Necrotizing enterocolitis is associated with ureaplasma colonization in preterm infants.

The study objective was to determine whether Ureaplasma respiratory tract colonization of preterm infants <33 wk gestation is associated with an increased risk for necrotizing enterocolitis (NEC). One or more tracheal or nasopharyngeal aspirates for Ureaplasma culture and PCR were obtained during the first week of life from 368 infants <33 wk gestation enrolled from 1999 to 2003 or from 2007 to 2009. NEC Bell stage ≥ 2 was confirmed by radiological criteria, and pathology, if available. Cord serum samples were analyzed for IL-6 and IL-1ß concentrations, and placentas were reviewed for histological chorioamnionitis in the first cohort. NEC was confirmed in 29 of 368 (7.9%) of the combined cohorts. The incidence of NEC was 2.2-fold higher in Ureaplasma-positive (12.3%) than Ureaplasma-negative (5.5%) infants <33 wk (OR, 2.43; 95% CI, 1.13-5.2; p = 0.023) and 3.3-fold higher in Ureaplasma-positive (14.6%) than Ureaplasma-negative (4.4%) infants ≤ 28 wk (OR, 3.67; 95% CI, 1.36-9.93; p = 0.01). Age of onset, hematologic parameters at onset, and NEC severity were similar between Ureaplasma-positive and negative infants. Cord serum IL-6 and IL-1ß concentrations were significantly higher in Ureaplasma-positive than in Ureaplasma-negative NEC-affected infants. Ureaplasma may be a factor in NEC pathogenesis in preterm infants by contributing to intestinal mucosal injury and/or altering systemic or local immune responses.

NICU admission hypothermia, chorioamnionitis, and cytokines.

OBJECTIVE: To determine whether neonatal intensive care unit (NICU) admission hypothermia is associated with an intrauterine inflammatory response. METHODS: We analyzed a cohort of 309 very low birthweight infants to determine relationships between admission hypothermia, chorioamnionitis, and serum and cerebrospinal fluid (CSF) interleukin (IL)-1ß, IL-6, and tumor necrosis factor-α. RESULTS: Admission hypothermia <36°C occurred in 72% of patients <26 weeks and 44% of patients ≥26 weeks gestational age. NICU admission hypothermia was not associated with histologic chorioamnionitis or with elevated serum cytokine concentrations. CSF IL-6 concentrations ≥6.3 pg/mL were associated with admission hypothermia in infants <26 weeks' gestation. Clinical chorioamnionitis was associated with a lower risk of admission hypothermia, while cesarean section delivery was associated with increased risk. CONCLUSIONS: NICU admission hypothermia is common among preterm infants and is not associated with the fetal inflammatory response syndrome. Hypothermia is less common in the setting of clinical chorioamnionitis and more common in cesarean section deliveries, identifying two groups in whom extra attention to appropriate thermoregulation is warranted.

Ultrasound-guided fine needle aspiration cytology diagnosis of a foregut cyst in the thyroid: a case report.

BACKGROUND: Foregut cysts represent abnormal budding of the tracheobronchial anlage during embryogenesis. They occur most commonly in the mediastinum and pulmonary parenchyma and very seldom in the neck. CASE: A 42-year-old woman developed neck swelling and discomfort. An ultrasound scan showed a cystic nodule with a thick wall extending from the left lower pole of the thyroid gland. The material obtained from fine needle aspiration of the cyst demonstrated numerous detached ciliated tufts (DCTs) in a background of amorphous debris and degenerated cells. A diagnosis of ciliated cell remnants or DCTs consistent with a foregut cyst was made. CONCLUSION: Although this entity is rare, awareness of its occurrence in the thyroid gland may prevent unnecessary surgery.

Fatal subcutaneous panniculitis-like T-cell lymphoma gamma/delta subtype (cutaneous gamma/delta T-cell lymphoma): report of a case and review of the literature.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a distinct type of peripheral T-cell lymphoma, which has gained recognition over the past decade. The disease presents complex clinical, pathologic, and immunohistochemical features, which warrant awareness by dermatologists, dermatopathologists, hematopathologists, hematologists, oncologists, and internists. SPTCL was initially included as a provisional entity in the Revised European-American Lymphoma classification, followed by the European Organization for Research and Treatment of Cancer classification as a primary cutaneous lymphoma, and subsequently as a distinct entity by the World Health Organization classification. It is known that patients diagnosed with SPTCL usually respond poorly to therapy, and the tumor progresses aggressively. Data from recent studies in a series of cases of SPTCL by the European Organization for Research and Treatment of Cancer Cutaneous Lymphoma Group have further identified SPTCL as a heterogeneous disease entity, which comprises an alpha/beta subtype (SPTCL-AB) and a gamma/delta subtype (SPTCL-GD). The latter has recently been included in the entity of "cutaneous gamma/delta T-cell lymphoma" by the World Health Organization, Pathology and Genetics of Skin Tumours. The clinical, histologic, and immunophenotypic data, treatment, and prognosis, appear different in the 2 subtypes of SPTCL. We report a case of fatal SPTCL-GD (cutaneous gamma/delta T-cell lymphoma), with detailed clinicopathologic features, immunohistochemical studies, treatment, and clinical course. In view of its aggressive behavior, identification of this disease is critical for proper management and treatment.

