Salah Ahmed Ibrahim, Haydar Abubakr Abdalla, Ahmed Mohammed El Hassan
{"title":"Case report: congenital salivary gland analage tumor presenting with neonatal respiratory distress.","authors":"Salah Ahmed Ibrahim, Haydar Abubakr Abdalla, Ahmed Mohammed El Hassan","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Stridor in the newborn is not an unusual entity. This article describes a rare presentation of neonatal respiratory distress caused by a benign nasopharyngeal salivary gland analage tumor.</p>","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"22 3","pages":"209-11"},"PeriodicalIF":0.0,"publicationDate":"2003-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22383756","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Nandita Kakkar, R K Vasishta, A K Banerjee, R K Marwaha, B R Thapa
{"title":"Familial hemophagocytic lymphohistiocytosis: an autopsy study.","authors":"Nandita Kakkar, R K Vasishta, A K Banerjee, R K Marwaha, B R Thapa","doi":"10.1080/pdp.22.3.229.242","DOIUrl":"https://doi.org/10.1080/pdp.22.3.229.242","url":null,"abstract":"<p><p>We describe four classical cases of familial hemophagocytic lymphohistiocytosis (FHL), a macrophage-related, autosomal recessive fatal disorder. Parental consanguinity was present in three cases. All patients presented with fever, neurological involvement of varying degrees, hepatosplenomegaly, cytopenias, deranged liver function tests, and coagulogram, hypofibrinogenemia (three cases), and hyperlipidemia (one case). An antemortem diagnosis could not be made, although it was suspected in one case. Necropsy (done in three cases and postmortem liver biopsy in one case) revealed classical features of FHL. Florid lymphohistiocytic infiltrate exhibiting hemophagocytosis was seen in the bone marrow, liver, spleen, lymph nodes and brain (examined in two case). In addition to this, focal infiltrates were seen in the kidneys, lung, pancreas, testes, adrenals, and skin. Marked lymphoid depletion was seen in one case in the lymph nodes and spleen.</p>","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"22 3","pages":"229-42"},"PeriodicalIF":0.0,"publicationDate":"2003-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/pdp.22.3.229.242","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22384269","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Z. Melegh, I. Bálint, E. Tóth, E. Csernák, Z. Szentirmay, Kálmán Nagy
{"title":"Detection of n-myc gene amplification in neuroblastoma by comparative, in situ,and real-time polymerase chain reaction.","authors":"Z. Melegh, I. Bálint, E. Tóth, E. Csernák, Z. Szentirmay, Kálmán Nagy","doi":"10.1080/15227950307737","DOIUrl":"https://doi.org/10.1080/15227950307737","url":null,"abstract":"We have used semiquantitative comparative and real-time quantitative polymerase chain reactions (PCR) to detect n-myc gene-amplification in 20 frozen neuroblastoma biopsies and IMR 32 cell line to predict biological behavior of the tumors. Two primer pairs were used for the semiquantitative method to co-amplify a 520-bp fragment of the beta-globin gene--used as a single copy reference standard--and a 258-bp fragment of the n-myc gene. After 30 cycles the PCR products were electrophoresed through an agarose gel and were compared to each other with use of a gel-densitometer. Real-time quantitative analyses were performed in a LightCycler instrument. A single primer pair was used to amplify a 120-bp fragment of the n-myc oncogene and a LC640-labeled fluorescent probe pair to detect the product. Calibration curve, set up from a serial dilution including samples with 1, 2, 10, 13, 25-fold n-myc oncogene amplification, was used for quantitative analysis. The semiquantitative method did not show distinct difference between tumor groups with no amplification and less than 10-fold amplification, whereas quantitative LightCycler analysis was able to detect even 2-fold amplification. Differentiated neuroblastomas seldom show n-myc amplification. In spite of this, we have found two partly differentiated tumor samples that contained n-myc amplification. In these cases in situ PCRs were performed to examine the tumor heterogeneity. We used biotinated ATP labeling and the same primer pair as for the LightCycler analysis. In both cases differentiated cells did not show n-myc gene amplification, whereas considerable amplification was detected in the neuroblasts.","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"22 3 1","pages":"213-22"},"PeriodicalIF":0.0,"publicationDate":"2003-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"59824857","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Placental calcium pump: clinical-based evidence.","authors":"John M