INHERITED EPIDERMOLYSIS BULLOSA IN NEWBORN (CASE STUDY)

2021 ◽  
Vol 74 (10) ◽  
pp. 2525-2529
Author(s):  
Veronika M. Dudnyk ◽  
Olga I. Izumets ◽  
Oksana S. Rubina ◽  
Valentin H. Furman
Keyword(s):  
Epidermolysis Bullosa ◽  
Malignant Tumors ◽  
Multidisciplinary Care ◽  
Optimal Care ◽  
Appropriate Treatment ◽  
Specialized Center ◽  
Mucous Membranes ◽  
Inherited Epidermolysis Bullosa

Inherited epidermolysis bullosa (IEB) is a group of genetically and clinically heterogeneous diseases characterized by the formation on the skin and mucous membranes blisters and erosion due to injury. Different forms of IEB can be accompanied by various extracutaneous complications, such as blisters and erosion on the cornea and mucous membranes, stenoses and strictures of the respiratory system, gastrointestinal tract, urinary system, muscle dystrophy, and malignant tumors. Therefore diagnosis and prescribing appropriate treatment and follow-up care is an important task for neonatologists and pediatric dermatologists. Because the manifestations of IEB are numerous, a specialized center is required for optimal care, where multidisciplinary care will be provided (neonatologists, pediatric surgeons, pediatric dermatologists, etc.). The purpose of this case report is to pay attention of specialists to a disease that is rare, to present clinical case of IEB in newborn who was admitted to the intensive care unit of newborns of Vinnitsa Regional Children’s Clinical Hospital.

scholarly journals CLINICAL CASES OF BULLOUS EPIDERMOLYSIS IN NEWBORNS

2020 ◽  
Vol 10 (4(38)) ◽  
pp. 87-91
Author(s):  
O. Rubina ◽  
K. Bertsun ◽  
O. Izyumetsʹ ◽  
R. Gomon ◽  
A. Zadorozhna
Keyword(s):  
Basement Membrane ◽  
Malignant Tumors ◽  
Clinical Manifestations ◽  
Multidisciplinary Care ◽  
Basal Membrane ◽  
Dna Diagnosis ◽  
Optimal Care ◽  
Mucous Membranes ◽  
Genes Encoding ◽  
Clinical Cases

Summary. Hereditary bullous epidermolysis (BE) is a group of genetically and clinically heterogeneous diseases characterized by the formation of blisters and erosion due to injury on the skin and mucous membranes. Different forms of BE can be accompanied by various extracutaneous complications, such as blisters and erosion on the cornea and mucous membranes, stenoses and strictures of the respiratory system, gastrointestinal tract, urinary system, muscular dystrophy, and malignant tumors. Hereditary bullous epidermolysis is divided into three types, depending on the level of blister formation: simple, borderline, and dystrophic. Simple BE is characterized by the stratification of epidermis due to the keratinocyte cytolysis.  Borderline BE means that blisters are formed at the border of the epidermis and dermis due to the splitting of the lamina of the basement membrane (lamina lucida), while dystrophic BE has blisters that are formed under the dense plate of the basement membrane (lamina densa), which exfoliates the dermis. Currently, mutations have been identified in more than 10 genes encoding the structural proteins of keratinocytes and the basal membrane of the skin and mucous membranes. A common feature of these proteins is their involvement in the formation of strong bonds between the epithelium and the basement membrane. The nature of the mutations and their localization determine the severity of the clinical manifestations of BE. Mutation information is a prerequisite for effective medical and genetic counseling, prenatal and preimplantation DNA diagnosis. Therefore diagnosis and prescribing appropriate treatment and follow-up care is an important task for neonatologists and pediatric dermatologists. As manifestations of hereditary BE are numerous, a specialized center is required for optimal care, where multidisciplinary care will be provided (neonatologists, pediatric surgeons, pediatric dermatologists, etc.). The purpose of clinical observation is to pay attention of specialists to this rare disease, and to present 2 clinical cases of bullous epidermolysis in newborns who were admitted to the anesthesiology and intensive care unit of newborns of Vinnytsia Regional Children's Clinical Hospital almost at the same time.

Primary Mammary (Non-Hodgkin) Lymphoma of Breast: a Rare Case Report

2018 ◽  
Vol 3 (1) ◽  
Author(s):  
Bamindele Johnson Alegbeleye ◽  
Benjamin Malikdoko
Keyword(s):  
Hodgkin Lymphoma ◽  
Malignant Tumors ◽  
Cell Type ◽  
Primary Breast Lymphoma ◽  
Non Hodgkin Lymphoma ◽  
Breast Lymphoma ◽  
Appropriate Treatment ◽  
Rare Case Report ◽  
Extranodal Lymphomas

