Subacute Course Research Articles

Segmental necrosis of the outlet of the stomach and duodenum in a premature newborn baby

Introduction. Gastric perforation in newborns is a polyetiological, severe disease that combines a number of destructive conditions, such as: isolated pinpoint perforations, wall necrosis and gastric rupture. The first description of this pathology in a premature infant was made by Siebold in 1825, and only in 1968, G. Reams reported a case of successful diagnosis and surgical correction of this disease. Currently, the frequency of gastric perforation occurs from 1 to 6 cases per 30 thousand live births, and the mortality rate is within 40–70%.In all infants, disorders in the integrity of stomach wall can be divided into iatrogenic and “idiopathic” which are caused, in most cases, by intrauterine hypoxia. Therefore, while examining such patients, differential diagnostics between gastric perforation and necrotizing enterocolitis is often made.Clinical case. The authors present their experience in diagnosing and treating segmental necrosis of the stomach and duodenum outlet in a child with an extremely low body weight of 800 gr. Predisposing factors in the antenatal period were considered to be manifestations of hereditary thrombophilia, severe preeclampsia, placental insufficiency, and intrauterine growth retardation. As far as the discussed pathology had a subacute course, clinical changes were registered only on the 21st day of infant’s life, when pneumoperitoneum was detected at the plain X-ray image. When the patient’s condition was stabilized, median laparotomy was performed, at which radial necrosis with detachment in the gastroduodenal junction was seen. An end-to-end gastroduodenoanastomosis was put at the defect zone. In the postoperative period, the course was complicated with bronchopulmonary dysplasia, oblique femoral fracture with angular fragment displacement because of premature osteopenia as well as cholestatic hepatitis. The patient was discharged from the hospital in satisfactory condition in 80 days.Conclusions. Thus, better understanding of specific features in the clinical picture of the discussed pathology and of the mechanisms of perforation development in hollow organs of the abdominal cavity will reduce the number of complications and adverse outcomes in patients with extremely low body weight.

The many faces of cryptogenic organizing pneumonia (COP).

Organizing pneumonia (OP) is interstitial pneumonia with an acute or subacute clinical course and a histological pattern compatible with acute lung injury. It usually arises after a recent infection of the peripheral bronchial system, whereby is called secondary OP. However, often OP arises with no recognizable cause for which it is called cryptogenic organizing pneumonia (COP). From a radiological standpoint, COP exhibits different imaging appearances and this review makes radiologists and clinicians familiar with the entire spectrum of expected disease findings. Successful interpretation of imaging is pertinent for a rapid diagnosis of COP and knowledge of the entire spectrum of imaging findings in COP is mandatory.

Lupus erythematosus: Management of cutaneous manifestations during pregnancy.

Lupus erythematosus is an autoimmune disease that often affects the skin. Cutaneous manifestations are generally subdivided into different subtypes, including acute, subacute, and chronic courses. Management of lupus erythematosus cutaneous manifestations during pregnancy remains a clinical challenge until nowadays. To date, no recommendations have been published specifically for the treatment of this condition in pregnant women, so therapeutic strategies are mainly based on recommendations for general population and other rheumatologic and dermatologic diseases during pregnancy. This challenge is compounded by a lack of evidence-based studies, as clinical trials in pregnant women are considered unethical in many circumstances, so data are often extrapolated from low-evidence sources. The aim of this article consists in review currently evidence of treatment of lupus erythematosus cutaneous lesion in pregnant women.

Nocardiosis

Nocardiosis is a rare infectious disease caused by the bacterium Nocardia, of the Actinomycetales order. It causes granulomatous inflammation in different organs and has a subacute or chronic course. Lung involvement is most common in our setting. There are also cutaneous and disseminated forms with a special predilection for the central nervous system, which entail a high mortality rate. It is more common in immunosuppressed patients, although it can also occur in immunocompetent subjects, especially its cutaneous form. The main risk factors are situations which entail deficient cellular immunity. Molecular biology and MALDI-TOF mass spectrophotometry techniques have improved diagnosis in regard to time and reliability. Co-trimoxazole is the first-line drug. However, treatment will depend on the clinical form, degree of immunosuppression, and initial severity. In severe cases and in cases of disseminated disease, the combination of at least two antimicrobial agents with activity against Nocardia is recommended. Treatment must be prolonged in order to avoid relapse.

