Management of the Hyperlipidemia of the Nephrotic Syndrome

Grundy, Scott M.; Vega, Gloria Lena

doi:10.1007/978-1-4613-1491-2_4

Cited by 3 publications

(4 citation statements)

References 51 publications

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“…However, even in children with severe hyperbetalipoproteinemia, typical lesions usually do not appear before adolescence. 3,11 The long period before the lesions occur highlights the rarity of the present case.…”

Section: Discussionmentioning

confidence: 65%

Unmasking of Childhood Hypothyroidism by Disseminated Xanthomas

Dötsch¹,

Zepf²,

Schellmoser³

et al. 2001

Pediatrics

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ABSTRACT. Secondary hyperlipidemia is a common laboratory finding in children with nephrotic syndrome, diabetes mellitus, and hypothyroidism. However, clinical signs of hyperlipidemia are extremely rare in childhood.We report on an 11-year-old girl who presented with a disseminated yellow papulomatous rash on the lower limbs and yellow skin creases on the palms of her hands. Blood tests yielded an opaque serum with a triglyceride concentration of 820 mg/dL and cholesterol of 1050 mg/ dL. Skin biopsy of one of the papules confirmed the diagnosis of xanthomas.Additional examinations revealed clinical (weight gain, diminished growth rate) and biochemical primary hypothyroidism (free T4: 0.4 ng/L [normal 8 -22 ng/L]; thyroid-stimulating hormone: >200 mU/L) as a consequence of Hashimoto thyroiditis (thyroid peroxidase and thyroglobulin: 4400 U/mL and >2000 U/mL, respectively; normal <60 U/mL). The patient was started on L-thyroxine, which led to a gradual decline of cholesterol and triglycerides to normal concentrations and a complete remission from the xanthomatous rash.For the first time, this case depicts disseminated xanthomas of the skin as the presenting complaint of severe hypothyroidism. Pediatrics 2001;108(5). URL: http:// www.pediatrics.org/cgi/content/full/108/5/e96; hyperlipidemia, hypothyroidism, xanthoma.ABBREVIATIONS. fT4, free T4; SDS, standard deviation score; TSH, thyroid-stimulating hormone.W ith an incidence of 1 in 500, hyperlipidemia is one of the commonest congenital disorders of metabolism. 1 Heterozygous type III hyperlipoproteinemia is clinically relevant, as it is frequently associated with the presence of xanthomas in adulthood. 2 However, although nonspecific skin lesions may occur in childhood type III hyperbetalipoproteinemia, xanthomas are extremely uncommon.Apart from inborn hyperlipidemia, secondary causes of elevated serum lipids have to be excluded. These diseases include the nephrotic syndrome, 3 diabetes mellitus, and hypothyroidism. 1 Particularly in childhood, however, secondary hyperlipidemia is rarely associated with clinical signs or symptoms. 4 We present a child with disseminated xanthomas and massively elevated serum lipid concentrations as a consequence of severe hypothyroidism. CASE REPORTAn 11-year-old girl presented to the pediatric endocrine outpatient department of the University of Erlangen with a disseminated yellow papulomatous rash on her lower limbs and yellow skin creases of the palms of her hands (Fig 1). The rash had been developing gradually over the past 2 years. On presentation, the patient was 137.5 cm tall (standard deviation score [SDS]: Ϫ2.4; Fig 2) and had a weight of 37 kg (50th percentile). Body mass index was 19.6 (SDS ϩ 1.8 according to 5). When the patient was 4 years old, her height had been at the 50th percentile (102 cm;

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Section: Discussionmentioning

confidence: 65%

Unmasking of Childhood Hypothyroidism by Disseminated Xanthomas

Dötsch¹,

Zepf²,

Schellmoser³

et al. 2001

Pediatrics

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“…6 The levels of total cholesterol, triglycerides, low-density lipoprotein (LDL), and very-lowdensity lipoprotein (VLDL) are increased, while high-density lipoprotein (HDL) levels are decreased. These abnormalities result from an increase in hepatic cholesterol and lipoprotein synthesis, a decrease in peripheral and hepatic catabolism of serum lipoproteins, and an increase in urinary excretion ofHDL.…”

Section: Manifestations Of Nephrotic Syndromementioning

confidence: 99%

“…Some patients respond to administration of a low dose of an angiotensinconverting enzyme inhibitor or calcium channel blocker with a reduction in proteinuria, although treatment with these agents has been demonstrated to be efficacious only in patients with diabetic nephropathy.~o Patients who have significant hyperlipidemia from chronic nephrotic syndrome are candidates for dietary manipulation and therapy with bile acid resin and lovastatin (Mevacor). 6 The latter agent should be used cautiously, since it has been associatcontinued ed with rhabdomyolysis in some nephrotic patients.…”

Section: Primary Nephrotic Syn-mentioning

confidence: 99%

Nephrotic syndrome in adults

Carome

Moore²

1992

Postgraduate Medicine

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The adult with nephrotic syndrome presents diagnostic and treatment challenges for the primary care physician. Early consultation with a nephrologist is advisable to assist in choosing between empirical therapy or renal biopsy to identify the specific causative lesion. Some patients respond to treatment of either the specific disorder or the underlying cause. All patients should be afforded specific therapy, where available, and nonspecific therapy to minimize the severity of the nephrosis and attenuate the incidence and severity of complications. Although the cause of most disorders resulting in nephrotic syndrome remains unclear, active research into the syndrome's pathogenesis and treatment options should prove fruitful.

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“…Μέρος της υγιεινής διατροφής που πρέπει να ακολουθείται είναι η χρήση πολυακόρεστων λιπαρών οξέων. Τελευταία συζητείται η πιθανή χορήγηση παραγόντων που μειώνουν τα Λίπη στην κυκλοφορία, όπως η χολεστυραμίνη (112)(113)(114)(115)(116)(117)(118)(119)(120)(121)(122)(123)(124)(125)194,195,215 Ως ολιγουρία ορίστηκε η αποβολή ούρων <200 ml/m 2 επιφάνειας σώματος/24ωρο, ενώ ως ανουρία η μηδενική αποβολή ούρων ή η αποβολή ολίγων ml το 8ωρο.…”

Section: δομη και συνθεση του πεπτιδιουunclassified

Το Κολπικο Νατριουρητικο Πεπτιδιο Στην Εναρξη Του Πρωτοδιαγνωστου Ιδιοπαθους Νεφρωσικου Συνδρομου Της Παιδικης Ηλικιας

Μεσσαριτακη¹

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Management of the Hyperlipidemia of the Nephrotic Syndrome

Cited by 3 publications

References 51 publications

Unmasking of Childhood Hypothyroidism by Disseminated Xanthomas

Unmasking of Childhood Hypothyroidism by Disseminated Xanthomas

Nephrotic syndrome in adults

Το Κολπικο Νατριουρητικο Πεπτιδιο Στην Εναρξη Του Πρωτοδιαγνωστου Ιδιοπαθους Νεφρωσικου Συνδρομου Της Παιδικης Ηλικιας

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