scholarly journals A case report on long segment hirschprung’s disease

2021 ◽  
Vol 8 (3) ◽  
pp. 235-237
Author(s):  
K. Arun Chander Yadav ◽  
D. Suganya ◽  
R. Kannan ◽  
A. Priya

Hirschsprung disease is a complex congenital condition of the intestine, usually recognized to be genetic origin and reverberates from a disturbance of the normal development of the enteric nervous system. It is evident to be the most common cause of a low intestinal obstruction in the neonates as well as the older children. Study reveals that occurrence rate of cases in 70% may be associated with other congenital abnormalities together with number of syndromic phenotypes. A number of distinct genetic sites have been identified in these syndromic phenotypes, identifying potential genetic predispositions. Presenting hereby a case of neonatal hirschsprung’s disease whereby the patient was surgically corrected along with stepwise pharmacological therapy and was able to get discharged with regular follow up advice.

2018 ◽  
Author(s):  
Charles Kalish ◽  
Nigel Noll

Existing research suggests that adults and older children experience a tradeoff where instruction and feedback help them solve a problem efficiently, but lead them to ignore currently irrelevant information that might be useful in the future. It is unclear whether young children experience the same tradeoff. Eighty-seven children (ages five- to eight-years) and 42 adults participated in supervised feature prediction tasks either with or without an instructional hint. Follow-up tasks assessed learning of feature correlations and feature frequencies. Younger children tended to learn frequencies of both relevant and irrelevant features without instruction, but not the diagnostic feature correlation needed for the prediction task. With instruction, younger children did learn the diagnostic feature correlation, but then failed to learn the frequencies of irrelevant features. Instruction helped older children learn the correlation without limiting attention to frequencies. Adults learned the diagnostic correlation even without instruction, but with instruction no longer learned about irrelevant frequencies. These results indicate that young children do show some costs of learning with instruction characteristic of older children and adults. However, they also receive some of the benefits. The current study illustrates just what those tradeoffs might be, and how they might change over development.


2021 ◽  
Vol 6 (1) ◽  
pp. e000677
Author(s):  
Evangelia Ntoula ◽  
Daniel Nowinski ◽  
Gerd Holmstrom ◽  
Eva Larsson

AimsCraniosynostosis is a congenital condition characterised by premature fusion of one or more cranial sutures. The aim of this study was to analyse ophthalmic function before and after cranial surgery, in children with various types of non-syndromic craniosynostosis.MethodsChildren referred to Uppsala University Hospital for surgery of non-syndromic craniosynostosis were examined preoperatively. Visual acuity was measured with Preferential Looking tests or observation of fixation and following. Strabismus and eye motility were noted. Refraction was measured in cycloplegia and funduscopy was performed. Follow-up examinations were performed 6–12 months postoperatively at the children’s local hospitals.ResultsOne hundred twenty-two children with mean age 6.2 months were examined preoperatively. Refractive values were similar between the different subtypes of craniosynostosis, except for astigmatism anisometropia which was more common in unicoronal craniosynostosis. Strabismus was found in seven children, of which four had unicoronal craniosynostosis.Postoperatively, 113 children were examined, at mean age 15.9 months. The refractive values decreased, except for astigmatism and anisometropia in unicoronal craniosynostosis. Strabismus remained in unicoronal craniosynostosis. Two new cases with strabismus developed in unicoronal craniosynostosis and one in metopic, all operated with fronto-orbital techniques. No child had disc oedema or pale discs preoperatively or postoperatively.ConclusionOphthalmic dysfunctions were not frequent in children with sagittal craniosynostosis and preoperative ophthalmological evaluation may not be imperative. Children with unicoronal craniosynostosis had the highest prevalence of strabismus and anisometropia. Fronto-orbital techniques used to address skull deformity may be related to a higher prevalence of strabismus postoperatively.


2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Filippo Migliorini ◽  
Nicola Maffulli ◽  
Filippo Spiezia ◽  
Giuseppe Maria Peretti ◽  
Markus Tingart ◽  
...  

Abstract Background Biochemical markers of bone turnover (BTMs), such as the bone alkaline phosphatase (bALP), procollagen type I N propeptide (PINP), serum cross-linked C-telopeptides of type I collagen (bCTx), and urinary cross-linked N-telopeptides of type I collagen (NTx), are used to manage therapy monitoring in osteoporotic patients. This systematic review analyzed the potential of these BMTs in predicting the clinical outcomes in terms of BMD, t-score, rate of fractures, and adverse events during the therapy setting in postmenopausal osteoporosis. Methods All randomized clinical trials (RCTs) reporting data on biomarkers for postmenopausal osteoporosis were accessed. Only articles reporting quantitative data on the level of biomarkers at baseline and on the outcomes of interest at the last follow-up were eligible. Results A total of 36,706 patients were retrieved. Greater values of bALP were associated with a greater rate of vertebral (P = 0.001) and non-vertebral fractures (P = 0.0001). Greater values of NTx at baseline were associated with a greater rate of adverse events at the last follow-up (P = 0.02). Greater values of CTx at baseline were associated with a greater rate of adverse events leading to discontinuation (P = 0.04), gastrointestinal adverse events (P = 0.0001), musculoskeletal adverse events (P = 0.04), and mortality (P = 0.04). Greater values of PINP at baseline were associated with greater rates of gastrointestinal adverse events (P = 0.02) at the last follow-up. Conclusion The present analysis supports the adoption of BMTs during pharmacological therapy setting of patients suffering from osteoporosis. Level of evidence I, systematic review of RCTs


