NEPHROTIC SYNDROME
Posted by Maria Ahmad on April 14th, 2012
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GENERAL CONSIDERATIONS
A combination of proteinuria, hypoalbuninemia, oedema and hyperlipidemia is termed nephrotic syndrome. The hallmark of nephrotic syndrome is massive proteinuria (> 1 g/m2/day). It is the commonest glomerular disease and is 15 times more common in children than in adults. Most cases occur between 1-5 years of age. Boys outnumber girls by 2-3 times at this age. There is no clear familial incidence.
Ninety percent cases in children are primary – without a discernible cause and about 10% secondary.
PATHOPHYSIOLOGY
Proteinuria: Occurs due to an increased permeability of the glomerular basement membrane. In the MCNS variety of primary nephrosis proteins of low molecular weight (70,000) are filtered out only (e.g. albumin). In other varieties larger sized molecules are also passed. The selectivity of proteinuria is helpful in suspecting the type of underlying renal pathology.
Hypoproteinemia
Low serum level of protein is the result of its reduced synthesis by liver, its loss in urine, and its catabolism. Liver responds to hypoalbuminemia by increased production of other proteins (gamma globulin is not produced by liver). Thus globulins like beta lipoproteins, are produced in excess. Serum cholesterol rises due to increased lipoprotein level.
Edema
Due to low serum albumin the intravascular colloidal pressure falls and oedema develops. There is also an increase of aldosterone and antidiuretic hormones resulting in retention of salt and water.
PATHOLOGY
Following histological types are seen on examination of renal biopsy with light, immunofluorescent and electron microscopy;
Minimal change Nephrotic Syndrome (MCNS)
This type fails to show any significant abnormality on light microscopy. No deposit of immune substances is detected by immunofluorescent microscopy. On electron microscopy fusion of foot processes of the epithelial cells is noted. This is the commonest type in children.
CHILD ABUSE
Posted by Maria Ahmad on April 13th, 2012
There is no universal definition of child abuse and the concept varies from country to country and society to society. Every child, despite his individual differences and uniqueness is to be considered of equal civil, and political rights, so that he may fully realize his inherent potential and share equally in life, liberty, and happiness. In accordance with these value premises then, “any act of commission or omission by individuals, institutions, or society as a whole, and any conditions resulting from such acts or inaction, which deprive children of equal rights and liberties, and/or interfere with their optimal development, constitute by definition, abusive or neglectful acts or conditions.”TYPE OF CHILD ABUSE
- Physical
- Sexual
- Emotional
PHYSICAL ABUSE
Physical abuse consists of any non accidental form of injury or harm, inflicted on a child by a person having charge of that child. Physical abuse includes beating, burning or wounding. The use of closed fist or instrument, kicking, throwing or shaking a child constitutes physical abuse, whether or no that incident caused internal injuries or eye damage. Corporal punishment which injures the child to the point where medical attention is required is outside the limits of normal corrective discipline. Such injuries could occur in an institutional setting as well as in the home.
INDICATORS OF PHYSICAL ABUSE
It is sometimes very difficult to determine which children brought to the emergency department of a hospital are victims of accidents and which children are, in fact, victims of physical abuse. Some physical indicators are listed below:
a) Physical Indicators
These include multiple injuries on the same child, injuries in various stages of healing, bruises on the face of an infant, a history that does not match the nature of the injuries sustained (eg) if fall was from a height of only two or three feet and child sustained long bone or skull fractures from that fall.
b) Skin Injuries
Bruises: If the bruised area shows marks which are identifiable as strap marks (linear or loop), human bite marks, hand marks, fingernail marks or marks which appear to have been made by an instrument they should be considered as inflicted bruises. The areas of the body most likely to be cause for suspicion of inflicted bruising are shoulders, lower back, buttocks, genitals, and inner thighs, cheeks, ear lobes, upper lip, frenulum, soft tissue of month, neck and abdomen.
Burns: The shape of the burns as well as the area of the body where the child was burned, will sometime suggest how the injury was caused. Most of the following categories should raise serious suspicion of physical abuse.
Immersion B urns: Symmetrical distribution on extremities, uniform burn of exposed area, absence of satellite burns, sparing of skin in creases.
