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Reflex sympathetic dystrophy (RSD) is characterized by severe pain, swelling, autonomic dysfunction and usually affects extremities following trauma. In this case we report a 16-year-old female patient who had undergone several operations on the distal of the right lower extremity over a period of 1.5 years. During the follow-up she was determined to have adolescent osteomalacia (primary vitamin D deficiency) and RSD findings at the right lower extremity. We also examined reports in the literature concerning the probable initiator role of vitamin D deficiency among the mechanisms of RSD occurrence. This is the first report of primary vitamin D deficiency accompanied by RSD in an adolescent with multiple fractures. [ABSTRACT FROM AUTHOR]

Objective: Thyroidectomy is a relatively uncommon procedure in pediatric patients. We aimed to review our 20-year experience of thyroid surgery., Methods: A total of 39 patients who underwent thyroid surgery from 2003 to 2023 were retrospectively evaluated. All patients were followed preoperatively and postoperatively by our institutional multidisciplinary board. Patients were divided into two groups based on their pathologies: benign and malignant., Results: In total, 39 patients (27 girls and 12 boys) underwent 47 thyroid surgeries (total thyroidectomy in 19 patients and subtotal thyroidectomy in 20 patients, with 8 of them having completion thyroidectomy). Notably, 20 (51%) patients had benign and 19 (49%) patients had malignant pathologies. Median age at operation was 157 (9-223) months in the benign group and 182 (1-213) months in the malignant group. In the benign group, 12 (60%) patients had colloidal goiter and 8 (40%) patients had other conditions. In the malignant group, 12 (63%) patients had papillary thyroid carcinoma, 3 (16%) patients had follicular thyroid carcinoma, 2 (11%) had medullary thyroid carcinoma, and 2 patients had other thyroid malignancies. Overall permanent complication rate was 2 out of 39 (5%), which was similar for both groups (1 hypocalcemia in each group). The median follow-up was 38 months (1-179 months) with no local recurrence or distant metastasis., Conclusion: Pediatric thyroidectomies are performed on a heterogeneous group of pediatric patients due to a diverse group of pathologies. A multidisciplinary approach is required for proper initial management and surgical strategy with decreased complication rate and event-free survival of these patients in experienced tertiary centers.