Infecciones por microorganismos del grupo HACEK y otros bacilos gramnegativos infrecuentes

There are a multitude of uncommon Gram-negative bacilli (GNB) capable of causing various infections in human beings; it is an extremely heterogeneous group of pathogens. Of note are those known as HACEK (Haemophilus, Aggregatibacter, Cardiobacterium, Eikenella, and Kingella) group GNB. They are slow-growing bacteria that are an infrequent cause of infective endocarditis with a subacute course and embolic complications. The majority are oral flora, causing infection following dental procedures. New microbiological techniques such as better culture media, MALDI-TOF, or molecular techniques such as DNA sequencing or the use of 16S rRNA PCR represent advances in their diagnosis. Treatment for them varies; amoxicillin-clavulanic acid or third-generation cephalosporins tend to be used. Of the uncommon non-HACEK GNB, the majority are ubiquitous bacteria that contaminate water and hospital surfaces, giving rise to nosocomial outbreaks of infections, including pneumonias, catheter-associated bacteremias, and other device-related infections. Of note are the genera Achromobacter, Alcaligenes, Chryseobacterium, and Elizabethkingia. Some genera, such as Actinobacillus, Aeromonas, or Chromobacterium, can give rise to infections following exposure to dirty water. Capnocytophaga and Pasteurella can produce infections following animal bites. Their treatment varies and tends to be based on beta-lactams with beta-lactamase inhibitors.

Acute intermittent porphyria in adults: a clinical case

Background. Porphyria unites genetic pathologies related to abnormal haem (an intermediate product of haemoglobin metabolism) synthesis and its toxic products accumulation in human body. Symptoms can vary, from photosensitivity, skin rashes and chronic abdominal pain towards partial or complete paralysis and acute psychosis. This metabolic disorder is diagnosed with molecular genetic and laboratory biosample tests. Drug therapy aims at reducing toxic metabolites concentration in patient’s blood.Clinical Case Description. A 28-yo female patient had an acute atypical porphyria attack with a later onset of neurovisceral manifestations (acute abdominal pain, tachycardia) progressing post-drug-treatment into acute sensorimotor polyneuropathy with flaccid, predominantly proximal, hands-prevalent tetraparaesis. Biochemical urine tests at the National Research Center for Hematology (by 30.06.2020) revealed porphobilinogen 55.3 mg/L at norm <3. Vital indications required an urgent haem arginate pathogenetic therapy (Normosang) in a 4-day course of 3 mg/kg/day drop infusion. The recommended course was well tolerated. Drug therapy and rehabilitation entailed a positive dynamics of restoring limb muscle strength towards an almost easy getting-up from chair and bed, and skin lightening. The patient was discharged on day 20 with diagnosis: “Acute intermittent porphyria. Axonal-demyelinating sensorimotor polyneuropathy. Severe flaccid asymmetric predominantly proximal hands-prevalent tetraparaesis. Subacute course, stabilisation phase. Condition after one course of haem arginate pathogenetic therapy (Normosang) at 3 mg/kg/day”. A resident haematologist surveillance was recommended, with a routine referral for inpatient examination and treatment at the Department of Orphan Diseases of the National Research Center for Hematology, Ministry of Health of Russia.Conclusion. Porphyria is relatively rarely diagnosed, about 12 cases per 100,000 people. The symptoms variety and nonspecificity conduce to a low detection rate, and untimely diagnoses can entail severe clinical manifestations, including lethal outcomes.

Pulmonary tularemia : a diagnosis not to be missed

Tularemia (caused by the facultative intracellular Gram-negative bacillus Francisella tularensis) is an endemic zoonotic disease in Europe, which exhibits different clinical patterns. Following the glandular form, pneumonia is the second most frequent manifestation in Switzerland. Pulmonary tularemia often has a subacute course and fails to respond to beta-lactam antibiotics. It can also mimic tuberculosis, because of the presence of systemic symptoms, such as fever, sweats and weight loss. History of animal exposure is not always reported. Clinicians should be aware of pulmonary tularemia. They should be able to diagnose it with appropriate tools (PCR, serology) and initiate appropriate therapy (fluoroquinolones, tetracyclines or aminoglycosides).

