bluuebunny/crossref_metadata_2025 huggingface dataset:
Connective Tissue Growth Factor Expression in Pediatric Myofibroblastic Tumors
Authors: Kasaragod Arvind B. , Lucia M. Scott , Cabirac Gary , Grotendorst Gary R. , Stenmark Kurt R.
Published: July 2002

Human connective tissue growth factor (CTGF) is a secreted cysteine-rich peptide and a member of the peptide family that includes serum-induced immediate gene products such as a v-src-induced peptide and a putative proto-oncogene, c-src. CTGF is secreted by endothelial cells, fibroblasts, smooth muscle cells, and myofibroblasts. Its expression is increased in various human and animal fibrotic diseases. We hypothesized that tumors with significant fibrous and vascular components would exhibit increased expression of CTGF. We examined the expression of CTGF mRNA by in situ hybridization in 12 pediatric tumors and tumor-like conditions, including angiofibroma, malignant fibrous histiocytoma, infantile myofibromatosis, and malignant hemangiopericytoma. All the tumors showed moderate to intense CTGF expression in tumor cells and/or endothelial cells of the associated vasculature. Angiofibromas expressed CTGF only in factor VIII–positive endothelial cells and vascular smooth muscle cells. In contrast, infantile myofibromatosis, malignant hemangiopericytomas, and fibrous histiocytomas expressed CTGF in both endothelial cells and in vimentin-positive tumor cells, particularly those around the blood vessels. CTGF mRNA was not detected in the inflammatory cells observed in many of the tumors. The presence of CTGF in the endothelial cells and tumor cells around blood vessels raises the possibility that CTGF is involved in the pathogenesis of these myofibroblastic tumors.
Renin Gene Expression in Fetal Kidneys of Pregnancies Complicated by Twin-Twin Transfusion Syndrome
Authors: Kilby Mark D. , Platt Craig , Whittle Martin J. , Oxley Jill , Lindop George B.M.
Published: July 2002

Twin-twin transfusion syndrome (TTTS) complicates one in five monochorionic pregnancies and is generally associated with high mortality and morbidity. One twin (the recipient) grows appropriately and has polyhydramnios while the other (the donor) may have a reduced growth velocity and severe oligohydramnios. The disparities in amniotic fluid volumes represent differences in fetal urine output. These differences occur secondary to hemodynamic changes, in which the vascular arrangement of placental anastomoses in TTTS leads to unidirectional flow from the donor to the recipient twin. A better understanding of the pathophysiology may contribute to improved management of this morbid condition.

We studied three consecutive prospectively diagnosed stillborn twin pairs affected by early-onset TTTS. Renin gene expression was studied in sections of fetal kidneys with immunocytochemistry using a renin anti-serum and with in situ hybridization using riboprobes complementary to renin mRNA, and renin-secreting cells (RCC) were counted. The overall maturation of the renal cortex was assessed by the percentage of immature glomeruli.

The donor twin kidneys were smaller than those of the recipients, but the maturation of the renal cortex was not significantly different (28.2% immature glomeruli in the donor and 24.4% in the recipient kidney). The donor kidney showed increased renin gene expression with hyperplastic juxtaglomerular apparatuses (JGAs) that contained excess RCCs (median 20.02 [25th–75th centiles, 5.4, 25.1 RCCs per 100 glomeruli]). In contrast, the recipient kidney was virtually devoid of these cells (0.04 [0, 0.36] RCCs per 100 glomeruli; P < 0.05).

In the donor kidney, increased renin release may, by a local action, contribute to renal vasoconstriction and oliguria. Increased renin and/or angiotensin II in the blood passing through the placental anastomoses may, by an endocrine action, suppress renin synthesis in the recipient kidney, thereby increasing renal blood flow and causing polyuria and polyhydramnios. These changes in the renal RAS could thus contribute to the pathogenesis of TTTS. The renal renin changes noted here may represent a contributory or compensating mechanism, the success of which may dictate the overall survival of the twin pregnancy and allow better understanding of the pathophysiology and perhaps therapy that may be employed in this condition.
Enteric Adenovirus Infection in Pediatric Small Bowel Transplant Recipients
Authors: Parizhskaya Maria , Walpusk Janet , Mazariegos George , Jaffe Ronald
Published: July 2002

Three of 70 small bowel transplant recipients were diagnosed with adenovirus enteritis. The biopsies were performed for surveillance in one patient at 2.7 years after transplantation and in two symptomatic children 1.5 years and 4.5 months after transplantation. In all three patients the characteristic epithelial changes were not noted by the primary observers. Two biopsies had been called “suggestive of acute rejection” and both patients had been so treated. One biopsy had been diagnosed as “regenerative”. Once the epithelial changes were recognized as being viral, confirmation was possible by stool culture in one patient, immunohistochemistry in two patients, or by lift technique of the H&E sections for electron microscopy. The immune suppression was reduced and none of the patients developed disseminated infection. As in other transplanted organs, such as lung and liver, adenovirus infection may be limited largely to the allograft but can be destructive. Early recognition of the characteristic changes that are illustrated can lead to confirmation of the virus and appropriate reduction of immune suppression. A mistaken diagnosis of rejection and augmentation of immune suppression can lead to viral dissemination and potential fatality.
Intrathoracic Petechiae in Sudden Infant Death Syndrome: Relationship to Face Position When Found
Authors: Krous Henry F. , Nadeau Julie M. , Silva Patricia D. , Blackbourne Brian D.
Published: July 2002

