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In 96 patients with congenital absence of the uterus and upper vagina, the Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, it proved possible to distinguish between the typical and the atypical form using laparoscopy. The typical form was characterized by symmetrical nonfunctioning muscular buds (the Müllerian duct remnants) and normal fallopian tubes, and the atypical form by aplasia of one or both buds, one bud smaller than the contralateral one, with or without dysplasia of one or both fallopian tubes. The atypical form was found in 52 patients (54.2%). Radiographs of the spine showed that congenital spinal abnormalities, especially the Klippel-Feil (KF) syndrome, were seen in 14 of the 52 patients with the atypical form only. Renal agenesis or ectopia together with the MRKH and KF syndromes, known as the MURCS association (MU: Müllerian duct aplasia; R: renal agenesis/ectopia; CS: cervical somite dysplasia), was diagnosed in 10/52 patients in the atypical group. From our results we conclude that additional cervical spine films in patients with the MRKH syndrome are indicated only in the atypical form the syndrome. In those cases where the MRKH syndrome is associated with the KF syndrome, the MURCS association should be considered.

The problem of zoonoses remains relevant in the context of reliable prevention of human disease and effective ways to achieve this result, in particular through the impact on susceptible animals by the efforts of veterinary medicine. Anthrax is an acute, particularly dangerous infectious disease of all species of farm, domestic and wild animals, as well as humans, which is caused by Bacillus anthracis microbes. The causative agent of anthrax belongs to the group of aerobic spore-forming bacteria and exists in two main forms: vegetative and spore. The vegetative form in the body of an infected animal can form a "capsule". In Ukraine, according to the provisions of the current "Instruction for the prevention and control of animal anthrax" (2000), the main method of preventing anthrax among animals is regular vaccination of animals susceptible to this disease. The authors conducted a retrospective analysis of the epizootic and epidemiological situation of anthrax in Ukraine for the period 1994–2021 and made a critical assessment of the performance of mandatory measures for specific prevention of susceptible animals. In order to find out the ecological and geographical features of the spread of anthrax, data on outbreaks of the disease in cattle, swine, small ruminants and humans on the territory of Ukraine were analyzed by regions for the time period under investigation. Over the past 28 years in Ukraine, animal anthrax was registered in in all areas except Zhytomyr region. In total, during the analyzed period, 177 affected points and 637 infected animals (cattle, small ruminants, pigs, horses, wild and fur-bearing animals, dogs) were registered (estimated at 3.59 animals per outbreak). Cattle were most often involved in the epizootic process, followed by pigs and small ruminants, while horses and other animal species were least infected. Ecological and geographical analysis showed that the largest number of affected points among animals during the analyzed period was found in Kyiv, Volyn, Kharkiv, Luhansk, Khmelnytskyi, Cherkasy, Odesa, and Vinnytsia regions. A small number of affected points during the analyzed period were found in Zakarpattia, Ternopil, Kherson, Autonomous Republic of Crimea, Poltava, Dnipropetrovsk and Ivano-Frankivsk regions. During the analyzed period, 68 people in 11 regions of Ukraine were infected with anthrax, 15 outbreaks were registered (4.46 people per outbreak). Most cases were reported in Donetsk, Kyiv and Odesa regions. The association between outbreaks of anthrax in animals and cases of anthrax among humans has been established, this dependency was 86.6% (the index of contiguity, which takes into account the number of years with simultaneous registration of animal and human cases, was 0.5). The authors thoroughly proved that it is vaccination among susceptible animals that will finally prevent the incidence of anthrax among people.

To assess the relationship between anatomical variants of sacroiliac joint (SIJ) and subchondral changes detected in magnetic resonance enterography (MRE) in patients with Crohn's disease (CD). This was a retrospective study of 60 CD patients, who were divided into two groups: with (n = 16) and without SIJ (n = 44) involvement, depending on the presence of inflammatory (bone marrow edema) and structural changes (sclerosis and erosions) in MRE. Anatomical variants of SIJ were assessed in CT of the abdomen and/or pelvis, distinguishing typical form with convex iliac surface and atypical forms. Univariate and multivariate analyses were performed to reveal an association between joint changes and forms. Our study included 60 patients (38 males; mean age 38.72 years ± 13.33). Patients with SIJ changes were older (p = .044). No significant differences in CD localization and behavior were found. The most common SIJ lesions were structural changes (in 75% of patients); the main atypical form was the iliosacral complex. The univariate and multivariate analyses showed a significant association of atypical forms with total subchondral changes (odds ratio [OR]: 3.429, 95% confidence interval [CI] 1.043-11.268; p = .042; OR: 5.066, 95% CI: 1.273-20.167; p = .021, respectively), and with structural changes (OR: 4.185, 95% CI: 1.155-15.160; p = .029; OR: 5.986, 95% CI: 1.293-27.700; p = .022, respectively). Atypical forms of SIJ are a risk factor for the occurrence of structural joint changes in CD patients. An association between bone marrow edema and atypical forms was not found.

