Introduction Lewis and Huff briefly described the presence of “microcystic cryptitis” in some of fetal vermiform appendices (VA) at autopsy. We further characterized these crypt changes (CC), their timing of occurrence, and tested their association with infection/inflammatory conditions. Methods Hematoxylin and eosin-stained slides of 345 VA were evaluated for the presence or absence of CC and their different morphologies. Autopsy reports were reviewed for evidence of amniotic fluid or fetal systemic infection and placental inflammatory conditions. Results Crypt dilatation with or without irregularity of the lumen, crypt dilatation with semiattenuated epithelium, intraluminal apoptotic debris and inflammatory cells, especially eosinophils, and foci of swirled spindled cells with calcifications or multinucleated giant cells were observed, either alone or in combination, in at least 58.5% (202/345) of the VA. CC began to appear at 17 weeks, peaked at 20 to 25 weeks (with up to 82% of VA exhibiting CC during this time), and followed by a steady decline beyond 28 weeks gestation. χ2 test of independence showed no significant association (P = .435; >0.05) between the presence and absence of CC and infection status of the fetus or placenta. Conclusion The underrecognized CC of the developing fetal vermiform appendix (VA) showed distinct temporal pattern of occurrence and did not seem to be affected by the presence or absence of infection, which so far favored their being a part of the normal gut developmental process.

To compare the histopathologic findings in placentas of fetuses who underwent fetal MMC (fMMC) repair versus placentas of those who underwent neonatal MMC repair. Single center retrospective cohort review of placentas from fetuses prenatally diagnosed with MMC between June 2008 to March 2014. All patients enrolled in the NIH sponsored MOMS trial were excluded. Two cohorts were identified: Group A (fMMC repair=89) and Group B (neonatal MMC repair=83). Mean GA at delivery in Group A was 33.9 weeks (22.0-37.2). Mean GA at delivery for Group B was 37.2 weeks (27-39). All mothers in Group A were maintained on chronic doses of calcium channel blockers for postoperative tocolysis from immediately after fMMC repair until delivery. Rates of chronic decidutis and laminar necrosis 34/89 (38%) were higher in fMMC placentas when compared to the placentas of fetuses who underwent neonatal repair 16/83 (19.2%), p value= 0.006. Placental weight did not differ significantly when compared to normative values for gestational age. An increased percentage of histopathologic lesions consistent with maladaptive changes in placental perfusion, such as chronic deciduitis and laminar necrosis, were identified the placentas of fetuses who underwent fMMC repair. These findings may indicate changes consistent with placental malperfusion as a result of chronic exposure to calcium channel blockers used for postoperative tocolysis. Further research is indicated to determine the effects of prolonged exposure to calcium channel blockers in this setting.

Background: The placenta is a complex organ that influences fetal growth and development, and through vascular programming may impact long-term postnatal health and well-being. Despite its likely i...

HomeCirculationVol. 118, No. 14Thoracopagus Conjoined Twins Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessReview ArticlePDF/EPUBThoracopagus Conjoined Twins R. Thomas CollinsII, MD, Tricia R. Bhatti, MD, Dale S. Huff, MD and Paul M. Weinberg, MD R. Thomas CollinsIIR. Thomas CollinsII From Division of Cardiology (R.T.C., P.M.W.) and Departments of Pathology and Laboratory Medicine (T.R.B., D.S.H., P.M.W.) and Radiology (P.M.W.), the Children’s Hospital of Philadelphia, Philadelphia, Pa; and the University of Pennsylvania School of Medicine (R.T.C., D.S.H., P.M.W.), Philadelphia, Pa. Search for more papers by this author , Tricia R. BhattiTricia R. Bhatti From Division of Cardiology (R.T.C., P.M.W.) and Departments of Pathology and Laboratory Medicine (T.R.B., D.S.H., P.M.W.) and Radiology (P.M.W.), the Children’s Hospital of Philadelphia, Philadelphia, Pa; and the University of Pennsylvania School of Medicine (R.T.C., D.S.H., P.M.W.), Philadelphia, Pa. Search for more papers by this author , Dale S. HuffDale S. Huff From Division of Cardiology (R.T.C., P.M.W.) and Departments of Pathology and Laboratory Medicine (T.R.B., D.S.H., P.M.W.) and Radiology (P.M.W.), the Children’s Hospital of Philadelphia, Philadelphia, Pa; and the University of Pennsylvania School of Medicine (R.T.C., D.S.H., P.M.W.), Philadelphia, Pa. Search for more papers by this author and Paul M. WeinbergPaul M. Weinberg From Division of Cardiology (R.T.C., P.M.W.) and Departments of Pathology and Laboratory Medicine (T.R.B., D.S.H., P.M.W.) and Radiology (P.M.W.), the Children’s Hospital of Philadelphia, Philadelphia, Pa; and the University of Pennsylvania School of Medicine (R.T.C., D.S.H., P.M.W.), Philadelphia, Pa. Search for more papers by this author Originally published30 Sep 2008https://doi.org/10.1161/CIRCULATIONAHA.108.789941Circulation. 2008;118:1496The incidence of conjoined twins is estimated at 1 in 50 000 births.1 Thoracopagus is the most common form of conjoined twins,2 with fusion from the anterior thorax to the umbilicus. A common pericardial sac is present in 90% of thoracopagus twins, and conjoined hearts are seen in 75%.3The present case was diagnosed prenatally, and the twins subsequently experienced intrauterine demise at ≈22 weeks gestation. Postmortem angiography demonstrated conjoined hearts with a left ventricle common to both twins (Figure). At autopsy, the cardiac anatomy for both hearts was found to be an unbalanced complete atrioventricular canal defect with a common left ventricle. Download figureDownload PowerPointFigure. Postmortem angiography via the umbilical arteries of thoracopagus twins demonstrating conjoined hearts. Ao indicates aorta; PA, pulmonary artery; LV, left ventricle; and RV, right ventricle.DisclosuresNone.FootnotesCorrespondence to R. Thomas Collins, II, MD, The Cardiac Center, Second Floor, Main Bldg, 34th St and Civic Center Blvd, Philadelphia, PA 19104. E-mail [email protected]References1 Amiel GJ. Conjoined entire twins: a case of thoraco-omphalopagus with discussion on nomenclature, obstetric management and anatomical note. Br J Clin Pract. 1967; 21: 141–146.MedlineGoogle Scholar2 Noonan JA. Twins, conjoined twins, and cardiac defects. Am J Dis Child. 1978; 132: 17–18.MedlineGoogle Scholar3 Tandon R, Sterns LP, Edwards JE. Thoracopagus twins: report of a case. Arch Pathol. 1974; 98: 248–251.MedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetails September 30, 2008Vol 118, Issue 14 Advertisement Article InformationMetrics https://doi.org/10.1161/CIRCULATIONAHA.108.789941PMID: 18824656 Originally publishedSeptember 30, 2008 PDF download Advertisement SubjectsCongenital Heart Disease