Context: Approximately 150 patients with juvenile gigantomastia have been reported in the literature but the underlying biologic mechanisms remain unknown., Objective: To conduct extensive clinical, biochemical, immunochemical, and genetic studies in 3 patients with juvenile gigantomastia to determine causative biologic factors., Methods: We examined clinical effects of estrogen by blockading estrogen synthesis or its action. Breast tissue aromatase expression and activity were quantitated in 1 patient and 5 controls. Other biochemical markers, including estrogen receptor α (ERα), cyclin D1 and E, p-RB, p-MAPK, p-AKT, BCL-2, EGF-R, IGF-IR β, and p-EGFR were assayed by Western blot. Immunohistochemical analyses for aromatase, ERα and β, PgR, Ki67, sulfotransferase, estrone sulfatase, and 17βHD were performed in all 3 patients. The entire genomes of the mother, father, and patient in the 3 families were sequenced., Results: Blockade of estrogen synthesis or action in patients resulted in demonstrable clinical effects. Biochemical studies on fresh frozen tissue revealed no differences between patients and controls, presumably due to tissue dilution from the large proportion of stroma. However, immunohistochemical analysis of ductal breast cells in the 3 patients revealed a high percent of ERα (64.1% ± 7.8% vs reference women 9.6%, range 2.3-15%); aromatase score of 4 (76%-100% of cells positive vs 30.4% ± 5.6%); PgR (69.5% ± 15.2% vs 6.0%, range 2.7%-11.9%) and Ki67 (23.7% ± 0.54% vs 4.2%). Genetic studies were inconclusive although some intriguing variants were identified., Conclusion: The data implicate an important biologic role for ERα to increase tissue sensitivity to estrogen and aromatase to enhance local tissue production as biologic factors involved in juvenile gigantomastia., (© The Author(s) 2024. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Purpose: On the new era of stem cell therapy, the present experimental study was conducted to investigate renal regenerative capacity related to kidney stem cell reserve in different nephrectomy (Nx) models., Methods: Three- and eight-week-old rats (n = 168) were randomly divided into four groups to include control and three Nx subgroups (1/6 Nx, 1/2 Nx, and 5/6 Nx) (Fig. 1). On post-Nx days 15, 30 and 60, kidney specimens were obtained to determine renal regenerative capacity. The specimens were examined with immunofluorescence. CD90/CD105 and Ki-67 expressions were determined as stem cell and cellular proliferation markers, respectively. Fig. 1 Intraoperative photographs showing three different types of nephrectomies (unilateral total Nx has not been shown in 5/6 Nx group) RESULTS: CD90 and CD105 expressions were stronger in glomeruli, but Ki-67 expressions were present only in tubuli. When all Nx types and post-Nx days were considered, both 3- and 8-week-old rats undergone 5/6 Nx had the highest glomerular CD90 and CD105 double expressions. While the expressions gradually increased toward the day 60 in 3-weeks old rats, 8-week-old rats had almost stable double expressions. The strongest tubular Ki-67 expressions were seen in 5/6 Nx groups of both in 3- and 8-week-old rats. The expressions were strongest on day 15 and then gradually decreased. Ipsilateral 1/6 Nx groups had stronger Ki-67 expression than contralateral ones in both age groups., Conclusions: Kidneys may pose a regenerative response to tissue/volume loss through its own CD90- and CD105-related stem cell reserve which mainly takes place in glomeruli and seems to have some interactions with Ki-67-related tubular proliferative process. This response supports that kidney stem cells may have a potential to overcome tissue/volume loss-related damage., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Competing Interests: The authors declare that they have no conflict of interest.

Introduction: There is an ongoing debate whether to perform orchiectomy or orchidopexy following testicular torsion (TT) in cases where the testis seems non-viable. The main problem is lack of objective criteria defining testicular viability. The aim of this study was to investigate the grade of injury in orchiectomy specimens obtained from cases of TT and its association with clinical findings., Methods: This multicenter retrospective study involved double-blinded reassessment of the patient files and the pathological specimens using Mikuz classification to analyze the relation between clinical and pathological findings., Results: A total of 289 patient charts from 14 centers were reviewed and 228 were included in this study. Twenty (8.8%) patients had grade 1 injury which refers to reversible injury. The clinical findings of these 20 patients were compared to 208 patients with higher grades of injury. As expected, there was statistically significant difference regarding duration of symptoms (p < 0.001); however, range was wide in both groups (as long as 96 h for grade 1 and as short as 7 h for higher grades). There was no statistically significant difference in any other variable including age (median 14 for both, p = 0.531), symptoms (pain: 19/20 vs. 189/202, p = 0.801; swelling: 13/19 vs. 168/197, p = 0.094), absence of blood flow in Doppler US (15/19 vs. 164/197, p = 0.635), or degree of torsion (median 720° for both, p = 0.172)., Conclusion: Our study revealed necessity for better criteria to define viability of testis following TT. Histopathological injury appeared to be reversible even in some patients with more severe perioperative findings, late admission, or high degree of twisting. Our findings support the tendency for testicular fixation instead of orchiectomy as none of the clinical or perioperative findings could be attributed to high-grade injury., (© 2023 S. Karger AG, Basel.)

