Oral supplementation corrects plasma lysine concentrations in lysinuric protein intolerance

Lukkarinen, Minna; Näntö‐Salonen, Kirsti; Pulkki, Kari; Aalto, Maija; Simell, Olli

doi:10.1016/s0026-0495(03)00089-1

Cited by 26 publications

(15 citation statements)

References 17 publications

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“…However, oral lysine supplementation did not correct hypocarnitinemia in our patients. The lysine doses need to be individually titrated to avoid gastrointestinal side effects [25]. In addition, even the maximum tolerated doses of lysine may insufficiently improve carnitine synthesis, as protein-bound trimethyllysine rather than free lysine is the precursor for carnitine in its biosynthesis.…”

Section: Discussionmentioning

confidence: 99%

Carnitine deficiency and l-carnitine supplementation in lysinuric protein intolerance

et al. 2008

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Section: Discussionmentioning

confidence: 99%

Carnitine deficiency and l-carnitine supplementation in lysinuric protein intolerance

et al. 2008

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“…In the study performed by Lukkarinen et al [13], lowdose oral l-lysine-HCl (0.05 mmol/kg) at mealtimes together with citrulline for 3 days was able to normalize plasma lysine concentrations in 6 patients with LPI. The small dose was well tolerated and did not induce hyperammonemia.…”

Section: Introductionmentioning

confidence: 90%

Long-term oral lysine supplementation in lysinuric protein intolerance

et al. 2007

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“…15 It has been suggested that lysine supplementation could be able to ameliorate the clinical symptoms of LPI that are not corrected by citrulline. 27 In a child presenting HLH, a wide range of exams should be performed to rule out an infection, an autoimmune disease or malignancy, since most of these causes are treatable. If primary HLH is suspected, an underlying immune deficiency like FLH, GS, CHS, XLP, should be screened.…”

Section: Differential Diagnosis and Further Investigationsmentioning

confidence: 99%

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et al. 2017

Mediterr J Hematol Infect Dis

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Abstract. Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammationcaused by uncontrolled proliferation of activated lymphocytes and histiocytes. Often, HLH is an acquired syndrome. We report a case of a nine month-old-boy presented with hepatosplenomegaly, severe anemia, thrombocytopenia, hypertriglyceridemia and high hyperferritinemia. These clinical features of HLH prompted a wide range of infectious and autoimmune tests to be performed. After an extensive diagnostic workup, he was referred to the immune-hematologic unit for HLH suspicion with an unknown cause. Primary HLH due to familial lymphohistiocytosis (FLH) was first evoked in front of consanguinity, probable HLH in the family, early onset, and in the absence of a causative pathology like infection or cancer. However, functional tests were normal. Atypical features like the: absence of fever, hypotonia, recurrent diarrhea since diversification, hematuria, and proteinuria suggested an inborn metabolism error with gastrointestinal involvement. Specific tests were performed to reach a final diagnosis.

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Oral supplementation corrects plasma lysine concentrations in lysinuric protein intolerance

Cited by 26 publications

References 17 publications

Carnitine deficiency and l-carnitine supplementation in lysinuric protein intolerance

Carnitine deficiency and l-carnitine supplementation in lysinuric protein intolerance

Long-term oral lysine supplementation in lysinuric protein intolerance

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