New effective treatment regimen for children infected with a double-resistant Helicobacter pylori strain.

BACKGROUND: The increasing number of pediatric patients infected with multiresistant Helicobacter pylori strains calls for evaluation of treatment regimens. Second-line antibiotics such as tetracycline or quinolones are not licensed for children. Because in vivo resistance to metronidazole may be overcome in vivo by a high dose and prolonged intake, we evaluated the eradication rate and side effects of a high-dose triple therapy in pediatric patients with culture-proven double resistance. PATIENTS AND METHODS: In this open multicentre trial, 62 children (<18 years, body weight >15 kg) infected with an H pylori strain resistant to metronidazole and clarithromycin were treated according to body weight classes with amoxicillin (∼ 75 mg/kg/day), metronidazole (∼ 25 mg/kg/day) and esomeprazole (∼ 1.5 mg/kg/day) for 2 weeks. Adherence and adverse events were assessed by a 2-week diary and telephone interviews at days 7 and 14 of treatment. Primary outcome was a negative C-urea breath test after 6 weeks. RESULTS: Of 62 patients, 5 were lost to follow-up, 12 were nonadherent, and 45 treated per protocol. Eradication rates were 66% (41/62) [confidence interval 54-78] (intention to treat) and 73% (33/45) [confidence interval 60-86] (per protocol). Success of treatment was not related to dose per kilogram body weight. Mild to moderate adverse events were reported by 21 patients, including nausea (10.8%), diarrhoea (8.9%), vomiting (7.1%), abdominal pain (5.4%), and headache (3.6%), and led to discontinuation in 1 child. CONCLUSION: High-dose amoxicillin, metronidazole, and esomeprazole for 2 weeks is a good treatment option in children infected with a double resistant H pylori strain.

[High percentage of clarithromycin and metronidazole resistance in Helicobacter pylori clinical isolates obtained from Spanish children].

OBJECTIVE: To determine the primary and secondary resistance to several antimicrobial agents in Spanish Helicobacter pylori clinical isolates obtained from paediatric patients from January 2002 to June 2006. METHODS: Samples were collected from gastric biopsies of symptomatic paediatric patients and H. pylori cultured according to standard microbiological procedures. Resistance was determined by E-test. Strains were considered resistant if minimal inhibitory concentration (MIC) > or = 2 mg/l for amoxycillin, > or = 4 mg/l for tetracycline, > or = 8 mg/l for metronidazole, > or = 1 mg/l for clarithromycin, MIC > or = 4 mg/l for ciprofloxacin, MIC > or = 32 mg/l for rifampicin and intermediate if MIC = 0.5 mg/l for clarithromycin, and MIC = 2 mg/l for ciprofloxacin. RESULTS: A total of 101 patients were included: 38 males and 63 females (sex ratio M/F: 0.6). Average age was 10 years (range: 4-18 years). All strains were susceptible to amoxycillin, tetracycline and rifampicin, 35.7% were resistant to metronidazole, 54.6% to clarithromycin and 1.8% to ciprofloxacin. 2.0% were intermediate to clarithromycin and 1.8% to ciprofloxacin. Double resistance to metronidazole and clarithromycin rated at 17.2%. Thirty-five patients (34.7%) had a history of treatment failure, and were considered as secondary H. pylori. Primary resistance rates to metronidazole and clarithromycin were 32.8% and 49.2%, respectively, and secondary resistance rates were 41.2% and 70.6%, respectively. CONCLUSIONS: Resistance to clarithromycin (56.6%) was higher than to metronidazole (35.7%) in the H. pylori strains studied. Clarithromycin resistance was very high even in strains from paediatric patients not previously treated for H. pylori infection.

High percentage of clarithromycin and metronidazole resistance in Helicobacter pylori clinical isolates obtained from Spanish children / Alto porcentaje de resistencia a claritromicina y metronidazol en aislamientos clínicos de Helicobacter pylori procedentes de pacientes pediátricos

