PRIMARY IMMUNODEFICIENCY IN A CHILD WITH HETEROTAXY

2021 ◽  
Author(s):  
Tatyana Pavlova
Keyword(s):  
Primary Immunodeficiency ◽  
Primary Care Physicians ◽  
Clinical Observation ◽  
Mirror Reflection ◽  
Internal Organs ◽  
Embryonic Tissues ◽  
Ivemark Syndrome ◽  
Abdominal Organs ◽  
Sporadic Cases ◽  
Symptom Complex

Heterotaxy is a complicated symptom complex in which the location of the main internal organs differs from their normal and complete mirror reflection. Ivemark syndrome - a combination of spleen agenesis with congenital heart disease and abnormalities in the location of the abdominal organs. The exact reason is unknown. Mostly sporadic cases. Genetic and infectious-toxic factors that damage the differentiation of embryonic tissues between the 31st and 36th days of embryogenesis are discussed. The article presents a clinical observation of a patient with Ivemark syndrome, illustrating the difficulties of diagnosing congenital malformations. The peculiarity of this clinical case was determined by the combination of the syndrome with primary immunodeficiency and vascular malformation. Ivemark syndrome is a rare disease, therefore, the awareness of primary care physicians in relation to this pathology is low. Early diagnosis of primary immunodeficiency before a child is at risk of infection is extremely important. There is demonstrated an example of pharmacological correction, which allows prolonging the patient's life.

PECULIARITIES OF THE RADIATION PATTERN OF THE BRAIN AND INTERNAL ORGANS OF NEWBORNS WHO UNDERWENT SEVERE PERINATAL ASPHYXIA AND UNDERWENT GENERAL THERAPEUTIC HYPOTHERMIA, ACCORDING TO THE PILOT STUDY RESULTS

2021 ◽  
Vol 100 (1) ◽  
pp. 30-35
Author(s):  
N.S. Vorotyntseva ◽  
◽  
V.V. Orlova ◽  
A.N. Motina ◽  
A.D. Novikova ◽  
...  
Keyword(s):  
Therapeutic Hypothermia ◽  
Perinatal Asphyxia ◽  
Control Group ◽  
Internal Organs ◽  
X Ray ◽  
Abdominal Organs ◽  
Study Results ◽  
Negative Effect ◽  
Chest X Ray ◽  
The Brain

Objective of the research: to study the features of the radiological picture of the brain and internal organs of newborns with severe perinatal asphyxia, who underwent general therapeutic hypothermia (GTH). Materials and methods: the study included 116 newborns with severe perinatal asphyxia. GTH was performed in 72 patients (group 1), 44 children did not receive hypothermia in the 2 (control) group. In the first 6 hours of life, children of groups 1 and 2 underwent ultrasound of the brain and abdominal organs and chest x-ray. The complex ultrasound scan was repeated at 3–5, 7–10, 14–16 and 21–28 days. Repeated chest x-ray was carried out strictly according to the indications. Results: GTH reduced the incidence of organic brain lesions by 18% in children with severe perinatal asphyxia (p0,05). The study revealed a number of undesirable consequences associated with GTH. Transient effusion into the abdominal and thoracic cavities was diagnosed by ultrasound in 8 (11%) patients after hypothermia, while there were no such changes in children in the control group (p<0,05). X-ray of the chest in newborns who received GTH during the first 14 days revealed edematous-hemorrhagic changes in the lungs more often than in patients of the control group – 55 (76%) and 24 (55%), respectively (p<0,05). Conclusions: GTH effectively prevented the development of severe post-hypoxic changes in the brain. The negative effect of hypothermia on microcirculation was manifested by the development of effusion into the serous cavities and edematous-hemorrhagic syndrome.

scholarly journals Ivemark syndrome with obstructed supracardiac TAPVC- case report

2021 ◽  
Vol 8 (2) ◽  
pp. 389
Author(s):  
Sravani Vemuri ◽  
Chaitanya Challa ◽  
Vala Ramgopal Rao ◽  
Sudharshan Raj C.
Keyword(s):  
Case Report ◽  
Cardiac Disease ◽  
Neonatal Period ◽  
Rare Disorder ◽  
Ivemark Syndrome ◽  
Abdominal Organs ◽  
Congenital Cardiac Disease ◽  
Left And Right ◽  
Thoracic And Abdominal ◽  
Anomalous Pulmonary Venous Connection

Heterotaxy disorder is a disturbance in the usual left and right distribution of the thoracic and abdominal organs. Ivemark syndrome is one such heterotaxy disorder. It is a rare disorder which affects males more than females with majority of cases presenting in the neonatal period mainly due to complex congenital cardiac disease. Here is a case report of rare disorder of the neonate with Ivemark syndrome with obstructed supracardiac total anomalous pulmonary venous connection (TAPVC). Management of obstructed supracardiac TAPVC is complicated as PGE1 infusion is contraindicated and immediate surgical correction is advised.

