Australasian Medical Journal
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Porphyria Cutanea Tarda (PCT) experience in Victoria, Australia
Get PDFBackgroundPorphyria Cutanea Tarda (PCT) is a metabolic disorder resulting from a deficiency of hepatic enzyme uroporphyrinogen decarboxylase (UROD). UROD deficiency results in the accumulation of porphyrins, which are phototoxic and hepatotoxic. PCT patients are at increased risk of developing hepatocellular carcinoma.AimsTo describe a series of PCT patients presenting to a tertiary center over 35-year period from the 1980s to December 2015 and review current literature to date on PCT, with a focus on PCT management.MethodsA search of the center’s dermatology department and biochemistry database were performed to identify patients diagnosed with PCT. Demographic data, underlying risk factors and management details were obtained. Statistical tests were performed to identify any possible association between the variables of interest.Results34 patients were included in this study. Mean age of diagnosis was 48 years and there was no gender difference. 12 patients had Hepatitis C infection, 25 had excessive alcohol consumption, 13 had hereditary haemochromatosis. Eight patients developed oestrogen-associated or hormonal replacement therapy (HRT) induced PCT. 33 patients (97 per cent) responded to venesection. Six (18 per cent) patients were prescribed hydroxychloroquine, either alone or concurrently with venesection. They all achieved remission. Average duration of follow up is 13 years. One patient developed hepatocellular carcinoma (HCC).ConclusionOur study has reinforced venesection as an effective treatment for PCT. Low dose hydroxychloroquine can be used in patients where venesection is contraindicated or not tolerated. General measures such as alcohol abstinence, visible violet light protection and trauma avoidance are recommended
The impact of the extent of thyroid resection complemented by radioiodine management on disease free status and discharge rates among Differentiated Thyroid Cancer patients: A retrospective analysis.
Get PDFBackgroundWell-differentiated thyroid cancers (DTC) are common endocrine cancers. They originate from follicular cells and parafollicular cells, and they have good prognosis. The pivotal treatment is surgery followed by radioiodine management. However, the extent of thyroid resection remains debatable. Currently both total thyroidectomy (TT) and thyroid lobectomy (TL) are performed followed by radioiodine management.AimsThe study aimed at evaluating the impact of the extent of thyroid resection complemented by radioiodine management on disease free status and discharge rates among DTC patients.MethodsA retrospective analysis of DTC patients’ files managed with radioiodine post-surgery in the Department of Nuclear Medicine at Dr George Mukhari Academic Hospital. The information extracted from the patient files included; the patient’s gender, histology, age, diagnosis, the nature of surgery (TT or TL), date of ablation, quantity of radioiodine dose administered and patients’ treatment status.ResultsSixteen patients met the study criteria; having had thyroid resection and later managed with radioiodine. A total of 5/16 (31.3 per cent) patients underwent TL and 11/16 (68.7 per cent) underwent TT. At the end of the study period, 5/11 (45.5 per cent) TT patients had been discharged compared to 1/5 (20 per cent) TL patients. The study also established that TL patients received higher cumulative doses compared to TT patients during the treatment, despite only two TL patients having been diagnosed with metastasis.ConclusionAdministration of radioiodine among TT patients increases disease free status while among TL patients its role is debatable. TT optimises the management DTC with radioiodine resulting in improved outcomes
Impact of muscle strength ladder training on disabled elders
Get PDFBackgroundCurrently, there are many studies focusing on the physical activity program of disabled elders, but little is known about effective and cheap appliances for the disabled elderly to take home exercise.AimsTo evaluate the impact of muscle strength ladder training on the activity ability of disabled elders.Methods A self-control trial was applied to 35 disabled elders in the communities in Shanghai for three months. The patients were evaluated for muscle strength and daily living activity (ADL) at the time of entry and intervention for three months.Results After the intervention, the patient's muscular strength grade was improved (P < 0.01), and the activity ability score was increased (P < 0.01).ConclusionMuscle strength ladder training could improve the muscle strength and activity ability of the upper and lower extremities. With the help of the muscle strength ladder training, the convenience, effectiveness and safety of exercise training could be increased
Contemporary challenges of warty carcinoma of cervix - Our experience and review of literature
