RESUMEN
Perineal groove is a well-defined clinical entity that belongs to a broader group of anorectal malformations. It is characterized by a non-epithelialized mucous membrane that appears as an erythematous sulcus in the perineal midline, extending from the posterior vaginal fourchette to the anterior anal orifice. The defect is gradually cicatrized, unless there are complications like infection, defecation disorders, trauma, and bleeding. The differential diagnosis includes several other conditions like trauma, infection, irritant dermatitis, lichen sclerosis, and ulcerated hemangioma. Since it is a rare malformation, it is often misdiagnosed and its presence often elicits unnecessary diagnostic workup and intervention. In this respect, neonatologists, dermatologists, or pediatric surgeons may under- or overestimate it. We report four cases of perineal groove out of 2250 live births at a Greek Maternity Hospital between September 2016 and April 2019. The "high" incidence of perineal groove cases in our Department allowed us to familiarize with this rare defect and minimize our clinical interventions.
Asunto(s)
Síndrome de Bandas Amnióticas/diagnóstico , Malformaciones Anorrectales/diagnóstico , Perineo/anomalías , Canal Anal/patología , Diagnóstico Diferencial , Femenino , Grecia , Maternidades , Humanos , Recién Nacido , Vagina/patologíaRESUMEN
The microbiome is vital for the proper function of the gastrointestinal tract (GIT) and the maintenance of overall wellbeing. Gut ischemia may lead to disruption of the intestinal mucosal barrier, resulting in bacterial translocation. In this systematic review, according to PRISMA (Preferred Reporting Items for Systematic Review and Meta-Analysis) guidelines, we constructed a search query using the PICOT (Patient, Intervention, Comparison, Outcome, Time) framework. Eligible studies reported in PubMed, up to April 2021 were selected, from which, 57 publications' data were included. According to these, escape of intraluminal potentially harmful factors into the systemic circulation and their transmission to distant organs and tissues, in utero, at birth, or immediately after, can be caused by reduced blood oxygenation. Various factors are involved in this situation. The GIT is a target organ, with high sensitivity to ischemia-hypoxia, and even short periods of ischemia may cause significant local tissue damage. Fetal hypoxia and perinatal asphyxia reduce bowel motility, especially in preterm neonates. Despite the fact that microbiome arouse the interest of scientists in recent decades, the pathophysiologic patterns which mediate in perinatal hypoxia/asphyxia conditions and gut function have not yet been well understood.
RESUMEN
BACKGROUND: The term "bacterial translocation" (BT) refers to the migration of bacteria or their products from the gastrointestinal tract to tissues located outside it, and may occur after intestinal ischemia-reperfusion injury. The term "endotoxin" is synonymous, and is used interchangeably with the term lipopolysaccharide (LPS). LPS, a component of Gram-negative gut bacteria, is a potent microbial virulence factor, that can trigger production of pro-inflammatory mediators, causing localized and systemic inflammation. The aim of this study is to investigate if neonatal asphyxia provokes BT and an increased concentration of LPS in an animal model of asphyxia in piglets. METHODS: Twenty-one (21) newborn male Landrace/Large White piglets, 1-4 days old, were randomly allocated into three groups, Control (A), Asphyxia (B) and Asphyxia-Cardiopulmonary Resuscitation (CPR) (C). All animals were instrumented, anesthetized and underwent hemodynamic monitoring. In Group A, the animals were euthanized. In Group B, the endotracheal tube was occluded to cause asphyxia leading to cardiopulmonary arrest. In Group C, the animals were resuscitated after asphyxia and further monitored for 30'. Bacterial translocation was assessed by the measurement of endotoxin in blood from the portal vein and the aorta, and also by the measurement of endotoxin in mesenteric lymph nodes (MLNs) at euthanasia. The results are given as median (IQR) with LPS concentration in EU/mL. RESULTS: BT was observed in all groups with minimum LPS concentration in the MLN and maximum concentration in the portal vein. LPS levels in the MLNs were higher in the Group B: 6.38 EU/mL (2.69-9.34) compared to the other groups (Group A: 2.1 EU/mL (1.08-2.52), Group C: 1.66 EU/mL (1.51-2.48), p = 0.012). The aorta to MLNs LPS difference (%) was lower in Group B: 0.13% (0.04-1.17), compared to Group A: 5.08% (2.2-10.7), and Group C: 3.42% (1.5-5.1)) (p = 0.042). The same was detected for portal to MLNs LPS difference (%) which was lower in Group B: 0.94% (0.5-3) compared to Group A: 4.9% (4-15), and Group C: 3.85% (1.5-5.1)) (p = 0.044). CONCLUSIONS: Neonatal asphyxia can provoke ΒΤ and increased LPS concentration in blood and tissue located outside the gastrointestinal system.
