RESUMO
Sarcoidosis is a disease of unknown etiology affecting most frequently young adults. Diagnosis is based on clinical, radiological and histopathological features and presentation is frequently of constitutional and respiratory symptoms, hilar and mediastinal adenopathies, with or without parenquimal involvement. Confirmation is histopathological with demonstration of non-caseificating granulomas in affected organs, with negative cultures for mycobacteria and fungi. We describe the clinical, radiological and histopathological findings in 8 patients. Mean age was 44 years (28-59) and half the patients were male. Two patients were symptom-free at diagnosis, thoracic findings were present in five (cough, retroesternal pain and arrhythmias) and one had extrathoracic symptoms (ankle arthritis). All showed mediastinal adenopathies in radiological and tomographic images (six were in stage 2 and two in stage 1). Two patients had spontaneous remission, four received oral steroids, one required oral steroids and azathioprine and there was no treatment data on one patient. In one patient, recidiva of severe and bilateral erithema nodosum required long term treatment with steroids and immunosupressors. Although sarcoidosis is a low prevalent disease in our country, a high index of suspicion is advisable when facing patients with respiratory symptoms, parenquimal involvement and mediastinal adenopathies, with or without extrathoracic manifestations.
Assuntos
Sarcoidose Pulmonar , Adulto , Diagnóstico Diferencial , Feminino , Glucocorticoides/uso terapêutico , Granuloma/diagnóstico por imagem , Granuloma/patologia , Humanos , Doenças Linfáticas/diagnóstico por imagem , Doenças Linfáticas/patologia , Masculino , Doenças do Mediastino/diagnóstico por imagem , Doenças do Mediastino/patologia , Pessoa de Meia-Idade , Prednisona/uso terapêutico , Prognóstico , Fibrose Pulmonar/diagnóstico por imagem , Fibrose Pulmonar/patologia , Radiografia , Sarcoidose Pulmonar/diagnóstico por imagem , Sarcoidose Pulmonar/tratamento farmacológico , Sarcoidose Pulmonar/patologiaRESUMO
La sarcoidosis es una enfermedad de etiología desconocida, que afecta con mayor frecuencia a adultos jóvenes. El diagnóstico es clínico, radiológico e histopatológico y la presentación frecuente es la de síntomas constitucionales y respiratorios, acompañados de linfoadenopatías hiliares y mediastinales, con o sin compromiso del parénquima pulmonar. La confirmación es anatomopatológica con la demostración de granulomas no caseificantes en los tejidos afectados, con cultivos negativos para micobacterias y hongos. Presentamos los hallazgos clínicos, radiológicos e histopatológicos en 8 pacientes. Cuatro eran de sexo masculino y la edad promedio fue de 44 años (28-59). Dos de los pacientes se encontraban asintomáticos al momento del diagnóstico, cinco de ellos se presentaron con hallazgos toráxicos (tos, dolor retroesternal y arritmias) y uno con síntomas extratoráxicos (artritis de ambos tobillos). Todos presentaron adenopatías mediastinales en los estudios radiológicos y tomográficos y seis de ellos se encontraban en el estadio 2. En cuanto al tratamiento, dos pacientes remitieron espontáneamente, cuatro pacientes recibieron prednisona, uno requirió prednisona y azatioprina y de un paciente no contamos con registros de tratamiento. Uno de nuestros pacientes evolucionó desfavorablemente con aparición y recidiva de lesiones de eritema nodoso severas y bilaterales que motivaron la necesidad de tratamiento permanente con esteroides e inmunosupresores. Si bien se trata de una enfermedad de baja prevalencia en nuestro país, deberíamos mantener un alto índice de sospecha ante la presentación de síntomas respiratorios con lesiones parenquimatosas y adenopatías mediastinales, con o sin manifestaciones extratorácicas.(AU)