Clinical Course of COVID-19 and Cycle Threshold in Patients with Haematological Neoplasms

The SARS-CoV-2 viral load in a respiratory sample can be inversely quantified using the cycle threshold (Ct), defined as the number of amplification cycles required to detect the viral genome in a quantitative PCR assay using reverse transcriptase (RT-qPCR). It may be classified as high (Ct < 25), intermediate (25-30) and low (Ct > 30). We describe the clinical course of 3 patients with haematological neoplasms who contracted COVID-19. None of them had been vaccinated. Firstly, a 22-year-old male with a refractory acute lymphoblastic leukaemia experienced an oligosymptomatic COVID-19 and had a Ct of 23 with an ascending curve. Another male, aged 23, had recently begun treatment for a promyelocytic leukaemia. He had a subacute course with high oxygen requirements. His Ct dropped from 28, when he only experienced fever, to 14.8, during the most critical period and on the edge of ventilatory support. Viral clearance was documented 126 days after the beginning of the symptoms. Finally, a 60-year-old male had received rituximab as maintenance therapy for a follicular lymphoma 3 months before contracting COVID-19. He had a fulminant course and required mechanical ventilation a few days later. We highlight the association between the course of CoViD-19 and the Ct. Viral shedding was longer than in immunocompetent hosts.

A giant colonic submucosal lipoma: An uncommon cause of colo-colonic intussusception with review of literature

Intestinal intussusception is a rare condition in adult. The early diagnosis is challenging and has chronic or acute subacute course. They often present with nonspecific symptoms. Colo-colonic intussusception in adults is often caused by malignant lesions colonic lipomas are rare mesenchymal tumors and the majority are asymptomatic and their size is usually <2 cm. Giant submucosal lipomas in sigmoid colon are rare mesenchymal tumors and rarely cause colo-colonic intussusception. Abdominal computed tomography and colonoscopy are the choice of modalities for preoperative diagnosis. Segmental colonic resection with lipoma is the choice of therapy in elderly males with symptomatic and larger submucosal lipoma.

РЕЗУЛЬТАТИ ПАТОГІСТОЛОГІЧНОГО ДОСЛІДЖЕННЯ ОПЕРАЦІЙНОГО МАТЕРІАЛУ З ВИЗНАЧЕННЯМ СТУПЕНЮ МОРФОЛОГІЧНОЇ АКТИВНОСТІ ТУБЕРКУЛЬОЗНОГО ПРОЦЕСУ ТА КЛІНІЧНА КАРТИНА ХВОРИХ З ТБ ПІСЛЯ VATS РЕЗЕКЦІЙ ТА VATS ПЛЕВЕКТОМІЙ З ДЕКОРТИКАЦІЄЮ ЛЕГЕНІ

