Epidemiology and medical cost of hospitalization due to rotavirus gastroenteritis among children under 5 years of age in the central-east of Tunisia

Data on the economic burden of rotavirus infection in Tunisia are needed to inform the decision to include rotavirus in routine childhood immunizations. This study aimed to describe the epidemiological profile of rotavirus disease in central-east Tunisia and to estimate its hospital cost. In the first stage - the prospective collection of epidemiological data - we enrolled all patients < 5 years old who were hospitalized for acute diarrhoea at 5 university paediatric departments in central-east Tunisia during the period 2009-2011. Rotavirus was responsible for 65 [23.3%] of the 279 cases enrolled. In the second stage, cost data were collected retrospectively using an activity-based costing method from the medical records of the children who were positively diagnosed with rotavirus. The average cost of care per child was TD 433 [SD 134]. This is a significant economic burden in Tunisia, where a safe and effective vaccine is available but not yet introduced to the immunization schedule

[Alcardi's syndrome in a monozygotic twin girl]

Aicardi's syndrome is a rare genetic disorder characterized by heterogeneity of clinical findings and prognosis. The characteristic features include infantile spasms, agenesis of the corpus callosum and chorioretinal lacunae. This syndrome is seem almost exclusively in female and it's often sporadic. The authors report a case of a premature female twins with typical Aicardi syndrome and her sister sibling was unaffected. At 4 months of age, the patient developed flexion in spasms and constipation. At that time she had microcephaly, abdominal ballonnement and generalized hypotonia. The electro- encephalography showed a characteristic hypsarrhythmia. The cranial ultrasonography and computed tomographic scan cerebral demonstrated agenesis of the corpus callous. Chest radiograph revealed multiple dorsal hemi vertebrae and scoliosis. The ophthalmologic examination showed chorioretinal lacunae. The karyotype was 46, XX. The patient also had peripheric hypothyroidism. The outcome under Vigabatrin and L-Thyroxin was characterized by a psychomotor retardation, a severe epilepsy, gastroesophageal reflux and recurrent pneumonia which caused the death at 2 years of age

[Management of hydronephrosis prenatally diagnosed]

Hydronephrosis, defined as dilatation of the renal pelvis and/or calyces is most frequently detected by prenatal ultrasound. The initial postnatal ultrasound performed in the first 48 hours of life underestimated the degree of hydronephrosis in most newborns. We recommend initial postnatal sonogram at 7 days of life. Hydronephrosis is most commonly assessed by the Society for Fetal Urology [SFU] grading system or anterior-posterior diameter [APD] of the renal pelvis in the transverse plane. This classification is valid by postnatal ultrasound. Even degree of postnatal hydronephrosis was predictive factor for the need for surgery; hydronephrosis does not mean ureteropelvic junction obstruction [PUJO]. The natural history of hydronephrosis has shown that most resolve spontaneously. Investigations are not recommended if APD<10 mm. Antibiotic prophylaxis is not required if vesicoureteral reflux is excluded. Diuretic renography is useful in many cases to evaluate the degree of obstruction and determine differential renal function. With the excellent results of pyeloplasty, it is proposed for babies with congenital PUJO and <40% split differential function. A management algorithm is proposed

[Persistent hyperinsulinemic hypoglycemia [HHI] of infancy: about 3 cases]

Hyperinsulinaemic hypoglycaemia [HHI] is the most frequent aetiology of recurrent severe hypoglycaemia in newborn and infant. This pathology arises the problem of early medical and surgical management to prevent brain damage. The aim of this work is to stress on the treatment emergency, to underline difficulties to distinguish damage. The aim of this work is to stress on the treatment emergency, to underline difficulties to distinguish between focal forms and diffuse forms, and to discuss our results with literature data. Our work is a retrospective study of 3 observations of symptomatic newborn HHI. Due to failure of glucose supplementation and medical treatment based on diazoxide and hydrocortisone, the 3 patients were operated between 50 and 107 days of life. Surgical exploration did not find any macroscopic pancreatic abnormality and intervention consisted of subtotal pancreatectomy [90-95 percent]. Anatomopathological study showed diffuse form in 2 cases and focal form in one case. After a follow up of 3 months to 2 years and a half the 3 patients have a normal glycaemia with no need to medical treatment. Yet 2 patients present brain damages .HHI is an emergency which needs a rapid medical management. Medical treatment failure should lead quickly to surgery to prevent neurological complications