Previous studies have not addressed the relationship of intrathoracic petechiae (IP) to the position of the face when a caretaker finds a victim of sudden infant death syndrome (SIDS). The aims of this retrospective study were to determine (1) the rate of the facedown position in SIDS (not to be confused with the prone body position), ( 2) if the facedown position occurred more frequently among SIDS victims with intrathoracic petechiae than those without petechiae, and (3) if the facedown position occurred more frequently among cases with more severe petechial hemorrhage of the thymus. We selected 199 SIDS cases from the San Diego SIDS Research Project database and grouped them as IP-present and IP-absent. Each case was analyzed with regard to the face position when found unresponsive or dead. Among these 199 cases, 37% were found facedown, which represents 51% of the 142 cases found prone. The two groups were similar with respect to age, sex, and rate of premature birth. Thirty-nine percent (39%) of the IP-present group and 9% of the IP-absent group were found in the facedown position ( P = 0.057; 95% confidence interval for the difference = 0.3%, 40%). Cases were also grouped by severity of thymic petechiae and analyzed regarding face position. Neither age nor the facedown position was associated with greater severity of thymic petechiae. The wide confidence interval yielded by our analysis of IP limits our ability to clarify the precise pathophysiologic role of external oronasal obstruction in SIDS. While it remains possible that a subset of SIDS cases occur as a result of external obstruction, we are unable to generalize its importance. Internal airway obstruction and rebreathing with terminal gasping, both of which have been documented in sudden infant death, remain other possible scenarios leading to the production of IP.
Tyrosinemia: A Review
Authors: Russo Pierre A. , Mitchell Grant A. , Tanguay Robert M.
Published: July 2002

Hypertyrosinemia encompasses several entities, of which tyrosinemia type I (or hepatorenal tyrosinemia, HT1) results in the most extensive clinical and pathological manifestations involving mainly the liver, kidney, and peripheral nerves. The clinical findings range from a severe hepatopathy of early infancy to chronic liver disease and rickets in the older child; gradual refinements in the diagnosis and medical management of this disorder have greatly altered its natural course, mirroring recent advances in the field of metabolic diseases in the past quarter century. Hepatorenal tyrosinemia is the inborn error with the highest incidence of progression to hepatocellular carcinoma, likely due to profound mutagenic effects and influences on the cell cycle by accumulated metabolites. The appropriate follow-up of patients with cirrhosis, the proper timing of liver transplantation in the prevention of carcinoma, and the long-term evolution of chronic renal disease remain important unresolved issues. The introduction of a new pharmacologic agent, NTBC, holds the hope of significantly alleviating some of the burdens of this disease. Mouse models of this disease have permitted the exploration of newer treatment modalities, such as gene therapy by viral vectors, including ex vivo and in utero methods. Finally, recent observations on spontaneous genetic reversion of the mutation in HT1 livers challenge conventional concepts in human genetics.
Cutaneous Ciliated Cyst with Interspersed Apocrine Features Presenting as a Pilonidal Cyst in a Child
Authors: Lee Maria Teresa , Heller Debra S. , Lambert W. Clark , Bethel Colin
Published: July 2002

We present a case of a cutaneous ciliated cyst that presented in a 13-year-old female. The lesion was felt to be a pilonidal cyst, based on its location and clinical appearance. This case is unusual because it is the youngest reported example of this very rare lesion. In addition, the admixture of apocrine and ciliated cells is extremely unusual. The histogenesis of cutaneous ciliated cysts is considered.
Cystic Fibrosis with Fibrosing Colonopathy in the Absence of Pancreatic Enzymes
Authors: Waters Brenda L.
Published: July 2002

Fibrosing colonopathy, characterized by dense submucosal fibrosis in the large bowel, is a disorder associated with bowel dysfunction in patients with cystic fibrosis who receive pancreatic enzyme supplementation. Most commonly, patients present with a distended abdomen and abdominal pain. Radiographs frequently demonstrate colonic wall thickening and luminal narrowing. Here I describe a neonate with cystic fibrosis who presented with both clinical and histological features of fibrosing colonopathy who had not received pancreatic enzymes. This report expands our understanding of the pathogenesis of fibrosing colonopathy.
Candida Funisitis: A Clinicopathologic Study of 32 Cases
Authors: Qureshi Faisal , Jacques Suzanne M. , Bendon Robert W. , Faye-Peterson Ona M. , Heifetz Stephen A. , Redline Raymond , Sander C. Maureen
Published: July 2002

We report on 32 cases of Candida funisitis and describe the associated clinicopathologic features. The Candida funisitis was characterized grossly by small, circumscribed, yellow-white nodules on the umbilical cord surface and, microscopically, by subamnionic microabscesses in which fungal organisms were demonstrable. Chorioamnionitis was present in all cases. Twenty-four (75%) of the 32 infants were premature. There were 7 perinatal deaths, all in immature fetuses. Five (16%) of the 32 fetuses had congenital candidiasis. Five (16%) of the mothers had a history of intrauterine foreign body, including intrauterine contraceptive device in three and cervical cerclage in two. The diagnosis of Candida funisitis should prompt a careful examination for fetal infection, even though it is associated with congenital candidiasis in only a minority of the cases.
Bone Marrow Metastasis in Ewing's Sarcoma and Peripheral Primitive Neuroectodermal Tumor: An Immunohistochemical Study
Authors: Lazda E.J. , Berry P.J.
Published: July 2002

