Atypical severe combined immunodeficiency caused by a novel homozygous mutation in Rag1 gene in a girl who presented with pyoderma gangrenosum: a case report and literature review.

Severe combined immunodeficiency (SCID) is a heterogeneous group of inherited defects involving the development of T- and/or B-lymphocytes. We report a female with atypical severe combined immunodeficiency caused by a novel homozygous mutation at cDNA position 2290 (c.2290C > T) in exon 2 of the RAG1 gene. The patient presented with bronchopneumonia, pyoderma gangrenosum (PG), pancytopenia and splenomegaly. She presented to us with pancytopenia and splenomegaly at the age of 11. Her condition was complicated by PG on left lower ankle at the age of 12. She experienced bronchopneumonia at the age of 15. She was diagnosed with RAG1 deficiency at the age of 16. Her immunological presentation included leucopenia and diminished number of B cells.

Pseudomonas biofilms, cystic fibrosis, and phage: a silver lining?

In contrast to usual laboratory conditions, most bacteria in the human body grow in biofilms. Encased in a structured matrix, many pathogens display heightened resistance to antibiotics. Pseudomonas aeruginosa lung infections in cystic fibrosis patients represent a prime example of the clinical challenges that antibiotic resistance in biofilms can represent. In the March 6, 2012 issue of mBio, Colin Hill and his colleagues report on experiments that add to the evidence that Pseudomonas phages are a potential treatment option for these infections.

Bacteriophages φMR299-2 and φNH-4 can eliminate Pseudomonas aeruginosa in the murine lung and on cystic fibrosis lung airway cells.

UNLABELLED: Pseudomonas aeruginosa is a common cause of infection in the lungs of patients with cystic fibrosis (CF). In addition, biofilm formation and antibiotic resistance of Pseudomonas are major problems that can complicate antibiotic therapy. We evaluated the efficacy of using bacteriophages to kill the pathogen in both biofilms and in the murine lung. We isolated and characterized two phages from a local wastewater treatment plant, a myovirus (φNH-4) and a podovirus (φMR299-2). Both phages were active against clinical isolates of P. aeruginosa. Together, the two phages killed all 9 clinical isolate strains tested, including both mucoid and nonmucoid strains. An equal mixture of the two phages was effective in killing P. aeruginosa NH57388A (mucoid) and P. aeruginosa MR299 (nonmucoid) strains when growing as a biofilm on a cystic fibrosis bronchial epithelial CFBE41o- cell line. Phage titers increased almost 100-fold over a 24-h period, confirming replication of the phage. Furthermore, the phage mix was also effective in killing the pathogen in murine lungs containing 1 × 10(7) to 2 × 10(7) P. aeruginosa. Pseudomonas was effectively cleared (reduced by a magnitude of at least 3 to 4 log units) from murine lungs in 6 h. Our study demonstrates the efficacy of these two phages in killing clinical Pseudomonas isolates in the murine lung or as a biofilm on a pulmonary cell line and supports the growing interest in using phage therapy for the control and treatment of multidrug-resistant Pseudomonas lung infections in CF patients. IMPORTANCE: Given the rise in antibiotic resistance, nonantibiotic therapies are required for the treatment of infection. This is particularly true for the treatment of Pseudomonas infection in patients with cystic fibrosis. We have identified two bacterial viruses (bacteriophages) that can kill Pseudomonas growing on human lung cells and in an animal model of lung infection. The use of bacteriophages is particularly appropriate because the killing agent can replicate on the target cell, generating fresh copies of the bacteriophage. Thus, in the presence of a target, the killing agent multiplies. By using two bacteriophages we can reduce the risk of resistant colonies developing at the site of infection. Bacteriophage therapy is an exciting field, and this study represents an important demonstration of efficacy in validated infection models.

[Treatment of congenital heart disease complicated by severe bronchopneumonia in infants].

OBJECTIVE: To study the treatment and the treatment outcome in infants with congenital heart disease complicated by severe pneumonia and heart failure. METHODS: The clinical data of 24 infants with congenital heart disease (left to right shunt) complicated by severe pneumonia and heart failure between January 2007 and December 2007 were retrospectively reviewed. RESULTS: Twenty-two infants recovered and 2 died. Severe pneumonia and heart failure were refractory even after 1-2 months medical treatment in 6 infants at ages of <6 months. They then underwent an open heart surgery under the mechanical ventilation and tracheal intubations and were successfully cured. The other 18 infants underwent a selective heart surgery after pneumonia and heart failure had been improved. Sixteen infants were successfully cured and 2 died of postoperative low cardiac output syndrome and diffuse intravascular clotting. CONCLUSIONS: The heart surgery should be performed early when the medical treatment does not work in infants with congenital heart disease complicated by severe pneumonia and heart failure. This may improve their outcome.

