Homoeopathic management of chronic replapsing pancreatitis in a paediatric patient: A case report

Chronic relapsing pancreatitis in the paediatric age group is a challenging case, especially when presenting in its acute exacerbation. This case report highlights the management of chronic relapsing pancreatitis in a 9-year-old female patient with homoeopathic treatment. The patient reported a year-long history of recurrent fever, abdominal pain, and raising titres of lipase and amylase; she had been admitted to a higher centre twice. The totality was constructed on day 1 and a homoeopathic remedy was prescribed. Detailed case taking, done after a week, confirmed the same remedy. Later, when the patient had an acute exacerbation, the same remedy-frequently repeated, helped settle the acute episode in a couple of days. The patient has been following up regularly for 3 years; the frequency and intensity of relapses reduced considerably over time and there have been no episodes for more than a year.

Haemophagocytic Lymphohistiocytosis in a Malay infant: Rare, Old and Often Forgotten Disease

Haemophagocytic lymphohistiocytosis (HLH) is a rare disease but potentially life threatening clinical syndrome. It is caused by a multisystemic hyperinflammatory process secondary to severe hypercytokinemia with excessive and uncontrolled activation of the immune response. We report a case of familial HLH with no apparent causes in 6 months-old Malay girl presented with recurrent fever associated with severe anaemia and bleeding tendency requiring extensive treatment but refractory to the treatment which lead to mortality due to neutropenic sepsis indicating of poor prognosis of this disease. This familial type of HLH should be suspected in all children after excluding all the secondary causes with collective laboratory features and requiring extensive management as it associated with high mortality. Bangladesh Journal of Medical Science Vol. 21(1) 2022 Page : 196-200

Abordaje biorregulador de Síndrome autoinflamatorio en un paciente pediátrico: Reporte de caso

Se presenta escolar con fiebre recurrente asociada a manifestaciones cutáneas y dolores articulares sin retraso pondoestatural, con respuesta inflamatoria durante los episodios, con diagnóstico de síndrome autoinflamatorio multifactorial tipo SURF (Syndrome of Undifferentiated Recurrent Fever), o uSAIDS (Enfermedad Autoinflamatoria Sistémica Indefinida) sin demostración de mutaciones clásicas, con una carga epigenética por antecedentes familiares sin respuesta a manejo usual con antipiréticos , antiinflamatorios y corticoides, quien respondió de forma beneficiosa a terapia biorreguladora vs curso natural de la enfermedad autoinflamatoria no clásica. La Medicina Biorreguladora de Sistemas (MBS) por tratarse de un paciente pediátrico se administró por vía nebulizada y nasal por 8 semanas, con lo que se demostró mejoría en la disminución de la fiebre en 80% y las lesiones cutáneas en un 65%, con la consecuente mejoría en la calidad de vida del paciente y su familia.

Safety & efficacy of antibiotic de-escalation and discontinuation in high-risk haematological patients with febrile neutropenia: a single-centre experience

Background There is currently no consensus on optimal duration of antibiotic treatment in febrile neutropenia. We report on the clinical impact of implementation of antibiotic de-escalation and discontinuation strategies based on the 4th European Conference on Infections in Leukaemia (ECIL-4) recommendations in high-risk haematological patients. Methods We studied 446 admissions after introduction of an ECIL-4 based protocol (= ECIL-4 group) in comparison to a historic cohort of 512 admissions. Primary clinical endpoints were the incidence of infectious complications including septic shock, infection-related intensive care unit (ICU) admission and overall mortality. Secondary endpoints included the incidence of recurrent fever, bacteraemia and antibiotic consumption. Results Bacteraemia occurred more frequently in the ECIL-4 group [46.9% (209/446) vs 30.5% (156/512); p<0.001], without an associated increase in septic shock [4.7% (21/446) vs 4.5% (23/512); p=0.878] or infection-related ICU admission [4.9% (22/446) vs 4.1% (21/512); p=0.424]. Overall mortality was significantly lower in the ECIL-4 group [0.7% (3/446) vs 2.7% (14/512); p=0.016], resulting mainly from a decrease in infection-related mortality [0.4% (2/446) vs 1.8% (9/512); p=0.058]. Antibiotic consumption was significantly reduced by a median of 2 days on antibiotic therapy (12 versus 14; p=0.001) and 7 daily antibiotic doses (17 versus 24; p<0.001) per admission period. Conclusions Our results support implementation of ECIL-4 recommendations to be both safe and effective based on real world data in a large high-risk patient population. We found no increase in infectious complications and total antibiotic exposure was significantly reduced.

