Vaso-occlusive crises are the most common manifestation of sickle cell disease (SCD) and the main cause of hospital admission in these patients. There is emerging evidence that vaso-occlusive pain has both nociceptive and neuropathic components. However, the treatment of SCD-related pain with neuropathic drugs has not yet been systematically studied, particularly in children. We describe a 14-year-old girl with SCD and multiple hospital admissions for pain management for severe acute vaso-occlusive pain episodes. The patient was evaluated by a multidisciplinary team of specialists which considered that the chronic, refractory pain she was experiencing for years was probably neuropathic in origin and it was decided to start oral gabapentin (300 mg/day). At 10 months follow-up, the patient reported remarkable improvement in her quality of life with a significant decrease in the number of hospital admissions (three admissions for acute vaso-occlusive pain episodes in 10 months versus the previously monthly recurrences).
Pituitary gland duplication is a particularly rare finding. Different theories have been proposed to explain its pathogenesis, however, this phenomenon is not yet totally understood. Recently, duplication of the pituitary gland (DPG)-plus syndrome has been described, associating DPG with other blastogenic defects. We present the clinical and imaging findings of a newborn girl with DPG, associated with multiple other midline anomalies, including a nasopharyngeal teratoma, palate cleft deformity, bifid nasal bridge, tongue and uvula, hypoplasia of the basis pontis and corpus callosum, duplication of the basilar artery and hypothalamic hamartoma. We describe our patient's multidisciplinary team approach and emphasise the importance of reporting upcoming cases, in order to give more insight into the understanding of this complex entity.
Immune reconstitution inflammatory syndrome (IRIS) represents paradoxical immune-mediated inflammation in response to an infecting pathogen, occurring after initiation of antiretroviral therapy (ART), concomitantly with immune system recovery. It has also been described in Kaposi's sarcoma (KS). We report a case of a 9-year-old Guinean girl, who developed Kaposi's sarcoma, following introduction of ART. KS associated with immune reconstitution inflammatory syndrome is rare, especially in children, but with the increased use of ART is becoming more prevalent.
Pediatric Dermatology
BRIEF REPORTA skin biopsy with immunohistochemistry for HHV8 confirmed the diagnosis of KS (Figure 2). In this context, a diagnosis of KS secondary to IRIS was made. The patient continued with ART, and after 1 year, she has a normal CD4 cell count, undetectable HIV load and the skin lesions resolved leaving only residual hyperpigmented patches.
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