Evolution in the management of Hirschsprung��s disease in the UK and Ireland: a national survey of practice revisited

INTRODUCTION

The management of Hirschsprung’s disease continues to evolve. This questionnaire survey aimed to determine current surgical management strategies for Hirschsprung’s disease in Britain.

SUBJECTS AND METHODS

The survey was sent electronically to all British paediatric surgeons. Initial questions explored individual experience and regional service provision. Additional questions, reserved for surgeons who perform definitive Hirschsprung’s disease surgery, addressed specific clinical scenarios.

RESULTS

Surveys were sent to 142 surgeons yielding 85 responses. After exclusions, 64 surveys from 21 centres were analysed. Forty-seven respondents worked in centres with designated ‘Hirschsprung’s disease surgeons’. Forty respondents perform definitive Hirschsprung’s disease surgery. In a well neonate with left-sided Hirschsprung’s disease, 34 of 40 surgeons favour primary pull-through following bowel decompression with rectal washouts; 35 of 40 surgeons aim to perform definitive surgery at less than 3 months of age, with 17 favouring laparoscopic-assisted Soave–Boley and 15 favouring an open Duhamel pull-through. Of the 40 surgeons, 36 use a staged approach to right-sided/total colonic Hirschsprung’s disease with 23 favouring a Duhamel or Long Duhamel pull-through.

CONCLUSIONS

The primary pull-through, using an open Duhamel or laparoscopic-assisted Soave–Boley technique, during the first 3 months of life, has become the operative strategy of choice in rectosigmoid Hirschsprung’s disease in Britain. Marked variation in practice remains for right-sided Hirschsprung’s disease.     

Patterns of cell proliferation and cell migration in the Sezary syndrome

The patterns of cell proliferation and cell migration were studied in three patients with the Sezary syndrome using autoradiographic techniques. Cell labeling patterns following pulse labeling with tritiated thymidine in vivo indicated that Sezary cells proliferate actively in skin and in lymph nodes but that few if any Sezary cells proliferate in the peripheral blood. In two of the patients serial samples were obtained. Label dilution patterns in skin and blood over time suggested that circulating Sezary cells originated in extracutaneous sites where cells were proliferating more rapidly than in the skin. Cells labeled in extracutaneous sites of proliferation appear rapidly in the blood, and their transit time through the peripheral blood compartment is short. Circulating Sezary cells may then be deposited in the skin where they resume proliferation at a low rate. Thus, while Sezary cells proliferate in both cutaneous and extracutaneous sites, proliferation appears to be more rapid in extracutaneous sites such as lymph nodes. This suggests that trials of systemic therapeutic approaches should be undertaken.     

The role of prolactin in the suppression of Crh mRNA expression during pregnancy and lactation in the mouse

Maternal stress is associated with negative health consequences for both the mother and her offspring. To prevent these adverse outcomes, activity of the hypothalamic‐pituitary‐adrenal (HPA) axis is attenuated during pregnancy and lactation. Although the mechanisms generating this adaptive change have not been defined fully, the anterior pituitary hormone prolactin may play a significant role. The present study investigated the role of prolactin in regulating the basal activity of the HPA axis during pregnancy and lactation in the mouse, focussing upon the corticotrophin‐releasing hormone (CRH) neurones. Using in situ hybridisation, a decrease in Crh mRNA‐expressing cell number in pregnant (55.6±9.0 cells per section) and lactating (97.4±4.9) mice compared to virgin controls was characterised (186.8±18.7, P<.01 Tukey‐Kramer test; n=6‐7 per group). Removal of the pups (24 hours) and thus the associated suckling‐induced prolactin secretion, restored CRH neurone number (180.1±19.7). To specifically test the role of prolactin in suppressing Crh mRNA expression in lactation, prolactin levels were selectively manipulated in lactating mice. Lactating mice were treated with ovine prolactin (1500 μg day^‐1, osmotic minipump, s.c.; n=7) or vehicle (n=6) for 24 hours following pup removal. This was sufficient to suppress Crh mRNA expression from 108.0±13.5 to 53.7±16.7 cells per section (P<.05 Student's t‐test). Additional cohorts of lactating mice were treated with bromocriptine (300 μg over 24 hours, s.c.; n=7) or vehicle (n=5) to suppress endogenous prolactin secretion; however, no change in Crh mRNA expression was detected. Thus, although prolactin was sufficient to suppress Crh mRNA expression in the paraventricular nucleus, it does not appear to be required for the ongoing regulation of the CRH neurones in lactation.     