Kasznica, Eugen B Petcu","doi":"10.1080/pdp.22.3.223.227","DOIUrl":"https://doi.org/10.1080/pdp.22.3.223.227","url":null,"abstract":"<p><p>Placenta can be considered as a pump of calcium necessary for the normal development of the fetus. We believe that the location of this pump is in the placental basement membrane. The calcification of this membrane has been described only in cases of in utero fetal death. In this study we describe for the first time a case of placental calcification in a living fetus. The fetus of a normal 21-year-old pregnant woman showed heart abnormalities but the genetic analysis showed a normal male karyotype. The histology of the placenta demonstrated multiple intravillous linear and granular calcific incrustations The hemtoxylin/eosin stain of the sections revealed basement membrane calcific incrustations and intravillous calcium deposits. We postulate that the fetal circulation in the villi was impaired and the calcium that reached the villi from the mother was deposited at this level.</p>","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"22 3","pages":"223-7"},"PeriodicalIF":0.0,"publicationDate":"2003-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/pdp.22.3.223.227","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22383757","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Pathology teach and tell: intravascular endothelial hyperplasia forming colonic polyps.","authors":"Nandita Kakkar, R K Vasishta, K L Narsimhan","doi":"10.1080/pdp.22.2.181.185","DOIUrl":"https://doi.org/10.1080/pdp.22.2.181.185","url":null,"abstract":"A12-year-oldmale childwasbrought to the emergency roomwith fresh rectalbleeding for the preceeding 24 h.There was a similar episode 6 months earlier that had subsided spontaneously. On examination, the child was pale, restless, and the peripheral pulses were thready. His blood pressure was 60=40 mmHg in the right upper limb; hemoglobin was 6.6 mmol=l, platelet count and coagulation pro¢le were normal.The child was resuscitated with fresh whole blood and explored.The entire colon up to the ileocecal junctionwas ¢lled with fresh blood. On careful palpation of the colon, a nodular circumscribed ¢rmmass,0.5 cm in diameter, was felt arising in the transverse colon near the splenic £exure. The segment of the colon containing the mass was resected and colon continuity was restored by end-to-end anastomosis. Gross examination of the colon showed small sessile polypoidal projections ranging in size from 0.3 to 0.6 cm in diameter. Few of the polyps were hemorrhagic. On microscopy, the vessels in the mucosa and submucosa revealed a variety of lesions. Many vessels showed an exhuberant solid intravascular endothelial proliferation forming tumor nodules within the vessels with total occlusion of their lumina (F|gure1).These intravascular nodules projected into the lumenof thebowel as polyps. The proliferating cells were plump endothelial cells with small lumina that did not showanyatypia ormitosis (F|gure 2).The endothelial nature of the cells was con¢rmed by a strong positive immunostaining for factor VIII^ related antigen. Some of the vessels were lined by large prominent endothelial cells only and few revealed multilayering with slight luminal compromise (F|gure 3). Few of the polyps containing vessels with intravascular endothelial proliferation revealed ulceration with active bleeding (F|gure 4). Only an occasional vessel showed endothelial proliferation admixed with ¢brin (F|gure 4).","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"22 2","pages":"181-5"},"PeriodicalIF":0.0,"publicationDate":"2003-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/pdp.22.2.181.185","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22219005","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The isoprenoid pathway and the pathogenesis of Reye's syndrome.","authors":"Ravi Kumar Kurup, Parameswara Achutha Kurup","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Unlabelled: </strong>The isoprenoid pathway produces three key metabolites: endogenous digoxin (regulator of neurotransmitter uptake), dolichol, and ubiquinone (free radical scavenger). Because a mitochondrial dysfunction has been described in Reye's syndrome, we thought it pertinent to assess the pathway in this disease. Since endogenous digoxin can regulate neurotransmitter transport, the pathway also was assessed in patients with right hemispheric, left hemispheric, and bihemispheric dominance to find out the role of hemispheric dominance in its pathogenesis. The plasma/serum activity of HMG CoA reductase, magnesium, digoxin, dolichol, ubiquinone, tryptophan/tyrosine catabolic patterns, free radical, and lipid levels as well as (red blood cell) RBC Na(+)-K(+) ATPase activity were measured in the above mentioned groups.