Sarcoidosis is a disease of unknown etiology affecting most frequently young adults. Diagnosis is based on clinical. Radiological and histopathological features and presentation is frequently of constitutional and respiratory symptoms, hilar and mediastinal adenopathies, with or without parenquimal involvement. Confirmation is histopathological with demonstration of non-caseificating granulomas in affected organs, with negative cultures for mycobacterium and fungi. We describe the clinical, radiological and histopatbological findings in 8 patients. Mean age was 44 years (28-59) and half the patients were male. Two patients were symptom-free at diagnosis, thoracic findings were present in five (cough, retroesternal pain and arrhytbmias) and one had extratboracic symptoms (ankle arthritis). All showed mediastinal adenopathies in radiological and tomographic images (six were in stage 2 and two in stage 1). 1\vo patients had spontaneous remission, four received oral steroids, one required oral steroids and azatbioprine and tbere was no treatment data on one patient. In one patient, recidiva of severe and bilateral erithema nodosum required long term treatment with steroids and immunosupressors. Although sarcoidosis is a low prevalent disease in our country, a high index of suspicion is advisable when facing patients with respiratory symptoms, parenquimal involvement and mediastinal adenopathies, with or without extrathoracic manifestations.(AU)
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Sarcoidose Pulmonar/patologia , Sarcoidose Pulmonar/diagnóstico por imagem , Sarcoidose Pulmonar/tratamento farmacológico , Fibrose Pulmonar/patologia , Fibrose Pulmonar/diagnóstico por imagem , Doenças do Mediastino/patologia , Doenças do Mediastino/diagnóstico por imagem , Granuloma/patologia , Granuloma/diagnóstico por imagem , Prognóstico , Prednisona/uso terapêutico , Glucocorticoides/uso terapêuticoRESUMO
La sarcoidosis es una enfermedad de etiología desconocida, que afecta con mayor frecuencia a adultos jóvenes. El diagnóstico es clínico, radiológico e histopatológico y la presentación frecuente es la de síntomas constitucionales y respiratorios, acompañados de linfoadenopatías hiliares y mediastinales, con o sin compromiso del parénquima pulmonar. La confirmación es anatomopatológica con la demostración de granulomas no caseificantes en los tejidos afectados, con cultivos negativos para micobacterias y hongos. Presentamos los hallazgos clínicos, radiológicos e histopatológicos en 8 pacientes. Cuatro eran de sexo masculino y la edad promedio fue de 44 años (28-59). Dos de los pacientes se encontraban asintomáticos al momento del diagnóstico, cinco de ellos se presentaron con hallazgos toráxicos (tos, dolor retroesternal y arritmias) y uno con síntomas extratoráxicos (artritis de ambos tobillos). Todos presentaron adenopatías mediastinales en los estudios radiológicos y tomográficos y seis de ellos se encontraban en el estadio 2. En cuanto al tratamiento, dos pacientes remitieron espontáneamente, cuatro pacientes recibieron prednisona, uno requirió prednisona y azatioprina y de un paciente no contamos con registros de tratamiento. Uno de nuestros pacientes evolucionó desfavorablemente con aparición y recidiva de lesiones de eritema nodoso severas y bilaterales que motivaron la necesidad de tratamiento permanente con esteroides e inmunosupresores. Si bien se trata de una enfermedad de baja prevalencia en nuestro país, deberíamos mantener un alto índice de sospecha ante la presentación de síntomas respiratorios con lesiones parenquimatosas y adenopatías mediastinales, con o sin manifestaciones extratorácicas.