PURPOSE:Cryptorchidism occurs in 25% of boys with myelomeningocele (MMC) compared to 3% of the general population. Testicular biopsy histopathology correlates with future sperm counts. We studied testicular histology in boys with cryptorchidism and MMC to investigate if the MMC influences histological findings.MATERIALS AND METHODS:The study group consisted of six patients with MMC and undescended testis (UDT) who underwent orchiopexy and bilateral testis biopsy. Twelve testicular biopsies from six patients were compared to 40 biopsies from 20 UDT-only controls. Total germ cell count per tubule (TGC/T) and the percentage of adult dark spermatogonia (%Ad) in undescended and contralateral descended testes from the patients were compared with controls.RESULTS:In the study group, two had total absence of germ cells (TGC/T=0) and three had severely reduced germ cells (TGC/T<0.2). Four had total absence of Ad spermatogonia and the remaining two had severely reduced Ad spermatogonia (%Ad=5). The mean TGC/T and %Ad in patients with UDT and MMC were conspicuously lower than controls. The differences did not reach statistical significance (P=0.09-0.29).CONCLUSION:These results suggest that patients with both MMC and UDT have a more severe reduction in total number and more severely delayed maturation of germ cells than do patients with UDT alone. With only six patients in this study, there was not the power to detect statistical significance. In addition to the reproductive problems due to erection and ejaculatory dysfunction in patients with MMC, this severe testicular histopathology may increase the risk of subfertility.

You have accessJournal of Urology1 Apr 2008THE POSITIVE PREDICTIVE VALUE OF PREPUBERTAL TESTIS BIOPSY ON ADULT SPERM DENSITY IN PATIENTS WITH BILATERAL UNDESCENDED TESTES Steve S Kim, Thomas F Kolon, Pasquale Casale, Michael C Carr, Stephen A Zderic, Douglas A Canning, Dale S Huff, and Howard M Snyder Steve S KimSteve S Kim More articles by this author , Thomas F KolonThomas F Kolon More articles by this author , Pasquale CasalePasquale Casale More articles by this author , Michael C CarrMichael C Carr More articles by this author , Stephen A ZdericStephen A Zderic More articles by this author , Douglas A CanningDouglas A Canning More articles by this author , Dale S HuffDale S Huff More articles by this author , and Howard M SnyderHoward M Snyder More articles by this author View All Author Informationhttps://doi.org/10.1016/S0022-5347(08)60413-9AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail "THE POSITIVE PREDICTIVE VALUE OF PREPUBERTAL TESTIS BIOPSY ON ADULT SPERM DENSITY IN PATIENTS WITH BILATERAL UNDESCENDED TESTES." The Journal of Urology, 179(4S), pp. 144–145 © 2008 by American Urological AssociationFiguresReferencesRelatedDetails Volume 179Issue 4SApril 2008Page: 144-145 Advertisement Copyright & Permissions© 2008 by American Urological AssociationMetricsAuthor Information Steve S Kim More articles by this author Thomas F Kolon More articles by this author Pasquale Casale More articles by this author Michael C Carr More articles by this author Stephen A Zderic More articles by this author Douglas A Canning More articles by this author Dale S Huff More articles by this author Howard M Snyder More articles by this author Expand All Advertisement PDF downloadLoading ...

