Clinical, virological and ecocardiographic study in children with dilated cardiomyopathy

Fifty consecutive patients diagnosed as having dialted cardiomyopathy [DCM] by echocardiography [30 males, ages from 5 months to 4 years] were studied. Thirty age and sex matched normal subjects were selected as a control group. All cases and controls were subjected to detailed history taking and clinical examination. Investigations included chest X-ray [CXR], standard electrocardiogram [ECG] and detailed echocardiographic examination. Laboratory investigations included: Complete blood count [CBC], erythrocyte sedimentation rate [ESR], serum C-reactive protein [CRP], serum creatinine phosphokinase [CPK], lactic dehydrogenase [LDH] and immunological studies for the detection of enteroviruses and adenovirus specific IgG and IgM antibodies by ELISA technique. Recurrent respiratory infections and dyspnea were the most frequent clinical symptoms in cases with DCM. Tachypnea and hepatomegaly were the most frequent clinical signs, while displaced cardiac apex and muffled first heart sound were the most frequent cardiac signs. Apical pansystolic murmur was asuscultated in only 30% of cases while mitral regurge was detected by echocardiography in 100% of cases [P<0.001]. Increased cardio-throracic [C/T] ratio was found in all case with DCM with mean value of 68.82%. The percentage frequencies of patients with DCM and increased ESR, CRP or LDH were significantly higher than those with DCM and normal ESR, CRP or LDH [p< 0.001]. No significant difference could be detected between the percentage frequencies of patients with increased and those with normal CPK level. Control cases showed normal ESR, CRP, LDH and CPK levels. The mean pulmonary [Pul] artery diameter indexed to the root of body surface area, the mean indexed diameter of the root of the ascending aorta [Ao], left atrial diameter, left ventricular end diastolic and end systolic diameters, the means E-Point Septal Separation [EPSS] were found to be significantly higher in cases with DCM than in controls [P<0.001]. The mean shortening fraction [SF] was significantly lower in cases than in controls [p<0.001]. The mean Ao and Pul acceleration time [AT] and ejection time [ET] were significantly lower in cases with DCM than in controls [p<0.001]. The mean CPK and LDH levels were significantly higher in patients with raised ESR or CRP than those with normal ESR or CRP [p<0.001 and 0.01]. The mean EPSS was significantly higher in patients with raised ESR than those with normal ESR. Ao ET was significantly lower in patients with raised ESR or CRP than those with normal ESR or CRP. Pul ET was significantly lower in patients with raised ESR than those with normal ESR [p<0.05]. The percentage frequencies of adenovirus or enterovirus IgM positive cases were significantly higher in patients than in control cases together [P<0.001] and separately [p<0.01]. The mean serum CPK and LDH were significantly higher in patients with studied viruses IgM positive than those with viruses IgM negative [p<0.05 and 0.01]. The mean Ao AT, ET and Pul ET were significantly lower in patients with studied viruses IgM positive than those with viruses IgM negative [p<0.01, 0.05 and 0.01]. Enteroviruses and adenovirus are important factors in the pathogenesis of DCM in our children. Cases, with such viral infection tend to be more severe at presentation and have poorer ventricular function. Detection of viral specific IgM with the use of ELISA technique provides both simple and accurate method to detect infection with enteroviruses or adenovirus in our patients with DCM. Increased C/T ratio in the CXR should always alert to the diagnosis of DCM in children with repeated respiratory symptoms particularly in the absence of significant cardiac signs. Serum CPK level detection is usually sufficient to give an idea about ongoing myocardial injury in cases with DCM. ESR, CRP and LDH usually signify infection elsewhere that could trigger heart failure in the compensated patient

study of some biochemical parameters of pulmonary hypertension in children with congenital and rheumatic heart disease