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BACKGROUND: Burn injury (BI) is one of the most serious causes of morbidity and mortality in the pediatric population. BI triggers an initial stage of hyperinflammation, followed by hypersecretion of both pro- and anti-inflammatory cytokines. IL- 18 is a vital pro-inflammatory cytokine, the effect of which has been investigated not only in animal models but also in adult patients. No study has yet examined the association of serum IL- 18 levels and the clinical significance in the course of pediatric BI. METHODS: We conducted a prospective study including all children with burn injuries who were hospitalized from December 2015 to December 2018 in a tertiary Children's Hospital. RESULTS: A total of 55 children with BI were included. In the present study, we found a strong positive correlation between total body surface area (TBSA) and the levels of IL-18 at admission and on the third day postburn, respectively. The WBC count, the number of lymphocytes and the CRP levels at admission revealed a strong, positive correlation with IL-18 levels. The correlation between IL-18 levels at admission and the length of stay (LOS) was moderate. CONCLUSIONS: This study has shown that the levels of IL-18 collected at admission correlate positively with the extent of TBSA and inflammatory indices in pediatric patients. Moreover, IL-18 levels at admission may not be the most accurate prognostic factor regarding the LOS. However, further research is needed in order to establish more accurate predictive factors for the outcome of BIs in pediatric patients.
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BACKGROUND: There is an endless debate on whether androgens mediate testis descent through developmental changes in the gubernacular or the cranial suspensory ligament. OBJECTIVE: To investigate the relation of any possible morphologic changes in the genital mesentery, that is, the system of genital peritoneal folds including the gubernacular and cranial suspensory ligaments, with the event of testis non-descent in rats prenatally exposed to the antiandrogen flutamide. MATERIALS AND METHODS: Time-pregnant Sprague Dawley rats received flutamide (100 mg/kg/d) or vehicle subcutaneously on gestational days 16-17. Flutamide-treated male offspring (n = 67), and vehicle-treated male (n = 34) and female (n = 28) offspring were surgically explored under microscope on postnatal day 50. Testicular position was examined bilaterally. Dimensions of genital mesentery parts were also assessed bilaterally. Association of flutamide-induced morphologic changes with descended (n = 61) and undescended (n = 50; 33 cryptorchid and 17 ectopic) testes was investigated with logistic regression analysis. RESULTS: The male genital mesentery comprised a cranial and a caudal fold converging on the vas deferens. Flutamide resulted in enlarged cranial and reduced caudal folds. Of all flutamide-induced alterations, the increased length of the posterior fixation of the cranial fold and the decreased length of the gubernacular ligament of the caudal fold were found to independently increase the odds of testis non-descent. Testicular ectopy, unlike cryptorchidism, was associated with a short gubernacular ligament only. The female genital mesentery consisted of a cranial fold only. CONCLUSION: Our findings showed a combined contribution of both cranial and caudal folds of the genital mesentery to testis non-descent, through an abnormally long mesentery root and an abnormally short gubernacular ligament, respectively. Inhibition of male-specific development of the genital mesentery with flutamide did not result in a feminized architecture.
