Perineal reconstruction for severe sequela of ecthyma gangrenosum

Ecthyma gangrenosum is a cutaneous gangrenous disorder which usually follows Pseudomona aeruginosa infection and is found mainly in immunosuppressed children. We describe a case of a five-year-old female with leukemia with a severe perineal ecthyma gangrenosum resulting in a cloaca-like deformity. One year later a perineoplasty with puborectalis interposition and overlapping external anal sphincteroplasty was successfully performed, achieving satisfactory continence.     

Bone Histomorphology May Be Unremarkable in Diabetes Mellitus

Abstract Background and Purpose:  Histomorphological studies on bone in human diabetes mellitus are scarce. The aim of this study was to observe the histomorphological appearance of bone in amputation specimens from feet of patients with diabetes mellitus. Material and Methods:  Routine histopathology reports on 45 amputation specimens were evaluated, provided the osteotomy was located in unaffected bone tissue. The bone morphology of regions affected with gangrene and/or osteomyelitis was considered, as well as the morphology at the dissection margin at a distance from the affected parts. The specimens were obtained from 43 diabetic patients, most of whom exhibiting the so-called neuroischemic diabetic foot with infection. The patients' age ranged from 50 to 92 years, duration of diabetes from 0 to 52 years; polyneuropathy was present in 36, peripheral ischemic vessel disease (PIVD) in 30, and renal failure in 24 of them, respectively. Results:  There were 22 clinical cases of osteomyelitis, 20 cases of gangrene (including three cases of necrosis without surrounding inflammation), and three cases of pressure ulcer, which were treated by amputation of 24 toes, and resection of 21 metatarsal bones, respectively. Histomorphology showed osteomyelitis (n = 29), bone necrosis (n = 1), myelofibrosis (n = 8), and normal bone (n = 7) at the affected sites, compared to normal bone (n = 26), myelofibrosis (n = 12), and osteoporosis (n = 7) at the osteotomy sites. In cases of clinical gangrene, bones were also affected by osteomyelitis, but less so than in cases of clinical osteomyelitis (8/18 vs. 22/22; p < 0.001). Bone tissue at the osteotomy sites was normal – with some myelofibrosis – in both conditions. Conclusion:  This pilot study shows that histomorphology of unaffected foot bone is mostly not abnormal in diabetic patients with neuropathy and PIVD. Further study is necessary to corroborate this preliminary evidence of absence of a “diabetic osteopathy” in the diabetic foot.      

Patients with Behcet's disease carry a higher risk for microvascular involvement in active disease period

BACKGROUND: Behcet's disease (BD) is characterized with remissions and exacerbations. However, to date, there is no study to investigate a possible association of disease activity (active versus inactive disease period) with cardiovascular complications. METHODS: Forty patients with BD were evaluated in both active and in inactive disease period. For the control group 45 healthy volunteers, age and sex matched, were registered. Subjects with at least a 15-day lesion-free period were regarded in inactive disease period, and subjects with any oral, skin, and/or genital lesion was regarded as in active disease period. In each subject coronary diastolic peak flow velocities (DPFV) were measured at baseline and after dipyridamole infusion (0.84 mg/kg over 6 minutes) using an Acuson Sequoia C256 echocardiography system. Coronary flow reserve (CFR) was defined as the ratio of hyperemic to baseline DPFV. RESULTS: CFR values were significantly lower in BD patients compared to the controls (2.57+/-0.50 versus 2.87+/-0.53, P = 0.006). In active disease period, basal DPFV (24.6+/-7.5 versus 27.3+/-6.6, P = 0.019) was significantly higher than in the inactive disease period. In the active disease period hyperemic DPFV (61.7+/-14.9 versus 56.8+/-16.7, P = 0.015) values decreased significantly. Therefore, in the active disease period CFR significantly decreased from 2.57+/-0.50 to 2.09+/-0.46, P<0.001. The only independent predictor of CFR within the active disease period was the disease duration (beta = -0.384, P = 0.012). CONCLUSION: Within the active disease period, coronary microvascular function is more prominently impaired in BD patients. Therefore, BD patients are possibly more vulnerable to cardiovascular manifestations when they are in an active disease period.     

