Background

Copper is an essential micronutrient, vital for various enzymatic functions. It is generally absorbed from stomach and duodenum with normal daily requirement of 0.2-0.5mg in children. Malnutrition and short bowel syndrome are known risk factors for copper deficiency, manifesting as myelodysplasia and neurological disorders.

Objectives

To raise awareness regarding copper deficiency in children with transpyloric feeds and highlight management challenges in paediatric intensive care.

Methods

A 2 years old boy with X-linked myotubular myopathy and severe gastro-oesophageal reflux had three admissions to PICU within 2 weeks due to respiratory distress and persistent tachycardia requiring invasive ventilation. There were no obvious reasons for the deterioration apart from severe anaemia Hb 69g/dL and severe copper deficiency with levels 0.4mmol/L (Normal values: 11-22).

Results

Intravenous copper replacement 0.2mg/day for 14 days, resulted in transient improvement in Copper levels to 14.7mmol/L, dropping to 5.9mmol/L once treatment was stopped and IV replacement was repeated. His tachycardia and respiratory deterioration had a temporal correlation with the copper deficiency and improved after supplementation. Copper replacement in this case was very challenging due to difficult intravenous access, risk of anaesthesia and inability to use gastric route due to severe reflux and risk of aspiration.

After multiple discussions, he was commenced on high dose Copper replacement via jejunal route i.e. 1.67mg/day that included his usual feeds + supplements ‘Seravit’. His Copper levels gradually improved to 23 and supplementation was adjusted.

Conclusion

Copper deficiency should be considered in patients on long-term trans-pyloric feeds and high dose jejunal replacements could be attempted in complex cases.

Background

Anthropometric measures provides monitor and asses the effectiveness of interventions and avoid harmful damages.

Objectives

Describing the weight profile of preterm infants at birth, beginning oral feeding, hospital discharge and at the first follow-up visit .

Methods

Data were collected regarding the birth, beginning oral feeding, hospital discharge and first follow-up visit (gestational age, corrected gestational age and weight). Weight data were placed on the Fenton Scale. Data were presented in median, minimum and maximum. The study used the sample including preterm infants, who were hospital discharged in full oral feeding.

Results

The sample consisted of 11 male and 7 female. The median gestational age at birth and birth weight was 30+6 weeks/ 1320g, minimum 25+4 weeks/775g, maximum 33 + 2 weeks/1940g. The median corrected gestational age in the beginning oral feeding and weight was 32+5 weeks/1423g, minimum 31+5 weeks/1090g, maximum 35 + 2 weeks/1860g. The median corrected gestational age and weight at the hospital discharge was 37+2 weeks/1928g, minimum 35+1 weeks/1860g, maximum 39+3/2190g. The median corrected gestational age and weight at the first visit was 39 weeks/2120g, minimum 35+5 weeks/1965g, maximum 40+4 weeks/2520g

Conclusion

During the hospitalization, the premature infants lose weight, meanly after the beginning oral feeding. Even though there was weight gain there wasn’t recovery of the weight profile on the Fenton scale.

Background

Advances in perinatal medicine allow the survival of newborns increasingly premature but with an uncertain neurological outcome due to multiple factors.

Objectives

To determine the clinical and epidemiological characteristics of preterm infants23-25weeks of gestational age and their neurological prognosis at two years of corrected gestational age.

Methods

Preterm infants of23+0-25+6 weeks of gestational age born in a tertiary hospital in2014-2015.They have been divided into two groups:Group 1:live newborns without sequelae or with minor sequelae at two years corrected age and group2:moderate-severe sequelae (cerebral palsy with GMFC>2,blindness of one or both eyes,hearing loss>40 dB)or death.

Results

40 newborns under26 weeks were collected(7 of 23 weeks,14 of 24 weeks and19 of 25 weeks).

There is a predominance of males in group 2(60%) compared to20% in the first group(p<0.05). The average weight of the first group was677 grams versus645 grams of the second(p 0.097).93.3% of patients in group1 received antepartum antibiotic treatment compared to 56% in the second group(p<0.05).

Regarding evolution,80% of patients in group2 received a transfusion of blood products compared to53% in group 2(p 0.091).There were no differences regarding gestacional age, the temperature at admission, treatment of the ductus or need for vasoactive drugs.

In cerebral ultrasound,44% of patients in group2 had grade4intraventricular hemorrhage compared to13% in group1(p 0.08).

Conclusion

In our series, male patients and those with HIV grade 4 have worse evolution. The use of prenatal antibiotics is recognized as an effective strategy to stop premature birth. It would be necessary to expand the number of patients in order to draw better conclusions.