Summary: Biventricular hypertrophy was noted at 24 weeks' gestation in a fetus with isolated cytochrome- c oxidase (COX) deficiency. Shock, caused by hypertrophic cardiomyopathy and severe pulmonary hypertension, led to the patient's death on day 6. His phenotype defines a new lethal variant of COX deficiency characterized by prenatal-onset cardiopulmonary pathophysiology.

This chapter concerning normal and abnormal testicular development represents 28 years of our experience based on semi-thin histologic sections of over 13,000 testicular biopsies mostly from patients with cryptorchidism and male infertility. The series also includes testicular biopsies from 30 testes of complete Androgen Insensitivity Syndrome (AIS), 20 of mixed gonadal dysgenesis, 24 of XXY Klinefelter syndrome, 6 of male Turner syndrome (Noonan syndrome), 10 of XX-male syndrome, and 10 of hernia uteri inguinalis (persistent Mullerian duct syndrome). Special effort was taken to compare abnormal testicular development from intersex patients with that of normal testicular development from birth to adulthood.

The WT1 gene encodes a transcription factor implicated in normal and neoplastic development. The purpose of this study was to evaluate the diagnostic utility of a commercial WT1 antibody on a variety of pediatric small round blue cell tumors (SRBCT). A mouse monoclonal antibody (clone: 6F-H2, DAKO) raised against the N-terminal amino acids 1–181 of the human WT1 protein was tested. Microscopic sections from 66 specimens were stained using an antigen retrieval protocol with trypsin. The tumors included peripheral neuroectodermal tumors (PNET/Ewing's), neuroblastomas, desmoplastic small round cell tumors (DSRCT), lymphomas, Wilms' tumors, and rhabdomyosarcomas (RMS). One RMS case was investigated by Western blot analysis and RT-PCR to confirm the antibody specificity. A strong cytoplasmic staining was demonstrated in all RMS (11/11). The Western blot analysis confirmed the WT1 protein in the tissue, and the RT-PCR confirmed the presence of WT1 mRNA in the peripheral blood and tissue of one RMS patient. The Wilms' tumors had a variable nuclear and/or cytoplasmic positivity in most (17/24) cases. All PNET/Ewing's were negative. The nuclei of two lymphoblastic lymphomas stained strongly. A weak nuclear or cytoplasmic staining was reported in a few DSRCT (3/5), lymphomas (2/10), and neuroblastomas (2/8). This is a useful antibody in the differentiation of RMS from other SRBCTs. A strong cytoplasmic staining favors an RMS, and a strong nuclear staining is suggestive of a Wilms' tumor. A role for WT1 in the pathogenesis of rhabdomyosarcomas is raised. The limited sampling precludes any conclusions regarding the value of tissue or peripheral blood analysis for WT1 mRNA in patients with rhabdomyosarcoma.

A potential consequence of systemic administration of viral vectors is the inadvertent introduction of foreign DNA into recipient germ cells. To evaluate the safety of in vivo recombinant adeno-associated virus (rAAV) mediated gene transfer approaches for hemophilia B, we explored the risk of germline transmission of vector sequences following intramuscular (IM) injection of rAAV in four species of male animals (mouse, rat, rabbit and dog). In vector biodistribution studies in mice and rats, there is a dose-dependent increase in the likelihood that vector sequences can be detected in gonadal DNA using a sensitive PCR technique. However, in dogs DNA extracted from semen is negative for vector sequences. To address this discrepancy, studies were done in rabbits, and both semen and testicular DNAs were analyzed for the presence of vector sequences. These studies showed that no AAV vector sequences were detected in DNA extracted from rabbit semen samples collected at time points ranging from 7 to 90 days following IM injection of 1 x 10(13) vector genomes rAAV (vg) per kg. In contrast, DNA extracted from gonadal tissue was positive for vector sequences, but the positive signals diminished in number and strength with time. By FISH analysis, AAV signals were localized to the testis basement membrane and the interstitial space; no intracellular signal was observed. We observed similar findings following hepatic artery administration of rAAV in rats and dogs, suggesting that our findings are independent of the route of administration of vector. Attempts to transduce isolated murine spermatogonia directly with AAV-lacZ were unsuccessful. In clinical studies human subjects injected IM with an AAV vector at doses up to 2 x 10(12) vg/kg have shown no evidence of vector sequences in semen. Together, these studies suggest that rAAV introduced into skeletal muscle or the hepatic artery does not transduce male germ cells efficiently. We conclude that the risk of inadvertent germline transmission of vector sequences following IM or hepatic artery injection of AAV-2 vectors is extremely low.