RESULTS OF HISTOLOGICAL EXAMINATION OF POST-OPERATIVE SPECIMENS WITH DETERMINATION OF DEGREE OF MORPHOLOGICAL ACTIVITY OF TUBERCULOSIS AND CLINICAL PRESENTATION OF TUBERCULOSIS PATIENTS AFTER VATS RESECTION AND VATS PLEUROECTOMY WITH DECORTICATION OF LUNG M. S. Opanasenko, I. V. Liskina, V. I. Lysenko, L. M. Zagaba, O. V. Tereshkovich, B. M. Konik, L. I. Levanda, S. M. Shalagai, M. I. Kalinichenko, M. U. Shamrai Abstract Patients with pulmonary tuberculomas of different histological structure, as well as pulmonary fibrous-cavernous tuberculosis (FCT) are subject to surgical treatment. These phase-forms require careful morphological examination, which provides an objective determination of the degree of activity of a specific inflammatory process. Materials and methods. 165 patients were examined: 130 (78,8 %) patients with different forms of tuberculosis and 35 (21,2 %) patients with tuberculosis of pleura. Morphological determination of the degree of activity of specific inflammation was performed according to the developed classification using hematoxylin and eosin staining. Specific lung lesions were detected in 130 (78.8 ± 3.2)% of cases, pleural TB was histologically detected in 35 (21.2%). Results and discussion. Most VATS resections were performed on tuberculosis - 98 (75.4%), 19 (14.6%) cases were operated on with FCT, 11 (8.5%) patients had post-tuberculosis changes. 2 (1.5%) patients underwent surgery for infiltrative tuberculosis. The following degree of morphological activity of tuberculous inflammation was established in the resection samples: high - 28 (21.5%), moderate - 42 (32.3%) and low - 60 (46.2%) cases. The degrees of morphological activity of the operative material and clinical symptoms were compared: low activity was found in 60 (46.2%) patients, 14 (10.8%) of them had typical symptoms of TB; measurable activity was found in 42 (32.4%), of which 21 (16.2%) patients had a clinical picture of TB; 28 (21.5%) patients had high activity, 22 (78.6%) of them showed symptoms, and in 8 (36.4%) patients of this subgroup the sputum was positive for Mycobacteria. These data do not demonstrate clear correlation, but suggest a a significant difference between groups. Chronic TB pleurisy was detected in 18 (51.4%) cases. It was characterized by the growth of coarse fibrous connective tissue with the presence of epithelioid-cell granulomas, and typical clinical and radiological symptoms. Conclusions. Determining the morphological degree of activity of specific inflammation allows to prescribe additional courses of anti-TB therapy. Low activity - 60 observations (46.2%), moderate degree - 42 cases (32.3%), high morphological degree - 28 (21.5%) cases. Different types of tuberculoma show different degrees of morphological activity. A significant difference was found between the presence of the clinical picture of TB in morphologically high and low levels of specific inflammatory activity. In tuberculosis of pleura patients with subacute or chronic course should receive surgery. Typical clinical presentation is usually observed in these patients. Key words: tuberculosis, morphological degree of activity, tuberculoma. Ukr. Pulmonol. J. 2022;30(4):28–33.

Diagnostic and Neurological Overview of Brain Tuberculomas: A Review of Literature.

Tuberculosis is a disease caused by a bacteria named Mycobacterium tuberculosis (M. tb). It is estimated by World Health Organization (WHO) that nearly a quarter of the world’s population is infected. Tuberculoma of the brain is one of the most severe extrapulmonary forms that affects patients younger than 40 years of age. Brain parenchymal tuberculoma develops in nearly one of 300 non-treated cases of pulmonary tuberculosis cases. In endemic regions, tuberculomas account for as many as 50% of all intracranial masses. Tuberculoma results in a hematogenous spread of M. tb from an extracranial source. Tuberculomas can mimic a variety of diseases and can present themselves in a subacute or chronic course, from asymptomatic to severe intracranial hypertension. Diagnosis is based on computed tomography (CT) scan and magnetic resonance imaging (MRI) studies with a similar ring-enhancing lesion. Treatment is primarily medical, and the duration for brain tuberculoma can vary from six to 36 months. In certain cases, surgery is recommended.

Budd-Chiari syndrome, review and illustration

Budd-Chiari syndrome is a rare vascular disorder characterized by obstruction of the hepatic venous outflow. Various diseases causing coagulopathy play a role in aetiology, such as myeloproliferative disorders. Acute vascular occlusion may lead to acute phlebitis with fever. The classic triad of acute liver failure may be present with ascites, hepatomegaly, and abdominal pain. However, subacute courses of disease were also observed. Because of the variable symptoms and severity extent, depending on the acuity of the course and the extent of the affected vessels, diagnosis is often difficult. Sonography, as a ubiquitously available and cost-effective diagnostic tool, plays a leading role. Doppler ultrasonography can be used to visualize hemodynamics in particular. In acute thrombotic occlusion, the affected hepatic veins usually cannot or only partially be visualized. In non-occluding thrombi, turbulent flow patterns may develop in the area of venous outflow obstruction, and flow velocity is then increased in the area of stenosis. Contrast enhanced ultrasound offers even better specificity of diagnosis. Computed tomography and magnetic resonance imaging can directly visualize thrombi and the cause of obstruction. Once the diagnosis is confirmed, anticoagulation must be initiated, but therapy of the underlying disease must also be started. If symptom-controlling measures are not sufficient, angioplasty/stenting to reopen short-segment stenoses or implantation of a TIPSS device may be considered. Liver transplantation remains ultima ratio. As studies on the precision of diagnostic methods are controversial, the characteristics of imaging for BCS are therefore summarized in this review on the basis of several illustrating case reports.

