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Endemic Cretinism

Fetal Iodine Syndrome


Presentation

  • In conclusion, our findings support the hypothesis that neurological features are present in all types of cretins, and are the consequence of maternal and fetal hypothyroxinemia due to severe iodine deficiency.[ncbi.nlm.nih.gov]
  • Seven patients presented clinically with thyroid atrophy of relatively small thyroid enlargements for the degree of chronic iodine deficiency that was present in the area.[ncbi.nlm.nih.gov]
  • Abstract Endemic cretinism is still present in an endemic goiter area of the central Apennines (Montefeltro) (goiter prevalence 55%; mean urinary iodine level 39 micrograms/g creatinine).[ncbi.nlm.nih.gov]
  • Both groups presented a marked increase in serum T3 after therapy (N 515 /- 89 ng/dl; EC 647 /- 149 ng/dl) followed by a decrease in basal and peak TSH response to TRH.[ncbi.nlm.nih.gov]
  • However, the marked height retardation, the observation of delayed bone maturation and the severity of mental deficiency suggest that thyroid failure was present in early life.[ncbi.nlm.nih.gov]
Disturbance of Growth
  • Disturbances of growth, puberty, and sexual function in endemic cretins are explained by the secondary effects of thyroid hormone deficiency on pituitary function.[ncbi.nlm.nih.gov]
Hematochezia
  • Haug Browse recently published Learning/CME Learning/CME View all learning/CME CME Partial Oral versus Intravenous Antibiotic Treatment of Endocarditis Case 4-2019: An 18-Year-Old Man with Abdominal Pain and Hematochezia Bridging the Gap Challenge Yourself[nejm.org]
Abdominal Pain
  • Haug Browse recently published Learning/CME Learning/CME View all learning/CME CME Partial Oral versus Intravenous Antibiotic Treatment of Endocarditis Case 4-2019: An 18-Year-Old Man with Abdominal Pain and Hematochezia Bridging the Gap Challenge Yourself[nejm.org]
Disproportionate Dwarfism
  • This offensive term should be avoided. cretinism (krē tənizəm) (congenital hypothyroidism), cretinism arrested physical and mental development with dystrophy of bones and soft tissues, resulting in disproportionate dwarfism.[medical-dictionary.thefreedictionary.com]
  • This offensive term should be avoided. cretinism arrested physical and mental development with dystrophy of bones and soft tissues, resulting in disproportionate dwarfism. Due to congenital or early-onset hypothyroidism.[medical-dictionary.thefreedictionary.com]
Abnormal Dermatoglyphic Pattern
  • Abstract We have tested the hypothesis that the abnormal development of the central nervous system seen in endemic cretinism might be accompanied by concurrent abnormal dermatoglyphic patterns.[ncbi.nlm.nih.gov]
Meningism
  • Nervous system Meninges Brain Spinal cord Peripheral nerves 9. Musculature 10. The skeleton General restriction in growth Physical proportions Development Bone form relationships Skull Trunk Upper limbs Lower limbs 11. Joints 12. Teeth 13.[bokus.com]
Babinski Sign
  • Nevertheless, all myxedematous cretins had some neurologic disorders (hyperreflexia, increased muscle tone, disorder of gait, Babinski sign, hypoacusia) that were similar to those present in neurologic cretins.[ncbi.nlm.nih.gov]
  • Vestibular function was sluggish in 96 per cent; gait was abnormal in 77.8 per cent, 46.1 per cent had a positive Babinski sign; 35.2 per cent had upper motor neuron paralysis; and many showed severe deformity of the extremities.[archive.unu.edu]
Hyperreflexia
  • Nevertheless, all myxedematous cretins had some neurologic disorders (hyperreflexia, increased muscle tone, disorder of gait, Babinski sign, hypoacusia) that were similar to those present in neurologic cretins.[ncbi.nlm.nih.gov]

Workup

  • […] lateral radiograph of the knee may be obtained to look for the distal femoral epiphysis; this ossification center appears at about 36 weeks' gestation, and its absence in a term or postterm infant indicates prenatal effects of hypothyroidism. [16] See Workup[emedicine.medscape.com]

Treatment

  • It can be prevented by iodine treatment before conception, but whether it can be prevented or ameliorated by treatment during pregnancy or after delivery is not known.[ncbi.nlm.nih.gov]
  • Further, mental disability and other clinical features of neurological damage were not altered by treatment.[ncbi.nlm.nih.gov]
  • To study this question we conducted a five-month follow-up of 51 patients with cretinism (age 14 and below), who were randomly assigned to treatment (0.5 ml of intramuscular iodized oil) and control groups.[ncbi.nlm.nih.gov]
  • It can be prevented by iodine treatment before conception, but whether it can be prevented or ameliorated by treatment during pregnancy or after delivery is not known. METHODS.[hero.epa.gov]
  • The interval from iodine treatment until delivery was used to define the trimester of treatment.[nejm.org]

