An analysis of children with brucellosis associated with pancytopenia

Brucellosis produces a variety of nonspecific hematologic abnormalities. Hematologic complications of mild anemia and leukopenia have been frequently associated with acute brucellosis, but pancytopenia is less frequently seen. In this study, records of children with brucellosis aged under or equal to 16 years, admitted to Yuzuncu Yil University Hospital between 2004 and 2010, were analyzed retrospectively. Over this time period, 187 patients with brucellosis were diagnosed. Twenty-five (13.3%) of 187 patients had pancytopenia during admission to hospital. The diagnosis of brucellosis was confirmed by standard tube agglutination test in all patients; titers were 1:320 in 1 patient and 1:1280 in 24 patients. Blood culture was positive for Brucella melitensis in 3 patients (12%). Fever was the most common manifestation, followed by malaise, anorexia, sweating, and weight loss. Fever and splenomegaly were the common signs in most patients. In addition, arthritis was observed in 5 patients, and epistaxis, headache, and abdominal pain were observed in 3 patients. The common bone marrow aspiration findings consisted of increased megakaryocytes and hyperplasia of erythroid series, with a shift to the left of the granulocytic series. Histiocytic hyperplasia was observed in the bone marrow smear of 2 patients. Mild hemophagocytosis was observed in the bone marrow of 3 patients. All patients recovered completely, and their peripheral blood counts returned to normal by 2 to 6 weeks after antibiotic treatment of brucellosis. In conclusion, the authors would like to emphasize that brucellosis should be considered in the differential diagnosis of children with pancytopenia.     

Detection and treatment of brucellosis by screening a population at risk.

Brucellosis presents a difficult diagnostic challenge in view of its protean manifestations, multiple organ involvement and variable clinical course. The purpose of the present study was to determine whether active screening of a population at risk, identified through index cases, would enhance the detection rate of brucellosis and improve treatment. During a 1-month period all 98 individuals sharing risk factors with 4 diagnosed symptomatic cases of brucellosis in one Bedouin town in southern Israel were approached and 86 agreed to undergo screening. Symptomatic brucellosis was found in 8 (9%) of the screened population and an additional 5 (6%) asymptomatic individuals were found to be seropositive. These 13 were followed for 12 months. All symptomatic cases were treated and cured. Of the 5 asymptomatic seropositive individuals 2 showed a further elevation of Brucella antibody titers. One became symptomatic, was treated and was cured. This screening program provided 53% of all reported cases from the Bedouin town during the entire year of the study. Screening a population at risk increased the detection rate of brucellosis and improved the treatment.     

Brucella melitensis in blood cultures of two newborns due to exchange transfusion

Brucellosis is a zoonotic infection, transmitted to humans primarily by consumption of unpasteurized milk and milk products. Transmission by blood transfusion is possible but very unusual. Herewith we present two newborns with positive blood cultures for Brucella melitensis after exchange transfusions. However, the standard tube agglutination titers against Brucella were not elevated and the newborns did not develop brucellosis. It is suggested that, in areas endemic for brucellosis, blood donors should be questioned about symptoms of brucellosis, and if suspected, serological tests for brucellosis should be indicated before blood transfusion. At the same time, the prevalence of the disease among animals should be reduced with effective animal disease control programs.     

Calcaneal apophysitis due to brucellosis

Brucellosis is still a major health problem in many geographical areas. Osteoarticular complications are important owing to their high prevalence. We report an unusual case of childhood brucellosis presenting with septic apophysitis of the calcaneus and abscess formation in a 12-year-old boy. The patient was successfully treated with a combination of antibiotics. The importance of early recognition of the disease and differential diagnosis is emphasized. Early recognition of infection, prolonged treatment, and long-term follow-up may improve the outcome.     

