Epidemiology and morbidity of Schistosoma mansoni infection in a fishing community along Lake Albert in Uganda

Schistosoma mansoni infection, associated morbidity and symptoms were studied in Piida fishing community at Butiaba, along Lake Albert, Uganda, from November 1996 to January 1997. The study revealed that S. mansoni is highly endemic with an overall prevalence of 72%, a mean intensity of 419.4 eggs per gram (epg) faeces (geometric mean for positives only), with 37.8% of males and 33.0% of females excreting over 1000 epg. Prevalence and intensity peaked in the 10-14 year old age group and decreased with increasing age. Females were less heavily infected than males. Differences were also shown between tribes. Diarrhoea and abdominal pain were commonly reported in Piida. However, no clear-cut correlation between intensity of S. mansoni infection and these conditions could be demonstrated, indicating that retrospective questionnaires concerning S. mansoni related-symptomatology are of limited value. Organomegaly, as assessed by ultrasonography, was frequent and hepatomegaly was associated with heavy S. mansoni infection. No correlation was demonstrated between splenomegaly and infection. This study emphasizes that schistosomiasis mansoni is a major public health problem in Piida fishing community and presumably also in many similar fishing communities. These observations call for immediate intervention and can help in planning long-term strategies for sustainable morbidity control.     

ARTIFICIAL ABDOMINAL HERNIA FOR THE TREATMENT OF HEPATOMEGALY IN A NEONATE WITH STAGE 4S NEUROBLASTOMA

Surgical widening of the abdomen by a silastic pouch has been used very rarely in the management of critically ill infants with hepatomegaly due to neuroblastoma stage 4S. A female newborn baby was referred on the second day of life because of local compressive effects of a massive hepatomegaly, which lead to multiorgan failure. An artificial abdominal hernia was created on the third day of life using a silastic pouch. During the operation oxygenation and ventilation improved and urinary output returned. After chemotherapeutic reduction of hepatic metastases and primary tumor the pouch was successfully removed on day 57 without local complications. The child has survived for more than 1 year and is in complete remission. An artificial abdominal hernia should be considered more often in the critically ill neonate with stage 4S neuroblastoma and massive hepatomegaly.     

新生儿期肝脏巨大血管瘤的外科诊治

目的探讨总结新生儿肝脏巨大血管瘤的外科诊治经验。方法回顾深圳市儿童医院 2014—2018年收治并采用外科手术治疗的 8例新生儿肝脏巨大血管瘤病例,分析其临床资料和外科治疗效果。结果 8例新生儿肝脏血管瘤首诊症状包括腹部包块、黄疸。 1例合并破裂出血、 6例合并动静脉瘘及严重的心衰表现, 1例合并呼吸衰竭。全部病例产后超声和 CT检查均有典型的血管瘤表现,单个病灶直径在 6.5～10 cm。所有病儿均接受手术并完整切除, 1例术后 1d出现胆漏,其他远期并发症随访期间尚未发现,手术效果良好。术后病理报告海绵状血管瘤 5例,血管内皮瘤 3例。术后随访 3～52个月,复查肿瘤均未复发,甲胎蛋白( AFP)恢复正常,所有病儿生长发育同正常儿。结论新生儿肝脏巨大血管瘤可通过临床表现、超声和 CT检查明确诊断。合并内科治疗棘手的破裂出血所致休克、心衰或者呼吸衰竭病儿,紧急手术治疗新生儿肝脏血管瘤是可行的,疗效满意。     

肝脏极度肿大的无反应性结核病——附一例尸检报告

一例表现为不明原因的发热和肝脏严重肿大(3600g)的无反应性结核病直至尸检才明确诊断,本例的最重要特点之一是在急性粟粒状结核性坏死灶周围几无渗出性或增生性反应.本文对无反应性结核病的发病机理、病理变化、临床特点及误诊原因等进行了讨论.     

Hepatic leukemia-associated macrophages exhibit a pro-inflammatory phenotype in Notch1-induced acute T cell leukemia

Tumor-associated macrophages (TAMs) are well accepted and the pathological role of macrophages in hematopoietic malignancies have been proposed. Hepatomegaly is frequently observed in T cell acute lymphoblastic leukemia (T-ALL) patients with poor prognosis. However, the role of leukemia-associated macrophages (LAMs) in hepatic microenvironment remains unclear. Here, the characteristics of hepatic LAMs (H-LAMs) were studied in Notch1 induced T-ALL model. Increase in proportion and absolute counts of H-LAMs was detected with infiltration of inflammatory cells. Furthermore, H-LAMs exhibited a more M1-like phenotype distinct from that of TAMs in hepatocellular carcinoma and LAMs from BM or spleen in leukemia. Moreover, H-LAMs expressed increased level of cytokines in charge of recruiting inflammatory cells, which contributed to pro-inflammatory hepatic microenvironment.     

Virilizing hepatoblastoma—Significance of alpha-1-fetoprotein and human chorionic gonadotropin as tumor markers in diagnosis and follow-up

Hepatoblastoma was diagnosed in a 12 month old boy presenting with hepatomegaly and isosexual precocious puberty. Preoperative levels of both alpha-l-fetoprotein (AFP) and human chorionic gonadotropin (HCG) were highly elevated. The tumor was removed by hepatic trisegmentectomy. Tumor tissue contained high concentrations of AFP and HCG. On combination chemotherapy with cyclophosphamide (CYC), vincristine (VCR), adriamycin (ADR) and 5-fluoruracil (5-FU) HCG dropped over a period of 9 months to normal values. In contrast, AFP was undetectable following surgery. One year after initiation of therapy there is no clinical or radiological evidence of recurrence of the malignancy but the observation period is too short to draw any conclusions on the effect of therapy and the final outcome.     

Autophagy and ethanol-induced liver injury

The majority of ethanol metabolism occurs in the liver. Consequently, this organ sustains the greatest damage from ethanol abuse. Ethanol consumption disturbs the delicate balance of protein homeostasis in the liver, causing intracellular protein accumulation due to a disruption of hepatic protein catabolism.Evidence indicates that ethanol or its metabolism impairs trafficking events in the liver, including the process of macroautophagy, which is the engulfment and degradation of cytoplasmic constituents by the lysosomal system. Autophagy is an essential, ongoing cellular process that is highly regulated by nutrients,endocrine factors and signaling pathways. A great number of the genes and gene products that govern the autophagic response have been characterized and the major metabolic and signaling pathways that activate or suppress autophagy have been identified. This review describes the process of autophagy, its regulation and the possible mechanisms by which ethanol disrupts the process of autophagic degradation. The implications of autophagic suppression are discussed in relation to the pathogenesis of alcohol-induced liver injury.     

Type Ib glycogenosis

Type Ib glycogenosis is a rare glycogen storage disorder resulting from a defect in the enzyme, glucose-6-phosphatase microsomal translocase. We report a case of Type Ib glycogenosis in an 18 month-old male child who presented with a history of hypoglycemic seizures and recurrent infections and had a massive hepatomegaly, recurrent hypoglycemia, hyperuricemia, hypertriglyceridemia, neutropenia and fasting lacticacidemia which decreased sharply on glucose administration.