Type I leucocyte adhesion deficiency in Yemenian family managed with appropriate treatment: A case series

2019 ◽  
Vol 32 (3) ◽  
Author(s):  
Carmen Cantisani ◽  
Ahmad Faiq Naqeshbandi ◽  
Mohamad Goldust ◽  
Salvatore Lampitelli ◽  
Franca Cantoresi ◽  
...  
Keyword(s):  
Case Series ◽  
Type I ◽  
Leucocyte Adhesion ◽  
Appropriate Treatment

scholarly journals Endocrine Dysfunction in Children with Zika-Related Microcephaly Who Were Born during the 2015 Epidemic in the State of Pernambuco, Brazil

Viruses ◽  
2020 ◽  
Vol 13 (1) ◽  
pp. 1
Author(s):  
Andréia Veras Gonçalves ◽  
Demócrito de B. Miranda-Filho ◽  
Líbia Cristina Rocha Vilela ◽  
Regina Coeli Ferreira Ramos ◽  
Thalia V. B. de Araújo ◽  
...  
Keyword(s):  
Optic Nerve ◽  
Viral Infections ◽  
Case Series ◽  
Optic Nerve Hypoplasia ◽  
Endocrine Dysfunction ◽  
Careful Monitoring ◽  
Early Puberty ◽  
Growth Impairment ◽  
Appropriate Treatment ◽  
The Brain

Congenital viral infections and the occurrence of septo-optic dysplasia, which is a combination of optic nerve hypoplasia, abnormal formation of structures along the midline of the brain, and pituitary hypofunction, support the biological plausibility of endocrine dysfunction in Zika-related microcephaly. In this case series we ascertained the presence and describe endocrine dysfunction in 30 children with severe Zika-related microcephaly from the MERG Pediatric Cohort, referred for endocrinological evaluation between February and August 2019. Of the 30 children, 97% had severe microcephaly. The average age at the endocrinological consultation was 41 months and 53% were female. The most frequently observed endocrine dysfunctions comprised short stature, hypothyroidism, obesity and variants early puberty. These dysfunctions occurred alone 57% or in combination 43%. We found optic nerve hypoplasia (6/21) and corpus callosum hypoplasia (20/21). Seizure crises were reported in 86% of the children. The most common—and clinically important—endocrine dysfunctions were pubertal dysfunctions, thyroid disease, growth impairment, and obesity. These dysfunctions require careful monitoring and signal the need for endocrinological evaluation in children with Zika-related microcephaly, in order to make early diagnoses and implement appropriate treatment when necessary.

Cartilage Regeneration with Cell-free Type 1 Collagen Matrix – Past, Present and Future (Part 1 – Clinical Aspects)

2020 ◽  
Author(s):  
Philip Peter Roessler ◽  
Turgay Efe ◽  
Dieter Christian Wirtz ◽  
Frank Alexander Schildberg
Keyword(s):  
Type I Collagen ◽  
Case Series ◽  
Cartilage Regeneration ◽  
Collagen Matrix ◽  
Treatment Method ◽  
Collagen Type I ◽  
Legal Framework ◽  
Clinical Aspects ◽  
Type I ◽  
Collagen Hydrogel

AbstractCartilage regeneration with cell-free matrices has developed from matrix-associated autologous cartilage cell transplantation (MACT) over ten years ago. Adjustments to the legal framework and higher hurdles for cell therapy have led to the procedures being established as an independent alternative to MACT. These procedures, which can be classified as matrix-induced autologous cartilage regeneration (MACR), all rely on the chemotactic stimulus of a cross-linked matrix, which mostly consists of collagens. Given the example of a commercially available type I collagen hydrogel, the state of clinical experience with MACR shall be summarized and an outlook on the development of the method shall be provided. It has been demonstrated in the clinical case series summarized here over the past few years that the use of the matrix is not only safe but also yields good clinical-functional and MR-tomographic results for both small (~ 10 mm) and large (> 10 mm) focal cartilage lesions. Depending on the size of the defect, MACR with a collagen type I matrix plays an important role as an alternative treatment method, in direct competition with both: microfracture and MACT.

