Histiocytoid Cardiomyopathy: Three New Cases and a Review of the Literature

1998 ◽  
Vol 1 (1) ◽  
pp. 56-69 ◽  
Author(s):  
Bahig M. Shehata ◽  
Kathleen Patterson ◽  
Jean E. Thomas ◽  
Diane Scala-Barnett ◽  
Sarada Dasu ◽  
...  
Keyword(s):  
Sudden Death ◽  
Lipid Droplets ◽  
Review Of The Literature ◽  
Ultrastructural Examination ◽  
The Third ◽  
Histiocytoid Cardiomyopathy ◽  
Pathologic Findings ◽  
Gross Lesions ◽  
Abnormal Mitochondria ◽  
In Infants And Children

Histiocytoid cardiomyopathy (HC), a rare arrhythmogenic disorder, presents as difficult-to-control arrhythmias or sudden death in infants and children, particularly girls. Three cases are described with autopsy findings. In two cases, yellow-tan nodules were grossly visible in the myocardium; in the third case, no gross lesions were identified. Microscopic examination in all three cases revealed multiple, scattered clusters of histiocytoid myocytes which on ultrastructural examination were filled with abnormal mitochondria, scattered lipid droplets, and scanty myofibrils. These pathologic findings are similar to those previously described. The pathogenesis of this entity remains controversial. It was recently proposed that this disorder is X-linked dominant with the associated gene located in the region of Xp22.

Sudden deterioration and death in patients with benign tumors of the third ventricle area

1986 ◽  
Vol 64 (2) ◽  
pp. 216-223 ◽  
Author(s):  
John W. Ryder ◽  
B. K. Kleinschmidt-DeMasters ◽  
Ted S. Keller
Keyword(s):  
Risk Factors ◽  
Sudden Death ◽  
Third Ventricle ◽  
Benign Tumors ◽  
Close Monitoring ◽  
Review Of The Literature ◽  
Content Type ◽  
The Third ◽  
Colloid Cysts ◽  
The Subject

✓ Although sudden deterioration and death is a widely recognized complication in patients with benign tumors of the third ventricle area, the exact incidence of this dreaded occurrence is unknown and the reports in the literature on the subject are largely anecdotal. Neither risk factors nor the etiology of the sudden death have been analyzed. The vast majority of these benign tumors are colloid cysts, and the presence and degree of ventricular dilatation and herniation associated with these tumors as cited in the reports are quite variable. The authors report a case of sudden death in a 27-year-old woman with a subependymoma of the left lateral and third ventricles. A review of the literature is included in an attempt to discern identifiable risk factors for sudden death in patients with tumors of the third ventricle area. Since this potential complication is known to exist in patients with otherwise benign tumors amenable to surgical resection, the authors recommend either prompt removal of the tumor on discovery or close monitoring of the patient if surgery is to be delayed.

scholarly journals Neonatal Sudden Death Due to Histiocytoid Cardiomyopathy

2021 ◽  
Vol 45 (3) ◽  
pp. 99-102
Author(s):  
Hongil Ha ◽  
Hyun Lyoung Koo
Keyword(s):  
Electron Microscopy ◽  
Cardiac Arrest ◽  
Heart Disease ◽  
Sudden Death ◽  
Papillary Muscles ◽  
Dense Granules ◽  
Histiocytoid Cardiomyopathy ◽  
Eosinophilic Cytoplasm ◽  
Nodular Lesions ◽  
In Infants And Children

Histiocytoid cardiomyopathy, an extremely rare heart disease in infants and children, usually occurring in girls under the age of 2 years, is characterized by cardiomegaly, ventricular arrhythmia, and sudden death. In the present study, we report a case of a 2-day-old female neonate who suddenly showed cyanosis and cardiac arrest in the neonatal unit and died without resuscitation. Autopsy revealed multifocal nodular lesions in the left ventricle wall and papillary muscles. Microscopically, these lesions were composed of discohesive round or polygonal cells with eosinophilic cytoplasm and were positive for desmin and negative for CD68. Electron microscopy findings displayed abnormal swollen mitochondria with disorganized cristae, dense granules, and diminished myofibrils in the periphery of the cytoplasm.