Get PDFBackgroundWarty carcinoma of the cervix is a rare subtype of squamous cell carcinoma. In general, it is not as aggressive as the other subtypes, and has a better prognosis.AimsThe aim of this study was to investigate overall and recurrence-free survival rate in patients with Warty carcinoma of the cervix.MethodsDuring the ten year period (2008–2017) in the Clinic of Gynaecologic oncology at the UMHAT - Pleven, Bulgaria were operated 714 cases with cervical cancer, 14 of which were histologically confirmed as a Warty carcinoma. Patients were investigated by retro- and prospective analysis for overall and recurrence-free survival rate.ResultsWarty carcinoma accounts for 1.94 per cent of all cervical carcinomas, operated in the clinic. The mean age of the patients was 48 years, ranging from 29–72 years. According to the FIGO staging systems for cervix, patients were staged as follows: in stage IB1 – 43 per cent and in stage IB2 – 57 per cent. Despite the high percentage of locally advanced process, only in one case out of all the patients there was local spreading of the lesion towards the uterine cavity and in one case there were metastases in the pelvic lymph nodes. Lymphovascular space invasion was not seen in any patient, and neither were perineural or perivascular invasion.ConclusionThis report highlights a favourable course and good prognosis of Warty carcinoma of the cervix. It is necessary to reconsider the aggressive surgical treatment and subsequent chemotherapy in women with Warty carcinoma of the cervix
Bilateral complete ureteral duplication with fixed dumbbell stone formation in the ureterocele
Get PDFBilateral ectopic ureteroceles are extremely rare. There are few reports of bilateral duplex system with ureterocele stone formation. Our case report represents a 35-year-old woman with bilateral complete ureteral duplication and stone in a right sided ureterocele. A Bugbee electrode was used to puncture the ureterocele at its most dependent part followed by fragmenting the stone using pneumatic lithotripsy. Management of the ureterocele stone depends mostly on stone size. Whereas smaller stones are amenable to endoscopic incision and lithotripsy, larger ones have required combination therapy with endoscopic incision, laser or pneumatic lithotripsy and even orificiotomy and ureterolithotomy
Direct bacterioscopic observation of Helicobacter in the oral cavity, stomach and rectum
Get PDFBackgroundBacterial cells of Helicobacter Pylori (HP) are often found in people with chronic gastritis and peptic ulcer disease (PUD). All patients with symptoms are usually screened for HP bacterial cells by different methods of detection. Studies have shown that HP can colonize the stomach and other parts of the gastorointestinal tract such as the oral cavity and rectum. AimsTo visualize and evaluate the bacterial cells of Helicobacter Pylori in vivo in the gastric, oral and rectal mucosa using immunocytochemical detection. MethodsStudies were carried out on smears from biopsies of the oral cavity, rectum and stomach (ICD-10K29.3) from seventy patients with chronic gastritis for the detection of Helicobacter pylori (HP) using immunocytochemistry. This technique allows detection of both coccoid and spiral forms of HP.ResultsOur research demonstrated that the stomach was dominated by spiral forms, with coccoid forms being much less common (on average about 5 per cent). There was a quite different distribution of spirals and cocci in the oral cavity and rectum. The oral cavity demonstrated almost exclusively coccoid forms of HP, rarely spiral and HP were detected only in coccoid forms in the rectum.Bacterioscopic investigation of gastrointestinal mucosa carried out via direct immunocytochemical staining clearly shows that HP - mucosal colonization occurs in the stomach (typically more than 50 helical cells in a single field of view), and that HP exits the body through the oral cavity and intestinal tract (5–10 cocci forms in 300 fields of view). Results of HP detection in the oral cavity and rectum corresponded with HP detection in the stomach in 80 per cent and 83 per cent of cases, respectively.ConclusionImmunocytochemical observation of HP in the oral cavity, stomach and rectal mucosa suggests that HP bacterial cells enter the gastrointestinal tract as coccoids, colonize stomach mucosa in vegetative spiral form and leave as coccoid forms. Thus, our data from direct bacterioscopy strongly supports the hypothesis that HP infection spreads and contaminates the gastrointestinal tract through its coccoid forms
Utility of 18-Fluorodeoxyglucose positron emission tomography in the prediction of ventricular tachycardia and advanced conduction disease in patients with cardiac sarcoidosis
Get PDFBackgroundThe diagnosis and management of cardiac sarcoidosis (CS) along with the recognition of high risk features remains a significant challenge. Failing to identify reliable disease prognosticators often results in fatal ventricular arrhythmia or heart failure.AimsThis study sought to determine the association of regional fluorodeoxyglucose positron emission tomography (18FDG PET) patterns and the presence of ventricular tachycardia (VT) storm and conduction disease in patients with CS.Methods In an observational cohort analysis, patients with a working diagnosis of CS who underwent 18FDG PET imaging were included. Baseline clinical data and 18FDG PET data were collected and analysed.Results A total of 57 patients with CS were studied. All had abnormal 18FDG PET imaging consisting of inflammation, scar or combination of both. VT storm was present in 33 per cent of the cohort, while 63 per cent had high grade conduction disease. Mean inflammatory burden noted on 18FDG PET in those with VT storm was 12 per cent. Inflammation involving ≥2 basal septal or mid septal segments (> 15 per cent myocardium) was an independent predictor of VT storm (OR: 9.62, 95 per cent CI 1.97–46.9, p=0.005). The degree of inflammatory burden and regional inflammation pattern on 18FDG PET demonstrated significant concordance with the presentation of VT storm. Inflammation in the anteroseptal segment was associated with high grade conduction disease (OR: 8.00, 95 per cent CI 0.94–68, p=0.029).ConclusionThe presence of > 15 per cent myocardial inflammation on 18FDG PET imaging and regional inflammation ≥2 basal or mid septal segment significantly correlated with risk of ventricular arrhythmia specifically VT storm regardless of scar burden