Orbital myositis occurs in typical and atypical forms. This review summarizes and updates the current state of knowledge of all forms of inflammation affecting extraocular muscle, excluding thyroid-associated orbitopathy. A comprehensive literature review of orbital myositis was performed. Orbital myositis typically occurs in an idiopathic acute form, with painful diplopia due to inflammation in 1 or more extraocular muscles of young adult females, which usually responds to a course of oral corticosteroids. Atypical forms include idiopathic chronic or recurrent orbital myositis, and myositis related to systemic autoimmune, inflammatory, and infective conditions. The commonest associated autoimmune condition is inflammatory bowel disease. Immunoglobulin G4-related ophthalmic disease often affects extraocular muscle. Drug reactions and rarely paraneoplastic disease may also cause extraocular muscle inflammation. Orbital myositis occurs in a typical acute steroid responsive form, but atypical forms related to specific autoimmune and inflammatory conditions are increasingly recognized. Orbital myositis has many similarities to uveitis and would benefit from a systematic approach to nomenclature, diagnosis, and treatment.

Key Clinical MessageVertebral hemangiomas are classified into typical and atypical forms. On imaging, atypical forms may look like malignant lesions. The confrontation of data from different imaging modalities (MRI; CT; scintigraphy) facilitates the diagnosis.

Objectives:Celiac disease (CD) is an autoimmune disorder that can be divided into typical and atypical forms. Atypical forms can show extraintestinal manifestations among which oral signs are very frequent. Considering that the pathogenesis of CD is related to a positivity to specific HLA-DQB1 haplotypes, we tested whether the presence of the HLA-DQB1*02 allele could be a hypothetical cause of the development of oral manifestations.Subjects and Methods:For this study was been examined the oral condition of 98 Sardinian patients, all affected by CD and all on a gluten-free diet for at least 1 year. Then was been determined each patient’s HLA-DQB1 haplotype and compared these results with clinical information. Results:The statistical analysis evidenced that the absence of the HLA-DQB1*02 allele predisposes to oral manifestations such as dental enamel defects (DED) and recurrent aphthous stomatitis (RAS) (Pvalue=5.98x10-05, OR = 0.23, CI: (0.10 - 0.45) per each copy of the HLA allele). Conclusions:These results showed that the presence of the HLA-DQB1*02 allele influences the development of oral signs in a dose-dependent manner and also how the HLA haplotype connected to oral signs could have a fundamental role for the diagnosis of atypical forms of CD.

Funding Acknowledgements Type of funding sources: None. Background Takotsubo syndrome (TKS) is characterized by the appearance of apical reversible dyskinesia in its typical form. Electrocardiogram (ECG) in the acute phase (<12 from symptom onset) generally shows anterior ST segment elevation. Nonetheless, other atypical forms of TKS have been described depending on the location of the dyskinetic segments, such as, mid-ventricular, basal and focal forms. Considering the different segments involved in these atypical forms, it seems reasonable to consider that ST changes in acute phase ECG could be different. Purpose To compare ECG in the acute phase of typical TKS versus mid-ventricular TKS, as it was the more frequent form of atypical TKS in our registry. Methods Patients included in the prospective TKS registry of our center according to the Mayo Clinic diagnostic criteria, with the first ECG performed less than 12 hours from the symptoms onset were reviewed. All cardiac left ventriculographies were reviewed to ensure a correct classification of the different types of TKS. Results A total of 297 patients were included in our local registry. 80 patients met our study inclusion criteria. 56 ECGs of typical apical TKS were compared to 24 ECGs of atypical midventricular TKS. There were no differences between the baseline characteristics in both groups, except for mid-ventricular TKS, that was more frequently triggered by physical stressor. Regarding the ECG analysis, the main difference found in our serie was related to ST-segment deviation (Table 1). While ST-segment elevation was more common in typical TKS than in atypical TKS (73% vs 50%), ST-segment depression (generally in inferior leads) was observed in 54% of patients with atypical TKS and in no patient with typical TKS (figure 1). Conclusion The different location of dyskinesia between typical TKS and mid-ventricular TKS is associated to significant differences in the ECG obtained in the first hours after the onset of the clinical symptoms. The presence of ST-segment depression is highly suggestive of mid-ventricular TKS. ECG characteristicsTypical (n = 56)Midventricular (n = 24)pSTe > 1mm, no (%)41 (73)12 (50)0,044STd >0,5 mm, no (%)013 (54)< 0,001T wave inversion, no (%)12 (21)4 (17)0,626Q wave, no (%)22 ( 39)12 (50)0,374cQT, mean (SD)445 (54)438 (37)0,578QRS low voltages*, n (%)9 ( 16)1 (4)0,328STe ST-segment elevation, STd: ST-segment depression, cQT: corrected QT interval *Voltages <5mm in all limb leads or <10mm in all precordial leads Abstract Figure. 12-lead ECG and left ventriculography