The study included 36 cases with congenital heart disease [CHD] all of them had left to right shunt [17 cases of them had pulmonary hypertension [PHT], 30 cases with valvular rheumatic heart disease [RHD] [15 of whom had PHT]. Ten healthy normal age-matched children were taken as control for each group. Full history and physical examination, pulse oximetry, standard 13-lead ECG and detailed echocardiographic examination were done for every patient and control case. Chest radiograph posterantrior view in erect position was done for all patients. Erythrocytic sedimentation rate, C-reactive rotein and antistreptolysin-O titer were done for patients with RHD. All cases and controls had the following done: plasma level of endothelin-1 [ET-1], angiotesin-II [ANG-II], nitric oxide [NO] and serum level of angiotesin-converting enzyme [ACE] activity. The mean peak pulmonary pressure [PP] in patients having CHD and PHT was 54.35 +/- 9.69 mm Hg, while it was 61.73 +/- 11.55 mm Hg in those with RHD and RHT. The mean peak PP in the group of patients having CHD without PHT was 22.64 +/- 4.11 mm Hg. It was significantly higher than in those with RHD without PHT [9.41 +/- 1.98 mm Hg, P<0.05] and than controls [4.12 +/- 1.25, P<0.01]. It was observed that the mean levels of ET-1, NO, ANG-II and ACE were significantly higher in all cases with CHD [3.42 +/- 0.77, 77.21 +/- 29.14, 81.27 +/- 37.3, 107.2 +/- 47.65, P<0.001], cases with PHT [3.76 +/- 0.81, 94.1 +/- 21.1, 101.4 +/- 33.8, 136.2 +/- 37.9, P<0.001] OR without PHT [2.92 +/- 0.6,61.36 +/- 27.5, 63.28 +/- 16.5, 85.36 +/- 22.6, P<0.5 and 0.01] than in their controls [1.86 +/- 0.6, 34.07 +/- 10.7, 19.15 +/- 10.9, 54.1 +/- 32.1]. It was observed that the mean level of ET-1, NO, ANG-II and ACE were significantly higher in cases with CHD with PHT than in those without PHT [p<0.05 for each]. In the group of patients with RHD, it was observed that the mean levels of ET-1, NO, ANG-II and ACE were significantly higher in all cases [3.89 +/- 1.4, 78.46 +/- 59, 70.35 +/- 37.4, P<0.01 and 0.001] and cases with PHT [4.9 +/- 1.0, 100.5 +/- 32, 95.7 +/- 36.8, 121 +/- 40.9, P <0.001] than in controls [1.55 +/- 0.5, 33.47 +/- 13.0, 22.68 +/- 11.1, 53.27 +/- 32.4]. It was also observed that the mean levels of ET-1, NO, ANG-II and ACE were significantly higher in cases with than in those without PHT [2.6 +/- 0.8, 56.5 +/- 18.0, 44.96 +/- 12.7, 63.3 +/- 37] [P<0.01]. It was observed that the mean levels of ET-1, ANG-II and ACE were significantly higher in cases with CHD having heart failure [3.8 +/- 0.73, 106.8 +/- 50.37, 155.3 +/- 65.66] than those without heart failure [3.26 +/- 0.7, 69.88 +/- 25.1, 91.85 +/- 32.8], P<0.05 and 0.001. There was statistically significant positive correlation between PP in all the studied patients and serum level of ET-1, NO, ANG-II and ACE [P<0.001]. A significant positive corrlation was found between serum level of ET-1 and NO in all the studied cases [P<0.05]. A significant positive correlation was also found between serum level of ANG-II and ACE in all the studied cases [P<0.03]. ET-1 and NO production is increased in cases with PHT secondary to CHD with left to right shunt and to RHD and its production correlates with the level of PP. It could be also concluded that the level of ANG-II and ACE activity is increased in cases with PHT secondary to CHD with left to right shunt and to RHD and its production correlates with the level of PP. The use of NO, NO donors, prostacyclin and ET-1 antagonists should be considered in the management of pulmonary hypertension secondary to hyperkinetic arterial PHT due to CHD with left to right shunt and in the management of venous pulmonary hypertension secondary to RHD. The long-term effect of such drugs on the pathological process in such cases should be studied. Still early intervention in cases with hyperkinetic arterial PHT due to CHD with left to right shunt before the end of the first year of life is highly recommended. It may be also suggested that the use of ACE inhibitors could be useful to prevent the effects of ANG-II on the pulmonary vascular pathology

Neurocognitive dysfunction in children treated from cancer

The study included 50 children with cancer [33 males and 17 females], aged from 6-15 years. All received chemotherapy and 23 of them received additional radiotherapy. One hundred and fifty matched healthy children were chosen as a control group. Thorough medical history, clinical examination and complete neurological evaluation were done to all cases. Wechsler Intelligence Scales for cases and controls were used to evaluate intellectual ability. A screening battery of neuropsychological tests was selected that measured elements of general, cognitive, language, perceptual, motor, affectional and nonverbal problem solving abilities. Three factors were calculated from the scales: Verbal score [VIQ], Performance score [PIQ] and Full-Scale Intelligence Quotient [FSIQ]. Neurologic affection was present in 26 patients, [7 had cranial nerve affection, 17 motor affection, cerebellar ataxia in 4 cases and sensory affection in 6 cases]. No statistical relation was found between neurologic affection and cognitive function. The percentage frequency of children having IQ level [85 was significantly higher in patients than in the control group P<0.007. The mean FSIQ, VIQ and PIQ were significantly higher in control cases than in patients. The mean results in subdivision tests; Vocabulary test, Picture composition test and Coding test were significantly higher in control cases than in patients. The mean FSIQ, VIQ and PIQ were significantly higher in control cases with IQ [85 than in patients. The mean results in subdivision tests; Similarity-test, Vocabulary test, Composition test and Coding test were also significantly higher in control cases than in patients with IQ >85. The percentage frequency of patients who received combined chemotherapy and radiotherapy was significantly higher in the group of cases having IQ level< 85 than in the group of patients having IQ level =/<85. It is recommended that psychosocial and neurologic rehabilitation should be an integral part of the lines of management of such children. Treatment modalities of malignant disease should avoid as much as possible CNS injury to avoid decrease in the intellectual and cognitive impairment that could occur in late survivors

