Neural larva migrans caused by the raccoon roundworm Baylisascaris procyonis

Baylisascaris procyonis, the common raccoon roundworm, is a rare cause of devastating or fatal neural larva migrans in infants and young children. We describe the clinical features of two children from suburban Chicago who developed severe, nonfatal B. procyonis neural larva migrans. Despite treatment with albendazole and high dose corticosteroids, both patients are neurologically devastated. In many regions of North America, large populations of raccoons with high rates of endemic B. procyonis infection live in proximity to humans, which suggests that the risk of human infection is probably substantial. In the absence of effective treatment, prevention of infection remains the most important public health strategy.     

Surgical treatment and outcome of mega-hydronephrosis due to pelviureteric junction stenosis

To study the surgical treatment and outcome of hydronephrosis secondary to pelviureteric junction obstruction (PUJS) that is so massive that the renal pelvis crosses over the vertebral column [mega-hydronephrosis (MH)]. Of the 40 cases of PUJS we treated over the past 5 years, 6 cases had MH secondary to PUJS (MH-PUJS) in 6 renal units and were reviewed. Incidence of MH was 15.0%. All had Anderson–Hynes dismembered pyeloplasty to treat obstructive renal pattern on technetium-99m diethylenetriaminepentaacetic acid (DTPA) scans in five and gait disturbance due to MH in one. The contralateral kidney was normal in five and dysplastic in one. Three had nephrostomy before pyeloplasty. Preoperative DTPA scans showed good renal function in four and poor in two. Plication of the entire dilated, lax renal parenchyma (“nephroplication”) was performed in three to decrease the intra-renal pelvic cavity. Prenatal diagnosis was performed in three cases. Mean age at pyeloplasty was 3.5 years and mean duration of follow-up was 1.5 years. Postoperatively, two of the three who did not have nephroplication had prolonged but not obstructed renal pattern on DTPA scans, and the other case who did not have nephroplication had nephrectomy for non-functioning kidney caused by recurrent pyelonephritis 2 years after pyeloplasty. In the three who had nephroplication, DTPA scans showed acceptable to relatively good passage in all cases. The surgical treatment of MH–PUJS is challenging with no fixed guidelines. However, nephroplication may be useful during pyeloplasty to decrease the size of MH–PUJS.     

Favourable long-term outcome after immediate treatment of neonatal hyperammonemia due to N-acetylglutamate synthase deficiency

Introduction  

N-Acetylglutamate synthase (NAGS) deficiency is a rare urea cycle disorder, which may present in the neonatal period with severe hyperammonemia and marked neurological impairment.     

Children with congenital hypothyroidism: long-term intellectual outcome after early high-dose treatment

We aim to determine long-term intellectual outcome of adolescents with early high-dose treated congenital hypothyroidism (CH). Sixty-three prospectively followed children with CH were assessed at age of 14 y with the Wechsler Intelligence Scale for Children-Revised and compared with 175 healthy controls. Median age at onset of treatment was 9 d (range 5-18 d) and median starting dose of levothyroxine (L-T4) was 14.7 microg/kg/d (range 9.9-23.6 microg/kg/d). Full-scale intelligence quotient (IQ) was significantly lower than in controls after adjustment for socioeconomic status (SES) and gender (101.7 versus 111.4; p < 0.0001). Children with athyreosis had a lower performance IQ than those with dysgenesis (adjusted difference 7.6 IQ scores, p < 0.05). Lower initial thyroxine (T4) levels correlated with poorer IQ (r = 0.27, p = 0.04). Lower SES was associated with poorer IQ, in particular in children with CH (interaction, p = 0.03). Treatment during childhood was not related to IQ at age 14 y. Adolescents with CH manifest IQ deficits when compared with their peers despite early high-dose treatment and optimal substitution therapy throughout childhood. Those adolescents with athyreosis and lower SES are at particular risk for adverse outcome. Therefore, early detection of intellectual deficits is mandatory in children with CH.     

