Liver stiffness assessed by real-time two-dimensional shear wave elastography predicts hypersplenism in patients with Wilson's disease: a prospective study.

BACKGROUND: The current study aimed to explore the value of liver stiffness assessed by two-dimensional real-time shear wave elastography (2D-SWE) to predict hypersplenism occurrence in Wilson's disease (WD) patients. METHODS: Ninety WD patients were enrolled in this prospective study between May 2018 and December 2018. Baseline clinical data and ultrasound imaging including 2D-SWE liver stiffness of WD patients were collected. After enrollment, patients had follow-ups for 24 months or until they developed hypersplenism. The hypersplenism risk factors were determined using Cox regressions and receiver operating characteristic curves (ROC). RESULTS: Twenty-nine (32.2%) patients developed hypersplenism. Age, portal vein diameter, and liver stiffness were independent hypersplenism risk factors in WD patients. The cutoff value of liver stiffness to predict hypersplenism was 10.45 kPa, with sensitivity and specificity of 75.9% and 73.8%, respectively. Patients were divided into two groups according to liver stiffness: ≥ 10.45 kPa (57.9% with hypersplenism) or < 10.45 kPa (13.5% with hypersplenism). The median time between enrollment and hypersplenism development was 15 months vs. 22 months (p < 0.001) for the two groups, respectively. CONCLUSION: The measurement of liver stiffness by 2D-SWE can be a reliable hypersplenism predictor in WD patients. Therefore, dynamic monitoring of WD patients using 2D-SWE is crucial for the early diagnosis of hypersplenism.

The spectrum of splenic complications in patients with sickle cell disease in Africa: a systematic review.

The majority of the global population of sickle cell disease (SCD) patients resides in Africa. Individuals with this condition are at great risk of serious infections and early mortality secondary to splenic dysfunction without preventative measures. This review investigated the spectrum of splenic complications encountered in SCD among populations in Africa. We systematically searched several databases for all articles published through March 3, 2020. We included 55 studies from 14 African countries. This review reveals the difference in frequency of splenic complications in SCD in Africa when compared with their counterparts in the United State and Europe. While several studies (n = 45) described splenomegaly with a prevalence of 12% to 73% among children, and 4% to 50% among adults with HbSS, the reported prevalence for acute splenic sequestration crisis (n = 6 studies) and hypersplenism (n = 4 studies) was <10% and <5% respectively. A total of 30 surgical splenectomy was reported across eight studies. Only two (3.7%) studies provided data on spleen function. A conflicting pattern was observed amongst studies that evaluated the relationship between splenomegaly and the presence of bacterial and malaria infections. This review reveals the paucity of studies describing the role of SCD-induced splenic dysfunction in morbidity and infection related mortality in Africa.

Etiology, presenting features and outcome of children with non-cirrhotic portal vein thrombosis: A multicentre national study.

OBJECTIVES: Non-cirrhotic portal vein thrombosis (PVT) is a main cause of portal hypertension in children. We describe the characteristics at presentation and outcome of a cohort of patients with PVT to determine clinical features and predictors of outcome. METHODS: We recorded: (1) Associated factors: prematurity, congenital malformations, neonatal illnesses, umbilical vein catheterization (UVC), deep infections, surgery; (2) congenital and acquired prothrombotic disorders; (3) features at last follow up including survival rate and need for surgery. RESULTS: 187 patients, mean age at diagnosis 4 ±â€¯3.7 years, had a history of prematurity (61%); UVC (65%); neonatal illnesses (79%). The diagnosis followed the detection of splenomegaly (40%), gastrointestinal bleeding (36%), hypersplenism (6%), or was incidental (18%). Of 71 patients who had endoscopy at presentation 62 (87%) had oesophageal varices. After 11.3 years' follow up 63 (34%) required surgery or TIPS. Ten-year survival rate was 98%, with 90% shunt patency. Spleen size, variceal bleeding and hypersplenism at presentation were predictors of surgery or TIPS (p < 0.05). CONCLUSION: PVT is associated with congenital and acquired co-morbidities. History of prematurity, neonatal illnesses and UVC should lead to rule out PVT. Large spleen, variceal bleeding and hypersplenism at presentation predict the need for eventual surgery in a third of cases.

