Anatomy of the porta hepatis as a basis for extended hepatoporto-enterostomy for extrahepatic biliary atresia--a new surgical technique.

Reports on long-term survival rates after Kasai's HPE reveal that 23% of patients are healthy ten and more years after surgery. Therefore we studied again the anatomy and histology of the porta hepatis and the portal tracts in order to improve this surgical procedure. METHODS: Autopsied livers of 6 children with and 6 without EHBA were investigated with regard to the topographical and histological anatomy of the major intra- and extrahepatic bile ducts. RESULTS: On the basis of anatomical findings we have modified the conventional Kasai's HPE and developed an extended exploration of the porta hepatis. The procedure and surgical technique is described in this paper.     

胆道闭锁患者肝门的病理解剖学研究

目的通过解剖胆道闭锁患者肝脏肝门组织,探讨Kasai术后肝门改变与肝内病理改变的差异,为改进Kasai手术提供理论依据。方法收集Kasai术后因胆汁淤积性肝硬化而行肝移植的胆道闭锁患儿13例,将其中术后胆汁引流情况较差者作为引流不良组,切取肝门及肝内组织。收集3例Kasai术后因肝门部囊肿,反复发作胆管炎而行肝移植的胆道闭锁患儿,3例术后胆汁引流较好,为引流良好组,自吻合口开始,沿左右肝管分别间隔1 cm连续取材。结果引流不良组:肝内病理改变以纤维化加重、胆管增生及血管增生为主,增生胆管直径较小,肝门淤胆程度较肝内相对重。引流良好组:靠近肝门部位胆管增生较肝内轻,并以大胆管为主。肝左叶胆管直径较大(最大750μm),具有引流功能;肝右叶胆管增生明显,胆管直径较小(最大390μm)。结论 Kasai术后胆汁引流较好的病例均呈现左叶胆管直径较大,可能提示解剖肝门广度需要增加。     

Myxoid liposarcoma of the porta hepatis in childhood

Liposarcoma in childhood is rare. A case of a myxoid liposarcoma arising at the porta hepatis is reported in a 3-year-old boy with initial favourable response to surgery combined with radio- and chemotherapy. Fatal recurrence occurred at age 15 years. The radiological and sonographic findings of liposarcomas are reviewed.     

The anatomy of the porta hepatis in patients with preduodenal portal vein,with special reference to portoenterostomy for biliary atresia

The aims of this study were to demonstrate the anatomic relationship between portal vein (PV) branches and hepatic ducts in the porta hepatis in individuals with preduodenal portal vein (PDPV) and to examine the validity of portoenterostomy (Kasai procedure) for patients with biliary atresia (BA) and PDPV. The porta hepatis of four subjects with PDPV without BA was dissected. Similar dissection supplemented by computer-aided three-dimensional reconstruction of the hilar structures was undertaken in a patient with BA and PDPV who underwent an unsuccessful Kasai procedure and died at 5 months of age. In three of the four subjects with PDPV alone, PV branches and extrahepatic bile ducts were abnormally arranged in the porta hepatis, some of the hepatic ducts entering the liver substance posterior, and occasionally lateral, to the main PV branches. The three-dimensional study showed that the original right main intrahepatic bile duct was located anterolateral to the portoenterostomy, which had been placed just anterior and to the right of the PV bifurcation. These findings strongly suggest that the PV may not be a reliable landmark for the Kasai procedure in patients with BA and PDPV.     

Comparison of the allergenicity of Actinidia deliciosa (kiwi fruit) and Actinidia chinensis (gold kiwi).

Actinidia chinensis (gold kiwi) is a newly available fruit which has been shown to have in vitro immunoglobulin E (IgE) cross-reactivity with green kiwi. This is the first study to investigate clinical reactivity of gold kiwi. Five patients clinically allergic to green kiwi were investigated by skin test and double-blind placebo controlled food challenge (DBPCFC) with gold kiwi fruit. IgE-binding patterns of individual sera from the five challenged patients and a pool of sera from a further nine patients with kiwi allergy were compared in the two fruits by Western blotting. Cross reactivity of proteins in the two fruits was assessed by inhibition of immunoblots and by IgE enzyme-linked immunosorbent assay (ELISA) inhibition. Four of the five patients had a positive DBPCFC to gold kiwi. Western blotting showed marked differences in the allergen patterns of green and gold kiwi. However, inhibition of the immunoblots and ELISA assay reveals extensive inhibition of IgE binding to proteins in each fruit by the alternative species. Gold kiwi fruit is allergenic and patients allergic to green kiwi are at risk of reacting to the gold kiwi fruit. Despite having different protein profiles and IgE-binding patterns, the two species have proteins that extensively cross-inhibit the binding to IgE.     

Home visiting the newborn baby as a basis for developmental surveillance at child welfare centres

Home visiting is a part of the Swedish child health surveillance programme. In the present study, part of a longitudinal prospective project, the predictive power of observations at home visits to 338 newborn babies was evaluated. The regular home visit was made by the nurse at the Child Welfare Centre also using a check-list developed for this project. Her check-list assessments seemed valid in identifying families with stressful psychosocial conditions. When the general home situation was judged as "poor" or "dubious", the boys showed signs of a delayed mental development at 4-5 years of age. Assessments of "feeding problems" among boys were associated with behavioural problems at 4-5 years of age. The results underline the importance of an early home visit as a base for the developmental surveillance at Child Welfare Centres. However, the results of the home visit observations were not followed by any extra interventions at CWC. It seems the nurse should feel confident in her check-list judgement and initiate interventions where appropriate.     

Infant stress and sleep deprivation as an aetiological basis for the sudden infant death syndrome

SIDS is almost invariably sleep-related. Viable syndrome aetiology must be compatible with its many epidemiologically diverse risk factors, each of which directly or indirectly associates with the creation of psychological and/or physiological infant stress, and the subsequent disruption of normal, contented sleep. During essential deep 'rebound' recovery sleep, arousal ability and upper airway muscle tone decrease further to that in normal sleep, with subsequent upper airway obstruction. When stress impact causes sufficient sleep disruption and physiological fatigue, a failure to arouse and so restore sufficient tone to overcome such obstruction results in sudden, unexpected death. SIDS has therefore many causes which share a final lethal mechanical pathway. Evidence is presented for obstructive apnoea during sleep as being the primary syndrome death mode, for sleep disruption, reduced arousal ability, and infant stress in SIDS, and for risk factor association with the creation of this stress. Specific infant vulnerability in the first 6 months of life to stress predominantly related to total dependency on a carer for gratification of need, and to obstructive sleep apnoea due to normal anatomical, physical, and respiratory immaturity, including rapid physiological fatigue, and peaks in sleep and thermal stress vulnerability, are discussed. Further reasons for the limited age period of SIDS, and for reduced neonatal risk, are given. Prone sleeping risk can relate to positional airway obstruction during normal sleep without prior infant stress. Much of SIDS aetiology appears to concern factors related to socio-economic deprivation and subsequent sub-optimal infant care.     

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