Thrombocytopenic purpura as only manifestation of brucellosis in a child

Thrombocytopenic purpura associated with brucellosis has been rarely reported in the world literature. Thrombocytopenic purpura is generally part of the array of manifestations of brucellosis such as fever, arthritis, malaise and hepatosplenomegaly. We describe a nine-year-old girl who presented with thrombocytopenic purpura as the sole manifestation of brucellosis, which resolved with anti-Brucella chemotherapy. Her physical examination was remarkable for hepatomegaly of 3 cm and splenomegaly of 2 cm palpable below the costal margin. Initial laboratory investigations revealed isolated thrombocytopenia with platelet count of 11,300/mm3 and positive serology for Brucella. Thrombocytopenia resolved promptly with proper antibiotics on 7th day of treatment. Brucellosis should be included in the differential diagnosis of thrombocytopenic purpura in Brucella-endemic areas.     

Presentation, complications, and treatment outcome of brucellosis in Turkish children

Background:  Brucellosis constitutes a public health problem in Turkey. In endemic Brucella melitensis areas such as Turkey, children represent 20–25% of cases.
Methods:  Hospital records of 90 children with brucellosis admitted during a 9 year period, 1997–2006, were evaluated retrospectively.
Results:  Of 90 patients, 27 (30%) were female and 63 (70%) were male ( P  < 0.05). Patients were aged between 1 and 16 years of age. The mean age was 9.02 ± 3.59 years. Fifty-two patients (57.8%) were from rural areas of Turkey. The mode of transmission was consumption of unpasteurized milk and milk products in 64 patients (71.1%). Parents of 41 patients (45.6%) worked in animal breeding. A positive family history for brucellosis was noted in 14 patients (15.6%). The most frequently involved joint with arthralgia was the knee joint. Arthritis was found in six patients (6.7%), and five of the six had monoarthritis. Serum agglutination test was ≥1/160 in 82 patients (91.1%). The joint symptoms rapidly responded to the treatment and the majority of the patients had significant improvement. The duration of treatment was 6 weeks in 81 patients (90%). Six patients experienced relapse. Four patients presented with complications that included neurobrucellosis, discitis and sclerosis of the hip joint.
Conclusions:  Treatment of childhood brucellosis with co-trimoxazole + rifampicin or doxycycline + rifampicin, according to patient age, is effective and has low relapse rates. Complications and relapse were successfully treated with triple-drug regimens with a low sequelae rate.     

Acute focal bacterial nephritis in childhood brucellosis

Brucellosis is an infectious disease transmitted from various animal hosts to man. In children the most common route of infection is unpasteurized milk or milk products. Brucellosis affects the urinary tract uncommonly, most frequently causing orchioepididymi-tis.

We report the case of a 4-year-old boy with brucellosis who developed acute focal bacterial nephritis (AFBN), documented by sonography, gallium scan, and computed tomography. After appropriate treatment his symptoms and his sonographic abnormality resolved completely. To the best of our knowledge, this is the first report of the association of AFBN and brucellosis.

     

A cross-sectional study of the seroprevalence and flock-level factors associated with ovine and caprine brucellosis in southeastern Iran

This cross-sectional study was conducted to estimate seroprevalence and to identify flock-level factors associated with seropositivity to brucellosis in small ruminants in Kerman province, southeastern Iran. In October-November 2011, serum samples were randomly collected from 1767 sheep and 1233 goats, older than 18 months, from 300 flocks. The sera were initially screened for the presence of anti-Brucella antibodies using the Rose-Bengal test; those found to be positive were then examined by Wright and 2-mercaptoethanol Brucella agglutination tests. A questionnaire was used to collect data on flock-level factors likely associated with the within flock seroprevalence of brucellosis. The associations were statistically evaluated for significance in multivariable logistic models. Sixty three flocks (21.00%; 95% CI: 16.80-26.60) had at least one seropositive animal. The mean within-flock seroprevalence was 3.10% (95% CI: 2.60-3.90). The presence of newly purchased animals (OR=3.42; 95% CI: 1.35-8.65) was significantly associated with seropositivity. Our findings highlight the role of animal movement among flocks in the epidemiology of brucellosis in this region. Thus, a control program for brucellosis in the region is suggested to impose appropriate restrictions on animal trade and improve knowledge of livestock owners about quarantine principles for newly purchased animals.Key Words: Brucellosis seroprevalence, Flock-level risk factors, Small ruminant, Iran     