先天性肾上腺皮质增生症早期治疗回顾分析

目的探讨先天性肾上腺皮质增生症(CAH,21-羟化酶缺乏)早期治疗的疗效。方法回顾性分析1997年至2011年出生,在6月龄内诊断并开始治疗的69例CAH患儿病史资料,了解早期治疗对患儿生长发育的影响,探讨治疗随访的评估指标。结果 69例患儿于生后4~180 d开始接受治疗,平均中位年龄47 d;平均生长速率为(7.4±1.1)cm/年;末次随访中位年龄3.4岁(0.67~9.92岁),身高均位于正常儿童生长水平的第25~50百分位;76%患儿的骨龄接近实际年龄;预测男女患儿终身高分别为(168.9±8.5)cm和(155.4±8.0)cm,71%位于遗传靶身高范围内,优于以往报道的诊断和治疗年龄>1岁患儿的终身高[(151.0±7.0)cm];真性性早熟发生率为5.79%(4/69),低于以往报道≤3岁及>3岁治疗者性早熟发生率(分别为14%及50%);治疗期间电解质、促肾上腺皮质激素(ACTH)、睾酮水平控制正常比例占86%~93%,17-羟孕酮波动较大;促肾上腺皮质激素、17-羟孕酮、睾酮呈正相关。结论早期治疗能改善CAH患儿的生长,降低性早熟发生率,提高终身高。     

Use of C-reactive protein to guide duration of empiric antibiotic therapy in suspected early neonatal sepsis

BACKGROUND: Serial C-reactive protein (CRP) measurements have been shown to be useful for guiding duration of antibiotic therapy in neonates. This study sought to determine whether this is a safe and practical approach in a developing country. METHODS: The study was conducted at the Johannesburg Hospital between September 15, 1998, and January 15, 1999. Subjects included all neonates evaluated for suspected sepsis in the first 24 h of life who had negative initial and repeat CRP values (< or = 10 mg/l) [corrected]. Repeat CRP measurements were performed between 24 and 48 h after birth. Antibiotic therapy was stopped in these infants at 24 to 48 h, and they were observed until 72 h, when the final blood culture results were available. The number of positive blood cultures in this group was determined. RESULTS: The repeat CRP estimation correctly identified 99 of 100 infants in the study as not requiring further antibiotic therapy (negative predictive value, 99%; 95% confidence intervals, 95.6 to 99.97%). The 1 infant with a positive blood culture was premature with a gestational age of 31 weeks. Eight babies required repeat evaluation for suspected sepsis, 4 presented on Day 3 to 4 and one of these babies died. All these neonates were of < or =33 weeks gestation. CONCLUSION: The use of serial CRP measurements to guide antibiotic therapy is a safe and practical approach in neonates with suspected sepsis in a developing country.     

Children with very early onset obsessive-compulsive disorder: clinical features and treatment outcome

Background: There is emerging evidence that early onset obsessive‐compulsive disorder (OCD) may be a phenomenologically distinct subtype of the disorder. Previous research has shown that individuals who report an early onset display greater severity and persistence of symptoms, and they may be less responsive to treatment. To date, this question has been investigated solely in adult samples. The present study represents the first investigation into the effect of age at onset of OCD on clinical characteristics and response to treatment in a paediatric sample. Method: A total of 365 young people referred to a specialist OCD clinic were included in the study. Clinical records were used to examine potential differences in key clinical characteristics between those who had a very early onset of the disorder (before 10 years) and those who had a late onset (10 years or later). Group differences in treatment responsiveness were also examined within a subgroup that received cognitive behaviour therapy (CBT) alone or CBT plus medication (n = 109). Results: The very early onset group were characterised by a longer duration of illness, higher rates of comorbid tics, more frequent ordering and repeating compulsions and greater parent‐reported psychosocial difficulties. There were no differences in treatment response between the groups, and when age at onset was examined as a continuous variable, it did not correlate with treatment response. Conclusions: Very early onset OCD may be associated with different symptoms and comorbidities compared with late onset OCD. However, these differences do not appear to impact on responsiveness to developmentally tailored CBT alone or in combination with medication. These findings further indicate the value in early detection and treatment of OCD in childhood.     

