Prevalence and Incidence of Bullous Pemphigoid
Ophanet, who are a consortium of European partners,
currently defines a condition rare when if affects 1 person per 2,000.
They list Bullous Pemphigoid as a "rare disease".
More information about Bullous Pemphigoid is available from Orphanet
Bullous Pemphigoid Prevalence: Book Excerpts
Prevalence/Incidence of Bullous Pemphigoid: Online Medical Books
16 MEDICAL BOOKS ONLINE!
Review excerpts from medical books online, free, without registration,
for more information about the prevalence and/or incidence of Bullous Pemphigoid.
Dermatitis:
Causes and incidence
(Professional Guide to Diseases (Eighth Edition))
The cause of atopic dermatitis is unknown, but a genetic predisposition may be exacerbated by such factors as food allergies, infections, irritating chemicals, temperature and humidity, and emotions. Approximately 10% of childhood cases are due to allergy to certain foods, particularly eggs, peanuts, milk, fish, soy, and wheat. Atopic dermatitis tends to flare up in response to extremes in temperature and humidity. Other causes of flare-ups are sweating and psychological stress.
An important secondary cause of atopic dermatitis is irritation, which seems to change the epidermal structure, allowing immunoglobulin (Ig) E activity to increase. Consequently, chronic skin irritation usually continues even after exposure to the allergen has ended or after the irritation has been systemically controlled.
Atopic dermatitis is most common in infants, usually developing between ages 1 month and 1 year, commonly in those with strong family histories of atopic disease. At least half of those cases clear by age 36 months. These children often acquire other atopic disorders as they grow older. Typically, this form of dermatitis flares and subsides repeatedly before finally resolving during adolescence. However, it can persist into adulthood. In adults, it’s generally chronic or recurring.
» READ BOOK EXCERPT ONLINE »
Source: Professional Guide to Diseases (Eighth Edition), 2005
Cough - Case 4-2: 7-Week-Old Boy:
III. Incidence and Epidemiology
(Pediatric Complaints and Diagnostic Dilemmas)
VSDs are the most common cardiac malformation seen in children. Recent studies
have shown the incidence of VSD in newborns to be 5 to 50 per 1,000 children,
with a slight female predominance. VSDs are the most common form of congenital
heart disease associated with chromosomal disorders.
VSDs are classified into four types: perimembranous (80%), outlet (5% to 7%),
inlet (5% to 8%), and muscular (5% to 20%). Muscular defects have the greatest
likelihood of undergoing spontaneous closure. Approximately 75% to 80% of all
VSDs close spontaneously, most often by 2 years of age.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Cough - Case 4-3: 7-Month-Old Girl:
III. Incidence and Epidemiology
(Pediatric Complaints and Diagnostic Dilemmas)
Measles is the infectious condition caused by the rubeola virus, an RNA virus of
the family Paramyxoviridae. Before the introduction of the measles vaccine in
1963, some 200,000 to 300,000 cases of measles were seen each year in the
United States. Since then, this has decreased by 99%. Currently, measles is
reported rarely in preschool children, some of whom are too young to be
vaccinated. Infrequently, cases of primary vaccine failure are reported.
Measles is spread as an airborne virus, and infection results from direct
contact with droplets from the respiratory secretions of infected patients. The
typical incubation period is 10 days. Children are most infectious when cough
and coryza are at their peak, which occurs during the late prodromal phase.
Children are considered contagious from several days before until 5 days after
the onset of the rash. The mortality rate for measles in the United States is 1
in 3,000 cases.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Cough - Case 4-6: 4-Month-Old Boy:
III. Incidence and Epidemiology
(Pediatric Complaints and Diagnostic Dilemmas)
B. pertussis, a gram-negative bacillus, is the causative organism for what is commonly
referred to as whooping cough. A whooping cough syndrome can also be seen with
Bordetella parapertussis, M. pneumoniae, C. trachomatis, Chlamydia pneumoniae, and some adenoviruses.
Pertussis is considered one of the most highly communicable diseases, with
transmission occurring via contact with respiratory tract secretions of an
infected patient. With waning immunity from childhood vaccination, adults and
adolescents are commonly the source of infection in infants and young children.