"Subacute" course of chronic radiation syndrome

Introduction. The use of ionizing radiation as a production factor in the late 1940s - early 1950s. began in the absence of a clear understanding of the permissible radiation doses for workers, as well as knowledge of diagnostic criteria and developed therapeutic measures for developing chronic radiation sickness (CRS). Since then, a great deal of experience has been accumulated in the diagnosis and treatment of CRS. Currently, there are no conditions at the workplace for chronic exposure of workers in doses exceeding the permissible ones. However, taking into account the constant expansion of the scope of using sources of ionizing radiation, it is necessary to remember about the possibility of CRS development due to prolonged exposure in case of violation of their storage or their loss. The study aimed to explore the formation of radiation bone marrow syndrome (RBS) due to chronic exposure in doses that exceed the maximum permissible, accumulated with different dose rates of radiation. Material and methods. We selected the medical records of 27 people (24 men and 3 women) who had RBS as a result of chronic professional gamma radiation exposure. The selection criteria were the diagnosis of grade II-III chronic radiation syndrome (CRS) in the presence of agranulocytosis or anemic syndrome in the period of the disease formation and, especially, in the development of myelodysplastic syndrome (MDS) or aplastic anemia in the period of the CRS consequences. Identified clinical and dosimetric CRS features of 27 patients exposed to chronic irradiation with a dose rate of 0.0002-0,009 Gy/h and the summary dose of 1.7 and 9.6 Gy, accumulated over a period of 6 to 96 months were compared the characteristics of 84 patients CRS exposed a lower dose rates (less than 0,0003 Gy/h) and 26 patients with acute radiation syndrome moderate (II) severity as a result of irradiation the dose rates of 0.14-3,7 Gy/h, total dose of 2 to 4 Gy. Results. The criteria of atypical subacute CRS course are identified: the rate of chronic radiation exposure - not less than 0.001-0.009 Gy/h with a summary dose of 1.7-9.6 Gy accumulated over a period of 6-96 months, the presence of agranulocytosis in the period of CRS formation and anemic syndrome in the periods of CRS formation and outcomes. These signs predict the development MDS in 60% of the patients in the period of the CRS consequences. Conclusion. Retrospective study determined that long-term human exposure to a dose rate of 0.001-0,009 Gy/h (0,005-0,05 Gy/day) and more in the accumulation of a summary dose of 1.7 and 9.6 Gy and duration of contact 6-96 months in 60% of cases can be expected development CRS with a subacute clinical course RBS. The main factor determining this feature of the course of RBS is the dose rate exceeding 0.001 Gy / h (2 Gy/year). In the subacute course of CRS, the early outcome in MDS is essentially deterministic. The development of agranulocytosis and anemic syndrome are typical signs of the subacute course of CRS.

AML with germline DDX41 variants is a clinicopathologically distinct entity with an indolent clinical course and favorable outcome.

Germline DDX41 variants in myeloid neoplasms (MNs) are not uncommon, and we explored the prevalence and characterized the clinical and pathologic features in a cohort of 3132 unrelated adult MN patients. By targeted next-generation sequencing, we identified 28 patients (20 men and 8 women) with pathogenic germline DDX41 variants who developed acute myeloid leukemia (AML), in which only 3 (11%) had a family history (FH) of MNs. A subacute clinical course of cytopenia (mean duration of 11.2 months, range 0-72 months) prior to the initial AML diagnosis was accompanied by a low blast count (median at 30%, range 20-70%) in hypocellular marrows (93% of all patients), in vast contrast to the typical proliferative subtypes of AML in the elderly. Most patients had a normal karyotype (75%) and acquired a second DDX41 variant (69%). A favorable overall survival (OS) was observed in comparison to that of common subtypes of AML with wild-type DDX41 in age-matched patients. Our study demonstrated that the frequent germline pathogenic DDX41 variants characterized a clinically distinct AML entity. Features characteristic of DDX41-mutated AML include male predominance, often lack of FH, indolent course, low proliferative potential, frequent somatic DDX41 variants, and a favorable OS.

