scholarly journals Early Desquamating Perineal Erythema in a Febrile Infant: A Characteristic Clinical Feature of Kawasaki Disease

2017 ◽  
Vol 14 (7) ◽  
pp. 710 ◽  
Author(s):  
Chiara Isidori ◽  
Lisa Sebastiani ◽  
Maria Cardellini ◽  
Giuseppe Di Cara ◽  
Donato Rigante ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Clinical Feature ◽  
Febrile Infant ◽  
Characteristic Clinical Feature

scholarly journals The landscape of circulating platelet aggregates in COVID-19

2021 ◽  
Author(s):  
Yuqi Zhou ◽  
Masako Nishikawa ◽  
Hiroshi Kanno ◽  
Tinghui Xiao ◽  
Takuma Suzuki ◽  
...  
Keyword(s):  
Large Scale ◽  
Multiorgan Failure ◽  
Young Patients ◽  
Clinical Feature ◽  
Platelet Activity ◽  
Multiple Organs ◽  
Characteristic Clinical Feature ◽  
Circulating Platelet Aggregates ◽  
Platelet Aggregates ◽  
D Dimer

A characteristic clinical feature of COVID-19 is the frequent occurrence of thrombotic events. Furthermore, many cases of multiorgan failure are thrombotic in nature. Since the outbreak of COVID-19, D-dimer testing has been used extensively to evaluate COVID-19-associated thrombosis, but does not provide a complete view of the disease because it probes blood coagulation, but not platelet activity. Due to this limitation, D-dimer testing fails to account for thrombotic events which occur despite low D-dimer levels, such as sudden stroke in young patients and autopsy-identified widespread microthrombi in multiple organs. Here we report the landscape of circulating platelet aggregates in COVID-19 obtained by large-scale single-cell image-based profiling and temporal monitoring of the blood of COVID-19 patients (n = 110). Surprisingly, our analysis shows the anomalous presence of excessive platelet aggregates in nearly 90% of all COVID-19 patients, including those who were not clinically diagnosed with thrombosis and those with low D-dimer levels (less than 1 ug/mL). Additionally, results indicate a strong link between the concentration of platelet aggregates and the severity and mortality of COVID-19. Finally, high-dimensional analysis and comparison with other diseases reveal that COVID-19 behaves as a product of thrombosis (localized) and infectious diseases (systemic), as a cause of systemic thrombosis.

scholarly journals Role of therapeutic phlebotomy in management of case of porphyria cutanea tarda (PCT) admitted in tertiary care hospital Vadodara  

2020 ◽  
Vol 8 (1) ◽  
pp. 4-6
Author(s):  
Ashu Dogra
Keyword(s):  
Aminolevulinic Acid ◽  
Tertiary Care Hospital ◽  
Porphyria Cutanea Tarda ◽  
Tertiary Care ◽  
Skin Lesions ◽  
Clinical Feature ◽  
Care Hospital ◽  
Skin Symptoms ◽  
Characteristic Clinical Feature

Porphyria cutanea tarda is the most frequent type of Porphyria worldwide & presents with skin symptoms mainly. Porphyrias can affect peripheral, autonomic and central nervous system. In Porphyria conditions there is accumulation of heme precursors 5 Aminolevulinic acid, Porphobilinogen and porphyrins which are associated with characteristic clinical feature with acute neurovisceral attacks and skin lesions. This case report summarizes Case of PCT that was successfully managed with Therapeutic Phlebotomy.

scholarly journals Mid-Frequency Hearing Loss Is Characteristic Clinical Feature of OTOA-Associated Hearing Loss

Genes ◽  
2019 ◽  
Vol 10 (9) ◽  
pp. 715 ◽  
Author(s):  
Sugiyama ◽  
Moteki ◽  
Kitajiri ◽  
Kitano ◽  
Nishio ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Clinical Features ◽  
Autosomal Recessive ◽  
Japanese Patients ◽  
Copy Number Variations ◽  
Clinical Feature ◽  
Single Nucleotide Variants ◽  
Characteristic Clinical Feature ◽  
Or Gene ◽  
Homozygous Deletions

