Long-term follow-up of patients with high-risk congenital diaphragmatic hernia

Background/Purpose

Recent advances including prenatal diagnosis, high-frequency oscillatory ventilation, and nitric oxide inhalation therapy have gradually improved the survival of high-risk congenital diaphragmatic hernia. However, the factors affecting the long-term outcome of these patients have not been well established.

Methods

Thirty-three children with ages 4.1 ± 2.5 years underwent clinical examination including growth measurements, echocardiography, ventilation, and perfusion scintigraphy.

Results

No late death was observed. Common complications were frequent respiratory tract infection (13 patients) and bowel obstruction (5 patients underwent surgery). Although frequent respiratory tract infection decreased with increasing age, patients with frequent respiratory tract infection had a decreased uptake of lung ventilation and perfusion scintigraphy on the affected side and had a decreased height for age and weight for height. No significant difference in lung ventilation and perfusion scintigraphy was observed between patients treated with and without extracorporeal membrane oxygenation, those requiring oxygen more than 1 month, and between those with and without prenatal diagnosis. Patients with a patch repair had decreased uptake on lung perfusion scintigraphy. Although frequent respiratory tract infection may be owing to hypoplasia of the ipsilateral lung, it may impair recovery of the hypoplastic lung.

Conclusion

These results indicate that monitoring for respiratory tract infection in addition to nutritional assessment should be required in the follow-up of patients with congenital diaphragmatic hernia at high risk.     

Assessment of obstructive apnea by using polysomnography and surgical treatment in patients with Beckwith-Wiedemann syndrome

Purpose

Obstructive apnea is sometimes seen in patients with Beckwith-Wiedemann syndrome. The cause of apnea is not limited to macroglossia, and the surgical indication for obstructive apnea has not yet been established. The authors performed polysomnography for the assessment of apnea.

Method

Overnight polysomnograms were obtained in 2 patients who developed obstructive apnea after 1-stage repair for omphalocele.

Case 1

Apnea index (AI), defined as apneic events per hour, indicated 17.3, and SpO₂ below 95% occupied 80% of the total sleep time. Computed tomography and magnetic resonance imaging indicated obstruction of the airway between macroglossia and the hypopharynx. Central tongue resection and the division of the frenulum linguae for associated ankyloglossia were performed 97 days after birth. One month after surgery, apneic events disappeared and SpO₂ below 95% occupied only 1% of the total sleep time.

Case 2

Obstructive AI indicated 28.1. Division of the frenulum linguae and anterior glossopexy were performed 55 days after birth. Postoperative polysomnogram indicated a marked reduction of AI.

Conclusions

These results indicated that polysomnography was useful for evaluating obstructive apnea and that advancement of the tongue by division of the frenulum linguae may be recommended for the treatment of obstructive apnea in patients with Beckwith-Wiedemann syndrome.     

Serum lipid survey and its recent trend in the general Japanese population in 2000

To determine the recent serum lipid levels and other serum variables in the general Japanese population and trends in their changes over the past 40 years, a nationwide survey of serum lipid levels was conducted in 36 institutes from various districts around Japan in 2000. The total number of subjects was 12,839, aged 4 through 99 years. The mean total cholesterol level was 201 mg/dl; 202 mg/dl in men and 200 mg/dl in women. The mean HDL-cholesterol level was 59 mg/dl; 55 mg/dl in men and 65 mg/dl in women. The mean LDL-cholesterol level was 118 mg/dl; 121 mg/dl in men and 115 mg/dl in women. The mean triglyceride level was 118 mg/dl; 136 mg/dl in men and 92 mg/dl in women. The total cholesterol level slightly increased by 5 mg/dl in 10 years. Although the triglyceride level in women did not change, the triglyceride level in men increased over 10 years, especially in the 30s through 70s age bracket, indicating a possible increase in metabolic syndromes in the future. The present results will become the standard serum lipid level data for the Japanese people, and succeeding 10-year surveys will clarify the trends of lipid levels in this country.     

Blink reflex R2 recovery curves in patients with facial palsy within ten days after onset

The purpose of this study was to investigate the acute changes in blink reflex responses in patients with facial palsy. We used the blink reflex R2 recovery curves as an index of the excitability of the blink reflex neural circuit and evaluated the excitability of the blink reflex within 10 days after onset. Twelve patients with peripheral facial palsy were selected on condition that the degree of facial palsy was so mild that R2 responses of the blink reflex were measurable on the affected side, and they were compared with 12 healthy volunteers. Conditioning and test electrical stimuli were delivered on the affected side of the supraorbital nerve. Ipsilateral R2 response (iR2) of the blink reflex in patients was significantly enhanced at interstimulus intervals (ISIs) between conditioning and test stimuli from 100 to 1,000 ms, in comparison with the control subjects. These findings suggested hyperexcitability of the blink reflex neural circuit in patients with facial palsy. In addition to iR2, contralateral R2 response (cR2) in the patients was also significantly increased at ISIs of 100-1,000 ms to the same extent as the enhancement of iR2. All these findings suggested that the hyperexcitable changes developed in a common pathway of iR2 and cR2, but not in the ipsilateral facial motor neurons. It is suggested that the hyperexcitability of the neural circuit of the blink reflex during an acute period of facial palsy is an adaptive response to compensate for impaired facial motor function.     