Introduction: There have been some significant changes regarding healthcare utilization during the COVID-19 pandemic. Majority of the reports about the impact of the COVID-19 pandemic on diabetes care are from the first wave of the pandemic. We aim to evaluate the potential effects of the COVID-19 pandemic on the severity of diabetic ketoacidosis (DKA) and new onset Type 1 diabetes presenting with DKA, and also evaluate children with DKA and acute COVID-19 infection., Methods: This is a retrospective multi-center study among 997 children and adolescents with type 1 diabetes who were admitted with DKA to 27 pediatric intensive care units in Turkey between the first year of pandemic and pre-pandemic year., Results: The percentage of children with new-onset Type 1 diabetes presenting with DKA was higher during the COVID-19 pandemic ( p < 0.0001). The incidence of severe DKA was also higher during the COVID-19 pandemic ( p < 0.0001) and also higher among children with new onset Type 1 diabetes ( p < 0.0001). HbA1c levels, duration of insulin infusion, and length of PICU stay were significantly higher/longer during the pandemic period. Eleven patients tested positive for SARS-CoV-2, eight were positive for new onset Type 1 diabetes, and nine tested positive for severe DKA at admission., Discussion: The frequency of new onset of Type 1 diabetes and severe cases among children with DKA during the first year of the COVID-19 pandemic. Furthermore, the cause of the increased severe presentation might be related to restrictions related to the pandemic; however, need to evaluate the potential effects of SARS-CoV-2 on the increased percentage of new onset Type 1 diabetes., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Kiral, Kirel, Havan, Keskin, Karaoglan, Yildirim, Kangin, Talay, Urun, Altug, Kesici, Tufan, Kacmaz, Bozan, Azapagasi, Uysal Yazici, Ozturk, Yesilbas, Karaguzel, Kaya, Barlas, Duyu, Boyraz, Sevketoglu, Akcay, Hancili, Guven, Dursun, Ulgen Tekerek, Ozcifci, Yazici, Turanli, Kendirli, Kahveci, Yetimakman, Citak, Şik, Bingol, Aygun, Durak, Yilmaz, Bugrul, Sari, Tekguç, Albayrak, Yener, Agin, Soydan, Yildizdas, Dilek, Yalindag, Incekoy-Girgin, Alacakir, Tutunculer, Arslanaoglu, Aydin, Bilgin, Simsek and Dinleyici.)

Herein, we aimed to present a child with extremely severe hypertriglyceridemia (ESHTG) secondary to diabetic ketoacidosis concomitant with type IX glycogen storage disease (GSD). Extremely severe hypertriglyceridemia (10 700 mg/dL) was detected through the apparent lipemic appearance of the sampled blood in a 17-year-old male patient with severe diabetic ketoacidosis. In spite of insulin infusion, the patient's clinical condition deteriorated to acute pancreatitis. Single sessions of therapeutic plasma exchange (TPE) along with insulin treatment have successfully intercepted the progression of the state of acute pancreatitis. The patient was also diagnosed with type IX GSD on the basis of the genetic analyses performed for the potential underlying metabolic diseases. In conclusion, underlying metabolic diseases, such as glycogen storage disease, should be investigated in patients with diabetic ketoacidosis accompanied by severe hypertriglyceridemia. If ESHTG does not relieve despite insulin infusion, and/or acute pancreatitis occurs as a complication, TPE should be kept in mind., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Aim: The causes of gonadotropin-independent precocious puberty are diverse, and often have overlapping clinical and biochemical features. With the exception of congenital adrenal hyperplasia (CAH), disorders that cause gonadotropin-independent precocious puberty (GIPP) are uncommon. The literature is devoid of any large-scale studies on the etiologic distribution of GIPP. The aim of this study was to determine the frequency of each etiology in a cohort of patients with GIPP (excluding those with CAH), and to evaluate the clinical and laboratory features of these patients., Materials and Methods: This multicenter, nationwide web-based study collected data on patients who presented with non-CAH GIPP in Turkey., Results: Data were collected for 129 patients (102 girls and 27 boys) from 29 centers. Based on the data collected, the estimated prevalence of non-CAH GIPP in the studied population was 14 in 1 000 000 children. Functional ovarian cyst was the most common etiology, accounting for 37% of all cases, followed by McCune-Albright syndrome (MAS) (26%). Among the patients with MAS, 11.7% had fibrous dysplasia, 32.3% had café-au-lait spots, and 52.9% had both. Human chorionic gonadotrophin-secreting tumors included choriocarcinoma of the liver, hepatoblastoma, and germ cell tumors of the sellar-suprasellar region and mediastinum. Patients with adrenocortical tumors presented at an earlier age than those with other etiologies. Ovarian tumors included mature cystic teratoma, dysgerminoma, juvenile granulosa tumor, and steroid cell tumor. Despite overlapping features, it was possible to identify some unique clinical and laboratory features associated with each etiology., Conclusion: This largest cohort of patients with non-CAH GIPP to date yielded an estimation of the frequency of non-CAH GIPP in the general pediatric population and showed that girls were affected at a rate 4-fold greater than that of boys owing to functional ovarian cysts and MAS, which were the two most common etiologies. The data collected also provided some unique characteristics associated with each etiology.

