Juvenile dermatomyositis in Thai children: Retrospective review of 30 cases from a tertiary care center

Background: Juvenile dermatomyositis is a rare condition, but it is the most common idiopathic inflammatory myopathy in pediatric patients. Aim: To study the clinical manifestations, investigations, treatment, clinical course, and outcomes of juvenile dermatomyositis in Thai children. Method: This retrospective study included juvenile dermatomyositis patients treated at Siriraj Hospital, a 2,300-bed national tertiary referral center in Bangkok, Thailand, from 1994 to 2019. Results: Thirty patients (22 females and 8 males) were included with a female to male ratio of 2.7:1. Median age at diagnosis was 5.1 years (range, 2.6-14.8 years). Median duration of illness before diagnosis was 6.5 months (range, 0.3-84.0 months). Acute and subacute onset occurred in the majority of patients. Presenting symptoms included muscle weakness in 27/30 (90%), skin rash in 26/30 (86.7%), muscle pain in 17/26 (65.4%), and arthralgia in 4/18 (22.2%) of patients. Dermatologic examination revealed Gottron’s rash, heliotrope rash, and periungual telangiectasia in 25/30 (83.3%), 21/30 (70.0%), and 15/24 (62.5%) of patients, respectively. Interestingly, scalp dermatitis was found in 8/21 (38.1%) of patients. The most commonly used treatment regimen in this series was a combination of prednisolone and methotrexate. During the median follow-up of 3.1 years (range, 0.0-18.5 years), only one-third of patients were seen to have monocyclic disease. Extraskeletal osteosarcoma at a previous lesion of calcinosis cutis was observed in one patient at 12 years after juvenile dermatomyositis onset. Limitations: This was a retrospective single-center study, and our results may not be generalizable to other healthcare settings. Prospective multicenter studies are needed to confirm the findings of this study. Conclusion: juvenile dermatomyositis usually poses a diagnostic and therapeutic challenge, which can be compounded by the ethnic variations in the clinical presentation, as observed in this study. Asian patients tend to present with acute or subacute onset of disease, and arthralgia and/or arthritis are less common than in Caucasian patients. Scalp dermatitis is not uncommon in pediatric juvenile dermatomyositis patients. An association between juvenile dermatomyositis and malignancy, though rare, can occur.

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Deep Cerebral Venous Thrombosis—A Clinicoradiological Study

Journal of Neurosciences in Rural Practice ◽

10.1055/s-0041-1730109 ◽

2021 ◽

Author(s):

Sujana Gogineni ◽

Dhananjay Gupta ◽

R. Pradeep ◽

Anish Mehta ◽

Mahendra Javali ◽

...

Keyword(s):

Venous Thrombosis ◽

Care Center ◽

Tertiary Care ◽

Clinical Manifestations ◽

Arterial Occlusion ◽

Venous System ◽

Common Risk Factor ◽

Imaging Characteristics ◽

Deep Cerebral Venous Thrombosis ◽

High Index Of Suspicion

AbstractStroke is a common neurological emergency. Almost 80% of strokes are due to arterial occlusion. Venous thrombosis comprises less than 1–2% of all strokes. Involvement of the deep cerebral venous system is still rare and accounts for about 10.9% of all cerebral venous thromboses (CVT). CVT diagnosis is often delayed or missed, because of its variable clinical manifestations. We retrospectively (2015–18) and prospectively (2018–20) reviewed all the cases of CVT in a tertiary care center in south India. Out of a total of 52 CVT cases, 12 were due to the involvement of deep cerebral venous system. Their clinical presentation, imaging characteristics, and outcomes were assessed. The most frequent presentation was headache followed by seizures. Hyperhomocysteinemia was the most common risk factor noted. Imaging characteristics were variable, and a high index of suspicion was required for early diagnosis. All patients had favorable outcome in our study, and except one, all were treated conservatively.

