Gender assignment and hormonal treatment for disorders of sexual differentiation.

Abstract:

PURPOSE:To study the gender assignment and hormonal treatment advocated for disorders of sexual differentiation (DSD). METHODS:A study was done on patients who were reviewed in the Pediatric Intersex Clinic to evaluate the pattern of gender assignment and hormonal treatment advocated. RESULTS AND CONCLUSION:The patients included male pseudohermaphrodite (MPH) 169; congenital adrenal hyperplasia (CAH) 91; mixed gonadal dysgenesis (MGD) 29; true hermaphrodite (TH) 25; pure gonadal dysgenesis (PGD) 2; persistent mullerian duct syndrome (PMDS) 2 and others (micropenis, severe hypospadias with cryptorchidism, 46XX male) 39. Out of 91 cases of CAH, 70 (76.9%) were on steroids (prednisolone, hydrocortisone) and/or mineralocorticoids (fluoricortisone) for adrenal suppression. Out of 146 cases of male pseudohermaphrodite and 21 cases of true hermaphrodite and 3 cases of mixed gonadal dysgenesis reared as males, testosterone was given for local application for phallic growth in 101 and/or as systemic injection for mental makeup after puberty in 41 cases. Systemic testosterone injection was also given for 7 cases of CAH reared as males. Out of 26, 15 cases with mixed gonadal dysgenesis and one out of 2 cases of pure gonadal dysgenesis that attained puberty after being reared as females, after female genitoplasty, were given conjugated oestrogen (Premarin) supplemented with progesterone, as the uterus was preserved. For 12 post-pubertal cases of complete androgen insensitivity syndrome (AIS), only premarin was given as there was no uterus. Growth hormone and Gn RH analogue was given in 2 patients with CAH to tide over the early bone maturation induced by hormones with equivocal results. Thus judicious hormonal supplementation based upon type of DSD and gender assigned can provide a psychological and cosmetic benefit to patients with DSD.

journal_name

Pediatr Surg Int

authors

Sharma S,Gupta DK

doi

10.1007/s00383-008-2232-7

subject

Has Abstract

pub_date

2008-10-01 00:00:00

pages

1131-5

issue

10

eissn

0179-0358

issn

1437-9813

journal_volume

24

pub_type

杂志文章
  • Increased intracellular levels of calcitonin gene-related peptide-like immunoreactivity in pulmonary endocrine cells in an experimental model of congenital diaphragmatic hernia.

    abstract::A congenital diaphragmatic hernia (CDH) model was induced in pregnant rats following administration of 100 mg nitrofen. The fetuses were stored and fixed in Bouin's solution for 24 h after caesarean section at term. After fixation, the lungs were dissected out. Immunostaining of the CDH lungs and controls with rabbit ...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/BF00180080

    authors: Yamataka T,Puri P

    更新日期:1996-08-01 00:00:00

  • Sibling vesicoureteral reflux in twins.

    abstract:AIM OF STUDY:Few studies have evaluated the incidence of familial vesicoureteral reflux (VUR) among multiple gestation births. The purpose of this study was to determine the incidence and characteristics of VUR in twins in a large cohort of families with VUR. METHODS:Between 1998 and 2010, data were collected on 251 f...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-010-2821-0

    authors: Giannotti G,Menezes M,Hunziker M,Puri P

    更新日期:2011-05-01 00:00:00

  • Immature enteric neurons in Ncx/Hox11L.1 deficient intestinal neuronal dysplasia model mice.

    abstract:AIM:The Ncx/Hox11L.1 gene is required for adequate development of enteric neurons in mice and Ncx/Hox11L.1 deficient (Ncx-/-) mice are used as a model for human intestinal neuronal dysplasia (IND) because of similar histopathology (hyperganglionosis), however, some 50% of Ncx-/- mice develop megacolon with a caliber ch...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-009-2451-6

    authors: Kato Y,Miyahara K,Hatano M,Hasegawa Y,Seki T,Frykman PK,Kusafuka J,Lane GJ,Yamataka A