Antibiotics ◽  
2021 ◽  
Vol 10 (2) ◽  
pp. 195
Author(s):  
Elena M. Varoni ◽  
Niccolò Lombardi ◽  
Giulio Villa ◽  
Alberto Pispero ◽  
Andrea Sardella ◽  
...  

Background: Medication-related osteonecrosis of the jaw (MRONJ) is a severe side effect of bisphosphonates and anti-resorptive drugs prescribed for treatment of severe osteoporosis, Paget’s disease, and bone malignancies. The aim of this study was to evaluate the clinical outcome of a combined pharmacological and surgical management strategy on patients affected by MRONJ. Materials and methods: Medical records of patients with MRONJ were retrospectively examined to collect clinical history data. Conservative management included an initial pharmacological phase with antibiotics and antiseptic agents, followed by surgical intervention to remove bone sequestrum. Primary outcomes were healing from MRONJ at short term (1 month after surgery) and at longer term (3 months after surgery). Secondary outcome was assessment of recurrences at longer-term follow-up. Results: Thirty-five patients were included in the study with mean follow-up of 23.86 ± 18.14 months. Seven cases showed spontaneous exfoliation of necrotic bone during pharmacological therapy, which in one case did not require any further intervention. At 1-month posttreatment, 31 out of 35 (88.5%) patients showed complete healing. The 25 patients who were followed for at least 3 months revealed a healing rate of 92% (23/25). Recurrences occurred in 7 patients out 23 who showed the long-term healing, after a mean period of 7.29 ± 3.45 months. The prognostic score (University of Connecticut Osteonecrosis Numerical Scale—UCONNS) was significantly higher (p = 0.01) in patients with poor healing as compared to complete healing, both at 1 and 3 months posttreatment. Conclusions: A MRONJ treatment approach based on a combined pharmacological and surgical treatment strategy showed a high rate of healing and few recurrences.


PEDIATRICS ◽  
1992 ◽  
Vol 89 (5) ◽  
pp. 834-838 ◽  
Author(s):  
Richard C. Wasserman ◽  
Candace A. Croft ◽  
Sarah E. Brotherton

In this cross-sectional study, the vision-screening process is described for 8417 children aged 3 to 5 seen for health supervision in a group of 102 pediatric practices in 23 states and Puerto Rico. Three hundred forty children who failed screening (63% of those who failed) were followed up 2 months after initial screening. The sample was 52% male, 86% white, 9% black, 3% Hispanic, and 1% Asian. Vision screening was attempted on 66% of children overall. Pediatricians' reasons for not screening were "not routine" (44%), "too young" (40%), and "screening done previously" (17%). Younger children were less likely to be screened than older children (39% of those aged 3), and Hispanics were less likely to be screened than other ethnic groups (P < .001). Thirty-three percent of children received no screening for latent strabismus. Two months later, 50% of parents whose child had failed a vision test were unaware of this fact on questionnaire follow-up. Eighty-five percent of children referred to an eye specialist had made or kept an appointment. It is concluded that pediatricians need to increase vision screening among younger preschool children and communicate more effectively to parents the results of screening failure.


2015 ◽  
Vol 87 (3) ◽  
pp. 243
Author(s):  
Massimo Massari ◽  
Patrizia Desideri ◽  
Paolo Menchinelli ◽  
Lucia Cerrito ◽  
Luciano De Giovanni

Aim of the study: Urge incontinence is considered to be a dysfunctional pathology of social interest due to the psychological and relational implications of such disability, the elevated number of affected patients and the consequent treatment costs. We propose an innovative non-pharmacological and non-invasive care methodology: Frequency rhythmic electrical modulation system (F.R.E.M.S.) therapy (FT), based on the administration of electric fields of monophasic pulsed, negative, asymmetric current, generated by a neurostimulator with the characteristics of low variable frequency, high voltage and very low impulse duration. Material and Methods: 30 patients were studied with urodynamic evaluation and radiological diagnostic techniques, and underwent 2 cycles of 15 days therapy, with a 12 months follow-up. Results: In 93% of cases, we obtained a positive result, with either disappearance or improvement of symptoms. Conclusion: Although the Authors believe that clinical results deserve further neurohistological and immunohistochemical studies, in order to define the anathomophysiological and biochemical changes induced by FT, they propose it as a possible alternative to traditional pharmacological therapy and electrical stimulation.


1996 ◽  
Vol 40 (12) ◽  
pp. 2732-2736 ◽  
Author(s):  
P Marchisio ◽  
N Principi ◽  
E Sala ◽  
L Lanzoni ◽  
S Sorella ◽  
...  

