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Los bezoares gastrointestinales son agregados no digeribles que fueron previamente ingeridos, el tricobezoar se sitúa dentro de la clasificación de éstos, su composición es de cabellos y restos alimentarios, tiene como ubicación más frecuente al estómago y rara vez se extiende hacia duodeno, yeyuno, íleon e incluso colon, condición entonces denominada síndrome de Rapunzel.Es una entidad rara, con incidencias que van desde 0.068% - 0.43%. Los pacientes a menudo muestran sintomatología digestiva inespecífica, como: dolor abdominal difuso, náuseas, vómitos, pobre ganancia de peso, ictericia obstructiva, enteropatía por pérdida de proteínas, masa abdominal y suele asociar además síntomas psiquiátricos 3-5. Se presenta el caso de una paciente de 7 años con hemorragia digestiva alta como debut de enfermedad, secundario a tricobezoar.

Obstructive respiratory stress and feeding difficulties in infants with Robin sequence (RS) may result in poor weight gain or loss. Following failure of conservative treatment, surgical options include mandibular distraction osteogenesis (MDO) and tongue-lip adhesion (TLA). Whilst both techniques have demonstrated to improve airway patency and feeding behavior, an advantage of either in restoring weight growth remains unknown. This study aimed to improve procedural selection by examining weight gain following MDO and TLA. A retrospective chart review was performed for 17 RS patients that had undergone MDO and 25 that had received TLA. The mean body weight in both groups was below the 50th population percentile at birth and fell further in the period before surgery. A mixed model analysis demonstrated that postoperative weight gain depended on the progression of time and preoperative weight. Conversely, biological sex, congenital comorbidities, method of feeding, the respective cleft team, and the type of surgery did not significantly influence the evolution of postoperative body weight. In conclusion, both MDO and TLA were able to restore weight growth in infants affected by RS, though a clear superiority of either technique could not be established.

Introduction Enteral feeding pumps at times may deliver different volumes than are prescribed, which can negatively impact growth, nutrition, and well-being. This study sought to assess whether challenges with pump accuracy for patients on food-based formulas contributed to challenges with weight gain. Methods Chart review identified complex feeding patients receiving food-based enteral nutrition via feeding pump with unexpected weight loss. Relevant data, such as enteral formula type, and anthropometric information were extracted. Results Five complex pediatric feeding patients were identified and 2 of these cases were summarized as representative examples, showing weight loss in children following the introduction of enteral food-based formulas because of feeding pump inaccuracy. Conclusions Complex pediatric feeding patients may display unexpected and poor weight gain and growth while receiving food-based enteral feeding interventions because of pump errors. It is vital for providers to be aware of these challenges for timely intervention.

Subcutaneous fat necrosis of the newborn should be considered in newborns with suggestive skin lesions, even in the absence of perinatal distress. SCFN may cause long‐standing complications, like hypertriglyceridemia or hypercalcemia. Hypercalcemia can be refractory to therapy and lead to poor weight gain and nephrocalcinosis, which should be closely monitored.

Clostridium difficile infection rates have been rising within the last decade and more pediatric patients have been suffering from this type of infection. However, this bacterium is a well-known contaminant in the normal flora of the colon in patients under the age of 2 years, and therefore consensus guidelines have recommended against routine testing for this infection, unless clinically indicated. We present here a case of a very young infant who presented with symptoms of food refusal, poor weight gain, abdominal distention, and colitis noted on imaging. Endoscopic evaluation demonstrated the presence of pseudomembranous colitis, so the patient was treated with antibiotics against C. difficile and recovered fully. This case demonstrates the importance of keeping C. difficile colitis in your differential diagnosis, even at a very young age.

Kabuki syndrome (KS) is a rare heterogeneous phenotypic genetic syndrome, characterized by hypotonia, developmental delay and/or intellectual disability with typical facial features. It is challenging to diagnose KS in newborn and young infant. We report a Thai girl who presented with two rare co-occurrence phenotypes, hyperinsulinemic hypoglycemia and midgut malrotation. She had not have distinctive facial dysmorphism during neonatal period. At 4 months of age, she had poor weight gain with some facial features suggestive KS. Singleton whole exome sequencing (WES) was carried out followed by Sanger sequencing of the supposed variant. The result indicated a novel de novo heterozygous KMT2D mutation, c.15364A>T (p.Lys5122*), confirming KS. Our patient revealed rare clinical manifestations from the diverse population and address the benefit of WES in establishing early diagnosis of KS before typical facial gestalt exhibited, which allows timely and appropriate management to maximize developmental achievement.

