CONTENTS:
Cholelihtiasis
Distrophia musculorum progresiva
Pericarditis constrictiva
Health News by Reuters
CHOLELITHIASIS IN CHILDREN.
Panteleeva E 1, Ch. Jelev 1, R. Kumanova 1, E. Lazarova 1, K. Kanchev 1, A. Bueva 2
Sofia Higher Medical School, Bulgaria
University Pediatric Hospital - Clinic of Gastroenterology and Hepatology-1, Clinic of Nephrology-2
Key words: cholelithiasis ( Ch.), ultrasound ( US ), risk factors
It was in 1737 when the first case of cholelithiasis ( Ch.) in a 12-year old boy was published by Gibson. Although that early recognition Ch. in children has been clearly defined as a diagnostic problem in the last three decades, some of the risk factors have been found. The disease rate is rapidly increasing mainly due to the widespread use of abdominal US.The true frequency has not been established yet, it is supposed that 0,1-0,2% of the patients with gall stones are children under the age of 15. The clinical presentation is typical, similar to the adults’ one. The asymptomatic presentation is much more common in children, bile stones are found accidentally or when looked for because of predisposing disease. We report 16 cases of Ch. diagnosed over 10-year period (1987 to September,1999). They are analysed and compared to the cases reported in the literature. There is a clear–cut sex distribution – 12 females and 4 males. The patients are distributed as follows: 8 in the age group 5-10 years, 7 above the age of 11, only one child was diagnosed at the age of 2,5 years.In this case bile stone formation was related to heterozygous b-Thalassemia and dehydration caused by 7-day lasting vomiting and fever. In our study associated conditions were found in 13 cases. Disorders of lipoproteins and abnormalities of the bile ducts are the most frequent predisposing factors in our series, cystic fibrosis – in 1 case, pregnancy – 1, Wilson disease – 3. Cholestatic jaundice and hydrops of the gall bladder were noted in 2 cases. An asymptomatic course was noted in 6 cases. The most common symptom is right upper quadrant abdominal pain accompanied by vomiting. In our experience as it is reported in literature abdominal US (fig. 1) has been found the most reliable method of diagnosing gallstones. Our asymptomatic patients have been followed once a year, two of the symptomatic ones were done ERCP ( fig 2 ), three of the children underwent surgery. Two children are treated with Ursofalk, the follow up is too short to report any results.
You can see the full text in the Bulgarian journal “Pediatria” 1994; 3; 34-37.
Fig. 1
Fig. 2
Duchene Type Muscular Dystrophy in the differential diagnosis of pediatric chronic liver diseases The finding of hyperaminotransferasemia ( hyper-elevated aminotransferase levels ) together with positive history of exposure to viral hepatitis, is suggestive of liver disease. It is well known that AST and ALT are intracellular enzymes, that is why the differential diagnosis of elevated liver enzymes with little or no clinical symptoms should include muscular and cardiac diseases, hemolytic anemias, poisoning, macroenzymes ( macroAST ). The distinction between Duchene Type Muscular Dystrophy (DTMD) and chronic liver diseases in early childhood could be quite difficult sometimes because the clinical symptoms are discrete at that time or the only common complain might be asthenia. Nine cases of DTMD have been diagnosed in our clinic since 1981. Six children were evaluated because of liver enlargement and elevated liver enzymes; one child was admitted to the hospital because of chronic diarrhea; two children underwent diagnostic evaluation because of dramatic elevation of liver enzymes. All the children were males, the age at diagnosis varied between 1 – 11 years. Family history of DTMD was noted in 3 cases. Only one child was found positive for anti-HBcor total and anti-HBs. The most common finding was insignificant hepatomegaly accompanied by unusually high aminotransferase levels. Muscular dystrophy was suspected in six of the patients soon after the admission. The other three children were recognized as DTMD later - 6 months to 3 years after the first visit to the hospital. During the follow up period they were treated as liver disease patients.
Enlargement of the musculature with characteristic involvement of calf and deltoid was noted in two cases. Both enlargement of the musculature and muscle weakness were found in 6 children. One child was diagnosed as DTMD in the process of screening ( by means of CK ) for unexplained hyperaminotransferasemia. Although the attractive hypothesis of liver disease in the clinical spectrum of muscular dystrophy is discussed in the literature, we support the idea of hyperaminotransferasemia as a consequence of muscular disease. In our experience the best approach to the patient in such cases, is the thorough and thoughtful examination of the muscles, gait and looking for muscle weakness. Finally, we recommend ( it is justified ) screening for DTMD in cases of "silent" hyperaminotransferasemia.
