Classic and "Dissecting" Gonadoblastoma in a Phenotypic Girl With a 46, XX Peripheral Karyotype and No Evidence of a Disorder of Sex Development.

Herein, we report a case of a 9-yr-old girl who had a 46, XX peripheral karyotype and apparent developmentally normal ovaries. She presented with abdominal pain and a right adnexal mass. No clinical or pathologic evidence of gonadal dysgenesis or undifferentiated gonadal tissue was detected. She underwent right salpingo-oophorectomy with rupture of the tumor at the time of operation due to recent adnexal torsion. The original pathologic diagnosis was gonadoblastoma and mixed germ cell tumor. Most significantly in our study, we identified a rare and novel pathway for the development of malignant mixed germ cell tumor from gonadoblastoma in the absence of identifiable dysgerminoma. The histologically identifiable steps of progression in our case were as follows: (1) residual islands of classic gonadoblastoma, (2) overgrowth by "dissecting" gonadoblastoma composed of transformed germ cells with clear cytoplasm and sex cord elements surrounded by a basement membrane, (3) stromal infiltration by dedifferentiated germ cells with loss of basement membrane, (4) formation of malignant mixed germ cell tumor. The dedifferentiated areas were composed of anaplastic germ cells with amphophilic cytoplasm that gradually replaced the sex cord elements by clonal expansion. Both the original transformed and the anaplastic germ cell components strongly expressed OCT4. We believe that the mixed germ cell tumor arose from the dedifferentiated germ cell component through neoplastic progression. This premise suggests that the germ cell component of "dissecting" gonadoblastoma rarely undergoes anaplastic change in the absence of transition to germinoma and can be the direct precursor of mixed germ cell tumor.

Functional Improvement and Expectations Are Diminished in Total Knee Arthroplasty Patients Revised for Flexion Instability Compared to Aseptic Loosening and Infection.

BACKGROUND: Instability has emerged as the most common noninfectious cause necessitating early revision after total knee arthroplasty (TKA). Although studies have documented improvement in outcomes with revision for flexion instability, it remains unknown how the outcomes compare to patients revised for other failure etiologies. The study purpose was to compare outcomes after revision TKA based on failure etiology. METHODS: A retrospective review of our prospectively collected revision TKA database was performed on patients who underwent revision TKA from October 1, 2010 to November 19, 2014. Demographic data; minimum 1-year Knee Society Scores; and University of California, Los Angeles activity level scores were obtained. RESULTS: One hundred seventy-seven consecutive revision TKAs were evaluated. After exclusion of revisions with confounding variables and diagnosis groups with small numbers, 92 patients with a revision diagnosis of flexion instability, infection, or loosening/osteolysis were compared. There were no group differences in Knee Society objective (P ≥ .460) and satisfaction (P ≥ .112) scores or UCLA activity level scores at final follow-up (P ≥ .118). Preoperative Knee Society function scores were significantly higher in patients with flexion instability (P = .019), but the amount of improvement in function relative to baseline was equivalent in the 3 groups (P = .170). Patients revised for flexion instability were significantly more likely than patients in the other 2 groups to report that their expectations were not met (P = .028). CONCLUSION: Patients and surgeons can expect that revision for isolated flexion instability may only obtain modest improvement compared with other diagnoses, potentially in part due to a higher preoperative functional level in patients with instability.

Can Intraoperative Sensors Determine the "Target" Ligament Balance? Early Outcomes in Total Knee Arthroplasty.

BACKGROUND: The optimal "target" ligament balance for each patient undergoing total knee arthroplasty (TKA) remains unknown. The study purpose was to determine if patient outcomes are affected by intraoperative ligament balance measured with force-sensing implant trials and if an optimal "target" balance exists. METHODS: A multicenter, retrospective study reviewed consecutive TKAs performed by 3 surgeons. TKA's were performed with standard surgical techniques and ligament releases. After final implants were made, sensor-embedded smart tibial trials were inserted, and compartment forces recorded throughout the range of motion. Clinical outcome measures were obtained preoperatively and at 4 months. Statistical analysis correlated ligament balance with clinical outcomes. RESULTS: One hundred eighty-nine consecutive TKAs were analyzed. Patients were grouped by average medial and lateral compartment force differences. Twenty-nine TKAs (15%) were balanced within 15 lbs and 53 (28%) were "balanced" greater than 75 lbs. Greater improvement in University of California Los Angeles activity level was associated with a mediolateral force difference <60 lbs. (P = .006). Knee Society objective, function, and satisfaction scores, and self-reported health state were unrelated to mediolateral balance in the knee. CONCLUSION: Intraoperative force-sensing has potential in providing real-time objective data to optimize TKA outcomes. These data support some early outcomes may improve by balancing TKAs within 60 lbs difference. Close follow-up is warranted to determine if gait pattern adaptations affect longer term outcomes with greater or less ligament "imbalance."