Background

Some infants with traumatic brain injury have been reported having biphasic clinical courses with abnormal head magnetic response imaging (MRI) images resembling acute encephalopathy with biphasic seizures and late reduced diffusion (AESD), which is the most common encephalopathy in Japanese children.

Objectives

We report a Japanese case of infantile traumatic brain injury with a biphasic clinical course and abnormal late head MRI image resembling AESD. It was diagnosed as abusive head trauma (AHT).

Methods

Case report.

Results

Case: A previously healthy 8-mouth-old boy fell backwards and presented with seizures for sixty minutes. Right after arrival, he was intubated because of consciousness disturbance. Brain computed tomography (CT) showed right acute subdural hematoma. He underwent barbiturate coma therapy (BCT) for the next 48 hours aiming at suppression-burst pattern on continuous electroencephalogram (cEEG) due to prolonged seizures. On day four, he had second phase of seizures detected by cEEG monitoring. Therefore, he underwent BCT for the next 48 hours again. On day five, he underwent brain MRI showing subcortical white matter lesions on diffusion-weighed image, mimicking AESD. On day thirty-six, he was discharged with developmental regression and tubal enteral feeding.

Conclusion

Late onset of subclinical seizures in infantile head trauma can be successfully detected by using cEEG for further neurological treatment.

Background

Cerebrovascular autoregulation (CA) is a physiologic mechanism aimed to preserve cerebral blood flow. CA can be continuously monitored processing commonly available signals and its impairment after brain injury is associated with poor outcome. Pressure Reactivity index (PRx) expresses the correlation between arterial blood pressure (ABP) and intracranial pressure (ICP). It has been previously used to measure optimal cerebral perfusion pressure (CPPopt) in adult and pediatric patients.

Objectives

To describe feasibility of PRx monitoring and CPPopt determination in a case of pediatric traumatic brain injury (TBI).

Methods

We prospectively collected data using the ICM+ Software (Cambridge University, UK) in a 6 years-old patient admitted after severe TBI. Despite treatment, he presented refractory intracranial hypertension and underwent decompressive craniectomy, with good neurological outcome at hospital discharge (GOS:5).

Results

ABP and ICP signals were successfully acquired and processed for a total of 83 hours. PRx was continuously displayed and recorded, on an observational basis, excluding during neuroimaging and neurosurgery time. CPPopt was calculated plotting PRx against CPP within periods of 4 hours. Where available, this resulted in a U-shaped curve where CPPopt is the minimum value (Figure).

Conclusion

PRx monitoring seems to be feasible, allowing CPPopt calculation. We hypothesize that it may be a valuable tool in the context of multi-modal monitoring after TBI in children. Further research is ongoing to define its role in clinical practice.

Background

Disseminated tuberculosis is a severe disease with high-mortality that requires early diagnosis and treatment.

Objectives

To report a case of severe disseminated tuberculosis treated with linezolid.

Methods

Review of medical records and literature.

Results

A 4-year-old girl, previously healthy, was admitted at our hospital reporting intermittent fever, diarrhea, night sweats, weight loss and cough. For the positive epidemiological history for tuberculosis and chest radiography with miliary pattern, ceftriaxone and first-line oral anti-tuberculosis agents (RHZ: rifampicin, isoniazid and pyrazinamide) were introduced. On the second day, she developed respiratory distress with mechanical ventilation requirement and septic shock. Considering the pulmonary worsening, linezolid and meropenem were associated. She presented a favorable response and was extubated. Linezolid were suspended after 14 days of treatment. However, she evolved with melena, haematochezia and hemorrhagic shock. Endoscopy, colonoscopy and angiography did not exhibit active bleeding. Abdominal tomography revealed lymphadenopathy and distal ileal wall thickening. Ciprofloxacin was associated for the possibility of intestinal tuberculosis with no more bleeding after 7 days. She was discharged after 35 days of hospitalization. Gastric washing identified Mycobacterium tuberculosis with rifampicin-sensitive molecular test and duodenal biopsy demonstrated positive acid-fast bacilli test, confirming the diagnosis of disseminated tuberculosis.

Conclusion

The intestinal hemorrhagic presentation is extremely rare. RHZ is primordial for management. However, due to the possibility of malabsorption and slow onset of action, it is important to highlight the correct use of intracellular agents, with rapid action and adequate infection site penetration in critical patients. In this case report linezolid, meropenem and ciprofloxacin were crucial for patient survival.

Background

Parenteral drug (PD) use is one of the prescribing indicators in terms of rational use of medicine. Though frequently preferred in children, PDs require to be carefully practiced in primary healthcare, particularly in infants.

Objectives

This study aimed to describe PDs prescribed to infants at primary healthcare centers (PHCs) in Turkey.

