Adrenal Tumor Complicating Untreated 21-Hydroxylase Deficiency in a 5½-Year-Old Boy

Bhatia, Vijayalakshmi; Shukla, Rishi; Mishra, SB; Gupta, Rakesh Kumar

doi:10.1001/archpedi.1993.02160360063020

Cited by 9 publications

(6 citation statements)

References 13 publications

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“…There have been few reports in the literature of the association of CAH and adrenal tumors 3 " 8 ' 10 . There have been few reports in the literature of the association of CAH and adrenal tumors 3 " 8 ' 10 .…”

Section: Discussionmentioning

confidence: 99%

Adrenal Nodules in Patients with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency: Regression after Adequate Hormonal Control

Giacaglia¹,

Mendonça²,

Madureira³

et al. 2001

Journal of Pediatric Endocrinology and Metabolism

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Adrenal nodules have been described in patients with 21-hydroxylase deficiency (21OHD). These nodules are usually considered to be ACTH-dependent, as is the commonly seen diffuse cortical hyperplasia. We evaluated the presence and behavior of adrenal nodules in patients with 21OHD. Based upon hormonal status and treatment compliance, the patients were classified into three categories: poor, regular and good control. Out of the 26 patients, eight had the non-classic, four salt-wasting and 14 simple virilizing forms. All patients underwent initial adrenal morphological studies, either by CT or MRI. Those with nodules were reevaluated after 12 months of adequate replacement therapy. Nodules were found in four of eight untreated patients and two of three patients with poor hormonal control, but not in the 15 patients with regular or good control. Adrenal nodules in these six patients demonstrated a considerable size reduction and even disappearance after adequate replacement therapy, showing that these nodules were ACTH-dependent. Thus, six out of 26 patients with 21OHD presented adrenal nodules, which were more frequent in the untreated or poorly-controlled patients, and all regressed in size after adequate therapy.

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Section: Discussionmentioning

confidence: 99%

Adrenal Nodules in Patients with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency: Regression after Adequate Hormonal Control

Giacaglia¹,

Mendonça²,

Madureira³

et al. 2001

Journal of Pediatric Endocrinology and Metabolism

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“…Immunoreactivity of P450 c21 is not absent, but apparently weaker than that of P450 c17 . Daeschner, 1965;Pang et al,1981;van Seters et al, 1981;Bauman & Bauman, 1982;Jaursch-Hancke et al, 1988;Takayama et al, 1988;Shimshi et al, 1992;Bhatia et al, 1993). The tumours were often revealed by progression of virilization (Hamwi et al, 1957;Pang et al, 1981;Bauman & Bauman, 1982;Takayama et al, 1988;Shimshi et al, 1992;Bhatia et al, 1993).…”

Section: Discussionmentioning

confidence: 99%

A case of silent 21‐hydroxylase deficiency with persistent adrenal insufficiency after removal of an adrenal incidentaloma

Nagasaka¹,

Kubota²,

Motegi³

et al. 1996

Clinical Endocrinology

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A case of an adrenal incidentaloma in a 57-year-old man with silent 21-hydroxylase deficiency is reported. Abdominal computed tomography revealed a right adrenal tumour of 6cm in diameter. There was no evidence of adrenal hormone excess. However, after surgical removal of the adrenal tumour, the patient developed acute adrenal insufficiency. Adrenocortical function has remained low ever since surgery. Pathological examination of the tumour revealed a cortical adenoma. On the basis of increased plasma renin activity and serum 17 alpha-hydroxyprogesterone concentration and decreased 21-hydroxylase activity of the adenoma tissue, the patient was diagnosed as having systemic 21-hydroxylase deficiency. There are two possible mechanisms for the persistent adrenal insufficiency; first the residual left adrenal gland may have been originally hypo-functioning for some reason and, second, the left gland may have lost the ability to regenerate following prolonged suppression by the adenoma. In cases of adrenal incidentalomas with 21-hydroxylase deficiency, the indications for surgical removal should be carefully considered.

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“…Due to wider application of increasingly sensitive radiological investigation techniques adrenal tumours are being detected more frequently in children with untreated congenital adrenal hyperplasia. There have been numerous such reports, including one from our own centre 15–17 . In one study the incidence of adrenal masses was found to be as high as 82% in homozygous and 45% in heterozygous patients with CAH 15 .…”

Section: Discussionmentioning

confidence: 99%

“…There have been numerous such reports, including one from our own centre. [15][16][17] In one study the incidence of adrenal masses was found to be as high as 82% in homozygous and 45% in heterozygous patients with CAH. 15 In contrast, virilizing adrenal tumour in female children can be confused with CAH.…”

Section: Adrenal Cortical Tumoursmentioning

confidence: 99%

Functioning adrenal tumours in children and adolescents: An institutional experience

Mishra

Agarwal

Misra

et al. 2001

ANZ Journal of Surgery

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Adrenal Tumor Complicating Untreated 21-Hydroxylase Deficiency in a 5½-Year-Old Boy

Cited by 9 publications

References 13 publications

Adrenal Nodules in Patients with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency: Regression after Adequate Hormonal Control

Adrenal Nodules in Patients with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency: Regression after Adequate Hormonal Control

A case of silent 21‐hydroxylase deficiency with persistent adrenal insufficiency after removal of an adrenal incidentaloma

Functioning adrenal tumours in children and adolescents: An institutional experience

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