Secondary Disorders Research Articles

COMMUNICATION SKILLS EXHIBITED BY A CHILD WITH ASPERGER SYNDROME

Autistic children experience verbal and nonverbal communication disorders categorized as primary language disorders and secondary language disorders. The disorder can affect all levels of language and persist into adulthood. In addition, the disorder can have a wide impact on the life of sufferers. On the other hand, the number of people with autism in Indonesia is increasing every year. This study aimed to examine an Asperger Syndrome child’s communication skills using Scovel’s theory which includes four stages, namely conceptualization, formulation, articulation, and self-monitoring. The research design used in this study is qualitative descriptive with the Single Subject Research (SSR) method. Data were gathered from an Asperger Syndrome child registered at SLB 1 in Makassar, through observation, interviews, and documentation. The research instruments were composed of the researcher as the primary instrument, and supporting instruments, which comprised an instrument to assess autism ability, observation guides, interview guides, and recording devices. The autism ability instrument is guided by the Diagnostic and Statistical Manual of Mental Disorders. Data analysis techniques are data transcription, data reduction, data presentation, and conclusion withdrawal. The findings revealed that the child’s level 2-conceptualization ability (requires great support) was influenced by his level of focus, frequency of learning topics, and expression of desire. The child’s formulation ability was at level 2 (needs considerable assistance) and was influenced by his attentiveness and comprehension of a topic. The articulation ability exhibited by the child was at level 1 (needs support) with several articulation disorders, including substitution (sound changes), omission, addition, and distortion (clutter). Due to the child’s inability to recognize speech errors, his self-monitoring ability was classified into level 3 (requires enormous support). Variations in the level of ability shown by the child are a manifestation of deficits in Asperger’s Syndrome.

Do Endocrinopathies Differ in Most Prevalent Hemoglobinopathy of Middle East: Beta-thalassemia?

Repeated blood transfusions in thalassemia patients is followed by endocrinopathies as diabetes, hypothyroidism, hypogonadism, hypoparathyroidism, and disorders in calcium and vitamin D homeostasis. The aimof this study was to evaluate the association of beta-thalassemia patientsendocrinopathies and osteoporosis. Serum level of some factors related tothe function of gonads, thyroid, adrenal, and pancreas along with serumlevels of calcium, phosphate, albumin, vitamin D, and iron were measured.Bone marrow density was tested via dual-energy x-ray absorptiometry(DXA densitometry). In this study, 56 patients with major thalassemia wereinvestigated. Paraclinical analysis indicated osteopenia in 17 (30.4%) andosteoporosis in 39 patients (69.6%) in addition to other types of endocrinedisorders, such as hypogonadism in 29 (51.8%), hypothyroidism in 13(23.2%), hypoparathyroidism in 1 (1.8%), hypocortisolism in 2 (3.6%), anddiabetes in 9 (16.1%) patients. Endocrinopathies had no significant relationship with osteoporosis and osteopenia in men. However, hypogonadismhad a significant relationship with osteoporosis and osteopenia in womenwith thalassemia. Estradiol level was lower in women with osteoporosisin comparison with women with osteopenia. Ferritin levels had neitherassociation with osteoporosis nor with LH levels (P>0.05). Secondary hypogonadism disorders are the main causes of osteoporosis and osteopeniain female beta-thalassemia patients.

British Society for Immunology and United Kingdom Primary Immunodeficiency Network (UKPIN) consensus guideline for the management of immunoglobulin replacement therapy.

SummaryCurrently, there is no guideline to support the use of immunoglobulin replacement therapy (IgRT) in primary and secondary immunodeficiency disorders in UK. The UK Primary Immunodeficiency Network (UK-PIN) and the British Society of Immunology (BSI) joined forces to address this need. Given the paucity of evidence, a modified Delphi approach was used covering statements for the initiation, monitoring, discontinuation of IgRT as well as home therapy programme. A group of six consultant immunologists and three nurse specialists created the statements, reviewed responses and feedback and agreed on final recommendations. This guideline includes 22 statements for initiation, 22 statements for monitoring, 11 statement for home therapy, and 19 statements for discontinuation of IgRT. Further areas of research are proposed to improve future delivery of care.

