Effect of rifampicin in the treatment of pruritus in hepatic cholestasis

Pruritus in hepatic cholestasis has been suggested to be secondary to a high concentration of serum bile acids. Rifampicin, which inhibits the uptake of bile acids by hepatocytes, has been used to treat pruritus. To determine the efficacy of rifampicin as a treatment for refractory pruritus, the medical records of 33 children (median age 25 months, range 4-135; 19 boys) with chronic cholestasis liver disease (21 with Alagille's syndrome, eight with progressive intrahepatic cholestasis, one with extrahepatic biliary atresia, one with an inborn error of bile acid metabolism, and one with cryptogenic cirrhosis) were reviewed retrospectively. The median dose of rifampicin was 5(4-10) mg/kg/day. The median duration of intake was 36(4-120) weeks. Complete relief of pruritus was noted in five (15%) patients and a partial response in 12 (36%). Overall, no significant difference was noted in the laboratory parameters before and after treatment with rifampicin. In the 21 patients with Alagille's syndrome, however, a significant decrease in alkaline phosphatase was seen before and after one and six months of starting treatment. No adverse side effects were seen. Rifampicin appears to be effective in the treatment of refractory pruritus. A prospective study is warranted to assess further the effect of rifampicin treatment in children with hepatic cholestasis.     

Analysis with antiidiotype antibody of a patient with chronic lymphocytic leukemia and a large cell lymphoma (Richter's syndrome)

A murine monoclonal antibody made against an idiotypic determinant (Id) of surface IgM/IgD lambda molecules on chronic lymphocytic leukemia (CLL) cells of a 71-year-old woman was used for clonal analysis by two- color immunofluorescence. The anti-Id antibody identified IgM+/IgD+/lambda+ B cells as the predominant cell type of her CLL clone. In addition, substantial proportions of the IgG and IgA B cells and most of the IgM plasma cells in her bone marrow and blood were Id+. Six years after diagnosis, the patient died of respiratory failure due to infiltration of lungs by malignant cells. Autopsy revealed a dramatic change in the tumor cell morphology. The lungs, hilar nodes, and liver were infiltrated by a diffuse large cell lymphoma admixed with the leukemic cells. By immunohistologic staining these anaplastic lymphoma cells were IgM+/IgD-/lambda+ B cells expressing the same Id noted earlier on the CLL cells. The immunoglobulin gene rearrangement pattern on Southern blot analysis was also the same in leukemic blood cells and in the tissues involved by the lymphoma. Thus, the combination of antiidiotype and immunoglobulin gene analyses in this patient with Richter's syndrome revealed that a CLL clone, seemingly "frozen" in differentiation, was actually undergoing isotype switching, differentiation into plasma cells, and evolution into a rapidly growing and fetal lymphoma.     

经内镜结扎及硬化联合治疗食管静脉曲张出血的探讨

食管静脉曲张破裂出血是肝硬化门静脉高压症常见的并发症,内镜食管静脉硬化（ＥＶＳ）和内镜食管静脉结扎术（ＥＶＬ）治疗食管静脉曲张出血（ＥＶＢ）,国内外已公认其止血效果,并列为首选治疗方法,但各有优缺点［１］。单纯多次ＥＶＳ治疗具有局部和全身并发症,止血...     

Transforming growth factor beta 1 directly and reversibly inhibits the initial cell divisions of long-term repopulating hematopoietic stem cells

Hematopoiesis appears to be regulated, in part, by a balance between extracellular positive and negative growth signals. Transforming growth factor beta-1 (TGF-beta 1) has been shown to be a negative regulator of primitive hematopoietic cells. This study examined the direct effect of TGF-beta 1 on the proliferation and differentiation of long-term repopulating hematopoietic stem cells (LTR-HSC) in vitro. We previously reported a cell fractionation approach that includes the selection of low Hoescht 33342/low Rhodamine 123 (low Ho/Rh) cell fractions that are highly enriched for long-term repopulating cells (LTR-HSC) and also clone to a very high efficiency in the presence of stem cell factor (SCF) + interleukin-3 (IL-3) + IL-6: 90% to 100% of individually cultured low Ho/Rh cells formed high proliferative potential clones. This high cloning efficiency of an LTR-HSC enriched cell population enabled proliferation inhibition studies to be more easily interpreted. In this report, we show that the continuous presence of TGF-beta 1 directly inhibits the cell division of essentially all low Ho/Rh cells (in a dose-dependent manner) during their 0 to 5th cell division in vitro. Therefore, it follows that TGF-beta 1 must directly inhibit the proliferation of LTR-HSC contained within these low Ho/Rh cells. The time required for some low Ho/Rh cells to undergo their first cell division in vitro was also prolonged in the presence of TGF-beta 1. Furthermore, when low Ho/Rh cells were exposed to TFG-beta 1 for varying lengths of time before neutralization of the TGF-beta 1 by monoclonal antibody, the ability to form macroclones was markedly decreased after approximately 4 days of TGF-beta 1 exposure. In addition, 1 to 10 ng/mL of TGF-beta 1 resulted in a maintenance of high proliferative potential-colony-forming cell (HPP-CFC) during 8 days of culture compared with loss of HPP-CFC in cultures with no added TGF- beta 1. In conclusion, this study shows that TGF-beta 1 directly inhibits the initial stages of proliferation of LTR-HSC and appears to slow the differentiation of daughter cells of low Ho/Rh cells.     

