Intestinal Lymphangiectasia: a Protein-Losing Enteropathy with Hypogammaglobulinemia, Lymphocytopenia and Impaired Homograft Rejection*

Strober, Warren; Wochner, R. Dean; Carbone, Paul P.; Waldmann, Thomas A.

doi:10.1172/jci105656

Cited by 188 publications

(71 citation statements)

References 38 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The serum IgA concentration was normal or slightly elevated in this patient, with an increased synthetic rate obscuring the short IgA survival. The pattern of a reduced albumin and IgG concentration associated with a shortened survival of albumin, IgG, and IgA is comparable to that observed in patients with protein-losing enteropathy (15). Therefore, to determine whether excessive gastrointestinal protein loss was present in these patients, In accordance with these observations with 5'Cr-labeled albumin, both patients excreted < 0.3% of the radioiodinated albumin pool catabolized daily in the daily stool collections, a rate comparable to the proportion of the catabolized radioiodinated albumin excreted in the feces as radioiodine observed in nine control individuals (range 0.1-1.0%) and markedly lower than the mean value of 4.6% observed in 18 patients with protein-losing enteropathy due to intestinal disease (16).…”

Section: Resultssupporting

confidence: 75%

“…The pattern of protein metabolism observed in the present cases is much closer to that observed in patients with nephrotic syndrome or especially those with protein-losing enteropathy who develop hypogammaglobulinemia secondary to excessive loss of albumin and immunoglobulin (1,2,15). Both patients The patients in the present study have a third major pathophysiological mechanism resulting in hypoalbuminemia and hypogammaglobulinemia, that is, a disorder in the endogenous catabolic pathways for these serum proteins.…”

Section: Discussionsupporting

confidence: 80%

See 1 more Smart Citation

Familial hypercatabolic hypoproteinemia. A disorder of endogenous catabolism of albumin and immunoglobulin.

Waldmann¹,

Terry²

1990

J. Clin. Invest.

View full text Add to dashboard Cite

The metabolism of albumin and IgG was investigated in two siblings, products of a first-cousin marriage, a female aged 34 yr and a male aged 17, who had a marked reduction in their respective serum concentrations of IgG (1.3 and 3.1 mg/ml) and albumin (19 and 21 mg/ml). The metabolism of radioiodinated IgG and albumin was studied in the two patients. The total circulating and body pools of IgG were < 28% of normal. The IgG synthetic rates were within the normal range. However, the IgG survival was short, with their respective fractional catabolic rates increased fivefold to 31% and 36% of the intravenous pool per day (normaL 6.7±2%/d). Furthermore, the patients had reduced total body pools, normal synthetic rates, and increased fractional catabolic rates for albumin. There was no proteinuria or abnormality of renal or liver function. In addition, the patients did not have circulating antibodies directed toward IgG, IgA, or albumin. Furthermore, both patients had normal fecal 51Cr-labeled albumin tests, thus excluding excessive gastrointestinal protein loss. We propose that these siblings have a previously unrecognized familial disorder characterized by reduced serum concentrations of IgG and albumin caused by a defect in endogenous catabolism, leading to a short survival ofthese proteins that is associated in this family with chemical diabetes and a skeletal deformity. (J. Clin.

show abstract

Section: Resultssupporting

confidence: 75%

Section: Discussionsupporting

confidence: 80%

Familial hypercatabolic hypoproteinemia. A disorder of endogenous catabolism of albumin and immunoglobulin.

Waldmann¹,

Terry²

1990

J. Clin. Invest.

View full text Add to dashboard Cite

show abstract

“…IgG was isolated from normal serum by DEAE-cellulose chromatography as previously described (11). IgA was prepared by GeonmPevicon2 block electrophoresis (11) of sera from two patients with marked monoclonal elevations of serum IgA.…”