幼年皮肌炎120例临床特点及治疗随访分析

目的 研究幼年皮肌炎(JDM)的临床特征、治疗效果以及转归.方法 回顾性分析2003年12月-2011年3月在北京儿童医院住院JDM患儿120例,分析其起病情况、临床表现、实验室检查及辅助检查、治疗方法、随访和预后.结果 120例患儿男55例,女65例;发病年龄1～14岁,平均年龄7岁.患儿均有典型的皮损及不同程度的肌肉症状,83例(69%)患儿有内脏受累,最常受累系统为呼吸系统(48%).所有患儿肌酶增高,肌电图均表现为肌源性损害.120例患儿均采用糖皮质激素治疗,均在早期加用甲氨蝶呤,有肺损害及重症患儿加用环孢素或环磷酰胺.早期治疗效果及远期预后均较好,120例在急性期死亡7例,死于肺部受累并感染致呼吸衰竭5例,并巨噬细胞活化综合征2例.结论 JDM是一种少见疾病,以肌无力和皮肤损害为突出表现,其皮损具有特征性,各脏器功能评估对诊断和判断疾病严重性非常有益;糖皮质激素联合免疫抑制剂治疗JDM安全有效,且预后较好.     

Characteristics of Kawasaki disease in infants younger than six months of age

BACKGROUND: Kawasaki disease is the leading cause of acquired heart disease in childhood. However, there are only a few reports in infants younger than 6 months. The objective of this study is to investigate the clinical and laboratory characteristics of Kawasaki disease in infants younger than 6 months. METHODS: From 1994 to 2003, 120 patients with Kawasaki disease diagnosed at our institution were included. Group 1 consisted of 20 (17%) patients younger than 6 months, and group 2 consisted of 100 (83%) patients older than 6 months. Clinical manifestations, laboratory results, echocardiographic findings, treatment and outcome were compared between these 2 groups. RESULTS: Clinical manifestations (hydrops of gallbladder: 0% versus 16%, P < 0.001) and laboratory results (white blood cell count 21,740 +/- 11,706 versus 11,830 +/- 4390/mm3, P < 0.001; hemoglobin 9.98 +/- 1.25 versus 10.8 +/- 1.37 g/dL, P = 0.015; platelet 483 +/- 393 versus 355 +/- 138 x 1000/mm3, P = 0.011; triglyceride 138 +/- 77.5 versus 107 +/- 17 mg/dL, P < 0.001) were different between patients with Kawasaki disease younger and older than 6 months, respectively. Younger infants were more likely to have incomplete presentation (35% versus 12%, P = 0.025), coronary involvement (65% versus 19%, P < 0.001), late intravenous immunoglobulin treatment and relatively poor outcome. CONCLUSIONS: Infants younger than 6 months with prolonged unexplained febrile illnesses should be suspected as having Kawasaki disease, despite the incomplete clinical presentation. Because early diagnosis and timely treatment are difficult in younger infants with Kawasaki disease because of delayed and incomplete clinical presentations, echocardiogram becomes an important implement for diagnosis. Early intravenous immunoglobulin treatment is required in view of the highest risk of coronary involvement in them.     