Algunas consideraciones sobre el tratamiento de la bronconeumonía bacteriana en ancianos hospitalizados / Some considerations on the treatment of the bacterial bronchopneumonia in hospitalized elderly

Se efectuó un estudio descriptivo y transversal en 112 ancianos y ancianas con diagnóstico de bronconeumonía bacteriana, ingresados en el Servicio de Medicina Interna del Hospital Provincial Docente Saturnino Lora de Santiago de Cuba durante el primer semestre del 2006, con vista a determinar la acción del tratamiento antimicrobiano seleccionado en la evolución de la enfermedad, para lo cual se revisaron las historias clínicas correspondientes. En la casuística predominaron el sexo femenino (58,0 por ciento), el grupo etario de 70 a 74 años y el criterio de selección del antibiótico empírico (61,7 por ciento); esto último fue generalmente la causa de una evolución desfavorable y una estadía hospitalaria prolongada en la mayoría de los pacientes.

A descriptive and cross-sectional study was carried out in 112 old men and old women with diagnosis of bacterial bronchopneumonia, admitted at the Service of Internal Medicine of Saturnino Lora Teaching Provincial Hospital of Santiago de Cuba during the first semester of 2006, aimed at determining the action of the antimicrobian treatment selected in the clinical course of the disease, for which the corresponding medical records were reviewed. In the case material prevailed the female sex (58,0 per cent), the age group of 70 to 74 years and the selection criterion of the empiric antibiotic (61,7 per cent); this last was generally the cause of an unfavorable clinical course and a prolonged hospital stay in most of the patients.

Resultados da técnica fisioterapêutica de aceleração de fluxo expiratório e seus efeitos em crinaças de até três anos de idade com broncopneumonia / Result of acceleration of expiratory flux physiotherapeutic thecnique and it effects on kids at tree years old for bronchopneumonia

A aceleração de fluxo expiratório(AFE) é uma técnica fisioterapêutica que visa a desobstrução brônquica, atuando sobre a dinâmica dos fluídos em vias áereas proximais e distais. Em razão de um grande número de crianças acometidas por broncopneumonias e, apresentando quadro de dispnéia provocado pela obstrução brônquica, foram estudados dezesseis crianças, com até três anos, internadas na enfermaria pediátrica do Hospital Universitário da UNOESTE, no período de janeiro à maio de 2001, sendo submetidas à técnica de aceleração de fluxo expiratório. Verificou-se através da freqüência respiratória a desobstrução brônquica ao final do tratamento fisioterapêutico e, após análise dos dados obtidos estatisticamente pelo teste de Tukey, evidenciou-se melhora na ventilação pulmonar com diminuição significativa da freqüência respiratória.

[Prevention and treatment of infectious complications after pulmonary resection--part I]. / Prevence a lécba zánetlivých komplikací po plicních resekcích--I. cást.

Pulmonary complications are still the most frequent cause of morbidity and mortality after lung resections. One of the most significant ones are infectious complications, particularly pneumonia. Pneumonia is very often caused by residual atelectasis. We evaluated a group of 75 patients after lung resection focused on occurrence of these two significant complications. We emphasize the prevention of these complications and draw attention to the bronchoscopy and "breathing reeducation" (physical therapy).

Concurrent Aelurostrongylus abstrusus infection and salmonellosis in a kitten.

A 14-week-old kitten had a history of vomiting, diarrhoea and pyrexia, all of which resolved without treatment. Three weeks later the kitten developed a violent non-productive dry cough. Thoracic radiographs revealed pneumothorax and nodular alveolar disease. Aelurostrongylus abstrusus larvae and intracellular Gram-negative bacilli were seen in bronchial wash fluid and pleural exudate, and Salmonella Typhimurium was cultured from both fluids but not from faeces. Therapy included unilateral closed-tube thoracostomy, enrofloxacin and fenbendazole. Historical signs were compatible with gastrointestinal salmonellosis and secondary broncho-pneumonia. Seeding of the lungs with salmonellae may have occurred as a result of migration of A abstrusus from a gastro-intestinal tract residually infected or colonised by S Typhimurium. Alternatively, the development of lungworm infection in the cat may have activated quiescent S Typhimurium pulmonary granulomata from bacteraemia secondary to gastro-intestinal salmonellosis. Two years after diagnosis the cat was reportedly in good health.

