Sleep-Related Breathing Disorders: When CPAP Is Not Enough

Three decades ago, continuous positive airway pressure (CPAP) was introduced to treat obstructive sleep apnea (OSA). Shortly after, bilevel positive airway pressure devices (BPAP) that independently adjusted inspiratory and expiratory positive airway pressure were developed to treat complex sleep-related breathing disorders unresponsive to CPAP. Based on the bilevel positive airway pressure platform (hardware) governed by propriety algorithms (software), advanced modes of noninvasive ventilation (NIV) were developed to address complex cardiorespiratory pathophysiology beyond OSA. This review summarizes key aspects of different bilevel PAP therapies (BPAP with/without backup rate, adaptive servoventilation, and volume-assured pressure support) to treat common sleep-related hypoventilation disorders, treatment-emergent central sleep apnea, and central sleep apnea syndromes.Supplementary InformationThe online version contains supplementary material available at 10.1007/s13311-020-00955-x.Key Words: Bilevel positive airway pressure (BPAP), Adaptive servoventilation (ASV), Volume-assured pressure support (VAPS), Sleep-related hypoventilation, Treatment-emergent central apnea, Central sleep apnea     

Rare causes of gastrointestinal hemorrhage in infancy

Gastrointestinal hemorrhage in children is a critical condition that demands quick and effective management. The differential diagnosis of gastrointestinal hemorrhage is wide. Heterotopic pancreas is a rare congenital anomaly and usually discovered incidentally. It is generally asymptomatic, but symptoms may occur when complicated by inflammation, bleeding, obstruction or malign transformation. Heterotopic pancreas may present throughout the gastrointestinal tract, but it is most commonly found in the stomach, duodenum and proximal jejunum. Juvenile polyps are common during childhood and present most often with painless rectal hemorrhage. They remain the most common colonic polyps in children. Colonoscopic polypectomy is the most effective procedure in the treatment of juvenile polyps. In this study, we describe rare causes of gastrointestinal system hemorrhage in infancy and discuss some diagnostic and therapeutic approaches.     

Hepatosplenic T-cell lymphoma: sinusal/sinusoidal localization of malignant cells expressing the T-cell receptor gamma delta

Peripheral T-cell lymphomas consist of a clinically heterogeneous group of malignant disorders whose immunophenotype usually corresponds to that of normal mature T cells. We describe and correlate the clinical, histopathologic, phenotypic, and genotypic findings in two patients with malignant lymphoma presenting with hepatosplenic disease. The morphologic pattern of lymphoma was that of a sinusal/sinusoidal infiltration in spleen, marrow, and liver. This morphologic characteristic was associated with the presence of a productive clonal rearrangement of the T-cell receptor (TCR) delta gene. Lymphoma cells expressed a CD3-TCR-gamma delta- phenotype. They were also double negative (ie, CD4-CD8-) and lacked the CD5 and CD7 antigens. In one patient, tumor progression was associated with phenotypic changes that resulted in a CD3-TCR-gamma delta- phenotype with the same delta-gene rearrangement as initially. These observations suggest the existence of a new type of peripheral T-cell lymphoma characterized by its hepatosplenic presentation, and by the sinusal/sinusoidal tropism and the TCR-gamma delta phenotype of the malignant cells.     

Further specificity characterization of von Willebrand factor inhibitors developed in two patients with severe von Willebrand disease

Circulating inhibitors against von Willebrand factor (vWF) that show the properties of heterologous IgG antibodies have been described in a few patients with severe von Willebrand disease (vWD). The present study provides further characterization of inhibitors from two patients with severe vWD. Inhibitors in both, like polyclonal rabbit antibody, detected all sizes of multimers and the complex structure of each multimer from platelets and plasma of normal individuals as well as from plasma of patients with IIA, IIB, and IIC vWD. Both inhibitors and the rabbit antibody reacted mainly with the intact 225-Kd vWF subunit and the 189-H and 140-Kd fragments in contrast to monoclonal antibodies specific for vWF fragments that detected a higher relative proportion of 176-Kd fragment. Furthermore, all these antibodies recognized fragment III, although one inhibitor and rabbit polyclonal antibody reacted poorly and the other inhibitor did not react at all with reduced fragment II of vWF digested with Staphylococcus aureus V-8 protease. These data suggest that although human inhibitors from severe vWD patients may behave, to some extent, as polyclonal heterologous antibodies against native vWF, the former show striking differences in their target specificity as well as a much broader specificity than that described for human factor VIII inhibitors.     

Complications in children with ventricular assist devices: systematic review and meta-analyses

Heart Failure Reviews - Heart failure is a significant cause of mortality in children with cardiovascular diseases. Treatment of heart failure depends on patients’ symptoms, age, and severity...     

Evaluation of burn severity in vivo in a mouse model using spectroscopic optical coherence tomography

Clinical management of burn injuries depends upon an accurate assessment of the depth of the wound. Current diagnostic methods rely primarily on subjective visual inspection, which can produce variable results. In this study, spectroscopic optical coherence tomography was used to objectively evaluate burn injuries in vivo in a mouse model. Significant spectral differences were observed and correlated with the depth of the injury as determined by histopathology. The relevance of these results to clinical burn management in human tissues is discussed.OCIS codes: (170.6510) Spectroscopy, tissue diagnostics; (110.4500) Optical coherence tomography     

A rare case of multilocus brain abscess due to Entamoeba histolytica infection in a child

Brain abscess due to Entamoeba histolytica (E. histolytica) may pose a diagnostic problem or a therapeutic challenge, as evidenced by the paucity of papers reporting complete recovery after treatment. An 11-year-old girl presented with progressive drowsiness, diminished movements of the left upper limb, and swallowing problems. Cranial MRI showed multiple, contrast-dense masses with fluid content. She was started on meropenem. Surgical drainage was performed. No bacterial or fungal growth was observed in drainage samples. Entamoeba histolytica trophozoites were detected in the tissue sample. Intravenous metronidazole was started and continued for 6 weeks, at the end of which abscesses were found and to have shrunk considerably. Intravenous therapy was switched to oral metronidazole, which was continued for 2 weeks. She regained all her preexisting abilities. Multiple brain abscesses due to E. histolytica is a very rare occurrence, and histopathologic evaluation is important in diagnosis.Brain abscess, a rare form of Entamoeba histolytica (E. histolytica) infection, is characterized by an abrupt onset of symptoms and rapid progression and early death if untreated. Although many erroneously consider it as a disease of economically-developing countries, brain abscess due to E. histolytica may pose a diagnostic problem or a therapeutic challenge as evidenced by the paucity of papers reporting complete recovery after treatment. Extra-abdominal amebiasis, including cerebral amebic abscess, develops as a complication of hepatic amebiasis, rather than direct dissemination from the intestine. Cerebral amebic abscess disease has an incubation period starting from a few days to several months. As cerebral bacterial infections have a medical profile similar to that of protozoans (including partial muscular weakness, lethargy, and headache) differential diagnosis is difficult. The aim of the current study is to present a case of a brain abscess not associated with hepatic amebiasis.1-3 Multiple brain abscesses due to E. histolytica is a rarely seen occurrence. Serological and histopathological evaluations may prove to be very important in the diagnosis.