Adult attachment style as mediator between childhood neglect/abuse and adult depression and anxiety

BACKGROUND: There has been little prospective investigation of the relationship between adult attachment style and clinical levels of anxiety and major depression. This paper seeks to address this, as well as examining the potentially mediating role of adult insecure attachment styles in the relationship between childhood adverse experience and adult disorder. METHODS: 154 high-risk community women studied in 1990-1995, were followed-up in 1995-1999 to test the role of insecure attachment style in predicting new episodes of anxiety and/or major depressive disorder. The Childhood Experience of Care and Abuse (CECA) and the Attachment Style Interview (ASI) were administered at first interview and the Structured Clinical Interview for DSM-IV (SCID) administered at first and follow-up interview. Major depression and clinical level anxiety disorders (GAD, Social Phobia or Panic and/or Agoraphobia) were assessed at first contact and for the intervening follow-up period. RESULTS: 55% (85/154) of the women had at least one case level disorder in the follow-up period. Only markedly or moderately (but not mildly) insecure attachment styles predicted both major depression and case anxiety in follow-up. Some specificity was determined with Fearful style significantly associated both with depression and Social Phobia, and Angry-Dismissive style only with GAD. Attachment style was unrelated to Panic Disorder and/or Agoraphobia. In addition, Fearful and Angry-Dismissive styles were shown to partially mediate the relationship between childhood adversity and depression or anxiety. CONCLUSION: In order to correctly interpret lifespan models of adult psychiatric disorder, it is necessary to test for mediating factors. Attachment theory provides a framework for explaining how dysfunctional interpersonal style arising from early childhood perpetuates vulnerability to affective disorders. This has implications for intervention and treatment to break cycles of risk.     

Recent unprecedented tree-ring growth in bristlecone pine at the highest elevations and possible causes

Great Basin bristlecone pine (Pinus longaeva) at 3 sites in western North America near the upper elevation limit of tree growth showed ring growth in the second half of the 20th century that was greater than during any other 50-year period in the last 3,700 years. The accelerated growth is suggestive of an environmental change unprecedented in millennia. The high growth is not overestimated because of standardization techniques, and it is unlikely that it is a result of a change in tree growth form or that it is predominantly caused by CO₂ fertilization. The growth surge has occurred only in a limited elevational band within ≈150 m of upper treeline, regardless of treeline elevation. Both an independent proxy record of temperature and high-elevation meteorological temperature data are positively and significantly correlated with upper-treeline ring width both before and during the high-growth interval. Increasing temperature at high elevations is likely a prominent factor in the modern unprecedented level of growth for Pinus longaeva at these sites.     

Combination protocols of cytokine therapy with interleukin-3 and granulocyte-macrophage colony-stimulating factor in a primate model of radiation-induced marrow aplasia

Single cytokine therapy with granulocyte-macrophage colony-stimulating factor (GM-CSF) or interleukin-3 (IL-3) has been shown to be effective in decreasing the respective periods of neutropenia and thrombocytopenia following radiation- or drug-induced marrow aplasia. The combined administration of IL-3 and GM-CSF in normal primates suggested that a sequential protocol of IL-3 followed by GM-CSF would be more effective than that of GM-CSF alone in producing neutrophils (PMN). We investigated the therapeutic efficacy of two combination protocols, the sequential and coadministration of recombinant human IL- 3 and GM-CSF relative to respective single cytokine therapy, and delayed GM-CSF administration in sublethally irradiated rhesus monkeys. Monkeys irradiated with 450 cGy (mixed fission neutron:gamma radiation) received either IL-3, GM-CSF, human serum albumin (HSA), or IL-3 coadministered with GM-CSF for days 1 through 21 consecutively postexposure, or IL-3 or HSA for days 1 through 7 followed by GM-CSF for days 7 through 21. All cytokines and HSA were injected subcutaneously at a total dose of 25 micrograms/kg/d, divided twice daily. Complete blood counts (CBC) and platelet (PLT) counts were monitored over 60 days postirradiation. The respiratory burst activity of the PMN was assessed flow cytometrically, by measuring hydrogen peroxide (H2O2) production. Coadministration of IL-3 and GM-CSF reduced the average 16-day period of neutropenia and antibiotic support in the control animals to 6 days (P = .006). Similarly, the average 10-day period of severe thrombocytopenia, which necessitated PLT transfusion in the control animals, was reduced to 3 days when IL-3 and GM-CSF were coadministered (P = .004). The sequential administration of IL-3 followed by GM-CSF had no greater effect on PMN production than GM-CSF alone and was less effective than IL-3 alone in reducing thrombocytopenia. PMN function was enhanced in all cytokine-treated animals.     

Evaluation of the semi-automated Autosperm semen analysis system. I. Accuracy and comparison with the conventional method and the automated Hamilton-Thorn system

The accuracy of measurements by the semi-automated Autosperm (Amsaten N.V.S.A. Corp., DePinte, Belgium) semen analysis system was assessed by recounting and manually tracking sperm recorded on videotape during analysis of 51 ejaculates. Mean inaccuracies in the analysis of sperm concentration and percentage motility were 15% and 22%, respectively. Measurements of sperm movement characteristics relied on the skill of the operator and discrepancies (means around 10%, maximum 57% to 184%) depended on the straightness of sperm paths. Although less expensive than the fully automated system, semen analysis by Autosperm is a subjective and labor-intensive method. Furthermore in comparison, data obtained using Autosperm also provide less information, and agreements of matched data with those obtained by the conventional methods were not significantly better.