Das Rapunzel-Syndrom

The Rapunzel syndrome is a rare manifestation of a gastric trichobezoar with a "tail" extending throughout the small intestine and sometimes even to the colon. We report on the surgical removal of such a bezoar in a 4-year-old patient by gastrotomy--the third published case in the German literature. The syndrome is mainly seen in young girls with trichophagia psychodynamically associated with early childhood deprivation and a high comorbidity of serious pediatric psychiatric disorders. The symptoms are nonspecific and may mimic those of other pathologic gastrointestinal conditions. Clinical characteristics are a movable mass in the epigastrium and alopecia. The therapy of choice is surgery of the trichobezoar together with the whole intestinal "tail," as in most cases endoscopic removal fails due to the large extension. Early diagnosis and treatment of the Rapunzel syndrome is of eminent importance in order to avoid later fatal complications such as gastric perforation and intestinal necroses. Intensive psychiatric follow-up is mandatory for preventing relapses.     

Association between sea recreational water and gastroenteritis

Gastrointestinal infections represent one of the main cause of morbidity both in developing and industrialized areas. Quality of coastal waters is particularly important for countries as Italy that has several regions on coasts. Public health and economy aspects could be hardly affected by poor quality of sea waters. Several studies have been addressed to the association between sea recreational water and development of gastrointestinal infection but in Italy only sporadic cases of infection have been reported especially in immunocompromised hosts. Results of this study, either in the case control or in the active surveillance study seem to confirm the absence of an association between sea exposure and intestinal infections. One of the main risk factors for the development of these infections is still represented by the seafood consumption especially shellfish.     

Peritoneal T cell responses can be polarized toward Th1 or Th2 in children on chronic peritoneal dialysis

Peritoneal T cell responses can be polarized toward Th1 or Th2 in children on chronic peritoneal dialysis. Previous studies on the peritoneal immune system described the presence of activated T lymphocytes in peritoneal effluents from subjects on chronic peritoneal dialysis (CPD). Since Th1/Th2 polarized response can influence the outcome of specific infectious diseases, we investigated if activated Th1/Th2 cells can be detected in peritoneal effluents during peritoneal dialysis, in order to better understand the role of T cells in the mechanisms of peritoneal defense. We have studied 8 children (4 males, 4 females, mean age 5.8 +/- 5.7 years, range 0.3-13.4) on CPD. Peritoneal cells have been isolated from peritoneal effluents by centrifugation. Immunofluorescent staining of intracellular cytokines for flow cytometric analysis was used to detect the percentage of T cells producing either IFN-gamma (Th1) or IL-4 (Th2). In the initial study 3 months after CPD initiation, high percentages of IFN-gamma positive peritoneal T cells (38% and 63%) were detected in two subjects; this finding is consistent with a Th1 polarization of peritoneal T cells. In another subject, high percentages of IL-4 positive T cells (31%) were detected, suggesting a Th2 polarization of peritoneal T cell response. Small amounts of either Th1 or Th2 T cells (2-4%) were also detected in the other subjects. At the 1 year follow-up, Th1 polarization persisted in one subject (18% IFN-gamma positive peritoneal T cells), in another a shift from Th1 to Th2 was observed, and in the other subject a down regulation of both T cell subsets occurred. The finding that a predominance of T cells producing either IFN-gamma or IL-4 was found in 3 out of 8 children strongly suggests that peritoneal T cell responses can be polarized toward Th1 or Th2. The decrease of Th1 and/or Th2 polarized T cells in the peritoneum of 4 out of 6 subjects (after 1 year) suggests that CPD can play an immunosuppressive role on T cell peritoneal responses. Further studies are needed in order to define whether different T helper activation patterns are associated with a higher risk of peritoneal infection or of peritoneal damage.     