Posttraumatic Headache.

Posttraumatic headache (PTH) is the most common secondary headache disorder, accounting for approximately 4% of all headache disorders. It is the most common symptom following concussion (mild traumatic brain injury) and can be debilitating for many who have persistent symptoms. With a recent increase in public awareness regarding traumatic brain injury, there has been a corresponding increase in PTH research. The pathophysiology of PTH remains poorly understood and the underlying mechanisms are likely multifactorial. Diagnosis of PTH is dependent on a temporal relationship to a head injury. PTH often resembles common primary headache phenotypes. Treatment of PTH utilizes known treatments for these other headache phenotypes, as there is no currently approved treatment specifically for PTH. Moving forward, further studies are needed to better define and validate the definition of PTH, understand the underlying pathophysiology, and find more specific treatments.

Movement Disorders Associated With Cerebral Artery Stenosis: A Nationwide Study.

BackgroundStudies of secondary movement disorder (MD) caused by cerebrovascular diseases have primarily focused on post-stroke MD. However, MD can also result from cerebral artery stenosis (CAS) without clinical manifestations of stroke. In this study, we aimed to investigate the clinical characteristics of MD associated with CAS.Materials and MethodsA nationwide multicenter retrospective analysis was performed based on the data from patients with CAS-associated MDs from 16 MD specialized clinics in South Korea, available between January 1999 and September 2019. CAS was defined as the >50% luminal stenosis of the major cerebral arteries. The association between MD and CAS was determined by MD specialists using pre-defined clinical criteria. The collected clinical information included baseline demographics, features of MD, characteristics of CAS, treatment, and MD outcomes. Statistical analyses were performed to identify factors associated with the MD outcomes.ResultsThe data from a total of 81 patients with CAS-associated MD were analyzed. The mean age of MD onset was 60.5 ± 19.7 years. Chorea was the most common MD (57%), followed by tremor/limb-shaking, myoclonus, and dystonia. Atherosclerosis was the most common etiology of CAS (78%), with the remaining cases attributed to moyamoya disease (MMD). Relative to patients with atherosclerosis, those with MMD developed MD at a younger age (p < 0.001) and had a more chronic mode of onset (p = 0.001) and less acute ischemic lesion (p = 0.021). Eight patients who underwent surgical treatment for CAS showed positive outcomes. Patients with acute MD onset had a better outcome than those with subacute-to-chronic MD onset (p = 0.008).ConclusionsThis study highlights the spectrum of CAS-associated with MD across the country. A progressive, age-dependent functional neuronal modulation in the basal ganglia due to CAS may underlie this condition.

Heterologous expression of naturally evolved putative de novo proteins with chaperones.

Over the past decade, evidence has accumulated that new protein‐coding genes can emerge de novo from previously non‐coding DNA. Most studies have focused on large scale computational predictions of de novo protein‐coding genes across a wide range of organisms. In contrast, experimental data concerning the folding and function of de novo proteins are scarce. This might be due to difficulties in handling de novo proteins in vitro, as most are short and predicted to be disordered. Here, we propose a guideline for the effective expression of eukaryotic de novo proteins in Escherichia coli. We used 11 sequences from Drosophila melanogaster and 10 from Homo sapiens, that are predicted de novo proteins from former studies, for heterologous expression. The candidate de novo proteins have varying secondary structure and disorder content. Using multiple combinations of purification tags, E. coli expression strains, and chaperone systems, we were able to increase the number of solubly expressed putative de novo proteins from 30% to 62%. Our findings indicate that the best combination for expressing putative de novo proteins in E. coli is a GST‐tag with T7 Express cells and co‐expressed chaperones. We found that, overall, proteins with higher predicted disorder were easier to express.StatementToday, we know that proteins do not only evolve by duplication and divergence of existing proteins but also arise from previously non‐coding DNA. These proteins are called de novo proteins. Their properties are still poorly understood and their experimental analysis faces major obstacles. Here, we aim to present a starting point for soluble expression of de novo proteins with the help of chaperones and thereby enable further characterization.