Cytologic findings and differential diagnosis in hepatic Epithelioid hemangioendothelioma: a case report.

BACKGROUND: Epithelioid hemangioendothelioma is a rare tumor of vascular origin with nonspecific clinical and radiologic presentation. Cytologic findings described in the few case reports are variable. CASE: We describe a rare case of unifocal, hepatic hemangioendothelioma in a 47-year-old woman; a broad differential diagnosis of malignant neoplasm was considered during on-site evaluation of fine needle aspiration (FNA); diagnosis was made on subsequent core biopsy. To better describe the cytologic findings, FNA was performed on the resected tumor. The cytologic feature of this tumor, comparison with histol ogy findings and the differential diag nosis are discussed in detail. CONCLUSION: A correct diagnosis of hepatic epithelioid hemangioendothelioma can be rendered in cytology when an adequate specimen is obtained. The smears are characterized by a discohesive population of atypical cells in a clean background, fragments of metachromatic stroma, scattered benign hepatocytes and bile duct epithelium. The atypical cells are polygonal with abundant, dense cytoplasm and occasional tailing. On careful review, a few cells with a sharp, punched out intracytoplasmic lumen can be seen. The nuclei are round to reniform, with fine chromatin and occasionally prominent nucleoli. Sharply defined intranuclear cytoplasmic inclusions are seen in many cells, better appreciated in Papanicolaou-stained smears.

Testicular juvenile granulosa cell tumor in a newborn: case report and review of the literature.

Juvenile granulosa cell tumor of the testis of neonates and infants is an uncommon lesion frequently associated with abnormal sex chromosome and ambiguous genitalia. This report describes a juvenile granulosa cell tumor arising in the testis of a neonate. Chromosome analysis of the tumor showed a normal male karyotype 46 XY. Histopathology and immunohistochemical studies revealed the occurrence of 2 well-differentiated epithelial-like and smooth muscle-like components in the neoplasm. The morphologic clues leading to the correct diagnosis of juvenile granulosa cell tumor and the possible histogenesis are briefly discussed.

Extra-oral plasmablastic lymphoma: report of a case and review of literature.

Plasmablastic lymphoma (PBL) of the oral cavity is classified as one subtype of diffuse large B-cell lymphoma that is most commonly seen in patients with human immunodeficiency virus infection. We report a rare case of PBL in the anal canal of a 33-year-old man with human immunodeficiency virus infection. The lymphoma cells were positive for CD138 and weakly positive for CD79a. In addition, these cells were also positive for CD10. The neoplastic cells were positive for Epstein-Barr virus and negative for human herpes virus 8. Review of the English medical literature revealed many more cases of extra-oral PBL. We propose that the term plasmablastic lymphoma of the oral cavity in World Health Organization classification be revised to simply plasmablastic lymphoma, which would include both oral and extra-oral PBLs, and the term to define the primary site of the lymphoma (ie, oral cavity) be dropped from the terminology used in World Health Organization classification.

Alveolar rhabdomyosarcoma of the paranasal sinuses in a 57-year-old woman with 1:16 translocation.

Alveolar rhabdomyosarcoma is an uncommon tumor. It is a rare malignancy in adults and rarely occurs in paranasal sinuses in those aged older than 50 years. This report describes a locally invasive and destructive alveolar rhabdomyosarcoma arising in the ethmoid sinus of a 57-year-old woman. The small round blue cell tumors are positive for myogenin by immunohistochemistry and have a karyotype of 45, XX, -5, -13, der(16)t(1;1) (q21;q13) by cytogenetic analysis. Fluorescence in situ hybridization demonstrated a complex translocation with break apart of the FKHR region, which supports a diagnosis of alveolar rhabdomyosarcoma. This report characterizes this tumor through microscopic and cyto-genetic analysis and emphasizes the importance of considering rhabdomyosarcoma in the differential diagnosis of small round cell tumors of the head and neck region in the middle-aged adults.

