RESUMO
OBJECTIVES: Using high resolution impedance manometry (HRIM), this study characterized the esophago-gastric junction (EGJ) dynamics in children with esophageal atresia (EA). METHOD: Esophageal HRIM was performed in patients with EA aged less than 18 years. Objective motility patterns were analyzed, and EGJ data reported. Controls were pediatric patients without EA undergoing investigations for consideration of fundoplication surgery. RESULTS: Seventy-five patients (M:F = 43:32, median age 1 year 3 months [3 months-17 years 4 months]) completed 133 HRIM studies. The majority (64/75, 85.3%) had EA with distal tracheo-esophageal fistula. Compared with controls, liquid swallows were poorer in patients with EA, as evident by significant differences in distension pressure emptying and bolus flow time (BFT). The integrated relaxation pressure for thin liquid swallows was significantly different between EA types, as well as when comparing patients with EA with and without previous esophageal dilatations. The BFT for solid swallows was significantly different when compared with EA types. CONCLUSIONS: We have utilized HRIM in patients with EA to demonstrate abnormalities in their long-term EGJ function. These abnormalities correlate with poorer esophageal compliance and reduced esophageal peristalsis across the EGJ. Understanding the EGJ function in patients with EA will allow us to tailor long-term management to specific patients.
Assuntos
Impedância Elétrica , Atresia Esofágica , Junção Esofagogástrica , Manometria , Humanos , Atresia Esofágica/cirurgia , Atresia Esofágica/fisiopatologia , Manometria/métodos , Feminino , Lactente , Masculino , Junção Esofagogástrica/fisiopatologia , Pré-Escolar , Criança , Adolescente , Deglutição/fisiologia , Estudos de Casos e Controles , Fístula Traqueoesofágica/cirurgia , Fístula Traqueoesofágica/fisiopatologiaRESUMO
BACKGROUND: Androgens control rodent inguinoscrotal testicular descent during a "programming window" (E12-17). It is proposed that androgen masculinises the genitofemoral nerve, but the mechanism remains unknown. We investigate androgen receptor (AR)-containing target organs: inguinal fat pad (IFP) and mammary bud (MB), supplied by the genitofemoral nerve, hypothesizing that neurotrophic factors may retrogradely masculinise the GFN. METHODS: The IFP, MB and bulbocavernosus (BC) muscle were collected at E12.5/E17.5 from androgen receptor knockout (ARKO) mice and wild-type (WT) littermates. Immunofluorescence and gene expression (RT-qPCR; n = 8/group) for Bdnf, active (TrkB) and inactive (truncated TrkB) receptors, Cntf and Cntf receptor were performed. RESULTS: In the IFP at E12.5, ARKO TrkB mRNA expression was significantly downregulated compared to WT males (p < 0.0026). By E17.5, there was increased Bdnf expression (p < 0.0233). The MB had no differences at E12.5 and had regressed in WT males by E17.5. The BC had no differences at E12.5, but at E17.5 had significant upregulation of Bdnf expression in ARKO, compared to WT males. There were no differences in CNTF or CNTF receptor expression. CONCLUSIONS: Androgen alters active TrkB and Bdnf expression in the IFP. IFP Bdnf signalling may regulate "masculinisation" of the GFN sensory nerves to indirectly control inguinoscrotal testicular descent. IMPACT: Androgen mediates neurotrophin release in the inguinal fat pad in mice, which may facilitate normal testicular descent by masculinising the GFN by peripheral uptake of neurotrophin. This is the first study to examine the role of neurotrophins in testicular descent. This suggests novel steps in the mechanical process of normal testicular descent that may be abnormal in some children with undescended testes.