Bone marrow metastases from small round cell tumors can present diagnostic difficulties. In this study, we assessed the value of immunohistochemistry, using two monoclonal antibodies to CD99, for the diagnosis of metastatic disease in bone marrow trephine specimens from patients with Ewing's sarcoma or primitive neuroectodermal tumor (PNET). The proportions of specimens showing metastases were 10.3% with routine staining and 20.7% with immunohistochemistry. The specimens that were negative on conventional light microscopy and positive with immunohistochemistry all showed other abnormalities. The results do not support the routine use of immunohistochemistry in specimens that are normal by conventional light microscopy, but indicate that useful information may be gained in cases where marrow histology is obscured by fibrosis, necrosis, or distortion artefact. Neither of the two antibodies tested was superior for this purpose.
Epidemiology of Intrathoracic Petechial Hemorrhages in Sudden Infant Death Syndrome
Authors: Becroft David M.O. , Thompson John M.D. , Mitchell Edwin A.
Published: July 2002

The possible effects of a wide range of sociodemographic and environmental factors on the incidence and distribution of petechiae were investigated in 485 sudden infant death syndrome (SIDS) cases from the New Zealand Cot Death Study. The number (nil, few, many) of macroscopic petechial hemorrhages in the visceral pleura, capsule of thymus, and epicardium was recorded in 458 of 474 autopsied SIDS cases. Other information was obtained from parental interview and obstetric records. Univariate analysis showed highly significant relationships ( P ≤ 0.005) between the frequency of petechiae at one or more sites and socioeconomic status, parity, breast feeding, age at death, time of death, sleep position, and head covering at death and lesser but significant relationships ( P ≤ 0.05) with Maori ethnicity, birth weight, gestation, pacifier use, and bed sharing. After multivariate analysis, significant associations remained between increased frequencies of thymic petechiae and parity (P = 0.0001), age at death (P = 0.0003), Maori ethnicity (P = 0.0019), pacifier use (P = 0.0001), and head covering at death (P = 0.0032); between increased frequencies of epicardial petechiae and head covering at death (P = 0.008) and an estimated time of death between 00:00 and 05:59 h ( P = 0.056); and between increased frequencies of pleural petechiae and maternal smoking ( P = 0.058) and parity ( P = 0.022). There was a decreased frequency of pleural petechiae in infants placed prone for their final sleep ( P = 0.058). The distribution and frequency of petechiae are affected by environmental factors, including known risk factors for SIDS, but these factors occur inconsistently across the three sites. The findings imply differences in the pathogenesis at each site but do not provide consistent support for previous theories of causation of petechiae.
Intraplacental Choriocarcinoma Associated with Viable Pregnancy: Pathologic Features and Implications for the Mother and Infant
Authors: Jacques Suzanne M. , Qureshi Faisal , Doss Barbara J. , Munkarah Adnan
Published: July 2002

Choriocarcinoma arising in the placenta, or intraplacental choriocarcinoma, has seldom been reported, particularly in the absence of maternal metastases. Reluctance to diagnose choriocarcinoma in the presence of chorionic villi can delay diagnosis; however, timely diagnosis of choriocarcinoma is prognostically important, both for the mother and infant. We report the clinicopathologic findings in five mothers and infants in whom choriocarcinoma was identified in the placenta. None of the mothers had a history of gestational trophoblastic disease in previous pregnancies. Three placentas were similar with a single small lesion grossly suggesting a small infarct; microscopically these consisted of infarcted areas surrounded by choriocarcinoma. These three mothers were unusual in that none had metastatic choriocarcinoma; two were treated with chemotherapy and remained disease-free; the third was lost to follow-up shortly following delivery. The remaining two mothers had known pulmonary metastases at time of delivery. One of these latter two placentas contained a large marginal lesion microscopically identified as choriocarcinoma. The fifth placenta had rare microscopic foci of choriocarcinoma, and sheets of necrotic choriocarcinoma were identified in “blood clot” submitted with the placenta. In four of the five cases the choriocarcinoma appeared to be arising from otherwise normal chorionic villi, and in no case was there invasion of the villous stroma. All of the infants survived, and none had evidence of choriocarcinoma. These cases support the concept that choriocarcinoma associated with otherwise normal pregnancy arises in the placenta and may be more common than reported.
Fibronectin Expression in Bronchopulmonary Dysplasia
Authors: Sinkin Robert A. , Roberts Michele , LoMonaco Michael B. , Sanders Raymond J. , Metlay Leon A.
Published: July 2002

Bronchopulmonary dysplasia (BPD) is a chronic fibrotic lung disease of neonates. Fibronectin (FN), a component of the extracellular matrix, is increased in the tracheobronchial effluent of neonates destined to develop BPD. Pulmonary FN is derived from plasma and local cellular synthesis. In order to identify which pulmonary cells synthesize FN and to test the hypothesis that FN is more abundant in lungs with BPD, we examined the distribution of pulmonary FN by in situ hybridization (for mRNA) and immunohistochemistry (for protein) in neonatal autopsy lung specimens, comparing lungs with BPD to those without. We used a staging system in which BPD is characterized by disruption of alveolar architecture, severe vascular changes, airway epithelial necrosis, smooth muscle hypertrophy, and peribronchial fibrosis. FN mRNA and protein were found in vascular endothelium, macrophages, fibroblasts, vascular and airway smooth muscle, and chondrocytes as well as in the pulmonary parenchyma in neonates with and without BPD. Hyaline membranes, when present, immunostained intensely for FN protein. FN mRNA was not seen in airway epithelial cells of either group. FN mRNA and protein were first increased in early acute BPD with their levels appearing greatest during the chronic reparative stage of BPD. In long-standing “healed” BPD, lower levels of FN mRNA and protein were seen. These findings are consistent with the association of increased FN with adult fibrotic lung disease and the previously reported increase in FN tracheal effluent levels in infants with BPD. Our results suggest an important role for pulmonary cell-derived FN in the early inflammatory and later proliferative stages of BPD.
Endocardial Fibroelastosis in Growth-Discordant Monozygotic Twins
Authors: Lazda E.J.
Published: July 2002

