Metabolic studies and autonomic function in children with partial lipodystrophy

West, R; Fosbrooke, Audrey S.; Lloyd, June K.

doi:10.1136/adc.49.8.627

Cited by 16 publications

(11 citation statements)

References 22 publications

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“…In our case no metabolic abnormalities were found, although metabolic disorders, particularly hyperinsulinemia, have been re ported in children with partial lipodystro phy [8],…”

Section: Discussioncontrasting

confidence: 40%

Lipoatrophia semicircularis

Ayala¹,

Lembo²,

Ruggiero³

et al. 1985

Dermatology

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“…In our case no metabolic abnormalities were found, although metabolic disorders, particularly hyperinsulinemia, have been re ported in children with partial lipodystro phy [8],…”

Section: Discussioncontrasting

confidence: 40%

Lipoatrophia semicircularis

Ayala¹,

Lembo²,

Ruggiero³

et al. 1985

Dermatology

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“…She had normal serum complement 3 levels. Reduced skinfold thickness from the upper half of body has been reported previously 13,158,185 .…”

Section: Body Fat Distributionmentioning

confidence: 61%

Clinical Features and Metabolic and Autoimmune Derangements in Acquired Partial Lipodystrophy

Misra

Peethambaram

Garg³

2004

Medicine

249

252

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We describe clinical features, body fat distribution, and prevalence of metabolic abnormalities in 35 patients with acquired partial lipodystrophy (APL) seen by us over 8 years, and also review 220 cases of APL described in the literature. Based on the review and our experience, we propose that the essential diagnostic criterion for APL is the gradual onset of bilaterally symmetrical loss of subcutaneous fat from the face, neck, upper extremities, thorax, and abdomen, in the "cephalocaudal" sequence, sparing the lower extremities. Analysis of the pooled data revealed that female patients were affected approximately 4 times more often than males. The median age of the onset of lipodystrophy was 7 years. Several autoimmune diseases, in particular systemic lupus erythematosus and dermatomyositis, were associated with APL. The prevalence rates of diabetes mellitus and impaired glucose tolerance were 6.7% and 8.9%, respectively. Approximately 83% of APL patients had low complement (C) 3 levels and the presence of polyclonal immunoglobulin C3 nephritic factor. Twenty-two percent of patients developed membranoproliferative glomerulonephritis (MPGN) after a median of approximately 8 years following the onset of lipodystrophy. Compared with patients without renal disease, those with MPGN had earlier age of onset of lipodystrophy (12.6 +/- 10.3 yr vs 7.7 +/- 4.4 yr, respectively; p < 0.001) and a higher prevalence of C3 hypocomplementemia (78% vs 95%, respectively; p = 0.02). The pathogenesis of fat loss and MPGN in patients with APL remains unclear, but activation of an alternate complement pathway has been implicated. Treating the cosmetic disfigurement by surgical procedures has yielded inconsistent results. The use of thiazolidinediones to treat fat loss in patients with APL remains anecdotal. Prognosis is mainly determined by renal insufficiency due to MPGN.

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“…vonTaylor 1961in Senior 1964 14 Pat. von Boucher 1973, Peters 1973, Williams 1973, West 1974 in Sissons 1976 …”

mentioning

confidence: 99%

“…Piscatelli et al(35), Boucher et al(9a) und West et al(50) fanden bei einem, 5 bzw. 6 Patienten eine Insulinresistenz in Form einer Hyperinsulinämie bei normaler Glukosetoleranz.…”

unclassified

,,Lipodystrophia progressiva" (Barraquer-Simon-Syndrom): Differentialdiagnose und Klinik

Böcker¹,

Weitbrecht²,

Neundörfer³

1986

Fortschr Neurol Psychiatr

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"Progressive lipodystrophy" or "partial lipodystrophy of the cephalothoracic type" is a rare, acquired condition of unknown aetiology with onset in childhood and a complete loss of subcutaneous fat of face, neck, trunk and upper extremities. The disease is more common in females than males and causes a disfigurement of the face; that it cannot be regarded only as a harmless variation, is above all due to a typical concomitant disease, membranoproliferative glomerulonephritis with hypocomplementaemia. Laboratory investigations nearly always reveal a complement-activation by the "alternative pathway" with consumption and lack of complement-component C3, a finding which allows a clear distinction between partial lipodystrophy and congenital or acquired forms of total lipodystrophy ("Berardinelli-Seip-Syndrom", lipoatrophic diabetes) and other circumscript lipodystrophies.

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Metabolic studies and autonomic function in children with partial lipodystrophy

Cited by 16 publications

References 22 publications

Lipoatrophia semicircularis

Lipoatrophia semicircularis

Clinical Features and Metabolic and Autoimmune Derangements in Acquired Partial Lipodystrophy

,,Lipodystrophia progressiva" (Barraquer-Simon-Syndrom): Differentialdiagnose und Klinik

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