Assuntos
Androgênios , Receptores Androgênicos , Tecido Adiposo , Androgênios/farmacologia , Animais , Fator Neurotrófico Derivado do Encéfalo , Fator Neurotrófico Ciliar , Humanos , Masculino , Camundongos , Camundongos Knockout , Receptor do Fator Neutrófico Ciliar , TestículoRESUMO
OBJECTIVE: To investigate the quality of life (QoL) impact on primary caregivers of children with esophageal atresia. STUDY DESIGN: We used a prospective cohort study design, inviting primary caregivers of children with esophageal atresia to complete the following questionnaires: Parent Experience of Child Illness (PECI), Patient-Reported Outcomes Measurement Information System (PROMIS) Anxiety, PROMIS Depression, 12-Item Short Form Survey (SF-12), and Pediatric Quality of Life Inventory (PedsQL). The PECI, PROMIS Anxiety and Depression, and SF-12 assessed caregiver QoL, and the PedsQL assessed patient QoL. Patients with Gross type E esophageal atresia served as controls. RESULTS: The primary caregivers of 100 patients (64 males, 36 females; median age, 4.6 years; range, 3.5 months to 19.0 years) completed questionnaires. The majority (76 of 100) of patients had Gross type C esophageal atresia. A VACTERL (vertebral anomalies, anorectal malformation, cardiac anomalies, tracheoesophageal fistula, renal anomalies, limb anomalies) association was found in 30, ≥1 esophageal dilatation was performed in 57, and fundoplication was performed in 11/100. When stratified by esophageal atresia types, significant differences were found in 2 PECI subscales (unresolved sorrow/anger, P = .02; uncertainty, P = .02), in PROMIS Anxiety (P = .02), and in SF-12 mental health (P = .02) and mental component summary scores (P = .02). No significant differences were found for VACTERL association, nor esophageal dilatation. Requirement for fundoplication resulted in lower SF-12 general health score, and lower PedsQL social and physical functioning scores. CONCLUSIONS: We have demonstrated that caring for a child with esophageal atresia and a previous requirement for fundoplication impacts caregiver QoL.
Assuntos
Sobrecarga do Cuidador/psicologia , Atresia Esofágica/enfermagem , Qualidade de Vida , Adolescente , Criança , Pré-Escolar , Atresia Esofágica/psicologia , Feminino , Humanos , Lactente , Masculino , Pais/psicologia , Estudos Prospectivos , Inquéritos e QuestionáriosRESUMO
Esophageal atresia (EA) is the most common congenital esophageal disorder. Radiological imaging facilitates diagnosis, surgical interventions, and follow-up. Despite this, standardized monitoring guidelines are lacking. We aimed to: (1) review the literature regarding radiation burden in children with EA; (2) establish the presence of guidelines for diagnosis and follow-up in children with EA. The systematic review was performed according to PRISMA protocol. Two investigators conducted independent searches (PubMed, Ovid, Cochrane Review) and data extraction. Analysis focused on pre- and post-operative imaging type and frequency to determine the radiation burden. Seven studies met the inclusion criteria (337 patients). All authors agreed upon the need to minimize radiation burden, recommending symptoms-guided management, use of dosimeters, and non-radiating imaging. One study identified a median 130-fold increase in cumulative lifetime cancer risk in children with EA compared with other babies in the special care unit. The most common investigations were X-ray and CT (pre-operatively), and X-ray and contrast swallow (post-operatively). Standardized guidelines focused upon the frequency and type of radiological imaging for children with EA are lacking. Children with EA are subjected to more radiation exposure than the general population. Implementation of non-radiating imaging (ultrasonography, manometry) is recommended.
Assuntos
Atresia Esofágica/diagnóstico , Esofagoplastia/métodos , Radiografia/métodos , Atresia Esofágica/cirurgia , Humanos , Lactente , Manometria , Exposição à RadiaçãoRESUMO
OBJECTIVE: To describe esophageal atresia mortality rates and their associations in our cohort. STUDY DESIGN: Patients with esophageal atresia, managed at The Royal Children's Hospital, Melbourne (1980-2018), who subsequently died, were retrospectively identified from the prospective Nate Myers Oesophageal Atresia database. Data collected included patient and maternal demographics, vertebral anomalies, anorectal malformations, cardiovascular anomalies, tracheoesophageal fistula, renal anomalies, and limb defects (VACTERL) associations, mortality risk factors, and preoperative, operative, and postoperative findings. Mortality before discharge was defined as death during the initial admission. RESULTS: A total of 88 of the 650 patients (13.5%) died during the study period; mortality before discharge occurred in 66 of the 88 (75.0%); mortality after discharge occurred in 22 of the 88 (25.0%). Common causes of mortality before discharge were palliation for respiratory anomalies (15/66 [22.7%]), associated syndromes (11/66 [16.7%]), and neurologic anomalies (10/66 [15.2%]). The most common syndrome leading to palliation was trisomy 18 (7/66 [10.6%]). Causes of mortality after discharge had available documentation for 17 of 22 patients (77.3%). Common causes were respiratory compromise (6/17 [35.3%]), sudden unexplained deaths (6/17 [35.3%]), and Fanconi anemia (2/17 [11.8%]). Of the patients discharged from hospital, 22 of 584 (3.8%) subsequently died. There was no statistical difference in VACTERL association between mortality before discharge (31/61 [50.8%]) and mortality after discharge (11/20 [55.0%]), nor in incidence of twins between mortality before discharge (8/56 [14.3%]) and mortality after discharge (2/18 [11.1%]). CONCLUSIONS: We identified predictors of mortality in patients with esophageal atresia in a large prospective cohort. Parents of children with esophageal atresia must be counselled appropriately as to the likelihood of death after discharge from hospital.