Phenotypic studies on dopamine receptor subtype and associated signal transduction mutants: insights and challenges from 10 years at the psychopharmacology–molecular biology interface

Background Mutants with targeted gene deletion (‘knockout’) or insertion (transgenic) of D₁, D₂, D₃, D₄ and D₅ dopamine (DA) receptor subtypes are complemented by an increasing variety of double knockout and transgenic-‘knockout’ models, together with knockout of critical components of DA receptor signalling cascades such as Gα_olf[Gγ₇], adenylyl cyclase type 5, PKA [RIIβ] and DARPP-32. However, it is increasingly recognised that these molecular techniques have a number of inherent limitations. Furthermore, there are poorly understood methodological factors that contribute to inconsistent phenotypic findings between laboratories. Objective This review seeks to document the impact of DA receptor subtype and related transduction mutants on our understanding of the behavioural roles of these entities, primarily at the level of unconditioned psychomotor behaviour. Methods It includes ethologically based and orofacial movement studies in our own laboratories, since these are the only studies to systematically compare each of the D₁, D₂, D₃, D₄ and D₅ receptor and DARPP-32 signal transduction ‘knockouts’. Discussion There is a particular emphasis on identifying methodological factors that might influence phenotypic effects and account for inconsistencies. The findings are offered empirically to (1) specify the extent of phenotypic diversity among individual DA receptor subtypes and transduction components and (2) indicate relationships between D₁, D₂, D₃, D₄ and D₅ receptor subtype proteins, associated Gα_i/Gα_s/Gα_olf[Gγ₇]–adenylyl cyclase type 5–PKA [RIIβ]–DARPP-32 signalling cascades and behaviour. The findings are also offered heuristically as a base for such phenotypic comparisons at additional levels of behaviour so that a yet more complete phenotypic profile might emerge.     

Prostaglandins E1 and E2 inhibit lipopolysaccharide-induced interleukin-18 production in monocytes

The purpose of this present study was to explore the therapeutic potential of prostaglandins E1 and E2 on the systemic inflammatory response evoked by endotoxin. Since interleukin-18, a monocyte-derived cytokine, is increased during sepsis, decreasing the production of interleukin-18 is important in treating this condition. Prostaglandin E1 and E2 inhibited interleukin-18 production in human monocytes treated with lipopolysaccharide and prostanoid IP-, EP2- and EP4-receptor agonists mimicked the effects of prostaglandins E1 and E2. Therefore, prostanoid IP, EP2- and EP4-receptors might be involved in the decrease in interleukin-18 production during sepsis.     

CT fluoroscopy-guided bronchoscopic dye marking for resection of small peripheral pulmonary nodules

STUDY OBJECTIVE: To determine the diagnostic reliability and safety of a new marking technique using transbronchoscopic dye injection under CT fluoroscopy for preoperative localization of a small pulmonary nodule. DESIGN: Prospective study. SETTING: Hyogo Medical Center for Adults and Shizuoka Cancer Center in Japan. PATIENTS: Seventeen patients who had a peripheral pulmonary nodule < 15 mm in size on CT scans that was suspected to be difficult to localize by visual inspection and manual palpation at our institutes between April 2000 and October 2002. INTERVENTIONS: After a bronchoscope was inserted orally under local anesthesia and was introduced into the related bronchus of the target nodule, a Teflon sheath catheter with metal tip was inserted transbronchoscopically and was advanced into the visceral pleura. By monitoring CT fluoroscopy, the catheter tip was positioned at the nearest pleural surface of the nodule, and 0.5 mL indigo carmine was injected under deep inspiratory breathhold. CT scans were obtained to confirm the relationship between the injected dye area and the nodule. MEASUREMENTS AND RESULTS: The dye injections were performed completely in all 17 patients, who subsequently underwent lung resection guided by the dye staining. There were no complications or harmful effects of the surgery. The area of injected dye was demonstrated as a hazy focal lesion about 10 mm beneath the pleura on the high-resolution CT scan, and was clearly visible as a patchy dark blue area about 20 mm in size on the visceral pleura at surgery. The mean distance between the nodule and the dye was 20 mm on the CT scan (distance range, 0 to 30 mm). The mean examination time with this technique was approximately 35 min (range, 25 to 45 min). The mean CT fluoroscopic time was 60 s (range, 30 to 120 s). CONCLUSIONS: Our transbronchial "tattooing" technique is safe and reliable. We think it is superior to previous marking methods.