scholarly journals Clinical and Paraclinical Features of Wilson’s Disease in Children in Shiraz, Southern Iran

2014 ◽  
Vol 3 (3) ◽  
pp. 160-66
Author(s):  
Seyed Mohsen Dehghani ◽  
Asma Erjaee ◽  
Mohammad Hadi Imanieh ◽  
Mahmood Haghighat ◽  
Zohre Bajalli ◽  
...  
Keyword(s):  
Medical Records ◽  
Autosomal Recessive ◽  
Clinical Findings ◽  
Medical Sciences ◽  
Progressive Degeneration ◽  
Urinary Copper ◽  
The Mean ◽  
High Index Of Suspicion ◽  
Biochemical Abnormalities

Background: Wilson disease (WD) is an autosomal recessive progressive degeneration of hepatolenticular tissue that causes the increase of copper deposition in the liver and other organs, with resultant hepatic, neurologic and psychological manifestations. WD is fatal if left untreated. The aim of the current study was to evaluate the clinical and Para-clinical findings in children with WD in Shiraz, Southern Iran.Patients and Methods: The Medical records of all children less than 18 years of age with definite diagnosis of WD, who were admitted in Nemazee Teaching Hospital from 2001 to 2009, or were under follow up at the Pediatric Hepatology Clinic affiliated to Shiraz University of Medical Sciences, were reviewed.Results: Overall, 70 patients with WD (41 males, 29 females) were studied. The mean age at the onset of diagnosis was 10.3±3.2 years and the most common first presentation in our patients was hepatic (90%). The most common biochemical abnormalities were increased urinary copper content, increased liver enzymes (92.9%), and increased prothrombin time (71.4%). Wilson index was ≥11 in 44.3% of the patients.Conclusion: WD is a rare and fascinating disorder that often poses a diagnostic and therapeutic challenge for the physician. Maintaining a high index of suspicion is critical in diagnosing this readily treatable disease and early treatment can decrease its mortality rate.

scholarly journals Clinical Characteristics of Intermittent Esotropia

2021 ◽  
Vol 62 (12) ◽  
pp. 1650-1656
Author(s):  
Young Chae Yoon ◽  
Nam Yeo Kang
Keyword(s):  
Medical Records ◽  
Clinical Characteristics ◽  
Age At Onset ◽  
Clinical Findings ◽  
Main Symptom ◽  
Angle Of Deviation ◽  
The Mean ◽  
Onset Angle ◽  
Dissociated Vertical Deviation

Purpose: To evaluate clinical findings and surgical outcomes of intermittent esotropia.Methods: We retrospectively reviewed the medical records of 23 patients (aged 9-42 years) who presented with intermittent esotropia between January 2007 and December 2020. We analyzed the age at onset, angle of deviation, presence of symptomatic diplopia, fusional state, near stereoacuity, and surgical results.Results: The mean age at onset was 19.9 ± 8.0 years and mean duration of esodeviation was 23.4 ± 27.1 months. Mean follow-up time was 13.0 ± 15.6 months. All patients except three had symptomatic diplopia, and 78.3% showed intermittent diplopia. Mean angles of deviation were 21.6 ± 10.3 prism diopters (PD) at distance and 20.2 ± 10.4 PD at near. Twenty-one patients (91.3%) were myopia. No patients had amblyopia and one had dissociated vertical deviation. On Worth’s four-dot test, five patients (21.7%) showed diplopia and four showed suppression at both distance and near. The other patients showed fusion at near or distance. Eleven patients underwent surgical correction. At the final examination, all patients achieved successful motor alignment and fusion with resolution of diplopia. Only two patients (18.2%) achieved normal 60 arcsec stereopsis, and six attained subnormal stereopsis.Conclusions: The main symptom of intermittent esotropia was diplopia. Surgical treatment was effective in achieving good postoperative motor alignment and fusion. However, successful motor alignment did not guarantee recovery of fine stereopsis.