Non-cirrhotic portal fibrosis at a tertiary care centre in South India.

BACKGROUND: Non-cirrhotic portal fibrosis (NCPF) is one of the important causes of upper gastrointestinal haemorrhage in patients in tropical countries. The aim of this study was to describe the clinical and laboratory profile of 68 patients with NCPF. MATERIAL AND METHODS: NCPF is defined as liver disease with: (1) evidence of portal hypertension; (2) a liver biopsy showing no cirrhosis or a Tc-labelled sulphur colloid scan showing a pattern suggestive of NCPF; and (3) a patent splenoportal axis. The clinical, laboratory and demographic features of 68 patients with such criteria were studied and analysed. RESULTS: NCPF was common in women (73.5%) in the fourth decade of life. The median duration of illness was 24 months (range, 1 month-28 years). Patients presented to hospital with the sensation of a mass in the abdomen (50%) or with haematemesis (26.5%). They had splenomegaly (95.6%) and thrombocytopenia (88.2%). The majority of patients had normal liver function tests. Abdominal ultrasonography showed increased periportal and peri gallbladder echoes (72%), spontaneous collaterals (41.2%) and ascites (19.1%). Liver biopsy revealed portal venous sclerosis (76.3%) and periportal fibrosis (55.3%). Tc-labelled sulphur colloid scan was suggestive of NCPF in the remaining 30 cases. CONCLUSION: NCPF is common in South India. Transient ascites occurs due to decompensation of liver function after variceal bleeding and in long standing cases of NCPF. Our study used Tc-sulphur scan for diagnosing NCPF in patients where liver biopsy was contraindicated in view of severe thrombocytopenia; however, the diagnostic utility of Tc-sulphur nuclear scan to diagnose NCPF in patients with severe hypersplenism needs to be further evaluated in future studies.

Liver disease in autosomal recessive polycystic kidney disease: clinical characteristics and management in relation to renal failure.

OBJECTIVES: We correlated liver and kidney manifestations in a national cohort of patients with autosomal recessive polycystic kidney disease (ARPKD). METHODS: A total of 27 consecutive patients with ARPKD were included. Hepatobiliary disorders were comparatively evaluated in 2 groups: children in group 1 (n = 10) displayed renal failure as infants and those in group 2 (n = 17) had normal kidney function through the first year of life. RESULTS: Median follow-up time was 10.6 (range, 0.4-40) years. Portal hypertension was diagnosed in 13 patients (48%) at the median age 5.0 (1.5-27.9) years. Esophageal varices developed in 8 patients (30%) at age 8.0 (2.1-11.9) years; 4 patients (15%) had variceal bleeding, and hypersplenism/splenomegaly occurred in 52%, similarly in both groups. Biliary tract dilatation was detected at 2.8 years in group 1 and at 7.9 years in group 2, significantly more frequently in group 1 (60% vs 18%, P = 0.039), causing cholangitis in 2 (20%) versus none in group 2 (P = 0.055). A total of 10 patients (37%) underwent cadaveric liver transplantation (LT) at a median age of 6.6 (1.0-20.0) years. In 1 patient LT was performed because of hepatoblastoma. Nine of these were combined liver-kidney transplantations (CLKT). Patients in group 1 required LT earlier (4.1 years vs 18.2 years, P = 0.017) and more frequently (70% vs 18%, P = 0.01). Overall survival beyond neonatal period was 85%. Two patients died because of infectious complications after CLKT, and 1 patient because of recurrent hepatoblastoma. CONCLUSIONS: Although correlation of renal and liver manifestations was variable, biliary dilatation was associated with early renal failure. CLKT may be a treatment for patients with ARPKD with marked hepatobiliary complications.

