[Acute lymphoblastic leukemia in a pediatric patient with Marfan's syndrome].

We report a rare case of acute lymphoblastic leukemia (ALL) in a 7-year-old boy with Marfan's syndrome. He was diagnosed as having Marfan's syndrome by clinical findings at the age of 2 years, and the diagnosis was confirmed by the detection of gene mutation in FBN1. He was referred to our hospital because of the swelling of cervical lymph nodes at the age of 7 years. Findings on bone marrow examination demonstrated T lymphoblastic ALL. He obtained complete remission after induction therapy, and had no serious side effects including cardiotoxicity during chemotherapy. He has remained in continuous complete remission for 34 months following diagnosis. To our knowledge, only three cases of leukemia in patients with Marfan's syndrome were reported previously. We speculate that increased activity of TGF-ß, which is known as a tumor suppressor factor, in patients with Marfan's syndrome may diminish the risk of developing leukemia, although such a thesis was not proven in this case.

Chronological diffusion-weighted imaging changes and mutism in the course of rotavirus-associated acute cerebellitis/cerebellopathy concurrent with encephalitis/encephalopathy.

Rotavirus is one of the most common causes of gastroenteritis in children and is known to accompany some neurological disorders such as encephalitis/encephalopathy and seizures. Although cerebellar disorders sometime occur as a complication of rotavirus gastroenteritis in Japan, few reports have addressed these issues. Here, we report three cases of insulted cerebellums in addition to encephalitis/encephalopathy associated with rotavirus. Similar to posterior fossa syndrome after surgery, mutism was a notable symptom that lasted about 1 month. Brain diffusion-weighted imaging (DWI) revealed chronological changes, i.e., marked hyperintensity in the bilateral dentate nucleus followed by the vermis and cerebellar hemisphere. The bilateral dentate nucleus is known to be a key lesion site for mutism, and these clinical and radiological findings may be tightly connected in rotavirus-associated cerebellitis/cerebellopathy.

Beneficial effect of chlorambucil in steroid-dependent and cyclophosphamide-resistant minimal change nephrotic syndrome.

BACKGROUND: Little information is available on the effect of second cytotoxic therapy in steroid-dependent children with minimal change nephrotic syndrome (MCNS). METHODS: Response to second cytotoxic therapy and side effects were reviewed in 33 steroid-dependent and cyclophosphamide-resistant children with MCNS who received chlorambucil (n=11, group 1) or cyclophosphamide (n=22, group 2). RESULTS: Age at onset of nephrosis, beginning of first and second therapy, sex ratio, duration of nephrosis before first cytotoxic therapy, interval between first and second cytotoxic therapy, number of relapses, cumulative doses of steroids and length of remission off steroids before second therapy were similar between groups. Four patients (36.4%, p<0.05) in group 1 remained in remission for a median 34.0 months, whereas only 1 patient (4.5%) in group 2 did for 53.0 months. Two patients in group 1 and 1 patient in group 2 became infrequent relapsers. Total number of nonrelapsers and infrequent relapsers was higher in group 1 (54.5%, p<0.05) than in group 2 (9.1%). Number of relapses and cumulative doses of steroids were reduced and length of remission off steroids was longer in group 1 than in group 2 (p<0.05). There was no difference between groups in frequency of side effects, and none had serious toxicity. However, the short period of follow-up in our study does not exclude the risk of azoospermia. CONCLUSIONS: Our data suggest a superior effect of chlorambucil over cyclophosphamide in steroid-dependent and cyclophosphamide-resistant children with MCNS. A future randomized controlled clinical trial is required to confirm our findings.

[Mucoepidermoid carcinoma of the parotid gland as a secondary malignancy developed ten years after chemotherapy for childhood acute lymphoblastic leukemia].

We report on a case of mucoepidermoid carcinoma of the parotid gland following treatment of acute lymphoblastic leukemia (ALL) in childhood. The female patient was diagnosed as having T-cell ALL at the age of 13 years. Treatment included multidrug chemotherapy and prophylactic 18 Gy cranial irradiation. She developed low-grade mucoepidermoid carcinoma of the right parotid gland at the age of 24 years, the most probable cause of the secondary malignancy being radiation. In a literature review of mucoepidermoid carcinoma following ALL, 11 out of 14 cases received radiation therapy for the initial treatment of ALL, but 3 cases had no radiation therapy. The parotid gland carcinoma as a secondary malignancy following ALL in childhood is rare, but it highlights the need for concern about the secondary malignancy in patients with painless parotid swelling after chemoradiotherapy.

A new method of blood sampling reduces pain for newborn infants: a prospective, randomized controlled clinical trial.

BACKGROUND: Using a new simple blood glucose measurement device (Free Style), blood can thus be sampled from the forearm, which is less sensitive to pain than from the heel. Measuring the blood glucose levels in newborn infants using Free Style is therefore a potentially less painful testing modality than traditional blood sampling methods. OBJECTIVE: To compare the pain intensity at the time of blood sampling from the forearm using the Free Style with the conventional method from the heel. DESIGN: A prospective, randomized controlled clinical trial. PATIENTS AND METHODS: Sixty healthy neonates were randomized by the sealed envelope method into two groups-Group F, in which blood was sampled from the forearm using the Free Style, and Group H, in which blood was conventionally sampled from the heel using a lancet. The pain intensity was assessed based on their crying, the Neonatal Facial Coding System (NFCS) and the Neonatal Infant Pain Scale (NIPS). RESULTS: After skin puncture, 12 (40%) of the infants cried in Group F and 27 (90%) in Group H. The duration of crying was also significantly shorter in Group F than in Group H. Both of pain scores between the two groups differed significantly, these findings indicated less pain for Group F. CONCLUSION: The new blood sampling method from the forearm was found to be less painful than the conventional method, thus making it possible to clinically use this product as an innovative method for blood sampling in neonatal medicine.

Serial diffusion-weighted imaging of neonatal herpes encephalitis: a case report.

We reported a patient with neonatal herpes simplex encephalitis in whom diffusion-weighted imaging was performed repeatedly. Diffusion-weighted imaging at 20h after the onset of seizures revealed scattered small spotty high intensity lesions in both hemispheres and a high intensity area in the left fronto-temporal lobe. There was no abnormal finding on conventional magnetic resonance imaging. Second diffusion-weighted imaging 72h after the onset revealed expanded scattered high intensity lesions in the bilateral hemisphere, a high intensity area in the left fronto-temporal lobe, and a new high intensity area in the right temporal lobe. There was no report on neonatal herpes simplex encephalitis that showed scattered high intensities in diffusion-weighted imaging. Scattered small high intensities on diffusion-weighted imaging may suggest endothelial cell infection with swelling and small vessel necrosis. Early diffusion-weighted imaging will be valuable for early detection and diagnosis of neonatal herpes simplex encephalitis.