</p><p><strong>Results: </strong>In the patient group as well as in individuals with right hemispheric dominance similar patterns were obtained. There was elevated digoxin and dolichol levels with low levels of ubiquinone in patients with Reye's syndrome as well as in those with right hemispheric dominance. The serum magnesium and RBC Na(+)-K(+) ATPase activity were reduced. There also was an increase in tryptophan catabolites and a reduction in tyrosine catabolites as well as increased free radical levels. Reye's syndrome is associated with an upregulated isoprenoid pathway, elevated hypothalamic digoxin secretion, and right hemispheric chemical dominance.</p>","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"22 2","pages":"159-70"},"PeriodicalIF":0.0,"publicationDate":"2003-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22219002","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ada Rivera, Suzanne Meleg-Smith, Igor Yosipiv, Samir El-Dahr, Frank Boineau
{"title":"Diagnosis of congenital nephrotic syndrome: a clinical and a pathologic challenge.","authors":"Ada Rivera, Suzanne Meleg-Smith, Igor Yosipiv, Samir El-Dahr, Frank Boineau","doi":"10.1080/pdp.22.2.105.116","DOIUrl":"https://doi.org/10.1080/pdp.22.2.105.116","url":null,"abstract":"<p><p>The diagnosis of congenital nephrotic syndrome (NS) is a challenge both for clinicians and for pathologists. We observed three cases in a series of 50 children with NS nonresponsive to therapy, corresponding to one case each of minimal change disease, Finnish-type glomerulopathy, and diffuse mesangial sclerosis--two histopathologic studies were performed in each case. The age at presentation did not predict the diagnosis nor the prognosis: The NS presented at 7 months of age in the patient with diffuse mesangial sclerosis, but it was present at birth in the patient with minimal change disease. In these 2 patients the final diagnosis was made with the first renal biopsy. Conversely, in the patient with Finnish-type glomerulopathy, the diagnosis was only possible in the repeat biopsy, as the early pathologic changes were nonspecific. This study shows the essential role of the renal biopsy in determining the etiologic diagnosis and prognosis in patients with congenital nephrotic syndrome. congenital nephrotic syndrome diffuse mesangial sclerosis Finnish-type glomerulopathy</p>","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"22 2","pages":"105-16"},"PeriodicalIF":0.0,"publicationDate":"2003-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/pdp.22.2.105.116","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22219659","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Nandita Kakkar, R K Vasishta, A K Banerjee, Arun Bansal
{"title":"Pathology teach and tell: melanotic ependymoma.","authors":"Nandita Kakkar, R K Vasishta, A K Banerjee, Arun Bansal","doi":"10.1080/pdp.22.2.171.174","DOIUrl":"https://doi.org/10.1080/pdp.22.2.171.174","url":null,"abstract":"A13-year-old male child presented with headache and vomiting of one-month duration.There was horizontal nystagmus, and fundus examination revealedbilateral papilloidema. There was no motor or sensory de¢cit. Contrast enhanced computed tomographs of the cranium (F|gure 1) revealed an inhomogenous poorly enhancing mass involving the fourth ventricle with gross obstructive hydrocephalus. A suboccipital craniectomy and near total excision of the tumor was done. The tumor was ¢lling the fourth ventricle and extending down to the third cervical vertebra. Microscopically, the tumor consisted of large polygonal cells arranged in sheets, occasionally showing a perivascular orientatation. A large number of tumor cells contained aprominent, ¢nely dispersed granular brownpigment (F|gure 2). These granules acquired a deep green color on Schmorl’s stain (F|gure 3). The pigment was Prussian blue negative, PAS positive (F|gure 4), and bleached by potassium permanganate treatment. Tumor cells did not express HMB-45.","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"22 2","pages":"171-4"},"PeriodicalIF":0.0,"publicationDate":"2003-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/pdp.22.2.171.174","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22219001","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Letter to the editor--multifocal myocardial apoptoses: distinctive cardiac lesions in cystic fibrosis and other diseases.","authors":"Jiri T Beranek","doi":"10.1080/pdp.22.2.187.188","DOIUrl":"https://doi.org/10.1080/pdp.22.2.187.188","url":null,"abstract":"Being interested in the mechanism of cardiomyocyte cell death [1], I have