La malattia di Kawasaki è una vasculite acuta sistemica che colpisce i vasi di medio calibro, tipica dell’età pe-diatrica e soprattutto dei bambini di età inferiore ai 5 anni, la cui complicanza principale è rappresentata dallo sviluppo di aneurismi coronarici. La diagnosi è essenzialmente clinica e la terapia si basa sulla somministrazione di immunoglobuline per via endovenosa (IVIG). Questo lavoro ha lo scopo di descrivere la malattia di Kawasaki in una popolazione di 60 pazienti e valutare i principali fattori di rischio per lo sviluppo del danno coronarico. La malattia si è presentata in forma tipica nel 77% dei pazienti, in forma incompleta nel 18%, in forma atipica nel 5%. Nel 77% dei pazienti si è ottenuta la remissione dopo un solo ciclo di IVIG e nella maggior parte dei casi (81%) le IVIG sono state somministrate entro 10 giorni dall’esordio della malattia. La compromissione coronari-ca è stata riscontrata nel 55% dei pazienti, ma solo il 21% di questi (12% di tutti i pazienti) ha presentato un danno coronarico permanente. Fattori di rischio per lo sviluppo del danno coronarico sono risultati: il sesso maschile, la forma incompleta o atipica, la mancata risposta a un solo ciclo di IVIG e la somministrazione delle IVIG oltre il 10° giorno dall’esordio.

Cryoablation is an alternative treatment for atrioventricular nodal reentrant tachycardia (AVNRT) and right anteroseptal and midseptal accessory pathways (APs) with a low complication rate. A high recurrence rate is still a concern in pediatric patients. From February 2015 to March 2017, all consecutive patients who underwent cryoablation for supraventricular tachycardia were included in this study. The demographic and clinical data of the patients were reviewed. Fifty-two patients (AVNRT 43, anteroseptal and midseptal AP 9) were enrolled, including 24 males and 28 females. The median age at the time of the procedure was 15.6 years. For patients with AVNRT, 34 (79.1%) had the typical form, 5 had the atypical form (11.6%), and another 4 had both forms. For AP, four patients had right midseptal and 5 had right anteroseptal APs. The median total procedure time was 114 min (range 69-331 min), and the median fluoroscopy time was 25.9 min (range 9.2-99.6 min). After a median 6 attempts of cryomapping and 3 of cryoablation, the arrhythmia substrate was successfully ablated in 51 of 52 patients (98.1%). Ten developed transient second degree atrioventricular (AV) block and one developed transient third degree AV block, but none had permanent AV block or other complications. After a mean follow-up of 1.95 ± 0.54 years (range 1.1-2.86 years), there were three cases of recurrence (5.9%). The mean number of cryoablations decreased from 6.6 ± 6.4 (early group) to 3.1 ± 2.6 (late group) (p = 0.01) after a 1-year learning period. Cryoablation for AVNRT and anteroseptal and midseptal APs in pediatric and adolescent patients is safe and effective.

Diffuse sclerosis, neuromyelitis optica, multiple sclerosis, subacute combined degeneration and acute disseminated encephalomyelitis are briefly described from the anatomical point of view, especially with reference to their variations and to those of their interlocking aspects that point to a unitary concept of their pathogeny. Their features of similarity are compared to the changes described in cases of experimental allergic encephalomyelitis (Ferraro) produced in laboratory animals. Illustrations stress some of the features that are common to those diseases, either in their typical or atypical forms.