Lesiones esplénicas en medicina interna / Splenic disorders in internal medicines

El bazo representa el mayor órgano linfopoyético, contiene el 25 % de la masa linfoide total. Participa en la inmunidad celular y humoral e interviene en la renovación de los glóbulos rojos y en la eliminación de las bacterias. Las funciones esplénicas están reducidas cuando el bazo está ausente, lo que implica entre otras complicaciones, una mayor susceptibilidad para padecer una sepsis por organismos encapsulados. Se presentan 6 casos clínicos ingresados en el servicio de Medicina Interna con patología esplénica y se hace una revisión del abordaje a realizar. El espectro de lesiones esplénicas en medicina interna es muy amplio. En ocasiones se puede sospechar patología esplénica por la historia clínica, la exploración física o por citopenias en los análisis. Disponemos de diversas pruebas complementarias para completar el estudio de dichas lesiones. En caso de duda diagnóstica se puede realizar esplenectomía siendo los diagnósticos más frecuentes la cirrosis hepática y el linfoma/leucemia (AU)

The spleen is the largest lymphopoietic organ, containing 25 % of total lymphoid mass. It participates in cellular and humoral immunity and intervenes in the renovation of red cells and the elimination of bacteria. Splenic functions are reduced when the spleen is absent, which entails, amongst other complications, greater susceptibility to suffering from sepsis due to encapsulated organisms. We present 6 clinical cases admitted to the Internal Medicine serve with splenic pathology and we make a review of the approach to be used. The spectrum of splenic lesions in internal medicine is very wide. On occasions, a splenic pathology can be suspected due to clinical history, physical exploration or because of cytopenias in the analyses. Different complementary tests are available for completing study of these lesions. A splenectomy can be carried out in case of diagnostic doubt, with the most frequent diagnoses being hepatic cirrhosis and lymphoma/leukaemia (AU)

Effect of antiviral therapy on the survival and incidence of major complications in HBV-associated cirrhotic patients after splenectomy for hypersplenism and portal hypertension.

BACKGROUND: Splenectomy remains a common approach for the management of hypersplenism and portal hypertension in hepatitis B virus (HBV)-associated cirrhotic patients in China and some other Asian countries. The effects of antiviral therapy on the survival and occurrence of complications in asplenic HBV-associated cirrhotic patients are unknown. This study analyzed the effect of antiviral therapy on survival and occurrence of major complications in HBV-associated cirrhotic patients after splenectomy for hypersplenism and portal hypertension. RESULTS: Of the 57 eligible patients for analysis, 28 patients received nucleos(t)ide analogs (treatment group) for antiviral treatment after splenectomy, while 29 patients received no antiviral treatment (control group). After a median of 3 years and 9 months, the overall survival and complication-free survival in the treatment group were higher though not statistically significant than those in the control group. Multivariate analysis showed that antiviral treatment was associated with increased but not statistically significant overall survival (hazard ratio (HR): 2.272, 95% confidence interval (CI): 0.952-5.424, P=0.064) and the antiviral treatment was significantly associated with increased complication-free survival of the patients (HR: 7.229, 95% CI: 1.271-41.117, P=0.026). The complication-free survival in patients aged ≤ 40 years was higher than that in patients aged>40 years in the antiviral treatment patients (P=0.020). CONCLUSIONS: Antiviral therapy initiating after splenectomy may reduce the incidence of complications and tend to improve the survival in asplenic HBV-associated cirrhotic patients, especially in younger patients, supporting the use of antiviral therapy in these patients after splenectomy.

Predictive factors for persistent splenomegaly and hypersplenism after adult living donor liver transplantation.

PURPOSE: The aim of this study was to evaluate predictive factors for persistent splenomegaly and hypersplenism after living donor liver transplantation (LDLT). PATIENTS AND METHODS: From January 2008 to June 2010, 159 adult patients (116 males and 43 females) who underwent living donor liver transplantation (LDLT) had pre- and post-LDLT computed tomography angiography and survived more than 6 months. Patients with post-LDLT portal vein stenosis were excluded from this study. We analyzed the impact for persistent splenomegaly and hypersplenism after LDLT of pre-LDLT spleen volume, main portal vein (PV) size, coronary vein (CV) size and platelet levels. RESULTS: While 38 patients displayed splenomegaly, 121 showed normal spleen volumes at 6 months after LDLT (LDLT). There were 119 thrombocytopenic versus 40 normal platelet patients at 6 months post-LDLT. The persistent splenomegaly patients showed significantly larger pre-LDLT spleen volume, larger PV and CV sizes as well as lower platelet levels before (×10,000/mL) and 1 month after LDLT (×10,000/mL). Multiple logistic regression analysis showed spleen volume and platelet count at 1 month posttransplant to be the only variables associated with persistent splenomegaly at 6 months post. Persistent thrombocytopenia at 6 months post-LDLT was associated with significantly larger pre-LDLT spleen volume, larger CV size, and lower platelet levels including P0 and P1 m. Multiple logistic regression analysis showed that platelet count at 1 week and at 1 month post-LDLT were the variables associated with persistent thrombocytopenia at 6 months post-LDLT. CONCLUSION: Spleen volume and platelet levels at 1 month after LDLT may predict persistent splenomegaly at 6 months post-LDLT. The predictive factors for hypersplenism at 6 months post-LDLT may be platelet levels at 1 week and at 1 month post-LDLT.

