Polycystic ovaries, precocious puberty and acquired hypothyroidism: the van wyk and grumbach syndrome

Chattopadhyay, Asis Kumar; Kumar, Vijay; Marulaiah, Mohan

doi:10.1016/s0022-3468(03)00403-2

Cited by 54 publications

(33 citation statements)

References 10 publications

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“…Besides, the expression of luteinizing hormone (LH) is suppressed as revealed by the elevation of LH-releasing hormone (LHRH). All of these results confirmed that VWGS is a gonadotropin-releasing hormone (GnRH)-independent type of precocious pseudopuberty (2)(3)(4)(5)(6)(7)(8)(9).…”

Section: Introductionsupporting

confidence: 57%

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VanWyk-Grumbach syndrome in a male pediatric patient: A rare case report and literature review

Zhang

Yang

Ruan

et al. 2017

Experimental and Therapeutic Medicine

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Abstract. Primary hypothyroidism usually leads to retardation of linear growth and delay or even arrest of puberty in juvenile patients. In rare cases, pediatric patients with hypothyroidism may present with signs of VanWyk-Grumbach's syndrome (VWGS), which includes juvenile hypothyroidism, delayed bone age and pseudoprecocious puberty. The present study reported on a rare case of VWGS and other complications, including obesity, short stature, hepatomegaly and pituitary hyperplasia. In addition, a comprehensive literature review was performed to illustrate the treatment and outcome of VWGS in pediatric patients. The present study contributed to the current knowledge regarding the diagnosis and treatment of VWGS in pediatric patients.

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Section: Introductionsupporting

confidence: 57%

“…However, enlargement of gonads and formation of ovarian cysts are usually (3,4,27). Thus, early diagnosis of the disease may help to identify cases that require surgery (5,6). Weight gain has been regarded as a symptom of hypothyroidism.…”

Section: Discussionmentioning

confidence: 99%

VanWyk-Grumbach syndrome in a male pediatric patient: A rare case report and literature review

Zhang

Yang

Ruan

et al. 2017

Experimental and Therapeutic Medicine

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“…Girls with this syndrome have breast development, follicular cysts and menstruation in the absence of pubic or axillary hair, which depends on adrenal androgens (2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22). Histopathological analysis of resected ovaries and ovarian cysts shows cystic follicles and little if any luteinisation.…”

Section: Discussionmentioning

confidence: 99%

“…Biochemically, low free thyroxine (T 4 ) is combined with raised levels of thyroid stimulating hormone (TSH), prolactin and oestradiol. Typically, luteinising hormone-releasing hormone (LHRH) stimulation shows an FSH-dominated prepubertal response with suppressed luteinising hormone (LH), confirming gonadotropin-releasing hormone (GnRH)-independent precocious pseudopuberty (2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22). Most cases in the literature are secondary to autoimmune thyroid disease but there are some case reports where the syndrome is secondary to unrecognised congenital hypothyroidism (5,10,12).…”

Section: Introductionmentioning

confidence: 99%

“…Most cases in the literature are secondary to autoimmune thyroid disease but there are some case reports where the syndrome is secondary to unrecognised congenital hypothyroidism (5,10,12). It is important to recognise this syndrome as symptoms regress with thyroid hormone replacement and patients spontaneously enter true puberty at an appropriate time (2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22).…”

Section: Introductionmentioning

confidence: 99%

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An unusual presentation of acquired hypothyroidism: the Van Wyk–Grumbach syndrome

Baranowski

Högler

2012

European Journal of Endocrinology

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The association in young females of long-standing primary hypothyroidism, isosexual precocious pseudopuberty and multicystic enlarged ovaries was first described in 1960 by Van Wyk and Grumbach. Since then, sporadic case reports have contributed to clarifying the key features of this syndrome. The unique elements that lead to this diagnosis are FSH-dominated sexual precocity combined with a delayed bone age in the presence of hypothyroidism. It is important to recognise this syndrome because initiating simple thyroid hormone replacement completely resolves symptoms and hormone abnormalities, avoiding unnecessary investigations for malignancies or surgical intervention. We describe an 8-year-old girl with autoimmune thyroiditis and severe long-standing hypothyroidism presenting with the clinical features of Van Wyk-Grumbach syndrome, a secondary TSH-secreting adenoma and hyperprolactinaemia. In addition, this girl presented with microcytic anaemia, elevated erythrocyte sedimentation rate (ESR) and two unusual features -a newly developed streaky hyperpigmented skin lesion and parathyroid hormone suppression despite vitamin D deficiency. Thyroxine replacement normalised all hormone abnormalities and shrunk the pituitary adenoma within 9 months, but the new skin lesion persisted. We review the literature and explore the pathophysiology of known and new features that give rise to speculation indicating stimulation of the FSH G protein-coupled receptor by excessive TSH, but LH suppression by hyperprolactinaemia.

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Ovarian tumor in a 12‐year old female with severe hypothyroidism: A case of Van Wyk and Grumbach syndrome

Hunold

Alzen

Wudy

et al. 2009

Pediatric Blood & Cancer

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We report a 12-year-old female presenting with an abdominal tumor. Diagnostic workup revealed giant bilateral ovarian cysts, severe hypothyroidism as well as an elevation of CA 125. We refrained from ovariectomy, which would be necessary for a malignant tumor, in view of an evident Van Wyk and Grumbach syndrome. The patient promptly responded to L-thyroxine with complete regression of all symptoms. Hypothyroidism should be considered in the evaluation of ovarian cysts. Although the Van Wyk and Grumbach syndrome is rare, it is crucial to rule it out in order to avoid unnecessary ovarian surgery when thyroid replacement is completely sufficient.

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Polycystic ovaries, precocious puberty and acquired hypothyroidism: the van wyk and grumbach syndrome

Cited by 54 publications

References 10 publications

VanWyk-Grumbach syndrome in a male pediatric patient: A rare case report and literature review

VanWyk-Grumbach syndrome in a male pediatric patient: A rare case report and literature review

An unusual presentation of acquired hypothyroidism: the Van Wyk–Grumbach syndrome

Ovarian tumor in a 12‐year old female with severe hypothyroidism: A case of Van Wyk and Grumbach syndrome

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