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Ateliotic Dwarfism

Congenital Isolated GH Deficiency


Presentation

  • Diabetes insipidus is frequently present, bitemporal hemianopia or complete blindness may occur, and skin is often yellow and wrinkled, but mentality is usually unimpaired.[medical-dictionary.thefreedictionary.com]
  • The fact that the incidence of diabetes is very high, at present 1 %-2 % of the population is affected and their number is gradually increasing - dis plays the timeliness of this subject.[books.google.com]
  • This reference guide will be the must-have of any internist or medical professional, as it takes a vast quantity of data across a sweeping grid of disorders, diseases, and presentations, and boils them down to individual parts, facilely indexed, and located[books.google.com]
Weight Gain
  • Clinical findings Galactorrhoea, amenorrhoea or oligomenorrhoea, secondary hypothyroidism, cold intolerance, hypotension, bradycardia, weight gain, hair loss, adrenal insufficiency, fatigue, loss of libido, hypoglycaemia, hyponatraemia. panhypopituitarism[medical-dictionary.thefreedictionary.com]
  • Hypothyroidism can cause many symptoms, including weight gain and fatigue. Other features of combined pituitary hormone deficiency include delayed or absent puberty and lack the ability to have biological children (infertility).[icdlist.com]
Dysostosis
  • Synonym: primordial dwarfism, true dwarfism. (05 Mar 2000) Robinow dwarfism Dwarfism associated with foetal face, acral dysostosis, and genital anomalies; there is also an autosomal recessive form. (05 Mar 2000) Seckel dwarfism An autosomal recessive[kmle.co.kr]
  • […] hypophosphatemic rickets 270.0 nutritional 263.2 ovarian 758.6 pancreatic 577.8 pituitary 253.3 polydystrophic 277.5 primordial 253.3 psychosocial 259.4 renal 588.0 with hypertension - see Hypertension, kidney Russell's (uterine dwarfism and craniofacial dysostosis[icdlist.com]
Cold Intolerance
  • Characteristic signs of the disorder are failure to lactate, amenorrhea (in females), weakness, cold intolerance, lethargy, and loss of libido and of axillary and pubic hair.[medical-dictionary.thefreedictionary.com]
Short Stature in Children
  • Growth hormone treatment for short stature in children born small for gestational age. Adv Ther. 2008;25:951-978. Chatelain P.[revistamedica.imss.gob.mx]
Loss of Hair
  • […] of hair Peripheral axonal neuropathy Pituitary cachexia Pituitary deficiency due to empty sella turcica syndrome Pituitary dwarfism Pituitary dwarfism with large sella turcica Pituitary dwarfism with normal somatotropin level AND low somatomedin Pituitary[icdlist.com]
Hypotension
  • There may be bradycardia or hypotension, and progression of the disorder leads to premature wrinkling of the skin and atrophy of the thyroid and adrenal glands. Treatment consists of the administration of the hormones of the target organs.[medical-dictionary.thefreedictionary.com]
  • Arterial hypotension is quite often noted. On an ECG owing to trophic disturbances of a myocardium the low voltage, decrease in height of teeth is noted.[bigmed.info]
Thrombosis
  • Postpubertal panhypopituitarism may be caused by postpartum pituitary necrosis (Sheehan's syndrome) resulting from thrombosis of pituitary circulation during or after delivery or other trauma to the pituitary.[medical-dictionary.thefreedictionary.com]
Hepatosplenomegaly
  • It is characterised by excretion of dermatan sulfate in the urine, growth retardation, lumbar kyphosis, sternal protrusion, genu valgum, usually hepatosplenomegaly, and no mental retardation. Onset occurs after two years of age.[kmle.co.kr]
Retinal Lesion
  • No retinal lesions were detected in any HGH-deficient dwarf.[ncbi.nlm.nih.gov]
  • No retinal lesions were detected in any HGH-deficient dwarf. Images[jci.org]
  • No retinal lesions were detected in any HGH-deficient dwarf. Full text Full text is available as a scanned copy of the original print version.[ncbi.nlm.nih.gov]
Skeletal Dysplasia
  • International Skeletal Dysplasia Society International Skeletal Dysplasia Society The principal aim of the Society shall be to promote scientific progress in the field of skeletal dysplasias and dysostoses ...[ibis-birthdefects.org]
  • Dysplasia Clinics Natural Histories of Dwarfism Types Research and Treatments Local Info District 1 CT, ME, MA, NH, RI, VT District 2 NJ, NY, PA District 3 DE, DC, MD, NC, SC, VA District 4 FL, PR District 5 IN, KY, MI, OH, WV District 6 IL, WI District[lpaonline.org]
  • His current work (2000) is on the skeletal dysplasias, therapy in bone dysplasias, and UCLA intercampus medical genetics training program. Bibliography D. Rimoin, T. Merimee, V.A.[whonamedit.com]
Genu Valgum
  • It is characterised by excretion of dermatan sulfate in the urine, growth retardation, lumbar kyphosis, sternal protrusion, genu valgum, usually hepatosplenomegaly, and no mental retardation. Onset occurs after two years of age.[kmle.co.kr]
Joint Subluxation
  • subluxed, spine: kyphoscoliosis with gradual decrease in interpediculate distance toward caudal end of lumbar spine, hands, hitch-hiker's thumb - widely abducted, hypoplastic first metacarpal, other metacarpals too broad, especially at growing end, carpal[kmle.co.kr]
Amenorrhea
  • Characteristic signs of the disorder are failure to lactate, amenorrhea (in females), weakness, cold intolerance, lethargy, and loss of libido and of axillary and pubic hair.[medical-dictionary.thefreedictionary.com]
Tantrums
  • Childishness, as characterised by a temper tantrum of an adolescent or adult. 3.[kmle.co.kr]

