Sociodemographic, clinical, laboratory, treatment and prognostic characteristics of 156 generalized pustular psoriasis patients in Turkey: a multicentre case series.

BACKGROUND: Generalized pustular psoriasis (GPP) is a rare and severe inflammatory disease characterized by widespread and superficial sterile pustules on an erythematous background. OBJECTIVES: This multicentre study aimed to determine the clinical profile and course in a large cohort of patients with GPP. METHODS: One hundred and fifty-six GPP patients (mean age, 44.2 ± 18.7 years) who met the diagnostic criteria of the European Consensus Report of GPP were included in the study. Sociodemographic characteristics, quality of life, triggering factors of the disease, clinical, laboratory, treatment and prognostic features were evaluated. RESULTS: 61.5% of the patients were female. The rate of working at or below the minimum wage (≤$332.5/month) was 44.9%. Drugs (36.5%) were the most common trigger. While hypocalcaemia (35.7%) was the most important cause of GPP during pregnancy, systemic steroid withdrawal (20%) was the most frequently reported trigger for infantile/juvenile and mixed-type GPP (15%) (P < 0.05). Acute GPP (53.8%) was the most common clinic. Nails were affected in 43.6% of patients, and subungual yellow spots (28.2%) were the most common change. In annular GPP, fever (P < 0.001) and relapse frequency (P = 0.006) were lower than other subtypes, and the number of hospitalizations (P = 0.002) was lower than acute GPP. GPP appeared at a later age in those with a history of psoriasis (P = 0.045). DLQI score (P = 0.049) and joint involvement (P = 0.016) were also higher in this group. Infantile/juvenile GPP was observed in 16.02% of all patients, and arthritis was lower in this group (24.4 vs. 16%). GPP of pregnancy had the worst prognosis due to abortion observed in three patients. CONCLUSIONS: Recent advances in treatment have improved mortality associated with GPP, but abortion remains a significant complication. Although TNF-α inhibitors have proven efficacy in GPP, they can also trigger the disease. Mixed-type GPP is more similar to acute GPP than annular GPP with systemic manifestations and course.

Clinical characteristics, quality of life and risk factors for severity in palmoplantar pustulosis: a cross-sectional, multicentre study of 263 patients.

BACKGROUND: Palmoplantar pustulosis (PPP) is a rare, chronic, inflammatory skin disease characterized by sterile pustules on palmar or plantar areas. Data on PPP are scarce. AIM: To investigate the clinical characteristics and risk factors for disease severity in a large cohort of Turkish patients with PPP. METHODS: We conducted a cross-sectional, multicentre study of patients with PPP recruited from 21 tertiary centres across Turkey. RESULTS: In total, 263 patients (165 women, 98 men) were evaluated. Most patients (75.6%) were former or current smokers. The mean Palmoplantar Pustulosis Area and Severity Index (PPPASI) was 8.70 ± 8.06 and the mean Dermatology Life Quality Index (DLQI) score was 6.87 ± 6.08, and these scores were significantly correlated (r = 0.52, P < 0.001). Regression analysis showed that current smoking was significantly associated with increased PPPASI (P = 0.03). Coexisting psoriasis vulgaris (PsV) was reported by 70 (26.6%) patients. Male sex prevalence, PPP onset incidence, disease duration, DLQI, and prevalence of nail involvement and psoriatic arthritis (PsA) were significantly increased among patients with PPP with PsV. Of the 263 patients, 18 (6.8%) had paradoxical PPP induced by biologic therapy, and these patients had significantly increased mean DLQI and prevalence of PsA (r = 0.03, P = 0.001). CONCLUSION: Our data suggest that smoking is a risk factor for both PPP development and disease severity. Patients with PPP with PsV present distinct clinical features and patients with biologic therapy-induced paradoxical PPP have reduced quality of life and are more likely to have PsA.

Skeletal muscle loss during anti-EGFR combined chemotherapy regimens predicts poor prognosis in patients with RAS wild metastatic colorectal cancer.