Control of organ transplant-associated graft-versus-host disease by activated host lymphocyte infusions

BACKGROUND: Prolonged persistence of donor-derived T cells after organ transplantation has been proposed to improve long-term allograft survival. However, surviving transplant-derived T cells are also able to mediate devastating graft-versus-host disease (GvHD). Currently, GvHD after organ transplantation is usually refractory to conventional therapy and the disease outcome fatal. METHODS: Graft-reactive host T cells were generated ex vivo from a patient suffering from a severe and refractory liver-transplant-associated GvHD. To control GvHD, activated alloreactive host T cells were repetitively retransferred into the patient (activated host lymphocyte infusion [aHLI]). RESULTS: Adoptive transfer of ex vivo activated alloreactive host T cells (aHLI) led to the control and complete resolution of severe GvHD without inducing allograft rejection. CONCLUSIONS: aHLI opens a novel therapeutic window to control solid-organ transplant-associated GvHD while preserving allograft integrity.     

A preliminary diffusion tensor and magnetization transfer magnetic resonance imaging study of early-onset multiple sclerosis

BACKGROUND: Early-onset multiple sclerosis (MS) typically has a more favorable course than adult-onset disease. OBJECTIVE: To assess the extent of microscopic tissue damage in the brain and cervical cord of patients with early-onset MS. DESIGN: During a single magnetic resonance imaging session, images of the brain and spinal cord were obtained using diffusion tensor and magnetization transfer magnetic resonance imaging. PATIENTS: We studied 13 patients with early-onset MS and 10 healthy volunteers. RESULTS: Compared with control subjects, patients with early-onset MS showed only a slight increase of the average mean diffusivity of the normal-appearing brain tissue. CONCLUSION: The relatively modest central nervous system damage detected in these patients might explain why early-onset MS typically has a more favorable clinical course than adult-onset MS.     

European study on intravenous immunoglobulin in multiple sclerosis: results of magnetization transfer magnetic resonance imaging analysis

BACKGROUND: Magnetization transfer magnetic resonance imaging (MT MRI) can provide in vivo markers reflecting the severity of multiple sclerosis-related brain damage occurring within and outside T2-visible lesions. OBJECTIVE: To investigate the effect of intravenous immunoglobulin (IVIG) treatment on the accumulation of brain damage in patients with secondary progressive multiple sclerosis (SPMS), measured using MT MRI.Design, Patients, and Intervention Seventy patients with SPMS participating in the European, multicenter, randomized, double-blind, placebo-controlled trial of IVIG in SPMS underwent brain T2-weighted and MT MRI at baseline and after 12 and 24 months. The MT MRI scans were post-processed and analyzed to obtain MT ratio values from T2-visible lesions and MT ratio histograms from the normal-appearing brain tissue (NABT). RESULTS: At baseline, a significant difference was found for NABT MT ratio histogram peak height (P =.003) between treated patients and patients receiving placebo. No significant differences between treated patients and those receiving placebo were found for any of the considered MT MRI-derived metrics in terms of treatment x time interaction. Nevertheless, over the 24-month period, the placebo patients experienced a 6.75% reduction of the NABT MT ratio histogram peak height, whereas treated patients experienced only a 0.92% reduction of the NABT MT ratio histogram peak height. CONCLUSIONS: This study did not show any statistically significant effect of IVIG on MT MRI quantities. Nevertheless, the markedly different percentage change of the NABT MT ratio histogram peak height over time between patients receiving placebo and treated patients suggests a possible role of IVIG treatment in preventing the loss of "truly" normal brain tissue in SPMS patients.     

Suppression of GABA<Subscript>B</Subscript> receptor function in vivo by disulfide reducing agent, <Emphasis Type="SmallCaps">dl</Emphasis>-dithiothreitol (DTT)

Rationale A recent in-vitro study demonstrated that the potent disulfide reducing agent, dl-dithiothreitol (DTT), may alter the structural stability of the GABA_B receptor, probably inactivating the disulfide bonds between four cysteine residues located in the GABA_B1(a) receptor structure.Objectives The present study was designed to evaluate whether DTT treatment was capable of antagonizing some behavioral effects of pharmacological stimulation of the GABA_B receptor.Methods Experiments on sedation/hypnosis induced by the GABA_B receptor agonists baclofen, SKF 97541, CGP 44532 and -hydroxybutyric acid (GHB) in DBA mice and selectively bred GHB-sensitive (GHB-S) rats, and a GHB drug discrimination study in Long Evans rats were conducted. Specificity of the DTT action on the GABA_B receptor was investigated by assessing its effect on the sedative/hypnotic effect induced by diazepam, ketamine and ethanol.Results DTT prevented the sedative/hypnotic effect of all GABA_B receptor agonists tested and also reversed baclofen-induced sedation/hypnosis. In contrast, DTT had no effect on, or even potentiated, sedation/hypnosis produced by diazepam, ketamine or ethanol. DTT completely blocked the discriminative stimulus effects of GHB.Conclusions These results are discussed in terms of DTT altering the stability of the binding domain of the GABA_B receptor, hindering the drug-receptor interaction.     