Workup

Delayed Bone Age
  • Pituitary Dwarfism type IV: (Normal Immunoreactive Growth Hormone and Low Somatomedin Pituitary Dwarfism Syndrome; Biodefective Growth Hormone Syndrome; Kowarski Syndrome) is characterized by growth retardation, pituitary dwarfism, and delayed bone age[accessanesthesiology.mhmedical.com]
Hypercholesterolemia
  • Hypercholesterolemia and hypertriglyceridemia occurred with greater frequency in both diabetics and HGH-deficient dwarfs than in normal controls. 8 of 21 diabetics and 6 of 21 sexual ateliotics exhibited significant hypertriglyceridemia.[ncbi.nlm.nih.gov]
Hypertriglyceridemia
  • Hypercholesterolemia and hypertriglyceridemia occurred with greater frequency in both diabetics and HGH-deficient dwarfs than in normal controls. 8 of 21 diabetics and 6 of 21 sexual ateliotics exhibited significant hypertriglyceridemia.[ncbi.nlm.nih.gov]

Treatment

  • Treatment consists of the administration of the hormones of the target organs. Panhypopituitarism may also be caused by pituitary apoplexy, hemorrhage, or head trauma. Also called hypophyseal cachexia, pituitary cachexia, Simmonds' disease.[medical-dictionary.thefreedictionary.com]
  • Primordial dwarfism causes, pictures, treatment and. Worlds smallest human beings gary parker. Dwarfism types, causes, treatments, and more webmd. Think you have problems consider primordial dwarfism.[block-appreciates.ga]
  • Growth hormone treatment regimens in Australia: analysis of the first three years of treatment for idiopathic growth hormone deficiency and idiopathic short stature. Clin Endocrinol. 2012;77:62-71. García RJ, Martinez-Aguayo A, Mericq V.[revistamedica.imss.gob.mx]
  • Scheme of treatment discontinuous: the period of rest makes about a half of term of treatment; during the accustoming breaks up to 4 — 6 months are shown.[bigmed.info]

Prognosis

  • What Is the Prognosis for a Person with Combined Pituitary Hormone Deficiency, PROP1-Related? Individuals with CPHD are typically able to live a normal lifespan.[myriadwomenshealth.com]
  • What is the prognosis for a person with PROP1-related Combined Pituitary Hormone Deficiency? People with PROP1-related CPHD are typically able to live a normal lifespan.[counsyl.com]
  • What is the Prognosis for a Person With PROP1-related Combined Pituitary Hormone Deficiency? People with PROP1-related CPHD are typically able to live a normal lifespan.[genesis.org.in]
  • Prognosis: Highly dependent upon age of diagnosis and success of hormone replacement. Ancillary treatments and support: Case-specific dependent on nature of hormonal deficiency. Specialists and specialty centers: Endocrinologist, pediatrician.[wohproject.org]
  • 22: Mammography and Imaging; half five: distinctive subject matters, part 23: particular subject matters in Endocrinology; half 6: DIABETOLOGY, part 24: Glucose Metabolism and Hypoglycaemia; part 25: Diabetes Mellitus and Insulin Resistance; part 26: prognosis[electricsexclothing.com]

Etiology

  • Increased fall in blood glucose and delayed return to normal following intravenous administration of insulin; however, spontaneous hypoglycemic episodes are rare Etiology.[kundoc.com]
  • However the first attempts of consideration of an etiology To. and classifications were undertaken at the end of 19 century Classification the First scientific classification To. it was offered by V. Hansemann in 1902.[bigmed.info]

Epidemiology

  • Relevant External Links for PROP1 Genetic Association Database (GAD) PROP1 Human Genome Epidemiology (HuGE) Navigator PROP1 Atlas of Genetics and Cytogenetics in Oncology and Haematology: PROP1 No data available for Genatlas for PROP1 Gene Mutations in[genecards.org]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology/Complications Abstract OBJECTIVE —Alström syndrome, with type 2 diabetes, and blindness could confer a high risk of foot ulceration.[care.diabetesjournals.org]

Prevention

  • The T2DM is possibly preventable with way of life alterations and drugs. equally dietary deficiency ailments like goitre, metabolic bone illnesses also are preventable.[electricsexclothing.com]
  • Prevention Prevention comes down to the prevention of infectious diseases, mental and physical. injuries at pregnant women, prevention of birth craniocereberal trauma at children, to providing good sots. - a gigabyte. conditions and good nutrition.[bigmed.info]
  • Laron Dwarfism information including symptoms, diagnosis, misdiagnosis, treatment, causes, patient stories, videos, forums, prevention, and prognosis. To offer friendship and support to people with dwarfism. A dwarf is a person of short stature.[block-appreciates.ga]
  • The fact that the kidney cannot retain excess homogentisic acid prevents major deviation from normal metabolism.[biologydiscussion.com]
  • Growth hormone treatment before the age of 4 years prevents short stature in young girls with Turner syndrome. Eur J Endocrinol. 2011;164:891-897. Ross JL, Quigley CA, Cao D, Feuillan P, Kowal K, Chipman JJ.[revistamedica.imss.gob.mx]

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