Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020639 (hypoproteinemia)
 1,134 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This study was aimed at defining the natural history of renal failure in obstructive jaundice due to cholangiocarcinoma, which is an important health problem in northeastern Thailand. Sixty-four patients among a total of 130 patients with obstructive jaundice secondary to cholangiocarcinoma who developed acute renal failure were studied retrospectively. Analysis was made with respect to clinical features, laboratory findings, and outcome. The development of renal failure before surgery was observed in all patients. It was nonoliguric in 80% and was associated with severe jaundice, gram-negative infection (42%), hypotension (31%), hypoproteinemia (30%), hyponatremia (56%), and hypokalemia (63%). The mean duration of renal failure was 2 weeks. All patients underwent surgery for the relief of jaundice. Seventy-seven percent of the patients survived and had recovery of renal function after the relief of jaundice. Twenty-three percent of the patients died of infection. Clinical data highlight the higher serum bilirubin levels and the frequent occurrence of hyponatremia, hypokalemia, and hypotension in renal failure. Their possible roles in contributing to the development of renal failure are discussed.
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PMID:Acute renal failure in obstructive jaundice in cholangiocarcinoma. 217 11

It's universally acknowledged that the mortality rate of postoperative patients with obstructive jaundice is higher than that of the non-icteric. Opinions, however, differ as to whether the mortality rate goes up with the worsening of jaundice. Some even regard hyperbilirubinemia as a contraindication in operation. The present article through a clinical research of 168 cases suggested that postoperative mortality registered no increase because of bilirubin level in plasma and the degree of severity of bilirubinemia was not a major factor affecting the postoperative prognosis. The 4 preoperative complications: hypoproteinemia, hepatocirrhosis, portal hypertension and acute cholangitis were responsible for postoperative prognosis, the conclusion is hyperbilirubinemia is not a contraindication in operation. A great portion of the patients should be given active operation to intercept persistent injury on liver and prevent the occurrence of acute cholangitis. To reduce the postoperative mortality, the treatment to be adopted is not to arrange biliary drainage before operation but to improve the patients general conditions by eliminating the complications mentioned above.
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PMID:A clinical study on the postoperative prognosis of 168 cases of surgical hyperbilirubinemia. 783 Oct 59

Neonatal intrahepatic cholestatic due to citrin deficiency (NICCD) is an autosomal recessive disorder caused by mutations in the SLC25A13 gene and characterized by neonatal/infantile-onset cholestatic hepatitis syndrome associated with conjugated hyperbilirubinemia and multiple aminoacidemias. We report the case of a Chinese female patient with NICCD disease who manifested prominent clinical features. The patient was diagnosed with NICCD based on cholestasis, aminoacidemia, and hypoproteinemia. She exhibited extreme aminoacidemia, coagulation disorders and untypical myocardial damage, which are rare in other NICCD patients genetically confirmed by us. This myocardial damage observed in obstructive jaundice could be caused by both hyperbilirubinemia and redundant blood bile acids. Screening the SLC25A13 gene revealed that this patient was compound heterozygous harboring two novel mutations, the c. 640C>T (p. Gln214X) in exon 7 and the c. 1709_1710insA (p. Ile570fs573X) in exon 16. Both mutations cause a premature stop codon and thereby truncated peptide or nonsense-mediated with loss of natural function accordingly. In conclusion, extremely manifested clinical features, including significant hyperbilirubinemia, multiple aminoacidemia, hypoproteinemia, coagulation disorders, and myocardial damage related to redundant blood bilirubin and bile acids, were observed in a NICCD patients with two novel mutations.
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PMID:Novel mutations in the SLC25A13 gene in a patient with NICCD and severe manifestations. 2538 44

The Clavien-Dindo (C-D) classification is a simple and feasible grading system of postoperative complications. The aim of the present study was to apply this system to retrospectively classify all types of post-pancreaticoduodenectomy (PD) complications (PPCs) and to systematically identify associated risk factors. Between January 2009 and December 2014, the C-D classification was applied to retrospectively classify PPCs for 1,056 patients who had undergone PD at the West China Hospital. Univariate and multivariate analyses were performed to link perioperative parameters and mortality with the severity of PPCs, which were subdivided into overall PPCs (Grade I-V), severe PPCs (Grade III-V) and mortality (Grade V). The number of patients with Clavien-Dindo grade I, II, IIIa, IIIb, IVa, IVb and V complications was 185 (17.5%), 128 (12.1%), 50 (4.7%), 25 (2.4%), 35 (3.3%), 19 (1.8%) and 33 (3.1%), respectively. A total of 475 (45.0%) patients experienced overall PPCs; 168 (15.9%) patients experienced severe PPCs; and 33 patients succumbed to mortality following PD. The following risk factors were identified following PD: Preoperative hypoproteinemia was correlated with all three subdivisions; obstructive jaundice was associated with severe PPCs and mortality; and older age was revealed to be an independent risk factor of mortality. A large retrospective study was performed in the present study and PD was correlated with a high occurrence of PPCs. The Clavien-Dindo system represents a broad applicable and feasible approach to evaluating PPCs in patients following PD. The independent risk factors of PPCs that were identified in the present study require further validation using the Clavien-Dindo classification in additional prospective studies.
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PMID:Use of Clavien-Dindo classification in evaluating complications following pancreaticoduodenectomy in 1,056 cases: A retrospective analysis from one single institution. 3000 96