ABSTRACT
@#Doege-Potter syndrome (DPS) is a rare paraneoplastic condition characterized by hypoinsulinemic hypoglycemia from a solitary fibrous tumor. The underlying mechanism is the secretion of a prohormone form of insulin-like growth factor II (IGF-II) by the tumor, which causes decreased release of glucose into the circulation. We report the case of a 27-year-old Filipino male with presumptive DPS from a recurrent right temporo-zygomatic hemangiopericytoma (HPC). The complexity of DPS requires a multidisciplinary approach. Early screening for metastases from HPC may prevent the undesirable sequelae of the disease process.
Subject(s)
Hemangiopericytoma , HypoglycemiaABSTRACT
Sirenomelia or mermaid syndrome is a rare congenital anomaly characterized by variable degree of fusion of lower extremities. Awareness to this rare condition is important for prenatal diagnosis and prognosticating the fetus. The exact etiopathogenesis is still an area of research. Two pathogenic hypotheses are the vascular steal hypothesis and the defective blastogenesis hypothesis with exceptions reported in literature.
ABSTRACT
El Síndrome de "Doege - Potter" es una entidad clínica rara con incidencia baja de difícil diagnóstico, poco conocida alrededor del mundo descrita en 1930, el cual consiste en un tumor intratorácico asociado a hipoglicemi asintomática. El objetivo del presente artículo es exponer un caso de "Síndrome de Doege-Potter", diagnosticado y tratado en el Centro de Especialidades Clínico - Quirúrgicas Jesús Obrero, en un paciente masculino de 55 años; tras la revisión sintomatológica, semiológica/topográfica, estudios imagenologicos, anatomopatologicos, y la revisión sistemática de la literatura internacional se llegó al diagnóstico final. Además se enfocara no solo conocimientos históricos, etiológicos y fisiopatológicos, sino medios diagnósticos estandarizados, que implica tomar en cuenta en esta patología una vez diagnosticada, para poder direccionar el tratamiento más adecuado según los hallazgos y el estado del paciente; siendo esta una manera de aportar en el levantamiento epidemiológico y casuístico de esta variedad rara y poco frecuente patología torácica a nivel mundial.
"Doege - Potter" syndrome is a rare clinical entity with low incidence of difficult diagnosis, little-known around the world described in 1930, which consists of an intrathoracic tumor associated with symptomatic hypoglycemia. The aim of this article is to expose a case of "syndrome of Doege-Potter", diagnosed and treated in the Centre of specialties Clinical - surgical Jesús Obrero, in a 55-year-old male patient; after reviewing symptomatology, / topographic, semiological studies imaging, pathological, and the systematic review of the international literature became the final diagnosis. In addition, focuses not only etiological, historical knowledge and physiopathological, but standardized diagnostic means, which implies taking into account in this disease diagnosed once, to be able to address the most appropriate treatment according to the findings and the patient's condition; this being a way to bring in the epidemiological and case lifting of this rare variety and frequent short thoracic pathology worldwide.
Subject(s)
HypoglycemiaABSTRACT
Potter’s syndrome is a rare congenital disorder characterized by characteristic atypical appearance of a newborn due to the oligohydroamnios experienced during the intrauterine life. Its incidence varies from 1 in 2000 to 1 in 5000. 40 years old G7P4L1 A2 at 37 weeks of period of gestation was admitted at a tertiary care institute with labour pains. Her previous obstetric history included three full term vaginal deliveries, two of the babies expired in postnatal period of 2 months and 7 days age respectively. One was alive and healthy. In fourth pregnancy she underwent emergency LSCS, but baby expired 5 months after birth. She had history of 2 abortions which were spontaneous and not followed by curettage. She also gave history of intake of some drugs at 6th week of gestation prescribed by a local Baba to have a male child. Her ultrasonography revealed fetus with bilateral renal agenesis. On per abdomen examination, clinically liquor was very much reduced, Fetal heart sounds were not heard. She delivered vaginally a still born fetus with ambiguous genitalia, anal atresia and malformed limbs. The fetus had typical facial appearance of “potter facies”. Potter’s Syndrome or Sequence is a rare fatal disorder. Apart from typical Potter’s facies, pulmonary hypoplasia, skeletal deformities are associated with this condition, severity depends on the degree and duration of oligohydramnios. Therefore, it is very important to pick up this condition during antenatal ultrasound at earlier gestation so that timely decision regarding further workup and pregnancy termination can be undertaken. This case report emphasizes upon the importance of regular antenatal check ups and examination in each and every patient as it picks up the suspicious cases which can lead to further workup, definite diagnosis of the condition and timely decision regarding management.
ABSTRACT
Objective@#To summarize the clinical rehabilitation nursing practice of a patient with pleural giant solitary fibroma with Dege-Port syndrome.@*Methods@#A preoperative and postoperative rehabilitation nursing of a patient with low pulmonary function pleural giant solitary fibroma and non-islet cell hypoglycemia was analyzed and summarized.@*Results@#After psychological, hypoglycemia, and pulmonary rehabilitation, the patient was tolerant to surgery and recovered well after surgery. He was discharged from hospital on the 13th day after surgery.@*Conclusions@#For patients with difficult diagnosis, long-term pleural giant solitary fibroma with hypoglycemia and low lung function, psychological nursing, hypoglycemia symptomatic treatment, pulmonary rehabilitation to improve lung function, close observation and prevention of serious complications in patients It plays a vital role in the course of the disease and rehabilitation.
ABSTRACT
Objective To summarize the clinical rehabilitation nursing practice of a patient with pleural giant solitary fibroma with Dege-Port syndrome. Methods A preoperative and postoperative rehabilitation nursing of a patient with low pulmonary function pleural giant solitary fibroma and non-islet cell hypoglycemia was analyzed and summarized. Results After psychological, hypoglycemia, and pulmonary rehabilitation, the patient was tolerant to surgery and recovered well after surgery. He was discharged from hospital on the 13th day after surgery. Conclusions For patients with difficult diagnosis, long-term pleural giant solitary fibroma with hypoglycemia and low lung function, psychological nursing, hypoglycemia symptomatic treatment, pulmonary rehabilitation to improve lung function, close observation and prevention of serious complications in patients It plays a vital role in the course of the disease and rehabilitation.
ABSTRACT
Potter's syndrome including bilateral renal agenesis or polycystic renal disease, bilateral pulmonary hypoplasia and characteristic face was first described in 1946. Although a great number of cases of Potter's syndrome was reported, Potter's syndrome with adult polycystic kidney disease(Potter type III) was very rarely found. In this report, we described an autopsy case of Potter's syndrome having adult polycystic kidneys disease, bilateral pulmonary hypoplasia and characteristic face in conjunction with multiple hepatic cysts, features of congenital hepatic fibrosis and a pancreatic cyst. Microscopically, all cysts were lined by cuboidal epithelial cells, showing positive for epithelial membrane antigen and cytokeratins.