Hepatitis C immune globulin (Biotest Pharmaceuticals)

A patient with DiGeorge syndrome showed a decrease of cytomegalovirus load after cord blood stem cell transplantation. A 3-month-old infant was admitted to hospital because of prolonged seizures. He was born at 37 weeks and 2 days gestation with a birth weight of 1864 g. He showed a significant decrease in serum Ca, Na and Cl levels, with inappropriate ADH secretion, and was treated with water restriction and Ca supplementation. Several minor anomalies, such as low-set ears, small-sized jaw, muscle hypotonia, retarded development and growth of body weight 4260 g (-3.0 SD) and body length 54 cm (–3 SD), and absence of thymus, genital hypoplasia, together with hypoparathyroidism (intact PTH <9 pg/ml), and lack of T-cells (5 cells/ll) led to a diagnosis of DiGeorge syndrome on day 100. The boy suffered from persistent fever, diarrhoea, and cough, with interstitial pneumonia, gastroenteritis, hepatitis, and retinopathy. Both cytomegalovirus (CMV) antigen and CMV-DNA were detected in blood and urine samples, suggestive of systemic CMV infection. He was treated with ganciclovir, c-globulin with high titres of CMV-IgG, and foscarnet sodium hydrate. The CMV load decreased from 60,000 genome copies/ 10 ll whole blood to 12,000, but no lower, and the symptoms such as fever, diarrhoea, and cough persisted (Table 1). Cord blood stem cell transplantation (CBSCT) was performed by infusion of 10.3·10 cord blood stem cells without preconditioning and with the use of cyclosporine (5 mg/kg per day) on day 220 (Table 1). Within 20 days after the CBSCT, his general condition, i.e. fever, cough, and general activities improved and a significant increase in T-cells (1690/ll) with dramatic reduction of CMV to 1000 copies was observed without any serious complications. However, respiratory distress and diarrhoea worsened gradually with a relapse of CMV infection up to 11,000 copies after the use of corticosteroids resulting in death on day 253. CMV infection is a fatal complication in T-cell deficient patients. CBSCT is an ideal treatment for introducing T-cell immunity, since premature T-cells do not induce serious graft-versus-host disease (GvHD) in the immune deficient host [1]. The reduction in CMV-DNA copies from 12,000/10 ll whole blood to 1,000 with increased T-cell counts suggests that CBSCT is effective for the treatment of CMV infection in DiGeorge syndrome. Although CBSCT successfully reduced the number of CMV-DNA copies, the respiratory distress and intestinal bleeding progressed. We cannot exclude the possibility that the introduction of lymphocytes accelerated the development of inflammation in the host organ. We do not suspect that these symptoms were caused by GvHD but possibly by systemic CMV disease, since the high CMV load was observed after CBSCT without any typical GvHD symptoms, i.e. skin lesions and liver dysfunction. Corticosteroid Y. Ohtsuka (&) AE T. Shimizu AE K. Nishizawa AE R. Ohtaki T. Someya AE A. Noguchi AE H. Fujita AE Y. Yamashiro Department of Paediatrics, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, 113-8421 Tokyo, Japan E-mail: yohtsuka@med.juntendo.ac.jp Tel.: +81-3-58021075 Fax: +81-3-58000216

A previously healthy, 8-month-old boy suffered from Kawasaki disease with rhabdomyolysis. Although Kawasaki disease (KD) is a systemic vasculitis affecting multiple organ systems, muscle involvement has rarely been reported [1, 2, 3, 4, 5,6]. This report describes rhabdomyolysis in a patient with KD. A previously healthy, 8-month-old boy was referred to another hospital because of a 5-day history of highgrade fever, cervical lymph node swelling, and exanthema. The patient was transferred to our hospital because of elevated creatine kinase (CK) levels. The patient did not take any anti-inflammatory drugs including ibuprofen before admission. Upon admission, physical examination revealed bilateral conjunctivitis, a strawberry-like tongue, enlarged cervical lymph nodes, polymorphic truncal rash and oedema of the peripheral extremities. His vital signs were as follows: body temperature 40.3 C; blood pressure 102/ 48 mmHg; and pulse rate 140 beats/min. The body weight was 9.6 kg (-0.4 kg before present illness). The patient did not suffer from apparent muscle pain, tenderness or weakness. Neurological examinations showed no remarkable findings. Laboratory studies revealed anaemia (red blood cell count 364·10/ll, haemoglobin 9.3 g/dl, and haematocrit 28.9%), elevated C-reactive protein (CRP) (5.34 mg/dl, reference range 0.01–0.31 mg/dl), increased levels of aspartate aminotransferase (AST, 528 IU/l, reference range 11–31 IU/l), alanine aminotransferase (ALT, 225 IU/l, reference range 7–42 IU/l) and lactate dehydrogenase (LDH, 1,716 IU/l, reference range 232–427 IU/l), and an elevated CK level (10,356 IU/l, reference range 48–280 IU/l) with 98.6% MM isozyme. Serum concentrations of aldolase (25.0 IU/l, reference range 2.5–5.8 IU/l) and myoglobin (380 ng/ml, normal <60 ng/ml) were also elevated. Serum sodium (138 mEq/l), potassium (4.7 mEq/l), chloride (102 mEq/l) and creatinine (0.2 mg/dl) and blood urea nitrogen (4.9 mg/dl) were normal. Urinalysis showed 1+ protein and 1+ occult blood without red blood cells. Urinary concentration of myoglobin (111 ng/mL, normal <10 ng/ml) was elevated. Urinary electrolytes were as follows: sodium 10 mEq/l, potassium 9.1 mEq/l and chloride 17 mEq/l. Urinary creatinine concentration was 15.3 mg/dl. Antinuclear antibody, hepatitis B virus surface antigen, and anti-hepatitis C virus antibody were negative. Bacterial and viral culture of throat swabs grew no pathogens. Echocardiograms and electrocardiograms revealed no abnormalities. The patient was diagnosed as having KD with rhabdomyolysis. Intravenous infusion of 1 g/kg c-globulin for 2 days and oral administration of flurbiprofen (2 mg/kg per day) promptly improved his condition. His body temperature normalised 4 days after the start of treatment and his serum CK and CRP levels normalised 7 and 10 days after treatment, respectively. He was discharged with elevated AST, ALT and LDH levels 13 days after he was admitted to the hospital. The levels of these enzymes returned to normal 4 weeks later. A follow-up examination 4 months later showed a complete recovery including normal CK levels. Our patient with KD showed rhabdomyolysis. Muscle involvement in KD has been described in only seven patients including four with myositis [1, 4, 6], one with acute neuropathic muscular injury mediated by immune complexes [2], one with macro CK [3] and one with rhabdomyolysis due to hyperthermia [5]. The precise pathogenic mechanisms for the development of rhabdomyolysis in our patient are unclear; however, because our patient exhibited high-grade fever, no neuromuscular signs or symptoms and a prompt decrease in serum CK levels after the fever abated, transient muscle damage due to hyperthermia is the most likely cause of rhabdomyolysis in our patient. Although muscle Eur J Pediatr (2003) 162: 891–892 DOI 10.1007/s00431-003-1329-1