    更新日期:2009-11-01 00:00:00

  • Is renal scintigraphy really a necessity in the routine diagnosis of congenital solitary kidney?

    abstract:BACKGROUND:For a definitive diagnosis of congenital solitary kidney, renal scintigraphy is suggested as being the gold standard of ruling out ectopic functioning renal tissue, possibly missed by ultrasound. The aim of our study was to test ultrasonography precision in comparison with renal scintigraphy on a larger coho...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-019-04478-1

    authors: Grabnar J,Rus RR

    更新日期:2019-06-01 00:00:00

  • Embryogenesis of a sac containing a Meckel's diverticulum.

    abstract::The embryogenesis of a previously unreported mesothelial pouch containing a Meckel's diverticulum is presented. ...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/BF01076960

    authors: Khatri HL,Singh RB,Sharma R,Aggarwal N,Singh K

    更新日期:1997-07-01 00:00:00

  • Surgical intervention for congenital diaphragmatic hernia: open versus thoracoscopic surgery.

    abstract:PURPOSE:To compare open repair (OR) with thoracoscopic repair (TR) for congenital diaphragmatic hernia (CDH) in neonates. METHODS:Twenty-four neonatal CDH cases diagnosed prenatally or within 6 h of birth at our institute from 2002 to 2012 with mild pulmonary hypertension managed without inhaled nitric oxide, were stu...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-013-3382-9

    authors: Tanaka T,Okazaki T,Fukatsu Y,Okawada M,Koga H,Miyano G,Ogasawara Y,Lane GJ,Yamataka A

    更新日期:2013-11-01 00:00:00

  • Differences in the characteristics and management of pyriform sinus fistula between neonates and young children.

    abstract:PURPOSE:Pyriform sinus fistula (PSF), which originates from the third or fourth branchial pouch remnant, is relatively rare. In young children and adults, it is characterized by lateral neck infections and suppurative thyroiditis, while neonatal PSF presents as a large cervical cystic mass and causes respiratory distre...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-011-3008-z

    authors: Amano H,Uchida H,Sato K,Kawashima H,Tanaka Y,Takazawa S,Jimbo T

    更新日期:2012-01-01 00:00:00

  • Redo transanal endorectal pull-through: a preliminary study.

    abstract::The aim of this study is to evaluate the safety and efficacy of the redo transanal endorectal pull-through (TEPT) for patients with persistent symptoms after pull-through for Hirschsprung's disease (HD). Seven children were included in the study, their ages ranged from 2.5 to 6 years (four males and three females). Th...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-006-1837-y

    authors: Gobran TA,Ezzat A,Hassan ME,O'Neill J

    更新日期:2007-02-01 00:00:00

  • Prenatally closed gastroschisis with midgut atresia.

    abstract::Spontaneous prenatal closure of gastroschisis (GS) is rare and usually associated with atresia of the midgut. We describe a case of GS diagnosed at 20 weeks' gestation that resolved spontaneously in utero. At delivery the infant had an ileus. A laparotomy with a jejunocolostomy was performed, but she died at 2 months ...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章,评审

    doi:10.1007/s00383-002-0815-2

    authors: Basaran UN,Inan M,Gücer F,Yardim T,Pul M

    更新日期:2002-09-01 00:00:00

  • Analysis of patients with congenital diaphragmatic hernia requiring pre-operative extracorporeal membrane oxygenation (ECMO).

    abstract::The purpose of this report was to analyze the patients requiring preoperative extracorporeal membrane oxygenation (ECMO) as the most critical group of patients with congenital diaphragmatic hernia (CDH) and to identify any special features. Over the past 11 years, out of 72 neonates with CDH admitted before 24 h of ag...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s003830050428

    authors: Nagaya M,Kato J,Niimi N,Tanaka S,Tanaka T

    更新日期:1998-11-01 00:00:00

  • Pulmonary artery size has prognostic value in low birth weight infants with congenital diaphragmatic hernia.

