Outbreak of Burkholderia cepacia bacteraemia in a tertiary care centre due to contaminated ultrasound probe gel.

BACKGROUND: Burkholderia cepacia is an important opportunistic organism in hospitalized and immunocompromised patients, particularly in cystic fibrosis. AIMS: To describe the epidemiological investigation of an outbreak of B. cepacia bacteraemia. METHODS: The study examined 14 patients during their admission to three intensive care units in a tertiary care hospital between January and June 2016. The outbreak involved nine (57%) female and six (43%) male patients. All patients were adults of ages ranging from 19 to 85 years with a median age of 52 years. Patients' medical charts, laboratory cultures, exposures, and central line insertion procedures were reviewed. FINDINGS: B. cepacia was isolated from the blood cultures of 14 patients resulting from contamination of the gel applied to the ultrasound probe used to guide the insertion of a central venous catheter. Molecular pathogen typing using pulsed-field gel electrophoresis showed 95% similarity between the B. cepacia isolates from the blood of these patients and those isolated from the ultrasound gel. CONCLUSION: Ongoing surveillance and prompt investigation of unusual disease outbreaks are vital for identifying sources of contamination of B. cepacia and protecting at-risk patients. Sound epidemiological methods are very important for identifying the source of any hospital infection outbreak.

Congenital para-oesophageal hiatal hernia in infancy.

Congenital para-oesophageal hiatal hernia (PEHH) is a rare problem in infancy, however, it constitutes a clinical entity that mandates surgical repair once the diagnosis is made. In the paediatric age group, acquired PEHH has been described as a major complication in a number of patients who were treated surgically for gastro-oesophageal reflux (GER) by Nissen fundoplication. PEHH is a frequently encountered condition in elderly patients; it accounts for 5% of diaphragmatic hiatal hernias. In both paediatric and adult patients PEHH, whether congenital or acquired in origin, is usually associated with potentially lethal complications such as gastric volvulus, incarceration, and perforation. In clinical practice true PEHH is extremely rare. The term has been expanded to include large gastric hiatal hernias where most of the stomach and the gastro-oesophageal junction are in the chest. Six infants with congenital PEHH are presented, together with an attempt to understand its possible aetiology and a review of its current surgical management.

Nonketotic hyperglycinemia: A life-threatening disorder in Saudi newborns.

Nonketotic hyperglycinemia (NKH) is an autosomal recessive disorder due to a fundamental defect in the glycine cleavage system, which leads to neuronal dysfunction caused by two receptor-mediated mechanisms. It is a life-threatening condition in the neonate. Until now, the disease has not been described from Saudi Arabia. We report on three Saudi newborns (two males and one female) who had NKH. Two of these were siblings (male and female). Following uneventful deliveries, they presented between the first and third day of life with progressive lethargy, poor feeding, recurrent apnea and severe hypotonia. Two newborns had myoclonic seizures, whereas electroencephalogram showed burst-suppression pattern in all of them. The diagnosis was confirmed by high cerebrospinal fluid/plasma glycine ratio (0.2 and 1.08) in two patients (normal < 0.030, whereas a sibling of one of the neonates had a high glycine level. Both siblings died during the second month of life despite therapy with dextromethorphan (an N-methyl-D-asparate [NMDA] receptor antagonist) in one of them. The third day had ketamine (noncompetitive NMDA receptor antagonist) and sodium benzoate (that conjugates with glycine, forming nontoxic hippuric acid). Although his seizures were controlled, he survived with severe neurological sequelae.

Down's syndrome in Saudi Arabia: incidence and cytogenetics.

A study has been carried out in Riyadh to determine the incidence and distribution of Down's syndrome births during a 9-year period from July 1982 to June 1991. Down's syndrome was ascertained in 42 (23 females and 19 males) of 23,261 consecutive babies born alive to Saudi women, giving an incidence of 1 in 554 live births (1.8 per 1,000). A trend towards an increased incidence of Down's syndrome with advanced maternal age or increased maternal parity was found. Cytogenetic studies were performed on 37 cases of which all but 1 were non-disjunction trisomy 21, while the remaining infant had a translocation. This study provides the first step for further epidemiological surveys of Down's syndrome in the Kingdom of Saudi Arabia in order to prepare the ground for an effective antenatal screening programme for chromosomal disorders.

Risk of thrombocytopenia in the infants of mothers with idiopathic thrombocytopenia.