PTEN hamartoma tumour syndrome: early tumour development in children

2014 ◽  
Vol 100 (1) ◽  
pp. 34-37 ◽  
Author(s):  
Patroula Smpokou ◽  
Victor L Fox ◽  
Wen-Hann Tan
Keyword(s):  
Renal Cell Carcinoma ◽  
Medical Records ◽  
Thyroid Nodules ◽  
Clinical Manifestations ◽  
Clinical Findings ◽  
Granulosa Cell Tumour ◽  
Tumour Development ◽  
Age Of Diagnosis ◽  
The Mean

ObjectiveThe aim of this study was to report the earliest age of diagnosis of common clinical findings in children with PTEN hamartoma tumour syndrome (PHTS).DesignMedical records of children with PHTS were reviewed; data included growth measurements, presence or absence of specific clinical manifestations and tumours, and documented ages of diagnosis.SettingChildren with PHTS evaluated at Boston Children's Hospital from 1996 to 2011.PatientsThe cohort included 34 children diagnosed with PHTS via genetic testing, under the age of 21 years. Of these, 23 were male and 11 female. The mean age at their last documented clinical evaluation was 13.6 years. The mean follow-up time was 7.5 years.ResultsMacrocephaly and developmental/intellectual disability were consistent findings. Pigmented penile macules were noted in all males examined for this finding. Thyroid nodules, found in half the children screened with ultrasound, were diagnosed as early as at 5 years of age. Thyroid carcinoma, identified in 12% of the children in this cohort, was diagnosed as early as at 7 years of age. Other tumours included renal cell carcinoma diagnosed at 11 years of age and granulosa cell tumour of the ovary and colonic ganglioneuroma, each diagnosed at 16 years of age.ConclusionsSpecific clinical findings and tumours are characteristic in children with PHTS. Tumour development occurs in young children with this condition, which necessitates early surveillance, especially of the thyroid.

Corneal densitometry in patients with keratoconus undergoing intrastromal Ferrara ring implantation

2021 ◽  
pp. 112067212110206
Author(s):  
Pablo Felipe Rodrigues ◽  
Bernardo Kaplan Moscovici ◽  
Guilherme Ferrara ◽  
Luciano Lamazales ◽  
Marcela Mara Silva Freitas ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Medical Records ◽  
Spherical Equivalent ◽  
Interventional Study ◽  
Data Comparison ◽  
Corneal Densitometry ◽  
Corrected Visual Acuity ◽  
Ring Segment ◽  
The Mean

Objective: Evaluation of central corneal densitometry changes following Ferrara corneal ring segment implantation in patients with keratoconus, especially the correlation between corneal densitometry and keratometry. Methods: Retrospective, non-comparative, interventional study based on the review of medical records of patients diagnosed with keratoconus who underwent Ferrara corneal ring segment implantation. Pre and post-operative corneal densitometry measurements obtained with Pentacam HR (Oculus, Wetzlar, Germany) were analyzed. The follow-up time was 3 months, and data comparison was made, using specific statistical analysis, with the data of 3 months postoperatively. Results: The study sample consisted of 43 eyes of 36 patients. The mean corrected visual acuity improved from 0.82 LogMAR preoperatively (SD ± 0.33) to 0.19 LogMAR (SD ± 0.13) postoperatively. The mean spherical equivalent varied from −4.63 (SD ± 3.94) preoperatively to −2.16 (SD ± 2.63) postoperatively. Asphericity varied from −0.69 (SD ± 0.32) preoperatively to −0.27 (SD ± 0.31) postoperatively. The mean maximum K was 54.01D (SD ± 3.38) preoperatively and 51.50D (SD ± 2.90) postoperatively. The mean anterior densitometric value was 18.26 (SD ± 2.03) preoperatively and 17.66 (SD ± 1.84) postoperatively. Conclusion: Corneal densitometry is an interesting technology that should be studied in keratoconus patients. Our results suggest that the corneal densitometry in the cornea’s anterior layer reduces after ICRS implantation and correlates with corneal keratometry. Further studies should be performed to increase the knowledge in this field.

scholarly journals Comparison of Intravitreal Aflibercept and Ranibizumab following Initial Treatment with Ranibizumab in Persistent Diabetic Macular Edema

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Ali Demircan ◽  
Zeynep Alkin ◽  
Ceren Yesilkaya ◽  
Gokhan Demir ◽  
Burcu Kemer
Keyword(s):  
Macular Edema ◽  
Diabetic Macular Edema ◽  
Medical Records ◽  
Poor Response ◽  
Intravitreal Aflibercept ◽  
The Mean ◽  
Retrospective Comparative Study ◽  
Switch Group ◽  
Persistent Diabetic Macular Edema