The true incidence of pertussis is unknown, because many cases in adolescents
and adults are unrecognized. However, it is known to be a worldwide threat,
with an estimated 40,000,000 cases and 360,000
deaths per year. In general, the disease is endemic, but there are 3- to 5-year
cycles of epidemics that occur in addition to the endemic levels. For unknown
reasons, girls are affected at much higher rates and with higher morbidity than
boys.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Diarrhea - Case 17-1: 2-Month-Old Boy:
III. Incidence and Epidemiology
(Pediatric Complaints and Diagnostic Dilemmas)
The incidence of inguinal hernia is estimated to be between 1% and 5%, or
approximately 10 to 20 cases per 1,000 live births. The incidence in premature
infants is significantly higher, approaching 30%. The ratio of boys to girls is
6:1. In boys, the right side is more frequently involved than the left,
presumably due to the embryologic origin of inguinal hernias through a patent
processus vaginalis and the fact that the right testis descends later during
gestation than the left. In both boys and girls, 60% of inguinal hernias occur
on the right, 30% on the left, and 10% bilaterally. Inguinal hernias are
usually diagnosed during the first year of life, most frequently during the
first month. There is often a family history of inguinal hernia. Undescended
testes may be associated with inguinal hernias. Other conditions associated
with inguinal hernias include Ehlers-Danlos syndrome, cystic fibrosis,
congenital cytomegalovirus infection, and testicular feminization. There is no
apparent ethnic or racial predisposition to inguinal hernia. Incarcerated
inguinal hernias occur most frequently before 6 months of age, are less common
after 2 years of age, and are rare after 5 years of age.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Diarrhea - Case 17-4: 15-Month-Old Boy:
III. Incidence and Epidemiology
(Pediatric Complaints and Diagnostic Dilemmas)
The annual incidence of neuroblastoma is approximately 8 per 1 million children
younger than 15 years of age. The median age at diagnosis is 22 months, and 95%
of cases are diagnosed by the age of 10 years. Neuroblastoma accounts for
approximately 6% of all pediatric tumors. There is a slight male preponderance,
with a ratio of 1.2:1. There also appear to be cases that are familial in
nature and manifest at a younger age, with a median age of 9 months at
diagnosis. These tumors derive from postganglionic sympathetic cells found in
the paraspinal sympathetic ganglia and in the adrenal chromaffin cells.
Neuroblastoma and ganglioneuroblastoma represent the malignant forms of these
neural crest tumors, whereas ganglioneuroma represents the most benign form,
with no metastatic potential.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Fever - Case 11-1: 18-Month-Old Girl:
III. Epidemiology and Incidence
(Pediatric Complaints and Diagnostic Dilemmas)
NF1 and NF2 are genetic disorders in which affected patients develop both benign
and malignant tumors at increased frequency. NF1 is associated with cutaneous
lesions, vision loss, and skeletal problems; cataract formation and hearing
loss are more typically associated with NF2. NF1, also known as von
Recklinghausen
's neurofibromatosis or peripheral neurofibromatosis, is an autosomal dominant
condition. Half of the cases occur in patients with a family history of NF1,
and the other half occur as spontaneous mutations. The incidence is
approximately 1 in 3,000. The clinical manifestations of NF1 result from
alterations of the NF1 gene located on chromosome 17. The gene product, termed
neurofibromin, is thought to function as a tumor suppressor, but research is
still ongoing.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Fever - Case 11-4: 7-Month-Old Girl:
III. Epidemiology and Incidence
(Pediatric Complaints and Diagnostic Dilemmas)
AIN can occur as an isolated phenomenon (primary AIN) or in association with a
known precipitating factor (secondary AIN), such as other autoimmune disorders,
infections, medications, and malignancies. In infants and young children, the
term
primary AIN usually refers to AIN in infancy (formerly known as chronic benign neutropenia).
The average age at diagnosis of AIN in infancy is 8 months (range, 1 to 38
months). Two thirds of patients are diagnosed between 5 and 15 months of age.
The estimated frequency is 1 per 100,000 children, making it more common than
the severe chronic neutropenias such as cyclic neutropenia.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Jaundice - Case 15-1: 14-Day-Old Boy:
III. Incidence and Pathophysiology
(Pediatric Complaints and Diagnostic Dilemmas)
Galactosemia is a rare inborn error of metabolism that occurs in 1 of every
60,000 infants. It is caused by the absence of an enzyme of galactose
metabolism. Presenting signs in the galactose-exposed, affected neonate can
include jaundice, hepatomegaly, seizures, lethargy, vomiting, hypoglycemia,
cataracts, and failure to thrive. In addition, babies with galactosemia exhibit
a heightened susceptibility to bacterial infection, particularly
Escherichia colisepsis. Although galactosemia is widely assessed in state newborn screening
programs, the onset of life-threatening clinical illness may precede the
completion of testing.
The hydrolysis of dietary lactose produces glucose and galactose. Galactose is
subsequently phosphorylated to galactose-1-phosphate. This compound, in turn,
is converted by the galactose-1-phosphate uridyl transferase enzyme to uridine
diphosphate (UDP)-galactose. These conversions enable galactose to enter the
glycolytic pathway of the cell. If the transferase enzyme is missing, as it is
in
“classic” galactosemia, galactose-1-phosphate accumulates in the tissues, and signs and
symptoms of the disease become evident. Classic galactosemia is caused by a
mutation in the
GALT gene, which codes for the galactose-1-phosphate uridyl transferase enzyme. In
addition, there are two other types of
“nonclassic” galactosemia. Galactokinase deficiency, a deficiency of the enzyme necessary
for the phosphorylation of galactose, can cause jaundice, cataracts, and
elevations of plasma galactose levels but does not result in mental deficiency.