Prognosis

  • PROGNOSIS Depends on: Early diagnosis Proper counselling Strict diet control Careful monitoring Compliance 32.[slideshare.net]
  • Cretinism Prognosis Early diagnosis of the disease leads to better prognosis. The newborns that are diagnosed and treated within their first month of life usually have normal intelligence.[howshealth.com]
  • Cretinism Prognosis When treated early, the outcome is good. Newborns diagnosed and treated within the first few weeks after birth generally have normal intelligence.[primehealthchannel.com]
  • Improved prognosis in congenital hypothyroidism treated before age three months. J Pediatr. 1972 Nov. 81(5):912-5. [Medline]. Klein AH, Agustin AV, Foley TP Jr. Successful laboratory screening for congenital hypothyroidism.[emedicine.medscape.com]

Etiology

  • The data indicate an autosomal recessive predisposition as a major etiological factor.[ncbi.nlm.nih.gov]
  • Etiology, pathogenesis and developmental sequelae of the condition are discussed and the physical and psychological characteristics are delineated.[researchers.mq.edu.au]
  • Most investigators maintain the opinion that the primary etiological factor is iodine deficiency. The thyroid enlarges as a result of this deficiency, and with chronic stimulation it becomes nodular in the course of time.[popline.org]
  • Cretinism Etiology Lack of thyroid hormones is the cause.[primehealthchannel.com]
  • This led to replacement of the descriptive term sporadic cretinism with the etiologic term congenital hypothyroidism.[emedicine.medscape.com]

Epidemiology

  • AMERICA CENTRAL IODINE EPIDEMIOLOGIA PATOLOGIA EPIDEMIOLOGIE PATHOLOGIE EUROPE AMERIQUE DU NORD AMERICA DEL SUR THERAPEUTIQUE AMERICA DEL NORTE OCEANIE CENTRAL AMERICA MORBIDITE ASIE DU SUD EST NORTH AMERICA ENDOCRINE DISEASES OCEANIA BOCIO SOUTH AMERICA EPIDEMIOLOGY[agris.fao.org]
  • Search for more papers by this author First published: August 1969 Cited by: 4 SUMMARY A study has been carried out of the clinical features, epidemiology and thyroid function in 254 cases of endemic cretinism in Eastern New Guinea.[onlinelibrary.wiley.com]
  • Neurologic Consequences of Malnutrition begins with an overview of the epidemiology and incidence of malnutrition and neurologic disorders.[books.google.ro]
  • Pediatrics and of Radioisotopes, Brussels University and IRSAC[ *** ], Lwiro, Democratic Republic of the Congo Further Information Publication History Publication Date: 08 January 2009 (online) Abstract The aim of the present work is to describe the epidemiological[thieme-connect.com]
Sex distribution
Age distribution

Pathophysiology

  • Its neuropathology and pathophysiology have been investigated very little. We report the clinical and magnetic resonance imaging brain scan findings of 3 adult Chinese cretins.[ncbi.nlm.nih.gov]
  • Furthermore, these data support the hypothesis that the primary pathophysiologic event in the different types of endemic cretinism is represented by maternal and fetal hypothyroidism, while differences may be explained by the extent and duration of postnatal[ncbi.nlm.nih.gov]
  • In conclusion, our hypothesis states that the clinical expression of endemic cretinism is determined by the the sum of two pathophysiologic processes.[liebertpub.com]
  • We found that categorization of the cretins into the conventional types did not reflect the pathophysiology of the condition, since an identical pattern and intensity of neurological, intellectual, and audiometric deficits were common to and equally present[ncbi.nlm.nih.gov]
  • In conclusion, our hypothesis states that the clinical expression of endemic cretinism is determined by the sum of two pathophysiologic processes.[ncbi.nlm.nih.gov]

Prevention

  • It can be prevented by iodine treatment before conception, but whether it can be prevented or ameliorated by treatment during pregnancy or after delivery is not known.[ncbi.nlm.nih.gov]
  • […] of Medicine, University of Adelaide ; now Foundation Professor of Social and Preventive Medicine, Monash University, Melbourne.[onlinelibrary.wiley.com]
  • The results showed that if the iodine supplement was given before conception the nervous form of endemic cretinism was prevented.[ncbi.nlm.nih.gov]
  • Endemic cretinism remains a major preventable cause of intellectual and physical disability in isolated communities in Third World countries.[researchers.mq.edu.au]
  • It can be prevented by iodine treatment before conception, but whether it can be prevented or ameliorated by treatment during pregnancy or after delivery is not known. METHODS.[hero.epa.gov]

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