Contact Burns: Hot object placed on skin, shape of burn suggests object.
Cigarette Burns: Round shape frequently on hands, arms or genital area.
c) Eye Injuries
Retinal Hemorrhage. This is often caused by shaking infants vigorously.
d) Head Injuries
Any head injury to a child should raise suspicion of possible child abuse including subdural haematoma and absence of hair in patches due to polling.
e) Bone Injuries
- Any of the following bone injuries should raise suspicion of physical abuse;
- Chip fractures of metaphysics
- Unusual fracture (ribs, scapula, sternum)
- Bucket handle fractures of metaphysics
- Repeated fracture to some site
- Fractures at different stages of healing
- Subperiosteal bleeding and calcification
- Dislocation of the hip and shoulder joints
- Spiral fractures
- Periosteal stripping
f) Whiplash, Shaken Infant Syndrome
This occurs when an infant is severely shaken. Symptoms include retinal hemorrhage and subdural hematomas with little or no external signs of injury.
PROBLEMS OF PRE-SCHOOL CHILDREN
Posted by Maria Ahmad on April 12th, 2012
Pre-school years (upto 5 years) are very important in the development of children as they are learning several kinds of social behavior and also reflect factors in the family and environment. Difficulties may very as the child passes through different stages of adaptation and adjustment within the family and thus is unlike those of older children where difficulties often involve experiences outside the home as well.The most common problems of this age group are bed wetting (44% for boys and 11% for girls), food fads (13%); difficulty in setting at night (13%), fears (12%) and soiling (125). Poor concentration (5%), severe tempers (5%), unhappiness (3%) and excessive worrying (2%) were all much less common. As different cultures are associated with different ways of bringing up children, marked differences have been found in the prevalence of psychiatric disorders among young children in different countries.
Some follow up studies have shown evidence of continuity in the type of disorders seen during early years of life.
Most of the problems of pre-school children are short lived and carry good prognosis. Poor outcome is however associated for those who come from broken homes, are placed in residential care and where marital relations of parents are not good.
The causes are mostly associated with family conflicts, poor marital relations of parents, rivalry with siblings and inadequate parental behavior.
The treatment includes day therapy, counselling for parents, firm but kind guidance for children and arrangements for the child to spend more time in play groups and play activities.
PSYCHIATRIC DISORDERS IN ADOLESCENCE
The adolescence period is defined as the developmental phase between childhood and adulthood. The common changes which take place during this period have major impacts on psycho-social developments. Attainment of physical growth, development of sexual activity, autonomy from the parents, ability to suppress impulses, and desires for close and sustained relationships are some of the components of maturity which may have long lasting effects on individual personalities. Although there are no specific psychiatric disorders of adolescence, certain experiences at this time often particularly merit treatment and advice from the experts.
The prevalence of these psychiatric disorders are only a little more common than in the middle years of the childhood, but the pattern is markedly closer to that of adulthood.
On the basis of these studies, a prevalence rate between 15-20% has been found for diagnosable psychiatric disorders at this age. During adolescence an increase is usually found for depression, schizophrenia, anorexia nerve’s, suicide, drug dependence and phobic disorders, whereas enuresis, encopresis, and over-activity disorders usually become less common.
GLYCOGEN STORAGE DISEASE
Posted by Maria Ahmad on April 9th, 2012
ETIOLOGY
Also termed “Glycogenosis” these disorder are characterized by;
Storage of glycogen in various organs. There is incomplete degradation of glycogen due to deficiency of various enzymes which break down glycogen at various stages of its metabolism. Various precursors/degradation products are formed and stored at various sites and organs in the body. To date at least 11 different types have been described and this number is likely to increase. Most types are inherited.
CLINICAL PRESENTATION
This varies according to the organs involved. Enlarged abdomen, enlarged liver heart, growth retardation, hypoglycemic episodes may be present. Three subdivisions can be made on this basis:
1. Glycogen storage diseases of the liver
Symptoms and signs may be present at birth or appear in early infancy with protuberant abdomen, hepatomegaly, stunted growth and a typical doll faces. Since glycogen cannot be converted into glucose by the liver readily, symptoms of hypoglycemia like irritability, convulsions or even coma may be the presenting symptoms in some patients.
