Expanding the mutation spectrum in ICF syndrome: Evidence for a gender bias in ICF2.

BACKGROUND: Immunodeficiency, centromeric instability, and facial anomalies (ICF) syndrome is a rare, genetically heterogeneous, autosomal recessive disorder. Patients suffer from recurrent infections caused by reduced levels or absence of serum immunoglobulins. Genetically, 4 subtypes of ICF syndrome have been identified to date: ICF1 (DNMT3B mutations), ICF2 (ZBTB24 mutations), ICF3 (CDCA7 mutations), and ICF4 (HELLS mutations). AIM: To study the mutation spectrum in ICF syndrome. MATERIALS AND METHODS: Genetic studies were performed in peripheral blood lymphocyte DNA from suspected ICF patients and family members. RESULTS: We describe 7 ICF1 patients and 6 novel missense mutations in DNMT3B, affecting highly conserved residues in the catalytic domain. We also describe 5 new ICF2 patients, one of them carrying a homozygous deletion of the complete ZBTB24 locus. In a meta-analysis of all published ICF cases, we observed a gender bias in ICF2 with 79% male patients. DISCUSSION: The biallelic deletion of ZBTB24 provides strong support for the hypothesis that most ICF2 patients suffer from a ZBTB24 loss of function mechanism and confirms that complete absence of ZBTB24 is compatible with human life. This is in contrast to the observed early embryonic lethality in mice lacking functional Zbtb24. The observed gender bias seems to be restricted to ICF2 as it is not observed in the ICF1 cohort. CONCLUSION: Our study expands the mutation spectrum in ICF syndrome and supports that DNMT3B and ZBTB24 are the most common disease genes.

Morphological variation of carotid artery bifurcation level in digital angiography.

Knowing of the level of carotid artery bifurcation (CB) is important for vascular surgery in the neck, radical neck dissections, carotid sinus baroreceptor stimulation, catheterisations, and aneurysms. The aim of this study was to determine the CB level in relation with the cervical vertebral levels, compare them on the right and the left sides, and investigate the relation of CB level with the length of neck. In this study, 100 conventional carotid angiographies were performed. The CB level was determined in relation with 10 different levels which were the levels of the cervical vertebrae and intervertebral disks, and the relation of CB level with the length of neck was investigated. The right and left CB levels of the patients were also determined, and compared. The highest level of CB was at the level of C2 vertebra, and the lowest level of CB was at the level of C6-C7 intervertebral disk in both male and female. When all patients were taken into consideration, CB level was most frequently seen at the level of C4-C5 (29%) on the right side, and at the level of C4 (26%) on the left side. The CB levels were not symmetrical in 10 female and 23 male. Knowing of the anatomical variations of CB level is important in surgical procedures. The anatomical differences must be taken into consideration since the neighbouring structures of CB change in case of variations. We believe that the results of this study will shed light to planning of all interventional methods concerning common carotid artery and its branches as well as surgery in the neck, and will help to minimise the complications.

Glycogen storage disease type 1b: an early onset severe phenotype associated with a novel mutation (IVS4) in the glucose 6-phosphate translocase (SLC37A4) gene in a Turkish patient.

Glycogen storage disease type I (GSD-I) is a group of autosomal recessive disorders that include types Ia and Ib. GSD-Ib is caused by a deficiency in the glucose-6-phosphate transporter (G6PT) caused by a mutation in the SLC37A4 gene coding for G6PT. Glycogen storage disease is characterized by poor tolerance to fasting, growth retardation and hepatomegaly resulting from accumulation of glycogen and fat in the liver and chronic neutropenia. Herein we describe a 4-month-old Turkish patient with early onset and severe typical clinical features of GSD-1b in which a novel mutation in the SLC37A4 gene was detected. After the bone marrow examination parenteral antibiotic therapy and subcutaneous granulocyte colony-stimulating factor (G-CSF) were started. Due to the severe neutropenia the patient had developed nosocomial sepsis and the dose of G-CSF was increased. After 2 months later from the initial treatment of the G-CSF he developed splenomegaly and urinary complications. Despite maximal therapy he had an extremely poor quality of life and life-threatening complications due to impaired bone marrow function. As the patient required continual hospitalization he was schedule for bone marrow transplantation.

B-cell subsets in patients with transient hypogammaglobulinemia of infancy, partial IgA deficiency, and selective IgM deficiency.

