Ophthalmic Evaluation of Diagnosed Cases of Eye Cystinosis: A Tertiary Care Center’s Experience

Background: We aimed to identify diagnosed cases of ocular cystinosis and describe clinical, epidemiological and therapeutic characteristics. Methods: This is a descriptive and retrospective case series. All patients underwent a full check-up examination every 4–6 months by ophthalmologists, nephrologists and other required specialists. Results: Of the seven cases, six (85.7%) were females and one (14.2%) was male. The infantile nephropathic form of cystinosis was observed in five patients and the juvenile nephropathic form in two patients. No patients with the ocular form of cystinosis were identified. Corneal cystine crystals (CCC) were found in all analyzed patients. Severe ocular and general complications of the disease that had been standing for years, connected to the infantile nephropathic form, delayed diagnosis or inappropriate treatment, were observed only in two patients. All patients received topical therapy. No adverse events related to the therapy were observed. Conclusions: Cystinosis is a rare, progressive disease. Early diagnosis and treatment prevent serious complications from numerous systemic organs. Patients require constant systematic monitoring by various specialists.

Download Full-text

Tracheo-bronchial foreign bodies: our experience at a tertiary care hospital

International Journal of Otorhinolaryngology and Head and Neck Surgery ◽

10.18203/issn.2454-5929.ijohns20161848 ◽

2016 ◽

Author(s):

Shashidhar S. Suligavi ◽

Mallikarjun N. Patil ◽

S. S. Doddamani ◽

Chandrashekarayya S. Hiremath ◽

Afshan Fathima

Keyword(s):

Foreign Body ◽

Early Diagnosis ◽

Foreign Bodies ◽

Tertiary Care ◽

Case Series ◽

Team Work ◽

Care Hospital ◽

Retrospective Case ◽

Presenting Symptoms ◽

Case Series Study

Background:Tracheo- bronchial foreign bodies have always posed a challenge to the ENT surgeon as they present with varied symptomatology ranging from a simple cough and fever to more grave respiratory distress. It requires a strong suspicion, early diagnosis and timely intervention to reduce the overall morbidity and mortality .This study was undertaken to highlight our experiences in handling cases of tracheo- bronchial foreign bodies (FB) at our setup.Methods:It is a retrospective case series study conducted in S. Nijalingappa Medical College between January 2011 and January 2015. Results:Most commonly affected were children between 1year to 3years of age. Chronic cough and wheeze were the commonest presenting symptoms. Vegetative foreign body was found to be the commonest variety of foreign body. The mortality rate in our study was 4.7% (n=3).Conclusions:A good clinical acumen, team work, early diagnosis and timely intervention are all needed to reduce the overall mortality and morbidity associated with tracheo- bronchial foreign bodies.

Download Full-text

Skin Autoimmunity Secondary to Alemtuzumab in a Tertiary Care Spanish Hospital

Neurology Clinical Practice ◽

10.1212/cpj.0000000000001126 ◽

2021 ◽

pp. 10.1212/CPJ.0000000000001126

Author(s):

Rocío López Ruiz ◽

Félix Sánchez Fernández ◽

María Ruiz de Arcos ◽

Julio Dotor García-Soto ◽

Alejandro Fuerte Hortigón ◽

...

Keyword(s):

Adverse Events ◽

Tertiary Care Hospital ◽

Tertiary Care ◽

Case Series ◽

Autoimmune Disorders ◽

Care Hospital ◽

Retrospective Case ◽

Spanish Hospital ◽

Infusion Reactions

AbstractBackground and Objective:The most common adverse events following Alemtuzumab include adverse infusion reactions, infections and autoimmune disorders. Skin adverse events are common during infusion but there are few reported cases of long-term skin autoimmune disease.Methods:A retrospective case series of patients developing long-term autoimmune skin disorders following alemtuzumab administration in a tertiary care hospital.Results:Of 133 patients treated with alemtuzumab, eight patients (6.02%) developed nine autoimmune cutaneous adverse events, including four events of alopecia areata, two of vitiligo, two of chronic urticaria and one of inflammatory atrichia. Three of them occurred between the first and the second infusion.Discussion:The lesions described are secondary to autoimmune disorders, probably related to immune dysregulation due to a differential lymphocyte repopulation following alemtuzumab. Autoimmune cutaneous adverse events may be frequent, and it would be recommended to monitor its appearance in order to treat them.