Two cases of fetal endocardial fibroelastosis are reported in the larger of growth-discordant monozygotic twins. The growth discordance in these two cases is regarded as a manifestation of twin-twin transfusion syndrome, but other causes are considered and the difficulties posed by the observed placental vascular anatomy are discussed. The literature on endocardial fibroelastosis in multiple pregnancy is briefly reviewed, and the relationship of endocardial fibroelastosis to the cardiovascular compromise seen in twin-twin transfusion syndrome is considered.
Cervical Spine Dysraphism with Teratoma Exhibiting Pulmonary Differentiation: Case Report and Review of the Literature
Authors: Ramdial Pratistadevi K. , Nadvi Sameer S. , Mallett Rene
Published: July 2002

Spinal dysraphism and teratomas are well-recognized spinal cord lesions, but both entities are rare in the cervical spinal cord. Rarely, teratomas have been described within dysraphic lesions in the thoracic and lumbosacral regions. To date, teratomas contained within cervical spine dysraphic lesions have not been described. Although mature cystic teratomas are renowned for the array of organoid tissue differentiation they display, mature lung differentiation is a rarity. While pulmonary differentiation has been described in six female genital tract teratomas, it has not been reported in spinal teratomas. The clinicopathologic features of a complex cervical lesion, comprising a combination of cervical spine dysraphism with a mature cystic teratoma that exhibited pulmonary differentiation, is presented, and the literature on teratomas exhibiting pulmonary differentiation is reviewed.
Yolk Sac Tumor of the Placenta in Wiedemann-Beckwith Syndrome
Authors: Drut Ricardo , Mortera Mónica , Drut Rosa Mónica
Published: July 2002

The present report describes an example of multifocal (two) yolk sac tumor (YST) with mesenchyme-like and enteroid patterns found in the placenta (730 g) of a newborn (4200 g) with Wiedemann-Beckwith syndrome (WBS) phenotype (macroglossia, omphalocele, hemihypertrophy, cardiomegaly, hypoglycemia). YST has not been previously reported to develop in the placenta. This case expands further the spectrum of alterations found in the placenta in the WBS and fits in the list of tumors related to WBS.
Placental Involvement in Congenital Hepatoblastoma
Authors: Doss Barbara J. , Vicari Joanne , Jacques Suzanne M. , Qureshi Faisal
Published: July 2002

We describe extensive placental involvement by hepatoblastoma in a 2600 g, 33-week estimated gestational age (EGA) hydropic female fetus with the hepatoblastoma otherwise limited to the liver. The placenta weighed 1190 g and histopathologic examination revealed diffuse tumor emboli in chorionic villous vessels. The placental tumor exhibited a cytologic appearance similar to the primary tumor and showed strong alpha-fetoprotein staining. Although unusual, other congenital tumors, including neuroblastoma and leukemia, have also been described metastatic to the placenta. This case emphasizes the important role of careful histopathologic examination of the placenta which, combined with immunohistochemistry and clinicopathologic correlation, may establish a diagnosis and possibly obviate the need for invasive neonatal diagnostic procedures.
Renal Tubular Dysgenesis in Twin-Twin Transfusion Syndrome
Authors: Oberg Kerby C. , Pestaner Joseph P. , Bielamowicz Lisa , Hawkins Edith P.
Published: July 2002

In twin-twin transfusion syndrome (TTTS), the disparity in circulation is reflected in discordant fetal growth, urine output, and amniotic fluid accumulation. The effect of uneven shunting of the growth factor and nutrient-rich vasculature on development and differentiation of the kidney has not been well studied. We analyzed renal tubular growth and differentiation in 25 fetal autopsies with TTTS (13 donors and 12 recipients, including 9 sibling pairs) between 18 and 33 weeks gestation. Immunohistochemical markers for fumarylace-toacetate hydrolase (FAH), Leu-M1, and Lotus tetragonolobus (LTA) were used to identify proximal convoluted tubules, and epithelial membrane antigen (EMA) was used to demonstrate distal convoluted and collecting tubules. FAH appeared to be more specific and reliable than either Leu-M1 or LTA in the identification of proximal tubules. Donors tended to demonstrate a paucity of proximal tubules with crowding of glomeruli characteristic of renal tubular dysgenesis (RTD). The degree of dysgenesis was greater in later gestations and associated with more severe growth restriction. Donors in TTTS are at risk for the development of RTD. Several authors suggest ischemia as the underlying cause of “acquired” RTD. However, in this setting there is no evidence of cell death or necrosis, and we suggest that hypoperfusion leading to decreased glomerular filtration is the underlying etiology, with the severity of RTD related to the degree of shunting.
Childhood Desmoplastic Fibroblastoma (Collagenous Fibroma) with a 12-Year Follow-up
Authors: Magro Gaetano , Venti Carmelo
Published: July 2002

We report the first case of a childhood desmoplastic fibroblastoma (collagenous fibroma). The tumor was located in subcutaneous tissue of the left buttock in a 5-year-old boy. No recurrence has been observed after 12 years, the longest follow-up available.
Correlation of Prenatal Ultrasound Diagnosis and Pathologic Findings in Fetal Anomalies
Authors: Sun Chen-Chih J. , Grumbach Kathryn , DeCosta Donna T. , Meyers Carol M. , Dungan Jeffrey S.
Published: July 2002

