Long-term renal follow up of preterm neonates born before 35 weeks of gestation.

BACKGROUND: The hypothesis of the Developmental Origins of Health and Disease states that environmental factors during fetal and infantile life are risk factors for some chronic diseases in adulthood. Few studies, however, have confirmed this hypothesis early in childhood. Therefore, we assessed how premature birth and low-birthweight (LBW) affect the renal function of Japanese children. METHODS: This retrospective study surveyed 168 patients who were born before 35 weeks of gestation and were cared for at the present neonatal intensive care unit. Follow-up duration was >2 years. Serum creatinine (sCr) and estimated glomerular filtration rate (eGFR) recorded in medical records were reviewed. RESULTS: The eGFR at 2 years of age was significantly correlated with birthweight and gestational age (P < 0.01). Approximately 10.7% of the children had low eGFR (<90 mL/min/1.73 m2 ) without clinical symptoms or abnormal urine examination. These children had high sCr on day 7 after birth (P < 0.01) and delayed recovery of these levels during the first month after birth. CONCLUSION: Premature gestational age and LBW directly affect renal function in young children. High sCr on day 7 after birth is a risk factor for chronic kidney disease in children. Careful follow up of renal function is therefore required for premature infants and infants with LBW beginning in early childhood to prevent renal dysfunction.

Increased community-acquired upper urinary tract infections caused by extended-spectrum beta-lactamase-producing Escherichia coli in children and the efficacy of flomoxef and cefmetazole.

BACKGROUND: Urinary tract infections caused by extended-spectrum beta-lactamase-producing bacteria are increasing worldwide. At our hospital, the number of pediatric patients hospitalized because of an upper urinary tract infection has dramatically increased since 2016. In total, 60.5% of urinary tract infections are caused by extended-spectrum beta-lactamase-producing Escherichia coli. Such a high prevalence of extended-spectrum beta-lactamase-producing E. coli has not been detected previously in Japan. Therefore, we evaluated the clinical and bacteriologic characteristics and efficacy of antibiotics against upper urinary tract infections caused by E. coli in children. METHODS: This retrospective study surveyed 152 patients who were hospitalized in the pediatric department of Shimane Prefectural Central Hospital because of upper urinary tract infections caused by E. coli. Medical records were reviewed to examine patient characteristics. O antigens, antibiotic susceptibility, gene typing, and pulse-field gel electrophoresis were studied at the Shimane Prefectural Institute of Public Health and Environmental Science. RESULTS: Urine sample analyses showed extended-spectrum beta-lactamase types such as CTX-M-9 and plural virulence genes. We changed the primary antibiotic treatment to flomoxef or cefmetazole to treat upper urinary tract infections caused by Gram-negative bacilli. After changing treatment, the time to fever alleviation was significantly shortened. CONCLUSION: Extended-spectrum beta-lactamase-producing E. coli should be suspected in community-acquired upper urinary tract infections. Therefore, when treating patients, it is necessary to focus on antibiotic susceptibility and the prevalence of extended-spectrum beta-lactamase-producing bacteria found in each area. Flomoxef and cefmetazole are useful primary treatments for upper urinary tract infections caused by extended-spectrum beta-lactamase-producing E. coli.

Effect of Intrathecal Baclofen on Delayed-Onset Paroxysmal Dystonia due to Compression Injury Resulting From Congenital and Progressive Spinal Bone Deformities in Chondrodysplasia Punctata.

BACKGROUND: Dystonia due to spinal lesions in adult patients is characterized by the provocation and/or amelioration of the spasm by somatosensory stimulation with a sensory trick. PATIENT DESCRIPTION: An infant with brachytelephalangic chondrodysplasia punctata developed flaccid tetraplegia due to cervical cord compression resulting from congenital atlantoaxial dislocation. Episodic, tonic extension of the extremities, neck, and trunk had appeared daily since age two years and was often provoked by tactile stimulation. Although decompression surgery was performed at age three years, progressive spinal deformity resulted in the aggravation of episodic dystonia thereafter, lasting for hours. Foot dorsiflexion and wearing a truncal brace for scoliosis inhibited these spasms. Intrathecal baclofen bolus injection transiently ameliorated the paroxysmal dystonia and detrusor-sphincter dyssynergia in the lower urinary tract. CONCLUSION: Paroxysmal dystonia is unusual in children with spinal cord lesions; however, it should be recognized for appropriate individualized clinical management.