Experience of prenatal diagnosis of true knots of the umbilical cord during ultrasound examination in the third trimester of gestation

2019 ◽  
Author(s):  
M.Y. Morozova, V.V. Zotov, M.S. Kovalenko et all
Keyword(s):  
Prenatal Diagnosis ◽  
Umbilical Cord ◽  
Ultrasound Examination ◽  
Technological Advance ◽  
Prenatal Ultrasound ◽  
Ultrasound Diagnosis ◽  
Review Of The Literature ◽  
Third Trimester ◽  
The Third

Despite the rapid technological advance, the expansion of prenatal ultrasound diagnosis, as well as the accumulation of experience by both domestic and foreign experts, prenatal recognition of true knots of the umbilical cord causes significant difficulties. Three cases of successful ultrasound diagnosis of true knots of the umbilical cord and brief review of the literature are presented.

Myocarditis Presenting as Sudden Death in Infants and Children: A Single Centre Analysis by ESGFOR Study Group

2021 ◽  
pp. 109352662110072
Author(s):  
Oana Neagu ◽  
Amparo Fernández Rodríguez ◽  
Domitille Callon ◽  
Laurent Andréoletti ◽  
Marta C Cohen
Keyword(s):  
Sudden Death ◽  
Influenza A ◽  
Group A Streptococcus ◽  
Acute Myocarditis ◽  
Clinical History ◽  
Infants And Children ◽  
Group A ◽  
Clinical Awareness ◽  
Type 3 ◽  
In Infants And Children

Background Acute myocarditis is an inflammatory disease of the heart mostly diagnosed in young people, which can present as sudden death. The etiology includes infectious agents (mostly viruses), systemic diseases and toxins. We aim to characterize infants and children with myocarditis at post-mortem presenting as sudden deaths. Methods Retrospective evaluation of 813 post-mortems in infants and children dying suddenly and unexpectedly between 2009–2019. Data retrieved included histological features, microbiology and clinical history. Results 23 of 813 post-mortems reviewed corresponded to acute myocarditis and 1 to dilated cardiomyopathy related to remote Parvovirus infection. PCR identified enterovirus (7), parvovirus (7 cases, 2 also with HHV6 and 1 case with EVB), Influenza A (1), Parainfluenza type 3 (1). Two cases corresponded to hypersensitivity myocarditis, 1 was Group A Streptococcus and 5 idiopathic myocarditis. Enterovirus was frequent in infants (7/10), and in newborns was associated with meningoencephalitis or congenital myocarditis. More than 50% were less than 2 years of age and all remained clinically unsuspected. Conclusion Myocarditis represents almost 3% of all sudden pediatric deaths. Enterovirus and parvovirus were the most common viruses. This retrospective analysis showed that patients experienced viral symptoms but remained unsuspected, highlighting the need for more clinical awareness of myocarditis.

Possible immunological damage to the tegument ofHymenolepis diminutain mice and rats

Parasitology ◽  
1975 ◽  
Vol 71 (3) ◽  
pp. 525-534 ◽  
Author(s):  
A. D. Befus ◽  
L. T. Threadgold
Keyword(s):  
Lipid Droplets ◽  
Cell Injury ◽  
Mechanical Damage ◽  
Neck Region ◽  
Host Immunity ◽  
Hymenolepis Diminuta ◽  
Intensity Of Infection ◽  
Variable Size ◽  
Abnormal Mitochondria ◽  
Worm Expulsion

Opaque or darkened areas (DA) of variable size and position occur onHymenolepis diminutain mice and rats. In mice DA normally first appear in the neck region of the worm but subsequently they appear elsewhere and increase in number until destrobilation or worm expulsion. The posterior of destrobilated worms is often darkened. In the more immunogenic infections with six cysticercoids there are more DA per worm than in infections with one cysticercoid. DA are areas of the tegument with a homogeneous increase in electron density; abnormal mitochondria; reduced granular endoplasmic reticulum, Golgi complexes and discoidal secretory bodies; and accumulation of lipid droplets. DA disappear from worms maintained for up to 4 h in Hanks' balanced salt solution and can be induced by mechanical damage to the worms.As the numbers of DA increase with the duration and intensity of infection and have similarities with types of cell injury, they are probably sites of worm pathology induced by host immunity.

scholarly journals Spontaneous aortic rupture during pregnancy

2011 ◽  
Vol 152 (23) ◽  
pp. 929-933 ◽  
Author(s):  
Zita Pánczél ◽  
Levente Sára ◽  
Péter Tóth ◽  
Márta Hubay ◽  
Éva Keller ◽  
...  
Keyword(s):  
Sudden Death ◽  
Aortic Dissection ◽  
Genetic Basis ◽  
Aortic Rupture ◽  
Relevant Literature ◽  
Ascending Aorta ◽  
Multiparous Woman ◽  
Rare Entity ◽  
Third Trimester ◽  
The Third

