hemicelluloses. Hemicellulose, which was already released from any architectural constraints, seemed to be hydrolyzed more extensively than the pectins. The ability of the beta-galactanases to markedly hydrolyze pectin and hemicellulose suggests that galactans provide a structural cross-linkage between the cell wall components. Collectively, the results support the case for a functional relevance of the papaya enzymes in softening related changes during ripening.
A new compound, chandonanol (1), along with four known compounds, chandonanthone (2), iso-chandonanthone (3), anastreptene (4), and (6R,7S)-sesquiphellandrene (5), was isolated from the MeOH extract of Bornean liverwort Chandonanthus hirtellus. The structure of the new metabolite was established by analyses of the spectroscopic data (1D NMR, 2D NMR, HRESIMS, and IR). These compounds were tested for their activity against antibiotic-resistant clinical strains. Chandonanol (1) exhibited potent bactericidal activity against Staphylococcus aureus and Escherichia coli.
The Bornean liverwort Gottschelia schizopleura was investigated phytochemically for the first time. Two new and four previously known clerodane-type diterpenoids were isolated from the MeOH extract of G. schizopleura through a series of chromatographic techniques. The structures of the new metabolites were established by analyses of their spectroscopic data (1D NMR, 2D NMR, HRESIMS and IR). All the isolated compounds 1-6 were tested against human promyelocytic leukaemia (HL-60), human colon adenocarcinoma (HT-29) and Mus musculus skin melanoma (B16-F10). Compound 1 and 2 showed active inhibition against HL-60 and B16-F10 cells.
A new compound, schistochilic acid D (1) and two known compounds (2 and 3) were isolated from MeOH extract of Bornean liverwort. Schistochila acuininata collected from Mount Trus Madi, Sabah. The structure of the new metabolite was established based on spectroscopic (ID NMR, 2D NMR, and IR). and HRESIMS data. In addition, another population of S. acuminata collected from Mount Alab (Sabah) yielded four known compounds, 2, 3, 4 and 5. These compounds were tested for their biological potential against the B 16-Fl0 cell line. Compounds 4 and 5 exhibited weak cytotoxic activity.
Paraneoplastic neurological disorder (PND) is a condition due to immune cross-reactivity between the tumour cells and the normal tissue, whereby the "onconeural" antibodies attack the normal host nervous system. It can present within weeks to months before or after the diagnosis of malignancies. Nasopharyngeal carcinoma is associated with paraneoplastic syndrome, for example, dermatomyositis, and rarely with a neurological disorder. We report on a case of nasopharyngeal carcinoma with probable PND. Otolaryngologists, oncologists and neurologists need to be aware of this condition in order to make an accurate diagnosis and to provide prompt treatment.
Multiple dermatofibromas is a rare entity consisting of more than fifteen lesions. Multiple clustered dermatofibroma is a distinct variant of multiple dermatofibromas and is defined as a well-demarcated plaque composed of individual dermatofibromas. We report a 16-year-old boy with multiple clustered dermatofibroma in a segmental distribution, which has previously not been reported in the literature.
Two new bicyclogermacrenes, capgermacrenes A (1) and B (2), were isolated with two known compounds, palustrol (3) and litseagermacrane (4), from a population of Bornean soft coral Capnella sp. The structures of these metabolites were elucidated based on spectroscopic data. Compound 1 was found to inhibit the accumulation of the LPS-induced pro-inflammatory IL-1b and NO production by down-regulating the expression of iNOS protein in RAW 264.7 macrophages.
BODIPYs are photosensitizers activatable by light to generate highly reactive singlet oxygen (1O2) from molecular oxygen, leading to tissue damage in the photoirradiated region. Despite their extraordinary photophysical characteristics, they are not featured in clinical photodynamic therapy. This review discusses the recent advances in the design and/or modifications of BODIPYs since 2013, to improve their potential in photodynamic cancer therapy and related areas.
Background: Patients' satisfaction is the key parameter to measure the quality of healthcare services. Value added-services (VAS) were introduced to improve the quality of medication deliveries and to reduce the waiting time at outpatient pharmacy.
Objective: This study aimed to compare the satisfaction levels of patients receiving VAS and traditional counter service (TCS) for prescription refills in Port Dickson Hospital.
Methods: A single-center, cross-sectional study was conducted in the outpatient pharmacy department of Port Dickson Hospital from 1 March to 30 June 2017. Systematic sampling method was utilized to recruit subjects into the study, except mail pharmacy in which universal sampling method was used. Data collection was done via telephone interviews for both groups.
Results: There was 104 and 105 in TCS and VAS group respectively. The response rate was 99.5%. Overall, a significant higher total mean satisfaction score in VAS group was observed as compared to TCS group (43.39 versus 40.49, p=0.002). The same finding was observed after confounding factors were controlled (VAS=44.66, 95% CI 43.07:46.24 versus TCS=39.88, 95% CI 38.29:41.46; p<0.001). VAS respondents reported more satisfaction than TCS respondents for both general and technical aspects. Among the VAS offered, mail pharmacy service respondents showed highest total mean satisfaction score, but no significant different was seen between groups (p=0.064).
Conclusion: VAS respondents were generally more satisfied than TCS respondents for prescription refills. A longitudinal study is necessary to examine the impact of other dimensions and other types of VAS on patients' satisfaction levels.
Study site: outpatient clinic, Hospital Port Dickson, Negeri Sembilan, Malaysia