Mixed Variant of Acrokeratosis verruciformis of Hopf: A rare entity

Acrokeratosis verruciformis of Hopf (AKV) is a rare autosomal dominant genodermatosis of unknown etiology. Here we present a case of a 20-year-female with multiple skin-colored flat papules over the dorsum of hands and feet interspersed with multiple hypopigmented macular lesions of 5 years duration. No family member showed a similar lesion. The presence of classical AKV with absent family history and definite histopathology findings make this case an unusual and rare entity.

Atezolizumab in patients with renal insufficiency and mixed variant histology: analyses from an expanded access program in platinum-treated locally advanced or metastatic urothelial carcinoma

BackgroundAtezolizumab is a treatment for locally advanced/metastatic urothelial carcinoma (mUC). However, its use in patients with renal insufficiency or UC with mixed variant histology (MVH) is not well characterized.ObjectiveTo report efficacy and safety of atezolizumab in these special subpopulations from an expanded access program (EAP).Design, setting, and participantsA total of 218 patients were enrolled at 36 US study sites (November 2015–August 2016), and the trial ended following the approval of atezolizumab by the US Food and Drug Administration. This post hoc analysis investigated outcomes in specific study subgroups.InterventionAtezolizumab 1200 mg was administered intravenously every 3 weeks until loss of clinical benefit, unacceptable toxicity, death, consent withdrawal, decision to discontinue, commercial availability, or study closure.Outcome measurements and statistical analysisResponse Evaluation Criteria in Solid Tumors V.1.1 responses and safety were evaluated by baseline renal function and histology.Results and limitationsObjective responses occurred in 0/6 (0%), 4/19 (21%), 1/27 (3.7%), and 12/62 (19%) of evaluable patients with creatinine clearance (CrCl) <30, 30–45, 45–60, and ≥60 mL/min, respectively, and stable disease was seen in three patients with CrCl <30 mL/min. Objective responses were seen in 13/102 patients (13%) with urothelial carcinoma (UC) histology only and in 4/12 patients (33%) with UC with MVH. Treatment-related adverse event frequencies ranged from 35% to 54% across the earlier indicated CrCl subgroups and they were also similar in patients with pure UC or UC with MVH (46%).ConclusionsIn this EAP mUC subgroup analysis, clinical benefit of atezolizumab occurred in patients with compromised renal function or MVH UC tumors. Safety was comparable across subgroups.Patient summaryWe examined the efficacy and safety of atezolizumab for UC in certain patients participating in an EAP. We found that responses to atezolizumab occurred, and safety was similar, in most patient subgroups with varying levels of kidney functioning or less common types of tumor tissue histology.

Expression of nectin-4 in bladder cancer with variant histology.

546 Background: The antibody-drug conjugate enfortumab vedotin is poised to change the bladder cancer (BC) treatment landscape by targeting Nectin-4, near ubiquitously expressed in urothelial cancer (UC). Less is known about this and other targets in BC with pure or mixed variant histology (VH). Methods: Immunohistochemistry (IHC) was performed on a Ventana Discovery Autostainer (Roche Diagnostics) using an ultraView DAB detection kit (Roche Diagnostics) and a Nectin-4 polyclonal antibody (1:100 dilution; Abcam, Cambridge, UK). The intensity and extent of Nectin-4 expression was determined by the histochemical scoring (H-score) used in preclinical testing, defined as the sum of the products of the staining intensity (score of 0–3) x % of cells (0–100) stained at a given intensity. Specimens were assessed by H score as: negative (0–14), weak (15–99), moderate (100–199), and strong (200–300). Results: Forty UC and VH BC were evaluated for Nectin-4 expression by IHC: 15 small cell (SCBC) (8 pure SCBC, 6 mixed SCBC/UC, 1 SCBC/CIS), 8 carcinosarcomas (CS) (7 pure CS, 1 HGUC/sarcomatoid features), and 17 pure HGUCs. Normal urothelium and stroma were negative. Eight of 8 (100%) pure SCBC were negative for Nectin-4. Six of 7 (85.3%) mixed SCBC+HGUC/CIS had weak staining and 1/7 (14.7%) had moderate staining in the urothelial components (comp) while 7/7 (100%) of the SCBC comp were negative. Seven of 7 (100%) pure CS were negative and 1/1 (100%) mixed CS+HGUC showed weak staining in the HGUC comp while the sarcomatoid comp was negative. Expression in UC was: 1/17 (5.9%) strong, 3/17 (17.6%) moderate, 10/17 (58.8%) weak, and 3/17 (17.6%) negative. Gene expression profiling confirmed Nectin-4 was downregulated in VH compared to UC samples, as was ERBB2 and Trop2. Conclusions: There is heterogeneity of expression of Nectin-4 and other targets in BC with VH compared to UC. This may have therapeutic implications, and highlights need for additional research in VH.[Table: see text]

