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622 result(s) for "13-18 years"
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Paracentral acute middle maculopathy: precursor to macular thinning in sickle cell retinopathy
We present a case of paracentral acute middle maculopathy (PAMM) in a patient with sickle cell disease (SCD). Though gradual capillary loss may contribute to pathophysiology of sickle cell retinopathy as well, our case suggests that PAMM may be the precursor lesion to macular thinning commonly observed in patients with SCD. In addition, fluorescein angiography may be unable to detect these acute vascular events occurring at the deep capillary plexus.
Low-dose ofatumumab for rituximab-resistant nephrotic syndrome
Drug resistant idiopathic nephrotic syndrome (DRNS) remains a therapeutic dilemma. In this pilot study, the efficacy of the new fully humanised, anti-CD20 monoclonal antibody ofatumumab was tested in 4 children with persistence of proteinuria for at least 12 months in spite of a full drug approach (including rituximab). We used a low-dose 2-infusion ofatumumab model (300+700 mg/1.73 m2 2 weeks apart) using specified premedication. Transient normalisation of proteinuria (persisting for 2 months) was achieved in 1 child while another presented stable remission after 12 months; both had normal renal function. The outcome was not modified in the remaining 2 children presenting an impaired renal function. These results demonstrate that low-dose ofatumumab may induce remittance of proteinuria in children with a long story of DRNS and normal renal function. Further studies are needed to test whether higher doses of ofatumumab can also modify proteinuria in patients with impaired renal function.
Front to back ocular injury from a vaping-related explosion
We describe a case of extensive ocular injury secondary to an electronic cigarette (e-cigarette)-related explosion. The explosion was the result of modifications made to a heating element of the e-cigarette device by a non-professional. Extensive ocular injuries that result from an explosion of an e-cigarette device can potentially cause significant and permanent visual impairment.
Idiopathic bilateral haemolacria
[...]of initial presentation, smear from the bloody tear film did not reveal any abnormal cells except for red blood cells, followed by lacrimal system syringing and probing revealed patent nasolacrimal system without any identifiable cause.
The entrapped twin: a case of fetus-in-fetu
Fetus-in-fetu (FIF) is a rare entity in which malformed parasitic twin grows inside the body of its twin. It is most commonly presented with mass in the abdomen. We present a case of a 15-year-old boy who presented with abdominal mass since infancy. Radiological investigations are suggestive of FIF. Intraoperatively, malformed fetus in a sac was found and excised. Postoperatively the patient recovers well and was put on follow-up.
Naphthalene ball poisoning: a rare cause of acquired methaemoglobinaemia
A 15-year-old boy presented to emergency services with accidental naphthalene ball ingestion. Following consumption he developed methaemoglobinaemia, massive intravascular haemolysis and acute kidney injury. He had no history suggestive of congenital haemoglobin M disease. Development of severe methaemoglobinaemia and intravascular haemolysis is quite unusual after consumption of a single ball of naphthalene. The patient was managed with ascorbic acid and intravenous N-acetyl cysteine. He also required haemodialysis for acute kidney injury that developed secondary to pigment nephropathy.
Migration of a Nexplanon contraceptive implant to the pulmonary artery
This is a case report of a 17-year-old woman who experienced distant intravascular migration of a radio-opaque Nexplanon contraceptive implant. She presented with an impalpable implant and this was located using CT in the left lower lobe pulmonary artery. Significant migration of a contraceptive implant is an uncommon risk. Using radio-opaque contraceptive implants such as Nexplanon increases the ability to locate them if migration occurs.
Swept source: optical coherence tomography angiography features of choroidal osteoma with choroidal neovascular membrane
A choroidal neovascular membrane (CNVM) with subretinal haemorrhage was seen at the lesion's inferior medial edge, involving the fovea ( figure 1 A). [...]diagnosis of choroidal osteoma 1 (CO) was made and confirmed on sonography, with a highly acoustic elevated lesion causing a corresponding shadow ( figure 1 B).
XIAP deficiency and MEFV variants resulting in an autoinflammatory lymphoproliferative syndrome
A 16-year-old boy of Caucasian ethnicity was evaluated for recurrent febrile episodes occurring during most of his life without establishment of any microbial aetiology. During febrile episodes he developed extensive splenomegaly, lymphadenopathy, anaemia, severe abdominal pain and general malaise. Lymph node biopsies demonstrated inflammation and sinus histiocytosis but no malignancy or granuloma. The patient underwent seroconversion for Epstein-Barr virus (EBV) infection during the hospitalisation. Genetic testing identified a hemizygous frameshift mutation in the X linked inhibitor of apoptosis (XIAP)-gene as well as variants in the MEFV gene indicating Familial Mediterranean Fever (FMF). XIAP expression was markedly reduced in the patient, while a functional assay assessing tumour necrosis factor (TNF)α production of monocytes in response to NOD2 stimulation displayed reduced activity. We suggest that the heterozygous MEFV variants and the hemizygous XIAP variant in combination triggered the prolonged and pathological inflammatory response to EBV infection.