ABSTRACT
Through the observation of seven cases we reported the epidemiological, clinical, paraclinical, evolutionary and therapeutical aspects of this disease. Epidemiologically, the sex ratio is of 0,75. The medium age is of six years and two months. Factors in favor of a hereditary origin are the consanguinity found in 28,6% of cases and the presence of a similar case in the family observed in 42,3%. Clinically, the quasi constantly neurolgical signs are ataxia [100%], dysarthria [71, 4%], areflexia of endon reflex in both lowner limbs [57,14], deep sensation troubles are less frequent [28,6%]. Vestibular affection with form of horizontal nystagmus is found in 14,3% of cases. The dysmorphic syndrome is in the form of claw feet found in [57,14%] and of dorsal scoliosis in [14,3%]. Cardiac affection in the form of cardiopathy is noted in two magnetic resonance imagery in one patient. The electromyography is disturbed in 28.6% of cases. The genetic study done to two cases [28,6%]. Paraclinically, we found cerebellar atrophy in 57,14% which was confirmed by cases showed an expansion of the triplet GAA at the level of the chromosome nine. Improvement under symptomatic treatment based on muscular and functional physiotherapy was progressively slow for all our patients
Subject(s)
Humans , Male , Female , Friedreich Ataxia/diagnosis , ChildABSTRACT
Rosai Dorfman Disease is in most cases a benign, rare and ubiquitous entity. Its prime interest lies in the fact that it must be distinguished from other malign histiocytosis and neoplasms which simulate it on a clinical and/or histological level. The authors report one case with massive lymphadenopathy, anemia and other auto-immuns signs
Subject(s)
Lymphadenitis/etiology , Child , Lymphatic DiseasesABSTRACT
The infantile visceral leishmaniasis in Morocco is due to the infantum Ieishmania for wich the dog is the principal home. The insects that may carry the parasite to man are numerous. It is the same for phiebotomes: phlebotomus ariasis and phiebotomus perniciosus are the main responsable in the transmission. In this study 216 cases of visceral leishmaniasis are gathered from 1957 to october 1989. Most of them are from the moroccan northen cities. Recently, we can add what this disease affects especially the child of 3 to 4 years old. The quick extension, of this disease from north to south, the affection of the adult and the infant in his carly years incite to intensify prophylaxis and treatement of the declared cases
Subject(s)
Child , LeishmaniasisABSTRACT
The acute epiduritis probably staphylococcus of which we report a particular observation, constitutes a medico-surgical emergency. it is question of a localization secondary to a septicemic state that we sometimes hematogenic external pachymeningitis. Particular antomical disposition of the epidural space explain the posterior dorsal localization and the sterectyped feature of the clinical picture making the affectiona real pathological entity. Improvement of this serious affection, which is uncommun but especially unknown, requires mainly early diangosis and therapeutical management
Subject(s)
Staphylococcal Infections/diagnosis , Acute Disease , Quadriplegia/etiology , ChildABSTRACT
The authors report one case of pulmonary Cryptococcosis in a child who is aged 11 years old struck down by a diffuse interstitial - pulmonary fibrosis. The idiopathic characteristic of cryptococcosis of the pulmonary affection remains difficult to precise. The universally recognized opportunist character of the neoformans Cryptococci has been insisted on. This aspect makes the Cryptococcosis a very feared affection on debilited grounds
Subject(s)
Child , MycosesABSTRACT
The authors reporte here a necroticohemorrhagic acute pancreatitis case in 30 months-child. This very uncommon disease requires early and affective diagnosis and therapeutical management. The authors discuss usefulness of complementary examinations and their sensitivity, the prognosis factors and the therapeutical attitude to manage uncommon and dreadful affection
Subject(s)
Acute Disease , Child , PancreasABSTRACT
The authors report the case of a 13 years old girl affected by the progressive systemic scleroderma, complicated by the oesophagenal and renal affection revealed by a nephrotic syndrom. With the helf of litterature facts, we trying to discuss the prognostic value of the renal affection which exceptionnally happens over the scleroderma
Subject(s)
Kidney/pathology , Nephrotic Syndrome/diagnosis , ChildABSTRACT
The authors report one case of a symmetrical gangrene of the feet noted during enteric fever in a 10 years old female child. Such complication is rare and it has a lot of pathogenic factors which all are incriminated in creating a low perfusion phenomenon occurring to a disseminated intravascular coagulation which is amplified or created by a blood circulation failure. A personal predisposition to this kind of complication is still hypothetic
Subject(s)
Typhoid Fever/complications , ChildABSTRACT
The study of cases of collodion baby previously published led to several conclusions-In the short-term, the child is threatened by pulmonary infections The increased skin permeability may result in the loss of water or intoxication of substance applied to the skin In the long-term the condition progresses to one of congenital disturbances in keratinisation simple or complex -The absence of parallelisme between initial intensity and the distant future imposes an extended supervision
Subject(s)
Ichthyosis/pathology , Infant, Newborn , Genetic CounselingABSTRACT
The infantile scurvy or Barlow's disease has not only fallen into oblivion by public but has been ignored by doctors as well. Authors report one case of infantile scurvy. It's a four years boy hospitalised because of total functional helplessness of lower limbs, kenees and ankles arthritis as well as distinctive hemmoragic signs. The diagnosis was precised by radiological tests and the confirmation by specific and exclusive therapeutic which allows walking recovery and the progressive disappearance of radiological anomalies