Duplications of 20q are rare. Here we report a 15 years old boy with de novo duplication of 17.1 Mb at chromosome 20q. We made a comparison with the other isolated 20q duplication cases. There are phenotypic similarities between the patients who have the same affected chromosomal regions. We also showed a clinical follow up of the patient. There may be a relationship with Glaucoma and Graves disease between the chromosomal region and these diseases may occur at the other patients when they get older.

Purpose: In a chick model of gastroschisis, we aimed to investigate the morphological/cellular, molecular, and ultrastructural changes taking place in gastroschisis-related intestinal damage (GRID)., Methods: 13-Day fertilized eggs were divided into two groups., Control Group: chorio-amnio-allontoic membranes opened and abdominal wall exposed. Gastroschisis group: an anterior abdominal wall defect created after opening membranes. Embryos from both groups were surgically removed on post-fertilization day 19. Intestinal samples were obtained for histopathology, immunohistochemistry, molecular biology, and electron microscopy., Results: The histopathological grade of intestinal damage which primarily involved mucosal structures was significantly higher in the gastroschisis group when compared to the control group (p<0.001). Immunohistochemically, E-cadherin and synaptophysin immunoreactivity in the gastroschisis group was significantly lower than control group (p<0.05 and p<0.01, respectively), whereas there was no significant difference in laminin and type-4 collagen immunoreactivity between the groups (p>0.05). Molecular analyses indicated a significant decrease in NFκB and IκB expression in the gastroschisis group (p<0.05 and p=0.001, respectively). Electron microscopy showed that the gastroschisis group had considerable ultrastructural damage, manifested by apoptosis in all layers., Conclusions: GRID affected all layers but was more prominent in mucosa. The damage may depend on E-cadherin and synaptophysin downregulation. Increased apoptotic activity, associated with decreased NFκB and IκB expression, may be an important component of this multifactorial damaging process., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Purpose: The pathogenesis of pulmonary hypoplasia associated with congenital diaphragmatic hernia (CDH) remains unclear. Interactions between the epithelium and surrounding mesenchyme play an important role in normal lung morphogenesis. Epimorphin, a stromal protein, plays a role in epithelial morphogenesis and lung branching, both of which are involved in pulmonary hypoplasia. In this study, we aimed to examine the relationship between epimorphin and pulmonary hypoplasia associated with CDH in an animal model., Methods: Time-pregnant rats were exposed to nitrofen or vehicle on gestational day 9 (D9). Fetuses were harvested on D16 and D20, and were divided into control, hypoplastic lungs with CDH (CDH+), and hypoplastic lungs without CDH (CDH-). Both lungs of each fetus were removed and subjected to morphometric and molecular biologic analyses. Lung-to-body weight ratios were calculated. Pulmonary RNA was extracted, and relative mRNA level of epimorphin was determined by quantitative real-time PCR (qRT-PCR). Protein expression of epimorphin was investigated by Western blotting., Results: In groups D16 and D20, lung-to-body weight ratios in subgroups CDH+ were significantly lower than those of controls and CDH-. The relative mRNA expression levels of epimorphin were significantly increased in both lungs in subgroup CDH+ compared with controls and CDH- on D16. Pulmonary epimorphin gene expression levels were significantly decreased in CDH+ group on D20 compared to controls. Western blotting confirmed the qRT-PCR results showing decreased pulmonary epimorphin protein expression in CDH+ hypoplastic lungs compared to controls on D20., Conclusion: Our study shows that there is an association between the epimorphin expression and pulmonary hypoplasia associated with CDH. Although the cause-effect relationship is far from being established, epimorphin-related mechanisms have a more critical role in early (D16) developmental stage.