Ураження суглобів при ANCA-асоційованих системних васкулітах Повідомлення І. Мікроскопічний поліангіїт

Артикулярний синдром є однією з основних ознак мікроскопічного поліангіїту (МПА), на частку якого в деяких регіонах земної кулі припадає понад 90 % усіх системних васкулітів (СВ), що пов’язані з антинейтрофільними цитоплазматичними антитілами (ANCA). Було встановлено, що за 5 років після дебюту МПА починають формуватися прогресуюче зменшення параартикулярної (епіфізарної) мінеральної щільності кістки й деструкція суглобів. Дуже багато питань, пов’язаних із характером перебігу суглобової патології при окремих ANCA-CB, вимагають подальшого вивчення. Мета: оцінити ураження суглобів у хворих на МПА та взаємозв’язки з екстраартикулярною патологією. Матеріал і методи. Під спостереженням перебували 89 хворих на МПА віком 18–78 років, серед яких було 44 % чоловіків і 56 % жінок. Тривалість хвороби від перших ознак її маніфестації становила 7 років. Гострий або підгострий перебіг МПА встановлено в 27 % випадків, у решти — хронічний; І ступінь активності патологічного процесу констатовано в 10 % спостережень, ІІ — в 32 %, ІІІ — в 58 %. Результати. Ураження суглобів спостерігається в 60 % від числа хворих на МПА, що у 40 % випадків перебігає у вигляді олігоартриту, залежить від наявності патології скелетних м’язів, пневмопатії і периферійної нейропатії. Клінічні, рентгенологічні та сонографічні ознаки артикулярної патології при МПА значно пов’язані з віком пацієнтів, тривалістю захворювання, ураженням серця, нирок і шкіри. У патогенетичних побудовах артропатії у хворих на МПА беруть участь С-реактивний протеїн (CRP) та циркулюючі імунні комплекси (CIC), а слабке значення має характер ANCA (антитіла до мієлопероксидази і протеїнази-3), при цьому певну прогнознегативну значущість щодо тяжкості суглобової патології мають високі параметри в крові ревматоїдного фактора (RF). Висновок. Прогнознегативними критеріями щодо розвитку патології суглобів у хворих на МПА є показники в крові: CRP &gt; 20 мг/л і CIC &gt; 130 ум.од., а значення RF &gt; 60 МО/мл вважаються чинниками ризику тяжкого перебігу артропатії.

ХРОНИЧЕСКАЯ ЛУЧЕВАЯ БОЛЕЗНЬ ПОДОСТРОГО ТЕЧЕНИЯ ОТ ВНЕШНЕГО НЕРАВНОМЕРНОГО ОБЛУЧЕНИЯ ПРИ КОНТАКТЕ С ПОТЕРЯННЫМ ИСТОЧНИКОМ

Purpose: To analyze the features of the clinical course of chronic radiation syndrome (CRS) due to external non-uniform chronic exposure to prolonged household contact with a lost source of ionizing radiation.&#x0D; Material and methods: Analysis of 2 clinical observations of patients who developed subacutecourse of CRS and chronic radiation dermatitis due to external non-uniform exposure of the lost sources of ionizing radiation are presented.&#x0D; Results: Boy A. K. from the age of 1 year for 7 years was external radiation exposed (for 1.5 years non-uniform exposure ) to the total dose according EPR tooth enamel about 6.3 Gy, according to the data of retrospective dose recovery on red bone marrow using voxel modeling – 26 (19–37) Gy.&#x0D; F. V. V., male, 38 years, was external non-uniform radiation exposed for 5 months, the total dose according cytogenetic studies of 7.9 Gy (dose rate about 0.035 Gy/h).&#x0D; During the examination in the hospital, the patients were diagnosed with CRS. Within the framework of the bone marrow syndrome, deep thrombocytopenia, moderate leuko- and neutropenia, and moderate anemic syndrome were observed. The latter is not typical for the typical course of CRS and is a criterion indicating a subacute course of the disease. In addition, signs of chronic radiation dermatitis were found in the projection of the action of the ionizing radiation beam. After stopping the radiation exposure, the patients did not recover their hematopoietic function, and in the period of immediate consequences, they developed myelodysplastic syndrome (MDS) with further transformation into acute leukemia.&#x0D; Conclusion: 1. Accidental prolonged household or criminal contact with a source of ionizing radiation can lead to the formation of CRS with an atypical subacute course and the formation of MDS with transformation to acute leukemia in the outcome of the disease or in the period of its consequences.&#x0D; 2. It can be assumed that with external non-uniform radiation exposure, leading to the development of CRS and chronic radiation damage to the skin, agranulocytosis in the subacute course of CRS may be absent.&#x0D; 3. Adverse prognostic signs for the development of MDS and leukemia in the outcome or in the period of the consequences of subacute CRS with non-uniform exposure are long-lasting deep thrombocytopenia and anemic syndrome after the end of radiation exposure.