Pemantauan Oksigen Otak Dengan Pengukuran Saturasi Oksigen Vena Jugularis (SjvO2) Sebelum Ekstubasi Pada Pasien Cedera Otak Traumatik

In the group of serious injuries due to traffic accidents, head injury ranks highest, and the discussion of injuries in Indonesia and America is extreme. head injury can cause shock or shock to the brain due to an accident or assault that causes impaired brain function and results in death or death. The main monitoring in the neurointensive care of head injury is the measurement of intracranial pressure (ICP) to combine cerebral perfusion pressure (CPP) and cerebral blood flow (CBF). Moderate to severe brain injury (TBI) will require treatment in an intensive care unit (ICU) with continued monitoring. The primary goal of monitoring is to detect and treat cerebral hypoperfusion for secondary disorders. SjvO2 is a method of monitoring brain perfusion by measuring cerebral venous saturation in the jugular bulb. Monitoring brain perfusion is a guide in managing head injuries, including ventilator weaning and extubation.

Fracture prediction tools in diabetes.

Type 1 (T1D) and 2 diabetes (T2D) are associated with increased risk of fracture independent of bone mineral density (BMD). Fracture risk prediction tools can identify individuals at highest risk, and therefore, most likely to benefit from antifracture therapy. This review summarizes recent advances in fracture prediction tools as applied to individuals with diabetes. The Fracture Risk Assessment (FRAX) tool, Garvan Fracture Risk Calculator (FRC), and QFracture tool are validated tools for fracture risk prediction. FRAX is most widely used internationally, and considers T1D (but not T2D) under secondary osteoporosis disorders. FRAX underestimates fracture risk in both T1D and T2D. Trabecular bone score and other adjustments for T2D-associated risk improve FRAX-based estimations. Similar adjustments for T1D are not identified. Garvan FRC does not incorporate diabetes as an input but does includes falls. Garvan FRC slightly underestimates osteoporotic fracture risk in women with diabetes. QFracture incorporates both T1D and T2D and falls as input variables, but has not been directly validated in individuals with diabetes. Further research is needed to validate and compare available fracture prediction tools and their performance in individuals with diabetes.

Psychological distress in breast cancer patients during oncological inpatient rehabilitation: incidence, triggering factors and correlation with treatment-induced side effects.

As a result of the cancer diagnosis and the mostly multimodal, stressful therapy, psychological distress is a common symptom in breast cancer patients. As part of this prospective study, 1400 patients who were admitted to the hospital for oncological rehabilitation were screened for distress and somatic, therapy-induced secondary disorders. Mean distress in the screened population was 5.6 ± 2.56 (range 0-10). In 942 cases (67.3%) a significant distress score (≥ 5) was detected and in 587 cases (41.9%) very high levels of distress (≥ 7) was observed. Psychological distress significantly correlated with younger age, presence of triple negative breast cancer (TNBC), tumor recurrence and metastasis, and inability to work. Treatment-induced side effects such as lymphedema, CIPN or extensive tumor resection (mastectomy) showed a (not significant) trend for higher distress. The high incidence of distress underscores the importance of psycho-oncological therapy offers and the great importance of occupational measures during oncological rehabilitation such as the importance of a successful return to work for a better quality of life in breast cancer patients.

Ocular pathology in patients referred to ophthalmology outpatient clinic at tertiary care hospital with headache

