scholarly journals A Rare Case of Congenital Methemoglobinemia with secondary polycythemia- Case Report and Literature Review

2019 ◽  
Vol 3 (01) ◽  
pp. 20-23
Author(s):  
Tasneem Ara ◽  
Qazi Smita Haque ◽  
Salma Afrose
Keyword(s):  
Skin Color ◽  
Rare Case ◽  
Heart Diseases ◽  
Final Diagnosis ◽  
Enzyme Deficiency ◽  
Type I ◽  
Methemoglobin Level ◽  
Hands And Feet ◽  
First Time

Congenital heart diseases are common cause of congenital cyanosis with polycythaemia. Congenital methemoglobinemia is a rare cause of lifelong cyanosis with polycythemia. Congenital methemoglobinemia is caused either by enzyme deficiency or by an abnormal Hb (Hb M). Asymptomatic despite presence of severe cyanosis indicates this rare disorder. We are reporting a rare case of polycythemia with cyanosis due to congenital methemoglobinemia. The patient was referred to our centre and attended Hematology OPD (out-patient department) when his routine CBC revealed erythrocytosis. At that time, we found him severely cyanosed especially apparent on lips, tongue, hands and feet. He was diagnosed as a case of congenital methemoglobinemia with 38% blood methemoglobin level (normal value-0.00-2.00%). On view of life long persistent cyanosis, without any cardiopulmonary and neurological abnormality, consanguinity of parent’s marriage, dark colored blood with high methemoglobin level, a final diagnosis of Type I enzyme deficiency congenital methemoglobinemia was made. He was treated with oral ascorbic acid 250 mg twice daily. At follow up after 6 months his skin color improved and RBC count returned to normal. We are reporting this case of congenital methemoglobinemia for the first time in Bangladesh to emphasize the importance of this rare entity in the differential diagnosis of asymptomatic cyanosis with polycythemia.

scholarly journals A rare case of a digital papillary carcinoma of the hand with secondary conservative management

2020 ◽  
Vol 2020 (1) ◽  
Author(s):  
Rabeet Khan ◽  
Renu Irri ◽  
Effie Katsarma
Keyword(s):  
Early Detection ◽  
Papillary Carcinoma ◽  
Management Strategy ◽  
Rare Case ◽  
Histological Analysis ◽  
Significant Risk ◽  
Sweat Glands ◽  
Hands And Feet ◽  
Palm Of The Hand

Abstract A digital papillary carcinoma (DPC) is a cancer of the sweat glands which develops on the digits of the hands and feet. With an incidence of 0.08 per 1 000 000 people/year, it is often initially misdiagnosed as a simple ganglion although it has a significant risk of metastasis. Histological analysis is beneficial in the formal diagnosis of DPCs. In this report, we present a rare case of DPC affecting the palm of the hand, rather than the more commonly reported DPC of the digits. We discuss the management strategy utilized in this case and the various prognostic considerations taken into account when deciding on an amputation versus local excision surgical approach. All cases of DPC require regular follow up imaging for early detection of recurrence or new metastases to maximize prognosis.

scholarly journals A Case of Chronic Ectopic Pregnancy Manifested by Rectal Bleeding

2017 ◽  
Vol 2017 ◽  
pp. 1-3 ◽  
Author(s):  
Nina Vukas Radulovic ◽  
Maria Bullarbo ◽  
Erling Ekerhovd
Keyword(s):  
Abdominal Pain ◽  
Ectopic Pregnancy ◽  
Rectal Bleeding ◽  
Rare Case ◽  
Final Diagnosis ◽  
Tubal Pregnancy ◽  
Low Levels

Ectopic pregnancy resulting in perforation of the rectum and rectal bleeding is clinically rare. We report an extremely rare case of chronic ectopic pregnancy with decreasing low levels of serum β-HCG resulting in rectal bleeding. A 31-year-old woman, gravida 3, para 3, with moderate abdominal pain and rectal bleeding was diagnosed with a tubal pregnancy. The tube was adherent to the rectum. Following salpingo-oophorectomy, the perforation of the rectum was sutured. Biopsies from the rectum as well as the tube confirmed chronic ectopic pregnancy. This case illustrates that diagnosing ectopic pregnancy is sometimes extremely challenging and it underlines the importance of follow-up consultations when the final diagnosis has not yet been reached.

scholarly journals Crossed complete fused left to right renal ectopia with solitary left ureter: A rare ‘Case Report’

2020 ◽  
Vol 26 (1) ◽  
Author(s):  
Ghanshyam Kumawat ◽  
Mohit Singla ◽  
Nishkarsh Mehta ◽  
Prashant Gupta ◽  
SS Yadav
Keyword(s):  
Urinary Bladder ◽  
Rare Case ◽  
Adolescent Male ◽  
Ct Urography ◽  
Renal Ectopia ◽  
Rare Case Report ◽  
Crossed Fused Renal Ectopia ◽  
General Physical ◽  
First Time