BACKGROUND: The pathogenesis of some primary humoral immunodeficiencies, such as transient hypogammaglobulinemia of infancy (THI) and immunoglobulin (Ig) A deficiency, remains unknown and can render diagnosis problematic. OBJECTIVE: In the present study, we used flow cytometry to analyze peripheral blood B-cell subsets in patients with THI and unclassified hypogammaglobulinemia (UCH), partial IgA deficiency, and selective IgM deficiency. METHODS: The study population comprised 41 patients with hypogammaglobulinemia (THI, 18; UCH, 23), 16 patients with partial IgA deficiency, and 16 patients with selective IgM deficiency who were admitted to Ankara University Department of Pediatric Immunology-Allergy between January 2010 and April 2011, as well as 29 healthy controls. B-cell subsets were examined according to the EUROclass classification. RESULTS: Age at diagnosis in the hypogammaglobulinemia group ranged between-14 months and 13 years (median, 26 months). Naive B-cell percentages were significantly higher and activated B-cell values lower in the THI patients than in the UCH patients and age-matched healthy controls. Nonswitched (IgM+CD27+IgD+) memory B-cell values were found to be significantly lower in patients with selective IgM deficiency than in healthy controls. No significant differences in B-cell subsets were found in patients with partial IgA deficiency. CONCLUSIONS: Previous reports show that reduced class-switched memory B cell values are associated with CVID, THI, and selective IgA deficiency. Our findings did not support these reports. Furthermore, we observed that naive B cell values were higher in patients with THI. A maturation defect could play a role in the pathogenesis of THI.

B-Cell Subsets in Patients With Transient Hypogammaglobulinemia of Infancy, Partial IgA Deficiency, and Selective IgM Deficiency

Antecedentes: La patogénesis de algunas inmunodeficiencias primarias tales como la hipogammaglobulinemia transitoria de la infancia (THI), el déficit de IgA permanece desconocida y a veces es motivo de un problema en su diagnóstico. Objetivos: El motivo de este estudio fue analizar mediante citometría de flujo, las subclases de células B en sangre periférica en pacientes con THI junto con hipogammaglobulinemias no clasificadas (UCH), deficiencias parciales de IgA y deficiencias selectivas de IgM. Métodos: Se incluyeron 41 pacientes con hipogammaglobulinemia (THI: 18 y UCH: 23 pacientes), 16 con déficit parcial de IgA, 16 con deficiencia selectiva de IgM y 29 controles sanos admitidos en Ankara University, Departamento de Pediatría e Inmunología -Alergia. Se examinaron las subclases de células B de acuerdo con la clasificación Euroclass. Los pacientes fueron vistos entre enero de 2010 y abril de 2011. Resultados: En el grupo de hipogammaglobulinemia, la edad en el diagnóstico se encontraba en un rango entre 14 meses y 13 años (Med: 26 meses). Las células B naive se encontraban significativamente elevadas y las células B activadas disminuidas en el grupo THI respecto al grupo UCH y los controles sanos. Las células B memoria (IgM+ CD27+ IgD+) se encontraban significativamente disminuidas en pacientes con diagnóstico de déficit selectivo de IgM. En la deficiencia parcial de IgA no se encontraron diferencias en las subclases de células B. Conclusiones: Nuestros resultados no confirman resultados previos de una reducción de células B memoria relacionada con CVID, THI y selectiva deficiencia de IgA. Encontramos un aumento de células B naive en pacientes con hipogammaglobulinemia transitoria, sugiriendo un defecto de maduración que puede jugar un papel en la patogénesis de esta enfermedad (AU)

Background: The pathogenesis of some primary humoral immunodeficiencies, such as transient hypogammaglobulinemia of infancy (THI) and immunoglobulin (Ig) A deficiency, remains unknown and can render diagnosis problematic. Objective: In the present study, we used flow cytometry to analyze peripheral blood B-cell subsets in patients with THI and unclassified hypogammaglobulinemia (UCH), partial IgA deficiency, and selective IgM deficiency. Methods: The study population comprised 41 patients with hypogammaglobulinemia (THI, 18; UCH, 23), 16 patients with partial IgA deficiency, and 16 patients with selective IgM deficiency who were admitted to Ankara University Department of Pediatric Immunology- Allergy between January 2010 and April 2011, as well as 29 healthy controls. B-cell subsets were examined according to the EUROclass classification. Results: Age at diagnosis in the hypogammaglobulinemia group ranged between 14 months and 13 years (median, 26 months). Naïve B-cell percentages were significantly higher and activated B-cell values lower in the THI patients than in the UCH patients and age-matched healthy controls. Nonswitched (IgM+CD27+IgD+) memory B-cell values were found to be significantly lower in patients with selective IgM deficiency than in healthy controls. No significant differences in B-cell subsets were found in patients with partial IgA deficiency. Conclusions: Previous reports show that reduced class-switched memory B cell values are associated with CVID, THI, and selective IgA deficiency. Our findings did not support these reports. Furthermore, we observed that naive B cell values were higher in patients with THI. A maturation defect could play a role in the pathogenesis of THI (AU)