Methods

PD prescriptions were collected from PHCs in 32 provinces of Turkey. After one hundred PD prescriptions per calendar month from each of the provinces were selected randomly, the prescriptions that were written to infants (<1-year-old) were analyzed based on some drug use parameters.

Results

A total of 156 prescriptions were identified to belong to infants, of which 54.5% were male. Among the total 168 PDs prescribed, 66.7% (n=112) were antibiotics. The most commonly prescribed antibiotic was ceftriaxone (56.3%), followed by cefuroxime (9.8%), sultamicilin (9.8%), and rifamycin (8.9%). In non-antibiotic PDs, the most commonly prescribed drug was sodium chloride (28.5%), methylprednisolone (21.4%), and colecalciferol (19.6%). Gender was not found to affect prescriptions of antibiotic vs. non-antibiotic PDs (males: 61.4% vs. 46.4%, respectively; p=0.07). Rifamycin (6.4%), enoxaparine (0.6%), and trimethobenzamide (0.6%) were the only PDs that were found to be prescribed as off-label in this patient population.

Conclusion

In conclusion, two-thirds of PDs prescribed to infants seem to be antibiotics, more than half of which constitute cephalosporins. It is also remarkable that near one in ten antibiotics were prescribed as off-label.

Background

DC is a controversial procedure at the treatment of resistant ICP in severe traumatic brain injury in children

Objectives

To study the outcome of DC in children after severe TBI (GSC<8).

Methods

A retrospective study was carried out about all children with TBI who were admitted to PICU of children’s hospital P. & A. Kyriakou during the period 1/1/2009-31/12/2016 (7 years) and a DC was performed. The evaluation of the outcome was based on both mortality rate and neurological outcome according to E-GOS at PICU discharge and after a 6-month follow up.

Results

During this period, 78 children with severe TBI were admitted to PICU, 30 of them with initial GSC<8. In 8 children DC with duroplasty was performed. Mean time of DC after brain injury:18h, mean ICP before DC:34mmHg and after DC:25mmHg. In 5 children DC was performed at the same time with drainage of cerebral hematoma. At PICU discharge (mean time of hospitalisation: 18days) mean E-GOS was 5 and at 6 months E-GOS was 7. Two children had DC due to refractory ICP to barbiturate coma. At PICU discharge (mean time of hospitalisation:17days) E-GOS was 3 and at 6 months E-GOS was 4. One child (initial GSC:3 and ICP:50) died. Complications: hydrocephalus(2), infection(1).

Conclusion

In the majority of children large DC was performed early and in combination with hematoma drainage. The early DC seems to correlate with the severity of the neurological outcome, a finding also suggested by the international literature which is limited in number and samples in pediatric patients.

Background

Flecainide is a class IC antiarrhythmic drug indicated for patients with supraventricular arrhythmias. It has proarrhythmic effects and although toxicity is rare, mortality rate can be as high as 10%.

Objectives

Flecainide is primarily metabolized by CYP2D6. We present a case of flecainide toxicity contributed by CYP2D6 polymorphism.

Methods

Patient was born at 29+5 weeks and was initially treated with propranolol for supraventricular tachycardia (SVT). However, he developed a recurrence of SVT and was switched to oral flecainide.

7 days after flecainide initiation, he became bradycardic. Electrocardiogram (ECG) showed sinus node dysfunction and atrioventricular block. The bradycardia was refractory to drugs, transcutaneous and transvenous pacing. In view of poor perfusion and severely reduced biventricular function on 2D-echocardiogram, extracorporeal membrane oxygenation (ECMO) was initiated. He was treated with IV calcium chloride and lipid emulsion and subsequently reverted to sinus rhythm with return of arterial pulsatility. Patient was supported on ECMO for 12 days and started on sotalol for SVT control.

Results

While other causes of flecainide toxicity were ruled out, investigations revealed supratherapeutic serum flecainide concentrations. Since flecainide is metabolised by CYP2D6 which exhibits polymorphism, a gene test was sent out to determine patient’s genotype. Patient has CYP2D6*10X2/*36 genotype that is predicted to have intermediate CYP2D6 enzyme activity which is associated with lower flecainide clearance.

Conclusion

Based on patient’s genotype, 25% dose reduction of flecainide is recommended by some national guidelines. This case report highlights the importance of ECG and flecainide levels monitoring when treating neonates. CYP genotype testing can further identify at-risk patients of toxicity.

Background

Dapsone (4,4 – diaminodiphenylsulphone) is used in various dermatological conditions and Pneumocystis carinii pneumonia. Accidental Dapsone overdose can lead to lifethreatening methhemoglobinemia (MethHb). We are presenting a case report on a toddler with symptomatic methemoglobinemia resistant to standard methylene blue treatment.

Objectives

To study the clinical profile and effectiveness of various treatment strategies in acquired Methemoglobinemia.