Purpose. To compare the visual and anatomic outcomes in patients with persistent diabetic macular edema (DME) who switched from ranibizumab to aflibercept with those who continued with previous ranibizumab therapy. Methods. In this retrospective comparative study, medical records of consecutive patients with center-involved DME ≥ 350 μm who had at least three recent consecutive monthly ranibizumab injections followed by as-needed therapy with either aflibercept or ranibizumab were reviewed. Data were collected at presentation (preinjection), at the intermediary visit, and at the last visit (at the end of the follow-up period). Results. Forty-three eyes of 43 patients were divided into two groups: the switch group (n=20) and the ranibizumab group (n=23). Though no significant improvement was found in the mean BCVA from the intermediary visit to the last visit, there was a difference in the mean CMT in the switch group and the ranibizumab group (p<0.001 and p=0.03, resp.). The mean CMT decreased after the intermediary visit by 188.6 ± 120.5 μm in the switch group and by 60.3 ± 117.1 μm in the ranibizumab group (p=0.003). Conclusions. Both aflibercept and ranibizumab decreased CMT in patients with persistent DME who showed a poor response to ranibizumab injections. However, switching to aflibercept provided only morphologic improvement.

Thoracolumbar Fascia Enthesopathy as a Cause of Low Back Pain: A Retrospective and Follow-up Study

2021 ◽  
Author(s):  
Ouidade A. Tabesh ◽  
Roba Ghossan ◽  
Soha H Zebouni ◽  
Rafic Faddoul ◽  
Michel Revel ◽  
...  
Keyword(s):  
Low Back Pain ◽  
Back Pain ◽  
Iliac Crest ◽  
Pain Syndrome ◽  
Clinical Findings ◽  
Complete Relief ◽  
Low Back ◽  
Thoracolumbar Fascia ◽  
The Mean

Abstract Aim. To evaluate ultrasonography findings of Thoracolumbar Fascia (TLF) enthesis in patients with low back pain (LBP) due to iliac crest pain syndrome (ICPS). Method. The ultrasonographic and clinical findings of 60 patients with LBP due to ICPS were compared to those of 30 healthy volunteers with no LBP. Thickness of the TLF was measured with ultrasound (US) at its insertion on the iliac crest. Results. Forty-eight women and 12 men with a mean age of 42.1±11.3 years were diagnosed with ICPS. In patients, the mean thickness of the TLF was 2.51±0.70mm in affected sides compared to 1.81±0.44mm in the contralateral unaffected sides. The mean thickness difference of 0.82mm between the affected and non-affected sides was statistically significant (95%CI, 0.64-0.99, P<0.0001). In volunteers, the mean thickness of the TLF was 1.6±0.2mm. The mean thickness difference of 0.89mm between the affected sides of patients and volunteers was statistically significant (95%CI, 0.73-1.06, P<0.0001). Forty-two patients who didn’t improve with conservative therapy, received injections of methylprednisolone acetate and 1% lidocaine around the TLF enthesis. All patients reported complete relief of their LBP within 20 minutes of the injections thanks to the lidocaine anesthetic effect. Fifty-six (93.3%) patients were reached by phone for a long-term follow-up. Among them, 33 (58.9%) patients experienced a sustained complete pain relief after a mean follow-up of 45±19.3 months (range, 3-74 months). Conclusion. our findings suggest that TLF enthesopathy is a potential cause of nonspecific LBP that can be diagnosed using US.

scholarly journals Evaluation of endometrial thickness in postmenopausal women by using 3.0-T MRI

2014 ◽  
Vol 18 (1) ◽  
Author(s):  
Junko Nakamura ◽  
Takeharu Yoshikawa ◽  
Eriko Maeda ◽  
Hiroyuki Akai ◽  
Hiroshi Ohtsu ◽  
...  
Keyword(s):  
Postmenopausal Women ◽  
Medical Records ◽  
Endometrial Thickness ◽  
Mr Images ◽  
Endometrial Thickening ◽  
3.0 T ◽  
The Mean ◽  
Magnetic Resonance Imaging Mri ◽  
3.0 T Mri