Relationship between blood glucose control, pathogenesis and progression of diabetic nephropathy

The present review briefly discusses evidence that the risk of a rapid decline of glomerular function abruptly increases when glycated hemoglobin is steadily higher than 7.5% and postprandial blood glucose is >200 mg/dl. The capacity to accomplish and to maintain steadily tightly controlled blood glucose levels is scanty using the currently implemented hypoglycemic drugs. Moreover, it must be highlighted that most patients with type 2 diabetes, particularly when renal damage does occur, have arterial hypertension. Several studies suggested that the development of ESRD is prevented significantly better by drugs that modulate the renin angiotensin system than by other compounds in patients with type 1 and 2 diabetes with overt diabetic nephropathy. However, a recent trial, the study Antihypertensive and Lipid Lowering Treatment to Prevent Heart Attack Trial (ALLHAT), which compared lisinopril, chlorthalidone, and amlodipine in a large population of patients with arterial hypertension, either associated or not with diabetes, demonstrated that the development of both coronary heart diseases and renal complications was equally prevented by the three drugs. One word of caveat, however, needs to be raised concerning one of the results of the ALLHAT study: the higher risk of developing new-onset diabetes among hypertensive patients who are not treated with lisinopril. Even if it is true that this latter side effect was not accompanied by a worse outcome of macrovascular and renal complications during the 5-yr follow-up period, one cannot rule out the possibility that this might be the case during more prolonged periods of follow-up in the future. Thus, the advantage of a lower cost in the treatment of hypertension with diuretics as compared with other drugs, with similar degree of success in the prevention of vascular complications, should be weighed also taking into consideration the burden of a higher rate of occurrence of new-onset diabetes.     

Longitudinal assessment of the middle cerebral artery peak systolic velocity in healthy fetuses and in fetuses at risk for anemia

OBJECTIVE: Our purpose was to assess the feasibility of longitudinal assessment of the middle cerebral artery peak systolic velocity (MCA-PSV) to predict fetuses who will have severe anemia. STUDY DESIGN: Doppler measurement of MCA-PSV was serially performed in 15 healthy fetuses (99 measurements; range: 4-9 per fetus), 8 fetuses who were mildly anemic (41 measurements; range: 3-10 per fetus), and 11 who were severely anemic (50 measurements; range: 2-7 per fetus) at their first cordocenteses. Linear models were fitted to the data from individual fetuses and the slopes were determined. The average rate of change (slope) of MCA-PSV as a function of gestational age in the 3 groups was calculated from these data. Estimated average slopes were computed using restricted maximum likelihood. F tests were used for hypothesis tests, with the degrees of freedom based on the Kenward and Roger approximation. The values of MCA-PSV and hemoglobin were expressed as multiples of the median (MoM). A P <.05 indicated statistical significance. RESULTS: Gestational age at the time of the Doppler studies ranged from 15.1 to 41 weeks in the healthy fetuses. It was between 15 and 33.4 weeks in the fetuses who became anemic. The estimated average slopes increased with the degree of anemia (P =.03). The difference in mean slope between the severely anemic sample and the healthy sample was statistically significant (estimated difference = 2.2, SE =.65, P =.01). The difference in mean slope between the mildly anemic and healthy samples was not statistically significant (estimated difference = 1.1, SE =.06; P =.08). CONCLUSIONS: We have demonstrated that the MCA-PSV slope is an excellent tool for identifying those fetuses who will become severely anemic and, therefore, need to be followed up more closely during the pregnancy. Our findings expand the clinical applications to which Doppler ultrasonography can be applied in monitoring pregnancies at risk for fetal anemia.