Abstract Background Crossed fused renal ectopia is a rare congenital anomaly where both the kidneys are fused and situated on one side and drain bilaterally into the urinary bladder. Rarely crossed fused ectopia can present with a single ureter draining ipsilaterally into the bladder, but here we are presenting for the first time a rare case of right crossed fused ectopia, where a solitary ureter crosses midline and drains contralaterally into the bladder. Case presentation A 19-year-old adolescent male patient presented with intermittent chronic right flank pain for 3 months. General physical and per abdominal examinations were normal. Computerized tomography (CT) urography showed right side crossed fused ectopic kidneys with a solitary ureter draining both the kidneys and opening into the left side of the urinary bladder. The patient was managed conservatively and is on regular follow-up. Conclusion Crossed complete renal fused ectopia with solitary contralateral ureteral is a rare case. Individualized case-based management is needed depending upon the symptoms, associated anomalies, and pathology. Reporting of all rare congenital cases will help in the future understanding and management of these conditions.

Psychological Resilience Provides No Independent Protection From Suicidal Risk

Crisis ◽  
2016 ◽  
Vol 37 (2) ◽  
pp. 130-139 ◽  
Author(s):  
Danica W. Y. Liu ◽  
A. Kate Fairweather-Schmidt ◽  
Richard Burns ◽  
Rachel M. Roberts ◽  
Kaarin J. Anstey
Keyword(s):  
Risk Factors ◽  
Well Being ◽  
Current Status ◽  
Psychological Resilience ◽  
Cross Sectional ◽  
Life Project ◽  
Resilience Factors ◽  
First Time

Abstract. Background: Little is known about the role of resilience in the likelihood of suicidal ideation (SI) over time. Aims: We examined the association between resilience and SI in a young-adult cohort over 4 years. Our objectives were to determine whether resilience was associated with SI at follow-up or, conversely, whether SI was associated with lowered resilience at follow-up. Method: Participants were selected from the Personality and Total Health (PATH) Through Life Project from Canberra and Queanbeyan, Australia, aged 28–32 years at the first time point and 32–36 at the second. Multinomial, linear, and binary regression analyses explored the association between resilience and SI over two time points. Models were adjusted for suicidality risk factors. Results: While unadjusted analyses identified associations between resilience and SI, these effects were fully explained by the inclusion of other suicidality risk factors. Conclusion: Despite strong cross-sectional associations, resilience and SI appear to be unrelated in a longitudinal context, once risk/resilience factors are controlled for. As independent indicators of psychological well-being, suicidality and resilience are essential if current status is to be captured. However, the addition of other factors (e.g., support, mastery) makes this association tenuous. Consequently, resilience per se may not be protective of SI.

If Looks Could Kill

2020 ◽  
Vol 3 ◽  
pp. 81-84
Author(s):  
Karen Chan
Keyword(s):  
Skin Color ◽  
Daily Rhythm ◽  
Significant Rate ◽  
Chinese Canadian ◽  
First Time ◽  
The Way

For me, rhythm means having consistency. The piece highlights my own experience with the disruption of my daily rhythm due to COVID-19. The first half shows my routine and interactions prior to COVID-19 while the second half shows my experiences in the present day. Prior to the virus, I had a day to day routine that was filled with noise. Everyday moved quickly and I established a daily rhythm. However, when COVID-19 spread, it changed everything. I felt like I didn’t have a routine anymore because I wasn’t allowed to go anywhere. Time was moving much slower and worst of all, xenophobia was growing at a significant rate. As a Chinese Canadian, this was the first time I truly felt the weight of the color of my skin. COVID-19 changed the way that I consistently assumed that the color of my skin wasn’t something that strangers would significantly care about. However, as I got on a bus, I unintentionally scared a woman simply because of my skin color. From that point, I knew that xenophobia would affect the way people perceived me everyday. The woman was scared of the virus— which in turn was scared of me—and I was scared that she would thwart her anger towards me because I am Chinese. If looks could kill, then the woman and I ironically both feared each other. Now, due to COVID-19, I am adapting to a new routine. A routine where the color of skin rings louder than any other sound.

Sustained normoglycemia in newly diagnosed type I diabetic subjects. Short-term effects and one-year follow-up

Diabetes ◽  
1984 ◽  
Vol 33 (10) ◽  
pp. 995-1001 ◽  
Author(s):  
K. Perlman ◽  
R. M. Ehrlich ◽  
R. M. Filler ◽  
A. M. Albisser
Keyword(s):  
Type I ◽  
Newly Diagnosed ◽  
Short Term ◽  
One Year ◽  
Short Term Effects

Radiological and clinical associations with scoliosis outcomes after posterior fossa decompression in patients with Chiari malformation and syrinx from the Park-Reeves Syringomyelia Research Consortium

2020 ◽  
Vol 26 (1) ◽  
pp. 53-59 ◽  
Author(s):  
Jennifer M. Strahle ◽  
Rukayat Taiwo ◽  
Christine Averill ◽  
James Torner ◽  
Jordan I. Gewirtz ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Chiari Malformation ◽  
Craniocervical Junction ◽  
Curve Progression ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Younger Age ◽  
Curve Magnitude ◽  
The Mean

