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Allgrove'S syndrome: case report and literature review.

Yasawy MI - J Family Community Med (2009)

Bottom Line: The results of these extensive investigations along with the clinical evaluations were consistent with Allgrove's syndrome.Glucocorticoid therapy was initiated.The management consisted of pneumatic cardiac dilatation and initiation of cortisone treatment.The patients' response was impressive and they resumed most of their usual activities.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, College of Medicine, King Faisal University, Dammam, Saudi Arabia.

ABSTRACT
This report concerns two brothers aged 10 and 18 years with long-standing dysphagia that started at age three and six years respectively. They had been diagnosed as achalasia and treated accordingly. The appearance of additional symptoms and clinical signs required further investigations including abdominal sonography, esophago-gastroduodenoscopy, barium swallow, esophageal manometry, computerized tomography (CT) of abdomen and brain, biochemical profiles, and neurologic and ophthalmic evaluations. The results of these extensive investigations along with the clinical evaluations were consistent with Allgrove's syndrome.Glucocorticoid therapy was initiated. The management consisted of pneumatic cardiac dilatation and initiation of cortisone treatment. The patients' response was impressive and they resumed most of their usual activities.

No MeSH data available.


Related in: MedlinePlus

Patient during admission in the hospital
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Figure 1: Patient during admission in the hospital

Mentions: On examination, he was cachectic, wasted and bedridden (Figure 1). The nasal speech of the young boy was understood only by his parents. He was pale with hyper-pigmentation of skin, gums, and palmar creases. His face was long and thin with narrowed upper lips down-turned mouth and microcephaly. His blood pressure was 80/50 mmHg. The joint motions were limited because of muscle contractures, spastic tetraparesis with mild ataxia, deep tendon reflexes were increased in all four limbs. His basic investigations showed moderate anemia of hemoglobin (Hb) of 10 g/dl. Urea and electrolytes were normal. Cortisol level at 8 am was less than 1.1 ug/dl and ACTH was 54.1. Adrenal imaging studies did not reveal any abnormality. Barium swallow study and esophageal manometry features were consistent with achalasia. He was seen and evaluated by an ophthalmologist and Schirmer's test was needed. This revealed dry eyes i.e. alacrima while, CT brain and orbit tomography showed reduced lacrimal gland tissue.


Allgrove'S syndrome: case report and literature review.

Yasawy MI - J Family Community Med (2009)

Patient during admission in the hospital
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3377019&req=5

Figure 1: Patient during admission in the hospital
Mentions: On examination, he was cachectic, wasted and bedridden (Figure 1). The nasal speech of the young boy was understood only by his parents. He was pale with hyper-pigmentation of skin, gums, and palmar creases. His face was long and thin with narrowed upper lips down-turned mouth and microcephaly. His blood pressure was 80/50 mmHg. The joint motions were limited because of muscle contractures, spastic tetraparesis with mild ataxia, deep tendon reflexes were increased in all four limbs. His basic investigations showed moderate anemia of hemoglobin (Hb) of 10 g/dl. Urea and electrolytes were normal. Cortisol level at 8 am was less than 1.1 ug/dl and ACTH was 54.1. Adrenal imaging studies did not reveal any abnormality. Barium swallow study and esophageal manometry features were consistent with achalasia. He was seen and evaluated by an ophthalmologist and Schirmer's test was needed. This revealed dry eyes i.e. alacrima while, CT brain and orbit tomography showed reduced lacrimal gland tissue.

Bottom Line: The results of these extensive investigations along with the clinical evaluations were consistent with Allgrove's syndrome.Glucocorticoid therapy was initiated.The management consisted of pneumatic cardiac dilatation and initiation of cortisone treatment.The patients' response was impressive and they resumed most of their usual activities.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, College of Medicine, King Faisal University, Dammam, Saudi Arabia.

ABSTRACT
This report concerns two brothers aged 10 and 18 years with long-standing dysphagia that started at age three and six years respectively. They had been diagnosed as achalasia and treated accordingly. The appearance of additional symptoms and clinical signs required further investigations including abdominal sonography, esophago-gastroduodenoscopy, barium swallow, esophageal manometry, computerized tomography (CT) of abdomen and brain, biochemical profiles, and neurologic and ophthalmic evaluations. The results of these extensive investigations along with the clinical evaluations were consistent with Allgrove's syndrome.Glucocorticoid therapy was initiated. The management consisted of pneumatic cardiac dilatation and initiation of cortisone treatment. The patients' response was impressive and they resumed most of their usual activities.

No MeSH data available.


Related in: MedlinePlus