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  • 1
    Electronic Resource
    Electronic Resource
    Case of the month: A newborn with tight skin and joint contractures (1996)
    Springer
    European journal of pediatrics 155 (1996), S. 987-989 
    Details
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02282893
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  • 2
    Electronic Resource
    Electronic Resource
    Familial progressive tubulo-interstitial nephropathy and cholestatic liver disease – a newly recognized entity? (1997)
    Springer
    European journal of pediatrics 156 (1997), S. 723-726 
    Details
    ISSN: 1432-1076
    Keywords: Key words Tubulo-interstitial nephropathy ; Cholestatic liver disease ; Primary sclerosing cholangitis ; Familial
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe two siblings (female and male) with progressive tubulo-interstitial nephropathy and cholestatic liver disease. The main characteristics were progressive renal failure and elevated liver enzymes (AST, ALT and γ-GT). Dialysis was started at the age of 1.9 and 6.5 years, respectively. Renal histology disclosed sclerosed glomeruli and atrophic tubules; the interstitium was fibrotic and infiltrated by lymphocytes. Endoscopic retrograde cholangiopancreatography revealed segmental irregularities and narrowing of the intrahepatic bile ducts, consistent with early primary sclerosing cholangitis. Liver histology showed enlarged portal triads, mild proliferation and inflammation of bile ducts, and fibrosis. At 5.9 years the girl underwent a successful renal transplantation whereas the boy is still on dialysis. Conclusion The association of progressive tubulo-interstitial nephropathy and cholestatic liver disease, consistent with early primary sclerosing cholangitis, constitutes a distinct autosomal recessive entity.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310050699
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  • 3
    Electronic Resource
    Electronic Resource
    Cystic nephroma: a rare benign renal tumor (1998)
    Springer
    Pediatric surgery international 13 (1998), S. 197-199 
    Details
    ISSN: 1437-9813
    Keywords: Key words Renal tumor ; Cystic nephroma ; Multilocular renal cyst ; Child
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report two boys aged 1 year and 2 years 2 months, respectively, with cystic nephromas. Both presented with a painless abdominal mass. Computed tomography showed a homogeneous, multicystic tumor of the lower pole of the kidney in both cases with thin septa without solid parts. Macroscopically, the surface of the tumor was smooth. Both patients underwent a renal-sparing procedure; histology confirmed the diagnosis of cystic nephroma.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s003830050290
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  • 4
    Electronic Resource
    Electronic Resource
    Acute appendicitis in neonates: complication or morbus sui generis? (1998)
    Springer
    Pediatric surgery international 14 (1998), S. 122-123 
    Details
    ISSN: 1437-9813
    Keywords: Key words Neonatal acute appendicitis with perforation  ;   Hirschsprung's disease  ;  Cystic fibrosis  ;  Necrotizing enterocolitis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Neonatal appendicitis (NA) is very rare and is associated with a very high perforation rate. Three cases treated at our hospital since 1969 are presented. All of them had a perforated appendix; two␣represented a complication of an underlying disease, i.e., Hirschsprung's disease and cystic fibrosis, respectively. These diseases should always be ruled out actively in cases of NA. If an underlying disease is not found, NA might be an isolated form of necrotizing enterocolitis limited to the appendix. Histology cannot distinguish between a simple appendicitis and a localized form of NEC, so NA must be regarded as a complication of an underlying disease as well as a morbus sui generis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s003830050457
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  • 5
    Electronic Resource
    Electronic Resource
    Incidental choriocarcinoma confined to a near-term placenta (1998)
    Springer
    Virchows Archiv 433 (1998), S. 89-91 
    Details
    ISSN: 1432-2307
    Keywords: Key words Choriocarcinoma ; Placenta ; Infarct
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  A large solitary choriocarcinoma was found incidentally in a placenta from a 36-week gestation following caesarean section performed because of intrauterine fetal distress. Macroscopically, there appeared to be a large old infarct in the centre of the placenta proper. Microscopically, there was extensive central necrosis with a rim of viable trophoblastic tumour that had the typical morphology of choriocarcinoma. Although the tumour was floating within maternal blood and was also detected in direct contact with fetal vessels, no metastatic disease was reported in the subsequent 11/2 years either in the mother or in the child. Placental infarcts are often not examined histologically, and an intraplacental tumour may thus be missed. Central friability and an unusual colour should alert the pathologist and lead to histological clarification. The management of an incidentally discovered intraplacental choriocarcinoma should be an expectant one, consisting of extensive workup for any evidence of metastases and serial β-HCG measurements in both mother and child.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004280050221
