Two types of left ventricular wall motion abnormalities with distinct clinical features in patients with hypertrophic cardiomyopathy

During the long-term follow-up of patients with hypertrophiccardiomyopathy (HCM), some patients develop left ventricular(LV) wall motion abnormalities in the absence of fixed coronaryartery disease. The purpose of this study is to clarify whichclinical features in patients with HCM seem to influence gradualdevelopment of LV wall motion abnormalities over an extendedperiod of time. The study investigates the incidence, mechanismand predictors of these abnormalities. In this retrospectivestudy of 162 patients with HCM, followed-up for an average of13.3 years, we focused our attention on 16 patients who graduallydeveloped two different forms of LV wall motion abnormality.In 11 of these 16 patients, apical segmental dysfunction withmidzone obstruction was recognized; the remaining five patientsshowed generalized hypokinesis, as seen in dilated cardiomyopathy.The 11 patients with apical segmental dysfunction presentedwith extensive apical hypertrophy reaching the midventricularlevel at first examination. The five patients with generalizedhypokinesis showed a slight decrease in LV contractility andreduced localized antero-apical wall motion even at initialexamination. None of the patients in either group developedthe other group's features during their clinical course. These two groups had different initial manifestations and pursueddifferent clinical courses, suggesting that the underlying mechanismscausing wall motion abnormalities are different.     

Megacystis and megacolon in an infant with Ehlers-Danlos syndrome

We report a 14 month old male infant with Ehlers-Danlos syndrome who became ‘anuric’ due to an acutely dilated urinary bladder. Although the patient was also found to have megacolon, no diverticulum was seen in his gastrointestinal tract or urinary bladder. In order to decompress the urinary bladder and colonic wall, we put an indwelling urinary catheter in place for 2 months, and carried out daily glycerin enema for 3 months. All urological and gastrointestinal symptoms subsided with this intensive medical treatment. The diagnosis of megacystis and megacolon was made very early in life for this patient. This may indicate that the striking extension of gastrointestinal and bladder wall may lead to the development of diverticula of gastrointestinal and urinary tracts in later life.     

A case of sub wakefulness syndrome

Abstract We report a patient, a 30-year-old male Japanese-Brazilian migrant construction worker, suffering from excessive daytime sleepiness for at least 6 months. Electroencephalogram recordings during his waking states showed that 10-Hz and 60-µV alpha activity was present prominently in the occipital regions. From the multiple sleep latency test, it was found that stages 1–2 NREM sleep episodes appeared repetitively without any REM episodes, and that the mean sleep latency was 10.2 min. These findings support the diagnosis that this patient suffers from subwakefulness syndrome.     

Varicella infection complicated with meningitis after immunization

A healthy 45 month old boy who had received varicella vaccine 21 months previously developed aseptic meningitis along with an episode of varicella. The presence of viral DNA in cerebrospinal fluid (CSF) detected by polymerase chain reaction (PCR) with Southern blot hybridization confirmed the relationship between the symptoms and the CSF pleocytosis. This is the first reported case of this complication of varicella meningitis occurring in a child with documented immunization and seroconversion.     

Inhaled nitric oxide and extracorporeal membrane oxygenation in persistent pulmonary hypertension of the newborn

Persistent pulmonary hypertension of the newborn (PPHN) may occasionally require an invasive treatment with extracorporeal membrane oxygenation (ECMO). Inhaled nitric oxide (NO) has recently been introduced as a selective pulmonary vasodilator for treatment of PPHN. We describe a case of PPHN in which neither inhaled NO nor ECMO was effective in reversing pulmonary hypertension. The clinical course of the patient suggested a potential role of NO inhalation in predicting the outcome of ECMO treatment for PPHN.