Breast lymphomas are quite rare extranodal lymphomas. They constitute a very small percentage of malignant tumors of the breast and a little subset of extranodal lymphomas. We report a case of primary mammary non-Hodgkin lymphoma in an 18 year old lady. FNAC was inconclusive and incisional biopsy confirmed primary breast lymphoma which was diagnosed as the diffuse large B-cell type; Non – Hodgkin’s lymphoma. She has complete disease remission to chemotherapy and also on follow-up. In conclusion, it is mandatory for clinician assessing breast mass to recognize this disease entity as a potential differential diagnosis and to do core biopsy so as to exclude PBL before appropriate treatment.

scholarly journals Parapharyngeal space tumors: a serial case study

2019 ◽  
Vol 47 (8) ◽  
pp. 4004-4013
Author(s):  
Kuang-Hsu Lien ◽  
Chi-Kuang Young ◽  
Shy-Chyi Chin ◽  
Chun-Ta Liao ◽  
Shiang-Fu Huang
Keyword(s):  
Parapharyngeal Space ◽  
Clinical Symptoms ◽  
Malignant Tumors ◽  
Neck Mass ◽  
Surgical Approaches ◽  
Common Symptom ◽  
Neck Tumors ◽  
The Mean

Objective Primary parapharyngeal space (PPS) tumors are one of the most challenging head and neck tumors to diagnose and treat. We analyzed our experience in patients with PPS tumors who were treated in our hospital over 13 years. Methods We retrospectively reviewed 16 patients with PPS tumors between 2006 and 2018. The study included clinical symptoms, histological types, surgical approaches, adjuvant therapies, postoperative complications, and prognosis. Results The mean age of the patients was 49.63 ± 17.03 years. A palpable neck mass (56.3%) was the most common symptom. In our series, 78.6% of the tumors were benign, and of these, schwannomas were the most common (6/14, 42.9%). Three surgical approaches were used in our patients, including transmandibular (57.1%), transcervical (21.4%) and transparotid (21.4%) approaches. Few complications were reported, including hoarseness and numbness. Adjuvant therapy was administered depending on pathological parameters of the tumors. No recurrence was observed during a mean follow-up of 60.6 months in patients with malignant tumors. Conclusions Radiological studies of PPS tumors are essential for diagnosis and surgical planning. Excision of PPS tumors using appropriate surgical approaches provides good outcomes.

Possible Trimethoprim–Sulfamethoxazole-Induced Hemolytic Anemia: A Case Report

2016 ◽  
Vol 30 (6) ◽  
pp. 653-657 ◽  
Author(s):  
Montgomery F. Williams ◽  
Emily P. Doss ◽  
Maggie Montgomery
Keyword(s):  
Hemolytic Anemia ◽  
Laboratory Testing ◽  
Signs And Symptoms ◽  
Probability Scale ◽  
Appropriate Treatment ◽  
Mucous Membranes ◽  
Mild Abdominal Pain ◽  
Oral Steroids ◽  
Packed Rbcs

Objective: To report a case of hemolytic anemia in a patient who received trimethoprim/sulfamethoxazole (TMP-SMX) for a urinary tract infection (UTI). Summary: A 47-year-old woman recently diagnosed with uncomplicated UTI received 3 doses of TMP-SMX. She developed yellowing of the skin and eyes, lethargy, mild abdominal pain, and dry mucous membranes. Laboratory testing demonstrated significant anemia with red blood cells (RBCs) of 1.99, hemoglobin (Hgb) of 6.3 g/dL, and hematocrit (Hct) of 18.1%. TMP-SMX was immediately discontinued. The patient was given methylprednisolone 60 mg intravenously (IV) followed by oral steroids and infused with 3 units of packed RBCs over the course of a 10-day inpatient admission. On discharge, the patient continued oral steroids. Outpatient follow-up indicated Hgb of 11.0 g/dL and Hct of 32.7%, 41 days after hospital discharge. Utilizing the Naranjo adverse drug reaction probability scale, there is a probable association between the patient’s hemolytic anemia and TMP-SMX. Conclusion: We report a case of hemolytic anemia resulting from the use of TMP-SMX. Although this is a rare adverse effect, clinicians should be aware of the signs and symptoms of hemolytic anemia, and so appropriate treatment can be administered should it occur.

Intensive Stuttering Therapy With Telepractice Follow-up: A Case Study

2011 ◽  
Vol 21 (1) ◽  
pp. 11-21 ◽  
Author(s):  
Farzan Irani ◽  
Rodney Gabel
Keyword(s):  
Case Report ◽  
Therapeutic Intervention ◽  
Intensive Therapy ◽  
Positive Outcome ◽  
Eclectic Approach

This case report describes the positive outcome of a therapeutic intervention that integrated an intensive, residential component with follow-up telepractice for a 21 year old male who stutters. This therapy utilized an eclectic approach to intensive therapy in conjunction with a 12-month follow-up via video telepractice. The results indicated that the client benefited from the program as demonstrated by a reduction in percent stuttered syllables, a reduction in stuttering severity, and a change in attitudes and feelings related to stuttering and speaking.

