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1.
J Fr Ophtalmol ; 45(9): 1021-1023, 2022 Nov.
Article in English | MEDLINE | ID: mdl-36137867

ABSTRACT

We report the case of an 85-year-old patient who developed trifascicular block with syncope, triggered by preoperative eye drops for cataract surgery. This life-threatening situation reopens the debate over the necessity of having an anesthesiologist present during cataract surgery.


Subject(s)
Cataract Extraction , Cataract , Humans , Aged, 80 and over , Mydriatics/adverse effects , Ophthalmic Solutions/adverse effects , Cataract Extraction/adverse effects , Cataract/complications , Cataract/diagnosis , Syncope/diagnosis , Syncope/etiology
2.
Orphanet J Rare Dis ; 17(Suppl 1): 170, 2022 04 19.
Article in English | MEDLINE | ID: mdl-35440056

ABSTRACT

Dunnigan syndrome, or Familial Partial Lipodystrophy type 2 (FPLD2; ORPHA 2348), is a rare autosomal dominant disorder due to pathogenic variants of the LMNA gene. The objective of the French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins), is to provide health professionals with a guide to optimal management and care of patients with FPLD2, based on a critical literature review and multidisciplinary expert consensus. The PNDS, written by members of the French National Reference Center for Rare Diseases of Insulin Secretion and Insulin Sensitivity (PRISIS), is available on the French Health Authority website (in French). Dunnigan syndrome is characterized by a partial atrophy of the subcutaneous adipose tissue and by an insulin resistance syndrome, associated with a risk of metabolic, cardiovascular and muscular complications. Its prevalence, assessed at 1/100.000 in Europe, is probably considerably underestimated. Thorough clinical examination is key to diagnosis. Biochemical testing frequently shows hyperinsulinemia, abnormal glucose tolerance and hypertriglyceridemia. Elevated hepatic transaminases (hepatic steatosis) and creatine phosphokinase, and hyperandrogenism in women, are common. Molecular analysis of the LMNA gene confirms diagnosis and allows for family investigations. Regular screening and multidisciplinary monitoring of the associated complications are necessary. Diabetes frequently develops from puberty onwards. Hypertriglyceridemia may lead to acute pancreatitis. Early atherosclerosis and cardiomyopathy should be monitored. In women, polycystic ovary syndrome is common. Overall, the management of patients with Dunnigan syndrome requires the collaboration of several health care providers. The attending physician, in conjunction with the national care network, will ensure that the patient receives optimal care through regular follow-up and screening. The various elements of this PNDS are described to provide such a support.


Subject(s)
Hypertriglyceridemia , Insulin Resistance , Lipodystrophy, Familial Partial , Lipodystrophy , Pancreatitis , Acute Disease , Female , Humans , Hypertriglyceridemia/complications , Lipodystrophy, Familial Partial/diagnosis , Lipodystrophy, Familial Partial/genetics , Lipodystrophy, Familial Partial/therapy
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