The OTOA gene (Locus: DFNB22) is reported to be one of the causative genes for non-syndromic autosomal recessive hearing loss. The copy number variations (CNVs) identified in this gene are also known to cause hearing loss, but have not been identified in Japanese patients with hearing loss. Furthermore, the clinical features of OTOA-associated hearing loss have not yet been clarified. In this study, we performed CNV analyses of a large Japanese hearing loss cohort, and identified CNVs in 234 of 2262 (10.3%, 234/2262) patients with autosomal recessive hearing loss. Among the identified CNVs, OTOA gene-related CNVs were the second most frequent (0.6%, 14/2262). Among the 14 cases, 2 individuals carried OTOA homozygous deletions, 4 carried heterozygous deletions with single nucleotide variants (SNVs) in another allele. Additionally, 1 individual with homozygous SNVs in the OTOA gene was also identified. Finally, we identified 7 probands with OTOA-associated hearing loss, so that its prevalence in Japanese patients with autosomal recessive hearing loss was calculated to be 0.3% (7/2262). As novel clinical features identified in this study, the audiometric configurations of patients with OTOA-associated hearing loss were found to be mid-frequency. This is the first study focused on the detailed clinical features of hearing loss caused by this gene mutation and/or gene deletion.

A ruptured middle cerebral artery aneurysm in a 13-month-old boy with Kawasaki disease

2010 ◽  
Vol 6 (2) ◽  
pp. 150-153 ◽  
Author(s):  
Jun Hyong Ahn ◽  
Ji Hoon Phi ◽  
Hyun-Seung Kang ◽  
Kyu-Chang Wang ◽  
Byung-Kyu Cho ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Middle Cerebral Artery ◽  
Cerebral Artery ◽  
Pediatric Population ◽  
Disease Process ◽  
Artery Aneurysm ◽  
Kawasaki Syndrome ◽  
Clinical Feature ◽  
Middle Cerebral Artery Aneurysm ◽  
Cerebral Artery Aneurysm

This 13-month-old boy, in whom Kawasaki disease had been diagnosed at the age of 6 months, presented with subarachnoid hemorrhage caused by the rupture of a middle cerebral artery aneurysm. The authors performed an emergency craniectomy and clip occlusion of the aneurysm, which was found to be partially thrombosed. The patient was discharged 4 weeks postoperatively without apparent neurological deficit. Intracranial saccular aneurysms in the pediatric population are rare, and are occasionally associated with various systemic disorders. Kawasaki disease is a systemic vasculopathy of unknown origin, but cerebral arteries are usually spared from the disease process. This is the second case report of a ruptured cerebral aneurysm in a patient with Kawasaki disease, providing a novel clinical feature that the authors call Kawasaki syndrome.

scholarly journals AB1002 IS KAWASAKI DISEASE OR SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS IN CHILDREN WITH FEVER WITHOUT A SOURCE?

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1794.2-1794
Author(s):  
B. Sözeri ◽  
F. Demir ◽  
T. Merter ◽  
M. Karacan
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Kawasaki Disease ◽  
Clinical Features ◽  
Systemic Vasculitis ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Clinical Feature ◽  
Unknown Etiology ◽  
Ivig Treatment ◽  
Factors Affecting ◽  
The Mean

Background:Fever without a source (FWS) is caused by various diseases, making differential diagnosis difficult. Clinical similarities between Kawasaki disease (KD) and systemic Juvenile Idiopathic Arthritis (sJIA) are well known. Kawasaki disease (KD), a self-limiting systemic vasculitis, remains of unknown etiology and can cause irreversible coronary artery aneurysms (CAAs). SoJIA is sometimes confused with incomplete KD because both diseases have overlapping clinical features and can be accompanied with CAAs and/or SJIA with macrophage activation syndrome (MAS).Objectives:In this study, the frequency of both KD and SJIA among the patients evaluated with FWS and the clinical features of patients diagnosed with Kawasaki disease.Methods:Medical records of patients who first visited our department between January 2016 and December 2019 were reviewedResults:A total of 107 patients were enrolled in this study, including 43 patients (40.2%, 23 males) who fulfilled the criteria of Kawasaki disease and 64 patients (59.8%, 39 males) who did not fulfill them. In patients who fulfilled the criteria of classical FWS, 36(33.6%, 20 males) patients were diagnosed with systemic juvenile idiopathic arthritis. The mean age of the patients with Kawasaki disease was 30.0±20,4 months (median 25 months), the mean age of other patients was 52,6±40 months (median 39,5 months). The mean age of the patients with sJIA patients was 87,6±49,8 (median 80months). Kawasaki patients were younger than others (p=0.01). There was no difference in gender between groups.In Kawasaki patients, the most common clinical feature at diagnosis was fever (100%) followed by conjunctival congestion and mucosal changes (69%). The last two findings are more significant in kawasaki patients than others (p<0,00). Twenty-six (59%) patients had completed KD while 25% had incomplete KD. 7 (16%) patients had atypical KD. The mean fever duration was longer in sJIA patients than KD and others (median 14,8 and 7 days, p<0.00). All patients with KD received IVIG (2 g/kg, infusion in 12 h) and aspirin (60 mg/kg/day). 13.6% of the patients also received oral corticosteroids because of IVIG resistance. Thirty-one patients (72.1%) responded to IVIG treatment, whereas 12 (6 female, 6 male) were IVIG resistant. CAI was detected in echocardiography at diagnosis in 10 (22.7%) (6 female; 4 male) patients. We also detected 4 patients pericarditis with /without CIA.Conclusion:The clinical presentations of KD and sJIA are quite similar with fever, rash, hepatomegaly, and lymphadenopathy. All 2 entities may provide clues to potentially shared immunopathology.References:[1]Arslanoglu Aydin E et al. The factors affecting the disease course in Kawasaki disease. Rheumatol Int. 2019 Aug;39(8):1343-1349[2]Dong S et al. Diagnosis of systemic-onset juvenile idiopathic arthritis after treatment for presumed Kawasaki disease. J Pediatr. 2015 May;166(5):1283-8.Disclosure of Interests:None declared