Assuntos
Atresia Esofágica/mortalidade , Bases de Dados Factuais , Atresia Esofágica/classificação , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Alta do Paciente/estatística & dados numéricos , Estudos Retrospectivos , Medição de RiscoRESUMO
OBJECTIVES: Evaluate safety and effectiveness of Polyethylene glycol (PEG) for chronic constipation in children aged younger than 24 months. Identify the optimum dose of PEG to manage chronic constipation in children aged younger than 24 months. METHODS: In this systematic review, Embase, Medline Ovid, Pubmed, and the Cochrane Library were searched between January 1, 2000 and February 1, 2019. Studies investigating functional constipation, in which patients younger than 24 months of age were treated with PEG, were considered as potentially eligible for review. Two authors screened the studies against inclusion/exclusion criteria. Study quality was assessed with the PEDro quality assessment, Cochrane risk of bias tool, and/or the Newcastle-Ottawa Scale. RESULTS: Five studies (2 randomized controlled trials, 3 retrospective chart reviews) satisfied selection criteria (nâ=â459). All studies employed different dosage categories: mean effective maintenance dose, mean initial dose, mean short-term and long-term dose, and mean daily dose. Dosage regimens were variable, with 0.45 to 1.1âgâ·âkgâ·âday for PEG3350 and 0.48 to 0.65âgâ·âkgâ·âday for PEG4000. Adverse effects were transient across all studies for all types of PEG; these included diarrhea and abdominal pain. CONCLUSIONS: This systematic review provided evidence for a lack of reported side effects from PEG for children aged younger than 24 months. Evidence to establish appropriate dosage regimens does not exist.An infographic accompanying this article can be found at http://links.lww.com/MPG/B839.
Assuntos
Constipação Intestinal , Polietilenoglicóis , Criança , Constipação Intestinal/tratamento farmacológico , Humanos , Polietilenoglicóis/efeitos adversos , Estudos RetrospectivosRESUMO
BACKGROUND/AIM: Closure of the processus vaginalis (PV) is considered as the last step of testicular descent. Therefore, patent processus vaginalis (PV), and inguinal hernias are linked to cryptorchidism. As the National Australian incidence of orchidopexy has decreased over the previous 20 years, we aimed to explore the incidence of inguinal herniotomy (including hydrocele) over time in Australia. METHODS: The National Department of Human Services (DHS) database, and Bureau of Statistics database were obtained for the years 1998-2017. The numbers of inguinal herniotomies in patients aged 0-4, 5-14 and 15-24 yearswere examined with ethical approval. RESULTS: Over the 20-year period, over 87,000 inguinal herniotomy procedures were performed in males. The incidence per year in males decreased across all ages over the 20-year period, but was most pronounced in infants and toddlers. Similar to males, the incidence in females decreased over time, with the ratio of procedures per head of population decreasing in children under 5 years of age. The ratio of male: females varied according to ages, and was between 2.8 and 6.2 males: 1 female. CONCLUSION: This study suggests that fewer 0-4-year olds are undergoing inguinal herniotomy, compared with 20 years ago. This is likely due to a change in practice for the management of unilateral symptomatic hernias, from routine bilateral herniotomies, to unilateral surgery. As well as less aggressive surgical intervention for hydroceles in boys. LEVEL OF EVIDENCE: III.