Twenty-nine of 27,662 pregnant women had autoimmune thrombocytopenia at the time of delivery at King Khalid University Hospital over 6 years starting June 1986. Twenty-six had idiopathic thrombocytopenic purpura (ITP), gave birth to 33 infants, of which 22 were by spontaneous vaginal delivery, eight by lower segment cesarean section, and two by forceps. Fourteen (44%) of the 32 living infants had platelets less than 150 x 10(9)/L and four (12.5%) had severe thrombocytopenia (platelets less than 50 x 10(9)/L). The mothers' platelets of less than 50 x 10(9)/L at delivery were found to be predictive of thrombocytopenia in their infants (P < 0.027), compared with mothers' platelet of more than 50 x 10(9)/L. Maternal treatment with prednisone did not seem to have significant effect on infants' platelets (P < 0.89). All infants with severe thrombocytopenia (less than 50 x 10(9)/L) at birth had ultrasound done and were found to be normal. We conclude that: (1) steroid given to pregnant women with ITP does not increase infants' platelet counts, (2) severe thrombocytopenia in the mothers (platelet counts less than 50 x 10(9)/L) is highly predictive of thrombocytopenia in their infants; (3) cesarean section should be limited to the mother with severe thrombocytopenia if fetal scalp platelets are less than 50 x 10(9)/L.

Neonatal Haemophilus influenzae infections.

Thirteen cases of neonatal Haemophilus influenzae (HI) infections were identified in Al-Baha Region, Saudi Arabia during 1 year: seven male, six female. The mean weight and age were 36.0 weeks (28-44) and 2.5 kg (1.1-4.5) respectively. All babies were delivered outside the hospital, five at home and eight at primary care centres. HI was isolated from the lungs in 12 (92%), eyes in seven (54%), and from the blood in four (31%). None of the neonates had meningitis. HI resistant to ampicillin and cefuroxime were identified in one case (7.7%) each. All survived and were discharged home in good condition. Our findings suggest that HI is becoming more recognised as a cause of neonatal infections especially in premature babies. The current starting regimen of antibiotics for suspected sepsis in neonates (ampicillin and gentamicin) adequately cover for HI sepsis and need not be changed unless lack of response to treatment is documented.

Acute rheumatic fever in Saudi Arabia: mild pattern of initial attack.

Fifty-one children with the initial attack of acute rheumatic fever (ARF) were studied prospectively to verify the sociodemographic and clinical profile and to compare results with those from other countries. Most children belonged to large families who lived in an urban setting with ready access to medical care. Unlike reports from many developing countries, the clinical manifestations in this study paralleled data from the West and included arthritis in 76% of the cases, carditis in 43%, and chorea in 8%. Among the 22 cases with carditis, 18 had mitral regurgitation, three developed combined mitral and aortic regurgitation, and one had aortic regurgitation. This study demonstrates the mild nature of ARF in Saudi Arabia and supports the concept that climate and geography appear to bear little relationship to the incidence and severity of ARF.

Osteopetrosis: brain ultrasound and computed tomography findings.

A case of osteopetrosis presenting with rare features of dysmorphism with proptosis due to hypoplasia of the orbits and the temporal bone is described. The case also had calcifications in the periventricular regions, the falx cerebri and the corpora colliculi. These features and sonographic findings of osteopetrosis have not been reported previously. The sensitivity and specificity of imaging modalities in the diagnosis of intracranial calcifications is discussed.

Comparison of continuous versus intermittent furosemide administration in postoperative pediatric cardiac patients.

OBJECTIVE: To compare the effects of furosemide administered by intermittent iv infusion vs. continuous iv infusion on urine output, hemodynamic variables, and serum electrolyte concentrations. DESIGN: Prospective, randomized trial. SETTING: Pediatric ICU. PATIENTS: Postoperative pediatric cardiac patients. INTERVENTIONS: Patients were assigned to either the continuous iv infusion or the intermittent infusion groups. The intermittent group received 1 mg/kg iv of furosemide every 4 hrs to be increased by 0.25 mg/kg iv every 4 hrs to a maximum of 1.5 mg/kg iv if the urine output was less than 1 mL/kg.hr. The continuous infusion group received an initial furosemide dose of 0.1 mg/kg iv (minimum 1 mg) followed by an iv infusion rate of 0.1 mg/kg.hr of furosemide to be doubled every 2 hrs to a maximum of 0.4 mg/kg.hr if the urine output was less than 1 mL/kg.hr. MEASUREMENTS AND MAIN RESULTS: Demographic variables, fluids, electrolyte and inotropic requirements were the same in both groups. A significantly (p = .045) lower daily dose of furosemide (4.90 +/- 1.78 vs. 6.23 +/- 0.62 mg/kg.day) in the continuous iv infusion group produced the same 24-hr urine volume as that of the intermittent group. There was more variability in urine output in the intermittent group as well as more urinary losses of sodium (0.29 +/- 0.15 vs. 0.20 +/- 0.06 mmol/kg.day, p = .0007) and chloride (0.40 +/- 0.20 vs. 0.30 +/- 0.12 mmol/kg.day, p = .045). CONCLUSION: Furosemide administered by continuous iv infusion is advantageous in the post-operative pediatric patient because of a more controlled and predictable urine output with less drug requirement and less urinary loss in sodium and chloride.