Paediatrics and International Child Health最新文献

A 16-year-old female presented to an outpatient clinic with a 13-year history of recurrent episodes of abdominal pain, vomiting and mild cutaneous swelling, either spontaneously or following minor trauma. The episodes occurred every 1-2 months. There was no family history of a similar complaint or hereditary angio-oedema (HAE). At the age of 16, evaluation confirmed the diagnosis of HAE type II, characterised by low C4 levels and reduced C1 esterase inhibitor function. The patient was prescribed tranexamic acid 1 g twice daily as well as C1 esterase inhibitor used as rescue medication during symptomatic episodes. This case report emphasises the importance of considering a diagnosis of HAE in patients with recurrent, unexplained abdominal pain, even in the absence of a positive family history of HAE.Abbreviations: ANA Antinuclear antibodies; C1-INH C1-inhibitor; CBC Complete blood count; FMF Familial Mediterranean fever; HAE Hereditary angioedema; IBD Inflammatory bowel diseases; SDP Solvent detergent-treated plasma; SLE Lupus erythematosus.

Background: Enteritis is one of the rare systemic manifestations in childhood-onset systemic lupus erythematosus and its diagnosis is very challenging. This is a rare case of an adolescent girl with recurrent non-specific gastro-intestinal symptoms which were later diagnosed to be owing to lupus enteritis, the only presenting manifestation of an active flare.

Case report: A 15-year-old girl was admitted with recurrent episodes of abdominal pain, vomiting and loose stools. She had diffuse abdominal tenderness. Abdominal ultrasonography demonstrated moderate ascites. A contrast-enhanced abdominal computerised tomography scan revealed thickening of the small bowel wall. On colonoscopy, there were rectal erosions, and microscopic examination of the biopsy specimens demonstrated mild inflammation. Non-specific enteritis was diagnosed and she was given antibiotics and supportive care. She was re-admitted 6months later with abdominal pain. An abdominal contrast-enhanced computerised tomography scan revealed thickening of the bowel wall and the target sign and comb sign in the small intestine. The anti-nuclear antibody was positive. Renal biopsy demonstrated grade 2 lupus nephritis. Lupus enteritis was diagnosed and the case satisfied the 2019 EULAR-ACR criteria and SLICC criteria. She was treated with methylprednisolone, cyclophosphamide and hydroxychloroquine. She improved with treatment and has remained asymptomatic during follow-up.

Conclusion: This case emphasises the need for healthcare providers to be alert to the possibility of lupus enteritis. It also highlights the importance of close follow-up of cases who have non-specific gastro-intestinal symptoms. Lupus enteritis should be considered in the differential diagnosis of recurrent non-specific gastro-intestinal symptoms in children, especially adolescents, to ensure timely diagnosis and treatment.Abbreviations: ACR American College of Rheumatology; ANA anti-nuclear antibody; CRP: C-reactive protein; CT: computerised tomography; CECT: contrast-enhanced computerised tomography; EULAR: European League Against Rheumatism; GI: gastro-intestinal; LE: lupus enteritis; SLE systemic lupus erythematosis; SLICC: Systemic Lupus International Collaborating Clinics; SLEDAI: SLE disease activity index.

Introduction: Raised serum bilirubin levels can cause kernicterus, and premature infants are at increased risk owing to metabolic immaturity. The standard treatment for neonatal jaundice is phototherapy, but probiotics alone can reduce the duration of phototherapy and hospitalisation.

Objectives: To determine the effectiveness of phototherapy with and without probiotics for the treatment of indirect hyperbilirubinaemia in preterm neonates.

Patients and methods: The open-labelled randomised controlled trial was conducted from January 2022 to January 2023 in the neonatal unit of the University of Lahore Teaching Hospital, Pakistan. A total of 76 preterm neonates who fulfilled the selection criteria were included and divided into two groups. Both groups received standard phototherapy. In Group B, a probiotic (Saccharomyces boulardii) 125 mg, twice daily, orally (in 5 cc of whichever milk the infant was receiving) was given until discharge from hospital. The primary outcome measurements were the duration of phototherapy and the length of hospitalisation.

Results: The mean (SD) duration of phototherapy was 36.55 (14.25) hours in Group A and 24.61 (9.25) hours in Group B (p <0.05). The mean (SD) duration of hospital stay was 47.36 (16.51) hours in Group A and 33.13 (8.93) hours in Group B (p <0.05).

Conclusion: Oral probiotics (Saccharomyces boulardii) have a significant effect on the duration of phototherapy for neonatal hyperbilirubinaemia, and they decrease the chances of nosocomial infection. Exploration of clinical outcomes by investigating faecal flora and undertaking large randomised controlled trials of various probiotics are needed.

Abbreviations: ABE: acute bilirubin encephalopathy; CNS: central nervous system; GA: gestational age; IVIG: intravenous immunoglobulin; KSD: kernicterus; NNU: neonatal unit; RCT: randomised controlled trial; S. boulardii: Saccharomyces boulardii.