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  • 6
    Electronic Resource
    Electronic Resource
    Aberrant positioning of trophoblast and lymphocytes in the feto-maternal interface with pre-eclampsia (1999)
    Springer
    Virchows Archiv 434 (1999), S. 207-211 
    Details
    ISSN: 1432-2307
    Keywords: Key words Pregnancy ; Placenta ; Pre-eclampsia ; Lymphocyte ; NK cell
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Pregnancy represents the growth of an allograft where fetal trophoblast cells evade immune rejection and invade maternal tissue. There should be a balance between fetal trophoblast and maternal immune-responsive cells and alterations in the proportion of these cells may relate to pregnancy disorders. To test this, the decidual tissue of placental bed biopsies was examined and trophoblast cells and lymphocytes were quantified morphometrically; spiral arteries were classified as unchanged, transformed or affected by acute atherosis. Normal pregnancy (n=19) was characterized by the transformation of about one half of all spiral arteries within the placental bed. We found that 40% of all lymphocytes were CD56+ uterine NK cells and 60%, CD3+ T-lymphocytes; about 30% of these were CD8+ T cells. Intrauterine growth retardation in the context of preeclampsia (n=15) was accompanied by reduced trophoblast numbers within smaller and more tortuous arteries and an increase in the proportion of CD56+ uterine NK cells and CD8+ T lymphocytes in the decidua (70% of all CD3+ cells). In the case of pre-eclampsia without fetal growth retardation (n=14) no increase in CD56+ uterine NK cells was seen, while CD8+ T lymphocytes were significantly increased compared with the normal level (50% of all CD3+ cells). Fetal growth retardation is associated with poor transformation of spiral arteries and characterized by an increase of uterine NK cells. Symptoms of pre-eclampsia are independently associated with an increase in the cytotoxic T subset of decidual lymphocytes. Pre-eclampsia and related fetal growth retardation are seemingly caused by an enhancement of the maternal cytotoxic defence against the fetal allograft.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004280050329
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  • 7
    Electronic Resource
    Electronic Resource
    Adrenal crisis presenting as hypoglycemic coma (2000)
    Springer
    Intensive care medicine 26 (2000), S. 105-108 
    Details
    ISSN: 1432-1238
    Keywords: Key words Glucocorticoid deficiency ; Adrenal malformation ; Isolated ACTH deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract An 18-month-old male infant presented with hypoglycemic coma and clinical signs of bronchopneumonia. He was suspected of suffering from septic shock. The patient progressed to irreversible multiple organ failure before the diagnosis of adrenal crisis was established. Plasma levels of ACTH and cortisol remained undetectable. Renin and aldosterone were normal. An autopsy failed to demonstrate any adrenal gland cortical tissue. Immunohistochemical staining demonstrated the presence of all pituitary hormones except ACTH, establishing the diagnosis of isolated ACTH deficiency. Intensive care clinicians should consider adrenal crisis in non-diabetic children with hypoglycemia and rapid circulatory deterioration.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001340050021
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  • 8
    Electronic Resource
    Electronic Resource
    Placental site trophoblastic tumor Morphologie, Differentialdiagnose und Prognose (1995)
    Springer
    Der Pathologe 16 (1995), S. 315-320 
    Details
    ISSN: 1432-1963
    Keywords: Schlüsselwörter Trophoblast ; „Placental site trophoblastic tumor“ ; „Exaggerated placental site“ ; „Placental site nodule“ ; Zytokeratin ; β -HCG ; HPL ; Key words Placental ; Site trophoblastic tumors ; Exaggerated placental site ; Placental-site nodule ; Sytokeratin ; β -Human chorionic gonadotrophin ; Human placental lactogen
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary A placental-site trophoblastic tumor is a rare neoplasia that is derived from the cells of the intermediate trophoblast. Morphological, biochemical, and Doppler ultrasound findings are presented regarding differential diagnosis using material from three recent cases. Essentially, placental-site trophoblastic tumors can be diagnosed if infiltration of the myometrium is seen by a monomorphic trophoblastic proliferation that is not interrupted by decidual cells. Necrosis and hemorrhages are not features of placental-site trophoblastic tumors. However, there is a peculiar behavior towards the uterine vasculature as spiral arteries are dilated and transformed the same way as occurs at the site of physiological implantation of pregnancy. It appears that as a result of this phenomenon there is a characteristic finding in ultrasound. Examination of this type of tumor demonstrates cystic spaces that can be defined as blood vessels by their Doppler signal. In two of the three cases a hysterectomy was performed, and criteria for the assumption of malignant placental-site tumors are therefore presented. However, only the mitosis rate seems to possess some predictive value.