Trichohepatoenteric syndrome (THES) is a rare autosomal recessive genetic disease characterized by severe early onset diarrhea, woolly and brittle hair, immunodeficiency, and liver disease. A mutation in either SKIV2L or TTC37 genes can cause the disease. We report a case of a 41-month-old girl who suffered from intractable watery diarrhea, hair abnormality, dysmorphic features, and poor weight gain. The diagnosis was made through whole-exome sequencing analysis. The analysis detected a new variant mutation (c.1201G > A) p. (Glu401Lys) in the SKIV2L gene. She was admitted once for poor weight gain and nasogastric tube (NGT) feeding, with which the patient showed improvement. She was discharged to go home on hypoallergenic baby formulas and a regular diet with improved weight gain.

Pseudohypoaldosteronism type1A (PHA1A) is the renal form of pseudohypoaldosteronism with autosomal dominant inheritance. PHA1A is caused by haploinsufficiency of the mineralocorticoid receptor, which is encoded by NR3C2. We encountered an infant who was diagnosed with PHA1A due to hyponatremia, hyperkalemia, and poor weight gain in the neonatal period. She carried a novel heterozygous mutation (NM_000901.5: c.1757 + 1 G > C) in the splice donor site of IVS-2 in NR3C2.

We report 20 newborns who developed, at a median age of 7 days, large abdominal patches of radially arranged purplish telangiectasia in a bilateral and symmetrical pattern in relation to the midline, creating a "butterfly wing" pattern. Clinical examination was normal in 13 newborns, six newborns had abdominal distention, and one newborn had poor weight gain due to inadequate breastfeeding. Most lesions spontaneously resolved within 3 months and did not reoccur for 19 newborns. Transient abdominal telangiectasia of the newborn (TATN) appears to be a distinctive entity that has not been previously described.

Azygos or hemizygous continuation of inferior vena cava (IVC) is diagnosed in the presence of intrahepatic interruption of IVC. We report a case of a 4-year-old, male child presenting with a history of poor weight gain. A detailed evaluation of the child revealed a diagnosis of hemizygous continuation of uninterrupted, but severely obstructed, IVC. This incidental finding has rarely been reported in the literature.

Summary Background and aims Children with extended hospital stays are at risk of nutritional deterioration making regular nutrition screening throughout their admission an integral part of the nutrition care pathway. The purpose of this study was to design and validate a simple, quick and universal weekly rescreening tool to identify hospital acquired nutritional deterioration during a child's hospital stay. Methods A prospective, longitudinal sample of children aged 0–16 years admitted to a paediatric tertiary hospital with a length of stay ≥7 days were included in the study. Agreement between nutritional deterioration markers of reduction in weight (kg), body mass index (kg/m2), energy intake (kcal/day) and protein intake (g/day) over a 7-day period and two proposed rescreening questions was determined using sensitivity, specificity, area under the curve and positive and negative predictive values. Results Sixty-one children were included in the study with 224 full 7-day datasets. The sensitivity and specificity of the rescreening question ‘Has the child had reduced nutritional intake in the last 7 days’ for identifying children with a ≥25% reduction in energy intake over the previous 7-day period were 61.9% (95% CI 41.1–82.7) and 82.2% (95% CI 76.9–87.5) respectively. The sensitivity of ‘Has the child lost weight or had poor weight gain’ at detecting weight loss was 71.4% (95% CI 54.7–88.2) and specificity 87.8% (95% CI 83.1–92.5). Conclusion The paediatric nutrition rescreening questions provide a valid and simple tool to detect nutritional deterioration in long stay paediatric patients and should be an integral part of the nutrition care process.

