How often is Klippel-Feil Syndrome associated with congential heart disease presentation of five cases and a review of the literature
Published on: 3rd September, 2019
OCLC Number/Unique Identifier: 8270717914
Introduction: Klippel-Feil syndrome (KFS), is a bone disorder characterized by the abnormal joining (fusion) of two or more spinal bones in the neck (cervical vertebrae), which is present from birth. Three major features result from this abnormality: a short neck, a limited range of motion in the neck, and a low hairline at the back of the head. In some individuals, KFS can be associated with a variety of additional symptoms and physical abnormalities which contribute in the deterioration and complication of the condition of the child.
Aim of presentation: Here, we report five children from Kosovo with KFS associated with different heart abnormalities, clinical presentation, diagnosis, management, and outcomes of selected conditions in resources-limited settings.
Methods: Retrospectively we analysed medical reports of five children, diagnosed at different age with congenital disease and clinical and lab signs of Klippel-Feil syndrome.
Conclusion: Basing on our cases, all diagnosed in a small country as a Kosovo, we can conclude that KFS is not such a rare condition. In addition, such syndrome is not so rarely associated with different congenital heart disease. In four cases cardiac surgery was indicated and successfully was done abroad Kosovo in the lack of such services in Kosovo.
Patellar fractures in children
Published on: 19th November, 2019
OCLC Number/Unique Identifier: 9269418298
We describe a rare case of patellar fracture in a seven year old girl. The case was reviewed for various aspects such as clinical diagnosis, imaging tests and most suitable treatment.
Treatment of autoimmune hemolytic anemia with erythropoietin: A case report
Published on: 20th November, 2019
OCLC Number/Unique Identifier: 9269420484
In this article, we describe the case of a fifty-year-old patient with autoimmune hemolytic anemia (AIHA) with constitutional symptoms, jaundice, unquantified fever and progressive dyspnea. The patient had history of smoking and Hepatitis A and following a physical exam she was found in a regular condition, icteric but with no other further signs. Her laboratory tests revealed hemolytic anemia with a hemoglobin of 8.5 g/dL, an increase of total and indirect bilirubin, an elevated ferritin, a decreased transferrin and haptoglobin and a positive result for direct Coomb’s test. Considering this, an immune profile was ordered finding a negative result of ANAs and ENAs and a decrease of complement C3 and C4. The patient was diagnosed with AIHA and as an initial step a corticosteroid treatment was administrated however the patient showed no clinical nor chemical improvement. At her third day of hospitalization, she was unstable hemodynamically requiring transfer to Intensive Care Unit (ICU) to optimize management. After 24 hours on ICU, due to persistence of deterioration of the patient, it was decided to manage with erythropoietin (EPO). In the following days, the patient showed a rise in her hemoglobin and an overall improvement made possible the transfer to hospitalization service. The AIHA is an uncommon disease and is not the first option that comes to mind with these symptoms, currently there are not controlled studies to the treatment due to its complexity and the heterogeneity of the results. We strongly support the use of EPO in refractory cases of this pathology.
Lack of applicability of the Enterocyte Chloride ion secretion paradigm to the Pathology of Cystic Fibrosis
Published on: 23rd October, 2017
OCLC Number/Unique Identifier: 7900080180
This review examines of the concept of a defective chloride channel in epithelial cells being a major cause of cystic fibrotic pathophysiology. The central concept of the defective chloride ion channel paradigm is that faulty CFTR protein or failed delivery of CFTR protein to the mucosal membrane of epithelial cells is the basis of cystic fibrosis. Defective placement or function of CFTR prevents hydration of bronchial mucus that is normally caused by epithelial cells; these are capable through chloride ion secretion of transporting fluid to the mucosal surface. This concept relies heavily on a paradigm taken from intestinal physiology-namely that the intestinal epithelial cell secretes chloride ion and fluid and that this has conferred heterozygote selective advantage in carriers of the cystic fibrosis gene. This present review examines the evidence for that hypothesis and assembles evidence from past studies that it is the smooth muscle cell that is of greater relevance. This review does not aim to provide an overview of current research into cystic fibrosis. The intention is to provide an overview of past research that led to the concept of a failure of epithelial cells to hydrate bronchial mucus because of compromised CFTR function. It is important to present all past evidence for aspects of the chloride secretion hypothesis and its associated heterozygote advantage concept so that the important evidential milestones can be re-assessed.
Foley catheter balloon tamponade as a method of controlling iatrogenic pulmonary artery bleeding in redo thoracic surgery
Published on: 20th November, 2019
OCLC Number/Unique Identifier: 9269426092
Background: Pulmonary artery bleeding secondary to iatrogenic injury is a troublesome intraoperative complication. The likelihood of encountering this complication is significantly higher in redo surgery for a number of reasons, including distortion of anatomical structures, adhesions and loss of tissue planes. Significant blood loss, although rare, remains a concern, and can occasionally be life-threatening. When significant bleeding from the pulmonary artery occurs, it can be a challenging situation to manage.
Case Report: A 65 year old female was undergoing redo thoracic surgery in the setting of a completion lobectomy for biopsy-proven primary adenocarcinoma of the lung. Iatrogenic injury to the pulmonary artery resulted in significant bleeding that could not be managed by gaining proximal control due to dense adhesions. The novel decision to utilize a Foley catheter for balloon tamponade was taken, in order to provide sufficient haemostasis for definitive surgical repair of the defect to be undertaken
Conclusion: The increased technical difficulties of redo thoracic surgery are well recognised. We describe the first case of Foley catheter balloon tamponade being utilized in the context of iatrogenic pulmonary artery bleeding during thoracic surgery.