Download Full-text

Peripartum hysterectomy: a five year review at a tertiary care centre

International Journal of Reproduction Contraception Obstetrics and Gynecology ◽

10.18203/2320-1770.ijrcog20173490 ◽

2017 ◽

Vol 6 (8) ◽

pp. 3590

Author(s):

Deepak A. V. ◽

K. J. Jacob ◽

Sumi P. Maria

Keyword(s):

Tertiary Care ◽

Case Series ◽

Case Fatality ◽

Tertiary Care Centre ◽

Retrospective Case ◽

College Hospital ◽

Peripartum Hysterectomy ◽

Medical College ◽

Obstetric Haemorrhage ◽

Suboptimal Care

Background: Peripartum hysterectomy is a life-saving procedure resorted to when conservative measures fail to control obstetric haemorrhage. Several predisposing factors, suboptimal care and lack of infrastructure may lead to this emergency procedure. We wanted to find out factors associated with peripartum hysterectomy and the adverse maternal outcomes at our centre.Methods: A retrospective case series analysis of 40 cases of peripartum hysterectomy performed over a period of 5 years from January 2010 to December 2014 at Government Medical College Hospital, Thrissur, Kerala was done.Results: The incidence of peripartum hysterectomy was 0.29%. The most common indication for peripartum hysterectomy was hysterectomy was uterine atony (50%). Thirty-five women (88%) were between 20 and 35 years. Most of the subjects were unbooked. There were two maternal deaths (case fatality rate of 5%) following peripartum hysterectomy during this period. All the subjects required blood transfusion.Conclusions: Prompt performance hysterectomy before the patient’s clinical condition deteriorates is the key to success. The incidence of adherent placenta is increasing, so every effort should be taken to reduce the caesarean section rates globally.

Download Full-text

Impact of SARS-COV-2 Pandemic on Kidney Cancer Management

Kidney Cancer ◽

10.3233/kca-210112 ◽

2021 ◽

pp. 1-14

Author(s):

Melissa Bersanelli ◽

Camillo Porta

Keyword(s):

Survival Rates ◽

Delayed Diagnosis ◽

Case Series ◽

Cost Benefit ◽

Original Data ◽

Immune Checkpoint Blockade ◽

Retrospective Case ◽

Renal Masses ◽

Term Survival ◽

Cancer Management

Background: The SARS-CoV-2 pandemic still has a huge impact on the management of many chronic diseases such as cancer. Few data are presently available reagarding how the management of renal cell carcinoma (RCC) has changed due to this unprecedented situation. Objective: To discuss the challenges and issues of the diagnosis and treatment of RCC in the COVID-19 era, and to provide recommendations based on the collected literature and our personal experience. Methods: Systematic review of the available Literature regarding the management of RCC during the SARS-CoV-2 pandemic. Results: Our review showed a prevalence of narrative publications, raising the issue of the real relevance of the evidence retrieved. Indeed, the only original data about RCC and COVID-19 found were a small retrospective case series and two surveys, providing either patients’ or physicians’ viewpoints. Conclusions: The expected delayed diagnosis of RCC could lead to an increase of advanced/metastatic cases; thus, proper therapeutic choices for patients with small renal masses should be carefully evaluated case by case, in order to avoid negative effects on long-term survival rates. The controversial interaction between immune checkpoint blockade and COVID-19 pathogenesis is more hypothetical than evidence-based, and thus immunotherapy should not be denied, whenever appropriate. To avoid treatments which won’t have an impact on patients’ survival, a honest and accurate evaluation of the cost/benefit ratio of each treatment option should be always performed. Finally, SARS-CoV-2 swab positivity should not prevent the continuation of ongoing active treatments in asymptomatic cases, or or after symptoms’ resolution.

