Van Wyk-Grumbach syndrome with hemangioma in an infant

2018 ◽  
Vol 31 (9) ◽  
pp. 1057-1060
Author(s):  
Moumita Biswas ◽  
Malay Kumar Sinha ◽  
Mrinal Kanti Das ◽  
Sumantra Sarkar
Keyword(s):  
Thyroid Hormone ◽  
Congenital Hypothyroidism ◽  
Hormone Replacement ◽  
Signs And Symptoms ◽  
Bone Age ◽  
Infantile Hemangioma ◽  
Thyroid Stimulating Hormone ◽  
High Serum ◽  
Primary Hypothyroidism ◽  
Thyroid Hormone Replacement

Abstract Background Van Wyk-Grumbach syndrome (VWGS) is characterized by juvenile primary hypothyroidism, delayed bone age and isosexual incomplete precocious puberty with reversal to the prepubertal state following thyroid hormone replacement. Case presentation In this case, an 18-month-old girl presented with premature menarche since 9 months of age, delayed bone age and enlarged bilateral multicystic ovaries along with a superficial infantile hemangioma over the upper anterior chest. VWGS was diagnosed based on the clinical features. High serum thyroid stimulating hormone and low free thyroxine with the absence of any carpal bones in the wrist X-ray were suggestive of congenital hypothyroidism. Interestingly, the coexisting hemangioma could also play a role in the etiology of the hypothyroidism through “consumptive hypothyroidism”. Thyroid hormone replacement resulted in the complete resolution of signs and symptoms. Conclusions Untreated congenital hypothyroidism of short duration, onset of symptoms in infancy and association of an infantile hemangioma in VWGS were the unique features in our case.

scholarly journals Effect of Adequate Thyroid Hormone Replacement on the Hypothalamo-Pituitary-Gonadal Axis in Premenopausal Women with Primary Hypothyroidism

2019 ◽  
Vol 8 (3) ◽  
pp. 152-158
Author(s):  
Bharath Bachimanchi ◽  
Suresh Vaikkakara ◽  
Alok Sachan ◽  
Ganji Praveen Kumar ◽  
Ashok Venkatanarasu ◽  
...  
Keyword(s):  
Thyroid Hormone ◽  
Hormone Replacement ◽  
Premenopausal Women ◽  
Primary Hypothyroidism ◽  
Thyroid Hormone Replacement

SERUM PROLACTIN IN PATIENTS WITH "FUNCTIONLESS" CHROMOPHOBE ADENOMAS BEFORE AND AFTER THERAPY

1977 ◽  
Vol 84 (3) ◽  
pp. 449-460 ◽  
Author(s):  
N. A. Samaan ◽  
M. E. Leavens ◽  
J. H. Jesse
Keyword(s):  
Hormone Replacement ◽  
Pituitary Surgery ◽  
Pituitary Tumour ◽  
Pituitary Hormones ◽  
Thyroid Stimulating Hormone ◽  
High Serum ◽  
Primary Hypothyroidism ◽  
Pituitary Function ◽  
Serum Prolactin ◽  
Before And After

ABSTRACT The immunoreactive serum human prolactin (PRL) level was measured before and after intravenous administration of 500 μg of thyrotrophinreleasing hormone (TRH) in 11 patients with "functionless" chromophobe adenomas before and after surgery and after radiotherapy in 6 of these patients. The results were compared to other pituitary function tests. Two of the patients studied had recurrent disease after previous pituitary surgery and radiotherapy. Five patients had pituitary surgery through the transfrontal route, while 6 had adenoma removal via the transnasal transsphenoidal route. Before surgery, the serum PRL concentration was abnormally high in 4 patients, before and after TRH administration. It was normal in 6 and subnormal in 1 patient who had had previous therapy. Two of the patients studied showed high serum thyroid-stimulating hormone (TSH) levels in the presence of low serum T3 and T4 suggesting primary hypothyroidism with a secondary TSH-producing pituitary tumour. After surgery all patients showed a significant decrease of the serum PRL level. This contrasts with more variable results in the measurements of other pituitary hormones. Post-operative radiotherapy produced no significant additional change in serum PRL levels in 5 of the 6 patients measured 6 months to 4 years after radiotherapy. Five of the 6 patients who had adenoma removed via the transsphenoidal route required no cortisol replacement and 4 remained euthyroid, whereas all 5 patients after transfrontal surgery required both cortisol and thyroid hormone replacement. These results indicate: (1) that measurement of serum PRL levels at basal and after TRH administration in patients with "functionless" chromophobe adenomas before and after treatment may be the best index for evaluating the effect of therapy; (2) that adenoma removal may be followed by preservation of normal pituitary function, but this is more likely to occur if the transsphenoidal approach is used; and (3) that primary thyroid insufficiency may be associated with a pituitary adenoma.