Purpose: Antiepileptic drugs may affect the endocrine system. We investigated the effects of valproic acid and topiramate on the levels of insulin, c-peptide and adipocytokines in pre-pubertal patients with idiopathic partial and generalized epilepsy., Methods: Forty-one children with epilepsy were included. The patients were divided into two groups (valproic acid; n = 21, topiramate; n = 20). The weight, height, body mass index and homeostasis model assessment of insulin resistance (HOMA-IR) were recorded and insulin, c-peptide, leptin, neuropeptide Y, adiponectin, visfatin and resistin levels were determined at 0, 6 and 12 months of therapy., Results: In the valproate group, weight and height increased significantly. Seven of 21 patients were overweight at the end of one year. Leptin was higher in the overweight subgroup. Although insulin and HOMA-IR increased (p < 0.05), none of the patients showed hyperinsulinism or IR. Resistin had decreased at the 6th and 12th months (p < 0.05). In the topiramate group, some statistically nonsignificant changes were demonstrated., Conclusion: The mechanisms behind valproate and topiramate-related weight control are still unclear, especially in children. Valproate and topiramate affect the weight, BMI, and insulin, leptin and adipocytokine levels in prepubertal children. We suggest that further studies including more patients with a long follow-up period are necessary to draw a firm conclusion regarding an association between the treatment with these drugs and the levels of leptin, insulin and adipocytokines., (Copyright © 2013 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

We report a patient with a de novo telomeric association between chromosomes 19 and Y in conjunction with mixed gonadal dysgenesis. The patient was first admitted to the clinic because of abnormal external genitalia. Laparoscopic evaluation revealed (1) a rudimentary uterus, one fallopian tube, and a small gonad resembling an ovary on the right side, and (2) an immature fallopian tube, a vas deferens, and a gonad resembling a testis on the left side. Conventional cytogenetic analysis performed on cultivated peripheral blood cells, and tissue obtained from the phallus and a gonadal structure which resembled a testis revealed two different cell lines with the 46,X,tas (Y;19)(p11.3;q13.4) and 45,X karyotype. Y chromosome microdeletion analysis showed that the patient did not have any genomic deletions in the AZFa, b, c, or SRY regions on the long arm of the Y chromosome. This is the first report of a patient with mixed gonadal dysgenesis that is accompanied by a telomeric association between chromosomes 19 and Y with 45,X mosaicism.

Here we present a male newborn with multiple congenital anomalies who also has an extremely rare form of testicular disorder of sex development (DSD). His karyotype was 45X, without any mosaicism. SRY gene was positive by polymerase chain reaction (PCR), and rearranged on distal part of the 7th chromosome by fluorescence in situ hybridization (FISH) analysis. SRY, normally located on the Y chromosome, is the most important gene that plays a role in the development of male sex. SRY gen may be translocated onto another chromosome, mostly X chromosome in the XX testicular DSD. On the other hand very few cases of 45 X testicular DSD were published to date. Other clinical manifestations of our patient were compatible with distal 7 q deletion syndrome. To the best of our knowledge this is the first case of 45 X testicular DSD with SRY gene rearranged on the 7th autosomal chromosome.