    abstract:AIM:The aim of this study was to examine the relationship between birth weight, pulmonary artery (PA) size, and outcome in congenital diaphragmatic hernia (CDH) to establish if PA size has prognostic value. METHODS:The subjects for this study were 39 consecutive left-sided CDH patients treated at our institution accor...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-011-2899-z

    authors: Takahashi T,Koga H,Tanaka T,Shoji H,Takeda S,Shimizu T,Lane GJ,Yamataka A,Okazaki T

    更新日期:2011-08-01 00:00:00

  • Home intravenous versus oral antibiotics following appendectomy for perforated appendicitis in children: a randomized controlled trial.

    abstract:PURPOSE:To compare the effect of home intravenous (IV) versus oral antibiotic therapy on complication rates and resource utilization following appendectomy for perforated appendicitis. METHODS:This was a randomized controlled trial of patients aged 4-17 with surgically treated perforated appendicitis from January 2011...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章,随机对照试验

    doi:10.1007/s00383-018-4343-0

    authors: Arnold MR,Wormer BA,Kao AM,Klima DA,Colavita PD,Cosper GH,Heniford BT,Schulman AM

    更新日期:2018-12-01 00:00:00

  • Evidence-based operations in paediatric surgery.

    abstract::It has been assumed that only 10% of medical interventions are supported by solid scientific evidence. The aim of this study was to determine the type of research evidence supporting operations in a tertiary referral paediatric surgical unit. All patients admitted over a 4-week period to two surgical firms were enroll...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s003830050332

    authors: Baraldini V,Spitz L,Pierro A

    更新日期:1998-07-01 00:00:00

  • Plasma-to-lumen clearance of para-aminohippurate can replace 51Cr EDTA clearance in the evaluation of intestinal mucosal injury.

    abstract::Intestinal mucosal injury of various degrees occurs in many clinical situations and is initially evidenced by altered mucosal permeability. The latter may be assessed in animal models by determination of plasma-to-intestinal lumen clearance of specific molecules, usually chromated 51Cr EDTA. The purpose of this study ...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s003830050261

    authors: Udassin R,Haskel Y,Seror D,Welbourne TC

    更新日期:1998-03-01 00:00:00

  • Decidual β-carotene-15,15'-oxygenase-1 and 2 (BCMO1,2) expression is increased in nitrofen model of congenital diaphragmatic hernia.

    abstract:BACKGROUND:Retinoids are essential for fetal and lung development. Beta-carotene(BC) is the main dietary retinoid source and beta-carotene-15,15'-oxygenase-1 and 2 (Bcmo1,2) is the primary enzyme generating retinoid from BC in adult mammalian tissues. Placenta has a major role in the retinol homeostasis in fetal life: ...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-014-3621-8

    authors: Takahashi H,Kutasy B,Pes L,Paradisi F,Puri P

    更新日期:2015-01-01 00:00:00

  • Management of gastrointestinal perforation from blunt and penetrating abdominal trauma in children: analysis of 96 patients.

    abstract:AIM:The objective of the present study was to evaluate the diagnostic methods, concomitant organ injuries, factors affecting mortality and morbidity, treatment methods, and outcomes of patients treated for traumatic gastrointestinal (GI) perforation. MATERIALS AND METHODS:We conducted a retrospective review of the med...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-016-3963-5

    authors: Arslan S,Okur MH,Arslan MS,Aydogdu B,Zeytun H,Basuguy E,Icer M,Goya C

    更新日期:2016-11-01 00:00:00

  • Pancreatic surgery for tumors in children and adolescents.

    abstract:PURPOSE:Pancreatic neoplasms are uncommon in children. This study sought to analyze the clinical and pathological features of surgically resected pancreatic tumors in children and discuss management strategies. METHODS:We conducted a retrospective review of patients ≤21 years with pancreatic neoplasms who underwent su...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-016-3925-y

    authors: Sacco Casamassima MG,Gause CD,Goldstein SD,Abdullah F,Meoded A,Lukish JR,Wolfgang CL,Cameron J,Hackam DJ,Hruban RH,Colombani PM

