Ghrelin levels are reduced in Rett syndrome patients with eating difficulties

Most patients with Rett syndrome (RTT) have both gastrointestinal problems and somatic growth failure, including microcephaly. Ghrelin is a peptide hormone involved in growth hormone secretion, interdigestive motility, and feeding behavior. Plasma ghrelin assays have previously been described for other neurodevelopmental disorders. To examine the pathophysiology of RTT, we measured plasma levels of ghrelin in patients with RTT. A case-control study examining plasma levels of ghrelin, serum growth hormone, and insulin-like growth factor-1 (IGF-1) was performed on 27 patients with RTT and 53 controls. Plasma levels of total (T)- and octanoyl (O)-ghrelin were significantly lower in patients with RTT than in controls. Plasma levels of T-ghrelin correlated significantly with serum IGF-1 levels and head circumference. Significantly lower levels of plasma T-ghrelin and O-ghrelin were observed in RTT patients with eating difficulties, while lower levels of plasma T-ghrelin were observed in RTT patients with constipation, in comparison to patients without either of these symptoms. Alterations in plasma ghrelin levels may reflect various clinical symptoms and signs in RTT patients, including growth failure, acquired microcephalus, autonomic nerve dysfunction, and feeding difficulties. We describe the role of ghrelin in RTT and suggest this peptide as a novel biological marker in patients with RTT.     

Long-term changes in the corneal endothelium after intraocular lens implantation]

The characteristics of the corneal endothelium of 134 eyes which underwent extracapsular cataract extraction and intraocular lens implantation from 1984 to 1990 were studied. We classified the initial 29 eyes as group 1 and the most recent 105 eyes as group 2. Endothelial cell loss of group 1 was much larger than that of the latter group, reflecting the degree of surgical damage. Endothelial cell of both groups decreased with index functions. The coefficient of variation of both groups showed no significant change during the observation period. The hexagonal cells of both groups decreased once after surgery, and it increased after one year in group 1, and after three months in group 2. However the late phase of the change of hexagonal cells appeared to have no relation to the degree of surgical damage. The change of hexagonal cells was supplemented by the appearance of pentagonal cells and heptagonal cells.     

Regression of an Aggressive Abdominal Desmoid Tumor in a Patient with Familial Adenomatous Polyposis by Hyperthermoradiotherapy

A case of huge desmoid tumor successfully treated by hyperthermoradiotherapy is described. A 23-year-old man with familial adenomatous polyposis was operated upon for a desmoid tumor in the mesenterium involving the right kidney and small intestine in 1988. In 1990, the tumor recurred and could not be resected because of the involvement of the vena cava. The tumor grew larger and larger, and occupied two-thirds of the right lower quadrant. Several therapies using sulindac, tamoxifen, prednisolone, indomethacin, luteinizing hormone-releasing hormone analogue, and ascorbate were all ineffective. Finally, the combination of radiation and hyper-thermia was used over a 6-month period. At the end of the hyperthermoradiotherapy, the tumor in the abdominal wail was markedly reduced in size, and the protruded abdominal wall became flat. To our best knowledge, this is the first report of the successful treatment of a huge desmoid tumor by hyperthermoradiotherapy.     

In vivo confocal microscopy of human cornea covered with human amniotic membrane

Purpose  Amniotic membrane transplantation has been widely performed to reconstruct the surface of the eye and treat chemical burns or epithelial defects. However, we have difficulty observing the cornea through the opaque transplanted amniotic membrane by slit-lamp biomicroscopy. We investigated the use of confocal microscopy for observation of human corneas covered with amniotic membrane. Methods  Human amniotic membrane was placed onto the normal corneas of five volunteers aged 22–24 years. Then, all layers of the covered corneas were observed by in vivo confocal microscopy. Results  Confocal microscopy displayed the epithelium, basement membrane, and stroma of the amniotic membrane. It also displayed the corneal epithelium. Furthermore, corneal stromal keratocytes and the corneal endothelium were clearly observed through the amniotic membrane by confocal microscopy. Conclusions  We demonstrated that in vivo confocal microscopy enabled us to observe all layers of corneas covered with amniotic membrane in normal human eyes. Our findings suggest that confocal microscopy may have advantages for clinical examination of the ocular surface, including all layers of the cornea.     

Development of a rapid semi-quantitative immunochromatographic assay for serum hepatocyte growth factor and its usefulness in acute liver failure.

Measurement of serum human hepatocyte growth factor (HGF) by enzyme-linked immunosorbent assay (ELISA) is useful for the early diagnosis and prediction of prognosis of patients with acute liver failure (ALF). This ELISA methodology, however, is neither rapid nor convenient for use at the bedside. In this study, we have developed a rapid semi-quantitative immunochromatographic (IC) assay and evaluated its usefulness in assessing patients with acute hepatic injury. Only 100mul of serum is required; the assay can be easily completed in 20min. The values obtained using this novel assay correlated well with the values obtained using the standard ELISA protocol. In addition, the values obtained in the IC assay correlated with clinical course; increased serum HGF levels were associated with an increased frequency of ALF and death. These results indicate that this rapid semi-quantitative IC assay for HGF is useful for the early diagnosis of ALF and prediction of clinical outcome in acute hepatic injury.     

Late aortic root redissection following surgical treatment for acute type A aortic dissection using Gelatin-Resorcin-Formalin glue

Objectives: Although Gelatin-Resorcin-Formalin (GRF) glue is widely used in surgery for acute aortic dissection, late complications possibly due to the glue, such as late aortic root redissection, have also been reported. We have experienced similar complications, some of which required redo surgeries, and these cases are reviewed.Methods: Twenty-six consecutive patients who underwent surgery for acute type A aortic dissection using GRF glue, from December 1996 to February 2001, were retrospectively studied, with a special focus on any late complications and any reoperation.Results: Of the 21 patients who survived and were followed as outpatients, false aneurysms were found in 5 patients (21%) at 24–42 (mean 34) months following the initial surgery. Of these, 2 patients required resternotomy because of the increasing aneurysm diameter. In both cases, the aortic root was redissected at the site of the GRF glue use where the anastomosis between the aortic root and the prosthesis had widely opened and had become the aneurysm entry point. Significant aortic regurgitation was noticed in 3 patients (14%, 1 of whom showed a false aneurysm), and 2 of these underwent reoperation for aortic root redissection.Conclusions: A high incidence of aortic root redissection with false aneurysm and/or aortic insufficiency was found following the surgery for acute aortic dissection using GRF glue. These patients should be carefully followed for years after surgery.     

Comparison of the effects of pirmenol and lidocaine on intraventricular conduction in canine myocardial infarction

Effects of pirmenol hydrochloride and lidocaine hydrochloride on intraventricular conduction were examined in the infarcted heart of anesthetized dogs. The effects of the drugs on the excitation induced by ventricular stimulations were determined with coupling intervals between 150 and 1,000 ms. Effects of the drugs on the His bundle electrocardiograms were also examined. Pirmenol in doses of 1-5 mg/kg prolonged the conduction time in the infarcted zones over a wide range of coupling intervals. Pirmenol at 5 mg/kg blocked the delayed conduction at a short coupling interval in the infarcted zones. The effect of pirmenol on the conduction time in the normal zone was slight. Lidocaine in doses of 3 and 10 mg/kg prolonged the conduction time in the infarcted zone at a short coupling interval. Pirmenol did not significantly prolong PQ or AH interval. In conclusion, pirmenol selectively depressed the delayed conduction in the infarcted zone, which could result in a reentrant pathway. The effect of pirmenol on delayed conduction was dependent on the coupling interval and was quite different from that of lidocaine.