Antenatal factors modulate hearing screen failure risk in preterm infants.

OBJECTIVE: The objective of this study was to characterise the effects of antenatal inflammatory factors and maternal therapies on neonatal hearing screen outcomes in very low birthweight infants. METHODS: We conducted a retrospective study of a cohort of infants <33 weeks' gestational age and <1501 g birth weight prospectively enrolled between 1999 and 2003 for whom placental pathology, cord blood interleukin (IL) 6, IL-1ß, tumour necrosis factor-α and neonatal hearing screen results were available. RESULTS: Of 289 infants with documented hearing screen results, 244 (84%) passed and 45 (16%) failed the hearing screen (unilateral, N=25 (56%); bilateral, N=20 (44%)). In the final logistic model, the fetal inflammatory response syndrome defined as the presence of fetal vasculitis and/or cord serum IL-6>18.2 pg/mL was the factor with greatest risk for hearing screen failure (OR 3.62, 95% CI 1.38 to 9.5). A patent ductus arteriosus treated with indomethacin significantly increased the risk (OR 3.3, 95% CI 1.3 to 8.26), while combined maternal steroid and magnesium sulfate exposure (0.37, 95% CI 0.11 to 0.81) reduced the risk for hearing screen failure. CONCLUSIONS: Intrauterine infection with a fetal inflammatory response is a risk factor for neonatal hearing loss while maternal therapies significantly reduced the risk of neonatal hearing loss in very low birthweight infants.

The severity of cholestatic injury is modulated by the genetic background.

Common bile duct ligation (CBDL) compromises the hepatic reticuloendothelial system by impairing the clearing of endotoxin and triggering an overwhelming inflammatory response. The response to endotoxin at the level of cytokine release and subsequent mortality depends on the genetic background in experimental mouse models. We hypothesized that the genetic make-up modulates the inflammatory responses after CBDL. The CBD was ligated in male A/J and B6 mice (8 weeks old). At 7 days post-CBDL, the presence of ascites was observed in 80% of B6 mice but in none of the A/J mice (P < 0.001). B6 mice showed higher mortality than A/J mice (P < 0.05). Both strains had marked cholestatic injury documented histologically. Liver chemistries were markedly elevated in both strains after injury. Plasma levels of the anti-inflammatory cytokine IL-10 were significantly higher in A/J than B6 mice at the 4- and 12-h time points (P < 0.05), whereas proinflammatory cytokine TNF-alpha levels were significantly higher in B6 than A/J mice at 2 h (P < 0.05). Both strains displayed activation of NF-kappaB after CBDL. In conclusion, the contrasting response observed after CBDL between A/J and B6 mice is largely attributable to genetic differences. Survival after CBDL was correlated with an increase in anti-inflammatory cytokines.

Choledochal cysts and multilocular cysts of the pancreas.

Choledochal cyst is a rare congenital anomaly of the biliary system that may be associated with other abnormalities of the hepatobiliary tract. We report a case of an 11-year-old boy in whom the preoperative evaluation revealed a choledochal cyst and intraoperative cholangiopancreatography showed a cystic mass in the pancreas. Examination of the choledocho-pancreatico-duodenectomy specimen showed a multilocular cyst in the pancreas in addition to a segmental dilation of the common bile duct. The findings in our case adds pancreatic cyst to the spectrum of abnormalities associated with choledochal cyst and may also support the theory that choledochal cyst is the predominant abnormality in a widespread spectrum of the pancreatobiliary duct dysplasia.

The value of autopsy in pediatric cardiology and cardiovascular surgery.

Autopsy has been considered extremely helpful in determining the accuracy of the premorbid diagnosis. With recent advances in diagnostic imaging, the value of the autopsy has been called into question. The aim of this study was to assess the value of the pediatric autopsy in the current era of advanced diagnostic technology and sophisticated surgical intervention for congenital heart disease (CHD) and also to determine the autopsy's potential impact on clinical management. We compared the data from the postmortem examination of 29 children with CHD with the data obtained before death and evaluated for the accuracy of the diagnosis of the cardiac anatomy as well as the cause of death. The diagnosis of the anatomy was confirmed in 13 cases (45%), additional cardiovascular abnormalities with potential clinical impact were found in 11 cases (38%), and additional abnormalities without clinical impact were found in 5 cases (17%). The cause of death was confirmed in 15 cases (52%), determined by autopsy in 12 cases (41%), and uncertain in 2 cases (7%). We conclude that the pediatric autopsy continues to provide clinically relevant information with a potential impact on patient management and to reveal unexpected causes of death in a significant proportion of patients with CHD.