Acute child poisonings in Oslo: a 2-year prospective study

AIM: To study the current epidemiology, clinical course and outcome of poisonings among children in Oslo and compare findings to a similar study from 1980. METHODS: Observational study with prospective inclusion of all children (<15 years of age) with a main diagnosis of acute poisoning treated in hospital or outpatient clinic in Oslo for 2 years. RESULTS: One hundred seventy-five episodes of acute poisoning were included at the outpatient clinic only (n = 65), the paediatric department only (n = 82) or both (n = 28 referrals). Annual incidence was 97 per 100 000, significantly lower than in 1980 (230 per 100 000). Highest incidence was in 1-year-old males (576 per 100 000). In children <8 years of age, the most common toxic agents were pharmaceuticals (39%) and household products (32%); children > or = 8 years ingested mainly ethanol (46%) or pharmaceuticals (36%). Five percent of all children were comatose, and complications were seen in 13%. All children survived without sequelae. Half of the admissions needed treatment; most commonly used treatments were activated charcoal (33%), gastric lavage (9%) and emetics (9%). CONCLUSION: The incidence of child poisonings in Oslo has significantly reduced since 1980. Only half of the poisonings needed treatment, most of the poisonings were mild and the clinical outcome was good.     

Pancytopenia, a rare hematologic manifestation of brucellosis in children

The records of 54 children with brucellosis were evaluated retrospectively. Among them, eight patients (14.8%) with pancytopenia were identified in a 7-year period between 1996 and 2003. Six of the eight patients with pancytopenia had Brucella melitensis isolated from blood cultures, and all eight patients had Brucella agglutination titers of at least 1:320. Agglutination test titers did not correlate with the degree of pancytopenia. Fever was the most common manifestation, followed by malaise, anorexia, sweating, weight loss, and gastrointestinal symptoms. Most patients had hepatosplenomegaly, and bone marrow aspiration specimens showed hyper-cellularity or normocellularity. Hemophagocytosis (3 patients) and histiocytic hyperplasia (4 patients) were observed in bone marrow examinations of eight patients, but bone marrow aplasia and granulomas were not detected. All children recovered completely; the pancytopenia was transient and resolved after the antibiotic treatment of Brucella infection. Brucellosis should be considered as a possible diagnosis among patients with pancytopenia.     

Acute hemorrhagic edema of infancy: the experience of a large tertiary pediatric center in Israel

Background:Acute hemorrhagic edema of infancy (AHEI) is a rare leukocytoclastic vasculitis of the small vessels occurring at a young age and considered as a benign self-limited disease.Due to its low prevalence,there are limited data on the presentation and complications of this disease.Methods:All computerized files of children who were hospitalized at a tertiary pediatric center due to AHEI over a 10 year period were reviewed.Clinical,laboratory and histopathological data were collected.Results:Twenty-six patients were included in our study,accounting for 0.7 cases per 1000 admissions of children aged 2 years or less.Mean age was 12.9 months.More than two thirds of the children had preceding symptoms compatible with a viral infection.Upon admission,all patients presented with typical findings of a rash and edema.Edema was most profound over the lower extremities (73％).Concomitant viral or bacterial infections were found in six children.Skin biopsy was performed in six patients revealing leukocytoclastic vasculitis.Thirteen children (50％) had systemic involvement including joint involvement (n=9),gastrointestinal hemorrhage (n=4),microscopic hematuria (n=1) and compartment syndrome of the limb (n=1).The latter was diagnosed in a patient with familial Mediterranean fever.Conclusions:Our largest data series highlighted what is known regarding clinical and histological findings in children with AHEI.However,contrary to what was previously reported,we found a higher rate of systemic involvement.Although AHEI is a rare entity,pediatricians should be familiar with its presentation,management and our reported complications.     

Clinical, laboratory, and epidemiologic features of murine typhus in 97 Texas children