Exercise as an Aging Mimetic: A New Perspective on the Mechanisms Behind Exercise as Preventive Medicine Against Age-Related Chronic Disease.

Age-related chronic diseases are among the most common causes of mortality and account for a majority of global disease burden. Preventative lifestyle behaviors, such as regular exercise, play a critical role in attenuating chronic disease burden. However, the exact mechanism behind exercise as a form of preventative medicine remains poorly defined. Interestingly, many of the physiological responses to exercise are comparable to aging. This paper explores an overarching hypothesis that exercise protects against aging/age-related chronic disease because the physiological stress of exercise mimics aging. Acute exercise transiently disrupts cardiovascular, musculoskeletal, and brain function and triggers a substantial inflammatory response in a manner that mimics aging/age-related chronic disease. Data indicate that select acute exercise responses may be similar in magnitude to changes seen with +10-50 years of aging. The initial insult of the age-mimicking effects of exercise induces beneficial adaptations that serve to attenuate disruption to successive "aging" stimuli (i.e., exercise). Ultimately, these exercise-induced adaptations reduce the subsequent physiological stress incurred from aging and protect against age-related chronic disease. To further examine this hypothesis, future work should more intricately describe the physiological signature of different types/intensities of acute exercise in order to better predict the subsequent adaptation and chronic disease prevention with exercise training in healthy and at-risk populations.

Diffuse lung disease in young children: application of a novel classification scheme.

RATIONALE: Considerable confusion exists regarding nomenclature, classification, and management of pediatric diffuse lung diseases due to the relative rarity and differences in the spectrum of disease between adults and young children. OBJECTIVES: A multidisciplinary working group was formed to: (1) apply consensus terminology and diagnostic criteria for disorders presenting with diffuse lung disease in infancy; and (2) describe the distribution of disease entities, clinical features, and outcome in young children who currently undergo lung biopsy in North America. METHODS: Eleven centers provided pathologic material, clinical data, and imaging from all children less than 2 years of age who underwent lung biopsy for diffuse lung disease from 1999 to 2004. MEASUREMENTS AND MAIN RESULTS: Multidisciplinary review categorized 88% of 187 cases. Disorders more prevalent in infancy, including primary developmental and lung growth abnormalities, neuroendocrine cell hyperplasia of infancy, and surfactant-dysfunction disorders, constituted the majority of cases (60%). Lung growth disorders were often unsuspected clinically and under-recognized histologically. Cases with known surfactant mutations had characteristic pathologic features. Age at biopsy and clinical presentation varied among categories. Pulmonary hypertension, presence of a primary developmental abnormality, or ABCA3 mutation was associated with high mortality, while no deaths occurred in cases of pulmonary interstitial glycogenosis, or neuroendocrine cell hyperplasia of infancy. CONCLUSIONS: This retrospective cohort study identifies a diverse spectrum of lung disorders, largely unique to young children. Application of a classification scheme grouped clinically distinct patients with variable age of biopsy and mortality. Standardized terminology and classification will enhance accurate description and diagnosis of these disorders.

At what age is a suction rectal biopsy less likely to provide adequate tissue for identification of ganglion cells?

OBJECTIVE: The objective of this study was to determine at what age suction rectal biopsy is less likely to provide adequate tissue to detect submucosal ganglion cells in a child being evaluated for Hirschsprung disease. PATIENTS AND METHODS: Children > or =1 year of age undergoing a rectal biopsy at a single children's hospital had 1 biopsy each obtained simultaneously with a suction biopsy device and a grasp biopsy forceps. The biopsies were examined by 2 pathologists for adequacy of the submucosa (none, scant, adequate, or ample) and the presence of ganglion cells. The 2 specimens were compared with each other. RESULTS: One hundred fifty-two children 1 to 17 years of age were included. Fifty-three were female. Subjects were grouped into 4 age categories: 1 to 3 years (group A), 4 to 6 years (group B), 7 to 9 years (group C), and > or =10 years (group D). Similar numbers of patients were recruited for each group. Ganglion cells were identified in 73% and 90% by the suction and grasp devices, respectively, in group A. In groups B through D, ganglion cells were identified in 50% to 53% vs 92% to 97% of the suction and grasp biopsies, respectively (P < 0.001). Submucosa was present in 88% (suction) vs 98% (grasp) in group A, 70% vs 95% in group B, 69% vs 94% in group C, and 45% vs 92% in group D. CONCLUSION: The suction rectal biopsy is less likely to provide adequate submucosa for identification of ganglion cells after 3 years of age.