Assuntos
Hérnia Inguinal/cirurgia , Herniorrafia/estatística & dados numéricos , Laparoscopia/estatística & dados numéricos , Adolescente , Adulto , Austrália/epidemiologia , Criança , Pré-Escolar , Bases de Dados Factuais , Feminino , Hérnia Inguinal/epidemiologia , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Adulto JovemRESUMO
PURPOSE: Undescended testes (UDT) are subjected to heat stress, which can disturb gonocyte transformation as well as apoptosis. This study aims to describe the apoptosis pathway occurring during minipuberty of children with unilateral (UDT), and to investigate the role of inhibin-B. METHODS: Testicular biopsies at unilateral orchidopexy of 10 boys (6-9 months old) with normal inhibin-B (n = 5) or low inhibin-B (n = 5) were selected for immunohistochemistry and TUNEL (Terminal deoxynucleotidyl transferase dUTP nick end labelling) assay. Testicular tubules were labelled with antibodies against Anti-Müllerian hormone (AMH, Sertoli cell marker), mouse Vasa Homolog (MVH) and placental alkaline phosphatase (PLAP) (both germ cell markers), cleaved caspase3 (apoptotic marker), and followed by confocal imaging and cell counting with Fiji/ImageJ. Data were analyzed with GraphPad Prism. RESULTS: In males with low and normal inhibin-B, there was no statistical difference (p > 0.05) in the percentage of testicular tubules containing TUNEL + cells, number of cleaved caspase3 ± germ cells/tubule, total number of germ cells/tubule, and the percentage of fibrotic tubules or number of Sertoli cells/tubule. CONCLUSIONS: These results suggest that inhibin-B does not regulate cell death of gonocytes and further studies are required to uncover any role of inhibin-B in gonocyte transformation.
Assuntos
Diferenciação Celular , Criptorquidismo/patologia , Inibinas/sangue , Túbulos Seminíferos/citologia , Apoptose , Caspase 3/metabolismo , Criptorquidismo/cirurgia , Células Germinativas/citologia , Células Germinativas/metabolismo , Humanos , Imuno-Histoquímica , Marcação In Situ das Extremidades Cortadas , Lactente , Masculino , OrquidopexiaRESUMO
OBJECTIVES: Fundoplication is commonly performed in patients with a history of esophageal atresia (EA), however, the success of this surgery is reduced, as reflected by an increased rate of redo fundoplication. We aimed to determine whether EA impacts the prevalence of fundoplication, its timing, and performance of a redo operation. STUDY DESIGN: A single-center, retrospective review of all patients undergoing fundoplication over a 20-year period (1994-2013) was performed. Redo fundoplication was used as a surrogate for surgical failure. RESULTS: A total of 767 patients (patients with EA 85, those who did not have EA 682) underwent fundoplication during the study period. Median age (months) at primary fundoplication was lower in patients with EA (7.2 vs those who did not have EA 23.3; P < .001). Redo fundoplication rates between groups were not significantly different (EA 11/85 vs 53/682; P = .14). Median time (months) between primary and redo fundoplication was greater in patients with EA (36.2 vs 11.7; P = .03). CONCLUSIONS: Contrary to popular belief, the incidence of redo fundoplication was not significantly increased in patients with a history of EA. However, patients with EA underwent fundoplication at younger ages, which may be related to early life-threatening events in these patients. These results inform perioperative counseling, and highlight the importance of sustained surgical follow-up in patients with EA.
Assuntos
Atresia Esofágica/complicações , Fundoplicatura , Refluxo Gastroesofágico/cirurgia , Pré-Escolar , Feminino , Seguimentos , Refluxo Gastroesofágico/etiologia , Humanos , Lactente , Laparoscopia , Masculino , Reoperação , Estudos Retrospectivos , Resultado do TratamentoRESUMO
There is no consensus in the literature about the necessity for excision of testicular remnants in the context of surgery for an impalpable testis and testicular regression syndrome (TRS). The incidence of germ cells (GCs) within these nubbins varies between 0 and 16% in previously published series. There is a hypothetical potential future malignancy risk, although there has been only one previously described isolated report of intratubular germ-cell neoplasia. Our aim was to ascertain an accurate incidence of GCs and seminiferous tubules (SNTs) within excised nubbins and hence guide evidence-based practice. The systematic review protocol was designed according to the PRISMA guidelines, and subsequently published by the PROSPERO database after review (CRD42013006034). The primary outcome measure was the incidence of GCs and the secondary outcome was the incidence of SNTs. The comprehensive systematic review included articles published between 1980 and 2016 in all the relevant databases using specific search parameters and terms. Strict inclusion and exclusion criteria were ultilised to identify articles relevant to the review questions. Twenty-nine paediatric studies with a total of 1455 specimens were included in the systematic review. The mean age of the patients undergoing nubbin resection was 33 months and the TRS specimen was more commonly excised from the left (68%). The incidence of SNTs was 10.7% (156/1455) and the incidence of GCs, 5.3% (77/1455). Histological analysis excluding the presence of either SNTs or GCs was consistent with TRS, fibrosis, calcification or haemosiderin deposits. There is limited evidence on subset analysis that GCs and SNTs may persist with increasing patient age. This systematic review has identified that 1 in 20 of resected testicular remnants has viable GCs and 1 in 10 has SNTs present. There is insufficiently strong evidence for the persistence of GCs and SNTs with time or future malignant potential. Intra-abdominal TRS specimens may contain more elements and, therefore, require excision, although this is based on limited evidence. However, there is no available strong evidence to determine that a TRS specimen requires routine excision in an inguinal or scrotal position.
