Premature ovarian insufficiency (POI) is a rare disease, especially in children and adolescents. It was previously called premature ovarian failure (POF). It can be manifested as delayed puberty, primary or secondary amenorrhea that occurred before the age of 40 years with no less than two abnormal serum sex hormones (low estrogen and high gonadotropin). It is reported that the incidence rate is 1% at the age of 40 years and 0.01% at the age of 20 years. Although the disease usually occurs in middle-aged and elderly women, clinical practice in recent years has shown that it has also been found in adolescents and even children. It is generally believed that the etiology of POI includes genetic factors, immune factors, and iatrogenic factors. So far, several genetic mutations that may cause POI have been found clinically, but the etiology of 90% of POI is still unknown. In recent years, the incidence of POI in children and adolescents has increased, and there are more urgent requirements for its early diagnosis, treatment, and clinical management. Based on this, this article will mainly review the research progress of the etiology, treatment, and clinical management of POI in children and adolescents.
Shellfish are extensively consumed worldwide because of their nutritional value. In general they are good sources of low-fat protein rich in several essential vitamins and minerals as well as in the essential nutrients omega-3 long-chain polyunsaturated fatty acids (n-3 LCPUFAs) .
Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a severe multiorgan hypersensitivity reaction mostly caused by several eliciting drugs in patients with a genetic predisposition. Incidence of DRESS in children is very variable, frome 1:1000 to 1:10.000, and the mortality rate seems to be lower than 10%. Anti-convulsants are the main drugs involved both in adults and in children. The treatment of choice is the prompt withdrawn of the offending drug and using intravenous immunoglobulins and corticosteroids used in synergy. In recent years, emerging studies have outlined the disease more clearly. We present a pediatric case in which the patient developed DRESS syndrome as a result of exposure to lamotrigine before and carbamazepine after and a relapse after exposure to omeprazole. Starting from this case report we provide an overview on DRESS Syndrome.
Background: Interleukin-6 (IL-6) promotes antibody production. The objective of this study was to investigate whether IL-6 C-572G single nucleotide polymorphisms (SNP) and clinical factors are associated with positive platelet antibody test.
Materials and methods: Thirty platelet recipients with platelet antibodies (responders) and 20 platelet recipients without platelet antibodies (non-responders) were randomly selected. The -572 C>G (rs 1800796) SNPs in the promoter region of IL-6 gene were genotyped by polymerase chain reaction (PCR)-restriction fragment length polymorphism (RFLP) method. Solid phase red cell adherence assay (SPRCA) was used for platelet antibody detection.
Results: Age, sex, percentage patients with benign diseases, and percentage of patients with homozygotes for the C allele at position -572 of the IL-6 gene were similar between responders and non-responders. Although the amounts of platelets pheresis transfused to patients with hematologic diseases were higher than those of non-hematologic diseases (47.2 ± 54.2 vs. 17.4 ± 13.8 units, p = 0.019), detection rate of platelet antibodies was lower in patients with hematologic diseases than that in patients with non-hematologic diseases (42.3% vs. 79.2%, p = 0.01).
Conclusion: There was no association between IL-6 C-572G gene polymorphism and positive reactivity in solid phase platelet antibody detection method in platelet recipients.
We explored if adolescents with Gilles de la Tourette syndrome (GTS) had functional postural control impairments and how these deficits are linked to a disturbance in the processing and integration of sensory information. We evaluated the displacements of the center of pressure (COP) during maximal leaning in four directions (forward, backward, rightward, leftward) and under three sensory conditions (eyes open, eyes closed, eyes closed standing on foam). GTS adolescents showed deficits in postural stability and in lateral postural adjustments but they had similar maximal COP excursion than the control group. The postural performance of the GTS group was poorer in the eyes open condition (time to phase 1 onset, max-mean COP). Moreover, they displayed a poorer ability to maintain the maximum leaning position under the eyes open condition during mediolateral leaning tasks. By contrast, during forward leaning, they showed larger min-max ranges than control subjects while standing on the foam with the eyes closed. Together, these findings support the idea that GTS produces subclinical postural control deficits. Importantly, our results suggest that postural control disorders in GTS are highly sensitive to voluntary postural leaning tasks which have high demand for multimodal sensory integration.