Bone marrow examination in pancytopenia.

Pancytopenia is defined by reduction of all the three formed elements of blood below the normal reference. It may be a manifestation of a wide variety of disorders, which primarily or secondarily affect the bone marrow. Haematological investigation forms the bedrock in the management of patients with pancytopenia and therefore needs detailed study. The total number of cases studied were 100 over a period of two years in the department of pathology, JSS Hospital, Mysore. Megaloblastic anaemia (33%) was the commonest cause of pancytopenia. Other causes were nutritional anaemia (16%), aplastic anaemia (14%), hypersplenism (10%), sepsis (9%) and leukaemia (5%). Less common causes were alcoholic liver disease, haemolytic anaemia, HIV, dengue, systemic lupus erythematosus, viral hepatitis, disseminated TB and multiple myeloma. Most of the patients were in the age group of 11-30 years with a male:female ratio of 1.6:1.Generalised weakness and fatigue (88%) were the commonest presenting complaints. Haemoglobin level varied from 1-10 g/dl with majorIty (70%) of them in the range of 5.1-10 g/dI. TLC was in the range of 500-4000 cells/cmm. Most (34%) of them had 3100-4000 cells/cmm. Platelet count was in the range of 4000-1,40,000 cells/cmm. Reticulocyte count varied from 0.1%-15% with majority (82%) of them ranging from 0.1%-2%. The bone marrow cellularity was hypocellular in 14%, hypercellular in 75%, and normocellular in 11% of the patients. Pancytopenia is a relatively common entity with inadequate attention in Indian subcontinent. A comprehensive clinical and haematological study of patients with pancytopenia will usually help in the identification of the underlying cause. However in view of wide array of aetiologies, pancytopenia continues to be a diagnostic challenge for haematologists.

Hypersplenism after living donor liver transplantation.

BACKGROUND/AIMS: It has not determined whether post-transplant persistent hypersplenism (PTPH) occurs after living donor liver transplantation (LDLT). METHODOLOGY: One hundred and ninety-four patients who survived more than 6 months after LDLTs were examined for the evaluation to determine the incidence of PTPH (leukocyte counts <3,500/microL and/or platelet counts <7.5 x 10(4)/microL). In addition, 154 patients without a splenectomy were evaluated for the risk factors for PTPH. RESULTS: The incidence of PTPH was 20.1% (n=31/154), and the occurrence ranged between 1.1 and 9.9 years after the LDLT, with the mean follow-up periods of 3.3+/-2.5 years. Multivariate analysis showed that portal pressure >30 mmHg at the time of the laparotomy (p<0.01) and post-LDLT small for size syndrome (p<0.01) are risk factors for PTPH. For those with severe portal hypertension (>30 mmHg at laparotomy), the splenectomy tended to give better survival rate (p=0.09) without increasing the rate of septic complications. CONCLUSIONS: Hypersplenism did persist in a proportion of patients after LDLT. A high-risk for PTPH, especially severe portal hypertension, may be an indication for a splenectomy during LDLT in order to achieve uncomplicated post-transplant recovery.

Hypersplenism is related to age of onset of liver disease.

BACKGROUND: The aim of this study was to investigate whether hypersplenism is related to the age of onset of liver disease. METHODS: Hypersplenism was assessed in 84 consecutive cirrhotic patients attending a university hospital liver clinic. RESULTS: Hypersplenism was present in 78% of patients and severe in 39%. Severe hypersplenism was more common in patients with cystic fibrosis liver disease (88%, P < 0.0001) and autoimmune hepatitis (60%, P = 0.0006) compared to alcoholic cirrhosis (12%). There was a significant correlation between patient age and platelet count r = 0.525, P < 0.0001 in the group as a whole and for the sub-group of patients with autoimmune hepatitis r = 0.54, P = 0.047. There was a negative correlation between age and spleen size r = -0.611, P < 0.0001. CONCLUSION: Younger patients had lower platelet counts, larger spleens and a higher incidence of hypersplenism suggesting that hypersplenism is significantly related to the age of onset of chronic liver disease.