Sarcoidosis is a disease of unknown etiology affecting most frequently young adults. Diagnosis is based on clinical. Radiological and histopathological features and presentation is frequently of constitutional and respiratory symptoms, hilar and mediastinal adenopathies, with or without parenquimal involvement. Confirmation is histopathological with demonstration of non-caseificating granulomas in affected organs, with negative cultures for mycobacterium and fungi. We describe the clinical, radiological and histopatbological findings in 8 patients. Mean age was 44 years (28-59) and half the patients were male. Two patients were symptom-free at diagnosis, thoracic findings were present in five (cough, retroesternal pain and arrhytbmias) and one had extratboracic symptoms (ankle arthritis). All showed mediastinal adenopathies in radiological and tomographic images (six were in stage 2 and two in stage 1). 1/vo patients had spontaneous remission, four received oral steroids, one required oral steroids and azatbioprine and tbere was no treatment data on one patient. In one patient, recidiva of severe and bilateral erithema nodosum required long term treatment with steroids and immunosupressors. Although sarcoidosis is a low prevalent disease in our country, a high index of suspicion is advisable when facing patients with respiratory symptoms, parenquimal involvement and mediastinal adenopathies, with or without extrathoracic manifestations.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Sarcoidose Pulmonar/patologia , Sarcoidose Pulmonar , Glucocorticoides/uso terapêutico , Granuloma/patologia , Granuloma , Doenças do Mediastino/patologia , Doenças do Mediastino , Prognóstico , Prednisona/uso terapêutico , Fibrose Pulmonar/patologia , Fibrose Pulmonar , Sarcoidose Pulmonar/tratamento farmacológicoRESUMO
OBJECTIVE: Histoplasmosis is a granulomatous fungal disease caused by Histoplasma capsulatum. The objective of the present paper was to describe the prevalence of oral histoplasmosis (OH) in two services from an endemic area in Argentina between 1991 and 2002 and to compare the clinicopathological profile of OH between HIV-positive and HIV-negative patients. METHODS: About 733 HIV+ (group A) and 14 260 patients (group B) were examined. Clinical diagnosis was confirmed by cytology, biopsy or culture. RESULTS: About 21 (3%) and 10 (0.07%) cases of OH were diagnosed in group A and B respectively. Most patients were male. A total of 90% of patients in group A were <45 years old whereas 70% of group B were more than 45 years old. Palate, gingiva and oropharynx were the most frequent locations. The importance of including histoplasmosis in the differential diagnosis of ulcerated oral lesions in immunocompromised patients was discussed.
Assuntos
Infecções Oportunistas Relacionadas com a AIDS/epidemiologia , Infecções por HIV/epidemiologia , Histoplasmose/epidemiologia , Doenças da Boca/epidemiologia , Adulto , Fatores Etários , Idoso , Argentina/epidemiologia , Diagnóstico Diferencial , Doenças Endêmicas , Feminino , Doenças da Gengiva/epidemiologia , Doenças da Gengiva/microbiologia , Soronegatividade para HIV , Humanos , Hospedeiro Imunocomprometido , Masculino , Pessoa de Meia-Idade , Doenças da Boca/microbiologia , Orofaringe/microbiologia , Palato/microbiologia , Doenças Faríngeas/epidemiologia , Doenças Faríngeas/microbiologia , Estudos Retrospectivos , Fatores SexuaisRESUMO
Massive orbital extension arising from a small posterior pole uveal melanoma is rare. A 75-year-old male followed for an asymptomatic choroidal nevus developed a two and a half month history of mild decrease in visual acuity. Upon clinical examination minimal enlargement of the tumor with surrounding serous retinal detachment was observed. Pre-operative magnetic resonance imaging disclosed massive extrascleral extension. Histopathology revealed that the tumor was composed of spindle B malignant melanoma cells. Multiple serial sections of the entire tumor area failed to demonstrate permeation through scleral lamellae. Instead, peripapillary perivascular short posterior ciliary vessel invasion was observed. This route of dissemination may explain the rapid growth and clinically undocumented behavior of small malignant melanomas at this location.
Assuntos
Neoplasias da Coroide/patologia , Melanoma/secundário , Neoplasias Orbitárias/secundário , Idoso , Neoplasias da Coroide/radioterapia , Neoplasias da Coroide/cirurgia , Enucleação Ocular , Humanos , Imageamento por Ressonância Magnética , Masculino , Melanoma/radioterapia , Melanoma/cirurgia , Invasividade Neoplásica , Órbita/diagnóstico por imagem , Órbita/patologia , Neoplasias Orbitárias/radioterapia , Neoplasias Orbitárias/cirurgia , Radiografia , Radioterapia Adjuvante , Resultado do TratamentoRESUMO
We report a woman with a history of allergies, polyuria, polydipsia, proteinuria, renal loss of electrolytes, renal tubular acidosis, nephrocalcinosis, and palpable purpura. A proximal defect was excluded by a normal bicarbonate reabsorption curve, and a distal tubular defect was shown because urine pH did not decrease to less than 6.4 despite ammonium chloride-induced systemic acidosis. Moreover, furosemide failed to improve urinary acidification. Urine-to-blood PCO(2) gradient was less than 14 mm Hg, although the urine bicarbonate level reached values as high as 89 mEq/L. Combining bicarbonate and neutral phosphate infusions increased the urine-to-blood PCO(2) gradient to only 20 mm Hg. These subnormal PCO(2) gradient values point to proton-pump dysfunction in the collecting tubule. Histological evidence of tubulointerstitial disease accompanied the tubular defects. The striking histological feature was the presence of immunoglobulin E (IgE) deposits in glomeruli, tubuli, and vessels. Concurrent with these findings, she had high serum IgE titers and CD23 levels. IgE antibodies from her serum were reactive against human renal tubuli, with binding to two regions that matched two different proteins present in cortex and medulla. One of these proteins corresponded to carbonic anhydrase II (31 kd); the second, to an unidentified protein that seems attached to cell membranes. We suggest that these IgE antibodies could have had a pathogenic role in this patient's glomerular, tubular, and small-vessel disease.
