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Kartagener's syndrome is a subset of primary ciliary dyskinesia, an autosomal recessive inherited disease, and is characterized by the triad of chronic sinusitis, bronchiectasis, and situs inversus. This paper reports the case of a 27-year-old female presenting with dyspnea on medium exertion, accompanied by chronic cough, non-productive or with clear expectoration. She had recurrent pneumonia until 15 years of age and underwent a lobectomy in the lower lobe of the left lung, probably due to bronchiectasis. Chest computed tomography showed situs inversus totalis, signs of previous surgical manipulation, and mild bronchial thickening. Computed tomography of the paranasal sinuses showed signs of chronic sinusitis due to a probable ciliary kinesis disorder. These finding suggest the diagnosis of Kartagener's syndrome. The prognosis reveals a slow rate of decline in lung function. However, repeated or chronic infections can negatively influence the quality of life of these patients.

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Situs inversus totalis is a rare recessive autosomal congenital abnormality in which the mediastinal and abdominal organs are in a mirrored position when compared to the usual topography. The literature reports some cases of situs inversus totalis and concomitant conditions: spinal abnormalities, cardiac malformations and hematological diseases, such as idiopathic thrombocytopenic purpura, which is an autoimmune disease that causes thrombocytopenia due to platelet destruction or suppression of its production. This article aimed to report the coexistence of situs inversus totalis and idiopathic thrombocytopenic purpura.

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ABSTRACT Situs inversus totalis is a rare recessive autosomal congenital abnormality in which the mediastinal and abdominal organs are in a mirrored position when compared to the usual topography. The literature reports some cases of situs inversus totalis and concomitant conditions: spinal abnormalities, cardiac malformations and hematological diseases, such as idiopathic thrombocytopenic purpura, which is an autoimmune disease that causes thrombocytopenia due to platelet destruction or suppression of its production. This article aimed to report the coexistence of situs inversus totalis and idiopathic thrombocytopenic purpura.

RESUMO Situs inversus totalis é uma anormalidade congênita autossômica recessiva rara em que os órgãos mediastinais e abdominais encontram-se em posição espelhada em relação à topografia habitual. A literatura relata alguns casos de concomitância do situs inversus totalis com outras condições: anomalias espinhais, malformações cardíacas e doenças hematológicas, como púrpura trombocitopênica idiopática, que é uma doença autoimune com plaquetopenia, devido à destruição dos trombócitos ou supressão da sua produção. Esse artigo teve o objetivo de relatar coexistência de situs inversus totalis e púrpura trombocitopênica idiopática.

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Dextrocardia with situs inversus is an uncommon anomaly affecting about 1 to 2 per 10,000 in the general population. This report describes an adult male patient with dextrocardia and in a Turkish subject. The photographic illustrations revealed transposition of some of the visceral organs such as the spleen was located right and the liver and gall bladder on the left. The heart was flattened and flipped to the right. Many people with situs inversus totalis are unaware of their unusual anatomy until they seek medical attention for an unrelated condition. So, early detection may lead to a successful surgical management and consequently offer a safer chance of survival. This report showed that dextrocardia and situs inversus can be seen amongst Turkish subjects.

La dextrocardia con situs inversus es una anomalía poco frecuente que afecta aproximadamente de 1 a 2 personas por 10.000 en la población general. Este informe describe un paciente masculino adulto con dextrocardia. Las figuras revelaron que la transposición de algunos de los órganos viscerales, como el bazo, se ubicada a la derecha y el hígado y la vesícula biliar a la izquierda. El corazón fue aplastado y girado hacia la derecha. Muchas personas con situs inversus totalis desconocen su anatomía inusual hasta que buscan atención médica por una afección no relacionada. Por lo tanto, la detección temprana puede llevar a un manejo quirúrgico exitoso y, en consecuencia, ofrecer una posibilidad más segura de supervivencia. Este informe mostró que la dextrocardia y el situs inversus se pueden encontrar entre los sujetos turcos.