Download Full-text

Safety of alemtuzumab in a nationwide cohort of Finnish multiple sclerosis patients

Journal of Neurology ◽

10.1007/s00415-021-10664-w ◽

2021 ◽

Author(s):

Ilkka Rauma ◽

Tiina Mustonen ◽

Juha Matti Seppä ◽

Maritta Ukkonen ◽

Marianne Männikkö ◽

...

Keyword(s):

Multiple Sclerosis ◽

Adverse Events ◽

Case Series ◽

Retrospective Case ◽

Serious Adverse Events ◽

Highly Active ◽

Disease Modifying Therapy ◽

Case Series Study ◽

Multiple Sclerosis Patients

Abstract Background Alemtuzumab is an effective disease-modifying therapy (DMT) for highly active multiple sclerosis (MS). However, safety concerns limit its use in clinical practice. Objectives To evaluate the safety of alemtuzumab in a nationwide cohort of Finnish MS patients. Methods In this retrospective case series study, we analyzed the data of all but two MS patients who had received alemtuzumab in Finland until 2019. Data were systematically collected from patient files. Results Altogether 121 patients were identified, most of whom had received previous DMTs (82.6%). Median follow-up time after treatment initiation was 30.3 months and exceeded 24 months in 78 patients. Infusion-associated reactions (IARs) were observed in 84.3%, 57.3%, and 57.1% of patients during alemtuzumab courses 1–3, respectively. Serious adverse events (SAEs) were observed in 32.2% of patients, serious IARs in 12.4% of patients, and SAEs other than IARs in 23.1% of patients. Autoimmune adverse events were observed in 30.6% of patients. One patient died of hemophagocytic lymphohistiocytosis, and one patient died of pneumonia. A previously unreported case of thrombotic thrombocytopenic purpura was documented. Conclusions SAEs were more frequent in the present cohort than in previous studies. Even though alemtuzumab is a highly effective therapy for MS, vigorous monitoring with a long enough follow-up time is advised.

Download Full-text

The Medially-Invasive Cholesteatoma: An Aggressive Subtype of a Common Pathology

Annals of Otology Rhinology & Laryngology ◽

10.1177/0003489420937731 ◽

2020 ◽

Vol 130 (1) ◽

pp. 38-46

Author(s):

Geoffrey Casazza ◽

Matthew L. Carlson ◽

Clough Shelton ◽

Richard K. Gurgel

Keyword(s):

Skull Base ◽

Tertiary Care ◽

Case Series ◽

Hearing Preservation ◽

Csf Leak ◽

Petrous Apex ◽

Otic Capsule ◽

Retrospective Case ◽

Presenting Symptoms ◽

Subtotal Petrosectomy

Objective: Describe the outcomes of treatment for patients with cholesteatomas that are medially invasive to the otic capsule, petrous apex, and/or skull base. Study Design: Retrospective case series Setting: Two tertiary care academic centers. Patients: Patients surgically managed for medially-invasive cholesteatoma at two tertiary care institutions from 2001 to 2017. Interventions: Surgical management of medially-invasive cholesteatomas. Main Outcome Measures: The presenting symptoms, imaging, pre- and post-operative clinical course, and complications were reviewed. Results: Seven patients were identified. All patients had pre-operative radiographic evidence of invasive cholesteatoma with erosion into the otic capsule beyond just a lateral semicircular canal fistula. Five patients had a complex otologic history with multiple surgeries for recurrent cholesteatoma including three with prior canal wall down mastoidectomy surgeries. Average age at the time of surgery was 41.3 years (range 20-83). Two patients underwent a hearing preservation approach to the skull base while all others underwent a surgical approach based on the extent of the lesion. Facial nerve function was maintained at the pre-operative level in all but one patient. No patient developed cholesteatoma recurrence. Conclusions: The medially-invasive cholesteatoma demonstrates an aggressive, endophytic growth pattern, invading into the otic capsule or through the perilabyrinthine air cells to the petrous apex. Surgical resection remains the best treatment option for medially-invasive cholesteatoma. When CSF leak is a concern, a subtotal petrosectomy with closure of the ear is often necessary.