PURPOSE: We aimed to assess whether anti-EGFR combined chemotherapy regimens are related with loss of skeletal muscle mass and to compare cetuximab and panitumumab therapies in the aspect of skeletal muscle area change as well as to assess whether skeletal muscle mass loss has prognostic significance in the RAS wild mCRC patients. MATERIALS AND METHODS: A total of 56 patients (30 patients in cetuximab arm and 26 patients in panitumumab) who had computed tomography images were retrospectively evaluated at the diagnosis and follow up during the treatment period before progression. RESULTS: During treatment period 24 patients (42.8%) had muscle loss. Of these, 7 (29.2%) patients were treated at first-line and 17 (70.8%) patients were treated at second-line setting. There was no significant difference in the aspect of skeletal muscle loss among cetuximab and panitumumab combined treatment regimens. Median PFS was 9.1 (8.6-9.6) months in muscle loss group and 13.9 (7.2-20.6) months in muscle stable group (p = 0.001). Median OS was 23.4 (95% CI 15.8-31.0) months in muscle stable group and 19.1 (95% CI 17.0-21.3) months in muscle loss group (p = 0.57) at first-line setting. For second-line, median OS was 21.2 (14.7-27.7) months in muscle stable group and 14.4 (6.0-22.4) months in muscle loss group (p = 0.003). CONCLUSIONS: Decrease in skeletal muscle mass before progression on CT imaging is an independent indicator for shorter PFS value in RAS WT mCRC patients who received anti-EGFR combined chemotherapy regimens at both the first and second-line settings. Beside that shorter overall survival values also were significantly seen in patients who had muscle loss during anti-EGFR therapy in the second-line setting.

Relation between sarcopenia and dose-limiting toxicity in patients with metastatic colorectal cancer who received regorafenib.

BACKGROUND: Sarcopenia is related to poor prognosis and drug toxicities in solid tumors. The aim of our study is to investigate the predisposition of patients with metastatic colorectal carcinoma who started regorafenib treatment to sarcopenia and prolonged survival. METHODS: Patients with metastatic colorectal carcinoma who receives regorafenib were search retrospectively. Dose-limiting toxicity was defined as dose reduction or toxicity requiring drug withdrawal. Sarcopenia evaluation was made with computed tomography performed within a month before treatment. Progression-free survival and overall survival were estimated. RESULTS: Thirty-six patients were found as suitable for the study. 63.9% of patients were found as basally sarcopenic. Dose-limiting toxicity occured 13 of 23 patients (56.5%) with basal sarcopenia, whereas only 1 of 13 patients (7.6%) with no sarcopenia exhibited dose-limiting toxicity (p = 0.005). Three patients suffered from grade 3-4 toxicity. Hand-foot syndrome, hypertension, and mucosal rash were the most seen side effects. Mean regorafenib treatment duration was 3.36 months. There was no significant difference in the progression-free survival (PFS) and the overall survival (OS) between sarcopenic patients and patients with no sarcopenia. Durations were as OS 24.2 weeks in patients with sarcopenia (95% CI 16.7-31.7), 28.1 weeks in patients with no sarcopenia (95% CI 20.5-35.7) (p = 0.36), and as PFS 14.2 weeks in patients with sarcopenia (95% CI 12.1-16.4), 14.8 weeks in patients with no sarcopenia (95% CI 9.7-20.1) (p = 0.65). CONCLUSION: Dose-limiting toxicity was significantly higher in basally sarcopenic patients who were started regorafenib as treatment of metastatic colorectal carcinoma. There was no significant relationship between overall survival and progression-free survival with sarcopenia.

Vitamin D deficiency in alopecia areata.