Acute gastroenteritis (AGE) is a common presenting complaint in paediatrics. Most often, the reason for admission into hospital is to initiate intravenous rehydration in patients with severe dehydration and inability to tolerate oral intake. We found that Ondansetron acts as a potent antiemetic to support an increased number of children receiving oral hydration, and subsequently leading to decreased rates of admission. This study aims to audit the use of Ondansetron to Oral Rehydration Therapy (ORT) on children with acute gastroenteritis, and its effect on admission rates from the emergency department in University Hospital, Limerick (UHL). Data collected over a 3-month period from June to August 2017 in which Ondansetron was not used was compared to another 3-month period when Ondansetron was used. Several outcomes were measured including admission to hospital.
The rate of admission decreased by 15% [26/74 (35%) in 2017 to 16/81 (20%) in 2019
p = 0.22]. 81 patients received Ondansetron, of which 79% were successfully rehydrated orally. The administration of Ondansetron reduced the need for intravenous fluids and hospital admission overall in these children with AGE. This reduction ultimately accounted for lower costs incurred by the Health Services Executive per patient, and also suggested the anti-emetic use as a cost effective measure for managing and treating patients with AGE.
Background: Diarrhea and nutrient deficiency worsen each other, and zinc is recommended to be included in clinical management of diarrhea. Therefore, this investigation was done to assess zinc prescription practice to children with diarrhea, identify factors associated with zinc prescription, and assess caregivers’ zinc’s perceived cost and willingness to pay for.
Methods: A health institution based cross-sectional study was done. Caretakers of 609 children with diarrhea attending health centers in Addis Ababa were included. Logistic regression was applied to identify variables associated with zinc prescription.
Results: Zinc was prescribed to 62.1% of children. About 74.9% of the caregivers were willing to pay for zinc. Previous use of zinc (AOR = 2.3; 95% CI: 1.34-4.01), exposure to zinc related message (AOR = 2.6, 95% CI: 1.53-4.60) and willingness to pay for zinc (AOR = 6.9; 95% CI: 3.84-12.66) were associated with zinc prescription.
Conclusion: Zinc was not administered to considerable proportion of children with diarrhea. Previous use of zinc, exposure to zinc related message and willingness to pay for positively contributed to zinc prescription. Health care workers shall be encouraged on zinc prescription. Intervention to increase willingness to pay for zinc and zinc benefit communication shall be strengthened in parallel with operational researches.
A term neonate was transferred from a Local Neonatal Unit to our surgical Neonatal Intensive Care Unit on Day 2 due to abdominal distension with radiological appearances suggestive of intestinal obstruction. He was born by Caesarean section with no risk factors for sepsis.
Background: Recent years have seen chaos in the neonatology use of antibiotics with diverse opinions and recommendations in international guidelines and societies. This has created great uncertainty in which cases to use, for how long, and which tests apply to make these decisions.
We conducted a retrospective cohort study about the use of antibiotics in the EpicLatino neonatal units and a Latin American network database, after noting these variations in the 2019 report.
Methods: For the year 2019 using the EpicLatino database, we included cases (only first admission) ≤ 32 weeks gestational age at birth, excluding one unit that did not accept to participate.
The number of cases and days receiving antibiotics were recorded as well as the progression for each unit. Inappropriate use of antibiotics was defined as greater than 3 days in patients with negative cultures (blood/CSF cultures) excluding: major malformations, urinary tract infections, necrotizing enterocolitis (NEC) and cases with suspected chorioamnionitis in the mother (the latter two only during the course of diagnosis of NEC or chorioamnionitis).
Results: A total of 6,543 days of antibiotics were observed, 49.5% of cases had at least one positive blood/CSF culture.
A total of 595 days of antibiotics without justification were found in 72 courses in 61 cases: 19.4% had no diagnosis of infection in the database, 9.7% did not document any culture throughout their stay, and 51,4% obtained only one blood/CSF culture during their entire stay. In the 58 cases with diagnosis of infection: 41% were clinical sepsis and a diagnosis of pneumonia with a poor positive culture correlation was found. Furthermore, 74% of the unit’s didn´t use pneumonia as a justification to use antibiotics.