Enchondroma Protuberans of the Ulna in a Pediatric Patient: A Case Report.

CASE: We report the case of a 3-year-old boy who presented with a distal ulnar fracture through a mixed sclerotic and lytic expansile lesion. The underlying lesion, an enchondroma protuberans, can mimic either benign or malignant bone tumors. It was successfully treated with casting and intralesional treatment. CONCLUSION: Enchondroma protuberans is a rare entity that mimics enchondroma, osteochondroma, periosteal chondroma, or chondrosarcoma. Diagnosis is typically made through both radiographic and histologic means. In this case, the pathologic fracture was successfully treated with casting followed by intralesional curettage and bone-grafting. There was no evidence of recurrence at 18 months.

Treatment of children and adolescents with stage II testicular and stages I and II ovarian malignant germ cell tumors: A Pediatric Intergroup Study--Pediatric Oncology Group 9048 and Children's Cancer Group 8891.

PURPOSE: To determine whether children with localized gonadal malignant germ cell tumors (MGCT) stage II testicular and stages I and II ovarian treated with four cycles of standard-dose cisplatin combined with etoposide and low-dose bleomycin (PEB) have an event-free survival (EFS) of at least 85% without significant toxicity. PATIENTS AND METHODS: Between May 1990 and July 1995, eligible pediatric patients with stage II or recurrent from stage I (as a stage II) testicular MGCT and stages I and II ovarian MGCT were enrolled onto this Pediatric Oncology Group and Children's Cancer Group study. PEB chemotherapy consisted of bleomycin 15 U/m2 on day 1, cisplatin 20 mg/m2/d on days 1 to 5, and etoposide 100 mg/m2/d on days 1 to 5. Patients received four cycles of therapy at 21-day intervals. RESULTS: Seventy-four patients with a median age of 10.5 years (range, 8.7 months to 16.7 years) were enrolled. Primary sites included: stage II testicular (n = 17), stage I ovarian (n = 41), and stage II ovarian MGCT (n = 16). Treatment with standard PEB resulted in 6-year EFS of 95% and overall survival (OS) of 95.7%. EFS and OS by primary site were as follows: stage II testicular, 100% and 100%; stage I ovarian, 95.1% and 95.1%; and stage II ovarian, 87.5% and 93.8%, respectively. Two patients died from recurrent disease, and one patient died of secondary acute myelocytic leukemia. Infrequent grade 3 to 4 hematologic toxicity was reported. No grade 3 to 4 renal, pulmonary, or ototoxicity was observed. CONCLUSION: Combination chemotherapy with PEB results in excellent EFS and OS with minimal toxicity in children and adolescents with localized gonadal MGCT.

Surveillance after initial surgery for Stage I pediatric and adolescent boys with malignant testicular germ cell tumors: Report from the Children's Oncology Group.

PURPOSE: The purpose of this study was to determine prognostic factors correlating with outcome in boys with Stage I malignant testicular germ cell tumors (MTGCT) initially managed with surveillance after surgical resection. METHODS: Between November 2003 and July 2011, 80 boys 0-15 years with Stage I MTGCT were enrolled in Children's Oncology Group Study AGCT0132. Those with residual or recurrent disease were treated with chemotherapy. RESULTS: Characteristics include: age (65, 0-5 years and 15, 11+years), pure YST (93.9%, 0-5 years and 0%, 11+years); and lymphovascular invasion (LVI) (50.6% present vs. 49.4% absent). At median follow-up of 4.94 years, 19 had persistent or recurrent disease, all detected by elevated AFP at a median of 87 days after study enrollment. The outcome from enrollment was 4-year EFS 74% (95% CI: 63%-83%) and 4-year OS 100%. 4-year EFS was improved with younger age (<11 years, 80% vs. 11+years, 48%, p<0.01); pure YST vs. mixed histology (81% vs. 45%, p<0.01), and lack of LVI (84% vs. 62%, p=0.03). CONCLUSIONS: Boys with Stage I MTGCT have excellent overall survival when treated with surgery alone. Age greater than 10 years, mixed histology and presence of LVI are each associated with relapse and may allow identification of high risk boys at time of enrollment.

