Literature DB >> 8333772

Neurological and adrenal dysfunction in the adrenal insufficiency/alacrima/achalasia (3A) syndrome.

D B Grant1, N D Barnes, M Dumic, M Ginalska-Malinowska, P J Milla, W von Petrykowski, R J Rowlatt, R Steendijk, J H Wales, E Werder.   

Abstract

Review of 20 patients with glucocorticoid deficiency (three cases also with salt loss) associated with absent tear secretion (19 cases) and achalasia of the cardia (15 cases) revealed neurological abnormalities in 17 including hyper-reflexia, muscle weakness, dysarthria, and ataxia together with impaired intelligence and abnormal autonomic function, particularly postural hypotension. These findings indicate that significant neurological problems are common in this multisystem disorder.

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Year:  1993        PMID: 8333772      PMCID: PMC1029374          DOI: 10.1136/adc.68.6.779

Source DB:  PubMed          Journal:  Arch Dis Child        ISSN: 0003-9888            Impact factor:   3.791


  24 in total

1.  Familial Addison's disease; case reports of two sisters with corticoid deficiency unassociated with hypoaldosteronism.

Authors:  T H SHEPARD; B H LANDING; D G MASON
Journal:  AMA J Dis Child       Date:  1959-02

2.  Familial glucocorticoid insufficiency.

Authors:  T Moshang; R L Rosenfield; A M Bongiovanni; J S Parks; J A Amrhein
Journal:  J Pediatr       Date:  1973-05       Impact factor: 4.406

3.  The syndrome of congenital adrenocortical unresponsiveness to ACTH. Report of six cases.

Authors:  C J Migeon; E M Kenny; A Kowarski; C A Snipes; J S Spaulding; J W Finkelstein; R M Blizzard
Journal:  Pediatr Res       Date:  1968-11       Impact factor: 3.756

4.  Esophageal Lewy bodies associated with ganglion cell loss in achalasia. Similarity to Parkinson's disease.

Authors:  S J Qualman; H M Haupt; P Yang; S R Hamilton
Journal:  Gastroenterology       Date:  1984-10       Impact factor: 22.682

5.  Glucocorticoid and partial mineralocorticoid deficiency associated with achalasia.

Authors:  R Lanes; L P Plotnick; T E Bynum; P A Lee; J F Casella; C E Fox; A A Kowarski; C J Migeon
Journal:  J Clin Endocrinol Metab       Date:  1980-02       Impact factor: 5.958

6.  [Familial glucocorticoid insufficiency (author's transl)].

Authors:  W Petrykowski; P Burmeister; N Böhm
Journal:  Klin Padiatr       Date:  1975-05       Impact factor: 1.349

7.  Isolated glucocorticoid insufficiency.

Authors:  E A Werder; R Haller; W Vetter; M Zachmann; R Siebenmann
Journal:  Helv Paediatr Acta       Date:  1975-07

8.  Infantile achalasia associated with deficient tear production.

Authors:  Y Efrati; A J Mares
Journal:  J Clin Gastroenterol       Date:  1985-10       Impact factor: 3.062

9.  Familial glucocorticoid deficiency with achalasia of the cardia and deficient tear production.

Authors:  J Allgrove; G S Clayden; D B Grant; J C Macaulay
Journal:  Lancet       Date:  1978-06-17       Impact factor: 79.321

10.  Glucocorticoid deficiency with achalasia of the cardia and lack of lacrimation.

Authors:  M Pombo; J Devesa; A Taborda; M Iglesias; F García-Moreno; G J Gaudiero; J M Martinón; M Castro-Gago; J Peña
Journal:  Clin Endocrinol (Oxf)       Date:  1985-09       Impact factor: 3.478

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  37 in total

1.  Long-term clinical follow-up and molecular genetic findings in eight patients with triple A syndrome.

Authors:  Miroslav Dumic; Nina Barišic; Vesna Kusec; Katarina Stingl; Mate Skegro; Andrija Stanimirovic; Katrin Koehler; Angela Huebner
Journal:  Eur J Pediatr       Date:  2012-04-28       Impact factor: 3.183

2.  Achalasia in siblings in infancy.

Authors:  P S Rao; P L Rao
Journal:  Indian J Pediatr       Date:  2001-09       Impact factor: 1.967

Review 3.  Diagnosis and management of pediatric adrenal insufficiency.

Authors:  Ahmet Uçar; Firdevs Baş; Nurçin Saka
Journal:  World J Pediatr       Date:  2016-04-08       Impact factor: 2.764

Review 4.  The molecular biology, biochemistry, and physiology of human steroidogenesis and its disorders.

Authors:  Walter L Miller; Richard J Auchus
Journal:  Endocr Rev       Date:  2010-11-04       Impact factor: 19.871

5.  Mutations of the AAAS gene in an Indian family with Allgrove's syndrome.

Authors:  Ashis Mukhopadhya; Sumita Danda; Angela Huebner; Ashok Chacko
Journal:  World J Gastroenterol       Date:  2006-08-07       Impact factor: 5.742

6.  Clinical and genetic characterisation of a series of patients with triple A syndrome.

Authors:  Erdal Kurnaz; Paolo Duminuco; Zehra Aycan; Şenay Savaş-Erdeve; Nursel Muratoğlu Şahin; Melişah Keskin; Elvan Bayramoğlu; Marco Bonomi; Semra Çetinkaya
Journal:  Eur J Pediatr       Date:  2017-12-19       Impact factor: 3.183

7.  Neurological features in adult Triple-A (Allgrove) syndrome.

Authors:  Anne-Evelyne Vallet; Annie Verschueren; Philippe Petiot; Nadia Vandenberghe; Marc Nicolino; Sabine Roman; Jean Pouget; Christophe Vial
Journal:  J Neurol       Date:  2011-06-09       Impact factor: 4.849

8.  Familial adrenocorticotropin unresponsiveness associated with alacrima and achalasia: biochemical and molecular studies in two siblings with clinical heterogeneity.

Authors:  C Heinrichs; C Tsigos; J Deschepper; R Drews; R Collu; C Dugardeyn; P Goyens; G E Ghanem; D Bosson; G P Chrousos
Journal:  Eur J Pediatr       Date:  1995-03       Impact factor: 3.183

9.  The "4A" syndrome: adrenocortical insufficiency associated with achalasia, alacrima, autonomic and other neurological abnormalities.

Authors:  M Gazarian; C T Cowell; M Bonney; W G Grigor
Journal:  Eur J Pediatr       Date:  1995-01       Impact factor: 3.183

10.  An 11-year-old boy with dark skin, swallowing difficulty and absence of tears.

Authors:  Debkrishna Mallick; Rajoo Thapa
Journal:  Indian J Dermatol       Date:  2009       Impact factor: 1.494

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