Morphological and Histological Study on the Foot Pads of the Anatolian Bobcat (Lynx lynx)

This study was conducted to reveal the morphometric and morphological features of foot pads in the Anatolian bobcat (Lynx lynx). To achieve this objective, dissection, histological, and radiography techniques were applied to two dead materials obtained from the Republic of Turkey Ministry of Forest and Water Works Sivas Branch Manager. Digit I is radiographically rudimentary in the forepaw. The paws of the forelimb of the Anatolian bobcat have a carpal, a metacarpal, and four digital pads, while the hind feet have a metatarsal pad and four digital pads. The metacarpal pad is cone‐like, while the metatarsal pad resembles a butterfly. The digital pads in the paws of the forelimb are longer and thinner than in the paws in the hind feet. The paws in both feet are situated as binary. Through histological examination, it was determined that the skin of the foot pads consists of epidermis, dermis, and pad cushion. The epidermis is subdivided into basal, spinous, granular, lucidum, and corneum layers. The dermis of each pad consists of papillar and reticular strata containing sweat glands, elastin, collagen, and reticular fibers. Anat Rec, 301:932–938, 2018. © 2017 Wiley Periodicals, Inc.     

The relationship between angiotensin converting enzyme gene polymorphism, coronary artery disease, and stent restenosis: the role of angiotensin converting enzyme inhibitors in stent restenosis in patients with diabetes mellitus

Patients with diabetes mellitus (DM) have advanced atherosclerosis compared with nondiabetics. Restenosis after intracoronary stent implantation occurs frequently in diabetic patients. Angiotensin II is an important growth factor for the development of neointimal hyperplasia after vascular injury. The aim of our study was to evaluate the relationships between angiotensin converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism and coronary artery disease (CAD) and stent restonosis in diabetic patients. One hundred and thirty consecutive patients with CAD and 47 consecutive patients (14 males, mean age, 58.0 +/- 10.0) without CAD were enrolled in the study. All patients had type 2 (noninsulin dependent) DM. The patients with CAD underwent percutaneous transluminal coronary angioplasty (PTCA) and stenting. Ninety-four (59 males, mean age, 60.3 +/- 9.8) underwent control coronary angiography at the end of the follow-up period (mean duration, 9.1 +/- 2.9 months). ACE gene I/D genotyping was identified in all patients. No significant difference was found among patients with and without CAD with respect to ACE gene I/D polymorphism (P = 0.460). In the control coronary angiography, stent restenosis and new lesion development were comparable in each genotyping subgroup. However, a significant relationship was observed between restenosis and the use of ACE inhibitors (ACEI) in patients with D allele (ACEI ratio, 43.5% in the restenosis group and 56.5% in non-restenotic group, P < 0.05). We did not find any relationship between ACE gene I/D polymorphism and CAD and stent restenosis and new lesion development in diabetic patients. On the other hand, ACEI treatment may reduce stent restenosis in type 2 diabetic patients with D allele (DD or ID).     

Pulmonary artery aneurysms in Behçet’s disease

Behçet’s disease is the most common cause of pulmonary artery aneurysms. Pulmonary artery aneurysms are rare, but they are life-threatening because of their high tendency to rupture. However, there is also a chance that the aneurysms may completely resolve with immunosuppressive therapy. A 30-year-old man was admitted with chest pain, painful oral and genital ulcers, skin rash, weakness, and intermittent hemoptysis. He had a history of Behçet’s disease for 6 years. The chest radiography showed a round opacity superior part of hilus on right. The helical thoracic computed tomography (CT) angiography demonstrated pulmonary aneurysms associated with Behçet’s disease. The patient was successfully treated with colchicine, corticosteroids, and cyclophosphamide. A discussion about pulmonary artery aneurysms associated with Behçet’s disease is provided in this case.     

Spontaneous endogenous hypermelatoninemia: a new disease?