    Notes: Zusammenfassung Beim „placental site trophoblastic tumor“ handelt es sich um eine seltene Neoplasie, die aus den Zellen des sog. intermediären Trophoblasten entsteht. Die vorliegende Arbeit präsentiert morphologische, laborchemische und dopplersonographische Befunde zur Differentialdiagnose anhand von 3 kürzlich beobachteten Fällen. Wesentlich für die Annahme eines „placental site trophoblastic tumor“ ist der Nachweis einer Infiltration des Myometriums durch eine monomorphe trophoblastäre Zellpopulation, die nicht von dezidualen Zellen unterbrochen wird. Nekrosen und Hämorrhagien größeren Ausmaßes fehlen im Bereich des infiltrativen Wachstums; stattdessen kommt die Umgestaltung und Aufweitung mütterlicher Spiralarterien zur Beobachtung, wie sie für die physiologische Implantationsregion bekannt ist. Aus diesem Phänomen scheint ein charakteristischer Ultraschallbefund zu resultieren: in der Uteruswand gelegene zystischen Herde weisen dopplersonographisch einen gefäßtypischen Blutfluß auf. Da in 2 Fällen anschließend der Uterus zur Untersuchung kam, werden auch Kriterien zur Annahme eines malignen Verhaltens des Tumors vorgestellt. Morphologisch scheint nur die Mitoserate in der Läsion einen gewissen prädiktiven Wert zu besitzen.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s002920050108
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  • 9
    Electronic Resource
    Electronic Resource
    Nachweis des intermediären Trophoblasten als Beleg einer intrauterinen Schwangerschaft (1994)
    Springer
    Der Pathologe 15 (1994), S. 28-31 
    Details
    ISSN: 1432-1963
    Keywords: Schlüsselwörter: Intermediärer Trophoblast – Zytokeratine – Abort – Extrauteringravidität – Y-Chromosom – In-situ-Hybridisierung ; Key words: Intermediate trophoblast – Abortion – Ectopic pregnancy – Cytokeratin – Y-chromosome – In situ hybridisation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Abstract. With spontaneous abortion the conceptus is often expelled and lost right at the beginning, and uterine curettings then contain only endometrial fragments and clotted blood. Even complete embedding of all available material for histologic examination will not reveal any chorionic villi, and ectopic pregnancy can thus not be excluded. In such cases, the intermediate trophoblast can sometimes still be demonstrated within the endometrial tissue. This highly invasive trophoblast is difficult to identify using conventional staining, but cytokeratin antibodies are reliable markers of this cell type. Using immunohistochemistry, these fetal components could be demonstrated in 27 of 95 specimens (28.5%), proving the intrauterine nature of the aborted pregnancy. In some cases the fetal derivation of intermediate trophoblast was demonstrated by using in situ hybridisation to mark repetitive sequences on the Y-chromosome in the interphase nucleus.