Pseudallescheriasis as an aggressive opportunistic infection in a bone marrow transplant recipient.

Pseudallescheria boydii is a low-virulence fungus that is the main causative agent of posttraumatic mycetoma in a nonimmunocompromised host. Immunocompromised patients are at high risk for locally invasive or disseminated Pseudallescheria infection. However, aggressive opportunistic infections due to P boydii are reported infrequently because it morphologically resembles other fungi, especially Aspergillus species, on tissue histology; therefore, such infections are not identified and treated properly. We report a case of disseminated P boydii infection in a patient following bone marrow transplantation. The identity of the fungus was not recognized until microbiologic culture results became available. Our case illustrates the importance of recognizing this fungus as an opportunistic infection in immunocompromised patients, as well as the need for culture of biopsy material for proper identification so that appropriate therapy can be instituted.

Discrepancy in pathologic diagnosis of placental lesions.

CONTEXT: Placentas are routinely examined by surgical pathologists, but peer review of placental diagnosis is rarely performed. OBJECTIVE: To determine the frequency of discrepant placental diagnosis between general surgical pathologists and a pediatric pathologist. DESIGN: One hundred fourteen placentas from infants with intrauterine growth restriction (IUGR) and 170 placentas from infants appropriate for gestational age (AGA) were reviewed for 10 lesion types using standardized criteria. The review diagnosis was compared with original reports. RESULTS: The review identified 333 lesions, 168 in the IUGR group and 165 in AGA group. Discrepant diagnosis occurred in 137 lesions (41.1%). There was no significant difference in the frequency of discrepant diagnosis between the IUGR (44.7%) and AGA groups (37.6%) (P >.05). Most discrepancies (92.7%) were due to underdiagnosis (identified on review but not mentioned in original diagnosis), but a few (7.3%) were due to misdiagnosis (mentioned in original report but disagreed on review). The common underdiagnoses with their corresponding rates were as follows: hemorrhagic endovasculitis (84.6%), fetal thrombotic vasculopathy (75%), massive perivillous fibrin deposition (68.4%), maternal floor infarction (66.7%), retroplacental hemorrhage (60.6%), intervillous thrombus (57.1%), decidual angiopathy (33.3%), placental infarction (25.4%), acute chorioamnionitis (22.7%), and chronic villitis (21.7%). Misdiagnosis was found in 10 cases: 5 cases of infarction (review diagnosis was perivillous fibrin deposits in 4, intervillous thrombus in 1), 3 cases of acute chorioamnionitis, and 2 cases of decidual angiopathy. Among the 8 general surgical pathologists involved, the frequency of discrepant diagnosis ranged from 31.5% to 58.6% (P >.05). The intraobserver discrepancy rate for the reviewer was 4.8%, significantly lower than the discrepancy rate for the 8 general surgical pathologists. CONCLUSION: It is common for general surgical pathologists not to recognize placental lesions, which may have clinical significance. Awareness of this deficiency, standardization of diagnostic criteria, and increased knowledge in placental pathology may improve the quality of diagnosis in this area.

Mycobacterium avium complex-associated cholecystitis in an HIV-infected woman.

Mycobacterium avium complex (MAC) is commonly associated with fever, fatigue, nausea, diarrhea, and cytopenias related to invasion of the intestine and bone marrow. Infection and clinical disease has been reported in other organs as well. We report the first case of cholecystitis associated with MAC infection of the gallbladder.

A study of gata3 and phox2b expression in tumors of the autonomic nervous system.