Neural maturation of breast and formula fed infants

Human milk provides infants with a full complement of all polyunsaturated fatty acids, including docosahexaenoic acid [DHA] and arachidonic acid [AA]. Formula milks contain only the precursors of DHA and AA, and linoleic acid and hence formula fed infants must synthesize their own DHA and AA. This work aimed at evaluating the hypothesis that the method of feeding; whether breast feeding or formula feeding in early infancy affects subsequent neurodevelopment and achievement of optimum brain function [Visual system, auditory and Sensory system]. The present study includes 53 infants [at the age of one year +/- I month], 30 infants were exclusively breast fed and 23 infants were exclusively formula fed. Each infant was subjected to a full neurological examination and neurophysiological studies including, flash Visual Evoked Potentials [FVEPs], Auditory Brainstem Evoked Potentials [ABEPs], and Somatosensory Evoked Potentials [SSEPs]. There was significant prolongation in the P100 wave latency of FVEP in formula fed infants [96.4 +/- 9.0] compared with breast-fed infants [90.2 +/- 8.5]. There was a significant prolongation of absolute latency of wave I with subsequent prolongation of wave Ill and wave V in formula-fed infants group compared with breast fed infants group. Meanwhile, there was no significant differences between the two groups as regards to interpeak latencies. There was significant prolongation in interpeak latencies between cortical and Erb's components in formula fed infants compared with breast fed infants. There was no significant correlation between sex, consanguinity, sibbling number, order of birth, parental educational level, age of supplementation of foods, social level, head circumference, anterior fontanel and different variables of evoked potentials. VEP, BAEP and SSEP are more mature in breast fed infants relative to formula fed infants at one year of age. Consequently we can speculate that breast milk helps earlier development and maturation of some aspects of the nervous system than milk formulas

Plasma nitric oxide, endothelin-1 and renin in children with acute renal parenchymal hypertension

Nitric oxide [NO], Endothetin-1 [FT-1] and plasma remin activity [PRA] in addition to complement component [C3], routine kidney function tests and peripheral hemogram, were studied in twenty four infants and children with acute renal parenchyma] hypertension [18 cases with post streptococcal glomerulonephritis and 6 cases with nephritis due to other causes diagnosed by renal biopsy]. Results were compared with those of 24 age and sex matched healthy controls. In patients group, both serum urea and creatinine levels revealed highly significant elevation compared to controls [p<0.001 for both], while glomerular filtration rate and C3 showed highly significant reduction compared to controls [p<0.001 for both]. Mean values of NO. FT-1 and PRA showed highly significant elevation [p<0.001 for all] in patients group compared to controls. No significant difference were found in biochemical tests studied in patients with posts-streptococcal in comparison to patients with nephritis due to other causes except for C[3] which reveated significant reduction in post-streptococcal glomerulonephritis compared to patients group with nephritis

Pulmonary tuberculous children and the Euthyroid Sick Syndrome.

This study was designed to investigate changes in the thyroid hormones in children with active pulmonary tuberculosis [TB] before and three months after starting antituberculous therapy. It was observed that the mean hemoglobin level was significant and lower in patients than controls. The mean serum albumin level was significantly lower in cases before treatment than three months after treatment and the control group. The mean serum level of AST and ALT was significantly higher in patients after treatment than before treatment and the control group. It was observed that the mean serum level of T3 was significantly lower in patients before treatment than after three months of antituberculous treatment or the control group. There were no significant differences in the serum levels of T4 or TSH between the control group and cases before and three months after starting antituberculous treatment. Cases with decreased serum T3 level had more severe clinical manifestations than those with normal serum T3 level

Serum level monitoring of digoxin in cardiac patients attending Assiut University Hospitals: utility in therapy optimizations.

This study addressed the utility of digoxin level monitoring in the optimization of drug therapy. The results revealed toxic serum levels [> ng/ml] only in 91 of the 147 suspected cases. Among 61 patients with inadequate response, low concentrations [<0.7 ng/ml] were noticed. Similarly, in ten patients with non-compliance, very low subtherapeutic levels were observed even to the nil level in 50% of the cases. Accordingly, therapeutic failure of digoxin resulting from under dosage or non-compliance could be detected by TDM that helped in appropriate intervention and in the achievement of optimum clinical outcome. On the other hand, the TDM in clinically controlled cardiac patients indicated that the target concentration of digoxin required to the control heart failure and/or supraventricular arrhythmias could range from 0.7 ng/ml to 2.0 ng/ml. These data indicated that the international therapeutic range [0.7-2.0 ng/ml] of digoxin could be also applied to Upper Egypt cardiac patients. In addition, this study demonstrated the importance of proper sampling time and measurement of serum potassium and creatinine in the interpretation of digoxin level data