Fibrillary glomerulopathy secondary to light chain deposition disease in a patient with monoclonal gammopathy

The pathologic manifestations of renal diseases related to monoclonal plasma cell dyscrasia include light chain deposition disease, the AL type of amyloidosis, and myeloma cast nephropathy. Light chain deposit disease (LCDD) is an uncommon condition in which monoclonal light chains are deposited in the glomeruli, tubules, and vessels causing varying degree of damage. We report a case of LCDD coincident with fibrillary glomerulonephropathy (FGN) in a 73-yr-old man with a diagnosis of monoclonal gammopathy of undetermined significance who presented with progressive renal insufficiency and mild proteinuria. The serum kappa light chain level was markedly raised. Immunofluorescent stains showed IgG along with C3 and kappa staining in glomeruli, but lambda staining was negative. Electron microscopic studies revealed diffuse punctuate-type deposits along the subendothelial areas. There were also scattered randomly oriented fibrils with a mean fibril thickness of 15-25 nm seen mainly in the glomerular mesangium, consistent with FGN. The congo red stain was negative on the histologic section. The present case illustrates that LCDD can progress to develop FGN in a patient with monoclonal gammopathy.     

Global Consortium Study of Neurological Dysfunction in COVID-19 (GCS-NeuroCOVID): Study Design and Rationale

Neurocritical Care - As the COVID-19 pandemic developed, reports of neurological dysfunctions spanning the central and peripheral nervous systems have emerged. The spectrum of acute neurological...     

Neurocritical Care Resource Utilization in Pandemics: A Statement by the Neurocritical Care Society

Neurocritical Care -     

Dexamethasone inhibits maturation and alters function of monocyte-derived dendritic cells from cord blood

Critically ill infants are treated with dexamethasone (Dx) and other glucocorticoids to reduce inflammation and to promote lung and cardiac function. The neonatal immune system is immature, so neonatal dendritic cells (DCs) might be especially sensitive to glucocorticoid-mediated immunosuppression. To test this, we compared Dx treatment of monocyte-derived DCs from cord (CB) and adult blood (AB). Dx decreased CD1a levels on both AB and CB DCs. CB-treated cells also exhibited decreased expression of CD83 and increased expression of CD14, alterations not observed in AB DCs. Characteristic immature endocytic activity was sustained and enhanced in Dx-treated CB DCs, whereas AB DCs matured normally. Maintenance of endocytosis corresponded with CD14 expression. Dx markedly increased CB DC IL-10, a T cell helper 2 (Th2)-preferential cytokine, while reducing IL-12, a counterbalancing Th1 cytokine. AB DCs were also affected, but increases in IL-10 and decreases in IL-12 were more modest. Dx treatment also inhibited DC-induced T cell proliferation, but CB DCs were inhibited more. In short, neonatal DCs seemed to be especially sensitive to the immunosuppressive effects of Dx as indicated by altered phenotype, endocytic function, ability to stimulate T cells, and cytokine shift favoring Th2. These alterations in DC function are consistent with an increased risk for certain infections and atopic diseases.     