Alterations in peripheral blood leukocytes functions during enzootic bronchopneumonia of calves. Effect of treatment with antibiotics and immunomodulators.

Twelve calves from over veal calf farm were divided into two groups: group I-6 calves which developed typical signs of enzootic bronchopneumonia and group II-6 calves with no symptoms of the disease. Both groups of calves were compared with respect to changes in several hematological parameters. Some functions of peripheral blood leukocytes as random migration, phagocytic index, percent of phagocytic cells and percent of NBT positive cells were also scored. In addition, changes in serum levels of interferon (IFN) and tumor necrosis factor (TNF) and the ability of peripheral blood leukocytes (PBL) to produce IFN and TNF were quantitated by biological methods. On the day of diagnosis, in group I of calves a significant increase in the total serum protein concentration, hemoglobin content, red and white blood cells counts in comparison to control calves (group II) was observed. The increased number of NBT positive neutrophils and moderate levels of serum IFN and TNF correlated with elevated body temperature, breathing and heart rates. Calves with bronchopneumonia (group I) after diagnosis of the disease were treated with Tylbian (tylosine derivative), Flumetazon (glucocorticoid), Emulselvet (immunomodulator), bromhexinum and sulphonamides. Seven days after the beginning of treatment with medicaments a significant improvement in clinical symptoms was observed, however, the ability of PBL to cytokine production increased significantly 2 weeks after beginning of treatment and correlated with significant increase in random migration of neutrophils and their phagocytic activity, measured by the percent of phagocytic cells. Unexpectedly, in control calves (group II), not exhibiting any symptoms of bronchopneumonia at the beginning of experiment, high serum IFN titers were detected which decreased significantly during the first week of observation. In contrast to that the ability of PBL of control calves to produce IFN increased significantly within 3 weeks of observation. The correlations between the ability of PBL to produce cytokine and the development of clinical symptoms of bronchopneumonia are discussed.

Hartmannella vermiformis isolated from the cerebrospinal fluid of a young male patient with meningoencephalitis and bronchopneumonia.

Meningoencephalitis and bronchopneumonia were documented in a patient from Peubla, Mexico. The patient began with symptoms and signs of a common flu and 12 days after the onset of his disease he was admitted to the hospital presenting symptoms and signs of meningoencephalitis. The clinical course evolved into an endocraneal hypertension syndrome with bronchopneumonia, coma and death. Wide-spectrum antibiotics, immunosuppressive and anti-tuberculosis therapy were unsuccessfully administered. Important antecedents were degree I malnutrition and repeated contact with polluted water. Post-mortem autopsy was not performed. Gram-positive cocci were isolated from the spinal fluid 2 days after admission, and then active amebae were isolated from three different samples of the spinal fluid at days 16, 18 and 19 after admission. Such samples were concentrated and inoculated onto specific culture media. Identification of amebae was based on their morphology and biochemistry. All amebae were Hartmannella vermiformis. Amebae were apparently not the cause of the disease and might be considered as an opportunistic colonizer which may have caused the evolution of the disease to become worse.

Penetration of ciprofloxacin into bronchial secretions from mechanically ventilated patients with nosocomial bronchopneumonia.

The aim of the study was to evaluate the penetration of ciprofloxacin into bronchial secretions from mechanically ventilated patients with nosocomial bronchopneumonia. For this purpose, in each patient studied, simultaneous serial blood and bronchial secretion samples were obtained over a 12-h period on days 2 and 4. Eight patients were included in the study. Ciprofloxacin was given at a dose of 200 mg over 30 min by using an automatic pump. Ciprofloxacin was measured by high-performance liquid chromatography. Peak levels of drug in serum were 2.95 +/- 1 mg/liter on day 2 and 2.43 +/- 0.7 mg/liter on day 4. Peak and trough levels in bronchial secretions were 0.95 +/- 0.51 and 0.21 +/- 0.12 mg/liter, respectively, on day 2 and 0.76 +/- 0.17 and 0.18 +/- 0.14 mg/liter, respectively, on day 4. The ratios of peak concentrations in bronchial secretions/serum were 0.32 +/- 0.11 and 0.33 +/- 0.06 on days 2 and 4, respectively. The ratios of the area under the concentration-time curve from 0 to 12 h (AUC0-12) for bronchial secretions/those for serum were 0.66 +/- 0.40 and 0.55 +/- 0.30 on days 2 and 4, respectively. A significant positive correlation was found on day 4 between the AUC0-12 for serum and the AUC0-12 for bronchial secretions. No significant correlations were found between peak values in serum and bronchial secretions.