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Juvenile dermatomyositis: a case of delayed recognition with unusual complication of nephrocalcinosis

BMJ Case Reports ◽

10.1136/bcr-2020-241152 ◽

2021 ◽

Vol 14 (4) ◽

pp. e241152

Author(s):

Geminiganesan Sangeetha ◽

Divya Dhanabal ◽

Saktipriya Mouttou Prebagarane ◽

Mahesh Janarthanan

Keyword(s):

Muscle Weakness ◽

Juvenile Dermatomyositis ◽

Inflammatory Myopathy ◽

Unusual Complication ◽

Ultrasound Screening ◽

Proximal Muscle Weakness ◽

Proximal Muscle ◽

Calcinosis Cutis ◽

First Case ◽

Medullary Nephrocalcinosis

Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy in children and is characterised by the presence of proximal muscle weakness, heliotrope dermatitis, Gottron’s papules and occasionally auto antibodies. The disease primarily affects skin and muscles, but can also affect other organs. Renal manifestations though common in autoimmune conditions like lupus are rare in JDM. We describe a child whose presenting complaint was extensive calcinosis cutis. Subtle features of proximal muscle weakness were detected on examination. MRI of thighs and a muscle biopsy confirmed myositis. Nephrocalcinosis was found during routine ultrasound screening. We report the first case of a child presenting with rare association of dermatomyositis, calcinosis cutis and bilateral medullary nephrocalcinosis.

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Frequency and clinical manifestations of post-poliomyelitis syndrome in a brazilian tertiary care center

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x2012000800002 ◽

2012 ◽

Vol 70 (8) ◽

pp. 571-573 ◽

Cited By ~ 4

Author(s):

Abrahão Augusto Juviniano Quadros ◽

Mônica Tilli Reis Pessoa Conde ◽

Luis Fabiano Marin ◽

Helga Cristina Almeida Silva ◽

Tatiana Mesquita e Silva ◽

...

Keyword(s):

Joint Pain ◽

Care Center ◽

Neuromuscular Disorders ◽

Tertiary Care ◽

Clinical Manifestations ◽

Paralytic Poliomyelitis ◽

Prior History ◽

Cold Intolerance ◽

Infection Age ◽

History Of

OBJECTIVE: To determine the frequency and clinical manifestations of patients with post-poliomyelitis syndrome (PPS) in a Brazilian division of neuromuscular disorders. METHODS: A total of 167 patients with prior history of paralytic poliomyelitis was investigated for PPS, based on international diagnostic criteria. Other variables analyzed were: gender, race, age at poliomyelitis infection, age at PPS onset, and PPS symptoms. RESULTS: One hundred and twenty-nine patients presented PPS, corresponding to 77.2% of the studied population. 62.8% were women and 37.2% were men. Mean age of patients with PPS at onset of PPS symptoms was 39.9±9.69 years. Their main clinical manifestations were: new weakness in the previously affected limbs (69%) and in the apparently not affected limbs (31%); joint pain (79.8%); fatigue (77.5%); muscle pain (76%); and cold intolerance (69.8%). CONCLUSIONS: Most patients of our sample presented PPS. In Brazil, PPS frequency and clinical features are quite similar to those of other countries.

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Dynamic Changes in Heparin-binding Protein as a Prognostic Biomarker in Patients with Sepsis

10.21203/rs.3.rs-111262/v1 ◽

2020 ◽

Author(s):

Qing-Li Dou ◽

Jiang-Ping Liu ◽

Wen-Wu Zhang ◽

Hoi Sin Tong ◽

Ya-Nan Gu ◽

...

Keyword(s):

