Tobacco counseling at well-child and tobacco-influenced illness visits: opportunities for improvement

We describe 2 children with cerebrovascular events caused by emboli from left atrial myxomas and review 7 other pediatric cases from the literature. Transient cutaneous eruptions involving the extremities preceded the cerebrovascular events and were most likely attributable to fragmentation of the atrial tumor with peripheral embolization. Our first case demonstrates the more common presentation with acute hemiplegia caused by cerebral infarction and the second case a transient ischemic attack manifested by more subtle features as a result of involvement of the vertebrobasilar circulation. Neither child had a history or other signs of cardiac disease. Atrial myxoma should be considered in the differential diagnosis when children present with neurologic symptoms or with signs of embolization, because surgical removal of the tumor is critical and may be curative.     

Pediatric West Nile virus infection: neurologic disease presentations during the 2002 epidemic in Cuyahoga County, Ohio

Knowledge is currently limited about West Nile virus (WNV) infection and its sequelae among children. Available evidence suggests that when compared with adults, children less than 18 years old can be at high risk for WNV exposure and infection yet manifest a lower risk for WNV-related morbidity and mortality. We detail clinical features of pediatric West Nile-associated neurologic disease (WNND) epidemic cases in Cuyahoga County during 2002. We present a structured review of pediatric and adult WNND cases hospitalized in Cuyahoga County, Ohio. During the epidemic, 5 children were hospitalized with confirmed WNND (estimated incidence = 1.4/100,000 children 5-17 years old at risk). Compared with adults, children had shorter hospitalization (mean, 4.6 versus 12.3 days), fewer neurologic symptoms, better neurologic outcomes, and lower mortality (0% versus 5.3%). Cerebrospinal fluid results were similar. When compared with adults, children had significantly lower rates of WNND. Children are at a decreased risk for severe WNV and less likely to present with neurologic signs or suffer neurologic sequelae.     

Acute Pericarditis in Childhood: A 10-Year Experience

Twenty children, aged 6 months to 13 years, with acute pericarditis admitted between 1987 and 1997 to a university hospital were analyzed retrospectively for their etiology, presentation, management, and prognosis. The most common types of pericarditis were purulent (40%), collagen vascular disease (30%), viral (20%), and neoplastic disease (10%). Most children presented with chest pain, fever, and tachypnea, but cardiac tamponade was not seen in any children. Staphylococcus aureus was the most frequent causative organism of purulent pericarditis and septic arthritis was the most common concurrent infection in the patients. Surgical drainage was performed for 11 cases, 9 underwent subxiphoid pericardial window, and 2 underwent thoracotomy. There was no constrictive pericarditis or reaccumulation of fluid after surgery. Two children died, one of staphylococcal septicemia and the other had a malignant mediastinal tumor. The remaining 18 made a complete recovery. We conclude that subxiphoid pericardial drainage is a simple, safe, and quick procedure and can be done easily in general hospitals by pediatric surgeons. The expensive facilities of cardiac surgeries are not needed.     

Clinical presentation and outcome of epidemic Kaposi sarcoma in Ugandan children

Background

Kaposi sarcoma (KS) is one of the most common pediatric cancers in sub‐Saharan Africa. Few data are available about the clinical presentation or response to treatment of children with epidemic (HIV‐associated) KS.

Methods

Medical records of all children with KS and HIV infection referred to the Uganda Cancer Institute in Kampala, Uganda from October 2004 to June 2007 were reviewed. Charts were abstracted for age, sex, location of KS lesions at presentation, biopsy results, CD4 T‐cell count and percentage, and KS treatment and outcome.

Results

Seventy‐three children with epidemic KS were identified, 37 males and 36 females. The median age was 10.1 years (range 2–18). KS presented with lymph node (LN) involvement in 60% of cases. The median absolute and percentage CD4 T‐cells at presentation were 210 cells/µl and 7.4%, respectively. Those children with lymphadenopathic KS were younger (mean difference 3.7 years; P = 0.01) and had higher CD4 T‐cell counts (mean difference 242 cells/µl; P = 0.03) than those without LN involvement. Of 32 patients for whom outcome data were available, a complete response to chemotherapy and/or antiretroviral therapy was documented in 20 (62.5%) patients.

