Norihisa Wada

Effect of therapeutic touch on brain activation of preterm infants in response to sensory punctate stimulus: a near-infrared spectroscopy-based study

Objective The purpose of this study was to determine whether therapeutic touch in preterm infants can ameliorate their sensory punctate stimulus response in terms of brain activation measured by near-infrared spectroscopy. Methods The study included 10 preterm infants at 34–40 weeks’ corrected age. Oxyhaemoglobin (Oxy-Hb) concentration, heart rate (HR), arterial oxygen saturation (SaO2) and body movements were recorded during low-intensity sensory punctate stimulation for 1 s with and without therapeutic touch by a neonatal development specialist nurse. Each stimulation was followed by a resting phase of 30 s. All measurements were performed with the infants asleep in the prone position. Results sensory punctate stimulus exposure significantly increased the oxy-Hb concentration but did not affect HR, SaO2 and body movements. The infants receiving therapeutic touch had significantly decreased oxy-Hb concentrations over time. Conclusions Therapeutic touch in preterm infants can ameliorate their sensory punctate stimulus response in terms of brain activation, indicated by increased cerebral oxygenation. Therefore, therapeutic touch may have a protective effect on the autoregulation of cerebral blood flow during sensory punctate stimulus in neonates.

Perinatal factors associated with long-term respiratory sequelae in extremely low birthweight infants

Objective To assess lung function at 8 years old in extremely low birthweight (ELBW) survivors and to identify perinatal determinants associated with impaired lung function. Design Retrospective cohort study. Setting Level III neonatal intensive care unit. Patients ELBW survivors born in 1990–2004 with available spirometry at 8 years old were studied. Children were excluded if they had a Wechsler Intelligence Scale for Children Third Edition full IQ <70. Main outcome measures Multivariate logistic regression analysis was used to identify perinatal determinants associated with airway obstruction (forced expiratory volume in 1 s (FEV1)/forced vital capacity (FVC) ratio <80%) at school age and the predictive power of potential determinants. Potential risk factors and predictors assessed in this study were gestational age, birth weight, small for gestational age, sex, chorioamnionitis, premature rupture of membranes, antenatal steroids, surfactant administration, respiratory distress syndrome, postnatal steroids, severe bronchopulmonary dysplasia and bubbly/cystic appearances of the lungs by X-ray during the neonatal period. Results Of 656 ELBW survivors, 301 (45.9%) had attended a school-age follow-up at 8 years old. A total of 201 eligible children completed the lung function test. Bubbly/cystic appearance of the lungs (OR 4.84, 95% CI 1.26 to 18.70) was associated with a low FEV1/FVC ratio. Children with bubbly/cystic appearance had characteristics of immaturity and intrauterine inflammation. Conclusions Within a cohort of ELBW infants, a bubbly/cystic appearance of the lungs in the neonatal period was the strongest determinant of a low FEV1/FVC ratio at school age.

Cyclosporine A causes maturation failure in embryonic-type glomeruli persisting after birth.

INTRODUCTION We analyzed renal histologic and immunohistologic findings in children with nephrotic syndrome (NS) who did (n=5) or did not (n=17) develop cyclosporine A (CyA) nephropathy despite appropriately low serum CyA concentrations being maintained over 2 years. METHODS To discriminate embryonic-type from mature glomeruli, we performed staining for type IV collagen a1, laminin ß1 and laminin ß2. Staining patterns were used to semiquantitatively assess glomerular immaturity (glomerular immaturity index, or GII). RESULTS In follow-up biopsy specimens, residual embryonic-type, collapsed embryonic-type and sclerotic glomeruli that had failed to differentiate were observed. Patients with early-onset CyA nephropathy had a high GII. In patients with a high GII, arteriopathy developed early in CyA treatment. Arteriopathy was observed mostly near embryonic-type glomeruli. Taken together, these glomeruli (surviving embryonic-type, collapsing embryonic-type, and sclerotic glomeruli) essentially equaled the total number of embryonic-type glomeruli in specimens obtained before CyA treatment. CONCLUSION Our findings indicate a need for caution in CyA therapy for patients with NS, even for a relatively short course of administration, because some patients may have embryonic-type glomeruli or immature arterioles that predispose them to CyA nephropathy.

A patient with Henoch-Schönlein purpura manifesting unusual symptoms and clinical course.

We evaluated and treated a girl with Henoch-Schönlein purpura (HSP), who initially developed redness, swelling, and pain in all 4 limbs accompanied by Raynaud syndrome and then had convulsions and disturbance of consciousness. HSP was diagnosed based on later findings of purpura in both legs and a decrease in factor XIII activity not accompanied by thrombocytopenia. She was normotensive. A skin biopsy specimen showed small-vessel vasculitis accompanied by immunoglobulin A deposition. The cause of erythema and limb pain, convulsions, and disturbed consciousness presumably was vasculitis. The possibility of HSP should be considered in patients with limb pain despite initial absence of purpura and in patients with central nervous system symptoms such as convulsions.