About the so-called "appendicular symptom complex" in malaria

1937 ◽  
Vol 33 (12) ◽  
pp. 1499-1501
Author(s):  
A. А. Shklyaev
Keyword(s):  
Abdominal Pain ◽  
Common Disease ◽  
Abdominal Organs ◽  
Symptom Complex ◽  
Cardinal Symptoms

Appendicitis, being the most common disease of the abdominal organs and at the same time the most "insidious" disease in which the cardinal symptoms take on all sorts of shades, should rightfully occupy the attention of a therapist, and especially a surgeon when patients complain of abdominal pain.

Genetic basis of human left–right asymmetry disorders

2014 ◽  
Vol 16 ◽  
Author(s):  
Hao Deng ◽  
Hong Xia ◽  
Sheng Deng
Keyword(s):  
Situs Inversus ◽  
Genetic Basis ◽  
Chromosomal Abnormalities ◽  
The Body ◽  
Disease Genes ◽  
Internal Organs ◽  
Abdominal Organs ◽  
Pathogenic Genes ◽  
Left Right Asymmetry ◽  
Reduced Penetrance

Humans and other vertebrates exhibit left–right (LR) asymmetric arrangement of the internal organs, and failure to establish normal LR asymmetry leads to internal laterality disorders, includingsitus inversusandheterotaxy.Situs inversusis complete mirror-imaged arrangement of the internal organs along LR axis, whereasheterotaxyis abnormal arrangement of the internal thoraco-abdominal organs across LR axis of the body, most of which are associated with complex cardiovascular malformations. Both disorders are genetically heterogeneous with reduced penetrance, presumably because of monogenic, polygenic or multifactorial causes. Research in genetics of LR asymmetry disorders has been extremely prolific over the past 17 years, and a series of loci and disease genes involved insitus inversusandheterotaxyhave been described. The review highlights the classification, chromosomal abnormalities, pathogenic genes and the possible mechanism of human LR asymmetry disorders.

scholarly journals REACTIONS IN SKIN AND IN INTERNAL ORGANS OF RABBITS SENSITIZED WITH STREPTOCOCCUS OR WITH PNEUMOCOCCUS VACCINE AND THEIR RELATIONSHIP TO ANTIBODIES

1943 ◽  
Vol 78 (2) ◽  
pp. 135-150 ◽  
Author(s):  
Paul F. deGara ◽  
D. Murray Angevine
Keyword(s):  
Antibody Titre ◽  
Specific Antibody ◽  
Anterior Wall ◽  
High Titre ◽  
Stomach Wall ◽  
Type I ◽  
Allergic Reactions ◽  
Local Inflammation ◽  
Internal Organs ◽  
Abdominal Organs

1. Homologous vaccine was injected into abdominal organs of rabbits after different intervals following sensitization by a single or by repeated intracutaneous injections of heat-killed pneumococci Type I or of formalin-killed hemolytic streptococci (Strain AB13). 2. Local inflammation with or without necrosis was observed in the liver and in the spleen of most of the rabbits. Injections into the kidney and into the anterior wall of the stomach usually caused hemorrhagic lesions at the site of injections, frequently accompanied by slight or moderate inflammation and seldom by necrosis. 3. No correlation was found between the skin and the organ reactions of the animals sensitized with killed streptococci or with killed pneumococci. There was also no correlation between skin or organ reactions and antibody titre of serum. 4. Allergic reactions occur in those organs, namely, liver and spleen, in which antibodies are demonstrable in a high titre, and they are usually absent in kidney and stomach wall in which the antibody titre is low or negative. It is probable that the reactions are caused by the union of antigen and its specific antibody in the tissue.

scholarly journals Recurrence of bladder cancer in the urethra

2021 ◽  
Vol 14 (2) ◽  
pp. 40-45
Author(s):  
V.V Protoshchak ◽  
◽  
A.A. Sivakov ◽  
V.K. Karandashov ◽  
L.M. Sinelnikov ◽  
...  
Keyword(s):  
Bladder Cancer ◽  
Clinical Observation ◽  
Cytological Study ◽  
Malignant Tumors ◽  
Abdominal Organs ◽  
Clinical Interest ◽  
Magnetic Resonance Imaging Mri ◽  
Recurrent Bladder Cancer ◽  
External Genital Organs

Introduction. Bladder cancer (ВС) is one of the most common malignant tumors of the urinary tract. In recent decades, recurrent BC in the urethra has become increasingly common. Given the rarity of this pathology, it is of clinical interest for oncologists and urologists. Materials and methods. A clinical case of recurrent bladder cancer in the urethra in a 63-year-old patient is presented. The results of magnetic resonance imaging (MRI) of the pelvic organs and external genital organs, as well as the results of computed tomography of the abdominal organs and observation by a hematologist are presented. Results. For early diagnosis of tumor changes in the urethra in patients with risk factors for the recurrence of BC after cystectomy, careful observation in the form of a cytological study of flushing water from it, performing urethroscopy with ascending urethrography and MRI of the external genital organs is advisable. Conclusions. This clinical observation demonstrates the importance of assessing the state of the urethra when follow up patients after radical cystectomy for BC.

