Introduction Common variable immunodeficiency (CVID) comprises a heterogeneous group of disorders characterized by impaired antibody production. Kidney involvement in CVID is described in isolated and sporadic case reports. The objective of this study was to study the renal function pattern in CVID patients through glomerular and tubular function tests. Methods Study of 12 patients with CVID diagnosis and 12 healthy control individuals. Glomerular filtration rate (GFR), fractional excretion of sodium (FENa+) and potassium (FEK+), urinary concentration, and acidification capacity were measured. In addition, microalbuminuria and urinary monocyte chemoattractant protein-1 (MCP-1) were evaluated as markers of selectivity of the glomerular barrier and inflammation, respectively. Results In relation to glomerular markers, all CVID patients had normal GFR (>90 mL/min/1.73 m2), and microalbuminuria and urinary MCP-1 levels were also similar to those of controls. Interestingly, CVID patients had reduced urinary concentration capacity, as demonstrated by lower U/POsm ratio, when compared to controls. Also, while all control subjects achieved a urinary pH less than 5.3, no CVID patients showed a decrease in urinary pH to such levels in response to acid loading with CaCl2, characterizing impaired urinary acidification capacity. Conclusion Patients showed a trend towards an elevated prevalence of tubular dysfunction, mainly related to urinary acidification and concentration capacities.

A 22-year-old man with 2 years of malaise and diffuse muscular enlargement complained of slow movements. Clinical examination showed myxedematous facies, lentification of deep tendinous reflexes, and a mounding phenomenon (video on the Neurology ® Web site at Neurology.org). Laboratory examinations revealed hypothyroidism and elevated muscular enzymes, suggesting the diagnosis of myxedematous myopathy.This intriguing physical examination finding known as myoedema, occasionally associated with hypothyroidism, 1 is due to prolonged muscle contraction caused by delayed calcium reuptake by sarcoplasmic reticulum, following local calcium ion release brought out by percussion of biceps belly. 2 AUTHOR CONTRIBUTIONSGiovany Gomes Capistrano: drafting/revising the manuscript, analysis or interpretation of data, accepts responsibility for conduct of research and final approval, acquisition of data. Gabriela Studart Galdino: drafting/ revising the manuscript, accepts responsibility for conduct of research and final approval, acquisition of data. STUDY FUNDINGNo targeted funding reported. DISCLOSUREThe authors report no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures.

Objetivo: relatar um caso de linfangiomatose difusa. Métodos: relatamos o caso de um paciente masculino, 27 anos, com derrames cavitários de repetição e diagnóstico de linfangiomatose disseminada sugerido por exames de imagem, com acometimento torácico, esplênico, vesical e ósseo. Submetido ao tratamento com propranolol, alendronato, talidomida e bevacizumabe. Resultados: apesar do tratamento instituído, não houve resposta clínica e radiológica satisfatória, evoluindo com coagulação intravascular disseminada (CIVD) e óbito. Conclusão: A linfangiomatose difusa é uma doença de prognóstico ruim na maioria dos casos. A CIVD no caso relatado pode estar associada a complicações da doença.