Introducción. Determinar la resistencia primaria y secundaria a varios agentes antibióticos en aislamientos clínicos de Helicobacter pylori procedentes de pacientes pediátricos desde enero de 2002 a junio de 2006. Métodos. Las muestras fueron biopsias gástricas procedentes de pacientes pediátricos sintomáticos. H. pylori fue cultivado acorde con los estándares de los procedimientos microbiológicos. La resistencia a antibióticos fue determinada mediante E-test. Los aislamientos fueron considerados resistentes si la CMI ≥ 2 mg/l para amoxicilina, ≥ 4 mg/l para tetraciclina, ≥ 8 mg/l para metronidazol, ≥ 1 mg/l para claritromicina, ≥ 4 mg/l para ciprofloxacino y ≥ 32 mg/l para rifampicina, y fue considerado intermedio si la concentración mínima inhibitoria (CMI) = 0,5 mg/l para claritromicina, y CMI = 2 mg/l para ciprofloxacino. Resultados. De los 101 pacientes incluidos en el estudio: 38 fueron hombres y 63 mujeres. La media de edad fue de 10 años. Todas las cepas fueron sensibles a amoxicilina, tetraciclina y rifampicina. El 35,7% de los aislamientos fueron resistentes a metronidazol, el 54,6% fue- ron resistentes a claritromicina y el 1,8% a ciprofloxacino. El 17,2% de los aislamientos tuvieron doble resistencia a metronidazol y claritromicina. Treinta y cinco pacientes (34,7%) tuvieron un fallo en el tratamiento frente a H. pylori, previamente. La resistencia primaria a metronidazol y claritromicina fue del 32,8 y 49,2%, respectivamente, y la resistencia secundaria fue de un 41,2 y 70,6%, respectivamente. Conclusión. La resistencia a claritromicina (56,6%) fue más alta que a metronidazol (35,7%) en los aislamientos clínicos de H. pylori estudiados. La resistencia a claritromicina fue alta incluso en los pacientes que no habían tenido tratamientos previos frente a H. pylori (AU)

Objective. To determine the primary and secondary resistance to several antimicrobial agents in Spanish Helicobacter pylori clinical isolates obtained from paediatric patients from January 2002 to June 2006. Methods. Samples were collected from gastric biopsies of symptomatic paediatric patients and H. pylori cultured according to standard microbiological procedures. Resistance was determined by E-test. Strains were considered resistant if minimal inhibitory concentration (MIC) ≥ 2 mg/l for amoxycillin, ≥ 4 mg/l for tetracycline, ≥ 8 mg/l for metronidazole, ≥ 1 mg/l for clarithromycin, MIC ≥ 4 mg/l for ciprofloxacin, MIC ≥ 32 mg/l for rifampicin and intermediate if MIC = 0.5 mg/l for clarithromycin, and MIC = 2 mg/l for ciprofloxacin. Results. A total of 101 patients were included: 38 males and 63 females (sex ratio M/F: 0.6). Average age was 10 years (range: 4-18 years). All strains were susceptible to amoxycillin, tetracycline and rifampicin, 35.7% were resistant to metronidazole, 54.6% to clarithromycin and 1.8% to ciprofloxacin. 2.0% were intermediate to clarithromycin and 1.8% to ciprofloxacin. Double resistance to metronidazole and clarithromycin rated at 17.2%. Thirty-five patients (34.7%) had a history of treatment failure, and were considered as secondary H. pylori. Primary resistance rates to metronidazole and clarithromycin were 32.8% and 49.2%, respectively, and secondary resistance rates were 41.2% and 70.6%, respectively. Conclusions. Resistance to clarithromycin (56.6%) was higher than to metronidazole (35.7%) in the H. pylori strains studied. Clarithromycin resistance was very high even in strains from paediatric patients not previously treated for H. pylori infection (AU)

Hematemesis secundaria a alergia a proteínas de leche de vaca / Hematemesis induced by cow milk allergy