OBJECTIVEIn patients with Chiari malformation type I (CM-I) and a syrinx who also have scoliosis, clinical and radiological predictors of curve regression after posterior fossa decompression are not well known. Prior reports indicate that age younger than 10 years and a curve magnitude < 35° are favorable predictors of curve regression following surgery. The aim of this study was to determine baseline radiological factors, including craniocervical junction alignment, that might predict curve stability or improvement after posterior fossa decompression.METHODSA large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and a syrinx (≥ 3 mm in width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°) in patients who underwent posterior fossa decompression and who also had follow-up imaging.RESULTSOf 825 patients with CM-I and a syrinx, 251 (30.4%) were noted to have scoliosis present at the time of diagnosis. Forty-one (16.3%) of these patients underwent posterior fossa decompression and had follow-up imaging to assess for scoliosis. Twenty-three patients (56%) were female, the mean age at time of CM-I decompression was 10.0 years, and the mean follow-up duration was 1.3 years. Nine patients (22%) had stable curves, 16 (39%) showed improvement (> 5°), and 16 (39%) displayed curve progression (> 5°) during the follow-up period. Younger age at the time of decompression was associated with improvement in curve magnitude; for those with curves of ≤ 35°, 17% of patients younger than 10 years of age had curve progression compared with 64% of those 10 years of age or older (p = 0.008). There was no difference by age for those with curves > 35°. Tonsil position, baseline syrinx dimensions, and change in syrinx size were not associated with the change in curve magnitude. There was no difference in progression after surgery in patients who were also treated with a brace compared to those who were not treated with a brace for scoliosis.CONCLUSIONSIn this cohort of patients with CM-I, a syrinx, and scoliosis, younger age at the time of decompression was associated with improvement in curve magnitude following surgery, especially in patients younger than 10 years of age with curves of ≤ 35°. Baseline tonsil position, syrinx dimensions, frontooccipital horn ratio, and craniocervical junction morphology were not associated with changes in curve magnitude after surgery.

Effectiveness of the Chiari Health Index for Pediatrics instrument in measuring postoperative health-related quality of life in pediatric patients with Chiari malformation type I

2020 ◽  
pp. 1-6
Author(s):  
Georgina E. Sellyn ◽  
Alan R. Tang ◽  
Shilin Zhao ◽  
Madeleine Sherburn ◽  
Rachel Pellegrino ◽  
...  
Keyword(s):  
Surgical Intervention ◽  
Type I ◽  
Health Related Quality ◽  
Health Index ◽  
Chiari Malformation Type I ◽  
Related Quality ◽  
Health Related ◽  
Over Time

OBJECTIVEThe authors’ previously published work validated the Chiari Health Index for Pediatrics (CHIP), a new instrument for measuring health-related quality of life (HRQOL) for pediatric Chiari malformation type I (CM-I) patients. In this study, the authors further evaluated the CHIP to assess HRQOL changes over time and correlate changes in HRQOL to changes in symptomatology and radiological factors in CM-I patients who undergo surgical intervention. Strong HRQOL evaluation instruments are currently lacking for pediatric CM-I patients, creating the need for a standardized HRQOL instrument for this patient population. This study serves as the first analysis of the CHIP instrument’s effectiveness in measuring short-term HRQOL changes in pediatric CM-I patients and can be a useful tool in future CM-I HRQOL studies.METHODSThe authors evaluated prospectively collected CHIP scores and clinical factors of surgical intervention in patients younger than 18 years. To be included, patients completed a baseline CHIP captured during the preoperative visit, and at least 1 follow-up CHIP administered postoperatively. CHIP has 2 domains (physical and psychosocial) comprising 4 components, the 3 physical components of pain frequency, pain severity, and nonpain symptoms, and a single psychosocial component. Each CHIP category is scored on a scale, with 0 indicating absent and 1 indicating present, with higher scores indicating better HRQOL. Wilcoxon paired tests, Spearman correlations, and linear regression models were used to evaluate and correlate HRQOL, symptomatology, and radiographic factors.RESULTSSixty-three patients made up the analysis cohort (92% Caucasian, 52% female, mean age 11.8 years, average follow-up time 15.4 months). Dural augmentation was performed in 92% of patients. Of the 63 patients, 48 reported preoperative symptoms and 42 had a preoperative syrinx. From baseline, overall CHIP scores significantly improved over time (from 0.71 to 0.78, p < 0.001). Significant improvement in CHIP scores was seen in patients presenting at baseline with neck/back pain (p = 0.015) and headaches (p < 0.001) and in patients with extremity numbness trending at p = 0.064. Patients with syringomyelia were found to have improvement in CHIP scores over time (0.75 to 0.82, p < 0.001), as well as significant improvement in all 4 components. Additionally, improved CHIP scores were found to be significantly associated with age in patients with cervical (p = 0.009) or thoracic (p = 0.011) syrinxes.CONCLUSIONSThe study data show that the CHIP is an effective instrument for measuring HRQOL over time. Additionally, the CHIP was found to be significantly correlated to changes in symptomatology, a finding indicating that this instrument is a clinically valuable tool for the management of CM-I.

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