Annals of Tropical Paediatrics最新文献

A retrospective review of ten patients (8 girls, 2 boys) admitted over a 9-month period with haemophagocytic lymphohistiocytosis (HLH) is presented. Presenting features included fever and hepatosplenomegaly (10), bleeding manifestations (7), lymphadenopathy (4), skin rash (4), shock (4), jaundice (3), CNS disorder (3), renal failure (2) and arthritis (2). Three infants had familial HLH (FHL) while the other seven patients had acquired (secondary) HLH. Two patients with FHL had very low perforin levels (0 and 0.05%). There was secondary HLH owing to systemic onset juvenile idiopathic arthritis in two patients, and one each had anaplastic large cell lymphoma, measles with pneumonia, disseminated tuberculosis, dengue hemorrhagic fever and lymphoproliferative disorder. Cytopenia affecting two or three lineages in peripheral blood was present in all while haemophagocytosis in bone marrow was documented in nine patients .Other important laboratory parameters were raised ferritin (9), raised LDH (9), hypertriglyceridaemia (7) and hypofibrinogenaemia (5). The patients were treated according to the HLH2004 protocol. Diagnosis of HLH should be considered early in patients presenting with unremitting fever, hepatosplenomegaly and cytopenias as without appropriate treatment HLH is usually fatal.

Background: Major obstacles remain in scaling up paediatric HIV treatment, including limited paediatric anti-retroviral drug options for resource-limited settings, challenges with adherence to liquid formulations and treatment fatigue with lifelong therapy.

Aim: To determine levels of adherence to HAART in HIV-infected children at 12, 24, 36 and 48 weeks of follow-up and to compare adherence levels before and after switching from syrup to fixed-dose combination (FDC)-tablet anti-retroviral formulations.

Methods: HIV-infected children aged between 6 months and 12 years were initiated on anti-retroviral therapy at Makerere University-Johns Hopkins University Care Clinic, Kampala. Good adherence to HAART was defined as taking ≥95% of prescribed medications. Adherence levels were measured using pharmacy refill data, quarterly unannounced home-visit pill counts and caregiver self-reports. Data were analysed using STATA(®) version 10.0.

Results: A total of 129 HIV-infected children were initiated on HAART with 14.7% on syrups and 85.3% on tablet formulations at enrollment. According to caregiver self-reporting, 99.2%, 100%, 100% and 99.2% achieved ≥95% adherence at 12, 24, 36 and 48 weeks, respectively. Using pharmacy refill data, the proportions were 89.9%, 95.4%, 93.8% and 93.0% and for unannounced home visits were 89.8%, 92.4%, 88.9% and 86.2%, respectively. Median adherence to syrup formulations (97%, IQR 93-98) was significantly lower than for tablets (100%, IQR 97-100, p = 0.012, n = 28) using pharmacy refill data. Viral suppression correlated with home visit and pharmacy refill adherence data.

Conclusion: The majority of children initiating HAART had good adherence when estimated by caregiver self-report and pharmacy refill data but lower adherence when measured by home-visit pill counts. Adherence to tablet formulation of HAART was significantly better than syrup formulation. Medication formulation did not significantly affect viral suppression.

Neurological manifestations of enteric fever are well known. However, isolated cranial nerve palsies are rare. A 4-year-old boy and two girls aged 5 and 11 years with culture-proven typhoid fever are described, two of whom developed palatal palsy and the other sixth cranial nerve palsy. Following treatment, there was complete resolution of the cranial nerve palsies within 5-7 weeks. While palatal palsy is reported rarely, isolated sixth cranial nerve palsy associated with typhoid has not been reported previously.

Nimesulide is a frequently used non-steroidal anti-inflammatory drug with analgesic and antipyretic effects in children. In view of fatal adverse drug reactions, however, its safety has been questioned. A 5-year-old boy developed Stevens-Johnson syndrome following use of nimesulide which was later complicated by rapidly progressive fatal bronchiolitis obliterans.

Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating disorder of the central nervous system which is usually precipitated by a viral infection or vaccination. A 3-month-old boy is reported who developed ADEM a week after full recovery from pertussis. MRI detected a high-intensity lesion extending from the pons to the mesencephalon, compatible with ADEM. Following the administration of intravenous immunoglobulins, the patient's clinical symptoms improved. This case report demonstrates that pertussis is capable of inducing an immune-mediated demyelinating disorder of the central nervous system.

A woman presented with polyhydramnios at 22 weeks of gestation with a structurally normal fetus and placenta. Biochemical analysis of amniotic fluid detected a very high level of chloride (582 mmol/L), which led to the diagnosis of Bartter syndrome. With serial amniocentesis and indomethacin therapy, the pregnancy continued to 36 weeks. Neonatal and subsequent investigations further supported the diagnosis of Bartter syndrome. The infant was well at birth and now, at 5 months of age, is gaining weight normally on indomethacin.

Orbital myositis in children is uncommon. Recurrence is a major challenge in management. An 11-year-old Malay girl who presented with bilateral orbital myositis with recurrent attacks of diplopia is reported.

Traumatic diaphragmatic rupture (TDR) is rare in children and can be easily overlooked because of lack of awareness of late presentation and concomitant injuries. A 4-year-old girl presented with respiratory distress 2 months after a road traffic accident. The initial differential diagnosis was pneumonia or pulmonary tuberculosis with associated pleural effusion. On further assessment, a diaphragmatic hernia was suspected. The initial radiograph showed left hydropneumothorax. Fluoroscopy, follow-up chest radiographs and barium swallow confirmed the diagnosis of left TDR. Surgery was undertaken but unfortunately she did not survive. Awareness of delayed presentation of TDR is essential for prompt management.