scholarly journals Cryoprobe biopsy for the diagnosis of acute hypoxemic respiratory failure of undetermined origin

2019 ◽  
Vol 21 (2) ◽  
pp. 119-123
Author(s):  
Marcos J Las Heras ◽  
Jose Dianti ◽  
Manuel Tisminetzky ◽  
Graciela Svetliza ◽  
Sergio E Giannasi ◽  
...  
Keyword(s):  
Respiratory Failure ◽  
Diagnostic Yield ◽  
Specific Treatment ◽  
Case Series ◽  
Safe Procedure ◽  
Hypoxemic Respiratory Failure ◽  
Acute Hypoxemic Respiratory Failure ◽  
Select Group ◽  
Lung Sample ◽  
Appropriate Treatment

Rationale Acute hypoxemic respiratory failure is a condition that comprises a wide array of entities. Obtaining a histological lung sample might help reach a diagnosis and direct an appropriate treatment in a select group of patients. Objective To describe our experience in the use of cryobiopsy for the diagnosis of acute hypoxemic respiratory failure of undetermined origin. Methods Retrospective analysis of case series of patients with acute hypoxemic respiratory failure who underwent lung cryobiopsy at the Intensive Care Unit of the Hospital Italiano de Buenos Aires, Argentina. Results Cryobiopsy yielded a histological diagnosis in all patients ( n = 10, 100%). This led to either a change in therapy or continuation of a specific treatment in eight of these patients. Cryobiopsy was found to be contributive in all the patients who did not meet Berlin criteria for acute respiratory distress syndrome. No major complications were associated with the procedure. Conclusions Cryobiopsy is a safe procedure with a high diagnostic yield in a selected group of patients.

Long-term outcomes of Boston keratoprosthesis type I: the Chinese People’s Liberation Army General Hospital experience

2021 ◽  
pp. bjophthalmol-2019-315617
Author(s):  
Li-Qiang Wang ◽  
Teng-Yun Wu ◽  
Xiao-Niao Chen ◽  
Ze-Quan Xu ◽  
Min Yang ◽  
...  
Keyword(s):  
General Hospital ◽  
Ocular Surface ◽  
Retention Rate ◽  
Case Series ◽  
Type I ◽  
Long Term Outcomes ◽  
Boston Keratoprosthesis ◽  
People's Liberation Army ◽  
People’S Liberation Army

PurposeTo report the long-term outcomes of Boston keratoprosthesis type I (B-KPro type I) implantation in the management of severe ocular surface disorders.MethodsRetrospective case series. Patients who underwent B-KPro type I implantation at the People’s Liberation Army General Hospital were enrolled between March 2011 and September 2019. Data regarding visual acuity (VA), B-KPro type I retention and postoperative complications were recorded and analysed.ResultsA total of 103 eyes of 100 patients who underwent B-KPro type I implantation were included. The main indications were chemical burn (59.2%), ocular trauma (25.2%), herpetic keratitis (11.7%) and autoimmune diseases (3.9%). The percentage of eyes with postoperative VA of 10/200 or better was 82.7% at 6 months, 82.8% at 12 months, 77.9% at 2 years, 72.4% at 3 years, 71.1% at 4 years, 69.4% at 5 years, 58.9% at 6 years, 56.8% at 7 years and 42.9% at 8 years. Preoperatively, 8.7% eyes were diagnosed with new-onset glaucoma. Retroprosthetic membrane formation occurred in 19.4% eye. Corneal melting occurred in 18.4% eyes. Sterile vitritis was diagnosed in 4.9% eyes and infectious endophthalmitis in 2.9% eyes. Retinal detachment occurred in 0.9% eyes.ConclusionsIn a Chinese patient group, B-KPro type I is a viable option for treating severe ocular surface disorders in eyes where conventional keratoplasty would have a poor prognosis, especially in patients with chemical and thermal burns. Improved visual outcomes and high retention rate can be achieved and maintained in most cases.

scholarly journals A Possible Association between Arnold-Chiari Type I Malformation and Epilepsy: Case Series and Review