Assuntos
Acidose Tubular Renal/etiologia , Vasos Sanguíneos/metabolismo , Imunoglobulina E/metabolismo , Rim/metabolismo , Vasculite/etiologia , Acidose Tubular Renal/imunologia , Adulto , Feminino , Humanos , Imunoglobulina E/sangue , Vasculite/imunologiaRESUMO
PURPOSE: To investigate whether commonly used vasodilating drugs ameliorate angiogenesis in experimental retinopathy of prematurity (ROP), and to study the response of these drugs to different growth factors. METHODS: We used a rat and mouse model of oxygen-induced ischemic retinopathy. Animals were treated with nimodipine, gingko-biloba and dipyridamole intraperitoneally starting the day before exposure to room air (day 1). Controls were injected with vehicle solution only. Eyes were processed histopathologically with serial sections and neovascularization was measured by counting the nuclei within the retinal internal limiting membrane, by a masked observer. Retinal and vitreous tissues were assayed by ELISA for VEGF, PDGF and TGFbeta2. RESULTS: Nimodipine significantly inhibited the growth of new vessels in rats. The number of nuclei was 310 +/- 69 in the control group (n:14) and 121 +/- 53 in the treated ones (n:14), (p<0.0005). Similar results were found with ginkgo-biloba extracts: 344 +/- 53 (n:15) in controls, and 136 +/- 29 (n:11) in treated ones (p<0.0005), and with dipyridamole: 303 +/- 69 (n:13) in controls, and 131 +/- 48.5 in treated rats (p<0.0005). Results were similar in mice. 186 +/- 45 (n:7) nuclei counted in controls against 90 +/- 25 (n:6) for dipyridamole treated (p<0.0005); and 81 +/- 21 for ginkgo-biloba treated animals (p<0.0005). A gradual, very significant increase in VEGF values in response to relative hypoxia (room air) contrasted with the significant inhibition noted both with ginkgo-biloba extracts and dipyridamole. TGFbeta2 and PDGF both showed a gradual increase in relative hypoxia at days 2 and 4 of room air (p<0.0005). Treated animals showed marked inhibition of the three growth factors. CONCLUSIONS: All three drugs markedly inhibited angiogenesis in experimental ROP. Growth factors were elevated in hypoxic conditions. Treated animals showed significant decreases of PDGF, VEGF, and TGFbeta2 in retinal and vitreous tissues.