scholarly journals Bellymount enables longitudinal, intravital imaging of abdominal organs and the gut microbiota in adult Drosophila

2019 ◽  
Author(s):  
Leslie Ann Jaramillo Koyama ◽  
Andrés Aranda-Díaz ◽  
Yu-Han Su ◽  
Shruthi Balachandra ◽  
Judy Lisette Martin ◽  
...  
Keyword(s):  
Tissue Level ◽  
Microbial Colonization ◽  
Internal Organs ◽  
Cell Lineages ◽  
Non Invasive ◽  
Abdominal Organs ◽  
Invasive Method ◽  
Longitudinal Imaging ◽  
Long Timescales ◽  
Adult Drosophila

Cell- and tissue-level processes often occur across days or weeks, but few imaging methods can capture such long timescales. Here we describe Bellymount, a simple, non-invasive method for longitudinal imaging of the Drosophila abdomen at sub-cellular resolution. Bellymounted flies remain live and intact, so the same individual can be imaged serially to yield vivid time series of multi-day processes. This feature opens the door to longitudinal studies of Drosophila internal organs in their native context. Exploiting Bellymount’s capabilities, we track intestinal stem cell lineages and gut microbial colonization in single flies, revealing spatiotemporal dynamics undetectable by previously available methods.

scholarly journals Lobular panniculitis as a manifestation of pancreatitis. Difficulties in diagnosis

2021 ◽  
Vol 59 (4) ◽  
pp. 471-477
Author(s):  
O. N. Egorova ◽  
A. L. Maslovsky ◽  
I. A. Zelenov ◽  
A. N. Goryachev ◽  
B. S. Belov
Keyword(s):  
Differential Diagnosis ◽  
Clinical Picture ◽  
Clinical Observation ◽  
Internal Organs ◽  
Therapeutic Practice ◽  
Diagnosis And Therapy ◽  
Total Damage ◽  
Lobular Panniculitis

The clinical picture of lobular panniculitis associated with damage to the pancreas can vary widely, accompanied by damage to the joints and internal organs, which complicates the diagnosis of the disease. A clinical observation of a torpid course of lobular panniculitis with total damage to organs and systems is presented, which clearly illustrates the difficulties of differential diagnosis and therapy that arise in real therapeutic practice.

Experience in studying blood alkalinity in eczema

1930 ◽  
Vol 26 (7) ◽  
pp. 83-88
Author(s):  
B. S. Bikkenin ◽  
N. N. Yasnitsky
Keyword(s):  
Metabolic Disorders ◽  
Point Of View ◽  
The Body ◽  
Autonomic Innervation ◽  
General State ◽  
Internal Organs ◽  
Symptom Complex ◽  
Biological Relationship ◽  
Relationship Of

The etiology of eczema is still one of the darkest and most controversial, still awaiting its resolution in dermatology. The peculiarity of the course and clinical varieties of eczema, which in some cases do not fit into the framework of a certain symptom complex, its occurrence in persons suffering from constitutional diseases, undoubtedly in some cases causing dependence on disorders in the field of autonomic innervation, finally, studied experimentally by Jadassohn, Bloch , Wеidеnfeld'om the reactivity of the skin of eczematics, its special state of readiness for eczema (Eczembereit-schaft), which is an expression of sensitization all this makes the view of eczema quite reasonable as a process that is etiologically closely associated with changes in the general state of the body. From this point of view, those studies that are aimed at finding out the intimate biological relationship of the skin with various systems of internal organs and to establish the ways in which the influence of metabolic disorders should be transmitted to the skin are of particular importance.

Weight Loss in Alzheimer's Disease: An International Review of the Literature

1994 ◽  
Vol 6 (2) ◽  
pp. 135-142 ◽  
Author(s):  
Gisele P. Wolf-Klein ◽  
Felix A. Silverstone
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Weight Loss ◽  
Primary Care Physicians ◽  
Clinical Observation ◽  
International Studies ◽  
Review Of The Literature ◽  
Adequate Food ◽  
Nutritional Strategies ◽  
The U.S

Alzheimer's disease affects an estimated 2 million elderly in the U.S. and challenges primary care physicians to assist caregivers in dealing with the daily management of these patients. To support the clinical observation of weight loss in Alzheimer patients despite adequate food intake, we reviewed the existing literature. To date, eight international studies have focused on nutrition in Alzheimer's disease and all have found weight loss. It is not clear whether this weight loss is a component of or a consequence of the disease.These findings suggest systemic, metabolic alterations in Alzheimer's disease. They require further investigation as to their nature and as to their appropriate recognition and management to retard the deteriorating effects of chronic weight loss and malnutrition. Finally, some reports lead to speculation that nutritional strategies may improve cognitive function.

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