Asunto(s)
Andrógenos/fisiología , Criptorquidismo/etiología , Genitales Masculinos/embriología , Mesenterio/embriología , Animales , Femenino , Flutamida , Masculino , Embarazo , Ratas Sprague-DawleyRESUMEN
Empty scrotum is a relatively rare entity, with few cases reported in the literature, so far; if it coexists with bilateral ectopic femoral testes, it then constitutes an extremely rare congenital abnormality. We report a case of empty scrotum revealed at the first physical examination of a neonate. The scrotum appeared empty and no testes could be palpated in the scrotum or the inguinal canal. Two solitary, oval masses were palpable laterally of each hemiscrotum, close to femoral canal and the diagnosis of bilateral femoral ectopic testes was confirmed by the consultant pediatric surgeon.
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We report on a neonate with a disorder of sex development, Prader 3-4 external genitalia and a palpable structure in the right inguinal canal suggestive of gonadal tissue. Chromosome studies on blood lymphocytes showed monosomy of chromosome X. Laparoscopy identified a streak-like gonad on the left side, unicorn uterus and a dysgenetic testis on the right, attached to a Fallopian tube. Because of the unilateral palpable gonad and the presence of ambiguous genitalia we investigated further for the presence of Y material. Quantitative fluorescent PCR analysis of material from the dysgenetic gonad and skin fibroblasts revealed the presence of chromosome Y-derived sequences, suggesting sex chromosome mosaicism. In 45,X/46,XY mosaicism, chromosome studies carried out on peripheral lymphocytes do not always reflect the proportion of cell lines in the gonads. The detection of Y chromosome material in a dysgenetic gonad is extremely significant, due to the high risk of malignant transformation.
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Cromosomas Humanos X/genética , Cromosomas Humanos Y/genética , Disgenesia Gonadal/genética , Gónadas/anomalías , Trastornos Ovotesticulares del Desarrollo Sexual/genética , Aberraciones Cromosómicas Sexuales , Células Cultivadas , Femenino , Fibroblastos/química , Fibroblastos/citología , Disgenesia Gonadal/patología , Humanos , Recién Nacido , Masculino , Mosaicismo , Trastornos Ovotesticulares del Desarrollo Sexual/patología , Piel/química , Piel/citologíaRESUMEN
We present a unique case of a 16-year-old male patient who was eventually diagnosed with a large enterolith arising from a Meckel's diverticulum. The enterolith had caused intermittent intestinal symptoms for three years before resulting in small bowel obstruction requiring surgical intervention. Meckel's enterolith ileus is very rare with only few cases described in the literature. To our knowledge, this is only the second case of Meckel's enterolith which had caused intermittent symptoms over a period of time, before resulting in ileus, and the first case where the intermittent symptoms lasted several years before bowel obstruction. The patient had been evaluated with colonoscopy, computerized tomography (CT), and magnetic resonance imaging enterography (MRIE); a calcified pelvic mass had been found, but no further diagnosis other than calcification was established. The patient presented at our emergency department, with symptoms of obstructive ileus and underwent exploratory laparotomy, where a large enterolith arising from a Meckel's diverticulum (MD) was identified, causing the obstruction. A successful partial enterectomy, enterolith removal, and primary end-to-end anastomosis took place; the patient was permanently relieved from his long-standing symptoms. Consequently, complications of Meckel's diverticulum and enterolithiasis have to be included in the differential diagnosis of abdominal complaints.