The incidence,spectrum and outcome of paediatric trauma managed by the Pietermaritzburg Metropolitan Trauma Service

Introduction

The Pietermaritzburg Metropolitan Trauma Service (PMTS) has run a systematic quality improvement programme since 2006. A key component included the development and implementation of an effective surveillance system in the form of an electronic surgical registry (ESR). This study used data from the ESR to review the incidence, spectrum and outcome of paediatric trauma in Pietermaritzburg, South Africa.

Methods

The ESR was reviewed, and all cases of paediatric trauma managed between 1 January 2012 and 30 July 2014 were retrieved for analysis.

Results

During the study period, 1,041 paediatric trauma patients (724 male, 69.5%) were managed by the PMTS, averaging a monthly admission of 36. The mean age was 10.9 years (standard deviation: 5.4 years). The mechanism of injury (MOI) was blunt trauma in 753 patients (72.3%) and penetrating trauma in 170 (16.3%). Pedestrian vehicle collisions accounted for 21% of cases and motor vehicle collisions for a further 11%. Intentional trauma accounted for 282 patients (27.1%) and self-inflicted trauma for 14 cases (1.3%). Ninety patients admitted to the intensive care unit and fifty-one required high dependency unit admission. There were 17 deaths, equating to an in-hospital mortality rate of 1.7%. A total of 172 children died on the scene of an incident. There were 35 road traffic related deaths, 26 suicides by hanging, 27 deaths from blunt assault and 23 deaths from penetrating assault. The overall mortality rate for paediatric trauma was 18.2%.

Conclusions

The ESR has proved to be an effective surveillance system and has enabled the accurate quantification of the burden of paediatric trauma in Pietermaritzburg. This has improved our understanding of the mechanisms and patterns of injury, and has identified a high incidence of intentional and penetrating trauma as well as road traffic collisions. These data can be used to guide strategies to reduce the burden of paediatric trauma in our environment.     

Dietary compliance in screening-detected coeliac disease adolescents

In 1992–94 we screened 6315 students for coeliac disease (CD) by testing antigliadin antibodies (AGA) as the first-level investigation. We found 28 biopsy-proven coeliac patients who were invited to start the gluten-free diet (GFD). The aim of this study was a clinical and laboratory follow-up in these screening–detected coeliac adolescents. Patients were 17 females and 11 males with a mean age at diagnosis of 12.8 ± 1 years (range 11–14). Mean follow-up duration time was 23 ± 7 months (range 9–37). Twenty-three of the 28 screening-detected coeliac patients came to the control visit, 3 refused the follow-up and 2 subjects were not found. Twelve patients (52.2%) stated that they never ate any gluten-containing food, while 11 of them (47.8%) reported occasional transgressions to the diet. GFD acceptance was reported as good ( n = 6), moderate ( n = 11) or low ( n = 6). After starting the GFD, signs of improvement were seen in most patients, such as weight gain, increased height velocity and increased feeling of well-being. AGA (both IgG and IgA classes) and antiendomysium antibodies (AEA) were normal in 19 subjects, 2 cases had IgG-AG A and AEA positivity, 1 patient showed abnormal AGA and AEA levels, while isolated IgA-AGA positivity persisted in 1 case. This study shows that even silent CD cases can clinically benefit from the GFD. The consequences of occasional transgressions to the GFD remain unclear.     

Characterization of carbohydrates in commercial infant formulae

Human milk has always been the reference parameter for the preparation of commercial formulae. The advent of more advanced technologies has enabled increasingly precise information on the composition of human milk to be obtained. Our knowledge in the field of carbohydrates also has improved considerably in the last few years following the pioneering studies of Montreuil (1, 2). From a quantitative point of view it has been demonstrated that in the different phases of lactation, in addition to lactose, human milk contains a consistent amount of oligosaccharides (about 20 g/1 in colostrum and 10–13 g/l in mature milk) (3, 4), whereas monosaccharides make up only about 1% of the total carbohydrates (4). More than 100 different types of oligosaccharides have been identified so far in human milk (5–7), mostly tri-octasaccharides (8). From a biochemical point of view, oligosaccharides are constituted by glucose, galactose, N -acetyl-glucosamine, fucose and sialic acid, and present a linear or branching structure (5). Little is known yet about their physiological role or metabolic fate (9); nevertheless, it has been demonstrated that, in addition to their nutritional function, they participate also in the regulation of the intestinal ecosystem, encouraging the growth of bifid flora (10) and contributing to the defense mechanisms against pathogens in various organs (11–13).     

A case of primary pulmonary hypertension

Primary lung hypertension (PLH) is a rare disease of unknown etiology seen largely in young persons. PLH shows rapid progression with lethal outcome 3-5 years after registration of the first symptoms. Early diagnosis of the disease is difficult. A case described in the article illustrates real difficulties in PLH diagnosis and treatment. The patient several times lost consciousness for a short time at insignificant exercise. This phenomenon has not been described in the literature so far.