Conclusions

In comparison to cutaneous involvement, LN involvement of epidemic KS occurs at younger ages and at higher CD4 levels. This clinical presentation may reflect recent infection with human herpesvirus 8 followed by a rapid progression to malignancy. Favorable response to treatment was observed in the majority of cases, but prospective studies are needed to determine optimal management. Pediatr Blood Cancer 2010;54:670–674. © 2010 Wiley‐Liss, Inc.     

Nosocomial intestinal infections in an infant ward. The importance of phone inquiries of the families]

Intestinal nosocomial infections remain a major concern in pediatric wards where they occur synchronously with syncytial respiratory virus infections. MATERIAL AND METHODS: During the 1997-1998 winter, we systematically listed the intestinal nosocomial infections that emerged in the pediatric wards of the Clermont-Ferrand hospital. After their discharge, the families of the children who were not hospitalized for acute diarrhea were contacted by telephone. RESULTS: Among 817 hospitalized children, 27% were admitted to the hospital for acute epidemic gastroenteritis. One hundred forty-four children were listed as having nosocomial gastroenteritis, 96 (66.6%) during the hospital stay and 48 (33%) after discharge, which represents 5.8% of all the admissions. Intestinal nosocomial infections increased with the number of admissions per day and per month, and occurred in 28.1% of the cases of syncytial respiratory virus bronchiolitis that concern children of the same age. Rotavirus was found in 28% of nosocomial infections, yet it was responsible for 85.5% of the hospital readmissions for nosocomial infections. CONCLUSION: Further studies should try to find solutions to decrease the frequency of those concomitant diseases.     

Early cardiac involvement in children carrying the A3243G mtDNA mutation

The phenotypic spectrum of the mitochondrial A3243G DKA mutation is highly variable, particularly when occuring in childhood. In contrast to the classical presentation in adulthood (MELAS syndrome; mitochondria! myopathy, encephalopathy, lactic acidosis and stroke-like episodes) children show a different pattern of symptoms, often without the typical encephalopathy or psychomotor regression. We present six children carrying the A3243G mtDNA mutation with a heteroplasmy above 50 % in muscle tissue. The age of diagnosis ranged from 2 weeks up to 14.5 years. The clinical presentation was rather non-specific including muscle weakness, developmental delay and epilepsy. In this small pediatric group we detected presymptomatic cardiac involvement in five out of six children already at an early stage of disease. The cardiac pathology included cardiomyopathy and biventricular hypertrophy with rhythm disturbances (for example long QT-syndrome). The observed cardiac changes do not always increase the risk of cardiac deterioration; however, two of our patients died early on. CONCLUSION: We hypothesize that the A3243G mutation might be underdiagnosed, as patients could suffer from an unexplained cardiac death before the diagnosis is made. We advise performing regular repeated ECGs and echocardiography in all children carrying a A3243G mtDNA mutation independently from the presence of cardiac symptoms.     

The clinical significance of lung hypoexpansion in acute childhood asthma

Background Many children experiencing acute asthmatic episodes have chest radiographs, which may show lung hyperinflation, hypoinflation, or normal inflation. Lung hypoinflation may be a sign of respiratory fatigue and poor prognosis.Objective To compare the clinical course in children with asthma according to the degree of lung inflation on chest radiographs.Patients and methods We conducted a retrospective study during a 24-month period (from July 1999 to July 2001) of children aged 0–17 years, who presented to a pediatric emergency department or outpatient clinic with an asthma exacerbation. Chest radiographs obtained at presentation were reviewed independently by three pediatric radiologists who were blinded to the admission status of the patient. The correlation between hypoinflation and hospital admission was assessed in three age groups: 0–2 years, 3–5 years, and 6–17 years.Results Hypoinflation on chest radiographs was significantly correlated with hospital admission for children aged 6–17 years (odds ratio 16.00, 95% confidence interval 1.89–135.43). The inter-reader agreement for interpretation of these radiographs was strong, with a kappa score of 0.76. Hypoinflation was not correlated with admission in younger children.Conclusion Lung hypoinflation is associated with a greater likelihood of hospital admission in children aged 6 years or older. Therefore, hypoinflation was a poor prognostic sign and may warrant more aggressive therapy.     