Introducción: La hematemesis es un proceso pocofrecuente en periodo de lactante, con diferentesetiologías como esofagitis por reflujo, ingesta deAINES, etc. La hematemesis por gastritis alérgica aproteínas de la leche de vaca (GAPLV) es una entidadrara cuya frecuencia va en aumento.Material y métodos: Análisis retrospectivo de lascaracterísticas clínicas, hallazgos endoscópicos yevolución de los lactantes diagnosticados deGAPLV que debutaron con hematemesis.Resultados: Se estudian 7 pacientes (4 niñas),edad media 4,2 meses (2-6). 6 se diagnosticaron enel periodo oct/04-nov/05. Todos referían historia devómitos previa al diagnóstico. El tiempo medio entrela introducción de la fórmula de inicio y el episodiode hematemesis fue de 9 semanas (4-15). Al diagnóstico5 pacientes tenían un peso inferior al paciente3. Se realizó endoscopia digestiva en todoslos niños observándose placas eritematosas en lamucosa gástrica en 5, con erosiones y petequias enlos otros 2. El examen histológico de la mucosamostró infiltrado inflamatorio mixto con aumento delnúmero de eosinófilos. El número de eosinófilos fueinferior a 20 por campo de gran aumento. El estudiode anticuerpos específicos frente a proteínas de lechede vaca (PLV) fue negativo en cinco y en dos seidentificó una sensibilización frente a caseína que senegativizó en la evolución. Ningún paciente presentabaeosinofilia periférica. La respuesta clínica a laexclusión de PLV y realimentación con hidrolizadoextenso de proteínas fue excelente, desapareciendola clínica y normalizándose la curva ponderal.Comentarios: En el diagnóstico diferencial de lahematemesis en el lactante debe valorarse la alergiaa proteínas de leche de vaca. La ausencia deespecificidad de las pruebas diagnósticas séricas yde los hallazgos endoscópicos hace que el diagnósticosea fundamentalmente clínico, con desapariciónde los síntomas tras la exclusión de las proteínas dela leche de vaca de la dieta

Background: Hematemesis is an uncommon entityin infant present several etiologies such as refluxesophagitis, gastritis due to NSAIDs,etc. Hematemesisinduced by cow milk allergy is rare, but increasingin the last years.Material and methods: Restrospectively clinicalcharts analysis endoscopic findings, histology andevolution of hematemesis due to cow’s milkallergy.Results: Seven infants were analized (4 girls). Sixof them were between October 2004 and November2005. The other one in 2000. All patients refered vomitingprevious hematemesis. Time after startingcow milk formulas and bloody vomiting was 9 weeks(4-15). At the time of diagnosis, five patients had lowweight (< 3th percentile). Upper gastrointestinal endoscopywas performed in all patiens showing areasof erythema in the gastric mucosa with erosion intwo infants. Histopathologic examination of biopsyspecimen revealed mixed inflammatory infiltrate withnumerous eosinophils. The number was < 20 perhigh-power field. Plasma measurement of cow milkspecific IgE was negative except in two children withsensibilitation to caseína, that disappear in evolution.Eosinophilia in hemogram was not observed.After diagnosis cow milk protein formula was excluded,replacing it with hidrolized formula. The result was total remission of symptoms and nutricional recovery.Comments: When realizing hematemesis differentialdiagnostic to infants, cow milk allergy must beconsidered. Laboratory test and endoscopic imagesare inespecific so the diagnosis relies on clinicalsuspicion and the cessation of symptoms after withdrawalof cow milk formula

Prospective multicentre study on antibiotic resistance of Helicobacter pylori strains obtained from children living in Europe.

AIM: To prospectively assess the antibacterial resistance rate in Helicobacter pylori strains obtained from symptomatic children in Europe. METHODS: During a 4-year period, 17 paediatric centres from 14 European countries reported prospectively on patients infected with H pylori, for whom antibiotic susceptibility was tested. RESULTS: A total of 1233 patients were reported from Northern (3%), Western (70%), Eastern (9%) and Southern Europe (18%); 41% originated from outside Europe as indicated by mother's birth-country; 13% were <6 years of age, 43% 6-11 years of age and 44% >11 years of age. Testing was carried out before the first treatment (group A, n = 1037), and after treatment failure (group B, n = 196). Overall resistance to clarithromycin was detected in 24% (mean, A: 20%, B: 42%). The primary clarithromycin resistance rate was higher in boys (odds ratio (OR) 1.58; 1.12 to 2.24, p = 0.01), in children <6 years compared with >12 years (OR 1.82, 1.10 to 3.03, p = 0.020) and in patients living in Southern Europe compared with those living in Northern Europe (OR 2.25; 1.52 to 3.30, p<0.001). Overall resistance rate to metronidazole was 25% (A: 23%, B: 35%) and higher in children born outside Europe (A: adjusted. OR 2.42, 95% CI: 1.61 to 3.66, p<0.001). Resistance to both antibiotics occurred in 6.9% (A: 5.3%, B: 15.3%). Resistance to amoxicillin was exceptional (0.6%). Children with peptic ulcer disease (80/1180, 6.8%) were older than patients without ulcer (p = 0.001). CONCLUSION: The primary resistance rate of H pylori strains obtained from unselected children in Europe is high. The use of antibiotics for other indications seems to be the major risk factor for development of primary resistance.

