RESUMO
H-type tracheo-oesophageal fistula (H-TOF) and congenital oesophageal stenosis (COS) are both rare anomalies. Only six cases of H-TOF in association with COS have been described in the literature. Characteristically, diagnosis is delayed, often well beyond the neonatal period. We report a patient who was accurately diagnosed on day 12 of life, and outline several of the key clinical dilemmas encountered in managing a neonate with this combination of rare congenital anomalies.
Assuntos
Estenose Esofágica/congênito , Estenose Esofágica/cirurgia , Fístula Traqueoesofágica/cirurgia , Estenose Esofágica/diagnóstico , Humanos , Recém-Nascido , Masculino , Fístula Traqueoesofágica/diagnósticoRESUMO
Renal candidiasis is a rare but well recognised complication of intensive care treatment in neonates. We report a problematic case with obstructing pelvi-ureteric junction (PUJ) fungal balls that was refractory to standard measures of adequate nephrostomy drainage, pelvic irrigation with amphotericin B, and systemic antifungal treatment. In an attempt to avoid potentially difficult open surgery, a novel treatment using a streptokinase solution (3,000 IU/ml) for irrigation was performed. This was successful in clearing the fungal balls, relieving obstruction and rendering the urine sterile. The present case highlights a potential role for streptokinase in cases of renal candidiasis refractory to current treatment protocols.
Assuntos
Antifúngicos/uso terapêutico , Candidíase/tratamento farmacológico , Recém-Nascido de Baixo Peso , Nefropatias/microbiologia , Pelve Renal/microbiologia , Estreptoquinase/uso terapêutico , Obstrução Ureteral/microbiologia , Seguimentos , Humanos , Hidronefrose/microbiologia , Recém-Nascido , Nefropatias/tratamento farmacológico , Masculino , Nefrostomia Percutânea , Resultado do Tratamento , Obstrução Ureteral/tratamento farmacológicoAssuntos
Doenças Uretrais/fisiopatologia , Cálculos da Bexiga Urinária/fisiopatologia , Pré-Escolar , Disuria/fisiopatologia , Humanos , Masculino , Ultrassonografia , Doenças Uretrais/diagnóstico por imagem , Doenças Uretrais/cirurgia , Cálculos da Bexiga Urinária/diagnóstico por imagem , Cálculos da Bexiga Urinária/cirurgiaRESUMO
OBJECTIVES: To determine if the indications for cutaneous vesicostomy have changed with the advent of prenatal diagnosis and to assess if temporary vesicostomy remains an effective measure in preventing urinary infection and stabilising renal function in children with severe uropathy. METHODS: Retrospective case note review. RESULTS: Nineteen patients underwent temporary cutaneous vesicostomy over a 12-year period. Of these, 10 had a prenatally detected uropathy. Children with prenatal findings were diverted at a significantly younger age than cases presenting clinically (33 (range 10-240) vs. 182 days (range 28-425), p = 0.02). In addition, there were significantly more boys diverted in the prenatally detected group (p = 0.02). The majority of clinically presenting infants were diverted because of severe or recurrent urosepsis whereas most prenatally detected cases underwent surgery for progressive renal impairment. In both groups vesicostomy was successful in preventing further urosepsis and resulted in improved renal function. Follow-up data, however, revealed 9 (50%) of 18 patients with chronic renal failure. One patient died prior to stoma closure from septicaemia and renal disease. CONCLUSIONS: The timing and indications for vesicostomy may be changing in the light of prenatal diagnosis. Temporary vesicostomy diversion remains a simple, effective and easily reversible method of preventing urosepsis and stabilising upper tract function. The poor renal outcome on follow-up may reflect the fact vesicostomy is reserved for infants and children with severe disease.