Assuntos
Células Germinativas/citologia , Disgenesia Gonadal 46 XY/patologia , Túbulos Seminíferos/patologia , Testículo/anormalidades , Criptorquidismo/patologia , Criptorquidismo/cirurgia , Disgenesia Gonadal 46 XY/cirurgia , Humanos , Masculino , Testículo/patologia , Testículo/cirurgiaRESUMO
BACKGROUND: Transcutaneous electrical stimulation (TES) using interferential current (IFC) is a new therapeutic treatment for constipation. Clinical studies show that TES-IFC for 3-6 months improves colonic transit, but it is not clear if short-term stimulation affects transit or the effect requires longer to develop. The aim of this study was to determine if TES-IFC for only four days affects oral-rectal transit time in healthy pigs. METHODS: Twenty-two 4-5-week old large white female piglets had transit studies during week 4 and week 5 by placing a capsule containing 18 radiopaque plastic markers in the esophagus under anesthetic followed by x-rays at 6, 30, 54, and 78 hours. Animals were randomly assigned to active or control groups. The active group received TES for 30 min daily for four days. Interferential current was applied through four electrodes (4 × 4 cm), with two para-spinal just below the last rib and two on the belly at the same level. Stimulation was at 4000 Hz and 4080-4160 Hz with currents crossing through the abdominal cavity. RESULTS: Whole bowel transit times ranged from 7.7 to 72.2 hours, stomach transit from <1 to 63 hours, and bowel with rectum transit time from 5 to 53 hours. Transit times were the same for the control (median 28.4 hours) and TES-IFC (23.0 hours) groups in the prestimulation and stimulation weeks (control 23.0, TES-IFC 19.8 hours) with no change within or between groups. CONCLUSION: Four days of half-hour TES-IFC daily in healthy 5-week-old piglets did not change oral-rectal transit time.
Assuntos
Abdome/inervação , Trânsito Gastrointestinal/fisiologia , Boca/fisiologia , Reto/fisiologia , Estimulação Elétrica Nervosa Transcutânea/métodos , Abdome/diagnóstico por imagem , Animais , Feminino , Boca/diagnóstico por imagem , Boca/inervação , Distribuição Aleatória , Reto/diagnóstico por imagem , Reto/inervação , Suínos , Fatores de TempoRESUMO
Undescended testis (UDT) occurs when something goes wrong with testicular descent from high in the abdominal cavity to the scrotum. Normal descent occurs in two steps, with the transabdominal phase controlled by a new testicular hormone, insulin-like hormone 3, and the inguinoscrotal phase controlled by androgens. The latter phase requires a complex process of migration from the inguinal abdominal wall to the scrotum and is commonly defective, leading to the high incidence (2-4%) of UDT at birth. The clinical examination of babies and infants aims to confirm the persistence of congenital UDT by 3-6 months, so surgery can be optimally timed at 6-12 months. For those boys who develop acquired UDT later in childhood, the 'ascending' testis often needs surgery between 5 years and 10 years, so all boys should be screened again for UDT at school entry.