Continuous chemoprophylaxis is effective in the prevention of new episodes of acute otitis media (AOM) in otitis-prone children, but compliance can be a problem and thus efficacy can be decreased. Intermittent chemoprophylaxis has so far shown conflicting results. Azithromycin, which has a peculiar pharmacokinetics, resulting, even after a single dose, in persistently elevated concentrations in respiratory tissues, could permit a periodic administration with higher compliance. We compared a 6-month course of once-weekly azithromycin (5 or 10 mg/kg of body weight) with that of once-daily amoxicillin (20 mg/kg) in a single-blind, randomized study of prophylaxis for recurrent AOM in 159 children aged 6 months to 5 years with at least three episodes of AOM in the preceding 6 months. In the amoxicillin group, 23 (31.1%) of 74 children developed 29 episodes of AOM, while in the 10-mg/kg azithromycin group, 11 (14.9%) of 74 children experienced 15 episodes. The 5-mg/kg/week azithromycin trial was prematurely interrupted after nine cases, due to the high occurrence rate of AOM (55.5%). During the 6-month prophylaxis period, the proportion of children with middle ear effusion declined similarly in both groups. No substantial modification of the nasopharyngeal flora was noted at the end of prophylaxis in both antimicrobial groups. In the 6-month-postprophylaxis follow-up period, about 40% of children in both groups again developed AOM. Azithromycin at 10 mg/kg once weekly can be regarded as a valid alternative to once-daily low-dose amoxicillin for the prophylaxis of AOM. Although in the present study no microbiological drawback was noted, accurate selection of children eligible for prophylaxis is mandatory to avoid the risk of emergence of resistant strains.


2017 ◽  
Vol 07 (01) ◽  
pp. e38-e41
Author(s):  
P. Dahlem ◽  
P. Biggar

AbstractMortality in newborn infants and children with sepsis is high with survival rates of generally more than 50% in recent studies. Longitudinal follow-up studies have the potential to reveal short-term and lifelong physical, mental, and psychological sequelae. Although no comprehensive follow-up research has yet been performed, a small number of follow-up studies have shown that there is a considerable impact on the patients' lives and their families after hospital discharge. Health-related quality of life also seems to be affected; however, it does not correlate with severity of sepsis or handicap per se. Prematurely born infants, who can develop sequelae directly attributable to prematurity and its consequences, suffer differently from sepsis-related lifelong sequelae compared with older children. Fortunately, time may heal some wounds due to the effect of growth in children. In future, large centers should establish structural follow-up programs for clinical and research purposes to learn more about the needs of affected children and their families.


1997 ◽  
Vol 13 (2) ◽  
pp. 205-211 ◽  
Author(s):  
Guilherme Borges ◽  
Roberto Tapia-Conyer ◽  
Malaquías López-Cervantes ◽  
María Elena Medina-Mora ◽  
Blanca Pelcastre ◽  
...  

In 1988, the General Directorate of Epidemiology and the Mexican Institute of Psychiatry conducted the first National Addiction Survey (ENA), providing regional and national data on alcohol, tobacco, and drug use. The ENA providing a subsample of women who have been pregnant at some time in their lives. There were 5,234 affirmative responses. Women were asked if they had suffered any of three adverse outcomes during their last pregnancy: spontaneous abortion, stillbirth, and congenital abnormalities. Prevalence of spontaneous abortion was 3.8%, stillbirth 1.2%, and congenital abnormalities 1.1 %. Multiple logistic-regression models were used to analyze the effect of alcohol consumption on these problems. Consumption during pregnancy was related only with the prevalence of congenital abnormalities, with prevalence odds of 3.4. Among habitual users during the last 12 months, oniy women in the highest use category showed an important relationship with the three problems mentioned. Follow-up studies on the Mexican population are recommended in order to obtain more conclusive findings.


2021 ◽  
Author(s):  
Yili Zhu ◽  
Jun Fan ◽  
Bo Huang ◽  
Ying Wu ◽  
Heshui Shi ◽  
...  

Abstract Background: Since the discovery of the first case of Anaplastic lymphoma kinase (ALK) -positive histiocytosis in 2008, originally described as a systemic, self-limiting disease in infants, the range of ALK-positive histiocytosis has recently been expanded to include localized diseases in older children and young adults. Case presentation: We present the case of an 18-year-old female with periumbilical painless mass for 5 months, who underwent a resection of the mass. Pathological examination showed the tumor consists predominantly of fascicular to storiform growth of nonatypical spindle cells, admixed with lymphocytic infiltrates. The tumor spindle cells were diffusely positive for CD68, CD163 and ALK. Further, molecular tests revealed ALK gene fusion: Kinesin Family Member 5B (KIF5B) (E24)-ALK (E20), confirmed ALK-positive histiocytosis. The tumor has not recurred one and a half years after resection by follow-up examination.Conclusion: ALK-positive histiocytosis in local lesion can achieve remission by complete resection and clinical follow-up showed a favorable prognosis.


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