Introduction: Headache has multi-factorial causes; many primary or secondary headache disorders have ocular manifestations and benefit from the systematic ocular examination. Physicians at the point of the first contact should therefore be familiar with common ocular pathologies associated with a headache for appropriate referral.&#x0D; Method: This hospital-based descriptive cross-sectional study was conducted in the Ophthalmology Department of Patan Hospital. All patients with headache as primary symptom and were referred to an ophthalmology clinic for ocular evaluation by other departments were included. The study was done for 15 mo and 354 patients were evaluated for the cause of headache. Findings and diagnoses of consenting participants were recorded and analyzed.&#x0D; Result: All enrolled patients completed all the study procedures and were analyzed. The majority 61% were in 14-40 years. Homemaker was commonly affected 30.2% followed by students 25.4%. Any form of ocular pathology was detected in 76 % of patients. The majority of patients had no refractive errors (67.8%). Although any form of refractive error was more common in the presbyopia age group there was no statistically significant difference between different age groups. Most patients were orthophoric and only 11% of patients had vergence insufficiency. Among the ocular pathology dry eye, glaucoma suspects were more common.&#x0D; Conclusion: This study supports the ocular morbidities and headache symptoms are linked very frequently. Good ocular history is needed for appropriate patient referral to a specialist. Correction of a minor degree of refractive error and lubricating eye drop may relieve worrisome headaches for many patients.

Alemtuzumab-induced immune phenotype and repertoire changes: implications for secondary autoimmunity.

Alemtuzumab is a monoclonal antibody that causes rapid depletion of CD52-expressing immune cells. It has proven to be highly efficacious in active relapsing–remitting multiple sclerosis; however, the high risk of secondary autoimmune disorders has greatly complicated its use. Thus, deeper insight into the pathophysiology of secondary autoimmunity and potential biomarkers is urgently needed. The most critical time points in the decision-making process for alemtuzumab therapy are before or at Month 12, where the ability to identify secondary autoimmunity risk would be instrumental. Therefore, we investigated components of blood and CSF of up to 106 multiple sclerosis patients before and after alemtuzumab treatment focusing on those critical time points.Consistent with previous reports, deep flow cytometric immune-cell profiling (n = 30) demonstrated major effects on adaptive rather than innate immunity, which favoured regulatory immune cell subsets within the repopulation. The longitudinally studied CSF compartment (n = 18) mainly mirrored the immunological effects observed in the periphery. Alemtuzumab-induced changes including increased numbers of naïve CD4+ T cells and B cells as well as a clonal renewal of CD4+ T- and B-cell repertoires were partly reminiscent of haematopoietic stem cell transplantation; in contrast, thymopoiesis was reduced and clonal renewal of T-cell repertoires after alemtuzumab was incomplete. Stratification for secondary autoimmunity did not show clear immununological cellular or proteomic traits or signatures associated with secondary autoimmunity. However, a restricted T-cell repertoire with hyperexpanded T-cell clones at baseline, which persisted and demonstrated further expansion at Month 12 by homeostatic proliferation, identified patients developing secondary autoimmune disorders (n = 7 without secondary autoimmunity versus n = 5 with secondary autoimmunity). Those processes were followed by an expansion of memory B-cell clones irrespective of persistence, which we detected shortly after the diagnosis of secondary autoimmune disease.In conclusion, our data demonstrate that (i) peripheral immunological alterations following alemtuzumab are mirrored by longitudinal changes in the CSF; (ii) incomplete T-cell repertoire renewal and reduced thymopoiesis contribute to a proautoimmune state after alemtuzumab; (iii) proteomics and surface immunological phenotyping do not identify patients at risk for secondary autoimmune disorders; (iv) homeostatic proliferation with disparate dynamics of clonal T- and B-cell expansions are associated with secondary autoimmunity; and (v) hyperexpanded T-cell clones at baseline and Month 12 may be used as a biomarker for the risk of alemtuzumab-induced autoimmunity.

FANCC deficiency mediates microglial pyroptosis and secondary neuronal apoptosis in spinal cord contusion