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Criptorquidismo/diagnóstico , Transtornos do Desenvolvimento Sexual/diagnóstico , Orquidopexia , Criança , Criptorquidismo/embriologia , Criptorquidismo/cirurgia , Transtornos do Desenvolvimento Sexual/complicações , Humanos , Lactente , Recém-Nascido , Laparoscopia , MasculinoRESUMO
INTRODUCTION AND METHODS: In this review, we describe the common clinical scenarios that may be present to a paediatric surgeon when a patient has a disorder of sex development (DSD). Our aim was to prepare surgeons so that they can respond with correct approaches to diagnose and manage the given situations. RESULTS: DSD present in three distinct clinical situations: in the neonate with some abnormality of the external genitalia; in the child undergoing surgical treatment for inguinal hernia or during open or laparoscopic orchidopexy or during hypospadias correction; and at or after puberty, which may be precocious or delayed or in an adolescent girl with masculinisation at puberty. We describe the clinical features, likely diagnoses and the recommended management pathway in these scenarios.
Assuntos
Transtornos do Desenvolvimento Sexual/diagnóstico , Transtornos do Desenvolvimento Sexual/cirurgia , Hipospadia/diagnóstico , Pediatria/métodos , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Hipospadia/cirurgia , Lactente , Recém-Nascido , Masculino , CirurgiõesRESUMO
BACKGROUND/AIM: Undescended testis (UDT) occurs in ~2 % of newborn males, and occasionally these infants also have posterior urethral valve (PUV). The cause of this relationship is uncertain. We aimed to review the literature to identify publications documenting co-occurrence of UDT and PUV, and to summarise the theories of co-occurrence. METHODS: A search of the literature (Embase, Medline, Pubmed; 1947-2015) was undertaken to identify publications describing the link between UDT in PUV patients, as well as PUV in UDT patients. Ten publications in English were found with both UDT and PUV: 9 articles describing the frequency of UDT in patients with PUV, and 1 article examining the frequency of PUV in infants with UDT. RESULTS: UDT occurred in 12-17 % of PUV compared with 1-2 % in the control population, consistent with a 10-fold increase. PUV occurred in 1.2 % of UDT patients compared with 0.01 % in the control population, consistent with a 100-fold increase. DISCUSSION: PUV leads to a 10-fold increase in occurrence of UDT, while the presence of UDT causes a 100-fold increase in occurrence of PUV. Four main theories of causation have been proposed, each of which have some merit but little supporting evidence, leaving the cause of simultaneous occurrence of PUV and UDT uncertain.
Assuntos
Criptorquidismo/etiologia , Uretra/anormalidades , Doenças Uretrais/etiologia , Anormalidades Urogenitais/embriologia , Humanos , Recém-Nascido , Nefropatias/complicações , Masculino , Doenças Uretrais/congênito , Doenças da Bexiga Urinária/complicaçõesRESUMO
BACKGROUND/PURPOSE: Long-term problems with faecal incontinence occur in up to 50 % of patients after pull-through for Hirschsprung disease (HSCR). The cause often remains unknown, leading to empirical treatments. Using nuclear transit study, we found some patients surprisingly had rapid proximal colonic transit, suspicious of occult diarrhoea. We aimed to assess whether these patients had unrecognized adverse reactions to food. METHODS: Patients (n = 10, all males, 9.6 year; 4.25-15.5 years) with persistent faecal incontinence following pull-through for HSCR referred to the senior author and after exclusion of anatomical defects, underwent nuclear transit studies. Most (8) subsequently underwent breath hydrogen tests for sugar malabsorption and were tested for adverse reactions to food. Exclusion diets for protein allergens, lactose or fructose were then trialed. RESULTS: Of the 10 patients with rapid intestinal transit proven on nuclear transit study, breath hydrogen tests for fructose and/or lactose malabsorption were done in 8, and were positive in 7/8 patients. Exclusion diets contributed to either resolution or improvement in faecal incontinence in 9/10 patients. CONCLUSIONS: Rapid transit in the proximal, ganglionated colon may be present in children with faecal incontinence following pull-through for HSCR, possibly secondary to adverse reactions to food. This study suggests that children with post-operative soiling may benefit from a transit study and hydrogen breath tests to diagnose adverse reactions to food caused by sugar malabsorption.