Peripartum depression is a common, serious complication in mothers. To assess the influence of infant care, sleep and growth on the risk of peripartum depression, 1,271 mothers of healthy one-month-old infants completed comprehensive questionnaires including the Edinburgh Postnatal Depression Scale. Relationships between high depression scores and variables related to infants’ care, sleep and growth were assessed adjusting for other variables. High depression scores were found in 233 mothers, which were associated with variables related to infants’ care (poor satisfaction with infant care support, p

Background Infant death in keratitis-ichthyosis-deafness (KID) syndrome is recognized; its association with specific genotypes and pathophysiology is inadequately understood. Objective We sought to discover characteristics that account for poor outcomes in lethal KID syndrome. Methods We collected 4 new cases and 9 previously reported, genotyped cases of lethal KID syndrome. We performed new molecular modeling of the lethal mutants GJB2 p.A88V and GJB2 p.G45E. Results Infant death occurred in all patients with GJB2 p.G45E and p.A88V; it is unusual with other GJB2 mutations. Early death with those 2 "lethal" mutations is likely multifactorial: during life all had ≥1 serious infection; most had poor weight gain and severe respiratory difficulties; many had additional anatomic abnormalities. Structural modeling of GJB2 p.G45E identified no impact on the salt bridge previously predicted to account for abnormal central carbon dioxide sensing of GJB2 p.A88V. Limitations This clinical review was retrospective. Conclusion GJB2 p.G45E and p.A88V are the only KID syndrome mutations associated with uniform early lethality. Those electrophysiologically severe mutations in GJB2 reveal abnormalities in many organs in lethal KID syndrome. All patients with KID syndrome may have subtle abnormalities beyond the eyes, ears, and skin. Early genotyping of KID syndrome births will inform prognostic discussion.

Summary We describe a case of an infant who presented with clinical features of hyperthyroidism. The child was found to be tachycardic, hypertensive and diaphoretic, she was noted to have poor weight gain and difficulty in sleeping. The child was admitted to the pediatric intensive care unit for care. She was found to have biochemical evidence of hyperthyroidism with positive thyroid stimulating immunoglobulin. She responded well to methimazole and propranolol and had a remarkable recovery. She is the youngest patient to be diagnosed with Graves disease in the English literature, at 12 months of life. Learning points Hyperthyroidism must always be considered even at very young age, for patient presenting with poor weight gain and hyperdynamic state. Autoimmune diseases are becoming more common in infancy. Craniosynostosis and increased height for age are well-documented consequences of untreated hyperthyroidism in developing children.

Background: Preterm delivery is among the major public health problems worldwide and the leading cause of morbidity and mortality among neonates. Postnatal poor weight gain, which can contribute to mortality, can be influenced by feeding practices, medical complications and quality of care that is provided to these high-risk neonates. This study aimed to investigate the proportion and predictors of poor weight gain among preterm neonates at Muhimbili National Hospital (MNH), from September 2018 to February 2019.Methods: A hospital-based prospective cohort study involving preterm neonates with Gestation age (GA) Results: A total of 227 preterm neonates < 37 weeks GA, with male to female ratio of 1:1.2 were enrolled in the study. The overall proportion of preterm with poor weight gain was 86.8% (197/227). Proportion of poor weight gain among the early and late preterm babies, were 88.5% (100/113) and 85.1% (97/114) respectively. Predictors of poor weight gain were low level of maternal education (AOR=2.58; 95%Cl: 1.02-6.53), cup feeding as the initial method of feeding (AOR=8.65; 95%Cl: 1.59-16.24) and delayed initiation of the first feed (AOR=10.06; 95%Cl: 4.14-24.43). A previous history of preterm delivery was protective against poor weight gain (AOR=0.33; 95% Cl: 0.11-0.79). Conclusion and recommendation: Poor weight gain was a significant problem among preterm receiving care at MNH. This can be addressed by emphasizing on early initiation of feed and tube feeding for neonates who are not able to breastfeed. Health education and counselling to mothers focusing on feeding practices as well as close supervision of feeding especially for mothers experiencing difficulties in feeding their preterm will potentially minimize risk of growth failure.

Background Preterm delivery is among the major public health problems worldwide and the leading cause of morbidity and mortality among neonates. Postnatal poor weight gain, which can contribute to mortality, can be influenced by feeding practices, medical complications and quality of care that is provided to these high-risk neonates. This study aimed to investigate the proportion and predictors of poor weight gain among preterm neonates at Muhimbili National Hospital (MNH), from September 2018 to February 2019. Methods A hospital-based prospective cohort study involving preterm neonates with Gestation age (GA) Results A total of 227 preterm neonates Conclusion and recommendation Poor weight gain was a significant problem among preterm neonates receiving care at MNH. This can be addressed by emphasizing on early initiation of feed and tube feeding for neonates who are not able to breastfeed. Health education and counselling to mothers focusing on feeding practices as well as close supervision of feeding especially for mothers experiencing difficulties in feeding their preterm will potentially minimize risk of growth failure.

