Isolated Congenital Palatal Fistula Without Submucous Cleft Palate

Congenital fistula of the palate is a rare deformity. It has been generally associated with cleft palate. Treatment of cleft palate is surgical intervention. We present a child with congenital fistula of palate that was not associated with submucous cleft and closed spontaneously at 18 months

EFFECT OF ADENOTONSILLECTOMY ON THE RIGHT VENTRICULAR DIASTOLIC FUNCTIONS IN CHILDREN WITH ADENOTONSILLAR HYPERTROPHY

WOS: 000435637700015The present study aimed to investigate the right ventricular function in children with obstructive adenotonsillar hypertrophy. For this purpose, right ventricular diastolic functions were evaluated via echocardiography before adenotonsillectomy and after a minimum of 6 months following surgery. Children with adenotonsillar hypertrophy who were admitted to our clinic with the complaints of snoring, mouth breathing, and/or witnessed apnea were included. Pre-operatively, a complete blood cell count, routine biochemical blood tests, chest radiography, electrocardiography, and tissue Doppler echocardiography were performed. Electrocardiography and tissue Doppler echocardiography were repeated on the follow-up visits of the patients a er a minimum of 6 months following adenotonsillectomy. Evaluation of the patients in the post-operative 6th month revealed no snoring or apnea in any of the patients. Although significant changes were detected in certain echocardiographic parameters a er the surgery, right ventricular morphological abnormalities were not detected. Also, echocardiographic measurements of le ventricular dimensions were within the normal limits. Nevertheless, the ratio of peak early to late tissue motion velocity of the tricuspid valve as determined by tissue Doppler imaging was higher in the post-operative period than in the pre-operative period. This might be attributed to the relative improvement in diastolic functions

A CASE OF DILATED CARDIOMYOPATHY DUE TO HYPOCALCEMIA IN NEWBORN

Dilation of one or both ventricles and systolic dysfunction occur dilated cardiomyopathy. Hypocalcemia is a rare cause of reversible heart failure and dilated cardiomyopathy. A seventeen-day old male infant was brought to our clinic with complaints of poor feeding and convulsion. On physical examination, the patient had worse general appearance and was apathetic. Examination of cardiovascular system revealed 2/6 diastolic murmur and signs of congestive heart failure. The value of serum calcium was 4.8 mg / dL. Cerebrospinal fluid examination was unremarkable. Echocardiographic evaluation showed enlarged heart chambers and globally hypokinetic left ventricular wall. Left ventricular ejection fraction was 39% and shortening fraction was 17%. After the treatment of calcium gluconate, a clinically dramatic improvement was observed. On the second day of hospitalization, the echocardiographic examination was normal. Therefore, we suggest that sermon calcium level in newborn with cardiomyopathy should be measured

Repeated Courses of Oral Ibuprofen in Premature Infants with Patent Ductus Arteriosus: Efficacy and Safety

BACKGROUND: There are limited data about the results of repeated oral ibuprofen (OIBU) treatment. This study aimed to describe patent ductus arteriosus (PDA) closure rates and adverse events after repeated courses of OIBU in premature infants with PDA. METHODS: Preterm infants with hemodynamically significant (hs)PDA were enrolled in the study. If the first course of OIBU treatment failed, a second and, if required, third course was administered. RESULTS: A total of 100 patients received OIBU. In six patients, treatment could not be completed due to death (n=3) and side effects (n=3). In three patients, adverse effects related to OIBU (thrombocytopenia and impairment of renal function) developed during the first course. During the second and third courses, no new adverse event occurred. After all courses, the PDA closure rate was determined as 88%. The rate was 71% after the first course, 40% after the second course, and 35% after the third course. Although the second course resulted in a significant increase in the closure rate (p0.05). The mean postnatal age at the start of the first dose of OIBU was not significantly different among the responders and non-responders to the first course (p>0.05). Clinical characteristics did not affect the closure rate significantly. The number of courses did not have a significant effect on death, when gestational age and birth weight were used as covariates [p=0.867, Exp(B)=0.901, 95% confidence interval=0.264-3.1]. CONCLUSION: A second course of OIBU seems effective and safe for use in preterm infants with hsPDA. Although a third course of OIBU results in PDA closure in some additional patients, the difference is not significant. Thus, surgical ligation should be considered after the second course, especially in patients with signs of severe heart failure

Clinical characteristics of neonates With VACTERL association

Background: The VACTERL association (VA) is the non-random co-occurrence of vertebral anomalies, anal atresia, cardiovascular malformations, tracheoesophageal fistula and/or esophageal atresia, renal anomalies, and/or limb anomalies, and is referred to by the first letters of its components. Studies investigating the clinical characteristics of VA patients and probing of the observed current six component types are limited, and none of them is focused on neonates. We investigated the clinical characteristics of our patients diagnosed as having VA in the newborn period. Methods: We retrospectively reviewed the neonates whose final diagnosis was VACTERL association. Presence of at least three components of previously reported six anomalies was accepted as VACTERL association. Sex, birthweight, gestational age, postnatal age, anomalies of the systems that are included in VA, and the other features were recorded. Results: There was a male predominance (14/11) of 28 patients; and there were three patients with ambiguous genitalia. The most common observed VACTERL component was vertebral anomalies (n= 26), followed by anal atresia (n= 19), tracheoesophageal fistula/esophageal atresia (n= 17), renal anomalies (n= 15), limb anomalies (n= 15) and cardiac anomalies (n= 14). The most frequent combination was VCTL (n= 4). Fifteen (57%) patients had non-VACTERL anomalies and the most frequent of these was ambiguous genitalia (n= 3). Conclusion: VA patients may have different clinical characteristics in different populations, and clinicians may miss some component features if the patients are evaluated after the neonatal period

Dilated cardiomyopathy secondary to rickets-related hypocalcaemia: eight case reports and a review of the literature

Introduction: Dilated cardiomyopathy is usually idiopathic and may arise secondary to infections or metabolic or genetic causes. Another rare cause is hypocalcaemia. Owing to the fact that calcium plays an essential role in excitation and contraction of myocardial muscle, myocardial contractility may decline in patients with hypocalcaemia. Materials and Methods: Patients with symptoms of congestive heart failure and rickets-related hypocalcaemia were assessed clinically and by echocardiography in a paediatric cardiology clinic. Echocardiography was performed for all patients. Rickets was diagnosed according to the clinical, laboratory, and radiologic findings. Maternal lifestyle and living conditions were investigated, and the maternal 25-OH vitamin D-3 blood level was measured. Results: We evaluated eight patients who developed heart failure as a result of severe hypocalcaemia associated with rickets between August, 1999 and June, 2012. The age distribution of the patients was 3-12 months. Laboratory results were consistent with advanced-stage rickets. Severe hypocalcaemia was detected in all patients. The maternal 25-OH vitamin D-3 levels were low. Echocardiography revealed increased pre-treatment left ventricle end-systolic and end-diastolic diameters for age and reduced ejection fraction and fractional shortening. After clinical improvement, the patients were discharged. Conclusions: Severe hypocalcaemia associated with rickets must always be kept in mind among the causes of dilated cardiomyopathy and impaired cardiac function in infants. If diagnosed and treated in time, dilated cardiomyopathy and severe heart failure related to rickets respond well