[Pediatric emergency in Mahdia hospital]

To study the profile of the emergency users, the attitude of the doctors providing medical care and the degree of satisfaction of those users. Prospective study concerning the pediatric emergency users into a 3rd level hospital, during 3 different periods between 26/05/2002 and 25/09/2003. The medium age of the consultants was 3 years, the sex ratio was 1, 03. 58,3 per cent belonged to modest socio-economic level, 65,7 per cent of then hadn't a treating doctor for following, and 68,3 per cent of the childs were brought by the proper initiative of their parents. The expectation delay before the consultation was less then 15 minutes in 57,3 per cent of the cases. After consultation, this delay was less than one hour in 50,7 per cent of the cases and between 1 and 3 hours in 44,3 per cent of the cases. 14,7 per cent of the children were hospitalised. The emergency case was considered simple or just a felt-emergency in 50,7 percent of the cases, and it was a simple consultation in 28,1 per cent of the cases. 46 per cent of the parents were "satisfied" of services. The major part of consultations at the emergency services is not justified. We have to educate the parents about how important is to have a family doctor, to supervise emergency structures to improve the quality of the offered services

[Recessive osteopetrosis: new mutation]

Autosomal Recessive osteopetrosis [ARO] is a severe bone disease, whose cellular defect is consisting in impaired osteoclast bone resorption, resulting in generalized osteosclerosis and obliteration of marrow. The molecular defect is heterogeneous. 50 per cent of ARO patients show an abnormality in the TCIR1 gene coding for the a3 subunit of vacuolar proton pump that plays a fundamemtal role in acidifying the osteoclast-bone interface. We report a new case with mutation in the TCIRG1 gene. The patient, a 4 months old male infant, presented with exophthalmia, macrocephaly, hepatosplenomegaly, a very severe bone sclerosis,anemia, thrombocytopenia, optic atrophy, with fatal outcome at second year. He has the mutation G11049T in homozygous state in the TCIRG1 gene, this mutation is in heterozygous state in the parents. Prenatal diagnosis was carried out in the mother by amniocentesis performed at 16 weeks of second gestation. The fetal DMA analysis showed that same mutation were present in heterozygous state. A healthy baby with no clinical, radiological or abnormalities was delivered

[Idiopathic pulmonary hemosiderosis rescue treatment with chloroquine: a case report]

Idiopathic pulmonary haemosiderosis is a rare but potentially lethal disorder. Long-term treatment with corticosteroids may improve the prognosis of this disease. Other immunosuppressive agents have been used in a small number of cases with encouraging results. We report a case of a twelve year old girl presenting a severe form of Idiopathic pulmonary haemosiderosis treated long-term high-dose steroids. That leeds to significant side effects. Chloroquine used after inefficiency of Azathioprine induced a subsequent remission

Deficiency in complex IV of mitochondria respiratory chain

Mitochondrial cytopathies are diseases due to a defect of mitochondrial respiratory chain and are characterized by the presence of morphological abnormalities of mitochondria. These diseases may be due to alterations of the mitochondrial or the nuclear genome. The clinical manifestations can be polymorphic as various organs may be involved. We report the case of a 2-year-old boy who has a declined development correlated with a distal renal tubular acidosis. His behavioural and motor development was normal until l2months when a regression of his motor milestones with a pyramidal syndrome was noted. The metabolic investigation and the cranial MRI revealed a Leigh syndrome. The biochemical and immunological studies on biopsied skeletal muscle and cultured skin fibroblasts showed a deficiency in the complex IV respiratory chain [cytochrome c oxidase or COX]

Right diaphragmatic paralysis in newborn

Isolated diaphragmatic paralysis is a rare affection in newborn. It is often associated to a brachial paralysis due to obstetrical factors. The authors report two cases of right diaphragmatic paralysis revealed by a respiratory distress developed immediately at birth in two male newborns, a premature of 34 weeks of gestation and a full-term infant, born after breech delivery and perinatal suffering. The chest radiography and the radioscopy confirmed the diagnosis of right diaphragmatic paralysis. It was isolated in the premature and associated to a facial paralysis and bilateral brachial paralysis in the full-term newborn. The evolution of the respiratory distress was favourable after a non invasive ventilation of some days in the both cases. Besides, the full-term infant kept a severe neurological distress and dies at 36 days of age by a nosocomial infection. At 16 months of age, the premature did not present respiratory abnormalities but keeps another ascent of the dome right hemidiaphragm. The prevention of this affection is very important by eutocic progress of the delivery

