Background
Van Wyk-Grumbach syndrome refers to the development of isosexual precocious pseudopuberty and multicystic enlarged ovaries in the presence of hypothyroidism and delayed bone age. It is a rare presentation of untreated hypothyroidism. The prepubertal response in Van Wyk-Grumbach syndrome is always isosexual and mediated by very high thyroid-stimulating hormone levels acting through the follicle-stimulating hormone receptors inducing a follicle-stimulating hormonal effect. Early recognition and thyroid hormone replacement can completely regress precocious puberty and ovarian enlargement, while improving the final height achievement.
Oligosyndactly is a congenital bony abnormality and can manifest either as an isolated malformation or as a component of a syndromic diagnosis. However, development of hypothyroidism in children with this peculiar bony deformity has rarely been described in the medical literature, with the exception of Cenani-Lenz Syndactyly syndrome.
Case presentation
We report the case of a 6-year-old Sri Lankan girl who presented with a 2-day history of vaginal bleeding and exertional dyspnea. She had marked short stature (well below −3 standard deviations) with an upper segment to lower segment ratio of 1.47. This girl had isolated breast development of Tanner stage 2. She was diagnosed to have acquired hypothyroidism secondary to autoimmune thyroiditis and also had macrocytic anemia, pericardial effusion, gonadotropin-releasing hormone-independent precocious puberty with radiological evidence of pubertal changes in the uterus, and multicystic ovaries. Interestingly, she also had post-axial oligosyndactyly in both feet and right-sided clubfoot. The diagnosis of Van Wyk-Grumbach syndrome was made based on the clinical and laboratory features. Her symptoms were successfully managed with L-thyroxine therapy.
Conclusions
Acquired hypothyroidism is a relatively common endocrine disorder among children and early recognition is important to prevent serious complications like Van Wyk-Grumbach syndrome. Sexual precocity with delayed bone age and stunting should direct our minds toward this unique diagnosis. It is always necessary to identify the other associated anomalies in addition to the primary diagnosis since these features may direct to a syndromic diagnosis.
Introduction: Urinary tract infection (UTI) has a significant morbidity highlighting the importance of preventing recurrences. Knowledge on aetiological agents and their antibiotic sensitivities is crucial for proper management of affected children.
Objectives:To determine the causative microorganisms and their antibiotic sensitivity pattern in patients with recurrent UTIs on uroprophylaxis and to compare the first episode of UTI with recurrent UTIs regarding causative organisms and antibiotic sensitivity.Method: A descriptive study was done at Teaching Hospital Peradeniya, recruiting children aged 1 month to 14 years, admitted from 1 st May 2019 to 30 th April 2020 with breakthrough febrile or symptomatic UTIs after being on antibiotic prophylaxis. Details were collected using a validated and pre-tested questionnaire.Results: A total of 141 UTIs of 52 children were analysed. There were 31 (59.6%) boys; 76.3% episodes were febrile and 22.7% were afebrile but symptomatic UTIs. Micturating cystourethrogram revealed that 29 (56%) children had vesico-ureteric reflux; renal scarring was noted in the majority; 24 (46.1%) children had bladder and bowel dysfunction. Constipation was found in 29 (55.7%). Main pathogens implicated in recurrent UTIs were Escherichia coli (E coli) (55%), Klebsiella species (14.6%) and Pseudomonas aeruginosa (13.5%). First episodes were caused by E coli (57.7%), Klebsiella species (17.3%) and Proteus mirabilis (11.5%). Imipenem (89.8%), amikacin (87.7%) and ciprofloxacin (87.7%) were the most sensitive _________________________________________
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.