Bacille Calmette-Guérin lymphadenitis and recurrent oral candidiasis in an infant with a new mutation leading to interleukin-12 receptor beta-1 deficiency.

Mendelian susceptibility to mycobacterial diseases (MSMD) is a rare syndrome characterized by predisposition to infections caused by weakly virulent mycobacteria, such as those in bacille Calmette-Guérin (BCG) vaccine and environmental mycobacteria. Salmonellosis has been reported in almost half of affected patients. Patients are also vulnerable to Mycobacterium tuberculosis infection. Several other infectious diseases may occur, albeit rarely. Mucocutaneous candidiasis is more common. Interleukin-12 receptor beta1 (IL-12Rbeta1) deficiency is the most frequent genetic cause of MSMD. Here, we describe an infant with a single episode of BCG lymphadenitis who also suffered from recurrent oral candidiasis. Genetic analysis revealed a new homozygous mutation (64+1G>T) in the IL12RB1 gene that caused complete IL-12R1beta1 deficiency. IL-12Rbeta1 deficiency should be considered in patients with BCG infection, even in those who experience a single episode of BCG lymphadenitis or recurrent mucocutaneous candidiasis. Every attempt should be made to heighten awareness in countries where BCG vaccination is performed.

CD27 expression on lymphocyte and sCD27 levels in children with asthma

Background: CD27, a lymphocyte specific member of the Tumour Necrosis Factor- Receptor (TNF-R) family is expressed on the majority of peripheral blood T cells. Activation of T cells via TCR/CD3 induces high CD27 surface expression and release of a soluble form (sCD27) of the molecule. sCD27 level increases in patients suffering from a variety of chronic inflammatory diseases. In the present study we aimed to measure both the serum sCD27 levels and CD27 expression on T cells in asthmatic patients, to evaluate the state of this molecule in allergic inflammation. Methods: Forty-three patients with asthma were included in to the study. CD27 molecule expression and soluble form of this molecule were analysed in atopic asthmatic (n:17) and non-atopic asthmatic (n:13) patients receiving inhaled corticosteroid treatment, in asthmatic patients whose treatment ceased at least for 6 months (n:13) and healthy control subjects (n:14). Results: There were no differences in the expression of CD27 molecule on peripheral blood lymphocyte nor in its soluble form sCD27 levels in sera between the atopic asthmatic and non-atopic asthmatic patients receiving ICS treatment, treatment free asthmatic patients and healthy control subjects. Conclusions: Neither the soluble form of CD27 nor its expression on T cells seem to be a reliable marker of atopic or non-atopic asthmatic inflammation

CD27 expression on lymphocyte and sCD27 levels in children with asthma.

BACKGROUND: CD27, a lymphocyte specific member of the Tumour Necrosis Factor- Receptor (TNF-R) family is expressed on the majority of peripheral blood T cells. Activation of T cells via TCR/CD3 induces high CD27 surface expression and release of a soluble form (sCD27) of the molecule. sCD27 level increases in patients suffering from a variety of chronic inflammatory diseases. In the present study we aimed to measure both the serum sCD27 levels and CD27 expression on T cells in asthmatic patients, to evaluate the state of this molecule in allergic inflammation. METHODS: Forty-three patients with asthma were included in to the study. CD27 molecule expression and soluble form of this molecule were analysed in atopic asthmatic (n:17) and non-atopic asthmatic (n:13) patients receiving inhaled corticosteroid treatment, in asthmatic patients whose treatment ceased at least for 6 months (n:13) and healthy control subjects (n:14). RESULTS: There were no differences in the expression of CD27 molecule on peripheral blood lymphocyte nor in its soluble form sCD27 levels in sera between the atopic asthmatic and non-atopic asthmatic patients receiving ICS treatment, treatment free asthmatic patients and healthy control subjects. CONCLUSIONS: Neither the soluble form of CD27 nor its expression on T cells seem to be a reliable marker of atopic or non-atopic asthmatic inflammation.