OBJECTIVE: To document the clinical, laboratory, and epidemiologic characteristics of pediatric patients with murine typhus. DESIGN: Pediatric patients were diagnosed using serologic testing, and clinical, laboratory, and epidemiologic data were retrospectively reviewed. SETTING: Of 97 patients, 77 (79%) were identified and treated as inpatients and 20 (21%) were treated as outpatients; most resided in south Texas. PATIENTS: Between 1979 and 1996, medical records and patient-physician interviews were available for 97 patients aged 16 years and younger with murine typhus. MAIN OUTCOME MEASURES: The frequency of clinical symptoms and signs, abnormal laboratory findings, epidemiologic findings, and measures of disease severity were determined. RESULTS: The clinical triad of fever, headache, and rash occurred in only 43 (49%) of 87 pediatric patients throughout the illness. Musculoskeletal symptoms were experienced by 43% of patients, whereas gastrointestinal tract symptoms (nausea, vomiting, anorexia, and diarrhea) occurred in 77%. Systemic involvement was evident by the frequent occurrence of abnormal laboratory findings referable to multiple organ systems, including the liver, kidney, blood, and central nervous system. CONCLUSIONS: Pediatric infection by Rickettsia typhi usually causes mild to moderate systemic illness. In children, the median duration of illness was 12 days (range, 5-29 days), but severe complications were rare. Length of illness was significantly related to the initial diagnosis, whereas the interval to defervescence was related to therapy with a tetracycline or chloramphenicol. Early recognition and treatment is important to prevent prolonged morbidity.     

Esophageal candidiasis in human immunodeficiency virus-infected pediatric patients after the introduction of highly active antiretroviral therapy

OBJECTIVE: To investigate epidemiologic trends, clinical features and outcome of esophageal candidiasis in the era of highly active antiretroviral therapy in a prospectively monitored population of HIV-infected children and adolescents followed at the National Cancer Institute. PATIENTS AND METHODS: The records of all HIV-infected pediatric patients (n = 266) followed between 1995 and 2000 were reviewed for a history of esophageal candidiasis. Proven esophageal candidiasis was defined as clinical plus radiographic and/or endoscopic findings of esophageal candidiasis. Probable esophageal candidiasis was defined as esophageal symptoms that responded promptly to appropriate antifungal therapy. The medical records of all patients fulfilling these criteria were reviewed for demographic, clinical and laboratory features at presentation, as well as therapeutic interventions and outcome. RESULTS: Of the 266 patients 9 (3.4%) had 18 documented episodes of proven (n = 16) or probable (n = 2) esophageal candidiasis. A history of prior mucosal candidiasis was present in 94% of all episodes. The median CD4+ count at the time of diagnosis was 7/microl (range, 0 to 550), and the median viral load was 98000 copies/ml (range, 22916 to 1278933). Concurrent oropharyngeal candidiasis was the most common clinical presentation (72%) followed by fever (55%), odynophagia (50%) and nausea or vomiting (39%). Treatment consisted of antifungal triazoles (61%) or amphotericin B (39%). Clinical cure was achieved in 15 cases, including all patients receiving triazoles. CONCLUSION: Esophageal candidiasis persists in the subgroup of patients not responding to highly active antiretroviral therapy and in that setting may present without concomitant oropharyngeal candidiasis or typical clinical symptoms, thus underscoring the need for a high index of suspicion in children with very low CD4+ counts.     

Fetal rhabdomyoma: prenatal diagnosis, clinical outcome, and incidence of associated tuberous sclerosis complex

OBJECTIVES: We reviewed our institution's experience with fetal cardiac rhabdomyoma to document the clinical outcome and incidence of associated tuberous sclerosis complex (TSC) and compared our findings with those of patients diagnosed with cardiac rhabdomyoma after birth. STUDY DESIGN: We reviewed the medical records of all cases diagnosed prenatally and postnatally with cardiac rhabdomyoma between January 1990 and June 2002. RESULTS: Twenty fetuses with cardiac rhabdomyoma were diagnosed at 28.4+/-6.0 weeks' gestational age. Of 19 continued pregnancies, there was one spontaneous intrauterine death, and 18 were delivered at term. Although none had prenatal hemodynamic complications, after birth seven had cardiac symptoms requiring medical (n=4) or surgical intervention (n=3). On follow-up, 15 of 19 with available outcome had TSC (79%), including six with neurodevelopmental disease. Over the same period, 26 patients were diagnosed with cardiac rhabdomyoma postnatally. Most (77%) were referred for cardiac assessment after findings suggesting TSC. On follow-up, TSC was confirmed in 25 (96%), including 22 with neurodevelopmental disease. The incidence of cardiac symptoms and TSC was not statistically different between the prenatal and postnatal diagnosis groups. CONCLUSIONS: Cardiac rhabdomyomas are benign from the cardiovascular standpoint in most affected fetuses. As observed in postnatally diagnosed cardiac rhabdomyoma, TSC is diagnosed in most cases of fetal cardiac rhabdomyoma.     