Thiamine (vitamin B₁) is available in common foods such as the outer husk of rice and is necessary for normal cardiovascular, neurological and metabolic processes. Thiamine deficiency is common in many parts of Asia and Africa, affecting up to a third or more of children and women of child-bearing age. The diagnosis is based on clinical suspicion, especially when noting heart failure in infants, encephalopathy in patients of any age, and peripheral neuropathy in older children and adults. Blood tests for whole-blood thiamine diphosphate (the quantity of biologically active thiamine present) and erythrocyte transketolase activity (the functional impact of thiamine) are not always readily available in areas where thiamine deficiency is common. Treatment is safe and effective, although dosing guidelines vary widely; 50 mg daily for 5 days is probably effective for treating acute thiamine deficiency disorders, and ongoing adequate thiamine intake is also needed. Prevention efforts depend on local and regional circumstances, including dietary diversification, food fortification, and/or supplementation of children and women at risk.Abbreviations: HIC: high-income countries; LMIC: low- and middle-income countries; MRI: magnetic resonance imaging; TDD: thiamine deficiency disorders.

Scurvy, a condition caused by vitamin C deficiency, is rare, especially in high-income countries. Symptoms of scurvy are typically characterised by dermatological disorders such as poor wound healing and tooth loss, but there is not usually cardiac involvement. A case of reversible pulmonary hypertension and right-sided heart failure owing to scurvy in a 7-year-old boy with autism spectrum disorder is reported. He had a very restricted diet and presented with polyarthralgia, gingival hyperplasia with ecchymosis, and fatigue. His condition, including pulmonary hypertension and right-sided heart failure, completely resolved with vitamin C supplementation. Paediatricians should have a high index of suspicion for scurvy in children with nutritional selectivity and be aware that it can manifest with cardiac symptoms. Scurvy may be life-threatening if not treated, but the symptoms can improve rapidly with vitamin C supplementation.

Vitamin D plays an active role beyond mineral metabolism and skeletal health, including regulation of the immune system. Vitamin D deficiency is widely prevalent, and observational studies link low vitamin D status to a risk of infections and auto-immune disorders. Reports indicate an inverse relationship between vitamin D status and such conditions. This review details vitamin D signalling interactions with the immune system and provides experimental and clinical evidence evaluating vitamin D status, vitamin D supplementation and host susceptibility to infections, inflammation and auto-immunity. The published literature including related reviews, systematic reviews, meta-analyses, randomised controlled trials (RCTs), observational studies and basic science reports have been synthesised. Meta-analyses of observational studies have demonstrated a link between low vitamin D status and risk of acute respiratory infections, COVID-19 disorders, multiple sclerosis, type 1 diabetes (T1DM), inflammatory bowel disease (IBD), systemic lupus erythematosus and other auto-immune disorders. Observational studies suggest that vitamin D supplementation may protect against several infectious and auto-immune conditions. Meta-analyses of RCTs had mixed results, demonstrating a small protective role for vitamin D supplementation against acute respiratory infections, especially in those with vitamin D deficiency and children, and providing modest benefits for the management of T1DM and IBD. Vitamin D status is inversely associated with the incidence of several infectious and auto-immune conditions. Supplementation is recommended for those with vitamin D deficiency or at high risk of deficiency, and it might provide additional benefit in acute respiratory infections and certain auto-immune conditions.

Increasing numbers of populations in the West are restricting their intake of meat and other animal products for reasons relating to health or ethics; in many countries, these restrictions are already common for cultural, religious, or socio-economic reasons. By following their parent's diet, children are exposed in parallel. This narrative review aims at assessing current data regarding vegetarian diets in children from birth to 18 years of age, which include, by increasing degree of restriction, flexitarian, lacto-ovo-vegetarian, lacto-vegetarian, pescatarian, vegan and macrobiotic diets. The 202 references include 45 studies in children. The more restrictive the diet and the younger the child, the greater the risk of nutritional deficiency. Of particular concern are vitamin B₁₂, iron, zinc, calcium, n-3 long-chain polyunsaturated fatty acids, and protein and energy intake, especially in pregnant and nursing women, infants and young children. Providing an adequate lacto-ovo-vegetarian diet is relatively easy, whereas the maintenance of more restrictive diets may be challenging. The benefits and risks of vegetarian diets in adults are relatively well documented, but data for children are scarce. Vegan and macrobiotic diets should be discouraged in pregnant and lactating mothers as well as in young children, who, otherwise, should pay careful attention to ensuring nutritional adequacy, blood testing and appropriate supplementation. The health consequences of a chosen diet should be discussed with parents and adolescents to ensure the best possible adherence to advice and prescriptions. There is a need for well conducted studies in children but also for better knowledge of nutrition in healthcare professionals.Abbreviations: ALA: α-linolenic acid; ARA: arachidonic acid; ASF: animal source foods; BMC: bone mineral content; BMD: bone mineral density; DHA: docosahexaenoic acid; DRV: dietary reference value; EPA: eicosapentaenoic acid; FLD: flexitarian diet; LA: linoleic acid; LC-PUFA: long-chain polyunsaturated fatty acids; LOVD: lacto-ovo-vegetarian diet; LVD: lacto-vegetarian diet; MAD: macrobiotic diet; OMD: omnivorous diet; PSF: plant-source foods; SFA: saturated fatty acids; VGD: vegetarian diets; VND: vegan diet.