Psychosocial aspects of adjustment disorders in women

The aim is to study the psychosocial aspects of adjustment disorders in women. Materials and methods: 54 women with diagnosed adjustment disorders (F43.2) who applied for advisory support were examined. The analysis of their anamnestic data with the help of a special questionnaire was performed, a clinical and psychopathological examination was conducted. To study various aspects of psychosocial maladjustment in this contingent of patients “The Stress Scale” by T.H. Holmes and R.H. Rahe (1967), “The Scale of Psychosocial Maladjustment” by L.O. Herasymenko, A. M. Skrypnikov and M. Rokeach methodology of studying of the value orientations (Fantalova O.B. modification, 1992) were used. Results: In 77.8% of cases a mixed type of maladjustment with dominance in the internal structure of factors of family and industrial maladjustment and in 29.6% a family maladjustment monovariant were identified. In most cases, the family and production variants were combined and a mixed variant of maladjustment was diagnosed. At the same time the most serious forms of maladjustment related to the sexual sphere. Analysis of the structure of psychosocial maladjustment of patients with adjustment disorders showed that the most typical manifestations of this disorder were the following: dissatisfaction with a sense of comfort (75,95 %), dissatisfaction with the psychological climate in the family (62,03 %) and with the period of marriage (62,03 %), dissatisfaction with psychological relationships with colleagues (60,76 %) and with the psychological relationship of the spouse (60,76 %) and a high multiplicity of irritation (54,43 %). Conclusions: The leading factors of maladjustment among the examined women were the stress in subjectively significant areas of activity and the conflictual nature of the desirability and accessibility of basic life values.

Features of depressive and anxiety manifestations in the first episode of bipolar affective disorder

Background. The study of depression and anxiety in the first episode of BAR is important for the timely detection, treatment and prevention of poor diagnosis of the disease. Objective – the study of the features of depressive and anxiety symptoms of the first episode of bipolar affective disorder, taking into account the gender factor and the clinical type. Materials and methods. We have clinically examined 65 men and 88 women diagnosed with first episode (FE) of bipolar affective disorder (BAD). Results. In patients with depressive FE of BAD, all indicators, with the exception of the undifferentiated depression indicator, in men are slightly higher than in women: the overall indicator (respectively 22.55±3.61 points and 22.16±3.03 points); adynamic depression (17.41±2.62 points and 16.76±2.48 points); pervasive depression (9.75±3.13 points and 9.69±2.66 points); depression with fear (9.34±2.55 points and 9.51±2.27 points); undifferentiated depression (4.89±0.95 points and 5.01±1.22 points); in patients with the mixed variant are not significantly different: accordingly 15,83±2,64 points and 17,00±3,32 points; 11.00±1.67 points and 11.80±1.64 points; 6.67±1.63 points and 6.60±2.07 points; 7.33±1.21 points and 8.00±1.87 points; 3.67±1.03 points and 3.40±1.14 points; in patients with a manic type there are no signs of depression. The indicators of anxiety in men and women do not differ significantly: in the depressive variant, the total indicator was accordingly 21.41±7.01 points and 23.36±7.01 points; psychic anxiety – 13.25±3.86 points and 14.35±3.87 points; somatic anxiety – 8.16±4.05 points and 9.01±4.10 points; when mixed, accordingly, 20,00±4,52 points and 22,00±4,90 points; 13.33±3.27 points and 15.20±2.39 points; 6.67±3.27 points and 6.80±3.70 points; the manic variant showed no signs of anxiety. The mean Zung score for the depressed variant was 68.82±8.30 points and 65.97±8.41 points, accordingly, for the mixed one, 44.00±5.55 points and 50.40±5.32 points, accordingly, at a manic variant 2.13±1.64 points and 2.50±1.60 points. Conclusions. Differences in manifestations of depression and anxiety in the first episode of bipolar affective disorder are determined by the clinical option; the impact of gender on these manifestations is insignificant.