Assuntos
Incontinência Fecal/fisiopatologia , Hipersensibilidade Alimentar/fisiopatologia , Trânsito Gastrointestinal/fisiologia , Doença de Hirschsprung/cirurgia , Síndromes de Malabsorção/fisiopatologia , Adolescente , Testes Respiratórios , Criança , Pré-Escolar , Frutose/metabolismo , Humanos , Hidrogênio/análise , Lactose/metabolismo , Masculino , Complicações Pós-OperatóriasRESUMO
AIM: As transcutaneous electrical stimulation (TES) increased defecation in children and adults with Slow-Transit Constipation (STC), we performed a pilot study to test if TES can improve symptoms (defecation and soiling) in children with chronic constipation without STC and transit delay in the anorectum. METHODS: Children with treatment-resistant constipation presenting to a tertiary hospital had gastrointestinal nuclear transit study (NTS) showing normal proximal colonic transit and anorectal holdup of tracer. TES was administered at home (1 hour/day for 3 months) using a battery-powered interferential stimulator, with four adhesive electrodes (4 × 4 cm) connected so currents cross within the lower abdomen at the level of S2-S4. Stimulation was added to existing laxatives. Daily continence diary, and quality-of-life questionnaires (PedsQL4.0) were compared before and after TES. RESULTS: Ten children (4 females: 5-10 years, mean 8 years) had holdup in the anorectum by NTS. Nine had <3 bowel motions (BM)/week. After three months TES, defecation frequency increased in 9/10 (mean 0.9-4.1 BM/week, p = 0.004), with 6/9 improved to ≥3 BM/week. Soiling reduced in 9/10 from 5.9 to 1.9 days/week with soiling, p = 0.004. Ten were on laxatives, and nine reduced/stopped laxative use. Quality-of-life improved to within the normal range. CONCLUSION: TES improved symptoms of constipation in >50% of children with treatment-resistant constipation with isolated holdup in the anorectum. Further studies (RCTs) are warranted in these children.
Assuntos
Constipação Intestinal/terapia , Estimulação Elétrica Nervosa Transcutânea/métodos , Criança , Pré-Escolar , Constipação Intestinal/psicologia , Feminino , Serviços de Assistência Domiciliar , Humanos , Masculino , Avaliação de Resultados em Cuidados de Saúde , Projetos Piloto , Qualidade de VidaRESUMO
PURPOSE: Boys with cryptorchidism have overall increased gonadotropin and decreased serum inhibin B levels compared to normal. Serum inhibin B levels, produced by Sertoli cells, may reflect the state of germinative epithelium in cryptorchid testes. We evaluated whether serum inhibin B levels differed between boys with unilateral vanished testis and those with unilateral cryptorchidism. MATERIALS AND METHODS: Blood samples from 297 boys 1.5 to 5 years old were included, of whom 222 had unilateral cryptorchidism, 29 had unilateral vanished testis and 46 had undergone unilateral orchiopexy 1 year previously. Serum inhibin B levels were measured using a commercially available ELISA kit and were compared to normal range. RESULTS: Serum inhibin B levels in boys with unilateral vanished testis were not different from those with unilateral cryptorchidism. Serum inhibin B values were above the normal median in 43% of boys previously operated on for unilateral cryptorchidism, compared to 17% at surgery (p = 0.0003). The percentage of patients with inhibin B levels below normal range was 14% in those with unilateral vanished testis, 23% in those with unilateral cryptorchidism and 11% in those who had undergone orchiopexy 1 year previously for unilateral cryptorchidism. The percentage of boys with inhibin B levels above normal median was 24% in those with unilateral vanished testis, 17% in those with unilateral cryptorchidism and 43% in those who had undergone orchiopexy. However, in boys with a vanished testis the frequency of serum inhibin B above normal median was only 5% before age 1.5 years, after which the rate was 67% (p = 0.0022). CONCLUSIONS: Our findings may reflect the development of contralateral testicular hypertrophy in boys with unilateral vanished testis. The initial low inhibin B values may be explained by impaired total number of Sertoli cells. Serum inhibin B values also indicated that in 6-month to 5-year-old boys with cryptorchidism orchiopexy was beneficial for the germinative epithelium.
