Rare association of schizophrenia and unilateral Graves’ disease with contralateral thyroid hemiagenesis in two cases of McCune-Albright syndrome

نویسندگان

  • Subramanyam Padma
  • Palaniswamy Shanmuga Sundaram
چکیده

The classical triad of McCune-Albright syndrome (MAS) consists of polyostotic fibrous dysplasia (FD), skin hyperpigmentation (café-au-lait spots), and endocrine dysfunction, frequently seen in females as precocious puberty. Etiology is genetically based and is explained by mosaicism of activating somatic mutations of the alpha-subunit of Gs protein. Clinical presentation is varied and is dependent on the particular distribution of affected cells, causing a broad spectrum of endocrine and non-endocrine manifestations. Typical endocrinopathies are precocious puberty, hyperthyroidism, growth hormone excess, hyperprolactemia, and hypercortisolism. Manifestations usually occur during infancy and childhood. We present 2 classical cases of MAS with rare association of cerebral and endocrine dysfunction (unilateral Graves’ disease with contralateral thyroid hemi agenesis). The first case is an adult onset MAS with hyperparathyroidism and schizophrenia; this association is hitherto unreported in literature. Literature search showed that mutations in the Gsα gene may be associated with the pathogenesis of schizophrenia which is similar to the underlying factor in MAS. The second is a child exhibiting classical MAS with hyperthyroidism (unilateral Graves' disease) which is common but is associated contralateral thyroid hemiagenesis.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Rare association of schizophrenia and unilateral Graves’ disease with contralateral thyroid hemiagenesis in two cases of McCune-Albright syndrome

The classical triad of McCune-Albright syndrome (MAS) consists of polyostotic fibrous dysplasia (FD), skin hyperpigmentation (café-au-lait spots), and endocrine dysfunction, frequently seen in females as precocious puberty. Etiology is genetically based and is explained by mosaicism of activating somatic mutations of the alpha-subunit of Gs protein. Clinical presentation is varied and is depend...

متن کامل

Mccune-Albright Syndrome: A Case Report Associated with Pamidronate Therapy and Literature Review

McCune-Albright Syndrome (MAS) is a rare sporadic disease characterized by bone fibrous dysplasia, Café au lait spots and a variable association of hyperfunction endocrine disorders. There is not any certain treatment available for this syndrome, and both physical and emotional disability in these patients is still a major concern for physicians. In present report we have described a 10- year-o...

متن کامل

McCune-Albright Syndrome: A Case Report and Literature Review

McCune-Albright syndrome (MAS) is a rare, heterogenous, clinical condition caused by a rare genetic mutation. The disorder is more common in females and is characterized by a triad of cutaneous, bone and endocrine abnormalities.  We describe a girl patient with MAS having precocious puberty and multiple cafe-au-lait macules and deforming polyostotic fibrous dysplasia of bone. Clinical presentat...

متن کامل

Unilateral Thyroid Agenesis—Curiosity or Predictor of Future Pathology?

Unilateral thyroid agenesis (thyroid hemiagenesis) is a rare disorder with less than 300 cases currently in the world literature. The current clinical importance of this condition lies in the possibility that such patients may be at an increased risk for a wide range of future pathology in the contralateral lobe. If this indeed proves to be the case in future, these patients need to be identifi...

متن کامل

Thyroid Gland Hemiagenesis with Multinodular Graves' Disease: A Case Report [Multinodüler Graves' Hastaliğinin Eşlik Ettiği Tiroid Hemiagenezili Olgu]

Thyroid hemiagenesis (THA) is a rare congenital anomaly. It occurs more frequently in the left lobe and predominantly in women. Most of the cases are asymptomatic. The most frequent associated thyroid disorders with THA are nontoxic nodular goiter and Hashimoto's thyroiditis whereas association of Graves' disease with THA is very rare. We present a man patient with THA and multinodular Graves' ...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:

دوره   شماره 

صفحات  -

تاریخ انتشار 2015