Hepatomegaly ( Liver congestion ) as an initial presentation of constrictive pericarditis Constrictive pericarditis(CP) is characterized by thickening and fibrosis of pericardium which may eventually obliterate pericardial sac. The venous flow to the cardiac chambers is restricted. The formation of adhesions develops over a period of years ( subacute or chronic form ). It is well tolerated if myocardium is intact. The arterial pressure and cardiac output are preserved due to the compensatory mechanisms of circulation. The most profound effect of adhesions is the elevated central venous pressure. In conclusion, our experience shows that CP is a rare disease in childhood. It should be suspected whenever isolated and resistant to treatment ascites accompanied by hepatomegaly, normal LFT and absence of portal hypertension, is present. The finding of systemic venous congestion is of high diagnostic value. The modern technical tools ( Echo, CT, MRI ) might easily establish the diagnosis, still the well known X-ray has not lost its diagnostic value. Tuberculosis is the most common cause for CP in Bulgaria and maybe it will keep that leading place for the near future. Surgery is successful when done on time.
In comparison with patients with right cardiac failure due to any other reason, the patients who suffer CP have nonspecific heart complains, they have no ankle edema. Most of them manifest with ascites and liver enlargement, they are suspected of liver cirrhosis or abdominal tumor. Those who present with pleural effusion are suspected of lung cancer or tuberculosis. The review of the literature found several cases of CP diagnosed after liver biopsy was done and the histology revealed cardiac cirrhosis.
CP is a rare disease in childhood.
Four cases of CP diagnosed over 10-year period (1986 – 1996) in our clinic are analysed and compared to the cases reported in the literature. We treated 3 boys and 1 girl. The age at diagnosis was one and the same – between 13 and 14 years. The most common clinical symptom is tiredness when doing physical exercises – 3 patients. Dark urine was noted in 2 cases, pale stools – in 1 case, enlargement of the abdomen – 3, ankle edema – in 1 case.
The initial diagnosis in a municipal hospital was acute hepatitis in two children, the other two patients were suspected of liver cirrhosis. That is why the children were sent to our hospital for further evaluation and treatment. The physical examination revealed hepatomegaly in all the patients, mild splenomegaly in 1, ascites in 3. The liver function tests ( LFT ) performed both in the municipal hospital and in our clinic did not find any liver disorders, there were no signs of portal hypertension. The treatment of edema by means of diuretics, human albumin and abdominal paracentesis failed.
The key symptom of CP when evaluating the patient is the finding of systemic venous congestion. It was found in all the patients.
The high diagnostic value of pulsus paradoxus is well known. Most of the patients, mainly in whom the diagnosis is delayed or suffer the so called non-elastic form of CP, do not manifest that sign. We did not find pulsus paradoxus in our patients.
Heart murmur in the early phase of diastolic filling may be audible. Pericardial friction rub is the cardinal physical sign. There were not present any heart murmurs in our patients.
ECG may show a low voltage QRS complexes or conduction disturbances. Most of the patients have mild shift of ST segment and T wave inversion. ECG showed nonspecific changes, e.g. flat T wave, in only one of our patients.
Usually cardiac X ray does not show cardiomegaly, but pericardial calcifications and pleural effusion might present in 50% of cases. We noted pericardial calcifications in two cases.
Echo is the most useful tool in establishing the diagnosis of CP. It was performed in 3 cases and was diagnostic in 2 ( see Fig.1 ). Cardiac CT was done in 2 cases, huge pericardial adhesions were seen ( see Fig.2 ).
While tuberculosis was the most common cause of CP in the past, today CP remains idiopathic in the majority of patients in the highly developed countries. Other rare causes include acute viral and bacterial infection, collagen diseases, oncologic diseases, trauma, dialysis and radiation as well as some metabolic and specific chromosomal syndromes ( Mulibrey nanism ). We found TBC infection in two cases, the other two are idiopathic. Successful surgical treatment was done in all cases.
( Fig. 2)
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