BackgroundTraumatic spinal cord injury (SCI)-induced neuroinflammation results in secondary neurological destruction and functional disorder. Previous findings showed that microglial pyroptosis plays a crucial role in neuroinflammation. Thus, it is necessary to conduct a comprehensive investigation of the mechanisms associated with post-SCI microglial pyroptosis. The Fanconi Anemia Group C complementation group gene (FANCC) has been previously reported to have an anti-inflammation effect; however, whether it can regulate microglial pyroptosis remains unknown. Therefore, we probed the mechanism associated with FANCC-mediated microglial pyroptosis and neuroinflammation in vitro and in vivo in SCI mice.MethodsMicroglial pyroptosis was assessed by western blotting (WB) and immunofluorescence (IF), whereas microglial-induced neuroinflammation was evaluated by WB, Enzyme-linked immunosorbent assays and IF. Besides, flow cytometry, TdT-mediated dUTP Nick-End Labeling staining and WB were employed to examine the level of neuronal apoptosis. Morphological changes in neurons were assessed by hematoxylin–eosin and Luxol Fast Blue staining. Finally, locomotor function rehabilitation was analyzed using the Basso Mouse Scale and Louisville Swim Scale.ResultsOverexpression of FANCC suppressed microglial pyroptosis via inhibiting p38/NLRP3 expression, which in turn reduced neuronal apoptosis. By contrast, knockdown of FANCC increased the degree of neuronal apoptosis by aggravating microglial pyroptosis. Besides, increased glial scar formation, severe myelin sheath destruction and poor axon outgrowth were observed in the mice transfected with short hairpin RNA of FANCC post SCI, which caused reduced locomotor function recovery.ConclusionsTaken together, a previously unknown role of FANCC was identified in SCI, where its deficiency led to microglia pyroptosis, neuronal apoptosis and neurological damage. Mechanistically, FANCC mediated microglia pyroptosis and the inflammatory response via regulating the p38/NLRP3 pathway.

Clinical features of hyperprolactinemia in children: modern methods of diagnosis and treatment

Hyperprolactinemia is a common endocrine disease that can be associated with significant morbidity and disability in the population. A systematic review of literature sources on the diagnosis and treatment results in patients with hyperprolactinemia, including drug-resistant micro- and macroadenomas of the pituitary gland, was carried out in order to highlight the most significant practical recommendations. The purpose of this literature review is to provide algorithms for differential diagnosis and clinical observation of patients, to compare the efficacy and side effects of drugs, surgeries, and radiation therapy in the treatment of hyperprolactinemia. Prolactinomas are the most common pituitary adenomas in older children. Their share in the structure of pituitary adenomas is about 50 %. The clinical picture depends on the age, sex of the child, volume and characteristics of tumor growth. The leading clinical manifestations of hyperprolactinemia are disorders of the reproductive system, including signs of the development of secondary hypogonadotropic hypogonadism, sexual dysfunction, psychoemotional disorders, and endocrine and metabolic disorders. The main criterion for the diagnosis of hyperprolactinemia is the concentration of prolactin in the blood serum, which is above the upper limit of the physiological norm. The primary step in the treatment of patients with pituitary macroadenoma is to control the compression effects of the tumor, including compression of the optic chiasm, followed by restoration of gonadal function. The main areas of treatment for hyperprolactinemia are restoration and maintenance of the normal function of the gonads; restoration of fertility; prevention of osteoporosis. Dopamine receptor agonists are the gold standard in the treatment of hyperprolactinemia. However, some people do not respond satisfactorily to these drugs. In this case, in the presence of pituitary tumors, in addition to drug treatment, surgery and radiation therapy are used. But surgery and radiation therapy are not the primary methods of choice for the management of patients with prolactin-secreting pituitary adenomas; first of all, preference is given to drug therapy. The prognosis of the course of prolactinomas is favorable and is based on the results of dynamic observation and the presence of markers of the aggressiveness of this adenoma.

Compensation Possibilities of Mental Disorders — Individual Case of a Child with Severe Neurological Disorder