The association of overweight and ankle injuries in children.

OBJECTIVES: Overweight children are at increased risk for many medical problems. Trauma is the leading etiology of childhood morbidity and mortality. No previous study has evaluated the association between overweight and acute ankle injuries in children. We hypothesized that being overweight is associated with an increased risk of ankle injury in children. METHODS: We conducted a case-control study in an urban pediatric emergency department. Subjects aged 5 to 19 years were recruited from June 2005 through July 2006. Children with acute ankle trauma were enrolled as cases. A convenience sample of children with a chief complaint of fever, headache, or sore throat was enrolled as controls. Demographic information and anthropometric measurements were obtained. Age- and gender-specific body mass index percentiles (BMI-Ps) were calculated using pediatric norms. Multivariate unconditional logistic regression was used to assess the relationship between overweight and ankle injury, adjusting for demographic variables. Through medical records, we obtained demographic information and weight, but not height, of all cases that were not enrolled. This allowed us to conduct a sensitivity analysis in which we combined the enrolled and nonenrolled cases into a single case group and made increasingly more unlikely assumptions about the height percentiles of the nonenrolled cases. RESULTS: One hundred eighty cases and 180 controls were enrolled in the study. We observed a significant association between overweight and ankle injury (multivariate-adjusted odds ratio 3.26, 95% confidence interval, 1.86-5.72; P value for trend <.0001). Although this result may be an overestimate of the magnitude of the association due to a possible bias in the selection of cases, sensitivity analysis demonstrated the robustness of the statistical significance of the finding. CONCLUSIONS: Overweight children may be at increased risk of ankle injury.     

Meningitis in pediatric cancer patients: a review of forty cases from a single institution.

BACKGROUND: Although the clinical features of bacterial meningitis in adult cancer patients and in healthy children have been described, no previous large series has described the clinical features of meningitis in pediatric cancer patients. We performed a retrospective review of bacterial or fungal meningitis in pediatric cancer patients to determine its clinical presentation, microbiology and outcome. METHOD: We reviewed the medical records of all patients younger than 18 years old with a diagnoses of any malignancy and bacterial or fungal meningitis at Children's Hospital and Regional Medical Center in Seattle, WA, from January, 1981, to June, 1998. RESULTS: During the study period there were 40 cases of bacterial or fungal meningitis in 36 pediatric cancer patients. Most patients (65%) had recent neurosurgery, a central nervous system device or cerebrospinal fluid leak. Neutropenia was present in 30% of patients. Fever and altered mental status were the most consistent signs at presentation. In addition at least one additional symptom or sign of meningitis (headache, neck pain or rigidity, seizures or photophobia) was present in 77% of cases. Staphylococcus aureus and Streptococcus pneumoniae were the most common microbiologic isolates. The five patients with fatal outcome were neutropenic. Neutropenia and seizures within 2 days of presentation were associated with long neurologic sequelae. CONCLUSIONS: Meningitis in pediatric cancer patients was associated with significant morbidity and mortality. Pediatric cancer patients with meningitis had clinical features and microbiology distinctly different from those of adult cancer patients and normal children with meningitis.     

Emergency department presentation of pediatric stroke

Pediatric stroke is not a common occurrence. When compared with adults, the pediatric population has a much more diverse group of risk factors, and while numerous rare congenital disorders are possible, most known etiologies are cardiac, vascular, or hematologic. The emergency department (ED) presentation of pediatric stroke does not differ greatly from that of adults, although posterior circulation ischemia is less common, and neurologic findings may be more difficult to recognize. ED treatment is also largely the same, with an attention to resuscitation and avoidance of hypoxia, hypotension, hyperthermia, and changes in blood sugar. Use of specialized agents such as aspirin and heparin should be considered in certain cases. It is important for the emergency physician to recognize acute neurologic events in pediatric patients to minimize complications.     