Autonomic neurons and chromaffin cells, which constitute the autonomic nervous system, are derived from a common progenitor from the neural crest, and its development is controlled by a network of transcription factors, including the master regulator, Phox2b, and its downstream, Gata3. Anti-Phox2b and anti-Gata3 antibodies were applied to a total of 77 autonomic nervous system tumors, including 35 paragangliomas, 21 pheochromocytomas, 9 neuroblastomas, 4 ganglioneuroblastomas, and 8 ganglioneuromas, as well as their potential morphologic mimics, including tumors of the small round cell tumor group, neuroendocrine carcinomas of lung and gastrointestinal tract (carcinoid tumors/neuroendocrine tumors, large cell neuroendocrine carcinomas, and small cell carcinomas), Merkel cell carcinomas, benign and malignant tumors of thyroid, parathyroid, and adrenal cortex, and malignant melanomas. A variety of nonendocrine/neuroendocrine carcinomas were also studied. Gata3 expression was seen in 89% of paragangliomas, 95% of pheochromocytomas, and all neuroblastomas, ganglioneuroblastomas, and ganglioneuromas, as well as in all parathyroid tumors, a majority of urothelial and mammary carcinomas, and a subset of squamous cell carcinomas, but all other tumors were negative. Phox2b expression was seen in all neuroblastomas, ganglioneuroblastomas, and ganglioneuromas and in 40% of paragangliomas, but pheochromocytomas and all other tumors were negative. Gata3 is a highly reliable marker for paragangliomas, pheochromocytomas, and neuroblastic tumors to distinguish from their simulators. This is an additional utility for this marker, which is used for the diagnosis of urothelial and mammary carcinomas. Phox2b is also highly specific, but its low sensitivity to paragangliomas and pheochromocytomas would limit the utility only to neuroblastic tumors.

Recurrent myxoid liposarcoma of the buccal mucosa in a young boy: a case report and review of the literature.

Liposarcomas are uncommon in the pediatric population. We present the case of a boy who had experienced multiple recurrences of liposarcoma in the buccal space before he reached the age of 13 years. We also provide a review of the literature and a discussion of adjuvant therapy, which are important to understanding the nature of this disease.

Dietary bile acid supplementation improves intestinal integrity and survival in a murine model.

PURPOSE: In vitro supplementation of the bile salt, taurodeoxycholic acid (TDCA), has been shown to stimulate proliferation and prevent intestinal apoptosis in IEC-6 cells. We hypothesize that addition of TDCA to a rodent liquid diet will be protective against induced intestinal injury. METHODS: C57Bl6 mice were fed a liquid diet with or without 50-mg/(kg d) TDCA supplementation. After 6 days, the mice were injected with lipopolysaccharide (LPS) (10 mg/kg) to induce intestinal injury. Specimens were obtained 24 hours later and evaluated for intestinal apoptosis, crypt proliferation, and villus length. A separate cohort of animals was injected with LPS (25 mg/kg) and followed 7 days for survival. RESULTS: Mice whose diet was supplemented with TDCA had significantly increased survival. After LPS-induced injury, mice supplemented with TDCA showed decreased intestinal apoptosis by both H&E and caspase-3. They also had increased intestinal proliferation by 5-bromo-2'deoxyuridine staining and increased villus length. CONCLUSIONS: Dietary TDCA supplementation alleviates mucosal damage and improves survival after LPS-induced intestinal injury. Taurodeoxycholic acid is protective of the intestinal mucosa by increasing resistance to injury-induced apoptosis, stimulating enterocyte proliferation, and increasing villus length. Taurodeoxycholic acid supplementation also results in an increased survival benefit. Therefore, bile acid supplementation may potentially protect the intestine from injury or infection.

Intraabdominal pulmonary sequestration presenting with elevated urinary normetanephrine levels.

Carrying a prenatal diagnosis of a left-sided intraabdominal mass, a term female newborn underwent postnatal imaging that confirmed a left suprarenal mass. Urinary normetanephrine levels were elevated. Given a preoperative diagnosis of neuroblastoma, the baby underwent an uneventful resection of the mass en bloc with the left adrenal gland. The pathologic examination returned pulmonary sequestration and a normal adrenal gland. Postoperative urinary catecholamines were normal. To the best of our knowledge, this is the first description of a newborn with an intraabdominal pulmonary sequestration presenting with elevated urinary catecholamines.

Placental Cryptococcus neoformans infection without neonatal disease: case report and review of the literature.

We report placental cryptococcosis in a woman with multidrug resistant human immunodeficiency virus (HIV) infection. She received antifungal therapy for cryptococcal meningitis prior to delivery. Cesarean section was performed with delivery of a single full-term male infant. There was no evidence of HIV or cryptococcal infection in the infant. The placenta grossly showed multiple white nodules. Microscopically, numerous encapsulated budding yeasts, morphologically consistent with cryptococci, were identified in the intervillous space and, to a lesser extent, in the chorionic villi. Cryptococcal infections are not uncommon, but only 2 cases of placental cryptococcosis have been reported. Our case is the 1st account documenting cryptococcal organisms within the chorionic villi, and yet there was no evidence of infection in the infant. Mother-to-fetal transmission of cryptococcal infection is not well defined. We review the literature and discuss its possible mechanisms.