    更新日期:2016-08-01 00:00:00

  • Surgeon-performed bedside ultrasound to assess volume status: a feasibility study.

    abstract:PURPOSE:Rapid assessment of volume status in children is often difficult. The purpose of this study was to evaluate the feasibility of surgeon-performed ultrasound to assess volume status in patients with hypertrophic pyloric stenosis. METHODS:Ultrasounds were performed on admission and before operation. The diameters...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-015-3798-5

    authors: Wyrick DL,Smith SD,Burford JM,Swearingen CJ,Dassinger MS

    更新日期:2015-12-01 00:00:00

  • In vitro and in vivo evaluation of photodynamic techniques for the experimental treatment of human hepatoblastoma and neuroblastoma: preliminary results.

    abstract:PURPOSE:The purpose of this study was to test the susceptibility of human hepatoblastoma and neuroblastoma cells to photodynamic diagnostics (PDD) and photodynamic therapy (PDT) using 5-aminolevulinic acid (5-ALA) as a photosensitizer. METHODS:Cell cultures of human hepatoblastoma (HuH6) and neuroblastoma (MHH-NB-11) ...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-008-2275-9

    authors: Bergmann F,Stepp H,Metzger R,Rolle U,Johansson A,Till H

    更新日期:2008-12-01 00:00:00

  • Multiple trauma in pediatric patients.

    abstract::We analyzed the causes and diagnoses, the treatment, short and long-term outcome of a consecutive series of 70 pediatric polytrauma patients. From 1989 to 1996, 70 children (aged 10 months to 16 years, mean 7.4 years) presented with multiple trauma. A follow-up investigation was performed 4.2 years (mean) after the ac...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-003-0954-0

    authors: Schalamon J,v Bismarck S,Schober PH,Höllwarth ME

    更新日期:2003-08-01 00:00:00

  • Successful treatment of severe refractory anastomotic stricture in an infant after esophageal atresia repair by endoscopic balloon dilation combined with systemic administration of dexamethasone.

    abstract::A boy with a history of esophageal atresia repair received an esophagoesophagostomy at 14 months for a severe refractory anastomotic stricture, which had already required repeated balloon dilation with intralesional dexamethasone injection. Anastomotic leakage and stricture was again evident after the second surgery, ...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-009-2367-1

    authors: Hishiki T,Kouchi K,Saito T,Terui K,Sato Y,Mitsunaga T,Nakata M,Yoshida H

    更新日期:2009-06-01 00:00:00

  • Crohn's disease in a Meckel's diverticulum: report of a case.

    abstract::Meckel's diverticulum has previously been reported to be present in patients with Crohn's disease. However, the finding is typically an incidental one, and involvement of the diverticulum in the disease process is uncommon. We report a case of an adolescent with known Crohn's disease who presented with symptoms though...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-007-1971-1

    authors: Diamond IR,Hayes-Jordan A,Phillips MJ,Langer JC

    更新日期:2008-03-01 00:00:00

  • The extent of pediatric orthopaedic research in low- and middle-income countries and the impact of academic collaboration on research quality: a scoping review.

    abstract:INTRODUCTION:This review aims to (1) assess the breadth of pediatric orthopaedic research in low- and middle-income countries (LMICs) and (2) determine the impact of academic collaboration (an LMIC and a non-LMIC investigator) in published LMIC research. METHODS:Pediatric orthopaedic clinical studies conducted in LMIC...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章,评审

    doi:10.1007/s00383-018-4412-4

    authors: Ibrahim JM,Liu M,Wu HH,Patel KR,Caldwell AM,Coughlin RR,Morshed S,Shearer DW

    更新日期:2019-03-01 00:00:00

  • Role of laparoscopy in treatment of choledochal cysts in children.

