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Tracheal access, accidental deccannulation, peristomal granulation, stenosis and difficult weaning are the laryngologist’s dilemma, wherever tracheostomy has been resorted to, in the paediatric age group. These major problems necessitate a modification in the procedure of tracheostomy where ‘stay’ and ‘maturation’ sutures are utilized. The stay sutures facilitate a quick midline tracheotomy and the maturation ones minimise parastomal granulation and easy tracheal recannulation.
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En diciembre de 1943, el Dr. Antonio María Béguez César detalló en el Boletín de la Sociedad Cubana de Pediatría los aspectos clínicos y hematológicos de una rara afección que padecieron tres niños de una familia santiaguera, quienes fallecieron en sus primeros años de vida. No había informes sobre hallazgos similares en la bibliografía médica, por lo cual se consideró la primera descripción de una enfermedad denominada por él como neutropenia crónica maligna familiar con granulaciones atípicas de los leucocitos, que aún hoy suele divulgarse erróneamente como síndrome de Chediak-Higashi y no como síndrome de Béguez-Steinbrinck-Higashi. Esta enfermedad es una inmunodeficiencia primaria causada por mutaciones en el gen regulador de la función lisosomal, capaz de alterar la formación del fagolisosoma en el neutrófilo y determinar la presencia de gránulos secretores gigantes en su interior, asociadas a un predominio de infecciones recurrentes generadas por bacterias piógenas. Aquí se realiza un recuento histórico del descubrimiento de esta entidad y se actualiza su fisiopatología(AU)
On December 1943, Dr. Antonio María Béguez César detailed in the Journal of the Cuban Pediatric Society the clinical and hematologic aspects of a rare disorder suffered by three children from a family in the locality, who expired during the first years of their lives in Santiago de Cuba. At that moment there was no report about similar findings in the medical literature, therefore it is considered the first description of a disease denominated by him as familial malignant chronic neutropenia with atypical granulations of leucocytes, misleadingly revealed as Chediak-Higashi syndrome instead of Béguez-Steinbrinck-Higashi syndrome. This disease consists of a primary immunodeficiency induced by mutations in the regulator gen of the lysosomal function, which is able to alter the formation of phagolysosoma in the neutrophil and determine the presence of giant secretor granules associated with the predominance of recurrent infections provoked by pyogen bacteria. Here, a brief history of it's discovery as well as an updating of it's physiopathology are carried out(AU)
Assuntos
Humanos , Síndrome de Chediak-Higashi/história , EpônimosRESUMO
Objective To explore the value of delay alternating with nutation for tailored excitation (DANTE)- sampling perfection with application optimized contrast using different flip-angle evolution (SPACE) sequences in quantitative evaluation of arachnoid granules (AG). Methods Thirty patients with arachnoid granules confirmed by conventional imaging were enrolled. All patients underwent SPACE and DANTE-SPACE sequences scanning. Distribution characteristics of arachnoid particles in sinus cavities and cranial plate on SPACE and DANTE-SPACE sequence images were evaluated, and the measurements were performed and statistically analyzed, including maximum cross-sectional area, longest diameter, SNR and CNR. Results A total of 43 arachnoid particles were detected with SPACE sequence, 29 in the sinus cavities and 14 in the cranial plate, while 45 arachnoid particles were found with DANTE-SPACE, 31 in the sinus cavities and 14 in the cranial plate. SPACE and DANTE-SPACE sequences showed no statistically significant difference in distribution of arachnoid particles (χ2=0.155, P=0.997). In the sinus cavity, SPACE and DANTE-SPACE sequences showed significant differences of the maximum cross-sectional area ([32.55±17.08]mm2, [29.15±16.24]mm2), the longest diameter ([7.13±2.00]mm, [6.42±1.81]mm), SNR (88.82±29.57, 83.30±32.11) and CNR (45.99±28.57, 65.33±30.96) of arachnoid particles (all P0.05). Conclusion DANTE-SPACE can effectively inhibit slow blood flow artifacts in the sinus, and accurately display arachnoid particles.
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Cholesteatoma is a disease of the middle ear characterized by the presence of squamous epithelium in the middle earcleft. By secreting chemical enzymes, it causes manifestations by bone erosion which is dangerous to the surrounding structures which are intracranial and extra cranial. Due to advent of antibiotics and early diagnosis of ear disease, cholesteatoma is becoming infrequent. Previously commonly seen complications of cholesteatoma like mastoid abscess, automastoidectomy and sigmoid sinus abscess and granulations are rarely seen nowadays.A retrospective study ofcases of ear discharge with cholesteatoma presented to Government Medical College/Government General Hospital, Nizamabad, Telangana, during the period of May 2013 to June 2015 was done. The experience was being presented in this study.42 cases of cholesteatoma presenting with these features we re recorded. Chronicity of the disease with varied presentations seen rarely and the prevalence of the disease in poor socioeconomic families who had no access to medical facilities and ignorance of the severity of the disease is the main factor for seeing the rare presentations not seen in other centres. Inaccessibility to medical facilities and lack of expert treatment was the reason for patients seeking medical attention with conditions due to chronicity which are rarely seen nowadays.