Melatonin, a major photoperiod-dependent hormone, regulates circadian rhythms and biological rhythms and acts as a prominent sleep promoter. Symptoms related to hypermelatoninemia have been reported in individuals supplemented with melatonin. However, spontaneous endogenous hypermelatoninemia has not been reported previously. A 6-year-old girl previously diagnosed with Shapiro's syndrome was admitted to our hospital on several occasions during a 1-year period with complaints of altered consciousness, syncope, hypothermia and episodes of sweating. The episodes occurred daily and during sleep and lasted for 1-6 h. During these episodes, she sweated profusely and felt faint and her skin was pale and cool. Other complaints included recurrent abdominal pain, urge incontinence and myopia. She was shown to have hypermelatoninemia (>1,000 pg/ml, normal range 0-150 pg/ml) during these episodes. The duration of her attacks decreased with phototherapy and she was successfully treated with propranolol. To our knowledge, this is the first case of hypermelatoninemia without any detectable organic pathology. We did not determine the exact mechanism of hypermelatoninemia in this patient; however, it might have been related to irregular control of pinealocytes by the suprachiasmatic nucleus or related pathways. Hypermelatoninemia should be considered in patients with spontaneous periodic hypothermia and hyperhidrosis, and also in patients with Shapiro's syndrome.     

Investigating transmission of Mycobacterium bovis in the United Kingdom in 2005 to 2008

Due to an increase in bovine tuberculosis in cattle in the United Kingdom, we investigated the characteristics of Mycobacterium bovis infection in humans and assessed whether extensive transmission of M. bovis between humans has occurred. A cross-sectional study linking demographic, clinical, and DNA fingerprinting (using 15-locus mycobacterial interspersed repetitive-unit-variable-number tandem-repeat [MIRU-VNTR] typing) data on cases reported between 2005 and 2008 was undertaken. A total of 129 cases of M. bovis infection in humans were reported over the period, with a decrease in annual incidence from 0.065 to 0.047 cases per 100,000 persons. Most patients were born pre-1960, before widespread pasteurization was introduced (73%), were of white ethnicity (83%), and were born in the United Kingdom (76%). A total of 102 patients (79%) had MIRU-VNTR typing data. A total of 31 of 69 complete MIRU-VNTR profiles formed eight distinct clusters. The overall clustering proportion determined using the n - 1 method was 33%. The largest cluster, comprising 12 cases, was indistinguishable from a previously reported West Midlands outbreak strain cluster and included those cases. This cluster was heterogeneous, having characteristics supporting recent zoonotic and human-to-human transmission as well as reactivation of latent disease. Seven other, smaller clusters identified had demographics supporting recrudescence rather than recent infection. A total of 33 patients had incomplete MIRU-VNTR profiles, of which 11 may have yielded 2 to 6 further small clusters if typed to completion. The incidence of M. bovis in humans in the United Kingdom remains low, and the epidemiology is predominantly that of reactivated disease.     

Beneficial effect of triple treatment plus immunoglobulin in experimental nephrotic syndrome

Combinations of antiproteinurics, including angiotensin I-converting enzyme inhibitors + angiotensin II receptor antagonist + statins, are promising choices in the treatment of steroid-resistant nephrotic syndrome. We aimed to investigate the effects of high doses of immunoglobulin in addition to these combinations in rats with adriamycin-induced nephrosis. The study included 40 rats allocated into five groups: control, nephrotic syndrome without treatment, dual therapy (DT) with enalapril + losartan, triple therapy (TT) with enalapril + losartan + simvastatin, and quadruple therapy (QT) with enalapril + losartan + simvastatin + a high dose of immunoglobulin. The proteinuria levels were not statistically different between DT, TT and QT groups at weeks 5, 8, 12 and 16. At week 16, serum creatinine levels in the QT group were significantly lower than those in the control, DT and TT groups. The glomerulosclerosis index in the DT group was significantly lower than in the TT and QT groups. The scores for interstitial fibrosis and TGF-β staining were similar among treatment groups. In conclusion, we showed that quadruple therapy including immunoglobulin had a beneficial effect on renal function in the late phase, but it had no additional effects in reducing proteinuria or in glomerulosclerosis score in experimental nephrotic syndrome. Further studies with angiotensin I-converting enzyme inhibitors (ACEIs), angiotensin II receptor antagonists (AIIRAs) and immunoglobulin combinations would offer some benefits in the treatment of nephrotic syndrome.