Predictive Accuracy ◽

Binding Protein ◽

Care Center ◽

Dynamic Change ◽

Tertiary Care ◽

Heparin Binding ◽

Dynamic Changes ◽

Multicenter Studies ◽

Absolute Level ◽

Heparin Binding Protein

Abstract BACKGROUNDWe aimed to investigate the prognostic value of dynamic changes in heparin-binding protein (HBP) within the first 48 hours of hospital admission in sepsis patients.MethodsWe conducted a prospective observational study in the emergency intensive care unit of a tertiary care center. Patients who met SEPSIS-3 criteria were prospectively enrolled from August 2019 to January 2020. Serum levels of HBP were measured at admission, 24 hours, and 48 hours. Dynamic change in HBP was calculated as a percentage change between admission and 24 hours, and between admission and 48 hours. Accuracies of absolute level of HBP, dynamic change of HBP, and other biomarkers were compared with ROC analysis.ResultsA total of 245 patients were enrolled. After excluding patients not fulfilling the eligibility criteria and those died before 48 hours of admission, 185 patients were included for final analysis, of which 117 had sepsis, 68(36.76%) had septic shock, and 48(30%) died in the hospital. Day 1-HBP was correlated with procalcitonin (r2=0.21, p=0.004). Of all predictors of 30-day mortality, HBP clearance within 48 hours had the highest predictive accuracy (AUC: 0.82), followed by Day 1-HBP (AUC: 0.79), PCT (AUC: 0.75) and HBPc-24(AUC: 0.6). HBPc-48 > -17.14% had an independent impact on 30-day survival after adjusting for age, gender, shock status, and requirement of mechanical ventilation support.ConclusionsHBPc-48 can predict survival in critically ill patients with sepsis and can assist clinicians with risk stratification of these patients. Future multicenter studies are necessary to assess the generalizability of these findings.

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Immunoglobulin A (IgA) Nephropathy in Protocol Graft Kidney Biopsy done at six months Post Transplantation in a Tertiary Care Center Hospital of Nepal

Journal of Nobel Medical College ◽

10.3126/jonmc.v6i1.18080 ◽

2017 ◽

Vol 6 (1) ◽

pp. 6-11

Author(s):

Midhan Shrestha ◽

Dibya Singh Shah

Keyword(s):

Iga Nephropathy ◽

Care Center ◽

Tertiary Care ◽

Clinical Signs ◽

Clinical Manifestations ◽

University Teaching ◽

Time Interval ◽

Post Transplantation ◽

Protocol Biopsy ◽

Post Transplant

Background: Renal transplantation is the treatment of choice for end stage renal disease. The focus of interest has been to increase the life of the transplanted graft. Recurrence of native kidney disease or occurrence of denovo glomerulonephritis has adverse effects in graft survival. Protocol graft biopsy done at fixed time interval after transplantation aids in early identification of post-transplant glomerulonephritis before development of clinical signs and symptoms. This study describes the incidence of post-transplant IgA Nephropathy in protocol renal graft biopsies done at six months post- transplantation.Materials and Methods: This is a hospital based observational descriptive study, done in Tribhuvan University Teaching Hospital, Kathmandu, Nepal, a tertiary medical referral center in the capital. Protocol biopsy of the graft kidney was performed at six months post-transplantation in all recipients who underwent kidney transplantation in this hospital between 2071 Kartik and 2072 Ashwin.Results: Protocol biopsy was performed in total 47 recipients. Mean age of the recipients was 33.7 years ±10.83 years. The study population consisted of 33 (70.2%) male and 14 (29.8%) female recipients. IgA Nephropathy was present in 6 (12.8%) recipients.Conclusion: Our study demonstrates that IgA Nephropathy does occur in patients with stable GFR and without any clinical or laboratory abnormalities. Protocol biopsy is valuable in detection of early histologic abnormalities before onset of clinical manifestations, thus helping in prompt management with aim to prolong the graft survival.Journal of Nobel Medical CollegeVolume 6, Number 1, Issue 10 (January-June, 2017)

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Incidental Petrous Apex Findings on Magnetic Resonance Imaging

Ear Nose & Throat Journal ◽

10.1177/014556130108000407 ◽

2001 ◽

Vol 80 (4) ◽

pp. 200-206 ◽

Cited By ~ 18

Author(s):

John P. Leonetti ◽

Harish Shownkeen ◽

Sam J. Marzo

Keyword(s):

Magnetic Resonance Imaging ◽

Magnetic Resonance ◽

Care Center ◽

Tertiary Care ◽

Clinical Manifestations ◽

Retrospective Chart Review ◽

Petrous Apex ◽

Resonance Imaging ◽

Mri Findings ◽

Clinical Surveillance

We performed a retrospective chart review to categorize a group of petrous apex findings that were noted incidentally on magnetic resonance imaging (MRI) in 88 patients. These patients were among those who had been seen at a tertiary care center between July 1988 and July 1998. These incidental findings, which were unrelated to the presenting clinical manifestations, included asymmetric fatty bone marrow (n = 41), inflammation (19), cholesterol granulomas (14), cholesteatomas (9), and neoplasms (5). Followup imaging and clinical surveillance of these patients has not demonstrated any significant change in the incidentally detected lesions. In all cases, the incidental MRI findings represented benign pathology.