This retrospective study compared the prenatal ultrasound (US) diagnosis with autopsy findings in 61 intact fetuses following induced abortion and 36 fragmented fetuses from dilatation and evacuation (D&E). In intact fetuses, complete agreement between US diagnosis and autopsy findings was achieved in 65.6% of cases in the central nervous system (CNS) and 47.5% in other somatic organ systems (SOS). There were major differences between US and autopsy findings involving the CNS in 6.5% of cases and SOS in 27.9%. Correlation was better for evaluation of renal anomalies (complete agreement in 63.6% of 11 suspected cases, 2 false-positive and no false-negative cases) than congenital heart disease (complete agreement in 27.3% of 11 suspected cases, 5 false-positive and 3 false-negative cases). In D&E specimens, a prenatal diagnosis of neural tube defect (NTD) was confirmed in 90% of cases. However, due to fragmentation of fetal parts, the US diagnosis in the CNS could not be confirmed totally (69.4%) or partially (2.8%) in fetuses with chromosomal abnormalities (ChA) or multiple congenital anomalies (MCA). Nonetheless, the US diagnosis of SOS was confirmed in six cases on D&E, including Meckel-Gruber syndrome, cystic hygroma, renal agenesis with contralateral renal dysplasia, cardiac defect, fetal hydrops, and tracheal atresia. Our results show that a thorough autopsy of an intact fetus after abortion is necessary to confirm prenatal diagnosis and allow proper management and counseling. The pathologic examination of D&E specimens can reliably confirm the US diagnosis of NTD, but it is very limited in identifying other fetal anomalies.
Variant EWS-WT1 Chimeric Product in the Desmoplastic Small Round Cell Tumor
Authors: Chan Agnes S. , MacNeill Sue , Thorner Paul , Squire Jeremy , Zielenska Maria
Published: July 2002

Chromosome translocations found in neoplasms often result in the creation of hybrid genes encoding chimeric proteins. Desmoplastic small round cell tumor (DSRCT) is a recently described aggressive malignancy associated with a unique chromosomal translocation t(11;22)(p13; q12). This translocation has recently been characterized, revealing the rearrangement and fusion of the WT1 gene on chromosome 11 to the EWS gene on chromosome 22. Fusion of these two genes results in the production of a putative oncogenic protein composed of the zinc finger DNA-binding domains of WT1 linked to the potential transcriptional regulatory domains of EWS. The typical chimeric transcript consists of the first 7 exons of EWS and the last 3 exons of WT1. We report here the first case of DSRCT with a variant EWS-WT1 chimeric product that includes 9 exons of EWS and 3 exons of WT1.
Glomerular Membranopathy in Children with IgA Nephropathy and Henoch Schönlein Purpura
Authors: Vogler Carole , Eliason Stephen C. , Wood Ellen G.
Published: July 2002

We evaluated renal biopsies from 34 children with IgA nephropathy or Henoch Schönlein purpura to further characterize the ultrastructural features of the glomerular membranopathy that occurs in these disorders. Focal glomerular basement membrane damage was identified in 29 children and was severe in 4 of the children. Alterations included focal and segmental attenuation, splitting, duplications, and spike-like subepithelial protrusions of the lamina densa, along with saccular glomerular microaneurysms arising at the paramesangium. Those cases with extensive glomerular basement membrane lesions had either moderate or severe glomerular alterations apparent by light microscopy. Over half of the cases with glomerular membranopathy had immunohistological or ultrastructural evidence of focal peripheral glomerular capillary wall immune deposits and electron-dense deposits occurred at sites of glomerular basement membrane splitting. Despite the focal attenuation of the glomerular basement membrane, we did not identify any biopsy with findings of thin basement membrane disease. The glomerular basement membrane ultrastructural findings we describe are characteristic of IgA nephropathy and Henoch Schönlein purpura, are common in children with these disorders, and are similar to the ultrastructural alterations of the basement membrane that occur in other glomerulonephritides. These basement membrane injuries may be inflammatory cell or immune mediated but their pathogenesis requires further study.
Approximate Doubling of Numbers of Neurons in Postnatal Human Cerebral Cortex and in 35 Specific Cytoarchitectural Areas from Birth to 72 Months
Authors: Shankle W.R. , Rafii Michael S. , Landing Benjamin H. , Fallon James H.
Published: July 2002

From 1939 to 1967, J.L. Conel quantitatively studied the microscopic features of the developing human cerebral cortex and published the findings in eight volumes. We have constructed a database using his neuroanatomical measurements (neuronal packing density, myelinated large fiber density, large proximal dendrite density, somal breadth and height, and total cortical and cortical layer thickness) at the eight age periods (0 [term birth], 1, 3, 6, 15, 24, 48, and 72 postnatal months) he studied. In this report, we examine changes in neuron numbers over the eight age-points for 35 von Economo areas for which Conel gave appropriate data. From birth to 3 months postnatal age, total cortical neuron number increases 23–30%, then falls to within 3.5% of the birth value at 24 months, supporting our previous work showing that the observed decrease in the number of neurons per column of cortex under a 1-mm2 cortical surface from birth to 15 months is almost entirely due to cortical surface expansion. The present study also shows a 60–78% increase in total cortical neuron number above the birth value from postnatal ages 24 to 72 months. The generalization, to humans at least, of the finding of no postnatal neurogenesis in rhesus macaques, a species belonging to a super-family that diverged from that of Homo sapiens more than 25 million years ago, is not warranted until explicitly proven for humans. The data of the present study support the existence of substantial postnatal neurogenesis in humans for the 35 cortical areas studied.
Adnexal-Centered Giant Congenital Melanocyte Nevus with Extensive Ganglioneuromatous Component and Trisomy 7
Authors: Drut Ricardo , Drut Rosa Mónica , Cohen Marta
Published: July 2002