Effects of fire season on flowering of forbs and shrubs in longleaf pine forests.

Effects of variation in fire season on flowering of forbs and shrubs were studied experimentally in two longleaf pine forest habitats in northern Florida, USA. Large, replicated plots were burned at different times of the year, and flowering on each plot was measured over the twelve months following fire. While fire season had little effect on the number of species flowering during the year following fire, fires during the growing season decreased average flowering duration per species and increased synchronization of peak flowering times within species relative to fires between growing seasons. Fires during the growing season also increased the dominance of fall flowering forbs and delayed peak fall flowering. Differences in flowering resulting from variation in fire season were related to seasonal changes in the morphology of clonal forbs, especially fall-flowering composites. Community level differences in flowering phenologies indicated that timing of fire relative to environmental cues that induced flowering was important in determining flowering synchrony among species within the ground cover of longleaf pine forests. Differences in fire season produced qualitatively similar effects on flowering phenologies in both habitats, indicating plant responses to variation in the timing of fires were not habitat specific.

Premature thelarche and granulosa cell tumors: a search for FSH receptor and G5alpha activating mutations.

Activating mutations of the Gsalpha gene are responsible for McCune-Albright syndrome and have also been identified in sporadic tumors of the pituitary and thyroid. When associated with malignancy, activating Gsalpha mutations are known as gsp-oncogenes. We hypothesized that similar activating mutations might also account for some cases of premature thelarche and/ or granulosa cell tumors. Polymerase chain reaction and DNA sequencing was used to screen for activating mutations of Gsalpha genes in children with premature thelarche and in pathologic specimens from juvenile and adult granulosa cell tumors. Because these disorders involve over-activity of the FSH-signaling pathway, we also screened for activating mutations of the FSH receptor. No mutations were detected in either the Gsalpha or the FSHR fragment studied. Previously reported polymorphisms (Ser680Asn and Ala307Thr) of the FSHR were detected in 25/27 tumor samples and 9/9 premature thelarche samples. We conclude that activating mutations in previously identified mutation 'hot-spots' in the Gsalpha and FSH receptor genes are probably not a major cause of premature thelarche or granulosa cell tumors. In contrast, polymorphisms of the FSH receptor are common.

Targeting receptor-activator of nuclear kappaB ligand in aneurysmal bone cysts: verification of target and therapeutic response.

Aneurysmal bone cyst (ABC) is a benign tumor of bone presenting as a cystic, expansile lesion in both the axial and appendicular skeleton. Axial lesions demand special consideration, because treatment-related morbidity can be devastating. In similar lesions, such as giant cell tumor of bone (GCTB), the receptor-activator of nuclear kappaB ligand (RANKL)-receptor-activator of nuclear kappaB (RANK) signaling axis is essential to tumor progression. Although ABC and GCTB are distinct entities, they both contain abundant multinucleated giant cells and are osteolytic characteristically. We hypothesize that ABCs express both RANKL and RANK similarly in a cell-type specific manner, and that targeted RANKL therapy will mitigate ABC tumor progression. Cellular expression of RANKL and RANK was determined in freshly harvested ABC samples using laser confocal microscopy. A consistent cell-type-specific pattern was observed: fibroblastlike stromal cells expressed RANKL strongly whereas monocyte/macrophage precursor and multinucleated giant cells expressed RANK. Relative RANKL expression was determined by quantitative real-time polymerase chain reaction in ABC and GCTB tissue samples; no difference in relative expression was observed (P > 0.05). In addition, we review the case of a 5-year-old boy with a large, aggressive sacral ABC. After 3 months of targeted RANKL inhibition with denosumab, magnetic resonance imaging demonstrated tumor shrinkage, bone reconstitution, and healing of a pathologic fracture. Ambulation, and bowel and bladder function were restored at 6 months. Denosumab treatment was well tolerated. Post hoc analysis demonstrated strong RANKL expression in the pretreatment tumor sample. These findings demonstrate that RANKL-RANK signal activation is essential to ABC tumor progression. RANKL-targeted therapy may be an effective alternative to surgery in select ABC presentations.

