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Cardiac hypertrophy associated with ACTH therapy for childhood seizure disorder.

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Hypertrophic cardiomyopathy is a newly recognized, potentially fatal complication of ACTH therapy. We report the clinical, echocardiographic, and pathologic findings of an infant who was treated with ACTH for seizure disorder and subsequently developed severe systemic hypertension. Echocardiography

Cardiac hypertrophy secondary to ACTH treatment in children.

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The usefulness of ACTH in the treatment of childhood epilepsy is assessed by improvement in the EEG and in the clinical condition. However, pronounced side effects, even serious ones, must be encountered. The most common complications are Cushing syndrome, infections, and arterial hypertension. We

Vigabatrin Therapy for Infantile Spasms in a Case of Cardiofaciocutaneous Syndrome with Cardiac Hypertrophy Developing during Adrenocorticotropic Hormone Treatment.

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In a patient with cardiofaciocutaneous syndrome complicated by intractable infantile spasms (West syndrome), cardiac hypertrophy developed during adrenocorticotropic hormone treatment. Various types of antiepileptic drugs, intravenous immunoglobulin, thyrotropin releasing hormone, and a ketogenic

Comparison of heart mass in seizure patients dying of sudden unexplained death in epilepsy to sudden death due to some other cause.

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Proposed mechanisms by which sudden unexplained death syndrome in epilepsy (SUDEP) occurs include cardiac dysrhythmias. We hypothesized that individuals dying of SUDEP would have enlarged hearts compared with normal, increasing the risk of sudden cardiac death should the autonomic nervous system

Polycythemia vera in a cat with cardiac hypertrophy.

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Polycythemia vera, a rare and poorly documented disease in cats, was diagnosed in a 4-year-old domestic shorthair cat admitted because of seizures. The diagnosis was made on the basis of high PCV, normal serum erythropoietin concentration (as determined by bioassay, using rabbit bone marrow cells),

Cardiac dysfunction in rats prone to audiogenic epileptic seizures.

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OBJECTIVE Cardiac dysfunction is one of the possible causes of sudden unexpected death in epilepsy (SUDEP). Therefore, the objective of this study was to evaluate cardiac and electrocardiographic parameters in rats with audiogenic epileptic seizures (WAR--Wistar audiogenic rats). METHODS In vivo

High incidence and variable clinical outcome of cardiac hypertrophy due to ACAD9 mutations in childhood.

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Acyl-CoA dehydrogenase family, member 9 (ACAD9) mutation is a frequent, usually fatal cause of early-onset cardiac hypertrophy and mitochondrial respiratory chain complex I deficiency in early childhood. We retrospectively studied a series of 20 unrelated children with cardiac hypertrophy and

Severe pulmonary congestion in a near miss at the first seizure: further evidence for respiratory dysfunction in sudden unexpected death in epilepsy.

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Sudden unexpected death in epilepsy (SUDEP) is the most important direct seizure-related cause of death, and most cases usually occur in patients with intractable, longstanding epilepsy. Suspected mechanisms for SUDEP include central and obstructive apnea, cardiac arrhythmia, postictal respiratory

An X-linked channelopathy with cardiomegaly due to a CLIC2 mutation enhancing ryanodine receptor channel activity.

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Chloride intracellular channel 2 (CLIC2) protein is a member of the glutathione transferase class of proteins. Its' only known function is the regulation of ryanodine receptor (RyR) intracellular Ca(2+) release channels. These RyR proteins play a major role in the regulation of Ca(2+) signaling in

Cardiac hypertrophy secondary to status epilepticus in the rat.

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Status epilepticus was induced in rats by sequential injections of lithium and pilocarpine. Seizure activity was aborted by a combination of MK-801 and diazepam, with status duration ranging from 3 to 180 min. When the hearts were examined 8-12 days later, rats that had experienced an episode of

Inhibition of carnitine palymitoyltransferase1b induces cardiac hypertrophy and mortality in mice.

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Recent reports suggest that short-term pharmacological carnitine palmitoyltransferase 1 (Cpt1) inhibition improves skeletal muscle glucose tolerance and insulin sensitivity. Although this appears promising for the treatment of diabetes, these Cpt1 inhibitors are not specific to skeletal muscle and

Cardiac manifestations of Pallister-Killian syndrome.

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Pallister-Killian syndrome (PKS) is a sporadic multisystem genetic diagnosis characterized by facial dysmorphia, variable developmental delay and intellectual impairment, hypotonia, hearing loss, seizures, differences in skin pigmentation, temporal alopecia, diaphragmatic hernia, congenital heart

Myocarditis complicated by complete atrioventricular block: nine years' experience in a medical center.

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BACKGROUND Myocarditis complicated with complete atrioventricular block (CAVB) is rare in children. The purpose of this study was to report the outcome of myocarditis with CAVB in our institution. METHODS Between June 1998 and June 2007, nine pediatric patients (aged from 1.5 to 16 years) were

Sudden unexpected nocturnal death syndrome in the Mariana Islands.

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Sudden unexpected nocturnal death syndrome (SUNDS) is a distinct clinical entity in previously healthy, young, Southeast Asian males. It is well known in the Philippines and more recently recognized in the U.S. by nonspecific autopsy findings, with no evidence of underlying disease and absence of

Perinatal (fetal and neonatal) tuberous sclerosis: a review.

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Tuberous sclerosis (TSC) is an autosomal-dominant disorder that presents with highly variable clinical manifestations including seizures, mental retardation, skin lesions, and hamartomas affecting multiple organ systems such as the heart, brain, eye, and kidney. A 42-year retrospective review of 70

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