Patients with DAX-1 gene mutations on chromosome Xp21 usually present with adrenal hypoplasia congenita and hypogonadotropic hypogonadism. Yet, neither correlation between the type of mutation and the age of onset of the disease nor mechanism of the mutation on puberty is fully understood. Here, we report a novel non-sense p.Gln208X mutation in the amino terminal domain of the DAX-1 gene observed in a large family with three boys presenting with adrenal manifestations at different ages. Furthermore, two boys developed spontaneous puberty that failed to progress at similar ages, whereas the other boy developed precocious puberty at 10 month of age. The unique structure of the DAX-1 gene may explain this phenotypic variability. However, more studies are needed to understand the role of the DAX-1 gene on development of the adrenal gland and hypothalamus-pituitary-gonadal axis.

Renal cell carcinoma is very rare in childhood. It is a different entity from its adult counterpart. We report a case of renal cell carcinoma presented with hypocalcemia in a 16-year-old girl that is, to the best of our knowledge, the first such published incident. The patient was treated by radical nephrectomy with regional lymphadenectomy. Hypocalcemia amended after surgery. Neither chemotherapy nor radiotherapy was given. She has remained disease free for 34 months.

Ectopic intrathyroidal thymus tissue that may be present as a thyroid nodule is rarely reported. We present a case of a 4-year-old boy with a solitary thyroid nodule. Real-time thyroid ultrasound showed a calcified nodule in the right lobe. Complete blood count, serum calcitonin, and thyroglobulin concentration were normal and antithyroid antibodies were negative. Fine-needle aspiration (FNA) biopsy was revealed as inadequate for cytological examination. During his follow-up, nodular enlargement was found, and the patient was subjected to surgical total excision of the right lobe of the thyroid gland. Pathological examination showed an ectopic intrathyroidal thymus tissue. In childhood, ectopic intrathyroidal thymus tissue can present as an enlarging microcalcified thyroid nodule that may mimic thyroid cancer and may grow during follow-up.

Osteopenia is a term to define bone density that is not normal but also not as low as osteoporosis. By definition from the World Health Organization osteopenia is defined by bone densitometry as a T score -1 to -2.5. There are many causes for osteopenia including calcium and vitamin D deficiency and inactivity. Genetics plays an important role in a person's bone mineral density and often Caucasian women with a thin body habitus who are premenopausal are found to have osteopenia. Correction of calcium and vitamin D deficiency and walking 3 to 5 miles a week can often improve bone density in the hip and spine. There are a variety of pharmaceutical agents that have been recommended for the treatment of osteopenia and osteoporosis including hormone replacement therapy, selective estrogen receptor modulator therapy, anti-resorptive therapy. In addition patients with osteoporosis who have failed anti-resorptive therapy can have a significant improvement in their bone density with anabolic therapy.

It is unknown whether noncomplicated acute appendicitis cause bacterial translocation. In this study, we aimed to test development of the bacterial translocation in the patients who were operated for acute appendicitis. In this prospective study, 10 control patients who underwent elective operations because of other reasons, and 18 patients with noncomplicated acute appendicitis were evaluated. No patients took prophylactic antibiotic. After laparotomy, samples were obtained from peritoneal leaf just close to wound edge, and peritoneal swab culture from right paracolic region. Before appendectomy, a mesenteric lymph node (MLN) adjacent to the terminal ileum was taken out. Tissue samples were placed in a sterile container for microbiological analysis, and 10% formalin for histopathological analysis. Control samples had no bacterial translocation. Only 3 of 18 (16.6%) patients with appendicitis included bacterial translocation to MLN. There was no significant difference between both groups. No bacterial colonization was detected in the peritoneal tissue and peritoneal swab culture. Peritoneal tissue injury score was 2 +/- 1.4 in controls and 2.8 +/- 1.7 in the patients with appendicitis (P>0.05). MLN injury score was 2.5 +/- 1.3 in controls and 3.2 +/- 1.5 in the patients with appendicitis (P>0.05). No patient developed wound and systemic infection. No significant bacterial translocation frequency and tissue injury score was identified in the children with noncomplicated acute appendicitis. This result suggests that antibiotic prophylaxis may be unnecessary in such patients.