Efektivitas Layanan Darurat Jakarta Siaga 112 dalam Penanganan Kebakaran oleh UPT. Pusat Data & Informasi Kebencanaan BPBD Provinsi DKI Jakarta

2020 ◽  
Vol 16 (1) ◽  
Author(s):  
Musa Musa
Keyword(s):  
Emergency Service ◽  
Fire Management ◽  
Emergency Services ◽  
The Public ◽  
Information Center ◽  
Disaster Data ◽  
In Fire ◽  
Document Review

This research was conducted to determine the Effectiveness of Jakarta Siaga 112 Emergency Services in Fire Management by UPT. Disaster Data & Information Center of BPBD DKI Jakarta Province by paying attention to aspects contained in the Effectiveness of the Jakarta Siaga Emergency Service Program 112. The research method was carried out with a case study method with data collection techniques using interview methods and document review. Interviews were conducted on 10 (ten) key informants, document review focused on documents related to the Jakarta Emergency Alert Service 112 Effectiveness research in Fire Management. The results showed that the Effectiveness of Jakarta Siaga 112 Emergency Services in Fire Management by UPT. The Center for Disaster Data & Information BPBD DKI Jakarta Province Its effectiveness is still low, due to the Implementation of Emergency Services Jakarta Standby 112 in Fire Management implemented by UPT. Disaster Data & Information Center of BPBD DKI Jakarta Province in terms of the Target Group Understanding of the Program, the Achievement of the Program Objectives aspects, and the Program Follow-up aspects. It is recommended to continue to disseminate this Emergency Service to the public, it is necessary to increase the firm commitment of the Head of 8 SKPD related to fire management so that all units play a role in accordance with the Standard Operating Procedures (SOPs) for Fire Management and the evaluation and follow-up of program services that are held periodically 3 once a month.Keywords: Effectiveness, Emergency Services, Fire Handling

Site specific agricultural soil management with the use of new technologies

2013 ◽  
Vol 16 (1) ◽  
pp. 59-67
Keyword(s):  
New Technologies ◽  
Digital Interface ◽  
Appropriate Treatment ◽  
Wide Range ◽  
Soil Variation ◽  
Soil Information ◽  
Remote Sensing Techniques ◽  
Agricultural Areas

<p>The Soil Science Institute of Thessaloniki produces new digitized Soil Maps that provide a useful electronic database for the spatial representation of the soil variation within a region, based on in situ soil sampling, laboratory analyses, GIS techniques and plant nutrition mathematical models, coupled with the local land cadastre. The novelty of these studies is that local agronomists have immediate access to a wide range of soil information by clicking on a field parcel shown in this digital interface and, therefore, can suggest an appropriate treatment (e.g. liming, manure incorporation, desalination, application of proper type and quantity of fertilizer) depending on the field conditions and cultivated crops. A specific case study is presented in the current work with regards to the construction of the digitized Soil Map of the regional unit of Kastoria. The potential of this map can easily be realized by the fact that the mapping of the physicochemical properties of the soils in this region provided delineation zones for differential fertilization management. An experiment was also conducted using remote sensing techniques for the enhancement of the fertilization advisory software database, which is a component of the digitized map, and the optimization of nitrogen management in agricultural areas.</p>

Paranasal Sinus Mucocele Clinical, imagistic and biochemical aspects

2018 ◽  
Vol 69 (8) ◽  
Author(s):  
Doina Vesa ◽  
Cristian Martu ◽  
Razvan Leata ◽  
Ludmila Lozneanu ◽  
luminita Radulescu ◽  
...  
Keyword(s):  
Facial Asymmetry ◽  
Retrospective Case ◽  
Mri Scans ◽  
The One ◽  
Ethmoid Sinuses ◽  
Retrospective Case Study ◽  
Cellular Components ◽  
Ct And Mri

Paranasal mucoceles are a type of cysts that evolve slowly and are asymptomatic; this poses a difficulty in diagnosing the patient because the symptoms can go unnoticed. The mucocele evolves unpredictably. On the one hand, it can become infected turning into pyoceles and on the other hand, it can invade important regions such as the orbital, cranial or genian regions, creating facial asymmetry. This is a retrospective case study of 37 patients diagnosed with sinus mucoceles, followed up by clinical examination and paraclinical tests such as CT and MRI scans. The biochemical components of the liquid from within the mucocele were analyzed and the following criteria were recorded: NaCl-, Cl-, Na+ and cholesterine as well as cellular components such as mastocytes, macrophages, hematocytes and leucocytes. In all cases, the treatment option was surgery with favorable post-operative and follow-up evaluation. The mucoceles that appeared post-operatively (maxillary and ethmoid sinuses) evolved more rapidly than the mucoceles that were induced byan external injury. Longer follow-up of operated patients permitted a more timely diagnosis of recurrences.

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