[Celiac disease and Bud-Chiari syndrome: an uncommon association]. / Enfermedad celíaca y síndrome de Budd-Chiari: una asociación infrecuente.

Celiac disease can present great clinical heterogeneity. Its association with a series of intestinal and non-intestinal diseases, whether immunologically mediated or otherwise, presents a higher than normal frequency. We present a patient with celiac disease and Budd-Chiari syndrome of unknown cause. This association has previously been described only in isolated cases in northern Africa. The appearance of this case in Spain reveals that the coexistence of both processes in a single patient is unlikely to be due to environmental or geographical factors.

Covariate effects on the apparent clearance of tacrolimus in paediatric liver transplant patients undergoing conversion therapy.

OBJECTIVE: To analyse the influence of covariates on the apparent clearance (CL) of tacrolimus in paediatric liver transplant recipients being converted from cyclosporin to tacrolimus. DESIGN: Retrospective modelling study. PATIENTS AND PARTICIPANTS: 18 children, 13 girls and 5 boys, aged 4 months to 16 years (median 9.1 years) who required conversion to tacrolimus because of acute or chronic rejection or cyclosporin toxicity. METHODS: 287 whole-blood tacrolimus concentrations from therapeutic drug monitoring were used to build a nonlinear mixed-effects population model (NONMEM program) for the apparent clearance of tacrolimus. Variables considered were age, total bodyweight (TBW), body surface area (BSA), time after initiation of treatment (T), gender, haematocrit (Hct), albumin (Alb), aspartate aminotransferase (AST), alanine aminotransferase (ALT), gamma-glutamyl transpeptidase (gammaGT), alkaline phosphatase (ALP), bilirubin (BIL), creatinine clearance (CL(CR)) and dosage of concomitant corticosteroids (EST). RESULTS: TBW, T, BIL and ALT were the covariates that displayed a significant influence on CL according to the final regression model: CL (L/h) = 10.4(TBW/70)3/4 x e(-0.00032 T) x e(-0.057 BIL) x (1 - 0.079 ALT). With this model, the estimates of the coefficients of variation were 24.3% and 29.5% for interpatient variability in CL and residual variability, respectively. CONCLUSIONS: The proposed model for tacrolimus CL can be applied for a priori dosage calculations, although the results should be used with caution because of the unexplained variability in the CL. We therefore recommended close monitoring of tacrolimus whole blood concentrations, especially within the first months of treatment. The best use of the model would be its application in dosage adjustment based on therapeutic drug monitoring and the Bayesian approach.

Prevalence of CagA and VacA antibodies in children with Helicobacter pylori-associated peptic ulcer compared to prevalence in pediatric patients with active or nonactive chronic gastritis.

VacA and CagA serological responses were detected in pediatric patients: 44 and 56%, respectively, in peptic ulcer (PU) patients, 33.3 and 44.4% in active chronic gastritis (ACG) patients, and 23.2 and 39.2% in non-ACG patients. Higher seroprevalence to CagA+VacA and to CagA+VacA+35-kDa antigen was found among PU patients. However, a low level of sensitivity and specificity was found for indirect detection of PU patients.

[Diagnosis and treatment of hepatic artery thrombosis after liver transplantation]. / Diagnosi e trattamento della trombosi dell'arteria epatica dopo trapianto di fegato.

