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IgG4-related disease is a rare chronic pathology manifested by lymphoplasmacytic infiltration of one or more organs, the formation of storiform fibrosis, tissue edema, and an increase of IgG4 in the blood. This disease was singled out as an independent nosological unit only in 2001. The incidence is less than 1 in 100,000 people per year. Almost any organ can be affected in IgG4-related disease. The association of Riedel's thyroiditis with IgG4 was established in 2010. Riedel's thyroiditis is an extremely rare inflammatory disease of the thyroid gland, which diagnosis is complicated by an atypical course and the absence of characteristic symptoms. Less than 300 clinical cases of the disease have been described in the world, only two from them were in children. This article presents a clinical case of a 6-year-old boy with Riedel's thyroiditis.

Background:  Primary hyperparathyroidism (PHPT) is an endocrine disorder characterized by excessive secretion of parathyroid hormone (PTH) with upper-normal or elevated blood calcium levels due to primary thyroid gland pathology. PHPT is a rare pathology in children, with a prevalence of 2-5:100,000 children according to the literature. Due to the non-specificity of clinical manifestations at onset (nausea, vomiting, abdominal pain, emotional lability), the disease may remain undiagnosed for a long time., Aim:  To study the features of the course and molecular genetic basis of primary hyperparathyroidism in children., Materials and Methods:  Retrospective observational study of 49 patients diagnosed with primary hyperparathyroidism. All patients underwent a comprehensive laboratory-instrumental and molecular genetic study at the Institute of Pediatric Endocrinology, Endocrinology Research Center of Russia in the period 2014-2022., Results:  The first clinical symptoms of PHPT were noted at the age of 13.8 years [10.6; 1 5.2], among which fatigue, headaches, dyspepsia, lower limb pain, and fractures were the most common. The age of diagnosis was 15.81 years [13.1; 16.8], all children were found to have high levels of PTH, total and ionized calcium, with hypophosphatemia in 93.9% of patients (n=46) and hypercalciuria in 43% (n=21). Five out of 49 patients (10.2%) were found to have ectopy of the thyroid: 3 showed an intrathyroidal location, 2 in the mediastinal region. Molecular genetic study revealed mutations in 32.7% of patients (n=16, CI (21; 47)), mutations in MEN1 being the most frequent (n=11). Pathogenic variants in CDC73 were detected in 3 patients, RET - in 2. Among the operated 39 patients, adenoma of the thyroid was detected in 84.6% of cases (n=33), hyperplasia in 7.7% (n=3), atypical adenoma in 5.1% (n=2), carcinoma in 5.1% of cases (n=2)., Conclusion:  The paper presents the peculiarities of the course and the results of molecular genetic study of pediatric PHPT. This sample is the largest among those published in the Russian Federation.

The presented clinical case of a girl with a salt-wasting form of congenital adrenal hyperplasia in combination with chronic recurrent infection and lower urinary tract dysfunction demonstrates the need to change conventional two-staged approach to surgical feminization in favor of a one-stage intervention in order to prevent a progression of genitourinary complications. After controlling for the underlying condition, the one-stage feminization was performed, including modified tightening introitoplasty using a Passerini-Glazel flap and a correction of hypertrophic clitoris and labia minora. Good short- and long-term results were achieved.

The authors analyse treatment of 3235 patients with prepuce pathology aged from 8 days to 16 years, the results of the treatment of preputial sac pathology; describe clinical manifestations of phimosis (physiological, hypertrophic, cicatricial) in children; formulate treatment policy, indications and contraindications for conservative and surgical treatment; provide practical recommendations on hygiene and care for infant and young age boys.

14 boys with balanic hypospadia (BH) and 23 boys with coronal hypospadia (CH) aged 9 months to 15 years were operated in 1997-2000. For 13 boys this operation was meatoglanuloplasty (MAGPI) by J. Duckett. Complications occurred in 4 boys: retraction (downwards) of the meatus (n = 2), urethral stenosis and urinary fistula (n = 2). 24 boys have undergone modified MAGPI operation. The modification consists in extended mobilization of the lateral parts of the head with longitudinal consecutive incisions along the head margin into the spongy bodies. The head is closed by suturing its mobilized margins with two rows of interrupted monofilamentous (PDS, prolene) sutures above the urethra. A variant of saving foreskin is possible when partial separation of the internal and external layers is made bilaterally with consecutive circular closure of the preputial sac. Phimosis is prevented by a relief longitudinal incision along the internal foreskin and transverse suturing. The patients were followed up for 2 months to 1.5 years. The original operation MAGPI provided good cosmetic and functional results in 9 boys(69.2%), satisfactory in 2 boys(15.4%), unsatisfactory also in 2 boys(15.4%). The modified MAGPI operation provided only good cosmetic and functional results. Thus, the proposed modification (MAGPI-Duckett) ensures a more reliable variant of meatoglanuloplasty in balanic and coronal hypospadia irrespective of the size and shape of the head, presence of the scaphoid fossa or meatostenosis. These additional procedures helped to reduce the number of postoperative complications and improved cosmetic and functional results.