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A rare medical condition characterized by the clinical presentation of discolored, hypoplastic nails, recurring pleural effusions, lymphedema, recurrent pneumonia and lymphedema.

Lymphedema and Yellow Nails.

Unknown (between 1927 and 1960, 10 cases reported in the literature), more frequent in the presence of severe rhinosinusitis symptoms and immunological disorders.

Autosomal dominant.

Can be caused by mutation in the forkhead family transcription factor gene MFH1 located on 16q24.3.

Two of the following criteria must be present: slow-growing nails (89% of cases), lymphedema (80% of cases), pleuropulmonary symptoms (63% of cases) (pleural effusion, recurrent pneumonitis, bronchiectasis, rhinosinusitis).

The characteristics of the nails include thickening, diminished growth, and onycholysis. The color may vary from a pale yellow to green. The edema is the initial symptom in one-third of cases. Although it mainly occurs in the lower limb, in time edema also affects the genitalia, hands, face, and vocal cords. Respiratory tract is involved with pleural effusion, restrictive or obstructive defects that are poorly responsive to bronchodilatators. Bronchiectasis, severe rhinosinusitis, and laryngeal edema can also be present. These patients may present chylous ascites and pericardial effusion. Some authors report a lack of IgG2. This illness is well known in adults, often occurring with a late onset. One case report described a nonimmune fetal hydrops and recurrent left chylothorax at 4 weeks of age in an infant with maternal Yellow Nail Syndrome.

In the presence of yellow nails, ask about recurrent respiratory infections and watch for the consequences of lymphedema (previous pleural, pericardial or ascitic effusion). Obtain an echocardiography for the exclusion of pericardial effusion. Pulmonary tests are indicated to assess the severity of the respiratory tract involvement. A chest radiograph should be obtained to eliminate the presence of bronchiectasia. Ask about any changes in the voice. If a voice change is present, an orolaryngotracheal examination is useful to assess the extent of the laryngeal edema.

If an alternative to tracheal intubation is available, it must be considered in the evaluation of the case because of the lymphedematous involvement of each part of the respiratory system. The venous access should prefer large veins and should be maintained for a duration as short as possible because of generalized, congenitally hypoplastic lymphatics. Postoperative chest physiotherapy should be considered. Consider patients as suffering from recurrent airway infection with high respiratory reactivity.

No interactions are known with anesthetic medications. If tracheal intubation is needed, the prophylactic administration of antibiotic is recommended. The use of muscle relaxants should be considered once the airway is secured and lung ventilation confirmed. Anticholinergic agents may make pulmonary secretions more tenacious and difficult to clear.

Govaert P, Leroy JG, Pauwels R, et al: Perinatal manifestations of maternal yellow nail syndrome. Pediatrics 89:1016, 1992.  [PubMed: 1594340]
Riedel M: Multiple effusions and lymphedema in the yellow nail syndrome. Circulation...

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