Molecular pathogenesis and clinical variability of homozygous beta0-thalassemia in populations of Jammu region of J&K state (India).

This study was undertaken to evaluate the variation in the clinical presentation of homozygous beta0-thalassemia from severe disease to a beta-thalassemia intermedia phenotype and to look for the contribution of associated factors in this variation of clinical course. Type of beta0-thalassemia mutations, associated alpha-thalassemia, and XmnI polymorphism in the gamma globin gene, which are known to affect the clinical course of the disease, were investigated from 15 homozygous beta0-thalassemia patients comprising 11 patients with beta-thalassemia major and 4 patients with beta-thalassemia intermedia. Transfusion dependency and the age at which the patient presented with symptoms were used to assess the degree of clinical severity of these patients. Three different beta0-thalassemia mutations viz. CD 41-42 (-TTCT), CD 8-9 (+G) and 619 bp deletion, were encountered among the 30 beta-thalassemia alleles. It was observed that the type of beta0-thalassemia mutations was not different between the two groups, but co-inheritance of one or more alpha-gene deletions and the presence of the XmnI polymorphism were associated with lesser severity of the disease.

Acute splenic sequestration in female children with sickle cell disease in the North of Jordan.

The objective of this study was to evaluate the rate of acute splenic sequestration (ASSC) in patients with sickle beta-thalassaemia and sickle cell anaemia, the risk of recurrence in those who survive the first episode, and the relationship between ASSC episodes and subsequent hypersplenism. All patients with confirmed diagnosis of sickle cell disease at a tertiary referral teaching hospital, between January 1994 and December 2002 were interviewed and had their medical records reviewed. Seventy-seven patients with sickle cell disease were identified. Their ages ranged between 2 and 18 years (mean, 10.1 years). There were 35 females and 38 males. Thirty-seven (50.6%) had sickle beta-thalassaemia, and 36 (49.4%) had homozygous sickle cell anaemia. Of these, 26 had high level of Hb F and 11 had normal level of fetal haemoglobin (Hb F). Twenty-one patients (28%) had 63 episodes of acute splenic sequestration. Thirty-seven episodes were experienced by 12 patients with sickle beta-thalassaemia; of these 11 were major attacks with one fatality. Twenty-six episodes were experienced by nine patients with sickle cell anaemia. Splenomegaly and hypersplenism were greater in the acute splenic sequestration group than in the rest of the sickle cell anaemia patients, and the differences were extremely significant. ASSC was found in nine siblings of sickle beta-thalassaemia group, while none were found in the sickle cell anaemia group. The mean age of the first episode was significantly higher in sickle beta-thalassaemia, with significant differences in the levels of Hb F, Hb S, size of spleen and severity of crisis between both groups. In the sickle cell anaemia group the only significant difference between patients with and these without acute splenic sequestration was the difference in the size of spleen. In this study, the rate of ASSC in the sickle beta-thalassaemia patients was 32%, in contrast to 25% in the sickle cell anaemia patients. The risk of recurrence was about 70% in those who survived their first episodes. There was a close relationship between ASSC and subsequent hypersplenism. Important predictable factors for ASSC in sickle beta-thalassaemia patients were the presence of splenomegaly of more than 5 cm below the costal margin, history of acute splenic sequestration in siblings and high Hb F. Most of first episodes in sickle cell anaemia occur under the age of 2 years, while in sickle beta-thalassaemia the majority of patients have their first crisis at the age of > or =3.5 years.

Surgical treatment of hepatocellular carcinoma in China: surgical techniques, indications, and outcomes.