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Background: Situs inversus (SI) is a rare congenital malformation characterized by the transposition of the viscera (thoracic and/or abdominal) to the opposite side of its normal topography, as a mirror image. In situs inversus totalis (SIT), all organs are inverted, in addition to the cardiac apex being directed to the right side of the body (dextrocardia). It may not present any clinical signs, and hence, it may be an accidental finding. Because of the rare occurrence of SIT, no epidemiological studies have been conducted in dogs. In 50% of the cases, it may be associated with primary ciliary dyskinesia (PCD), which causes respiratory clinical signs. SIT can be diagnosed using routine examinations such as ultrasonography, radiography, tomography, and echocardiography. The objective of this paper was to describe the radiographic and echocardiographic findings in a dog with SIT.Case: A 4-month-old Yorkshire Terrier bitch was admitted to the veterinary clinic, with a clinical complaint of cough. A physical examination showed that the dog’s general conditions were good; it had normal appetite and the mucous membranes had a normal color. Radiographic examination revealed changes in the topographic anatomy of the organs. The cardiac silhouette was inverted, adopting a “D” form; this suggested that the left-side chambers were positioned on the right side, and the right-side chambers were located in the left topographic region. The aortic arch was visible on the right side of the thorax. A diffuse non-structured pulmonary interstitial pattern was observed on latero-lateral projections, which could be associated with an inflammatory process. The fundic region of the stomach, spleen, and thymus were visible on the right side. The liver was on the left side of the patient’s abdomen. The stomach contained a foreign body of radiopaque nature that was considered a radiographic finding.[...]

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Background: Situs inversus (SI) is a rare congenital malformation characterized by the transposition of the viscera (thoracic and/or abdominal) to the opposite side of its normal topography, as a mirror image. In situs inversus totalis (SIT), all organs are inverted, in addition to the cardiac apex being directed to the right side of the body (dextrocardia). It may not present any clinical signs, and hence, it may be an accidental finding. Because of the rare occurrence of SIT, no epidemiological studies have been conducted in dogs. In 50% of the cases, it may be associated with primary ciliary dyskinesia (PCD), which causes respiratory clinical signs. SIT can be diagnosed using routine examinations such as ultrasonography, radiography, tomography, and echocardiography. The objective of this paper was to describe the radiographic and echocardiographic findings in a dog with SIT.Case: A 4-month-old Yorkshire Terrier bitch was admitted to the veterinary clinic, with a clinical complaint of cough. A physical examination showed that the dogs general conditions were good; it had normal appetite and the mucous membranes had a normal color. Radiographic examination revealed changes in the topographic anatomy of the organs. The cardiac silhouette was inverted, adopting a “D” form; this suggested that the left-side chambers were positioned on the right side, and the right-side chambers were located in the left topographic region. The aortic arch was visible on the right side of the thorax. A diffuse non-structured pulmonary interstitial pattern was observed on latero-lateral projections, which could be associated with an inflammatory process. The fundic region of the stomach, spleen, and thymus were visible on the right side. The liver was on the left side of the patients abdomen. The stomach contained a foreign body of radiopaque nature that was considered a radiographic finding.[...](AU)

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OBJECTIVE: Klippel-Feil syndrome is characterized by faulty segmentation of two or more cervical vertebrae and, in its most severe form, consists of massive cervical vertebral fusion, short neck, low posterior hairline, and limitation of head movement. Several cases associating Klippel-Feil syndrome with situs inversus totalis have been reported. In the present study, we describe the clinical features of a novel case of Klippel-Feil syndrome associated with situs inversus totalis and searched for mutations in GDF1, GDF3 and GDF6 genes, which were recently implicated in the development of skeletal and visceral anomalies. METHODS: A case of Klippel-Feil syndrome associated with situs inversus totalis underwent a full clinical examination including X-ray of cervical spine and thorax, abdominal ultrasound, and computerized tomography scanning of thorax and abdomen. PCR amplification and automated nucleotide sequencing of coding exons and intron-exon junctions of GDF1, GDF3, and GDF6 genes were performed in genomic DNA. RESULTS: No molecular alterations were found in GDF1, GDF3 and GDF6 genes in this patient. CONCLUSION: An additional patient associating Klippel-Feil syndrome and situs inversus totalis is reported. Mutations in GDF1, GDF3, and GDF6 genes were excluded as the cause of this unusual clinical association.

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El objetivo del presente reporte es dar a conocer un caso de situs inversus y colecistitis aguda diagnosticado y tratado en el Hospital Al Wahda Mabar Thamar de Yemen. Paciente de sexo femenino, de 50 años, con dolor en epigastrio después de la ingestión de alimentos grasos. Dos días antes de su ingreso incrementa su intensidad y se mantiene en hipocondrio izquierdo, y además, presenta vómitos. Utilizando antibioticoterapia perioperatoria, se realiza incisión subcostal izquierda, y se encuentra colecistitis aguda con dilatación moderada del colédoco. Se realiza colecistectomía y coledocotomía, con buena evolución posoperatoria(AU)