[Meckel Gruber syndrome a case report]

Meckel Gruber syndrome is a rare lethal autosomal recessive disorder characterized by a clinical and genetical heterogenicity. Currently, the diagnosis is based on some major criteria: occipital meningo-encephalocele, bilateral polycystic displastic kidneys and postaxial polydactily. Prenatal diagnosis by a transvaginal ultrasound is possible at the 11th week of gestation justifying therapeutic abortion. The prevalence little valued in our country and the variability of the genes according to ethnics as well as their expression phenotypic in the same ethnic opens perspectives in the fundamental research as for the positioning of genes and the survey of their expression

Atypical Aicardi's syndrome on two sisters not twins

Aicardi's syndrome is a rare genetic disorder characterized by heterogeneity of clinical findings and prognosis. The characteristic features include infantile spasms, agenesis of the corpus callosum and chorioretinal lacunae. This syndrome is seen almost exclusively in female and is often sporadic. However, some familial cases were reported in the literature. The prognosis is unpredictable. It is grave in most of cases with severe psychomotor retardation. The authors report two sisters not twins, from a consanguineous familily with atypical Aicardi syndrome diagnosed at 2 months of age. Clinical feautures were infantile spasms, partial agenesis of the corpus callosum and atypical ophthalmologic abnormalities. Evolution was marked by severe psychomotor retardation, scoliosis and severe recurrent pneumonia entailing the death of one of the patients at 3 years of age

[Chediak- Higashi Syndrome. report of one case]

Chediak - Higashi syndrome is a rare hereditary disease. Most patients develop an accelerated phase of the disease. Once accelerated phases occurred, the disease is invariably fatal before the use of etoposide. We report the case of a 5 year-old girl with consanguineous parents, presenting a partial oculocutaneaous hypopigmentation admitted because of protracted fever, lethargy, paler and hepatosplenomegaly. Laboratory results confirmed the diagnosis of hemophagocytic syndrome. The finding of a large cytoplasm granules in born marrow leucocytes confirmed the diagnosis of Chediak Higashi syndrome. Treatment with immunoglobulin, steroids and cyclosporine induced a transitory remission and a relapse occured after 3 weeks. The use of etoposide associated with precedent treatment leads to a prolonged remission and bone marrow transplantation was succefuly performed after 5 months. This case reports the severity of accelerated phase of Chediak- Higashi syndrome and the efficiency of the treatment with etoposide

[Forein bodies in the tracheo-bronchial tree. about 94 cases]

This is a study about 94 tracheo-bronchial foreign bodies observations. The most frequent involved age is between 1 and 2 years [64.5 per cent]. The penetration syndrome found in 74, 4 percent of case. Physical examination is often abnormal [79.7 per cent]. The chest radiography shows emphysema [39.9 per cent] or atelectasis [18.08 per cent]. No mortality case was reported

[Hereditary osteo-onychodysplasia. a case report]

Hereditary osteo-onycho-renal dysplasia is a rare genetic disorder also known as nail patella syndrome. It is an autosomal dominant hereditary disease with complete penetration but variable expressivity. The basic manifestations include: iliaque horns, finger nail abnormalities, patella hypoplasia or aplasia and elbow deformities. Nephropathy accompanied by mild proteinuria is also thought to be a commun manifestation. We report the case of 7 years old boy and his family who show clinical features of NPS

[ malignant adrenocortical carcinoma of the child to a comment]

Malignant corticossurrenaloma is very rare in childhood. It occurs more often before the age of 5 years. Its pathogeny remains unknown but a genetic predisposition was suggested. The tumor is usually of secreting type and revealed by virilisation and/or Cushing syndrome, seldom by feminization and exceptionally by Conn syndrome. We report the case of a 7 years old girl who presented with a hetero-sexual precocious pseudo-puberty associated to a Cushing syndrome. The clinical examination found an abdominal mass. Hormonal investigation confirmed androgen and gluco-corticoid hypersecretion. Malignancy of the tumor was evoked by MRI. Evolution was marked by a tumoral rupture which required a laparotomy in emergency. The anatomo-pathological examination confirmed the diagnosis. In spite of a favorable immediate post operative evolution, the child died eight days later. Through this observation, we discuss the clinical, biological, therapeutic and evolutive aspects of this affection