SummaryEctopic secretion of ACTH is an infrequent cause of Cushing's syndrome. We report a case of ectopic ACTH syndrome caused by a nasal paraganglioma, a 68-year-old female with clinical features of Cushing's syndrome, serious hypokalaemia and a right paranasal sinus' lesion. Cranial magnetic resonance image showed a 46-mm mass on the right paranasal sinuses. Endocrinological investigation confirmed the diagnosis of ectopic ACTH production. Resection of the tumour normalised ACTH and cortisol secretion. The tumour was found to be a paraganglioma through microscopic analysis. On follow-up 3 months later, the patient showed nearly complete clinical recovery. Ectopic ACTH syndrome due to nasal paraganglioma is extremely uncommon, as only two other cases have been discussed in the literature.Learning points
Ectopic Cushing's syndrome accounts for 10% of Cushing's syndrome etiologies.Most paraganglioma of the head and neck are not hormonally active.Nasal paraganglioma, especially ACTH producing, is a very rare tumour.
Thyroid lymphomas are rare clinical entities that may result from either the primary intrathyroid de novo or secondary thyroid gland involvement of a lymphoma. Among these, the Hodgkin's subtype is quite uncommon, accounting for 0.6–5% of all thyroid malignancies. The authors report on a 76-year-old female presenting with a thyroid nodule that, upon surgical excision, was found to be a nodular lymphocyte predominant Hodgkin lymphoma of the thyroid. So far, thyroid involvement by this variant has never been reported. Upon reporting on this clinical case, the authors emphasize the difficulties usually found in establishing the diagnosis and in defining the best management strategy. A thorough review of the available literature is done.
Tubulointerstitial nephritis and uveitis (TINU) syndrome is a rare disease characterised by the association of acutetubulointerstitial nephritis and uveitis. It affects mainly children and young women. Drugs and infections may be precipitating factors. It is a diagnosis of exclusion. The mainstays of treatment are topical and systemic corticosteroids. Prognosis is usually favourable. We report a case of TINU which occurred in our unit. A 37-year-old woman presented with an influenza-like illness, bilateral ocular pain and blurred vision. Ophthalmological evaluation revealed bilateral anterior uveitis and later renal involvement was seen as acute tubulointerstitial nephritis. A diagnosis of TINU was assumed after exclusion of other systemic diseases. She was treated with topical corticosteroids for the uveitis and evolved favourably, with resolution of ocular symptoms and normalisation of serum creatinine and proteinuria. This case highlights the importance of a high degree of clinical suspicion to make the diagnosis of TINU syndrome.
In comparison to oral steroids, the intravenous pulse regimen was associated with a lower risk of relapse in IgAN, a known independent negative predictor of renal survival. No differences were found regarding the other renal outcomes.
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