二氧化硫对低氧性肺动脉高压大鼠肺动脉胶原堆积的影响

目的 探讨二氧化硫(SO2)对低氧性肺动脉高压大鼠肺动脉胶原堆积的影响.方法 将雄性Wistar大鼠34只分为对照组(8只)、低氧组(8只)、低氧加SO2组(腹腔注射亚硫酸钠/亚硫酸氢钠混合液0.5 mL/d)(9只)和低氧加天冬氨酸异羟肟酸(HDX)组[腹腔注射HDX 3.7 ms/(kg·d)](9只).对低氧组、低氧加SO2组和低氧加HDX组大鼠进行低氧处理21 d,氧体积分数为(100±5)mL/L,低氧处理6 k/d.采用羟脯氨酸水平测定及天狼猩红染色法分析低氧性肺动脉高压大鼠肺组织及肺动脉胶原的变化.采用SPSS 13.0软件进行统计学分析.结果 4组大鼠肺组织羟脯氨酸水平有显著性差异(F=12.363 Pa<0.01).与对照组比较,低氧组大鼠羟脯氨酸水平升高34.53%(P<0.01),随着低氧性肺动脉高压的形成,大鼠肺动脉中羟脯氨酸水平升高.与低氧组比较,低氧加SO2,组大鼠羟脯氨酸水平降低17.58%(P<0.05),低氧加HDX组大鼠羟脯氨酸水平升高21.30%(P<0.05).天狼猩红偏振光法分析显示,与对照组比较,低氧组大鼠血管壁胶原纤维明显增加;与低氧组比较,低氧加SO2组大鼠血管壁Ⅰ型胶原较少,呈红色或黄色粗纤维状,与Ⅲ型胶原伴行,低氧加HDX组大鼠血管壁胶原明显增加.结论 SO2能显著降低低氧大鼠的肺动脉胶原堆积.     

Nephrotic range proteinuria: rare manifestation of scleroderma renal crisis

The nephrotic range of proteinuria is uncommon in scleroderma renal crisis. This 46-yr-old woman with a medical history of scleroderma presented with very high blood pressure, a sudden elevation of serum creatinine, and proteinuria in the nephrotic range. Renal biopsy revealed onion-skin type of arterial changes with necrosis, confirming the presence of scleroderma nephropathy. Electron microscopy showed diffuse fusion of foot processes. Immunohistochemical staining (IHC) revealed increased expression in glomeruli of phosphorylated mammalian target of rapamycin (p-mTOR). These findings suggest that fusion of foot processes and activation of mammalian target of rapamycin-dependent pathways in podocytes are most likely responsible for the severe proteinuria in this patient with scleroderma nephropathy.     

A Callosal Catastrophe: Toxic Leukoencephalopathy Associated with Thermogenic Weight Loss Supplement Use

Background

The use of weight loss drugs and dietary supplements is common, but safety profiles for these drugs are largely unknown. Reports of toxicity have been published, and the use of these agents should be considered in clinical differential diagnoses.

Methods

We report the case of a patient with toxic leukoencephalopathy and hyponatremia associated with oral consumption of a thermogenic dietary supplement and essential oils.

Results

A 30-year-old woman presented after 2 days of headache, blurred vision, photophobia, vomiting, and hand spasms. She was taking a thermogenic dietary supplement daily for 6 months as well as a number of essential oils. Examination revealed mild right sided ataxia and diffuse hyperreflexia. Neuroimaging demonstrated bilaterally symmetric T2 hyperintensities of the corpus callosum and periventricular white matter. Approximately 18 h after admission she became unresponsive with brief extensor posturing and urinary incontinence. She partially recovered, but 1 h later became unresponsive with dilated nonreactive pupils and extensor posturing (central herniation syndrome). She was intubated, hyperventilated, and given hyperosmotic therapy. Emergent imaging showed diffuse cerebral edema. Intracranial pressure was elevated but normalized with treatment; she regained consciousness the following day. She was extubated one day later and discharged on hospital day 5. She was seen 2 months later with no further symptoms and a normal neurologic examination.

Conclusions

The pathophysiology of this patient’s hyponatremia and toxic leukoencephalopathy is unknown. However, physicians must be aware of the association between thermogenic dietary supplements and toxic leukoencephalopathy. Vigilance for life-threatening complications including hyponatremia and cerebral edema is critical.

     

Oral findings in a child with lipoid proteinosis: a case report and review

Lipoid proteinosis, a very rare autosomal recessive genodermatosis, results in hyaline material deposition in the skin and mucous membrane of various organs leading to multisystem involvement. A case report of a 12-year-old female child is presented here who showed classic features of the disease with generalized thickening, hardening, and scarring of the skin and vocal cord infiltration causing voice changes. The patient also had numerous oral mucosal and dental findings. The knowledge of the clinical features of the disease may help the oral health professional in rendering the appropriate treatment in order to improve the quality of life deteriorated by the disease.