Objective: To evaluate our data related with epidemiologic features, clinical presentation, and laboratory findings in children with type 1 diabetes mellitus (DM1) and to compare specific characteristics of immune-mediated subtype (DM 1A) with idiopathic one (DM 1B)., Methods: We classified 115 children with DM1 according to the presence (DM 1A, n=77) or absence (DM 1B, n=38) of diabetes-related autoantibodies in Akdeniz University Hospital, Turkey from January 2000 to December 2005., Results: A total of 43 patients (37%) in the whole group, had onset of DM1 during the winter months and the lowest frequency occurred in summer (p<0.005). The duration of breast-feeding, introduction time of cow's milk, and seasonal distribution of birth-month or onset of disease did not significantly differ in both groups. When compared with patients who had no documented honeymoon period, the patients who had a documented honeymoon period had lower HbA1c levels (p<0.01) at the onset. A large percentage of patients with DM 1A were positive for glutamic acid decarboxylase antibody (GAD65)., Conclusion: There was no significant difference between patients with DM 1A and DM 1B with respect to epidemiologic features, and clinical presentation suggested that these factors do not play a major role either in creating a disease-initiating effect or in the development of islet autoimmunity. However, determination of GAD65 with HbA1c levels at the onset of the disease may ensure some useful information regarding clinical course.

Unlabelled: We report a 40-d-old female infant who presented to our clinic with afebrile generalized convulsive episodes due to severe hypomagnesaemia with secondary hypocalcaemia. Laboratory investigations revealed mild hypoparathyroidism, which was to return normal following magnesium (Mg) treatment, and normal fractional renal excretion of Mg (0.8%). After the diagnosis of primary hypomagnesaemia was established, the patient was discharged with oral Mg subcarbonate and intramuscular Mg sulphate. On regular follow-up until the age of 4 y, the child was asymptomatic. Mean serum calcium levels remained normal, while mean serum Mg levels remained subnormal. During this 4-y period, oral Mg dose was gradually increased while the doses and frequency of administration of parenteral Mg were decreased. Additionally, oral Mg subcarbonate was switched to Mg citrate because of its side effects. Finally, parenteral Mg was discontinued as the dosage of oral Mg supplementation reached a level of 90 mg/kg/d elemental Mg citrate, without any gastrointestinal side effects. Some screening tests were performed to evaluate the complications of chronic hypomagnesaemia. Bone age, bone densitometry, ECG and renal sonography were all normal. Our patient is now 5 y old and symptom free., Conclusion: Treatment with high doses of oral Mg was successful in keeping our patient symptom free and normocalcaemic, but cannot fully normalize serum Mg concentrations. Thus, during the regular follow-up of patients with primary hypomagnesaemia, the main target must be to increase oral Mg supplementation to a dosage which can maintain normocalcaemia rather than normomagnesaemia.

Purpose: Midline dorsal plication is an efficient and safe surgical technique to correct chordee. We investigated the efficacy of midline dorsal plication for recurrent chordee in complicated hypospadias reoperations., Materials and Methods: We retrospectively evaluated the charts of 25 boys who underwent reoperation between 1999 and 2004 due to complications of primary hypospadias repair other than meatal stenosis. A total of 15 cases were initially managed elsewhere for primary repair or complications. The etiology of recurrent chordee was defined at surgical correction. When recurrent chordee was noted a midline dorsal plication was performed., Results: Of 25 patients 10 had previously undergone chordee repair. Nine of these patients were observed to have recurrent chordee and 1 had de novo chordee. A total of 10 patients had recurrent or delayed onset chordee. Mean patient age at primary repair was 6.28 years (range 1 to 33). Mean age at last operation for chordee was 15.9 years (range 4 to 66). Mean interval to recurrent chordee was 6 years (range 1 to 16), excluding a 66-year-old blind patient who did not know when recurrent chordee developed. Five patients had chordee recur before puberty at a mean interval of 2.6 years. Mean reoperation rate was 2.4 for recurrent chordee cases and 2.6 for chordee-free cases. Mean followup after midline dorsal plication for recurrent chordee repair was 22 months (range 8 to 56), while mean followup in pubertal and postpubertal cases was 20 months. No recurrence of chordee or surgery related morbidity was observed after recurrent chordee repair by midline dorsal plication., Conclusions: Chordee may recur during puberty following successful chordee repair. The midline dorsal plication technique is simple, efficient and safe even in patients who have undergone multiple surgeries for hypospadias and chordee repair.