Adequate interpretation of clinical and histopathologic features of giant congenital melanocytic nevus (GCMN) in newborns is a continued challenge. A GCMN with three large nodules and three polypoid exophytic tumors presented in the dorsum of a female full-term newborn, the borders exhibiting a spotted grouped pattern. Microscopic examination revealed a peculiar adnexal-centered (eccrine sweat gland ducts, acrosiringia, and hair infundibula) compound nevus expressing pagetoid intraepidermal spreading of epithelioid melanocytes. The nodules represented an extensive ganglioneuromatous component. The neurons and their neuropil were positive for neuron-specific enolase, S-100, synaptophysin, tyrosine hydroxilase, and PGP 9.5. In addition to these components, a poorly differentiated, fusiform, low-mitotic rate population of cells undergoing epithelioid differentiation (and probably neuronal differentiation) with nodular arrangement was also present in the polypoid tumors and deeper parts of the nevus, in part intermixed with the neurons. These cells were vimentin positive but S-100 negative. FISH studies revealed these cells to express three signals for the centromeric probe for chromosome 7 whereas the neuronal component showed just two. Adnexal-centered arrangement of melanocytes has not been emphasized in GCMN. Ganglioneuromatous differentiation has been rarely reported in this condition. Trisomy 7 in GCMN has been reported only once previously.
Consistency of Isochromosome 7q and Trisomy 8 in Hepatosplenic γδ T-cell Lymphoma: Detection by Fluorescence in Situ Hybridization of a Splenic Touch-Preparation from a Pediatric Patient
Authors: Coventry Susan , Punnett Hope H. , Tomczak Ewa Z. , Casher Danielle , Koehler Maria , Borowitz Michael J. , Griffin Constance A. , de Chadarévian Jean-Pierre
Published: July 2002

Hepatosplenic gamma-delta (γδ) T-cell lymphoma is a rare but increasingly recognized lymphoid malignancy predominantly affecting young adult males. It is not well appreciated in the pediatric population. We report the third case of this aggressive lymphoma in a child as well as additional support for the consistency of the recently discovered cytogenetic abnormalities, isochromosome 7q and trisomy 8, which in this case were documented using fluorescence in situ hybridization (FISH) of a touch-preparation of the spleen.
Demonstration of Communication between Alveolus and Interstitium in Persistent Interstitial Pulmonary Emphysema: Case Report
Authors: Yao Jorge L. , Fasano Maria , Morotti Raffaella , Caprio Martha , Greco M. Alba
Published: July 2002

Persistent interstitial pulmonary emphysema (PIPE) is an uncommon complication of premature infants suffering from hyaline membrane disease who have been treated with mechanical ventilation. The presumed mechanism for the development of the disease is via a break in the bronchioalveolar system that allows air to escape into the interstitium. We report a case of a 9-week-old child who developed the localized form of the disease and underwent a lobectomy. Immunohistochemical stains helped to demonstrate the communication between the airway system and interstitium. This report strengthens the theory that the disease develops from airway rupture at the alveolar level.
Castleman's Disease of the Left Triceps in a Child Suspected to be a Small round Cell Tumor of Childhood
Authors: Fiel-Gan Mary D. , Voytek Theresa M. , Weiss Richard G. , Brown R. Timothy , Joshi Vijay V.
Published: July 2002

Castleman's disease (CD) is histologically characterized by a proliferation of polyclonal small lymphocytes and plasma cells. The clinical presentation varies widely, but most commonly manifests as a solitary mediastinal mass, incidentally found on radiographic examination. We present a case of a 10-year-old girl who exhibited a left arm mass which, preoperatively and on frozen section, was diagnosed as a small round cell tumor of childhood (SRCT). This report emphasizes the unusual location of CD in the soft tissue and as a rare entity to be considered in the differential diagnosis of SRCT.
Transient repression of the lac operon — the effect of a lac promoter deletion
Authors: Yudkin M.D.
Published: July 2002

Experiments have been done to show whether the lac promoter delection L1, which partly alleviates catabolite repression, also affects transient repression of lac. In stain L1/F′M15 all of the β‐galactosidase is synthesized from a chromosomal gene cis to L1, whereas 98% of the thiogalactosidase transacetylase is synthesized from an episomal gene cis to an intact i‐p‐o region. The addition of glucose to induced cultures of strain L1/F′M15 growing in glycerol medium caused extensive transient repression of transacetylase but almost no transient repression of β‐galactosidase. In control experiments with a diploid stain of genotype p
+
z
+
a
−/F′p
+
z
−
a
+ the two enzymes suffered equal transient repression. Thus L1 substantially relieves transient repression.
Does translational ambiguity increase during cell differentiation?
Authors: Picard-Bennoun Marguerite
Published: July 2002

Several observations made in the fungus Podospora anserina suggest that translational ambiguity may increase, and possibly must increase, at specific stages of the life cycle. Such changes in the properties of the translational apparatus seem to occur as well in the yeast S. cerevisiae and in the alga C. reinhardii. A slight increase of the misreading level would allow readthrough or frameshifting necessary to synthesise regulatory proteins in low amount at key points of cellular differentiation.
Proteolysis of the neurofilament 68 kDa protein explains several previously described brain proteins of unique composition and high acidity
Authors: Weber Klaus , Geisler Norbert
Published: July 2002

Neurofilaments follow the structural principles of non‐neuronal intermediate filaments but contain additional sequences which are carboxyterminally located and increase in length between triplet proteins (68 kDa, 160 kDa and 200 kDa). The tailpiece domain has been sequenced in the case of the porcine 68 kDa protein. It has a unique amino acid composition. Within 106 residues there are only 12 different amino acid types, and glutamic acid accounts for 46% of the sequence. Examination of the literature on highly acidic brain proteins leads us to the proposal that microglutamic acid‐rich protein, Glu‐50, macroglutamic protein, as well as some unusual components of the S100 class, are most likely proteolytic degradation products of the neurofilament 68 kDa protein.
The hexose uptake system of Chlorella: Is it a proton symport or a hydroxyl ion antiport system?
Authors: Komor Ewald , Schobert Christian , Cho Bong-Heuy
Published: July 2002