Preliminary observations on growth and developmental fine structure of Rickettsia canada in various organs and tissues of the hard tick, Dermacentor andersoni Stiles, are reported. R. canada is typically rod-shaped, being delimited with a three-layered wall having a velvety coating adsorbed to its exterior surface. A finely reticulated cytoplasmic matrix containing prominent ribosomes is delimited with a three-layered unit membrane. Average length and width of these organisms are 1.6 by 0.4 mum. Although R. canada produces a generalized infection in D. andersoni, hypodermal and muscle tissues experience heaviest growth. Three morphologically distinct rickettsial forms were observed in individual hypodermal cells: (i) typical growth forms with a finely reticulated cytoplasmic matrix and distinct ribosomes; (ii) atypical forms with lightly to densely staining cytoplasm and a coagulated appearance in which ribosomes cannot be distinguished from the matrix; and (iii) forms with crystalline bodies that have a striated to beaded lattice structure and, at times, a fibrillar body in the cytoplasm as well. Occasional finger-like to irregular invaginations of the plasma membrane are noted. Intranuclear growth was demonstrated by electron microscopy in gut epithelial cells only. Growth and development of R. canada were manifest in all tissues examined.

Pyoderma Gangrenosum of Penis

Optic neuritis (ON) is characterized as an inflammation of the optic nerve. Based on etiology, ON is classified to typical and atypical forms. Typical ON is often related to multiple sclerosis while atypical ON can be caused by various infections, autoimmune disorders or different inflammatory diseases. Although the cause of ON is multifactorial, a lot of attention is given to inflammatory and immune processes which are considered to contribute to demyelination of axons. Typical ON occurs as subacute unilateral decrease in visual acuity (VA) and pain which intensifies during eye movement. Atypical ON is characterized by intense pain or no pain, bilateral decrease in VA, hemorrhages and exudate in optic discs. In typical ON, fast and significant recovery of VA is common while in atypical ON, the prognosis of VA recovery is poor. Thanks to recent advances in the field of genetics, it is possible to identify an increasing number of genes that could be associated with ON pathogenesis.In this article we briefly review the epidemiology, causes, pathophysiology, symptoms, diagnostics and treatment of ON as well as its relation to multiple sclerosis.

Disorders that cause congenital scoliosis include Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. We present the case of a 46-year-old karyotypical (XX) woman with müllerian agenesis (MRKH type A, typical form), a rudimentary bicornate uterus, a blind vaginal pouch, and adenocarcinoma of both ovaries with subsequent bilateral salpingo-oophorectomy. She also had scoliosis of the thoracic and lumbar spine, an association thus far seen only among patients with type B (atypical) MRKH. We describe typical and atypical forms of MRKH and emphasize how these various anomalies associated with müllerian agenesis have affected the classification of the syndrome. We also outline possible embryologic etiologies of müllerian agenesis.

Purpose: To determine the state of local and general nonspecific resistance of the organism in the dynamics of surgical treatment of patients with odontogenic maxillary sinusitis in the presence of galvanic pathology in the oral cavity and to clarify the causes that cause the development of postoperative inflammatory complications. Methods: We examined 56 patients with odontogenic maxillary sinusitis in the dynamics of surgical treatment (sparing highmorotomy) in the presence of fixed metal dentures in the oral cavity. The age of the patients ranged from 29 to 58 years. Results: In patients with odontogenic maxillary sinusitis with the presence of oral cavity galvanosis (atypical and typical forms), i.e. in the subjects of the II observation group, gingivitis was detected in almost 100% of cases, which were located in the area of fixed metal dental prostheses. The most pronounced inflammatory phenomena in the oral cavity were in subjects with stamped-soldered metal constructions, as well as in patients with chromium-cobalt (chromium-nickel) metal inclusions or in persons with defects in the metal-protective coating with titanium nitride (areas of damage and abrasion of titanium nitride metal-protective coating). Conclusions: On the basis of the conducted examination of patients with odontogenic maxillary sinusitis in the presence of galvanic pathology in the oral cavity, it was found that in some forms of this pathology, i.e. galvanosis, there is a decrease in local and general nonspecific resistance of the body, which in turn lead to the development of inflammatory complications in the maxillary soft tissues (in 100%) and in the bone wound of the jaw, i.e. osteomyelitis (in 34.6%).