Background: We aimed to randomly compare intubating conditions, recovery characteristics and neuromuscular effects of single dose of mivacurium (0.2 mg.kg(-1)) during sevoflurane vs. propofol anesthesia in 60 healthy children, undergoing inguinal surgery., Methods: All children were randomly allocated to receive 2 mg.kg(-1) propofol iv or sevoflurane 8% inspired concentration for induction of anesthesia. Anaesthesia was maintained with 66% nitrous oxide in oxygen and 100-120 microg.kg(-1) propofol or sevoflurane approximately 2-3% inspired concentration with controlled ventilation. The ulnar nerve was stimulated at the wrist by a train-of four (TOF) stimulus every 20 s and neuromuscular function was measured at the adductor pollicis. When the response to TOF was stable, 0.2 mg.kg(-1) mivacurium was given. The trachea was intubated successfully at the first attempt in all patients., Results: Onset time following a single dose of mivacurium was shorter in the sevoflurane group (2.99 min), than in the propofol group (4.42 min). The times to 25, 50, 75, and 90% recovery were significantly longer in the sevoflurane group (13.1, 15.7, 18.6, and 21.2 min, respectively) than in the propofol group (11.4, 13.2, 14.4, and 17.2 min respectively). TOF ratios of 50, 70, and 90% were significantly occurred later in sevoflurane group than propofol group., Conclusions: Our results indicate that when compared with propofol group, the sevoflurane group had an accelerated onset and a delayed recovery of neuromuscular block induced by mivacurium in children.

Purpose: Data about the differences between the vascularization of normal and hypospadiac prepuce are lacking. We investigated the course of the preputial arterial blood vessels in normal controls and children with hypospadias by using transillumination, arterial methylene blue injection and 3-dimensional (3-D) reconstruction of serial histological sections focusing on arterial vessels., Materials and Methods: Prepuce of 48 normal controls and 15 children with hypospadias was transilluminated by a front and back lighting technique and then photographed. All of the normal and 12 of hypospadiac prepuces not used for urethroplasty or penile body skin reconstruction were removed. The blood vessels of normal prepuce were also identified after arterial injection of methylene blue. Selected prepuce of normal controls and children with hypospadias was serially sectioned, and arterial and venous vessels were histologically distinguished. A 3-D computer reconstruction of the arterial system of normal and hypospadiac prepuces was performed., Results: We confirmed the reliability of the transillumination technique to describe the arterial vascular anatomy of the prepuce by comparing the transillumination to methylene blue injection and 3-D reconstruction of histological sections. We classified the arterial vascular anatomy of normal prepuce as 1 artery predominant (41.67%), 2 arteries predominant (25%), H-type arching artery (12.5%) and net-like arterial system (20.83%). However, hypospadiac prepuce revealed a net-like arterial system more frequently (50%). We noted that the frequency of net-like arterial system was higher in more severe hypospadiac prepuce., Conclusions: Understanding the differences between normal and hypospadiac prepuce vascular anatomy is germane to hypospadias surgery. The arterial blood supply of the hypospadiac prepuce is different than normal. A better knowledge of the vascular anatomy of the hypospadiac prepuce may improve the surgical results of hypospadias repair.