Correction of displaced peritoneal dialysis catheters with an angular stiff rod.

BACKGROUND: Fluoroscopically guided guidewire manipulations are readily available and inexpensive methods of correcting malfunctioning peritoneal dialysis catheters, with reported success rates ranging from 25% to 67%. PURPOSE: To improve the success rates of guidewire manipulations with a modified technique. MATERIAL AND METHODS: Using a stiff rod and a stiff wire under fluoroscopy guidance, catheters that had migrated were drawn back into the rectovesical pouch. An angular rod was used to lever the catheter downward, and the guidewire was used to push the catheter down. RESULTS: No complications developed, and immediate success was achieved in 13 of 14 interventions. With this technique, catheter patency in chronic ambulatory peritoneal dialysis (CAPD) patients (11/12) was higher than that of previously reported methods. Durable success was maintained in nine of 12 patients after a single intervention. All re-manipulations (2/2) were successful. CONCLUSION: Although used in only 14 interventions in 12 patients, the outcome was promising. This method is a safe and favorable alternative to other guidewire manipulations, based on absence of complications and high success.

Endovascular stent placement in patients with hepatic artery stenoses or thromboses after liver transplant.

Hepatic artery stenosis or thrombosis following liver transplant is a potentially life-threatening complication. Successful liver transplant depends on uncompromised hepatic arterial inflow. Early diagnosis and treatment of complications prolong graft survival. Interventional radiologic techniques are frequently used to treat hepatic artery complications. Twenty patients with hepatic artery stenoses (n = 11) or thromboses (n = 9) were included in this study. Eighteen of the 20 patients were successfully treated by stent placement. In 9 patients, early endovascular interventions were performed 1 to 7 days after surgery. Two patients were operated owing to the effects of dissection and bleeding from the hepatic artery. Repeat endovascular interventions were performed 10 times in 6 patients. Follow-up ranged from 5 months to 4.5 years. Nine patients with patent hepatic arteries died during follow-up owing to reasons unrelated to the hepatic artery interventions. In 3 patients, the stents became occluded at 3, 5, and 9 months after surgery but no clinical symptoms were present.

Accuracy of multidetector computed tomographic angiography for detecting hepatic artery complications after liver transplantation.

AIMS: The aim of this study was to evaluate the accuracy of multidetector computed tomographic angiography (MDCTA) for detecting hepatic artery complications after liver transplantation. METHODS: Between July 2001 and September 2006, 212 patients underwent liver transplantation including 110 (41 female and 69 male patients); of mean age, 24 years (range=6 months to 66 years) who were assessed with MDCTA. First, arterial phase images obtained after intravenous injection of 150 mL of contrast at a rate of 4 mL/s were acquired using the bolus triggering technique. Then portal and late-phase images were obtained. Axial and coronal maximum intensity projection (MIP) images and volume-rendered images were produced from the axial image data. Arterial vascular complications were noted. Stenosis was defined as severe (>75%), moderate (>or=50%), or mild (<50%) according to its diameter. Twenty-nine of the 38 individuals with hepatic artery complications detected by MDCTA had correlative digital subtraction angiography (DSA). Seven of 110 patients with normal hepatic artery and venous pathologies in MDCTA also had DSA to investigate venous complications. RESULTS: MDCTA showed hepatic artery complications in 38 of the 110 patients who were assessed with this modality. DSA confirmed the MDCTA findings in all but 1 of the 29 patients assessed with catheter angiography. Fourteen of the 38 individuals also underwent percutaneous interventions and treatment. Fifteen patients had early hepatic artery complications, and 23 late hepatic artery complications. The most common early complications were thrombosis (66.6%) and stenosis (26.6%). The most common late complications were stenosis (56.5%) and thrombosis (26%). If we evaluate the early and late complications, the incidence of late complications was greater than that of the early complications (61% vs 39%). There was no statistically significant difference in cadaveric and living donor liver transplants for early versus late or for type of complications. CONCLUSIONS: MDCTA is noninvasive imaging modality that accurately shows a variety of vascular complications after liver transplantation. We suggest that if we suspect any vascular complication with Doppler ultrasound, we must perform MDCTA for diagnosis. If we detect severe/moderate stenosis, the patient must undergo DSA.

Arterial steal syndrome after orthotopic liver transplantation.