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Aim To establish a method to evaluate lymphatic drainage of macromolecular tracer in cerebrospinal fluid in rats.Methods Rat cervical lymphatic blockade(CLB)models were established by occlusion of cervical lymphatic tubes and removal of cervical lymphatic nodes.The rats were divided into non CLB(normal controls) and CLB groups.~(125)I-labeled human serum albumin(~(125)I-HSA)was injected into the left lateral cerebral ventricle,and blood samples were collected and ~(125)I-HSA concentrations were detected continually within 24 hours.Concentration-time curve was drawn according to the single compartment model in pharmacokinetics.Parameters of pharmacokinetics such as area under curve(AUC),maximum concentration(C_(max)),transfer rate constant K_a and peak time(T_(max))were derived.The AUC,C_(max),K_a,and T_(max) regarding the lymphatic drainage of ~(125)I-HSA were calculated based on the differences between the two groups.Results AUC,C_(max),K_a of ~(125)I-HSA by lymphatic drainage were 51.97 mg·L~(-1)·h~(-1),2.91 mg·L~(-1),and 0.64 h~(-1),respectively.The proportion of AUC,C_(max),K_a of ~(125)I-HSA by lymphatic drainage to those of drained by both arachnoid granulations and lymphatics was 71.53%,44.02%,58.18%,respectively.T_(max) in CLB group(8.36±0.82 h)was much longer than that in non CLB group(3.57±0.54 h).Conclusions A method to evaluate lymphatic drainage of macromolecular tracer in cerebrospinal fluid in rats is successfully established.The lymphatic drainage pathway plays an important role in eliminating macromolecular substances in cerebrospinal fluid.
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Objective To further improve the morphological materials of AGs by micro-dissection, histology and CT, we observed the arachnoid granulations (AGs) in middle cranial fossa. Methods Thirty-three adult cadaveric heads were used for microsurgical dissection;Histological sections of AG specimens from 3 cadaver heads were examined. Forty patients who had both normal conventional brain CT and computed tomographic venography (CTV) were retrospectively reviewed. Results In middle cranial fossa the AGs occur in the following situations in order of frequency: the middle meningeal sinus, sphenoparietal sinus, lateral foramen rotundum and cavernous sinus. AGs usually show round, oval in shape and irregular in shape. AGs can be divided into individual type and leaflet type under light microscope. The numbers of AGs were observed by microanatomy and CTV were 8.72 and 3.52 respectively. The AGs of cavernous sinus was not localized precisely on CTV. Conclusion Study of the AGs in the middle cranial fossa systematically and comprehensively enriches anatomy and image knowledge. It is helpful in neurosurgical planning and choosing operalion procedure to avoid postoperative complications.
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Este trabalho visou investigar a associação da coexistência da presença de granulações tóxicas com resultados de hemocultura positivas, idade dos pacientes, condições de internamento e tipos de agentes bacterianos. Foi realizada análise retrospectiva e prospectiva, cega, para a presença de granulações tóxicas em amostras sangüíneas de trezentos pacientes, de ambos os sexos, internados em hospitais da Cidade de Belém - Pará, com solicitação de hemocultura, num período de dois anos. Com os hemogramas e as hemoculturas realizadas por métodos de automação, e todos os dados submetidos à metodologia de comparação estatística pelo Qui-quadrado (método de clump). Nossos resultados mostraram a existência de associação estatística entre: (1) a presença de granulações tóxicas e os resultados de hemoculturas positivas; (2) a menor idade dos pacientes (neonatos) associadas a hemocultura positiva; (3) a condição de internamento em UTI com hemocultura positiva; e (4) a presença de granulações tóxicas e a observação de leucocitose e desvio à esquerda, em pacientes internados em UTI, com hemoculturas positivas. E que os cinco principais agentes bacterianos identificados nas hemoculturas deste estudo foram Klebsiella oxytoca (22 por cento), Acinetobacter calcoaceticus (20 por cento), Escherichia coli (18 por cento), Enterobacter cloacae (14 por cento), e Pseudomonas aeruginosa (8 por cento).
This work aims at investigating the association of the presence of toxic granulation with positive blood cultures, age of patients, conditions of hospitalization and types of bacterial agents. Blind prospective and retrospective, analyses were carried out for the presence of toxic granulations-in blood samples of 300 patients of the both genders hospitalized in the City of Belém, Pará, Brazil. Request blood tests over a two year period were evaluated. The blood tests and cultures were performes using automated methods. All the data were statistically compared using the Qui-square test (clump method). The results show statistical associations between: (1) the presence of toxic granulations and positive blood cultures; (2) lower ages of patients (the newborn) and positive blood cultures; (3) hospitalization in the ICU and positive blood cultures and (4) toxic granulations-and the observation of leucocytosis and right-left shunts in patients hospitalized in the ICU with positive blood cultures. The commonest bacterial agents identified were klebsiella oxytoca (22 percent), Acinetobacter calcoaceticus (20 percent), Escherichia coli (18 percent), Enterobacter cloacae (14 percent), and Pseudomonas aeruginosa (8 percent).