Clinical Subtypes, Molecular Features and Treatment Strategies of Aggressive NK-Cell Leukemia: A Multicenter Analysis of 113 Patients

Aggressive NK-cell leukemia (ANKL) represents a rare form of NK cell neoplasm and a distinct disease entity within the continuous spectrum of EBV-T/NK-LPDs. This disease is more prevalent among people from Asia and Central and South America. The median overall survival (OS) is less than two months, irrespective of treatments. Due to the rarity of ANKL, the clinical features and underlying pathogenesis are still lack of understanding.To date, less than 350 cases of ANKL have been described in English literature worldwide. Most of these cases were sporadic case reports or studies with cohort scales no more than 40 patients. From October 2003 to July 2016, we conducted a retrospective study with 113 confirmed ANKL patients enrolled in 10 clinical centers located in different geographic regions across China. This study was approved by the institutional review board of Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology. All cases were centrally reviewed by three hematopathologists and three hematologists.All the patients were of the Han nationality living in the mainland China and had no history of CAEBV, SMBA or other EBV-T/NK-LPDs. Sudden fever, pancytopenia, hepatosplenomegaly, hypofibrinogenemia, hyperferritinemia and elevated lactate dehydrogenase (LDH) levels with dismal survival had characterized in these patients. The median age of the entire cohort was 37 years. The median overall survival was 55 days and 1-year survival rate was 4.42% (5/113). OS was estimated from the onset of disease to the date of death or the end of the study.A subacute clinical course was demonstrated in 18 patients (15.93%, 18/113). Prior to the fulminant onset, they manifested infectious mononucleosis (IM)-like symptoms for more than three months (median 4 months, range: 3-15 months). Female predominance (P = 0.007), alleviated hyperferritinemia (P < 0.001), transaminitis (ALT, P = 0.009) and hypofibrinogenemia (P = 0.038) and a marked survival advantage (P <0.001) were noted at diagnosis in these patients, irrespective of whether patients administered with allo-HSCT were excluded or not. Notably, even if the period of prodromal phases were not included in the estimation of the OS of these patients, this marked survival advantage could still be observed when patients received allo-HSCT were excluded (P = 0.042). All these data suggested that patients with prolonged prodromal phases represent a clinical subtype of ANKL which differed with the others. Therefore, we propose to define ANKL with IM-like symptoms lasting for more than 3 months as “subacute ANKL”, while the others who had acute clinical course as “classic ANKL”.To clarify the underlying pathogenesis, genes of interest were screened by Ion Torrent AmpliSeq™. The panel contained 18 candidate genes identified in our previous WGS analysis of 8 ANKL patients, including transcriptional factors (TP53 and MYC), JAK-STAT pathway genes (JAK2, JAK3, STAT3, STAT5A, STAT5B and STAT6), other signaling pathway genes (PIK3CB, NFKB1, NFKBIA, MAP3K13, MAPK10, NRAS and FGFR1) and epigenetic regulators (PRDM9, CREBBP and TET2). Our results showed TP53 gene had a significant lower mutation rate in subacute ANKL than that in classic ANKL (P =0.038). Except for that, the other gene mutation patterns including genes in JAK-STAT pathway, were similar between the two groups. Especially, TP53 mutations were not found in patients of subacute ANKL subtype, while enriched in 13 classic ANKL patients (44.83%, 13/29). In addition, most of the detected TP53 missense mutations (61.54%, 8/13) were located in the DNA binding domain.Survival analysis revealed L-asparaginase, methotrexate and dexamethasone (AspaMetDex) was an effective induction regimen with no therapy-related mortality. The complete remission rate and overall response rate in newly diagnosed patients was 30.77% (4/13) and 76.92% (10/13), respectively. Allo-HSCT was the only curative treatment with 2-year survival rate of 43.86% (3/7). Multivariate analysis showed serum LDH level, clinical subtype and administration of L-asparagine based chemotherapy or allo-HSCT were factors independently predictive for survival.In conclusion, these data depicted the clinical characteristics and molecular features of ANKL. It also provided insights into the treatments and outcomes of this deadly type of leukemia. Future prospective studies or clinical trials hold great promise for ANKL. [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Epizootological features and clinical manifestation of cat chlamydiosis in Poltava