Complete resection of giant pericardial synovial sarcoma in a 7-year-old boy: a case report

Background: Synovial sarcoma is a rare soft-tissue malignant tumor most commonly occurring in the extremities and head and neck region, and rarely occurring in the pericardium. Case presentation: We report a 7-year-old boy was admitted to the hospital with recurrent fever and chest pain over the past four months. A cardiac magnetic resonance imaging (MRI) revealed a tumor beneath the heart in the pericardial, and we surgical resection it completely. The postoperative histopathological examination resulted in a diagnosis of monophasic spindle cell type synovial sarcoma. After two weeks of hospitalization, the patient was discharged. Three months after discharge, the positron emission tomography (PET/CT) scans did not show any signs of recurrence. Conclusion: Pericardial synovial sarcoma is a rare disease that is detected early, and complete resection improves patient survival. We recommend CT be performed in patients with recurrent fever and sizeable pericardial effusion to rule out possible pericardial synovial sarcoma considering the echocardiography limitations.

A20 Haploinsufficiency in East Asia

A20, encoded by the TNFAIP3 gene, is a negative regulator of tumor necrosis factor (TNF)-nuclear factor-κB signaling. It was recently demonstrated that A20 haploinsufficiency (HA20), caused by a heterozygous mutation in the TNFAIP3 gene, can present as an early onset autoinflammatory disease resembling Behçet’s disease (BD). In addition to autoinflammatory symptoms, HA20 was also reported to be associated with autoimmune diseases and immunodeficiency. Because the phenotypes associated with HA20 are broad, with different severities observed even among individuals in the same family with identical mutations, it has been assumed that the symptoms of HA20 may depend on genetic background and environmental factors. In this review, we summarize the characteristics of patients with HA20 in East Asia and compare these with patients in other regions, mainly the USA and Europe. Patients with HA20 in East Asia developed recurrent fever more frequently than patients in other regions, but were less likely to develop typical BD symptoms such as skin rashes and genital ulcers. In addition, patients with HA20 in East Asia had low rates of complication with autoimmune diseases and low autoantibody detection rates. While anti-TNF-α agents were the primary treatments for severe HA20 in East Asia, anti-interleukin-1 agents and Janus kinase inhibitors were also administered in other regions. Future studies will need to establish methods for analyzing the pathophysiology of HA20 and determining optimal treatment strategies for each patient.

X-linked agammaglobulinemia rare disease with a rarer presentation

X-linked agammaglobulinemia (XLA) is a rare disorder, characterized by absence of mature B cells leading to severe antibodies deficiency. This translates to recurrent sinopulmonary infections in affected children. The most common age group of presentation is 6 months to 2 years. Being an X-linked recessive disorder males are affected, females are carriers. Intravenous immunoglobulins and antibiotics remains the corner stone of treatment. Here in, we report a case of 11-year-old male having recurrent episodes of fever with one episode of hospitalization 3 years back. Child was treated at healthcare facility elsewhere for recurrent fever. He presented to our institute with signs and symptoms suggestive of meningitis, investigated, had culture proven Staphylococcus aureus meningitis with a low Absolute Lymphocyte Count (ALC). On further work up found to have low serum immunoglobulins (IgG, IgM, IgA) and Flowcytometry showing absence of B cells (CD19/CD20). Child was diagnosed to have XLA. This case highlights the importance of having strong clinical suspicion of XLA, despite not having recurrent sinopulmonary infections.

P084 Recurrent fever in children: why not a mevalonate kinase deficiency?

Background Mevalonate kinase deficiency (MKD) is a rare autosomal recessive auto inflammatory disease. The clinical spectrum of this disease is a continuum ranging from the moderate form of Hyper-IgD syndrome (HIDS) to lethal forms of mevalonic aciduria (MA). An autoinflammatory disease should be considered in children with recurrent fever of unexplained origin. Case report M, 28 months old boy from a non-consanguineous marriage of Algerian parents, presented since the age of two months’ episodes of unexplained fever resistant to antibiotics and requiring several hospitalizations. Family history found that the mother and a maternal uncle experienced an unexplained recurrent fever. Personal history found episodes of fever lasting in average 6 days, with no obvious cause and recurring every 15–20 days; associated with bilateral cervical adenopathy, mouth ulcers, arthralgia, abdominal pain with vomiting and diarrhea. Clinical examination confirmed the fever with temperature of 38.7°, Chills and irritability, Multiple cervical adenopathy oral aphthae, tongue of geographical aspect, cervical pain and large joint arthralgia, tender abdomen with no organomegaly. Biologic workups found major inflammatory syndrome with hyper leucocytosis at 14X103/mm3 predominantly polynuclear, high CRP level, high ESR at 110 mm. Blood cultures and viral serologies were negative. Immunoelectrophoresis found elevated IgA, IgG and IgM with a normal IgD level. Chest X-ray and abdominal ultrasound were normal. The diagnosis of MKD in its moderate form was supported by elevated urinary mevalonic acid excretion to 5.1 mmol/mol during the febrile episode and subsequently confirmed by a 2nd contributory urine assay during another febrile episode. Conclusion MKD is a rare disease. The clinical spectrum of this condition is variable and could benefit of an effective treatment. Our patient developed a moderate form with a better long-term prognosis.