Thyroid function tests (TFTs) abnormalities in patients (pts) with metastatic renal cell carcinoma (mRCC) treated with sunitinib

2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 4605-4605 ◽  
Author(s):  
P. E. Shaheen ◽  
I. R. Tamaskar ◽  
R. N. Salas ◽  
B. I. Rini ◽  
J. Garcia ◽  
...  
Keyword(s):  
Treatment Outcome ◽  
Growth Factor ◽  
Thyroid Hormone ◽  
Kinase Inhibitor ◽  
Hormone Replacement ◽  
Signs And Symptoms ◽  
Thyroid Function Tests ◽  
Thyroid Hormone Replacement ◽  
Treatment Tolerance ◽  
Endothelial Growth Factor

4605 Background: Sunitinib is a multi-targeted receptor tyrosine kinase inhibitor of vascular endothelial growth factor and platelet-derived growth factor receptors. It has anti-tumor activity in mRCC pts with toxicity including fatigue. We investigated TFTs abnormalities and related signs and symptoms in pts with mRCC receiving sunitinib. Methods: The medical records of pts with mRCC enrolled in 4 ongoing clinical trials of sunitinib were reviewed. TFTs assessment (TSH, T3 and T4) was undertaken based on the clinical suspicion of treating physicians. Patient demographics, frequency and values of TFTs and any signs and symptoms of thyroid dysfunction were collected. Abnormal TFTs and treatment outcome were correlated. Results: Between 5/2004 and 12/2005, 62 pts (43 males, 19 females) were treated with sunitinib. The median age was 58 years (range, 23–72). Fifty-five pts had TFTs assessed while on treatment and 40 pts (65% of total) had one or more abnormality. Two pts had well-controlled hypothyroidism prior to initiation of sunitinib. TFTs abnormalities were consistent with hypothyroidism in all pts including one who initially developed transient hyperthyroidism. Signs and symptoms possibly related to hypothyroidism were found in 33 pts (53% of total) with abnormal TFTs and were initially attributed to sunitinib. Signs and symptoms included fatigue in 33 pts, anorexia in 20 pts, fluid retention in 17 pts, and skin/hair changes in 13 pts. Thyroid hormone replacement was undertaken in 12 pts and resulted in improvement of symptoms in 6 pts. Among the 40 pts with abnormal TFTs 29 pts had tumor evaluation; 13 had SD, 8 had PR, 2 had CR. There was no correlation between abnormal TFTs and treatment outcome. Conclusions: TFTs abnormalities are common in pts with mRCC treated with sunitinib. Thyroid hormone replacement is indicated in such pts to improve hypothyroidism-related symptoms and possibly to improve treatment tolerance. [Table: see text]

scholarly journals Van Wyk- Grumbach Syndrome – An Unusual Presentation of Severe Hypothyroidism

2016 ◽  
Vol 11 (1) ◽  
pp. 85-88
Author(s):  
Lubna Naznin ◽  
Suchitra Saha ◽  
Debashish Saha ◽  
Sarmin Sultana ◽  
Md Jahangir Chowdhury
Keyword(s):  
Hormone Replacement ◽  
Armed Forces ◽  
Bone Age ◽  
High Serum ◽  
Thyroid Hormone Replacement ◽  
Medical College ◽  
Thyroid Hormone Replacement Therapy ◽  
Precocious Pseudopuberty ◽  
Complete Reversal ◽  
Serum Tsh

Van Wyk- Grumbach syndrome (VWGS) is characterized by juvenile hypothyroidism, delayed bone age, and precocious puberty with a complete reversal to the pre-pubertal state following thyroid hormone replacement therapy. In this study, a 7 years and 1 month old girl presented with precocity having premature menarche, short stature, constipation, delayed bone age and enlarged bilateral multicystic ovaries. She presented with acute abdomen due to torsion of left ovary and had to undergo left sided oophorectomy and right ovarian cystectomy. High serum TSH, low FT4 with high FSH but low LH within pre-pubertal range suggestive of ‘severe hypothyroidism with FSH dominant precocious pseudopuberty’ confirmed the diagnosis of VWGS.Journal of Armed Forces Medical College Bangladesh Vol.11(1) 2015: 85-88

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