Mildly affected patients reach adolescence and subsequently symptoms tend to abate. Others die early as a result of hypoglycemia or lactic acidosis
Children also present with hepatomegaly at a later age, but symptoms of hypoglycemia are mild or absent. These children reach adulthood and some may have normal life span.
2. Glycogen storage disease affecting heart muscle
(Pompe’s) disease: There is abnormal collection of glycogen in the cardiac muscle. The disease presents at birth or in early infancy with symptoms and signs of congestive heart failure, cardiomegaly, cyanosis or shock. Usually no murmur is heard. Heart is grossly enlarged on chest X-ray. Death is almost inevitable in the first year of life.
3. Glycogen storage disease affecting skeletal muscles
Patients present during adult life or childhood with hypotonia, muscle cramps, muscle pain, easy fatiguability and occasionally myoglobinuria. The disease varies in severity. CNS may be involved in some cases.
DIAGNOSIS
- Low blood glucose and pre diabetic type glucose tolerance curve.
- I.V. glucose tolerance test is abnormal in Type-1.
- Glucagon stimulation test shows poor response.
- Abnormal liver function tests.
- Low blood pH. Raised blood lactic acid and Keaton bodies in blood and urine may be present.
- Phospholipids and cholestrol may be raised.
- Amino-acid-uria, phosphaturia and glycosuria, may be present. Liver biopsy – may show ballooned hepatocytes (due to the accumulation of glycogen).
- Glycogen content in the affected tissues is raised.
- Specific enzyme defects may be detected in the liver.
- CPK may be raised in the varieties affecting skeletal muscle.
IDENTITY DISORDER ADOLESCENT CRISIS
Posted by Maria Ahmad on April 9th, 2012
IDENTITY DISORDER ADOLESCENT CRISIS
The conceptual boundaries of identity disorder or adolescent crisis are very vague and psychiatrists differ in their opinions about the diagnostic issues in this regard. These conditions are usually defined as severe subjective distresses over an inability to reconcile to the new changes of life with marked role confusions. The external events such as family crisis, social disabilities, personal problems and serious alienation from norms of the society are all precipitating factors and may cause numerous setbacks. These disturbances are manifested by uncertainty about a variety of issues, like career choice, friendship patterns, religious identification, sexual orientation, behavior, values and loyalties.
The presentation of most of these cases may be in the form of either emotional disorders or conduct disorders and at times frank psychotic states are also observed. The diagnosis is not valid if the reaction is symptomatic of another mental disorder. The associated features frequently found in identity disorder include a marked discrepancy between the person’s view of himself and the view that others have of him; moderate anxiety and depression; self doubts; impulsive experimentation’s and aggressive out bursts.
The clinical features are characterized by inability to make decisions, a sense of isolation and inner emptiness, difficulty to achieve relationships and sexual intimacy and assumption of a negative identity.
The main etiological factors for these disorders underlines the conflicts and disturbances in interpersonal and intrapersonal relationships. The onset may be acute but there is usually evidence of a gradual onset of this disorder in most of these cases. The course is usually brief but an extensive propagation of this disorder may also be found in certain individuals.
Therapeutic efforts in identity disorder are directed towards encouraging growth and development. The use of drugs is not commonly recommended and treatment is mostly directed towards supportive psychotherapy.
LYSOSOMAL DISEASES
Posted by Maria Ahmad on April 6th, 2012
GENERAL CONSIDERATIONS
Lysosomes are cellular organelles which break down complex macromolecules by the action of certain enzymes. If these enzymes are absent these macromolecules or their incomplete break down products accumulate in the tissues which break down and produce characteristic clinical features. These “storage diseases” are mucopolysaccharidoses, lipidoses or mucolipidoses depending on what the stored material is. Only the first two groups are discussed below.
LIPIDOSES
GENERAL CONSIDERATIONS
There are three main types. These are autosomal recessive. Some cases maybe autosomal dominant. Jews are commonly affected.
- Gaucher’s disease.
- Niemann- Pick’s disease.
- Tay Sachs disease.
In addition thirteen other rare forms have been identified. These are differentiated according to the type of abnormal lipid which is deposited in each type. The characteristic features of these disease is the formation of foam cell histiocytes with collection of intracytoplasmmic lipid in various body tissues and organs.
