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.     

Developmental changes in electroencephalogram for term and preterm infants using an autoregressive model

The purpose of the present study was to clarify the developmental changes of electroencephalogram (EEG) background activity in term and preterm infants that take place with increasing conceptional age (CA) through an autoregressive (AR) model. Polygraphical EEG recordings were obtained from 76 healthy term and preterm infants with a CA ranging between 31 and 40 weeks. Decreases in total power and component power of δ were noted with CA during burst activity (BA) in quiet sleep (QS) and during active sleep (AS). Increases in total power and component power of δ were noted with CA during interburst interval activity (IBIA) of QS. Regression analysis (RA) of the information amount (IA) indicated a negative correlation with increasing CA in AS and in the monopolar EEG tracings Fp1, C3, O1 and O2 in BA, and a positive correlation in O1 in IBIA. Regression analysis of the IA of δ indicated a negative correlation with increasing CA in AS and in Fp1, C3, O1 and O2 in BA, and a positive correlation in O1 in IBIA. Regression analysis of the IA of δ indicated a negative correlation with increasing CA in BA and AS. These results showed that the high voltage slow wave component changed to a low voltage slow one with development in AS and that BA was longer in duration and lower in power with increasing CA, while IBIA was shorter in duration and higher in power. In conclusion, significant developmental changes occur in all derivatives of AS. Even though the EEG of BA and IBIA change separately, they are followed by EEG of QS in a continuous pattern.     

Visual training and correction of articulation disorders by use of dynamic palatography: serial observation in a case of cleft palate

The dynamic palatograph is an electrical apparatus that generates a visual display of constantly changing palatolingual contact as a function of time, using an artificial palatal plate with affixed electrodes. This paper describes a technique of speech therapy incorporating dynamic palatography for a cleft palate patient. The patient, a 6-year-old Japanese girl with a repaired unilateral cleft lip and palate, had been judged to demonstrate articulation disorders involving contact of the tongue with the hard palate or alveolus following surgical improvement of velopharyngeal function. Prior to therapy the tongue tended to contact the hard palate more posteriorly than normal. After therapy with the dynamic palatograph, palatolingual contact was normal in comparison with average speakers. Our findings suggest that the facility of constant visual indication of tongue posture to the clinician and patient during corrective speech therapy using dynamic palatography may expedite results with cleft palate patients in the speech clinic when implemented in a carefully structured treatment plan.     

Coping with voices: selective attention training for persistent auditory hallucinations in treatment refractory schizophrenia

Attentional difficulties in people with schizophrenia are common and can be disabling. A number of cognitive rehabilitation interventions aim to improve attention span by having patients practice paying attention to various types of stimuli for increasing lengths of time. However, such interventions typically assume that the attention deficit is a form of negative symptom, reflecting a reduced ability to maintain an attentional focus. In the following case study, we describe a cognitive intervention for a patient whose inability to pay attention was the result of persistent, distracting, and disabling auditory hallucinations. The dichotic listening paradigm was modified to enable the patient to repeatedly practice attending to a source of relevant information, while ignoring irrelevant information that was gradually introduced to one, two, and then three spatial locations. A graded technique was used wherein difficulty level (i.e., number of stimulus sources and stimulus intensity) was increased only after near perfect performance was achieved on prior trials. Embedding this intervention within the context of a therapeutic relationship led to clear improvement in task performance over time, with concomitant better attentiveness on the inpatient unit, and an improved ability to engage in interviews at residential settings, leading to subsequent hospital discharge.     

Pulmonary hypertension in systemic lupus erythematosus: evaluation of clinical characteristics and response to immunosuppressive treatment