Arterial steal syndrome after orthotopic liver transplantation (OLT) is characterized by arterial hypoperfusion of the graft, which is caused by a shift in blood flow into the splenic or gastroduodenal arteries. In this report, we present mechanisms by which this syndrome caused ischemia in our patients. Steal was suspected by elevated levels of liver enzymes and the results of Doppler ultrasonography and computed tomographic angiography; it was confirmed by celiac angiography. Patients with established hepatic arterial thrombosis before angiography were excluded from this study. Steal was treated by embolization with a coil or by placement of an endoluminal narrowing stent. Ten patients at our institution (seven men and three women; mean age, 24.7 +/- 11 years; range, 6 to 40 years) exhibited biochemical evidence of liver ischemia and graft failure at 1 to 170 days after having undergone orthotopic liver transplantation. Nine of those patients had splenic steal, and one had both splenic and left gastric artery steal syndrome. None of the patients had gastroduodenal artery steal syndrome. The eight patients with splenic steal syndrome and the patient with both splenic and left gastric steal syndrome were treated by transcatheter occlusion with a coil. The remaining patient with splenic steal syndrome was treated with an endoluminal narrowing stent placement. All patients improved clinically within 24 hours after treatment, exhibiting significant changes in their biochemical and radiological parameters. Follow-up ranged from 1 to 22 months (mean, 6.7 +/- 6.6 months). One patient died from sepsis 1 month after having undergone coil embolization. He had no vascular anomalies at the time of death. We conclude that steal is a significant problem after OLT. Embolization and stenting are minimally invasive and successful treatments for steal, usually resulting early clinical improvement.

Stent placement in pediatric patients with hepatic artery stenosis or thrombosis after liver transplantation.

Hepatic artery stenosis (HAS) and thrombosis (HAT) after orthotopic liver transplantation remain significant causes of graft loss. Postoperative HAT follows approximately 5% to 19% of orthotopic liver transplantation. It is seen more frequently in pediatric patients. In the past, repeat transplantation was considered the first choice for therapy. Recently, interventional radiological techniques, such as thrombolysis, percutaneous transluminal angioplasty, or stent placement in the hepatic artery, have been suggested, but little data exist related to stent placement in the thrombosed hepatic artery during the early postoperative period in pediatric patients. Between March 2000 and March 2005, percutaneous endoluminal stent placement was performed in seven pediatric liver transplant patients. HAT or HAS initially diagnosed in all cases by Doppler ultrasound then confirmed angiographically. We intervened in four cases of hepatic artery stenosis and three cases of hepatic artery occlusion. Stents were placed in all patients. Three ruptures were seen during percutaneous transluminal angioplasty of the hepatic artery using a covered coronary stents on the first, fifth day, or 17th postoperative day. In one patient, dissection of the origin of the common hepatic artery developed owing to a guiding sheath, and a second stent was placed to cover the dissected segment. The other two hepatic artery stents remained patent. In one stent became occluded at 3 months after the intervention with no clinical problems. Follow-up ranged from 9 to 40 months. In conclusion, early and late postoperative stent placement in the graft hepatic artery was technically feasible.

Renal autotransplantation for the treatment of complex renovascular hypertension.

OBJECTIVE: In individuals with complicated renal vascular disease, renal autotransplantation has been used as an alternative to percutaneous transluminal angioplasty, which may be unsuccessful or hazardous in these situations. We evaluated the outcomes of renal autotransplantation. PATIENTS AND METHODS: Between February 1989 and December 2005, we performed 5 renal autotransplantation procedures. The surgical strategy included renal explantation, ex vivo renal preservation, ex vivo reconstruction of the renal artery if necessary, and renal heterotopic autotransplantation. RESULTS: The study subjects (3 men and 2 women) exhibited one of the following indications for surgery: fibromuscular dysplasia (2 patients), Takayasu's arteritis (1), or atherosclerosis (2). All patients exhibited uncontrolled hypertension before renal autotransplantation. Renal arteries of patients were anastomosed either to the external or internal iliac arteries or to both when there were multiple renal arteries. The renal vein was anastomosed end-to-side to the external iliac vein, and ureteral reimplantation was not performed. Mean posttransplantation follow-up was 9.8 +/- 5.7 years (range, 1-16 years). Mortality and morbidity were not observed during the follow-up, and hypertension and renal function normalized or improved in all 5 patients. CONCLUSIONS: Renal autotransplantation is a highly effective procedure to treat complex renovascular lesions; ex vivo renal repair is a safe and effective surgical procedure in the clinical setting.

Effect of secondary interventions on patency of vascular access sites for hemodialysis.

