Ceftriaxone associated urolithiasis in a child with hypercalciuria.

We present a 5-year-old boy with pneumonia who complained of right lumbar pain on the 7(th) day of treatment with Ceftriaxone. Ultrasound examination revealed mild to moderate right hydronephrosis. Under spasmoanalgetic therapy and hydration there was spontaneous passage of three small calculi. Infrared spectroscopy showed that the calculi were composed of calcium-ceftriaxonate. Full metabolic investigation was performed and moderate hypercalciuria was detected, suggesting the role of hypercalciuria in ceftriaxone-associated nephrolithiasis.

A giant osteochondroma in a boy with multiple exostoses.

Hereditary multiple exostoses (HME) is an inherited autosomal dominant disorder characterised by the presence of multiple exostoses, in fact benign cartilaginous tumors (enchondromata on the long bones). A six-year-old boy was found to have multiple osteochondromas on the legs, arms and ribs. Unusually, one of the osteochondromas on the right arm was huge (5 x 6 cm) and painful. X ray confirmed the benign nature of the osteochondromas. The family history was uneventful as well as the pregnancy and delivery. His intelligence is normal, and ultrasound did not detect any anomalies of the heart or kidneys. The occurrence of a large osteochondroma in a young boy is rare. In spite of its size and growth the lesion is so far benign. Frequent follow-up is recommended for the timely detection of eventual malignant transformation.

Four generations in a family with neurofibromatosis 1: precocious puberty and optic nerve tumor (OPT).

Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder with varied clinical manifestations. The proband is a 6-year-old boy with signs of precocious puberty. His penis was 10 cm, testicles 8 ml, pubic hair P2-3, and the genital skin was hyperpigmented. Multiple cafe au lait spots well above 5 mm were noticeable on his skin, as well as hard subcutaneous nodules, mostly on his trunk. His intelligence and hearing are normal. He has no history of seizures. Laboratory analysis showed: LH LH 1.4 mIU/ml, FSH 6.2 mIU/ml, testosterone 183 ng/ml. Bone age was 9 years. LHRH stimulation was characteristic of true precocious puberty (LH 9.8 mIU/ml and FSH 8.9 mIU/ml after 30 minutes). The MRI of the brain showed a tumor of the suprasellar region with compression of the pituitary stalk. At present the boy is 6 years old and has been treated with triptoreline acetate for 3 months. The volume of the testicles has decreased to 7 ml and a slight loss of pubic hair was noted. In addition, his mother and his grandfather exhibited dermal masses, and focal cutaneous and subcutaneous growths. The great-grand father had had the same cutaneous changes and died at the age of 75 from unrelated causes. It has already been well documented that NF is associated with an increased risk of malignancy and precocious puberty. Hence, we emphasize the need for a close and regular clinical follow-up of the OPT, puberty and patterns of growth.

Type I Gaucher disease (GDI) in three siblings: enzyme replacement treatment (ERT) required.

(Full text is available at http://www.manu.edu.mk/prilozi). This is a family of three children, born to healthy Macedonian parents after uneventful pregnancies and delivery. The index child was an eight-year-old girl admitted for abdominal discomfort and distension: the spleen was 14cm below the costal margin (BCM), the liver 8cm BCM. No bone pain or pathology was reported. There was mild pancytopaenia (hemoglobin 11.2 gm/L; WBC counts 4.6 x 10;3; platelets 70 x 10;3). Liver function tests, renal ultrasound, bone scan, and a chest radiograph were within normal limits. Bone marrow analysis in this child and her two brothers (11 and 6.5 years old) revealed Gaucher cells. Both brothers had only mild anaemia, but the older brother had been splenectomized prior to diagnosis of GD1. Enzyme analysis revealed low activity (2.59, 1.62, and 2.55 nmol/h/mg protein, respectively); plasma chitotriosidase levels were also elevated. Genetic testing revealed homozygosity for the N370S/N370S mutation in all three siblings. In the absence of available enzyme replacement treatment (ERT), the girl was splenectomized. Removing an important immune organ (the spleen) introduces further risk for the patients. In addition, this does not solve the bone involvement characteristic for GD. ERT should be introduced for all GD1 patients in Macedonia. Key words: Gaucher disease, N370S mutation, siblings, enzyme replacement therapy.

Tensile bond strength of a light-cured glass ionomer cement when used for bracket bonding under different conditions: an in vitro study.

The purpose of this study was to investigate the tensile bond strength of a new light-cured resin reinforced glass ionomer cement (Fuji Ortho LC), following the bonding of stainless steel brackets to 40 extracted human premolar teeth under four different enamel surface conditions: (1) non-etched, moistened with water; (2) etched, moistened with water; (3) etched, moistened with human saliva; and (4) etched, moistened with human plasma. The etched surface produced a higher bond strength than the non-etched surface when contaminated with distilled water. Contamination with human saliva resulted in a further increase in bond strength whilst plasma contamination produced an even higher strength. However, one-way analysis of variance showed no statistically significant difference between the various groups. After debonding, enamel and bracket base surfaces were examined for residual adhesive. The location of the adhesive also indicated improved bonding to etched enamel. This investigation shows that regardless of enamel surface pretreatment or environment, Fuji Ortho LC provides an adequate strength for bonding of orthodontic brackets.