BACKGROUND: Alopecia areata (AA) is a T cell-mediated autoimmune disease that causes inflammation around anagen-stage hair follicles. Insufficient levels of vitamin D have been implicated in a variety of autoimmune diseases. Previous reports have described the effects of vitamin D on hair follicles. OBJECTIVES: To evaluate the status of vitamin D in patients with AA, and the relationship between vitamin D levels and disease severity. METHODS: A cross-sectional study of 86 patients with AA, 44 patients with vitiligo and 58 healthy controls was conducted. The serum vitamin D levels of the study group were determined by liquid chromatography/tandem mass spectrometry. RESULTS: Serum 25-hydroxyvitamin D [25(OH)D] levels in patients with AA were significantly lower than those of the patients with vitiligo and the healthy controls (P = 0·001 and P < 0·001, respectively). The prevalence of 25(OH)D deficiency was significantly higher in patients with AA (91%) compared with patients with vitiligo (71%) and healthy controls (33%) (P = 0·003 and P < 0·001, respectively). Furthermore, a significant inverse correlation was found between disease severity and serum 25(OH)D level in patients with AA (r = -0·409; P < 0·001). CONCLUSIONS: Deficient serum 25(OH)D levels are present in patients with AA and inversely correlate with disease severity. Accordingly, screening patients with AA for vitamin D deficiencies seems to be of value for the possibility of supplementing these patients with vitamin D.

Synthesis, crystal structure and ab initio/DFT calculations of a derivative of dithiophosphonates.

The compound 2 has been synthesized from the reaction of 2,4-Bis(4-methoxyphenyl)-1,3,2,4-dithiadiphosphetane-2,4-disulfide and (R)-1-[3,5-Bis(trifloromethyl)phenyl]ethanol in toluene. The obtained crude dithiophosphonic acid 1 has been treated with the excess of N(C2H5)3 to give rise to 2, [(+HN(C2H5)3][O-CH3CH-C6H3(CF3)2)(CH3OC6H4)PS2(-)]. The compound 2 has been characterized by using the spectroscopic methods such as IR, (1)H, (13)C, (31)P NMR and structural analysing method such as X-ray crystallography. It crystallizes in the orthorhombic system, whose space group is P212121. It consists of a dithiophosphonate bridged methoxyphenyl and bis(triflorophenylethyl) groups and a triethylammonium moiety linked by N-H⋯S and C-H⋯F hydrogen bonds. In the crystal structure, the C17H14F6O2PS2 molecule is elongated along the b-axis and stacked along the a-axis. The triethylammonium, N(CH2CH3)3, molecule fill in the cavities between the C17H14F6O2PS2 molecule. Moreover, ab initio methods based on Hartree-Fock (HF) and Density Functional Theory (DFT) calculations with the basis set of 6-31G(d) are also carried out to determine the molecular structural properties and to calculate FT-IR and NMR spectrum of the compound 2. The experimental results and theoretical calculations have been compared, and they are found to be in good agreement.

Transdermal methyl alcohol intoxication cause of pain relief.

A 60-year old female patient was found comatosed at home and taken to the hospital's Emergency Department by her relatives. It was learnt that she wrapped her knees with spirit-impregnated cotton pad for pain for one week. On physical examination, only a colour change of purple violet on her knees was noted. Metabolic acidosis with increased anion gap was detected by arterial blood analysis. The patient underwent haemodialysis. She was discharged from the hospital with no complaints, alert and rational following five days of follow-up treatment, with the diagnosis of methyl alcohol poisoning.

Transdermal methyl alcohol intoxication cause of pain relief / Intoxicación transdérmica con alcohol metílico tratando de aliviar un dolor

A 60-year old female patient was found comatosed at home and taken to the hospital's Emergency Department by her relatives. It was learnt that she wrapped her knees with spirit-impregnated cotton pad for pain for one week. On physical examination, only a colour change of purple violet on her knees was noted. Metabolic acidosis with increased anion gap was detected by arterial blood analysis. The patient underwent haemodialysis. She was discharged from the hospital with no complaints, alert and rational following five days of follow-up treatment, with the diagnosis of methyl alcohol poisoning.

Una paciente de 60 años de edad fue hallada en estado comatoso en su casa, y trasladada por sus familiares al departamento de emergencias del hospital. Se supo que la paciente había sentido dolor en sus rodillas, y las cubrió con almohadillas de algodón impregnadas de metanol por espacio de una semana. Al realizarse el examen físico, sólo se observó un cambio de color violeta púrpura en sus rodillas. El análisis de sangre arterial reveló acidosis metabólica con hiato iónico elevado. A la paciente se le practicó una hemodiálisis. Fue dada de alta del hospital sin dolencias, consciente, y en su sano juicio, luego de cinco días de seguimiento de su tratamiento, tras de haber sido diagnosticada con envenamiento por alcohol metílico.