Guillain-Barré Syndrome and Crohn disease: A Case Report

Development of both Crohn disease and Guillain-Barré syndrome likely involves autoimmunity associated with excessive inflammatory cytokines. We treated a girl who developed Guillain-Barré syndrome during the course of Crohn disease. Although high-dose γ-globulin therapy administered initially for Guillain-Barré syndrome was ineffective, plasmapheresis ameliorated her acute neuropathic symptoms. Crohn disease was managed with Salazopyrin administration and enteral feeding. Chronic inflammation of the intestinal mucosa caused by Crohn disease can allow presentation of microbial intestinal antigens normally hidden from the immune system. Such presentation could incite an extraintestinal immune response on the basis of molecular mimicry, leading to activation of systemic autoimmunity against the nervous system. Accordingly, concurrence of Guillain-Barré syndrome and Crohn disease in our patient appeared to result from shared autoimmune mechanisms and systemic and local increases in cytokine concentrations. The patient also developed erythema nodosum and gall stones, relatively common complications of Crohn disease. However, Guillain-Barré syndrome is rare.

Intravitreal Injection of Bevacizumab for Retinopathy of Prematurity in an Infant with Peters Anomaly

Purpose: To report our findings in an infant with Peters anomaly type II whose retinopathy of prematurity (ROP) was treated with an anti-VEGF agent and surgeries. Case Report: A male infant weighing 548 g was born prematurely at 23 weeks and 1 day with corneal opacity and shallow anterior chambers in both eyes. At the postmenstrual age of 35 weeks and 3 days, the infant was tentatively diagnosed with stage 3 ROP because of a dilated tunica vasculosa lentis and ultrasonographic findings. The boy was treated with bilateral intravitreal injections of bevacizumab (IVB) because laser photocoagulation of the retina could not be performed due to the corneal opacity. The retina in the right eye detached 3 times, namely 5 days, 16 days, and 7 months after the IVB; encircling the scleral buckle and a vitrectomy with endolaser photocoagulation were therefore required. In his left eye, the retina was reattached after the initial IVB, and no additional treatment was required. ROP was not reactivated in both eyes until the last examination at the age of 2 years and 6 months. Conclusions: Our results showed that IVB is a useful treatment for ROP in patients with Peters anomaly. However, a retinal detachment can be a complication after IVB. The optimal timing of IVB for ROP in infants with hazy media needs to be determined.

Renal tubular dysgenesis : the first case reported in Japan

Renal tubular dysgenesis (RTD) is a fatal congenital disease characterized by a defect in the differentiation of the proximal and distal convoluted tubules. This disorder is clinically associated with oligohydramnios, intrauterine growth retardation, and acute renal failure, and the diagnosis is made only at autopsy. We report a very low birth-weight infant with RTD. The infant was delivered at 32 weeks of gestation by cesarian section, because of fetal distress, and weighed 631 g. She had no micturition after birth and developed acute renal failure on day 3 of life. Because ultrasound scan did not show any abnormalities of the kidneys, she was treated aggressively with various blood purification procedures, but she died of sepsis and disseminated intravascular coagulation (DIC) on day 13 after birth. Postmortem examination of the kidneys showed glomerular crowding and undifferentiated tubules. Positive staining of tubular epithelial cells for epithelial membrane antigen supported a diagnosis of RTD. When renal failure occurs in a neonate without any gross morphological abnormalities of the kidneys on ultrasound imaging, RTD should be considered. A review of the literature showed that this is the first case of RTD reported in Japan.

Liver fibrosis with hypereosinophilia causing transient abnormal myelopoiesis

Transient abnormal myelopoesis is mostly self‐resolving and has a good prognosis, but some patients subsequently die of liver fibrosis. We report the case of an infant with Down syndrome who developed life‐threatening liver fibrosis at the same time as the blasts were about to disappear. This patient also had a marked increase in eosinophils, which were possibly harboring a GATA1 mutation and were expressing a high level of platelet‐derived growth factor‐B mRNA; these may have been involved in the development of liver fibrosis. Low‐dose cytosine arabinoside therapy effectively treated both hypereosinophilia and liver fibrosis.

Non-invasive renal artery embolization for renal dysplasia accompanied by hypertension

Renovascular hypertension caused by renal dysplasia often is resistant to drug therapy. For a 14‐year‐old girl with such refractory hypertension, a non‐invasive right renal ablation by embolization with anhydrous ethanol using a shepherd ‘s‐crook’ balloon catheter, was done. Blood pressure then rapidly normalized. Apart from mild fever after the procedure, no adverse effects occurred. In patients with mild renal artery stenosis and hypertension resistant to anti‐hypertensive drug therapy, renal artery embolization may be a useful option.