监测心排量在重症手足口病患儿中的临床应用

目的：部分重症手足口病患儿有明显心功能改变,心功能衰竭是其致死的主要原因,对重症患儿进行左心功能检测对其救治具有重要意义。本研究探讨监测重症手足口病患儿心排量的临床应用价值。方法：选取2011年4～9月入住儿科重症监护室的重症手足口病患儿107例,临床分期为2、3、4期分别有73、23、11例。采用超声心输出量监测仪（USCOM） 床旁监测患儿的心排量,包括心输出量、每搏输出量。其中95例患儿进行了MRI检查,并按照不同MRI结果分组。MRI异常41例（其中9例延髓受累）,未见异常54例。比较不同临床分期及不同MRI结果组间患儿心排量情况。结果：与临床2、3期患儿比较,临床4期患儿心排量明显降低（P<0.05）;心排量在正常、异常MRI组间比较差异无统计学意义,但延髓受累组患儿心排量明显低于其他部位受累组及MRI正常组（P＜0.05）。结论：心排量明显降低提示病情危重,并提示病变可能已累及脑干的心血管中枢位置,监测心排量对重症患儿的救治具有指导意义。     

Focal encephalitis with enterovirus infections

We report on four pediatric patients with Enterovirus infections who were admitted to the hospital with signs or symptoms of acute, focal encephalitis. All four experienced focal seizures. Each had a cerebrospinal fluid pleocytosis at the initial lumbar puncture. In all four patients the diagnosis of herpes simplex encephalitis was entertained. Each child improved spontaneously within a few days of admission to the hospital, and only one had residual neurologic abnormalities at the time of discharge. A brief review of these cases, and three additional cases from the literature, indicate that the enteroviruses, particularly the group A Coxsackieviruses, are rare causes of acute focal encephalitis in children and adolescents.     

Hemorrhagic stroke associated with pulmonary edema and catastrophic cardiac failure

Cerebral arteriovenous fistula (AVF) is a vascular malformation that is rare in the pediatric population. Older children with cerebral AVF tend to present with neurologic problems related to intracranial venous hypertension or intracranial hemorrhage. Cardiac and pulmonary complications following acute neurologic injury such as subarachnoid hemorrhage are common in adults, but are rarely reported in children. However, complications have been reported in cases of enterovirus 71 rhombencephalitis in infants and children and can cause high morbidity and mortality. Here, we report a 14-year-old boy who presented with cardiac failure associated with pulmonary edema following cerebral hemorrhagic stroke due to AVF. After aggressive investigation and management, we intervened before significant hypoxia and hypotension developed, potentially reducing the risk of long-term adverse neurologic consequences in this patient.     

Lung resections in children for congenital and acquired lesions

We reviewed a single-center experience of pediatric lung resections for various congenital and acquired benign lung conditions. Thirty-five children underwent lung resections between 1998 and 2006, their age ranging from 8 days to 12 years (mean 3 years), with a male:female ratio of 4:1. Twelve patients were neonates. Antenatal diagnosis was available in only one patient. The presenting symptoms were respiratory distress and respiratory tract infections. Imaging with chest X-ray with/without a CT scan picked up the lesion in all cases. Preoperative ventilation was required for five patients. One patient had pneumothorax at presentation; however, ten patients had inadvertent intercostal tube insertion before surgical referral. The surgical procedures performed included lobectomy (28), segmentectomy (3), and pneumonectomy in 4 cases. Twenty-one patients underwent emergency surgery. Six patients required postoperative ventilation. The histopathological diagnosis was congenital lobar emphysema (CLE) (9), congenital cystic adenomatoid malformation (CCAM) (9), bronchiectasis (9), sequestration (3), atelectasis (1), lung abscess (1), unilobar tuberculosis (1), hydatid cyst (1), and foreign body with collapse (1). There was considerable discrepancy between the preoperative diagnosis based on imaging and the postoperative histopathological diagnosis. Postoperative complications included atelectasis (2), pneumothorax (2) and fluid collection (4 cases). Three patients died, one from compromised cardiac function, one from overwhelming sepsis and one from respiratory failure due to severe bilateral CCAM; the rest of the patients made a satisfactory recovery. At short-term follow-up all patients were doing well. Pulmonary resections are necessary for various congenital and acquired lung lesions in children and can be done safely in a pediatric hospital setup. Proper preoperative diagnosis can avoid inadvertent intercostal tube insertion in patients with congenital cystic lung lesions. The histopathological diagnosis often differs from the radiological diagnosis. Emergency lobectomies for acute respiratory distress, even in neonates, result in a satisfactory outcome.     