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Acute Kidney Injury and Atypical Features during Pediatric Poststreptococcal Glomerulonephritis

International Journal of Nephrology ◽

10.1155/2016/5163065 ◽

2016 ◽

Vol 2016 ◽

pp. 1-5 ◽

Cited By ~ 3

Author(s):

Rose M. Ayoob ◽

Andrew L. Schwaderer

Keyword(s):

Acute Kidney Injury ◽

Care Center ◽

Tertiary Care ◽

Elevated Serum ◽

Rapidly Progressive Glomerulonephritis ◽

Kidney Injury ◽

Small Subset ◽

Acute Glomerulonephritis ◽

Poststreptococcal Glomerulonephritis ◽

Presenting Symptoms

The most common acute glomerulonephritis in children is poststreptococcal glomerulonephritis (PSGN) usually occurring between 3 and 12 years old. Hypertension and gross hematuria are common presenting symptoms. Most PSGN patients do not experience complications, but rapidly progressive glomerulonephritis and hypertensive encephalopathy have been reported. This paper reports 17 patients seen in 1 year for PSGN including 4 with atypical PSGN, at a pediatric tertiary care center. Seventeen children (11 males), mean age of 8 years, were analyzed. Ninety-four percent had elevated serum BUN levels and decreased GFR. Four of the hospitalized patients had complex presentations that included AKI along with positive ANA or ANCAs. Three patients required renal replacement therapy and two were thrombocytopenic. PSGN usually does not occur as a severe nephritis. Over the 12-month study period, 17 cases associated with low serum albumin in 53%, acute kidney injury in 94%, and thrombocytopenia in 18% were treated. The presentation of PSGN may be severe and in a small subset have associations similar to SLE nephritis findings including AKI, positive ANA, and hematological anomalies.

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Clinical Characteristics and Management of the Hydatid Cyst of the Liver: A Study from a Tertiary Care Center in Nepal

Journal of Parasitology Research ◽

10.1155/2020/8867744 ◽

2020 ◽

Vol 2020 ◽

pp. 1-7

Author(s):

Utsav Joshi ◽

Roshan Subedi ◽

Amar Jayswal ◽

Vishakha Agrawal

Keyword(s):

Hydatid Cyst ◽

Imaging Finding ◽

Care Center ◽

Descriptive Analysis ◽

Tertiary Care ◽

Clinical Manifestations ◽

Public Health Problem ◽

Imaging Studies ◽

Abdominal Ultrasonography ◽

Number Of Patients

A hydatid cyst of the liver is a significant yet neglected public health problem in Nepal. The present study was carried out to evaluate the demographic characteristics, clinical presentations, and management of the patients of the hydatid cyst of the liver in the setting of a developing country. It was a retrospective, descriptive analysis of 53 patients admitted in the department of surgery with the diagnosis of hydatid cyst of the liver based on clinical manifestations, imaging studies, or serology between 2016 and 2019. The median age of the patients was 36 years, with the age group of 25-45 years being the most commonly affected (23, 43.4%). 58.5% of the patients were female. Abdominal pain (49, 92.5%) and a palpable liver (17, 32.1%) were the most common complaint and physical finding in our study population, respectively. Abdominal ultrasonography and computed tomography scan were the major imaging studies used to establish a diagnosis. A unilocular and anechoic cystic lesion was the most frequent imaging finding. The right lobe of the liver harbored the cysts in the maximum number of patients. Surgery was the preferred modality of treatment (23, 43.4%), with pericystectomy being the most common form of surgical intervention. The hydatid cyst of the liver is a common cause of morbidity in Nepal. Clinical evaluation supplemented by imaging studies is required for diagnosis, and surgery remains the treatment of choice in most cases. To explain the epidemiological pattern of the disease, multicentric studies involving a larger sample of patients should be conducted.