A second and still rarer type of galactosemia is caused by uridyl
diphosphogalactose 4-epimerase deficiency. This condition behaves very much
like classic galactosemia.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Jaundice - Case 15-3: 2-Month-Old Boy:
III. Incidence and Epidemiology
(Pediatric Complaints and Diagnostic Dilemmas)
Congenital hypothyroidism has been estimated to occur in about 1 of every 4,000
newborns, and about one third of these infants demonstrate prolonged
hyperbilirubinemia. It appears that T4 is necessary to the bilirubin
conjugating process. The baby in this case had hypothyroidism and protracted
unconjugated bilirubinemia as dominant features of an even rarer
endocrinopathy, congenital hypopituitarism. Of note, the hyperbilirubinemia
seen in panhypopituitarism can also be
cholestatic, particularly when growth hormone or corticotropin (ACTH) deficiencies dominate
the pathophysiology.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Jaundice - Case 15-4: 6-Week-Old Girl:
III. Incidence and Etiology
(Pediatric Complaints and Diagnostic Dilemmas)
Biliary atresia occurs in about 1 in every 10,000 to 15,000 infants worldwide.
The disease is characterized by postinflammatory obliteration of some or all of
the extrahepatic biliary ducts. The extent of biliary tree involvement varies.
If the disease is limited to the distal segment, surgical correction may be
possible. Far more common, however, is diffuse involvement of the extrahepatic
biliary ducts, for which hepatic portoenterostomy (the Kasai procedure) or
liver transplantation is required.
The etiology of EHBA remains a mystery. It is presumably caused by an insult,
perhaps viral or ischemic, to the developing biliary tree. Not even the timing
of the disease onset is clear. Some children with biliary atresia are born with
other true congenital anomalies (e.g. malrotation, polysplenia, heart defects).
On the other hand, most infants with EHBA have no other malformations and are
clinically well until several weeks of age, suggesting a progressive, acquired
process with relatively late onset. Likewise, the range of histopathologic
findings seen in biliary atresia is heterogeneous. Therefore, it seems likely
that multiple etiologies of biliary atresia exist.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Pallor - Case 10-1: 3-Week-Old Boy:
III. Incidence and Epidemiology
(Pediatric Complaints and Diagnostic Dilemmas)
The precise incidence of Diamond-Blackfan anemia is not known. For all the RBC
aplasias, it is estimated that there are 300 to 1,000 new cases annually in the
United States. Diamond-Blackfan anemia occurs primarily in infancy. In some
studies, 10% of patients are anemic at birth, 25% by 1 month, 50% by 3 months,
and 70% by 1 year. This anemia is seen in all ethnic groups, but primarily in
Caucasians. There is no gender predominance.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Pallor - Case 10-2: 12-Month-Old Girl:
III. Incidence and Epidemiology
(Pediatric Complaints and Diagnostic Dilemmas)
In developed countries, routine iron fortification of formulas and cereals has
led to a significant decrease in early childhood anemia. However, iron
deficiency remains a leading cause of anemia. Currently, the prevalence of iron
deficiency is approximately 7%. One third of affected children develop anemia.
Children living below the poverty level are at greatest risk. More worrisome is
the increasing recognition of anemia as only one manifestation of iron
deficiency. Even in the absence of anemia, children with iron deficiency may
have neurocognitive and behavioral problems. Only some of these problems are
reversible with iron supplementation, suggesting the importance of prevention.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Pallor - Case 10-3: 5-Month-Old Boy:
III. Incidence and Epidemiology
(Pediatric Complaints and Diagnostic Dilemmas)
AHA occurs as the result of binding of antibody, antibody and complement
complex, or complement to the RBC. The resulting immunologic reaction destroys
RBCs and causes anemia. Infectious agents, drugs, and other agents may
stimulate the process. Some autoimmune diseases, such as systemic lupus
erythematosus, may also generate anti-RBC antibodies and RBC destruction. The
true incidence of AHA is unknown, but is estimated to be 1 to 3 cases per
100,000 population per year. Acute AHA usually manifests in the first 4 years
of life.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Rash - Case 9-2: 7-Week-Old Girl:
III. Incidence and Epidemiology
(Pediatric Complaints and Diagnostic Dilemmas)
Child abuse is an all too common diagnosis. Soft tissue trauma or skin injuries
such as bruising are frequently the earliest and most common manifestation of
physical maltreatment. A number of studies have shown that many seriously
injured children had been evaluated previously for bruises or burns, just as in
this case. Johnson and Showers showed in an epidemiologic study of injury
variables that children with evidence of chronic maltreatment, such as these
bruises, are at a 50% risk for further abuse and at a 10% risk for fatal
injury.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
About prevalence and incidence statistics:
The term 'prevalence' of Bullous Pemphigoid usually refers to the estimated population
of people who are managing Bullous Pemphigoid at any given time.
The term 'incidence' of Bullous Pemphigoid refers to the annual diagnosis rate,
or the number of new cases of Bullous Pemphigoid diagnosed each year.
Hence, these two statistics types can differ:
a short-lived disease like flu can have high annual incidence but low prevalence,
but a life-long disease like diabetes has a low annual incidence but high prevalence.
For more information see about prevalence and incidence statistics.
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