Introduction: BPD is the commonest adverse outcome of premature birth, hence appropriate management is essential. Methods: We undertook a survey of UK neonatal units to determine their management of infants with BPD. Results: Fifty-two neonatal units responded: 37% were level 3 NICUs, 49% level 2 and 14% level 1 units. Sixty percent of units had a BPD management guideline. Thirty-six units used patient triggered ventilation and 14 high frequency oscillatory ventilation. The majority (69%) used volume targeting. Nitric oxide was used by 29.9% of units with no consensus over dosage, range 5-20ppm. Diuretics were used by the majority (64.7%) for fluid overload. They were used routinely by 9.8% of units, reasons included poor weight gain (3.9%) or to facilitate weaning from invasive ventilation (2%). Chlorthiazide, spironolactone and furosemide were the most commonly used diuretics, but in varying combinations. Only 24% of units undertook regular renal ultrasound examinations for infants receiving chronic diuretic therapy. Nineteen units used a course of systemic corticosteroids >9 days, whereas 11 units a course Conclusion: Our survey demonstrates there is no consensus for the management of infants with BPD and emphasizes the need for appropriately designed trials to establish evidence based management.

BackgroundPyriform aperture stenosis is a rare form of congenital nasal obstruction; it poses a management dilemma for otolaryngologists and physicians alike. It can result in poor weight gain and potentially life-threatening airflow obstruction. The challenge lies in the difficulty to predict which patients will require invasive operative management versus conservative therapy alone.Case reportThis case demonstrates the successful use of high-flow nasal cannula therapy in a young child with pyriform aperture stenosis.

Cases of isolated duodenal stenosis in the neonatal period are minimally reported in pediatric literature. Causes of small bowel obstruction such as duodenal atresia or malrotation with midgut volvulus have been well documented and are often diagnosed due to their acute clinical presentation. Duodenal stenosis, however, causes an incomplete intestinal obstruction with a more indolent and varying clinical presentation thus making it a diagnostic challenge. We present a neonate with a unique case of congenital duodenal stenosis. The neonate presented with poor weight gain and frequent "spit-ups" as per the mother at the initial newborn visit. The clinical presentation was masked as the patient was being fed infrequently and with concentrated formula. We postulate that this may be due to the fact that the mother was an adolescent and relatively inexperienced with newborn care. During the hospital course, the patient had recurrent episodes of emesis with notable electrolyte abnormalities including hypochloremia and metabolic alkalosis. Further investigation with an abdominal X-ray showed dilated loops of bowel. Pyloric stenosis was ruled out via abdominal ultrasound. An upper gastrointestinal (GI) series ultimately confirmed a diagnosis of duodenal stenosis and the infant underwent surgical repair with full recovery. Congenital duodenal stenosis may have atypical presentations in neonates requiring pediatricians to have a high index of suspicion for diagnosis and to ensure timely therapy.

Neonates with CHD are at increased risk of developing necrotising enterocolitis due to mesenteric hypoperfusion. Necrotising enterocolitis results in repeated feed interruptions contributing to poor growth during the early post-operative phase. Poor weight gain and longer hospital stay are risk factors for death in neonates with CHD. Abdominal radiography is used as a diagnostic tool for necrotising enterocolitis; however, its utility is limited in the early stages of necrotising enterocolitis when pneumatosis intestinalis is absent. Calprotectin is a neutrophil activation biomarker, and elevated levels are evident in inflammatory diseases such as necrotising enterocolitis. The aim of this study was to determine whether there is a correlation between faecal calprotectin concentration and gut inflammation in neonates with CHD. This prospective single-centre study recruited newly diagnosed term patients with duct-dependent CHD between March 2018 and March 2019. Faecal calprotectin concentrations were measured in post-surgical patients using enzyme-linked immunosorbent assay methods. A total of 30 patients were included in the analysis. Calprotectin concentration for patients who developed necrotising enterocolitis was 3528 µg/g compared with 390 µg/g without, compared with 1339 µg/g in patients with suspected necrotising enterocolitis (p = 0.0001). Patients with suspected necrotising enterocolitis had a significantly longer length of hospital stay, on average 18 days longer compared to patients without necrotising enterocolitis (p = 0.03). Faecal calprotectin concentrations may reflect severity of gut inflammation in neonates with CHD. Suspected necrotising enterocolitis contributes to longer days nil by mouth and an increase in length of hospital stay.