Pediatric granulomatous orchitis: Case report and review of the literature.

An 11-year-old boy presented with fever and abdominal pain, and was diagnosed with retroperitoneal lymphadenitis. At the same time, a painless right scrotal mass was observed. On imaging the testis and the epididymal mass both had abundant blood flow, although tumor markers were negative. Although the right testis had shrunk after antibiotic treatment, swelling was persistent and incisional biopsy was therefore performed, resulting in diagnosis of granulomatous orchitis (GO). No recurrence was found. In cases of scrotal swelling in both the testis and the epididymis of an older child, it is necessary to consider the possibility of inflammatory GO, and orchiectomy should not be performed without careful consideration.

Induction of differentiation of myeloid leukemia cells in primary culture in response to lithocholic acid acetate, a bile acid derivative, and cooperative effects with another differentiation inducer, cotylenin A.

Lithocholic acid (LCA) acetate induced the differentiation of human leukemia cells. Treatment with a combination of LCA acetate and cotylenin A, an inducer of the differentiation of leukemia cells, was more effective than that with LCA acetate or cotylenin A alone at inducing monocytic differentiation. LCA acetate activated mitogen-activated protein kinase (MAPK) before inducing differentiation. Cotylenin A did not activate MAPK, suggesting that cotylenin A has a different mode of action. The cooperative effects of LCA acetate and cotylenin A on inducing differentiation were, at least partly, due to the enhancement of LCA acetate-induced MAPK activation by cotylenin A.

Clinical and electrophysiological features of Japanese pediatric long QT syndrome patients with KCNQ1 mutations.

BACKGROUND: The purpose of the present paper was to determine the clinical and electrophysiological features of Japanese pediatric long QT syndrome (LQTS) patients with KCNQ1 mutations (LQT1). METHODS: KCNQ1 mutations were analyzed in 13 Japanese pediatric patients with prolonged QT interval on electrocardiogram (ECG). These LQT1 patients were reviewed, retrospectively, for the presence of past and family histories of LQTS-related cardiac events, other complications, and their ECG findings evaluated at rest and during exercise). RESULTS: KCNQ1 mutations were identified in eight patients (61.5%) from six unrelated families. Four missense mutations were identified in seven patients and an insertion/deletion in one. The mutations were located in the transmembrane domain in four patients and the C-terminal domain in four. Syncope did not occur in patients with the C-terminal domain mutations up to the age of 6-9 years, but family members of patients with the C-terminal domain mutations had a history of syncope in their elementary school days. Compared with a non-LQTS control group, peak heart rate (HR) on exercise and the HR increase during exercise were significantly lower in the LQT1 group (LQT1 vs control, 155 +/- 16 beats/min vs 182 +/- 13 beats/min, P < 0.01, 66 +/- 16 beats/min vs 99 +/- 24 beats/min, P < 0.01, respectively). CONCLUSIONS: The risk of LQTS-related cardiac events may not be different in pediatric LQT1 patients with C-terminal domain mutations than in patients with transmembrane domain mutations. Possible sinus node dysfunction or a poor HR response to sympathetic stimulation has been suggested in pediatric LQT1 patients.

Mesoblastic nephroma: a case report of prenatal detection by MR imaging.

We report a case of mesoblastic nephroma detected prenatally by magnetic resonance (MR) imaging. MR imaging could provide valuable information about the origin and nature of a fetal abdominal mass and help define the relationship of the mass to adjacent structures.

Two Japanese families with hyperekplexia who have a Arg271Gln mutation in the glycine receptor alpha 1 subunit gene.

We report two Japanese patients from two families with hyperekplexia who have a Arg271Gln mutation in the glycine receptor alpha 1 subunit gene. The clinical course of both patients was typical for hyperekplexia, characterized by neonatal hypertonia and exaggerated startle response, and which improved gradually with age. One was associated with umbilical hernia and hip dislocation, diagnosed at 11 months, while the other was diagnosed at 1 month. Both showed positive head retraction reflex. Four Japanese families have been reported as having hyperekplexia including our cases, of which three have shown the same missense Arg271Gln mutation, most frequently found in patients from Northern Europe and the United States.