El lactante broncobstruído / The infant with bronchial obstruction

Se analizan las historias de 50 pacientes que ingresaron al Servicio de Atención Cardio Respiratoria (SACR) en el lapso comprendido entre enero de 1987 y setiembre de 1990, con diagnóstico semiológico de síndrome canalicular obstructivo.Las edades de los pacientes estuvieron comprendidas entre 1 mes y 1 año con una distribución homogénea para los intervalos de edad.El 75 por ciento de toda la población estudiada fue del sexo masculino.El 42 por ciento de los pacientes tenían antecedentes familiares de asma.La mayoría de los pacientes, 52 por ciento (N=26)no tenían patología respiratoria previa.La mayoría de los ingresos (N=31) fueron enviados de otros centros de atención pediátrica, siendo el resto ingresados por consulta directa en SACR.El 58 por ciento de los pacientes ingresaron directamente en CTI.La mayoría de los pacientes no habían recibido tratamiento broncodilatador previo (N=34).Presentaron hipoxemia-hipercapnia o acidosis mixta, 15 pacientes, de los cuales sólo fueron ventilados 9 (18 por ciento ).Se comprobó foco de condensación clínico o radiológico en el 46 por ciento (N=23) de pacientes y en el 26 por ciento (N=13) se evidenció infección bacteriana por cultivos o índices hematológicos.Fueron tratados con broncodilatadores el 80 por ciento de los pacientes (N=40): solo con Beta 2 estimulantes el 38 por ciento (N=11), con la combinación de beta 2 y xantinas 34 por ciento (N=17).Los corticoides se emplearon sólo en 9 pacientes

Simultaneous independent lung ventilation in pediatric patients.

Current methods of ventilation do not allow adequate ventilation of the affected lungs in the presence of unilateral disease, e.g., unilateral atelectasis, diaphragmatic hernia, or lobar emphysema. Using a bilumen endotracheal tube and two independent ventilators, synchronized simultaneous independent lung ventilation (SILV) can be achieved. This technique provides a method of treating unilobar, unilateral, or multifocal lung disease effectively. This article describes the author's methodology and clinical experience with SILV.

Sarampión y enfisema subcutáneo: presentación de tres casos / Measles and subcutaneous emphysema: a three case study

Se presentaron tres casos de sarampión complicados con bronconeumonía y enfisema subcutáneo, en niños de una familia campesina, no vacunados contra el sarampión. En seis meses se trataron 183 casos, de los cuales sólo tres presentaron enfisema subcutáneo, haciendo una incidencia de 1.6%; además se fundamentó en ellos la presencia de bronconeumonía, siendo relevantes los accesos de tos, lo cual sugiere que la fisiopatología de esta complicación se explica por la teoría del gradiente de presión propuesta por Bloch en 1968. Los factores encontrados en estos pacientes demuestran un sarampión más agresivo y con mayor probabilidad de presentar complicaciones; sin embargo, el valor pronóstico de la severidad del cuadro sarampionoso ante la presencia del enfisema subcutáneo es limitado y el manejo debe encaminarse más que nada a tratar el proceso bronconeumónico agregado

Incidencia de bronconeumonia en pacientes desnutridos de III grado / Bronchopneumonia incidence in III grade malnutrition patients

Se estudio en forma retrospectiva a 150 pacientes con diagnostico de desnutricion de III grado asociado con bronconeumonia del Servicio de Medicina Interna del Hospital del Nino. Se seleccionaron 94 pacientes, 35 correspondieron a pacientes vivos y 59 a pacientes fallecidos, de estos se les practico a 27 estudios postmortem. Se concluyo que la bronconeumonia ocupa el mayor procentaje de complicaciones en pacientes con desnutricion de III grado. El mayor riesgo de la poblacion fueron pacientes menores de 1 ano, ademas de la importancia de la rehabilitacion esta tambien relacionado con la prontitud del manejo y la recuperacion nutricional, superacion economica y cultura de la poblacion