    abstract::Laparoscopy enables surgeons to approach the surgical conditions from a new perspective. Laparoscopic surgery has revolutionized the treatment of choledochal cysts (CDC). Yet, this new technique requires objective evaluations. We have examined the controversies about the CDC dissection, distal common bile duct ligatio...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章,评审

    doi:10.1007/s00383-013-3266-z

    authors: Diao M,Li L,Cheng W

    更新日期:2013-04-01 00:00:00

  • Signalling molecules: clues from development of the limb bud for cryptorchidism?

    abstract::Recent studies of testicular descent suggest not only that the gubernaculum does not initially attach to the scrotum, but also that it must migrate from the groin. Two findings suggest that the gubernaculum may behave like an embryonic limb bud during this phase. First, the active growth centre is at the distal tip of...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章,评审

    doi:10.1007/s00383-007-1907-9

    authors: Huynh J,Shenker NS,Nightingale S,Hutson JM

    更新日期:2007-07-01 00:00:00

  • Fecal and urinary continence after scope-assisted anorectovaginoplasty for female anorectal malformation.

    abstract:AIM:We assessed continence after scope-assisted anorectovaginoplasty (SARVP) for female anorectal malformation (FARM). METHODS:Five FARM cases were assessed; cases 1 and 2: cloacal malformation; case 3: urogenital sinus, and rectovestibular fistula (RF); case 4: RF, absent vagina, and sacral anomaly; case 5: covered c...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-012-3141-3

    authors: Yamataka A,Goto S,Kato Y,Koga H,Lane GJ,Okazaki T

    更新日期:2012-09-01 00:00:00

  • Congenital lobar emphysema: experience with 21 cases.

    abstract::A retrospective study of all cases (n = 21) of congenital lobar emphysema (CLE) treated at the Royal Hospital, Muscat, from September 1988 to August 1999 was performed. The presentation, diagnosis, treatment, and outcome are reviewed. All 21 patients were nonwhite. Thirteen had left-upper-lobe, 7 right-middle-lobe, an...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s003830000506

    authors: Thakral CL,Maji DC,Sajwani MJ

    更新日期:2001-03-01 00:00:00

  • Toldt's fascia flap: a new technique for repairing large diaphragmatic hernias.

    abstract::The most popular techniques for repairing large diaphragmatic defects involve the use of synthetic patches. We present an alternative approach using living tissue. We reviewed our cases of congenital diaphragmatic hernia (CDH) diagnosed within the first 24 h of life from 1991 to 2003. Toldt's fascia (TF) flap was used...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-004-1267-7

    authors: Okazaki T,Hasegawa S,Urushihara N,Fukumoto K,Ogura K,Minato S,Kawashima S,Kohno S

    更新日期:2005-01-01 00:00:00

  • Esophageal atresia and tracheo-esophageal fistula associated with coarctation of the aorta, CHARGE association, and DiGeorge syndrome: a case report and literature review.

    abstract::CHARGE association and DiGeorge syndrome (DGS) rarely occur together and only eight cases have been reported in the English literature. Two were associated with esophageal atresia (EA) and severe congenital heart anomalies. We report a third case of EA with tracheoesophageal fistula (EA-TEF) associated with coarctatio...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章,评审

    doi:10.1007/s00383-008-2222-9

    authors: Lee KD,Okazaki T,Kato Y,Lane GJ,Yamataka A

    更新日期:2008-10-01 00:00:00

  • Perineal-mound defects.

    abstract::Perineal-mound (PMD) and genital-fold defects cause anorectal malformations (ARM) in both sexes. They are common in females and usually present as a low anomaly (except rectovestibular fistula). They are rare in males and present as an intermediate anomaly. A common embryological explanation for these defects with var...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章,评审

    doi:10.1007/s003830050562

    authors: Sharma AK,Goel D,Kothari SK

    更新日期:1999-01-01 00:00:00