The whole truth: comparative analysis of diaphragmatic hernia repair using 4-ply vs 8-ply small intestinal submucosa in a growing animal model.

BACKGROUND: Diaphragmatic reconstruction remains a challenging problem. There is limited information concerning the use of small intestinal submucosa (SIS) in congenital diaphragmatic hernia repair. A canine model was used to evaluate the use of a SIS patch in diaphragmatic reconstruction. METHODS: Eleven beagle puppies (1.6-4.2 kg, 8 weeks old) underwent left subcostal laparotomy, central left hemidiaphragm excision (2 x 7 cm, 50% loss), and reconstruction with a 4-ply group I (n = 5) or 8-ply group II (n = 6) SIS patch. Chest radiographs were taken at time of operation and 3 and 6 months postoperatively. Animals were killed at 6 months. Adhesion formation (both pleural and abdominal), gross visual evaluation of the patch, and histology were compared. RESULTS: In group I (4-ply), 1 animal died at 3 months from patch deterioration accompanied by stomach herniation that resulted in respiratory failure. In the 4 remaining animals, chest radiographs showed no evidence of herniation or eventration. On physical examination, there was no evidence of chest wall deformity. During gross surgical examination, the 4-ply patches showed thinning, multiple defects, and liver herniation in 3 animals. In 1 pup, the patch was thickened, intact, well incorporated at the repair site, and adherent to the liver and spleen. In group II (8-ply), 1 animal died of cardiopulmonary failure in the early postoperative period. In the other 5 animals, chest radiographs showed evidence of eventration in 1. On gross examination the patch adhered to the liver in all 5 surviving animals. In 4, the patches were thickened, viable, but had some shrinkage. One patch pulled away from the native diaphragm laterally; however, no visceral herniation was present. In the 1 animal with eventration, there was no evidence of a patch. Adhesion scores (AS) were graded and determined by the sum of extent (0-4), type (0-4), and tenacity (0-3). Average abdominal AS in group I was 5.6 +/- 0.8 vs 10.2 +/- 0.2 (P = .079) for group II. Average lung AS was 0.6 +/- 0.6 in group I vs 3.8 +/- 1.1 (P = .0476) for group II. Histological examination showed group II patches had greater collagen deposition with central calcification and mild inflammation within the residual graft, whereas group I patches were much thinner and were composed of granulation tissue without evidence of residual graft. CONCLUSIONS: These data indicate that 8-ply SIS repair of diaphragmatic defects was superior (80%; 4/5 to 4-ply, 20%; 1/5, success). Organ adherence appears to be necessary for neovascularization of the SIS composite. Eight-ply grafts appear to be more durable and persist for a longer period, which may improve neovascularization. Long-term follow-up to evaluate remodeling characteristics of the patch material is required.

Germline p53 mutation presenting as synchronous tumors.

Li-Fraumeni syndrome and the LF-like syndrome, rare heritable conditions that predispose to the development of malignancy, are associated with germline mutations of the tumor suppressor gene p53. The authors describe a 14-month-old boy who presented with synchronous rhabdomyosarcoma and adrenal cortical carcinoma and a novel mutation of the p53 gene. Analysis of exons 2 through 11 of the p53 gene using the polymerase chain reaction and DNA sequencing revealed a mutation of codon 273. Although codon 273 is a known hotspot region for p53 mutation, the patient's mutation, R273H, has not been associated with development of adrenal cortical carcinoma.

Amifostine does not protect against the ototoxicity of high-dose cisplatin combined with etoposide and bleomycin in pediatric germ-cell tumors: a Children's Oncology Group study.