A case series review of patients with Thrombocytopenia and Absent-Radii syndrome (TARS) and their management during pregnancy
Published on: 12th August, 2020
OCLC Number/Unique Identifier: 8667871373
Bleeding diatheses due to platelet-related disorders can present challenges to treating clinicians especially in the context of peri- and post-partum patients in the obstetric setting. TARS is an inherited disorder characterised by reduced bone marrow platelet production, skeletal deformities affecting radii and other limbs; cardiac, renal, and other heterogeneous anomalies may occur. It is caused by co-inheritance of a microdeletion and a nucleotide polymorphism in the RBM8A gene on chromosome 1.
Bleeding phenotype is more severe than platelet numbers might predict especially in infants but improves with age. There is minimal literature regarding impact in pregnancy and puerperium.
We describe management of three pregnancies in the haematology-obstetrics clinic. As platelet counts normally decrease through pregnancy, close monitoring is required in TAR syndrome. No major bleeding was seen antenatally but two required platelet transfusion during labour. No other treatment definitely improves bleeding, although case reports of steroids claim variable success.
Tranexamic acid may be helpful, and thrombopoietin agonists represent a potential future option.
Delayed penile prosthesis implantation in the delayed presentation of ischemic priapism
Published on: 6th February, 2020
OCLC Number/Unique Identifier: 8541455754
Priapism is currently defined as a prolonged painful erection without sexual stimuli, if priapism persists > 36 hours, conservative treatment does not lead to detumescence.
The most common etiologies are: medications into corpora cavernosa, anti-psychotics, anti-hypertensives, hematological disorders (chronic myeloid leukemia).
The Penile prosthesis is the choice treatment for restoring the erectile function after the failure of the conservative treatment.
But the corporal fibrosis makes it very difficult, with high risk of complications [1].
Applications to reduce the amount of excess cement for cement-retained implant supported prostheses: Mini review
Published on: 12th June, 2020
OCLC Number/Unique Identifier: 8620529291
Cement-retained implant-supported restorations has been preferred by many clinicians due to its ease of production, low cost and similarity to dental supported restorations [1]. In the literature, many complications caused by residual cement, ranging from acute severe bone resorption to implant loss, have been published as a case report/ series [2-7]. In another study [8], residual cement was seen in 81% of implant cases that are clinically identified as peri- implantitis. Hence it has been indicated that a strong relationship has been determined between residual cement and development of chronic peri-implant infection [9].
Only low intensity of aerobic exercise improves respiratory compliance in pulmonary hypertensive rats
Published on: 20th November, 2019
OCLC Number/Unique Identifier: 8440622691
Objective: To investigate in an animal model of Pulmonary Hypertension (PH) by monocrotaline whether a lower exercise intensity, which has lower potential to provoke dyspnea symptoms, could prevent the increase the right ventricle pressure and the decrease in respiratory compliance.
Setting: A research laboratory. ANIMALS: twenty-one Wistar rats were randomized to the groups: Control (CO; saline solution); PH-sedentary; PH-low and PH-moderate intensity of exercise training (ET).
Interventions: They received a single saline or monocrotaline subcutaneous injection (50 mg/kg). The exercise program was performed during 3-weeks.
Main Outcome Measures: Rats were evaluated by their morphometric and hemodynamic changes and by the respiratory mechanic responses induced by the exercise protocols.
Results: Both protocols of ET significantly (p < 0.05) attenuated the increase in the right ventricular systolic pressure. However, the lower intensity was more effective to prevent the impairment in the respiratory and quasi-static compliance.
Conclusion: Collectively, our results showed for the first time the benefits of ET to the respiratory system mechanics. We also demonstrated that intensity is crucial in PH, probably due to the difficulty to match VO2 capacity and O2 demand during exercise. The improvement in quasi-static compliance not only might improve the ability to breathe, and capture oxygen, but also welfare.
Depression and anxiety in patients with suspected renal artery stenosis
Published on: 6th September, 2021
OCLC Number/Unique Identifier: 9244771644
Objective: To investigate the occurrence of depressive and anxiety disorders in patients remitted for endovascular treatment of renal artery stenosis (RAS), and whether symptoms of depression and anxiety can be reduced after the treatment of RAS with percutaneous transluminal renal angioplasty (PTRA).Methods Patients: Selected for renal angiography with pressure measurement in the renal arteries due to suspected RAS were invited to participate in the study. 37 patients agreed to answer a questionnaire. Eighteen patients also agreed to a clinical psychiatric examination including the International Neuropsychiatric Interview (M.I.N.I. 6.0) to be diagnosed according to the DSM-IV. Results: Twenty-one of the 37 patients completing the questionnaire were found to have a pressure gradient of ≥ 10 mmHg at angiography, indicating significant stenosis. Ten of the 18 examined patients met the criteria for current DSM-IV anxiety diagnosis and two patients had a current depression. Three of the 18 examined patients had previously made a suicide attempt. Conclusion: The results of this study suggest that patients with suspected RAS have a high psychiatric comorbidity, as 56% of the patients undergoing psychiatric examination met the DSM-IV criteria for a current anxiety disorder.
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