    Notes: Zusammenfassung. Wird bei einem Spontanabort zu Beginn die eigentliche Fruchtanlage ausgestoßen und nicht asserviert, sind im Untersuchungsgut häufig nur noch endometriale Schleimhautfragmente und Blutkoagula anzutreffen. Auch bei vollständiger Aufarbeitung des Gewebes sind oft keine Chorionzotten mehr erkennbar. Somit kann das Vorliegen einer Extrauteringravidität nicht ausgeschlossen werden. Fetales Gewebe in Form des intermediären Trophoblasten (IT) ist manchmal aber noch innerhalb der bei der Kürettage geförderten Dezidua nachzuweisen. Diese invasive Trophoblastkomponente ist wegen ihrer Ähnlichkeit zum Deziduagewebe bei konventioneller Färbung kaum sicher zu identifizieren. Sie kann aber in zuverlässiger Weise immunhistochemisch mit Zytokeratinantikörpern dargestellt werden. Auf diese Weise konnten bei 95 intrauterinen Schwangerschaften ohne Chorionzotten im Abrasionsmaterial in 27 Fällen (28,5%) fetale Anteile in Form des intermediären Trophoblasten festgestellt werden und damit der Beweis einer ehemals intrauterin gelegenen Schwangerschaft erbracht werden. Darüber hinaus wurde an einigen Fällen die fetale Abkunft des IT mittels In-situ-Darstellung des Y-Chromosoms am Interphasenkern bewiesen.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s002920050021
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  • 10
    Electronic Resource
    Electronic Resource
    Glomerulopathie beim Denys-Drash-Syndrom Kasuistik einer Modellkrankheit (1998)
    Springer
    Der Pathologe 19 (1998), S. 230-234 
    Details
    ISSN: 1432-1963
    Keywords: Schlüsselwörter Denys-Drash-Syndrom ; Glomerulopathie ; Nephrotisches Syndrom ; WT1-Gen ; Nephroblastom ; Key words Denys-Drash Syndrome ; Glomerulopathy ; Nephrotic syndrome ; WT1 gene ; Nephroblastoma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary About 10% of all nephroblastomas (Wilms’ tumor) present as part of malformation syndromes. The Denys-Drash syndrome (DDS) comprises pseudohermaphroditism, glomerulopathy and, early, often bilateral Wilms’ tumors. A nephrectomy was performed in a 4-month-old girl because of a Wilms’ tumor. Two months later, low serum albumin levels and proteinuria had developed. A biopsy from the remaining kidney showed a glomerulopathy which could also be seen in the nephrectomy specimen. The morphology was highly characteristic: the innermost layer of the kidney cortex exhibited augmentation of the mesangial matrix only; the intermediate layer showed severe sclerosis of glomeruli with deposition of fibrillary material; and the subcapsular layer revealed very small glomeruli and atrophic tubuli. Fifteen months later, peritoneal dialysis was necessary and due to the high risk of tumor development in the remaining kidney, a nephrectomy was performed. Molecular analysis revealed a point mutation within exon 9 of the WT1 gene (394 ARG→TRP), which was homozygous in the tumor and heterozygous within renal parenchyma. The DDS is caused by a mutation in the WT1 gene on chromosome 11p13 which occurs during oogenesis or spermiogenesis. The WT1 gene is highly expressed during the development of the genitalia and the kidney; damage in one allele only causes the malformation syndrome. Loss of the second allele of the WT1 gene constitutes the second step of tumorigenesis. The appearance of Wilms’ tumors derived from cells homozygous for the mutation reveals the function of the WT1 gene as a tumor suppressor gene.
    Notes: Zusammenfassung Knapp 10% aller Nephroblastome (Wilms-Tumor) treten im Rahmen von Fehlbildungssyndromen auf. Das Denys-Drash-Syndrom (DDS) beinhaltet einen Pseudohermaphroditismus, eine Glomerulopathie und frühe, häufig beidseitige Wilms-Tumoren. Ein 4 Monate altes Mädchen wird wegen eines Wilms-Tumors nephrektomiert. Zwei Monate später wird ein nephrotisches Syndrom festgestellt. Eine Nierenbiopsie zeigt eine Glomerulopathie mit charakteristischer Morphologie: in den inneren Schichten der Nierenrinde ist die mesangialen Matrix vermehrt; in den mittleren Schichten finden sich stark sklerosierte Glomeruli, und die subkapsuläre Nierenrinde zeigt sehr kleine Glomeruli und undifferenzierte Tubuli. 15 Monate später ist das Kind dialysepflichtig. Wegen des Risikos einer Tumorentstehung in der verbliebenen Niere wird auch kontralateral nephrektomiert. Die molekularbiologische Analyse ergibt eine Punktmutation im Exon 9 des WT1-Gens (394 ARG→TRP), welche im Tumor homozygot ist und im Nierengewebe heterozygot. Das DDS entsteht durch eine in den Keimzellen auftretende Neumutation im WT1-Gen auf Chromosom 11p13. Das WT1-Gen wird in Genitalien und Nieren während der Embronalperiode stark exprimiert. Der heterozygote Zustand mit dem funktionellen Verlust nur eines Allels bedingt das Fehlbildungssyndrom. Mit dem Auftreten von Wilms Tumoren im homozygoten Zustand offenbart sich die Funktion des WT1-Gens als eines Tumorsuppressor-Gens.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s002920050279
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