PURPOSE: To determine the impact of secondary procedures performed to maintain arteriovenous fistula (AVF) and arteriovenous graft (AVG) patency. METHODS: There hundred and eighty six vascular access procedures were retrospectively evaluated. 156 (40.4%) patients required radiological interventions to treat acute thrombosis, swelling of the extremity with the access site, insufficient hemodialysis, or stenosis at an anastomotic site. RESULTS: The 386 cases comprised 106 AVGs and 280 AVFs. In 138 of the 156 cases, which required a radiological intervention, the treatment was successful and saved the vascular access site. The unassisted post-intervention patency time for these 138 successful cases was 13.1 +/- 12 months (range, 1-65 months). Twenty-nine (63%) of the 46 access sites treated with surgical thrombectomy were saved. CONCLUSIONS: Frequent, regular follow-up of hemodialysis patients with vascular access sites is the best way to diagnose problems early and allow the best chance of long-term function.

Ureteroscopic treatment of proximal ureter stones with the aid of an antegrade occlusion balloon catheter.

PURPOSE: To define the role of an antegrade occlusion balloon catheter in preventing migration of proximal ureteral stones to the dilated proximal ureter during endoscopic treatment. MATERIAL AND METHODS: An occlusion balloon catheter was used in 8 of 21 patients with proximal ureteral stones who underwent ureterorenoscopy. Five of the eight patients had solitary kidneys admitting with anuria and had percutaneous nephrostomy. In the other three patients, percutaneous nephrostomy and occlusion balloon catheters were placed a day before the procedure, since these patients had total obstruction and massive dilatation of the proximal ureter and renal collecting system. The balloons of occlusion catheters were inflated with 1 ml of sterile saline proximal to the stones just before ureterorenoscopy. RESULTS: All stones could be reached by ureterorenoscopy and treated successfully with the aid of an ultrasonic lithotripter, and no stone migration to the upper dilated collecting system was observed. Just after the operation, while the patient was still lying on the operation table, the occlusion catheter was removed. The nephrostomy catheter was removed a day later. All patients were totally stone-free after the procedures. CONCLUSION: Occlusion balloon catheters increase the ureteroscopic treatment success rate in proximal ureter stones. This should be kept in mind especially when dilatation of the proximal collecting system is prominent and in cases with unsuccessful previous intervention with a retrograde stone cone catheter.

Comparison of two different percutaneous splenic artery interventions in the treatment of hypersplenism: preliminary report.

BACKGROUND: Splenomegaly and hypersplenism occur in patients with chronic liver disease and liver transplant recipients. The traditional treatment for hypersplenism is surgical removal. Percutaneous interventional methods, such as partial splenic embolization, are alternatives to surgery for hypersplenism. This article gives preliminary findings for a new percutaneous technique in which a narrowed stent is placed in the splenic artery. METHODS: The study focused on 10 patients (eight males and two females) who were treated for hypersplenism. Partial splenic embolization was performed in six patients (age range, 1-43 years) who were waiting for liver transplantation, and narrowed stents were placed in four patients (age range, 12-47 years) who had undergone either orthotopic two patients) or heterotopic two patients) liver transplantation. For embolization, the splenic artery was catheterized and polyvinyl alcohol particles were infused to the distal branches, reducing blood flow in the spleen by 40% to 50%. In the other cases, a narrowed stent was deployed to the middle portion of the splenic artery. RESULTS: Hypersplenism was successfully treated in all 10 cases. Compared with partial splenic embolization, placement of narrowed stents was associated with lower frequencies of postintervention fever and pain, shorter hospital stay, and decreased need for antibiotics. In addition to treating hypersplenism, narrowed-stent placement also completely resolved splenic artery steal syndrome in the two patients (orthotopic liver transplant recipients) with this condition. CONCLUSION: Percutaneous placement of a narrowed stent in the splenic artery is a promising new technique for treating hypersplenism and splenic arterial steal syndrome.

Polyarteritis nodosa complicated by intrahepatic-perihepatic hemorrhage and acute appendicitis: successful treatment with cyclophosphamide and corticosteroids.

Polyarteritis nodosa (PAN) is a systemic vasculitic disease characterised by necrotising inflammation of small and medium-sized arteries. Abdominal complications due to PAN are rare, but the death rate for these cases is high. We describe a serious case of PAN that involved intrahepatic-perihepatic haemorrhage and acute appendicitis. Exploratory laparotomy and appendectomy were performed, and the patient was successfully treated with cyclophosphamide and corticosteroids.