Invasive pneumococcal disease in children prior to implementation of the conjugate vaccine in the Zurich region,Switzerland

Objective: To describe symptoms, disease manifestations and outcome of invasive pneumococcal disease in children prior to implementation of the pneumococcal vaccine. Patients and methods: Analysis of children younger than 16 years of age with invasive pneumococcal disease (IPD; n = 119). Children with culture‐confirmed IPD, without underlying illness at risk for invasive disease, were included. Results: IPD in 90 children (age: median 2, mean 3.2 years) included 15 with meningitis, 16 with septicaemia, 14 with bacteraemia, 24 with pneumonia and 21 with skin, bone and joint infections. Symptoms of IPD most often described were fever and gastrointestinal symptoms (abdominal pain, vomiting, or diarrhoea), and coughing. More than 90% of children with pneumonia were coughing. Most importantly, clinical signs significantly predictive for severe IPD included tachycardia for sepsis, tachypnea for pneumonia, and meningeal signs for meningitis. Leukocyte, neutrophil and platelet counts were lower and C‐reactive protein concentrations were higher on admission in children with complicated than in children with uncomplicated IPD but, due to wide overlap of these numbers, the difference was not of prognostic help to predict clinical course and outcome. Overall, 40% of children with IPD manifested complications and IPD showed a mortality rate of 6.6%. Conclusions: IPD is a serious disease with a high complication rate and mortality. The clinical signs tachycardia, tachypnea, and meningism were highly predictive for severe IPD. The initial clinical presentation and laboratory evaluation were mostly unpredictable with respect to complications and outcome in contrast to the clinical signs.     

Childhood brucellosis: a study of 102 cases

One hundred two children, 45 days to 14 years of age, with proven brucellosis were studied to illustrate the epidemiologic, clinical and laboratory findings and to assess the outcome of antimicrobial therapy. The main source of infection was the consumption of raw milk in 80% of the patients. The predominant presenting symptoms and signs were fever, arthralgia, malaise, weight loss, arthritis, hepatosplenomegaly and lymphadenopathy. Brucella melitensis was isolated from 75% of 87 patients. Diverse hematologic and biochemical abnormalities were found. Different durations and combinations of trimethoprim-sulfamethoxazole or tetracycline plus streptomycin or rifampin were used for therapy. Eight-five patients were followed for an average of 14 months. Twelve (85.7%) of 14 patients treated with two-antibiotic combinations for 3 weeks relapsed, as did 5 (8%) of 62 patients treated for at least 6 weeks (P less than 0.001). No relapses occurred in 9 patients treated with trimethoprim-sulfamethoxazole and rifampin for 8 to 12 weeks plus streptomycin for the first 3 weeks. Longer duration and combination of antibiotic therapy seem warranted to improve outcome and to prevent relapses.     

The role of magnetic resonance cholangiography in the management of children and young adults after liver transplantation