read with attention the article by Nezelof et al. [in this publication; 2] about multifocal myocardial necrosis in cystic ¢brosis and other diseases.Today, it is known that necrosis, called more suitably accidental cell death, leads to in£ammatory reaction whereas apoptosis, the secondmechanismof cell death, does notprovoke in£ammation. Since there is no in£ammatory reaction in the cases described by Nezelof et al. [2], wemayask justi¢ably whether the cardiomyocytes in question have not died by apoptosis instead of necrosis. Indeed, cardiomyocyte apoptosis is present in F|gure 2 of the article [2].Numerous atrophic cardiomyocytes are scattered amid di¡use ¢brosis in the upper two thirds of the ¢gure. Amajority of themmanifest a fragmentation into small apoptotic bodies. The reader may be mislead by their similarity to vessels ¢lled with red cells [1], a phenomenon already described in the hearts of systemic sclerosis patients [3]. Acarefulmorphologic analysis shows, however, that apoptoticbodies are inside cardiomyocytes and cannot be, therefore, red cells. In the bottom third of F|gure 2, cardiomyocytes are less damaged and it is easy to ¢nda clearly recognizable myo¢ber fragmenting into apoptoticbodies [2].The lackof in£ammatory reaction in F|gure 2 also con¢rms the conclusion that the cardiomyocytes in question are dying by apoptosis [2]. F|gures1, 3, and 4 are, unfortunately, of too lowmagni¢cation tovisualize cellular details [2].They show, however, numerousmyocardial clefts that are not present in the normal heart and that often contain tissue remains.The latter may be either artefacts (the shrunken interstitium) or deadcardiomyocytes. I would advance that the second alternative is true because the tissue remains often manifest similar tinctorial properties as surrounding them, cardiomyocytes and=or contain dark small apoptototic bodies inside them. Again, the lack of in£ammatory reaction indicates","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"22 2","pages":"187-8"},"PeriodicalIF":0.0,"publicationDate":"2003-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/pdp.22.2.187.188","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22217751","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Pathology teach and tell: neonatal ELS.","authors":"Pratibha A Ankola, Ashraf Morkos","doi":"10.1080/pdp.22.2.175.179","DOIUrl":"https://doi.org/10.1080/pdp.22.2.175.179","url":null,"abstract":"A2790-gs full-termmalebornby normal spontaneous vaginal delivery to a 28year-old gravida 2 para1with antenatal fetal diagnosis of cystic left lungmass associated with mediastinal shift to the right and no hydrops fetalis. The lung mass was ¢rst seen at 20 weeks gestation on ultrasound and increased in size over 9 weeks. Follow-up ultrasound at 34 weeks showed decrease in size of the mass. Karytotypewas 46XY. Apgar scores were 9 and 9 at1and 5 min, respectively. Physical examination of the infant at birth revealed tachypneawith respiratory rate 80=min min and mild lower intercostal retractions. Air entry was equal and good bilaterally. Heart soundswere heardbest to the right of themidline.Oxygen saturation remained 96^99%. Initial blood gas was within normal limits. Baby was placed on nasal CPAP 4 with F|02 0.25 due to increasing tachypnea anddesaturation. ChestX-ray showed left lower lobe density, withmediastinal shift to the right. Left diaphragmwasvisualizedwell (F|gure1).Computedtomography (CT) scanwith POcontrast con¢rmedthe cystic left lungmass (F|gure 2). Di¡erential diagnosis included bronchopulmonary sequestration and congenital cystic adenomatoid (CCAM) malformation of the lung.The baby had increased pCO2 with tachypnea needing intubation and mechanical ventilation from the 5th day. Echocardiogram showed tiny patent ductus arteriosus (PDA), large left atrial appendage, and a large £ow in left pulmonary veins.Thebabyhadelective surgeryonthe11th day. At surgery, amassmeasuring 5 4 cmwas removedandcon¢rmedtobe a left extralobar sequestrationwithblood supply fromavessel arising fromthe aorta.Thebabywas extubated uneventfully on the 3rd postoperative day and placed on nasal CPAP for1day before weaning o¡ to room air.The infant remained tachypneic intermittently with respiratory rate up to 80=min for 2 days.The baby was discharged on the10th postoperative day in stable condition.Histopathologyof themass con¢rmedthe diagnosis (F|gure 3).","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"22 2","pages":"175-9"},"PeriodicalIF":0.0,"publicationDate":"2003-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/pdp.22.2.175.179","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22217752","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}