The aim of this study was to examine the clinical presentation and time of hepatic artery thrombosis (HAT) after orthotopic liver transplantation (OLT), stressing the role of imaging modalities. Therapeutic options are described, such as retransplantation (Re-OLT), hepatic resections and revascularization procedures, focusing on complications and outcome in a consecutive series of 687 OLT. Over the period from 1986 to 1999, 687 OLT were carried out in 601 patients, 592 of whom were adults and 95 pediatric subjects. Of these operations 601 were primary OLT and 86 Re-OLT (71 I Re-OLT, 14 II Re-OLT and 1 III Re-OLT). In this retrospective study, we reviewed rejection episodes, time of HAT (early or late), possible cause of HAT, day of suspected diagnosis of HAT and day of confirmation of diagnosis. Clinical presentation, management, complications, outcome, survival rates and the need for Re-OLT were also recorded. The incidence of HAT was 2.47% (17/687). Early HAT (n = 9, < 30 days) was diagnosed 15.6 days after OLT (range: 3-25 days), whereas late HAT (n = 8, > 30 days) occurred 295.1 days after OLT (range: 38-1830 days). In two asymptomatic patients (2/17: 11.7%), HAT was discovered incidentally. Most of the patients (11/17: 64.7%) presented with increased liver function test values and fever. Relapsing bacteremia occurred in 7/17 cases (41.1%), whereas a biliary stricture and biliary leak were diagnosed in 3/17 (17.6%) and in 1/17 patients (5.8%), respectively. Fulminant hepatic failure was the clinical presentation in 2/17 cases (11.7%). In one case the clinical presentation was acute and chronic rejection (1/17: 5.8%). Intrahepatic abscesses were diagnosed in one case (1/17: 5.8%), as well as an intrahepatic haemorrhage (1/17: 5.8%). Doppler ultrasound (DUS) correctly revealed HAT in 9 of the 17 patients (52.9% sensitivity). In 8 of the 9 patients (88.8%) in whom HAT was diagnosed by DUS, angiography was also performed to confirm the diagnosis. Overall, angiography detected HAT in 14/17 patients (82.3% sensitivity). HAT management consisted of immediate Re-OLT in 6 patients 6.8 days (range: 3-12 days) after diagnosis. Delayed Re-OLT was performed in 6 patients 529.1 days (range: 68-1920 days) after diagnosis. The overall retransplantation rate was 70.5% (12/17). Two patients died despite undergoing intraarterial urokinase treatment. Three grafts were salvaged, but suffered biliary stricture due to ischemic cholangitis and underwent hepatico-jejunostomy. A II Re-OLT was carried out in 4 of 12 patients (33.3%). The overall mortality rate was 41.1% (7/17). One-year and 3-year overall survival rates were 58.8% (10/17) and 47.0% (8/17), respectively. Both 5- and 10-year overall survival rates were 11.7% (2/17). Although the results of OLT have improved dramatically over the past few years, HAT is still associated with substantial morbidity, a high incidence of graft failure and high mortality rates. The use of DUS to screen for HAT has permitted earlier diagnosis, but early angiographic evaluation of the hepatic arteries is still needed for accurate diagnosis of HAT and remains the gold standard. Retransplantation is the definitive solution for HAT in the majority of cases, though it is essentially the patient's clinical condition that dictates the form of management.

[A patient with visceromegaly: risk factor for performing percutaneous endoscopic gastrostomy. A clinical case]. / Paciente con visceromegalia: situación de riesgo en la realización de una gastrostomía endoscópica percutánea. Caso clinico.

Percutaneous endoscopic gastrostomy is an easy and safe technique to provide an enteral access for patients needing long-term enteral nutrition. Minor complications may occur in 9% to 13% of patients. Life-threatening complications appear in 1-3% of cases. Perforation of a hollow viscus leading to peritonitis is a rare condition; hepatic perforation after placing a percutaneous endoscopic gastrostomy (PEG) has not been reported previously. In patients with massive visceromegaly an abdominal ultrasound may help in localizing the place of punction avoiding surrounding organs.

Monitoring of tacrolimus as rescue therapy in pediatric liver transplantation.

The introduction of tacrolimus as rescue therapy represents a significant advance in the prevention of late graft failure and second liver transplantation. The authors report the blood level monitoring of tacrolimus as a rescue therapy in 21 children who underwent liver transplantation, and they report the dose-concentration relationship in the presence or absence of hepatitis C virus (HCV) in these patients. This was a retrospective study conducted from May 1993 to January 1997. Indication for the conversion from cyclosporine (CsA) to tacrolimus were acute rejection (62%), chronic rejection (33%), and CsA toxicity (5%). Mean daily dose in the first month was 0.32 mg/kg, whereas at the end of the follow-up period it was 0.14 mg/kg. Tacrolimus mean whole blood concentration levels were between 7.1 ng/ml and 9.4 ng/ml, without significant differences over time. Mean daily doses in HCV+ and HCV- patients were 0.08 and 0.24 mg/kg, respectively (p < 0.01), and mean concentrations were 8.3 and 8.4 ng/ml (NS). HCV+ children required a mean dose three times lower than the dose used in HCV- children to obtain the same tacrolimus trough blood level. Therefore, doses in HCV+ children must be decreased to achieve levels within the therapeutic range.

Treatment of esophageal varices by endoscopic ligation in children.