Features of dyssomnyas in primary episode of bipolar affective disorder

Bipolar affective disorder (BAD) is one of the actual problems in modern psychiatry. The aim of the study is to investigate dyssomnia in patients with primary episode of BAD, taking into account, the sex and clinical features of the diseases debut. We have clinically examined 65 men and 88 women diagnosed with bipolar affective disorder in the period 2015–2019. It was found that dyssomnia is present in 98.3% of all patients, in 97.7% of men and in 98.7% of women with depressive variant of the primary episode of BAD, accordingly in 30.4%, 26.7% and 37.5% of patients with manic variant and at 100,0% of patients with mixed variant. The structure of sleep disorders varies significantly depending on the variant of the primary episode: with depressive, prevail night and early awakenings (100.0%), sleep disturbance (75.6%, 70.5%, and 78.7%), unstable sleep (47.9%, 59.1% and 41.3%, p<0.05), nightmares (12.6%, 13.6% and 12.0%); at manic — unstable sleep (73.9%, 73.3% and 75.0%), sleep disturbance (17.4%, 13.3% and 25.0%) and frequent night awakenings (13.0%, 13.3% and 12.0%), in the mixed variant — falling asleep (72.7%, 66.7% and 80.0%), unstable sleep (72.7%, 83.3% and 60.0%), frequent night awakenings (54.5%, 66.7%, and 40.0%), early awakening (36.4%, 50.0%, and 20.0%) and nightmares (27.3%, 16.7% and 40.0%). Thus, the primary episode of bipolar affective disorder is accompanied by a wide range of dyssomnias, among which the leading are disorders of depth and stability of sleep. Manifestations of dyssomnia vary depending on the clinical type of the primary episode of BAD: with the depressive variant, prevail night awakenings, early awakenings and sleep disturbances, with unstable sleep, and with mixed sleep disturbance and unstable sleep. Dyssomnia prevalence is highest for mixed and lowest for manic variants. In depressive men, unstable sleep was more common, and the other disorders of sleep in men and women were not significantly different.

Socio-demographic characteristics of patients with diff erent clinical variants of the primary episode of bipolar aff ective disorder

The analysis of the medical records of 402 patients with a primary episode of bipolar aff ective disorder revealed early onset of the disease, low educational, occupational and social levels of patients, as well as high aff ection by their addictive behavior. Characteristics of these changes depend on the clinical variant of the initial episode and are the worst in patients with mixed and manic variants, and relatively better — in patients with depressive variant of the initial episode. During life, the level of professional, social and family implementation of patients remains low, with the worst social consequences of the disease in patients with a manic and mixed variant of the initial episode, and relatively better — in patients with depressive variant. These features should be taken into account when developing diagnostic, therapeutic and prophylactic approaches. Keywords: bipolar aff ective disorder; primary episode; socio-demographic features

A rare clinical case of retrograde small-bowel intussusception in pediatric practice

Intestinal intussusception in children is a mixed variant (with elements of obstruction and strangulation at the same time) of intestinal obstruction caused by isoperistaltic insertion of the proximal intestine into the distal. The article describes a rare clinical case of retrograde small bowel intussusception in a teenage girl aged 17 years with severe neurological pathology. Anamnesis, clinical symptoms and results of paraclinical methods of research, methods and results of the conducted treatment are given. The aim of the article is to familiarize doctors of practical health care with an extremely rare variant of intestinal intussusception. Retrograde (antiperistaltic) small bowel intussusception in children is very rare. Given the neurological status of the patient, the probable cause of intussusception was spastic neurogenic small intestinal dyskinesia. The direct cause of the disease was feeding mistakes in the form of feeding large amounts of strained food through the gastrostomy.