BACKGROUND AND AIMS: Surgery remains the most effective treatment for hepatocellular carcinoma (HCC). While resection and liver transplantation achieve the best outcomes in patients with small HCC, controversy surrounds treatment of large HCC, HCC with portal vein tumor thrombus, and HCC with hypersplenism. PATIENT/METHODS: From January 1988 to December 2002, 2,102 patients with large HCC underwent hepatectomy in our hospital. The traditional resection method was used on 959 patients, after which the improved new method was used on 1,143 patients. Meanwhile, from January 1990 until December 2003, hepatic resection +/- thrombectomy has been performed in 438 patients with HCC and portal vein tumor thrombus. Among them, 286 patients showed portal vein tumor thrombus located in the primary and secondary branch of the main portal vein (group A), and 152 patients showed portal vein tumor thrombus (PVTT) involved in the main portal vein (group B). Additionally, out of 204 HCC patients with cirrhotic hypersplenism, 94 patients had hepatectomy and splenectomy, and 100 patients had only hepatectomy without hospital death. RESULTS: The 3- and 5-year survival after resection of large HCCs (over 5 cm) with improved new method in China was between 50.7 and 58.8% and 27.9 and 38.7%, respectively. Tumor recurrence in the liver within 1 year after hepatic resection + thrombectomie was detected in 45% of group A and in 78.8% in group B. The cumulative 5-year overall survival rates were 18.1% for group A and 0% for group B. The 1-, 3-, and 5-year overall survival in HCC plus portal vein tumor thrombus (PVTT) was 58.7, 22.7, and 18.1%. The hepatectomy/splenectomy group had a 5-year tumor-free survival rate of 37.2% and the hepatectomy group alone had 27.2%. CONCLUSION: The new resection methods, hepatic resection + thrombectomy and hepatectomy + splenectomy, are very effective treatments for large HCC, HCC with portal vein tumor thrombus, and HCC with hypersplenism, respectively. Local treatment modalities, e.g. percutaneous ethanol injection, cryosurgery, and radiofrequency ablation as well as microwave coagulation are used in patients with poor liver function in small and large HCCs.

Clinical significance of RBC alloantibodies and autoantibodies in sickle cell patients who received transfusions.

BACKGROUND: The clinical significance of alloimmunization to RBC antigens in sickle cell patients was analyzed by a retrospective review of the records of pediatric and adult sickle cell patients who received transfusions and who were followed over a 10-year period. STUDY DESIGN AND METHODS: Charts of pediatric and adult sickle cell patients followed at Schneider Children's Hospital (SCH) and Long Island Jewish Medical Center between 1989 and 1999 were retrieved. Patients followed at SCH were classified as pediatric, regardless of age. Data on transfusion history, alloimmunization, and transfusion reactions from 1990 were retrieved from computerized blood bank records. Transfusion history, development of alloantibodies and autoantibodies, and transfusion reactions were correlated with clinical evidence of hemolysis or other adverse reactions from the charts. All patients received ABO- and Rh-compatible blood transfusions for which a partial or extended antigen match was not performed. RESULTS: Among pediatric patients, 29 percent developed clinically significant alloantibodies, and 8 percent developed autoantibodies. Seven patients developed delayed hemolytic and/or serologic transfusion reactions, two with hyperhemolysis, two with clinical evidence of hemolysis, and three with serologic evidence only. The two patients with hyperhemolysis had received extended antigen-matched RBC transfusions to provide blood compatible with their existing antibodies. Among adult patients, 47.0 percent developed significant alloantibodies, and 9.7 percent developed autoantibodies. Five incidences of delayed hemolytic and/or serologic transfusion reactions occurred, one with hyperhemolysis and four with serologic evidence only. CONCLUSION: The alloimmunization rate is 29 percent in pediatric and 47 percent in adult sickle cell patients when partial or extended RBC antigen match is not performed. However, the delayed serologic and/or hemolytic transfusion reactions did not result in severe clinical outcome in most instances. The most important adverse event was hyperhemolysis, which may be triggered by a transfusion, but was not prevented by matching for RBC antigens. In most instances, the cause of hyperhemolysis was multifactorial.

Wandering spleen presenting as a right hypochondrial mass and intestinal obstruction.