Factors associated with increased risk of inappropriate empiric antibiotic treatment of childhood bacteraemia

To identify bacteraemic children who are at increased risk of inappropriate empiric antibiotic therapy, we performed univariate and multivariate analyses of prospectively-studied bacteraemic episodes. Appropriateness of therapy was defined according to the in vitro susceptibility of the isolate. Inappropriate empiric therapy was found in 38% of 516 bacteraemic episodes and was associated with higher mortality. The rate of inappropriate treatment was lower in neonates and infants (28% and 33%, respectively) but higher in children 1- to 5-years old (51%,P=0.0029). The rate was dependent on the source of bacteraemia (range, 18%–70%,P=0.0092), underlying conditions (range, 26%–53%,P=0.0001), the specific paediatric section in which the child was hospitalized (range, 24%–70%,P=0.0002), and the causative micro-organism (range, 15%–75%,P<0.0001). Four clinical variables that independently and significantly affected the rate of inappropriate antibiotic treatment were identified by multivariate stepwise logistic regression analysis (odds ratios in parenthese): hospital-acquired bacteraemia (2.3), age of 1- to 5-years (2.1), cytotoxic therapy (1.8) and presence of central IV line (1.6).     

Impaired neuromotor outcome in school-age children with congenital hypothyroidism receiving early high-dose substitution treatment

Congenital hypothyroidism (CH) can lead to intellectual deficits despite early high-dose treatment. Our study aimed to determine whether motor impairments can occur despite early high-dose treatment. Sixty-three children with CH and early (median age of onset of treatment 9 d), high-dose treatment (median starting dose of levothyroxine 14.7 μg/kg/d) were tested with the Zurich Neuromotor Assessment (ZNA) at a median age of 13.8 y (range 7.0-14.2 y). Median z-scores in the children with CH were -0.95 in the pure and -0.56 in the adaptive fine motor component, significantly lower than in the ZNA test norms (p < 0.001 and p = 0.01, respectively). The 26 children with athyreosis were more affected than the 33 children with dysgenesis, particularly in the pure motor (-1.55 versus -0.76, p = 0.03), adaptive fine motor (-1.31 versus 0.13, p < 0.01), and static balance task (-0.47 versus 0.67, p = 0.01). Boys performed worse than girls. Older age at onset of treatment was related to poorer adaptive fine motor performance. Movement quality (assessed by associated movements) was not affected. We conclude that severe CH can cause neuromotor deficits persisting into adolescence. These deficits cannot completely be reversed by postnatal treatment, but earlier age at treatment may reduce the degree of impairment.     

Continuous infusion of ceftazidime in the empiric treatment of febrile neutropenic children with cancer

PURPOSE: Infection remains one of the most important complications in cancer therapy. The choice of antibiotics and the method of administration can affect results. Beta-lactam antibiotics can be administered by several short injections per day or by continuous infusion. The latter modality may provide superior pharmacokinetics. PATIENTS AND METHODS: The authors studied the pharmacokinetics of ceftazidime in children treated for malignancy and in febrile aplasia after chemotherapy. They received a continuous infusion of ceftazidime (200 mg/kg/day) after a loading dose (65 mg/kg/day) administered with amikacin (25 mg/kg/day) and vancomycin (50 mg/kg/day).RESULTS Twenty-three pharmacokinetic studies were performed. Mean ceftazidime serum levels were 31.1 +/- 11.9, 31.2 +/- 10, 32.4 +/- 11.6, 33 +/- 11.6, and 30.4 +/- 12.1 mg/L at 25, 27, 30, 36, and 43 hours, respectively. Treatment was tolerated well. There were no toxic or infectious deaths. CONCLUSIONS: Ceftazidime's time-dependent pharmacokinetics shows the advantage of continuous infusion. This study confirmed the feasibility and safety of this administration schedule in the empiric treatment of febrile neutropenic children with cancer.     

宫内治疗与新生儿近期预后(附2例报告)

目的评估宫内治疗的新生儿结局。方法对2例先天疾病胎儿(1例胸腔积液,1例胎儿巨大膀胱)采用超声波引导下的穿刺治疗。结果新生儿出生时情况稳定,为诊断、治疗赢得了时间,经过产科、新生儿科、外科共同合作,患儿均健康出院。结论在多学科合作下的宫内介入性治疗将提高围产医学的诊疗水平。