ABSTRACT
OBJECTIVES: To present the results of feminising genitoplasty done in female patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. DESIGN: Case series. SETTING: A tertiary referral centre in Hong Kong. PATIENTS: Female patients with congenital adrenal hyperplasia undergoing corrective surgery for virilisation between 1993 and 2012. MAIN OUTCOME MEASURES: The operative result was judged with a scoring system (1-3) for four areas: appearance of clitoris, labia and vagina, plus requirement for revision surgery. RESULTS: A total of 23 female patients with congenital adrenal hyperplasia with a median age of 17.5 (range, 1.5-33.8) years were identified. Of these individuals, 17 presented in the neonatal period and early infancy, of which four had an additional salt-losing crisis. Six patients-including four migrants from mainland China-were late presenters at a median age of 2 (range, 0.5-14) years. Twenty-two patients had corrective surgery at a median age of 2 (range, 1-14) years. Clitoral reduction was performed in all, and further surgery in 21 patients. The additional surgery was flap vaginoplasty in 10 patients, a modified Passerini procedure in six, and a labial reconstruction in five; one patient with prominent clitoris was for observation only. Minor revision surgery (eg mucosal trimming) was required in three patients; a revision vaginoplasty was done in one individual. Of the 23 patients, 18 (78%) with a median age of 20 (range, 9.3-33.8) years participated in the outcome evaluation: a 'good' outcome (4 points) was seen in 12 patients and a 'satisfactory' (5-9 points) result in five patients. CONCLUSIONS: Nearly three quarters of our cohort (n=17) presented with classic virilising form of 21-hydroxylase deficiency. Only four (25%) patients experienced a salt-losing crisis. Female gender assignment at birth was maintained for all individuals in this group. 'Good' and 'satisfactory' outcomes of surgery were reported in nearly all participants.
Subject(s)
Adrenal Hyperplasia, Congenital/surgery , Adolescent , Adult , Child , Child, Preschool , Cohort Studies , Female , Hong Kong , Hospitals , Humans , Infant , Plastic Surgery Procedures , Steroid 21-Hydroxylase , Treatment OutcomeABSTRACT
An acquired ileal atresia is a rare occurrence. A 3-week-old neonate is presented, who developed postnatally a type 2 ileal atresia and an ileal stenosis within a pre-existing internal hernia secondary to an adhesion band. The literature reports a total of eight cases (4 females) with acquired ileal atresia in babies ranging in age from 3 weeks to 2 years (median 4 months). Mechanical forces (eg, adhesion band, intussusception and volvulus) onto the intestine are most frequently (75%) implicated as the primary event. The distal ileum is most often affected and a type 3A atresia is identified in six of eight (75%) cases.
Subject(s)
Ileal Diseases/etiology , Infant, Newborn, Diseases/etiology , Intestinal Atresia/etiology , Constriction, Pathologic , Female , Humans , Ileal Diseases/pathology , Ileal Diseases/surgery , Ileum/pathology , Ileum/surgery , Infant, Newborn , Infant, Newborn, Diseases/pathology , Infant, Newborn, Diseases/surgery , Intestinal Atresia/pathology , Intestinal Atresia/surgery , PressureABSTRACT
A study of postantibiotic effects (PAE) in vitro of amikacin and ofloxacin on Mycobacterium fortuitum isolates from sternotomy wounds by use of the dilution method for drug removal showed that both drugs exhibited good bactericidal activities, with the PAE of amikacin lasting from 13.5 to 27.6 h and the PAE of ofloxacin lasting from 1.2 to 5.0 h. These laboratory results concur with our experience of the efficacy of once-daily dosing with these drugs in the treatment of infections caused by M. fortuitum. These data may have therapeutic implications in guiding the scheduling of the administration of drugs in these infections, which require a long duration of therapy.
