Peritoneal Sclerosis in a Patient on Long-term Continuous Ambulatory Peritoneal Dialysis (CAPD). : An Autopsy Case.

若年性ネフロン癆による慢性腎不全でCAPD (continuous ambulatory peritoneal dialysis)導入し, 6年6ヵ月後に死亡した20歳男性の1剖検例を報告した。CAPD導入数カ月後, 腹膜炎による除水能低下を起こしたが, 約5ヵ月後に回復した。CAPD導入1年5ヵ月以降重症な腹膜炎罹患により除水能低下状態が遷延したが, 次第に回復した。しかし, 体液貯留傾向のため, 3年2ヵ月後より高張透析液を使用し除水量の増加を得たが, 3年9ヵ月後に不可逆的な除水能低下状態となった。一方, クレアチニンの透析排液/血漿濃度比(D/P)から見た溶質除去能は, その約半年後まで保たれており, 血清クレアチニン値の上昇は軽度であった。剖検にて腹膜の線維性肥厚と高度の内腔狭窄を伴う動静脈硬化を認め, 腹膜硬化症と診断した。本例の腹膜硬化症は, 頻回の腹膜炎と高張透析液の使用が主な原因と考えられた。腹膜機能を長期に維持するためには, 腹膜炎の予防と高張透析液の使用を最小限にすることが重要と考えられた。A 20-year-old man, treated with continuous ambulatory peritoneal dialysis (CAPD) for 6.5 years because of-end-stage renal disease due to juvenile nephronophthysis, died of ultrafiltration failure, and the morphological examination of peritoneum was carried out at autopsy. Nine episodes of peritonitis developed, and ultrafiltration transiently decreased after each episodes. At 2 years after the start of CAPD, severe peritonitis occurred, and then his body weight and blood pressure gradually increased. At 4 years after the beginning of CAPD, when hyperosmotic dialysate was frequently used, ultrafiltration was irreversively deteriorated. On the other hand, creatinine dialysate/plasma ratio remained within normal limits for about several months, and the increase in the level of serum creatinine was very little. The peritoneum obtained at autopsy revealed marked fibrous thickening as well as the conspicuous luminal narrowing of arteries and veins due to intimal thickening. The development of peritoneal sclerosis seemed to be related with the frequency and severity of peritonitis and the use of hyperosmotic dialysate

Factors associated with initial ALA size larger than 69 mm.

<p>Multivariate analysis for 86 patients (missing data in 4 cases). ° : patients born and living in France who travelled to tropical areas °° : patients born in France living in an endemic region °°° : patients born in an endemic region and living in France who traveled back to their country to visit friends and relatives °°°° : patients born in an endemic region and living in France who did not return to their country since they immigrate* : White Blood Cells, ** C-Reactive Protein.</p

Main characteristics for 90 patients with amoebic liver abscess.

<p>° : patients born and living in France who travelled to tropical areas °° : patients born in France living in an endemic region °°° : patients born in an endemic region and living in France who traveled back to their country to visit friends and relatives °°°° : patients born in an endemic region and living in France who did not return to their country since they immigrated * : White Blood Cells, ** C-Reactive Protein.</p

Kaplan Meier curve evaluating disappearance of ALA.

<p>Kaplan Meier curve evaluating disappearance of ALA.</p

Flow chart.

<p>Flow chart.</p

Distinct immunofluorescence pattern on HEp-2000™ cells detected in the serum of patients with malaria.

<p><b>A</b> Nuclear speckled and cytoplasmic diffuse pattern with perinuclear enhancement detected by indirect immunofluoresence on HEp-2000™ cells (left) and on human neutrophils fixed with ethanol (top right) with the serum of a patient with HMS. Cytoplasmic diffuse pattern with perinuclear enhancement on neutrophils fixed with formalin. The nuclear speckled pattern is faded as observed with usual antinuclear antibodies (right middle). Negative control for ANCA testing (right bottom). <b>B</b> Common immunofluorescence patterns with cytoplasmic fluorescence that are diagnostic for SLE (anti-ribosome+anti-DNA), anti-synthetase syndrome (Anti-Jo1), necrotizing myopathies (anti-SRP) and primary biliary cirrhosis (anti-mitochondria) (left) compared with the distinct immunofluorescence pattern observed with malaria autoantibodies (right). <b>C</b> titers of autoantibodies with the distinct nucleo-cytoplasmasmic pattern in patients with HMS (n = 14) or non-HMS malaria (n = 22). Comparison performed using a U Mann-Whitney non parametric test. <b>D</b> Correlation of the titers of anti-malarial antibodies with the titers of autoantibodies with the distinct nucleo-cytoplasmic pattern. Correlation assessed using a Spearman non parametric test.</p

Nuclear speckled and cytoplasmic diffuse with perinuclear enhancement pattern is diagnostic for smoldering malaria.

<p>Occurrence or past history of malaria in patients with the distinct nucleo-cytoplasmic pattern was prospectively studied. The pattern was observed in 19 independent sera out of 10 400 ANA tests performed over a 12 month-period. For each patient, the occurrence of malaria was first searched in the medical clinical record file. If history of malaria was absent, serology, PCR or thin/thick blood smear were performed. Diagnosis for each case is indicated. (ND = not done).</p

Index case: Clinical and biological characteristics.

<p>Index case: Clinical and biological characteristics.</p

Patients with malaria: Clinical and biological characteristics.

<p>Patients with malaria: Clinical and biological characteristics.</p

Polyclonal autoantibodies of IgG isotype are responsible for the malaria related pattern.

<p><b>A</b> Detection by Western blotting of autoantibodies in the serum of malaria patients recognizing HEp-2 cells proteins. Each lane corresponds to an individual experiment with the serum obtained from indicated patient. Positive control are shown with anti-actin and anti-tubulin monoclonal antibodies and anti-Scl70 positive serum obtained from a patient with systemic sclerosis. <b>B</b> Comparison of the number of bands observed on Western blotting gels in non-HMS (n = 12) and HMS (n = 14) malaria. Comparison made using a non-parametric U Mann-Whitney test. <b>C</b> Determination of the targets of malarial autoantibodies. Targets of the IgGs isolated from three patients with HMS and from pooled sera of five patients with acute malaria were compared with the targets of IgGs from five healthy donors using a protein array (four experiments for malaria and five for healthy donors). Each of the 9 400 protein targets were plotted on a volcano plot. Targets with a fold change superior to eight with a statistical significance inferior to 0.01 that we considered as highly specific for malaria are shown in the chart. For each target, the cellular localization of the protein is indicated.</p