OBJECTIVE: To clarify the clinical features of systemic lupus erythematosus (SLE) complicated with pulmonary hypertension (PH) and to evaluate the efficacy of immunosuppressive treatment. METHODS: Case records were reviewed for 194 patients with SLE who were admitted to Aoyama Hospital of Tokyo Women's Medical University between 1992 and 1999. There were 12 patients with PH [8 SLE and 4 SLE + systemic sclerosis (SSc) overlap syndrome]. These patients were compared with 59 age and sex matched patients with SLE for clinical characteristics and laboratory findings. The efficacy of treatments for PH was also evaluated. RESULTS: In our cohort of 194 patients with SLE, 6.2% had PH. The plasma thrombin-antithrombin III complex and plasma D-dimer levels were significantly higher in patients with PH compared with those without PH. Eight patients with PH (4 SLE and 4 SLE + SSc) were treated with corticosteroids (CS) +/- cyclophosphamide (CYC). Right ventricular systolic pressure (RVSP) was improved in 7 of 8 patients. In 6 of 7 responders to the therapy, the treatment was started as soon as they were diagnosed with PH. PH relapsed in 2 patients treated with oral CS +/- CS pulse therapy, but their RVSP was decreased again by immunosuppressive treatment. CONCLUSION: CS +/- CYC was effective for PH associated with SLE. Immunosuppressive treatment should be performed during the early stage of PH to improve prognosis.     

PLENARY LECTURES-PL1 Toxicity of perfluorooctane sulfonate and perfluorooctanoate

Persistent perfluorinated organic compounds, such as perfluorooctane sulfonate (PFOS) and perfluorooctanoate (PFOA) are used in a variety of industrial applications. They are very stable in the environment, distribute widely in the global environment and in wild life, and are detected in human sera.     

Two color flow cytometric analysis of lymphocytes in HTLV-I-carrier children

Phenotypic changes in lymphocytes from healthy children with antibody for human T lymphotrophic virus type I (HTLV-I) were studied using two color flow cytometry. The subjects were high school students within a small district of Kyushu, Japan. In the carrier group, a subset of lymphocytes which expressed CD8⁺CD57⁺ was significantly lower in percentage (P < 0.01) and in number (P < 0.05). There was no significant difference between carriers and non-carriers in the percentage and number of CD4⁺ Leu8⁺cells or CD4⁺ HLA-DR⁺ cells. The IgG levels were slightly higher in carriers than in non-carriers (P < 0.05). These findings suggest that HTLV-I-infected cells may affect the immune system in healthy carrier children.     

The safety of a nitric oxide inhalation system using a conventional infant respirator

Attention is becoming increasingly focused on inhalation of nitric oxide (NO) as a selective pulmonary vasodilator. Its metabolite nitrogen dioxide (NO₂), however, is a toxic molecule. The purpose of the present study was to evaluate the safety of a NO inhalation system using a conventional infant respirator from the viewpoint of NO₂ production. The NO inhalation system consisted of a standard neonatal ventilator, a neonatal circuit and a test lung. The NO concentration was increased from 0 up to 19 ppm. At each level of NO, the oxygen (O₂) concentration was changed from 21 to 100%. The NO and NO₂ concentrations were measured with a chemiluminescence analyzer using a molybdenum converter. The NO₂ concentration was increased when either the O₂ or the NO concentration was increased. The maximum concentration of NO₂ was 0.10 ± 0.02 ppm when the concentrations of NO and O₂ were 19 ppm and 100% respectively. The NO inhalation system, using a conventional infant respirator, can be used safely when monitoring NO and NO₂ concentrations.     

Variations in Palatal Rugae in Near Term Fetuses from Untreated Jcl:ICR Mice

ABSTRACT The oral surface of the mouse palate has eight or nine pairs of transverse ridges, or rugae. Abnormalities in the pattern of palatal rugae have been reported in mutant mice and mice exposed to teratogens in utero. The purpose of this study was to describe control data of ruga variations for proper definition of "anomalous" ruga patterns. Jc1:ICR mice on gestation day 18 were killed, and the fetuses were fixed in Bouin's solution. Fetal palates were examined under a dissecting microscope. In total, 251 fetuses from 19 dams were observed. Among these fetuses 88% had one or more variations in the palatal rugae. Common variations were supernumerary anterior to the fourth ruga, division, and lateral bifurcation, and these were regarded as variations in the "normal" range. Variations rare in fetuses from untreated dams were shortness, fusion, cross, and supernumerary posterior to the fifth ruga, and these should be defined as "anomalous" ruga patterns in teratology experiments. Key words: mouse, palate, rugae, developmental toxicity test