Abstract 210: Kawasaki Disease in Patients Older Than 10-years Old in a Children’s Hospital In Mexico City

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
ITZEL E RIOS-OLIVARES ◽  
LUIS M GARRIDO-GARCIA
Keyword(s):  
Kawasaki Disease ◽  
Mexico City ◽  
Systemic Vasculitis ◽  
Clinical Manifestations ◽  
Cervical Lymphadenopathy ◽  
Skin Lesions ◽  
Clinical Feature ◽  
Unknown Etiology ◽  
Cardiac Complications ◽  
Increased Risk

Background: Kawasaki Disease (KD) is an acute febrile illness characterized by systemic vasculitis of unknown etiology. Cardiac sequelae, such as coronary artery aneurysms (CAA), are one of the most important aspects of this disease. Actually KD is most frequently presented in children younger than 5-years old. Objective: To describe the clinical and laboratory features, cardiac sequelae and outcome in children older than 10-years old with KD who were attended at the Instituto Nacional de Pediatría in Mexico City. Methods: An observational, descriptive, retrospective and transversal case study. We reviewed the medical records of patients older than 10-years diagnosed with KD from August 1995 to May 2014, and analyzed gender, age, clinical manifestations, hemoglobin, leucocyte count, platelet count, ESR, CRP, albumin, sodium, potassium, AST, ASL, time from the onset of the symptoms to diagnosis, treatment used, the development of CAA and outcome in the acute phase of the disease. Results: We studied 18 cases of KD in patients older than 10-years old, 72.2% (13 of 18) were male with a mean age of 154 months (range 120 to 200). The time from the onset of the fever to diagnosis was 10.6 ± 5.8 days, (range 3 to 21 days). Skin lesions were the most common manifestation of KD and cervical lymphadenopathy was the least common clinical feature. 2 patients presented with KD shock syndrome. Complete KD was diagnosed in only 50% (9 of 18) of our cases. 16 patients received IVGG, 2 patients required a second GGIV dose and 10 patients also received steroids. 6 of 18 patients (30%) developed CAA. There were no deaths in our group. Conclusions: KD in patients older than 10-years old represent a clinical challenge because in the majority of the cases they presented with an atypical clinical picture which contribute to a delayed diagnosis. Also there is an increased risk of developing cardiac complications and CAA in this group of patients.

Abstract 200: Afebrile Kawasaki Disease Beyond Diagnostic Guideline Evolving Coronary Artery Complication

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Sun Hwa Lee ◽  
Na Yeon Kim
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Diagnostic Algorithm ◽  
Clinical Feature ◽  
Z Score ◽  
Bacille Calmette Guerin ◽  
Inoculation Site ◽  
Diagnostic Guideline ◽  
Specific Finding