Assuntos
Cistostomia/métodos , Ultrassonografia Pré-Natal , Doenças da Bexiga Urinária/cirurgia , Bexiga Urinária/anormalidades , Bexiga Urinária/diagnóstico por imagem , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Falência Renal Crônica/prevenção & controle , Testes de Função Renal , Masculino , Gravidez , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Doenças da Bexiga Urinária/congênito , Doenças da Bexiga Urinária/diagnóstico por imagem , Infecções Urinárias/prevenção & controleRESUMO
PURPOSE: We attempted to determine if the degree of second trimester dilatation and/or other qualitative sonographic features of the fetal urinary tract are predictive of postnatal outcome in male neonates with posterior urethral valves. MATERIALS AND METHODS: We reviewed ultrasound reports and/or hard copy imaging in 17 cases of posterior urethral valves initially detected on second trimester scans (median gestation 18 weeks, range 15 to 23.5). Ultrasound appearance was categorized according to the pattern and severity of dilatation, cystic change or echogenicity of the renal cortex and presence of oligohydramnios. Outcome was reviewed at a median followup of 5.7 years (range 4.4 to 10). RESULTS: Of the 17 cases there was a poor outcome in 10, including death in 4 and chronic renal failure in 6. Seven patients were alive and well with normal renal function at followup. The prognosis in cases of moderate or severe upper tract dilatation was poor. Of the 9 patients with marked prenatal hydroureteronephrosis 8 (89%) were dead or had chronic renal failure at followup. In contrast, only 2 of the 8 patients (25%) with mild upper tract dilatation or dilatation limited to the bladder had chronic renal failure at review (p = 0.05). Three cases of prenatal renal parenchymal change and 3 of subsequent oligohydramnios had a poor outcome postnatally. CONCLUSIONS: The prognosis for boys with prenatally detected posterior urethral valves is closely associated with qualitative aspects of second trimester scan findings. This information may be of clinical value in the prenatal counseling of parents.
Assuntos
Ultrassonografia Pré-Natal , Uretra/anormalidades , Uretra/diagnóstico por imagem , Obstrução Uretral/diagnóstico por imagem , Feminino , Seguimentos , Humanos , Recém-Nascido , Masculino , Valor Preditivo dos Testes , Gravidez , Segundo Trimestre da GravidezRESUMO
Integrins are thought to be essential adhesion receptors for the maintenance of tissue histioarchitecture. The purpose of this study was to determine integrin expression patterns in the human stratified transitional epithelium of the urinary tract (urothelium). In situ expression patterns were compared with in vitro expression, using a normal cell culture model system in which the effects of cell stratification can be studied independently of differentiation. By immunohistological criteria, the urothelia of bladder, ureter and renal pelvis expressed alpha 2 beta 1 and alpha 3 beta 1 integrins in all layers at intercellular junctions, and cytoplasmically in the lower strata. By contrast, alpha 6 beta 4 and occasionally alpha v beta 4 were expressed only by basal cells and localised to the basal lamina. These expression patterns were unaltered in specimens where an inflammatory cell infiltrate was present. In long-term cultures of normal urothelial cells maintained in a low-Ca++ serum-free medium, the monolayer cultures expressed alpha 2 beta 1, alpha 3 beta 1 and alpha 5 beta 1 integrins at intercellular junctions and in cytoplasmic inclusions, whereas alpha 6 beta 4 was distributed in a random pattern over the substratum. Increasing exogenous Ca++ concentrations induced cell stratification and desmosome formation, but not cytodifferentiation. Under these conditions, alpha 6 beta 4 became cell-, rather than substratum-associated, localising particularly to filopodia and lamellipodia. Quantitation of integrin expression by flow cytometry confirmed increased surface expression of alpha 6 beta 4 in high Ca++ media, and also of alpha 3 and alpha 5, but not alpha 2, subunits. These results suggest that alpha 2 beta 1 and alpha 3 beta 1 integrins, although differentially regulated, are mainly involved in homotypic cell-cell interactions and the maintenance of a stratified morphology, whereas alpha 6 beta 4 is the principal integrin involved in substratum adhesion.