The article presents epizootological data and clinical signs of feline chlamydia in Poltava. According to the results of studies in 2018–2020, a wide spread of chlamydia infection among cats in the city of Poltava was established. The largest number of the studied cats, were pedigree or crossbred. There was no explicit clear seasonality, but a greater number of cases of respiratory diseases were recorded in the autumn-winter period of the year. In the structure of infectious diseases during the research period, chlamydia mono-infection was registered in 68 % of cats, association of chlamydia with infectious rhinotracheitis in 18.5 %, kalitsivirus infection in 8.6 %, with panleukopenia in 4.9 %. The greatest number of the registered cats had acute and subacute course of chlamydia. In the acute course, there was an increase in temperature, oppression and refusal to eat. In the majority of cases of acute, subacute and associated chlamydia, the structures of the eye were damaged. Conjunctivitis, keratitis, chemosis, uveitis, third eyelid inflammation, changes in the shape of the pupil developed. A Sphynx breed cat showed signs of acute chlamydial arthritis with pronounced inflammatory edema. The latent course was accompanied by abortions, which were mainly registered in young cats. Animals older than four years more often showed stillborn and weak kittens. The main clinical signs of chlamydia in kittens born from sick cats were eye lesions: swelling of the conjunctiva, its reddening and secretion of a significant amount of brown purulent exudate with the viscosity of the catarrh and as a consequence of eyelid adhesion and complications as ankyloblepharon or symblepharon. Submandibular and other lymph nodes in the head area were significantly enlarged. Often pus dried up near the mouth or nostrils. Pus crusts accumulated in the nasal cavity and prevented breathing. Gastrointestinal tract disorders were observed in some cases. Part of the affected litter died within the first three days after birth. Neonatal chlamydia was more often manifested by conjunctivitis, keratitis, panophthalmitis and growth retardation. On the second or third day after birth, the kittens showed weakness and lack of appetite. In two cases of neonatal chlamydia, bronchopneumonia developed. When chlamydia and infectious rhinotracheitis were associated, the structures of the eye were affected in all cases. These lesions could be different and depended on the stage of the infectious process.

Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 4. Cryptogenic Organizing Pneumonia.

Cryptogenic organizing pneumonia (COP) is a type of idiopathic interstitial pneumonia with an acute or subacute clinical course. Bilateral lung consolidations located in the subpleural area and bronchovascular bundle are the most common findings on chest high-resolution computed tomography. The pathologic manifestations include granulation tissue in the alveoli, alveolar ducts, and bronchioles. COP responds fairly well to glucocorticoid monotherapy with rapid clinical improvement, but recurrence is common. However, treatment with combined immunosuppressant agents is not recommended, even if the COP patient does not respond to glucocorticoid monotherapy with expert opinion.

The State of Systemic and Local Immunity with Non ? Specific Vulvovaginitis in Adolescents

The study showed that there is a secondary immune deficiency in the subacute course, and in acute immune deficiency it was not detected. In acute course compared with subacute one, there is an increased production of IFN-γ, which indicates the direct dependence of the pro-inflammatory cytokines activity level on the clinical course of the inflammatory process. A study of vaginal secretion phagocytosis revealed that in acute cases, the phagocytic function of the local secretion is satisfactory, while in the subacute case, satisfactory absorption function decreases the bactericidal activity of the vaginal secretion, which indicates the need for correction of the phagocytic link. The use of Roncoleukin parenterally in acute course does not affect normal immune status indicators, i.e. has a modulating effect depending on the initial immune status. In subacute case, local application leads to an increase in the absorption and oxygen-dependent bactericidal ability of the vaginal secretion, which contributes to the rapid regression of the clinical manifestations of vulvovaginitis, parenterally leading to normalization of the main indicators of the subpopulation composition of lymphocytes.