We reviewed the results of 50 magnetic resonance (MR) cholangiograms to evaluate their usefulness in directing clinical management in young patients after liver transplantation (LTx). Thirty-two patients underwent 50 MR cholangiograms on a 1.5-T unit. Studies were performed from 1 week to 16 yr after LTx. Indications included biochemical abnormalities with (n = 19) or without (n = 16) biopsy evidence for chronic rejection, sepsis (n = 14), and intractable ascites (n = 1). Original interpretations were compared to laboratory and ultrasound findings, and clinical outcome. Of 19 studies performed on 14 patients with biopsy evidence of chronic rejection, 16 were abnormal on MR (but only one was abnormal on ultrasound), resulting in corrective surgery (n = 1), re-Tx (n = 1), and endoscopic dilatation (n = 1). Of 16 studies on 16 patients with biochemical abnormalities without evidence of chronic rejection on biopsy, 14 were abnormal on MR (but only five of 13 on ultrasound), leading to corrective surgery (n = 3) and re-listing for Tx (n = 3). Thirteen of 14 studies on six patients with sepsis were abnormal on MR (five of nine were abnormal on ultrasound), identifying surgically correctable strictures (n = 2), and leading to re-Tx (n = 1) and percutaneous biliary drainage procedures (n = 2). The one patient with ascites had a normal study. We advocate usage of MR cholangiography for the detection of biliary complications after LTx, particularly in those patients who present with biochemical abnormalities that are not easily explained by acute cellular rejection or viral infection and in those with biliary sepsis.     

Xanthogranulomatous pyelonephritis in children: diagnostic and therapeutic aspects.

BACKGROUND: Xanthogranulomatous pyelonephritis (XGP) is an uncommon chronic, inflammatory disease of the kidney. Etio-pathogenesis, diagnosis and management of pediatric XGP is still obscure due to the limited number of cases. Therefore, a retrospective clinical study was carried out to present an updated picture of the entire spectrum of pediatric XGP based on our 30 years' experience covering one of the largest non-collected series treated in a single medical center. METHODS: Records of children who were treated for XGP in our unit from 1970 to 1999, inclusive, were reviewed retrospectively.Information recorded for each patient included age, sex, past medical history, clinical characteristics, diagnostic procedures,treatment methods, histopathologic findings and outcome. RESULTS: 17 children with a mean age of 6.6 +/- 0.8 years, consisting of 15 males and 2 females, were treated for XGP. Eight patients had a urological disease history and 4 of them underwent surgery for urinary calculi and exstrophia vesica repair. The most common presenting symptoms were abdominal pain, fever,weight loss and anorexia. Palpable flank mass was the most common physical examination finding. Left and right kidneys were involved in 10 and 7 patients, respectively. Diagnostic procedures were intravenous pyelography, retrograde pyelography, ultrasound scan and computerized tomography (CT). Renal calculi and/or calcifications and non-functioning kidney were the most frequent findings in radiologic investigations. By combining all these investigations, the preoperative diagnoses were XGP (n = 2), renal and/or perirenal abscess (n = 5), psoas abscess(n = 2), non-functioning kidney due to recurrent pyelonephritis(n = 4), Wilms' tumor and/or renal clear-cell carcinoma (n = 4). XGP was correctly diagnosed in only two patients based on cr findings. Drainage of the renal or psoas abscess was performed in 8 patients as an initial procedure (surgical drainage in 6 and ultrasound-guided percutaneous drainage in 2 patients). Surgical procedures included nephrectomy (n= 13), partial nephrectomy (n=2), nephrectomy and diversion of reno-colic fistula(n= 1), and renal biopsy and nephrostomy (n= 1). Operative and postoperative complications were colonic perforation (n= 3) and wound infection (n = 3). Complications were noted only in patients who underwent nephrectomy without initial drainage procedures. Histopathologic examinations showed diffuse and focal XGP in 14 and 3 patients, respectively. CONCLUSION: XGP should be included in the differential diagnosis of all children presenting with perirenal or psoas abscess, renal mass and/or non-functioning kidney associated with/or without urolithiasis. Clinical awareness and a high index of suspicion is required to achieve the correct preoperative diagnosis and appropriate management. CT seems to be the most valuable imaging method for the diagnosis. We strongly recommend percutaneous drainage of the abscess and adjunctive antibiotic therapy prior to nephrectomy to avoid complications. Complete nephrectomy is the proper treatment for the diffuse form whereas frozen section biopsies followed by partial nephrectomy are mandatory for the proper treatment of focal disease.