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BACKGROUND: Solid pseudopapillary tumors (SPTs) of the pancreas are a rare occurrence, not exceeding 1-2% of all exocrine pancreatic tumors. SPT was first described in 1959 as "papillary tumor of the pancreas, benign or malignant" and affects mainly young women, in their second or third decade of age. These tumors are of low malignant potential, unclear pathogenesis, grow gradually and become considerably large before causing symptoms. A typical clinical presentation is often described by affected patients and, in some cases, an SPT is an incidental finding during the time the patient undergoes medical imaging studies for other health issues. SPT is frequently located at the head or tail of the pancreas. Metastases are rare but, when present, affect predominantly the liver. PATIENTS AND METHODS: We report a series of five SPT cases in female patients 13-47 years old, presenting with almost identical symptoms of upper abdominal discomfort and non-tender palpable mass. Two out of five patients also reported vomiting, nausea and poor appetite as co-existing non-diagnostic symptoms. Only one patient presented without any symptoms. Tumor location and dimensions varied. One patient underwent a pancreatoduodenectomy (Whipple's procedure), while the remaining patients underwent distal pancreatectomy with concomitant splenectomy. RESULTS: Perioperative morbidity and mortality was zero. All five patients are disease-free at a follow-up from 3 months to 13 years. Histopathology reports supported the diagnosis of SPT and no metastatic disease was present in any of the patients. CONCLUSION: The overall prognosis of SPT of the pancreas is excellent due to its favorable biological features, even in the presence of distal metastasis. Although surgical resection is often curative, a close follow-up is advised in order to diagnose a possible local recurrence or distal metastasis and choose the proper therapeutic option for the patients.
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Carcinoma Papilar/cirugía , Páncreas/cirugía , Neoplasias Pancreáticas/cirugía , Pronóstico , Adolescente , Adulto , Carcinoma Papilar/patología , Femenino , Humanos , Persona de Mediana Edad , Metástasis de la Neoplasia , Recurrencia Local de Neoplasia/patología , Páncreas/patología , Pancreatectomía , Neoplasias Pancreáticas/patología , Adulto JovenRESUMEN
BACKGROUND: Inflammatory myofibroblastic tumor is a rare tumor of a borderline malignancy. Although it is commonly seen in children, the number of childhood cases in the current literature is limited. The lung is the most commonly affected location. However, cases that have been documented in the mesentery-omentum have mostly been located in the mesentery of the small bowel and not in the antimesenteric edge as in our patient. CASE PRESENTATION: A 6-year-old Greek boy was referred to our hospital with acute abdominal pain mimicking appendicitis. Ultrasound and computed tomography revealed a solid mass in the abdomen. The patient underwent laparoscopic resection of the tumor, and histopathology and immunohistochemical analysis favored an omental-mesenteric myxoid hamartoma, which is a variant of an inflammatory myofibroblastic tumor. The patient's postoperative course was uneventful, and he has been asymptomatic during follow-up. CONCLUSIONS: Inflammatory myofibroblastic tumor of the small intestine is a rare, benign neoplasm in children that should be considered as a possible cause of acute abdomen. A precise diagnosis can be made on the basis of histologic findings. Surgical excision is the treatment of choice.
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Apendicitis/diagnóstico , Neoplasias Intestinales/diagnóstico , Intestino Delgado , Neoplasias de los Tejidos Conjuntivo y Blando/diagnóstico , Niño , Diagnóstico Diferencial , Humanos , MasculinoRESUMEN
Dieulafoy's lesion is an extremely rare cause of upper gastrointestinal bleeding in the neonatal age group. Till now, only 6 cases of Dieulafoy's lesion in neonatal period have been reported in the international literature. Herein, we report an extremely rare case of Dieulafoy's lesion in a preterm neonate.
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Venous access device is critically needed for long-term parenteral nutrition (PN), especially in children with chronic disease such as cystic fibrosis, short bowel syndrome, and permanent intestinal failure. When traditional sites are unavailable and venous access is very limited, alternative central routes are required. The access to the azygos system has been shown to be a safe, relatively easy solution in those special situations. We report the case of an adolescent who benefited from this central venous access for long-term PN and antibiotic treatment for >7 years with a limited number of complications.
