We report the case of a 2-year-old boy who developed a small bowel intussusception during treatment failure of his first episode of nephrotic syndrome. Despite the absence of typical symptoms other than abdominal pain, the intussusception was diagnosed by ultrasonography and computed tomography and successfully reduced by air enema. No pathological lead point was discovered, and no symptoms of Henoch-Schönlein purpura developed later. Intussusception should be considered in the differential diagnosis of abdominal pain in patients with nephrotic syndrome, especially in patients exhibiting prolonged edema. Ultrasonography or computed tomography should be performed, even in the absence of other typical symptoms suggestive of intussusception. We should also bear in mind that the intussusception associated with nephrotic syndrome might occur at regions other than the typical ileocolic region, such as within the small intestine.

We report on an 11-year-old boy who developed rhabdomyolysis and acute renal failure following Salmonella enteritidis gastroenteritis. Rhabdomyolysis should be considered as a potentially fatal complication in patients with Salmonella gastroenteritis.

Bilateral lesions in the anterior temporal lobe result in a neurobehavioral condition termed Klüver-Bucy syndrome (KBS). 1,2 This syndrome is characterized by psychic blindness (visual agnosia with inability to recognize objects), excessive oral tendencies, hypermetamorphosis (impulsive reaction to visual stimuli), placidity, changes in feeding behavior, and hypersexuality. Young animals and children with bi-temporal lesions, however, have different symptoms from those listed above. Psychic blindness in human children often manifests as an inability to recognize significant persons (discriminate family members from strangers), while hypermetamorphosis and hypersexuality are not usually significant. 3,4 These individuals also exhibit limited social contacts and have prominent self-directed stereotypic behaviors. 4 Herpetic encephalitis is one of the most frequent causes of bi-temporal lesions during childhood, 3 possibly due to viral invasion of the amygdala via the olfactory tract. We report here a patient with sequelae of neonatal Herpes simplex encephalitis who had childhood KBS. Selective serotonin re-uptake inhibitors (SSRI) affected the social behavior of this patient. We discuss the significance of this effect with regard to the pathophysiology of autism. Case reportThe patient was a boy who suffered from febrile illness 8 days after birth. He was diagnosed with neonatal herpetic meningoencephalitis based on an elevated cell count, positive polymerase chain reaction (PCR) for Herpes simplex virus-1 (HSV-1) in the cerebrospinal fluid, and elevation of serum HSV-IgM. The baby was discharged after treatment with acyclovir for 3 weeks. Computed tomography (CT) on discharge showed high-density regions over the bilateral anterior temporal lobe and insular cortex (Fig. 1a,b). Infantile spasms appeared at 5 months of age, and were successfully treated with adrenocorticotropic hormone. Hypsarrhythmia disappeared, and electroencephalography showed rare and equivocal spikes in the central areas. Psychomotor developmental delay, however, became evident thereafter. Although the patient could roll over at 8 months of age, choreic movements of the extremities rendered him unable to hold objects in his hands. These involuntary movements disappeared by 11 months of age, and soon after he could maintain a sitting position without support. Brain CT at this stage showed marked atrophy with calcification of the bilateral amygdala and anterior hippocampus (Fig. 1c). Calcification was also evident over the bilateral insular cortex, but no other brain structures showed abnormalities (Fig. 1d). He showed the stereotypical habit of placing his fingers into his mouth, along with toys, clothing, and the hands of other individuals. He had been eating paste food due to mild dysphagia, and appetite loss appeared during this period. He showed no emotional empathy for other individuals, and even smiled when scolded. Eye contact was brief and inconsistent. He did not express sadness when separated from his mother. He enjoyed tactile and a...

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Pheochromocytoma, a catecholamine-secreting tumor arising from the chromaffi n cells of the sympathetic nervous system, is uncommon in children and accounts for <1% of pediatric hypertension. 1,2 Besides severe hypertension, patients suffer from headaches, diaphoresis, palpitations, and exhaustion due to catecholamine excess. As a result of hypermetabolism, poor weight gain may also be prominent despite good appetite. However, no association between growth failure and pheochromocytoma has been reported in the literature. Here we report an unusual case of childhood-onset pheochromocytoma complicated by short stature as a presenting feature. Case reportA 13-year-old Japanese boy was admitted to Matsue Red Cross Hospital for severe headache and palpitation. He was born uneventfully at term via cephalic presentation with a length of 50.3 cm and a weight of 3280 g. There was no family history of short stature (his father and mother were 174 cm and 156 cm tall, respectively), hypertension, endocrine disease, or phacomatosis. The boy was well until 10 years of age when his short stature became apparent ( Fig. 1 ). From age 11 on, he began to suffer from episodic headaches, nausea and palpitations. Diaphoresis and polydipsia had also developed.On admission the patient was fl ushed, diaphoretic and appeared exhausted. Remarkable short stature (133.8 ± 3.0 cm) was found with normal proportions (bodyweight, 29.5 ± 1.9 kg; body mass index [BMI], 16.5 kg/m 2 ) and prepubertal external genitalia. Marked hypertension (fl uctuating, 170 -240/110 -130 mmHg) was noted on his initial examination, while his pulse rate was regular at 90 beats per min. Neither a goiter nor an abdominal tumor was palpable. Ophthalmoscopy indicated mild retinal hemorrhage.Routine laboratory fi ndings were normal, including complete blood counts, serum chemistries, and urinalysis (Table 1). The plasma and urinary levels of norepinephrine were signifi cantly elevated at 22 500 pg/mL (normal range, 90 -420 pg/mL) and 2900 g/24 h (normal range, 19 -151 g/24 h), respectively, while plasma and urinary epinephrine or dopamine levels were all normal. The spot urinary vanillylmandelic acid level was also elevated. No evidence of adrenocortical hyperfunction, thyrotoxicosis or multiple endocrine neoplasia was found. Abdominal ultrasonography, computed tomography (CT; Fig. 2 ) and magnetic resonance imaging (MRI) demonstrated a well-defi ned, 5.0 × 4.5 cm solid mass in the right infrarenal area: both adrenal glands appeared normal. Scanning with radiolabeled m -iodebenzylguanidine, just once after 24 h of the infusion, failed to show uptake by the tumor. Both cranial and chest CT also did not produce any other tumor images.Under a clinical diagnosis of solitary, extra-adrenal, norepinephrine-producing pheochromocytoma, 50 g tumor tissue was surgically removed. The pathological fi nding of the tumor sections were consistent with pheochromocytoma on HE staining, with partial immunoreactivity of vasoactive intestinal peptide but not of somatostatin . 3 Electron micro...