Section: Methodsmentioning

confidence: 99%

Hypercatabolism of IgG, IgA, IgM, and albumin in the Wiskott-Aldrich syndrome

Blaese¹,

Strober²,

Levy³

et al. 1971

J. Clin. Invest.

View full text Add to dashboard Cite

A B S T R A C T The Wiskott-Aldrich syndrome is an immune deficiency disorder with an impairment of both humoral and cellular immune responses. Metabolic turnover studies of IgG, IgA, IgM, and albumin were conducted in seven patients with the Wiskott-Aldrich syndrome using purified radioiodinated proteins. The survival of each of the proteins studied was significantly shortened with a half-time of 7.5 days for IgG (normal 22.9 ±4 SD), 3.0 days for IgA (normal 5.8 +1), 5.0 days for IgM (normal 10.1 ±2.1), and 8.6 days for albumin (normal 17, range [13][14][15][16][17][18][19][20]; the fractional catabolic rates were correspondingly elevated and the distribution of protein among the body compartments was normal. For three of the four proteins, IgG, IgA, and albumin, the steady-state synthetic rates were generally elevated leading to normal or even elevated serum proteins levels. Thus, in the case of IgA, the synthetic rate averaged five times normal while the fractional degradative rate was twice normal. The resulting serum concentration was, therefore, significantly elevated. IgM represented an exception to this pattern in that the increased rate of degradation was not counterbalanced by an increased synthetic rate and, therefore, the serum levels were low.Albumin clearance studies using albumin-"Cr showed gastrointestinal protein loss in these patients to be slightly greater than normal, but this could account for only a small fraction of the hypercatabolism observed. There was no proteinuria or abnormalities of thyroid, adrenal, renal, or liver function. Thus, none of the previously recognized causes of increased serum protein catabolism were present. Patients with the Wiskott-Aldrich syndrome, therefore, have a unique disorder of Part of this study appeared in abstract form (1).

show abstract

“…Nestes casos o doente tem perda importante de proteínas tanto para a luz intestinal, quanto para o interior da cavidade peritoneal, além de apresentar má absorção de gorduras 5 . A perda de proteínas determina hipoalbuminemia com todas as suas consequências, além da depressão das imunidades celular e humoral 9 , agravando ainda mais as condições do doente.…”

Section: Introductionunclassified

Paracoccidioidomicose subaguda progressiva: tratamento de um doente com anfotericina B e nutrição parenteral

Pereira

Barraviera

Marcondes

et al. 1985

Rev. Inst. Med. trop. S. Paulo

View full text Add to dashboard Cite

RESUMOOs Autores descrevem um caso de paracoccidioidomicose subaguda progressiva, com quadro clínico sugestivo de síndrome de má absorção, em que o doente não se beneficiara apenas com o tratamento antifúngico convencional. Ao se introduzir como medida auxiliar a nutrição parenteral houve evidente melhora clí-nica e laboratorial. Desta maneira os Autores propõe o uso associado da nutrição parenteral no tratamento de doentes com esta forma clínica de paracoccidioidomicose. INTRODUÇÃOA Paracoccidioidomicose da criança e do adolescente manifesta-se com freqüência pela forma denominada juvenil da doença 6 , que evolui com acometimento ganglionar predominante. A poliadenopatia aguda ou subaguda com localização preferencial nas cadeias linfáticas: cervical, sub-mandibular, axilar e mesentérica é comum na primeira e segunda décadas da vida. Nestes doentes os gânglios enfartados são pouco dolorosos, de tamanho variáveis e costumam fistulizar. Baço, fígado e medula óssea frequentemente são acometidos. O comprometimento do tubo digestivo, nesta forma de paracoccidioidomicose, é comum sendo o quadro clínico decorrente das alterações anatômicas ou apenas funcionais de alguma parte do intestino delgado ou dos segmentos eólicos l . Muitas vezes associam-se às alterações intestinais da doença, às do sistema linfático abdominal, acarretando quadro clínico semelhante ao encontrado em portadores de linfangiectasia intestinal 8 . Nestes casos o doente tem perda importante de proteínas tanto para a luz intestinal, quanto para o interior da cavidade peritoneal, além de apresentar má absorção de gorduras 5 . A perda de proteínas determina hipoalbuminemia com todas as suas consequências, além da depressão das imunidades celular e humoral 9 , agravando ainda mais as condições do doente.A denominação de paracoccidioidomicose subaguda progressiva, parece ser mais adequada para representar a forma juvenil com comprometimento linfático abdominal acompanhada de hipoalbuminemia e má absorção, por dar idéia da gravidade e do caráter evolutivo dessa forma de doença, ao invés de ater-se apenas ao grupo etário.A nutrição parenteral, à base de carboidratos, aminoácidos, vitaminas, gorduras e minerais é indicação importante para o tratamento de doentes debilitados, incapacitados para a nutrição oral e com defeitos de absorção in-(1) Auxiliar

show abstract

Intestinal Lymphangiectasia: a Protein-Losing Enteropathy with Hypogammaglobulinemia, Lymphocytopenia and Impaired Homograft Rejection*

Cited by 188 publications

References 38 publications

Familial hypercatabolic hypoproteinemia. A disorder of endogenous catabolism of albumin and immunoglobulin.

Familial hypercatabolic hypoproteinemia. A disorder of endogenous catabolism of albumin and immunoglobulin.

Hypercatabolism of IgG, IgA, IgM, and albumin in the Wiskott-Aldrich syndrome

Paracoccidioidomicose subaguda progressiva: tratamento de um doente com anfotericina B e nutrição parenteral

Contact Info

Product

Resources

About