Dysrhythmia as a cause of syncope in children without neurological or cardiac morphological abnormalities

BACKGROUND: Arrhythmias are among the malignant causes of syncope. This study has been undertaken to determine the relative incidence and significance of dysrhythmia in the pathogenesis of syncope among patients referred to a pediatric cardiology unit. METHODS: Between March 1997 and March 1999, 105 consecutive patients (59 female, 46 male) aged 11.5 +/- 3.6 years without neurologic or cardiac morphologic causes were evaluated for at least one episode of syncope. A pediatric cardiologist and a pediatric neurologist evaluated all the patients. Routine chest X-ray, 12-lead electrocardiogram (ECG), electroencephalography (EEG), 24-h Holter monitoring and echocardiography were carried out. When deemed necessary, further tests were undertaken for the cases of syncope which were unexplained by routine tests. RESULT: The cause of syncope was identified as vasovagal in 25.7% (n = 27) and related to dysrhythmia in 30.5% (n = 32). The cause was migraine-associated syncope in two children, psychogenic syncope in three children and orthostatic hypotension in one patient. The cause was unknown in 36.2% (n = 38). CONCLUSION: We conclude that dysrhythmia is a significant and frequent cause in children referred to pediatric cardiology units. The combination of ECG, Holter monitoring, electrophysiologic study, transtelephonic ECG and head-up tilt test can identify the underlying cause of syncope in as many as 58% of these patients that present with syncope.     

The challenges of prompt identification and resuscitation in children with acute fulminant myocarditis: case series and review of the literature

Aim: To describe the clinical presentation, triage, resuscitation and outcome of acute fulminant myocarditis in children presenting to district hospitals and referred for cardiac intensive care. Methods: Case series describing five patients (from 2 weeks to 12 years old) with a diagnosis of acute fulminant myocarditis, presented to outlying hospitals between December 2006 and December 2007 and retrieved to a cardiac intensive care unit. Results: All children were admitted with non‐specific symptoms such as vomiting, cough and poor feeding to their local hospital, where various provisional diagnoses such as viral gastroenteritis, bronchitis or renal failure were considered. Acute physiological deterioration usually prompted the referral for intensive care. Two children died at the referring hospital during stabilisation by the retrieval team. Three children survived transport to intensive care and to hospital discharge; two received mechanical support and one underwent urgent orthotopic heart transplantation. Enterovirus and parvovirus were identified as causative agents in two patients. In one case, macrophage activation syndrome was diagnosed although no clear viral trigger was identified. Median length of hospitalisation among survivors was 33 days, and mechanical cardiac support was required for a median of 12 days. Conclusions: The diagnosis and initial management of acute fulminant myocarditis is extremely challenging. Prognosis for patients admitted to a cardiac centre for early mechanical support can be very favourable, while a delay in considering the diagnosis may result in poor outcome. The diagnosis of myocarditis should be considered in any previously well child presenting with a viral prodrome and non‐specific organ dysfunction associated with dysrhythmias, shock or acute heart failure, even in the absence of cardiomegaly.     

Analysis of status epilepticus related presumed encephalitis in children.