Assuntos
Integrinas/metabolismo , Sistema Urinário/metabolismo , Adolescente , Adulto , Cálcio/farmacologia , Moléculas de Adesão Celular/análise , Células Cultivadas , Criança , Pré-Escolar , Células Epiteliais , Epitélio/metabolismo , Proteínas da Matriz Extracelular/análise , Feminino , Humanos , Lactente , Masculino , Sistema Urinário/citologiaRESUMO
BACKGROUND: The purpose of the work was to establish urothelium as an in vitro model for the study of proliferation, stratification, and differentiation in "complex" epithelia. EXPERIMENTAL DESIGN: Normal human urothelial cells were cultured in a serum-free medium. The effects of epidermal growth factor (EGF), cholera toxin (CT), extracellular calcium and 13-cis-retinoic acid on cell growth, morphology, phenotype, and cytodifferentiation were studied using phase-contrast microscopy and indirect immunofluorescence. Stratification-related changes were additionally analyzed by transmission electron microscopy. RESULTS: Under optimized conditions, long-term cultures were successful in 44 (74.5%) out of 59 specimens. Bacterial infection was the most common cause of failure (9 cases). Primary urothelial cells required an initial plating density of > or = 10(4) cells/cm2 for survival; passaged cells survived much lower plating densities (> or = 2.5 x 10(2) cells/cm2). CT significantly improved cell attachment, but neither CT nor EGF were essential for growth. By contrast, cells failed to proliferate without bovine pituitary extract. In media containing bovine pituitary extract, CT, and EGF, cultures had a mean population doubling time of 14.7 +/- 1.8 hours, maintained a nonstratified phenotype, and expressed the cytokeratin (CK) profile of basal/intermediate urothelium: CK7, CK8, CK17, CK18 and CK19, with variable expression of CK13. CK20 was not expressed in vitro. CK14 and CK16 were also expressed, suggestive of squamous metaplasia in culture, which could be inhibited with 13-cis-retinoic acid. Increasing extracellular calcium from 0.09 to 0.9-4.0 mM slowed cell proliferation, induced stratification and desmosome formation, and increased expression of E-cadherin. High calcium, EGF, CT, and retinoic acid did not induce markers of late/terminal urothelial cytodifferentiation. CONCLUSIONS: We describe a simplified technique for the isolation and long-term culture of human urothelial cells. Urothelial cells in vitro are capable of rapid proliferation and can be induced to form integrated stratifying cell layers in high calcium medium. Stratification-related changes are not necessarily accompanied by urothelial cell maturation and differentiation.
Assuntos
Bexiga Urinária/citologia , Adolescente , Adulto , Cálcio/farmacologia , Diferenciação Celular/efeitos dos fármacos , Divisão Celular/efeitos dos fármacos , Células Cultivadas , Criança , Pré-Escolar , Toxina da Cólera/farmacologia , Meios de Cultura Livres de Soro/farmacologia , Fator de Crescimento Epidérmico/farmacologia , Células Epiteliais , Epitélio/efeitos dos fármacos , Epitélio/ultraestrutura , Feminino , Imunofluorescência , Humanos , Lactente , Masculino , Microscopia Eletrônica , Microscopia de Contraste de Fase , Fenótipo , Fatores de Tempo , Tretinoína/farmacologia , Bexiga Urinária/efeitos dos fármacos , Bexiga Urinária/ultraestruturaRESUMO
Between 1982 and 1992, 67 boys with posterior urethral valves were managed at our hospital, including 32 (48%) in whom the condition was detected prenatally. We examined the relationship between gestational age at detection and outcome at a median followup of 3.9 years (range 4 months to 10 years). Detection at or before 24 weeks of gestation predicted a poor outcome with 9 of 17 patients (53%) dead or in chronic renal failure at followup. Of the cases detected later in pregnancy only 1 had a poor outcome (p = 0.01). All of the cases detected after 24 weeks of gestation had had normal second trimester scans. Growth parameters for boys in the early and late detection groups were not statistically different. However, there was a significant association between renal failure and growth with 67% versus 14% having heights less than the 3rd percentile (p = 0.05). Respiratory distress at birth predicted a poor outcome, while the presence of palpable abdominal abnormalities or vesicoureteral reflux failed to predict outcome.
Assuntos
Doenças Fetais/diagnóstico por imagem , Ultrassonografia Pré-Natal , Uretra/anormalidades , Feminino , Seguimentos , Idade Gestacional , Transtornos do Crescimento/etiologia , Humanos , Recém-Nascido , Falência Renal Crônica/etiologia , Masculino , Gravidez , Prognóstico , Síndrome do Desconforto Respiratório do Recém-Nascido/etiologia , Estudos Retrospectivos , Uretra/diagnóstico por imagem , Refluxo Vesicoureteral/etiologiaRESUMO
The purpose of this study was to assess the accuracy of prenatal ultrasound in correctly diagnosing fetal structural abnormality requiring surgical intervention and to evaluate its effect on management and eventual outcome. Information was collected from existing departmental databases. From 1985 to 1991, 101 surgical disorders were suspected prenatally in the Yorkshire region; termination of pregnancy was requested in 6. Prenatal diagnoses were incorrect in 22/101, six being normal infants. In 28/101 cases the diagnosis was made after 30 weeks of gestation. Transfer to the tertiary referral centre was postnatal in 12/95 cases, although prenatal diagnosis should have allowed earlier referral. Mortality data for the 95 infants showed a survival of 73% overall. Definite parental anxiety resulting from either an incorrect diagnosis, inappropriate counselling, or unnecessary investigations was identified in 16 cases. Although prenatal diagnosis is helpful in the majority of cases, these findings suggest the need for improvements in diagnostic accuracy, inter-departmental communications and patient care.