Background: The accepted threshold for normal endometrial thickness is 5 mm; lesions with endometrial thickness < 5 mm are considered benign, whilst those > 5 mm areconsidered malignant. However, endometrium ≥ 5 mm on transvaginal ultrasonography inpostmenopausal woman is considered as asymptomatic endometrial thickening. However, recent studies suggest that asymptomatic endometrial thickness of even 8 mm – 11 mm in postmenopausal women may be normal.Objectives: The present study investigated the normal endometrial thickness range in 297 asymptomatic postmenopausal women using 3.0-T magnetic resonance imaging (MRI) T2-weighted sagittal images measured retrospectively by a single radiologist.Method: The data were classified according to patient age and postmenopausal duration, and the medical records and follow-up MR images were reviewed to assess the clinical outcome.Results: The mean endometrial thickness was 2.4 ± 0.1 mm (range: 0.1–11.6). The endometriumin 21 of 297 subjects was ≥ 5 mm thick. Follow-up MR images were obtained in 17 of these 21 women, and their endometrial thickness was found to have decreased in all of them. To date,none of the subjects has been diagnosed with endometrial cancer.Conclusion: Although 5 mm is considered the conservative threshold of normal endometrial thickness on MRI of postmenopausal women, this figure should not, to avoid excessive false-positive diagnoses, be assumed as an indication of malignancy.

scholarly journals Surgical outcomes of acute acquired comitant esotropia of adulthood

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Dae Hyun Kim ◽  
Ha Jeong Noh
Keyword(s):  
Success Rate ◽  
Surgical Outcomes ◽  
Medical Records ◽  
Large Angle ◽  
Sudden Onset ◽  
Prism Diopter ◽  
Factors Affecting ◽  
The Mean ◽  
Motor Outcomes

Abstract Background Acute acquired comitant esotropia (AACE) is a type of strabismus characterized by a sudden onset of large angle esotropia with diplopia, which often occurs in children after infancy, teenagers, and young adolescents. However, studies on the surgical outcomes of only adults are rare. The purpose of this article is to analyze the surgical outcomes for adult patients diagnosed with AACE. Methods Medical records of 24 patients who had undergone surgery for AACE were retrospectively analyzed. The main outcome measures were the final motor and sensory success rate after surgery and factors affecting motor and sensory outcomes. Motor success was considered alignment within 8 prism diopter (PD) at both near and distance and sensory success was stereoacuity ≥ 60 sec/arc. Results The preoperative mean esodeviation angles were 33.1 ± 10.4 PD at distance and 33.3 ± 11.2 PD at near. The mean period of postoperative follow up was 7.5 ± 4.5 months (range 1–8 months). The postoperative mean esodeviation angles at final follow-up time were 3.4 ± 6.1 PD at distance and 3.8 ± 6.7 PD at near. The surgical motor success rate at final follow-up was 79.2% (19/24). The sensory success rate at final follow-up was 50.0% (12/24). The factor affecting the motor outcome was the type of surgery (p < 0.05). The factor affecting sensory outcome was postoperative follow-up time (p < 0.05). Conclusions Surgery type appears to affect surgical motor outcomes in adults with AACE. Although the sensory outcome was favorable, it seems that regaining bifoveal fixation takes time.

scholarly journals The effect of amblyopia on clinical outcomes of children with astigmatism

2021 ◽  
Vol 13 ◽  
pp. 251584142110408
Author(s):  
Burçin Çakır ◽  
Nilgün Özkan Aksoy ◽  
Sedat Özmen ◽  
Özlem Bursalı
Keyword(s):  
Visual Acuity ◽  
Clinical Outcomes ◽  
Medical Records ◽  
Convergence Insufficiency ◽  
Corrected Visual Acuity ◽  
Ocular Deviation ◽  
The Mean ◽  
The Difference ◽  
Best Corrected Visual Acuity

Background: Amblyopia is more common in children with high astigmatism, but factors contributing to development of amblyopia and visual outcomes are not fully understood. Objective: To evaluate the effect of amblyopia on the clinical outcomes in children with ⩾1.75 diopter (D) astigmatism. Methods: We reviewed the medical records of children with ⩾1.75 D astigmatism with and without amblyopia (amblyopes group and non-amblyopes group). The mean age, gender, amount and type of ocular deviation, presence of convergence insufficiency (CI), stereopsis, time of initial spectacle use and follow-up time, differences in best-corrected visual acuity (VoD) and spherical equivalent (SE) between eyes were assessed and compared between the groups. Best-corrected visual acuity (BCVA), mean SE, astigmatism measurements were assessed and compared between amblyopic, fellow, and non-amblyopic eyes. Results: The records included 68 eyes of 34 children with amblyopia and 56 eyes of 28 children without amblyopia. The mean age, gender, amount and type of ocular deviation, presence of CI, stereopsis, time of initial spectacle use, follow-up time, and the difference in SE did not differ between groups. In amblyopes, exodeviation was more common and statistically greater in near (33 cm) than at distance (6 m) (p = 0.005). The mean BCVA and astigmatism values were statistically different between amblyopic, fellow, and non-amblyopic eyes. Conclusion: A greater near than distance exodeviation and higher mean astigmatism value were found in amblyopic children with astigmatism.