Download Full-text

Transcanal Endoscopic Antrostomy Hole : Its Utility in Restoring Ventilation Pathway to Epitympanum and Mastoid Antrum During Tympanoplasty

Bangladesh Journal of Endosurgery ◽

10.3329/bje.v2i1.19588 ◽

2014 ◽

Vol 2 (1) ◽

pp. 9-14

Author(s):

Sheikh Shawkat Kamal

Keyword(s):

Middle Ear ◽

Tertiary Care ◽

Case Series ◽

Care Hospital ◽

Retrospective Case ◽

Middle Ear Mucosa ◽

Case Series Study ◽

Temporal Bones ◽

Mastoid Antrum

Aim: To evaluate the role of newly created transcanal endoscopic antrostomy hole as a passage in restoring the aeration of epitympanum and mastoid antrum.Materials & Methods: Study design: Retrospective case series study. Study duration: From January 2013 to January 2014. Study place: Private tertiary care hospital. Patients: 27 ears of 23 adult patients (age ranging from 15years to 54years) underwent transcanal endoscopic tympanoplasty for their chronic middle ear diseases in presence of inflamed middle ear mucosa along with radiological shadows of stagnant fluid in their epitympanum and mastoid antrum. Structured three months follow-up was ensured in every case. Intervention: A hole was created at a selected site of posterior meatal wall purely through transcanal endoscopic approach that established direct communication between mastoid antrum and external auditory canal. Thereafter this newly created passage was used for three purposes- assessment of the condition of mastoid antrum, performing the water test for checking epitympanic patency and in few cases placement of temporary tube for postoperative ventilation and drainage of middle ear. Main out come measures: The feasibility, performance and management of transcanal endoscopic antrostomy hole as passage for reestablishing the aeration of epitympanum and mastoid antrum.Results: The chosen site for antrostomy hole was found effective and safe in providing convenient entrance into the mastoid antrum in every case in this study with out facing technical complexity and failure. Postoperative healing of skin over antrostomy hole was found complete in all ears without any inward growth of skin in to mastoid antrum. Available post operative CT scan imaging of temporal bones showed improved aeration in their epitympanum and mastoid antrum.Conclusions: The role of transcanal antrostomy hole has been proved worthy in restoring ventilation pathway to epitympanum and mastoid antrum during tympanoplasty. This antrostomy hole has the potentiality to be considered in future for placement of long term mastoid ventilation tube in order to treat persistent atelectatic middle ear.

Download Full-text

Relationship between Drug-Induced Sleep Endoscopy Findings, Tonsil Size, and Polysomnographic Outcomes of Adenotonsillectomy in Children

Otolaryngology ◽

10.1177/0194599819860777 ◽

2019 ◽

Vol 161 (3) ◽

pp. 507-513 ◽

Cited By ~ 6

Author(s):

Derek J. Lam ◽

Natalie A. Krane ◽

Ron B. Mitchell

Keyword(s):