Kawasaki disease (KD) is diagnosed with clinical features. Presence of at least five days of fever is major criteria. Herein we report a 7 month -old boy diagnosed as afebrile kawasaki disease who initially presented with inflammation at the Bacille Calmette-Guerin (BCG) inoculation site (BCGitis) with multiple erythematous papular rash, followed by desquamation of finger tips at 11 day of illness. Laboratory test showed elevated ALT (110 U/L) and otherwise showed no specific finding. Clinical feature disappeared spontaneously except BCGitis. The patient did not fulfill the diagnostic criteria but progressive coronary arterial dilatation was noticed. Left coronary artery (LMCA) was dilated from Z-score 1.6 (3 day of illness) to 2.8 (11 day of illness). After treated with intravenous immunoglobulin (2g/kg), BCGitis disappeared and follow up echocardiogram showed normalized LMCA lesion (Z-score 1.0 at 17 day of illness). BCGitis was considered to be pathognomonic feature of this patient. Diagnostic algorithm and guidelines are useful tool for the incomplete KD patients but clinicians should also be cautious for the patient with BCGitis even they are excluded by diagnostic guideline.

Temporal evolution of remission following multiple sclerosis relapse and predictors of outcome

2012 ◽  
Vol 18 (8) ◽  
pp. 1152-1158 ◽  
Author(s):  
Claire L Hirst ◽  
Gillian Ingram ◽  
Trevor P Pickersgill ◽  
Neil P Robertson
Keyword(s):  
Multiple Sclerosis ◽  
Therapeutic Intervention ◽  
Physical Disability ◽  
Temporal Evolution ◽  
Initial Assessment ◽  
Clinical Feature ◽  
Patient Counselling ◽  
Physical Support ◽  
Impact Scale ◽  
Characteristic Clinical Feature

Background: Relapse is a characteristic clinical feature of multiple sclerosis (MS) and is commonly employed as a measure of efficacy following therapeutic intervention. However, less is known about the temporal evolution of subsequent disability or factors predicting recovery. Objectives: The objective of this study was to assess the pattern of recovery following relapse and identify factors which predict recovery and residual disability following relapse. Methods: A total of 226 relapses were studied prospectively in a cohort of 144 patients with standardised clinical assessments of physical disability including Expanded Disability Status Scale (EDSS), 10-m timed walk, 9-hole peg test and Multiple Sclerosis Impact Scale (MSIS-29) at 0, 2, 6 and 12 months. A total of 82 patients completed 12 months of follow up without further relapse. Results: Thirty per cent of relapses were severe (change in EDSS >2.0) of which 11% failed to recover. All measures showed significant improvement at 2 months but additional improvement was also observed in 9-hole peg test and MSIS-29 up to 12 months following initial assessment. Mean time to second relapse was 382 days. The only predictor of relapse severity in the model tested was younger age; however, increasing age and initial relapse severity were also predictors of poor outcome. Conclusions: This study shows that the majority of improvement in physical disability following relapse occurs by 2 months but that more subtle recovery can take place over 12 months in a small sub-group of patients. These data will aid in patient counselling and will also inform the timing of therapeutic intervention and physical support.

scholarly journals Preauricular pilomatrixoma in a 3 year-old girl: A case study

2012 ◽  
Vol 18 (1) ◽  
pp. 84-86
Author(s):  
H Hirbod ◽  
F Ameli ◽  
M Swamintanhan ◽  
BS Goh
Keyword(s):  
Foreign Body Reaction ◽  
Histopathological Examination ◽  
Excisional Biopsy ◽  
Pyogenic Granuloma ◽  
Clinical Feature ◽  
Sebaceous Cyst ◽  
Characteristic Clinical Feature ◽  
History Of ◽  
High Index Of Suspicion

Objectives: To report a case of preauricular pilomatrixoma in a 3-year-old girl.Methods: Retrospective review on a case reportResults: A three-year-old girl presented with six month history of painless right pre-auricular swelling that was gradually increasing in size. The mass was small, soft, non tender with normal overlying skin. She underwent excisional biopsy of lesion and histopathological examination showed pilomatrixoma. She was well post operatively and no recurrence after 2 years of follow up.Conclusion: Pilomatrixoma is often misdiagnosed clinically as epidermoid cyst, sebaceous cyst, dermoid cyst, foreign body reaction, calcification in lymph node, fat necrosis, pyogenic granuloma, chalazion and keratoacanthoma, but a high index of suspicion and careful histological examination of its characteristic clinical feature can help clinicians to differentiate it from other tumors.   DOI: http://dx.doi.org/10.3329/bjo.v18i1.10426  Bangladesh J Otorhinolaryngol 2012; 18(1): 84-86

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