Other diagnosis found: Conjunctivitis, NEC 1A and rotavirus NEC.
Conclusion: Although the method of reviewing the use of antibiotics in a database has a number of limitations, especially the cause that motivated the use of antibiotics and other tools used for diagnosis of infections, the notable differences between units is striking.
Although it is difficult to make recommendations to all units, it is important to control infections in some units and in others to reduce the excessive use of antibiotics, especially with diagnosis of pneumonia in neonates and negative blood/CSF cultures.
Despite critical care advances, robust antibiotic therapy and improved strategies in early detection and prevention of infection, the incidence of morbidity and mortality from neonatal sepsis worldwide in preterm and low birth weight neonates remains overwhelmingly high. Neonatal sepsis is characterised by a clinical syndrome of systemic signs of infection and bloodstream bacteraemia in newborns within the first months of life.
The risk of sepsis in neonates is inversely proportional to gestational age and birth weight due to deficiency in humoral immunity and the need for more invasive supportive neonatal intensive care unit interventions. Adverse effects such as necrotising enterocolitis associated with antimicrobial therapy are serious enough to warrant exploration of alternative therapeutic strategies. Immunoglobulin replacement therapy offers hope of enhancing immune competence and reducing infection rates in vulnerable populations.
It is evident from the relevant studies to date that the benefits offered by intravenous immunoglobulin prophylaxis may not be significant enough for routine hospital implementation. Further research to better understand the mechanisms underlying immunodeficiency will lead to the realisation of alternative therapeutic and prophylactic interventions.
Background: In Wilms’ tumor, the time elapsed between clinical diagnosis and the start of treatment is clearly associated with morbidity and mortality. As treatment delay can influence patient survival, identification of possible causes can mitigate the consequences arising from prolonged diagnostic uncertainty.
Objective: To ascertain whether an initial diagnosis of Wilms’ tumor in the emergency department influences patient prognosis depending on the type of referral for definitive treatment.
Patients and methods: Retrospective chart review of 98 children receiving treatment for Wilms’ tumor at the Brazilian National Cancer Institute (INCA) between April 2003 and December 2016. Patients were categorized into two groups: those referred directly from an emergency public department to INCA and those first transferred to another hospital before being referred to INCA.
Results: Of the 98 cases included in the study, 42.9% were direct referrals and 57.1% were indirect referrals. Presence of an abdominal mass was the most common presenting complaint, followed by abdominal pain. In cases with larger tumors, the mean tumor volume was greater than reported elsewhere in the literature, suggesting longer disease duration. Significantly higher tumor volumes were observed in patients with a palpable abdominal mass as compared to those with the second most frequent complaint (abdominal pain).
Conclusion: The findings of this study support the hypothesis that patients diagnosed with kidney masses in the emergency department are at greater risk of delayed diagnosis when they are referred first to a non-specialized outside hospital than when referred directly to a specialized cancer treatment unit.
Introduction: In Madagascar, plague is a highly contagious acute endemic infectious disease. The diagnosis of the most severe form of pneumonic plague remains difficult in children, hence the objectives of the present study; which is to identify the clinical signs of this clinical form in children and to describe its epidemiological and evolutionary profile.
Methods: A retrospective case-control study was conducted in four pediatric wards in Antananarivo during the urban pneumonic plague outbreak from September 2017 to January 2018.
Those cases were defined as children aged 0-15 years old suspected of having plague with positive RDT and PCR, and they were defined as children aged 0-15 years old with negative RDT and PCR.
Results: Fifty-two cases of pneumonic plague were identified, half of which (50%) were under 24 months of age.
A male predominance was noted with a sex ratio of 1.23 and 86.54% of the patients were from urban areas.
Several clinical signs were found but none was specific for pneumonic plague: cough (59.62% p: 0.5), dyspnea (3.85% p: 0.16), chest pain (3.85% p: 0.26%), hemoptysis (7.69% p: 0.17), vomiting (9.62% p: 0.14), diarrhea (11.54% p: 0.45), altered general condition (38.46% p: 0.24%).
Two deaths were noted (3.8%).
Conclusion: No specific clinical warning signs have been identified in childhood pneumonic plague. In the event of an epidemic of urban pneumonic plague, any bacterial pneumonia should at least initially include active treatment against Yersinia pestis.