Updated meta-analysis of the relation between heart disease and androgenic alopecia or alopecia areata
Get PDFBackgroundThe relationship between baldness and heart disease is still controversial. We performed an updated meta-analysis of observational studies to evaluate the relation between heart disease and androgenic alopecia or alopecia areata.AimsTo evaluate the relation between heart disease and androgenic alopecia or alopecia areata.Methods Studies were identified by searching Medline and Embase up to October 20, 2017 without language restriction. Meta-analysis was performed by using a random-effects model.Results Nine studies were included in the meta-analysis (eight on androgenic alopecia and one on alopecia areata: 44,806 participants). Compared to men without baldness, men with androgenic alopecia had an increased risk of heart disease (relative risk (RR): 1.32, 95 per cent CI: 1.08 to 1.63, p=0.01, I2=25 per cent), and younger men (< 55 or ≤ 60 years) showed a stronger association (RR: 1.44, 95 per cent CI: 1.11 to 1.86, p=0.01, I2=0 per cent). The positive relation depended on the severity of baldness and decreased in order of severe vertex (RR: 1.60, 95 per cent CI: 1.19 to 2.16, p=0.002), moderate vertex (RR: 1.41, 95 per cent CI: 1.22 to 1.64, p < 0.001), mild vertex (RR: 1.18, 95 per cent CI: 1.05 to 1.33, p=0.007), and frontal baldness (RR: 1.10, 95 per cent CI: 0.92 to 1.32, p=0.28)). In contrast, there was no significant relation between alopecia areata and heart disease (RR: 0.91, 95 per cent CI: 0.60 to 1.39, p=0.66).ConclusionAndrogenic alopecia is associated with heart disease, but alopecia areata is not
Neuron- specific enolase level in patients with metabolic syndrome and its value forecasting acute stroke
Get PDFBackgroundPatients with metabolic syndrome are at a greater risk of experiencing a cerebrovascular event. Several studies show that patients with metabolic syndrome have asymptomatic ischemic brain injury. In this case, there is a need for rapid determination of asymptomatic brain lesions and prediction of acute stroke.AimsThe aim of the study was to determine the neuron-specific enolase (NSE) serum level in patients with metabolic syndrome and the value of this level for forecasting acute stroke.Methods The study used the following information to determine metabolic syndrome: waist circumference, total cholesterol, triglycerides, high-density lipoprotein cholesterol, blood pressure, and blood glucose. Doppler sonography mapping of the brachiocephalic trunk was held to determine the percentage of the carotid artery stenosis. To determine asymptomatic ischemic brain injury, the NSE serum marker was measured. Statistical processing of the measurements was performed using the H test and the Mann–Whitney test. The possible link between MS and NSE were determined by logistic regression analysis. Mathematical modeling was performed using logistic regression.Results There are statistically significant differences in NSE concentrations in groups with metabolic syndrome and ischemic stroke patients. This assertion is confirmed by logistic regression analysis, which revealed the existence of a relationship between metabolic syndrome and increased concentration of NSE.ConclusionPatients with metabolic syndrome have an increased concentration of NSE. This indicates the presence of asymptomatic ischemic neuronal damage. A prognostic model for determining the probability that patients with metabolic syndrome will have an acute stroke was developed
Blood lead level and correlation with pregnancy-associated anaemia
Get PDFBackgroundThe most common reason that leads to anaemia is related to the reduction in certain trace elements such as Fe. However, it has been found that an elevation in some other heavy metals such as Pb could also lead to anaemia.AimsThis research aims to assess the Correlation between Pb blood levels and Fe, Haemoglobin levels during pregnancy among Jordanian women.Methods A cross-sectional study was conducted in the Northern area of Jordan. Venous blood samples collected from 167 pregnant women for the determination of haemoglobin (Hb), Fe and Pb levels of which 17 in the first trimester, 19 in second trimesters, 131 in third trimesters. Women were classified into two groups, the first group included cases with blood Pb Levels ≥10µg/dL (high blood Pb level group, n=118) and the second with blood Lead levels less than 10µg/dL (low blood Lead level group, n=49).Results About 71.4 per cent of women had anaemia (n=120; Hb>10.5g/dl) and 70.7 per cent of women had a high blood Pb level of ≥10µg/dL). Pb blood levels ranged from 6.45 to 28.0μg/dL. The mean (SD) of blood Pb level was 12.1 (4.1) µg/dL. The mean haemoglobin and Fe levels did not differ significantly between women with low and high levels of Pb. Blood Pb levels were not significantly correlated with haemoglobin levels (r=-0.025; P=0.747) nor with iron levels (r=0.099; P=0.241). After adjusting for important variables, Pb was not significantly associated with haemoglobin (P=0.223) and with iron (P=0.116).Conclusion The level of Pb in the blood of pregnant women has no any association with haemoglobin and Fe levels during pregnancy