2‐Amino‐2‐deoxyglucose is taken up by the hexose transport system of Chlorella. The uptake of the neutral sugar proceeds together with stoichiometric alkalization of the medium. The uptake of the positively charged sugar (i.e., with a protonated amino group) proceeds as uniport and is not accompanied by net proton uptake but by charge compensating exit of proton and potassium. Thus the proton at the amino group replaces the cotransported ion, which therefore has to be a proton and not a hydroxyl ion. A depolarization of the membrane from − 130 mV to − 70 mV increased the K
m‐value 3‐fold without change of V
max. It is concluded that neutral sugar and proton are bound in close vicinity at a site in the middle of the membrane and that transport of sugar and proton occurs simultaneously.
In vivo 31P‐NMR studies of myocardial high energy phosphate metabolism during anoxia and recovery
Authors: Neurohr Klaus J. , Gollin Gerald , Barrett Eugene J. , Shulman Robert G.
Published: July 2002

31P NMR spectra of heart in‐situ in live guinea pigs were obtained continuously in 20.5 s time blocks during 3 min of anoxia, during subsequent reoxygenation and, in separate animals, during terminal anoxia. Reversible anoxia resulted in rapid degradation of phosphocreatine (t
½ = 54.5 ± 2.5 s) which recovered fully during reoxygenation. Heart Pi increased during anoxia and returned to basal levels after oxygen was restored. During 3 min of anoxia, no significant changes in ATP levels or pH were detected. The results demonstrate that it is feasible to measure rapid fluxes of high energy phosphates by 31P NMR in intact animals during and after anoxic stress to the myocardium.
Vitamin K‐dependent carboxylase: Synthesis of an inhibitor of the glutamyl binding site
Authors: Rich Daniel H. , Kawai Megumi , Goodman Hedda L. , Engelke Jean , Suttie J.W.
Published: July 2002

Liver microsomes contain a vitamin K and O2‐dependent carboxylase that converts peptide‐bound glutamyl residues to γ‐carboxyglutamyl residues. The peptide Boc‐O‐phospho—Ser‐O‐phospho—Ser—Leu‐OMe has now been synthesized. This peptide inhibits the carboxylation of endogenous protein precursors by a detergent‐solubilized preparation of the carboxylase and is an apparent competitive inhibitor of the carboxylation of Phe—Leu—Glu—Glu—Leu.
Site of tubulin synthesis in Tetrahymena pyriformis
Authors: Barahona Isabel , Rodrigues-Pousada Claudina
Published: July 2002

Free and membrane‐bound polysomes were isolated from the protozoa Tetrahymena pyriformis, and the contribution of these two types of polysomes to tubulin synthesis were studied using immunoprecipitation of the 35S‐translational products in a rabbit reticulocyte lysate. One‐dimensional electrophoretic analysis shows that tubulin is synthesized by polyadenylated RNA isolated from free and membrane‐bound polysomes. Non‐polyadenylated RNAs of free polysomes are also able to direct tubulin synthesis. Two‐dimensional electrophoretic analysis using O'Farrell's system confirms these results and also reveals the existence of the α‐ and β‐tubulin subunits.
Specific and reversible activation and inactivation of the mitochondrial phosphate carrier by cardiolipin and nonionic detergents, respectively
Authors: Mende P. , Hüther F.-J. , Kadenbach B.
Published: July 2002

The phosphate carrier of pig heart mitochondria was solubilized with Triton X‐100 and purified by chromatography on hydroxylapatite. Incubation of the phosphate carrier fraction with cardiolipin stimulated the reconstituted [32P]phosphate exchange activity in liposomes, whereas increased Triton X‐100 concentrations inhibited it. The effects of cardiolipin and Triton X‐100 are reversible. The activation by cardiolipin is highly specific and could not be obtained with any other applied phospholipid.
Schistosoma mansoni: antigenic community between schistosomula surface and adult worm incubation products as a support for concomitant immunity
Authors: Dissous Colette , Capron André
Published: July 2002

The use of protective monoclonal antibodies has enabled us to demonstrate antigenic community between a 38‐kDa schistosomula surface molecule and a 115‐kDa component derived from adult worms. Injection of adult worms in rats also led to the production of antibodies specific for the 38‐kDa antigen, suggesting that the 115‐kDa adult worm molecule could act as an inducer of the protective immune response raised against young invading parasites.
Isolation of a photosystem 2 preparation from higher plants with highly enriched oxygen evolution activity
Authors: Ford R.C. , Evans M.C.W.
Published: July 2002

Detergent‐treatment of higher plant thylakoids with Triton X‐100 at pH 6.3 has been used to purify a PS2 fraction with very high rates of oxygen evolution (1000 μmol.mg chl−1.h−1). A photosynthetic unit size of about 300 chlorophyll (chl) molecules has been determined by optical methods, suggesting an average turnover time for PS2 of about 2 ms. The donor system for P680+is particularly well preserved in the preparation, as judged by P680+reduction kinetics, the detection by EPR of Signal IILTand the presence of the high potential form of cytochromeb‐559 (at a ratio of 1:1 with the reaction centre).
Stimulation of an exocytotic event, the hamster sperm acrosome reaction, by cis‐unsaturated fatty acids
Authors: Meizel Stanley , Turner Kenneth O.
Published: July 2002