Background: Rapidly involuting congenital hemangioma (RICH) is a rare vascular tumor that is present at birth and involutes during the first year of life. Kasabach-Merritt syndrome (KMS) is a complication of some vascular tumors such as kaposiform hemangioendothelioma and tufted angioma associated with thrombocytopenia and coagulopathy.
Results: The case of a 2-month-old infant with a diagnosis of RICH with thrombocytopenia and coagulation disorder, successfully treated with surgical excision without complications or recurrence is presented.
Conclusion: The association between RICH and KMS is rare. Histopathological study, immunohistochemistry and ultrasound findings are important for the diagnosis.
Brief summary: This report covers the rare association between rapidly involuting congenital hemangioma and Kasabach-Merritt syndrome in a 2-months-old female infant.
Microvillositary inclusion disease also known as microvillositary atrophy is a rare congenital enteropathy containing a border abnormality in the brushes of enterocytes, manifesting as severe rebellious diarrhea in newborns and infants. It was first described in 1978 by Davidson, et al. The autosomal recessive mode of transmission is suggested because of the frequency of familial cases and inbreeding. Histopathology plays an essential role in establishing the diagnosis. In 2008, a common mutation was identified in most of the patients studied in the MYO5B gene that codes for the Myosin Vb protein, which helped in understanding the etiopathogeny of this pathology poorly described in the literature. The prognosis for this pathology is extremely bleak, requiring total parenteral nutrition for child survival. Intestinal transplantation is for the moment the only long-term solution.
Materials and methods: We report the case of an infant aged 6 months, with no perinatal antecedent. There is 1st degree consanguinity, the mother has a history of deaths in younger siblings in undetermined circumstances. Who since the age of 3 days presents profuse liquid diarrhoea with malnutrition, dehydration and enormous abdominal distension? Several diagnoses were suspected before the jejune biopsy was carried out, which led to the diagnosis of a microvilliositary inclusion disease.
The aim of our work is to highlight the rarest cause of neonatal rebel diarrhoea and to know how to include it among other differential diagnoses.
Introduction: Sepsis remains a major cause of death in neonatal period. Although significant advances in diagnosis, therapeutic and prevention strategies have been noted, sepsis remains a common concern in clinical practice especially in low-resource countries. The aim of this study was to determine the predictors of mortality in neonatal sepsis in Lubumbashi city (Democratic Republic of Congo).
Methods: The records of newborns with sepsis managed in Neonatal Intensive Care Units in two University Hospitals between November 2019 and October 2020 were studied. Binary and multiple logistic regressions have been used to observe the association between independent variables and dependent variable.
Results: A total of 162 cases of neonatal sepsis were reviewed. The mortality rate of neonatal sepsis was 21% of babies admitted. Very low birth weight (< 1500 grams) and primiparity were significantly associated with mortality in neonatal sepsis (AOR = 12.66; 95% CI 2.40 to 66.86; p = 0.003 and AOR = 3.35; 95% Cl 1.31 to 8.59; p = 0.012, respectively).
Conclusion: The mortality rate of neonatal sepsis was 21%. Very low birth weight and primiparity were significantly associated with mortality in neonatal sepsis.
Introduction: Pneumonia, defined as infection of lung parenchyma, is associated with severe complications especially in the very young and old patients. It is the world’s leading cause of childhood mortality. The World Health Organization (WHO) classification and guidelines are commonly used in Sudan in the diagnosis and management of pneumonia patients. This review was the outcome of some researches done in Sudan by the author and his colleagues. Management Systems were evaluated to give complete end to end solutions for serving patients along with their records in hospitals and clinics in Sudan. The objective of the study was: To reflect author experience in management of childhood pneumonia in Sudan and to determine feasible, affordable approach to pneumonia in Sudan.
Methodology: Searching through PubMed for the author publication and review of publication by author in Sudan regarding management of pneumonia.
Conclusion: Simple tests like chest X-ray, high WBC high-reactive protein, together with high temperature can predict the need for urgent blood culture. Antibiotic treatment for childhood pneumonia weather that recommended by WHO, b-lactam inhibitors or 3rd generation cephalosporin has the same outcome.
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