An Implantable Pulse Generator Indicating Asystole or Extreme Bradycardia

The design of a pacemaker which indicates the occurrence of significant bradyarrhythmia is described. Significant bradyarrhythmia is defined as either asystole for 6 seconds, bradycardia with three successive RR-intervals greater than 2 seconds each, or five or more RR-intervals of 2 seconds or more over a period of 25 seconds, but without three such intervals in succession. During bradyarrhythmia the generator initially stimulates at an interval of 2 seconds (30 per minute) and when the described criteria have been fulfilled the pacemaker automatically converts to a conventional ventricular inhibited pulse generator stimulating at a rate of 70 per minute.     

Radiofrequency Catheter Ablation of AV Nodal Reentrant Tachycardia in Situs Inversus Totalis

A 41‐year‐old male with situs inversus totalis had paroxysmal supraventricular tachycardia. On electrophysiology study, the mechanism of the tachycardia was determined as slow/fast atrioventricular nodal reentrant tachycardia and slow pathway was successfully performed under fluoroscopic guidance.     

Maternal uniparental isodisomy is responsible for serious molybdenum cofactor deficiency

Molybdenum cofactor (MoCo) deficiency is a rare autosomal recessive inherited metabolic disorder resulting in the combined deficiency of aldehyde oxidase, xanthine dehydrogenase, and sulfite oxidase. We report a male infant with MoCo deficiency whose clinical findings consisted of microcephaly, intractable seizures soon after birth, feeding difficulties, and developmental delay. Sequencing of MOCS1, MOCS2, and GEPH genes, and single nucleotide polymorphism genotyping array analysis showed, to our knowledge, unusual inheritance of MoCo deficiency/maternal uniparental isodisomy for the first time in the literature. At 10 months of age, he now has microcephaly and developmental delay, and his seizures are controlled with phenobarbital, clonozepam, and vigabatrin therapy.     

Effect of hypothyroidism on the nitrergic relaxant responses of corpus cavernosal smooth muscle in rabbits

BACKGROUND: The incidence of hormonal dysfunction as a cause of impotence remains controversial. However, several recent studies have reported evidence of hormonal abnormalities in 25-35% of impotent men. Hypothyroidism has been reported to occur in 6% of impotent men. METHODS: In the present study, we examined nitrergic responses in hypothyroidism in rabbit corpus cavernosum and compared them with controls. RESULTS: Carbachol-induced relaxation responses and electrical field stimulation (EFS)-induced frequency-dependent relaxations decreased significantly in hypothyroid rabbits. Papaverine and sodium nitroprusside (SNP)-induced relaxation responses did not change significantly in hypothyroid rabbits. The contraction responses of phenylephrine and EFS-induced frequency-dependent contractions were significantly decreased in the hypothyroid group. CONCLUSIONS: We can speculate that the reduction of relaxant responses to EFS and carbachol in hypothyroid rabbits can depend on a decreased release of nitric oxide (NO) from nitrergic nerves and endothelium or a reduction of muscarinic receptor density. Also, decreases in contraction responses may depend on diminished adrenoceptor density.     

Bilateral double testis: Evaluation by magnetic resonance imaging

Bilateral double testis, a varient of polyorchidism, is a rare malformation of which six cases have been reported in the published literature. Magnetic resonance imaging was used in the presented case to confirm the diagnosis without surgical exploration.