Methods

A 2.5 year old girl presented 12 hours after accidental ingestion of dapsone with irritability and central cyanosis with saturations in the 80s. Initial ABG showed normal paO2 levels, but Methemoglobin (MetHb) levels of 32%. Even after 2 bolus doses of methylene blue (G6PD status normal) and Ascorbic acid, she continued to be symptomatic with rebound increase in MetHb levels. Hence methylene blue infusion was started. In view of worsening metabolic acidosis, abnormal LFTs and hemolytic anemia, single volume exchange transfusion was done and high maintenance doses of Ascorbic acid was administered. There was dramatic clinical improvement with drop in methemoglobin levels. She was discharged on day 9, on methemoglobin levels of 5.4%.

Results

Failure of methylene blue therapy in this child might be due to inadequate decontamination, sulfhemoglobinemia or underlying hemoglobin M disease and might explain the response to whole blood exchange transfusion. Addition of ascorbic acid, an antioxidant, might have reduced the formation of new MetHb.

Conclusion

Methylene blue is the standard treatment for Methemoglobinemia. Failure to respond to this, warrants prompt initiation of alternate treatment strategies like exchange transfusion as well as high dose ascorbic acid administration.

Background

Desmoplastic small round cell tumours are rare, aggressive neoplastic tumours with a predisposition to affect the intra-abdominal cavity.

Hyperthermic intraperitoneal chemotherapy (HIPEC) is a mode of delivering concentrated, heated chemotherapy directly into the abdominal cavity.

Objectives

We describe the first UK case of HIPEC for a desmoplastic small round cell tumour in a child.

Methods

An 8 year old girl (AB) was diagnosed with desmoplastic small round cell tumours after presenting with a ten day history of abdominal discomfort. Initial assessment revealed a large palpable abdominal mass. An ultrasound demonstrated a large heterogenous mass with central cystic necrosis. Two lesions were noted within the liver in addition to an omental deposit. Bone marrow aspirates and biopsies confirmed the diagnosis.

AB had eleven rounds of chemotherapy in total . A follow-up MRI showed a residual pelvic tumour of 3.1cm indenting the superior wall of the bladder.

After MDT discussion the decision was made to proceed with macroscopic resection of tumours followed by cisplatin HIPEC. Intraoperatively multiple intra-abdominal lesions were noted. After resection was completed, cisplatin heated to 41 degrees C was washed over the peritoneum. This was tolerated with noradrenaline used to optimise haemodynamics. Postoperatively she was admitted to PICU for monitoring and observation.

Results

During PICU admission AB remained clinically stable. Active interventions included GCSF, analgesia, electrolyte optimisation and hyperhydration. AB was dischargable from PICU after 4 days.

Conclusion

We described the first UK case of HIPEC being used in paediatrics. This technique could be considered in other cases of intra-abdominal neoplasia with metastases.

Background

Hypoxic-ischemic encephalopathy (HIE) following perinatal asphyxia often leads to brain injury. Hypothermia is the only established beneficial therapy. Near-infrared spectroscopy (NIRS) could be a useful, noninvasive tool to establish prognosis.

Objectives

To evaluate the prognostic value of NIRS in neurodevelopmental outcome at 18-36 months in neonates with HIE and to establish timing and cut-off points of regional cerebral oxygen saturation (rcSO₂) that best relate to prognosis.

Methods

All term neonates with HIE, submitted to hypothermia and NIRS, between 2013-2016, were included. Regarding outcome three groups were considered: normal neurodevelopment (NND), severe disability (SD): death, cerebral palsy, global development delay and/or severe hearing impairment; moderate disability (MD): borderline developmental delay, mild to moderate sensory abnormalities and/or epilepsy.

Results

Twenty-eight neonates were included. Neurodevelopment outcome was normal in 28.6%, 35.7% had MD and 35.7% had SD (3 died). rcSO₂ values tended to increase from 65% to 85% (medians) at 48 hours of life. There was a statistical difference between the groups at 48 hours (NND 71%, MD 78.5%, SD 95%; p=0.005) and after hypothermia (NND 66%, MD 71%, SD 87%; p=0.011). Considering the NND group vs the others, the ROC curve at 48 hours revealed a significant area under de curve (AUC) of 0.872 (p=0.001), with a rcSO₂ cut-off point of 83.5%. After hypothermia, rcSO₂ values below 67.5% (AUC 0.763; p=0.033) predicted NND while values above 91.5% (AUC 0.831; p=0.004) indicated SD.

Conclusion

NIRS accurately predicts neurodevelopmental outcome during therapeutic hypothermia, with higher rcSO₂ values in patients with disability, mainly at 48 hours and after hypothermia.