IntroductionNeurological diseases often lead to mental disorders in children. After the structure of disorder is identified, an important task is to examine the abilities to compensate developmental delay (Lev S. Vygotsky).ObjectivesThe goal of the study was to explore potential of children with opsoclonus-myoclonus syndrome (OMS), which is important for psychocorrection.MethodsCase study of a boy with idiopathic OMS (aged 3 years 5 months) who was patient at the Psychoneurological Department no. 2 of the Russian Children Clinical Hospital. The following methods were used: analysis of anamnestic data, analysis of patient’s medical record, pathopsychological assessment.ResultsAnamnestic data and medical records indicate the absence of pregnancy pathologies and normal early development. OMS was first detected when the child was aged 2 y. 7 m. Pathopsychological assessment gave the following results: 1) movement disorder (primary disorder) leads to secondary mental disorders; 2) locomotor activity disorders inhibit the child’s use of space and orientation of body in it; 3) secondary defects are detected in constructive activity, speech and drawing. Intact components of the mental processes: 1) the child demonstrates motivation for independent activity despite operational difficulties; 2) in certain activities, the general plan of actions remains intact, i.e., the goal set is actualized in movements and actions; 3) notions about objects, actions with them, planning and performance of movements are intact.ConclusionsThe research demonstrates disbalance between operational difficulties and integrity of semantic orientation, internal planning of actions. The data prove the importance of discussion on abilities of children with OMS to compensate mental disorders.DisclosureNo significant relationships.

Investigation of alpha-synuclein in patients with late-onset schizophrenia

IntroductionThere is no consensus about whether late-onset schizophrenia(LOS) is a type of schizophrenia or a secondary psychotic disorder. One of the theories of the occurrence of late-onset psychoses is neurodegeneration caused by the imbalance of proteostasis.ObjectivesTo study the concentration and expression of alpha-synuclein in patients with LOS compared with controls.MethodsThe study involved 42 patients with the ICD-10 criteria of schizophrenia with the onset of the disease after 45 years and 104 controls with no dementia and severe somatic pathology, comparable in age and gender. The alpha-synuclein level was estimated in a lymphocytic cell fraction from patients with LOS N=42 and controls N=104 using the Human alpha-synuclein ELISA kit. The expression of the SNCA gene was studied in 22 LOS patients and 22 controls and determined by PCR using the SYBR Green Supermix kit and the CFX96 instrument in comparison with the referent genes using previously developed primers. LOS group underwent psychopathological examination including scales. Statistics: SPSS 12.0; the Mann–Whitney test; exponential regression analysis; data is given as median (min–max). The level of significance was set at p&lt;0.05.ResultsThe alpha-synuclein level is higher in patients with LOS (9.21 (0.78-29.52)) compared to control (6,355 (0.46-35.44)), p=0.024. The mRNA level of the SNCA gene is higher in the LOS group (0.533 (0.089-1.406)) compared to controls (0.087 (0.016-0.266) , p&lt;0.001.ConclusionsOur study shows the relationship of alpha-synuclein with the manifestations of LOS. Obtained novel data can open up new targets for therapy and bring it closer to understanding the phenomenon.DisclosureNo significant relationships.

Structural Equation Modelling of Primary and Secondary Vulnerability of Psychopathic Disorder based on Behavioral Brain Systems in Medical Students

Background. The level of activity of behavioral brain systems is one of the effective factors in the occurrence or persistence psychopathic behaviors. Therefore, the aim of this study was to structurally model primary and secondary psychopathic disorder based on behavioral brain systems. Methods. This research is applied in terms of purpose and descriptive in terms of correlational research method. The statistical population of this study includes all medical students of Tabriz University of Medical Sciences. A total of 284 subjects were selected as a sample by convenience sampling method and they performed Lonson, Kiel and Fetispatrick Psychopathy Scale and the Carver and White Brain-Behavioral Systems Scale. Data were analyzed using Pearson correlation coefficient and structural equation modelling with SPSS software version 24 and LISREL version 8.80. Results. The results showed that the behavioral activation system has a positive and significant relationship with primary and secondary psychopathy and the behavioral inhibition system has a negative and significant relationship with primary and secondary psychopathy (P &lt;0.01). The results of structural equation model showed that behavioral activator system and behavioral inhibition system have an effective role on primary and secondary psychopathy (P &lt;0.01). Also, the results of structural equation model showed acceptable model (2/df= 2.50, RMSEA= 0.075, CFI= 0.96, NFI= 0.94, GFI= 0.94). Conclusion. According to the function of activating systems and behavioral inhibition, pathological personality traits can be oriented or corrected before the formation and stabilization of these traits.