Assuntos
Dipiridamol/uso terapêutico , Ginkgo biloba , Substâncias de Crescimento/metabolismo , Nimodipina/uso terapêutico , Plantas Medicinais , Neovascularização Retiniana/prevenção & controle , Retinopatia da Prematuridade/prevenção & controle , Vasodilatadores/uso terapêutico , Animais , Animais Recém-Nascidos , Modelos Animais de Doenças , Fatores de Crescimento Endotelial/metabolismo , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Recém-Nascido , Linfocinas/metabolismo , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Extratos Vegetais/uso terapêutico , Fator de Crescimento Derivado de Plaquetas/metabolismo , Ratos , Ratos Sprague-Dawley , Neovascularização Retiniana/metabolismo , Neovascularização Retiniana/patologia , Vasos Retinianos/efeitos dos fármacos , Vasos Retinianos/patologia , Retinopatia da Prematuridade/metabolismo , Retinopatia da Prematuridade/patologia , Fator de Crescimento Transformador beta/metabolismo , Fator A de Crescimento do Endotélio Vascular , Fatores de Crescimento do Endotélio Vascular , Corpo Vítreo/metabolismoRESUMO
Bacillary angiomatosis (BA) is an infectious disease characterized by proliferative vascular lesions; it mainly affects HIV-positive patients. Multiple cutaneous nodular lesions together with fever, chills, malaise, anorexia, vomiting and headache are the most important clinical manifestations. It may also involve the heart, liver, spleen, bones, lung, muscles, lymph nodes, central nervous system and other organs. Erythromycin, 500 mg four times a day, is the drug of choice. The importance of this lesion lies in its clinical and histological similarity with other diseases. Cutaneous and oral lesions of BA clinically resemble Kaposi's sarcoma (KS). Histopathologically, BA may be confused with angiosarcoma, pyogenic granuloma and epithelioid hemangioma. We report two HIV-positive men with BA lesions in the oral mucosa. Diagnosis was confirmed by biopsy and Warthin-Starry silver staining.
Assuntos
Infecções Oportunistas Relacionadas com a AIDS/diagnóstico , Angiomatose Bacilar/diagnóstico , Doenças da Boca/microbiologia , Adulto , Angiomatose Bacilar/patologia , Antibacterianos , Biópsia , Corantes , Diagnóstico Diferencial , Eritromicina/uso terapêutico , Evolução Fatal , Granuloma Piogênico/patologia , Soropositividade para HIV , Hemangioma/patologia , Hemangiossarcoma/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Doenças da Boca/patologia , Neoplasias Bucais/diagnóstico , Neoplasias Bucais/patologia , Sarcoma de Kaposi/diagnóstico , PrataRESUMO
Se analiza el material de 10 pacientes tratados con IFN alfa leucocitario por vía peri e intralesional. Sus edades oscilan entre 21 y 38 años (promedio 26,6 años). En 3 casos el diagnóstico histopatológico fue de CIN II a III y en 7 casos fue de CIN III. La respuesta al tratamiento fue evaluada por colposcopia, citología e histología. Las parejas masculinas se controlaron con penoscopias. El tiempo promedio de seguimiento fue de 19,3 meses con un rango de 12 a 30 meses. Del total, nueve pacientes prestaron remisión completa histológica d esus displasias. Una paciente no respondió a la terapia. Al año del tratamiento ninguna de las pacientes que completaron este control presentaron recidivas. Las recurrencias histológicas observadas fueron hasta el momento a CIN I. Cuatro de las cinco pacientes con condilomas detectados en la penoscopia remitieron luego del tratamiento. En función de estos resultados se podría considerar el tratamiento con IFN alfa una opción terapÛutica válida (AU)
Assuntos
Adulto , Neoplasias do Colo do Útero/terapia , Interferon Tipo IRESUMO
Se analiza el material de 10 pacientes tratados con IFN alfa leucocitario por vía peri e intralesional. Sus edades oscilan entre 21 y 38 años (promedio 26,6 años). En 3 casos el diagnóstico histopatológico fue de CIN II a III y en 7 casos fue de CIN III. La respuesta al tratamiento fue evaluada por colposcopia, citología e histología. Las parejas masculinas se controlaron con penoscopias. El tiempo promedio de seguimiento fue de 19,3 meses con un rango de 12 a 30 meses. Del total, nueve pacientes prestaron remisión completa histológica d esus displasias. Una paciente no respondió a la terapia. Al año del tratamiento ninguna de las pacientes que completaron este control presentaron recidivas. Las recurrencias histológicas observadas fueron hasta el momento a CIN I. Cuatro de las cinco pacientes con condilomas detectados en la penoscopia remitieron luego del tratamiento. En función de estos resultados se podría considerar el tratamiento con IFN alfa una opción terapêutica válida
Assuntos
Adulto , Interferon Tipo I , Neoplasias do Colo do Útero/terapiaRESUMO
It is clear that CP is present in a higher or lower degree in different gastric-duodenum pathologies, especially in active superficial chronic gastritis, gastric ulcer and duodenum ulcer with gastric metaplasia. It is also found in atrophic chronic gastritis and, to a lesser extent, if it has intestinal metaplasia, as well as in some normal stomachs. It is not found in a histologically normal duodenum, nor in the oesophagus. As the fact that there was no publication on BE drew our attention, we set to make a retrospective research of CP of spinal metaplasia of distal oesophagus. Its presence proved to be high, 88% even in those cases with intestinal metaplasia and with ulcer of Barrett. We have used Gram coloration and Warthin Starry with Alcian-Blue and we have classified it within the degrees set by Marshall and Warren. We have also carried out a discussion on certain physiopathological facts, such as the presence of infiltrated PMN in all the cases, and its importance in keeping metaplasia, of ulcers of Barrett and its possible role in the development of adenocarcinoma.