Aortic dissection is a rare entity. Half of the aortic dissection cases occur during pregnancy in women under the age of 40. The authors report a case of a multiparous woman at the third trimester of her sixth pregnancy, who died from a sudden and intractable cardiovascular shock. Autopsy revealed the dissection of the ascending aorta. The case is interesting, especially because in the pregnant woman’s family it was not the first sudden death during pregnancy. Authors review the relevant literature regarding the symptoms and the genetic basis of this rare but potentially lethal complication of pregnancy. Orv. Hetil., 2011, 152, 929–933.

scholarly journals Von hippel-lindaus disease: Report of three cases and review of the literature

1995 ◽  
Vol 53 (4) ◽  
pp. 782-788 ◽  
Author(s):  
Luiz F. Bleggi-Torres ◽  
Lúcia de Noranha ◽  
J. Fillus Neto ◽  
José E. Queiroz Telles ◽  
Luiz E. Madalozzo
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Clear Cell ◽  
Review Of The Literature ◽  
The Third ◽  
The Family ◽  
Cerebellar Haemangioblastoma ◽  
Atypical Meningiomas ◽  
Cns Lesions

The authors present the autopsy findings of two related patients and the biopsy findings of a thrid member of the family. The oldest member was 34 years old at death and on postmortem examination he had haemangioblastomas in the retina, cerebellum, medulla and spinal cord. Other findings were renal cell carcinoma, phaechromocytoma, cysts of kidney and pancreas, hydromyelia and atypical meningiomas. His brother died when 30 years old. The autopsy revealed haemangioblastomas of cerebellum, renal cell carcinoma and a clear cell cystadenoma of epididymus. The third patient was the daughter of the first and presented with headache and dizziness. CT-scan showed a cerebellar haemangioblastoma. Epidemiological considerations on the commonest visceral and CNS lesions and a review of current diagnostic criteria are discussed.

scholarly journals Awareness of disease in dementia: Development of a multidimensional rating scale

2007 ◽  
Vol 1 (1) ◽  
pp. 74-80 ◽  
Author(s):  
Marcia Dourado ◽  
Valeska Marinho ◽  
Cláudia Soares ◽  
Eliasz Engelhardt ◽  
Jerson Laks
Keyword(s):  
Cognitive Deficits ◽  
Rating Scale ◽  
Age At Onset ◽  
Assessment Scale ◽  
Second Step ◽  
Review Of The Literature ◽  
The Third ◽  
Diagnosis Of Dementia ◽  
Preliminary Study ◽  
Affective Relations

Abstract Objective: To describe the development of the Assessment Scale of Psychosocial Impact of the Diagnosis of Dementia (ASPIDD), a multidimensional scale to evaluate awareness of disease in dementia. Method: The development of this scale was conducted in four steps. In step one, questions were drawn up after a review of the literature. The second step involved the suggestions offered by a neurologist regarding the skills considered important for the scale. The third step involved the re-writing and review of the domains and questions in the scale followed by a semantic evaluation performed by two independent psychiatrists. Step four consisted of the preliminary study aimed at evaluating the applicability of the ASPIDD. Results: In the semantic evaluation only minor changes were proposed. The preliminary sample had 52 patients, comprising 23 CDR 1 (male=9; female=14) and 29 CDR2 (male=13; female=16). Mean age of patients was 69.7±5.51 (CDR1) and 73.6±9.4 (CDR2), and age at onset was 66.4±5.7 years (CDR1) and 68.3±9.3 year (CDR2). Mean schooling was 9.0±4.3 years (CDR1) and 8.8±4.4 years (CDR2). Mean MMSE was 21.0±3.3 (CDR1) and 17.6±3.5 (CDR2). Mean Cornell was 4.8±2.3 (CDR1) and 4.2±1.9 (CDR2). The patient and caregiver dyads were aware of problems, mainly of those related to social, family and affective relations. The higher rates of discrepant responses were found on the awareness of cognitive deficits and changes in ADL. Conclusion: The ASPIDD is a multidimensional instrument to assess awareness of disease among AD patients.

Sign in / Sign up

Export Citation Format

Share Document