The objective of present paper is to present a case of situs inversus and cholecystitis diagnosed and treated in the Al Wahda Mabar Thamar Hospital of Yemen. A female patient aged 50 with epigastric pain after ingestion of fatty foods; two days before its admission increase its intensity and remains in left hypochondrium and also vomiting. With the use of perioperative antibiotic-therapy, a left subcostal incision is made detecting an acute cholecystitis with a choledochal slight dilatation. A cholecystectomy and choledochotomy with a good postoperative evolution were performed(AU)

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Primary ciliary dyskinesia (PCD), previously known as immotile cilia syndrome, is an autosomal recessive hereditary disease that includes various patterns of ciliary ultrastructural defects. The most serious form is Kartagener syndrome (KS), which accounts for 50% of all cases of PCD. The incidence of PCD ranges from 1:20,000 to 1:60,000. Since PCD causes deficiency or even stasis of the transport of secretions throughout the respiratory tract, it favors the growth of viruses and bacteria. As a result, patients have lifelong chronic and recurrent infections, typically suffering from bronchitis, pneumonia, hemoptysis, sinusitis, and infertility. Bronchiectasis and other chronic conditions infections can be the end result of the irreversible bronchial alterations, leading to chronic cor pulmonale and its consequences. Only half of the patients affected by PDC present all of the symptoms, a condition designated complete KS, compared with incomplete KS, typically defined as cases in which situs inversus does not occur. The diagnosis is made clinically and confirmed through transmission electron microscopy. Since there is no specific therapy for PCD, it is recommended that, upon diagnosis, secondary infections be treated with potent antibiotics and prophylactic interventions be implemented. In this paper, we report six cases of PCD (five cases of complete KS and one case of KS) and review the related literature, focusing on the diagnostic, therapeutic and clinical aspects of this disease.

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Abdominal situs inversus is a rare condition usually associated with malformations of asymmetric organs such as the heart, liver, spleen and malrotation of the intestines. A case of abdominal situs inversus with intestinal malrotation and preduodenal portal vein is reported. Patient underwent prophylactic Ladd's procedure and preduodenal portal vein was left undisturbed during surgery. This case highlights the importance of rigorous investigation of anatomic features prior to surgery in a patient with heterotaxia. The authors advocate radiological investigation of patients with heterotaxia and prophylactic Ladd's procedure in those with intestinal malrotation.

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The Bochdaleck's hernia is an anatomical defect of posterolateral region of the diaphragm that lets abdominal structures go inside the thorax. It has an incidence of 1 in 2000 to 5000 of newborn alive, without difference of sex. This defect is very common in the neonatal period and rare in adult age. We present a case of a male patient 32 years old, with the definitive diagnosis of Bochdalek hernia. The diagnosis was made by radiographic findings, with a few digestive symptoms. Confirmatory diagnosis was made by axial tomography computerized that found part of bowel in right hemithorax and pulmonary hypoplasy, with liver in situs solitus. The patient received medical treated only. The patient did not receive surgical treatment because it is supposed that this kind of treatment has more risks than benefits.

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To date, almost all patients reported with hypoplasia of the left heart have had usual atrial arrangement, with a small proportion known to have left or right isomerism. As far as I am aware, however, only one patient has previously been described with mirror-imaged arrangement of the heart and organs. In this report, I describe the second case.

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RESUMEN. Se presenta el caso clínico de una paciente femenina de 49 años, tosedora crónica, a quien se confirmó la presencia de bronquiectasias y situs inversus y que correspondó a un síndrome Kartagener. Este síndrome es un variante fenotípica del síndrome discinecia ciliar primaria o síndrome del ciclo inmóvil, uan enfermedad genética rara que causa inmovilidad ciliar primaria o síndrome del ciclo inmóvil, una enfermedad genética rara que causa inmovilidad cicliar. El curso clínico incluye manifestaciones secundarias al acúmulo de secreciones en vía aérea, bronquiectásias, sinusitis, otitis y esterilidad. Se revisa el tema de tos crónica, bronquiectasias y sindrome de Kartagener. Se discuten los hallazgos radiológicos encontrados

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Presentamos un caso de Síndrome de Kartagener en un individuo de sexo masculino, 41 años, soltero, trabajador rural, con una historia de más de 20 años de procesos rinosinusales y bronquiales de etiología infecciosa, secreciones nasales purulentas permanentes y espectoración de iguales características. Disnea de esfuerzo en progresión y episodios de exacerberación de la disnea con ruidos endobronquiales configurando una verdadera hiperreactividad bronquial. Los estudios revelaron bronquiectasias masivas en ambos pulmones, insuficiencia ventilatoria mixta, sinusitis, situs inversus total, disquinesia ciliar (test de sacarina) e inmovilidad espermática

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