Assuntos
Esôfago de Barrett/microbiologia , Campylobacter/isolamento & purificação , Doenças do Esôfago/microbiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Esôfago de Barrett/diagnóstico por imagem , Neoplasias Esofágicas/microbiologia , Esofagoscopia , Esôfago/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , RadiografiaRESUMO
It is clear that CP is present in a higher or lower degree in different gastric-duodenum pathologies, especially in active superficial chronic gastritis, gastric ulcer and duodenum ulcer with gastric metaplasia. It is also found in atrophic chronic gastritis and, to a lesser extent, if it has intestinal metaplasia, as well as in some normal stomachs. It is not found in a histologically normal duodenum, nor in the oesophagus. As the fact that there was no publication on BE drew our attention, we set to make a retrospective research of CP of spinal metaplasia of distal oesophagus. Its presence proved to be high, 88
even in those cases with intestinal metaplasia and with ulcer of Barrett. We have used Gram coloration and Warthin Starry with Alcian-Blue and we have classified it within the degrees set by Marshall and Warren. We have also carried out a discussion on certain physiopathological facts, such as the presence of infiltrated PMN in all the cases, and its importance in keeping metaplasia, of ulcers of Barrett and its possible role in the development of adenocarcinoma.
RESUMO
We present the patterns for the diagnosis, checking the clinical, radiological, endoscopical and histological data of 35 patients suffering from Barrett's Esophagus (BE) (columnar metaplasia lining the lower esophagus). The clinical characteristics are those of a severe esophagitis of long evolution, although metaplasia itself is asymptomatic, and its features depend on the inflammation degree. Radiology can bring out some data as GE reflux, hiatal hernia, ulcers or stricture, and perhaps double contrast may show any sign by means of which endobrachyesophagus (EBE) can be suspected. Endoscopy provides us with accurate data about EBE, ulcers, stricture and inflammation. Histology reveals the type of columnar metaplasia (junctional or cardial, gastric fundic, intestinal or specialized, or composite). Acquired or congenital etiology can be clarified by an immunohistochemical method, Peroxidase anti-Peroxidase (PAP), showing the presence of gastrin secretory cells (G cells) in the congenital cases.
Assuntos
Esôfago de Barrett/diagnóstico , Sistema Cromafim/patologia , Células Enterocromafins/patologia , Doenças do Esôfago/diagnóstico , Adulto , Idoso , Esôfago de Barrett/etiologia , Esôfago de Barrett/patologia , Esofagoscopia , Esôfago/diagnóstico por imagem , Esôfago/patologia , Feminino , Gastrinas/metabolismo , Humanos , Masculino , Pessoa de Meia-Idade , RadiografiaRESUMO
We present the patterns for the diagnosis, checking the clinical, radiological, endoscopical and histological data of 35 patients suffering from Barretts Esophagus (BE) (columnar metaplasia lining the lower esophagus). The clinical characteristics are those of a severe esophagitis of long evolution, although metaplasia itself is asymptomatic, and its features depend on the inflammation degree. Radiology can bring out some data as GE reflux, hiatal hernia, ulcers or stricture, and perhaps double contrast may show any sign by means of which endobrachyesophagus (EBE) can be suspected. Endoscopy provides us with accurate data about EBE, ulcers, stricture and inflammation. Histology reveals the type of columnar metaplasia (junctional or cardial, gastric fundic, intestinal or specialized, or composite). Acquired or congenital etiology can be clarified by an immunohistochemical method, Peroxidase anti-Peroxidase (PAP), showing the presence of gastrin secretory cells (G cells) in the congenital cases.