Tear Rates of the Ipsilateral ACL Graft and the Contralateral Native ACL Are Similar following ACL Reconstruction

2020 ◽  
Author(s):  
Nadia Nastassia Ifran ◽  
Ying Ren Mok ◽  
Lingaraj Krishna
Keyword(s):  
Acl Reconstruction ◽  
Medical Records ◽  
Cruciate Ligament ◽  
Acl Graft ◽  
Increased Risk ◽  
Significant Difference ◽  
The Mean ◽  
Anterior Cruciate ◽  
Mean Time

AbstractThe aim of the study is to compare the tear rates of ipsilateral anterior cruciate ligament (ACL) grafts and the contralateral native ACL as well as to investigate the correlation of gender, age at time of surgery, and body mass index (BMI) with the occurrence of these injuries. The medical records of 751 patients who underwent ACL reconstruction surgery with follow-up periods of 2 to 7 years were retrospectively analyzed. Survival analyses of ipsilateral ACL grafts and contralateral native ACL were performed. Univariate and multivariate logistic regression analyses were performed to identify risk factors that were associated with these injuries. The tear rates of the ipsilateral ACL graft and contralateral ACL were 5.86 and 6.66%, respectively with no significant difference between groups (p = 0.998). The mean time of tears of the ipsilateral ACL and contralateral ACL was also similar (p = 0.977) at 2.64 and 2.78 years, respectively after surgery. Both the odds of sustaining an ipsilateral ACL graft and contralateral ACL tear were also significantly decreased by 0.10 (p = 0.003) and 0.14 (p = 0.000), respectively, for every 1-year increase in age at which the reconstruction was performed. However, graft type, gender, and BMI were not associated with an increased risk of these injuries. There was no difference between tear rates of ipsilateral ACL graft and contralateral ACL following ACL reconstruction. Patients who undergo ACL reconstruction at a young age are at an increased risk of both ipsilateral graft and contralateral ACL rupture after an ACL reconstruction. Patients who are young and more likely to return to competitive sports should be counselled of the risks and advised to not neglect the rehabilitation of the contralateral knee during the immediate and back to sports period of recovery. This is a Level III, retrospective cohort study.

Fibromuscular Dysplasia of the Internal Carotid Artery

Neurosurgery ◽  
1982 ◽  
Vol 10 (1) ◽  
pp. 39-43 ◽  
Author(s):  
Ralph P. Wells ◽  
Robert R. Smith
Keyword(s):  
Carotid Artery ◽  
Internal Carotid Artery ◽  
Medical Records ◽  
Fibromuscular Dysplasia ◽  
Internal Carotid ◽  
Clinical Course ◽  
Oral Anticoagulants ◽  
Specific Therapy ◽  
The Mean

Abstract The natural course of fibromuscular dysplasia (FMD) of the internal carotid artery (ICA). a stenosing angiopathy associated with cerebrovascular insufficiency, has not been described. A search of medical records located 16 female patients with angiographically demonstrated FMD of the ICA. The identical twin of 1 patient was included in the registry on the basis of noninvasive studies consistent with FMD of the ICA. The mean age at diagnosis was 58 years. Follow-up examinations were performed an average of 3.8 years after diagnosis (range, 1 to 9 years); the evaluation included clinical, angiographic, and Doppler studies. Fifteen patients showed no evidence of progression of FMD, whereas 2 patients with coincident atherosclerotic disease had suffered strokes. One patient had undergone surgical dilatation of the ICA. 3 had received oral anticoagulants, and 13 had received either aspirin or no specific therapy. In light of the apparently benign clinical course of uncomplicated FMD of the ICA, it is concluded that dilatation is rarely warranted.

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