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Cardiac Health Awareness Among Women Presenting for Routine Health Checkup at Tertiary Care Center: Time Trends Over 15 Years

Indian Journal of Cardiovascular Disease in Women WINCARS ◽

10.1055/s-0039-1695652 ◽

2019 ◽

Vol 04 (02) ◽

pp. 079-084

Author(s):

Shibba Takkar Chhabra ◽

Gurleen Kaur ◽

Samir Kapoor ◽

Gagandeep Nagi ◽

Devanshi Kajal ◽

...

Keyword(s):

Care Center ◽

Tertiary Care ◽

Treatment Modalities ◽

Age Group ◽

Tertiary Care Center ◽

Health Awareness ◽

Health Checkup ◽

Cardiac Health ◽

Presenting Symptoms ◽

The Past

Abstract Background Women have been thought to be protected against the perils of cardiovascular disease (CVD) till late in their lives. But the literature suggests quite the opposite with CVD being a major cause of death even in young women. In contrast, the lack of awareness among women is disheartening and needs to be addressed radically. Methods The study was designed and conducted as retrospective cohort at a tertiary care center. Data was collected from patients presenting for routine cardiac health checkup over the past 15 years. The parameters observed included age at presenting, symptoms and/or signs, plus area of residence or domicile. Results A total of 32,831 patients presented for routine cardiac health checkup, of which 9,211 (28.1%) patients were women and 23,620 (71.9%) were men. On 5 yearly cumulative assessment, the mean attendance of women was 28.1 ± 2.5% as compared with 71.9 ± 2.6% men. Trend observed over the past 15 years revealed little change in the number of women versus men presenting for cardiac health checkup annually. Statistical significance was seen at p < 0.01. Among the women presenting for the checkup, it was observed that most women were in the postmenopausal age group (42.8%), followed by perimenopausal age group (34.6%), and least in premenopausal age group (22.5%) during the timeline of the study. A similar trend was observed in the male attendance; the input of males being higher at all instances. Noteworthy were trends of urban women (69.6%) presenting for health checkup more often as compared with 30.4% visiting from rural residence. Conclusions Coronary artery disease (CAD) is not uncommon in female gender. It is accompanied by varying symptom presentation with high mortality. It is seen that cardiac health awareness is significantly lacking among women as compared with men. There are almost static trends observed over the past 15 years, especially in premenopausal age group and rural domicile. Corrective actions inclining toward campaigns and communication to distribute information on cardiac disease prevention and treatment modalities among women are needed to curb CAD. This may promote early detection of CAD leading to early interventions to promote a healthy heart among women. Recommendations and necessary actions steps for a woman oriented cardiac program are the need of the hour.

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Scrub Typhus with Varied Clinical Presentations

Journal of Pediatric Infectious Diseases ◽

10.1055/s-0037-1605363 ◽

2017 ◽

Vol 13 (04) ◽

pp. 319-322

Author(s):

Susrita Banerjee ◽

Satyabrata Roychowdhuri ◽

Mihir Sarkar

Keyword(s):

Scrub Typhus ◽

Care Center ◽

Tertiary Care ◽

Clinical Manifestations ◽

Case Series ◽

Dengue Shock Syndrome ◽

Tertiary Care Center ◽

Eastern India ◽

Clinical Presentations ◽

Diagnostic Difficulties

AbstractWe present four cases of pediatric scrub typhus from a tertiary care center of eastern India, emphasizing on varied unusual clinical manifestations posing diagnostic difficulties. A low index of suspicion is required to prevent under diagnosis. We report a case series of four patients with scrub typhus having uncommon clinical manifestations (two patients with hemophagocytic lymphohistiocytosis, one patient with myocarditis, and one patient presenting with shock reminiscent of dengue shock syndrome). The response to doxycycline was good, with fever subsiding within 48 to 72 hours of starting the treatment. Three out of four cases completely recovered once appropriate medication was given.

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