This is a case report of a 23 year old multiparous woman who presented with intestinal obstruction and a right hypochondrial mass. Laparatomy revealed an infarcted 1.4 Kg spleen in the right lumbar region compressing the ascending colon. There was also ileal volvulus around the splenic pedicle. This is probably the first documented case of wandering spleen in the right hypochondrium, presenting as right large bowel obstruction, to be reported in our region. Wandering spleen is a rare condition, often asymptomatic, but may present as an acute abdomen. Pre-operative diagnosis is difficult and rarely made. Laboratory tests are seldom useful, but imaging studies do assist. Up to 1971 only 350 cases had been reported in the western literature. Review of English literature from 1900 to 1991 reported only 51 cases in children. In our region 11 cases were reported in Uganda between 1968 and 1971. No other literature is available from our region. Clinical presentation, aetiology, investigation, and management of wandering spleen is discussed.

Splenomegaly, hypersplenism and coagulation abnormalities in liver disease.

Splenomegaly is a frequent finding in patients with liver disease. It is usually asymptomatic but may cause hypersplenism. Thrombocytopenia is the most frequent manifestation of hypersplenism and may contribute to portal hypertension related bleeding. A number of therapies are available for treating thrombocytopenia due to hypersplenism including splenectomy, partial splenectomy, partial splenic embolization, TIPS etc. None is entirely satisfactory. Hypersplenism usually improves following liver transplantation. Therapy with cytokines such as thrombopoietin may offer hope for the future. Patients with liver disease also have abnormalities in coagulation. This is not surprising as all coagulation proteins (except for von willebrand factor vWF) and most inhibitors of coagulation are synthesized in the liver. Genetic or acquired abnormalities of coagulation may predispose to thrombosis of the hepatic or portal veins with significant clinical sequelae. An understanding of the mechanisms involved in coagulation and thrombosis is valuable in choosing from the increasing treatment options available. These include clotting factors, haemeostatic drugs and newer therapies such as recombinant factor VIIa. Splenic artery aneurysms are the most common visceral artery aneurysms in man. Rupture is frequently catastrophic. These aneurysms are being increasingly recognized in liver transplant patients and require treatment before or during transplant surgery.

[Selective portal-systemic shunts for bleeding portal hypertension]. / Derivaciones portosistemicas selectivas en el manejo de la hemorragia por varices esofagogastricas.

At the beginning of the seventies, we began to perform regularly selective shunts for the treatment of portal hypertension. In a 15 year period, 177 patients (155 with liver cirrhosis) were operated with three kinds of selective shunts: 128 with a Warren shunt, 29 with an end to end renosplenic shunt and 20 with a splenocaval shunt. 167 cases were operated in an elective fashion. The 15 years global operative mortality, was 14.4%. Operative mortality of the Child A patients, was 11.6%. Survival for the Child A group was 74.6% at 1 year, 68.2% at 5 years and 64.6% at 15 years. Incapacitating encephalopathy was observed in 6.9%, rebleeding 6.2% and shunt thrombosis in 6.2%. Portal vein alterations in the postoperative period were observed: in 13.3% a reduction in diameter ocurred and in 20.5%, thrombosis was recorded. It is concluded that when feasible, the selective shunts are the treatment of choice for portal hypertension in those patients with good liver function.

Effect of portacaval anastomosis on hypersplenism.

Leukopenia, thrombocytopenia, and hemolytic anemia occur commonly in advanced cirrhosis. Some investigators have reported that portacaval anastomosis (PCA) abolished hypersplenism while others have not found PCA to be uniformly beneficial. We compared the frequency of hypersplenism before and after admission to a controlled investigation of the effects of PCA in 52 unoperated control subjects and 38 patients with patent PCA. The two groups were followed for an average period of 5 1/2 years. On admission to the study leukopenia was present in about 2% of patients, thrombocytopenia in 6%, and hemolytic anemia in 4%. Splenomegaly was present in 48% and hypersplenism in 11%. After randomization splenomegaly disappeared more frequently in the shunted group. In addition, fewer patients with PCA developed splenomegaly for the first time after inclusion into the study than did unoperated control subjects. Leukopenia, thrombocytopenia, and hemolytic anemia, when present at inclusion into the study, disappeared with equal frequency in the shunted and unshunted patients, and appeared with equal frequency in both groups after randomization in previously unaffected patients. In no instance was hypersplenism clinically significant nor was splenectomy considered or carried out in any of these 90 patients. In additional uncontrolled studies we observed that therapeutic PCA did not affect hypersplenism differently from prophylactic PCA. We conclude that PCA has neither clinically nor statistically significant effects on hypersplenism.