Severe COVID-19 is a T cell immune dysregulatory disorder triggered by SARS-CoV-2

ABSTRACT Introduction COVID-19 has had a calamitous impact on the global community. Apart from at least 6 M deaths, hundreds of millions have been infected and a much greater number have been plunged into poverty. Vaccines have been effective but financial and logistical challenges have hampered their rapid global deployment. Vaccine disparities have allowed the emergence of new SARS-CoV-2 variants including delta and omicron, perpetuating the pandemic. Areas covered The immunological response to SARS-CoV-2 is now better understood. Many of the clinical manifestations of severe disease are a consequence of immune dysregulation triggered by the virus. This may explain the lack of efficacy of antiviral treatments, such as convalescent plasma infusions, given later in the disease. Expert opinion T cells play a crucial role in both the outcome of COVID-19 as well as the protective response to vaccines. Vaccines do not prevent infection but reduce the risk of a chaotic and destructive cellular immune response to the virus. Severe COVID-19 should be considered a virus-induced secondary immune dysregulatory disorder of cellular immunity, with broad host susceptibility. This perspective of COVID-19 will lead to better diagnostic tests, vaccines, and therapeutic strategies in the future.

A Review Article on Vishopadrava Chikitsa with special reference to Brihathrayee

Ayurveda is oldest medical science known to mankind and mainly aims at healthy living and long life unlike other medical science which simply focus on the treatment of diseases and secondary disorders. In a Visa (poisoning) condition not diagnosed properly or no treated in properly in their earlier stages it will leads manifestation of Visopadrava (secondary disorders). Visopadrava plays very important role in diagnosis, prognosis and treatment of the diseases. Hence in the present article effort is made to understand the concept of Visopadrava with betterment approach proper treatment and Pathya-Apathya.

Pediatric hepatobiliary disease: Genetic and clinical manifestations

In children, hepatobiliary diseases are rarely and mainly due to congenital defects during formation, development of the liver and biliary tract or due to disorders of metabolism. The liver and biliary tract of infant have the incomplete development of physiology during the perinatal until the childhood period. In the process of complete development of the child's liver and biliary tract, there are important changes and affected by genetic and environmental factors. Therefore, the liver and biliary tract are very vulnerable leading to hepatobiliary diseases in children. Disorders in the formation of bile ducts, bile secretion, hepatocellular metabolism, disturbances of metabolism all lead to the formation associated with hepatobiliary diseases. Based on pathophysiology, the hepatobiliary diseases in children can be divided into two groups: hepatobiliary diseases due to the incomplete development of the structure and function of the liver and biliary tract, and hepatobiliary diseases due to the disruption of metabolic processes in liver cells. The secondary effects of hepatobiliary disease can threaten a child's life, and is the cause of metabolic disorders such as hypoglycemia, secondary coagulation disorder due to low concentration of vitamin K-dependent factors leading to intracranial hemorrhage in children, infections caused by immunodeficiency, malnutrition, increased portal venous pressure leading to severe gastrointestinal bleeding... Therefore, the hepatobiliary disease in children should be detected and treated early to avoid adverse complications. In the context of this paper, we focus on hepatobiliary diseases with genetic causes in children. Genetic factors and research situation in the World and Vietnam will be also mentioned. The information about genetic and clinical manifestations will be aggregated to contribute to the general understanding of hepatobiliary diseases in children and to orientate the accurate and effective treatment for patients.

Preprint Highlight: Kinesin-binding protein remodels the kinesin motor to prevent microtubule binding

Kinesin-binding protein (KIFBP) interacts with a subset of 8 of the 45 human kinesin family members and prevents their binding to microtubules, yet it was unclear how KIFBP selectively binds to this subset. By combining cryoelectron microscopy and cross-linking mass spectrometry, it was revealed that KIFBP uses a multivalent interaction to bind to at least two different kinesins, Kif18a and Kif15. This confirms a recent study of Kif15, extends the structural analysis to Kif18a, and uses molecular dynamics simulations to suggest KIFBP selectivity is determined by whether a kinesin loop prefers secondary structure or disorder. This work will be important to researchers studying molecular motors and structural biologists because it reveals uncommon mechanisms for molecular interaction.