GAUCHERS DISEASE
Infantile Form
The activity of enzyme glucocerebrosidase is low and glucocerebroside accumulatef in the tissues. It presents in the first year of life. Progressive hepatosplenonegaly, delayed motor and mental milestones, strabismus, neck rigidity and bulbar paralysis develop. Most infants die under the age of one year.
Adult Form
Usually presents in the second decade. There is progressive enlargement of the liver. Massive splenomegaly may lead to hypersplenism and its complications. Bone pains and joint swellings leading to disability and fractures may be present. There may be delayed neurological development.
Juvenile Form (Subacute neuropathic)
This has features both of adult and infantile forms. Initially there is hepatosplemomegaly and later progressive neurological symptoms set in. There are behavioral changes, apraxia, extra-pyramidal and cerebellar signs, seizures and developmental regression are common. Many live in to early adulthood.
EMOTIONAL ABUSE
Posted by Maria Ahmad on April 5th, 2012
Emotional or mental abuse is probably the most difficult type of child abuse to define and to identify. It leads to psychological as opposed to physical harm of children. It would include a parent or care giver’s constant and continual overt rejection and criticism of a child or open disowning of a child. There is a component of emotional abuse co-existing in most child abuse cases.INDICATORS OF EMOTIONAL ABUSE
It is unlikely that the emotionally abused child will be brought to the hospital with emotional abuse as his/her chief complaint. The most likely reasons for these children to come to the hospital’s attention would include suicide threats and attempts, behavior problems, psychosomatic complaints and failure to thrive. It is very difficult to intervene in a case of emotional abuse without very careful documentation of parent or care giver’s behavior and the consequent damage to the child. Behavioral indicators for both children and parents are therefore listed below;
a) The Child: The types of symptoms exhibited by emotionally abused children may include
- Anxiety
- Bizarre behavior
- Sleeping problems
- Suicide threats and/or attempts
- Depressed/withdrawn behavior
- Bed-wetting
- Aggressive/defiant/demanding behavior
- Fecal incontinence
- Development lag
- facial tics
- Poor social relationships
- School failure
- Attention seeking behavior
- Low self-esteem
- Overly compliant and apologetic behavior.
b) The Parents
(S): Some of the following characteristics may be found in parents who emotionally abuse their children;
- Blames or belittles child at home or in public place
- Withholds comforting when child is really frightened or distressed
- Is over-critical of child on regular basis
- Describes child in negative terms
- Identifies child with disliked or hated relative
- Usually assumes child is at fault
- Openly admits dislike or hate for the child.
FAILURE TO PROVIDE OR CHILD NEGLECT
Failure to provide consists of omission on the part of the parent or care giver to the basic needs such as food, clothing and shelter to a child. It also includes lack of adequate parental supervision to prevent injury, abandonment and provide medical attention when required. Also included is the category of “failure to thrive”, whereby a child can fail to get adequate nourishment for a variety of reasons e.g. it may be determined that the child has organic problems or non-organic problems or the cause of “failure to thrive” may remain undetermined.
NUTRITION COUNSELLING
Posted by Maria Ahmad on March 22nd, 2012
This involves:-
a) Optimum Breast-feeding (Lactation Management).
b) Correct Weaning.
c) Feeding during Illness.
LACTATION MANAGEMENT
Part of the Nutrition strategy for children is the promotion of breast-feeding. In Pakistan over 90% of mothers breast-feed their young initially. It is the culturally accepted mode of feeding. But on doing a National newborns only breast-milk. There were certain
practices which interfered lacteal feeds (feeds given before putting the baby to mothers breast after birth).
These include honey, water and ghutti (a honey and vegetable mixture). Sometimes harmful prelates like laxatives are also used to clean the intestines of the newborn. Other practices like giving mixed feeding (water or other milk) in post to breast feeding is also very common. All of these interfere with “optimum breast feeding” and can lead to lactation failure. Since this involves using bottles to feed babies, the resulting contamination causes diarrhea.
Socioeconomically poor mothers usually over-dilute milk when feeding their babies, thus resulting in decreased caloric intake and malnutrition.