2018 ◽  
Vol 2 (1) ◽  
Author(s):  
Hadelsberg Uri P ◽  
Goldberg Hadassah ◽  
Marianayagam Neelan J ◽  
Rajz Gustavo
Keyword(s):  
Case Series ◽  
Type I ◽  
Chiari Type ◽  
Chiari Type I Malformation ◽  
Chiari Type I

scholarly journals TRADITIONAL BONESETTERS

2016 ◽  
Vol 23 (06) ◽  
pp. 699-704
Author(s):  
Waqar Alam ◽  
Faaiz Ali Shah ◽  
Ashfaq Ahmed ◽  
Saeed Ahmad ◽  
Abdullah Shah
Keyword(s):  
Compartment Syndrome ◽  
Study Design ◽  
Low Cost ◽  
Cultural Beliefs ◽  
Case Series ◽  
Non Union ◽  
Appropriate Treatment ◽  
Accident And Emergency ◽  
Set Up ◽  
Study District

Objectives: To analyze the reasons for treatment by Traditional Bone Setters(TBS) and the frequency of complications in patients treated by traditional bone setters in our setup. Study Design: Descriptive case series. Place and Duration of Study: District HeadquarterHospital (DHQ) Temargarah & Lady Reading Hospital Peshawar and, Ghurki Trust TeachingHospital, Lahore from Dec 2014 to Nov 2015. Material and Methods: Patients of either genderor all ages received in outpatient department or accident and emergency of DHQ Temargarah,Lady Reading Hospital Peshawar and Ghurki Trust Teaching Hospital, Lahore with history oftrauma followed by treatment by local bonesetters were included in our study. After properhistory, examinations and investigations, appropriate treatment were given and complicationswere noted. Results: We received a total of 267 patients who were treated by Traditional bonesetters.186 were males and 81 were females. Age ranges from 1.5 years to 87 years. In majorityAdvice or pressure from family/friends taking the lead. 77(28.84%) of patients suffer because offamily or friends. 66(24.72%) of patients affected because of socio cultural beliefs, 46(17.23%)because of low cost, 35(13.11%) because of ignorance, 24(8.98%) because of fear of operation,19(7.12%) because of fear of amputation took their treatment from bone setters. The mostfrequent complication we received was malunion were found in 67(25.10%) and non-union in55(20.60%), Conclusion: Pressure from friends and family was the main reason for consultingTraditional Bone Setters for treatment in our set up and complications caused by their treatmentwere frequent and ranged from immediate compartment syndrome and gangrene of the limb tolate onset mal union, non-union and avascular non-union.

scholarly journals GYNAECOMASTIA

2010 ◽  
Vol 17 (02) ◽  
pp. 205-210
Author(s):  
ASIYA SHABBIR ◽  
TARIQ MAHMOOD REHAN ◽  
SHAHID Hussain Jaffery ◽  
Muhammad Ubaidullah Baig
Keyword(s):  
Benign Lesion ◽  
Case Series ◽  
Spontaneous Resolution ◽  
Subcutaneous Mastectomy ◽  
Appropriate Treatment ◽  
Case Series Study ◽  
Causative Factors ◽  
Male Patients ◽  
Series Study ◽  
Surgical Unit

Objectives: To determine the causative factors and management of Gynaecomastia. Study Design: Observational case series study. Place and Duration of Study: Surgical Unit-1 Bahawal Victoria Hospital Bahawalpur, from 1st January 200 till 31st December 2007. Patient and Methods: Thirty male patients having breast swelling were included in this study. Relevant history was obtained. Appropriate physical examination was performed. Necessary investigations were done and after making a diagnosis appropriate treatment was done. A total of 30 patients were studied in the study period of 18 months. Those male patients who were having breast lesion other than benign enlargement (e.g. Ca. Breast, Breast/Abscess) were not included in the study. Necessary investigations were done. Subcutaneous Mastectomy was performed. Results: The most common age group developing gynaecomastia was of 20-30 years (60%). Bilateral gynaecomastia was observed in 76.66% and unilateral gynaecomastia in 23.33%. Idiopathic gynaecomastia was observed in 73.33% cases. Medical treatment was given with tamoxifen 10mg twice a day for the period of three months in 6 case (20%) and this remained effective in 5 case (83.33%). Subcutaneous mastectomy was performed in 17 cases (56.66%). Post-operative complications were seen in 4 cases (23.52%), the most common complication being wound hematoma in 2 cases (11.76%). Most of the patients (88.9%) were fully satisfied with the results of subcutaneous mastectomy. Conclusion: Gynaecomastia is the most common benign lesion of the male breast. As far as physiological gynaecomastia is concerned, patients should be observed for at least 2 years from the onset of their condition. In most of the cases spontaneous resolution occurs. Surgical treatment should be planned in whom spontaneous resolution does not occur. Surgery remains the mainstay of therapy and is frequently indicated for psychological and cosmetic reasons. 