BACKGROUND: High-dose cisplatin combined with etoposide and bleomycin (HDPEB) improves event-free survival (EFS) in advanced pediatric germ-cell tumors (PGCT), but has significant ototoxicity. Amifostine appears to protect against toxicity. The authors combined amifostine with HDPEB and evaluated the efficacy and toxicity, specifically whether ototoxicity decreased. METHODS: Eligibility criteria included age < 15 years and unresectable Stage III/IV extracranial, extragonadal PGCT. Patients received bleomycin 15 IU/m(2) on Day 1, then etoposide 100 mg/m(2) per day, amifostine 825 mg/m(2) per day, and cisplatin 40 mg/m(2)per day on Days 1-5, intravenously. The cycles were repeated every 3-4 weeks with imaging evaluation after 4 cycles. Patients with residual radiographic abnormalities underwent resection. Patients with residual tumor received two additional HDPEB cycles. Hearing evaluations were required at diagnosis and after two and four cycles. Audiologic results were reviewed and compared with historical controls treated with HDPEB. RESULTS: Twenty-five eligible patients were enrolled between April 2000 and April 2002. Their median age was 1.6 years (range, 0.64-13.9 years), 17 patients were female, 11 had metastases, and 24 had a yolk sac carcinoma component histologically. Primary sites included sacrococcygeal area/pelvis (n = 15), vagina (n = 5), and other (n = 5). Two-year EFS and overall survival were 83.5% +/- 12.8% and 85.6% +/- 12.3%, respectively. Eight patients were removed from the study (four had progressive disease/disease recurrence and four had ototoxicity). Grade 3/4 toxicities included neutropenia (n = 20), thrombocytopenia (n = 14), electrolyte imbalances (n = 14), and gastrointestinal toxicity (n = 12). Twenty-four of 25 patients received hearing evaluations, and 75% had significant hearing loss. CONCLUSIONS: Amifostine did not protect against HDPEB-associated ototoxicity.

Esthesioneuroblastoma in children.

Esthesioneuroblastoma (olfactory neuroblastoma) is a rare tumor of the olfactory epithelium. Approximately 1,000 cases have been described in the literature since the original description in 1924. It occurs in older individuals and is rare in children. The authors describe the clinicopathologic presentation in a series of five children treated with neoadjuvant/adjuvant chemotherapy and review the English literature for previously described patients younger than 18 years to assess clinical presentation, mode of treatment, and outcome in this age group.

Complications of small intestinal submucosa for corporal body grafting for proximal hypospadias.

PURPOSE: Small intestinal submucosa (SIS) has been described for corporal body grafting in cases of severe penile curvature. We reviewed our experience with a 2-stage repair using corporal body grafting with SIS for proximal hypospadias and severe chordee. MATERIALS AND METHODS: A retrospective chart review was performed on 12 patients with penoscrotal hypospadias and severe chordee. Corporal grafting was performed with a 4-layer SIS graft (STRATASIS, Cook Biotech, Spencer, Indiana). Patients were evaluated postoperatively with clinic visits after both stages of repair to assess results. RESULTS: Corporal body grafting with SIS was performed in 12 patients between June and December 2001. Average patient age at the time of stage 1 repair was 9 months. Of the 12 patients 8 (66%) had no complications and 10 (83%) have a straight phallus following stage 2 repair. Complications occurred in 4 patients (33%), 2 of which were minor and did not require surgical correction. There were 2 major complications related to the SIS graft, 1 of which required excision of the graft and replacement with a tunica albuginea flap, and the other required 3 dorsal plications to correct residual chordee. CONCLUSIONS: Our experience using the 4-layer SIS resulted in 2 major complications requiring surgical correction during stage 2 repair. This rate exceeds the complications reported with either dermal or tunica vaginalis grafts. Currently we have stopped using SIS for corporal grafting.

White specks in the esophageal mucosa: An endoscopic manifestation of non-reflux eosinophilic esophagitis in children.

BACKGROUND: White specks in the esophageal mucosa have been observed in children with eosinophilic esophagitis. The aim of this study was to determine the relationship between white specks in the esophageal mucosa and allergic (non-reflux) eosinophilic esophagitis. METHODS: Endoscopic data, pH probe results, and histopathology reports for children with esophageal endoscopic abnormalities seen during a 17-month period were reviewed. Eosinophilic esophagitis was grouped according to the number of eosinophils per high power field (non-allergic, <15 eosinophils/high power field; allergic, > or =15 eosinophils/high power field). RESULTS: Of 1041 endoscopies performed during the study period, 153 revealed evidence of eosinophilic esophagitis. Of these 153, 61 had fewer than 15 eosinophils/high power field and 92 had 15 or more eosinophils/high power field. At 31 of the 153 procedures, white specks were noted in the esophageal mucosa. The sensitivity of white specks in the esophageal mucosa for allergic eosinophilic esophagitis was only 30%, but the specificity was 95%. pH probe testing was performed in 21 patients with white specks and was normal in all. CONCLUSIONS: This report describes a new endoscopic finding associated with allergic eosinophilic esophagitis in children. Eosinophilic esophagitis tends to be severe when white specks are present (> or =15 eosinophils/high power field) and is not associated with pathologic gastroesophageal reflux, as demonstrated by pH probe testing.