Endoscopic variceal ligation (EVL) is an alternative technique to endoscopic variceal sclerotherapy (EVS) to treat esophageal varices. This method consists of mechanical ligature and thrombosis of varices using elastic rubber rings. During an 11-month period, nine pediatric patients with esophageal varices secondary to portal hypertension were treated by EVL. Extrahepatic portal vein obstruction was the cause of portal hypertension in 5 patients and in 4 cases the cause was intrahepatic disease. The average age of the patients was 8 years (range: 2-15). Five patients had bled from esophageal varices previously. Two were actively bleeding at the time of the procedure. Endoligature was performed prophylactically in four patients. Fifty varix ligations were performed in 26 separate sessions. In children older than ten years, EVL was performed under intravenous sedation. Two mild rebleeds have occurred in this group and responded to repeated ligature. Varices were reduced in grade in all patients. Six patients achieved variceal eradication. Recurrence of varices was not encountered. No major complications occurred and there were no treatment failures. These early results suggest that endoscopic ligation is a safe and effective method for the control of esophageal varices in children.

[Wilson's disease. A retrospective analysis of 12 cases]. / Enfermedad de Wilson. Análisis retrospectivo de doce casos.

We reviewed retrospectively 12 patients with Wilson's disease diagnosed during a 16-year period (1974-1989). The prevalence rate was 0.6 per 100,000 individuals. Clinical onset was hepatic (50%) or neurologic (50%), but at diagnosis (6.4 years later) 67% of patients showed several clinical manifestations: hepatic, neurologic, renal and haematologic. Among the essential diagnostic indices we find false negative results for Kayser-Fleischer ring (25%), serum ceruloplasmin (8%) and total serum copper (34%). Ten patients were treated with penicillamine. This drug was effective and well tolerated, although one patient (10%) developed membranous nephritis and required to change successively to BAL and trien. In a 61 months follow-up 5 patients (42%) died from severe liver failure. Patients with poor prognosis had a diagnostic delay and a liver failure degree significantly greater than patients with good prognosis. Our results suggest the following conclusions: a) in Spain the prevalence rate of Wilson's disease is near the lower reported rate; b) the early diagnosis of Wilson's disease is rare; c) diagnosis should be made only when several essential indices are positive; d) early hepatic transplantation showed carried out in patients with acute or chronic severe liver failure.

Arterial reperfusion in human orthotopic liver transplantation by anastomosis of the celiac trunk to the right or left hepatic artery of the recipient.

Fifteen orthotopic liver transplants were performed from 23 April 1986 to June 1987 in 14 patients (age range 3-56 years). In 12 transplants, extracorporeal bypass was used. The installation was effected by suprahepatic and infrahepatic cava-caval and portaportal anastomoses. Arterial anastomosis was realized after reperfusing the graft through the vena porta. In 13 transplants the donor celiac trunk was anastomosed to the receptor's right hepatic artery. In one ten year-old girl, the donor celiac trunk was anastomosed to the left hepatic artery. In one patient who underwent retransplantation for rejection, the donor organ had two separate hepatic arteries and the right and left hepatic arteries were respectively anastomosed to the right and primitive hepatic arteries of the receptor. All patients were followed up periodically by Doppler echography and trimethyl-Br IDA 99 mTc scan, which, consistently confirmed the permeability of the anastomosis and dependent vessels, and the good perfusion and function of the grafts, which were free of infarcted areas. Three patients died at 30 and 31 days and postmortem studies demonstrated vascular permeability. Angiography was performed in one patient, evidencing a good vascular caliber in the hepatic arteriography.

[Functional evaluation of pancreas cystic fibrosis. Correlations and value of the echocardiogram versus other methods (author's transl)]. / Evaluación funcional de la fibrosis quística de páncreas. Correlaciones y valor del ecocardiograma frente a otros métodos.

The results of a correlation of various parameters used in the functional evaluation of 12 cases of pancreas cystic fibrosis (PCF) are reported. Three clinical scores were measured by four separate observers and average score was taken. Electrocardiogram, phono, vectorcardiogram, pH, blood gases, radiology, spirometry, erythropoietin, blood counts and echocardiogram were also recorded. Each of these were correlated. In the echocardiogram (EC), right and left systolic time intervals, wall and cavity of the right ventricle and the left ventricular function were measured. Some correlations were found to have a statistical significance: the data attained from the EC, clinical score and the spirometry. However, blood count, blood gases, erythropoietin and pH were found to have no statistical significance. The EC was found to be the most accurate and sensitive method of recognising the initial stages of pulmonary hypertension in PCF; detecting its presence at as early an age as six years (on average). These results present a strong argument for the use of the EC as a vital part of each exam of PCF.