Three Patients with CPT1A Deficiency: Literature Review and Case Series

2020 ◽  
Author(s):  
Naser Ali Mirhosseini ◽  
Mahdieh Saatchi ◽  
Sana Taghiyar
Keyword(s):  
Genetic Disorder ◽  
Case Series ◽  
Autosomal Recessive Inheritance ◽  
Abnormal Liver Function Test ◽  
Medical Procedures ◽  
Main Method ◽  
Appropriate Treatment ◽  
Liver And Kidney ◽  
Renal Tubular

Background: Carnitine palmitoyltransferase 1A deficiency is a rare genetic disorder with autosomal recessive inheritance pattern of fatty acid metabolism secondary to CPT1A mutation. Several dozen infants and children have been described with a deficiency of the liver and kidney CPT1 isoenzyme (CPT1-A). Clinical manifestation includes fasting-induced hypoketotic hypoglycemia, occasionally with extremely abnormal liver function test (LFT) and rarely with renal tubular acidosis. Acyl carnitine analysis has been the main method for the diagnosis of CPT1A deficiency.  Prompt treatment of hypoglycemia includes intravenous fluid containing 10% dextrose. To prevent hypoglycemia, infants should eat frequently during the day and have cornstarch continuously at night. Fasting should not last more than 12 hours during illness, surgery, or medical procedures. Case Presentation: We reported three patients with CPT1A deficiency presented with hypoglycemia and Reye like syndrome in early childhood that with early diagnosis and treatment they are well in follow-up. Conclusion: Prognosis of this genetic disorder will be good with appropriate treatment.

scholarly journals Kounis syndrome uncovers severe coronary disease: an unusual case of acute coronary syndrome secondary to allergic coronary vasospasm

2019 ◽  
Vol 12 (12) ◽  
pp. e232472
Author(s):  
Luca Conti ◽  
Kelly Gatt ◽  
Christopher Zammit ◽  
Karen Cassar
Keyword(s):  
Myocardial Infarction ◽  
Acute Coronary Syndrome ◽  
High Signal ◽  
Type I ◽  
Kounis Syndrome ◽  
Coronary Syndrome ◽  
Triple Vessel Disease ◽  
Appropriate Treatment ◽  
St Elevation ◽  
Type I Hypersensitivity

Acute coronary syndrome occurring during the course of a type I hypersensitivity reaction constitutes Kounis syndrome. We report a case of a 64-year-old man who presented with a non-ST elevation myocardial infarction and peripheral blood eosinophilia. He had rhinitis and constitutional symptoms for several days prior to presentation. Blood investigations revealed moderate eosinophilia and elevated IgE levels. A cardiac MRI showed generalised oedema with a subtle wall motion abnormality in basal inferior/inferolateral wall, and subendocardial high signal on late gadolinium enhancement suggesting a localised myocardial infarction. A coronary angiogram then revealed triple vessel disease. A diagnosis of Kounis syndrome was made. Within days of starting appropriate treatment, the patient’s eosinophil count returned to normal with improvement of clinical picture.

scholarly journals Orofacial clinical features in arnold chiari type i malformation: a case series

2019 ◽  
Vol 48 ◽  
pp. 274
Author(s):  
E.L.D. Figueiredo ◽  
B. Neres ◽  
B. Vasconcelos ◽  
R. Vasconcellos ◽  
M.D. Bortoli ◽  
...  
Keyword(s):  
Clinical Features ◽  
Case Series ◽  
Type I ◽  
Chiari Type ◽  
Chiari Type I Malformation ◽  
Chiari Type I
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