Quantifying hospital-associated costs, and accompanying travel costs and productivity losses, before and after withdrawing TNF-α inhibitors in juvenile idiopathic arthritis.

OBJECTIVE: To quantify differences in hospital-associated costs, and accompanying travel costs and productivity losses, before and after withdrawing TNF-α inhibitors (TNFi) in JIA patients. METHODS: This was a retrospective analysis of prospectively collected data from electronic medical records of paediatric JIA patients treated with TNFi, which were immediately discontinued, spaced (increased treatment interval) or tapered (reduced subsequent doses). Costs of hospital-associated resource use (consultations, medication, radiology procedures, laboratory testing, procedures under general anaesthesia, hospitalization) and associated travel costs and productivity losses were quantified during clinically inactive disease until TNFi withdrawal (pre-withdrawal period) and compared with costs during the first and second year after withdrawal initiation (first and second year post-withdrawal). RESULTS: Fifty-six patients were included of whom 26 immediately discontinued TNFi, 30 spaced and zero tapered. Mean annual costs were €9165/patient on active treatment (pre-withdrawal) and decreased significantly to €5063/patient (-44.8%) and €6569/patient (-28.3%) in the first and second year post-withdrawal, respectively (P < 0.05). Of these total annual costs, travel costs plus productivity losses were €834/patient, €1180/patient, and €1320/patient in the three periods respectively. Medication comprised 80.7%, 61.5% and 72.4% of total annual costs in the pre-withdrawal, first and second year post-withdrawal period, respectively. CONCLUSION: In the first two years after initiating withdrawal, the total annual costs were decreased compared with the pre-withdrawal period. However, cost reductions were lower in the second year compared with the first year post-withdrawal, primarily due to restarting or intensifying biologics. To support biologic withdrawal decisions, future research should assess the full long-term societal cost impacts, and include all biologics.

[Borrelial lymphocytoma]. / Borrelia-lymfocytoom.

BACKGROUND: Borrelial lymphocytoma is a relatively rare but typical presentation of Lyme disease. Predilection sites are the ears in children and chest/nipples in adults. It is treated like an erythema migrans and has a good prognosis. CASE DESCRIPTION: A 16-year-old boy presented with a swollen, red and painful right nipple since several months. An ultrasound showed normal breast tissue. The patient was referred to the pediatric surgeon who performed an incision biopsy. Histopathological examination revealed follicular hyperplasia without signs of malignancy. An infectious cause, most likely Lyme disease, was suspected. Serological analysis and PCR of the tissue confirmed the diagnosis of a borrelial lymphocytoma, and the patient was treated with doxycycline with good result. CONCLUSION: Early recognition of the characteristic clinical presentation of borrelial lymphocytoma, supported by positive results from serologic testing for Lyme disease, avoids the need for additional and invasive diagnostic tests.

A girl with autoimmune cytopenias, nonmalignant lymphadenopathy, and recurrent infections.

We describe a girl, now 9 years of age, with chronic idiopathic thrombocytopenic purpura, persistent nonmalignant lymphadenopathy, splenomegaly, recurrent infections, and autoimmune hemolytic anemia. Her symptoms partly fit the definitions of both autoimmune lymphoproliferative syndrome (ALPS) and common variable immunodeficiency disorders (CVIDs). Genetic analysis showed no abnormalities in the ALPS-genes FAS, FASLG, and CASP10. The CVID-associated TACI gene showed a homozygous polymorphism (Pro251Leu), which is found also in healthy controls.

Transient hypertransaminasemia in paediatric patients with Crohn disease undergoing initial treatment with enteral nutrition.

OBJECTIVES: Total enteral nutrition (TEN) is frequently used as monotherapy in children with Crohn disease to prevent steroid toxicity. Liver disease is a known complication in inflammatory bowel disease, and liver enzymes are regularly obtained in these patients. PATIENTS AND METHODS: Prospective follow-up of liver enzymes was performed in 11 new consecutive patients ages 7.6 to 17.1 years who were primarily treated with TEN for 6 weeks. Liver enzymes were measured before starting TEN and after 3, 6, and 12 weeks. RESULTS: At the beginning of TEN, the mean aspartate aminotransferase (ASAT) was 18.4 U/L and the mean alanine aminotransferase (ALAT) 17.1 U/L. The mean ASAT and ALAT were 202.0 U/L and 269.0 U/L after 3 weeks and 109.6 U/L and 180.9 U/L at 6 weeks. After 12 weeks values decreased to 22.8 U/L (ASAT) and 20.9 U/L (ALAT). Overall, 9 of 11 patients had transient elevated ASAT and 10 patients showed elevated ALAT. Gamma-glutamyl transpeptidase was slightly elevated in 3 patients during therapy, but alkaline phosphatase and bilirubin showed no changes. None of the patients developed liver disease during follow-up, and prolonged clinical remission was achieved in 9 patients. CONCLUSIONS: This study shows that TEN can be associated with transient hypertransaminasemia without evidence of liver disease. We hypothesise that insulin resistance in patients with Crohn disease in combination with standard TEN formulae can result in transient hepatic steatosis causing the hypertransaminasemia. For the clinician it is important to be aware of this benign TEN-associated condition to prevent unnecessary investigations.