Assuntos
Criptorquidismo/sangue , Inibinas/sangue , Pré-Escolar , Criptorquidismo/patologia , Humanos , Lactente , MasculinoRESUMO
PURPOSE OF REVIEW: Normal testicular descent is now recognized to occur in two steps with the first, transabdominal stage controlled by insulin-like hormone 3. The second, inguinoscrotal stage is controlled by androgens, mostly indirectly via the genitofemoral nerve, which appears to direct the migration of the gubernaculum to the scrotum. Undescended testis (UDT) is multifactorial, with only some of the genes identified. This review highlights recent developments that are leading to changes in practice. RECENT FINDINGS: There is an emerging consensus among pediatric surgeons and urologists about the management of UDT with recommendations that the diagnosis of congenital UDT should be confirmed at 3-6 months of age and orchidopexy done at 6-12 months of age. With the recommendations for early surgery, recent studies focus on the complications of orchidopexy, to determine whether this is higher in infants than older children. In addition, there is general acceptance of the existence of 'acquired' UDT, which develops after about 2 years of age, but treatment for this group remains controversial. SUMMARY: Evaluation of children with UDT now needs to be separated into the assessment of possible congenital UDT in infants at 0-6 months, for orchidopexy before 12 months, and preschool boys, who may be developing acquired UDT.
Assuntos
Criptorquidismo/diagnóstico , Orquidopexia/métodos , Testículo/fisiopatologia , Pré-Escolar , Criptorquidismo/fisiopatologia , Criptorquidismo/cirurgia , Humanos , Lactente , Masculino , Guias de Prática Clínica como Assunto , Testículo/crescimento & desenvolvimento , Resultado do TratamentoRESUMO
AIM: Constipation is a common cause of admission to hospital for disimpaction, as oral laxatives are often inadequate. High-dose oral laxative protocols are used for complete bowel clearance prior to colonoscopy, but have not been reported for treating faecal impaction. The aim of this study was to assess the effectiveness of a high-dose oral protocol using polyethylene glycol with electrolytes (PEG + E) (Movicol Rx) combined with sodium picosulphate (SP) (Dulcolax SP Rx) in faecal impaction in children presenting to a suburban clinic. METHODS: Forty-four children presented with acute/chronic faecal impaction were given six to eight sachets of PEG + E were given on day 1, with decreasing doses on subsequent 3 days, while 15-20 SP drops were given on days 2 and 3. Compliance with medication was achieved using a simple method of motivation, with the child drinking the laxatives in a race. On day 4, PEG + E was reduced to one sachet and SP to 10 drops as an ongoing maintenance dose. Defecation, soiling, diet and water intake was monitored daily for 7 days in a diary. RESULTS: Forty-four children (aged 2-17 years) seen over 8 months were reviewed retrospectively. Children began defecating within 10-12 h reaching a maximum volume of stool/day (four cups) on day 2. All patients were disimpacted successfully and in the week following disimpaction there was no reported faecal soiling or complications. CONCLUSIONS: A high-dose oral protocol combining PEG + E sachets and SP drops successfully and safely disimpacted a cohort of children with acute/chronic constipation presenting to a suburban continence clinic. This protocol appears to be useful to control faecal disimpaction in an outpatient setting, thereby avoiding hospital admission.
Assuntos
Citratos/uso terapêutico , Constipação Intestinal/tratamento farmacológico , Eletrólitos/uso terapêutico , Impacção Fecal/tratamento farmacológico , Laxantes/uso terapêutico , Compostos Organometálicos/uso terapêutico , Picolinas/uso terapêutico , Polietilenoglicóis/uso terapêutico , Administração Oral , Adolescente , Catárticos/uso terapêutico , Criança , Pré-Escolar , Citratos/administração & dosagem , Combinação de Medicamentos , Quimioterapia Combinada , Eletrólitos/administração & dosagem , Feminino , Humanos , Laxantes/administração & dosagem , Masculino , Compostos Organometálicos/administração & dosagem , Picolinas/administração & dosagem , Polietilenoglicóis/administração & dosagem , Solventes/uso terapêutico , Serviços de Saúde Suburbana , Resultado do TratamentoRESUMO
Slow-transit constipation (STC) is a newly described subtype of intractable constipation in children which we originally identified with deficiency of substance P in axons supplying the proximal colonic muscle. When nuclear transit studies became available, the patients were found to have slow proximal colonic transit, and responded to antegrade enemas. Using the appendicostomy, we found that there was reduced frequency in propagating sequences throughout the colon. We began testing whether transcutaneous electrical stimulation (TES) could improve motility and symptoms, and over several trials have now shown that TES is remarkably effective in treating children with STC, with long-lasting effects. TES holds promise for treating STC, as well as a range of gastrointestinal motility disorders.