Assuntos
Anormalidades Congênitas/diagnóstico por imagem , Doenças Fetais/diagnóstico por imagem , Ultrassonografia Pré-Natal , Aborto Eugênico , Anormalidades Congênitas/epidemiologia , Aconselhamento , Erros de Diagnóstico , Feminino , Doenças Fetais/epidemiologia , Humanos , Recém-Nascido , Masculino , Auditoria Médica , Pais/psicologia , Gravidez , Reprodutibilidade dos TestesRESUMO
We report two cases of focal nodular hyperplasia of the liver in childhood. Operative resection has resulted in complete relief of symptoms, with no evidence of recurrence on follow-up. We suggest that asymptomatic patients may be managed conservatively following appropriate radiological imaging and needle biopsy to exclude a malignant lesion. Surgical intervention is indicated in cases of diagnostic difficulty and for relief of symptoms.
Assuntos
Hepatopatias/patologia , Criança , Feminino , Humanos , Hiperplasia , Fígado/patologia , Hepatopatias/cirurgia , MasculinoRESUMO
An in vitro system for the growth of normal human urothelial cells has been developed. Urothelial cells were isolated from tissue samples in 38 patients and cultured in a defined serum-free medium. Confluent cell monolayers of 25 cm.2 were produced after 7 days in 32 cases. Subsequent subcultures at a 1:20 split ratio achieved confluency within another 7 days and a consistently high rate of growth was sustained for at least 7 passages. Characterization by immunofluorescence with a panel of antibodies demonstrated that the cultured cells were exclusively epithelial and retained the characteristic antigenic profile of normal urothelium, even after extended periods in culture. The only consistent cause of failure (6 of 38 cases) was bacterial contamination secondary to an underlying urinary tract infection in these patients.
Assuntos
Técnicas de Cultura , Epitélio/transplante , Bexiga Urinária/cirurgia , Adolescente , Antígenos/análise , Divisão Celular , Criança , Pré-Escolar , Células Epiteliais , Epitélio/imunologia , Feminino , Humanos , Lactente , Masculino , Bexiga Urinária/citologia , Bexiga Urinária/imunologiaRESUMO
This case report describes the development of a leiomyosarcoma in the chest wall of a patient previously treated for Hodgkin's disease. Although a similar case has not been reported previously, we managed our patient with wide local excision.
Assuntos
Doença de Hodgkin/terapia , Leiomiossarcoma/patologia , Segunda Neoplasia Primária/patologia , Neoplasias Torácicas/patologia , Adulto , Neoplasias Ósseas/patologia , Criança , Terapia Combinada , Humanos , Masculino , Costelas , Neoplasias de Tecidos Moles/patologiaRESUMO
Tailgut cysts are derivatives of the embryonic post-anal gut. Usually asymptomatic, they are discovered in adult life as an incidental retrorectal mass. Complications include infection, with the formation of retrorectal abscesses and occasionally and fistulae, and a long-term risk of malignant change, which means that once discovered surgical excision is advised. We present a case that was assessed pre-operatively with transrectal ultrasound. The findings were of a well defined cystic lesion, with no evidence of invasion. We believe that transrectal ultrasound is of value in assessing retrorectal lesions.
Assuntos
Cistos/diagnóstico por imagem , Doenças Retais/diagnóstico por imagem , Reto/diagnóstico por imagem , Adulto , Feminino , Humanos , UltrassonografiaRESUMO
Intraoperative enteroscopy was performed in 12 patients (median age 68 years) with obscure gastrointestinal bleeding probably of small bowel origin, six of whom were men. All the patients were evaluated by routine haematological, coagulation and biochemical profiles, upper and lower gastrointestinal endoscopies, visceral angiography and/or isotope scanning. All the patients were anaemic. Visceral angiography was useful on three of the 12 occasions on which it was used and isotope scanning was valuable on eight of the 11 occasions it was used. Nine patients had undergone previous laparotomy. Enteroscopy was performed successfully in all cases, with fresh blood and discrete vascular lesions being the chief findings (10 of 12 cases). Segmental resections (n = 8) and local resections (n = 2) were performed in ten patients, with two patients having more than one laparotomy for rebleeding. Five patients developed postoperative complications and there was an operative death and one late death. Three of the ten surviving patients experienced further rebleeding. Intraoperative enteroscopy is now an essential adjunct to laparotomy for gastrointestinal bleeding which has been localized to the small bowel before operation.