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Vena Ácigos , Fibrosis Quística/terapia , Nutrición Parenteral en el Domicilio/métodos , Adolescente , Cateterismo Venoso Central , Femenino , Humanos , Nutrición Parenteral en el Domicilio/efectos adversos , Nutrición Parenteral en el Domicilio/instrumentación , Resultado del TratamientoRESUMEN
OBJECTIVES: The objective of this study was to assess the ability of acute dimercaptosuccinic acid (DMSA) scintigraphy for revealing vesicoureteral reflux (VUR) in young children after a first febrile urinary tract infection (UTI). METHODS: Children agedAsunto(s)
Radiofármacos
, Ácido Dimercaptosuccínico de Tecnecio Tc 99m
, Infecciones Urinarias/etiología
, Reflujo Vesicoureteral/complicaciones
, Reflujo Vesicoureteral/diagnóstico por imagen
, Femenino
, Humanos
, Lactante
, Masculino
, Estudios Prospectivos
, Cintigrafía
, Ultrasonografía
RESUMEN
Henoch-Schönlein purpura (HSP)-associated stenosing ureteritis represents a rare complication of the disease, typically presenting with severe manifestations. This article reports on a 3.5-year-old boy with HSP and severe nephritis who developed a unilateral stenosing ureteritis with atypical manifestations, resulting in a nonfunctional kidney and consequent nephrectomy. The urinary tract ultrasound was normal in the first week of illness, and the diagnosis was made during follow-up 8 months after onset. The predominance of nephritic manifestations may have masked any signs of ureteritis, leading to the delay in diagnosis. To clarify the clinical spectrum of this complication, an extensive review of the literature was performed. We emphasize the necessity of repeated urinary tract ultrasound both early and later in the course of HSP, especially in cases with renal involvement, so that an early diagnosis of this complication can prevent a potentially serious renal outcome.
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Vasculitis por IgA/complicaciones , Obstrucción Ureteral/etiología , Biopsia , Preescolar , Diagnóstico Diferencial , Humanos , Vasculitis por IgA/diagnóstico , Masculino , Nefrectomía , Índice de Severidad de la Enfermedad , Tomografía Computarizada por Rayos X , Ultrasonografía , Uréter/diagnóstico por imagen , Uréter/patología , Obstrucción Ureteral/diagnóstico , Obstrucción Ureteral/cirugíaRESUMEN
The existence of congenital obstructive posterior urethral membrane (COPUM), extending proximally from the verumontanum toward the bladder neck, has been widely debated. Although COPUM often leads to chronic renal disease, a milder degree of obstruction and protective pressure pop-off mechanisms have been reported. We present a male infant with a rare combination of unilateral renal agenesis, COPUM proximally from the verumontanum to the bladder neck, and enlarged prostatic utricle. The posterior urethra was dilated, without bladder abnormalities or hydroureteronephrosis, indicating milder obstruction. Moreover, the enlarged prostatic utricle could contribute in the urinary tract protection, serving as an uncommon pressure pop-off mechanism.
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Anomalías Múltiples , Riñón/anomalías , Próstata/patología , Obstrucción Uretral/congénito , Anomalías Múltiples/diagnóstico , Humanos , Lactante , Masculino , Obstrucción Uretral/diagnósticoRESUMEN
In an attempt to evaluate first urinary tract infection (UTI) in neonates and infants, we estimated retrospectively in 296 patients (62 neonates and 234 infants) clinical and laboratory findings, occurrence of vesicoureteral reflux (VUR), urinary tract abnormalities and pyelonephritis. First UTI occurred more often in male than female neonates, whereas male and female infants/young children were affected at an equal rate. The pathogens isolated in urine cultures of neonates and infants did not statistically significantly differ (P>0.05); Escherichia coli predominated. Gram-negative bacteria other than E. coli affected boys more often than girls (P=0.0022). Fever was the most frequent symptom. Neonates had lower-grade fever of shorter duration than infants (P<0.05). The incidence of reflux and urinary tract abnormalities did not differ between neonates and infants, male and female neonates and infants (P>0.05). Pyelonephritis affected neonates and infants at an equal rate; it was more prevalent among female patients (P=0.038) and patients with VUR or urinary tract abnormalities other than VUR (P<0.0001). Neonates with reflux were more often affected by Gram-negative bacteria other than E. coli than were neonates without reflux (P=0.0008).