This cis‐unsaturated fatty acids oleic, arachidonic and cis‐vaccenic stimulated the hamster sperm acrosome reaction in vitro (an exocytotic event which occurs in the sperm head and which is essential for fertilization). The trans‐isomers of oleic and vaccenic acids did not stimulate the acrosome reaction, nor did the cis‐unsaturated fatty acids petroselenic and docosahexaenoic or the saturated fatty acids lauric, myristic or stearic. This is the first report of a stimulatory effect of cis‐unsaturated fatty acids on an exocytic event in an intact viable cell.
Proton NMR studies of denatured lysozyme
Authors: Dobson Christopher M. , Evans Philip A. , Williamson Kenneth L.
Published: July 2002

Evidence is presented from 1H NMR studies for non‐random conformational behaviour in denatured lysozyme in aqueous solution. A method is presented which permits the assignment of resonances in the 1H NMR spectrum of the denatured protein by observing magnetisation transfer from resonances of the native state. The use of these experiments in characterising the denatured state and the significance of these studies for the investigation of protein folding are discussed.
On the change of the charges in the four photo‐induced oxidation steps of the water‐splitting enzyme system S
Authors: Saygin Ö. , Witt H.T.
Published: July 2002

After dark adaptation of oxygen‐evolving PS II complexes, oscillatory absorption changes (stable > 0.5 s) with a periodicity of 4 were detected at 514 nm. They have been correlated with the four oxidation states of the water‐splitting enzyme system S. Supposing that the changes are due to electrochromic shifts, they might indicate a positive surplus charge in states S2and S3. This means that the electron release pattern 1:1:1:1 is accompanied by a pattern of charge formation 0: + : + : 0 and an intrinsic proton release stoichiometry 1:0:1:2 for the transitions So→S1, S1→S2, S2→S3, S3→S0.
Synthesis of diadenosine‐5',5'''‐p1,p3‐triphosphate in yeast at heat shock
Authors: Denisenko O.N.
Published: July 2002

Heat shock leads to the accumulation of diadenosine‐5',5'''‐p1,p3‐triphosphate (ApppA) in Saccharomyces cerevisiae. Its intracellular concentration increases up to 30 μM after 45‐min incubation at 41°C. I propose that ApppA can be an inducer of heat shock proteins.
Differential inhibition by plastoquinone analogues of photoreduction of cytochromeb‐559 in chloroplasts
Authors: Tsujimoto Harry Y. , Arnon Daniel I.
Published: July 2002

The high‐potential form of cytochromeb‐559 (b‐559 HP) is closely linked to the oxygenic photosystem (photosystem II) but its relation to other redox components of the photosynthetic apparatus, including plastoquinone, is still obscure. We investigated the photoreduction of cytochromeb‐559 HP by isolated chloroplasts in the presence of 3 antagonists of plastoquinone, of which, DBMIB (dibromothymoquinone) and DNP‐INT (dinitrophenyl ether of iodonitrothymol) are known to inhibit the oxidation of the plastoquinone pool (PQ) by the FeS‐cytochrome ƒ/b6complex and one, UHDBT (5‐n‐undecyl‐6‐hydroxy‐4,7‐dioxobenzothiazole) is known to inhibit the reduction of PQ by QB.QBis a protein‐bound plastoquinone that serves as a two‐electron gate for the reduction of PQ. We found that DBMIB and DNP‐INT did not inhibit but low concentrations of UHDBT severely inhibited the photoreduction of cytochromeb‐559 HP. These results suggest that the electron donor for the reduction of cytochromeb‐559 HP was either QBor a portion of the PQ pool that was oxidized by a new pathway free of binding sites for DBMIB and DNP‐INT.
Relationships between phosphoinositide metabolism, Ca2+ changes and respiratory burst in formyl‐methionyl‐leucyl‐ phenylalanine‐stimulated human neutrophils
Authors: Rossi F. , Bianca V.Delia , Grzeskowiak M. , De Togni P. , Cabrini G.
Published: July 2002

The relationships between the changes of cellular Ca2+ the activation of phosphoinositide turnover and the functional responses induced by the stimulus‐receptor interactions in neutrophils are matter of controversy. By measuring the concentration dependency of different formyl‐leucyl‐methionyl‐phenylalanine (FMLP)‐induced changes, the following values of ED50 were found: 1.6 and 0.8 nM for the rise in [Ca2+]i monitored with Quin‐2, in the presence and absence of exogenous Ca2+ respectively; 20 nM for the activation of phosphoinositide metabolism, monitored as change in the 32Pi of phosphatidate; 14 nM for membrane‐bound Ca2+ mobilization, monitored with chlorotetracycline (CTC); 34 nM for 45Ca2+ influx and 32 nM for the respiratory burst. Furthermore, low dose of FMLP causes an increase in [Ca2+]i in absence of activation of breakdown of phosphatidylinositol, phosphatidylinositol 4‐monophosphate and phosphatidylinositol 4,5‐biphosphate monitored as changes in [3H]glycerol radioactivity. The results clearly demonstrate that the increase in [Ca2+]i, due to the release from intracellular stores, is not caused by the breakdown of phosphatidylinositides. On the other hand, the data of the similarity of ED50 are compatible with an involvement of phosphoinositide response in the release of membrane bound Ca2+, monitored with CTC, and in the 45Ca influx and in the respiratory burst.
High sensitivity of carnitine acyltransferase I to malonyl‐CoA inhibition in liver of obese Zucker rats
Authors: Clouet Pierre , Henninger Catherine , Pascal Marc , Bézard Jean
Published: July 2002

Carnitine acyltransferase of liver mitochondria prepared from obese Zucker rats has a higher sensitivity to inhibition by malonyl‐CoA compared with carnitine acyltransferase of mitochondria prepared from lean Zucker rats.