Other harmful practices such as delaying weaning foods beyond 6 mothers of age, mothers taking restricted diets during lactation (due to food taboos of hot and cold foods affecting the baby), discontinuing breast-feeding before child is 2 years of age, also contribute to malnutrition and increased infections in the infant.
Considering these problems a Programmed by the name of “baby Friendly Hospital Initiative” was launched by UNICEF and Government of Pakistan, in 1992. The major objectives of this Programmed are to improve optimum breast-feeding practices of mothers in Hospitals and other health facilities. It is hoped that by doing so health workers in the community will also adopt correct practices.
PSYCHOSES OF CHILDHOOD
Posted by Maria Ahmad on March 22nd, 2012
Infantile autism, dis-integrative psychosis and schizophrenia are included in this group of disorders.INFANTILE AUTISM
Infantile autism is the most common form of psychoses in children with a prevalence of 0.02 percent and about 3 fold male predominance. It was at one time thought that autism was caused by insufficient maternal warmth and stimulation in infancy. Marked differences have still been observed between parents of autistic and of control subjects suggesting that social relationship anomalies (impaired rapport, lack of empathy, suspiciousness and low emotional responsiveness) are usually present in these parents.
Etiology of this disorder is however not clear. An organic basis is suggested by the presence of neurological abnormalities, development of spasms and encephalitis. Perinatal complications are also noted in many cases. Twin studies also indicate a genetic propensity. New imaging techniques (like CT scanning and MRI) have also suggested selective sub-cortical structural changes in particular, brain stem involvement in many cases.
The onset is usually within two and half years of and age and the common classical features include autistic aloofness (aloof, poor gaze contact, no persistent friendship and indifference to social conventions) abnormalities of language (absence or delayed, immature and poor comprehension) and obsessive desires for sameness. Fears, abnormal movements, self destructive behavior and low intelligence are additional abnormalities. Most autistic children are of limited intelligence (about 75% have I.Q. under 70) and
I.Q. also serves the most important prognostic guide. Although no specific treatment is available, various approaches like educational placement, behavior modifications and supportive treatment are helpful in autistic children.
Parents of these autistic children require long term support in understanding and caring of these patients. In certain cases long term residential placement in a hostel or special community may need to be considered.
Medication is only helpful in the control of over-activity and aggressive outbursts. The findings of high blood serotonin levels and abnormalities in the endogenous opioid systems have shown the hopes for the efficacy of drugs like Fenfluramine and Naltrazone.
Autistic children do not develop into adult schizopharenics, but they may become autistic adults.
About 40 percent of children with this condition improve to a major extent and the remainder improve little or are unable to lead an independent life.
SCHIZOPHRENIA
Posted by Maria Ahmad on March 22nd, 2012
The overall prognosis is therefore poor, particularly if no speech is acquired by the age of five and if I.Q. remains below average.It seldom begins before late adolescence. The symptoms are almost the same as seen in adult schizophrenia and diagnosis is usually difficult in the early stages.
DIS-INTEGRATIVE PSYCHOSES
Dis-integrated psychoses are disorders where normal or near normal development in the first few years is followed by loss of social skills and speech along with severe disorder of emotion and behavior. Most cases are progressive and are left with mental retardation. There is uncertainty about the extent to which this condition differs from autism. In some cases the disorder can be shown to be due to some associated encephalopathy but the diagnosis should be made on the behavioral features.
SPECIFIC DELAYS IN DEVELOPMENT
These disorders are characterized by inadequate development of specific academic, language, speech and motor skills, and are not due to any demonstrable physical or neurological illnesses. These disorders include specific arithmetic disorders, specific reading retardation (dyslexia) and specific articulation disorders. These disorders are associated with impairment in academic functioning and usually represent the disturbance at functional levels.
Specific reading disorders show significant impairment in the development of reading skills which are not solely accounted for by mental age, visual problems or inadequate schooling.
The dyslectic children, generally, show an excess of boys, developmental speech delay in infancy, poor right/left differentiation, lower verbal than performance I.Q. and usually come from a large family. The etiology is unknown but a strong case has been advocated for genetic, neurological and social factors, the general prognosis is not bad but varies with the severity of the condition.