Management of endograft infections.

Endovascular abdominal aortic aneurysm repair (EVAR) and thoracic endovascular aneurysm repair (TEVAR) have become well-accepted alternatives to traditional open surgery because of the diminished perioperative complications. Aortic stent-graft infection is an uncommon complication and little is known about the general features of and potential risk factors for aortic stent-graft infection, and treatment is administered on a case-by-case basis with no consensus guidelines. Despite a low infection rate, the associated mortality rates are extremely high and the morbidity rate, even with aggressive surgical interventions, is also high. Since 1991 only 117 cases of thoracic and abdominal endograft infections have been reported in the literature. This paper presents a detailed analysis of the features of all cases reported to date and reviews the epidemiology, diagnosis, management, and outcomes of thoracic and abdominal endograft infections.

Alkalinisation of local anaesthetics prescribed for pain relief after surgical decompression of carpal tunnel syndrome.

PURPOSE: To compare the duration of analgesia achieved following administration of buffered prilocaine versus plain prilocaine to patients undergoing surgical decompression of the median nerve. METHODS: 40 (32 female and 8 male; mean age, 50.5 years) patients who underwent surgical decompression of carpal tunnel syndrome were recruited. Patients were randomly allocated to 2 groups: the alkalinised group was given 10 ml of prilocaine hydrochloride 2% buffered with 1 ml of sodium bicarbonate 8.4%, whereas the non-alkalinised group received 10 ml of plain prilocaine hydrochloride 2% solution. Patients were asked to rate their comfort level at the operation site in the first 6 hours following surgery and after discharge from hospital using a visual analogue scale (VAS). The duration of analgesic effect was evaluated every 3 hours. Additional oral analgesia in the form of paracetamol 500 mg tablets was available to patients if required for break-through pain. RESULTS: Significantly lower VAS scores were reported by the alkalinised group during the first postoperative 12 hours. The change of VAS scores over time was significantly higher in the non-alkalinised group. The mean analgesic requirement for paracetamol tablets in the alkalinised and non-alkalinised groups was 4 and 34, respectively. CONCLUSION: Buffered prilocaine provided a longer postoperative pain-free period for patients undergoing surgical decompression of the median nerve. It is easy, safe, and cost-effective.

The effect of growth hormone on 24-h urinary creatinine levels in burned patients.

Catabolism is increased in burned patients. Creatinine excreted in urine is accepted as an indicator of catabolism of muscle mass. Growth hormone (GH) is one of the most potent anabolic agents. We investigated the effect of GH on 24-h urinary creatinine levels as an indicator of catabolism of muscle mass in burned patients. In 20 patients with severe burns, 24-h urinary creatinine levels were investigated for 3 days following hospitalisation. Then the patients were divided into two groups of 10. In the study group, following investigation of 24-h urinary creatinine levels for 3 days, GH 0.1 mg/kg was injected subcutaneously three times in a week. Following the last dose of GH, 24-h urinary creatinine levels were investigated for 3 days again. In the control group, an equal volume of isotonic saline solution was injected at the same times instead of GH, and 24-h urinary creatinine levels were investigated for 3 days again. Mean burn size and age were not significantly different between the groups. 24-h urinary creatinine level obtained in the early period was 48.5+/-16.6 mg/day in the study group and 49.9+/-11.3 mg/day in the control group. There was no statistical difference between these two values (p>0.5). 24-h average urinary creatinine level obtained in the late period was 36.6+/-16.4 mg/day in the study group and 50.6+/-9.9 mg/day in the control group, and the difference was statistically significant (p<0.05). In the comparison of early and late 24-h urinary creatinine levels in the study group, there was a statistically significant difference between these two values (p<0.05). In the control group, there was no difference between early and late 24-h urinary creatinine levels (p>0.5). We concluded that GH is effective in decreasing urinary creatinine excretion. This decrease in urinary creatinine excretion may be associated with diminished muscle catabolism.