Rating Scale ◽

Tertiary Care ◽

Case Series ◽

Apnea Hypopnea Index ◽

Strongly Correlated ◽

Sleep Endoscopy ◽

Retrospective Case ◽

Drug Induced ◽

Failure Assessment ◽

The Relationship

Objective (1) Determine the correlation of awake tonsil scores and preadenotonsillectomy (pre-AT) sleep endoscopy findings. (2) Assess the relationship between polysomnographic AT outcomes with awake tonsil scores and sleep endoscopy ratings of tonsil and adenoid obstruction. Study Design Retrospective case series with chart review. Setting Tertiary care children’s hospital. Subjects and Methods Children aged 1 to 18 years who underwent sleep endoscopy and AT from January 1, 2013, to August 30, 2016, were included. Pre-AT sleep endoscopy findings were scored with the Sleep Endoscopy Rating Scale. Awake tonsil scores and sleep endoscopy ratings were compared with Spearman correlation. Associations between changes in pre- and post-AT polysomnography parameters and (1) awake tonsil scoring and (2) sleep endoscopy scoring were assessed with 1-way analysis of variance and linear regression. Results Participants included 36 children (mean ± SD age, 6.8 ± 4.3 years; 68% male, 44% obese). Awake tonsil scores and sleep endoscopy ratings were strongly correlated ( R = 0.58, P = .003). Awake tonsil scores were not associated with changes in any polysomnography parameters after AT (all P > .05), while sleep endoscopy ratings of adenotonsillar obstruction were significantly associated (all P < .05, R2 = 0.16-0.35). Patients with minimal adenotonsillar obstruction during sleep endoscopy had less improvement than those with partial or complete obstruction (mean obstructive apnea-hypopnea index change: −8.2 ± 11.5 vs −15.9 ± 14.3, and −46.8 ± 31.3, respectively; P < .001). Conclusions In children at risk for AT failure, assessment of dynamic collapse with sleep endoscopy may better predict the outcome of AT than awake tonsil size assessment, thus helping to inform surgical expectations.

Download Full-text

Over-under cartilage tympanoplasty: technique, results and a call for improved reporting

The Journal of Laryngology & Otology ◽

10.1017/s0022215120001978 ◽

2020 ◽

Vol 134 (10) ◽

pp. 875-881

Author(s):

I D Erbele ◽

M R Fink ◽

G Mankekar ◽

L S Son ◽

R Mehta ◽

...

Keyword(s):

Tertiary Care ◽

Case Series ◽

Senior Author ◽

Retrospective Case ◽

Closure Rate ◽

Hearing Results ◽

Middle Ear Cholesteatoma ◽

Cartilage Tympanoplasty ◽

The Mean ◽

Clinically Significant

AbstractObjectiveThis study aimed to describe the microscopic over-under cartilage tympanoplasty technique, provide hearing results and detail clinically significant complications.MethodThis was a retrospective case series chart review study of over-under cartilage tympanoplasty procedures performed by the senior author between January 2015 and January 2019 at three tertiary care centres. Cases were excluded for previous or intra-operative cholesteatoma, if a mastoidectomy was performed during the procedure or if ossiculoplasty was performed. Hearing results and complications were obtained.ResultsSixty-eight tympanoplasty procedures met the inclusion criteria. The median age was 13 years (range, 3–71 years). The mean improvement in pure tone average was 6 dB (95 per cent confidence interval 4–9 dB; p < 0.0001). The overall perforation closure rate was 97 per cent (n = 66). Revision surgery was recommended for a total of 6 cases (9 per cent) including 2 post-operative perforations, 1 case of middle-ear cholesteatoma and 3 cases of external auditory canal scarring.ConclusionOver-under cartilage tympanoplasty is effective at improving clinically meaningful hearing with a low rate of post-operative complications.

Download Full-text

Intralesional Methotrexate Treatment for Keratoacanthoma Tumors: A Retrospective Case Series

Journal of Cutaneous Medicine and Surgery ◽

10.1177/120347541201600316 ◽

2012 ◽

Vol 16 (3) ◽

pp. 212-217 ◽

Cited By ~ 11

Author(s):

Nicolas Aubut ◽

Jimmy Alain ◽

Joël Claveau

Keyword(s):

Adverse Events ◽

Total Dose ◽

Effective Treatment ◽

Complete Resolution ◽

Case Series ◽

Response Rates ◽

Initial Diagnosis ◽

Retrospective Case ◽

Methotrexate Treatment

Background: Intralesional methotrexate (IL-MTX) is an effective treatment for keratoacanthoma. Objective: We sought to determine the response rates and adverse events in KA treated with intralesional methotrexate. Methods: All cases of KA treated with intralesional methotrexate at our institutions from 2001 to 2009 were systematically reviewed. Results: Forty-six cases of KA treated with IL-MTX were identified. A complete resolution was achieved in 74% of patients, requiring an average of 1.8 injection sessions, for a mean total dose of 10 mg. Adverse events did not occur. Limitations: The follow-up period was short, and there was no histologic confirmation of the initial diagnosis. Conclusion: IL-MTX is an effective and well-tolerated alternative to surgery for the treatment of KA.

Download Full-text