OBJECTIVE: Encephalitis is an acute infection of brain parenchyma characterized clinically by fever, headache, and an altered level of consciousness. There may also be focal or multifocal neurologic deficits, and focal or generalized seizure activity. Here we report an analysis of status epilepticus (SE) related presumed encephalitis in a series of children. METHODS: We retrospectively reviewed cases of SE related presumed encephalitis treated in the pediatric intensive care unit, between February 2002 and June 2006. Factors evaluated included age, sex, clinical symptoms, seizure type, presence of SE or refractory status epilepticus (RSE), initial electroencephalogram (EEG) finding, neuroimaging study, cerebrospinal fluid (CSF) and outcome. RESULTS: There were 46 patients (19 girls and 27 boys), aged 8 months to 16 years. Twenty (43.4%) of 46 children developed RSE. The major clinical symptoms included fever (100%), upper respiratory symptoms (56.5%) and altered level of consciousness (45.6%). The initial seizure type was categorized as focal (23.9%), generalized (34.8%), primary focal and secondary generalized (41.3%). Initial EEG revealed a focal (30.8%), or multifocal (19.2%) epileptiform discharge in the SE group and a focal (5%), or multifocal (70%) or generalized (25%) epileptiform discharge in the RSE group. The time of follow-up for this study was 6 months to 51 months. In the SE group, 4 died, 16 developed epilepsy and/or neurologic deficits, and 6 returned to baseline. In the RSE group, 6 died, 13 developed epilepsy and/or neurologic deficits, and none returned to baseline. All survivors were discharged on antiepileptic medications. CONCLUSIONS: Our data indicated that children of SE related presumed encephalitis had a high mortality and morbidity. Outcome was related to multifocal or generalized abnormalities of the initial EEG and presence of RSE.     

Pediatric appendicitis in "real-time": the value of sonography in diagnosis and treatment.

OBJECTIVES: To determine the accuracy of sonography in the diagnosis of clinically equivocal appendicitis, and to identify the factors leading to an inaccurate ultrasound diagnosis. The impact of sonographic findings on clinical management and outcome of children with appendicitis is examined. METHODS: We performed a retrospective review of 317 children who attended the emergency department (ED) of a children's hospital for acute abdominal pain for which acute appendicitis was the main differential diagnosis. They had ultrasound because the diagnosis was uncertain clinically. RESULTS: The positive predictive value of ultrasound for appendicitis was 0.92, and the negative predictive value was 0.88. The sensitivity and specificity could not be determined because there were 43 patients with equivocal ultrasound results. The pitfalls hindering the accuracy of ultrasound diagnosis included a high incidence of perforated appendicitis at presentation, the retrocecal appendix, and other technical factors such as abdominal guarding, excessive bowel gas, obesity, inadequate bladder filling, and the uncooperative patient. When ultrasound findings were combined with clinical judgment in clinical management, there were only five cases of non-therapeutic laparotomy and eight cases of delayed surgery due to missed diagnosis in our cohort. CONCLUSIONS: Ultrasound is a useful for the evaluation of acute abdominal pain in children. However, in the setting of a pediatric hospital ED, the accuracy of ultrasound and its ability to improve early hospital triage may be reduced. Repeated clinical review is still essential and in selected cases, appendiceal CT scan may be required to guide therapeutic decision making.     

Post-infectious acute cerebellar ataxia in children

Acute cerebellar ataxia is a relatively common neurologic disorder among children. Our aim was to characterize the clinical picture, etiology, and prognosis of acute cerebellar ataxia. The medical records of all children with a diagnosis of acute cerebellar ataxia hospitalized in our center and Hasharon Medical Center from 1990 to 2001 were reviewed. The diagnosis of acute cerebellar ataxia was based on the following criteria: acute onset of ataxia with or without nystagmus; absence of known genetic predisposing factors, such as familial degenerative disorders; and absence of drug intoxication, bacterial meningitis, and metabolic disorders. Thirty-nine children were identified; 54% were male; mean age at presentation was 4.8 +/- 3.8 years. All patients were observed for at least 1 year. A prodromal febrile illness was noted in 74.4%: varicella, 31%; mumps, 20%; nonspecific viral infection, 15.4%; mycoplasma, 5%; Epstein Barr virus, 3%. Latency from the prodromal illness to the onset of ataxia was 8.8 +/- 7.4 days. The most common associated neurologic findings were nystagmus and dysmetria. Full gait recovery took less than 2 weeks on average, and the longest duration of neurologic signs was 24 days (mumps-related). Acute cerebellar ataxia in childhood is a self-limited disease. The recovery was faster than that reported in previous publications and was complete in all children without any neurologic sequelae. Imaging studies are needed only in atypical presentation or if there is no spontaneous improvement after 1 to 2 weeks.     

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.